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Sickle Cell Anemia
Sickle cell anemia is a chronic, hereditary blood disorder marked by the production of an abnormal form of hemoglobin called hemoglobin S.
Sickle Cell Anemia
Overview of Sickle Cell Anemia
Sickle cell anemia is a chronic, hereditary blood disorder marked by the production of an abnormal form of hemoglobin called hemoglobin S. This faulty protein causes red blood cells to distort into a crescent or sickle shape, unlike the normal, flexible, disc-shaped cells. These abnormally shaped cells tend to become stiff and sticky, leading to clumping and blockages in small blood vessels. The impaired circulation results in pain episodes, tissue damage, and an array of systemic complications.
Sickle cell anemia predominantly affects individuals of African, Mediterranean, Middle Eastern, and Indian descent. The condition is inherited in an autosomal recessive pattern—meaning a person must inherit the defective gene from both parents to develop the disease. Those who inherit only one defective gene are carriers and typically show no or very mild symptoms.
While there is currently no universal cure, significant advancements in treatment have improved the lifespan and quality of life for those affected. At Konkan Cancer Hospital, we offer specialized care through evidence-based treatment protocols, comprehensive diagnosis, pain management, and long-term follow-up, supporting both children and adults battling this condition.
Signs & Symptoms
Early identification of symptoms enables better management and prevention of complications. Some hallmark symptoms include:
Anemia: Due to rapid breakdown of sickled cells, patients often experience fatigue, pallor, and shortness of breath.
Pain Crises: Also called vaso-occlusive crises, these are intense episodes of pain resulting from blocked blood flow, commonly affecting bones, joints, chest, and abdomen.
Swelling: Dactylitis or painful swelling in hands and feet is often among the earliest signs in children.
Frequent Infections: Damage to the spleen compromises immunity, increasing susceptibility to infections like pneumonia.
Delayed Growth and Puberty: Chronic anemia and nutrient malabsorption can lead to stunted physical development in children.
Vision Problems: Blocked retinal vessels may cause visual disturbances or even blindness over time.
When to Consult a Specialist:
Persistent pain or fatigue
Fever over 101°F
Difficulty breathing
Sudden weakness or slurred speech (possible stroke)
Konkan Cancer Hospital provides 24/7 access to hematologists, emergency care, and pain management experts for timely intervention.
Diagnostic Tests & Procedures
Effective management begins with accurate diagnosis. At Konkan Cancer Hospital, we utilize advanced diagnostic tools, including:
Complete Blood Count (CBC): Indicates overall blood health and detects anemia.
Peripheral Blood Smear: Reveals presence of sickle-shaped red cells.
Hemoglobin Electrophoresis: The gold standard for identifying hemoglobin variants like HbS, HbC, or fetal hemoglobin.
High-Performance Liquid Chromatography (HPLC): Offers precise quantification of different hemoglobin types.
Genetic Testing: Confirms mutations in the HBB gene and helps in prenatal diagnosis.
Newborn Screening: Mandatory in many countries and highly recommended for early detection and intervention.
Once diagnosed, patients are enrolled in a personalized care plan including regular monitoring, vaccination schedules, and education on symptom awareness and emergency care.
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Treatment Options at Konkan Cancer Hospital
Managing sickle cell anemia involves a multidisciplinary approach tailored to each patient. Treatment goals include pain control, preventing complications, and enhancing quality of life.
Medications:
Hydroxyurea: Increases fetal hemoglobin levels, reduces pain episodes, and lowers the need for blood transfusions.
L-glutamine: Helps in reducing the frequency of sickle cell crises.
Folic Acid: Supports red blood cell production.
Antibiotics & Vaccinations: Prevent infections, especially in young children.
Blood Transfusions:
Used to treat severe anemia, prevent stroke, and manage complications.
Exchange transfusions help reduce sickled cells during crises.
Bone Marrow or Stem Cell Transplant:
The only potential cure, though limited by the need for a compatible donor.
Recommended for severe cases and younger patients.
Supportive Therapies:
Pain management
Nutritional guidance
Psychological counseling
At Konkan Cancer Hospital, we offer all these modalities under one roof with constant monitoring and compassionate care.
Prognosis & Long-Term Management
Thanks to modern interventions, many individuals with sickle cell anemia now live into their 50s and beyond. Factors contributing to better outcomes include:
Early diagnosis
Consistent use of hydroxyurea
Routine check-ups with a hematologist
Prevention of infections via vaccination
Lifestyle modifications such as staying hydrated and avoiding extreme temperatures
Konkan Cancer Hospital’s dedicated sickle cell clinic ensures continuity of care through regular monitoring, emergency access, and a patient-centric support system. We emphasize long-term follow-up to mitigate complications and improve overall prognosis.
Most Asked FAQs on Sickle Cell Anemia
Yes. Staying hydrated, avoiding extreme temperatures, eating a nutrient-rich diet, and managing stress can help reduce complications.
Triggers include dehydration, infection, strenuous activity, and cold weather. Prevent by staying hydrated, taking medications regularly, and avoiding known triggers.
Low-intensity exercises like walking and swimming are generally safe. Always consult your hematologist before beginning a new routine.
Yes, it can damage the spleen, liver, kidneys, lungs, eyes, and even the brain. Regular screening helps detect these complications early.
Yes. It is inherited when both parents pass on a sickle cell gene.
A hematologist is the primary specialist. Depending on complications, other experts such as pulmonologists or nephrologists may be involved.
With regular treatment and preventive care, many patients can live into their 50s or longer with a good quality of life.
For Expert Consultation: Book an appointment with our Sickle Cell Specialist Team at Konkan Cancer Hospital. We’re committed to advanced diagnostics, compassionate care, and world-class treatment standards.
