Pediatric Hematology & Immunology – NUR 320 Notes

Pediatric Hematology & Immunology – NUR 320 Notes

Anemia

• Definition: A reduction in red blood cells (RBC) and/or hemoglobin (Hgb) and hematocrit (Hct) concentration. This condition diminishes the oxygen carrying capacity of the blood, leading to reduced oxygen availability to tissues.

• Causes:

  • Nutritional deficiency

  • Blood loss/hemorrhage

  • RBC destruction

  • Decreased RBC production

• Signs & Symptoms:

  • Lack of energy

  • Easily fatigued

  • Pallor

  • Headaches

  • Tachycardia

• Laboratory Findings:

  • RBC: The number of RBCs in the blood is decreased.

  • Hgb: The amount of hemoglobin in each cell is decreased.

  • Hct: The percent volume of packed RBCs is decreased.

• Normal Ranges:

  • RBC: $4.5-5.5 imes 10^6 ext{ cells/µL}$

  • Hgb: $11.5-15.5 ext{ g/dL}$

  • Hct: 35 ext{% to } 45 ext{%}

Types of Anemia

• Iron Deficiency Anemia: Most common type caused by a lack of iron.

• Sickle Cell Disease: Genetic disorder affecting hemoglobin structure.

• Thalassemia: Genetic condition affecting hemoglobin production.

• Aplastic Anemia: Resulting from the failure of bone marrow to produce sufficient blood cells.

Iron-Deficiency Anemia

• Definition: Decreased amount of hemoglobin due to lack of iron; iron is necessary to form hemoglobin.

• Causes:

  • Diet insufficient in iron

  • Preventable and reversible illness

  • Dietary habits such as consuming "white foods" (refined grains) or vegetarian diets.

  • Transition from formula to cow's milk.

• Peak Incidence: Occurs most frequently at ages 6-24 months.

• Signs and Symptoms:

  • Fatigue

  • Irritability

  • Headache

  • Splenomegaly

  • Pallor

Nursing Interventions for Iron-Deficiency Anemia

• Prevention and Teaching:

  • Encourage breastfeeding with iron supplementation starting around 4-5 months.

  • Use iron-fortified formula until 6 months of age.

  • Introduce iron-fortified foods at 6 months.

  • Limit cow's milk consumption during toddlerhood to 16-24 ounces per day.

  • Promote a varied diet throughout adulthood.

Iron Supplementation

• Recommended Dosage: Ferrous sulfate at $3 ext{ mg/kg/day}$ for a minimum of 3 months.

• Administration Tips:

  • Take with food/drink rich in vitamin C to enhance absorption.

  • Be cautious as liquid ferrous sulfate may stain teeth; recommend using a syringe or straw, and brushing teeth immediately post-ingestion.

  • Stool may turn tarry green or black; this is normal.

  • Keep out of children’s reach to prevent accidental overdose.

Sickle Cell Disease

• Definition: Genetic alteration causing sickling of red blood cells.

• Demographics:

  • Highest risk in individuals of African and Mediterranean descent.

  • Approximately 8% of African Americans carry the sickle cell trait, with 1 in 500 affected by sickle cell disease.

• Genetics: Autosomal recessive pattern.

Pathophysiology

• Genetic Mutation: The 6th amino acid in the hemoglobin molecule is altered, leading to the formation of sickled hemoglobin.

• Mechanism of Disease:

  • Sickled RBCs can cause occlusions and tissue ischemia due to impaired oxygen perfusion.

  • Crises:

  • Acute Sequestration Crisis: Occurs when the spleen traps damaged RBCs, resulting in rapid decreases in hemoglobin and blood volume, potentially causing hypovolemic shock.

  • Vaso-occlusive Crisis: Triggered by stressors; sickling leads to clumping of RBCs, causing severe pain and possible strokes.

  • Aplastic Crisis: Severe anemia due to parvovirus B19 impacting RBC production; life-threatening circumstance.

  • Acute Chest Syndrome: Similar to pneumonia; clumping in the lungs reduces gas exchange, contributing to hypoxia.

Diagnosis

• Screening: Newborn screening and hemoglobin electrophoresis to distinguish between sickle cell trait and disease.

• Clinical Presentation: Often not recognized until the first crisis in toddlers or preschoolers.

Nursing Interventions for Sickle Cell Disease

• Prevention Education:

  • Frequent handwashing to reduce infection risk.

  • Penicillin prophylaxis for young children (aged 2 months to 5 years).

  • Maintain up-to-date vaccinations (Hib, PCV 23, Meningococcal).

  • Administer folic acid to support RBC production.

  • Hydroxyurea treatment to stimulate production of fetal hemoglobin.

  • Encourage adequate hydration.

  • Promptly address any fevers (above $101.3^ ext{o}F$) and respiratory issues.

Management during Vaso-Occlusive Crisis

• Hospital Treatment:

  • Administer antibiotics for infections.

  • Provide IV hydration and blood transfusions.

  • Manage pain with controlled analgesics and monitor respiratory status for acute chest syndrome or stroke.

Signs and Symptoms of Acute Chest Syndrome

• Severe chest or back pain

• Fever above $101.3^ ext{o}F$

• Cough and dyspnea (breath shortness)

• Tachypnea (rapid breathing)

• Retractions (inward movement of the chest wall during inhalation)

• Declining oxygen saturation levels.

Signs and Symptoms of CVA (Cerebrovascular Accident)

• Severe, persistent headaches

• Vomiting

• Jerking or twitching of the face, limbs

• Seizures or abnormal behavior

• Inability to move an arm or leg

• Staggering or difficulty walking

• Slurred speech

• Vision changes.

Thalassemia

• Types:

  • Beta Thalassemia: Most common type.

  • Forms:

    • Minor: Asymptomatic carrier.

    • Intermedia: Moderate to severe anemia with splenomegaly.

    • Major (Cooley anemia): Severe anemia requiring transfusion for survival.

• Management: Regular blood transfusions and chelation therapy to manage excess iron from transfusions.

Aplastic Anemia

• Overview: Rare, life-threatening condition due to bone marrow failure.

• Consequences: Profound anemia, leukopenia, and thrombocytopenia affecting RBCs, white blood cells, and platelets.

• Etiology: Can be congenital (e.g., Fanconi syndrome) or acquired (e.g., due to viruses or exposure to toxic agents).

• Management Approach: Similar treatment strategies to leukemia.

Clotting Disorders

• Characteristics: Decreased clotting leading to abnormal bleeding such as:

  • Epistaxis (nosebleeds)

  • Gingival bleeding (gum bleeding)

  • Ecchymosis from minor injuries

  • Heavy menstrual bleeding.

• Examples:

  • Immune Thrombocytopenia Purpura (ITP)

  • Hemophilia

  • Von Willebrand's Disease.

Immune Thrombocytopenic Purpura (ITP)

• Definition: Condition wherein antibodies destroy platelets.

• Clinical Presentation: Commonly seen in children < 10 years old following a viral illness; presents with petechiae, purpura, and low platelet counts (< 50,000).

• Management: Primarily supportive; activity restrictions to prevent injury and bleeding. Medications include prednisone, IVIG, and anti-D antibodies as indicated.

Hemophilia

• Overview: Results from missing coagulation factors (factor VIII or IX); primarily affecting males due to its X-linked inheritance pattern.

• Management Strategies:

  • Avoidance of high-risk activities.

  • Administration of factor replacement therapies as needed.

  • Ensure patients wear medical alert bracelets.

Von Willebrand's Disease

• Definition: Genetic disorder resulting from abnormalities in von Willebrand factor, the most common bleeding disorder.

• Genetics: Autosomal dominant inheritance.

• Management:

  • Administration of von Willebrand factor concentrate.

  • Use of desmopressin to stimulate vWF release, with caution to limit usage to three consecutive days, maintaining at least a 24-hour interval between doses.

Nursing Interventions for Decreased Clotting Disorders

• Patient Education:

  • Importance of wearing medical alert bracelets.

  • Recognizing signs of low platelet counts.

  • Strategies for avoiding injury (e.g., sneezing with an open mouth).

• Emergency Treatment:

  • Elevate injured extremities above heart level.

  • Apply cool compresses and maintain pressure for bleeding injuries.

  • Treatment specifics will depend on the underlying condition (e.g., factor administration for hemophilia, desmopressin for Von Willebrand's).

HIV & AIDS

• Transmission Modes in Children:

  • Mother-to-child transmission

  • Risky behaviors among adolescents and young adults (unprotected sex, IV drug use).

• Decrease in Perinatal Transmission: 90% reduction in the US through prevention methods such as antiretroviral therapy (ART) during pregnancy and delivery; avoiding breastfeeding and pre-chewed foods by HIV+ caregivers.

• Clinical Characteristics: HIV, a retrovirus, primarily infects CD4 T-cells, presenting symptoms in children can include:

  • Lymphadenopathy

  • Hepatosplenomegaly

  • Oral candidiasis

  • Chronic or recurrent diarrhea

  • Failure to thrive

  • Developmental delays

  • Parotitis.

Laboratory and Diagnostic Testing for HIV Infection

• PCR Test: Positive result in infected infants older than 1 month old.

• ELISA Test: May yield false positives in infants with maternal antibodies persisting up to 24 months.

• Platelet counts: Can be elevated (> 500,000) in severe HIV infections.

• CD4 Counts: Typically low in HIV-positive status.

Quality Patient Outcomes in HIV Care

• Early recognition and timely management of HIV can lead to:

  • Controlled infection status.

  • Support for optimal growth and development.

  • Prevention of infectious complications or cancer.

  • Adherence to ART leading to prolonged survival and enhanced quality of life.