Post on 15-Dec-2015
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Major functions Production of cells Oxygenation Nutrient distribution to cells Immune protection Waste collection from cells heat
production
Common Diagnostic Assessments Family History Child’s health history Physical exam Medication use Dietary pattern Lab studies: CBC w/diff, PT, PTT, Hb, Hct,
iron studies, bone marrow aspiration/bx Prepare child & family for lab tests
Definition http://www.nhlbi.nih.gov/health/dci/
Diseases/Sca/SCA_WhatIs.html Sickle cell anemia results from the hereditary
presence of abnormal Hgb S in place of Hgb A. When Hgb S becomes deoxygenated (loses oxygen), it tends to form polymers that deform the red blood cells (RBCs) into a sickle shape. Sickled RBCs cannot pass freely through capillaries and cause blockage of small vessels. This can compromise the blood supply to various organs.
Incidence Hereditary hemoglobinopathy;
autosomal recessive; 1 African Americans
Hgb S trait is found in 8 to 10% of the American black population. Sickle Cell Anemia– homozygous form
(HbSS)—most prevalent in US In Western African native blacks, up to
40% of the population carry Hgb S trait.
Basic Defect A defect in the structure of Hemoglobin A—
substitution of 1 amino acid (valine) for another (glutamine) @ the 6th position of the beta polypeptide chain.
Hgb partially/completely replaced by Hgb S When triggered, Hgb S sickles & obstructs
cap. blood flow, s blood viscosity tissue ischemia
Results hemolysis (RBC destruction) & anemia;
Sickling reversible until repeated cycles leads to irreversible sickling
Diagnostic Tests Rarely seen before 4-6 mos. Sickledex: sickle turbidity test; finger or
heel stick; results in 3 min Sickledex does not distinguish between
sickle cell trait and sickle cell disease. Hemoglobin electrophoresis is required to distinguish between these 2 disorders.
Hgb electrophoresis to “fingerprint” protein Hx, PE, CBC CXR if symptoms
Diagnostic Tests (cont’d) Peripheral Blood Smear
The routine peripheral blood smear does not contain sickled red blood cells unless hypoxemia (inadequate levels of oxygen in the blood) or a precipitating factor (such as a red blood cell poison or drug) is present.
Diagnostic Tests (cont’d)
Electrophoresis Both sickle cell disease (homozygous for
hemoglobin S) and sickle cell trait (heterozygous for Hgb S) can be detected by this test.
This test separates different types of hemoglobin based on electrical charge. Hgb S can be differentiated from Hgb A and other types of Hgb.
Diagnostic Tests (cont’d)
Normal Results In hemoglobin electrophoresis, no Hgb S
should be present. Normal hemoglobins in an adult are mostly Hgb A with small amounts of Hgb A2 and Hgb F.
What do abnormal results mean? sickle cell trait sickle cell anemia
Pathophysiology Spleen—enlarged, if untreated,
becomes fibrous & ineffective susceptibility to infection.
Liver—failure & necrosis 2ndary to impaired circulation. Hepatomegaly and tenderness are common sx.
Kidney— perfusion 2ndary to congestion of glomerular capillaries & tubular arterioles hematuria, inability to concentrate urine, enuresis, & possible nephrotic syndrome.
Pathophysiology (cont’d)
Bones— Hyperplasia of bone marrow osteoporosis
weakened bones lordosis and kyphosis or other skeletal deformities
Chronic hypoxia possible osteomyelitis from salmonella
“Hand-foot Syndrome”—occurs in children 6 mos-2yrs. Caused by infarction of short-tubular bones.Sx: swelling & pain of soft tissue of hands & feet. Usually resolves in 2-3 wks.
Pathophysiology (cont’d)
CNS—primarily R/T ischemia MAJOR INFARCT—Stroke or CVA
paralysis MINOR INFARCT—HA, aphasia, weakness,
convulsions, visual changes 2ndary to retinopathy & retinal detachment.
Heart—Decompensation & failure 2ndary to chronic anemia CHF, tachycardia
Pathophysiology (cont’d)
Blood— Rate of destruction of RBC’s > rate
of RBC production RBC’s lifespan is to 10-15 days
(compared to 120 days for nml RBC) Hgb, Hct & RBC count Reticulocyte count, bilirubin &
uric acid
Pathphysiology (cont’d)
Other sx: Generalized weakness Anorexia Joint, back, and abdominal pain Fever Vomiting Possible leg ulcers Failure to thrive, rate of growth &
development
Sickle Cell Crisis Triggers
Acute illness Dehydration Strenous exercise Hypoxic conditions Trauma Respiratory illness
Results: acute pain, cell destruction, infarcts, organ damage
Types of Crises Vaso-occlusive: PAIN, swollen joints,
vomiting, fever, anorexia, dactylitis Acute sequestration: liver & spleen,
HR dyspnea, weakness, pallor, shock
Aplastic crisis: weakness, pallor, dyspnea, tachycardia, shock
Acute chest syndrome: chest pain, cough, fever, abdominal pain
Other complications Megaloblastic anemia Hyperhemolytic crisis Cerebrovascular injury (stroke) Overwhelming infections
TREATMENT Primary Goals:
1. Prevent Crisis (avoid sickling phenomenon)—through adequate oxygenation & hydration in every day life activities. Hydroxyurea may be used prophylactically in adolescents and older adults. Folic Acid 1mg daily is also recommended to restore folate stores needed for erythropoiesis.
2. Treat crisis as an emergency when it occurs—determine etiology quickly and treat appropriately
Nursing Management Adequate hydration (also any electrolyte
replacement if needed) Pain management Promote rest Treat any infections Blood transfusions if needed Monitor O2 – supplement if needed Oral hygiene Maintain nutrition Family support and education
NURSING CARE Pain Control—strive to prevent pain.
Opioids as morphine, oxycodone, hydromorphone & methadone are preferred. Give IV or PO for severe pain round-the-clock. PCA pumps are great! Avoid Demerol (Meperidine) - ↑seizure risk
Tylenol & Ibuprofen may be used after dx is made Use non-pharmacologic methods also Heat (e.g. K-pad)to affected joints may be
soothing by O2 with vasodilation.
NURSING CARE Hydration–
Accurate I & O essential IV/PO fluids up to 150ml/kg/day will promote
hemodilution Note color of urine—sometimes brown
Prevent tissue deoxygenation Avoid strenuous activity Avoid environments with low O2 concentration Avoid extremes in temp- esp. cold environment Avoid emotional stress Avoid known sources of infection
Child/Family Education How to manage & minimize crisis:
avoid dehydration, cold, stress, hypoxia, acute illness, etc.
Signs of infection Importance of immunizations Antibiotic administration if ordered Education & support re: long term
effects Genetic counseling
Thalassemia Major
Autosomal recessive Defective Hgb; RBCs fragile w/short
life span anemia, hemolysis Main Tx: blood transfusions Hemochromatosis – iron overload –
occurs from hemolysis of transfused and defective RBCs
Chelation tx done to eliminate excess Fe
Nursing Considerations Numerous blood transfusions Assess s/s of hemosiderosis Assess s/s infection Folic acid supplementation Iron chelation tx as ordered Family support Possible bone marrow transplant
Aplastic Anemia
Definition: Condition where all formed elements of the blood are depressed.
Pancytopenia or Triad: Anemia (↓ RBC's) Leukopenia (↓ WBC's) Thrombocytopenia (↓ platelets)
Etiology of A.A. Primary (congenital) Secondary (acquired) -- often
secondary to drugs e.g. chemotherapy or other meds that “shut down” bone marrow function
Diagnosis -- Bone marrow aspiration or biopsy
Therapeutic Management -- Goal: Restore bone marrow function Immunosuppressive therapy Bone marrow transplantation ** choice
85% survival rate Nursing Considerations -- Similar to
child with leukemia Prepare family for diagnostic &
therapeutic procedures Preventing complications from severe
pancytopenia Emotionally supporting them in terms
of a potentially fatal outcome
Hemophilia
Deficiency in specific clotting factor X-linked recessive trait; 2 most
common Hemophilia A (classic) Hemophilia B (Christmas Disease)
Uncontrolled bleeding from wounds into joints, muscles, & other body tissues
Spontaneous & severe uncontrolled bleeding in severe form
Common Labs Hx & PE, symptoms PTT prolonged Factor VIII, IX levels Normal: PT, thrombin, bleeding
time, fibrinogen, platelet count May do liver Bx to r/o other causes
Signs & symptoms Excessive bleeding from circumcision Bleeding w/teething Hemarthosis – cardinal sign Bruises in various stages Hematuria Nose bleeds Oral bleeding
Nursing Management
Control localized bleeding: Direct pressure for extended period of time like
10 minutes Ice Elevation of affected limb
Avoid injections when possible No rectal temps Monitor for bleeding (joints, urine, stool, etc) Provide pain relief Monitor neuro status if possible head bleeds
Nursing Management
Administration of Factor replacement as needed
Factor VIII concentrate (Antihemophilia factor {AHF} ), or DDAVP , a synthetic form of vasopressin that is the treatment of choice in mild hemophilia & von Willebrand disease. Should increase Factor VIII activity 3-4 fold.
Teaching Considerations Teach family factor administration/storage DDAVP administration Avoid ASA type products Avoid contact sports Medic alert tag Avoid constipation Good nutrition Good oral hygiene Genetic counseling Protective environment for infants/toddlers
Idiopathic Thrombocytopenia Purpura (ITP)
Excessive destruction of platelets Purpura (from petechiae under skin)
Normal bone marrow Most common bleeding disorder in
children 80% between 2-5 yrs old and usually last
~ 6m Usually after viral infection Symptoms when platelet ct < 20,000
Therapeutic Management
IF medical tx is indicated, then may use one of the following regimens Prednisone IVIG—expensive and takes long to
administer and monitor pt Anti-D antibody—controversial but may
prolong survival of platelets. Can be given in 1 dose over 5-10 minutes.
Nursing Considerations Stress activity restriction Prevention of head injuries; instruct
parents to get med eval if head or abd injury
Assess for bleeding/bruising Oral care support No rectal temps – No ASA products Splenectomy if has for 1 yr and tx not
successful Family support & teach on how to control
bleeds