What do we actually mean by the Sickle Cell Disease?
Sickle cell disease! Sounds familiar? Yes, it is a common chronic blood disorder which generally shows anemia-like symptoms in the affected population. Sickle-cell disease is a rare disorder in India with less than 1 million cases per year. But it is a fairly common condition in African and Mediterranean population.
Basically, it is an inherited blood disorder in which the shape of RBCs is characteristically “sickle-shaped” or “crescent-shaped” instead of its normal biconcave shape. Now, let us understand how is this change in shape affects the patient suffering from it. Since the main function of an RBC is to carry oxygen to all organs of the body, a change in the shape of a red blood cell drastically affects its oxygen-carrying capacity.
The life expectancy of a person suffering from the sickle cell disease is nearly 40-47 years as per certain studies. This is not a definite number as it can increase or decrease depending upon the quality of care a patient receives.
Why is the Sickle cell Disease Problematic?
The sickle-shaped cells cause many problems for the individual. Firstly, the cells are not flexible as a result of which they burst apart more often than usual. This reduces the lifespan of the RBCs to 10-12 days from 90-120 days. Thus, there is a discontinuity in the rate of production of new cells and destruction of the old ones. Also, the sickle-shaped cells can stick to the blood vessels causing an obstruction to the blood flow. The lack of oxygen to certain vital organs can cause sudden severe pain and ischaemic crisis.
What are its Symptoms?
Which leads us to the symptoms of the Sickle Cell disease. The symptoms of the sickle cell disease vary from mild to severe anemia in the patients carrying the sickle cell gene. The patient can just be a carrier of the trait with no apparent clinical symptoms or can be suffering from the severe form where the patient receives the sickle cell trait from both the parents. Some of the common symptoms of sickle cell disease are fatigue and tiredness, pain, or infections. Painful swelling can usually be seen in the hands and limbs of the affected patients. Presence of jaundice or “yellowish-discoloration” of the skin and in eyes (“Icterus”) is also observed.
Are there any complications associated with the Sickle Cell disease?
There might also occur a number of complications in sickle cell disease patients. The children suffering from sickle cell disease may have delayed growth and are generally shorter but usually, regain their height by adulthood. Seizures, strokes, or even coma can result from sickle cell disease. Blindness and skin problems may occur if the vessels of these respective organs are blocked. Gallstones are other sets of problems which are not related to the blockage of arteries but due to the accumulation of pigment- bilirubin which is the result of the constant breakdown of red blood cells.
The diagnosis of the sickle cell disease is mostly done as per the presented symptoms by the patients. Also, the doctor may prescribe certain diagnostic tests just to be sure. These could be blood tests (blood counts can reveal abnormal Hb levels) and “Hb electrophoresis” which determines the type of hemoglobin in the blood.
Is it preventable?
The Sickle Cell disease can be prevented with early diagnosis. Genetic counseling of the parents is a necessary step. It informs the parents about their genetic make-up and the possibility of transmission of the disease to their offspring. The new intra-natal diagnostic techniques even allow detection of the occurrence of sickle cell disease in the child before it is born. This is important as it becomes easier for the doctor to manage the sickle cell disease from early on.
How can it be treated? Is it curable?
As far as the cure for sickle cell disease is concerned, a bone marrow transplant is the only possibility. But it poses severe risks and is very vulnerable to failure and rejection. This treatment option is not indicated for severe cases of the sickle cell disease. For patients with a mild form of the disease, the bone marrow transplant can still be considered because it’s less risky and complicated for them.
Otherwise, the physician generally manages the disease symptomatically. They generally prefer treating the complications occurring in the individual such as the artery disease or the pain crisis or jaundice and anemia.
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