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Medical Treatments

Sickle Cell Anemia

Sickle cell anemia is an inherited blood disorder primarily affecting hemoglobin, the protein in red blood cells responsible for carrying oxygen throughout the body. In individuals with this condition, a mutation in the HBB gene causes the production of abnormal hemoglobin called hemoglobin S (HbS). Under low-oxygen conditions, HbS molecules stick together, causing red blood cells to distort into a rigid, sickle (crescent) shape. Unlike normal round red blood cells, sickle cells are less flexible and tend to clump together, leading to blockages in small blood vessels.
These blockages impair blood flow, reducing oxygen supply to tissues and organs, which can result in episodes of intense pain known as "sickle cell crises." Over time, sickle cell anemia can lead to complications like chronic anemia, organ damage (particularly in the spleen, kidneys, and liver), stroke, and an increased risk of infections. Sickle cells also have a shorter lifespan than normal red blood cells, surviving for only 10-20 days compared to the typical 120 days, further contributing to anemia.
The condition is most common among individuals of African, Mediterranean, Middle Eastern, and Indian ancestry. Management of sickle cell anemia includes hydration, pain relief, blood transfusions, and, in some cases, bone marrow transplants. Advances in gene therapy offer hope for potential cures in the future. However, ongoing care and treatment are critical to improving the quality of life for those affected.

Signs & Symptoms

 People with sickle cell anemia often experience the following:

  • Pain Crises: Severe pain episodes caused by blocked blood flow. This can occur in the chest, abdomen, joints, or bones.
  • Fatigue: Tiredness due to anemia (low RBC count).
  • Swelling: Especially in the hands and feet.
  • Frequent Infections: The spleen (part of the immune system) can be damaged, making it harder to fight infections.
  • Delayed Growth: Children may grow more slowly and hit puberty later.
  • Vision Problems: Tiny blood vessels in the eyes may get blocked, leading to vision loss.
Causes

 Sickle cell anemia is caused by a mutation in the gene responsible for hemoglobin (the protein in RBCs that carries oxygen). This mutation is inherited from both parents. If a person inherits one sickle cell gene from each parent, they develop the disease. If they inherit only one sickle cell gene, they are carriers (called "sickle cell trait") but may not show symptoms.

When to seek medical attention?

 People with sickle cell anemia often experience the following:

  • Pain Crises: Severe pain episodes caused by blocked blood flow. This can occur in the chest, abdomen, joints, or bones.
  • Fatigue: Tiredness due to anemia (low RBC count).
  • Swelling: Especially in the hands and feet.
  • Frequent Infections: The spleen (part of the immune system) can be damaged, making it harder to fight infections.
  • Delayed Growth: Children may grow more slowly and hit puberty later.
  • Vision Problems: Tiny blood vessels in the eyes may get blocked, leading to vision loss.
Diagnostics & Tests

 Doctors diagnose sickle cell anemia using:

  • Blood Test: To check for the abnormal hemoglobin (called hemoglobin S).
  • Newborn Screening: In many countries, newborns are tested for sickle cell disease at birth.
  • Genetic Testing: For families with a history of the condition, testing can identify carriers or affected individuals.
  • Hemoglobin Electrophoresis: A test to measure the different types of hemoglobin in the blood.
Do’s & Don’ts

 Do's:

  • Stay Hydrated: Drink plenty of water to keep blood thin and reduce blockages.
  • Get Vaccinated: Especially against pneumonia, meningitis, and the flu, as infections can worsen symptoms.
  • Take Prescribed Medications: Follow your doctor's advice on treatments like hydroxyurea and pain relief.
  • Regular Checkups: Monitor your health to catch complications early.
  • Eat a Balanced Diet: Include foods rich in folic acid, such as leafy greens.

 Don'ts:

  • Avoid Dehydration: Don't skip fluids, especially in hot weather or after exercise.
  • Avoid Overexertion: Extreme physical activity can trigger pain crises.
  • Avoid Smoking and Alcohol: These can reduce oxygen in the blood and worsen symptoms.
  • Avoid High Altitudes: Lower oxygen levels can trigger sickle cell crises.

NOTE:

Our medical content authors have diligently gathered and synthesized information on this topic to offer valuable insights to our readers. Drawing from a range of reputable medical journals and health resources, this content aims to enhance understanding of the subject. It's important to remember that while this information is informative, it should not replace personalized consultation or treatment from a qualified physician. For further details, please refer to our Editorial Policy.

For this topic, our authors used some of the following resources:

American Society of Hematology | Sickle Cell Disease

Johns Hopkins Medicine | Sickle Cell Disease

National Institutes of Health (NIH) (.gov) | What Is Sickle Cell Disease? - NHLBI

National Sickle Cell Anemia Elimination Mission

nhs.uk | Sickle cell disease

Frequently Asked Questions For Sickle Cell Anemia

No, it is a genetic condition and cannot be passed from person to person like an infection.

There is no widespread cure, but bone marrow transplants may be curative for some patients. Treatments aim to manage symptoms and complications.

With proper care, many people with sickle cell anemia can live into adulthood and lead fulfilling lives. However, they may face health challenges and require lifelong medical care.

Yes, it can increase the risk of complications during pregnancy for both the mother and the baby. Regular prenatal care is essential for managing these risks.

If only one parent has the sickle cell trait, the child may inherit the trait but usually won't develop the disease. Both parents must carry the sickle cell gene for the child's anemia.

Dehydration, stress, cold temperatures, or infections can trigger pain crises, which sometimes happen without an apparent reason.

Best Hospitals in Sickle Cell Anemia

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BLK-MAX Super Specialty Hospital, Delhi – State-of-the-art medical facility
BLK-MAX Super Specialty Hospital

Pusa Road, Radha Soami Satsang, Rajendra Place New Delhi, 110005 India

High-tech medical equipment at Artemis Hospital, Gurgaon, ensuring accurate diagnostics and advanced treatment solutions.
Artemis Hospital

Artemis Hospitals Sector 51, Gurugram 122001 Haryana, India

SHALBY Sanar International Hospital, Gurugram – trusted healthcare destination for global patients via VivaVel.com
SHALBY Sanar International Hospitals, Gurugram

Golf Course Rd, Parsvnath Exotica, DLF Phase 5, Sector 53, Gurugram, Haryana Gurgaon, 122022 India

PARAS Health, Gurugram – top multispecialty hospital for international patients via VivaVel.com
PARAS Health, Gurugram

C-1, Sushant Lok- 1, Sector-43, Phase- I, Gurugram, Haryana, 122002

Amrita Hospital, Faridabad – India's largest multispecialty hospital with cutting-edge healthcare infrastructure and global standards.
Amrita Hospital

Mata Amritanandamayi Marg, Sector 88, Faridabad, Haryana, India 121002

High-end cardiac and orthopedic surgery at Max Hospital Dehradun – trusted by overseas patients with VivaVel.com support
Max Super Speciality Hospital, Dehradun

Mussoorie, Diversion Road, Dehradun, Uttarakhand 248001

Top Doctors in Sickle Cell Anemia

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A. V. S. Suresh
  • Dr. A. V. S. Suresh
  • Senior Consultant
  • Medical Oncology | Hematology
  • 21 Years Experience
  • Continental Hospitals

Dr. A. V. S. Suresh is a distinguished Senior Consultant Medical Oncologist and Hematologist at Continental Hospitals, Hyderabad. With over two decades of clinical ex...

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Gaurav Dixit
  • Dr. Gaurav Dixit
  • Unit Head Of Hemato-Oncology
  • Hematology | Bone Marrow Transplantation...
  • 13 Years Experience
  • Artemis Hospital

Dr. Gaurav Dixit is a highly experienced Hematologist and Bone Marrow Transplant (BMT) Specialist who currently leads the Haemato-Oncology Unit at Artemis Hospital, Gurugram....

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Rayaz Ahmed
  • Dr. Rayaz Ahmed
  • Senior Director - Oncology
  • Oncologist | Hematologist
  • 14 Years Experience
  • Max Super Speciality Hospital, Saket

Dr. Rayaz Ahmed is a director with expertise in Cancer Care/Oncology, Bone Marrow Transplant, Hematology Oncology, and hematology (Hematology) at Max Super Speciality Hospita...

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Soumya Bhattacharya
  • Dr. Soumya Bhattacharya
  • Consultant
  • Haematologist
  • 25 Years Experience
  • Apollo Gleneagles Hospital, Kolkata

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Roshan Dikshit
  • Dr. Roshan Dikshit
  • Senior Consultant
  • Hematologist & Transplant Specialist
  • 16 Years Experience
  • Aakash Healthcare Super Speciality Hospital, Dwarka

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Nitin Sood
  • Dr. Nitin Sood
  • Vice Chairman
  • Haemato-Oncology & Bone Marrow Transplan...
  • 29 Years Experience
  • Medanta - The Medicity, Gurgaon

Dr. Nitin Sood is a distinguished Clinical Hematologist, Hemato-Oncologist, and Bone Marrow Transplant Specialist with nearly thirty years of clinical, academic, and research exper...

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