PEDS 602 Hematologic disorders - Chamberlain College of Nursing | PEDS602 Hematologic disorders

Document Content and Description Below

PEDS 602 Hematologic disorders - Chamberlain College of Nursing PEDS602 Hematologic disorders Hematologic DisordersHematologic DisordersAnemia • Hemoglobin content of the blood is insufficient... to satisfy bodily needs for oxygen • Causes: • Blood loss – GI or menstrual periods • Accelerated hemolysis • Decreased production • Typical Clinical Manifestations: • Fatigue • Dizziness • Weakness • Pallor • ↑ heart rate • ↑ breathing rateClassifications of Anemia: Nutritional Anemias: Iron-deficiency-most common in children Vitamin B12 Folic acid Bone Marrow Depression Aplastic Anemia- less RBC, WBC, PLATELETS. Can be caused by radiation, drugs, Hemolytic Anemias Sickle cell Thalassemia Acquired hemolyticIron Deficiency Anemia Inadequate supply of dietary iron so new RBCs are smaller with less Hgb Due to nutrition, malabsorption, blood loss, and PICA • Generally is preventable • Iron-fortified infant cereals/formulas – start at 6 mos when maternal supply no longer sufficient • Special needs of premature infants – maternal stores only last 2-3 mos • Adolescents at risk due to rapid growth, poor eating habits & heavy periodsIron Deficiency Anemia • Clinical Symptoms: depends on severity • Brittle, ridged, spoon-shaped nails • Irritability • Poor muscle tone • Growth retardation • Headache • Pica (especially ice, dirt or pure starch) • Prolonged anemia: • Nailbed deformities • Tachycardia • Growth and developmental delaysIron Deficiency Anemia (cont.) Therapeutic Management: • High iron foods – beef, liver, green leafy vegetables, bread, eggs, some cereals • Iron supplements on empty stomach or with Vitamin C • Limit milk<16 oz after 1 year of age because it limits food intake • Supplements can stain teeth, use a straw • Supplements can cause constipation and will color the stool black!Sickle Cell Anemia • Autosomal recessive condition where normal hemoglobin is partially or completely replaced by the sickle-shaped, ab- - - - - - - - - - -- - - - - - - - - - - - - - - - - - - -ene therapy • http://www.youtube.com/watch?v=ujf72mjy0BgClinical Manifestations Anemia Thrombocytopenia (Plt) & Leukopenia (WBC) Pain- obstructed blood flow Hand-foot syndrome (often 1st sign in babies)– swollen, blood congestion, pain/fever Jaundice – liver overwhelmed by RBC breakdown – Spleen picks up RBC fragments Frequent infections –spleen cannot filter infections– Stroke - confusion Stunted growth- lack of O2 for growth Vision problems – damage to eye vessels Chest syndrome – pulmonary hypertension PriapismTherapeutic Management: • Rest - Decrease energy expenditure • O2 use – hypoxia leads to more sickling due to metabolic acidosis • Hydration!! Oral, IV, hydrate • Analgesics – often PCA pump • Transfuse - treat anemia • “aplastic crisis” – bone marrow clogged with sickled RBC stops producing RBC, WBC, plt – transfusions necessary • Splenectomy – sequestration = rupture • Antibiotics – treat infection quickly & prophylactic • Preventative vaccinations: pneumococcal, meningococcal and influenzaComplications Frequent transfusion leads to hemosiderosis (too much iron in tissues) • Treat with iron-chelation such as (Desferal: deferoxamine) + vitamin C to promote iron excretion Antibodies – increased risk of anaphylaxis – harder to match Slide 14Psychosocial impact • Coping mechanisms • Support with genetic counseling • Financial needs • Caregiver role strain • Living with chronic illness in the familyAplastic Anemia-will be bleeding a lot! • Bone marrow does not produce an appropriate number of circulating blood cells – congenital or acquired. • Causes – radiation, drugs, insecticide, sulfa drugs, mono, hepatitis or idiopathic • Clinical Manifestations: • Less platelets – no clots, bleed, ITP • Less RBC – tachycardia, pale, CHF, tired • Less WBC – immunosuppressed • May need stem cell/ bone marrow transplant!Hemophilia • Hereditary clotting factor disorder with prolonged coagulation times • Types: • Hemophilia A (classic) Factor VIII – 80% • Hemophilia B (Christmas disease)- Factor IX – 20% - less severe • Who’s at Risk? X linked recessive transmitted by female carriers – seen mostly in males Form platelet plugs at site of bleeding but cannot make the thromboplastin or fibrin necessary for coagulation – no clot stabilityHemophilia Clinical Manifestations: First may be circumcision, tooth eruption, nosebleed, or a fall as toddler Prolonged bleeding – increased PT, PTT, INR Hemarthroses Excessive bruising Blood in urine or stool Treatment: Prevent bleeding Beware of chest, brain, abdomen, large muscle bleeds Support family and prepare for home care Tylenol or Morphine – for joint pain careful of NSAIDS (inhibit platelets!) Corticosteroids – for joint swelling Factor replacement – administer in morning due to short half life – most effective when child most active. Can still make antibodies to the factors.Hemophilia • Bleeding is prolonged…not faster • Clotting is prolonged…does exist - - - - - - - - - - - - - - - - - - - - - - - - - r • Use blood within 30 minutes of arrival • Infuse over 4 hours maximum • In first 15 min, transfuse slowly, check VS, and stay with patient at all times • Watch closely for transfusion reactionTransfusion Reactions • Hemolytic: the most severe, but rare – starts destroying all RBC • Febrile reactions: fever, chills • Allergic reactions: urticaria, pruritus, laryngeal edema • Hypothermia • Electrolyte disturbances: hyperkalemia from massive transfusions or with renal problems • Delayed reactions: destruction of RBC 5-10 days after tx, hepatitis, syphilis, HIV [Show More]

Last updated: 1 year ago

Preview 1 out of 23 pages