Nursing Care of a Child with a Hematological Disorder

Flashcards for reviewing hematological disorders in children, covering causes, symptoms, treatments, and nursing care.

What happens to blood components in hematologic disorders?

Components are formed incorrectly or increase/decrease beyond normal ranges.

What is the first stage of blood coagulation?

Platelets adhere to each other, forming a platelet plug.

What vitamin is essential for the third stage of blood coagulation?

Vitamin K

What is the insoluble, impermeable clot called that forms a protective seal?

Fibrin

What characterizes normochromic, normocytic anemia?

Normal RBC color and size but too few for adequate oxygen transport.

What are some causes of acute blood loss anemia?

Trauma leading to hemorrhage, internal bleeding, acute nephritis, placenta previa, or inadequate nutrition.

What lab findings are expected in a patient with acute blood loss anemia?

Normal MCV and MCH (if due to hemorrhage), potential changes if underlying deficiencies exist.

What are the interventions for acute blood loss?

Control bleeding, transfusions, keep child flat and warm, IV fluids, oxygen.

What is the primary treatment focus for anemia of acute infection?

Treating the underlying infection.

What is characteristic appearance of RBCs in hypochromic anemias?

Pale and reduced in diameter.

What is a preventative measure for iron deficiency anemia in infants?

Breastfeeding or iron-fortified formula.

What are potential causes of iron deficiency anemia?

Inadequate dietary iron, GI lesions, ulcerative colitis, Crohn's, parasitic infections, menstruation, overweight.

What physical signs are seen in iron deficiency anemia?

Pale mucous membranes, enlarged heart/spleen, poor muscle tone, spoon-shaped nails, pica.

What lab tests are relevant for iron deficiency anemia?

Decreased hemoglobin and hematocrit, low iron levels.

What is included in the treatment plan of Iron Deficiency Anemia?

Iron supplements, dietary changes (increase iron), ferrous sulfate, vitamin C (to aid absorption).

What are the treatments for chronic infection anemias like Sickle Cell Anemia?

Pain relief, hydration, oxygenation, possible transfusion or stem cell transplant, hydroxyurea.

What characterizes hemolytic anemias?

Increased erythrocyte destruction causing low erythrocyte count.

Describe the pathophysiology of sickle cell anemia.

Autosomal recessive disorder where RBCs become elongated and crescent-shaped, obstructing blood flow.

What precipitates a sickle cell crisis?

Dehydration, vomiting/diarrhea, respiratory infection, impaired oxygen exchange.

List S/S of sickle cell crisis

Fever, anemia, swelling of hands and feet, thin build with long limbs, protruding abdomen, tachypnea, wheezing/cough

What interventions are important during a sickle cell crisis?

Pain relief (acetaminophen/narcotics), hydration (IV fluids), oxygenation, vasodilatation (heating pads).

What is the primary issue in Idiopathic Thrombocytopenia Purpura (ITP)?

Decreased number of circulating platelets.

What causes ITP?

Immune response leading to platelet destruction, often post-viral infection.

What are the signs and symptoms of ITP?

Petechiae, large ecchymotic areas (especially on legs), epistaxis, joint bleeding/pain.

What is the treatment plan for ITP?

Oral prednisone, IVIG, anti-D immunoglobulin, platelet transfusion, acetaminophen (avoid NSAIDs/aspirin), immunosuppressants, splenectomy.

What lab results are expected in a patient with ITP?

Low platelet count

What is the cause of Henoch-Schonlein Purpura?

Increased vessel permeability due to hypersensitivity reaction to an allergen.

What are the primary signs and symptoms of Henoch-Schonlein Purpura?

Rash on buttocks/legs/arms/ears, tender/swollen joints, abdominal pain, vomiting, blood in stool.

What are signs of abnormal urine for a patient with Henoch-Schonlein Purpura?

Urine for protein and glucose.

How is Henoch-Schonlein Purpura treated?

Oral corticosteroids (prednisone), mild analgesics.

What is a potential complication of Henoch-Schonlein Purpura?

Chronic nephritis (kidney inflammation).

What is the underlying issue in hemophilia?

Inherited deficiency in blood coagulation factors (e.g., factor VIII in Hemophilia A).

What is lacking in Hemophilia A?

Deficiency of factor VIII

How is Hemophilia A inherited?

Inherited.

What are common assessment findings in hemophilia?

History of bleeding, nosebleeds, bruising, hemarthrosis (joint bleeding).

What are the S/S of Hemophilia?

Excessive bleeding post-circumcision, easy/heavy bruising, soft tissue/joint hemorrhage, loss of joint mobility, GI bleeds.

What lab results are expected for Hemophilia A?

Prolonged PTT, low factor VIII levels.

What is the treatment approach for hemophilia A?

Factor VIII administration, DDAVP (synthetic vasopressin), corticosteroids (for hematuria/hemarthrosis), NSAIDs (with caution), avoid aspirin, supportive care/PT, blood transfusions.

What are important education points for hemophilia patients and their families?

Prevent injuries, use helmets, activity restrictions, avoid contact sports, use soft toothbrushes, venipuncture instead of finger sticks, medical alert tag.

What is the deficient factor in Hemophilia B (Christmas Disease)?

Factor IX

What characterizes Thalassemia?

Autosomal recessive anemia with abnormalities in the beta chain of adult hemoglobin.

What are the signs and symptoms of Thalassemia Minor?

Pallor, hypochromic/microcytic blood cells.

What might labs reveal about a patient with Thalassemia Minor?

Normal RBC count, decreased hemoglobin.

What is the distinctive feature of Thalassemia Major (Cooley anemia)?

Inability to make beta hemoglobin.

What are the S/S of Thalassemia Major?

Pallor, irritability, anorexia (typically after 9 months of life).

What are the effects of Thalassemia Major on the body organs?

Widening of bones, bronze skin, splenomegaly, cirrhosis/cholelithiasis, diabetes, heart failure, high iron levels.

How is Thalassemia Major treated?

Stem cell transplant; while waiting: digitalis, diuretics, low sodium diet, packed RBC transfusions every 2-4 weeks.

What factors should be included when assessing children with Hematological Disorders?

History/PMH (low birth weight, blood loss at birth, lack of vitamin K, family history), complaints of fatigue, easy bruising, epistaxis, picky eating/PICA, increased milk intake.

Name some Tx related to assessing Hematological DOs

bone marrow aspiration, biopsy, blood transfusion, hematopoietic stem cell transplantation, splenectomy, corticosteroids

What are common treatments or interventions for hematological disorders?

Bone marrow aspiration/biopsy, blood transfusion, hematopoietic stem cell transplant, splenectomy, corticosteroids.

What are some important considerations when providing recreational activities for children with hematological disorders?

Include other children for socialization, focus on fun alternatives, build self-esteem/body image.

List the six competencies of Quality & Safety Education for Nurses (QSEN)

Patient centered care, teamwork and collaboration, evidenced-based practice, quality improvement, safety, informatics.