Sickle Cell Anemia

•Factors causing a sickle cell/ vaso-occlusive crisis

-Being overly tired or fatigued -Dehydration -Exposure to cold -Excessive physical exertion -Infection -Emotional or mental stress -Smoking

Prognosis for Sickle Cell Disease

-Median age of death is 42 years old for men and 48 years old for women -Mortality in African American children (1 to 14 years old) has decreased 53% to 41% from 1968 to 1992 -Patients that survive passed 50 years old usually develop progressive renal damage

3 Sickle Cell Syndromes

-Sickle cell anemia (HbSS) •Most common variant •Inherits a substitution of valinefor normal glutamicacid in the 6thamino acid position of the beta globinchain of hemoglobin from both parents •Alters hemoglobin molecule so it crystalizedand deforms into the sickle shape when it loses O2 -Hemoglobin SC (HbSC) -Hemoglobin S beta thalassemia(HbSBeta-thal)

Describe the leading cause of death in sickle cell

Acute Chest Syndrome -Leading cause of death -New pulmonary infiltrate accompanied by at least one of the following: •Fever •Chest pain •Wheezing •Coughing •Tachypnea

Other complications of sickle cell

Other Complications: Anemia, febrile ilness, infection, CVA, acute splenic sequestrian crisis, pulmonary hypertention, aplastic crisis, cholellthiasis, dactylitis, priapism, retinopathy, chronirenal insufficiency, leg ulcers,

Pathophysiologic steps in sickle cell

Presence of acidosis, dehydration, extreme temperatures, fever, change in altitude, strenuous physical exertion, emotional stress stress released too soon and sickles in capillaries Potassium and water efflux Cell density increases Increase in hemoglobin S polymerization Chemical on sickle cells binds to blood vessel walls Cells cannot pass through capillaries Blocks blood flow in microcirculation and produces tissue injury Increases hypoxia More erythrocytes sickle

Interventions to improve pulmonary function

Spirometry Breathing Techniques Huff cough to clear sputum

Risk Factors for Sickle Cell

•AutosomalRecessive Disorder -Both parents are unaffected carriers •25% chance of having a child with Sickle Cell Disease (SCD) •25% chance of having a child who is unaffected •50% chance of having a child who is a carrier but is unaffected -One parent affected and one parent a carrier •50% chance of having a child with Sickle Cell Disease

Long Term goals for PT in sickle cell

•Bed mobility -Pt will perform all bed mobility tasks Independently to prevent skin breakdowns •Transfers -Pt will perform transfers Independently to aid in self-care. •Endurance -Pt will increase endurance to Fair+ to aid in gait •MMT -Pt will increase strength of both B LEs and UEs to 4+/5 to perform ADLs •Gait -Pt will ambulate > 200ft with least assistive device Independently to aid in community ambulation

Medical Management of Sickle Cell

•Bone Marrow transplantation/Stem Cell Transplant •Streptococcus pneumoniae vaccine •Prophylactic use of penicillin •RBC Transfusion •Hydroxyurea •Medication: Analgesics or corticosteroids •Being researched: Nitrous oxide and fetal hemoglobin

Describe the definitive test to diagnose sickle cell

•Definitive test is hemoglobin electrophoresis -HbSand HbCamino acid substitutions change the electrical charge of the protein -Migration patterns of the hemoglobin focuses on diagnostic patterns with each of the different variants

Describe Sickle Cell

•Group of inherited hemoglobin disorders characterized by -Chronic hemolytic anemia -Heightened susceptibility to infections -End-organ damage -Intermittent episodes of vascular occlusion causing both acute and chronic pain

HBSS PAIN CRISIS acronym

•H-hemolysis, Hand-Foot syndrome •B-bone marrow hyperplasia/infarction •S-stroke: thrombotic or hemorrhagic, subarachnoid bleeds •S-skin ulcers, primarily leg •P-pain episodes, priapism, psychosocial problems •A-anemia, aplasticcrisis, avascularnecrosis •I-Infections: CNS, pulmonary, GU, bone, joints •N-nocturia, urinary frequency from hyposthenuria •C-cholelithiasis, cardiomegaly, CHF, chest syndrome •R-retinopathy, renal failure, renal concentrating defects •I-infarction: bone, spleen, CNS, muscle, bowel, renal •S-sequestration crisis involving spleen or liver •I-increased fetal loss during pregnancy •S-sepsis

Clinical Manifestations of Sickle Cell

•Increased blood viscosity •Vascular obstruction •Deoxygenationof hemoglobin molecule •Increased percentage of HbSin RBC •Low pH •Increased temperature •Blood stasis •Reduced life span of RBC from 120 days to 10-30 days

Describe Vasoocclusive crisis

•Leading cause for hospitalization •Ischemic tissue damage caused by occlusion of microvascularbed by sickledblood cells •Extreme pain typically in long bones, joint, the back, the abdomen, and the chest •May last five to six days causing hospitalization •Treatment: nonsteroidalanti-inflammatory drugs (NSAIDs), opioids, and adjuvant medications

Describe Acute Chest Syndrome

•Leading cause of death •Combination of pneumonia and pulmonary infarction •Symptoms: fever, cough, chest pain, and difficulty breathing •Children: caused by infection •Adults: caused by pulmonary fat embolism •Can lead to chronic respiratory insufficiency •Treatment: Analgesics, hydration, oxygen supplementation, incentive spirometry, and antibiotics

Prevalence statistics for sickle cell

•Most common inherited hematologic disorder -250,000 babies are born worldwide each year -75,000-90,000 American affected -1 in 400 African American newborns in the United States -1 in 10-12 African Americans carry the sickle cell trait •Prevalent in South America, Cuba and Central America

Describe pain episodes in sickle cell

•Most common problem •Self limiting and reversible in extremities, back, chest, and abdomen •Range from mild transient attacks of 5 min to lasting days or weeks requiring hospitalization •Believed to be caused by inflammatory process to bone marrow necrosis, ischemic muscle, and ischemic bowel resulting from obstruction and sludgingof blood flow from sicklederythrocytes •Cumulative ischemic tissue damage and fibrosis can lead to chronic pain

Overall goals for PT in sickle cell

•Pain management •Increase Strength, Functional Mobility, Endurance, and Gait. Improve Pulmonary Function Improve Functional Capacity

Describe Sickle Cell Disease

•Sickle Cell Disease -Autosomalrecessive disorder -Abnormal hemoglobin present (hemoglobin S) •Causes red blood cells to change shape (biconcave crescent)during deoxygenation -Various Types: Hemoglobin S (HbS),Homozygous Hemoglobin S (HgbSS),Heterozygous Hemoglobin SC (HgbSC),Heterozygous hemoglobin beta-thalassemia(HgbSB-thal)

2 other diagnostic tests for sickle cell

•Sickledex -5 min solubility test -Detects presence of HbSin emergency setting -Little diagnostic value as it does not differentiate -False negatives are frequent in newborns and with severe anemia •Analysis of DNA from fetal cells (amniocentesis) -16thgestational week -Sickle, HbC, HbS, B-thalassemiagenes can be detected