This post will help you get a better understanding of bone marrow transplant as a cure for sickle cell disease.
Bone Marrow Transplant for Sickle Cell Disease
Sickle-cell disease (SCD), also known as sickle-cell anemia (SCA), is a hereditary blood disorder, caused by an abnormality in the oxygen-carrying protein hemoglobin found in red blood cells. This leads to a propensity for the cells to assume an abnormal, rigid, sickle-like shape under certain circumstances. Sickle-cell disease is associated with a number of acute and chronic health problems, such as severe infections, attacks of severe pain (“sickle-cell crisis”), stroke, and an increased risk of death.
The terms “sickle-cell crisis” or “sickling crisis” may be used to describe several independent acute conditions occurring in patients with SCD. SCD results in anemia and crises that could be of many types including the vaso-occlusive crisis, aplastic crisis, sequestration crisis, hemolytic crisis, and others. Most episodes of sickle-cell crises last between five and seven days. Although infection, dehydration, and acidosis (all of which favor sickling) can act as triggers, in most instances, no predisposing cause is identified.
What are the Causes of Sickle Cell Disease?
Sickle-cell disease occurs when a person inherits two abnormal copies of the hemoglobin gene, one from each parent. Several subtypes exist, depending on the exact mutation in each hemoglobin gene. A person with a single abnormal copy does not experience symptoms and is said to have sickle-cell trait. Such people are also referred to as carriers.
Symptoms of Sickle Cell Disease
The symptoms of sickle cell disease are:
- Eye Damage
- Other Features
- Leg Ulcers
- Fatigue and Anemia
- Bacterial Infections
- Lung and Heart Injury
- Aseptic Necrosis and Bone Infarcts (death of portions of bone)
- Splenic Sequestration (sudden pooling of blood in the spleen) and Liver Congestion
- Pain Crises Dactylitis (swelling and inflammation of the hands and/or feet) and Arthritis
Some features of sickle cell anemia, such as fatigue, anemia, pain crises, and bone infarcts can occur at any age. Many features typically occur in certain age groups. Infants and younger children can suer with fever, abdominal pain, pneumococcal bacterial infections, painful swellings of the hands and feet (dactylitis), and splenic sequestration. Adolescents and young adults more commonly develop leg ulcers, aseptic necrosis, and eye damage. Symptoms in adult typically are intermittent pain episodes due to an injury to bone, muscle, or internal organs.
Treatment of Sickle Cell Disease
Though medical management and blood transfusion are looked at managing symptoms of sickle cell disease, Bone Marrow Treatment (BMT) is the only proven effective treatment which can be called as a cure for Sickle Cell Disease. Bone Marrow Treatment requires a donor who is willing to donate. Ideally, a twin family member (syngeneic) or close relative (allogeneic) would donate the bone marrow necessary for transplantation.
Cost of Bone Marrow Treatment
The cost of the BMT is very high in advanced countries. In the USA, Bone Marrow Transplant will cost (autologous): USD 360,000. & Bone Marrow (allogeneic):USD 800,000.
But Treatment Possible through its association with Hospitals in India arranges the same BMT ranging from USD 25000 to USD 30000.
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