Remission Induction: Almost immediately after confirmation of the diagnosis, induction therapy is begun and lasts for 4 to 6 weeks (Margolin, Rabin, Steuber, et al, 2011). The principal drugs used for induction in ALL are the corticosteroids (prednisone or dexamethasone), vincristine, and l-asparaginase, with or without doxorubicin. Oral steroids are administered daily in divided doses to maintain consistently high blood levels. Vincristine is given by IV infusion once a week for a total of four to six doses, and l-asparaginase or doxorubicin is given at various schedules. A complete remission is determined by the absence of clinical signs or symptoms of the disease and the presence of less than 5% blast cells in the bone marrow. With AML the drug therapies differ from those used for lymphoblastic leukemia. The principal drugs used for induction therapy in AML are doxorubicin or daunomycin and cytosine arabinoside; various other drugs may be added.

Because many of the drugs also cause myelosuppression of normal blood elements, the period immediately after a remission can be critical. The body is defenseless against invading organisms (especially normal bacterial flora) and highly susceptible to spontaneous hemorrhage. Consequently, supportive therapy during this time is essential.

Intensification, or Consolidation, Therapy: Intensification, or consolidation, therapy is used to further decrease the number of leukemic cells in the child’s body. Intensification therapy incorporates some of the following agents: l-asparaginase, high-dose methotrexate or intermediate-dose methotrexate with leucovorin rescue, vincristine, doxorubicin, steroids, cytarabine, intramuscular or oral methotrexate, and 6-mercaptopurine. The intensification phase consists of pulses of these agents given periodically during the first 6 months of treatment. The specific agents used for intensification therapy depend on the type of leukemia and the child’s risk factors.

Maintenance: The goal of maintenance therapy is to preserve remission and further reduce the number of leukemic cells. Combined drug regimens have been more successful in maintaining remissions and preventing drug resistance. A variety of agents are used during maintenance therapy, including a daily dose of oral 6-mercaptopurine, weekly doses of methotrexate, and intermittent pulses of steroids and vincristine, which are standard in most treatment regimens.

During maintenance therapy, weekly or monthly complete blood counts are taken to evaluate the marrow’s response to the drugs. If myelosuppression becomes severe (usually indicated by an ANC <1000/mm³), or if toxic side effects occur, therapy is temporarily stopped or the dose decreased.

Central Nervous System Prophylactic Therapy: Children with leukemia are at risk for invasion of the CNS by the leukemic cells. For this reason, all children receive CNS prophylactic therapy. Before the 1980s children with ALL received cranial-spinal irradiation. Because of the concern regarding late effects of cranial irradiation and secondary malignancies, this mode of therapy is now generally reserved for high-risk patients or those with resistant CNS disease. Depending on protocol, intrathecal methotrexate or triple intrathecal chemotherapy (consisting of methotrexate, cytarabine, and hydrocortisone) is used during induction, intensification, and maintenance therapy to prevent CNS disease.

Duration of therapy has been based on clinical experience comparing survival rates for various time intervals and is concerned with preventing deleterious effects of excessive treatment. Although the optimum time for discontinuing therapy is not known, current practice is to continue treatment for to 3 years. All children after cessation of therapy require regular medical evaluation for surveillance of relapse and long-term sequelae of treatment. Most relapses (16%) occur during the first year off therapy, about 2% to 3% of the relapses occur during each of the next 3 years, and very few relapses occur after 6 years (Margolin, Rabin, Steuber, et al, 2011).

Reinduction After Relapse: For many children, additional therapy becomes necessary when a relapse occurs, as evidenced by the presence of leukemic cells within the bone marrow. Usually reinduction for ALL includes the use of prednisone and vincristine with a combination of other drugs not previously used. Although remissions may be achieved after more than one relapse, each relapse indicates an increasingly poor prognosis. However, more long-term second and subsequent remissions are occurring, and these may have better outlooks than previously thought.

A site that is resistant to chemotherapy and is responsible for leukemic relapse is the testes. A minority of males experience relapses during maintenance therapy or have occult disease after cessation of therapy. Treatment for testicular disease includes bilateral testicular irradiation, intensive systemic chemotherapy, and CNS prophylactic therapy (Landier, 2001).

Bone Marrow Transplantation: BMT has been used successfully in treating some children with ALL and AML. In general, BMT is not recommended for children with ALL during the first remission because of the excellent results possible with chemotherapy. The group with the best results has been those with ALL who received the graft during the second remission (Bollard, Krance, and Heslop, 2011). Because of the poorer prognosis in children with AML, transplantation may be considered during the first remission when a suitable donor is available (Bollard, Krance, and Heslop, 2011).

Prognosis: The majority of children with newly diagnosed leukemia who receive effective multiagent chemotherapy will survive. More than 80% of the children achieved long-term disease-free survival, and the majority of these children developed no obvious health problems from the leukemia or its treatment (Margolin, Rabin, Steuber, et al, 2011). Prognosis after transplantation varies with the timing of the procedure and the type of leukemia; reported ranges for long-term survival are between 25% and 50% (Bollard, Krance, and Heslop, 2011). However, because many of these children faced almost certain death without transplantation, even these low figures represent a major advance. Still, the use of BMT remains controversial.

Prepare the Family for Diagnostic and Therapeutic Procedures: From the time before diagnosis to cessation of therapy, children must undergo several tests, the most traumatic of which are bone marrow aspiration or biopsy and LP. Multiple finger sticks and venipunctures for blood analysis and drug infusion are common occurrences for several years after the diagnosis. Therefore the child needs an explanation of why each procedure is done and what can be expected. (See Preparation for Diagnostic and Therapeutic Procedures, Chapter 27.)

Depending on the child’s age, one way of beginning diagnostic preparation is to explain the tests, procedures, and treatment plan.* Using a drawing or letting the child look at a drop of blood under a microscope not only teaches, but also fosters trust between the nurse and the child. It also allows the nurse to assess the child’s level of understanding. An error many health professionals make is to overestimate children’s knowledge about their bodies. For example, a bone marrow aspiration makes sense only when it is clarified that the center of a bone is hollow and contains the cells that later become “working” blood cells or leukemic cells.

Provide Continued Emotional Support: Nursing care of the child with leukemia is based on typical problems the family confronts during the treatment phases. It is not unusual for a child who discontinues therapy after 2 or 3 years and maintains a permanent remission to experience many side effects. Therefore the nurse’s role is one of continual support, guidance, clarification, and judgment. Parents need to know how to recognize symptoms that demand medical attention. Although some of the reactions discussed are expected, parents should still report them to their practitioner. Warning parents of their possible occurrence beforehand also allows parents to prepare. At the same time, it reassures them that these reactions are not caused by a return of leukemic cells.

The nurse must also use judgment in recognizing which side effects are normal reactions and which indicate toxicity. Frequently it is the office or clinic nurse who screens such telephone calls and gives advice, when appropriate. Usually nausea and vomiting are not indications for drug cessation. However, severe vomiting may require immediate intervention to prevent dehydration. Signs of infection, mucosal ulceration, hemorrhagic cystitis, peripheral neuropathy, and constipation require medical evaluation.

Another aspect of continued emotional support involves prognosis. Leukemia is not invariably fatal, but present statistics must be correctly interpreted. Although more than 95% of children with ALL achieve an initial remission and almost 80% of them live 5 years or longer, remember that these are average estimates and apply to those children treated with the most successful protocols since diagnosis (Shusterman and Meadows, 2000). For the low-risk child the chances may be better, but for the high-risk child they may be significantly poorer. Of those who do survive after discontinuing therapy, a portion will relapse. At present only the passage of time is positive confirmation that the child is “cured” of the disease.

The nurse must be familiar with these statistics to interpret them correctly to parents. At the same time, the nurse must realize that a realistic understanding of the chances for survival requires an adjustment period. For example, it is not unusual for parents to interpret the “95% remission” as the probability for a cure. When a relapse occurs, parents may for the first time be able to “hear” the facts.

Statistics are numbers. Sometimes they bring hope, and at other times they bring despair. Although they are important in terms of research, better treatment, and identification of high- or low-risk populations, they present a general picture of what to expect. The nurse who is working with family members must individualize the numbers to relate to the people. An understanding of each member’s emotional needs, as well as competent care of physical ones, is essential to the positive, growth-promoting support of the family. Chapter 23 discusses comprehensive emotional support for the family through all phases of the illness.

Clinical Staging and Prognosis: Accurate staging of the extent of disease is the basis for treatment protocols and expected prognosis. More than one staging system exists; Box 36-3 shows the Ann Arbor Staging Classification.

Each stage is further subdivided into A or B. A denotes absence of associated general symptoms. B indicates presence of symptoms, such as night sweats, fever (38° C [100.4° F]), or weight loss of 10% or more during the preceding 6 months. In stages II and III, subtype B has a significantly poorer prognosis than subtype A.

The prognosis for patients with Hodgkin disease has improved dramatically, largely as a result of the systematic staging procedure and improved treatment protocols. The prognosis is excellent in children with localized disease. The overall 10-year survival rate is as high as 90%. For relapses, complete remission may occur in 20% to 40% of patients; BMT may represent hope for a cure (Lanzkowsky, 2005). Even in those with disseminated disease, long-term remissions are possible in more than half the patients. Fortunately, now there is a trend toward response-based therapy, and often when radiation is used, it involves field radiotherapy.

Clinical Manifestations: Hodgkin disease is characterized by painless enlargement of lymph nodes. The most common finding is enlarged, firm, nontender, movable nodes in the supraclavicular or cervical area. In children the sentinel node located near the left clavicle may be the first enlarged node. Enlargement of axillary and inguinal lymph nodes is less frequent (see Fig. 36-4).

Other signs and symptoms depend on the extent and location of involvement. Mediastinal lymphadenopathy may cause a persistent, nonproductive cough. Enlarged retroperitoneal nodes may produce unexplained abdominal pain. Systemic symptoms include low-grade or intermittent fever (Pel-Ebstein disease), anorexia, nausea, weight loss, night sweats, and pruritus. Generally, such symptoms indicate advanced lymph node and extralymphatic involvement.

Diagnostic Evaluation: The history and physical examination often yield important clues to the disease, such as fevers; night sweats; weight loss; and enlarged lymph nodes, spleen, or liver. Because of the multiple organs that can become involved, diagnosis consists of several tests to confirm the presence of Hodgkin disease and to assess the extent of involvement for accurate staging. Tests include complete blood count, uric acid levels, liver function tests, erythrocyte sedimentation rate or C-reactive protein, alkaline phosphatase, and urinalysis. Radiographic tests include CT scans of the neck, chest, abdomen, and pelvis; a gallium or PET scan (to identify metastatic or recurrent disease); a chest x-ray film; and, if clinically indicated, a bone scan to detect metastasis.

A lymph node biopsy is essential to establish histologic diagnosis and staging. The presence of Sternberg-Reed cell is considered diagnostic of Hodgkin disease because it is absent in the other lymphomas; however, it may occur in infectious mononucleosis. A bone marrow aspiration or biopsy is also usually performed. With the advent of the CT and gallium scans to identify metastatic disease and multiagent chemotherapy and radiotherapy to eradicate metastatic disease, a laparotomy is avoided except in selected cases.

Therapeutic Management: The primary modalities of therapy are chemotherapy and irradiation. Each may be used alone or in combination based on the clinical staging. The goal of treatment is obviously a cure; however, aggressive therapy increases the chances of complications in the disease-free state and can seriously compromise the quality of life. Consequently, numerous research studies are currently investigating treatment options to minimize long-term complications. Because of the diversity of approaches to treatment, the following is an overview of general principles that may not apply to all children. One of the major concerns with combined radiation and antineoplastic drug therapy is the serious late effects in children with an excellent prognosis.

Radiation may entail involved field radiation, extended field radiation (involved areas plus adjacent nodes), or total nodal irradiation (the entire axial lymph node system), depending on the extent of involvement. In stage IV disease, chemotherapy is the primary form of treatment, although limited irradiation may be given to areas of bulky disease. The most effective combination of chemotherapy widely used in the past has been MOPP (mechlorethamine [Mustargen], vincristine [Oncovin], prednisone, and procarbazine), alternating with ABVD (doxorubicin [Adriamycin], bleomycin, vinblastine, and dacarbazine). However, this therapy combination of MOPP and ABVD caused severe late effects, including secondary malignancies. For this reason care providers are now using other drug combinations such as BEACOPP (bleomycin, etoposide, doxorubicin [Adriamycin], cyclophosphamide, vincristine [Oncovin], procarbazine, and prednisone) or ABVD (Hudson, Krasin, Metzger, et al, 2011).

Follow-up care of children no longer receiving therapy is essential to identify relapse and second malignancies. In children with splenectomy because of laparotomy, prophylactic antibiotics are administered for an indefinite period. Also, immunizations against pneumococci and meningococci are recommended before the splenectomy. (See Chapter 12.)

Nursing Care Management: Nursing care involves (1) preparation for diagnostic and operative procedures, (2) explanation of treatment side effects, and (3) child and family support (see Nursing Care Plan, pp. 1470-1472). Once the child is hospitalized for suspected Hodgkin disease, a battery of diagnostic tests is ordered. The family needs an explanation of why each test is performed, since many of them, such as bone marrow aspiration and lymph node biopsy, are invasive procedures. (See Chapter 27.)

Explanations of chemotherapeutic reactions vary with the specific drug regimen. The most common side effects, such as nausea and vomiting, body image changes, neuropathy, and mucosal ulceration, are discussed under Nursing Care of the Child with Cancer. Radiation results in few side effects, sometimes consisting only of a mild skin reaction. With external field radiation to the chest and abdomen, nausea and vomiting, weight loss, and mucosal ulceration (esophagitis, gastric ulcers) are common. The usual measures for providing relief are discussed on p. 1473 and outlined in Table 36-2.

The most common side effect of extensive irradiation is malaise, which may result from damage to the thyroid gland, causing hypothyroidism. Lack of energy is particularly difficult for adolescents because it prevents them from keeping up with their peers. Sometimes adolescents push themselves to the point of physical exhaustion rather than admit fatigue and succumb to the decreased activity tolerance. Parents should observe for such behavior, such as extreme fatigue at the end of the day, falling asleep at the dinner table, inability to concentrate on homework, or an increased susceptibility to infection. Regular bedtimes and periodic rest times are important for these children, especially during chemotherapy, when myelosuppression increases the risk of infection and debilitation. Before discharge, the nurse should discuss a feasible school schedule with the parents and child. If alterations are necessary, such as elimination of strenuous physical education, they are discussed with the teacher, school nurse, and principal. Follow-up care is essential to diagnose hypothyroidism early and institute thyroid replacement.

An area of concern for adolescents is the high risk of sterility from irradiation and chemotherapy. Both irradiation to the gonads and drugs, particularly procarbazine and alkylating agents, may lead to infertility. Younger patients with a greater complement of oocytes are more likely to retain ovarian function.

Although sexual function is not altered, the appearance of secondary sexual characteristics and menstruation may be delayed in the pubescent child. Adolescents should be informed of these side effects early in the course of the diagnosis and treatment. Delayed sexual maturation may be an extremely sensitive and painful area for children. (See Chapter 20.)

• The disease is usually diffuse rather than nodular.

• The cell type is either undifferentiated or poorly differentiated.

• Dissemination occurs earlier, more often, and more rapidly.

• Mediastinal involvement and invasion of meninges typically occur.

Staging and Prognosis: NHL is heterogeneous, exhibiting a variety of morphologic, cytochemical, and immunologic features, not unlike the diversity seen in leukemia. Classification is based on the pattern of histologic presentation: (1) lymphoblastic, (2) Burkitt or non-Burkitt, or (3) large cell. Immunologically these cells are also classified as T cells; B cells (an example of which is Burkitt lymphoma); or non-T, non-B cells, which lack specific immunologic properties.

The clinical staging system used in Hodgkin disease is of little value in NHL, although that system has been modified for NHL and other systems have been developed. A favorable prognosis is defined by (1) lymph node involvement only and limitation to one or two adjacent lymphatic regions (excluding the mediastinum); (2) an extranodal site in the nasopharynx, oropharynx, or other isolated extranodal site, with or without regional lymphadenopathy; or (3) gastrointestinal involvement, with or without regional lymphadenopathy, limited to the mesentery (Gross and Perkins, 2011). Box 36-4 presents the most commonly used staging system.

The use of aggressive combination chemotherapy has had a major impact on the survival rates of children with NHL. The most effective treatment regimens result in cure in almost all children with limited disease involvement; 75% to 90% of children with extensive disease are cured (Gross and Perkins, 2011; Kavan, Kabickova, Gajdos, et al, 1999).

Clinical Manifestations: Clinical manifestations depend on the anatomic site and extent of involvement. Many of the manifestations seen in Hodgkin disease may be present in NHL, although rarely does a single symptom give rise to the diagnosis. Rather, metastasis to the bone marrow or CNS may produce signs and symptoms typical of leukemia. Lymphoid tumors compressing various organs may cause intestinal or airway obstruction, cranial nerve palsies, or spinal paralysis.

The exception to the usual presentation of NHL is Burkitt lymphoma, a type of cancer that is rare in the United States but endemic in parts of Africa. It is a rapidly growing neoplasm that is most commonly seen as a mass in the jaw, abdomen, or orbit. However, no anatomic site appears exempt from involvement. Peripheral lymphadenopathy, hepatosplenomegaly, or signs of conversion to leukemia are rarely seen.

Diagnostic Evaluation: Because most children with NHL have widespread disease at diagnosis, thorough pathologic staging is unnecessary. Current recommendations for staging include a surgical biopsy for histopathologic confirmation of disease with immunophenotyping and cytogenetic evaluation; bone marrow aspiration; radiologic studies, especially CT scans of the lungs and gastrointestinal organs; and LP.

Therapeutic Management: The present treatment protocols for NHL include an aggressive approach using irradiation and chemotherapy. Similar to leukemic therapy, the protocols include induction, consolidation, and maintenance phases, some with intrathecal chemotherapy. At present the differentiation between lymphoblastic lymphoma and all other lymphomas is widely used as a way to categorize patients for specific treatment regimens (Gross and Perkins, 2011). Children with lymphoblastic lymphoma are treated with several drug protocols, most containing several chemotherapeutic agents. One of the most commonly used regimens includes cyclophosphamide or ifosfamide, vincristine, intrathecal chemotherapy, prednisone, daunomycin, 6-thioguanine, cytosine arabinoside, carmustine (BCNU), and l-asparaginase.

Children with nonlymphoblastic lymphoma are treated with cyclic drug combinations, including cyclophosphamide and intermediate- or high-dose methotrexate (Gross and Perkins, 2011). Most protocols also include an anthracycline. These children receive CNS prophylaxis with combination intrathecal chemotherapy. These multiagent regimens are administered for 6 to 24 months.

Nursing Care Management: Nursing care of the child with NHL is similar to the care discussed under Nursing Care of the Child with Cancer. Because of the intensive chemotherapy protocol, nursing care is primarily directed toward managing the side effects of these agents.

Clinical Manifestations

The signs and symptoms of brain tumors are directly related to their anatomic location and size and to some extent the child’s age. For instance, in infants whose sutures are still open, a bulging fontanel indicates hydrocephalus. Head circumference measurements allow for detection of increased head size. Even in older children, clinical manifestations may be nonspecific. However, the most common symptoms of infratentorial brain tumors are headache, especially on awakening, and vomiting that is not related to feeding. Tumors in this area of the brain often obstruct the flow of cerebrospinal fluid, causing increased ICP and the symptoms mentioned above. In addition, patients may have symptoms related to the specific structure involved. Tumors of the cerebellum often cause nystagmus, ataxia, dysarthria, and dysmetria (Blaney, Haas-Kogan, Young-Pouissant, et al, 2011; Strickler and Phillips, 2000). Supratentorial symptoms more commonly include seizures, personality or behavioral changes, and contralateral weakness. Tumors involving the structures of the midbrain, including the hypothalamus and pituitary gland, may cause endocrinopathies such as diabetes insipidus, delayed or precocious puberty, and growth failure. Table 36-4 presents the common presenting symptoms of brain tumors.

Diagnostic Evaluation

Diagnosis of a brain tumor is based on presenting clinical signs and diagnostic imaging. Because the signs and symptoms may be vague and easily overlooked, early diagnosis necessitates a high index of suspicion during history taking. A number of tests may be employed in the neurologic evaluation (see Table 37-2), but the gold standard diagnostic procedure is MRI, which permits early diagnosis of brain tumors and assessment of tumor growth during or after treatment. Magnetic resonance (MR) angiography can be performed during the same session as MRI to determine the vascularity of the tumor (Brunelle, 2000). Another test is CT, which permits direct visualization of the brain parenchyma, ventricles, and surrounding subarachnoid space. Through the IV injection of radiographic contrast agents, intracranial blood vasculature can be demonstrated (Blaney, Haas-Kogan, Young-Pouissant, et al, 2011). MR spectroscopy is a new radiographic technique that is able to differentiate between malignant tumors and areas of necrosis (Brunelle, 2000; Lanzkowsky, 2005).

When a positive CT scan is obtained, angiography may be done to provide information about the tumor’s blood supply and degree of vascularity, which may assist the surgeon in planning the operative approach. Other tests may include an MRI of the spine, functional MRI, and electroencephalography. LP is dangerous in the presence of increased ICP because of possible brainstem herniation after sudden release of pressure.

Definitive diagnosis is based on tissue specimens obtained during surgery. Occasionally, special techniques are required for determining the cell type. This period of waiting is one of anxiety for family members, who are aware of the link between cell type and prognosis. Because of the location of some brain tumors, such as brainstem tumors, a biopsy is not possible and the diagnosis is made by imaging findings alone.

Therapeutic Management

Treatment may involve the use of surgery, radiotherapy, and chemotherapy. All three may or may not be used, depending on the type of tumor. The treatment of choice is total removal of the tumor without residual neurologic damage. Patients with the most complete tumor removal have the greatest chance of survival. Several surgical advances have allowed the biopsy and removal of tumors in areas previously considered too dangerous for traditional operative techniques. Stereotactic surgery involves the use of CT and MRI in conjunction with other special computer techniques to reconstruct the tumor in three dimensions. With computer-assisted instruments, removal is sometimes possible. Stereotactic biopsy is performed with CT or MRI computer guidance for inserting the biopsy needle. This procedure has the benefit of a short hospital stay and a lower morbidity and mortality rate in comparison with an open craniotomy (Blaney, Haas-Kogan, Young-Pouissant, et al, 2011). Other procedures include the use of lasers to vaporize tumor tissue and brain mapping to determine the precise location of critical brain areas to avoid during surgery.

Radiotherapy is used to treat most tumors and to shrink the size of the tumor before attempting surgical removal. The use of chemotherapy has emerged in the past decades with an increasingly important role, either in combination with irradiation or alone. The drugs most commonly used are vincristine, cisplatin, carboplatin, cyclophosphamide, and etoposide (Strickler and Phillips, 2000). The problems of treatment are compounded by the serious late effects of all three modes of therapy. Surgery can cause injury to important areas of the brain, especially when the surgeon is attempting to remove invasive tumors. Irradiation has serious long-term consequences, which may include tissue necrosis, secondary malignancies, endocrine dysfunction, and behavioral or intellectual deficits. For these reasons, the use of irradiation is deferred for as long as possible in young children. Although there is limited information regarding a “safe age” for giving radiation, most centers consider it to be over age 3 years, but focal radiation is often given at a younger age (Mainprize, Taylor, and Rutka, 2000).

Nursing Care Management

Nursing care of the child with a brain tumor is similar regardless of the type of intracranial lesion. Because a brain tumor is potentially fatal, the reader is urged to incorporate the psychologic interventions discussed in Chapter 23 with those elaborated on in this section. However, remember that many brain tumors are curable. Medulloblastoma, for instance, has a survival rate of more than 80% in those patients without metastatic disease. Despite the grave nature of some brain tumors, it is important to realize the hope that new standard therapies and emerging therapies have brought to the families of many pediatric brain tumor patients.

Clinical Manifestations

The signs and symptoms of neuroblastoma depend on the location and stage of the disease. With abdominal tumors, the most common presenting sign is a firm, nontender, irregular mass in the abdomen that crosses the midline (in contrast to Wilms tumor, which is usually confined to one side). Compression of the kidney, ureter, or bladder may cause urinary frequency or retention.

Distant metastasis frequently causes supraorbital ecchymosis, periorbital edema, and proptosis (exophthalmos) from invasion of retrobulbar soft tissue. Lymphadenopathy, hepatomegaly and skeletal pain are also present in patients with disseminated disease. Vague symptoms of widespread metastasis include pallor, weakness, irritability, anorexia, and weight loss.

Other primary tumor sites may cause significant clinical effects such as neurologic impairment, respiratory obstruction from a thoracic mass, or varying degrees of paralysis from compression of the spinal cord. Infrequently a child may have symptoms of increased catecholamine excretion, such as flushing, hypertension, tachycardia, and diaphoresis (Weinstein, Katenstein, and Cohn, 2003).

Diagnostic Evaluation

Diagnostic evaluation is aimed at locating the primary site and areas of metastasis. A CT of the abdomen, pelvis, or chest is the preferred imaging modality to locate the primary tumor. A bone scan and MIBG (iodine-131 metaiodobenzylguanidine) scan should be performed to evaluate for the presence of skeletal metastases. Examination of the bone marrow with bilateral aspirates and biopsies should be performed in all patients. Neuroblastomas, particularly those arising on the adrenal glands or from a sympathetic chain, excrete the catecholamines epinephrine and norepinephrine. Urinary excretion of catecholamines is detected in approximately 95% of children with adrenal or sympathetic tumors. Analyzing the breakdown products that are normally excreted in the urine, namely, vanillylmandelic acid, homovanillic acid, dopamine, and norepinephrine, permits detection of a suspected tumor both before and after medical-surgical intervention. Amplification of the N-myc gene and abnormalities in chromosomes have been associated with a poorer prognosis (Grosfeld, 2000; Lau, Tai, Weitzman, et al, 2004). Increased ferritin and neuron-specific enolase are also seen in neuroblastoma.

Staging and Prognosis

Neuroblastoma is a “silent” tumor. In more than 70% of cases, diagnosis is made after metastasis occurs, with the first signs caused by involvement in the nonprimary site, usually the lymph nodes, bone marrow, skeletal system, skin, or liver. Because of the frequency of invasiveness, the prognosis for neuroblastoma is generally poor.

The child’s age and the stage of the disease (Box 36-5) at diagnosis are important prognostic factors. Survival is inversely correlated with age. If all stages are grouped together, the survival rates are 75% for children under 1 year of age and less than 50% for children over 1 year of age. This marked difference in survival rates by age is partly accounted for by the larger proportion of very young children with stage I, II, or IV-S disease and the absence of the N-myc gene amplification (Brodeur, Hogarty, Mosse, et al, 2011).

Infants who remain free of disease for 1 year after treatment are usually cured, but older children have experienced relapses several years after cessation of treatment. Surgical resection of the tumor in stage I infants diagnosed by ultrasonography done for other reasons appears to be almost 90% curative (Grosfeld, 2000). Neuroblastoma is one of the few tumors that demonstrate spontaneous regression (especially stage IV-S), possibly as a result of maturity of the embryonic cell or development of an active immune system.

Therapeutic Management

Accurate clinical staging is important for establishing initial treatment. Therefore the purpose of surgery is both to remove as much of the tumor as possible and to obtain biopsies. In stages I and II, complete surgical removal of the tumor is the treatment of choice. If the tumors are large, partial resection is attempted, with a course of irradiation postoperatively to shrink the tumor in the hope of complete removal at a later date. Surgery is usually limited to biopsy in stages III and IV because of the extensive metastasis, although additional surgery to assess tumor regression or remove a regressed tumor is not unlikely.

The precise role of radiotherapy is unclear. It does not appear to be of any benefit in children with stage I and II disease. It is commonly used with stage III disease, although it may not improve survival expectancy. It may make a large tumor operable. Radiotherapy provides emergency management of a massive neuroblastoma causing spinal cord compression (Nguyen, Sallah, Ludin, et al, 2000). It also offers palliation for metastatic lesions in bones, lungs, liver, or brain.

Chemotherapy is the mainstay of therapy for extensive local or disseminated disease. The drugs of choice are vincristine, doxorubicin, cyclophosphamide, cisplatin, etoposide, ifosfamide, and carboplatin; they are administered in a variety of combinations according to specific protocols. In addition, the use of consolidative myeloablative therapy using autologous marrow or peripheral stem cells followed by 13-cis-retinoic acid has improved the outcome of patients with high-risk disease.

Nursing Care Management

Nursing care management is similar to that discussed under Nursing Care of the Child with Cancer, including psychologic and physical preparation for diagnostic and operative procedures; prevention of postoperative complications for abdominal, thoracic, or cranial surgery; and explanation of chemotherapy and radiotherapy and their side effects (see Tables 36-2 and 36-3).

Because this tumor carries a poor prognosis for many children, evaluate and address the needs of the family in terms of coping with a life-threatening illness. (See Chapter 23.) Because of the high degree of metastasis at the time of diagnosis, many parents suffer guilt for not having recognized signs earlier. Often the guilt is expressed as anger toward professionals for not diagnosing it sooner. Parents need much support in dealing with these feelings and expressing them to the appropriate people.

Clinical Manifestations

Most malignant bone tumors produce localized pain in the affected site, which may be severe or dull and may be attributed to trauma or the vague complaint of “growing pains.” The pain is often relieved by a flexed position, which relaxes the muscles overlying the stretched periosteum. Frequently it draws attention when the child limps, curtails physical activity, or is unable to hold heavy objects. A palpable mass is also a common manifestation of bone tumors, but systemic symptoms such as fever and other clinical symptoms such as spinal cord compression and respiratory distress are more frequent in patients with Ewing sarcoma.

Diagnostic Evaluation

Diagnosis begins with a thorough history and physical examination. A primary objective is to rule out causes such as trauma or infection. Careful questioning regarding pain is essential in attempting to determine the duration and rate of tumor growth. Physical assessment focuses on functional status of the affected area; signs of inflammation; size of the mass; and any systemic indication of generalized malignancy, such as anemia, weight loss, and frequent infection.

Definitive diagnosis is based on radiologic studies, such as plain films and CT or MRI of the primary site, CT of the chest, and radioisotope bone scans to evaluate metastasis and bone marrow examination in patients with Ewing sarcoma. A needle or surgical biopsy is necessary to establish the diagnosis. Ewing sarcoma most commonly involves the pelvis, long bones of the lower extremities, and chest wall and radiographically involves the diaphysis with detachment of the periosteum from the bone (Codman triangle). In osteosarcoma, lesions are most commonly located in the metaphyseal region of the bone, often involving the long bones. Radial ossification in the soft tissue gives the tumor a “sunburst” appearance on plain radiograph.

Prognosis

A better understanding of the biology of neoplastic growth has resulted in more aggressive treatment and an improved prognosis. The natural history of osteogenic sarcoma and Ewing sarcoma suggests that multiple submicroscopic foci of metastatic disease are present at the time of diagnosis despite clinical evidence of localized involvement. Before the use of aggressive multimodal therapy, pulmonary metastasis appeared in the majority of patients who were treated with surgical excision alone (Gorlick, Bielack, Teot, et al, 2011). With current therapies that include surgery and chemotherapy for osteosarcoma and surgery, radiotherapy, and chemotherapy for Ewing sarcoma, more than two thirds of patients with localized disease can be cured.

Therapeutic Management

Optimum treatment of osteosarcoma includes surgery and chemotherapy. The surgical approach consists of surgical biopsy followed by either limb salvage or amputation. To ensure local control, all gross and microscopic tumors must be resected. A limb salvage procedure involves en bloc resection of the primary tumor with prosthetic replacement of the involved bone. For example, with osteosarcoma of the distal femur, a total femur and joint replacement is performed. Frequently children undergoing a limb salvage procedure receive preoperative chemotherapy in an attempt to decrease the tumor size and make surgery more manageable (Lanzkowsky, 2005; Gorlick, Bielack, Teot, et al, 2011).

Critical Thinking Exercise—Osteogenic Sarcoma

Chemotherapy plays a vital role in treatment of osteosarcoma. Antineoplastic drugs, such as high-dose methotrexate with citrovorum factor rescue, doxorubicin, cisplatin, ifosfamide, and etoposide, may be administered singly or in combination and may be employed both before or after surgical resection of the tumor. The use of postoperative chemotherapy after amputation has comparable results to trials using preoperative chemotherapy followed by limb salvage surgery. Preoperative chemotherapy allows for examination of the surgical specimen at the time of definitive surgery, which predicts clinical outcome. When pulmonary metastases are found, thoracotomy and chemotherapy have resulted in prolonged survival and potential cure. These combined-modality approaches have significantly improved the prognosis in osteosarcoma to approximately 78% for nonmetastatic patients (Lanzkowsky, 2005). Newer trials have recently been completed and have incorporated muramyl tripeptide phosphatidylethanolamine to eradicate micrometastases by stimulating macrophages to kill tumor cells not eliminated by chemotherapy (Lanzkowsky, 2005).

Nursing Care Management

Nursing care depends on the type of surgical approach. Obviously the family may have more difficulty adjusting to an amputation than a limb salvage procedure. In either instance, preparation of the child and family is critical. Straightforward honesty is essential in gaining the child’s cooperation and trust. The diagnosis of cancer should not be disguised with falsehoods such as “infection.” To accept the need for radical surgery, the child must be aware of the lack of alternatives for treatment. Although the responsibility of telling the child is generally left to the physician, the nurse should be present at the discussion or be aware of exactly what is said. The child should be told a few days before surgery to allow him or her time to think about the diagnosis and consequent treatment and to ask questions. (See Nursing Care Plan: The Child with a Bone Tumor.*)

Sometimes children have many questions about the prosthesis, limitations on physical ability, and prognosis in terms of cure. At other times they react with silence or with a calm manner that belies their concern and fear. Either response must be accepted, since it is part of the grieving process of a loss. For those who desire information, it may be helpful to introduce them to another amputee before surgery or to show them pictures of the prosthesis.† However, the nurse must be careful not to overwhelm children with information. A sound approach is to answer questions without offering additional information. For those who do not pursue additional information, the nurse expresses a willingness to talk.

The child is also informed of the need for chemotherapy and its side effects before surgery. Exercise caution about offering too much information at one time. When discussing hair loss, emphasize positive aspects, such as wearing a wig. Because bone tumors affect adolescents and young adults, it is not unusual for them to become angry over all the radical body alterations.

If an amputation is performed, the child is usually fitted with a temporary prosthesis immediately after surgery, which permits early functioning and fosters psychologic adjustment. If this is not done, the child requires stump care, which is the same as for any amputee. A permanent prosthesis is usually fitted within 6 to 8 weeks. During hospitalization the child begins physical therapy to become proficient in the use and care of the device.

Phantom limb pain may develop after amputation. This symptom is characterized by sensations such as tingling, itching, and, more frequently, pain felt in the amputated limb. The child and family need to know that the sensations are real, not imagined. Amitriptyline (Elavil) has been used successfully in children to decrease the pain (Olsson, 1999). In addition, an epidural is often used preoperatively as a nerve block in an effort to decrease or eliminate the occurrence of phantom limb pain. Much research is needed to further delineate the best care for these patients (Ong, Arneja, and Ong, 2006).

Discharge planning must begin early in the postoperative period. Once the child has begun physical therapy, the nurse should consult with the therapist and practitioner to evaluate the child’s physical and emotional readiness to reenter school. It is an opportune time to involve a community nurse in the child’s home care. Every effort is made to promote normalcy and gradual resumption of realistic preamputation activities.* Role-playing in anticipation of such experiences is beneficial in preparing the child for the inevitable confrontation by others. Environmental barriers, such as stairs, are assessed in terms of the accessibility in the school and home, especially because the child may need to use crutches or a wheelchair before complete healing and prosthetic competency are achieved.

The nurse encourages the child to select clothing that best camouflages the prosthesis, such as pants or long-sleeved shirts. Well-fitted prostheses are so natural looking that girls can usually wear sheer stockings without revealing the device. Emphasizing feminine or masculine apparel helps the child regain a feeling of self-identity. Even during the postoperative period, encouraging the child to wear blue jeans and a T-shirt may distract attention from the deformity and focus it on familiar aspects of appearance.

The family and child need much support in adjusting not only to a life-threatening diagnosis but also to alteration in body form and function. Because loss of a limb entails a grieving process, those caring for the child need to recognize that the reactions of anger and depression are normal and necessary. Often parents view the anger as a direct affront to them for allowing the amputation to occur, or they see the depression as rejection. These are not personal attacks but the child’s attempts to cope with a loss.

Therapeutic Management

Limb salvage procedures might be feasible in extremity lesions, and amputation may be considered if the results of radiotherapy render the extremity useless or deformed (e.g., from retarded growth in young children). The treatment of choice for the majority of lesions is involved field radiotherapy and chemotherapy. A widely used drug regimen includes vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide, and etoposide. The addition of ifosfamide and etoposide has increased the 3-year survival to 78% for patients with localized disease (Lanzkowsky, 2005).

Nursing Care Management

The psychologic adjustment to Ewing sarcoma is typically less traumatic than it is to osteosarcoma because of the preservation of the affected limb. Many families accept the diagnosis with a sense of relief in knowing that this type of bone cancer does not necessitate amputation, and initially they may not be aware of the damaging effects on the irradiated site. Consequently they need preparation for the various diagnostic tests, including bone marrow aspiration and surgical biopsy, and adequate explanation of the treatment regimen. High-dose radiotherapy often causes a skin reaction of dry or moist desquamation followed by hyperpigmentation. The child should wear loose-fitting clothes over the irradiated area to minimize additional skin irritation. Because of increased sensitivity, protect the area from sunlight and sudden changes in temperature, such as from heating pads or ice packs. Encourage the child to use the extremity as tolerated. Occasionally the physical therapist may plan an active exercise program to preserve maximum function.

The child needs the same considerations for adjusting to the effects of chemotherapy as any other patient with cancer. The drug regimen usually results in hair loss, severe nausea and vomiting, peripheral neuropathy, and possibly cardiotoxicity. Make every effort to outline a treatment plan that allows the child maximum resumption of a normal lifestyle and activities. (See Nursing Care Plan: The Child with a Bone Tumor.†)

Clinical Manifestations

The most common presenting sign is painless swelling or mass within the abdomen. The mass is characteristically firm, nontender, confined to one side, and deep within the flank. If it is on the right side, it may be difficult to distinguish from the liver, although, unlike that organ, it does not move with respiration. Parents usually discover the mass during routine bathing or dressing of the child.

Other clinical manifestations are the result of compression from the tumor mass, metabolic alterations secondary to the tumor, or metastasis. Hematuria occurs in less than one fourth of children with Wilms tumor. Anemia, usually secondary to hemorrhage within the tumor, results in pallor, anorexia, and lethargy. Hypertension, probably caused by secretion of excess amounts of renin by the tumor, occurs occasionally. Other effects of malignancy include weight loss and fever. If metastasis has occurred, symptoms of lung involvement, such as dyspnea, cough, shortness of breath, and pain in the chest, may be evident.

Diagnostic Evaluation

In a child suspected of having Wilms tumor, special emphasis is placed on the history and physical examination for the presence of congenital anomalies; a family history of cancer; and signs of malignancy, such as weight loss, enlarged liver and spleen, indications of anemia, and lymphadenopathy. Specific tests include radiographic studies, such as abdominal ultrasound, CT, and MRI of the abdomen; CT of the chest to look for metastases in the lung; and Doppler ultrasound of the inferior vena cava. Laboratory studies should include a complete blood count (polycythemia is sometimes present if the tumor secretes excess erythropoietin), biochemical studies, and urinalysis. Studies to demonstrate the relationship of the tumor to the ipsilateral kidney and the presence of a normally functioning kidney on the contralateral side are essential.

Staging and Prognosis

Wilms tumor probably arises from a malignant, undifferentiated metanephrogenic blastoma (a cluster of primordial cells capable of initiating the regeneration of an abnormal structure). Its occurrence slightly favors the left kidney, which is advantageous because surgically this kidney is easier to manipulate and remove. Although the tumor may become large, it remains encapsulated for an extended period. During surgery the tumor is staged to maximize the effectiveness of treatment protocols (Box 36-6).

The histology of the tumor cells is also identified and classified according to two groups: favorable histology (FH) and unfavorable histology (UH). Only about 12% of Wilms tumors demonstrate UH, which is associated with a poorer prognosis and demands a more aggressive treatment protocol, regardless of the clinical stage.

Survival rates for Wilms tumor are one of the highest among all childhood cancers. Children with localized tumor (stages I and II) have a 90% chance of cure with multimodal therapy. For those children who relapse, a better expectancy of disease-free survival is associated with FH of the tumor, more than 12 months elapsing from the first complete remission, and nonabdominal recurrence (Dome, Liu, Krasin, et al, 2002; Plesko, Kramarova, Stiller, et al, 2001).

Therapeutic Management

Combined treatment with surgery and chemotherapy, with or without irradiation, is based on the clinical stage and histologic pattern. In unilateral disease a large transabdominal incision is performed for optimum visualization of the abdominal cavity. The tumor, affected kidney, and adjacent adrenal gland are removed. Great care is taken to keep the encapsulated tumor intact because rupture can seed cancer cells throughout the abdomen, lymph channel, and bloodstream. The contralateral kidney is carefully inspected for evidence of disease or dysfunction. Regional lymph nodes are inspected, and a biopsy is performed when indicated. Any involved structures, such as part of the colon, diaphragm, or vena cava, are removed. Metal clips are placed around the tumor site for exact marking during radiotherapy.

If both kidneys are involved, the child may be treated with radiotherapy or chemotherapy preoperatively to shrink the tumor, allowing more conservative therapy (Graf, Tournade, and deKraker, 2000; Lanzkowsky, 2005). In some cases a partial nephrectomy is performed on the less affected kidney, with a total nephrectomy performed on the opposite side. When a transplant is feasible, such as from a twin, sibling, or parent, bilateral nephrectomy is considered as a last resort.

Postoperative radiotherapy is indicated for children with large tumors, metastasis, residual disease at the primary tumor site, UH, or recurrence. Chemotherapy is indicated for all stages. The most effective agents for treating Wilms tumor are actinomycin D and vincristine; doxorubicin and cyclophosphamide may be used for UH or advanced-stage disease (Fernandez, Geller, Ehrlich, et al, 2011; Lanzkowsky, 2005). The duration of therapy ranges from 6 to 15 months.

Nursing Care Management

The nursing care of the child with Wilms tumor is similar to that of other cancers treated with surgery, irradiation, and chemotherapy. However, some significant differences are discussed for each phase of nursing intervention.

Clinical Manifestations

The initial signs and symptoms are related to the site of the tumor and compression of adjacent organs (Table 36-5). Some tumor locations, such as the orbit, manifest early in the course of the illness. Other tumors, such as those of the retroperitoneal area, only produce symptoms when they are relatively big and compress adjacent organs. Unfortunately, many of the signs and symptoms attributable to rhabdomyosarcoma are vague and frequently suggest a common childhood illness, such as “earache” or “runny nose.” Rarely, the site of the primary tumor site is never identified.

Diagnostic Evaluation

Diagnosis begins with a careful history and physical examination. Radiographic studies to delineate the primary tumor site should include CT or MRI. Metastatic evaluation should include a CT of the chest, bone scan, and bilateral bone marrow aspirates and biopsies. For patients with tumors in the parameningeal area, perform an LP to examine the spinal fluid. An excisional biopsy or surgical resection of the tumor, when possible, is done to confirm the diagnosis.

Staging and Prognosis

Careful staging is extremely important for planning treatment and determining the prognosis. The Intergroup Rhabdomyosarcoma Study has developed a surgicopathologic staging system, shown in Box 36-8 (Lanzkowsky, 2005; Helman, 2011).

With the use of contemporary multimodal therapy, more than 80% of patients with nonmetastatic disease are expected to survive (Lanzkowsky, 2005; Helman, 2011). If relapse occurs, the prognosis for long-term survival is poor.

Therapeutic Management

All rhabdomyosarcomas are high-grade tumors with the potential for metastases. Therefore multimodal therapy is recommended for all patients. Complete removal of the primary tumor is advocated whenever possible. However, because the tumor is chemosensitive, radical procedures with high morbidity should be avoided. In the majority of cases, a biopsy is followed by chemotherapy, irradiation, or both. Patients with embryonal tumors and group I disease can be treated with chemotherapy alone, whereas all others require chemotherapy and radiotherapy. Drugs that are used most often for the treatment of rhabdomyosarcoma include vincristine, actinomycin D, cyclophosphamide (VAC), ifosfamide, topotecan, irinotecan, and doxorubicin, which are administered for about 1 year (Lanzkowsky, 2005).

Nursing Care Management

The nursing responsibilities are similar to those for other types of cancer, especially the solid tumors when surgery is employed. Specific objectives include (1) careful assessment for signs of the tumor, especially during well-child examinations; (2) preparation of the child and family for the multiple diagnostic tests (see p. 1478); and (3) supportive care during each stage of multimodal therapy. The reader is urged to review the Nursing Care Management section for cancer and Chapter 23 for emotional support of the family in the event of a poor prognosis.

Clinical Manifestations

Retinoblastoma has few grossly obvious signs. Typically the parents are the ones who first observe a whitish “glow” in the pupil, known as the cat’s eye reflex, or leukocoria (Fig. 36-6). The reflex represents visualization of the tumor as the light momentarily falls on the mass. When a tumor arises in the macular region (area directly at the back of the retina when the eye is focused straight ahead), a white reflex may be visible when the tumor is small. It is best observed when a bright light is shining toward the child as the child looks forward. Sometimes parents accidentally discover it when taking a photograph of their child using a flash attachment.

When the tumor arises in the periphery of the retina, it must grow to a considerable size before light can strike it sufficiently to produce the cat’s eye reflex. In this situation it is visible only when the child looks in certain directions (sideways) or if the observer stands at an oblique angle to the child’s face as the child looks straight ahead. The fleeting nature of the reflex often results in a delayed diagnosis because health care professionals fail to appreciate the ominous significance of the parents’ findings.

The next most common sign is strabismus resulting from poor fixation of the visually impaired eye, particularly if the tumor develops in the macula, the area of sharpest visual acuity. Blindness is usually a late sign, but it frequently is not obvious unless the parent consciously observes for behaviors indicating loss of sight, such as bumping into objects, slowed motor development, or turning of the head to see objects lateral to the affected eye. Other signs and symptoms include heterochromia (different color of the iris), glaucoma, and pain.

Diagnostic Evaluation

A detailed family history and recording of eye symptoms is essential. Children suspected of having this disorder are referred to an ophthalmologist; the diagnosis is usually based on indirect ophthalmoscopy, ultrasound, and CT scans.

Metastatic disease at the time of retinoblastoma diagnosis is rare (Singh, Shields, and Shields, 2000); therefore staging procedures such as bone marrow aspiration, bone scan, and LP are not routinely performed.

Staging and Prognosis

Staging of retinoblastomas is done under indirect ophthalmoscopy before surgery to accurately determine the tumor size (measured in disc diameters [DD]) and location (according to an imaginary line called the equator drawn on the midplane of the eye) (Hurwitz, Shields, Shields, et al, 2011; Lanzkowsky, 2005).

Various classification systems have been used to stage retinoblastoma. The Reese-Ellsworth system (Box 36-9) classifies patients according to five groups and predicts survival when patients are treated with radiotherapy. A new classification system is designed based on the extent and location of the intraocular tumor and better predicts globe salvage using contemporary treatments. Cure rates for survival are much better than for retention of useful vision. The overall 10-year survival rate is nearly 90% for unilateral and bilateral tumors (Hurwitz, Shields, Shields, et al, 2011; Singh, Shields, and Shields, 2000). Retinoblastoma is one of the tumors that may spontaneously regress.

Of major concern in long-term survivors is the development of secondary tumors, especially osteosarcoma. Children with bilateral disease (hereditary form) are more likely to develop secondary cancers than are children with unilateral disease. Currently providers think these individuals are predisposed to developing cancer, and radiation increases their risk.

Therapeutic Management

Treatment of retinoblastoma is complex. Enucleation may be used to treat advanced disease with optic nerve invasion in which there is no hope for salvage of vision. Irradiation can be used when there is vitreous seeding. Chemotherapy has been used to decrease the tumor size to allow treatment with local therapies such as plaque brachytherapy (surgical implantation of an iodine-125 applicator on the sclera until the maximum radiation dose has been delivered to the tumor), photocoagulation (use of a laser beam to destroy retinal blood vessels that supply nutrition to the tumor), and cryotherapy (freezing of the tumor, which destroys the microcirculation to the tumor and the cells themselves through microcrystal formation). Vincristine, carboplatin, and etoposide are the agents most commonly used.

The use of chemotherapy in advanced disease, even in group V, is controversial and has not shown improved survival. Drugs that may be used in the treatment of metastatic disease include vincristine, cyclophosphamide, doxorubicin, cisplatin, carboplatin, and etoposide. In the case of CNS disease, intrathecal chemotherapy may be administered (Hurwitz, Shields, Shields, et al, 2011; Lanzkowsky, 2005).

Nursing Care Management

Nursing Care Management

To supplement routine health assessment, every adolescent male should know how to perform frequent testicular self-examination (TSE) to familiarize himself with his own anatomy and to ensure early detection of any abnormality. Ideally self-examination should be performed once a month beginning when physical development reaches Tanner stage 3, usually about age 13 or 14 years. (See Fig. 19-6.) Each testicle is examined individually, preferably after a warm bath or shower (when scrotal skin is more relaxed), using the thumbs and fingers of both hands and applying a small amount of firm, gentle pressure. The normal testicle is a firm organ with a smooth egg-shaped contour. The epididymis can be palpated as a raised swelling on the superior aspect of the testicle and should not be confused with an abnormality. The efficacy of teaching TSE to adolescent males has been tested, and it has been found to be successful (Han and Peschel, 2000).

1. Yes, there is sufficient evidence to arrive at conclusions.

2. a. It is important to note that approximately 10 days after administration of chemotherapeutic agents, patients hit their nadir (time at which their blood counts are at the lowest). At this time in the patient’s treatment it is crucial that parents call with any fever (as defined by the treating institution), since this may be the only sign of an infection. Other areas of concern include the appearance of the central line site and dressing. Is there any drainage on the dressing, foul odors, bleeding at the site, or erythema and pain at the site?

3. Initially medications and laboratory tests ordered should be reviewed for an acetaminophen order, for antibiotic or antifungal agents, and for parameters on how often blood should be drawn and cultures obtained. Any missing orders should be brought to the attention of the provider (physician or nurse practitioner). If a blood culture is required, it should be drawn before acetaminophen administration. Avoid use of aspirin- or ibuprofen-based medications. It is important with each assessment to pay careful attention to the signs of sepsis, which include fever or hypothermia, unexplained tachycardia, or tachypnea. A late sign of sepsis or septic shock is a drop in the patient’s blood pressure. Report any changes in the patients’ condition to the provider.

4. Yes, the documented vital signs and physical assessment support the need for careful assessment of infection and for continued monitoring for sepsis.

1. Yes, there is sufficient evidence to arrive at some possible conclusions.

2. a. Normal platelet counts are typically between 150,000 and 450,000/mm³ with some minor variations from laboratory to laboratory. Patients are at risk for spontaneous bleeding when the platelet count falls below 20,000/mm³. In some patients spontaneous bleeding from the nose, gums, or rectal area can occur at any time regardless of the platelet count. Certain medications such as ibuprofen- or aspirin-based products can interfere with platelet function regardless of the actual platelet count.

3. The immediate intervention would include assessing whether the oxygen is humidified. The nose is vascular and can bleed easily if the mucosa is dried by oxygen. Inspect the length and placement of the nasal prongs and the nasal mucosa for any signs of irritation. Other interventions include transfusing platelets as ordered by a physician or nurse practitioner and having the patient use a soft toothbrush or Toothette (sponge toothbrush) for oral care.

4. Yes, the laboratory results, timing of chemotherapy, and expected nadir support these conclusions; since we know that Paul received therapy 10 days ago, we would expect to see his counts drop to their lowest point around this time.