AN INHERITED RED BLOOD CELL DISORDER
Sickle Cell Disease
An inherited condition where red blood cells become sickle-shaped under stress, causing anaemia, painful crises, infections and organ complications. Managed with comprehensive lifelong care.
ABOUT THIS CONDITION
What is Sickle Cell Disease
Sickle cell disease is an inherited blood disorder in which red blood cells contain abnormal haemoglobin (haemoglobin S) that causes them to take on a rigid, sickle (crescent) shape under conditions of low oxygen, dehydration or stress. These sickle-shaped cells block small blood vessels, leading to pain, organ damage and reduced oxygen delivery. They also break down more quickly than normal cells, causing chronic anaemia. Typical features include chronic anaemia with tiredness and pallor, episodes of severe pain (sickle cell crises) in the bones, chest, abdomen or joints, frequent infections, jaundice, delayed growth in children, leg ulcers, eye problems, kidney issues and risk of stroke. The disease is more common in people of African, Mediterranean, Middle Eastern and Indian origin. Diagnosis is confirmed with blood tests including haemoglobin electrophoresis. Treatment includes preventive measures, pain management, hydration, blood transfusions, specific medical therapy and, in selected patients, bone marrow transplantation. Dr. Patnam Pravallika Reddy provides initial evaluation and co-management with haematology at Lux Hospitals, Hyderabad.
SIGNS TO WATCH
Common Symptoms
Symptoms that need attention
WHY IT HAPPENS
Causes & Risk Factors
- Inherited from both parents (recessive condition)
- Abnormal haemoglobin S in red blood cells
- More common in African, Mediterranean, Middle Eastern and Indian populations
- Triggers for crises: dehydration, infection, cold, stress, low oxygen
- Carrier state (sickle cell trait) usually causes no symptoms
- Identified at birth or in childhood in many regions
- Genetic counselling important for families
CLINICAL DETAILS
KeyFacts
Complete blood count, blood film and haemoglobin electrophoresis
Sickle cell anaemia (homozygous) · Sickle cell trait (heterozygous, carrier)
Preventive care, pain management, transfusions, specific medical therapy and bone marrow transplant in selected cases
Hydration, infection prevention and avoiding triggers
Pneumococcal and other vaccinations are essential
Available at Lux Hospitals, Hyderabad — co-managed with haematology
HOW WE TREAT IT
Treatment Approach
Comprehensive Lifelong Care
The most effective approach is comprehensive lifelong care with preventive measures, vaccinations, early treatment of pain crises with hydration and pain relief, regular monitoring, specific disease-modifying therapy in selected patients, and specialist haematology co-management.
- 1
Consultation & Assessment
Dr. Pravallika reviews symptoms, family history and prior crises, examines the patient and arranges blood tests including haemoglobin electrophoresis.
- 2
Treatment Planning
A personalised plan is created in coordination with haematology, including preventive care, crisis management and long-term monitoring.
- 3
Medical Management
Pain crisis management, hydration, infection prevention, vaccinations, transfusions when needed and specific disease-modifying therapy.
- 4
Recovery & Follow-up
Long-term follow-up with haematology including screening for complications and ongoing support.
AVAILABLE TREATMENTS
Treatment Options
Pain Crisis Management
Acute crises are managed with hydration, oxygen, effective pain relief and treatment of any precipitating cause such as infection or dehydration.
Hydration and Infection Prevention
Adequate hydration, prompt treatment of infections, and vaccinations against pneumococcus, influenza and other organisms are essential.
Blood Transfusions When Needed
Transfusions are used for severe anaemia, acute complications and stroke prevention in selected patients.
Disease-Modifying Therapy in Selected Patients (Referral for haematology)
Specific medical therapy reduces the frequency of crises, transfusion needs and complications in many patients with sickle cell disease.
Bone Marrow Transplantation in Selected Patients (Referral for haematology / transplant evaluation)
Bone marrow or stem cell transplantation can be curative in carefully selected patients, typically children with severe disease.
COMMON QUESTIONS
Frequently Asked Questions
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