What is Sickle Cell Anemia Disease?
Sickle cell anemia or sickle cell disease (SCD)is genetic health disorder of your red blood cells (RBCs). Usually, Red blood cells are in shape of a disc which gives them full freedom to travel via even smallest blood vessels in the body. However, with this disorder, the red blood cells shape abnormally like a crescent shape resembling a tool called sickle. This makes these red blood cells sticky & rigid prone to likely getting trapped in the small vessels which partially blocks or stops the blood from reaching different parts of your body. This condition can cause pain and damage of tissues.
There are multiple types of Sickle cell disease but they all have similar type of symptoms which may vary in severity. These include as below-
- Irritability and excessive fatigue from anemia
- In babies, fussiness.
- Bedwetting from associated kidney disorders
- Pain and swelling in hands and feet
- Frequent infection episodes.
- Pain in legs, arms, chest and back.
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Let us know, who is at risk for sickle cell disease?
Children are only at risk for this sickle cell anemia, Incase of both parents carrying sickle cell trait. A blood investigation that is called hemoglobin electrophoresis can also reveal which type does a patient have.
People from regions which have more cases of malaria disease are more likely to be carriers. This regions include people from:
- Saudi Arabia
- the Mediterranean
What complications may arise from sickle cell anemia disease?
Sickle cell anemia may cause severe complications which appear when these abnormally shaped cells block the vessels in different parts of the body. Painful and damaging blockages are called as sickle cell crises. They can be caused by a number of reasons, including:
- Change in temperatures
- Altitude (Heights)
- Poor Hydration
The following types of complications may result from sickle cell anemia-
Severe anemia – Shortage of RBCs in the body.
Hand-foot syndrome- This can cause swelling in the hands and the foot.
Splenic sequestration- This is a blockage of the splenic vessels by the sickle cells. It causes a sudden and painful enlargement of your spleen. A patient require to undergo a procedure for removal of spleen in this scenario.
Delayed growth- Delayed growth often occurs in patients with Sickle cell anemia disease.
Neurological complications – Seizures, stroke and even sometimes coma can result from sickle cell disease. These are caused by blockages in the brain. Urgent treatment is needed in such conditions.
Eye problems- Blindness may caused by blockages in the vessels which supply blood to your the eyes, it can also damage retina.
Skin ulcers- Skin ulcers in the legs can be there in this disease due to vessel blockage.
Heart disease &chest syndrome- Since this interferes with blood oxygen supply, it can also cause cardiac problems.
Lung disease- May cause decreased blood flow which may result in high blood pressure in the lungs.
Priapism- Painful erections may happen due to blockages in the blood vessels.
Gallstone- This does not happen due to blockage in blood vessels but due breakdown of RBCs, the byproduct of this breakdown is bilirubin and High levels can lead to gallstones.
Sickle chest syndrome- It is a severe type of sickle cell crisis. It causes severe chest pain and is associated with problems like cough, fever, shortness of breath, sputum production and also low blood oxygen levels.
How is sickle cell disease diagnosed?
In children and in the adults, one or more of the following procedures may be used to diagnose this disease
Detailed history of the patient
- Severe pain in bones
- Acute pain in hands and legs
- Painful enlargement of spleen
- Growth issues
- Lung infections
- ulcers in the legs
- Cardiac problems
A doctor would want to test you for sickle cell anemia incase you any symptoms mentioned above.
- Blood counts can reveal an abnormal Hb levels.
- Blood films may reveal RBCs that appear irregular.
- Sickle solubility tests.
This is needed to confirm the diagnosis of sickle cell disease.
What is the treatment of sickle cell anemia ?
A number of treatments are available for sickle cell disease-
- Use of intravenous fluids helps RBCs(red blood cells )return to a normal state.
- Treatment of underlying or associated infections is a vital part of managing the crisis by SCD.
- Blood transfusions help in improvement of transport of oxygen and the nutrients as needed for the body.
- Supplemental O2 is given which makes breathing easier and improves the oxygen levels.
- Pain killer drugs are used to relieve the pain caused during a sickle crisis.
- Immunizations/Vaccination can help prevent the infections the body. Patients usually tend to have low immunity.
Bone marrow transplant or Stem cell transplant has been used to treat sickle cell anemia patients. The Children who are younger than 16 years of age with severe complications, and those who have aHLA matching donor are considered the best candidates for a stem cell transplant.
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