The Child with a Condition of the Blood, Blood-forming Organs, or Lymphatic System

Hematological System in Children

• The hematological system includes blood and blood-forming organs. Blood is essential for all bodily functions. Blood dyscrasias (disorders) occur when blood components don't form correctly or are outside normal ranges.
• Blood cells form mostly in the yolk sac in the early stages of gestation. Later, the spleen, liver, thymus, lymph system, and bone marrow contribute to blood formation.
• Fetal blood formation mainly occurs in the liver until the third trimester. During childhood, red blood cells (RBCs) are produced in the marrow of long bones (e.g., femur, tibia). By adolescence, hematopoiesis (blood cell formation) takes place in various bone marrows (ribs, sternum, vertebrae, pelvis, skull, clavicle, scapulae).
• RBC production is controlled by erythropoietin.
• The blood components are plasma and formed elements (erythrocytes, leukocytes, thrombocytes).

Blood Component Differences in Infants

• Newborns have high hemoglobin and RBC counts due to high erythropoietin levels, placental blood shift, and lower extracellular fluid volume.
• White blood cell (WBC) counts are high at birth, decreasing to a stable level within a week.
• Low vitamin K levels in newborns require immediate administration after birth. Vitamin K is crucial for blood clotting factor development.

Lymphatic System in Children

• The lymphatic system (lymphocytes, lymphatic vessels, lymph nodes, spleen, tonsils, adenoids, thymus gland) drains the body to lymph nodes. Here, infection-fighting organisms are destroyed, and antibody production is stimulated.
• Tonsils and adenoids are larger in preschool and school-aged children, acting as part of the body's defense.
• The thymus gland plays a vital role in newborn immune system development. Premature and term infants are at increased risk for infections due to immature T-cell activity.

Anemias

• Anemia results from various causes, reducing circulating hemoglobin and oxygen-carrying capacity. Hemoglobin levels below 8 g/dL increase cardiac output and blood flow to vital organs.
• Anemia's general manifestations include pallor, weakness, tachycardia, shortness of breath, and possible congestive heart failure.

Iron Deficiency Anemia

• The most common nutritional deficiency in U.S. children, particularly during infancy and adolescence due to rapid growth.
• Causes include severe hemorrhages, poor iron absorption, excessive growth needs, and inadequate diet (e.g., cow's milk).
• Manifestations include pallor, irritability, anorexia, decreased activity, and even growth retardation and cognitive changes in long-standing cases.
• Treatment aims at providing iron, typically ferrous sulfate orally between meals; Vitamin C aids absorption. Liquid preparations are often given via straw.
• Parent education regarding breastfeeding (at least 6 months, recommended 12 months), iron-fortified formula, and iron-rich solid foods is crucial.
• Stools may be tarry or green. Iron shouldn’t be given with milk.
• Monitor treatment response with follow-up visits.

Sickle Cell Anemia

• Inherited hemoglobin defect results in abnormal hemoglobin S (sickling).
• Predominantly affects African Americans.
• Sickling (clumping) is triggered by decreased oxygen, dehydration, infection, stress, and cold exposure.
• Sickle cells obstruct capillaries, causing clots and infarcts (tissue damage) in various organs.
• Acute pain often results in affected areas.
• Sickle cell trait (AS) does not cause disease; genetic counseling is important.

Sickle Cell Crisis Types

• Vasoocclusive: Most common, blood flow obstruction and vasospasm. Symptoms include dactylitis (painful hands and feet), joint pain, abdominal pain, strokes, etc.

• Splenic Sequestration: Blood pools in the spleen, causing abdominal pain, circulatory collapse, and potentially sudden death.

• Aplastic: Bone marrow stops producing RBCs, often triggered by infections, resulting in severe anemia

• Hyperhemolytic: Accelerated RBC breakdown superimposed on existing anemia, rare but severe.

Sickle Cell Diagnostic Evaluation and Treatment

• Diagnosis can occur prenatally (chorionic villus sampling, amniocentesis).
• Screening tests (sickledex), hemoglobin electrophoresis.
• Treatment is supportive and symptomatic. Bedrest, analgesics (IV morphine for severe pain, PCA for older children).
• Frequent blood transfusions.
• Oral hydroxyurea therapy increases fetal hemoglobin type to reduce sickling and pain. Maintaining hydration, monitoring sodium levels and minimizing risk of thrombosis are important.

Thalassemia

• Hereditary blood disorders causing insufficient adult hemoglobin production.
• RBCs are abnormal, leading to rapid destruction.
• Thalassemia major (Cooley's anemia) results in severe anemia, bone marrow expansion, enlarged spleen and liver, and potentially cardiac failure and death. Thalassemia minor has less severe symptoms.
• Treatment mainly involves frequent blood transfusions, chelation therapy (deferasirox, deferiprone), and potential stem cell transplantation to manage iron overload.

Bleeding Disorders

Hemophilia

• Hereditary inability to clot blood due to factor VIII (Hemophilia A) or factor IX (Hemophilia B) deficiency.
• Common in males, transmitted by females.
• Prolonged bleeding, hemarthrosis (bleeding into joints).
• Treatment mainly involves replacement of missing factors (recombinant antihemophilic factor) or using desmopressin (DDAVP) for less severe cases.
• Avoid aspirin and NSAIDs.
• Educate parents/patients on preventing injuries, and on managing bleeding episodes.

Idiopathic Thrombocytopenic Purpura (ITP)

• Autoimmune disorder causing decreased platelets; unknown cause, likely autoimmune response to a virus.
• Manifestations include easy bruising, petechiae, purpura, bleeding from mucous membranes.
• Treatment involves avoiding drugs affecting platelets, limited activity during acute stages, and use of prednisone (first-line treatment).
• Close monitoring, especially for intracranial signs.

Henoch-Schönlein Purpura

• Autoimmune condition involving blood vessel inflammation; likely triggered by infections or allergies.
• Clinical presentations include palpable purpura, abdominal pain, gastrointestinal bleeding, and hematuria. Treatment is largely supportive.

Disorders of White Blood Cells

Leukemia

• Cancer of blood-forming tissues characterized by uncontrolled immature WBC production; most common childhood cancer.
• Manifestations include fever, pallor, bleeding tendencies, leg/joint pain, listlessness, abdominal/lymph node enlargement, skin discoloration, petechiae, purpura, anorexia, vomiting, dyspnea, hematuria, anemia, thrombocytopenia.

Leukemia Diagnostic Evaluation and Treatment

• Diagnosed through blood tests, bone marrow biopsy, X-rays, and spinal tap (to check for central nervous system involvement).
• Treatment involves a multidisciplinary approach (often chemotherapy phases, possibly radiation).
• Close observation for infections, bleeding, and other chemotherapy side effects is critical.
• Bone marrow transplant might be an option

Hodgkin's Disease

• Lymphatic malignancy with specific Reed-Sternberg cells.
• Typically presents with painless lumps (often in the neck), low-grade fever, night sweats, and unexplained weight loss.
• Treatment primarily involves radiation and chemotherapy, based on the stage.
• Follow-up care addresses concerns from therapy (e.g., radiation sensitivity, fertility).

Nursing Care of the Chronically Ill Child

• Chronic illnesses significantly impact growth and development.

• Nursing interventions focus on ensuring emotional well-being, enabling self-determination, and preventing isolation.

• School-based programs and recreational activities maintain normality.

• Home care programs promote normalcy in routines, decision-making, and independence.

• Nursing priorities in end-of-life care involve supporting the child and family.

• Nurses must be empathetic and honest with the family and child with regard to the child's awareness and emotional responses.

• Open communication, honesty, fostering of control, and awareness of cognitive and developmental differences are critical.

• Siblings and other family members often face challenges and also deserve the nurse's attention.

Test your knowledge on blood formation, components, and disorders with this Hematology Quiz tailored for Biology Class 12. Explore topics like erythrocytes, iron deficiency anemia, and the effects of vitamin K in newborns. Perfect for reviewing crucial concepts related to blood health and diseases.