PEDI | Hematology P1 (NO Drugs)

1. Major Hematologic Disorders

• Iron Deficiency Anemia: The most common anemia in children, often caused by excessive milk intake (>24 oz/day) displacing iron-rich foods.

◦ Management: Administer iron supplements (give with Vitamin C/juice, avoid milk) and limit milk intake. Stools may turn tarry green.

• Sickle Cell Disease (SCD): Genetic disorder where HgbS replaces normal HgbA, causing RBCs to sickle, obstructing blood flow.

◦ Crisis Management: Prioritize hydration (1.5–2x maintenance), oxygenation, and pain control (opioids, NSAIDs). Medical emergency: Acute chest syndrome or splenic sequestration.

• Hemophilia: X-linked recessive clotting deficiency (A=Factor VIII, B=Factor IX).

◦ Safety: Prevent bleeding (no contact sports, soft toothbrush). Treat bleeds with RICE (Rest, Ice, Compression, Elevation) and factor replacement.

2. Pediatric Oncology

Unlike adult cancers (epithelial/environmental), childhood cancers are largely embryonal (tissue-based), grow rapidly, and are highly responsive to treatment.

• Leukemia (ALL/AML): The malignancy of bone marrow/blood. ALL is the most common. Diagnosis via bone marrow biopsy; lumbar puncture checks CNS involvement.

• Solid Tumors:

◦ Wilms Tumor: Renal tumor. Never palpate the abdomen pre-op to prevent rupture/metastasis.

◦ Neuroblastoma: Neural crest tumor, often presents as an abdominal mass crossing the midline.

◦ Retinoblastoma: Signaled by "cat's eye reflex" (whitish glow in pupil).

3. Critical Pharmacology & Safety

Chemotherapy requires specialized handling due to toxicity.

• Vincristine: A mitotic inhibitor.

◦ FATAL WARNING: For IV use only. Fatal if given intrathecally.

◦ Side Effects: Peripheral neuropathy (foot drop), constipation, vesicant (extravasation risk).

• Methotrexate: Folate antimetabolite.

◦ Risks: Myelosuppression, hepatotoxicity, mucositis, renal failure (requires hydration/alkalinized urine).

◦ Rescue Agent: Leucovorin is used to neutralize toxic effects.

• Daunorubicin/Doxorubicin: Anthracyclines.

◦ Risks: Severe cardiotoxicity (lifetime cumulative dose limits apply) and red/orange urine.

• Etoposide: Topoisomerase inhibitor.

◦ Admin: Watch for hypotension during rapid infusion (infuse over 30–60 mins).

• Prednisone: Corticosteroid used for induction/palliation.

◦ Side Effects: Hyperglycemia, mood changes, immunosuppression, Cushing’s syndrome. Must taper to avoid adrenal insufficiency.

• Mesna: A cytoprotectant agent.

◦ Use: Must be given with Ifosfamide or Cyclophosphamide to prevent hemorrhagic cystitis (bladder bleeding).

4. Nursing Priorities

• Neutropenia: Infection is the leading cause of death. Calculate ANC; implement protective isolation if ANC <500. No fresh flowers/fruit; monitor temp closely.