Sickle Cell Anemia
Sickle Cell Anemia
This is included in a group of disorders related to other sickle cell disorders. This type of anemia occur when there occur deficiency of good, reliable and healthy red blood cells resulting in inability to deliver oxygen properly.
Signs and symptoms manifested in 1st decade of life. Clinical features include;
- Fatigue, pallor and weakness occur due to breakdown of rbcs and development of anemia.
- Episodic pain occur which occur in chest and abdomen and is severe in intensity. This is characteristic of sickle cell disease and is known as pain crises.
- Hands and feet become swollen.
- Increase risk of infections.
- Growth retardation and delayed puberty.
- Blurring of vision and other visual problems.
Sickle cell disease occur when there is sudden mutation in the gene which encodes the hemoglobin as a result of which blood cell go tendency to become sickle shaped when there are episodes of low oxygen tension in the body. When such Red Blood cells become deoxygenated there shape changes to sickle shape which make it difficult to pass through thin diameter capillaries especially of spleen sinusoids. There RBCs breakdown occur when deformed RBCs are stuck in the sinusoids leading to the development of anemia. It is the inherited disorders and can present as sickle cell trait only.
Following complications can result from this disease;
- Blood flow blocked to brain causes stroke
- Acute chest syndrome
- Visual disturbances
- Pulmonary hypertension
- Sores/ulcers on leg
- Major organ damage.
- Gallstones formation
- Priapism which refers to long, painful erection in men
- Still births or abortions and other pregnancy complications
Detailed history, family history and complete general physical examination is necessary for diagnosis. Other investigations include;
- Blood test to detect the condition of anemia
- Routine blood test on newborn babies.
- Ultrasound for stroke probability assessment
- Medications like hydroxyurea, L glutamine oral powder, Crizanlizumab (Adakveo), pain killers, voxelotor can be prescribed.
- Prevention of infections by properly taking vaccination and penicillin at young age can help to lower the complications.
- Blood transfusion and bone maroon transplantation are mainly considered.
- Properly take folic acid and iron supplements
- Stay hydrated.
- Avoid to get exposure of extreme temperatures
- Light exercise
- Limit smoking.
- Be careful when using over-the-counter medications.