- Bone marrow or stem cell transplant is a permanent cure for the disease
- Diagnosis can be done during pregnancy too
- This is possible in case of family history of sickle cell disease
World Sickle Cell Day is observed on June 19. The day is meant to raise awareness about sickle cell disease, which is a group of disorders that affect haemoglobin, the molecule in red blood cells which deliver oxygen to cells throughout the body. People with this disorder have haemoglobin S, an atypical haemoglobin molecule which can distort red blood cells into a sickle or a crescent shape. This disease is a blood disorder which is inherited and passed down from parents to the child. In 2008, the UN General Assembly recognised sickle cell disease as a public health problem, and one of the world's foremost genetic diseases.
World Sickle Cell Disease Day 2020: Know the symptoms
Symptoms of sickle cell disease usually appear around five months of age. The symptoms vary from person to person and change over time.
- Pain: Sickle cell disease causes period episodes of pain. When sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints, it causes pain. The pain can also occur in bones.
- Anaemia: Sickle cells break apart easily and die, leaving a person with very few red blood cells. This can cause anaemia. Red blood cells usually live for around 120 days before they need to be replaced. In case of sickle cell disease, they die in 10 to 20 days, leaving a shortage of red blood cells.
- Delayed growth or puberty: Red blood cells provide your body with oxygen and nutrients that are needed for growth. Shortage of healthy red blood cells can slow down growth and delay puberty.
- Frequent infections: Sickle cell disease can damage your spleen and make you more vulnerable to infections. Infants and children with sickle cell anaemia are giving vaccinations and antibiotics in order to prevent infections like pneumonia.
- Swelling of hands and feet: Sickle-shaped red blood cells block blood flow to the hand and feet and cause swelling.
- Vision problems: Blood vessels in eyes can become plugged with sickle cells. This can damage retina and lead to vision problems.
Symptoms of sickle cell disease can appear from two to five months of age. But in milder cases, the symptoms may appear till teenage.
Type of sickle cell disease
One can get infected with sickle cell disease if both parents have the problem gene and pass it on to their child.
1. HbSS or sickle cell anemia is a severe kind of sickle cell disease. It occurs when child inherits sickle cell gene from each parent.
2. HbSc occurs when one parents has sickle cell gene and the other has a gene from abnormal haemoglobin.
3. Hbs beta thalassemia occurs when sickle cell gene is passed from one parent, and beta-thalassemia is passed on from the other.
If only one parent has the problem gene, then the child will not have symptoms but will have the gene known as sickle cell trait.
Sickle cell disease diagnosis and treatment
Sickle cell can be detected in an infant during newborn screening process. In case of family history, it can be detected during pregnancy.
Bone marrow or stem cell transplant is the only permanent cure for sickle cell disease. Early detection and timely treatment can help in managing symptoms, offer relief from pain and prevent severe complications.
Apart from stem cell transplant, the symptoms can be managed with the help of periodic blood transfusion, pain killers, vaccinations and antibiotics.
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