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Sickle Cell Anaemia
- Days In Hospital 10 days
- Outside Hospital Stay 30 days
- Success Rate 95%
Minimum Cost
$ 20000
Maximum Cost.
$ 40000
Most Asked Procedures
Overview of Sickle Cell Anaemia in India
A blood condition known as sickle cell anaemia occurs when red blood cells have structural abnormalities. Oxygen is carried and supplied to the body by red blood cells. Several body processes may be affected when these red blood cells are aberrant because they are unable to carry oxygen. This condition is known as sickle cell anaemia because the red blood cells in it have a crescent-shaped appearance.
In India, the cost of treating sickle cell anaemia ranges from $20,000 to $40,000. In India, there is a greater than 95% chance of successful therapy for sickle cell anaemia. The increased likelihood is a result of improvements in medical diagnosis and care.
What Is Sickle Cell Anaemia?
The most severe form of sickle cell disease, an inherited blood condition, is sickle cell anaemia. You get sickle cell anaemia when you inherit a genetic abnormality that results in the development of sickle-shaped or C-shaped red blood cells. These cells may be referred to as "sickled cells" by medical professionals.
Red blood cells are typically spherical, pliable discs. They deliver oxygen to your organs and tissues by gliding through your blood arteries. Instead of moving throughout your body, sickled cells become lodged in your blood arteries because they are sticky and rigid. Additionally, sickled cells degrade and expire sooner than healthy red blood cells. Your red blood cell supply is impacted by sickle cell anaemia, leading to severe anaemia (low red blood cell levels).
Life-threatening complications can arise from sickle cell anaemia. In the past, only a few infants born with this syndrome survived to adulthood. More people are now living into their 50s because of innovative treatments and early detection.
How Common Is Sickle Cell Anaemia?
At least 100,000 Americans are thought to be affected by sickle cell anaemia, according to experts. Black people are more affected by it than white people. According to experts, the genetic mutation that causes the illness is present in 1 in 365 Black individuals. People with South Asian, Middle Eastern, or Southern European ancestry may also be affected.
Types of Sickle Cell Anaemia:
The genes inherited from parents can be used to identify the different forms of sickle cell anaemia that manifest. Here are a few varieties of sickle cell anaemia:
Haemoglobin SS (HbSS): 65% of individuals with sickle cell disease have haemoglobin SS (HbSS), the most severe kind. It develops when two haemoglobin S genes are inherited from both parents, leading to more severe symptoms and chronic anaemia.
Haemoglobin SC (HbSC): About 25% of people with the condition have haemoglobin SC (HbSC), a less to more severe form. This is a less severe form of anaemia than HbSS, caused by inheriting one haemoglobin S gene from one parent and one haemoglobin C gene from the other.
Haemoglobin (HbS) beta thalassemia: The condition known as haemoglobin (HbS) beta thalassaemia occurs when a person inherits the beta thalassaemia gene from one parent and the haemoglobin S gene from the other. Of the two kinds, "Plus" (HbS beta +) affects about 8% of SCD patients, while "Zero" (HbS beta 0) affects about 2%. The latter, like HbSS, is more severe, whereas the former is gentler.
Other Rare Forms: Other uncommon forms are haemoglobin SO (HbSO), haemoglobin SE (HbSE), and haemoglobin SD (HbSD). They arise from the inheritance of one gene for haemoglobin S and another gene that produces another faulty haemoglobin variant (D, E, or O). Less frequently, these kinds are associated with less severe symptoms.
Procedure:
The primary goals of treatment for sickle cell disease (SCD) are to manage symptoms, prevent complications, and improve quality of life. Essential choices for treatment include:
Medications
- Hydroxyurea: This medication helps prevent pain crises and blood transfusion frequency by increasing foetal haemoglobin production, which reduces sickling of red blood cells.
- Pain Relief: If you experience more severe pain crises, you may require more potent, prescribed painkillers. Otherwise, over-the-counter pain medications such as acetaminophen or ibuprofen can help manage mild pain.
- Antibiotics: Penicillin and/or other antibiotics can help prevent infections in people with sickle cell disease who have a compromised spleen and are at increased risk for infections.
Blood Transfusions
Routine blood transfusions can help reduce the likelihood of stroke and other complications by increasing the number of healthy red blood cells in circulation.
Bone Marrow or Stem Cell Transplant
Sickle Cell Disease (SCD) can be cured with a bone marrow or stem cell transplant. An appropriate donor with healthy bone marrow is found, and his/her healthy bone marrow takes the place of the damaged bone marrow. However, it is often only saved when the SCD is severe and the risks are significant.
Gene Therapy
New gene therapy treatments are intended to correct the genetic defect that causes sickle cell disease (SCD). These are still experimental treatments, but they are creating the possibility for a permanent cure.
Supportive Care
- Hydration: Drinking enough fluids helps minimise the chances of sickle cell crises.
- Healthy Lifestyle: The disease can be managed by diet, exercise and avoiding things that cause stress (like high heat),
- Regular Check-Ups: Regular visits with the doctor are necessary to monitor the disease and treat any complications.
Patient Education and Counselling
Patients and their families must be educated about sickle cell disease (SCD) management and compliance with their treatment plan. Counselling and psychological support can also help with the chronic nature of the disease.
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Cost of Sickle Cell Anaemia in India
In India, the cost of treating sickle cell anaemia ranges from $20,000 to $40,000. In India, there is a greater than 95% chance of successful therapy for sickle cell anaemia. The increased likelihood is a result of improvements in medical diagnosis and care.
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Indications of Sickle Cell Anaemia:
Typical signs and symptoms include:
- Anaemia-related fatigue: Infants with sickle cell anaemia may exhibit exceptionally high levels of fussiness and irritability.
- Regular infections: Because the illness damages your spleen, it impairs your immune system, making infections more likely to occur. However, the risk of contracting illnesses has now decreased significantly due to the widespread use of preventive medications and vaccinations.
- Pain: Because sickle cell anaemia deprives your tissues of oxygen, it damages them. You may experience pain in your arms, legs, chest, and back due to tissue damage. Excruciating pain may begin as an ache that worsens or develops suddenly.
- Painful swelling in hands and feet: One of the earliest signs of sickle cell anaemia in infants is painful swelling in the hands and feet. Babies' hands and feet cannot get blood because sickled cells become lodged in their blood arteries.
- Yellow-colored eyes and skin from jaundice: Jaundice causes yellow eyes and skin because, among other things, your liver filters red blood cells. Fast-dying sickled cells in sickle cell anaemia emit bilirubin, which accumulates in the body and results in jaundice.
The signs of sickle cell anaemia usually appear in infants between the ages of 6 and 9 months. As your body produces more aberrant (sickled) cells, your symptoms will fluctuate over time.
Sickle Cell Disease Causes
The HBB gene, which produces haemoglobin, a protein found in red blood cells that transports oxygen throughout the body, is altered or mutated in sickle cell disease. Typically, haemoglobin keeps red blood cells pliable and spherical. The body produces haemoglobin S, an aberrant form of haemoglobin, when the HBB gene is mutated in sickle cell disease. Because of this aberrant haemoglobin, red blood cells are rigid and have a sickle or crescent moon shape.
Typically, parents pass this genetic alteration on to their offspring. A person must inherit the sickle cell gene from both parents to have sickle cell disease. They can still pass the gene on to their offspring, even though they are a carrier (have the sickle cell trait), if they only have one parent who carries the gene, and often show no symptoms. We refer to this pattern of inheritance as autosomal recessive.
Sickle Cell Disease Diagnosis
Several tests are used to diagnose sickle cell disease, determining the severity of the condition and verifying the presence of the sickle cell gene. These consist of:
Prenatal Screening: Early intervention and management are made possible by the ability to do tests to identify sickle cell genes even before delivery. By taking a sample of the amniotic fluid around the foetus, sickle cell disease can be identified in the unborn child. Expectant parents should inquire about this screening from their healthcare team if they have sickle cell trait or sickle cell anaemia.
Newborn Screening: Using a heel-prick blood sample, infants are regularly screened for sickle cell disease (SCD) in many countries shortly after delivery. Prompt treatment and efficient disease management are made possible by early diagnosis.
Haemoglobin Electrophoresis: This test can detect the presence of haemoglobin S, validating a diagnosis of sickle cell disease, and differentiates the various forms of haemoglobin in the blood.
Genetic Testing: The HBB gene, which causes sickle cell disease (SCD), can have mutations that can be found through genetic testing. In addition to helping identify individuals who are carriers of the sickle cell trait, these tests can also validate the diagnosis.
Complete Blood Count (CBC): A complete blood count measures haemoglobin, red blood cells, and other blood components. Anaemia, a prevalent condition associated with sickle cell disease (SCD), can be diagnosed with its assistance.
Blood Smear: To detect the distinctive sickle-shaped red blood cells associated with sickle cell disease (SCD), a sample of blood is examined under a microscope.
Risk Factors:
The following are the risk factors associated with the development of sickle cell anaemia:
Genetic Factors
Genetic factors of abnormal haemoglobin, inherited from parents to children, are the commonest risk factor for sickle cell anaemia. A vast majority of newborns have sickle cell anaemia due to faulty genes.
Family History
A positive correlation has been shown between the development of sickle cell anaemia and family history.
Ethnicity
There is a higher prevalence of sickle cell anaemia among certain ethnic groups. People from South Asia, the Middle East, the Mediterranean, and Africa have a greater likelihood of developing it.
Parental Carriers
The child has an increased chance of getting sickle cell anaemia if both parents have the faulty gene.
Sickle Cell Disease Complications
The sickle-shaped red blood cells that cause sickle cell disease (SCD) can obstruct blood flow, resulting in several other complications that can be life-threatening. The most common complications include:
- Pain crises: Sudden and severe attacks of pain in the chest, muscles, joints, and bones.
- Acute chest syndrome: A life-threatening lung disease that arises due to lung infection or obstruction of blood flow to the lungs.
- Stroke: Is responsible for causing irreversible damage to the brain due to the blood supply to the brain being blocked.
- Organ damage: SCD can damage the heart, lungs, liver, spleen, and kidneys.
- Anaemia: A long-lasting reduction of red blood cells caused by their destruction.
- Infections: Individuals with sickle cell disease are more susceptible to getting infections as their immune systems are impaired.
- Pulmonary hypertension: High blood pressure affecting the blood vessels of the lungs.
- Leg ulcers: Non-healing areas of open sores on the legs.
- Priapism: A sustained and painful erection of the penis.
Frequently Asked Questions (FAQ’s)
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Haematopoietic stem cell/ hematopoietic progenitor cell transplantation (HSCT or HPC), also known as a bone marrow transplant, is the only cure for sickle cell anaemia and is the best treatment option for reversing sickle cell disease. Other treatment options are focused on the treatment of symptoms and preventing complications for individuals who are not candidates for HSCT or who do not have a donor match. Examples of these treatment options are blood transfusion therapy, pain management techniques and medications like hydroxyurea.
There are numerous potential adverse impacts of sickle cell disease (SCD), which could severely impact a patient’s life, for example, acute pain crises, constant pain, and possible organ damage. They may experience lower quality of life, decreased work productivity, and increased healthcare usage. SCD is a chronic disease and can be managed appropriately with the proper care; however, it can be unpredictable and requires care and surveillance on an ongoing basis.
Haematopoietic stem cell transplantation (HSCT), or bone marrow transplantation, is the only effective cure for sickle cell anaemia, and can provide a cure. Other treatments focus on treating symptoms and preventing complications for people who are not eligible for HSCT or do not have a compatible donor, including blood transfusions, treating pain, and taking medications like hydroxyurea.