Growth of bone originates from the epiphyseal plate, and damage to this structure can result in unequal lengths of the extremities. Surgical intervention involving the epiphysis on the affected extremity or the epiphyseal line on the opposite extremity is the usual treatment.
Bone healing and callus formation can span and repair only a limited space between bone fragments. When bone fragments cannot be maintained in correct alignment for repair due to inadequate reduction, poor immobilization, or a damaged or softened cast, bone healing is impaired. The factors most likely to interfere with bone healing and cause delayed union or nonunion, based on the physiologic needs for bone healing, are listed in Box 39-9.
The hematoma, which becomes the matrix for bone deposition in the break, must be free of infection or bits of adipose or connective tissue. The constant supply of nutrients and bone-forming cells brought to the area by way of the bloodstream provides the vital ingredients for repair.
Sometimes artificial means are employed to facilitate bone healing. Bone grafting becomes necessary when bone nonunion occurs. The donor site is usually the tibia or the iliac crest. Bleeding of bone ends may need to be artificially stimulated, and at times holes are drilled near the bone ends in an attempt to increase circulation. Postsurgical immobilization of the recipient area is crucial to the success of the graft. The Ilizarov external fixator and protocol for care are frequently used to assist bone healing in patients with nonunion (see p. 1650).
Malunion is fracture union with increased angulation or deformity at the fracture site. It can be detected at any stage in the healing process or after complete healing. Unsatisfactory reduction is the usual reason for malunion. A cast or splint that allows fracture movement will also likely result in malunion. Periodic radiographic examinations help detect this complication and prevent it from becoming a major problem over a long period.
Excessive deformity can be corrected during the healing process through realignment and reimmobilization. However, attempts at correction may cause delayed union or nonunion; therefore the degree of deformity is carefully evaluated in light of these complications. The probability that sufficient spontaneous alignment will occur with growth and continuation of the healing process also is considered. Correction of the malunion when healing is near completion requires surgical intervention.
Osteomyelitis, infection of the bone, is often secondary to a bloodstream infection but is a potential problem when the fracture is an open one, when pressure ulcers develop, or when bone surgery has been performed. Any bacterial organism can cause this infectious process; however, Staphylococcus aureus is the pathogen most frequently identified (see p. 1673 for a discussion of osteomyelitis).
Although uncommon in children, development of renal calculi is a potential risk whenever the child has a limb that is non–weight bearing for a long time, especially if the circumstances also produce urinary stasis. Measures to prevent the formation of renal calculi include maintaining optimal hydration, mobilizing the child as much as possible, and checking closely the amount and characteristics of urinary output. Any urinary tract infection should be treated promptly with appropriate antimicrobials and urine acidification, since the nucleus of a calculus is often composed of bacterial debris or calcium and the buildup of stone is precipitated by alkaline urine. An associated problem, hypercalcemia, was reviewed in the section on problems of the immobilized child.
Blood, air, or fat emboli can be a hazard to the child with a fracture. As postinjury bleeding and clotting occur, a small piece of the clot can travel to vital organs, such as the lung, heart, or brain, and produce a life-threatening vascular obstruction and ischemia. Generally the pulmonary system is the most frequent site of emboli deposition, but it may not occur until 6 to 8 weeks after the injury.
Fat emboli are the greatest threat in an individual with multiple fractures, particularly fractures of the long bones such as the femur. Fat droplets from the marrow are transferred to the general circulation by the venous-arterial route, where they can be transported to the lung or brain. This type of embolism occurs within the first 24 hours, generally in the second 12 hours after the injury occurs. Adolescents are those usually affected in the pediatric age-groups.
Intermittent compression devices are used to prevent venous pooling in the lower extremities when prolonged immobilization is required. These devices are inflatable sleeves that allow cyclic emptying and filling of leg veins; the devices are used in children with spinal cord injury once mobilization is initiated to decrease the effects of orthostatic intolerance. Anticoagulant drug therapy, passive and active range of motion, and early mobilization are also used to decrease venous stasis and prevent thrombus development.
A child may be born with the congenital absence of a body part, experience a traumatic loss of an extremity, or require a surgical amputation for a pathologic condition such as osteogenic sarcoma. With today’s surgical technology and the quick thinking of bystanders who save a traumatically amputated body part, some children have had fingers and arms sewn back on with variable degrees of functional use regained. A severed part should be rinsed in saline; wrapped gently but completely in sterile gauze; and placed in a watertight bag labeled with the child’s name, the date, and the time. The bag should then be placed in ice water to keep the limb chilled but not frozen. The part should not be packed in ice, since damage to the tissue may make reimplantation impossible.
Surgical amputation or the surgical repair of a permanently severed limb focuses on constructing an adequately nourished stump. For lower extremities the presence of a smooth, healthy, padded stump, free of nerve endings, is important for prosthesis fitting and subsequent ambulation. In some situations in which there is no vascular or neurologic deficit, a cast is applied to the stump immediately after the procedure, and a pylon, metal extension, and artificial foot are attached so that the patient can walk on the temporary prosthesis within a few hours.
Lower extremity stump shaping is done postoperatively with special elastic bandaging using a figure-8 compression bandage, which applies pressure in a conical fashion. This technique decreases stump edema, controls hemorrhage, and aids in developing desired contours so that the child will bear weight on the posterior aspect of the skin flap rather than on the end of the stump. When appropriate, a stump shrinker, in addition to an elastic wrap, may be used. Some surgeons apply a prosthetic device in surgery after the amputation to reduce edema and promote early ambulation.
The stump may be elevated for the first 24 hours, but after this time the extremity should not be left in this position because contractures in the proximal joint will develop and seriously hamper ambulation. Monitoring proper body alignment further decreases the risk of flexion contractures. Postoperatively, children who undergo amputation of a lower extremity should be turned not only from side to side but also from front to back. Postoperative complications for which the nurse should be vigilant include hemorrhage and infection of the operative site. As the child progresses, encourage him or her to lie prone at least three times a day, increasing the time prone to tolerance of an hour at a time.
For older children and adolescents, arm exercises, bed pushups, and exercises with parallel bars (which are used in prosthesis training programs) help to build up the arm muscles necessary for walking with crutches. An overhead trapeze bar enhances mobilization and upper body strength building in the early postoperative period when a lower limb has been amputated. Full range-of-motion exercises of joints above the amputation must be performed several times daily, using active and isotonic exercises. Young children are spontaneously active and require little encouragement.
Depending on the child’s age, the child or parents need to learn stump care, including careful washing with soap and water every day and checking for skin irritation, breakdown, or infection. A tube of stockinette or talcum powder is used to make the prosthesis slide on more easily. A careful skin check must be performed every time the prosthesis is removed, and prosthesis tolerance time must be adjusted to prevent skin breakdown.
Evaluate limb pain, especially pain that increases with ambulation, to check for a possible neuroma at the free nerve endings in the stump or a poorly fitting prosthesis. Chronic pain may also be related to weakness or joint instability, injury to the nerve, or fibrosis of soft tissues. Pain in the contralateral extremity may result from asymmetric weight bearing.
For children who have had an amputation, phantom limb pain is an expected experience because the nerve-brain connections are still present. Phantom pain is real pain and should be treated appropriately with analgesics and other pain-relieving measures. Gradually these sensations fade, although in many amputees they persist for years. Preoperative discussion of this phenomenon helps a child understand these feelings and not hide the experience from others.
Adolescents probably spend more time and energy practicing and participating in sports activities than members of any other age-group. The practice of sports and games contributes significantly to growth and development, to the education process, and to good health. It provides exercise for growing muscles, interactions with peers, and a socially acceptable means of enjoying stimulation and conflict. In addition, competitive activities help the older child and adolescent engage in self-appraisal and develop self-respect and concern for others.
Every sport has some potential for injury to the participant—whether the young person participates in serious competition or purely for enjoyment. Serious injury is not limited to the athlete who competes in rough contact sports; a large number of severe or fatal injuries occur to persons who engage in milder physical activity but are not physically prepared for it. For example, a person’s body build may not be suited to the sport, muscles and support systems (respiratory and cardiovascular) may not have been sufficiently conditioned to withstand the rigors of the physical stress, or the child or adolescent may not possess the insight and judgment to recognize when an activity is beyond his or her capabilities. Rapidly growing bones, muscles, joints, and tendons are especially vulnerable to unusual strain.
The awkward and inexperienced child or adolescent may suffer more injury than the more skilled and experienced one. Strong muscles are less easily damaged than weak ones and provide better protection to the joints they cross. Fatigue significantly impairs muscle function and judgment. More injuries occur during recreational sports participation than in organized athletic competition. Likewise, most injuries occur in practices rather than in games. Although team sports give rise to frequent injuries, serious injuries resulting from recreational and individual sports are generally more common. The increase in strength and vigor in adolescence may tempt children to overextend themselves. This is especially true of boys who are egged on by teammates or coaches or are stimulated by the admiration of female observers.
Not only does the activity itself pose a hazard of greater or lesser degree, but the environment and the sports or recreational equipment present additional risks. Adolescents participate in physical activities in a variety of environments, both indoors and outdoors, on floors, on the ground, on snow, on or beneath water surfaces, and sometimes in free air space. These activities frequently involve equipment that intensifies the risk.
Among adolescents of the same age, the degree of physical maturation varies greatly, and many of the physical characteristics important in sports are related to hormone production. Consequently, physical strength, coordination, endurance, and size vary considerably among children and adolescents who wish to compete against each other. Sports competition between young people who differ markedly in strength and agility is unfair and hazardous. Matching of candidates for sports should be based on physical maturity, height, weight, and physical fitness and skills, particularly in sports involving rigorous body contact. Age is a less important consideration.
The American Academy of Pediatrics (Rice and Council on Sports Medicine and Fitness, 2008) has developed a classification that categorizes sports according to probability of collision and strenuousness (Box 39-10). Collision sports such as tackle football, basketball, hockey, and soccer have the highest injury rates, followed by other contact sports (Figs. 39-29 and 39-30). In addition, the American Academy of Pediatrics (Rice and Council on Sports Medicine and Fitness, 2008) has developed and published guidelines that provide criteria for determining inclusion or exclusion of the young athlete based on common medical and surgical conditions and relative risks in various sports categories. This serves as a useful guideline for the health professional in counseling youth regarding sports activities.
Fig. 39-29 Football is an example of a strenuous collision sport with a high risk of serious injury.
Fig. 39-30 Gymnastics is an example of a strenuous limited contact, limited collision sport with a high risk of serious injury.
The American Academy of Pediatrics (2000a; Brenner and Council on Sports Medicine and Fitness, 2007) encourages sports participation by young persons and encourages adults close to sports activities to be aware of the early warning signs of fatigue, dehydration, and injury. Athletes should seek assistance when an injury is suspected and not “work through” injuries caused by overuse (shin splints, stress fractures, tendinitis, and apophysitis).
The role of health care professionals, specifically nurses, in relation to sports injuries focuses on prevention, treatment, and rehabilitation. Of these areas, prevention is perhaps the most important. It is difficult to “sell” prevention, however, especially to children. Anticipatory guidance is an important aspect of preventive counseling for sports injuries, since many injuries occur when children are tired and are not concentrating on their activities; injuries are also more common when participants are distracted by events off the field of play, such as not really wanting to play or thinking about personal problems.
Everyone wants to play the game, but not necessarily to practice. Often, if an 8- to 15-minute warm-up is suggested, adolescents and children warm up for 30 seconds and say they are ready to go. Youth who are actively involved in athletic programs need to undergo medical evaluation as a prerequisite to participation and to receive education in sports skills using correct training and conditioning methods. Tactics that are dangerous beyond the ordinary risk associated with the specific sport should be omitted. In addition, children should use appropriate protective equipment, properly maintained and suited to the individual. The sports environment should make maximum provision for safety and availability of first-aid and medical services.
The same protective principles apply to enthusiasts in noncompetitive sports. They need the same education in basic safety precautions, encouragement to acquire proper instruction in the skills required for performing the activity (such as water safety, skiing techniques), and proper maintenance of equipment.
The injuries sustained in sports or recreational activities can involve any part of the body and range from relatively minor cuts, bruises, and abrasions to severe closed head injuries such as concussion or totally incapacitating central nervous system injuries or death. Some of these injuries are discussed in chapters devoted to the major topic (e.g., spinal cord injuries [Chapter 40] and head injuries [Chapter 37]). Fractures are discussed earlier in this chapter.
Some sports are particularly dangerous for children and adolescents. Snowmobiling, snowboarding, use of all-terrain motor vehicles, in-line skating, skateboarding, motorcycle riding, full contact hockey, bicycle riding, wave running, and trampoline use are examples of sports and recreational activities that can lead to significant injuries in connection with inappropriate use, failure to wear protective equipment, or participation by children who are under age. According to the American Academy of Orthopaedic Surgeons (2007), sports injuries account for approximately 20% of visits to the emergency department for injury. Basketball and pedal cycling account for the majority of injuries, followed by football, baseball or softball, and skate boarding or in-line skating. One study reported a high incidence of injuries in high school football athletic competition; boys’ soccer had a high rate of head, neck, and face injuries; and high rates of concussion injuries occurred in boys’ soccer (Rechel, Yard, and Comstock, 2008). Ankle injuries are the most common competitive sports-related injuries, with significant numbers occurring in boys’ and girls’ basketball (Nelson, Collins, Yard, et al, 2007).
A variety of injuries can result when an external force is applied that causes severe stress on tissue, muscle, and skeletal structures (Fig. 39-31). The body structures attempt to absorb the force, but when they are unable to do so, injuries occur. Two general types of injury are recognized. The first is acute trauma, which is defined as a sudden, acute injury from a major force. Among such injuries are fractures of long bones and the axial skeleton; sprains of joint ligaments; strains of muscle tendon units; and contusions, including those of muscle tendon units and overlying soft tissue. The second type is repetitive overuse injuries, or microtrauma, which result from repetitive injury to tissue over a long period. Overuse injuries include stress fractures, bursitis, tendonitis, apophysitis of tendon insertions, and at times injuries of the joint surface.
More than 95% of sports injuries involve the soft tissues, not the bony skeleton. About two thirds of these consist of strains and sprains, and most injuries involve the extremities. Acute overload injuries are those that occur suddenly during an activity and produce immediate symptoms. They can be caused by a blow or overstretching, twisting, or any other forces that cause a sudden stress to tissues.
Contusions are probably the most common sports injuries and are often considered to be “part of the game.” A contusion is damage to the soft tissue, subcutaneous structures, and muscle. The tearing of these tissues and small blood vessels and the ensuing inflammatory response lead to hemorrhage, edema, and associated pain when the child or adolescent attempts to move the injured part. The escape of blood into the tissues is observed as ecchymosis, a black-and-blue discoloration.
The most serious contusions are those involving the quadriceps; they are common in strenuous, collision-type sports and usually result from being kicked or kneed in the thigh. Large contusions cause gross swelling, pain, and disability and usually receive immediate attention from health care personnel. The less spectacular smaller injuries may go unnoticed, so that continued participation is allowed. They can become disabling after rest, however, because of pain and muscle spasm. The young athlete is frequently instructed to “work it out” or disregard the pain. Myositis ossificans may occur from deep contusions to the biceps or quadriceps muscles; this condition may result in a restriction of flexibility of the affected limb.
Immediate treatment of a contusion consists of application of cold for no more than 20 to 30 minutes, as in the treatment of sprains described below. Return to participation is allowed when the strength and range of motion of the affected extremity are equal to those of the opposite extremity.
Although they are not always directly related to sports, crush injuries occur in children when they slam their fingers (in doors, folding chairs, or equipment) or hit their fingers (as when hammering a nail). A severe crush injury involves the bone, with swelling and bleeding beneath the nail (subungual) and sometimes laceration of the pulp of the distal phalanx. A subungual hematoma can be released by a special cautery device that “burns” a hole at the proximal end of the nail.
Long bones are held in approximation to one another at the joint by ligaments. Joints can be tight or loose, and loose joints are more likely to be dislocated. For certain sports (e.g., gymnastics and acrobatic dancing) the joints need to be limber. A tight joint is needed for sports such as football. One of the most vulnerable joints is the shoulder, which is structurally insecure, having only a rotator cuff to maintain the shoulder in place. The joint is shallow with relatively little muscle protection; therefore the capsule becomes stretched and the joint dislocates easily. There is a high incidence of shoulder injuries in male gymnasts and an even greater incidence of shoulder injuries in players of contact sports, such as football. Temporary restriction of the joint with a sling or bandage that secures the arm to the chest can provide sufficient comfort and immobilization until the individual receives medical attention.
Dislocations are less common in children than in older persons, but some types are specific to the younger age-groups. Before final closure of the epiphyses, injuries to the joints are more likely to cause epiphyseal separation than dislocation. For example, shoulder dislocation occurs most often in older adolescents, and dislocation unaccompanied by fracture is rare. Dislocations of the phalanges are the most common type seen in children, followed by elbow dislocations. Injury to the hip causes dislocation more frequently than femoral neck fracture (often experienced by persons in the older age-groups).
In children younger than 5 years of age, the hip can be dislocated by a fall. The greatest risk after this injury is the potential loss of blood supply to the head of the femur. Children with naturally lax joints, such as those with Down syndrome, are more prone to recurrent dislocation of the hip.
A dislocation occurs when the force of stress on the ligament is great enough to disrupt the normal position of the opposing bone ends or the bone end and its socket. The predominant symptom is pain that increases with attempted passive or active movement of the extremity. In dislocations there may be an obvious deformity and inability to move the joint. Temporary restriction of the joint with a sling or bandage that secures the arm to the chest in a shoulder dislocation provides sufficient comfort and immobilization until the child or adolescent can receive medical help.
In hip dislocation the best chance for prevention of damage to the head of the femur is to relocate the hip within 60 minutes after the injury occurs. As the length of time between injury and hip relocation increases, the risk of irreparable damage increases. Simple dislocations should be reduced as soon as possible with the child under mild sedation and often local anesthesia. Sedative agents such as midazolam or propofol (Diprivan), and analgesics such as ketamine or fentanyl, can be used to produce partial or complete analgesia during the reduction. Increased swelling, which makes reduction difficult and increases the risk of neurovascular problems, can complicate an unreduced dislocation. Treatment depends on the severity of the injury.
Dislocation of the patella occurs spontaneously in some children; in others it is a result of injury. It is common among adolescent girls. The patella is always dislocated laterally. Most dislocations are reduced either spontaneously or by a companion before the child is seen by a practitioner. Therapy is immobilization for 3 to 4 weeks. Surgery may be needed to treat recurrent dislocations.
The most common dislocation injury is subluxation or partial dislocation of the radial head in the elbow, also called pulled elbow or nursemaid’s elbow. In the vast majority of cases the injury occurs in a child between ages 1 and 3 years who receives a sudden longitudinal pull or traction at the wrist while the arm is fully extended and the forearm pronated. It usually occurs when an adult is holding the child by the hand or wrist and gives a sudden jerk to prevent a fall or attempts to lift the child by pulling the wrist, or the child suddenly pulls away by dropping to the floor (or ground). The child has an anxious expression, whines, complains of pain in the elbow and wrist, refuses to move the arm, and holds it with the opposite hand and in a slightly flexed and pronated position against his or her body.
The practitioner manipulates the arm by applying firm finger pressure to the head of the radius and then supinates and flexes the forearm to return the bone structures to normal alignment. A click or clunk may be heard or felt, and functional use of the arm returns within minutes. Immobilization of the arm is not necessary (Cornwall, 2007). The longer the subluxation is present, however, the longer it takes for the child to recover mobility after treatment. A radiograph may be needed if attempts to reduce the dislocation are not successful.
A sprain occurs when trauma to a joint is so severe that a ligament is either stretched or partially or completely torn by the force created as a joint is twisted or wrenched. This is often accompanied by damage to associated blood vessels, muscles, tendons, and nerves. As a guideline for management and prognosis, sprains are classified according to the degree of injury (Box 39-11). Because of the number of ligaments required to maintain knee stability, the knee is one of the joints most commonly injured in sports. It is also the largest joint and consequently is more prone to injury. Ankle sprains in children account for approximately 75% of all ankle injuries; these injuries are common in individuals who participate in sports, especially in the pediatric age-group (Chorley, 2005).
The presence of joint laxity is the most valid indicator of the severity of a sprain. With a severe injury the athlete complains that the joint “feels loose” or as if “something is coming apart” and may describe hearing a “snap,” “pop,” or “tearing.” Pain is seldom the principal subjective symptom. There is a rapid onset with swelling, often diffuse, accompanied by immediate disability and appreciable reluctance to use the injured joint.
A strain is a microscopic tear to the musculotendinous unit and has features in common with sprains. The area is painful to the touch and is swollen. The severity is evaluated as grade I, II, or III, as for sprains, except that the degree of laxity does not apply. Even with severe grade III injuries, complaints of laxity are rare. Most strains happen over time rather than suddenly, and the rapidity of the appearance provides clues regarding severity. In general the more rapidly the strain occurs, the more severe the injury. When the strain involves the muscular portion, there is more bleeding, often palpable soon after injury and before edema obscures the hematoma.
The first 6 to 12 hours is the most critical period for virtually all soft tissue injuries. Basic principles for managing sprains and other soft tissue injuries are summarized in the acronyms RICE and ICES:
The trend with ankle sprains is to encourage early mobilization and rehabilitation rather than prolonged immobilization and rest. The acronym PRICEMMS is used to describe measures to implement early mobilization and prevent chronic ankle instability and pain (Chorley, 2005):
P—Proprioception or balance exercises
R—Rest, alteration of activity, use of crutches, progressive weight bearing
M—(Range of) Motion exercises (active or passive)
S—Strengthening exercises, isometric or with movable resistance
Soft tissue injuries should be iced immediately. This is best accomplished using crushed ice wrapped in a towel or encased in a screw-top ice bag or plastic bag (e.g., a resealable storage bag). A wet elastic wrap is applied to provide compression and to keep the ice pack in place. A single layer of the wrap or cloth is placed over the injured area to protect the skin under the ice pack, and the remainder of the bandage secures the pack in place. The wet wrap transfers the cold better than a dry wrap. Athletic trainers often keep wet elastic wraps refrigerated for ready use.
There is still controversy over whether heat or ice should be used during the rehabilitative phase of management. Regardless of the method used, it is accompanied by appropriate exercise, depending on the severity of the injury, and carried out under the direction of a competent professional experienced in the care of sports injuries.
Ice has a rapid cooling effect on tissues that reduces pain and the magnitude of the stretch reflex by decreasing muscle spindle response, afferent nerve discharge, and the afferent loop response (monosynaptic reflex). Secondary effects are achieved by vasoconstriction, decrease in muscle nerve velocity, and increase in muscle viscosity. Also, the decreased temperature slows metabolism, which reduces tissue oxygen requirements. Edema formation is reduced when fewer histamine-like substances are released. Nine to 15 minutes of ice exposure produces deep-tissue vasodilation without increased metabolism. However, the effects last up to 7 hours. Ice therapy should be intermittent, and ice should never be applied for more than 30 minutes at a time to prevent tissue damage.
Elevating the extremity uses gravity to facilitate venous return and reduce edema formation in the damaged area (see Research Focus box). The point of injury must be kept several inches above the level of the heart for therapy to be effective (Fig. 39-32). Several pillows can be used effectively for elevation. Allowing the extremity to be dependent causes excessive fluid accumulation in the area of injury, which delays healing and causes painful swelling. Ivins (2006) recommends a 72-hour rest period for a sprained ankle to allow ligament healing.
Fig. 39-32 Correct and incorrect methods for elevating a lower extremity. A, Correct method: lower leg elevated on pillows; ankle above heart level. B, Incorrect positioning: ankle below level of heart.
Major sprains or tears to the ligamentous tissue rarely occur in growing children. Ligaments are stronger than bone, and the epiphysis and growth plate are the weakest areas of the bone; therefore the more usual site of injury is at the growth plate (see Fractures, p. 1636). Torn ligaments, especially those in the knee, are usually treated by immobilization with a knee immobilizer or range-of-motion brace until the child is able to walk without a limp. Crutches are used for mobility to rest the affected extremity. Passive leg exercises, gradually increased to active ones, begin as soon as sufficient healing has taken place. Caution parents and children against using any form of liniment or other heat-producing preparation before examination. If the injury requires casting or splinting, the heat generated in the enclosed space can produce extreme discomfort and may even cause tissue damage. In some cases torn knee ligaments are managed with arthroscopy and ligament repair or reconstruction as necessary, depending on the extent of the tear, the ligaments involved, and the child’s age. Surgical reconstruction of the anterior cruciate ligament may be performed in young athletes who wish to continue in active sports.
Postoperative mobilization of the affected joint is implemented immediately using a continuous passive motion device. This device provides passive range-of-motion exercise to the injured extremity and decreases postoperative complications related to restricted mobility. The patient often is ambulatory within hours on crutches and is discharged late on the day of surgery or the following day.
To excel in sports, the young athlete is forced to train longer, harder, and earlier in life than previously. The rewards are an increased level of fitness, better performance, faster times, and the satisfaction of attaining a personal goal. With the increase in the number of children participating in a wide variety of sports year-round, more overuse injuries are being seen in the pediatric age-group (Brenner and Council on Sports Medicine and Fitness, 2007).
The risk of overuse injury is always present and can be related to several factors: training errors, muscle-tendon imbalance, anatomic malalignment (e.g., femoral anteversion, excessive lumbar lordosis, tibial torsion), incorrect footwear or playing surface, an associated disease state, and growth (growth cartilage is less resistant to microtrauma). Athletes who run extensively frequently experience shin splints. The ligaments tear away from the tibial shaft, and this creates the pain. Ice, rest, and NSAIDs, such as ibuprofen or naproxen, are the usual treatment. Shin splints are rarely serious.
Chronic pain in athletes is often associated with overuse injury, which can occur at any level of athletic participation. The common feature in overuse injuries is the repetitive microtrauma that occurs to a particular anatomic structure (Brenner and Council on Sports Medicine and Fitness, 2007). Performing the same movements time and time again can cause several types of injury: (1) frictional, or rubbing of one structure against another; (2) tractional, or repeated pull on a ligament or tendon; and (3) cyclic, or repetitive loading of impact forces (stress fractures). The end result is inflammation of the involved structure with complaints of pain, tenderness, swelling, and disability.
Bursae, tendons, muscles, ligaments, joints, and bones are all subject to overuse. Table 39-4 outlines some of the common overuse syndromes. Plantar fasciitis is common in athletes, and Osgood-Schlatter disease often occurs in children who do a lot of jumping. The occurrence of overuse-type injuries, such as sore shoulders and strained elbows, may indicate that too much is being asked of the child in too short a period.
TABLE 39-4
DISORDER | CAUSE | MANIFESTATIONS |
Plantar fasciitis | Repetitive stretching of the plantar fascia (calcaneus to metatarsal heads) | Pain in arch or heel |
Achilles tendinitis | Repeated forcible traction on short tendon | Pain on palpation; pain with plantar flexion against resistance |
Sever disease | Epiphysitis of the calcaneus | Pain over insertion of Achilles tendon into tip of calcaneus |
Anterior leg pain (shin splints) | Irritation of posterior tibial muscle in unconditioned athlete or one not conditioned to a new sport | Pain in leg along anterior or medial edge of midshaft or distal third of tibia |
Osgood-Schlatter disease | Traction apophysitis of tibial tubercle | Pain and tenderness; overprominence of involved tubercle |
Sinding-Larsen-Johansson syndrome (jumper’s knee) | A variant of Osgood-Schlatter disease; traction apophysitis on inferior pole of patella | Same as above; pain slightly lower than in Osgood-Schlatter disease |
Patellofemoral syndromes | Malalignment of extensors, increased patellar compression, and increased training intensity | Chronic knee pain, especially following forced leg extension from flexion or after running |
Tennis elbow | Lateral epicondylitis from repetitive strain on elbow | Pain in elbow, aggravated by use |
Little League elbow | Osteochondritis of the capitellum; tendinitis of flexor-origin medial epicondyle from repetitive valgus strain to elbow from throwing | Pain in elbow that increases with activity |
Little League shoulder | Microfracture of proximal humeral growth plate from repetitive throwing | Pain and characteristic contracture; loss of internal rotation and increased external rotation |
Swimmer’s shoulder | Supraspinatus tendinitis from repetitive shoulder movement | Pain in shoulder that increases with activity |
Given the intensity and duration of sports training, many young athletes suffer stress fractures, especially after a recent increase in training regimens. These fractures occur as a result of repeated muscle contraction and occur most often in sports involving repetitive weight bearing such as running, gymnastics, and basketball. They occur less often in swimming (in the upper extremities). Tibial fractures are most common.
The most common symptom of stress fracture is a sharp, persistent, progressive pain or a deep, persistent dull ache located over the bone. Sometimes there is pain on impact (heel strike), but the most important clinical sign is pain over the involved bony surface. Diagnosis is based on clinical observation. Plain radiographs are rarely diagnostic of stress fractures during the initial few weeks, since callus formation is not yet evident. Occasionally a bone scan will indicate a “hot spot.”
Inflammation is common to all overuse syndromes; therefore management involves rest or alteration of activities, physical therapies, and medication. Rest is the primary therapy, usually interpreted as reduced activity and the use of alternative exercise—not bed rest or immobilization with casting. The main purpose is to alleviate the repetitive stress that initiated the symptoms. It is important to keep the child or adolescent mobile, and training can be continued. Alternative exercise is selected that maintains conditioning without aggravating the injury. For example, pool running (treading water in the deep end of a pool) can use the same movements as running but without the weight bearing; bicycling, swimming, and rowing are viable alternatives.
Other modalities include cryotherapy and cold whirlpool baths, and sometimes taping, bracing, splinting, and other orthoses are employed, depending on the injury. Medications such as NSAIDs (see Table 7-4) are sometimes prescribed to reduce inflammation and pain. Topical medications are of questionable value.
Infants, children, and adolescents are at greater risk for heat-related illness than adults (American Academy of Pediatrics, 2000a). Several characteristics of infants and children render them more vulnerable to heat stress. The greater ratio of surface area to body mass in infants and young children leads to increased transfer of heat between the body and the environment. Children produce more metabolic heat for body mass during exercise and have a reduced capacity to convey heat from the body core to the skin. Also, children do not have the sweating capacity of adults and take longer to become acclimated to hot conditions. Young children may not feel the need to drink a sufficient amount of fluid during extended exercise.
Heat cramps are caused by sodium depletion, which in turn potentiates the effects of calcium on skeletal muscle. They most often occur as a result of strenuous exercise in a hot environment. Cramps most frequently involve the leg muscles. Vital signs are usually normal, but the core temperature may be elevated. The child sweats profusely, but mentation is normal. Treatment is rest and replacement of fluid and electrolytes. Ingestion of dilute sports drinks or electrolyte replacement liquids is helpful. Electrolyte replacement solutions are now available as popsicles and gelatin (like Jell-O), which are well tolerated with less vomiting. Replacement electrolyte strips that easily dissolve in the mouth are also available; small sips of a clear fluid such as water can be taken as tolerated.
Heat exhaustion, or heat stress, is a common condition that usually occurs during vigorous exercise in a hot environment. It results from excessive loss of fluids, especially in poorly acclimated and dehydrated children. The onset may be gradual, with initial complaints that include thirst, headache, fatigue, dizziness, anxiety, or nausea and vomiting. The child usually has a clear sensorium but may be somewhat disoriented. The temperature can be normal or mildly elevated; sweating is profuse. Tachycardia, hypotension (usually postural), and syncope may be observed secondary to intravascular volume depletion. Treatment is to move the child to a cool environment, provide rest, and replace fluid volume. The child with a clear sensorium can receive oral replacement fluids, but often IV fluids are required due to vomiting. External cooling methods are not necessary.
Heatstroke represents a failure of normal thermoregulatory mechanisms. Heatstroke usually occurs during or immediately after physical activity, especially in the unacclimated adolescent who is exercising vigorously. The onset is rapid with initial symptoms of headache, weakness, and disorientation. Central nervous system manifestations may be agitation, confusion, and lethargy. Loss of consciousness may occur without warning and may be accompanied by nuchal rigidity, posturing, and convulsions. Sweating may not be present. The temperature is typically higher than 40° C (104° F), and there is severe volume depletion. Immediate care is relocation to a cool environment, removal of clothing, application of cool water (wet towels or immersion), and use of fans. The child is transported to the hospital by EMS for intensive care.
Acute care includes rapid cooling until core temperature reaches 38.9° C (102° F) to prevent overcooling. Antipyretics are not used because they are metabolized by the liver, which is already not functioning properly. Renal and liver failure are common sequelae to heatstroke. Treatment includes careful monitoring of temperature and other vital signs, supportive care such as supplemental oxygen administration, and cautious fluid and electrolyte replacement. Prevention remains the best treatment for hyperthermia. If the temperature is elevated, time in the sun should be decreased. Activity should be stopped if the humidity is elevated as well. The athlete should drink plenty of fluids, preferably with low sugar content.
Nonexertional, or classic, heatstroke has a slow onset with insidious development of anorexia, nausea, vomiting, headache, mental manifestations, and loss of intravascular volume. Classic heatstroke occurs primarily in children with abnormal thermoregulation (e.g., children with cystic fibrosis) and infants subjected to prolonged neglect in a hot environment.
Some athletes are motivated to enhance their performance by any and all means available. They are eager to learn about nutrition, and many are influenced by misconceptions, fads, and superstitions regarding certain foods. Physical performance is affected by energy and body composition. The young athlete must maintain a diet that provides sufficient nutrients and energy to meet metabolic needs for optimum functioning. Physical training increases the need for energy and for more nutrients that convert food energy into chemical energy for physical performance.
There is no evidence to indicate that food supplements, extra vitamins, sports bars, or high-protein diets are needed to meet the demands of heavy physical exercise or improve physical performance. In addition, there are no scientific data, other than anecdotal reports, supporting the benefits of such supplements in increasing physical performance. Athletes should be given accurate information regarding the lack of proven safety for such supplements (Rodriguez, DiMarco, Langley, et al, 2009). Young athletes need considerably more calories than the recommended dietary allowance (RDA). When the basic requirements for growth and activity are met by a balanced diet of protein, grains and cereals, fruits and vegetables, and dairy products,* the additional calories needed for the extra exertion can be selected as desired. The athlete can obtain these extra calories by eating additional helpings from any of the basic four food groups, but many of the additional calories are provided by complex carbohydrates found in foods such as vegetables, pastas, and bread.
The recommended dietary energy intake for adolescents involved in sports is at least 50% of caloric intake from carbohydrates (6 to 10 g/kg/day), protein intake of 1.2 to 1.4 g/kg/day, and 25% to 30% from fat (American Academy of Pediatrics, 2009). It should be noted, however, that energy requirements vary depending on the sport and the child’s age and body build. Adolescent athletes need additional iron and calcium intake from appropriate food sources to meet growth and developmental needs and to replace amounts lost in competition. Box 39-12 presents nutrition pointers for young athletes.
Water and Electrolytes: Considerable water is lost from the body through perspiration, urination, and evaporation from the respiratory tract. Water losses, especially from the skin, increase as the duration and intensity of exercise increase and as environmental temperature rises. Although thirst is experienced early in dehydration, it is unreliable as an indicator of fluid deficit. Athletes should hydrate with water regardless of thirst during strenuous exercise or activity. Water is recommended as the best drink for most athletes, and current recommendations are to take 5 to 8 oz of water every 15 to 20 minutes (150 ml for 40 kg [88 lb] athletes and 250 ml for athletes >40 kg); fluids should never be restricted during activity. A flavored, colored sports beverage containing 6% to 8% carbohydrate, however, may be preferred by children for the taste (American Academy of Pediatrics, 2009). Drinking carbonated beverages is discouraged. Athletes participating in multiple daily exercise sessions in warm environments are at risk for dehydration and should receive all the water they desire.
Very little water is exchanged in the stomach, and it must reach the intestines for absorption. The best fluids for rapid gastric emptying are cold, have low osmolality, and have a large volume. Gatorade and other sports drinks contain excess carbohydrate (6% to 8%); they should be diluted with one or two parts water to one part drink for children, but older adolescents may tolerate the carbohydrate load. New sports drinks are commercially available that reportedly boost physical performance. These new carbohydrate-electrolyte drinks, as well as other commercially available carbohydrate-electrolyte drinks, may cause dental enamel erosion, although the research results vary (Venables, Shaw, Jeukendrup, et al, 2005). Studies outlining the effects of these newer sports drinks on actual physical performance and their untoward effects in adolescent athletes have yet to receive adequate attention in the medical and scientific community.
Small amounts of electrolytes, especially sodium and chloride, are lost during exercise. Because sweat is quite dilute relative to plasma, excessive perspiration can result in excessive loss of water and an increase in plasma concentrations of sodium chloride. Therefore it is more important to replace water than sodium and chloride. Children should be well hydrated before beginning strenuous exercise or sports, especially in warm climates or environments. Periodic drinking breaks are encouraged, and adults or other team members should be alert to the child who has complaints such as headache, cramping, nausea, or vertigo. The use of salt tablets or table salt is unnecessary and may actually be harmful. Athletes usually derive sufficient salt replacement from the diet.
Minerals: The basic diet does not satisfy the iron requirement of 10% to 15% of female athletes, most of whom are teenage girls who tend to become iron depleted after menarche. Young boys who are experiencing rapid adolescent growth and who have irregular and inadequate diets also are at risk of iron depletion. These children need iron supplements.
Adequate calcium intake during puberty is essential to promote mineralization of the growing skeleton. The recommended dietary reference intake (DRI) for calcium in adolescents ages 13 to 18 years is 1300 mg/day, yet few adolescents meet this goal. Calcium plays a vital role in nerve transmission, muscle contraction, and blood coagulation. Female athletes who engage in intensive training may develop amenorrhea, with subsequent decreased bone mineral density, osteopenia, and osteoporosis. Although the last two conditions may not occur immediately, stress fractures and impaired muscle contractions may be seen with low calcium intake. The best sources of additional calcium for athletes are nonfat dairy products. In addition, foods such as regular or low-fat yogurt and cheese, calcium-fortified orange juice, low-fat chocolate milk, and pudding may help meet daily calcium requirements. Consider the amount of calcium in the following: 1 glass of calcium-fortified orange juice contains 200 to 250 mg of calcium; 1 cup of skim milk has 300 mg of calcium; and 8 oz of yogurt contains approximately 410 mg of calcium. A well-balanced diet can provide the necessary calcium intake if adolescent athletes are made aware of the requirements and of the long-term consequences of poor nutrition.
Glycogen: Energy comes primarily from glycogen previously stored in muscles and the liver. Energy for prolonged exercise is derived from high-carbohydrate foods (e.g., bread, cereals, pancakes, potatoes, rice, spaghetti) consumed 24 to 48 hours before the activity, not from a meal eaten just before the activity. The meal before a physical contest should be eaten at least 2 to 4 hours before the exertion and should consist mainly of carbohydrates. Carbohydrate (glycogen) loading is a technique reserved for competition in prolonged aerobic endurance events and requires dietary changes a week before the competitive event. For more information regarding carbohydrate loading and other techniques for improving athletic performance, the reader is directed to texts on sports medicine and sports training.
Weight: Control of body weight by restricting water or food intake or increasing sweat loss is dangerous. Weight loss should not exceed 1.5% of total body weight per week (or 1 to 2 lb/wk) (American Academy of Pediatrics, 2009). Young athletes need appropriate information about nutrition to dispel the allure of fads and fallacies about diet and performance. A sports nutritionist should be consulted for determining an optimal diet based on amount of energy expenditure and energy requirements. The optimum diet for an athlete is one that contains the essential food groups and that is adjusted to the energy requirements of the sport in which the child or adolescent is engaged. Such a dietary plan should provide adequate nutrition for top physical efficiency and performance, maintenance of physical fitness and desirable body weight, and optimum function of all organ systems.
The syndrome known as female athlete triad consists of amenorrhea, osteoporosis, and disordered eating (American Academy of Pediatrics, 2009). The triad was originally described in athletes in sports for which thinness was desired (gymnasts, ballet dancers, figure skaters, and long distance runners) but is now recognized in virtually all sports. The phenomenon has been attributed to a complex interplay of physical, genetic, hormonal, nutritional, psychologic, and environmental factors that include the stress of competition, decreased protein consumption, and altered lean-to-fat body ratio.
Amenorrhea has been reported among girls who engage in strenuous exercise. Except in swimmers, menarche is attained later in athletes than in nonathletes. Gymnasts, figure skaters, and ballet dancers have the latest mean ages of menarche; track athletes have less of a delay in maturity than do gymnasts and ballet dancers, who also tend to be smaller, lighter, and leaner than other female athletes. Swimmers, who tend to be larger, have a mean age of menarche that approximates that for nonathletes. Also, there appears to be an association between delayed menarche and more advanced competitive levels; that is, athletes at more advanced levels have a greater delay than those at lower competitive levels.
One topic for counseling of the female athlete with delayed menarche is pregnancy. Sexually active teenagers, regardless of menstrual status, need to consider contraceptive precautions. Most teenage girls erroneously believe that if they do not menstruate, they cannot become pregnant.
Osteoporosis from decreased levels of estrogen in these athletes, complicated by poor nutritional intake, leads to loss of bone density and stress fractures. The peak of bone density is reached in late adolescence and is related to circulating estrogen levels. Girls with diminished estrogen secretion in delayed menarche will reach late adolescence with low bone density and will be subject to stress fractures and osteoporosis. Hypoestrogenic bone loss greatly increases the risk of injury. It is recommended that bone density (DEXA scan) be evaluated in the athlete who has been amenorrheic for more than 6 months (Joy, Van Hala, and Cooper, 2009).
Disordered eating is less severe and more subtle than eating disorders such as anorexia and bulimia. Disordered eating includes food restrictions, rigid food patterns, fasting, vomiting, and the use of diet pills and laxatives. The goal is to achieve a specific body image that is seen as desirable for the sport and is influenced by others such as a coach, teammates, or peers. Disordered eating results in poor protein intake, low fat intake, and inadequate caloric intake. Adolescent females should increase calcium intake to four to six servings per day of low-fat dairy products (1500 mg of calcium and 400 to 800 international units of vitamin D are the RDAs for amenorrheic athletes [American Academy of Pediatrics, 2000b]) (see Nutrition, p. 1660). In addition, they should consume adequate protein and calories to meet the energy and metabolic needs of exercise. Trainers and coaches also need to be aware of the potentially long-term results of intensive, prolonged exercise in pubertal girls.
Treatment may be long term and often involves development of an appropriate nutritional plan with a registered dietitian, decrease in exercise, behavioral change therapy, and nutritional supplementation aimed at preventing osteoporosis (Waldrop, 2005). However, education alone may not provide adequate incentive to change behavior, and further psychologic health interventions may be required.
An excellent source of further information for parents, coaches, and athletes regarding nutrition for female athletes is “Female Athlete Triad.”*
Young athletes have used various performance-enhancing substances in an attempt to augment their athletic performance. These substances, also known as ergogenic aids, are believed by athletes to increase strength and endurance, delay the onset of fatigue, increase the ability to concentrate, and decrease sensitivity to pain. Although use of these substances is prohibited in international Olympic competition, there are no means at present to enforce a prohibition on their use in other sports settings.
Examples of substances used by athletes include psychomotor stimulants (e.g., amphetamines), anabolic-androgenic steroids (AAS), ephedra, androstenedione (andro), dehydroepiandrosterone (DHEA), creatine, guarana, ginseng, amino acid and protein supplements, and excess amounts of vitamins (niacin, vitamin A, vitamin B6). Because many of these substances are considered natural, many people willingly use them without further investigating potential hazards. The belief is that anything “natural,” even if consumed in excess of the DRI (see Chapter 6), must be perfectly fine for the body because it will be rapidly metabolized and excreted without causing harm. This is not necessarily true for all substances, however, and parents, coaches, trainers, athletes, and health care workers should be knowledgeable about the effects of such substances. Rather than prohibiting their use, a better approach, especially for adolescents, is to provide open, informed discussion on the availability of appropriate substitutes that exist in foods that are indeed healthy to consume yet provide beneficial effects for athletic performance. Schools may include such discussions in existing curricula for their athletes and encourage participation in these educational programs.
Amphetamines and related drugs, such as methylphenidate (Ritalin), as well as caffeine and ephedra or other stimulants, may be taken to provide a sense of increased alertness and relief of fatigue; however, obscuring fatigue may permit participants to exceed their limits and precipitate a sudden collapse. Some stimulants such as ephedra are used to burn fat when used in combination with caffeine. These substances can also make the users more aggressive, which can contribute to injuries to themselves and others.
Other misused drugs include stimulants intended for bronchodilation, decongestants, agents for weight gain or loss, physiologic agents used to enhance oxygen-carrying capacity, and nutritional supplements taken in doses greater than required (American Academy of Pediatrics, 2005). Anabolic steroids are a source of concern to health professionals. The majority of these drugs are no longer manufactured in the United States by legitimate companies. Black market supplies of anabolic steroids are of poor quality and potency. In an attempt to enhance muscle strength, these drugs may be administered to athletes by coaches, managers, athletic trainers, and even physicians. The user develops larger-appearing muscles and increased body weight and body water, but reports on the side effects of these drugs outweigh any benefits for performance during athletic competition (Gregory and Fitch, 2007). Although the psychologic effect may be beneficial, many valid studies have failed to demonstrate any improvement in performance (Calfee and Fadale, 2006).
The precise incidence of anabolic steroid use by adolescent athletes remains debatable but may range from 5% to 11% of high school athletes. Middle-school children are reporting an increased use of anabolic steroids to enhance athletic performance. Coaches and health professionals who work with youth report a trend toward increased use of these agents. Adolescents and young adults rely on poor sources of information about the potential hazards of steroid use (friends, television, muscle magazines) and are generally poorly informed about their potential negative side effects. Health care professionals need to be aware of the clinical manifestations of steroid use. Clinical signs such as severe acne, a sudden increase in strength and muscle mass, a sudden decrease in body fat, a male pattern of baldness, and water retention are common. In females a male pattern of hair growth and a deepening voice are significant observations.
The dangers of continued use are well known and include virilization in females; oligospermia, prostatic hypertrophy, myocardial infarction, stroke, testicular atrophy, infertility, and gynecomastia in males; and premature closure of the epiphyses, acne, increased blood cholesterol levels, hypertension, and hepatocellular carcinoma in both genders. Mood changes have been observed, including aggressiveness, changes in libido, depression, anxiety, and psychosis (Gregory and Fitch, 2007). Health hazards outweigh any potential gain that the drug might provide.
Other drugs that are often misused include nutritional aids, local anesthetic agents, narcotic analgesics such as nalbuphine (Nubain), growth hormone, erythropoietin, creatine, beta blockers (to reduce levels of circulating catecholamines and thus reduce anxiety related to the somatic type of stress), antiinflammatory drugs such as dimethyl sulfoxide (DMSO) (which is not approved for use and is available only as a veterinary or an industrial preparation), and corticosteroids. The possibility of their use by the adolescent athlete should be considered when performing a health assessment.
The American Academy of Pediatrics (2005) recommends that parents and coaches be involved in educating athletes about the adverse effects of performance-enhancing substances and that schools and other sports organizations discourage the use of such substances among athletes. In addition, it is recommended that interventions for encouraging substance-free competition that are positive, rather than punitive, are promoted and encourage sound nutrition and training practices. The pressure to win at all costs is high, and athletes face pressures not only from peers and immediate authority figures but also from others who promise financial gain and potential fame. The problem is often compounded by the existence of poor role models in professional sports activities who earn thousands or even millions of dollars yearly and are known by their colleagues to take performance-enhancing substances yet deny the practice.
A death associated with sports produces renewed anxiety in both parents and health care professionals. The term sudden or instantaneous death is applied to death that occurs within minutes of the onset of the cause of death or within 24 hours of the episode. Sudden cardiac arrest is also a term used to describe the athlete who experiences a sudden death. The incidence of sudden death among high school athletes has been estimated to be 1 per 200,000 per year (Maron, Gohman, and Aeppli, 1998; Maron, 2003). Maron, Doerer, Haas, and colleagues (2009) reported that between 1980 and 2006 most causes of sudden death in athletes occurred as a result of cardiovascular disease (56%), followed by blunt trauma (22%), commotio cordis (3%), and heat stroke (2%). One study of sudden cardiac deaths in young athletes reported an average of 69 cases per year during the years 2000 to 2006 (Drezner, Chun, Harmon, et al, 2008). Overall survival rates for young athletes experiencing sudden cardiac arrest during an athletic event between 2000 and 2006 were 11%, with a trend toward improved survival in the latter years of the study. More males (83%) than females (17%) experienced sudden cardiac arrest, and females were more likely to survive the arrest than were males (Drezner, Chun, Harmon, et al, 2008).
Causes of sudden death are related to three main risk factors: (1) sports with a high inherent risk for sports-related sudden death, (2) recognized or unknown underlying medical problems in child participants, and (3) the sports environment (e.g., the rules, equipment, practice fields or areas of sport participation, and ambient temperature of the geographic area). (Chapter 23 discusses the impact of sudden death on the family and relevant nursing interventions.)
Sports: Sports associated with the greatest risk of sudden death are those involving collision and frequent body contact. Examples of collision sports are football, ice hockey, rugby, and boxing. There is a high potential for serious injury or fatality in sports such as mountain or rock climbing and hang gliding. Sports that involve high-velocity objects, such as baseball and ice hockey, may result in death from serious head or chest injuries. Riding vehicles such as snowmobiles, mopeds, water jet skis, all-terrain vehicles, snowboards, minibikes, and motorcycles can also be considered high-risk sports.
Medical Conditions: The most frequent medical causes of sudden death during sports activity are cardiac abnormalities, especially idiopathic hypertrophic subaortic stenosis (hypertrophic cardiomyopathy). Manifestations suggestive of hypertrophic cardiomyopathy include a typical triad of severe chest pain, dizziness, and dyspnea. Unfortunately some affected individuals never display signs of disease until they collapse during a sports event. A history of sudden death of a relative or relatives in the second or third decade of life often offers a clue to recognition.
Well-trained athletes often display evidence of hypertrophic cardiomyopathy, the so-called athlete’s heart, but the condition is not pathologic. Congenital coronary artery malformation is the second most common cause of sudden death in athletes. Additional causes include valvular heart disease, atherosclerotic coronary artery disease, dilated cardiomyopathy, Marfan syndrome, and myocarditis. Children with systemic hypertension, some types of cardiac arrhythmias such as prolonged QT syndrome, and some forms of heart block will face restrictions in the type and amount of exercise they can tolerate safely. Commotio cordis is a common cause of sudden death in athletes without previous history of heart disease. This occurs following a blunt, nonpenetrating blow to the chest, which produces ventricular fibrillation. Commotio cordis is more common in children and adolescents, with a mean age at occurrence of 13 years, and the blow may not be perceived as being that unusual or significant enough to produce such drastic results.
Appropriate use of an automatic external defibrillator (AED) by civilian bystanders or health care workers may save the life of an athlete who experiences a life-threatening cardiac emergency. A school-based AED program provides a high survival rate for student athletes and nonathletes who experience sudden cardiac arrest on school grounds (Drezner, Rao, Heistand, et al, 2009). (See also Cardiopulmonary Resuscitation, Chapter 31.)
Environmental Causes: Environmental factors that are potential causes of death include playing conditions, clothing, equipment, rules used by officials governing a sport, and outdoor temperature. Heatstroke and hypothermia (see Chapter 18) are the most serious environment-related causes of death in athletes.
The American Academy of Pediatrics has introduced an emergency response plan for schools that includes training teachers and school workers in cardiopulmonary resuscitation and first aid, collecting data to evaluate the risk for injuries in the school environment, and acting to reduce such risk (Hazinski, Markenson, Neish, et al, 2004). Encourage nurses in school programs and in the community to become involved in the establishment of such programs and to assist in training and in planning and implementation of effective risk prevention strategies designed to minimize child deaths in schools and local communities.
The American Academy of Pediatrics also recommends implementing a lay rescuer AED program in schools and that communication regarding the location of such equipment be properly managed (Hazinski, Markenson, Neish, et al, 2004). A consensus statement was developed the National Athletic Trainers’ Association in conjunction with 15 national health care organizations for emergency preparedness and response in the event of sudden cardiac arrest in young athletes. This statement includes recommendations for those involved in high school and collegiate athletics for the prompt recognition and treatment of sudden cardiac arrest, including the use of an AED (Drezner, Courson, Roberts, et al, 2007).
Nurses may become involved in children’s sports activities in preparation and evaluation of children for activities, provision of anticipatory guidance and counseling about athletic competition and nutrition, prevention of injuries, treatment of injuries, and rehabilitation after injuries. Selecting an appropriate sport for both recreation and competition is a joint effort of the child or adolescent, parents, and health professionals. Children are introduced to sports as part of family activities, neighborhood games, and school physical education programs, and both parents and children are influenced by media exposure to a variety of sports. Children are highly influenced by the popularity of and exposure afforded athletics in the school setting, especially in high school.
The best approach to counseling children and parents regarding sports participation is to encourage activities that are most likely to provide pleasure and physical benefits throughout childhood and into adulthood. Exposure to a variety of sports activities is probably better for young children than limitation to only one sport. Caution parents against overprogramming children to allow ample time for other activities and associations. Burnout among children and adolescents who continuously participate in sports is increasing in the United States. Children and adolescent are encouraged to take periodic breaks from such activities to allow physical healing, refresh the mind, and work on strength and conditioning (Brenner and Council on Sports Medicine and Fitness, 2007).
Nurses are sometimes members of a sports medicine team. Although certified sports trainers and other specialists in sports medicine usually manage training and rehabilitation, the nurse should have input regarding injury prevention. Nurses should be able to provide emergency treatment for most types of injuries and know when to refer the injured child for evaluation and care. Sports injuries can occur in free play as well as in organized athletic programs, and a school nurse is often the first person who attends an injured child.
When children sustain athletic injuries, nurses are often responsible for instructing the children and their parents regarding care and rehabilitation. Instructions regarding, for example, the need and schedule for follow-up appointments, application of ice, and any restrictions in activity should be delivered clearly and preferably accompanied by written directions. Emphasize the importance of taking medications as prescribed, since they may be needed for an extended period and compliance may be difficult. For children continuing with activities, nonnarcotic pain medication administration an hour before practice or competition is advantageous.
Prevention of sports injuries is probably the most important aspect of any athletic program. Nurses collaborate with coaches and athletic trainers to ensure that safety measures are carried out. Stretching exercises, warming-up and cooling-down activities, and an appropriate training program are only some of the requisites for safe participation. Protective measures, such as padding, taping, wrapping, or use of other devices, are employed for areas at risk. Nurses are also on the alert for environmental safety risks.
Participation of youth in sports programs has grown significantly in the past several decades. This trend toward greater participation by both genders has been encouraged because of its demonstrated effect in reducing obesity, lowering blood pressure, and lowering cholesterol and lipid levels.
For some athletes, their whole life revolves around sports participation. When a serious injury occurs, the athlete’s self-esteem and self-image may suffer a devastating blow. Nursing assessment may reveal an athlete who appears to have difficulty dealing with this event and actually rejects any positive reinforcement. The nurse may help the child and family in establishing a support system. The athlete may need to learn new coping skills and explore other avenues to foster feelings of increased self-worth and a sense of accomplishment.
Lack of participation, exercise aversion, and declining interest in sports are sometimes the aftermath of participation during the school years and adolescence. Motivation can be altered or permanently destroyed by failure to appreciate the child’s or adolescent’s needs related to sports activities. Ridicule or derogation during acquisition of motor skills can shatter a child’s or adolescent’s self-esteem, producing anxiety and self-doubt that may result in a lifelong aversion to sports. Every child should have the opportunity to develop a strong sense of personal worth through the process of motor learning and acquisition of skills. All participants should have the opportunity to participate and be rewarded with positive encouragement for their contribution, no matter how small; all participants should be rewarded for what they do right.
Torticollis (wry neck), which can be either congenital or acquired, is a condition of limited neck motion in which the neck is flexed and the head is drawn or tilted laterally to the affected side while the chin is pointed toward the opposite side. It is a manifestation rather than a disease entity and may be associated with a number of conditions, including congenital abnormality of the cervical spine or a traumatic lesion of the sternocleidomastoid muscle. Congenital muscular torticollis may occur as a result of abnormal positioning in utero, which causes contracture of the sternocleidomastoid muscle (Spiegel, Hosalkar, Dormans, et al, 2007). Infants with positional plagiocephaly may have muscular torticollis as well, which can be successfully treated with neck stretching exercises (Graham, Gomez, Halberg, et al, 2005; Persing, James, Swanson, et al, 2003).
In early infancy a firm, nontender mass may be felt in the midportion of the muscle. The mass regresses and is replaced by fibrous tissue. If the condition remains untreated, permanent limitation of neck movement results, and the head and face become asymmetric, probably because of impaired blood supply to the depressed side of the head. Plagiocephaly and facial asymmetry often occur as a result of the contractured sternocleidomastoid muscle. Patients may have other associated musculoskeletal conditions, including calcaneovalgus foot deformity and metatarsus adductus (Spiegel, Hosalkar, Dormans, et al, 2007).
Treatment of simple torticollis consists of gentle stretching exercises. The face is turned toward the affected muscle while the head is tilted in the opposite direction with the neck extended. A physical therapist typically establishes the treatment regimen to be followed by the nurse and family. The exercises are best performed by two persons—one to control the torso and one to manipulate the head. If stretching exercises are unsuccessful, surgical release of the sternocleidomastoid muscle may be needed. Increasingly, surgical correction by age 12 to 18 months is recommended to prevent muscle contractures and further progression of plagiocephaly. Other forms of torticollis occur in infancy or may develop at a later age but are not discussed in this text.
Nurses should be alert to the possibility of torticollis in infants with limited head movement. After diagnosis it is frequently the nurse’s responsibility to teach the exercises and supervise the family in performing them. The exercises require very explicit instructions to the family, and compliance is mandatory. The nurse should also suggest that the child be placed in the crib or playpen in a way that encourages turning the head away from the deformity to observe activities and interesting items. Parents can encourage the child to turn the head in the direction desired for correction through feeding and playing with the child.
Legg-Calvé-Perthes disease, sometimes called coxa plana or osteochondritis deformans juvenilis, is a self-limiting disorder in which there is aseptic necrosis of the femoral head. The disease affects children ages 2 to 12 years, but most cases occur as an isolated event in boys between 4 and 8 years of age. In approximately 10% of cases the involvement is bilateral; most of the affected children have a skeletal age significantly below their chronologic age (Hosalkar, Horn, Friedman, et al, 2007). The male/female ratio is 4 : 1 or 5 : 1. Caucasian children are affected 10 times more frequently than African-American children.
Critical Thinking Case Study—Legg-Calvé-Perthes Disease
The cause of the disease is unknown. A disturbance of circulation to the femoral capital epiphysis produces an ischemic aseptic necrosis of the femoral head. During middle childhood, circulation to the femoral epiphysis is more tenuous than at other ages, being supplied almost entirely by lateral retinacular vessels. This circulatory impairment appears to extend to the epiphysis and acetabulum as well. The pathologic events seem to take place in four stages (Box 39-13), although there is controversy regarding prognostic classification. The entire disease process may encompass as few as 18 months or continue for several years. The reformed femoral head may be severely altered or appear entirely normal.
The onset of Legg-Calvé-Perthes disease is usually insidious, and the history may reveal only intermittent appearance of a limp on the affected side or a symptom complex, including hip soreness, ache, or stiffness that can be constant or intermittent. The parents may report seeing the child limping, and the limp becomes more pronounced with increased activity. The pain may be experienced in the hip, along the entire thigh, or in the vicinity of the knee joint. The pain and limp are usually most evident on arising and at the end of a long day of activities. The pain is usually accompanied by joint dysfunction and limited range of motion. There may be a vague history of trauma. The diagnosis is established by radiographic examination, with the definitive diagnosis being made by MRI, which demonstrates osteonecrosis.
Because deformity occurs early in the disease process, the aims of treatment are to eliminate hip irritability; restore and maintain adequate range of hip motion; prevent capital femoral epiphyseal collapse, extrusion, or subluxation; and ensure a well-rounded femoral head at the time of healing. Treatment varies according to the child’s age at the time of diagnosis and the appearance of the femoral head vasculature and position within the acetabulum. Nonsurgical containment of the femoral head may be accomplished with abduction casts, whereas a pelvic or femoral osteotomy may be used to contain the femoral head. Activity causes microfractures of the soft ischemic epiphysis, which tend to induce synovitis, stiffness, and adductor contracture. The initial therapy is rest and non–weight bearing, which help reduce inflammation and restore motion. Later, active motion is encouraged. In some cases traction is applied to stretch tight adductor muscles.
Containment can be accomplished in several ways. One is the use of non–weight-bearing devices, such as an abduction brace (e.g., Atlanta Scottish Rite orthosis), leg casts, or a leather harness sling, which prevent weight bearing on the affected limb. Another includes the use of various weight-bearing appliances, such as abduction-ambulation braces or casts after a period of bed rest and traction. A third option consists of surgical reconstruction and containment procedures. Conservative therapy must be continued for 2 to 4 years, although braces constructed from lightweight materials allow the child to maintain a nearly normal activity level. Surgical correction, although subjecting the child to additional risks (e.g., from anesthesia, infection, blood transfusion), returns the child to normal activities in 3 to 4 months. The use of home traction has also been explored.
The disease is self-limiting, but the ultimate outcome of therapy depends on early and efficient treatment and the child’s age at the onset of the disorder. Children 5 years and younger, whose epiphyses are more cartilaginous, have the best prognosis for complete recovery. Children over 9 years old have a significant risk for degenerative arthritis, especially if they have femoral head deformity at the time of diagnosis (Hosalkar, Horn, Friedman, et al, 2007). The later the diagnosis is made, the more femoral damage will have occurred before treatment is implemented. In many cases, with good patient compliance, the prognosis is excellent.
Nurses are often the first health care professionals to identify affected children and to refer them for medical evaluation. Because these children are largely cared for on an outpatient basis, the major emphasis of nursing care is on teaching the family the required care. The family needs to learn the purpose, function, application, and care of the corrective device and the importance of compliance with the prescribed regimen to achieve the desired outcome.
One of the most difficult aspects of the disorder is the need to cope with normally active children who feel well but must remain relatively inactive. It is important to emphasize that children should continue to attend school and engage in former activities that can be adapted to the therapeutic appliance. Adaptation of school activities may need to be arranged with school personnel.
Suitable activities must be devised to meet the needs of the child in the process of developing a sense of initiative or industry. Activities that fulfill the creative urges are well received. This is also an opportune time to encourage the child to begin a hobby such as assembling collections, building models, or engaging in crafts.
Slipped capital femoral epiphysis (SCFE), or coxa vara, refers to the spontaneous displacement of the proximal femoral epiphysis in a posterior and inferior direction. It develops most frequently shortly before or during accelerated growth and the onset of puberty (children between the ages of 10 and 16 years; median age, 13 for boys, 12 for girls) and is most frequently observed in boys and obese children. Bilateral involvement occurs in up to 60% of cases. Osteonecrosis is a common complication of SCFE and is reported to occur in 17% to 47% of all patients (Hosalkar, Horn, Friedman, et al, 2007).
Most cases of SCFE are idiopathic, although it can be associated with endocrine disorders, renal osteodystrophy, and radiotherapy. The cause of idiopathic SCFE is multifactorial and includes obesity, physeal architecture and orientation, and pubertal hormone changes that affect physeal strength. Approximately 65% of patients with SCFE are above the 90th percentile in weight-for-age profiles; therefore obesity is believed to play a significant role in the development of the condition (Hosalkar, Horn, Friedman, et al, 2007). Although obesity stresses the physeal plate, SCFE can also occur in children who are not obese. Radiographs show medial displacement of the epiphysis and uncovered upper portion of the femoral neck adjacent to the physis. There is a widened growth plate and irregular metaphysis. The capital femoral epiphysis remains in the acetabulum, but the femoral neck slips, deforming the femoral head and stretching blood vessels to the epiphysis.
The following different types of clinical manifestation have been observed: (1) an episode of minor trauma in which the epiphysis is acutely displaced in a previously functional joint; (2) gradual displacement without definite injury, with progressively increased hip disability; (3) intermittent bouts of displacement alternating with periods of well-being, with the gradual appearance of symptoms associated with ambulation (e.g., external rotation); and (4) a combined gradual and traumatic displacement in which there is gradual slippage, with further displacement caused by injury.
SCFE is suspected when an adolescent or preadolescent, especially one who is obese or tall and lanky, begins to limp and complains of pain in the hip continuously or intermittently. The pain is frequently referred to the groin, anteromedial aspect of the thigh, or knee. Physical examination reveals early restriction of internal rotation on adduction and external rotation deformity with loss of abduction and internal rotation as the severity increases. The child or adolescent often lies still with the lower extremity flexed, abducted, and externally rotated because of the intense pain; any attempts to move the limb are met with significant resistance (Loder, 2006). The diagnosis is confirmed by radiographic examination.
The treatment goals of SCFE are to (1) prevent further slipping until physeal closure, (2) avoid further complication such as avascular necrosis, and (3) maintain adequate hip function (Loder, 2006). Once the diagnosis is established, the child should be made completely non–weight bearing to prevent further slippage. Surgical treatment varies with the degree of displacement. Traditional methods included presurgery bed rest and traction followed by surgical pinning. Some surgeons prefer to take the child to surgery within 24 hours of the onset of acute symptoms and avoid further risk for avascular necrosis (Loder, 2006). Surgical pinning involves the placement of a single pin or alternatively two cannulated screws through the femoral neck into the proximal femoral epiphysis to prevent further slippage (Hart, Grottkau, and Albright, 2007; Loder, 2006). Several other surgical treatment options are also available and are described in the Loder (2006) reference. Postsurgical care includes non–weight bearing with crutch ambulation until acceptable, painless range of motion is achieved.
SCFE is an emergency and requires early diagnosis and treatment to increase the likelihood of a satisfactory cure. The two most severe complications of SCFE are avascular necrosis of the proximal femoral physis and chondrolysis, which involves the loss of articular cartilage, decreased range of motion, and pain (Hart, Grottkau, and Albright, 2007). Avascular necrosis is a complication of an unstable hip, which may result in degenerative hip disease in later life (Loder, 2006).
Nursing care involves preparing the child and family for the surgical procedure and recovery. The child may be placed on a patient-controlled analgesia (PCA) pump for postoperative pain management and should receive appropriate instructions preoperatively for activating the dosing by the pump. If traction is used preoperatively, nursing care is the same as that for a child in traction, as discussed earlier in this chapter. Postoperative care involves hemodynamic stabilization and assessment for complications. The adolescent is taught the proper use of crutches and the importance of avoiding any weight bearing on the affected hip (if unilateral). The adolescent may be involved in building upper body strength during the convalescent period to increase mobility from bed to wheelchair, as appropriate. Self-care and performance of activities of daily living to capability are encouraged to promote confidence and decrease a sense of helplessness.
Pain management is essential, and observation for major complications associated with major surgery and immobilization (thrombus, pneumonia, constipation) is an important part of nursing care.
The spine, which consists of numerous segments, can acquire deformation curves of three types: kyphosis, lordosis, and scoliosis (Fig. 39-33).
Fig. 39-33 Defects of spinal column. A, Normal spine. B, Kyphosis. C, Lordosis. D, Normal spine in balance. E, Mild scoliosis in balance. F, Severe scoliosis not in balance. G, Rib hump and flank asymmetry seen in flexion caused by rotary component. (Redrawn from Hilt NE, Schmitt EW: Pediatric orthopedic nursing, St Louis, 1975, Mosby.)
Kyphosis is an abnormally increased convex angulation in the curvature of the thoracic spine (see Fig. 39-33, B). It can occur secondary to disease processes such as tuberculosis, chronic arthritis, osteodystrophy, or compression fractures of the thoracic spine. The most common form of kyphosis is postural. Children, especially during the time when skeletal growth outpaces growth of muscle, are prone to exaggeration of a tendency toward kyphosis. This is particularly common in self-conscious adolescent girls who assume a round-shouldered slouching posture in an attempt to hide their developing breasts and increasing height. Scheuermann kyphosis is a thoracic curve of greater than 45 degrees with wedging of more than 5 degrees of at least three adjacent vertebral bodies and vertebral irregularity.
Postural kyphosis is almost always accompanied by a compensatory postural lordosis, an abnormally exaggerated concave lumbar curvature. Treatment of kyphosis consists of exercises to strengthen shoulder and abdominal muscles and bracing for more marked deformity. With adolescents who are significantly self-conscious about their appearance, the best approach is to emphasize the cosmetic value of corrective therapy and to place the responsibility on the adolescent for carrying out an exercise program at home, with regular visits to and assessments by a physical therapist. Treatment with a brace may be indicated until skeletal maturity; surgical spinal fusion may be considered for severe, painful, or progressive deforming thoracic curves such as Scheuermann kyphosis.
Lordosis is an accentuation of the lumbar curvature beyond physiologic limits (see Fig. 39-33, C). It may be a secondary complication of a disease process, the result of trauma, or idiopathic. Lordosis is a normal observation in toddlers, and in older children it is often seen in association with flexion contractures of the hip, obesity, congenital dislocated hip, and SCFE. During the pubertal growth spurt, lordosis of varying degrees is observed in teenagers, especially girls. In obese children the weight of the abdominal fat alters the center of gravity, which causes a compensatory lordosis. Unlike kyphosis, severe lordosis is usually accompanied by pain.
Treatment involves management of the predisposing cause when possible, such as weight loss and correction of any existing orthopedic or neuromuscular conditions. Postural exercises or support garments are helpful in relieving symptoms in some cases; however, these do not usually effect a permanent cure.
Spondylolisthesis is the forward slipping of one vertebral body on another (“slipped disk”). It usually involves L5 and S1. Retrospondylolisthesis, or retrolisthesis, is the posterior slipping or displacement of one vertebral body on another. Either condition can have multiple causes, including congenital deficiency or fracture of part of the vertebra. The condition may be asymptomatic, or it may cause lower back pain or neurologic compromise. Spondylolisthesis can usually be treated nonsurgically, although spinal fusion may be indicated in cases of severe, progressive slip.
Scoliosis is a complex spinal deformity in three planes, usually involving lateral curvature, spinal rotation causing rib asymmetry, and thoracic hypokyphosis (see Fig. 39-33, E-G, and Fig. 39-34). It is the most common spinal deformity and can be further classified according to age of onset: congenital, occurs in fetal development; infantile, at birth or up to 3 years of age; childhood (juvenile), occurs in children ages 4 to 10 years of age; or adolescent, during the growth spurt of early adolescence (the most common type). Scoliosis can be caused by a number of conditions and may occur alone or in association with other diseases, particularly neuromuscular conditions (neuromuscular scoliosis). In most cases, however, there is no apparent cause, hence the name idiopathic scoliosis. The following discussion involves the adolescent type, which is often called adolescent idiopathic scoliosis. There appears to be a genetic component to the etiology of idiopathic scoliosis; however, the exact relationship has yet to be established.
Fig. 39-34 Moderate thoracic idiopathic adolescent scoliosis. Forward flexion reveals a mild rib hump deformity. (From Zitelli BJ, Davis HW: Atlas of pediatric physical diagnosis, ed 4, St Louis, 2002, Mosby.)
Critical Thinking Case Study—Scoliosis
Idiopathic scoliosis is most noticeable during the preadolescent growth spurt. Parents frequently bring a child for follow up on an abnormal school scoliosis screening or because of ill-fitting clothes, such as poorly fitting jeans. School screening is somewhat controversial, since there are no controlled studies to demonstrate improved outcomes and a reported number of false positives lead to referrals (Bunnell, 2005). The American Academy of Orthopaedic Surgeons and the American Academy of Pediatrics have recently published a joint statement favoring scoliosis screening for preadolescents and adolescents either in the school, physician’s office, or nurses’ clinic (Richards and Vitale, 2008). According to the American Academy of Orthopaedic Surgeons (Richards and Vitale, 2008), girls should be screened at ages 10 and 12 years, whereas boys should be screened once either at age 13 or 14 years. The benefits of early detection, referral, and medical treatment are considered to be significant, but the persons performing the screenings must be educated in the detection of spinal deformity.
The standing child, wearing only underpants and viewed from behind, may exhibit asymmetry of shoulder height, scapular or flank shape, or hip height, or may demonstrate pelvic obliquity. Cutaneous changes may also be observed. When the child bends forward at the waist so that the trunk is parallel with the floor and the arms hang free (the Adams position), asymmetry of ribs and flanks may also be appreciated (see Figs. 39-33, G, and 39-34). A scoliometer is also used in the initial screening to measure truncal rotation (as does the Adams test). Often a primary curve and a compensatory curve will place the head in alignment with the gluteal cleft. With an uncompensated curve, however, the head and hips are not aligned. By stabilizing the pelvis and asking the child to twist to both sides, the practitioner can evaluate the flexibility of the curve.
Definitive diagnosis is made by radiographs of the child in the standing position and use of the Cobb technique (standard measurement of angle curvature), which establishes the degree of curvature. The Risser scale is used to evaluate skeletal maturity on the radiographs. This scale assists in making a determination of the likely progression of the spinal angulature as the child’s bones mature. The Tanner maturity rating is also used to evaluate the risk of curve progression in adolescents. Not all spinal curvatures are scoliosis. A curve of less than 10 degrees is considered a postural variation. Curves of less than 20 degrees are mild and, if nonprogressive, do not require treatment.
Intraspinal conditions or other disease processes that can cause scoliosis must be ruled out. The presence of pain, sacral dimpling or hairy patches, cutaneous vascular changes, absent or abnormal reflexes, bowel or bladder incontinence, or left thoracic curve may indicate an intraspinal abnormality such as syringomyelia, diastematomyelia, or tethered cord syndrome. An MRI scan is usually obtained for evaluation.
Current management options include observation with regular clinical and radiographic evaluation, orthotic intervention (bracing), and surgical spinal fusion (Fig. 39-35). Treatment decisions are based on the magnitude, location, and type of curve; the age and skeletal maturity of the child or adolescent; and any underlying or contributing disease process.
Fig. 39-35 Radiographs showing severe scoliosis before surgical correction (A) and after surgical correction of scoliosis, including internal fixation (B).
Bracing and Exercise: For many curves in the growing child and adolescent, bracing may be the treatment of choice. It is important to realize that bracing is not curative, but that it may slow the progression of the curvature to allow skeletal growth and maturity. The two most common types of bracing are (1) the Boston and Wilmington braces, which are underarm orthoses customized from prefabricated plastic shells, with corrective forces for each patient using lateral pads and decreasing lumbar lordosis; and (2) a TLSO, which is an underarm orthosis made of plastic that is custom molded to the body and then shaped to correct or hold the deformity (Fig. 39-36). The Milwaukee brace, which is an individually adapted brace that includes a neck ring, is rarely used in scoliosis but is sometimes used in the treatment of kyphosis. The Charleston nighttime bending brace is worn only when the child is in bed because it prevents walking because of the severity of the trunk bend.
Fig. 39-36 A, Standard thoracolumbosacral orthosis (TLSO) brace for idiopathic scoliosis. Note the color and design incorporated into the brace to make it more acceptable to children and adolescents. B and C, Variation of a standard TLSO brace that fastens in the back to provide needed support for the spinal curvature.
Bracing, although used as the gold standard treatment for mild to moderate curvatures, has not proved to be entirely effective in the treatment of scoliosis. Compliance in wearing the brace is difficult because of the adolescent’s age and preoccupation with body image and appearance. In addition, bracing was historically intended to be a 23-hour treatment. In some cases treatment may involve bracing at nighttime only, since this may enhance compliance in adolescents with scoliosis (Jarvis, Garbedian, and Swamy, 2008). Experts recognize that brace treatment in some children with significant scoliosis may help avoid surgical intervention by slowing curve progression; however, further studies are needed to clarify the effectiveness of bracing (Richards and Vitale, 2008).
Exercises alone and chiropractic treatment are rarely of value in managing scoliosis; transcutaneous electrical nerve stimulation has also proved to be an ineffective treatment for this condition. Exercises are of benefit when used in conjunction with bracing to maintain and increase the strength of spinal and abdominal muscles during treatment.
Operative Management: Surgical intervention may be required for correction of severe curves (see Fig. 39-35, A) (usually 45 degrees or more in skeletally immature patients and 50 to 55 degrees or more in the skeletally mature [Spiegel, Hosalkar, and Dormans, 2007]). The degree of curvature and the cause determine the decision regarding surgery. Bracing and exercise have been universally disappointing in treating curves greater than 40 degrees, and paralytic and congenital curves, which eventually progress, are best treated with early surgical stabilization if the child’s health status will allow major surgery. The child’s age and location of the curvature influence the decision for surgery, and any progressive or severe curve that does not respond to more conservative orthotic measures requires surgical correction. Difficulties with balance or seating, respiratory excursion, or pain are also considered.
A number of surgical techniques are now available. One surgical technique consists of realignment and straightening with internal fixation and instrumentation combined with bony fusion (arthrodesis) of the realigned spine. The goals of surgical intervention are to correct the curvatures on the sagittal and coronal planes and to provide a solid, pain-free fusion in a well-balanced torso, with maximum mobility of the remaining spinal segments.
Many instrumentation systems, including Harrington, Dwyer, Zielke, Luque, Cotrel-Dubousset, Isola, TSRH (Texas Scottish Rite Hospital), and Moss Miami, are available. Selection of the system is individualized according to the patient’s needs and surgeon’s preference. Posterior or anterior surgical approaches can be used.
The Harrington system, the first internal spinal instrumentation device, consists of distraction and compression rods, hooks, and nuts. The posterior elements are decorticated, and bone from the iliac crest or donor bone is placed across the vertebrae to provide fusion. Postoperatively the child is log-rolled to prevent spinal motion, and a molded plastic jacket is used to stabilize the spine until the fusion is solid.
The Luque-rod segmental spinal instrumentation provides segmental stability by the use of wires and L-shaped rods. By way of a posterior approach, the wires are threaded beneath the lamina of each vertebra and tightened around the rods resting along the transverse processes to stabilize the spine. Bone from the iliac crest or donor bone is used to fuse the spine. The advantage of this method is that the patient can be mobile within a few days and requires no postoperative immobilization. The disadvantage is the risk of nerve damage.
The Cotrel-Dubousset instrumentation combines the Harrington and L-rod approaches by using bilateral rods and hooks at many sites. Anterior approaches using the Dwyer or Zielke instrumentation involve screws into the vertebral bodies connected by a cable or rod. These systems require postoperative immobilization with a custom-fitted plastic jacket.
Advances in surgical technology currently being evaluated include thoracoscopic spinal fusion and placement of implants; metallic staples may also be placed into the vertebral bodies to achieve spinal fusion and to correct the deformity (Spiegel, Hosalkar, and Dormans, 2007). The use of minimally invasive surgery techniques such as video assisted thoracoscopic surgery has gained widespread acceptance for its decreased recovery time, decreased pain, fewer intraoperative and postoperative complications, small incision, less impact on pulmonary function, and less chest wall disruption (Lonner, 2007).
Treatment for scoliosis extends over a significant portion of the affected child’s period of growth. In adolescents this period is the one in which their identity, physical and psychologic, is formed. The identification of scoliosis as a “deformity,” in combination with unattractive appliances and a significant surgical procedure, can have a negative effect on the already fragile adolescent body image. The adolescent and family require excellent nursing care to meet not only physical needs, but also psychologic needs associated with the diagnosis, surgery, postoperative recovery, and eventual rehabilitation (Slote, 2002).
Although these adolescents are encouraged to participate in most peer activities, necessary therapeutic modifications are likely to make them feel different and apart. Nursing care of the adolescent who is facing scoliosis surgery, potential social isolation, pain, and uncertainty, not to mention misunderstood emotions and body image issues, must be evaluated from the adolescent’s perspective to be successful in meeting the individual’s needs (Napierkowski, 2007).
When a child or adolescent first faces the prospect of a prolonged period in a brace, jacket, or other device, the therapy program and the nature of the device must be explained thoroughly to both the child and the parents so that they will understand the anticipated results, how the appliance corrects the defect, the freedoms and constraints imposed by the device, and what they can do to help achieve the desired goal. The management involves the skills and services of a team of specialists, including the orthopedist, physical therapist, orthotist (a specialist in fitting orthopedic braces), nurse, social worker, and sometimes a thoracic or pulmonary specialist.
It is difficult for a child to be restricted at any phase of development, but the adolescent needs continual positive reinforcement, encouragement, and as much independence as can be safely assumed during this time. Guidance and assistance regarding anticipated problems, such as selection of clothing and participation in social activities, are appreciated by adolescents. Socialization with peers is strongly encouraged, and every effort is expended to help the adolescent feel attractive and worthwhile.
Preoperative Care: The preoperative workup usually involves a radiographic series, including bending and traction films, pulmonary function studies, and a number of routine laboratory studies (including prothrombin, partial thromboplastin, and bleeding times; blood count; electrolyte levels; urinalysis and urine culture; and blood levels of any medications). Because spinal surgery usually involves considerable blood loss, several options are considered preoperatively to maintain or replace blood volume. These options include autologous blood donations obtained from the patient before the surgery; intraoperative blood salvage; intraoperative hemodilution; erythropoietin administration; and controlled induced hypotension, which must be carefully monitored at all times to prevent physiologic instability (Newton and Wenger, 2001).
Surgery for spinal fusion is complex, and often adolescents who require the procedure because of idiopathic scoliosis are not familiar with medical terms or procedures. Preoperative teaching is critical for the adolescent to be able to cooperate and participate in his or her treatment and recovery. Because the surgery is extensive, the patient is taught how to manage his or her own PCA pump; how to log-roll; and the use and function of other equipment, such as a chest tube (for anterior repair) and Foley catheter. It is recommended that the child or adolescent bring a favorite toy (age dependent) or personal items such as a favorite stuffed animal, laptop computer, cellular phone (for web surfing, texting, and e-mailing), movie player, MP3 player, or portable compact disc player for postoperative use. Meeting with a peer who has undergone a similar surgery is also valuable (Slote, 2002).
Postoperative Care: After surgery, patients are monitored in an acute care setting and log-rolled when changing position to prevent damage to the fusion and instrumentation. In some cases an immobilization brace or cast is used postoperatively depending on the type of surgical intervention. Skin care is important, and pressure-relieving mattresses or beds may be needed to prevent pressure wounds. (See Maintaining Healthy Skin, Chapter 27.)
In addition to the usual postoperative assessments of wound, circulation, and vital signs, the neurologic status of the patient’s extremities requires special attention. Prompt recognition of any neurologic impairment is imperative because delayed paralysis may develop that requires surgical intervention. Common postoperative problems after spinal fusion include neurologic injury or spinal cord injury, hypotension from acute blood loss, wound infection, syndrome of inappropriate antidiuretic hormone, atelectasis, pneumothorax, ileus, delayed neurologic injury, and implanted hardware complications (Freeman, 2007; Newton and Wenger, 2001). Superior mesenteric artery syndrome may occur several days after spinal surgery; this involves duodenal compression by the aorta and superior mesenteric artery and may result in acute partial or complete duodenal obstruction. Clinical manifestations include epigastric pain, nausea, copious vomiting, and eructation; symptoms are aggravated in the supine position and often relieved with the patient in a left lateral decubitus or prone position.
The adolescent usually has considerable pain for the first few days after surgery and requires frequent administration of pain medication, preferably opioids administered intravenously on a regular schedule. For children able to understand the concept, PCA is recommended. (See Pain Assessment; Pain Management, Chapter 7.) In most cases the patient begins walking as soon as possible. Depending on the instrumentation used and the surgical approach, most patients are walking by the second or third postoperative day and are discharged by 1 week. In addition to pain management, the patient is evaluated for skin integrity, adequate urinary output, fluid and electrolyte balance, and ileus (Slote, 2002). Discharge planning should include a timetable for follow-up with the practitioner and resumption of regular activities.
All patients are started on physical therapy as soon as they are able, beginning with range-of-motion exercises on the first postoperative day and many of the activities of daily living in the following days. Self-care, such as washing and eating, is always encouraged. Throughout the hospitalization, age-appropriate activities and contact with family and friends are important parts of nursing care and planning (see Immobilization, p. 1623).
Encourage the family to become involved in the patient’s care to facilitate the transition from hospital to home management (see Nursing Care Plan). An organization that provides education and services to both families and professionals is the National Scoliosis Foundation.* The American Academy of Orthopaedic Surgeons† and Scoliosis Research Society,‡ an organization of physicians and scientists, have published an excellent book, Scoliosis, and the Scoliosis Research Society has educational information on its website.
Osteomyelitis, an infectious process in the bone, can occur at any age but most frequently is seen in children 10 years of age or younger. S. aureus is the most common causative organism (Box 39-14). Neonates are also likely to have osteomyelitis caused by group B streptococci. Since the advent of Haemophilus influenzae type b immunization in the late 1980s, H. influenzae has become a less common causative pathogen. Children with sickle cell anemia may develop osteomyelitis from Salmonella organisms as well as S. aureus. Neisseria gonorrhoeae is a potential causative organism in the sexually active adolescent.
Acute hematogenous osteomyelitis results when a blood-borne bacterium causes an infection in the bone. Common foci include infected lesions, upper respiratory tract infections, otitis media, tonsillitis, abscessed teeth, pyelonephritis, and infected burns. Exogenous osteomyelitis is acquired from direct inoculation of the bone from a puncture wound, open fracture, surgical contamination, or adjacent tissue infection. Subacute osteomyelitis has a longer course and may be caused by less virulent microbes with a walled-off abscess or Brodie abscess, typically in the proximal or distal tibia. Chronic osteomyelitis is a progression of acute osteomyelitis and is characterized by the presence of dead bone, bone loss, and drainage and sinus tracts. Generally healthy bone is not likely to become infected. Factors that contribute to infection include inoculation with a large number of organisms, presence of a foreign body, bone injury, high virulence of an organism, immunosuppression, and malnutrition. Certain types and locations of bone are also more vulnerable to infection.
In acute osteomyelitis bacteria adhere to bone, causing a suppurative infection with inflammatory cells, edema, vascular congestion, and small-vessel thrombosis; the result is bone destruction, abscess formation, and dead bone (sequestra). Infection within the bone can rupture through the cortex into the subperiosteal space, stripping loose periosteum and forming an abscess. As dead bone is resorbed, new bone is formed along the live bone and infection borders. This surrounding sheath of live bone is called an involucrum. Sinus tracts from perforations in the involucrum may drain pus through soft tissue to the skin.
The pathology of osteomyelitis is different in infants, children older than 1 year of age, and adults. In infants blood vessels cross the growth plate into the epiphysis and joint space, which allows infection to spread into the joint. In children the infection is contained by the growth plate, and joint infection is less likely (unless the infection is intracapsular) (Fig. 39-37). Adults have no growth plate to contain infection, and again the joint is compromised. Adult periosteum is attached to bone; consequently, rupture through the periosteum and sinus drainage is more common in adults.
Fig. 39-37 Pathogenesis of acute osteomyelitis differs with age. A, In infants younger than 1 year the epiphysis is nourished by penetrating arteries through the physis, allowing development of the condition within the epiphysis. B, In children up to 15 years of age the infection is restricted to below the physis because of interruption of the vessels. (From McCance KL, Huether SE: Pathophysiology: the biological basis for disease in adults and children, ed 6, St Louis, 2010, Mosby.)
In children severe pain, fever, irritability, and tenderness with or without local signs of inflammation suggest osteomyelitis. The extremity is tender, and the child may hold it in semiflexion and resist movement. In infants these symptoms may be minimal or absent, and pain may be difficult to localize. The infant may demonstrate pain with movement of the extremity or hold it immobile. Fever is uncommon in infants, and they often do not appear to be ill (Lampe, 2007). Infants may have an adjacent joint effusion. Typically the metaphysis of long bones, the tibia and femur, is involved. In a small portion of children more than one bone may be affected.
Organism identification and antibiotic susceptibility testing are essential for effective therapy. Obtain cultures of aspirated subperiosteal pus along with cultures of blood, joint fluid, and infected skin samples. Bone biopsy is indicated if blood culture results and radiographic findings are not consistent with osteomyelitis. Supporting evidence for osteomyelitis includes leukocytosis and elevated erythrocyte sedimentation rate. Radiographic signs, except for soft tissue swelling, are evident only after 2 to 3 weeks. A three-phase technetium bone scan can show areas of increased blood flow, such as occurs in early stages in infected bone, and is useful in locating multiple sites; however, it is not a diagnostic test. CT can detect bone destruction, and MRI provides anatomic details useful in delineating the area of involvement, especially if surgical intervention is planned. The differential diagnosis includes trauma, malignant lesions, leukemia, juvenile rheumatoid arthritis, and acute rheumatic fever. Sometimes the osteomyelitis may be unrecognized if it occurs as a complication of a severe toxic and debilitating disease.
After culture specimens are obtained, empiric therapy is started with IV antibiotics covering the most likely organisms. For S. aureus nafcillin or clindamycin is generally used; methicillin-resistant S. aureus may require vancomycin. When the infective agent is identified, administration of the appropriate antibiotic is initiated and continued for at least 4 weeks, but the length of therapy is determined by the duration of the symptoms, the response to treatment, and the sensitivity of the organism. In selected cases oral antibiotic therapy may follow a shorter IV course. Because of the prolonged duration of high-dose antibiotic therapy, it is important to monitor for hematologic, renal, hepatic, ototoxic, and other potential side effects.
Surgery may be indicated if there is no response to specific antibiotic therapy, persistent soft tissue abscess, or infection that spreads to the joint. Opinions differ regarding surgical intervention, but many advocate sequestrectomy and surgical drainage to decompress the metaphyseal space before pus erupts and spreads to the subperiosteal space, forming abscesses that strip the periosteum from bone or form draining sinuses. When these complications occur, a chronic infection usually persists. When surgical drainage is carried out, polyethylene tubes are placed in the wound. One tube instills an antibiotic solution directly into the infected area by gravity, and the other, connected to a suction apparatus, provides drainage. (See Chapter 18 for a discussion of wound care.)
During the acute phase of illness, any movement of the affected limb causes discomfort to the child; therefore the child is positioned comfortably with the affected limb supported. Moving and turning are carried out carefully and gently to minimize discomfort. The child may require pain medication (see Chapter 7) or sedation. Take vital signs and record them frequently, and implement measures to reduce a significant temperature elevation.
Antibiotic therapy requires careful observation and monitoring of the IV equipment and site. Because more than one antibiotic is usually administered, the compatibility of the drugs must be determined and care taken to avoid mixing incompatible drugs. The stability of the drugs and their toxic nature are also considered when determining the rate of administration. The infusion device must be well situated in the vein to ensure that the drug does not infiltrate into surrounding tissues, where it may produce tissue damage. For long-term antibiotic therapy, a venous access device, such as a peripherally inserted central catheter, is the preferred method of IV administration. (See Chapter 28.)
Standard Precautions are put in effect for children with open wounds, depending on the institution’s policies. The wound is managed according to the practitioner’s directions. Administration of antibiotic solution directly into the wound is most efficiently accomplished using a regular infusion setup that is prepared and regulated in the same manner as for any IV infusion. Intake and output are measured and recorded, and the character of both the wound and drainage is noted. The amount and character of drainage on the wound dressing are also noted.
As the infection subsides, physical therapy is instituted to ensure restoration of optimum function. The child is usually discharged on a regimen of oral antibiotics, and progress is followed closely for some time.
Septic (suppurative) arthritis is a bacterial infection in the joint. It usually results from hematogenous spread or from direct extension of an adjacent cellulitis or osteomyelitis. Direct inoculation from trauma accounts for 15% to 20% of septic arthritis cases. The most common causative organism is S. aureus (Lampe, 2007). Community-acquired methicillin-resistant S. aureus is commonly a cause of septic arthritis (Gutierrez, 2005). In addition to S. aureus, pathogens seen in neonates include group B streptococci, Escherichia coli, and Candida albicans. In children 2 months to 5 years of age, S. aureus, Streptococcus pyogenes, Streptococcus pneumoniae, and Kingella kingae are the primary organisms causing infection, whereas children older than 5 years are more likely to be infected by S. aureus and S. pyogenes; sexually active adolescents may be infected by N. gonorrhoeae (Gutierrez, 2005).
Knees, hips, ankles, and elbows are the most commonly affected joints. Clinical manifestations include severe joint pain, swelling, warmth of overlying tissue, and occasionally erythema. The child is resistant to any joint movement. Features of systemic illness such as fever, malaise, headache, nausea, vomiting, and irritability may also be present.
The affected joint is aspirated and the specimen evaluated by Gram stain, culturing (including separate cultures for H. influenzae and N. gonorrhoeae), and determination of leukocyte count. In addition, perform blood culture and obtain complete blood count with differential and erythrocyte sedimentation rate or C-reactive protein level. Early radiographic findings are limited to soft tissue swelling but may reveal a foreign body, and such films always provide a baseline for comparison. Technetium scans reveal areas of increased blood flow but will not differentiate between sites. MRI and CT scans provide more detailed images of cartilage loss, joint narrowing, erosions, and ankylosis of progressive disease. An infection involving the hip, however, is considered a surgical emergency to prevent compromised blood supply to the head of the femur (Lampe, 2007).
Treatment is IV antibiotic therapy based on Gram stain results and the clinical presentation. The benefits of serial aspirations to demonstrate sterility of synovium fluid and reduce pressure or pain are controversial. Pain management is an important aspect of nursing care, particularly with involvement of a large joint such as the hip. Surgical intervention may also be required if there was a penetrating wound or a foreign object was possibly involved. Physical therapy may be initiated for the child who is immobilized in a cast or traction to prevent flexion contractures. Additional nursing care is the same as for osteomyelitis.
In children tubercular infection of the bones and joints is acquired by lymphohematogenous spread at the time of primary infection. Occasionally it is from chronic pulmonary tuberculosis. Skeletal tubercular infection is not common in the United States but should be considered in communities with high tuberculosis case rates. The infection is most likely to involve the vertebrae, causing a tubercular spondylitis. If the infection is progressive, it causes Pott disease with destruction of the vertebral bodies and results in kyphosis. Symptoms are insidious. The child may report persistent or intermittent pain. Other findings include joint swelling and stiffness; fever and weight loss are not common. Tubercular arthritis can also affect single joints such as a knee or hip and tends to cause severe destruction of adjacent bone. Infection in the fingers causes spina ventosa, a tuberculous dactylitis.
As with pulmonary tuberculosis, the index case should be located. A family and environmental history needs to be obtained and skin tests performed. (See also Tuberculosis, Chapter 32.) Results of tuberculin skin tests are positive for the majority of children with tuberculous arthritis; however, the results are not diagnostic, and the clinical and laboratory features do not differentiate tubercular arthritis from a nontubercular septic arthritis. Diagnosis requires isolation of Mycobacterium tuberculosis from the site. Patients with the susceptible organism start treatment with combined antituberculosis chemotherapy (isoniazid, rifampin, and pyrazinamide); directly observed therapy is preferred.
Nursing care depends on the site and extent of infection. Tuberculous spondylitis and hip infection may require immobilization, casting, and fusion. Nursing care is the same as for osteomyelitis with the addition of isolation requirements.
OI is the most common osteoporosis syndrome in children, characterized by excessive fractures and bone deformity. There are at least five types of OI, accounting for significant disease variability. Clinical features include varying degrees of bone fragility, deformity, and fracture; blue sclerae; hearing loss; and dentinogenesis imperfecta (hypoplastic discolored teeth). The inheritance pattern is autosomal dominant in the majority of cases, although the most severe form demonstrates autosomal recessive inheritance.
Most types of OI have defects in the COL1A1 or COL1A2 genes, which code for polypeptide chains in type 1 procollagen, a precursor of type 1 collagen, a major structural component of bone. The error results in faulty bone mineralization, abnormal bone architecture, and increased susceptibility to fracture.
Classifications for OI are based on clinical features and patterns of inheritance (Box 39-15). Clinically, type I is the most common, with wide variability of bone fragility; some affected family members have significant deformity and disability, whereas others lead agile, active lives. Type II variants are the most severe and are considered lethal in infancy. Type III OI is characterized by multiple fractures, bone deformity, and severe disability; affected individuals rarely live to 30 years of age. Type IV is similar to type I with blue or white sclerae. Another variant, or type V, has been described in which those affected have a hyperplastic callus, a radiodense metaphyseal band, and calcification of the interosseous membrane of the forearm; no collagen mutations are noted in this group (Marini, 2007). A type VI has been described with a characteristic mineralization defect, which does not respond to pamidronate therapy as do types I to V (Land, Rauch, Travers, et al, 2007). Children affected with this type have no dental involvement and normal sclerae; a bone biopsy is the only way to establish a diagnosis because of the similarities to other types.
The treatment for OI is primarily supportive, although patients and families are optimistic about new research advances. Bone marrow transplant for severe OI was first reported in 1999 with positive results; however, this is still considered an experimental treatment. Bisphosphonate therapy with pamidronate, olpadronate, neridronate, or alendronate to promote increased bone density and prevent fractures has become standard therapy for many children with OI. Bisphosphonate therapy reportedly is more beneficial for increasing vertebral bone density but is considered less effective for long bones (Marini, 2007). Others report effectiveness of pamidronate in children with moderate to severe OI (Alharbi, Pinto, Finidori, et al, 2009). Bachrach and Ward (2009) suggest data are inadequate to recommend the use of bisphosphonate therapy in children with OI for sole treatment of bone mineral density reduction. Oral risedronate has been reported to be mildly effective in treating children with type I OI, but is not superior to pamidronate therapy (Rauch, Munns, Land, et al, 2009).
The rehabilitative approach to management is directed to preventing (1) positional contractures and deformities, (2) muscle weakness and osteoporosis, and (3) malalignment of lower extremity joints prohibiting weight bearing. Lightweight braces and splints help support limbs, prevent fractures, and aid in ambulation. Physical therapy helps prevent disuse osteoporosis and strengthens muscles, which in turn improves bone density. Surgery is sometimes used to help treat the manifestations of the disease. Surgical techniques are used to correct deformities that interfere with bracing, standing, or walking. For the child with recurrent fractures, inserting an intramedullary rod provides stability to bones.
Because there is a 50% risk of an affected individual passing the gene to an offspring, genetic counseling is recommended.
Infants and children with this disorder require careful handling to prevent fractures. They must be supported when they are being turned, positioned, moved, and held. Even changing a diaper may cause a fracture in severely affected infants. These children should never be held by the ankles when being diapered but should be gently lifted by the buttocks or supported with pillows.
Children with current fractures or healing fractures should be screened for OI; the assumption that abuse or neglect is the cause of fractures in children must be carefully evaluated by a multidisciplinary team. A detailed history, no evidence of associated soft-tissue injury, and the presence of other symptoms related to OI help determine the diagnosis.
Both parents and the affected child need education regarding the child’s limitations and guidelines in planning suitable activities that promote optimum development and protect the child from harm. Realistic occupational planning and genetic counseling are part of the long-term goals of care. The parents can obtain educational materials and information from the Osteogenesis Imperfecta Foundation, Inc.* which also has a network that can put a family in contact with other families with a similar problem.
Juvenile idiopathic arthritis (JIA), also commonly called juvenile rheumatoid arthritis (JRA), refers to chronic childhood arthritis. JIA is gradually replacing JRA in the research literature and is increasingly used in clinical practice; consequently, both JIA and JRA classifications are discussed here. The revision of JRA nomenclature to JIA was due in part to the minimally applicable reference to “rheumatoid” in childhood arthritis, which is relevant to only a small proportion of affected children yet burdens the family with images of disfiguring adult rheumatoid arthritis. Furthermore, the old subtyping of JRA into systemic, pauciarticular, and polyarticular disease reflects disease onset and not disease progression, which is of greater importance (Warren, Perez, Curry, et al, 2001).
JIA is a group of idiopathic chronic inflammatory diseases affecting the joints and other tissues in approximately 1 in 1000 children. Some theories speculate that the disorder arises when an infectious agent activates an autoimmune inflammatory process in a genetically predisposed child. Although a genetic susceptibility to JIA is known, such as human leukocyte antigen (HLA) polymorphisms and the PTPN22 gene, this accounts for less than half of the genetic susceptibility; additional genetic factors are being researched (Prahalad and Glass, 2008). There is a female predominance of 2:1 and two peak ages of onset: between 1 and 3 years and 8 and 10 years of age.
The disease process is characterized by a chronic inflammation of the synovium with joint effusion and eventual erosion, destruction, and fibrosis of the articular cartilage. Adhesions between joint surfaces and ankylosis of joints occur if the process persists.
Whether a single joint or multiple joints are involved, stiffness, swelling, and loss of motion develop in the affected joints. They are swollen and warm to the touch but seldom red. The swelling results from edema, joint effusion, and synovial thickening. The affected joints may be tender and painful to the touch or relatively painless. The limited motion early in the disease is a result of muscle spasm and joint inflammation; later it is caused by ankylosis or soft tissue contracture. Morning stiffness, or “gelling,” of the joint(s) is characteristic and present on arising in the morning or after inactivity. Infections, injuries, and surgical procedures often precipitate a flare-up of the arthritis; therefore prompt recognition and treatment of infections are necessary.
In severe, long-standing cases growth is significantly restricted. Corticosteroid therapy is also a contributing factor. There may be growth disturbances, either overgrowth or undergrowth, adjacent to the inflamed joints (e.g., altered leg length after knee involvement) and micrognathia (receding chin) from temporomandibular arthritis.
Classification: The older JRA classification has been replaced in pediatric rheumatology centers and in research by JIA, but in many pediatrician offices, the term JRA remains in use; consequently the JRA classification is still pertinent for review. JRA is a variable disease with three major disease courses: systemic onset, pauciarticular (involving few joints, usually less than five), and polyarticular (involving four or more joints simultaneously). The International League of Associations for Rheumatology classification of JIA, developed in 1997, revised and published in 1998, and revised again in 2001, lists seven disease categories, each with its own set of criteria and exclusions: systemic arthritis, oligoarthritis, rheumatoid factor (RF)–negative polyarthritis, RF-positive arthritis, psoriatic arthritis, enthesitis-related arthritis, and undifferentiated arthritis (Petty, Southwood, Baum, et al, 1998; Petty, Southwood, Manners, et al, 2004) (Box 39-16).
The outcome of JIA is variable and unpredictable. Even in severe forms, the disease is rarely life threatening and is significantly different from adult rheumatoid arthritis. Features that distinguish JIA from adult disease include onset before 16 years of age; a negative test result for RF (in 90% of cases); classic symptoms of systemic arthritis such as quotidian fever, rash, and pericarditis; development of uveitis (inflammation of the iris and ciliary body) as a complication (in 8% to 20% of cases); and a tendency for the arthritis to become inactive.
The arthritis tends to wax and wane and eventually becomes inactive in approximately 60% of the cases. These children may have severe or minimal joint damage remaining when active arthritis abates. Forty percent of the children have progressive arthritis into adulthood. Their arthritis can cause significant joint deformity and functional disability requiring medication, physical therapy, and perhaps future joint replacement. Chronic and acute uveitis is an extraarticular complication of JIA that may cause permanent vision loss if undiagnosed and not aggressively treated. Although many children have minimal arthritis, it can produce severe physical, functional, and emotional impairment.
JIA and JRA are diagnoses of exclusion; there are no definitive tests. Both diagnoses are based on the clinical criteria of age of onset before 16 years, arthritis in one or more joints for 6 weeks or longer, and exclusion of other causes (Petty, Southwood, Manners, et al, 2004). Laboratory test results may provide supporting evidence of disease. An elevated sedimentation rate or C-reactive protein may or may not be present. Leukocytosis is frequently present during flares of systemic disease. Tests for RF give positive results in only 10% of the children with JIA. The presence of antinuclear antibodies is common in JIA, but they are not specific for arthritis; however, their presence helps to identify children with pauciarticular disease, who are at greater risk for uveitis.
There is no cure for JIA. The major goals of therapy are to control pain, preserve joint range of motion and function, minimize the effects of inflammation such as joint deformity, and promote normal growth and development. Achievement of these goals requires a family-centered approach with collaboration among the child, the family, and the health care team. The team includes the primary care physician; pediatric rheumatologist; rheumatology nurse educator; social worker; physical and occupational therapists; subspecialists (e.g., pediatric ophthalmologist); and a community of friends, relatives, and teachers. The treatment plan is individualized and varies, but it can be complicated and intrusive, including medications, physical and occupational therapy, slit-lamp eye examinations, splints, comfort measures, dietary management, modification of school activities, and psychosocial support.
Outpatient care is the mainstay of therapy; lengthy hospital admissions for rehabilitation used to be common but are now limited in the era of managed care. Chronic uveitis can cause permanent vision loss, glaucoma, and cataracts. Slit-lamp ophthalmologic examinations at regular intervals are required to diagnose chronic anterior uveitis (iridocyclitis), inflammation of the anterior segments of the eye, iris, and ciliary body. The majority of affected children have a relatively good visual prognosis if the inflammation is detected and treated early; however, most cases are asymptomatic. Consequently, routine slit-lamp examinations are critical. The children at greatest risk for development of uveitis have pauciarticular disease and a positive antinuclear antibody (Cassidy, Kivlin, Lindsley, et al, 2006; American Academy of Pediatrics, 1993).
Medications: A variety of antirheumatic drugs are available, and most are effective in suppressing the inflammatory process and relieving pain. The drugs may be given alone or in combination. NSAIDs are the first drugs used. Common NSAIDs include ibuprofen, naproxen, tolmetin, diclofenac, indomethacin, and meloxicam. (See Table 7-4.) Aspirin, once the initial drug of choice, is seldom used in children. NSAIDs offer an immediate analgesic effect, but the antiinflammatory effect requires larger doses and more time to achieve. The child must take an NSAID for at least 3 weeks before effectiveness can be evaluated. Patient and family education regarding potential gastrointestinal, renal, and hepatic side effects and reduced clotting is essential. Parents should monitor the child for abdominal pain and blood in the stool. Naproxen has the potential side effect of skin fragility in individuals with fair skin so families need to take precautions regarding sun exposure and report unusual skin lesions.
Additional medication is required in approximately 65% of children with arthritis. The agents used are slower-acting antirheumatic drugs (SAARDs) and include methotrexate, sulfasalazine, and hydroxychloroquine. Weekly low-dose methotrexate therapy is usually the first SAARD regimen used. Families may be overwhelmed by the potential adverse effects, including liver disease, bone marrow suppression, gastrointestinal disturbance, teratogenic effects, and the alarming but unconfirmed risk of carcinogenesis. Methotrexate is effective, however, and the potential benefits outweigh the potential risks. Methotrexate therapy has also improved uveitis in children with uveitis resistant to steroid treatment (Foeldvari and Wierk, 2005). Laboratory monitoring of liver enzyme levels and blood counts is crucial. A daily folic acid supplement can help reduce the occurrence of oral ulcers. Taking methotrexate at bedtime may help reduce nausea.
Frank discussion about sexual activity and birth defects is critical. Sexually active teenagers need effective birth control and documented periods, as well as pregnancy tests if periods are not regular. As a precaution, pregnant caregivers and those trying to conceive need to avoid contact with methotrexate.
Alcohol consumption is another sensitive topic that needs to be discussed honestly because it increases the risk of hepatotoxicity. Most children require both an NSAID and methotrexate, and parents may be notified by pharmacists about combination toxicity. This is a known interaction of which the rheumatologist is aware; however, instruct patients to avoid additional over-the-counter NSAIDs and to take acetaminophen for episodes of fever. They should also avoid sulfa antibiotics and other bone-marrow–suppressing drugs. Parents should always discuss methotrexate drug interactions with providers prescribing medications for interval illness. During some illnesses, especially varicella, methotrexate should be discontinued because it can suppress the immune response. Sulfasazaline may be selected as the first SAARD in children with axial arthritis, a positive test result for HLA-B27, or symptoms of inflammatory bowel disease, given this drug’s success in these select groups of patients.
Corticosteroids are the most potent antiinflammatory agents; however, they will not cure arthritis, and the significant adverse effects of long-term steroid use are undesirable. Steroids are administered when other medications have failed to control a disease flare and the child has substantial physical disability. Prednisone is given orally in a burst or at the lowest effective dosage. Use of an alternate-day schedule may help reduce side effects. High-dose IV steroids may provide sustained improvement for children with severe arthritis and pericarditis associated with systemic disease. Intraarticular injections of long-acting steroids have proven effective in treating limited arthritis with minimal adverse effects. Children may require conscious sedation or general anesthesia, which affects risk-benefit considerations, but it is critical to have a cooperative patient for good procedure outcome.
Biologic Agents: Etanercept and adalimumab are approved for use in children with JIA. Biologic agents are typically used in children with moderate to severe arthritis after unsuccessful treatment with NSAIDs and methotrexate. Etanercept blocks the binding of tumor necrosis factor (TNF) with cell surface receptors, thereby reducing proinflammatory activity. Adalimumab is a monoclonal antibody TNF blocking agent, which also reduces the proinflammatory response that promotes arthritis. Studies have shown etanercept and adalimumab to be effective and well tolerated (Lovell, Giannini, Reiff, et al, 2003; Lovell, Ruperto, Goodman, et al, 2008).
Although etanercept and adalimumab have been found safe and effective, parents need to inform providers of any unusual symptoms in the child given the relatively limited experience with these drugs and the potential for long-term side effects (Lovell, Reiff, Ilowite, et al, 2008; Burmester, Mease, Dijkmans, et al, 2009). The potential for malignancy, particularly lymphoma, is continuing to be monitored in children on TNF blockers. Increased infection risk is the most common adverse effect. Parents should withhold etanercept and adalimumab during a concurrent infection and promptly report symptoms of infection to their provider for assessment and treatment. A negative tuberculin skin test should to be obtained before starting etanercept and adalimumab; yearly follow-up skin testing has been suggested. Other biologic agents that interrupt cytokine activity are approved by the U.S. Food and Drug Administration for the adult rheumatoid arthritis population and are available off label and may be useful in treating children with JIA (Ilowite, 2008).
The cytotoxic agents cyclophosphamide, azathioprine, cyclosporine, and chlorambucil have been used to treat severe refractory arthritis that has not responded to other medications. Experience is limited with these drugs, and the toxicity risks and potential benefits are not well defined.
Physical Management: Programs of physical management are individualized for each child and are designed to reach the ultimate goal of preserving function and preventing deformity. Physical therapy is directed toward specific joints and focuses on strengthening muscles, mobilizing restricted joints, and preventing or correcting deformities. Occupational therapists assume responsibility for increasing general mobility and improving performance of activities of daily living.
General treatment and maintenance programs vary. Physical therapists may be involved several times weekly to monthly in management of a home program, or their visits may be limited to infrequent review of the home program for compliance, effectiveness, and need. Muscle strength is frequently lost around the involved joints, and inactivity leads to generalized weakness. However, performance of the normal activities of daily living and the child’s natural tendency to be active are usually sufficient to maintain muscle strength and joint mobility. Unless there is a specific risk of injury related to arthritis, the child should not be restricted from regular play, dance, exercise programs, and even individual and team sports. Activity modifications may be needed to accommodate joint limitations, but exercise should be encouraged; a sedentary lifestyle contributes to a deconditioned state, which further limits physical activity and ultimately influences the child’s quality of life (Klepper, 2008).
Exercising in a pool is excellent because it allows freedom of movement with support. When joints are inflamed, heavy resistance aggravates the pain. At such times, simple isometric or tensing exercises that do not involve joint movement are generally tolerated and should be encouraged. Range-of-motion exercises are an important aspect of therapy and are continued after evidence of disease has disappeared in order to detect any signs of recurrence.
Practitioners may recommend splinting and positioning during rest to help minimize pain and prevent or reduce flexion deformity. Joints most frequently splinted are knees, wrists, and hands. Positioning during rest is also important. The child should rest on a firm mattress with no pillow or a very low one. Loss of extension in the knee, hip, and wrist causes special problems. Vigilance is required to detect the earliest signs of involvement, and vigorous attention must be given to specialized passive stretching, positioning, and resting splints to prevent deformity.
Surgery: The benefits of synovectomy, an established preventive and therapeutic procedure in adults, are questionable in children with arthritis. Synovectomy is used primarily in pauciarticular disease. In cases of synovitis, intraarticular steroid injection is an alternative to synovectomy and may be tried once or twice before surgery is performed. Joint replacement is proving to be successful in older children who are fully grown.
Nursing care of children with JIA involves assessment of their general health, the status of involved joints, and their emotional responses to all of the ramifications of the disease—pain; physical restrictions; therapies; and self-concept, especially in preadolescents and adolescents.
The effects of the disease are manifested in every aspect of the child’s life, including physical activities, social experiences, and personality development. Children’s adjustment to the stresses and demands of the disease and the level of functioning they achieve are related largely to the reaction and support they receive from their family and the health care professionals involved in their care and management (see Nursing Care Plan).
Relieve Pain: Multiple factors influence the pain of arthritis: disease severity, functional status, individual pain threshold, family variables, and psychologic adjustment. Although complete pain relief is desirable, it is probably unrealistic. The aim is to provide as much relief as possible with antiinflammatory medication and other therapies to help children tolerate the pain and complete the activities of daily living. At present, opioid administration is not a routine therapy for the chronic pain of arthritis. Nonpharmacologic modalities such as relaxation may be helpful. (See Pain Management, Chapter 7.)
Diet and Exercise: The general health of children with arthritis and their siblings must be considered but may be overlooked as parents and health personnel concentrate on the disease. Maintenance of a well-balanced diet and assessment of nutritional status are integral parts of health supervision. A daily children’s complete multivitamin with iron is a reasonable dietary supplement, but there is no “arthritis diet” or foods to avoid that are specifically associated with arthritis. Unfortunately children with arthritis have not been spared the nationwide obesity epidemic. After assessing the child growth chart, make a referral to a dietitian for children who are underweight or overweight due to malnutrition. Excessive weight causes additional strain on inflamed joints. Joint pain during or after exercise impedes active play, which perpetuates the vicious cycle of inactivity and weight gain. Encourage daily physical exercise, starting with a gradual program of walking and slowly advancing to more active play as tolerated. During the school year this can be accomplished with participation in physical education classes as tolerated. When school is out, parents and children should devise a family plan for exercise that includes a variety of options such as games, sports, dance, yoga, swimming, bike riding, and walking. This builds good habits for an active lifestyle for the entire family.
Sleep and Rest: Children with JIA report frequent disrupted nighttime sleep, daytime sleepiness, fatigue, and sleep anxiety (Bloom, Owens, McGuinn, et al, 2002). Restorative sleep is essential. Children should get 8 to 10 hours of nighttime sleep. Daytime naps are discouraged, especially because inactivity provokes stiffness and prolonged naps can interfere with sleepiness at bedtime. Fatigue should be handled with rest rather than sleep. Thirty to 60 minutes of relaxation—viewing television, reading, playing video games, using the computer, or listening to music—is refueling and less likely to disrupt nighttime sleep than a nap. Use of a firm mattress to maintain alignment of spine, hips, and knees is recommended. A thin pillow or no pillow better aligns the spine. Lying prone during rest is encouraged to straighten the hips and knees. Children with joint contractures may wear nighttime splints, which may take some time to get used to but should not be painful and should not hamper falling asleep.
Encourage School Attendance: School-aged children should attend school, even on days when there is joint pain. Staying home and lying around will not improve arthritis. If joint pain and stiffness prevent school attendance, the rheumatologist should be notified and the child assessed. The rheumatology team can make recommendations to the school to maximize attendance and participation. Enlisting the school nurse to administer scheduled medications and as-needed analgesics such as acetaminophen for pain rescue will help keep the child at school. Requesting one set of books for use at home and one for the classroom eliminates the need for heavy backpacks and the subsequent strain on arthritic joints. The child’s participation in the physical education program as tolerated is another way to maximize attendance and performance. If more extensive modifications to the school routine are indicated, the development of an individualized education plan for the school setting (see Chapter 24) may ensure that the child’s needs are met. Examples of provisions included in such plans are half-day programs, special transportation, and in-school physical therapy. Although home teaching is rarely indicated, in some circumstances it is necessary; it is initiated with the goal of returning the child to the classroom as soon as possible.
Facilitate Compliance: For any medical or physical plan of therapy to be effective, the family must agree to it and understand the benefits of treatment and the problems associated with noncompliance. Review a simple written list of exercise benefits and complications of joint immobility. At the outset, elicit barriers to a plan from the child and parents. If a child cannot swallow pills or refuses injections, the given modality is not acceptable. If parents know they cannot monitor or enforce a complicated medication and physical therapy schedule, the plan needs to be simplified to honestly reflect the actual care that is being provided. If joint range-of-motion exercises are too painful and emotionally difficult for parents to implement despite use of pretherapy analgesics and comfort measures, then physical therapy home visits or outpatient physical therapy sessions need to be considered.
In addition to developing a plan that is attainable, recognizing a child’s compliance by providing inexpensive weekly or monthly rewards fosters goal setting and ultimately boosts self-esteem. It is not just the child who needs therapy rewards; the nonprimary child care provider needs to step in and offer relief to the primary caregiver to prevent burnout, as well as to verbally acknowledge the extra parenting responsibilities being performed.
Everyone needs reminders; a simple written schedule of medications and physical therapy exercises should be provided and reviewed with the family at each visit.
Encourage Comfort Measures and Activities of Daily Living: Application of heat has been beneficial to children with arthritis. Moist heat is best for relieving pain and stiffness, and the most efficient and practical method is via the bathtub. The temperature and duration of the bath are specified by the therapist but usually do not exceed 10 minutes at 37.8° C (100° F). Sometimes a daily whirlpool bath, paraffin bath, or hot packs may be used as needed for temporary relief of acute swelling and pain. Hot packs are easily applied at home using a towel that is wrung out after being immersed in hot water or heated in a microwave oven, applied to the area, and covered with plastic for 20 minutes. Painful hands or feet can be immersed in a pan of water for 10 minutes two or three times daily as an adjunct to tub baths.
Pool therapy is the easiest method for exercising a large number of joints. Swimming activities strengthen muscles and maintain mobility in larger joints. Very small children who are frightened of the water can perform their exercises in the bathtub. Small children love to splash, kick, and throw things in the water.
Activities of daily living provide satisfactory exercise for older children to maintain maximum mobility with minimum pain. These children should be encouraged in their efforts and patiently allowed to dress and groom themselves, to assume daily tasks, and to care for their belongings. It is often difficult for stiff fingers to manipulate buttons, comb or brush hair, and turn faucets, but parents and other caregivers should not readily offer assistance. In addition, children should be helped to understand why others do not assist them. Many helpful devices, such as self-adhering fasteners, tongs for manipulating difficult items, and grab bars installed in bathrooms for safety, can be used to facilitate tasks. An elevated toilet seat often makes the difference between dependent and independent toileting, since weak quadriceps muscles and sore knees inhibit the ability to raise the body from a low sitting position.
A child’s natural affinity for play offers many opportunities for incorporating therapeutic exercises. Throwing or kicking a ball, hanging from monkey bars, and riding a tricycle (with the seat raised to achieve maximum leg extension) are excellent moving and stretching exercises for a young child whose daily living activities are physically limited.
An effective approach to beginning the day’s activities is to awaken children early to give them their medication and then allow them to sleep for an hour. On arising, children take a hot bath (or shower) and perform a simple ritual of limbering-up exercises, after which they commence the activities of the day, such as going to school. Exercise, heat, and rest are spaced throughout the remainder of the day according to individual needs and schedules. Instruct parents in exercises that fit the needs of the child.
The Arthritis Foundation and the American Juvenile Arthritis Organization* (an organization within the Arthritis Foundation) provide information and services for both parents and professionals, and nurses can refer families to these agencies as an added resource.
The Child: Arthritis affects every aspect of the child’s daily life. The physical pain and limitations interfere with performance of normal tasks and provision of self-care. Even simple tasks, such as dressing, combing the hair, using the bathroom, cutting food, climbing stairs, manipulating doors and faucets, and using public transportation, are difficult or impossible. The child may have school difficulties related to transportation to and from school, stair climbing, and loss of time as a result of exacerbations and hospitalization. Physical limitations interfere with participation in many activities, both curricular and extracurricular, which limits peer contacts and interaction and increases social isolation. These problems are especially critical for adolescents, for whom peer acceptance and relationships are vital to personality development (see Family-Centered Care box). Many children with arthritis increasingly turn to solitary activities and to the family at a time when they are expected to move into greater independence and relationships with peers.
Changes in personality may accompany JIA, as with any chronic illness. These changes may be temporary, such as demanding, irritable behavior, or may be persistent, such as passive hostility, uncommunicativeness, and manipulativeness. Families need confirmation that adjusting to a chronic illness is difficult. Consider and encourage support referrals to social workers, counselors, and psychologists. (See Chapter 22.)
The Family: The beginning of the disease is often sudden and frightening for the family, and its variable course with cycles of remission and exacerbation is discouraging. Many parents become susceptible to experimenting with unorthodox cures advanced by advertisers and well-meaning friends. Access to the Internet and more than 200 sites dedicated to arthritis provides families a welcome relief to the isolation of living with arthritis, but the Internet is also a bottomless source of unsubstantiated information that necessitates frank discussion and review with the family to help them sort out what is opinion versus fact, and safe versus dangerous. Sometimes health care providers do not know the benefits or risks of a nutritional, herbal, or other complementary therapy. It is hoped that the new surge of legitimate scientific investigations of these remedies will provide future answers. Carefully evaluate these therapies. Obviously harmless measures such as wearing a copper bracelet need not be discouraged, but dissuade parents from engaging in questionable or obviously harmful practices. Parents’ understanding of the disease and their attitude toward the child can determine the success or failure of a treatment program, and major focuses of nursing intervention are parental education and support.
Nurses should be alert to cues that signal undue anxiety and guilt, which may lead to unhealthy overprotection, such as preoccupation with causative factors, constant analysis of the effects of various therapies, experimentation with diets, and continual searching for a magical cure. Parental overprotection and overindulgence can be especially harmful to the child’s progress. Sometimes parents avoid prescribed medications, keep the child home from school unnecessarily, restrict interaction with age-mates, do not discipline the child, and assume self-care activities that are best performed by the child.
Parents and patients need to hear that nurses promote independence. The child’s participation in extracurricular activities, including play with friends, scouting, youth groups, dancing, and sports, is recommended. Nurses also support assigning children family chores and allowing older children to hold a part-time job, which foster self-reliance.
Most of the reactions, problems, and concerns of families of a child with JIA are those of any parents of a child with a chronic illness or disability. The impact of the diagnosis is felt most acutely by the parents, who demonstrate anxiety, guilt, and all the manifestations of the grief process. The concerns and needs of these families are discussed extensively in Chapter 22, and the reader is directed to this chapter for additional guidance in planning care.
Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disease of the blood vessels and connective issue. The Lupus Foundation of America (2009) estimates 1.5 million individuals have lupus, and 10% to 15% of these adults were diagnosed with SLE as children or adolescents. It typically manifests between the ages of 10 and 19 years, and onset before age 5 years is unusual. There is a 5 : 1 female/male predominance.
Its course and symptoms are variable and unpredictable, with mild to life-threatening complications. SLE in children tends to be more severe at onset and has a more aggressive clinical course than adult-onset disease. Other types of lupus erythematosus include chronic cutaneous lupus erythematosus (discoid lupus erythematosus), drug-induced lupus erythematosus, subacute cutaneous lupus erythematosus, and neonatal lupus erythematosus. Neonatal lupus erythematosus occurs when maternal autoantibodies cross the placenta and cause transient lupuslike symptoms in the newborn, with the potential lethal complication of heart block. The remaining discussion focuses on SLE.
The cause of SLE is not known. It appears to result from a complex interaction of genetics with an unidentified trigger that causes the disease to activate. Suspected triggers include exposure to ultraviolet light, estrogen, pregnancy, infections, and drugs. Although a specific gene or genes have not yet been identified as the cause of SLE, an expanding volume of genetic research has identified specific loci associated with SLE.
There is a documented genetic predisposition to SLE as evidenced by an increased concordance rate in twins (tenfold), increased incidence within family members (10% to 16%), an increased frequency of certain gene alleles in population-based studies, and increased risk of autoimmune disease in first-degree relatives of individuals with SLE (Priori, Medda, Conti, et al, 2003; Tsao and Wu, 2007). The incidence of pediatric SLE is also increased in Hispanic, Asian, and African-American pediatric populations.
SLE is the result of an abnormal immune response causing production of abnormal antibodies and formation of immune complexes. These immune complexes are deposited in tissues, causing inflammation and inciting other proinflammatory mediators that result in tissue injury and damage. Immune complex deposition in the glomerulus of the kidney causes lupus nephritis, a life-threatening complication of SLE. Almost any tissue in the body can be damaged by this abnormal inflammatory response, including the brain, heart, lungs, liver, gastrointestinal tract, spleen, joint tissues, muscles, and skin.
The onset of SLE can be insidious, with intermittent constitutional symptoms such as fever, fatigue, weight loss, and arthralgia. However, rapid involvement of vital organs, primarily the kidneys, can herald an accelerated course with potentially fatal outcome. Recent reports suggest that survival rates in children with SLE have significantly improved; 5-year survival rates are said to be almost 100%, and 10-year survival rates are close to 90% (Ravelli, Ruperto, and Martini, 2005).
Box 39-17 lists the manifestations related to the various tissues involved.
Rash is a common feature in SLE. The erythematous malar “butterfly” rash that spares the nasolabial fold is a suggestive feature but not pathognomonic. Maculopapular rashes are frequent and can occur anywhere but typically are found on sun-exposed skin. Nails and hair can be involved, with red, cracked cuticles; periungual telangiectasia; and patchy or diffuse alopecia. Raynaud phenomenon, or spasm of the blood vessels, causes cool hands and feet with pain and a characteristic tricolor (purple or blue–white-red) change. Raynaud phenomenon usually appears as a response to cold exposure and can cause significant tissue damage. In addition to color changes in the extremities, vascular necrosis and digital ulceration can occur. Arthritis and tenosynovitis are common in SLE. The arthritis is usually painful and typically of short duration; joint deformity is unusual.
Renal involvement is a serious complication caused primarily by deposition of circulating immune complexes in the glomerular basement membrane with cellular infiltrates. Lupus nephritis is usually asymptomatic; consequently, monitoring of urine and renal function is required to detect disease. Kidney biopsy is required for lupus nephritis classification. There are six classes depending on the type and extent of the renal lesion. Specific treatment is based on the class of nephritis. Although outcomes have improved for children with renal disease, the course is difficult to predict. Some children get better, whereas some remain the same or progress to renal failure requiring dialysis and transplantation.
Neuropsychiatric lupus is another serious complication found in approximately 25% of pediatric SLE patients. The majority of these children manifest central nervous system involvement within the first year of diagnosis (Benseler and Silverman, 2007). Symptoms can vary from manifestations as subtle as inability to concentrate to frank psychosis and seizure. Assess school performance and emotional stability at each visit as possible indicators of central nervous system involvement.
Cardiovascular disease results in significant mortality and morbidity in lupus. Young women with SLE ages 35 to 45 years are 50 times more like to have a myocardial infarction compared with population controls (Manzi, Meilahn, Rairie, et al, 1997). The immune dysregulation of SLE directly contributes to premature atherosclerosis. Children with SLE have an increased rate of dyslipidemia (Hayata, Borba, Bonfa, et al, 2005). Treatment includes exercise and dietary changes to promote a healthy weight, cardiovascular fitness, and management of hypertension. Studies are currently underway to assess the usefulness of statins in pediatric lupus for future evidence-based treatment of premature atherosclerosis in SLE (Sandborg, Ardoin, and Schanberg, 2008).
SLE is a clinical diagnosis supported by specific abnormal results on laboratory tests. The American College of Rheumatology criteria for the classification of SLE in adults has a sensitivity of 96% and a specificity of 96% if 4 of the 11 criteria are present (Box 39-18). The SLE workup includes an extensive history taking and physical examination with inquiry about school performance and behavior change. Initial laboratory tests include complete blood count with differential; comprehensive metabolic chemistry panel; microscopic urinalysis; rapid plasma reagin test; quantitative determination of immunoglobulin levels; and tests for antinuclear antibodies, anti–deoxyribonucleic acid antibodies, complement 3 (C3), complement 4 (C4), lupus anticoagulant, and antiphospholipid antibodies.
A diagnosis of lupus should not be made without consideration of all medications being taken and their side effects. Some commonly used drugs such as minocycline, procainamide, hydralazine, and chlorpromazine can cause lupuslike symptoms. Minocycline, a common acne treatment, may not be considered important by the teenager and omitted from the history, so an accurate recent past and present medication history is essential for treatment. Drug-induced lupus resolves with time after the triggering medication has been discontinued (Tucker, 2007).
There is no cure for SLE; the management goal is to reverse or minimize disease activity with appropriate medications while helping the child and family cope with the complications of the disease and treatment.
Medications: Since the 1950s, corticosteroids have been the mainstay of SLE therapy. They are effective antiinflammatory and immunosuppressive agents. Unfortunately, the use of steroids is hampered by side effects, which include growth delay, decreased resistance to infection, osteoporosis, weight gain, hypertension, development of cushingoid features and cataracts, and diabetes risks. Generally, a dosage sufficient to control symptoms is prescribed, and then the dosage is tapered to the lowest level possible to achieve an acceptable balance between disease activity and steroid side effects. For severe disease IV pulse (high-dose) steroids are given on an intermittent schedule, which may allow reduction in the daily steroid dose with better compliance and fewer cushingoid features (Klein-Gitelman and Pachman, 1998). Topical steroids are used for cutaneous lesions, but prolonged therapy thins the skin; consequently, facial application needs to be brief or with medication of lower concentration. Because of increased immunosuppression with steroid use, a tuberculin skin test should be performed before starting steroid therapy, especially in high-risk communities. A medical identification tag should be worn by children undergoing chronic steroid therapy so that administration of stress steroids can be considered in emergency situations.
Other medications used include NSAIDs such as naproxen and ibuprofen for pain associated with arthritis, arthralgia, and myalgia. Nurses need to instruct patients to take NSAIDs with food to help prevent gastrointestinal side effects. Hydroxychloroquine, an antimalarial drug, is an effective therapy for skin and joint manifestations. Possible untoward effects include skin, gastrointestinal, and retinal toxicity. A complete ophthalmologic examination is indicated before treatment begins and every 6 months thereafter. Methotrexate may be used in patients with stubborn arthritis that has not responded to NSAIDs and hydroxychloroquine and allows a lower dose of glucocorticoids to be used. Azathioprine, another steroid sparer, has been useful in treatment of SLE thrombocytopenia. Both methotrexate and azathioprine have significant potential adverse effects, including potential for increased infection, malignancy risks, liver and lung toxicity, and birth defects. Well-documented discussions of these risks with patients and parents are required.
Cyclophosphamide, a potent immunosuppressive chemotherapy agent, used in combination with corticosteroids, is effective in treating proliferative lupus nephritis and neuropsychiatric lupus. A detailed cyclophosphamide education session should be held for patient and family to clearly describe potential benefits and risks, including infertility and future malignancy.
Mycophenolate mofetil, a purine inhibitor, has been used with success in adult lupus nephritis and is currently being used as a steroid-sparing agent in active pediatric lupus and as maintenance therapy after standard cyclophosphamide treatment for lupus nephritis. It is also being evaluated as an alternative to standard treatment of lupus nephritis with cyclophosphamide (Paredes, 2007). Mycophenolate mofetil is an attractive alternative, if successful, because it is less toxic and better tolerated than cyclophosphamide; however, it is not without potential side effects, including increased infection risk, liver toxicity, and birth defects.
New biologic treatments are being developed that focus on the immune dysregulation in SLE, cancer, and other autoimmune diseases. Rituximab is a monoclonal antibody that eliminates CD20-positive B cells without affecting early B cells or plasma cells. This results in decreased antibody formation and has been used off label in pediatric lupus patients who have not responded to standard therapy (Nwobi, Abitbol, Chandar, et al, 2008).
General Measures: In addition to medication, treatment includes general measures such as patient and family education, rest and exercise, proper diet, sun avoidance, and social support.
SLE is complex and requires ongoing patient education. Families and patients need up-to-date, understandable information so they can become informed decision makers and participate in disease management. Nurses are duty bound to discuss information families bring to appointments from the Internet, friends, and family. As health care providers critically evaluate disease information with families, families learn the skills needed to become self-advocates. Families also want to hear about the impact of SLE on growth and development, childbearing, schooling, and career choice. The message should be optimistic and clear, with few exceptions: “Prepare for the future; you will attend school, graduate, have children, and work.”
Diet, exercise, and rest are the daily elements under direct patient control. The family needs to maximize the power of these normal functions to their benefit. There is no specific SLE diet, but a balanced diet that does not exceed calorie expenditure is essential for maintaining appropriate weight on corticosteroid therapy. A low-salt diet may be required if the patient becomes nephrotic or hypertensive. A low-fat diet is indicated in children with dyslipidemia. Maximizing peak bone mass in adolescent SLE is essential, especially since both SLE and its treatment with glucocorticoids increase the risk of osteoporosis. A diet rich in calcium and vitamin D is essential to prevent osteoporosis. If dietary calcium is not sufficient, calcium and vitamin D supplements need to be recommended. Consultation with a registered dietitian will help the family develop an individualized diet that meshes with their lifestyle.
The benefits of a regular exercise program include weight maintenance, cardiovascular fitness, and osteoporosis prevention, all of which help minimize SLE complications and corticosteroid side effects. Unfortunately many children stop participation in sports after diagnosis. Children with SLE report more fatigue and have lower aerobic fitness (Houghton, Tucker, Potts, et al, 2008). Encourage continuation of sports and recreational activities, if possible; if not, try to modify the activity or find alternatives to encourage the child and parents to view daily exercise as an essential part of the treatment plan and a continuation of the child’s normal lifestyle. Additional rest is necessary during disease exacerbations but not to the extent that it interferes with regular sleep patterns.
Given the frequency of photosensitive rash, the dangers of excessive ultraviolet A and B light exposure (including exposure to uncovered fluorescent lights) need to be stressed. This can be a sensitive topic for sun-loving teenagers and outdoor athletes. Discuss the use of sunscreen (with an SPF [sun protection factor] ≥30), hats, and protective clothing. Cosmetics and moisturizers that contain sunscreen are attractive options for adolescent girls. One useful rule to share with the adolescent who may be surrounded by peers who regularly seek out sun exposure is the “slip, slop, slap” rule: slip on a shirt, slop on sunscreen, and slap on a hat before going out in the sun. (See Chapter 18.) Scheduling outdoor activities in the morning and evening can reduce exposure without limiting participation in recreational activities. Make every effort to encourage children to participate in peer activities and to make sun-shielding modifications as inconspicuous as possible.
Social support from family, friends, teachers, counselors, and professional social workers and therapists can help the child and family through difficult times and promote adaptation to an illness that is not going to go away. Destructive coping mechanisms need to be identified and replaced with behaviors that enhance adaptation and healthy outcomes. Organizations that can help children and families learn about and adjust to the disease are the Lupus Foundation of America* and the Arthritis Foundation† (see p. 1682).
Fostering adaptation and self-advocacy is the primary nursing goal. Patient and family acceptance and understanding of this life-threatening and therapeutically intrusive disease are big challenges for any nurse. Patient education is started at diagnosis and continued at every opportunity; repetition is good. Encourage family members to call with questions and concerns. Advise patients to write down their questions so they are prepared during the appointment. Consider adolescent development, with heightened concerns about body image and looking different. The nurse should be open about this. Skin care, cosmetics, and unobtrusive moisturizers with sunscreen and sun block should be discussed.
Weight gain is an emotional issue, and it must be approached honestly with a workable plan for family dietary changes and a realistic exercise program. The nurse should work with a dietitian and a trainer or physical therapist to individualize a nutrition and fitness program for the child. A parent or sibling should be involved in the program, so the child does not feel that restrictions are punitive.
With older children, sexual activity and birth control must be discussed, especially because pregnancy is a potential trigger for disease flare. Honest discussion about healthy, responsible reproductive choices with both the teenager and parents is important for establishing communication. The parents and teenager should know that the teenager can come to the nurse with reproductive concerns. Because estrogen can trigger disease flare, low-dose estrogen or progestin-only oral contraceptives are preferred. Some teenagers choose the compliance-friendly Depo-Provera (medroxyprogesterone) 3-month injections. Again, frank discussion about risks and benefits is essential.
Prevention of infection includes hand washing (especially at school) and preprocedure antibiotic coverage for routine events such as dental cleaning. Immunization vaccinations should be maintained in SLE, except live and attenuated vaccines should be withheld in patients on immunosuppressive therapy.
To compensate for the side effects of some drugs such as the corticosteroids, teenagers often go on fad or starvation diets. It is critical that nutritional counseling be available to ensure that the adolescent understands the role that a healthy diet plays in the management of SLE. School attendance may decrease because of loss of self-esteem, depression, feelings of inadequacy, or poor academic performance. Assessment of common adolescent risk taking behaviors, including tobacco and recreational drug use, needs to be performed as it would for any teenage patient. Treatment compliance is a significant issue in adolescence, especially given the medication side effects and restrictions on sun exposure. Adolescents need to understand what the function of each drug is, how each drug helps to manage the disease, and what effect missing doses may have on their health. The nurse needs to keep track of prescription refills to evaluate compliance and medication efficacy. Barriers to medication compliance should be investigated, and patients should be helped to devise a workable plan. If friends are going to the beach, the patient should consider going late in the day with a beach umbrella, sun block, and a wide-brim hat. Give instructions to reapply sun block after swimming and to limit time in direct sun. (See Chapter 18.)
Teaching patients how to find ways to adapt positively to SLE with normal growth and development as the goal will give them self-advocacy skills. Nurses should apply the principles of adjusting to a chronic illness that are discussed in Chapter 22.
• Trauma is the leading cause of death in children and is caused by accidental injury, child abuse injury, and birth injury.
• Immobility has a profound effect on all elements of growth and development.
• The major consequences of immobilization are loss of muscle strength, endurance, and muscle mass; bone demineralization leading to osteoporosis; circulatory stasis and thrombus formation; loss of joint mobility; and contractures.
• In the care of the immobilized child, nurses are concerned with position changes, adequate dietary intake, adequate hydration, promotion of activity, and involvement of the child in self-care.
• Features of children’s bones not observed in those of adults include presence of a growth plate, a thicker and stronger periosteum, greater bone porosity, more rapid healing, and less joint stiffness.
• Types of fractures seen in children include bend, buckle, greenstick, and complete fractures.
• Goals of fracture management in children are to reestablish alignment and length of the bony fragments, retain alignment and length, and restore function to injured parts.
• The method of fracture reduction is determined by the child’s age, the degree of displacement, the amount of overriding bone, the amount of edema, the condition of the skin and soft tissues, and the integrity of sensation and circulation distal to the fracture.
• The primary purposes of traction are to fatigue involved muscle and reduce muscle spasm, to realign bone ends, and to immobilize the fracture site until realignment has been achieved to permit casting or splinting.
• Complications of fractures are circulatory impairment, nerve compression syndromes, compartment syndromes, epiphyseal damage, nonunion, malunion, infection, kidney stones, and pulmonary emboli.
• Participation in sports predisposes adolescents to acute injuries, such as contusions, dislocations, sprains, and strains, and to overuse syndromes, such as stress fractures.
• Health concerns associated with sports include menstrual dysfunction, substance misuse, and sudden death from sudden cardiac arrest.
• Musculoskeletal disorders in children include torticollis, Legg-Calvé-Perthes disease, SCFE, kyphosis, lordosis, and scoliosis.
• Observation for idiopathic scoliosis is an important part of a routine physical assessment.
• Management of idiopathic scoliosis includes bracing or surgery.
• Postoperative nursing care of the child with idiopathic scoliosis demands careful attention to peripheral neurovascular function, respiratory function, pain control, and skin care.
• Nursing care of the child with osteomyelitis is directed at positioning for comfort, administering antibiotics, monitoring IV equipment and site, and ensuring adequate nutrition.
• Osteomyelitis is acquired by direct or secondary invasion or hematogenous spread of infectious organisms.
• Goals of therapy for arthritis in children are to control pain, preserve joint range of motion and function, minimize the effects of inflammation such as joint deformity, and promote normal growth and development.
• Nursing care of the child with arthritis consists of relieving pain, promoting general health, preventing deformity, preserving optimum function, and encouraging self-care and maximum involvement in school and other activities.
• SLE is a chronic multisystem autoimmune disorder that affects the blood vessels and connective tissues of the body.
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*SafeKids USA is an excellent resource for those interested in child safety; www.usa.safekids.org.
*Basic guidelines for dietary intake can be accessed at www.MyPyramid.gov. This interactive site allows the individual to enter her or his age, sex, and average exercise pattern to see the types of food and total estimated number of calories that should be consumed. Athletes may use this site as a basis for formulating a balanced diet and add the recommended number of calories for athletic activity.
*American Academy of Pediatrics: Female athlete triad, Sports Shorts, Issue 8, July 2002, available at www.aap.org/sections/sportsmedicine/SportsShorts.cfm.
*Five Cabot Place, Stoughton, MA 02072; 800-673-6922; www.scoliosis.org.
†6300 N. River Road, Rosemont, IL 60018-4262; 847-823-7186; www.aaos.org.
‡555 E. Wells St., Suite 1100, Milwaukee, WI 53202; 414-289-9107; www.srs.org.
*804 W. Diamond Ave., Suite 210, Gaithersburg, MD 20878; 800-981-2663; www.oif.org.
*PO Box 7669, Atlanta, GA 30357; 800-283-7800; www.arthritis.org. In Canada: The Arthritis Society, 393 University Ave., Suite 1700, Toronto, Ontario, Canada M5G 1E6; 416-979-7228; fax: 416-979-8366; www.arthritis.ca.
*2000 L St. NW, Suite 710, Washington, DC 20036; 202-349-1155; fax: 202-349-1156; www.lupus.org.
†A recommended booklet available from the Arthritis Foundation is Meeting the Challenge: A Young Person’s Guide to Living with Lupus.