Module Seven: Pediatric Oncology, MSK, Neuromuscular, Endocrine, Mental Health, and Emergencies

Fundamentals of Pediatric Patient Care and Development

Pediatric patients must not be viewed simply as "little adults." Effective pediatric care requires interventions that specifically support growth and development while accounting for how various diseases impact these processes. Recognizing the differences in disease presentation between children and adults is a core competency. Professionals must be adept at pediatric-specific medication administration and the subsequent monitoring of effects. Furthermore, education must be provided comprehensively to both the patient and the family. A defining characteristic of pediatric care is the integration of play into all aspects of treatment and interaction to facilitate coping and development.

Pediatric Oncology and Organ Neoplasms

Wilms’ Tumor is the most common childhood cancer of the kidney. Approximately $75\%$ of cases occur in children under the age of $5$, and about $5\%$ of cases are familial in nature. Expected clinical findings include a painless abdominal mass that uniquely does not cross the midline. Other symptoms include weight loss, anemia, and enlargement of the liver and spleen. The primary treatment modalities are surgery and chemotherapy. A critical nursing intervention is to never palpate the abdomen, as this action could lead to the rupture of the tumor capsule and the subsequent spread of tumor cells.

Neuroblastoma is the most common cancer seen in infancy, typically occurring in children under $22$ months of age. It is a solid, extracranial tumor that originates from fetal cells forming the adrenal medulla and the sympathetic nervous system (SNS). While the primary site is usually the abdomen, it is highly aggressive, with $70\%$ of cases having already metastasized prior to diagnosis. Unlike Wilms’ tumor, the firm, painless abdominal mass associated with neuroblastoma typically crosses the midline. Patients may experience urinary frequency or retention due to bladder compression. Symptoms of metastasis include periorbital edema, supraorbital ecchymosis, exophthalmos, hepatomegaly, respiratory obstruction, fatigue, anorexia, and weight loss. Treatment involves surgery, radiation, and chemotherapy, though the tumor may occasionally regress spontaneously.

Brain neoplasms are the most common organ tumors in children, representing $25\%$ of all childhood cancers. Most of these tumors occur in the brainstem or cerebellum. While they can originate from any cranial cell, glial tumors are the most prevalent, followed by astrocytomas. Expected findings depend heavily on location and size, but often include headaches upon awakening, weight loss, and anemia. Treatment includes surgery and chemotherapy. Similar to Wilms’ tumor protocols, specific precautions are taken to prevent the spread of malignant cells during assessment.

Soft Tissue and Bone Neoplasms

Rhabdomyosarcoma arises from muscle tissue and can occur in any location throughout the body. The expected findings are entirely dependent on the tumor's location; clinicians must understand the function of the involved organ to recognize signs of dysfunction, such as obstruction. Treatment requires a multidisciplinary approach involving surgery, radiation, and chemotherapy.

Osteosarcoma is the most common bone cancer in children, most frequently diagnosed in patients older than $10$ years of age. More than $50\%$ of these tumors occur in the femur, specifically at the metaphysis growth plate. Symptoms include localized pain, which is often relieved when the limb is flexed, and changes in gait or a noticeable limp. Treatment involves chemotherapy and surgery, which may range from limb-salvage procedures to amputation. Ewing’s Sarcoma is rarer than osteosarcoma and typically presents in the diaphysis (shaft) of the bone.

Blood Neoplasms and Cancer Treatment Modalities

Hodgkin’s Lymphoma (HD) is a malignancy of the lymph system that most frequently occurs in adolescents between the ages of $15$ and $19$. Clinical findings include painless enlargement of the subclavicular or cervical nodes, a nonproductive cough, abdominal pain, low-grade fever, anorexia, pruritus (itching), night sweats, and weight loss. Treatment consists of chemotherapy and radiation.

Acute Lymphoblastic Leukemia (ALL) is a malignancy of the bone marrow characterized by the overproduction of immature white blood cells (WBCs). It is most commonly found in children between the ages of $2$ and $6$, with a higher incidence in white males and children with Down syndrome. Symptoms include an unresolving low-grade fever, pallor, bruising (petechiae), lethargy, joint pain, nausea, vomiting, weakness, and anorexia. Treatment involves IV or intrathecal chemotherapy, corticosteroids, and potentially a bone marrow or hematopoietic stem cell transplant.

Chemotherapy agents are selected based on the specific type of cancer. Administration often requires the placement of a port or central line. Chemotherapy may also be used as an adjuvant to radiation to make cells more susceptible to treatment. Because it affects rapidly growing cells throughout the body, common side effects include immunosuppression and pancytopenia. Radiation doses are meticulously calculated and delivered in divided treatments over several weeks. Tattoos may be used to mark the skin for accurate beam delivery. While it affects rapidly growing cells, the effects of radiation are localized to the irradiated area and can result in lymphedema and the formation of scar tissue.

Musculoskeletal Disorders and Fractures

Fractures in children can be caused by obesity and poor nutrition. Clinical indicators include pain, crepitus, deformity, edema, ecchymosis, and a decrease in the use of the affected area. Fracture types are diverse, including plastic deformation, buckle, greenstick, transverse, oblique, spiral, phyal, stress, complete, incomplete, closed (simple), open (compound), complicated, and comminuted. Emergency care must be provided at the time of injury, followed by pain assessments, range of motion evaluations, and neurovascular checks (the 5 P’s: Pain, Pulselessness, Pallor, Paresthesia, Paralysis). Monitoring includes sensation, temperature, color, capillary refill, and movement. Complications can include Compartment Syndrome, Volkmann contracture, renal calculi, embolism, and osteomyelitis.

Traction methods used in pediatric orthopedics include Skin Traction, Skeletal Traction, Halo Traction, and Manual Traction. Each requires specific nursing care to maintain alignment and skin integrity.

Congenital musculoskeletal disorders include Clubfoot (Talipes Equinovarus), characterized by the heel tilting down and toes turning in. Treatment involves serial casting, followed by surgical correction of tightened tendons, and then bracing for $2$ to $4$ years. Legg-Calve-Perthes Disease involves a temporary, significant reduction in blood supply to the head of the femur, leading to avascular necrosis of the hip joint. It presents as a limp without initial pain, progressing to pain in the hip, knee, or thigh. Bisphosphonates are currently being tested as a treatment.

Developmental Dysplasia of the Hip (DDH) is commonly associated with breech births. Signs include limited abduction, leg length differences, asymmetric gluteal folds, and an audible click during assessment. The Pavlik Harness is used for $12$ weeks, worn $23$ hours per day to prevent hip flexion and adduction. Straps are readjusted weekly, and skin/neurovascular checks should be performed twice daily. Parents should be instructed to use undershirts and knee socks while avoiding lotions or powders. If the harness is unsuccessful, surgical reduction and the application of a spica cast are necessary.

Osteogenesis Imperfecta is a genetic disorder resulting in brittle bones. Clinical findings include multiple bone fractures, blue sclera, early hearing loss, and small, discolored teeth. Diagnosis is confirmed via bone biopsy. Nursing care involves frequent oral care and manual blood pressure monitoring to prevent fractures. Scoliosis is an abnormal lateral and rotational curvature of the spine. If the curve is less than $25$ degrees, no intervention is required. Curves between $25$ and $45$ degrees require a brace, while curves exceeding $50$ degrees require spinal fusion surgery. Signs include uneven clothes, uneven shoulders or hips, and a rib hump.

Chronic Neuromuscular Disorders

Cerebral Palsy (CP) is a permanent but variable condition of the brain affecting muscle tone, posture, and movement. Spastic CP, the most common type, involves stiff and rigid muscles and can manifest as diplegia ($2$ limbs) or quadriplegia ($4$ limbs). Dyskinetic CP involves oscillating muscle tone, while Ataxic CP affects coordination. Risk factors are categorized into prenatal (anoxia, maternal infection, genetics), perinatal (low birth weight, birth trauma), and postnatal (stroke, meningitis, head trauma). Medications include Baclofen, Diazepam, Botulinum Toxin A, and Valproic acid for seizures. Interprofessional care involves physical, occupational, and speech therapy, along with various surgical specialties.

Spina Bifida occurs when the neural tube fails to close, often linked to folic acid deficiency or radiation exposure. It results in varying neurological impairments, including paralysis below the lesion level, affecting bowel and bladder control. Comorbidities include hydrocephalus and Chiari II malformation. Complications involve skin ulcerations, latex allergies, and increased intracranial pressure.

Juvenile Idiopathic Arthritis is an autoimmune disorder targeting synovial joints. Inactivity increases stiffness, leading to delayed growth, irritability, and rashes. Management focuses on medical control of inflammation. Muscular Dystrophy is an X-linked genetic disease causing muscle fiber degeneration. Duchenne (DMD) is the most common type. Symptoms appearing around age $3$ to $4$ include proximal weakness and a toe gait. The Gower sign (using hands to climb up one's own body to stand) is diagnostic. Cardiac dysfunction is a prominent concern.

Endocrine Disorders: Diabetes and Growth

Type 1 Diabetes involves an absolute lack of insulin due to genetics, toxins, or viruses. Type 2 involves insulin resistance linked to obesity and high triglycerides. Hyperglycemia is defined as blood glucose (BG) greater than $250\,mg/dL$ and presents with the three P’s (polyuria, polydipsia, polyphagia), flushed dry skin, and Kussmaul respirations. Hypoglycemia is defined as BG less than $60\,mg/dL$ and presents with hunger, diaphoresis, tachycardia, and changes in behavior. Diagnostic thresholds include a Fasting Glucose > 126\,mg/dL, Random Glucose > 200\,mg/dL, and HbA1c > 6.5\%.

Diabetic Ketoacidosis (DKA) is life-threatening, characterized by a BG > 330\,mg/dL, glycosuria, pH < 7.3, and bicarbonate < 15\,mmol/L. Management involves rapid isotonic fluid replacement and an insulin drip at $0.1\,\text{units/kg/hr}$. BG drops must be monitored to ensure they do not exceed $100\,mg/dL$ per hour to avoid increased intracranial pressure.

Growth Hormone (GH) Deficiency results in short stature, delayed epiphyseal closure, and increased insulin sensitivity. It is treated with Somatropin via SQ injections administered $6$ or $7$ days per week at bedtime. If growth does not reach $2.5\,cm/year$, the medication is stopped. Addison’s Disease involves adrenal insufficiency. In children, the most significant result of an adrenal crisis triggered by stress or illness is hypoglycemia.

Mental Health and Pediatric Emergencies

Mental health conditions include Depression (treated with SSRIs like Sertraline), ADHD (characterized by inattention and impulsivity, treated with Methylphenidate), and Autism Spectrum Disorder (marked by social delays and distress with routine changes). Intellectual disabilities and Failure to Thrive (weight < 5\%)) require early intervention and nutritional support. Maltreatment (physical, sexual, emotional, or neglect) and bullying are critical social-health issues requiring reporting and support.

Pediatric respiratory emergencies include Respiratory Insufficiency (increased work of breathing), Respiratory Failure (inability to oxygenate), and Apnea (respiratory cessation for $20\,seconds$ or longer). Airway obstruction is often caused by foreign body aspiration. Other emergencies include drowning (can occur in only $1\,inch$ of water) and poisoning. Tylenol poisoning progresses from N/V in the first $24$ hours to RUQ pain and jaundice by $72$ to $96$ hours. Lead poisoning can cause cognitive delays, seizures, or death.

Burn management involves classifying severity (Superficial to Full Thickness). For major burns, $100\%$ humidified oxygen and fluid replacement using the Parkland formula are critical. Effective fluid resuscitation is measured by an output of $0.5$ to $1\,mL/kg/hr$. Colloid fluids are added after the first $24$ hours. Management also addresses pain (Morphine), infection prevention, and potential complications like inhalation injury or hypertrophic scarring.

Pediatric Death, Dying, and Grief

Care at the end of life involves Palliative Care and Hospice Care. Grief responses are categorized as Anticipatory, Complicated, Parental, or Sibling grief. Children's understanding of death varies by age: Infants/Toddlers (birth to $3$ years) lack an understanding of permanence; Preschoolers ($3$ to $6$ years) may view death as reversible or a form of sleep; School-age ($6$ to $12$ years) begin to understand the finality; and Adolescents ($12$ to $20$ years) have an adult-like understanding but may struggle with their own mortality.