sickle-cell anemia is a inherited blood disorder presenting in childhood characterized by red blood cells which become abnormal, rigid, sickle shape decreasing the cells' flexibility due to the mutations in the hemoglobulin gene causing decreased oxygen carrying capacity

 

Sickle Cell Anemia is a childhood disorder which remains lifelong requiring frequent blood transfusions

Symptoms:

• Anemia and associated symptoms like fatigue and weakness
• Episodes of pain in chest, abdomen, joints and bones
• Swollen hands and feet may be the first signs of sickle cell anemia in babies.
• Jaundice with yellowish discoloration of eyes
• Frequent infections
• Delayed growth and puberty
• Vision problems

Complications:

• Stroke and its signs include seizures, weakness or numbness of your arms and legs, sudden speech difficulties, and loss of consciousness
• Pulmonary hypertension people develop high blood pressure in their lungs (pulmonary hypertension). Shortness of breath and difficulty breathing are common symptoms of this condition, which can ultimately lead to heart failure.
• Organ damage that affects kidneys, liver and spleen
• Blindness
• Skin ulcers
• Gallstones
• Priapism painful erections

Diagnosis:

• Complete Blood Count and blood smear to detect low blood hemoglobulin and high Reticulocyte count
• Gel Electrophoresis to detect abnormal hemoglobulin
• High-performance liquid chromatography
• Genetic testing for gene mutations
• Ultrasound and CT Scan for detection of organ damage or infections

Treatment:

• Dietary cyanate and thiocyanate, from foods containing cyanide derivatives, has been used as a treatment for sickle- cell anemia
• Painful crises are treated symptomatically with analgesics, Opiods and NSAIDS
• Folic acid and penicillin for controlling infections
• Antibiotics, Simple blood transfusion and exchange transfusions
• Hydroxyurea
• Decreases the number and severity of attacks
• Bone marrow transplants