What is sickle cell disease?
Sickle cell disease (SCD) is an inherited blood disorder. It’s passed down from parent to child. Abnormal hemoglobin is created in the body when this disease strikes you. Haemoglobin is the protein in red blood cells that carries oxygen to all parts of your body. When you have SCD, your body’s tissues and organs don’t get enough oxygen.
Healthy RBC’s are able to move around easily. With Sickle cell disease, the red blood cells become hard and sticky. Shaped like the letter ‘C’, these damaged red blood cells (sickle cells) clump together. Easy movement through the blood vessels does not become possible. Blood vessels get stuck in small blood vessels and block the blood flow. This blockage stops the movement of healthy oxygen-rich blood which can cause pain or damage major organs.
Causes of sickle cell disease
Sickle cell is an inherited disease. It is caused by a defect in a gene. If only two genes are inherited one from each parent, then you have SCD. If you have just one gene you are healthy, but you are a carrier of the disease. If two carriers have a child, there is a greater chance their child will have SCD.
Parents who are carrier sickle cell gene have a 25% chance of having a child with SCD.
What are the risk factors for sickle cell disease?
Having a family history of sickle cell disease increases your risk for the disease. SCD mainly affects people from Africa and the Carribean. But the gene has also been found in people with origin from the Middle East, India, Latin America, and Mediterranean countries. It has also been found in Native American Indians.
What are the symptoms of sickle cell disease?
Symptoms vary from person to person. The symptoms may be mild or severe.
Symptoms may include:
   1. Anaemia
This is the most common symptom of the disease. Having fewer red blood cells causes’ anemia. Severe anemia can make you feel dizzy, short of breath, and tired.
   2. Yellowing of the skin, eyes, and mouth (jaundice)
This is a common symptom. Sickle cells don’t live as long as normal red blood cells. They die at a rate faster than what the liver can filter. The yellow color is caused by a substance (bilirubin) that is released when red blood cells die.
   3. Acute chest syndrome
This is when sickle cells stick together and block oxygen flow in the tiny vessels in the lungs. It often happens suddenly, when the body is under stress from infection, fever, or dehydration. Symptoms may seem like pneumonia. It can include fever, pain, and a violent cough.
   4. Splenic sequestration (pooling)
The spleen becomes enlarged and painful when sickle cells get stuck there so fewer red blood cells are able to move. This causes a sudden drop in hemoglobin.
   5. Stroke
This is another sudden and severe problem that occurs with this disease. The sickle cells can block the major blood vessels that bring oxygen to the brain. Any interruption in the flow of blood and oxygen to the brain can cause severe brain damage. If you have a stroke from SCD, you are more likely to have a second and third stroke.
How is sickle cell disease diagnosed?
Many hospitals routinely screen newborns for sickle cell. And, a blood test called hemoglobin electrophoresis may be done. It can tell if you are a carrier of sickle cell. It can also tell if you have any of the diseases linked with the sickle cell gene.
Treatment of SCD (Sickle Cell Disease) :
1. Pain medicines
These are used for sickle cell crisis.Â
2. Drinking plenty of water daily (8 to 10 glasses)
This is to prevent and treat pain crises. In some cases, IV (intravenous) fluids may be needed.
3. Blood transfusions
These may help treat anemia and prevent stroke. They are also used to dilute the sickle cell hemoglobin with normal hemoglobin. This is done to treat chronic pain, acute chest syndrome, splenic sequestration, and other emergencies.
4. Vaccinations and antibiotics
These are used to prevent infections that may arise.
5. Folic acid
This helps prevent severe anemia.
 6. Hydroxyurea
This medicine helps reduce the frequency of pain crises and acute chest syndrome. It may also help decrease the need for blood transfusions.
7. Bone marrow transplant
A transplant can cure some people with SCD. The decision to have a transplant is based on the severity of the disease and finding a suitable donor.
Living with sickle cell disease
SCD is an ongoing (chronic) condition. You may not be able to fully prevent the complications of this disease. However, living a healthy lifestyle can reduce some of the problems. This includes doing things such as:
- Eating a healthy diet with lots of fruits, vegetables, whole grains, and protein
- Getting enough sleep
- Drinking lots of fluids
Avoid things that may trigger a crisis, such as:
- High Altitudes
- Cold weather
- Swimming in cold water
- Heavy physical labor
- Medicines for nasal congestion (decongestants). They cause blood vessels to narrow
How to avoid sickle cell diseaseÂ
- Getting the flu shot each year
- Washing your hands often
- Avoiding people who are sick
- Regular dental exams
These were some of the ways with which we can counter sickle cell disease. Remember, prevention is better than cure.