Sickle Cell disease is a complex disorder:
Sickle cell disease has a large number of complications along with a variety of types. It is basically an autosomal recessive trait. This means that the gene for the sickle cell disease should come from both the parents. A patient with only one sickle cell gene is known to be a carrier of sickle cell trait.
It is a condition in which the patient suffers from various chronic symptoms which occur due to deficient blood or oxygen supply to various important organs. In sickle cell crisis, the abnormally shaped sickle cell red blood cells tend to stick to the arterial wall and cause attacks of severe, sudden pain or “Pain Crisis”. The intensity of pain and frequency of episodes of pain can increase with increasing age. The number of hospitalizations just tend to show only a marginal amount of patient suffering. The severity and intensity of pain for those who do not reach the hospital is still unknown. The crisis has its manifestation in a variety of organs of the body such as eyes, heart, lungs, limbs etc. In order to have a better understanding of the sickle cell crisis, we shall try to understand its effects on the body.
Firstly, we shall look into the neurological deficits. A patient suffering from sickle cell crisis may have seizure episodes, strokes or coma in some patients. It occurs due to deficient supply of blood to the brain.
The spleen is an important organ which consists of a lot of small and big arteries. When during the cell crisis, the splenic vessels are blocked, doctors call it “splenic sequestration”. As a result, of which spleen may become enlarged and may have to be removed. Also, in some cases, the spleen itself stops working due to lack of oxygen and starts shrinking. It shrinks until it is no longer viable. This is termed as “auto splenectomy”. The operation is known a “splenectomy”. The major function of the spleen is to protect against infections. The patients which have undergone splenectomy are more prone to infections such as haemophilus, influenza, and streptococcus.
When in the crisis, the blood vessels of the hand and feet are blocked, the result is the swelling of hands and feet. It can also lead to leg ulcers. It is one of the first signs of sickle cell disease in the infants.
Another complication associated with the sickle cell crisis is heart and chest disease symptoms. The lack of oxygen in the blood cause problems such as heart attack, heart failures and abnormal heart rhythms. As a result, there is the weakening of heart muscle. Over a period of time, chronic sickle cell crisis causes moderate to severe damage to the lungs. The result of which is pulmonary hypertension (high blood pressure in the lungs) or pulmonary fibrosis (scarring of the lungs). The long-term damage to lung further reduces the oxygen supply and can cause frequent episodes of sickle cell crisis.
A severe type of crisis is “sickle chest syndrome”. It is a condition which is associated with chest pains, cough, fever, sputum production, shortness of breath, and low blood oxygen levels. X-ray shows either pneumonia or dead lung tissue. The sickle chest syndrome worsens the prognosis for the patients.
But let us talk about the individuals with the so-called “sickle cell trait”. These individuals do not suffer from any of the complications of the sickle cell disease. They carry the gene for the disease and can even transfer the disease but do not suffer from the disease. If a person with sickle cell trait marries another person with the disease or the trait, their child is likely to develop the severe form of the disease. Since it is an inherited disease, pre-marital genetic counseling should be mandatory. Various hospitals in India, offer good quality counselors and gene tests which can adequately tell the patients about their genetic make-up. It helps one to understand preventive measures, possible treatments, and reproductive options.
So, we can safely say that a person having a sickle cell trait does not suffer from sickle cell crisis. He/she might suffer from mild anaemia though which can/cannot be attributed to the sickle cell syndrome.
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