Sickle Cell Disease
Sickle cell disease is an inherited disorder in which red blood cells contain an abnormal form of haemoglobin that causes them to become rigid and crescent- or sickle-shaped under certain conditions. These misshapen cells can block small blood vessels, causing pain episodes and organ damage, and break down prematurely, causing chronic anemia. It is diagnosed through haemoglobin electrophoresis or genetic testing, often as part of newborn or carrier screening.
Understanding Sickle Cell Disease
Sickle cell disease is caused by a mutation in the gene for haemoglobin, the oxygen-carrying protein in red blood cells, resulting in an abnormal form called haemoglobin S. Under conditions such as low oxygen, dehydration, or infection, red cells containing haemoglobin S can distort into a rigid, crescent (sickle) shape. These sickled cells do not flow smoothly through small blood vessels and can block circulation, and they also break down more quickly than normal red cells, causing chronic anemia.
Sickle cell disease is inherited in an autosomal recessive pattern, meaning a child needs to inherit the abnormal gene from both parents to have the disease. A person who inherits the gene from only one parent has sickle cell trait, which is usually asymptomatic but can still be passed on to children. Because of this, couples with a family history of sickle cell trait or disease are encouraged to consider genetic counselling before or during pregnancy.
Sickle cell disease is a lifelong condition with recurring complications, including painful vaso-occlusive crises, increased susceptibility to infection due to reduced spleen function, and potential effects on the lungs, kidneys, and other organs over time. Because of this broad and evolving impact, coordinated, ongoing haematology care is central to managing the condition and reducing complications.
Signs of Sickle Cell Disease to watch for
When to see a haematologist
A sudden, severe pain crisis, chest pain, or difficulty breathing in a known sickle cell patient is a medical emergency and needs immediate attention.
Fever in a person with sickle cell disease should be treated urgently given the increased risk of serious infection.
Couples with a family history of sickle cell disease or trait should seek genetic counselling before or during pregnancy to understand the risk to their children.
Tests offered at the centre
Haemoglobin Electrophoresis / HPLC
Identifies and measures haemoglobin S and other haemoglobin variants, confirming the diagnosis of sickle cell disease or trait.
Complete Blood Count and Peripheral Smear
Shows characteristic sickle-shaped red cells and a reticulocyte count, providing supporting evidence and helping monitor disease activity.
Genetic (Molecular) Testing
Confirms the specific haemoglobin gene mutation, useful for diagnosis confirmation, carrier testing, and family counselling.
Newborn and Carrier Screening
Identifies sickle cell disease or trait early, allowing preventive care and family counselling to begin before complications arise.
Treatment options
Your treatment plan is personalized to your diagnosis, subtype and overall health.
Pain Management Protocols
Structured approaches to managing acute pain crises aim to control pain effectively and identify and treat any triggering factor.
Hydroxyurea Therapy
A medication that can reduce the frequency and severity of pain crises in eligible patients by increasing a protective form of haemoglobin.
Blood Transfusion Support
Used for severe anemia or specific complications, and in some cases as a preventive strategy for patients at higher risk of certain complications.
Infection Prevention
Vaccination and, in children, prophylactic antibiotics help reduce the risk of serious infection related to reduced spleen function.
Bone Marrow / Stem Cell Transplant
A potentially curative option for select eligible patients with a matched donor, considered on an individual basis.
Why choose AP Blood & Blood Cancer Centre
Sickle cell disease requires both emergency readiness for pain crises and structured long-term care to prevent complications, which benefits from a haematology-focused, single-specialty environment rather than fragmented general care.
Dr. Amarnadh Polisetty, MBBS, MD (General Medicine), DNB (Clinical Haematology), has over 7 years of experience in clinical haematology and has been part of specialist transplant teams managing more than 500 bone marrow transplant patients, relevant to evaluating transplant eligibility in appropriate sickle cell disease cases. Care plans are personalized, combining crisis management, hydroxyurea therapy where appropriate, and preventive strategies.
Common questions about Sickle Cell Disease
Medically reviewed by Dr. Amarnadh Polisetty, MD, DNB (Clinical Haematology)
Last reviewed on 1 July 2026
Living with sickle cell disease or trait?
For pain crisis management, ongoing care, or genetic counselling, consult a clinical haematologist for a personalized care plan.