Things You Must Know About Sickle Cell Disease

Sickle cell disease (SCD) is a class of red blood cell diseases that is inherited by offspring from their parents. This disease distresses the protein in red blood cells called hemoglobin. The role of hemoglobin is to deliver oxygen throughout the body, which is aberrant in sickle cell disease. This abnormal hemoglobin, called hemoglobin S, bunches together, beginning the red blood cells to turn sickle-shaped. These sickle-shaped cells are not flexible and can stick to the blood vessel wall, which causes obstructions in blood flow. Sickle cell disease can lead to anemia, pain, infections, and other severe complications.

Things You Must Know About Sickle Cell Disease

Sickle cell disease (SCD) is a class of red blood cell diseases that is inherited by offspring from their parents. This disease distresses the protein in red blood cells called hemoglobin. The role of hemoglobin is to deliver oxygen throughout the body, which is aberrant in sickle cell disease. This abnormal hemoglobin, called hemoglobin S, bunches together, beginning the red blood cells to turn sickle-shaped. These sickle-shaped cells are not flexible and can stick to the blood vessel wall, which causes obstructions in blood flow. Sickle cell disease can lead to anemia, pain, infections, and other severe complications.

The Problem with Sickled Cells

Sickle cell disease is a genetic condition that occurs when a child receives a sickle cell trait from each parent that codes for abnormal hemoglobin.

 

Healthy red blood cells are round, and they transfer through small blood vessels to carry oxygen to all parts of the body. However, red blood cells with sickle cell disease are under stress; they "sickle" or take on the appearance of a banana, which might impede their ability to pass through blood vessels. Contrary to red blood cells in healthy individuals that are spherical, pliable, and easily able to flow through blood vessels, certain red blood cells in sickle cell disease patients take on the appearance of a crescent moon and adhere to blood channel walls. As a result, blood flow via vessels is slowed or even stopped, which can cause pain, damage to organs and tissues, or even result in a stroke.

 

Also, sickle cells barely survive 10 to 20 days, compared to the normal lifespan of red blood cells which ranges from 90 to 120 days. Red blood cells are constantly being produced by the body to replace worn-out ones. The body may, however, find it difficult to keep up with the rate at which the aberrant cells are being destroyed in sickle cell disease. As a result, red blood cell counts are typically lower in individuals with sickle cell disease, causing anemia, known as sickle cell anemia.

 

Symptoms of Sickle Cell Disease

The signs and symptoms of sickle cell disease includes a wide range of symptoms that vary from person to person and change over time. Symptoms can be mild or severe enough to require frequent hospitalizations. They may include:

• Anemia (looking pale)
• Dark urine
• Yellow eyes
• Painful swelling of the hands and feet
• Abdominal swelling
• Frequent pain episodes, called sickle cell crisis, in the chest, abdomen, joints, or bones
• Frequent infections and fever
• Delayed growth
• Vision problems
• Signs or symptoms of stroke

 

Treatment of Sickle Cell Disease

The sickle cell illness cannot be cured with conventional therapies. Nonetheless, there are therapies that support patients in controlling and enduring their illness. Treatment reduces organ damage, eliminates pain, stops infections, and manages consequences. It may involve blood transfusions, prescription drugs, analgesics, and other measures as required.

 

If you think you might have sickle cell disease, you should consult your physician. Make sure you inform your doctor before becoming pregnant if you have sickle cell trait. Your doctor might recommend that you see a hematologist, or physician who specializes in blood disorders, based on the state of your illness.

 

Currently, a bone marrow transplant or stem cell transplant is the only cure for sickle cell disease. For a patient to have the best chance of a successful transplant, they need a donor who is well-matched. However, this procedure is not easy, as many patients do not have a donor who is a good genetic match or they are too old for a transplant. 

Researchers are also exploring genetic therapies. Genetic therapies aim to repair a faulty gene or add

a missing or new gene. These may lead to new treatments or help cure sickle cell disease.

If you have the sickle cell trait, then seeing a genetic counsellor prior to becoming pregnant can be beneficial. You can better understand your child's risk of sickle cell anemia by speaking with a counsellor. Additionally, you can gain knowledge about potential therapies, preventive measures, and safer ways to conceive.

 

Lifestyle Modifications and Self-Care of Sickle Cell Disease

The lifestyle modifications listed below are recommended to help manage this illness, that include:

• Daily folic acid supplements to produce new red blood cells.
• Consume a wide range of fresh, brightly colored fruits and vegetables, as well as whole grains.
• Sip a lot of water. A dehydration crisis could worsen a sickle cell disease. Try to have eight glasses of water a day and drink a lot of it throughout the day.
  
  
• Steer clear of extremely hot or cold temperatures since they may raise your chance of having a sickle cell crisis.
  
  
• Engage in regular exercise; discuss with your physician the appropriate amount of activity for you.
  
  
• Take caution when using over-the-counter drugs. Because of the detrimental effects on your kidneys, take pain relievers like ibuprofen or naproxen sodium only after consulting your doctor.
  
  
• Quit smoking as it raises your likelihood of experiencing pain crisis.

The term "sickle cell disease" describes a collection of illnesses brought on by hemoglobin gene abnormalities that can cause red blood cells to become deformed into a sickle shape. Sickle cell disease is a chronic illness with only a few treatment options like blood transfusions and stem cell transplants. Early detection and intervention might lessen your symptoms and risk of consequences. You can continue to lead a full and active life with continued care with the help of your healthcare provider.

If you have any questions about sickle cell disease, you can check with our specialist at Ask a doctor, 24x7.