15.2 Pediatric Hematologic and Oncologic Disorders
Key Takeaways
- Sickle cell vaso-occlusive crisis is managed with hydration, oxygen when hypoxaemic, warmth, and adequate opioid analgesia; cold, tourniquets, and meperidine are avoided, and massage of an affected limb is contraindicated.
- Beta-thalassaemia major requires regular transfusion with iron chelation, because transfusional iron overload rather than anaemia becomes the main threat to the heart, liver, and endocrine organs.
- In haemophilia, the priority in an acute bleed is factor replacement first, then RICE measures; intramuscular injections, aspirin, and non-steroidal anti-inflammatory drugs are avoided.
- A palpable abdominal mass in a young child suggests Wilms tumour, and the abdomen must not be palpated because palpation can rupture the renal capsule and seed tumour cells.
- Iron supplements are given between meals with a vitamin C source, through a straw when liquid to protect the teeth, and dark stools are an expected harmless effect rather than a reason to stop.
15.2 Pediatric Hematologic and Oncologic Disorders
The haemoglobinopathies carry particular weight in this exam. Sickle cell disease and beta-thalassaemia have significant prevalence across the Arabian Gulf, and Qatar operates a national premarital screening programme precisely because of it. Expect these conditions in stems set in Qatari and regional practice.
Iron Deficiency Anaemia
The commonest childhood anaemia, peaking between 6 and 24 months when maternal iron stores are exhausted, and again in adolescence. Excessive cow's milk intake in toddlers is a classic contributor: it is low in iron, displaces iron-rich foods, and can cause occult gastrointestinal blood loss.
Presentation is pallor, irritability, fatigue, poor feeding, and — in prolonged deficiency — impaired cognitive development and pica. The blood picture is microcytic hypochromic with low ferritin.
Iron teaching for parents is a standard exam item:
- Give between meals with a vitamin C source such as orange juice to enhance absorption; avoid giving with milk, tea, or antacids.
- Give liquid iron through a straw or place it toward the back of the mouth, and rinse or brush afterwards, because it stains the teeth.
- Stools will turn dark green or black — this is expected and harmless, not a reason to stop.
- Expect constipation; increase fluid and fibre.
- Store iron securely out of reach. Accidental iron overdose is a leading cause of fatal poisoning in young children.
- Continue therapy for the full prescribed duration, usually well beyond the point at which the haemoglobin normalises, to replenish stores.
Sickle Cell Disease
An autosomal recessive haemoglobinopathy in which deoxygenated haemoglobin S polymerises, distorting red cells into rigid sickle shapes that occlude the microvasculature and haemolyse early.
Triggers of crisis: hypoxia, dehydration, infection, fever, acidosis, extreme temperatures, physical or emotional stress, and high altitude.
| Crisis type | Features |
|---|---|
| Vaso-occlusive (most common) | Severe pain in bones, joints, abdomen, or chest; dactylitis (painful swollen hands and feet) is often the first presentation in infants |
| Splenic sequestration | Sudden splenic enlargement trapping blood; profound anaemia and hypovolaemic shock — a paediatric emergency |
| Aplastic | Transient marrow shutdown, often triggered by parvovirus B19; severe anaemia with low reticulocytes |
| Acute chest syndrome | Fever, chest pain, hypoxia, new infiltrate; the leading cause of death |
Management of vaso-occlusive crisis — the four pillars: generous hydration, adequate analgesia (do not undertreat; scheduled opioids or patient-controlled analgesia for severe pain), oxygen when hypoxaemic, and warmth and rest.
What not to do matters as much: no cold applications or ice (vasoconstriction worsens sickling), no tourniquets or restrictive clothing, no massage of an affected limb, and avoid meperidine (pethidine), whose metabolite normeperidine accumulates and causes seizures.
Long-term care includes penicillin prophylaxis from infancy and full immunisation including pneumococcal and meningococcal vaccines, because functional asplenia leaves the child vulnerable to encapsulated organisms; hydroxyurea to raise fetal haemoglobin; folic acid; transcranial Doppler surveillance for stroke risk; and parental teaching to seek care urgently for fever above 38.5 °C, which is treated as a potential sepsis until proven otherwise.
Thalassaemia
Beta-thalassaemia major presents in the first year as the switch from fetal to adult haemoglobin completes: severe anaemia, failure to thrive, hepatosplenomegaly, and — untreated — the characteristic frontal bossing and maxillary prominence of marrow expansion.
Treatment is regular transfusion to suppress ineffective erythropoiesis, which creates the central long-term problem: transfusional iron overload. Iron deposits in the heart, liver, pancreas, and endocrine organs, causing cardiomyopathy, cirrhosis, diabetes, and delayed puberty. Iron chelation — deferoxamine by subcutaneous infusion, or oral deferasirox — is therefore the treatment that determines survival, and adherence support is the central nursing role, particularly with adolescents for whom nightly infusions are burdensome. Monitor ferritin, cardiac and hepatic function, growth, and pubertal development. Because of Qatar's premarital screening programme, genetic counselling for the family is routinely part of the care plan.
Bleeding Disorders
Haemophilia is an X-linked recessive deficiency of factor VIII (haemophilia A) or factor IX (haemophilia B), so it typically affects males, with females as carriers. Presentation is haemarthrosis — a warm, swollen, painful joint with reduced movement, most often knee, ankle, or elbow — plus prolonged bleeding after minor procedures, and intracranial bleeding as the most feared event.
Acute bleed management order: factor replacement first, then RICE — Rest, Ice, Compression, Elevation. The joint is immobilised briefly, then range of motion is resumed early to prevent contracture and chronic arthropathy.
Avoid: intramuscular injections (use subcutaneous or intravenous routes; if immunisation must be intramuscular, use the smallest gauge needle with prolonged pressure, after factor cover as prescribed), aspirin and non-steroidal anti-inflammatory drugs, rectal temperatures and suppositories, and contact sports. Encourage swimming and cycling with a helmet, use a soft toothbrush, and advise a medical alert identification.
Immune thrombocytopenic purpura in children usually follows a viral illness and presents with petechiae, purpura, and epistaxis in an otherwise well child. Most cases resolve spontaneously; management is observation plus bleeding precautions, with immunoglobulin or corticosteroids for significant bleeding. Teach activity restriction proportionate to the platelet count and avoidance of aspirin and non-steroidal anti-inflammatory drugs.
Childhood Cancers
Acute lymphoblastic leukaemia
The commonest childhood malignancy. Marrow replacement produces the triad that maps directly onto the three cell lines: anaemia (pallor, fatigue), neutropenia (fever, infection), and thrombocytopenia (bruising, petechiae, epistaxis), often with bone pain, lymphadenopathy, and hepatosplenomegaly. Diagnosis is by bone marrow aspiration; lumbar puncture assesses central nervous system involvement, and intrathecal chemotherapy is given for prophylaxis.
Nursing priorities are those of the neutropenic, thrombocytopenic child: infection precautions, bleeding precautions, no rectal temperatures, suppositories, or intramuscular injections, meticulous mouth care for mucositis, nutritional support, and structured family support through a long treatment protocol.
Wilms tumour (nephroblastoma)
A renal embryonal tumour presenting as a firm, non-tender, smooth abdominal mass that does not cross the midline, usually in a child under 5, sometimes with haematuria or hypertension.
The single highest-yield nursing action is a prohibition: do not palpate the abdomen. Palpation risks rupturing the renal capsule and disseminating tumour cells, upstaging the disease. Post a clear sign at the bedside — "Do not palpate abdomen" — handle the child gently during bathing and positioning, and communicate the precaution at every handover.
Neuroblastoma
A tumour of neural crest origin, most often arising in the adrenal gland or sympathetic chain, presenting as an irregular abdominal mass that does cross the midline — the key discriminator from Wilms tumour. It frequently presents with metastatic disease, and periorbital ecchymosis ("raccoon eyes") from orbital metastases is a recognised sign.
Brain tumours
Most childhood brain tumours are infratentorial, so the presentation is raised intracranial pressure and cerebellar signs: morning headache relieved by vomiting, ataxia, nystagmus, head tilt, and — in infants with open sutures — a bulging fontanel and increasing head circumference. Measure head circumference on every infant assessment.
A 9-year-old with sickle cell disease is admitted with a vaso-occlusive crisis affecting both knees, rating the pain 9 out of 10. Which prescription should the nurse question?
A 3-year-old is admitted for investigation of a firm, smooth, non-tender left-sided abdominal mass that does not cross the midline, with a working diagnosis of Wilms tumour. Which nursing action is the highest priority?
A 5-year-old with haemophilia A falls at school and arrives with a swollen, warm, painful right knee that he is holding flexed. What is the correct order of nursing actions?