Paediatric Hematologic, Oncologic & Genetic Disorders - Concept Map
Paediatric Hematologic, Oncologic & Genetic Disorders: Blood, Cancer, and Inherited Conditions in Children
Related concepts
Hematologic Disorders
Sub concepts
- Iron-Deficiency Anemia (most common nutritional anemia)
- Sickle Cell Disease (autosomal recessive; HbS crisis management)
- Thalassemia Major (chronic transfusions; iron overload)
- Hemophilia A & B (X-linked recessive; bleeding disorder)
- Immune Thrombocytopenic Purpura (ITP; low platelets)
Relationship to central
Blood cell disorders affecting oxygen transport, clotting, and immunity
Oncologic Disorders
Sub concepts
- Acute Lymphoblastic Leukemia (ALL; most common childhood cancer)
- Acute Myeloid Leukemia (AML)
- Chemotherapy Toxicities & Management
- Pancytopenia & Consequences (infection, bleeding, anemia)
- Neutropenic Precautions (critical safety measures)
Relationship to central
Malignant proliferation requiring chemotherapy and intensive nursing support
Genetic & Chromosomal Disorders
Sub concepts
- Down Syndrome (Trisomy 21; most common chromosomal disorder)
- Patterns of Inheritance (autosomal recessive, X-linked, chromosomal)
- Phenylketonuria (PKU; inborn error of metabolism)
- G6PD Deficiency (X-linked; hemolysis risk)
- Philippine Newborn Screening (RA 9288; early detection)
Relationship to central
Inherited or chromosomal abnormalities affecting physical/metabolic development
Nursing Care Priorities (NCM Framework)
Sub concepts
- Safety & Infection Prevention (neutropenic precautions, no live vaccines)
- Bleeding Prevention & Management (soft toothbrush, no aspirin/NSAIDs)
- Pain Management (crisis control, opioids for severe pain)
- Nutritional Support & Feeding
- Family Education & Genetic Counselling
- Growth & Development Monitoring
- Psychosocial Support (alopecia, body image, coping)
Relationship to central
Evidence-based, priority-driven clinical management aligned with Maslow hierarchy
Concept connections
- Leukemia (Pancytopenia)Infection Risk (Neutropenia)Uncontrolled WBC proliferation crowds out functional neutrophils, leading to severe infection risk; fever is the only sign of infection in neutropenia and must be treated as medical emergency
- Leukemia (Pancytopenia)Bleeding Risk (Thrombocytopenia)Immature blast cells displace megakaryocytes, causing severe platelet deficiency and hemorrhagic manifestations (petechiae, bruising, mucosal bleeding)
- Leukemia (Pancytopenia)Anemia (RBC deficiency)Bone marrow crowding by leukemic blasts displaces erythropoiesis, causing microcytic or normocytic anemia with pallor and fatigue
- Hemophilia (X-linked recessive)Factor VIII or IX DeficiencyGenetic mutation on X chromosome prevents synthesis of clotting factors; males affected (hemizygous), females carriers (heterozygous)
- HemophiliaHemarthrosis & Joint DamageFactor deficiency leads to prolonged bleeding into joint spaces; recurrent hemarthrosis causes chronic inflammation, cartilage damage, and progressive joint disability (arthropathy)
- Sickle Cell Disease (Autosomal recessive)Vaso-Occlusive CrisisHbS polymerization under hypoxia, dehydration, or stress causes RBCs to sickle and occlude vessels; results in ischemic pain, tissue infarction, and organ damage
- Sickle Cell DiseaseHHOP Crisis ManagementEvidence-based protocol (Hydration, Heat, Oxygen, Pain control) directly addresses pathophysiology: hydration and oxygen prevent sickling, heat prevents RBC polymerization, pain control manages ischemic pain
- Thalassemia MajorIron Overload (Hemosiderosis)Chronic transfusion therapy to correct severe anemia causes cumulative iron deposition in heart, liver, and endocrine organs; leads to cardiomyopathy, cirrhosis, and diabetes
- Thalassemia MajorChelation TherapyIron-binding agents (deferoxamine, deferasirox) are essential to prevent iron overload toxicity; must monitor compliance and organ function during treatment
- Iron-Deficiency AnemiaFerrous Sulfate AdministrationFirst-line treatment requiring specific administration (empty stomach, with vitamin C, not with milk) to maximize absorption and minimize side effects; education is critical for compliance
- Down Syndrome (Trisomy 21)Congenital Heart DefectsChromosomal abnormality causes developmental defects in endocardial cushion; AV canal defects are most common associated cardiac lesion requiring echocardiography screening
- Down SyndromeHypotonia & Feeding DifficultiesMuscle tone deficiency and small oral cavity with protruding tongue create feeding challenges; nursing support with slow feeding, head positioning, and adapted techniques is essential
- Down SyndromeEarly Intervention ProgramsEvidence-based developmental support through PT/OT/speech therapy addresses hypotonia, motor delays, and language development; improves functional outcomes and independence
- PKU (Autosomal recessive)Newborn Screening (RA 9288)Heel-prick test at 24-72 hours detects elevated phenylalanine; early diagnosis allows lifelong low-phenylalanine diet to prevent intellectual disability
- G6PD Deficiency (X-linked)Hemolysis from Oxidative TriggersEnzyme deficiency impairs antioxidant defense; exposure to triggers (fava beans, sulfa drugs, antimalarials, naphthalene) causes acute hemolytic crisis; prevention through education is key
- Chemotherapy (Myelosuppression)Neutropenic PrecautionsChemotherapy destroys rapidly dividing bone marrow cells; severe neutropenia (WBC <500) requires strict protective isolation, avoiding foods/procedures that introduce infection risk
- ChemotherapyNausea/Vomiting ManagementAntiemetics must be given BEFORE chemotherapy administration to prevent acute/delayed emesis; supportive care (bland diet, frequent meals) minimizes nutritional impact
- ChemotherapyMucositis CareCytotoxic effects damage oral mucosa; frequent saline rinses, soft foods, and antifungal agents prevent secondary infection and maintain nutrition/hydration
- ITP (Autoimmune Thrombocytopenia)Isolated Platelet DestructionAutoimmune attack on platelets causes selective thrombocytopenia; WBC and RBC counts remain normal, distinguishing ITP from leukemia; often self-limiting in children
- Patterns of InheritanceGenetic CounsellingUnderstanding inheritance pattern (autosomal recessive, X-linked, chromosomal) is essential for non-directive counselling; guides family planning and risk communication
- Nursing Priority: Infection PreventionLive Vaccine ContraindicationImmunosuppression from chemotherapy or severe immunodeficiency contraindicates live vaccines (MMR, varicella); inactivated vaccines may be given with careful monitoring
- Nursing Priority: Bleeding PreventionAspirin & NSAID AvoidanceThese drugs impair platelet function and increase bleeding risk in thrombocytopenia or hemophilia; acetaminophen is preferred analgesic in these populations
- Pediatric Hematologic DisordersFamily-Centered Nursing CareAll hematologic disorders require integrated family education, psychosocial support, and adherence to chronic management plans; family coping directly impacts child outcomes
- PancytopeniaInfection (leading cause of death in leukemia)Severe neutropenia eliminates body's primary defense against bacterial/fungal infection; infection is the most common fatal complication requiring aggressive prevention and early treatment
- Sickle Cell Crisis TriggersHypoxia, Dehydration, Cold, InfectionAll triggers promote RBC sickling by altering oxygen availability, pH, or osmolarity; identifying and avoiding triggers is central to crisis prevention
- Down Syndrome (Trisomy 21)Atlantoaxial InstabilityLaxity of ligaments around C1-C2 vertebrae increases risk of subluxation with neck flexion; caution with contact sports and intubation; X-ray screening recommended
- Hemophilia Factor ReplacementPTT NormalizationFactor VIII or IX concentrate restores clotting cascade; PTT corrects to normal range, indicating adequate factor replacement and hemostatic function
Diagrams
Paediatric Hematologic, Oncologic & Genetic Disorders: Complete Concept Map
Comprehensive mind map showing all major pediatric hematologic, oncologic, and genetic disorders, their characteristics, clinical manifestations, management strategies, and nursing care priorities organized hierarchically from central concept through disorder types to specific nursing interventions aligned with NCM framework.
Nursing Assessment & Care Priority Framework for Pediatric Hematologic Disorders
Clinical decision-making flowchart showing systematic nursing assessment of common pediatric hematologic disorders, leading to appropriate priority interventions (infection prevention for leukemia, crisis management for sickle cell, factor replacement for hemophilia) and family education, aligned with NCM framework for prioritization.
Chemotherapy Toxicity Management & Nursing Interventions
Comprehensive nursing care flowchart for managing chemotherapy toxicities in pediatric cancer patients, showing systematic assessment of myelosuppression (infection and bleeding priorities), nausea, mucositis, alopecia, and drug-specific effects with evidence-based interventions and family education.
Sickle Cell Disease Crisis Management: HHOP Protocol
Step-by-step sickle cell crisis management flowchart emphasizing the evidence-based HHOP protocol (Hydration, Heat, Oxygen, Pain control) with assessment of common crisis triggers (hypoxia, dehydration, infection, cold), immediate interventions, and prevention education aligned with NLE priority management.
Down Syndrome (Trisomy 21): Clinical Features & Nursing Assessment
Clinical assessment flowchart for Down syndrome (Trisomy 21) showing characteristic physical features (hypotonia, facial features, hand/foot signs), priority screening for associated conditions (congenital heart defects, GI anomalies, hypothyroidism, hearing/vision, atlantoaxial instability, leukemia risk), and comprehensive nursing care including early intervention, growth monitoring, and family-centered support.
Iron-Deficiency Anemia: Assessment, Treatment & Family Education
Comprehensive nursing assessment and management flowchart for iron-deficiency anemia in children, covering risk identification, clinical presentation, laboratory confirmation, evidence-based medication administration (ferrous sulfate with vitamin C on empty stomach, via straw, with critical safety precautions for poisoning prevention), expected outcomes, and preventive strategies aligned with Philippine health programs.
Philippine Newborn Screening (RA 9288): Process & Nursing Responsibility
Comprehensive nursing flowchart detailing the Philippine Newborn Screening program (RA 9288), covering optimal timing (24-72 hours after protein feeding), heel-prick collection technique, transport procedures, conditions screened (congenital hypothyroidism, CAH, PKU, galactosemia, G6PD deficiency), result interpretation, and nursing responsibilities for education, follow-up, and support for early detection and prevention of disability.
Distribution of Common Pediatric Hematologic Disorders by Frequency
Pie chart showing the relative frequency of common pediatric hematologic disorders, with iron-deficiency anemia being the most prevalent (45%), followed by leukemia (20%), sickle cell disease (15%), thalassemia (10%), hemophilia (7%), and ITP and other conditions (3%). This distribution reflects epidemiologic prevalence and NLE testing frequency.
Hemophilia Nursing Care: X-Linked Inheritance & Clinical Management
Detailed mind map of hemophilia A and B covering X-linked recessive inheritance, factor deficiencies, clinical manifestations (hemarthrosis, easy bruising, prolonged bleeding), laboratory findings (prolonged PTT with normal platelets), factor replacement management, RICE protocol for acute joint bleeds, critical safety precautions (no aspirin/NSAIDs/IM injections), activity modifications, and comprehensive family education aligned with NCM nursing care priorities.