The pathophysiology involves a lack of iron to produce hemoglobin, which leads to a decreased ability to transport oxygen. To compensate, the body increases cardiac output, shunting blood from the periphery to vital organs. This results in clinical manifestations such as:
- Increased heart rate (tachycardia)
- Pallor (paleness)
- Fatigue and weakness
- Brittle nails
Nursing Care and Nutritional Interventions
Nursing management focuses on replenishing iron stores and preventing complications like developmental delays.
- Supplementation: Iron supplements may be started as early as 2 months of age for preterm babies, or by 4–6 months for full-term babies via baby cereal.
- Absorption Optimization: Always administer iron supplements with Vitamin C to enhance absorption.
- Preventing Discoloration: Use a straw when administering liquid iron to prevent staining of the teeth.
- Dietary Education: Encourage iron-rich foods such as leafy greens, liver, dry beans, nuts, and whole-grain bread.
- Milk Regulation: In toddlers, limit cow's milk intake to no more than 32 oz per day to prevent interference with iron absorption.
Sickle Cell Anemia
Sickle cell anemia is an autosomal recessive genetic disorder that primarily affects individuals of African, Indian, Mediterranean, and Middle Eastern descent. The disease is characterized by the production of abnormal hemoglobin that causes red blood cells to take on a crescent or "sickle" shape.
Pathophysiology and Crises
The sickle-shaped cells increase blood viscosity and obstruct blood flow, leading to tissue hypoxia (lack of oxygen) and ischemia (tissue damage), which results in intense pain. Patients may experience several types of "sickle cell crises":
- Vaso-occlusive Crisis: Caused by acute ischemia, this leads to severe pain, swollen joints, hematuria, obstructive jaundice, and visual disturbances.
- Sequestration Crisis: This occurs when blood pools excessively in the spleen or liver, reducing circulating blood volume and potentially leading to shock.
- Aplastic Crisis: An extreme form of anemia triggered by a viral infection that decreases the production of red blood cells.
Management and Monitoring
Treatment aims to reduce oxygen consumption and maintain stability:
- Hydration: Maintain strict fluid and electrolyte balance.
- Infection Prevention: Provide prophylactic oral penicillin to prevent infections.
- Oxygenation: Administer oxygen and blood products as needed.
- Stroke Risk Assessment: An annual Transcranial Doppler is performed to monitor vascular blood flow and assess the risk of a cerebrovascular accident (CVA/stroke).
Hemophilia
Hemophilia is a hereditary, sex-linked recessive clotting disorder caused by a deficiency in one of the body's clotting factors.
- Hemophilia A: Deficiency in Factor VIII (the most common form).
- Hemophilia B: Deficiency in Factor IX.
Clinical Manifestations:
The hallmark symptom is hemarthrosis (bleeding into the joints). Other signs include easy bruising, hematuria (blood in urine), and abnormal bleeding from the umbilical cord or circumcision sites.
Treatment Precautions:
- Factor Replacement: Administer the missing clotting factor.
- Medication Safety: Avoid NSAIDs (like ibuprofen) and salicylates (like aspirin), as these can interfere with platelet function and increase bleeding risk.
- Injury Prevention: Delay invasive procedures like heel sticks or IM injections when possible.
Pediatric Malignancies
Childhood cancers involving the blood and lymph systems require rapid diagnosis and intensive management.
Acute Lymphocytic Leukemia (ALL)
ALL is the most common form of childhood cancer, typically affecting children between ages 2 and 5, particularly Caucasian males and those with Down syndrome. It is a malignancy of the bone marrow that causes an overproduction of immature white blood cells (WBCs) and an underproduction of red blood cells (RBCs) and platelets.
- Symptoms: Low-grade unresolving fever, pallor, bruising, bone pain, weakness, and abdominal pain due to enlarged lymph nodes.
- Diagnosis: Confirmed via bone marrow aspiration (typically from the iliac crest), blood tests, and potentially a spinal tap to check for CNS involvement.
- Nursing Care: Focus on reducing infection risks, maintaining hydration, and providing oral hygiene.
Hodgkin Disease
Hodgkin disease is a malignancy of the lymph system that most commonly affects adolescents (ages 15–19).
- Symptoms: The presence of painless, enlarged lymph nodes is a primary sign, often accompanied by night sweats, weight loss, low-grade fever, itching, and a non-productive cough.
- Diagnosis: Confirmed through a lymph node biopsy.
- Treatment: Typically involves chemotherapy and radiation therapy.
Study Tip: When distinguishing between ALL and Hodgkin disease, remember that ALL is a bone marrow malignancy characterized by immature WBC overproduction, while Hodgkin is a lymph system malignancy characterized by painless lymph node enlargement.
