SICKLE CELL DISEASE

John Marie Christian C. Protacio

SICKLE CELL DISEASE
• Causes severe anemia
• Results from sickle hemoglobin (HbS) gene
• Low oxygen level in venous blood
• Erythrocyte loses its round, pliable, biconcave disc shape
• Cell becomes rigid, dehydrated and sickle shaped
• Can be reverted if exposed to adequate amount of oxygen
CLINICAL MANIFESTATION
• Results from chronic hemolysis or thrombosis
• Anemia (5 – 11g/dL)
• Jaundice (sclera)
• Enlargement of bones of the face and skull
• Tachycardic, murmurs and enlarged heart
 COMPARISON OF SODIUM AND POTASSIUM
LEVELS IN PLASMA
mEq/L Sodium (135-145) Potassium (3.5-5.5)
HBS stable 136.22 +/- 3.19 3.56 +/- 0.42
HBS crisis 135.17 +/- 2.77 3.28 +/- 0,35
HBS rehydration 137.57 +/- 2.15 4.42 +/- 0.15

African Journal of Biochemistry Research Vol.3 (11), pp. 370-374, November, 2009
SICKLE CELL CRISIS
• Three Types:
– Acute vaso-occlusive crisis
• Entrapment of erythrocytes and leukocytes in the microcirculation
– Tissue hypoxia, inflammation and necrosis
– Endothelium becomes dysfunctional and vasculopathy develops
– Aplastic Crisis
• Infection with human parvovirus
– Hgb falls, marrow is unable to compensate
– Sequestration crisis
• Organs pool the sickle cell
– Kidneys = child (splenic infarction)
– Liver = adults
– Lungs = WARNING
ACUTE CHEST SYNDROME
• Manifested by fever, respiratory distress
• Infiltrates on chest X-ray
• Causes
– Chlamydia and mycoplasma pneumoniae
– Respiratory syncytial virus & parvovirus
– Pulmonary embolism

• Medical Management
– RBC transfusion
– Antimicrobial therapy
– Bronchodilators
PULMONARY HYPERTENSION
• Common sequence of SCD
• Px complaints
– Fatigue, dyspnea on exertion, dizziness, chest pain, syncope

• Pulse Oximetry and breath sounds = normal until latter

• Screening
– Doppler echocardiography
– High level of brain natriuretic peptide
– CT scan of the chest
STROKE
• Hemorrhagic stroke
• Individuals with SCD at risk for overt stroke
• Management
– RBC transfusion
• Reduce HbS to less than 30%
– Prevents cerebral edema
ASSESSMENT AND DIAGNOSTIC FINDINGS
• Px with sickle cell trait
– Normal Hgb level, Hct level and blood smear

• Patient with Sickle cell disease

– Low Hct, present on blood smear
– Increased WBC and platelet count

• Hemoglobin electrophoresis
– Confirms diagnosis
PROGNOSIS
• Usually diagnosed during childhood (1-2yrs of age)
• Young adults live with multiple complications
• For Some, symptoms may diminish at 30yrs
• Death is common
– Heart disease (32%)
– Lung disease (28%)
– GI or liver disease (9%)
– Cancer (less than 1%)
MEDICAL MANAGEMENT
• Hematopoietic Stem Cell Transplant
– Only available to small affected patients
• Incompatible or severe organ damage
PHARMACOLOGIC THERAPY
• Hydroxyurea (for pregnant women)
– Chemotherapy drug
– Increase Fetal Hgb level with px with SCD
– Side effects
• Chronic suppression of leukocyte formation
• Teratogenesis

• Folic Acid
– Increase hematopoiesis

• Antibiotic
– Specific for determined infection

• Analgesics (aspirin, NSAIDS)

– Pain management
TRANSFUSION THERAPY
• Diminishes episodes of sickle cell crisis
• Complication
– Poor venous access
– Delayed hemolytic transfusion reaction
– Infections
– Iron overload
– Increase blood viscosity
– Alloimmunization
ASSESSMENT
• Determine characteristic and location of pain, swelling, fever
• Determine impact of fatigue in ADL’s
• Laboratory Findings (Urine, CBC, X-rays, CT scan)
• Physical assessment
– Abdomen – splenic infarction
– Cardiopulmonary
• Auscultate breath sounds
• Measure O2 Saturation
• Edema
• Cardiomegaly (Xray)
– Signs of dehydration
• Monitor I/O
• Skin turgor
• Serum creatinine and blood urea
DIAGNOSIS
• Acute pain and fatigue r/t tissue hypoxia due to agglutination of
sickled cells within blood vessels
• Risk for infection
• Risk for powerlessness r/t illness-induced helplessness
• Deficient knowledge regarding sicke crisis prevention
COMPLICATIONS
• Hypoxia, ischemia, infection
• Dehydration
• Cerebrovascular disease
• Anemia
• Acute/chronic kidney failure
• Heart failure, pulmonary hypertension
• Impaired fertility
• Cognitive dysfunction
• Pain
NURSING INTERVENTION
• Pain
– Support and elevate if swelling
– Breathing exercise, relaxation techniques
– Physical therapy
– Use of analgesics as prescribed

• Fatigue
– Exercise and rest balance
– Maximize nutrition, hydration, sleep cycle

• Infection
– Determine Signs and Symptoms
– Administer medication as ordered
– Health education for antibiotic therapy