Pediatric Hematologic Disorders and Cancer Presented by Marlene Meador RN, MSN, CNE
Hematologic System Adult Pedi • Life cycle of RBC- 120 days • Cell production- marrow and spleen • RBC’s= 4.1 to 4.9 million/ml • Hemoglobin= • Hematocrit= • Life cycle of RBC- 100 days (neonate) • Cell production- red bone marrow (infant) • #RBC’s= 5million/ml at birth • Hemoglobin= 17-18 g • Hematocrit= 45-50%
Iron Deficiency Anemia • Cause • Signs and symptoms • Diagnostic tests • Nursing interventions • Oral supplements- What significant side effects does the nurse need to remember? • Dietary teaching- what specific foods?
What Parents Want to Know: (p 1281) • Specific foods: (based on age of child) • Cream of wheat or iron fortified cereal • Apricots, prunes, raisins and other dried fruits • Egg yolks • Dark green leafy vegetables • Administration of Iron Supplements: • Give with vitamin C –rich fluids • Prevent staining from liquid iron supplements • Changes in stool patterns • Avoid mixing supplement with food/drink containing calcium
Sickle Cell disease • Sickle cell trait- genetic disorders characterized by production of elongated, crescent shaped erythrocyte in the place of normal Hbgp 1282-1283 • Precipitating factors (p 1284) • Signs and symptoms
Three Forms of Sickle Cell Crisis • Vaso-occlusive • Acute sequestration • Aplastic
Types of Sickle Cell Crisis • Vaso-occlusive- most common effects • Pain • Hand and foot syndrome (dactylitis) • CVA- hemiplegia, aphasia, seizures, LOC changes, vision changes, and headache • Acute chest syndrome- chest pain, fever cough (leading cause of death in SCD) • Priapism • Hepatomegaly • Hematuria
Types of Sickle Cell Crisis • Aplastic Crisis: • Decreased RBC production- S&S malaise, headache, pallor, lethargy, and fainting (precipitated by infection) • Splenic sequestration- life threatening S&S pallor, irritability, tachycardia, hypovolemic shock • Hyperhemolytic crisis- (not in text)- RBC’s destroyed more rapidly than usual (immature cells)
Quick Review: • What is most common reason for admission to the ED for a child with SCD? • What precipitates a sickle cell crisis? • How does sickling effect the life span of an RBC? • what organs experience complications as a result of chronic sickling crisis?
Diagnosis & Treatment • Cord blood testing if one parent is known to carry trait • Blood transfusions • Complications • Nursing interventions before/during/after
Treatment • Patient/family teaching • Medications • Immunizations- why important?
Clinical Judgment: • Why are blood transfusions ordered for the patient in sickle cell crisis? • Can a neonate have a diagnosis of sickle cell disease? • What ethical issues relate to this diagnosis?
Hemophilia (p 1291) • X-linked trait • What factor is missing or defective? Factor VIII • Who is the carrier, and who is effected by this disorder?
Diagnosis & Treatment • When does diagnosis most commonly occur? • What specific laboratory tests and values? • What are signs & symptoms?
Nursing Care: • Factor VIII- when should the patient receive this medication? • What does the family need to know about factor VIII? • Human plasma • Vasopressin (DDAVP)
Nursing Care cont… • What is the primary nursing goal for a patient with hemophilia? • Prevent or stop bleeding • What are specific interventions to achieve this goal? • Administer Factor VIII • Apply local pressure for 10-15 minutes • Elevate the joint and immobilize • Apply cold compresses
Complications of hemophilia • Hemarthrosis- assess child for joint pain, edema, or permanent deformity. Where most common? • At risk for hemorrhage Death
Childhood Cancers “…communication promotes understanding and clarity; with understanding, fear diminishes; in the absence of fear, hope emerges; and in the presence of hope, anything is possible” (Stovall, 1995)
Childhood Cancer • C- continual unexplained weight loss, fatigue malaise • H- headaches with vomiting (early morning) • I- increased edema or pain in joints • L- lump or mass, persistent lymphadenopathy • D- development of whitish appearance in pupil of the eye • R- recurrent or persistent fevers, night sweats • E- excessive bruising or bleeding • N- noticeable pallor
What signs and symptoms would lead to the diagnosis of leukemia? • Fever • Pallor • Overt signs of bleeding • Lethargy or malaise • Anrexia • Large joint or bone pain • Petechiae, frank bleeding • Enlarged liver or spleen, changes in lymph nodes • Neurologic changes
Lab values for a diagnosis of leukemia: examination of CBC with at least 25% blasts confirm the diagnosis Normal Leukemia • Leukocytes < 10,000 • Leukocytes> 10,000 • Platelets 20-100,000 • Hemoglobin 7-11
Further diagnostic findings: • Bone marrow aspiration- iliac crest (why this site?) • How does the nurse prepare the child/family for this procedure? • What are the nurse responsibilities for this procedure?
Treatment and Plan of Care: (p 1313/1321) • Chemotherapy: three phases • Induction phase • Consolidation • Delayed intensification • Remission and maintenance
Nursing Plan of Care for a Child Undergoing Chemotherapy: • Myelosupression- protect from injury • Infection/sepsis (neutropenia)- protect from infection • Renal damage • GI disturbances • Metabolic emergencies
Intrathecal Medication • Chemotherapy instilled into spinal canal Assess and monitor for placement of intrathecal catheter and assess neuro checks
Cranial Radiation • Head and neck tumors are more sensitive to radiation than chemotherapy. • When would chemotherapy become an adjunct to radiation therapy?
Tumor Lysis Syndrome: • What causes tumor lysis syndrome? • What are signs and symptoms of this complications • What nursing interventions apply to treatment?
Bone marrow and Stem Cell Transplantation • Used to treat leukemia, neuroblastomas and some noncancerous conditions-aplastic anemia • Goal to administer a lethal dose to kill the cancer, and resupply the body with stem cells from the child’s own bone marrow, or a compatible donor
Develop a plan of care for the child undergoing treatment for leukemia: • Risk for injury: • Soft tissue/mucous membranes • Generalized trauma • Risk for infection: • Risk for alteration in bowel elimination • Risk for GI distress • Fluid volume delicate
Nephroblastoma- Wilm’s Tumor • Soft renal tumor - one or both kidneys • (p 1332) Metastasis or seeding spread by palpation • Nephrectomy treatment of Wilm’s tumor
Nursing treatment of Wilms’ tumor: • Pain management • Frequent reposition • Noninvasive and pharmacologic pain interventions • Prevent circulatory overload • Weigh daily • I&O, urine for specific gravity • Prevent infection • Hand washing • Protective isolation • Homecare needs
Clinical manifestations of Neuroblastoma(p 1327) • Smooth, hard, non-tender along sympathetic nervous system • Frequent location is abdomen • Neck and facial edema from vena cava syndrome • Increased ICP • Limp if metastasis to bone • Pancytopenia
Nursing Management • Assess by observation and inspection (not palpation) • Document bowel and bladder function • Record height & weight, observe gait • Chemotherapy, radiation, surgery • Teach parents S&S of infection. Why?
Osteosarcoma- most common primary bone malignancy in children • Goal of treatment- remove tumor and prevent spread of disease • Biopsy Chemo Surgery Chemo (radiation=palliative pain control) • Promote self esteem • Side effects of chemotherapy • Amputation of extremity • Separation from friends and family
Ewing Sarcoma- second most common bone tumor associated with children • Pain, soft tissue swelling • Anorexia, fever, malaise with metastasis • Diagnosis same as osteosarcoma • Management • Chemo • Surgery (decrease tumor bulk) • Radiation
Pathophysiology and Manifestations • Most common soft tissue malignancy • Divided by young (<10 yrs) and older (adolescents) in location • 60% have positive prognosis • Soft to hard, nontender mass (depends on location) • In pelvic tumors, may disrupt organ function
Diagnosis and Treatment • CT, BM aspiration and biopsy • Renal function and liver function tests • Treated with chemo, surgery and radiation
Retinoblastoma- rare malignant tumor of the neural retina • “cat’s eye” reflex seen as a white light in the pupil is the most common “leukocoria” • May have strabismus of involved eye • Red painful eye is late symptom • Staging based on extent of disease
Nursing care of the child/family with a malignant disease: • Initial focus on support of family members • Nurses facilitate the educational process to allay fears of unknown • Encourage family members to verbalize fears and questions • Postoperative care if indicated • Community resources (through the discharge planner, case worker)
Death and Dying: Understanding of death according to developmental age: • < 3 years- no understanding/concept of death • 3-5 years- afraid of separation from parents • 5-9 years- understand death is permanent, irreversible and sad. Concerns for fear of pain, being left alone and leaving parents and friends. • Age 10> have adult’s concept of death
Nursing Care and Grief • Child- encourage child to express feelings, allow choices, help maintain independence • Family- listen, answer questions, provide information, encourage expression of feelings and fears