Hematologic Disorders

Case Study
You have been assigned to care for MA.

He is a 4 year old male who was admitted for pneumonia. He is on 1L O2. He is afebrile. HR 136, RR 26, B/P 100/60. Oxygen saturation is 93%. His mother is at the bedside. During shift report you are given information that he is eating and drinking well. His IV fluids were decreased to TKO rate. He is on day 3 of antibiotics.

You complete the 8 am assessment. Findings: Skin pale and cool to touch. Cap refill 2 seconds. Lethargic. No appetite. Not interested in playing. Lungs with bilateral course sounds. S1 S2 heart sounds with splitting of S2. Generally thin, well appearing child. What additional information would you like?

Case Study

Eats pasta, cheese, crackers, chicken nuggets and McDonalds. Drinks primarily soda and Juicy Juice No MVI Favorite activities: video games, watching cartoons Sleeps 16 hours per day. Falls asleep easily and eveywhere Lives in St.Charles. 70 year old home. Lives with parents and 8 year old female sibling.

CBC
WBC: 6.5 range: 5.5-15.5 RBC: 4.1 range: 3.1-4.5 HGB: 10.9 range: >11.5 HCT: 29.5 range: > 33.5 MCV: 69 range: > 80 MCHC: 19 range: 24-30 Retic: 0.8% range: 0.5% 2.0%

Iron Related Tests

Serum ferritin: 2 range: 7-142 ng/ml

Total Iron: 30 range: 50-120 mcg/dl

Pb Level

Lead level: 6 range: <10mg/dl

Iron Deficiency Anemia

Most common cause of anemia Decreased iron intake Increased iron or blood loss Increased growth Rare before 4-6 months of age Can be associated with Pb poisoining Family education is critical

The Plan of Care

Administer iron (elemental iron) Iron therapy continued for at least 3 months after Hgb has normalized Hgb should increase within 2 weeks Increase iron rich foods Give iron on empty stomach in an acidic environment Clean teeth after iron administration Administer iron with vitamin C Black stools Consider social service referral

Sickle Cell Disease

African Americans, Mediterranean, Indian, and Middle Eastern individuals affected Trait found in 8% of AA Autosomal recessive Sickle cell trait individuals may be symptomatic under extreme conditions

Genetic Transmission
1 parent (+) and 1 parent (-) then, 50% risk trait 2 parents (+) then, 25% unaffected, 25% disease, 50% trait

Pathophysiology

Pathophysiology
HbS Increased RBC destruction Decreased RBC lifespan Microcirculation obstructions High reticulocyte count Brain, Heart, Kidney, Eye, Spleen

Manifestations

Stressful event causes sickling crisis (deoxygenation, acidosis, dehydration) Vaso-occlusive, acute sequestration, or aplastic crisis Infection Delayed growth/puberty Pallor, jaundice, fatigue Avascular necrosis of hips and shoulders Renal dysfunction Retinopathy Priapism Cerebral Infarct Acute chest syndrome Cardiomyopathy

PAIN!!!!

Nursing Management

Fever (>101.3) IV Abx, cultures, hydration Prophylactic penicillin therapy Influenza and pneumovax vaccine Oxygen therapy Hydration Strict I and O Transfusion Pain Management

General Patient Education Guidelines

Fluid Intake Frequent urination Discuss risk for infection Importance of primary care Caution against extremes in temperature Pain Management Adequate Rest

Beta-Thalassemia
Thalassemia major or Cooleys anemia Beta-polypeptide chain abnormality Abnormality of Hgb synthesis resulting in hemolysis of erythrocytes Exagerrated RBC production (erythropoiesis)/destruction(hemolysis) Increased intestinal absorption of iron resulting in hemosiderosis

Etiology and Incidence

Autosomal recessive Can have mild expression of gene thalassemia minor Mediterranean descent primarily affected

Clinical Manifestations

Frontal bossing Maxillary prominence Wide-set eyes Flat nose Hepatosplenomegaly Jaundice, pallor Growth retardation Severe anemia

Therapeutic Management

RBC transfusion Chelation therapy (deferoxamine) Splenectomy Bone Marrow Transplant

Nursing Interventions

Oxygenation Monitor Hgb level, iron level Hydration Management of sequelae of hemosiderosis Chelation therapy education Psychosocial support Vigilant primary care (immunizations, prompt treatment of infection, etc.) Genetic counseling

Hemophilia

Factor VIII deficiency

**Can see symptoms with factor IX and XI deficiency

Etiology

X-linked Autosomal recessive

Manifestations
Epistaxis Bleeding gums Hematuria Petechia Frequent bruising Muscle/joint bleeding Circumcision bleeding excessive

Diagnostic Evaluation
Quantitative immunoelectrophorectic assay Factors VIII and IX assays Platelet count Fibrinogen level Bleeding time PT PTT

Management

Avoid rectal temperature measurement Measure the affected joint/extremity Assess LOC Apply gentle pressure to superficial wounds Administer factor (prophylactically) Administer clot stabilizing medication RICE Consider co-morbidity (?) Extensive family education (safe environment, prompt treatment, acceptable activities, medication education)

Von Willebrands Disease

Most common inherited bleeding disorder Autosomal dominant inherited Dysfunction of Von Willebrands protein (carrier protein for VIII and co-factor for platelet binding to endothelial tissue) Variable severity Prolonged bleeding Treat with DDAVP FFP and cryoprecipitate prophylactically Family education

Immune Thrombocytopenic Purpura

Acquired bleeding disorder ? Autoimmune Platelet < 150,000, purpuric rash, normal bone marrow Treatment: watchful waiting, IVIG, steroids, platelet transfusion

Nursing Interventions

Soft tooth brush Gentle pressure to superficial wounds Monitor labs (?) Discuss home environment/safe environment Appropriate play activities for diagnosis and developmental age Steroid use education (risk for infection, tapering medication) Caregiver support Reinforce need for vigilant follow-up IVIG delay MMR for at least 10 months Avoid medications that affect platelet function

Pediatric HIV Infection

9000 children < age 13 diagnosed (2001) Transmission: perinatal = 90% Other causes: blood products, abuse African American and Latinos affected Half of new diagnosis in the US occur in adolescents age 15-24 years Chronic condition

Vertical Transmission

General risk is multifactoral (viral load, PROM, prematurity, low birth weight, treatment regimen) Combination therapy with Protease Inhibitors = risk of transmission 1% General risk without treatment = 20-25% No breastfeeding Prenatal diagnosis of HIV critical Consider mandatory screening of all newborns

Pediatric Specific Manifestations

Typically faster progression Higher viral load FTT and developmental delay is a common finding High risk for PCP (start prophylaxis empirically) Lymphocytic interstitial pneumonitis Higher CD4 counts indicate severe immune suppression (<750 in children under 1 year of age) Review table 41-1 p. 1053

LIP

Other Manifestations

Lymphadenopathy Hepatosplenomegaly Recurrent URI, otitis media, sinusitis Parotitis Cardiomyopathy Chronic diarrhea/malabsorption Peripheral neuropathy Persistent candidiasis Skin Infections

Pediatric Diagnosis
Elisa and Western Blot (after 18 mos.) PCR: qualitative and quantitative Known exposed: test within 48 hours Positive result confirmed with a repeat assay Negative at birth: test at 1-2 months, 6 months, and 18 months If positive: use CD4 and viral load to assess response to therapy

PCP Prophylaxis
Initiate on exposed infant at 4-6 weeks Trimethoprim-sulfamethoxazole Continue until definitive negative diagnosis Can administer M-W-F, or 1 dose daily Specific caregiver education

Immunizations
Consider immune status when immunizing Pneumococcal and Influenza required Follow usual immunization schedule Consider Varicella although often contraindicated MMR allowed if health immune system If exposed to varicella, give immuneglobulin (V-zig) within 24-48 hours

Nursing Management

Continual and repeated education Assistance with adherence to medication regimen (HAART) Psychosocial assessment and attention to the individual patient and family needs Disclosure (when to, when not to) Dispelling myths Integrating cultural practices, family beliefs, and resources into the plan of care Education regarding transmission precautions Nutrition (high calorie, high protein) Support respiratory status Skin care Develop a plan that allows the child to maximize function for as long as possible

Specific Adolescent Issues

Isolating nature of the disease Loss of other affected family members Disclosure (dating, intimacy, life planning) Incorporate the adolescent into decision making process Medication education Promoting integration with peers

Systemic Lupus Erythematous

Frequent onset at puberty (9-15 years) Uncommon in children <10 years More common in females: approx. 8:1 More common in African American, Asian, and Latino descent Characterized by exacerbation:remission More severe in children 5-year survival: 90% with renal disease 100% without renal disease

Etiology
Genetic predisposition Immune system dysregulation Environmental stimuli (triggers) Hormonal influences Multisystem, autoimmune disease Variability in disease severity

Manifestations

1997 American College of Rheumatology

Specific Manifestations
Malar rash Nephritis: proteinuria >0.5 g/d, cellular casts Seizures Psychosis Pleuritis Pericarditis

Management

Immunosuppression (steroids, Cytoxan) Support symptoms Monitor vital sign trends Caution with live vaccine administration particularly with immunecompromoised status Hep B, influenza, pneumococcal Nephritis = low NA diet (2gm/day), may have HTN as a result of renal involvement Avoid alfalfa (trigger) Sun Protection Physical therapy Individualized psychosocial plan based on type and severity of symptoms Family education (medication, triggers, natural history of disease, integrating illness into life goals)

Pediatric Considerations

Most frequent cause of death with SLE = overwhelming infection Team approach to care (social work is a critical component) Reinforce early intervention of any symptoms Always assess mouth for ulcers (early sign of exacerbation) Caution against pregnancy, OCP use and possible exacerbations related to hormonal shifts (adolescent)