Things To Know About Sickle Cell Anemia

Sickle cell anemia is a rare disease, found in fewer than 1 million cases per year in India.

It is usually diagnosed at birth or in some cases symptoms may appear after 4 months of age.

Normally red blood cells are round and flexible, which can move easily from blood vessels. But in the case of Sickle cell anemia, red blood cells change their shape into the crescent moon or sickle, also they are rigid and sticky and unable to move from blood vessels and block the path of blood flow and oxygen. Which results in low oxygen and blood supply to the different parts of the body.

Symptoms

1. Fatigue – due to less supply of oxygen to the body, you may experience fatigue, lethargy.

2. Pain – You can experience pain where sickle cells block the path in a blood vessel, that may be chest, abdomen, joints, or bones.

In some adults, chronic pain leads to joint damage.

3. Swelling – Swelling on hands and legs due to blockage in blood vessels is more common.

4. Fever – Sickle cells can damage the liver, spleen, and many organs. Due to that chances of infection are high. 

5. Delayed growth – Decrease in red blood cells provides fewer nutrients to the body, which can lead to slow growth.

Causes

Sickle cell anemia is an inherited disease, caused by the mutation of genes. 

If the mother and father pass defective genes to the child, it leads to affection for the child.

If only one parent passes the sickle cell gene to the child, that child can have sickle cell trait. 

People with sickle cell traits make normal and sickle cell hemoglobin. And might not show any symptoms of sickle cell anemia.

Risk factor

If both parents carry sickle cell genes, the risk for the baby is much higher to get sickle cell anemia.

Diagnosis

Blood test – To rule out defective forms of hemoglobin. Electrophoresis is the main investigation to confirm the diagnosis of Sickle cell anemia.

Amniotic fluid – In unborn babies, it can be diagnosed by taking a sample of amniotic fluid from the mother’s womb.

Complications

Pulmonary hypertension – this is a more common complication seen in adults. Sickle cells affect the lungs and symptomized of fatigue and shortness of breath.

Blindness – Sickle cells can block the blood vessels of the eyes, which leads to blurring vision and gradually turns into complete loss of vision.

Stroke – This is the most common complication of sickle cell anemia. Sickle cells block the vessels which supply blood to the brain and due to less supply of blood and oxygen, a person can get a stroke or seizure.

Gallstone – Breakdown in the red blood cells produces bilirubin. An excessive amount of bilirubin in the body creates gall stone.

Pregnancy – In pregnant women, it can lead to high blood pressure and can cause miss abortion or premature delivery.

Priapism –  Sickle cell can block the blood vessels in the penis and men with sickle cell anemia can get a painful long erection. Eventually leads to impotency.

Treatment –

Hydroxyurea is a drug of choice. Which helps to reduce flares of pain. 

Voxelotor improves sickle cell anemia. Voxelotor recently approved by the FDA.

Morphine, Fentanyl use to reduce pain. 

Penicillin use as a high-spectrum antibiotic between the age of 2 months to 5 years of age in patients. An adult treated with Penicillin throughout their life.

Blood transfusion is initiated in most cases. 

Bone marrow transplantation is only the cure for sickle cell anemia. Which is only a transplant by an identical family member.

Prevention

There is no prevention for sickle cell anemia, But you can avoid complication with the following measures –

• Make a habit to take a healthy diet and include lots of yellow-orange fruits and green vegetables.
• Take Folic acid and vitamin supplements after consulting your physician.
• Drink 8-10 glasses of water daily.
• Walk or exercise daily. Don’t overexert yourself.
• Avoid extremely hot or cold exposure.
• Avoid smoking.
• Children with sickle cell anemia can complete a normal vaccination schedule. 
• After 6 months of age, children can take Flu vaccine once a year
• Children can additionally take the Pneumococcal vaccine at the age of 2 and 5 years. To avoid pneumonia.

Conclusion

The life span of red blood cells is normally 120 days, and in sickle cell anemia patients, it is only 10 to 20 days. 

They require a moderate amount of folic acid after consulting with a physician.

Keep yourself protected from infection.

Do a complete health check-up. Consult with your doctor before planning pregnancy, if there is any history of sickle cell anemia in the family.

If your child develops a persistent fever, frequent abdominal, chest, or joint pain, pale skin, yellow sclera, weakness in arms, legs, or difficulty in walking please consult a physician for early diagnosis.