Sickle Cell Anemia

Sickle cell anemia:  A congenital hemolytic anemia that occurs primarily but not exclusively in black people, sickle cell anemia results from a defective hemoglobin molecule ( hemoglobin S ) that causes RBC's ( red blood count ) to roughen and become sickle shape. These cells impair circulation, resulting in chronic ill health, periodic crises, long term complications, and premature death.

This inherited disease involves an abnormality of hemoglobin, the component of red blood cells that carries oxygen.  The normal hemoglobin molecule is of the A type.  In sickle cell disease much of the hemoglobin is of the type called S type, giving it it's shape.  Unlike normal, flexible red cells, the sickled cells are stiff; groups of them may become entangled in such a way as to obstruct smaller blood vessels.  When obstruction occurs there is severe joint swelling and pain, more common in the fingers, and toes, a development often referred to as sickle cell crisis.  There may also be paralysis and convulsions resulting from cerebral thrombosis.

Symptoms: 

Joint swelling
Pain
Jaundice
Pallor
Chest pains
Aching bones
Ischemic leg ulcer ( especially around the ankles )
Increase susceptibility to infection
Paralysis
Convulsions
Tachycardia ( rapid heart rate )
Cardiomegaly ( enlarge heart )
Systolic and diastolic murmurs
Chronic fatigue
Unexplained dyspnea ( difficulty breathin/labored )
Hepatomegaly ( enlarge liver )
Splenomegaly ( enlarge spleen )
Patient with painful vaso - occlusive crisis may manifest severe abdominal, thoracic, muscular, or bone pain; possible increased jaundice and dark urine; low grade fever; and, in long term disease, spleen shrinkage.

Treatment:

Therapy for this anemia is primarily symptomatic and can usually be done at home.  Hemoglobin level should be check frequently, if hemoglobin drops suddenly or if the patient condition deteriorates rapidly, he must be hospitalized for possible transfusion of packed RBCs.  In a sequestration crisis, treatment may include sedation and administration of analgesics, blood transfusion, oxygen administration, and administration of large amounts of oral or I.V. fluids

 * Watch for signs of sickle cell crises:  includes; pale lips, tongue, palms, or nail beds; lethargy; listlessness; sleepiness, with difficulty awakening; irritability; severe pain temperature over 104 degree C or fever of 100 degree that persist for 2 days.
** CALL YOUR DOCTOR PROMPTLY if you experience the above symptoms.

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