Cushing Syndrome

Cushing syndrome is a characteristic group of manifestations caused by excessive circulating free cortisol. It can result from a variety of causes, which generally fall into one of five categories (Box 38-12 and Table 38-2). Cushing syndrome in young children may be due to an adrenal tumor (Moshang, 2003).

BOX 38-12   ETIOLOGY OF CUSHING SYNDROME

Pituitary—Cushing syndrome with adrenal hyperplasia, usually attributed to an excess of adrenocorticotropic hormone (ACTH)

Adrenal—Cushing syndrome with oversecretion of glucocorticoids, generally the result of adrenocortical neoplasms

Ectopic—Cushing syndrome with autonomous secretion of ACTH, most often caused by extrapituitary neoplasms

Iatrogenic—Frequently the result of administration of large amounts of exogenous corticosteroids

Food dependent—Inappropriate sensitivity of adrenal glands to normal postprandial increases in secretion of gastric inhibitory polypeptide

TABLE 38-2

CLINICAL MANIFESTATIONS OF CUSHING SYNDROME

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Cushing syndrome is uncommon in children. When seen, it is often caused by excessive or prolonged steroid therapy that produces a cushingoid appearance (see Fig. 38-4). This condition is reversible once the steroids are gradually discontinued. Abrupt withdrawal precipitates acute adrenal insufficiency. Gradual withdrawal of exogenous supplies is necessary to allow the anterior pituitary an opportunity to secrete increasing amounts of ACTH to stimulate the adrenals to produce cortisol.

Clinical Manifestations

Because the actions of cortisol are widespread, clinical manifestations are equally profound and diverse (see Table 38-2). Those symptoms that produce changes in physical appearance occur early in the disorder and are of considerable concern to school-age and older children (Fig. 38-5). The physiologic disturbances, such as hyperglycemia, susceptibility to infection, hypertension, and hypokalemia, may have life-threatening consequences unless recognized early and treated successfully. Children with short stature may be responding to increased cortisol levels, resulting in Cushing syndrome. Cortisol inhibits the action of GH.

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Fig. 38-5 Characteristics of Cushing syndrome.

Diagnostic Evaluation

Several tests are helpful in confirming Cushing syndrome. Serum cortisol levels should be measured at midnight and in the morning, along with corticotropin hormone, urinary free cortisol, fasting blood glucose levels for hyperglycemia, serum electrolyte levels for hypokalemia and alkalosis, and 24-hour urinary levels of elevated 17-hydroxycorticoids and 17-ketosteroids. Imaging of the pituitary and adrenal glands to assess for tumors, bone density studies for evidence of osteoporosis, and skull x-rays examination to determine enlargement of the sella turcica may also aid in the diagnosis. Another procedure used to establish a more definitive diagnosis is the dexamethasone (cortisone) suppression test (Batista, Riar, Keil, et al, 2007). Administration of an exogenous supply of cortisone normally suppresses ACTH production. However, in individuals with Cushing syndrome, cortisol levels remain elevated. This test is helpful in differentiating between children who are obese and those who appear to have cushingoid features.

Therapeutic Management

Treatment depends on the cause. In most cases surgical intervention involves bilateral adrenalectomy and postoperative replacement of the cortical hormones (the therapy for this is the same as that outlined for chronic adrenal insufficiency). If a pituitary tumor is found, surgical extirpation or irradiation may be chosen. In either of these instances, treatment of panhypopituitarism with replacement of GH, thyroid extract, ADH, gonadotropins, and steroids may be necessary for an indefinite period.

Nursing Care Management

Nursing care also depends on the cause. When cushingoid features are caused by steroid therapy, the effects may be lessened with administration of the drug early in the morning and on an alternate-day basis. Giving the drug early in the day maintains the normal diurnal pattern of cortisol secretion. If given during the evening, it is more likely to produce symptoms because endogenous cortisol levels are normally low and the additional supply exerts more pronounced effects. An alternate-day schedule allows the anterior pituitary an opportunity to maintain more normal hypothalamic-pituitary-adrenal control mechanisms.

If an organic cause is found, nursing care is related to the treatment regimen. Although a bilateral adrenalectomy permanently solves one condition, it reciprocally produces another syndrome. Before surgery, parents need to be adequately informed of the operative benefits and disadvantages. Postoperative teaching regarding drug replacement is the same as discussed in the previous section.

image NURSING ALERT

Postoperative complications of adrenalectomy are related to the sudden withdrawal of cortisol. Observe for shocklike symptoms (e.g., hypotension, hyperpyrexia).

Anorexia and nausea and vomiting are common and may be improved with the use of nasogastric decompression. Muscle and joint pain may be severe, requiring use of analgesics. The psychologic depression can be profound and may not improve for months. Parents should be aware of the physiologic reasons behind these symptoms in order to be supportive of the child.

Congenital Adrenal Hyperplasia

Congenital adrenal hyperplasia (CAH) is a family of disorders caused by decreased enzyme activity required for cortisol production in the adrenal cortex. The most common defect (accounting for >90% of cases) is 21-hydroxylase deficiency (American Academy of Pediatrics, 2000 [reaffirmed 2005]). This deficiency is an autosomal recessive disorder that results in improper steroid hormone synthesis. It occurs in approximately 1 per 12,000 to 15,000 births. In its most severe form, it can be life threatening (Glatt, Garzon, and Popovic, 2005).

Pathophysiology

Interference in the biosynthesis of cortisol during fetal life results in an increased production of ACTH, which stimulates hyperplasia of the adrenal gland. Depending on the enzymatic defect, increased quantities of cortisol precursors and androgens are secreted. There are six major types of biochemical defects. The most common is partial or complete 21-hydroxylase deficiency. With partial deficiency, enough aldosterone is produced to preserve sodium, and adequate cortisol is produced to prevent signs of adrenocortical insufficiency.

In the complete, or salt-losing, form, insufficient amounts of aldosterone and cortisol are produced. If salt-losing CAH is not diagnosed and treated at birth, infants will exhibit symptoms of failure to thrive, weakness, vomiting, and dehydration, and a salt-losing crisis will ensue (Behrman, Kliegman, Jenson, et al, 2009). In 11-hydroxylase deficiency there is an increase in the mineralocorticoid 11-desoxycorticosterone, which leads to hypertension. In each of these types excess production of androgens causes ambiguous genitalia in females and precocious genital development in males. Other forms of CAH do not result in excess production of androgens but cause various degrees of hypoaldosteronism or hyperaldosteronism.

Clinical Manifestations

Excessive androgens cause masculinization of the urogenital system at approximately the tenth week of fetal development. The most pronounced abnormalities occur in the female, who is born with varying degrees of ambiguous genitalia. Masculinization of external genitalia causes the clitoris to enlarge so that it appears as a small phallus. Fusion of the labia produces a saclike structure resembling the scrotum without testes. However, no abnormal changes occur in the internal sexual organs, although the vaginal orifice is usually closed by the fused labia. (See also Ambiguous Genitalia, Chapter 11.) The label ambiguous genitalia should be applied to any infant with hypospadias or micropenis and no palpable gonads, and a diagnostic evaluation for CAH should be contemplated. Males do not display genital abnormalities at birth (New and Ghizzoni, 2003), so it may go undetected.

Increased pigmentation of skin creases and genitalia caused by increased ACTH may be a subtle sign of adrenal insufficiency. A salt-wasting crisis frequently occurs, usually within the first few weeks of life (Behrman, Kliegman, Jenson, et al, 2009). Infants fail to gain weight, and hyponatremia and hyperkalemia may be significant. Cardiac arrest can occur.

Untreated CAH results in early sexual maturation, with enlargement of the external sexual organs; development of axillary, pubic, and facial hair; deepening of the voice; acne; and marked increase in musculature with changes toward an adult male physique. However, in contrast to precocious puberty, breasts do not develop in the female, and she remains amenorrheic and infertile. In the male the testes remain small, and spermatogenesis does not occur. In both sexes linear growth is accelerated, and epiphyseal closure is premature, resulting in short stature by the end of puberty.

Diagnostic Evaluation

Clinical diagnosis is initially based on congenital abnormalities that lead to difficulty in assigning sex to the newborn and on signs and symptoms of adrenal insufficiency. Newborn screening is currently done in all 50 U.S. states by measurement of the cortisol precursor 17-hydroxyprogesterone. Definitive diagnosis is confirmed by evidence of increased 17-ketosteroid levels in most types of CAH (American Academy of Pediatrics, 2000 [reaffirmed 2005]). In complete 21-hydroxylase deficiency, blood electrolytes demonstrate loss of sodium and chloride and elevation of potassium. In older children bone age is advanced, and linear growth is increased. DNA analysis for positive sex determination and to rule out any other genetic abnormality (e.g., Turner syndrome) is done in any case of ambiguous genitalia.

Another test that can be used to visualize the presence of pelvic structures is ultrasonography, a noninvasive imaging technique that does not require anesthesia or sedation. It is especially useful in CAH because it readily identifies the presence of female reproductive organs or male testes in a newborn or child with ambiguous genitalia. Because ultrasonography yields immediate results, it has the advantage of determining the child’s gender before the more complex laboratory results for chromosome analysis or steroid levels are available.

Therapeutic Management

After diagnosis is confirmed, medical management includes administration of glucocorticoids to suppress the abnormally high secretions of ACTH and adrenal androgens (Glatt, Garzon, and Popovic, 2005). If cortisone begins early enough, it is very effective. Cortisone depresses the secretion of ACTH by the adenohypophysis, which in turn inhibits the secretion of adrenocorticosteroids, which stems the progressive virilization. The signs and symptoms of masculinization in the female gradually disappear, and excessive early linear growth is slowed. Puberty occurs normally at the appropriate age.

The recommended oral dosage is divided to simulate the normal diurnal pattern of ACTH secretion. Because these children are unable to produce cortisol in response to stress, it is necessary to increase the dosage during episodes of infection, fever, or other stresses. Acute emergencies require immediate IV or intramuscular administration. Emergency situations include bacterial and viral infections, vomiting, surgery, fractures, major injuries, and sometimes insect stings.

Children with the salt-losing type of CAH require aldosterone replacement, as outlined under chronic adrenal insufficiency, and supplementary dietary salt. Frequent laboratory tests are conducted to assess the effects on electrolytes, hormonal profiles, and renin levels. The frequency of testing is individualized to the child.

Gender assignment and surgical intervention in the newborn with ambiguous genitalia is complex and controversial. It is a significant stress for families, who need support and education from a multidisciplinary team of experienced specialists. Factors that influence gender assignment include genetic diagnosis, genital appearance, surgical options, fertility, and family and cultural preferences. Generally, genetically female (46XX) infants should be raised as girls. Early reconstructive surgery should be considered only in the case of severe virilization (Lee, Houk, Ahmed, et al, 2006). Emphasis is on functional rather than cosmetic outcomes, and surgery can often be delayed. Reports concerning sexual satisfaction after partial clitoridectomy indicate that the capacity for orgasm and sexual gratification is not necessarily impaired. Male infants may require phallic reconstruction by an experienced surgeon.

Unfortunately, not all children with CAH are diagnosed at birth and raised in accordance with their genetic sex. Particularly in the case of affected females, masculinization of the external genitalia may have led to sex assignment as a male. In children with milder forms of CAH, especially males, diagnosis may be delayed until early childhood, when signs of virilism appear. In these situations it is usually advisable to continue rearing the child as a male in accordance with assigned sex and phenotype. Hormone replacement may be required to permit linear growth and to initiate male pubertal changes. Surgery is usually indicated to remove the female organs and reconstruct the phallus for satisfactory sexual relations. These individuals are not fertile.

Nursing Care Management

Of major importance is early recognition of ambiguous genitalia and diagnostic confirmation in newborns. As with any congenital defect, the parents require an adequate explanation of the condition and time to grieve for the loss of perfection. In this instance they may also need to grieve for the loss of the desired-sex child. For example, the birth of a phenotypically male infant may fulfill their wish for a son. Knowledge of the child’s actual sex may leave them disappointed. Such situations may also lead them to discuss the possibility of raising the child as a boy despite the actual sex. This is a difficult question that requires thoughtful discussion among the parents and members of the health team.

In general, rearing the genetically female child as a girl is preferred because of the success of surgical intervention and the satisfactory results with hormones in reversing virilism and providing a prospect of normal puberty and the ability to conceive. This is in contrast to the choice of rearing the child as a boy, in which case the child is sterile and may never be able to function satisfactorily in heterosexual relationships. If the parents persist in their decision to assign a male sex to a genetically female child, request a psychologic consultation to explore their motivations and ensure their understanding of the future consequences for the child.

Parents need an explanation regarding this disorder that helps them explain it to others. When referring to the external genitalia, it is preferable to refer to them as sex organs and to emphasize the similarity between the penis-clitoris and scrotum-labia during fetal development. Explain that the sex organs were overdeveloped because of too much male hormone secretion. Using a correct vocabulary allows parents to explain the abnormalities to others in a straightforward manner, just as if the defect involved the heart or an extremity.

Parents often fear that the infant will retain “male behavioral characteristics” because of prenatal masculinization and will not be able to develop female characteristics. It is important to stress that gender identity and psychosexual development depend on multiple influences. Because the prognosis for normal sexual development is excellent after early treatment, the nurse should foster identification with the child as one sex only. Ambiguous genitalia have no relationship to sexual preference for partners later in life.

As soon as the sex is determined, inform parents of the findings and encourage them to choose an appropriate name and identify the child as a male or female, with no reference to ambiguous sex. If the appearance of the enlarged genitalia in a girl concerns the parents, encourage them to discuss their feelings. Suggesting ways to avoid questioning remarks from visitors, such as diapering the child in a separate room, is also helpful. If surgery is anticipated, showing parents before-and-after photographs of reconstruction helps to reinforce the expected cosmetic benefits.

Nursing care management regarding cortisol and aldosterone replacement are the same as those discussed for chronic adrenocortical insufficiency. A follow-up visit by a home health nurse may be desirable to ensure that parents understand and comply with the treatment regimen. Nurses in well-child facilities should assume responsibility for guidance and supervision regarding this aspect of care during each visit. Monitor vital signs closely for early signs of hypertension due to cortisol therapy.

Because infants are especially prone to dehydration and salt-losing crises, parents need to be aware of signs of dehydration and the urgency of immediate medical intervention to stabilize the child’s condition. Parents should have injectable hydrocortisone available and know how to prepare and administer the intramuscular injection. (See Chapter 27.) Parents, and later the child, need to understand that the medical regimen must be a lifelong commitment; therefore provide them with the education and counseling that is most likely to ensure informed and willing compliance. They also need to know that growth retardation that may have occurred before therapy cannot be overcome and that normal stature is not a realistic expectation, even though growth velocity may improve with medication. Assess males for development of testicular tumors.

In the unfortunate situation in which the sex is erroneously assigned and the correct sex determined later, parents need a great deal of help in understanding the reason for the incorrect sex identification and the options for sex reassignment or medical-surgical intervention.

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Advise the parents that there is no physical harm in treating for suspected adrenal insufficiency that is not present, whereas the consequence of not treating acute adrenal insufficiency can be fatal.

Because the hereditary form of adrenal hyperplasia is an autosomal recessive disorder, refer parents for genetic counseling before they conceive another child. The nurse’s role is to ensure that parents understand the probability of transmitting the trait or disorder with each pregnancy. Prenatal diagnosis and treatment for CAH are available. Affected offspring also require genetic counseling, since both sexes are generally able to reproduce. (See Chapter 5 for recurrence risks and genetic counseling.)

Hyperaldosteronism

Excessive secretion of aldosterone may be caused by an adrenal tumor or, in some types of adrenogenital syndromes, result from enzymatic deficiency. The signs and symptoms are caused by increased sodium levels, water retention, and potassium loss. Hypervolemia causes hypertension and resultant headaches. Paradoxically, funduscopic changes resulting from increased blood pressure and edema from water retention are minimal. Hypokalemia results in muscular weakness, paresthesia, episodes of paralysis, and tetany and may be responsible for polyuria and consequent polydipsia.

The clinical diagnosis is suspected when there are findings of hypertension, hypokalemia, and polyuria that fail to respond to ADH administration. Renin and angiotensin titers are abnormally low. Urinary levels of 17-hydroxycorticosteroids and 17-ketosteroids are normal in primary hyperaldosteronism caused by an aldosterone-secreting tumor but are usually abnormal in adrenogenital syndrome.

Therapeutic Management

Temporary treatment of the disorder involves replacement of potassium and administration of spironolactone (Aldactone), a diuretic that blocks the effects of aldosterone, thereby promoting excretion of sodium and water while preserving potassium. Definitive treatment is similar to that for chronic adrenocortical insufficiency.

Nursing Care Management

An important nursing consideration is recognition of the syndrome, particularly in children with high blood pressure. Other clues include bed-wetting, excessive thirst, and unexplained weakness. After the diagnosis, nursing care is related to the treatment regimen. If diuretics are used, they should be administered in the morning to avoid accidents during the night. Children need unrestricted restroom privileges at school. Potassium supplements should be mixed with fruit juice such as grape juice to increase their acceptability, and potassium-rich foods should be encouraged. Parents need to be aware of the signs of hypokalemia and hyperkalemia. After an adrenalectomy, nursing care is similar to that for chronic adrenocortical insufficiency.

Pheochromocytoma

Pheochromocytoma is a rare tumor characterized by secretion of catecholamines. The tumor most commonly arises from the chromaffin cells of the adrenal medulla but may occur wherever these cells are found, such as along the paraganglia of the aorta or thoracolumbar sympathetic chain. Approximately 10% of these tumors are located in extraadrenal sites. In children they are frequently bilateral or multiple and are generally benign. Often there is a familial transmission of the condition as an autosomal dominant trait (Behrman, Kliegman, Jenson, et al, 2009).

Clinical Manifestations

The clinical manifestations of pheochromocytoma are caused by an increased production of catecholamines, producing hypertension, tachycardia, headache, decreased gastrointestinal activity with resultant constipation, increased metabolism with anorexia, weight loss, hyperglycemia, polyuria, polydipsia, hyperventilation, nervousness, heat intolerance, and diaphoresis. In severe cases, signs of congestive heart failure are evident.

Diagnostic Evaluation

The clinical manifestations mimic those of other disorders, such as hyperthyroidism or DM. Tests specific to these conditions may be performed as part of the differential diagnosis. In a small number of instances a palpable tumor suggests the diagnosis. Usually the tumor is identified by a CT scan or MRI. Definitive tests include 24-hour measurement of urinary levels of the catecholamine metabolites; histamine stimulation, which provokes a hypertensive attack from sudden release of large amounts of catecholamines; and α-adrenergic blocking agents, which produce a hypotensive episode by inhibiting the action of circulating catecholamines.

Therapeutic Management

Definitive treatment consists of surgical removal of the tumor. In children the tumors may be bilateral, requiring a bilateral adrenalectomy and lifelong glucocorticoid and mineralocorticoid therapy. The major complications that can occur during surgery are severe hypertension, tachyarrhythmias, and hypotension. The first two are caused by excessive release of catecholamines during manipulation of the tumor, and the latter results from catecholamine withdrawal and hypovolemic shock.

Preoperative medication to inhibit the effects of catecholamines is begun 1 to 3 weeks before surgery to prevent these complications. The major group of drugs used is the α-adrenergic blocking agents with or without β-adrenergic blocking agents. The most commonly used α-adrenergic blocker is phenoxybenzamine (Dibenzyline), a longer-acting medication given orally every 12 hours. The shorter-acting phentolamine (Regitine) is equally effective but less satisfactory for long-term use, although it is useful for acute hypertension. To control catecholamine release when α-adrenergic blocking agents are inadequate, the child is given β-adrenergic blocking agents.

Success of therapy is judged by lowering of blood pressure to normal, absence of hypertensive attacks (flushing or blanching, fainting, headache, palpitations, tachycardia, nausea and vomiting, profuse sweating), heat tolerance, decrease in perspiration, and disappearance of hyperglycemia. A disadvantage of these drugs is their inability to block the effects of catecholamines on β-receptors.

Nursing Care Management

An initial nursing objective is identification of children with this disorder. Outstanding clues are hypertension and hypertensive attacks. Because of behavioral changes (nervousness, excitability, overactivity, even psychosis), increased cardiac and respiratory activity may appear to be related to an acute anxiety attack. Therefore a careful history of the onset of symptoms and association with stressful events is helpful in distinguishing between an organic and a psychologic cause for the symptoms.

Preoperative nursing care involves frequent monitoring of vital signs and observation for evidence of hypertensive attacks and congestive heart failure. Therapeutic effects are evidenced by normal vital signs and absence of glycosuria. Note daily blood glucose levels and urine acetone; report any signs of hyperglycemia immediately.

The environment is made conducive to rest and free of emotional stress. This requires adequate preparation during hospital admission and before surgery. Encourage parents to room-in with their child and to participate in care. Play activities need to be tailored to the child’s energy level without being overly strenuous or challenging because these can increase metabolic rate and promote frustration and anxiety.

image NURSING ALERT

Do not palpate the mass. Preoperative palpation of the mass releases catecholamines, which can stimulate severe hypertension and tachyarrhythmias.

After surgery observe the child for signs of shock from removal of excess catecholamines. If a bilateral adrenalectomy was performed, the nursing interventions are those discussed for chronic adrenocortical insufficiency.

Disorders of Pancreatic Hormone Secretion

Diabetes Mellitus

image DM is a chronic disorder of metabolism characterized by a partial or complete deficiency of the hormone insulin. It is the most common metabolic disease, resulting in metabolic adjustment or physiologic change in almost all areas of the body. Approximately one in three children born in the United States will develop diabetes. The odds are higher for African-American and Hispanic children: nearly 50% of them will develop diabetes (Urrutia-Rojas and Menchaca, 2006). The age of presentation of childhood-onset type 1 DM has a bimodal distribution, with one peak at 4 to 6 years of age and another at early puberty (10 to 14 years of age) (Felner, Klitz, Ham, et al, 2005). The incidence in boys is slightly higher than in girls (1:1 to 1.2:1).

imageCase Study—Diabetes Mellitus

Type 1 DM is more prominent in Caucasians, with an incidence of 24 per 100,0000 (Dabelea, Bell, and D’Agostino, 2007). The incidence in African-Americans is 11 per 100,0000; the incidence in Hispanics is 15.2 per 100,0000; and the incidence in Cubans is 2.6 per 100,0000. Native Americans tend to develop type 2 DM rather than type 1 DM, even when diagnosed in childhood. The Pima Tribe reports a greater than 55% incidence of type 2 DM.

Classification

Traditionally DM had been classified according to the type of treatment needed. The old categories were insulin-dependent diabetes mellitus (IDDM), or type I; and non–insulin-dependent diabetes mellitus (NIDDM), or type II. In 1997 these terms were eliminated because treatment can vary (some people with NIDDM require insulin) and because the terms do not indicate the underlying problem. The new terms are type 1 and type 2, using Arabic symbols to avoid confusion (e.g., type II could be read as type eleven) (American Diabetes Association, 2001). The characteristics of type 1 DM and type 2 DM are compared in Table 38-3.

TABLE 38-3

CHARACTERISTICS OF TYPES 1 AND 2 DIABETES MELLITUS

CHARACTERISTIC TYPE 1 TYPE 2
Age at onset <20 yr Increasingly occurring in younger children
Type of onset Abrupt Gradual
Sex ratio Males slightly more than females Females outnumber males
Percentage of diabetic population 5%-8% 85%-90%
Heredity:
 Family history Sometimes Frequently
 Human leukocyte antigen Associations No association
 Twin concordance 25%-50% 90%-100%
 Ethnic distribution Primarily Caucasians Increased incidence in Native Americans, Hispanics, African-Americans
Presenting symptoms 3 Ps common: polyuria, polydipsia, polyphagia May be related to long-term complications
Nutritional status Underweight Overweight
Insulin (natural):
 Pancreatic content Usually none >50% normal
 Serum insulin Low to absent High or low
 Primary resistance Minimum Marked
Islet cell antibodies 80%-85% <5%
Therapy:
 Insulin Always 20%-30% of patients
 Oral agents Ineffective Often effective
 Diet only Ineffective Often effective
Chronic complications >80% Variable
Ketoacidosis Common Infrequent

Type 1 diabetes is characterized by destruction of the pancreatic beta cells, which produce insulin; this usually leads to absolute insulin deficiency (Fig. 38-6). Type 1 diabetes has two forms. Immune-mediated DM results from an autoimmune destruction of the beta cells; it typically starts in children or young adults who are slim, but it can arise in adults of any age. Idiopathic type 1 refers to rare forms of the disease that have no known cause.

Pathophysiology Review

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Fig. 38-6 The pancreas. A, The main and accessory ducts. B, Glandular cells of the pancreatic islets. (From Patton KT, Thibodeau GA: Anatomy and physiology, ed 7, St Louis, 2010, Mosby.)

Type 2 diabetes usually arises because of insulin resistance, in which the body fails to use insulin properly, combined with relative (rather than absolute) insulin deficiency. People with type 2 can range from predominantly insulin resistant with relative insulin deficiency to predominantly deficient in insulin secretion with some insulin resistance. It typically occurs in those who are over 45, are overweight and sedentary, and have a family history of diabetes.

Several other specific types of DM have been defined, such as those resulting from genetic defects of beta cell function, pancreatic diseases (e.g., cystic fibrosis), and defects in insulin action. Maturity-onset diabetes of the young (MODY) is associated with monogenetic defects in beta cell function that are characterized by impaired insulin secretion with minimum or no defects in insulin action. The disease is inherited as an autosomal dominant pattern, and the onset of hyperglycemia occurs at an early age (generally before age 25 years).

Etiology

The clinical syndrome of DM results from a large variety of etiologic and pathogenic mechanisms. Type 1 DM is an autoimmune disease that arises when a person with a genetic predisposition is exposed to a precipitating event, such as a viral infection. Type 2 DM is more likely to be influenced by stronger, but as yet unknown, genetic factors. Thus its origin is considered to be polygenic.

Genetic Factors: Type 1 DM is not inherited, but heredity is a prominent factor in the etiology. In more than 40 rare genetic syndromes, diabetes is a major feature (Harris, 2003). No simple mendelian pattern is found for DM. Children born to fathers with type 1 DM are about three times more likely to develop type 1 DM (approximately 7% frequency) than children born to mothers with type 1 DM (approximately 2% frequency) (see Research Focus box).

image RESEARCH FOCUS

Genetic Factors and Diabetes

Studies of type 2 diabetes mellitus (DM) in identical twins demonstrate a 100% concordance throughout the life span, whereas studies of type 1 DM in identical twins demonstrate a 30% to 50% concordance rate, suggesting that both environmental and genetic factors are important in the development of type 1 DM (Stephenson, 2003). Children diagnosed with type 1 diabetes before 5 years of age may have different autoimmune and genetic characteristics related to their diabetes than older children (Hathout, Hartwick, Fagoaga, et al, 2003).

At least 60% of the genetic susceptibility to type 1 diabetes is conferred by human leukocyte antigen (HLA) on chromosome 6. Several alleles have been implicated, including DR3, DR4, and DQ8. The highest-risk alleles (DR3 and DR4) are found in 95% of patients with diabetes. Only 50% of nondiabetic persons have these alleles. Certain alleles, such as DR2, may actually protect the individual from diabetes (Barker and Eisenbarth, 2003).

Autoimmune Mechanisms: Pancreatic islet cell antibodies (ICAs) are found in about 70% to 85% of patients newly diagnosed with type 1 DM. The antibodies disappear by 1 year after diagnosis in most persons, but in some they may persist for years. The current theory is that the presence of the HLA genes causes a defect in the immune system that renders the possessor susceptible to a trigger event, which can be a dietary source, a virus, bacteria, or a chemical irritant. The predisposing factor initiates an autoimmune process that gradually destroys beta cells. Without beta cells, the body cannot produce insulin. It is unclear whether the ICAs are a result of the inflammatory process or a significant aspect of the beta cell destruction. Controversy exists regarding whether the autoimmune response is primarily mediated by the lymphocyte response or the humoral (antibody) response or is a result of the two.

There is a strong association between type 1 DM and other autoimmune endocrine disorders. An increased incidence of other autoimmune endocrine disorders, such as thyroiditis and Addison disease, has been found in families of children with DR3-associated type 1 DM.

Researchers have also found anti-ICAs in a number of unaffected first-degree relatives of children with type 1 DM (Bingley and Gale, 2006). These findings offer hope of identifying persons at risk for diabetes with the eventual possibility of screening and implementation of therapy. Research is also continuing to identify genetic risk and environmental triggers with the hope of developing prevention strategies such as immunizations.

Treatment with cyclosporine or other forms of immunosuppression has been tested as an early intervention in the newly diagnosed person with type 1 DM. The effects of lifelong immunosuppression must be carefully weighed against the lifelong effects of diabetes.

Diet: Cow’s milk has been implicated as a possible trigger of the autoimmune response that destroys pancreatic cells in genetically susceptible hosts, thus causing DM (Goldfarb, 2008). Further research is needed.

Viruses: A variety of viruses, including mumps, coxsackievirus B, and congenital rubella, have been implicated as the prime environmental factor in the etiology of DM. Islet cells appear to be particularly susceptible to either direct viral damage or chemical insult. The body reacts to this damaged or changed tissue in an autoimmune phenomenon. Thus the virus serves as a precipitating factor, or “trigger.” A viral etiology also helps explain the seasonal variation in the onset of DM. Although this seasonal variation is not evident in children under 5 years of age, the marked increase in older children during the winter months strongly suggests an infectious disease relationship in either cause or expression of diabetes in children.

Type 2 Diabetes: Type 1 DM is the predominant form of diabetes in the pediatric age-group. However, changes in food consumption and exercise patterns have increased the rate of type 2 DM in children and adolescents in the Unites States (Ramchandani, 2004; Kiess, Bottner, Raile, et al, 2003; Steinberger and Daniels, 2003; Stephenson, 2003). The disturbed carbohydrate metabolism of type 2 DM may be a result of a sluggish or insensitive secretory response in the pancreas or a defect in body tissues that requires unusual amounts of insulin, or it may be that the insulin secreted is rapidly destroyed, inhibited, or inactivated in affected persons. Children with type 2 diabetes often have other features of insulin resistance syndrome: polycystic ovary syndrome and acanthosis nigricans (AN). AN is found in as many as 90% of children with type 2 diabetes and is characterized by velvety hyperpigmented areas in skinfolds. Risk factors include non-European ancestry, family history of type 2 DM, obesity, insulin resistance, and older age (American Diabetes Association, 2007). While performing routine scoliosis screenings, school nurses often identify AN on the child’s neck. Refer such children to their primary care provider for further metabolic evaluation.

Pathophysiology

Insulin is needed to support the metabolism of carbohydrates, fats, and proteins, primarily by facilitating the entry of these substances into the cell. Insulin is needed for the entry of glucose into the muscle and fat cells, prevention of mobilization of fats from fat cells, and storage of glucose as glycogen in the cells of liver and muscle. Insulin is not needed for the entry of glucose into nerve cells or vascular tissue. The chemical composition and molecular structure of insulin are such that it fits into receptor sites on the cell membrane. Here it initiates a sequence of poorly defined chemical reactions that alter the cell membrane to facilitate the entry of glucose into the cell and stimulate enzymatic systems outside the cell that metabolize the glucose for energy production.

With a deficiency of insulin, glucose is unable to enter the cell, and its concentration in the bloodstream increases. The pathophysiology review in Fig. 38-6 shows the cells responsible for secreting glucagon and insulin. The increased concentration of glucose (hyperglycemia) produces an osmotic gradient that causes the movement of body fluid from the intracellular space to the interstitial space, then to the extracellular space and into the glomerular filtrate in order to “dilute” the hyperosmolar filtrate. Normally the renal tubular capacity to transport glucose is adequate to reabsorb all the glucose in the glomerular filtrate. When the glucose concentration in the glomerular filtrate exceeds the renal threshold (6180 mg/dl), glucose spills into the urine (glycosuria) along with an osmotic diversion of water (polyuria), a cardinal sign of diabetes. The urinary fluid losses cause the excessive thirst (polydipsia) observed in diabetes. This water “washout” results in a depletion of other essential chemicals, especially potassium.

Protein is also wasted during insulin deficiency. Because glucose is unable to enter the cells, protein is broken down and converted to glucose by the liver (glucogenesis); this glucose then contributes to the hyperglycemia. These mechanisms are similar to those seen in starvation when substrate (glucose) is absent. The body is actually in a state of starvation during insulin deficiency. Without the use of carbohydrates for energy, fat and protein stores are depleted as the body attempts to meet its energy needs. The hunger mechanism is triggered, but increased food intake (polyphagia) enhances the problem by further elevating blood glucose (Fig. 38-7).

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Fig. 38-7 Body systems respond to hypoglycemia in various ways to increase blood glucose level.

Ketoacidosis: When insulin is absent or there is altered insulin sensitivity, glucose is unavailable for cellular metabolism, and the body chooses alternate sources of energy, principally fat. Consequently fats break down into fatty acids, and glycerol in the fat cells is converted by the liver to ketone bodies (β-hydroxybutyric acid, acetoacetic acid, acetone). Any excess is eliminated in the urine (ketonuria) or the lungs (acetone breath). The ketone bodies in the blood (ketonemia) are strong acids that lower serum pH, producing ketoacidosis.

Ketones are organic acids that readily produce excessive quantities of free hydrogen ions, causing a fall in plasma pH. Then chemical buffers in the plasma, principally bicarbonate, combine with the hydrogen ions to form carbonic acid, which readily dissociates into water and carbon dioxide. The respiratory system attempts to eliminate the excess carbon dioxide by increased depth and rate—Kussmaul respirations, or the hyperventilation characteristic of metabolic acidosis. Sodium and potassium in the plasma buffer the ketones. The kidney attempts to compensate for the increased pH by increasing tubular secretion of hydrogen and ammonium ions in exchange for fixed base, thus depleting the base buffer concentration.

With cellular death, potassium is released from the cell (intracellular fluid) into the bloodstream (extracellular fluid) and excreted by the kidney, where the loss is accelerated by osmotic diuresis. The total body potassium is then decreased, even though the serum potassium level may be elevated as a result of the decreased fluid volume in which it circulates. Alteration in serum and tissue potassium can lead to cardiac arrest.

If insulin therapy in combination with correction of the fluid deficiency and electrolyte imbalance does not reverse these conditions, progressive deterioration occurs with dehydration, electrolyte imbalance, acidosis, coma, and death. Diabetic ketoacidosis (DKA) should be diagnosed promptly in a seriously ill patient and therapy instituted in an intensive care unit.

Long-Term Complications: Long-term complications of diabetes involve both the microvasculature and the macrovasculature. The principal microvascular complications are nephropathy, retinopathy, and neuropathy. Microvascular disease develops during the first 30 years of diabetes, beginning in the first 10 to 15 years after puberty, with renal involvement evidenced by proteinuria and clinically apparent retinopathy.

With poor diabetic control, vascular changes can appear as early as image to 3 years after diagnosis; however, with good to excellent control, changes can be postponed for 20 or more years. Intensive insulin therapy appears to delay the onset and slow the progression of clinically important retinopathy, including vision-threatening lesions, nephropathy, and neuropathy, by 35% to more than 70%, according to studies on treatment and complications of type 1 DM (American Diabetes Association, 2002).

The postpubertal duration, not the total duration, of type 1 DM is implicated as a risk factor for the development of microvascular disease. The process appears to be one of glycosylation, wherein proteins from the blood become deposited in the walls of small vessels (e.g., glomeruli), where they become trapped by “sticky” glucose compounds (glycosyl radicals). The buildup of these substances over time causes narrowing of the vessels, with subsequent interference with microcirculation to the affected areas (Rosenson and Herman, 2008). Macrovascular disease develops after 25 years of diabetes and creates the predominant problems in patients with type 2 DM.

Other complications have been observed in children with type 1 DM. Hypertension and atherosclerotic cardiovascular disease also contributes to the mortality and morbidity of type 1 DM (Karik, Fields, and Shannon, 2007). Hyperglycemia appears to influence thyroid function, and altered function is frequently observed at the time of diagnosis, as well as in poorly controlled diabetes. Limited mobility of small joints of the hand occurs in 30% of 7- to 18-year-old children with type 1 DM and appears to be related to changes in the skin and soft tissues surrounding the joint as a result of glycosylation.

Mild Diabetes: Although most cases of childhood diabetes are recognized during the rapid initial deterioration in carbohydrate metabolism, other cases with more benign disease are being identified with increasing frequency. A few are detected accidentally by urinalysis before overt symptoms occur. MODY, for instance, is a rare genetic type of diabetes resulting from mutations at one of five genes and transmission by an autosomal dominant inheritance (Fajans and Bell, 2003). Children with this type of diabetes phenotypically resemble type 2 DM but are usually not obese. MODY is characterized by beta cell dysfunction and is often treated similarly to type 2 DM.

Clinical Manifestations

The symptomatology of diabetes is more readily recognizable in children than in adults, so it is surprising that the diagnosis may sometimes be missed or delayed. Diabetes is a great imitator; influenza, gastroenteritis, and appendicitis are the conditions most often diagnosed when it turns out that the disease is really diabetes. Diabetes should be suspected in children with a strong family history of diabetes, especially if another child in the family has the disease.

The sequence of chemical events described previously results in hyperglycemia and acidosis, which produce weight loss and the three “polys” of diabetes—polyphagia, polydipsia, and polyuria—the cardinal symptoms of the disease. In type 2 DM the insulin values are elevated. Eighty percent to 90% of this population are overweight, and fatigue and frequent infections (such as candidal infections in females) are often present.

image NURSING ALERT

Recurrent vaginal and urinary tract infections, especially with Candida albicans, are often an early sign of type 2 DM, especially in adolescents.

The variability of clinical manifestations in type 1 DM at diagnosis is best understood if the autoimmune destruction of islet cells is considered an ongoing process (Box 38-13). Symptoms of hyperglycemia may be apparent only during stress (such as an illness) in early stages of disease because of near-normal levels of insulin production. Progressive islet cell destruction of later stages produces more obvious signs and symptoms. By the time there are overt diabetic symptoms, 80% to 90% of islet cell function has been destroyed. Frequently identified symptoms of overt diabetes include enuresis, irritability, and unusual fatigue.

BOX 38-13   CLINICAL MANIFESTATIONS OF TYPE 1 DIABETES MELLITUS

Polyphagia

Polyuria

Polydipsia

Weight loss

Enuresis or nocturia

Irritability; “not himself” or “herself”

Shortened attention span

Lowered frustration tolerance

Fatigue

Dry skin

Blurred vision

Poor wound healing

Flushed skin

Headache

Frequent infections

Hyperglycemia

• Elevated blood glucose levels

• Glucosuria

Diabetic ketosis

• Ketones and glucose in urine

• Dehydration in some cases

Diabetic ketoacidosis

• Dehydration

• Electrolyte imbalance

• Acidosis

• Deep, rapid breathing (Kussmaul)

Abdominal discomfort is common. Weight loss, though observable on the charts, may be a less frequent presenting complaint because the family might not have noticed the change over time. Another outstanding feature of diabetes is thirst. One couple reported that their child, during a trip from California to Kansas, drank the contents of a gallon jug of water between each gas station stop. As abdominal discomfort and nausea increase, the child may actually refuse fluid and food, increasing the state of dehydration and malnutrition. Other symptoms include dry skin, blurred vision, and sores that are slow to heal. More commonly in children, fatigue and bed-wetting are the chief complaints that prompt parents to take their child for evaluation.

At diagnosis, the child may be hyperglycemic, with elevated blood glucose levels and glucose in the urine; ketotic, with ketones measurable in the blood and urine, with or without dehydration; or suffering from DKA, with dehydration, electrolyte imbalance, and acidosis.

Diagnostic Evaluation

Three groups of children who are candidates for diabetes are (1) children who have glycosuria, polyuria, and a history of weight loss or failure to gain despite a voracious appetite; (2) those with transient or persistent glycosuria; and (3) those who display manifestations of metabolic acidosis, with or without stupor or coma. In every case consider diabetes if there is glycosuria, with or without ketonuria, and unexplained hyperglycemia.

Glycosuria by itself is not diagnostic of diabetes. Other sugars, such as galactose, can produce a positive result with certain test strips. Other conditions, such as infection, trauma, emotional or physical stress, hyperalimentation, and some renal or endocrine diseases can cause a mild degree of glycosuria.

An 8-hour fasting blood glucose level of 126 mg/dl or more, a random blood glucose value of 200 mg/dl or more accompanied by classic signs of diabetes, or an oral glucose tolerance test (OGTT) finding of 200 mg/dl or more in the 2-hour sample is almost certain to indicate diabetes (American Diabetes Association, 2005). Postprandial blood glucose determinations and the traditional OGTTs have yielded low detection rates in children and are not usually necessary for establishing a diagnosis. Serum insulin levels may be normal or moderately elevated at the onset of diabetes; delayed insulin response to glucose indicates the presence of impaired glucose tolerance.

Ketoacidosis must be differentiated from other causes of acidosis or coma, including hypoglycemia, uremia, gastroenteritis with metabolic acidosis, salicylate intoxication encephalitis, and other intracranial lesions. DKA is a state of relative insulin insufficiency and may include the presence of hyperglycemia (blood glucose level 330 mg/dl), ketonemia (strongly positive), acidosis (pH 7.30 and bicarbonate 15 mmol/L), glycosuria, and ketonuria (Dunning, 2009). Tests used to determine glycosuria and ketonuria are the glucose oxidase tapes (Keto-Diastix).

Therapeutic Management

The management of the child with type 1 DM consists of a multidisciplinary approach involving the family; the child (when appropriate); and professionals, including a pediatric endocrinologist, diabetes nurse educator, nutritionist, and exercise physiologist. Often psychologic support from a mental health professional is also needed. Communication among the team members is essential and extends to other individuals in the child’s life, such as teachers, school nurse, school guidance counselor, and coach (see Community Focus box).

image COMMUNITY FOCUS

The Adolescent with Type 1 Diabetes Mellitus

As a nurse caring for adolescents with type 1 diabetes mellitus (DM), I am constantly aware of the wide range of adolescent behaviors that affect the course of this disease. Education of the child and the parents can often make the difference between a disease in control of the teen and a teen in control of the disease.

I have cared for many adolescent girls who have episodes of hyperglycemia at the time of menstruation that can result in diabetic ketoacidosis. I have found that education regarding sick-day protocol with sliding-scale regular insulin instituted at the first sign of hyperglycemia, which may occur 1 to 2 days before onset of menses, can keep the adolescent girl out of the intensive care unit and in control of her diabetes.

Eating disorders, such as bulimia or anorexia nervosa, in the teenager with type 1 DM pose a serious health hazard (Mannucci, Rotella, Recca, et al, 2005). Also, insulin manipulation or omission has been identified as a weight loss method used by some adolescent girls (Hoffman, 2001). Nurses working with these adolescents, especially females, must be aware of the hazards and openly discuss the risks with the young person. A referral for specialized intervention may be needed.

Another group of adolescents with diabetes who are at risk are those who drink alcohol. I have found that confusion about the effects of alcohol on blood glucose is common. Teens may believe that alcohol will increase blood glucose levels, when in fact the opposite occurs. Ingestion of alcohol inhibits the release of glycogen from the liver, thereby resulting in hypoglycemia. Teens with diabetes who drink alcohol may become hypoglycemic but be treated as though they were inebriated. Behaviors may be similar, such as shakiness, combativeness, slurred speech, and loss of consciousness.

Education regarding the effects of alcohol is important and must be included in a teaching plan. If teens insist on drinking alcohol, they can be cautioned to use sweetened mixers or eat snacks when consuming alcoholic beverages.

Episodes of hyperglycemia or hypoglycemia may become a serious issue for adolescents who are leaving home for the first time. One teenager confided that her mother always recognized her combative, antisocial behavior as impending hypoglycemia and treated her with the appropriate intervention. The teen feared that a college roommate might be offended by the behavior and leave her alone with impending hypoglycemia.

One young man realized he could not live alone when he took a nap because he “felt tired” and woke up 4 days later in the hospital. Fortunately, his family realized he was in a coma and summoned emergency medical service. The fatigue signaled the beginning of a viral infection, which led to a blood glucose level of 410 mg/dl. Nurses need to address these fears openly and facilitate ways in which the teen can enlist the aid of significant peers who may be available during hyperglycemic or hypoglycemic episodes.

Susan Zekauskas, RN, MSN, PNP

The definitive treatment is replacement of insulin that the child is unable to produce. However, insulin needs are also affected by emotions, nutritional intake, activity, and other life events such as illnesses and puberty. The complexity of the disease and its management requires that the child and family incorporate diabetes needs into their lifestyle. Medical and nutritional guidance are primary, but management also includes continuing diabetes education, family guidance, and emotional support.

Insulin Therapy: image Insulin replacement is the cornerstone of management of type 1 DM. Insulin dosage is tailored to each child based on home blood glucose monitoring. The goal of insulin therapy is maintaining near-normal blood glucose values while avoiding too frequent episodes of hypoglycemia. Insulin is administered as two or more injections per day or as continuous subcutaneous infusion using a portable insulin pump.

imageEvidence-Based Practice—Medication Safety and Insulin Therapy

Healthy pancreatic cells secrete insulin at a low but steady basal rate with superimposed bursts of increased secretion that coincide with intake of nutrients. Consequently insulin levels in the blood increase and decrease coincidentally with rise and fall in blood glucose levels. In addition, insulin is secreted directly into the portal circulation; therefore the liver, which is the major site of glucose disposal, receives the largest concentration of insulin. No matter which method of insulin replacement is used, this normal pattern cannot be duplicated. Subcutaneous injection results in absorption of the drug into the general circulation, thus reducing the concentrations of insulin to which the liver is exposed.

Insulin Preparations:

Insulin is available in highly purified pork preparations and in human insulin biosynthesized by and extracted from bacterial or yeast cultures. Most clinicians suggest human insulin as the treatment of choice. Insulin is available in rapid-, intermediate-, and long-acting preparations, and all are packaged in the strength of 100 units/ml. Some insulins are available as premixed insulins, such as 70/30 and 50/50 ratios, the first number indicating the percentage of intermediate-acting and the second number the percentage of rapid-acting insulin. Box 38-14 lists the different types of insulin.

BOX 38-14   TYPES OF INSULIN

There are four types of insulin, based on the following criteria:

• How soon the insulin starts working (onset)

• When the insulin works the hardest (peak time)

• How long the insulin lasts in the body (duration).

However, each person responds to insulin in his or her own way. That is why onset, peak time, and duration are given as ranges.

• Rapid-acting insulin (lispro) reaches the blood within 15 minutes after injection. The insulin peaks 30 to 90 minutes later and may last as long as 5 hours.

• Short-acting (regular) insulin usually reaches the blood within 30 minutes after injection. The insulin peaks 2 to 4 hours later and stays in the blood for about 4 to 8 hours.

• Intermediate-acting (NPH and Lente) insulins reach the blood 2 to 6 hours after injection. The insulins peak 4 to 14 hours later and stay in the blood for about 14 to 20 hours.

• Long-acting (Ultralente) insulin takes 6 to 14 hours to start working. It has no peak or a very small peak 10 to 16 hours after injection. The insulin stays in the blood between 20 and 24 hours.

Some insulins come mixed together. For example, you can buy regular insulin and NPH insulins already mixed in one bottle, which makes it easier to inject two kinds of insulin at the same time. However, you cannot adjust the amount of one insulin without also changing how much you get of the other insulin.

Lispro-H (Humalog) and insulin aspart (NovoLog) are human insulin analogs. One unit of the analog has the same glucose-lowering effect as 1 unit of human rapid-acting insulin, but the effect is even more rapid and of shorter duration. One benefit is a decreased risk of hypoglycemia, since the peak effect is reached in 1 to image hours. Because of its rapid onset, each of the analogs must be injected within 15 minutes before eating.

image DRUG ALERT

Insulin Preparations

The human insulins from various manufacturers may be interchangeable, but human insulin and pork insulin or pure pork insulin should never be substituted for one another.

Dosage:

Conventional management is a twice-daily insulin regimen combining rapid-acting (regular) and intermediate-acting (NPH or Lente) insulin drawn up into the same syringe and injected before breakfast and before the evening meal. The amount of morning regular insulin is determined by patterns in the late morning and lunchtime blood glucose values. The morning intermediate-acting dosage is determined by patterns in the late afternoon and supper blood glucose values. Fasting blood glucose patterns at breakfast help determine the evening dose of intermediate insulin, and the blood glucose patterns at bedtime help determine the evening dose of rapid-acting (regular) insulin. For some children, better morning glucose control is achieved by a later (bedtime) injection of intermediate-acting insulin.

Regular insulin is best administered at least 30 minutes before meals. This allows sufficient time for absorption and results in a significantly greater reduction in the postprandial rise in blood glucose than if the meal were eaten immediately after the insulin injection. Intensive therapy consists of multiple injections throughout the day with a once- or twice-daily dose of long-acting (Ultralente) insulin to simulate the basal insulin secretion and injections of rapid-acting insulin before each meal. A multiple daily injection program reduces microvascular complications of diabetes in young, healthy patients who have type 1 DM. The precise dose of insulin needed cannot be predicted. Therefore the total dosage and percentage of regular- to intermediate-acting insulin should be determined empirically for each child. Usually 60% to 75% of the total daily dose is given before breakfast, and the remainder before the evening meal. Furthermore insulin requirements do not remain constant but change continuously during growth and development; the need varies according to the child’s activity level and pubertal status. For example, less insulin is required during spring and summer months, when the child is more active. Illness also alters insulin requirements. Some children require more frequent insulin administration. This includes children with difficult-to-control diabetes and children undergoing the adolescent growth spurt.

Methods of Administration:

Daily insulin is administered subcutaneously by twice-daily injections, by multiple-dose injections, or by means of an insulin infusion pump. The insulin pump is an electromechanical device designed to deliver fixed amounts of regular or lispro insulin continuously (basal rate), thereby more closely imitating the release of the hormone by the islet cells (Olohan and Zappitelli, 2003). Although the pump delivers a programmed amount of basal insulin, the child or parent must program a dose for the pump to deliver before each meal.

The system consists of a syringe to hold the insulin, a plunger, and a computerized mechanism to drive the plunger. The insulin flows from the syringe through a catheter to a needle inserted into subcutaneous tissue (the abdomen or thigh), and the lightweight device is worn on a belt or a shoulder holster. The child or parent changes the needle and catheter every 48 to 72 hours, using aseptic technique, and tapes it in place.

Although the pump provides more consistent insulin delivery, it has certain disadvantages. Pump therapy is expensive and requires commitment from the parent and child. A certain level of math skills is required to calculate infusion rates. It should not be removed for more than 1 hour at a time, which may limit some activities. Skin infections are common; and, as with any other mechanical device, it is subject to malfunction. However, the pumps are equipped with alarms that signal problems that may arise, such as a depleted battery, an occluded needle or tubing, or a microprocessor malfunction.

Future Therapies: Islet cell or whole pancreas transplantation may offer hope to patients in the future. Viable insulin-producing cells have been injected into the portal vein, where they are transported via the circulation to the liver and eventually produce up to two thirds of the required insulin. The major use of transplants has been in persons who have serious complications, particularly those whose deteriorating kidneys have required renal transplantation and who are receiving immunosuppressive therapy. However, islet cell and pancreatic transplants tend not to be sustainable over time despite continuation of therapy. The use of nonhuman islet cells encapsulated in immunoprotective, semipermeable membranes may have a future in the treatment of type 1 DM (Campbell, 2004).

Other routes for insulin administration are being studied. Inhaled insulin, for instance, has been an effective and safe means to control postprandial glucose levels (Garg, Rosenstock, Silverman, et al, 2006).

Monitoring: Daily monitoring of blood glucose levels is an essential aspect of appropriate DM management.

Blood Glucose:

Self-monitoring of blood glucose (SMBG) has improved diabetes management and is used successfully by children from the onset of their diabetes. By testing their own blood, children are able to change their insulin regimen to maintain their glucose level in the euglycemic (normal) range of 80 to 120 mg/dl. Diabetes management depends to a great extent on SMBG. In general, children tolerate the testing well. Table 38-4 lists plasma blood glucose and hemoglobin A1c goal ranges.

TABLE 38-4

PLASMA BLOOD GLUCOSE AND HEMOGLOBIN A1c GOALS FOR TYPE 1 DIABETES MELLITUS BY AGE-GROUP

image

*Plasma blood glucose goal range.

Modified from American Diabetes Association: Standards of medical care in diabetes, Diabetes Care 28(Suppl):S4-S36, 2005.

Glycosylated Hemoglobin:

The measurement of glycosylated hemoglobin (hemoglobin A1c) levels is a satisfactory method for assessing the control of the diabetic patient. As red blood cells circulate in the bloodstream, glucose molecules gradually attach to the hemoglobin A molecules and remain there for the lifetime of the red blood cell, approximately 120 days. The attachment is not reversible; therefore this glycosylated hemoglobin reflects the average blood glucose levels over the previous 2 to 3 months. The test is a satisfactory method for assessing control, detecting incorrect testing, monitoring effectiveness of changes in treatment, defining patients’ goals, and detecting nonadherence. Nondiabetic hemoglobin A1c values are generally between 4% and 6% but can vary by laboratory. Diabetes control for children depends on age, with hemoglobin A1c levels of 6.5% to 8% indicating a slightly elevated but acceptable result (American Diabetes Association, 2005). Table 38-5 gives comparisons of hemoglobin A1c to blood glucose levels.

TABLE 38-5

COMPARISON OF HEMOGLOBIN BLOOD GLUCOSE LEVELS TO HEMOGLOBIN A1c

image

Modified from American Diabetes Association: Standards of medical care in diabetes: clinical practice recommendations, Diabetes Care 28(Suppl):S4-S36, 2005.

Urine:

Urine testing for glucose is no longer used for diabetes management. There is poor correlation between simultaneous glycosuria and blood glucose concentrations. However, urine testing can be carried out to detect evidence of ketonuria.

image NURSING ALERT

It is recommended that urine be tested for ketones every 3 hours during an illness or whenever the blood glucose level is over 240 mg/dl when illness is not present.

Nutrition: Essentially the nutritional needs of children with diabetes are no different from those of healthy children. Children with diabetes need no special foods or supplements. They need sufficient calories to balance daily expenditure for energy and to satisfy the requirement for growth and development. Unlike the child without diabetes, whose insulin is secreted in response to food intake, insulin injected subcutaneously has a relatively predictable time of onset, peak effect, duration of action, and absorption rate depending on the type of insulin used. Consequently the timing of food consumption must be regulated to correspond to the time and action of the insulin prescribed.

Meals and snacks must be eaten according to peak insulin action, and the total number of calories and proportions of basic nutrients must be consistent from day to day. The constant release of insulin into the circulation makes the child prone to hypoglycemia between the three daily meals unless a snack is provided between meals and at bedtime. Calculate the distribution of calories to fit the activity pattern of each child. For example, a child who is more active in the afternoon needs a larger snack at that time. This larger snack might also be split to allow some food at school and some food after school. Food intake should be altered to balance food, insulin, and exercise. Extra food is needed for increased activity.

The food intake may be planned in a variety of ways but is based on a balanced diet that incorporates six basic food groups: milk, meat, vegetables, fat, fruit, and starch. There are several meal-planning approaches, including the exchange system and carbohydrate counting. The exchange system from the American Diabetes Association groups foods by nutrient content. Within each group, portion sizes of foods are calculated to give an equivalent amount of the nutrient. In the fruit list, for instance, one small apple has the equivalent amount of carbohydrate as a half banana. In the exchange system, food groups are important: fruits exchange with fruits, starches exchange with starches.

Carbohydrate counting has been popular since the Diabetes Control and Complications Trial. Portion sizes are still important, but all carbohydrates are considered equivalent. In this system, food groups are not as important as carbohydrate content. For example, one small apple and one slice of bread have the same carbohydrate amount (15 g) and may be used interchangeably.

Concentrated sweets are discouraged, and because of the increased risk for atherosclerosis in persons with DM, fat is reduced to 30% or less of the total caloric requirement. Dietary fiber has become increasingly important in dietary planning because of its influence on digestion, absorption, and metabolism of many nutrients. It has been found to diminish the rise in blood glucose after meals.

Correctly used, the diet allows for flexibility and the incorporation of preferred foods in most instances. For the growing child, never use food restriction for diabetic control, although caloric restrictions may be imposed for weight control if the child is overweight. In general, the child’s appetite should be the guide for the amount of calories needed, with the total caloric intake adjusted to appetite and activity. Box 38-15 outlines basic principles of diet management.

BOX 38-15   NUTRITIONAL MANAGEMENT IN TYPE 1 DIABETES MELLITUS

Goal

Attain metabolic control of glucose and lipid levels

Objectives

Appropriate meal and snack planning:

• Achieve a dietary balance of carbohydrates, fats, and proteins.

• Provide extra food during periods of exercise.

• Time meals consistently to prevent hypoglycemia.

• Avoid high-sugar, high-carbohydrate foods to prevent hyperglycemia.

Develop an appropriate insulin regimen and physical activity program:

• Administer insulin as directed before eating.

• Increase insulin dose or activity level when extra food is eaten.

• Decrease insulin dose during periods of strenuous activity.

Exercise: Exercise is encouraged and never restricted unless indicated by other health conditions. Exercise lowers blood glucose levels, depending on the intensity and duration of the activity. Consequently exercise should be included as part of diabetes management, and the type and amount of exercise should be planned around the child’s interests and capabilities. However, in most instances children’s activities are unplanned, and the resulting decrease in blood glucose can be compensated for by providing extra snacks before (and, if the exercise is prolonged, during) the activity. In addition to a feeling of well-being, regular exercise aids in utilization of food and often results in a reduction of insulin requirements.

Physical training tends to increase tissue sensitivity to insulin, even in the resting state. Consequently it is especially important to understand the relationship between the activity and the diabetic regimen. Vigorous muscular contraction increases regional blood flow and accelerates the absorption and circulation of insulin that is injected into the area, which can contribute to development of hypoglycemia. If exercise involving leg muscles is planned, it is recommended that nonexercised sites (arm or abdomen) be used for insulin injection. This practice may replace the need for further increased carbohydrate intake or a reduced insulin dose (or both) to avoid exercise-induced hypoglycemia.

Children with poorly controlled diabetes are particularly at risk for hyperglycemia with exercise, or exercise may actually stimulate ketoacid production. Therefore discourage the child who has marked hyperglycemia (blood glucose level >240 mg/dl) and ketonuria from strenuous physical activity until the diabetes is controlled by appropriate adjustments of insulin and diet.

Athletes and youngsters who regularly participate in organized sports should adjust their insulin dosage in anticipation of sustained physical activity during the part of the day devoted to strenuous exercise. Team sports may encourage overexertion and subsequent hypoglycemia. Insulin dosages are decreased for participation in organized sports or prolonged activities, such as swimming for several hours. It is important that the patient consult with the practitioner for advice on insulin dose adjustment.

Hypoglycemia: Occasional episodes of hypoglycemia are an integral part of insulin therapy, and an objective of diabetes management is to achieve the best possible glycemic control while minimizing the frequency and severity of hypoglycemia. Even with good control, a child may frequently experience mild symptoms of hypoglycemia. If the signs and symptoms are recognized early and promptly relieved by appropriate therapy, the child’s activity should be interrupted for no more than a few minutes

image NURSING ALERT

Hypoglycemic episodes most commonly occur before meals, or when the insulin effect is peaking.

The most common causes of hypoglycemia are bursts of physical activity without additional food, or delayed, omitted, or incompletely consumed meals. Reglycosylation of muscles may occur over the ensuing 24 hours. Particular vigilance related to hypoglycemia may be necessary during the night after vigorous exertion. Occasionally, hypoglycemic reactions occur unexpectedly and without apparent cause. They may be the result of an inadvertent or deliberate error in insulin administration.

Gastroenteritis, in which there is gastric stasis, may impede the absorption of food, even though the child is eating reasonably well. It can also occur when the blood glucose level is so low that it causes stasis. Then the child may eat a meal or snack and still have an insulin reaction. Continued feeding does not seem to alter the blood glucose level because the simple glucose or sugar remains in the stomach.

The signs and symptoms of hypoglycemia are caused by both increased adrenergic activity and impaired brain function. The increased adrenergic nervous system activity plus increased secretion of catecholamines produces nervousness, pallor, tremulousness, palpitations, sweating, and hunger. Weakness, dizziness, headache, drowsiness, irritability, loss of coordination, seizures, and coma are more severe responses and reflect CNS glucose deprivation and the body’s attempts to elevate the serum glucose levels.

It is often difficult to distinguish between hyperglycemia and a hypoglycemic reaction (Table 38-6). Because the symptoms are similar and usually begin with changes in behavior, the simplest way to differentiate the two is to test the blood glucose level. The blood glucose level is low in hypoglycemia, whereas in hyperglycemia the glucose level is significantly elevated. Urinary ketones may be present after hypoglycemia as a result of starvation ketone production. In doubtful situations it is safer to give the child some simple carbohydrate. This will help alleviate the symptoms in the case of hypoglycemia but will do little harm if the child is hyperglycemic.

TABLE 38-6

COMPARISON OF MANIFESTATIONS OF HYPOGLYCEMIA AND HYPERGLYCEMIA

image

Children are usually able to detect the onset of hypoglycemia, but some are too young to implement treatment. Parents should become adept at recognizing the onset of symptoms—for example, a change in a child’s behavior, such as tearfulness or euphoria. In the majority of cases, 10 to 15 g of simple carbohydrate, such as 1 tbsp of table sugar, will elevate the blood glucose level and alleviate the symptoms. The simpler the carbohydrate, the more rapidly it will be absorbed (8 oz of milk equals 15 g of carbohydrate). The rapid-releasing sugar is followed by a complex carbohydrate such as a slice of bread or a cracker and by a protein such as peanut butter or milk.

For a mild reaction, milk or fruit juice is good to use in children. Milk supplies them with lactose or milk sugar, as well as a more prolonged action from the protein and fat (helps decrease absorption). Other glucose sources include Insta-Glucose (cherry-flavored glucose), carbonated drinks (not sugarless), sherbet, gelatin, or cake icing. All children with diabetes should carry with them glucose tabs, Insta-Glucose, sugar cubes, or sugar-containing candy such as LifeSavers or Charms. A difficulty with candies or icing is that the child may learn to fake a reaction to get the sweets; therefore commercial treatment products such as Insta-Glucose or glucose tabs may be preferred.

When in doubt, it is best to assume hypoglycemia and treat, but overtreatment could result in hyperglycemia. The treatment may be repeated in 10 to 15 minutes if the initial response is not satisfactory. Rest and the addition of food should be part of the plan.

An insulin reaction is often the most feared aspect of diabetes because severe brain symptoms may develop. In a severe reaction the various areas of the brain respond in sequence: the forebrain with increased drowsiness and perspiration, the hypothalamus and thalamus with tachycardia and loss of consciousness, the midbrain with seizure activity that may be started from stimulation initially from the hypothalamus, and finally the hindbrain with responses of deeper coma and decreasing reflexes. The treatment of choice for severe hypoglycemia is 50% glucose administered intravenously.

Glucagon is sometimes prescribed for home treatment of hypoglycemia. It is available as an emergency kit that must be mixed at the time of use and is administered intramuscularly or subcutaneously. Glucagon functions by releasing stored glycogen from the liver and requires about 15 to 20 minutes to elevate the blood glucose level.

image NURSING ALERT

Vomiting may occur after administration of glucagon; therefore take precautions against aspiration (e.g., placing the child on the side), since the child will be unconscious.

Once the child is responsive, the lost glycogen stores are replaced by small amounts of sugar-containing fluid administered frequently until the child feels comfortable about trying solid foods.

Morning Hyperglycemia:

The management of elevated morning blood glucose levels depends on whether the increase is a true dawn phenomenon, insulin waning, or a rebound hyperglycemia (the Somogyi effect). Insulin waning is a progressive rise in blood glucose levels from bedtime to morning. It is treated by increasing the nocturnal insulin dose. The true dawn phenomenon shows a relatively normal blood glucose level until about 3 am, when the level begins to rise. The Somogyi effect may occur at any time but often entails an elevated blood glucose level at bedtime and a drop at 2 am with a rebound rise following. The treatment for this phenomenon is decreasing the nocturnal insulin dose to prevent the 2 am hypoglycemia. The rebound rise in the blood glucose level is a result of counterregulatory hormones (epinephrine, GH, and corticosteroids), which are stimulated by hypoglycemia. More frequent blood monitoring (especially at times of anticipated peak insulin action) usually identifies these conditions. Trace amounts of urinary ketones aid in identifying undetected hypoglycemia.

Illness Management: Illness alters diabetes management, and maintaining control is usually related to the seriousness of the illness. In the well-controlled child an illness runs its course as it does in the unaffected child.

The goals during an illness are to restore euglycemia, treat urinary ketones, and maintain hydration. Monitor blood glucose levels and urinary ketones every 3 hours. Some hyperglycemia and ketonuria are expected in most illnesses, even with diminished food intake, and are an indication for increased insulin. Insulin should never be omitted during an illness, although dosage requirements may increase, decrease, or remain unchanged, depending on the severity of the illness and the child’s appetite. Often the child needs supplemental insulin between usual dose times. If the child vomits more than once, if blood glucose levels remain above 240 mg/dl, or if urinary ketones remain high, notify the health care practitioner. Simple carbohydrates may be substituted for carbohydrate-containing exchanges in the meal plan. Although insulin and diet are important tools in sick-day care, fluids are the most important intervention. Fluids must be encouraged to prevent dehydration and to flush out ketones.

Surgery: The physiologic and emotional stresses related to surgery require careful adjustment of insulin. Because the child receives IV glucose during surgery and the stress of the surgery itself also raises the blood glucose level, the risk of an insulin reaction is slight. Short-acting insulins should be continued until the child is able to tolerate oral feedings and return to the routine pattern of insulin administration.

Prevention: Major advances have been made in the ability to detect susceptibility to type 1 DM, and animal studies indicate that the disease can be prevented by various immunologic interventions (Skyler and Marks, 2003). Early immunosuppression may preserve long-term endogenous insulin secretion in individuals with type 1 DM.

Cyclosporine, mycophenolic acid, and nicotinamide have shown promise in delaying beta cell destruction or lowering the incidence of type 1 DM in relatives of children with the disease. Much progress has been made in the identification of islet cell antigens targeted by the immune response. Many potential treatments to prevent type 1 DM have appeared, and human trials have begun.

Therapeutic Management: Diabetic Ketoacidosis

image DKA, the most complete state of insulin deficiency, is a life-threatening situation. Management consists of rapid assessment, adequate insulin to reduce the elevated blood glucose level, fluids to overcome dehydration, and electrolyte replacement (especially potassium).

imageNursing Care Plan—The Child with Diabetic Ketoacidosis

DKA constitutes an emergency situation; therefore the child should be admitted to an intensive care facility for management. The priority is to obtain venous access for administration of fluids, electrolytes, and insulin. The child should be weighed, measured, and placed on a cardiac monitor. Blood glucose and ketone levels are determined at the bedside, and samples are obtained for laboratory measurement of glucose, electrolytes, blood urea nitrogen, arterial pH, Po2, Pco2, hemoglobin, hematocrit, white blood cell count and differential, calcium, and phosphorus.

Oxygen may be administered to patients who are cyanotic and in whom arterial oxygen is less than 80%. Gastric suction is applied to unconscious children to avoid the possibility of pulmonary aspiration. Antibiotics may be administered to febrile children after appropriate specimens are obtained for culture. A Foley catheter may or may not be inserted for urine samples and measurement. Unless the child is unconscious, a collection bag is usually sufficient for accurate assessments.

Fluid and Electrolyte Therapy: All patients with DKA suffer from dehydration (10% of total body weight in severe ketoacidosis) because of the osmotic diuresis, accompanied by depletion of electrolytes, sodium, potassium, chloride, phosphate, and magnesium. Serum pH and bicarbonate reflect the degree of acidosis. Prompt and adequate fluid therapy restores tissue perfusion and suppresses the elevated levels of stress hormones.

The initial hydrating solution is 0.9% saline solution. Traditionally deficits have been replaced at a rate of 50% over the first 8 to 12 hours and the remaining 50% over the next 16 to 24 hours. Current trends suggest more cautious fluid management to reduce the risk of cerebral edema. Consequently, the recommendations now are to replace the deficit evenly over 36 to 48 hours (Cooke and Plotnick, 2004).

image DRUG ALERT

Potassium and Serum Potassium levels

Potassium must never be given until the serum potassium level is known to be normal or low and urinary voiding is observed. All maintenance IV fluids should include 30 to 40 mEq/L of potassium unless the potassium concentration is elevated or urinary output is absent (Cooke and Plotnick, 2004). Never give potassium as a rapid IV bolus, or cardiac arrest may result.

Serum potassium levels may be normal on admission, but after fluid and insulin administration the rapid return of potassium to the cells can seriously deplete serum levels, with the attendant risk of cardiac arrhythmias. As soon as the child has established renal function (is voiding at least 25 ml/hr) and insulin has been given, vigorous potassium replacement is implemented. The cardiac monitor is used as a guide to therapy, and the nurse should observe the configuration of T waves every 30 to 60 minutes to determine changes that might indicate alterations in potassium concentration (widening of the Q-T interval and the appearance of a U wave following a flattened T wave indicate hypokalemia; an elevated and spreading T wave and shortening of the Q-T interval indicate hyperkalemia).

Insulin should not be given until after obtaining urine ketones and a blood glucose level. Continuous IV regular insulin is given at a dosage of 0.1 units/kg/hr. Insulin therapy should be started after the initial rehydration bolus, since serum glucose levels fall rapidly after volume expansion. Blood glucose levels should decrease by 50 to 100 mg/dl/hr. When blood glucose levels fall to 250 to 300 mg/dl, dextrose is added to the IV solution. The goal is to maintain blood glucose levels between 120 and 240 mg/dl by adding 5% to 10% dextrose. Sodium bicarbonate is used conservatively; it is used for pH less than 7.0, severe hyperkalemia, or cardiac instability. Because sodium bicarbonate has been associated with increased risk for cerebral edema, children receiving this substance must be carefully monitored for changes in level of consciousness (Brown, 2004). If bicarbonate treatment is necessary, 1 to 2 mEq/kg should be added to the IV fluids to run over 1 to 2 hours (Cooke and Plotnick, 2004).

When the critical period is over, the task of regulating insulin dosage in relation to diet and activity is started. Children should be actively involved in their own care and are given responsibility according to their ability and the guidance of the nurse.

image DRUG ALERT

Insulin and Tubing

Because insulin can chemically bind to plastic tubing and in-line filters, thereby reducing the amount of medication reaching the systemic circulation, an insulin mixture is run through the tubing to saturate the insulin-binding sites before the infusion is started.

Nursing Care of the Child with Diabetes Mellitus: Acute Care

Children with DM may be admitted to the hospital at the time of their initial diagnosis; during illness or surgery; or for episodes of ketoacidosis, which may be precipitated by any of a variety of factors. Many children are able to keep the disease under control with periodic assessment and adjustment of insulin, diet, and activity as needed under the supervision of a practitioner. Under most circumstances these children can be managed well at home and require hospitalization only for a serious illness or upset (see Research Focus box).

image RESEARCH FOCUS

Outpatient Treatment of Type 1 Diabetes

A Cochrane systematic review of seven studies evaluating whether children newly diagnosed with type 1 diabetes should be admitted to a hospital or treated in the outpatient setting found no disadvantages to allowing the child to remain as an outpatient. Studies evaluated metabolic control, acute diabetic complications and hospitalizations, psychosocial variables and behavior, and total care costs (Clar, Waugh, and Thomas, 2007).

However, a small number of children with diabetes exhibit a degree of metabolic lability and have repeated episodes of DKA that require hospitalization, which interferes with education and social development. These children appear to display a characteristic personality structure. They tend to be unusually passive and nonassertive and to come from families that are inclined to smooth over conflicts without resolution. Children in this type of setting experience emotional arousal with little, if any, opportunity or ability to resolve it. Other children from psychosocially dysfunctional families display behavioral and personality problems. This emotional stress causes an increased production of endogenous catecholamines, which stimulate fat breakdown, leading to ketonemia and ketonuria.

Loving discipline is a supportive measure for any child; however, children with poorer diabetic control come from predominantly disruptive family units with little or no discipline. Lack of control is psychologically harmful. Because many of the psychosocial problems are not immediately apparent, psychosocial assessment by professionals is required, together with ongoing emotional support and counseling to reverse the patterns of ketoacidosis (Wolsdorf, Glaser, and Sperling, 2006).

Hospital Management: The child with DKA requires intensive nursing care. Observe vital signs and record them frequently. Hypotension caused by the contracted blood volume of the dehydrated state may cause decreased peripheral blood flow, which can be particularly hazardous to the heart, lungs, and kidneys. An elevated temperature may indicate infection and should be reported so that treatment can be implemented immediately.

Maintain careful and accurate records, including vital signs (pulse, respiration, temperature, and blood pressure), weight, IV fluids, electrolytes, insulin, blood glucose level, and intake and output. Use a urine collection device or retention catheter to obtain the urine measurements, which include volume, specific gravity, and glucose and ketone values. The volume relative to the glucose content is important because 5% glucose in a 300-ml sample is a significantly greater amount than a similar reading from a 75-ml sample. A diabetic flow sheet maintained at the bedside provides an ongoing record of the vital signs, urine and blood tests, amount of insulin given, and intake and output. Assess the level of consciousness and record it at frequent intervals. The comatose child generally regains consciousness fairly soon after initiation of therapy but is managed like any unconscious child during that time.

When the critical period is over, the task of regulating insulin dosage to diet and activity begins. The same meticulous records of intake and output, urine glucose and acetone levels, and insulin administration are maintained. Capable children should be actively involved in their own care and are given responsibility for keeping the intake and output record, testing the blood and urine, and, when appropriate, administering their own insulin—all under the nurse’s supervision and guidance (see Nursing Care Plan).

image NURSING CARE PLAN

The Child with Diabetes Mellitus

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Nursing Care of the Child with Diabetes Mellitus: General Care

image Diabetes management involves a constant state of assessment. Daily monitoring of blood glucose levels; periodic urinalysis for ketones; and observation for signs of hypoglycemia, hyperglycemia, or other complications are part of the daily life of children with diabetes and their families. Suspect diabetes in any child who exhibits the manifestations of hypoglycemia or hyperglycemia, and refer the child for further assessment and appropriate testing.

image Nursing Care Plan—The Child with Diabetes Mellitus

QUALITY PATIENT OUTCOMES

Diabetes Mellitus

• Blood glucose levels maintained within normal range

• HbA1c range from 6.5% to 8%

• DKA prevented

The nurse should be alert to evidence of complications, although these are usually not manifested until adulthood. Assessment of skin for evidence of breakdown is important so that appropriate care can be implemented to facilitate healing and prevent infection. Because illnesses, such as respiratory tract infections or gastrointestinal upsets, complicate the diabetes management, they should be detected early.

Education is the cornerstone of diabetes management and the major responsibility in diabetes nursing care. This includes education and reinforcement of information for the family and for children who are old enough to participate in self-management of the disease. With younger children, parents must supervise and manage their therapeutic program, but children should assume some responsibility for self-management as soon as they are capable. Children can assist with blood glucose testing at a relatively young age, and most should be able to administer their own insulin at about 9 years of age. In situations in which the parents are inconsistent or unreliable, the child can learn self-care at an earlier age. However, education programs cannot be conducted as one-time activities with the expectation that they will achieve permanent behavior changes. Education is an ongoing nursing activity as family and patient needs change and new findings are applied.

Concepts of Child and Family Education: Children and their families vary in educational background and the capacity to learn and understand the various aspects of the therapeutic program. Some families respond best to simple explanations and directions, whereas others expect thorough, in-depth information about the physiologic processes and responses associated with the disease and its therapy. All the principles of teaching and learning are applied in the educational process; therefore, before beginning, the nurse must determine the optimum time, place, method, and content to be taught. Self-management, the ultimate goal for children with diabetes, is more likely to occur when children understand the disease and the care it requires. Properly educated and motivated, most families should be able to follow a program of regulated control satisfactorily.

When to teach a family and child is best judged by their psychologic state and emotional readiness. When a child is newly diagnosed, the psychologic adjustment to the disease can block the learning process completely. For example, in a follow-up visit family members may state that they are hearing a certain bit of information for the first time even though the material had been covered several times in the course of teaching.

Certainly the first 3 or 4 days after diagnosis are not an optimum time for complex learning. In fact, the later the more complex material is presented, the better. For example, one successful program teaches only essential, or survival, information first and intense information a month later. Another program advocates teaching 1 week after diagnosis followed by a review of survival techniques 2 weeks after discharge. Probably the worst time for teaching is the day or so after diagnosis, when the education must be compressed into a few hours or days so that the child can be discharged early.

Regardless of the teaching plan, the nurse must accurately assess individual ability to learn. This includes assessment of the individuals’ educational background and emotional stability and the use of appropriate measurement tools, such as a pretest or an objective assessment of the learners’ educational level. The stepped approach to patient education employs the method of simple to complex. The stepped approach involves (1) using good interpersonal skills, (2) teaching about the illness and regimen, and (3) overcoming obstacles to behavior change.

The setting for the educational process can facilitate learning. If the child must be hospitalized, bedside education may be necessary in some cases, but the coming and going of a number of people is distracting. At times in the educational process individual instruction is needed, but contact with other children or parents can assist in adjustment to the reality of the disease and the implications of having a chronic condition.

A child learns best when sessions are short, no more than 15 to 20 minutes. The parents do best with periods of 45 to 60 minutes, or longer if they are inquisitive. Education should involve all the senses. Although visual aids are valuable tools, participation is the most effective method for learning. For example, to teach blood glucose testing, the nurse explains the technique, demonstrates the procedure, and allows the learner to perform the procedure; this is followed by a review of the material using visual aids, with learning validated by some testing method that includes feedback. A variety of teaching methods and teaching aids can be used. Some visual aids may be beautifully illustrated but miss a major point; therefore materials should be previewed for accuracy and appropriateness. Varying the presentation with a variety of audiovisual materials, including films, slide-tape programs, and books, stimulates the senses and helps the individual learn.

Several organizations are prepared to assist with education and dissemination of knowledge about diabetes. The American Diabetes Association,* Canadian Diabetes Association, Juvenile Diabetes Research Foundation International, and American Association of Diabetes Educators§ are valuable resources for a wide variety of educational materials. The National Institute of Diabetes and Digestive and Kidney Diseases publishes a number of comprehensive annotated bibliographies, including “Educational Materials for and About Young People with Diabetes,” a compilation of resource materials for children, siblings, parents, teachers, and health professionals; and “Sports and Exercise for People with Diabetes.”

The content of the educational course must include all aspects of the disease as they relate to the individual child. Many aspects of the disease may not be covered in an initial educational course but can be postponed until subsequent office or clinic visits or can be done through referral sources such as the American Diabetes Association. The minimum information needed should help the family manage from one day to the next; expanded information helps the individual with long-term adjustment to the disease. The more the family understands about the disease in relation to body needs, the better they are able to maintain a high degree of control. Important content needed for minimum management is discussed briefly in the following sections.

Identification: One of the first things the nurse should call to the parents’ attention is the need for the child to wear some means of medical identification. Usually recommended is the Medic-Alert identification, a stainless steel, silver, or gold-plated identification bracelet that is visible and immediately recognizable. It contains a collect telephone number that medical personnel can call around the clock for medical records and personal information.

Nature of Diabetes: The better the parents understand the pathophysiology of diabetes and the function and action of insulin and glucagon in relation to caloric intake and exercise, the better they will understand the disease and its effects on the child. Parents need answers to a number of questions (voiced or unvoiced) to increase their confidence in coping with the disease. For example, they may want to know about the various procedures performed on their child and treatment rationale, such as what is being put in the IV bottle and the expected effect.

Meal Planning: Normal nutrition is a major aspect of the family education program. The nutritionist usually conducts diet instruction, with reinforcement and guidance from the nurse. The emphasis is on adequate intake for age, consistent menus, complex carbohydrates, and consistent eating times. The family learns how the meal plan relates to the requirements of growth and development, the disease process, and the insulin regimen. Meals and snacks are modified based on the child’s preferences and current menu, preserving cultural patterns and preferences as much as possible. Extensive exchange lists are available that include foods compatible with most lifestyles.

Learning about foods within specific food groups helps in making choices. Weights and measures of foods are used as eye-training devices for defining serving sizes and should be practiced for about 3 months, with gradual progression to estimation of food portions. Even when the child and family become competent in estimating portion sizes, reassessment should take place weekly or monthly and when there is any change of brands.

Family members should also be guided in reading labels for the nutritional value of foods and food content. They need to become familiar with the carbohydrate content of food groups. Substitution with foods of equal carbohydrate content is the skill needed for successful carbohydrate counting. Substitution might be necessary if a food is not available in sufficient quantity or for the teenager who wishes to eat fast food with peers. The use of a multiple daily injection program lends flexibility to the timing of meals.

Educating children or teenagers to make healthy food choices is an ongoing task. Teach younger children to choose from a special treat box stocked with sugar-free items when others bring high-sugar treats to the classroom. Discussions with school-age children might include situations encountered at school or parties, such as choosing food in the cafeteria or bringing substitute treats to parties. Role-playing and discussion help teenagers deal with food choices when on dates, with friends, or on a food break after school.

Lists of popular fast-food items and items served at the major fast-food chains can be obtained from the restaurants to help guide food selections. It is important that the child know the nutritional value of these items (the major chains are remarkably uniform), but the child should be cautioned to avoid high-fat, high-sugar, and high-carbohydrate items. For example, the child could choose a plain hamburger instead of a double cheeseburger.

Children should use sugar substitutes with moderation in items such as soft drinks. Artificial sweeteners have been shown to be safe, but if there is any question about amounts, the physician, dietitian, or nurse specialist can provide guidelines based on body weight. Sugar-free chewing gum and candies made with sorbitol may be used in moderation by children with DM. Although sorbitol is less cariogenic than other varieties of sugar substitutes, it is an alcohol sugar that is metabolized to fructose and then to glucose. Furthermore, large amounts can cause osmotic diarrhea. Most dietetic foods contain sorbitol. They are more expensive than regular foods. Also, although a product may be sugar free, it is not necessarily carbohydrate free.

Traveling: Traveling requires advance planning, especially when a trip involves crossing time zones. A number of tips are included in pamphlets available free of charge. Suggestions for traveling encompass what will be needed from the practitioner before leaving, what and how much to take along, needs in transit, what to consider at the destination, and planning for when the child returns home. Planning is needed no matter what type of travel is considered—automobile, plane, bus, or train.

Insulin: Families need to understand the treatment method and the insulin prescribed, including the effective duration, onset, and peak action. They also need to know the characteristics of the various types of insulins, the proper mixing and dilution of insulins, and how to substitute another type when their usual brand is not available (insulin is a nonprescription drug). Insulin need not be refrigerated but should be maintained at a temperature between 15° and 29.5° C (59° and 85° F). Freezing renders insulin inactive.

Insulin bottles that have been “opened” (i.e., the stopper has been punctured) should be stored at room temperature or refrigerated for up to 28 to 30 days. After 1 month these vials should be discarded. Unopened vials should be refrigerated and are good until the expiration date on the label. Diabetic supplies should not be left in a hot environment.

Injection Procedure: image Learning to give insulin injections is a source of anxiety for both parents and children. It is helpful for the learner to know that this important aspect of care will become as routine as brushing the teeth. First, the basic injection technique is taught, using an orange or similar item and sterile normal saline for practice. To gain children’s confidence, the nurse can demonstrate the technique by giving a skillful injection to the parent and then having the parent return the demonstration by giving the nurse an injection. With practice and confidence, the parents soon are able to give the insulin injection to their children, and their children trust them. Another effective strategy is to instruct the children and then have them teach the technique to the parents while the nurse observes. Both parents should participate, and as little time as possible should elapse between instruction and the actual injection, especially with parents and teenage learners.

imageAnimation—Insulin Injection

Insulin can be injected into any area in which there is adipose (fat) tissue over muscle; the drug is injected at a 90-degree angle. Newly diagnosed children may have lost adipose tissue, and care should be exerted not to inject intramuscularly. The pinch technique is the most effective method for tenting the skin to allow easy entrance of the needle to subcutaneous tissues in children. The site selected sometimes depends on whether children or parents administer the insulin. The arms, thighs, hips, and abdomen are usual injection sites for insulin. The children can reach the thighs, abdomen, and part of the hip and arm easily but may require help to inject other sites. For example, a parent can pinch a loose fold of skin of the arm while the child injects the insulin.

The parents and child are helped to work out a rotation pattern to various areas of the body to enhance absorption, since insulin absorption is slowed by fat pads that develop in overused injection areas. The most efficient rotation plan involves giving about four to six injections in one area (each injection about 2.5 cm [1 inch] apart, or the diameter of the insulin vial from the previous injection) and then moving to another area.

Remember that the absorption rate varies in different parts of the body (Table 38-7). Methodically using one anatomic area and then moving to another (as described in the previous paragraph) minimize variations in absorption rates. However, vigorous exercise, which enhances absorption from exercised muscles, also alters absorption. Therefore it is recommended that a site be chosen other than the exercising extremity (e.g., avoiding legs and arms when playing in a tennis tournament).

TABLE 38-7

ONSET AND DURATION OF ACTION RELATED TO INJECTION SITE

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From Albisser AM, Sperlich M: Adjusting insulins, Diabetes Educ 18(3):211-218, 1992.

Injection sites for an entire month can be determined in advance on a simple chart. For example, the “paper doll” (body outline) described on p. 138 can be constructed and insulin sites marked by the child. After injection, the child places the date on the appropriate site. To keep in practice, it is a good idea for the parent to give two or three injections a week in areas that are difficult for the child to reach.

The same basic methodology is used when teaching children to give their own insulin injections (Fig. 38-8). They should practice first on an orange or a doll, building courage gradually. The first attempt is usually awkward because children tend to slowly push the needle through the skin rather than using a quick approach. It is best not to pressure them into assuming this responsibility until they are ready. When children participate in a group learning situation or have an opportunity to observe their peers giving their own injections, they may become more motivated. Warn parents that at some time children will give themselves an uncomfortable injection at home and that they will need parental support and encouragement. Otherwise children may not wish to give themselves injections for some time.

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Fig. 38-8 School-age children are able to administer their own insulin.

Other devices are available for insulin injection and may offer advantages to some children. Children who do not wish to give themselves injections can learn to use a syringe-loaded injector (Inject-Ease). With the device, puncture is always automatic. Adolescents respond well to a self-contained and compact device resembling a fountain pen (NovoPen), which eliminates conventional vials and syringes. Preloaded pens may also improve adherence to intensive insulin regimens, improve lifestyle flexibility, and decrease pain (Rex, Jensen, and Lawton, 2006).

Teaching includes the proper way to equalize pressure in the bottle by injecting an amount of air equal to the amount of solution withdrawn and how to remove air bubbles from the syringe. When insulin doses are small, an air bubble in the syringe can displace a significant amount of medication. Since the introduction of the 0.5-ml and 0.3-ml syringes, the risk of incorrect dosage has diminished. Advise patients who have small doses of mixed insulins to use one of these syringes. Insulin syringes should be compared for accuracy, comfort, and strength. The family and child should be able to choose both “their” insulin and “their” syringe from a variety of samples. The needle length and gauge are also factors to consider from the point of view of comfort (e.g., use the shortest and smallest-gauge needle available). Some brands of syringes may be more comfortable than others. When currently available syringes are used, insulin injections of less than 2 units of U100 may have an unacceptably large error. Diluted insulin is sometimes used if the prescribed dose is less than 2 units. Special diluent is available from the insulin manufacturers (Eli Lilly, 2007).

When the child’s dosage requires the injection of both short- and intermediate-acting insulin at the same time, most families prefer to mix the two and use a single injection. Insulin can be premixed and stored in the refrigerator for later use. Commercially prepared insulin mixtures are also available (e.g., 70/30 and 50/50). To obtain maximum benefit from mixing insulins, the recommended practice is to (1) inject the measured amount of air (equivalent to the dosage) into the long-acting insulin; (2) inject the measured amount of air into the rapid-acting (clear) insulin and, without removing the needle; (3) withdraw the clear insulin; and (4) insert the needle (already containing the clear insulin) into the long-acting (cloudy) insulin and then withdraw the desired amount.

image DRUG ALERT

Mixing Insulin

When mixing types of insulin, always withdraw the clear, rapid-acting insulin into the syringe first, then the long-acting insulin. This avoids contaminating the short-acting insulin with the longer-acting insulin.

Inject the mixture either less than 5 minutes after mixing (before the zinc content of the long-acting insulin affects the action time of the short-acting insulin) or 15 or more minutes after mixing (to allow the insulins to resume long-acting and short-acting properties).

Nurses should also teach proper disposal of equipment after use in the home. Although not standard practice in the hospital, the use of a needle clipper is recommended to safely remove and house the used needle. The syringe plunger can be broken before disposal. An excellent means for syringe disposal is in an opaque, puncture-resistant container, such as an empty coffee can, bleach bottle, or milk carton. The container is labeled “biohazardous waste” and is discarded with similar material only, not with household refuse and consistent with local regulations.

Continuous Subcutaneous Insulin Infusion:

Some children are candidates for use of a portable insulin pump, and even some young children with unsatisfactory metabolic control can benefit from its use. The child and the parents learn to operate the device, including the mechanics of the pump, battery changes, and alarm systems. A number of devices are available on the market that vary in the basal rates they are able to deliver and in the cost of the equipment. Families can investigate the various devices and select the model that best suits their needs. Product information is available from pump manufacturers and distributors.

Parents and children learn (1) the technical aspects of the pump and self-monitoring of blood glucose; (2) prevention and treatment for hyperglycemia, sick-day management, and meal planning; (3) the effects of exercise, stress, and diet on blood glucose levels; and (4) decision-making strategies to evaluate blood glucose patterns and how to make adjustments in all aspects of the regimen.

Numerous blood glucose measurements (at least four times per day) are an essential part of infusion pump use. Intensive education and supervision are critical to obtaining maximum efficiency and control. This is particularly important if the family has been accustomed to a conventional insulin regimen. They must realize that simply wearing the pump will not normalize blood glucose. The pump is merely an insulin delivery device, and frequent, routine blood glucose determinations are necessary to adjust the insulin delivery rate.

The major problem with use of the insulin pump is inflammation from irritation or infection at the insertion site. The site should be cleaned thoroughly before the needle is inserted and then covered with a transparent dressing. The site is changed and rotated every 48 to 72 hours (this may vary) or at the first sign of inflammation. Nurses working where pumps are part of the therapeutic regimen should become familiar with the operation of the specific device being used and the protocol of disease management. Others should be aware of this management technique and be prepared to assist patients using the pump.

Monitoring: Nurses should also be prepared to teach and supervise blood glucose monitoring. SMBG is associated with few complications, and although it does not necessarily lead to improved metabolic control, it provides a more accurate assessment of blood glucose levels than can be obtained with the historical urine testing. Blood glucose monitoring has the added advantage that it can be performed anywhere (see Atraumatic Care box).

ATRAUMATIC CARE

Minimizing Pain of Blood Glucose Monitoring

• To enhance blood flow to the finger, hold it under warm water for a few seconds before the puncture.

• When obtaining blood samples, use the ring finger or thumb (blood flows more easily to these areas), and puncture the finger just to the side of the finger pad (more blood vessels and fewer nerve endings).

• To prevent a deep puncture, press the platform of the lancet device lightly against the skin and avoid steadying the finger against a hard surface.

• Use lancet devices with adjustable-depth tips. Begin with the shallowest setting.

• Use glucose monitors that require small blood samples (e.g., Ascensia Elite) to avoid repeated punctures.

Blood for testing can be obtained by two different methods: manually or with a mechanical bloodletting device. A mechanical device is recommended for children, although the child and family should learn to use both methods in the event of mechanical failure. Several lancet devices are available from which to choose, and each provides a means for obtaining a large drop of blood for testing (Fig. 38-9).

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Fig. 38-9 Child using finger-stick device to obtain blood sample.

image NURSING ALERT

Caution children not to allow anyone else to use their lancet because of the risk of contracting hepatitis B virus or human immunodeficiency virus infection.

The blood sample may be obtained from fingertips or alternate sites such as the forearm. Alternate site testing requires a meter that can test a small volume of blood. Not all meters are capable of this.

The practitioner should examine signs of redness and soreness at the site of finger puncture. It may be evidence of poor technique, poor hygiene, or poor skin healing relative to poor control. Many types of blood-testing meters are available for home use. Newer technology has brought about improvements in meter size and ease of use. The family should be shown features of several meters, including advantages and disadvantages, and allowed to choose equipment that best meets their needs.

The least expensive testing method uses a reagent strip to which blood is applied (Fig. 38-10). After blotting, the color change is compared to a color scale for an estimation of the blood glucose level. The strips can be cut in half (although not all professionals recommend this) to obtain two readings per strip. This method is not accepted practice but may be necessary for some families or situations.

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Fig. 38-10 Child using blood glucose monitor and reagent strips to test blood for glucose.

Urine Testing:

Testing for urinary ketones is recommended during times of illness or when blood glucose values are elevated. Information on a specific ketone-testing product should include correct procedure, storage, and product expiration. Families need a clear understanding of home management of ketones: fluids and additional insulin as directed by the health care team.

Shopping: Diabetic maintenance is an expensive necessity. Families should investigate all sources of obtaining supplies for managing the disease. Prices are often lower when supplies are purchased in volume; however, it is not advisable to buy bulk items that are unfamiliar, since the new items may not be satisfactory for the individual child or may become outdated before they are used. Costs vary considerably among pharmacies and other suppliers, including the numerous discount mail-order establishments. When buying by mail, it is important to find a supplier that responds to the family’s satisfaction and to allow ample time for delivery to avoid running out of supplies. Parents are also cautioned not to substitute insulins or the type of insulin syringe (e.g., a 1-ml syringe for the low-dose type) simply to save money. Parent groups and the local American Diabetes Association can offer some suggestions for investigation. Most states have legislation mandating that insurance companies cover the cost of diabetes supplies and education.

Hyperglycemia: Severe hyperglycemia is most often caused by illness, growth, emotional upset, or missed insulin doses. Emotional stress from school finals or examinations or physical response to immunizations are examples of causes of hyperglycemia. With careful glucose monitoring, any elevation can be managed by adjustment of insulin or food intake. Parents should understand how to adjust food, activity, and insulin at the time of illness or when the child is treated for an illness with a medication known to raise the blood glucose level (e.g., steroids). The hyperglycemia is managed by increasing insulin soon after the increased glucose level is noted.

Hypoglycemia: Hypoglycemia is caused by imbalances of food intake, insulin, and activity. Ideally hypoglycemia should be prevented, and parents need to be prepared to prevent, recognize, and treat the problem. They should be familiar with the signs of hypoglycemia and instructed in treatment, including care of the child with seizures. (See Chapter 37.) Early signs are adrenergic, including sweating and trembling, which help raise the blood glucose level, much like the reaction when an individual is startled or anxious. The second set of symptoms that follow an untreated adrenergic reaction are neuroglycopenic (also called brain hypoglycemia). These symptoms typically include difficulty with balance, memory, attention, or concentration; dizziness or lightheadedness; and slurred speech. Severe and prolonged hypoglycemia leads to seizures, coma, and possible death (Cryer, 2003). In particular, infants are at an increased risk because of the developing brains’ vulnerability to the potential neurotoxic insult of hypoglycemia (Cooke and Plotnick, 2004). Hypoglycemia can be managed effectively (see Emergency Treatment box).

image EMERGENCY TREATMENT

Hypoglycemia

Mild Reaction: Adrenergic Symptoms

Give child 10 to 15 g of a simple, high-carbohydrate substance (preferably liquid, e.g., 3 to 6 oz of orange juice).

Follow with starch-protein snack.

Moderate Reaction: Neuroglycopenic Symptoms

Give child 10 to 15 g of a simple carbohydrate as above.

Repeat in 10 to 15 minutes if symptoms persist.

Follow with larger snack.

Watch child closely.

Severe Reaction: Unresponsive, Unconscious, or Seizures

Administer glucagon as prescribed.

Follow with planned meal or snack when child is able to eat, or add a snack of 10% of daily calories.

Nocturnal Reaction

Give child 10 to 15 g of a simple carbohydrate.

Follow with snack of 10% of daily calories.

It is advisable for parents to plan for anticipated excitement or exercise. In addition, gastroenteritis may decrease insulin needs slightly as a result of poor appetite, vomiting, or diarrhea. If the blood glucose level is low but urinary ketones are present, the family should be aware of the increased need for simple carbohydrates and liquids.

Hygiene: All aspects of personal hygiene should be emphasized for the child with diabetes. Caution the child against wearing shoes without socks, wearing sandals, or walking barefoot. Correct nail and extremity care tailored to the individual child (with the guidance of a podiatrist) can begin health practices that last a lifetime. Eyes should be checked once a year unless the child wears glasses, and then as directed by the ophthalmologist. Regular dental care is emphasized, and cuts and scratches should be treated with plain soap and water unless otherwise indicated. Diaper rash in infants and candidal infections in teens may indicate poor diabetes control.

Exercise: Exercise is an important component of the treatment plan. If the child is more active at one time of the day than at another time, food or insulin can be altered to meet that activity pattern. Food should be increased in the summer, when children tend to be more active. Decreased activity on return to school may require a decrease in food intake or increase in insulin dosage. The child who is active in team sports needs a snack about a half hour before the anticipated activity. Races or other competition may call for a slightly higher food intake than practice times.

Food intake usually needs to be repeated for prolonged activity periods, often as frequently as every 45 minutes to 1 hour. Families should be informed that if increased food is not tolerated, decreased insulin is the next course of action. If the timing of the exercise is changed so that the supper meal is delayed, the insulin in the second or third dose of the day may be moved back to precede the mealtime. Sugar may sometimes be needed during exercise periods for quick response. Elevated blood glucose levels after extreme activity may represent the body’s adrenergic response to exercise. If the blood glucose level is elevated (>240 mg/dl) before planned exercise, urine ketones should be checked and the activity may need to be postponed until the blood glucose is controlled.

Without adequate insulin levels the cells are unable to receive glucose, the preferred fuel, despite the high level of blood glucose. The low insulin level allows glucagon to act, uninhibited, to increase hepatic glucose production, further raising the blood glucose level with no means to use sugar at the muscle site. Breakdown of fat (lipolysis) is the alternative, and the end product of lipolysis is ketone body production (Cooke and Plotnick, 2008).

image NURSING ALERT

Ketonuria in the presence of hyperglycemia is an early sign of ketoacidosis and a contraindication to exercise.

Record Keeping: Home records are an invaluable aid to diabetes self-management. The nurse and family devise a method to chart insulin administered, blood glucose values, urine ketone results, and other factors and events that affect diabetes control. The child and family should observe for patterns of blood glucose responses to events such as exercise. If lapses in management occur (such as eating a candy bar), the child should be encouraged to note this and not be criticized for the transgression.

Complications: The nurse should present the implications of the disease in a tactful, clear, and nonthreatening manner. Knowledge of the complications of diabetes and their relationship to control provides a basis for knowledgeable decision making. Eye and kidney diseases are the greatest threats, with neurologic complications close behind. Clear explanations of these problems clarify false information often given by well-meaning friends. The information should include discussion of research so that the family is left with the positive impression that others are concerned about finding answers and preventing complications. It also gives them hope that somehow, some way, a prevention or cure will be possible.

Self-Management: Self-management is the key to close control. Being able to make changes when they are needed rather than waiting until the next contact with health care professionals is important for self-management and gives the individual and family the feeling they have control over the disease. Psychologically this helps family members feel they are useful and participating members of the team. Allowing the child to learn to look at records objectively promotes independence in self-management support. As children grow and assume more responsibility for self-management, they develop confidence in their ability to manage their disease and confidence in themselves as persons. They learn to respond to the disease and to make more accurate interpretations and changes in treatment when they become adults.

Self-management techniques to be mastered are the testing and adjustment of insulin and diet with alterations in day-to-day activities and anticipation of unusual occurrences. However, the nurse should provide guidelines regarding when to consult with the health care professionals. For instance, the degree of metabolic control before an illness is a determining factor in seeking medical help during the illness. In an individual with poor control, it takes only a few hours before the trouble is severe, whereas if control is good before the illness, several days may elapse before help is needed. Also caution patients and families to seek assistance if glucose levels are elevated and urine is not clear of ketones after 24 hours of self-management.

Child and Family Support: Just as the physiologic responses affect the child, the parents and other family members of the child with newly diagnosed DM experience various emotional responses to the crisis. Care in the acute setting is short but may create fears and frustrations. The prospect of a chronic illness in their child engenders all the feelings and concerns that are faced by parents of children with other chronic illnesses. (See Chapter 22.) The threat of complications and death is always present, as well as the continuing drain on emotional and financial resources.

Certain fears may develop as a result of past experiences with the disease. A severe insulin reaction with seizures can contribute to fear of repetition. Once parents observe a seizure or the adolescent has one in a public place, the desire to maintain better control is reinforced. They must understand how to prevent problems and how to handle problems calmly and coolly if they occur, and they must understand the complexities of the body, the disease, and its complications. Young children usually adjust well to problems related to the disease. With toddlers and preschoolers, insulin injections and glucose testing may be difficult at first. However, they usually accept the procedures when the parents use a matter-of-fact approach without calling attention to a “hurt” and treat the procedure like any other routine part of the child’s life. After the injection, time with some special and positive attention, such as reading, talking, or another pleasant activity, is one way to convert children who initially refuse injections to those who accept them.

In the years before adolescence children probably accept their condition most easily. They are able to understand the basic concepts related to their disease and its treatment. They are able to test blood glucose and urine; recognize food groups; give injections; keep records; and distinguish between fear, excitement, and hypoglycemia. They understand how to recognize, prevent, and treat hypoglycemia. However, they still need considerable parental involvement.

NURSING TIP

Ongoing motivation to adhere to a regimen is difficult. An older child and parent (or another caregiver) may enjoy negotiating a day off when the responsibility for testing and recording blood glucose is delegated from the child to the caregiver (or vice versa).

Adolescents appear to have the most difficulty adjusting. Adolescence is a time of stress in trying to be perfect and like one’s peers, and no matter what others say, having diabetes is being different. Some adolescents are more upset about not being able to have a candy bar than about injections, diet, and other aspects of management. If children can accept the difference as a part of life—in other words, that each person is different in some way—then with adequate parental support they should be able to adjust well.

Problems of adjustment to diabetes are especially difficult for the young person whose disease is diagnosed in adolescence. Denial is sometimes expressed by omitting insulin, not performing tests, and eating incorrectly, although denial of the disease usually diminishes during this period as the adolescent with DM begins to feel competent and worthy. Diabetes makes the teenager different when conformity and sameness are desired; having the disease emphasizes vulnerability and imperfection when the search for identity is the foremost developmental task of adolescence. It is often difficult for the adolescent to know what to tell friends.

Camping and other special groups are useful. At diabetes camp, children learn that they are not alone. As a result, they become more independent and resourceful in other settings. Useful information about such camps and organizations can be obtained from the American Diabetes Association. A list of accredited camps specifically for children and teenagers with diabetes is also available from the American Camp Association.*

Puberty is associated with decreased sensitivity to insulin that normally would be compensated for by an increased insulin secretion. Health care professionals should anticipate that pubertal patients will have more difficulty maintaining glycemic control. Insulin doses commonly need to be increased, often dramatically (Tfayli and Arslanian, 2007). Patients should learn to give themselves additional doses of rapid-acting insulin (5% to 10% of their daily dose) when their blood glucose levels are increased. The use of supplemental rapid-acting insulin is preferred to withholding food in the adolescent.

Eating disorders, such as bulimia or anorexia nervosa, in the teenager with type 1 DM (see Chapter 21) pose a serious health hazard (Ackard, Vik, Neumark-Sztainer, et al, 2008). The nurse should be alert to a history of preoccupation with weight, food faddism, excessive caloric restriction, or unexplained hypoglycemia. Moreover, insulin manipulation or omission has been identified as a weight loss tool used by some female adolescents (Tierney, Deaton, Webb, et al, 2008).

Inaccurate doses of insulin may occur inadvertently or, if they occur frequently, may be an attention-seeking device; in a number of cases they may demonstrate adolescent depression and, more seriously, a subconscious but socially accepted method of suicide. Excessive intake of food leads to obesity and may also be symptomatic of depression. Psychiatric counseling may be needed if suicidal tendencies are amplified by the diabetes.

Rehospitalizations are most often related to poor control of the disease, although they may be indirectly related to poor coping and are a method of avoiding the pressures caused by family and peers. The hospital may represent an environment that is peaceful and free of stress. The goal for this problem is to determine the cause of the hospitalization. It may be related to poor control, poor self-management, or the need for better supportive management at home. Evaluation should be based on the physiologic and psychologic adjustment of the child and family.

Parents:

Parents develop guilt feelings when they have a child with any chronic disease, especially one with a hereditary component. They cope with these feelings in a number of ways. For example, they may be overprotective or neglectful. Guilt-ridden parents may blame themselves for the disease, consciously or subconsciously. Nevertheless, they must come to realize through education and counseling that they could not have done anything to prevent the disease and it was not their fault, since both environmental and hereditary factors may be involved in the development of diabetes.

Parents who are overprotective of the child suffer from feelings of guilt and fear of the unknown. Overprotection is a mechanism that alters the guilt responses to justify the parents’ own needs—for example, “If the child is in my sight, nothing worse will happen than getting diabetes.” The overprotective parent becomes the smothering parent, one who hampers the child’s growth, development, and maturation.

The neglectful parent, on the other hand, has a different problem. This response is a mechanism developed to block feelings that give pain and provide relief from feelings of guilt—”This is your disease, and I have no responsibilities related to your disease; therefore, if anything bad happens to you as a result of this disease, it is not my fault.” The neglectful parent assigns responsibilities to the child before the child is mature enough to accept them.

Threatened parents look at the disease as a way to keep the child tied to them. If the child learns to be independent, as is expected during a camping experience, the parent may feel threatened and place obstacles in the child’s path to independent development. Problems in the parental response provide a challenge for the nurse to assist by counseling or, if severe enough, by referring the parents to resources designed to help them alter their behavior (see Critical Thinking Exercise).

image CRITICAL THINKING EXERCISE

Type 1 Diabetes Mellitus

Rebecca, a 15-year-old with a 3-year history of type 1 diabetes mellitus (DM), has been admitted to the pediatric intensive care unit for treatment of diabetic ketoacidosis (DKA). This is her fifth hospital admission for DKA in the past year. Rebecca’s parents are divorced, and she has four younger siblings, none of whom has diabetes. Rebecca’s mother has maintained two jobs for the past 5 years and frequently leaves Rebecca in charge of the household. In anticipation of her discharge you are planning a patient education program for Rebecca and her mother. What important issues regarding Rebecca’s unstable diabetes management must you consider to plan the education program?

1. Evidence—Is there sufficient evidence to draw conclusions about Rebecca’s recurrent episodes of DKA?

2. Assumptions—Describe an underlying assumption about each of the following:

a. Type 1 DM in adolescence

b. Type 1 DM and menses

c. Emotional stress and elevated blood glucose levels

d. Blood glucose monitoring for insulin management

3. What priorities for nursing care should be established for Rebecca?

4. Does the evidence support your nursing intervention?

Children who are sufficiently mature may be seen alone by the health care professional, although the parents should not be made to feel left out. Time should be set aside during the child’s health visit or afterward to meet the parents’ needs. Parents should also be included in special sessions to keep them abreast of the child’s management, to help them continue to participate in the child’s care, and to provide an opportunity to express their feelings concerning their own or their child’s adjustment to the disease. The amount of information that they offer at this time can give clues to their level of support of the child and assist in decisions concerning therapeutic management. This helps guide the child through the most disruptive time of life—the teenage years.

Health care professionals must be aware of parents who voice support and appear to be supporting the child to the optimum level but who, with more in-depth interviewing, are found not to be supportive. These parents seldom see the need for following through from verbalizing to meeting the child’s real needs, and they unknowingly place obstacles in the child’s path. They may be helping the child grow up too fast and therefore insecurely. These parents urgently need counseling so they can realize how their behavior affects the child. The classroom experience, group therapy, or parenting programs can guide the parents’ relationships with their children. All parents should be helped to recognize that, as children grow and develop, they are children first and children with diabetes second. The ultimate goal for these parents is to be supportive of their children, to communicate more effectively with them, and to help their children develop in an acceptable manner (Wysocki, Harris, Buchloh, et al, 2006).

Key Points

• The endocrine system has three components: the cell, which sends a chemical message via a hormone; target cells, which receive the message; and the environment through which the chemical is transported from the site of synthesis to the sites of cellular action.

• Pituitary dysfunction is manifested primarily by growth disturbance.

• The main physiologic action of TH is to regulate the basal metabolic rate and control the processes of growth and tissue differentiation.

• Disorders of thyroid function include hypothyroidism, autoimmune thyroiditis, goiter, and hyperthyroidism.

• Therapy for hyperthyroidism is directed at retarding the rate of hormone secretion and may include drug therapy, thyroidectomy, or radioiodine therapy.

• Classic forms of hypoparathyroidism in childhood are idiopathic (deficient production of PTH) and pseudohypoparathyroidism (increased PTH production with end-organ unresponsiveness to PTH).

• The adrenal cortex secretes three important groups of hormones: glucocorticoids, mineralocorticoids, and sex steroids.

• Disorders of adrenal function include acute adrenocortical insufficiency, chronic adrenocortical insufficiency, Cushing syndrome, CAH, and hyperaldosteronism.

• Five categories of Cushing syndrome are pituitary, adrenal, ectopic, iatrogenic, and food dependent.

• Management of CAH includes assignment of a sex according to genotype, administration of cortisone, and, possibly, reconstructive surgery.

• The focus of type 1 DM is insulin replacement, diet, and exercise.

• Education of families includes explanation of diabetes, meal planning, administering insulin injections, monitoring general hygiene practices, promoting exercise, record keeping, and observing for complications.

Answers to Critical Thinking Exercise

Type 1 Diabetes Mellitus

1. Yes. Rebecca has had five hospital admissions for DKA in the past year. Numerous factors must be involved with her unstable disease.

2. a. The normal tasks of adolescence can play a significant role in blood glucose instability.

b. Adolescent girls with diabetes have frequent fluctuations of blood glucose levels immediately before, during, or after their menses.

c. Rebecca’s personal loss from the divorce, her mother’s absence because of a heavy work schedule, and the added responsibilities of the household may cause significant stress, resulting in elevated blood glucose levels.

d. Careful, frequent, consistent monitoring of blood glucose levels is essential for effective insulin management during adolescence.

3. The first priority would be to focus directly on the issues of hyperglycemia. Determination of Rebecca’s practice of monitoring and management of her diabetes at home is essential. Areas of diabetes management that should be emphasized include careful dietary management, an appropriate exercise program, conscientious self-testing of blood glucose, appropriate administration of daily insulin, and adherence to sliding-scaling insulin therapy. Discussion of the emotional stressors she identifies at this time is appropriate.

4. Yes, Rebecca’s history of DKA over the past year supports her inability to monitor and manage her diabetes.

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