World Sickle Cell Awareness Day is observed every year on June 19. It is a genetic blood disorder that affects millions across the globe.
On the occasion, Dr Nakul Pathak, consultant in the department of pediatrics and pediatric intensive care at Surya Mother & Child Super Speciality Hospital, shared with HT Lifestyle how the disorder affects individuals, and debunked five related myths.
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According to Dr Pathak, “Sickle cell disease affects haemoglobin, the protein that enables red blood cells to carry oxygen. Healthy red blood cells are soft and flexible, allowing them to pass through narrow blood vessels.”
“In sickle cell disease, some cells become stiff, sticky, and crescent-shaped. These cells can break down prematurely, causing chronic anaemia, or obstruct small blood vessels and restrict the supply of blood and oxygen to tissues.”
In children, the early symptoms of sickle cell may be episodic. A child with sickle cell disease may appear well between episodes, noted the physician. This can make the condition easy to underestimate, even though changes are continuing within the blood.
The doctor went on to debunk five myths about the condition. They are presented as follows.
Myth 1: Sickle cell disease is contagious
Fact: It is an inherited genetic condition and cannot spread through touch, food, coughing, living together, or contact with blood. A child develops the disease after inheriting genes for abnormal haemoglobin from both parents.
Myth 2: Sickle cell trait means a person has the disease
Fact: A person with the trait has usually inherited one sickle cell gene and one normal haemoglobin gene. Most carriers do not experience the symptoms associated with sickle cell disease, although complications may occur in rare and extreme circumstances. They can still pass the gene to their children.
“When both parents have the trait, each pregnancy carries a 25 per cent chance of the child having sickle cell disease,” noted Dr Pathak.
Myth 3: Sickle cell disease only causes pain
Fact: Pain is its most recognisable symptom, but the damage can extend much further. Blocked blood flow can affect the lungs, brain, spleen, kidneys, and eyes. Children may experience persistent anaemia, fatigue, delayed growth, and increased vulnerability to serious infections.
Acute chest syndrome and stroke are among the most dangerous complications. Fever, chest pain, difficulty breathing, seizures, facial drooping, or sudden weakness require urgent medical attention.
Myth 4: Patients asking for pain relief are addicted to medicines
Fact: A pain crisis can be severe, sudden, and disabling. “Research shows that people with sickle cell disease are sometimes unfairly labelled ‘drug-seeking’, which can delay appropriate pain relief,” noted Dr Pathak. Their symptoms require careful assessment and timely medical management.
Myth 5: Treatment is limited to transfusions and painkillers
Fact: Prescription medication such as hydroxyurea can reduce pain crises and acute chest syndrome. However, there are preventive measures that can be taken in early childhood.
Vaccination, penicillin for eligible young children, and transcranial Doppler screening can help prevent infections and identify stroke risk. Stem-cell transplantation can cure selected patients, while recently gene therapies have opened another treatment pathway for some people aged 12 years and above, shared the physician.
“World Sickle Cell Day is an opportunity to challenge the misconceptions that continue to shape how the disease is understood and treated. Pain crises must be taken seriously, sickle cell trait should not be confused with the disease, and families need accurate information about how the condition is passed from parents to children, its warning signs, and the treatments available,” he stated.
Note to readers: This article is for informational purposes only and not a substitute for professional medical advice. Always seek the advice of your doctor with any questions about a medical condition.
