Healthcare News

Sickle cell disease in kids: Early detection, intervention, long-term management | Health


Sickle cell anaemia is a serious health problem that affects millions of people in India where in the Indian belt, which includes the states of Gujarat, Maharashtra, Chhattisgarh, Orissa and some of Bengal, sickle cell anaemia is most common while there are also areas in Tamil Nadu, Kerala, the South and Telangana that are affected by it. According to the Indian Journal of Medical Research, India is home to over 20 million patients with sickle cell disease but even though this inherited blood illness is so common, it is still largely unaddressed.

Sickle cell disease in children: Early detection, intervention and long-term management (Photo by Twitter/EllenDomaeko)
Sickle cell disease in children: Early detection, intervention and long-term management (Photo by Twitter/EllenDomaeko)

In an interview with HT Lifestyle, Dr Santanu Sen, Consultant, Paediatrics, Paediatric Hematology, Oncology and Stem Cell Transplantation at Kokilaben Dhirubhai Ambani Hospital in Mumbai, explained, “Sickle cell disease is a hereditary blood disorder that parents pass on to their children. The red blood cells’ irregular shape, which resembles a sickle and gives sickle cell disorders their name, causes the symptoms of the condition. Because of their sickle shape, red blood cells become stiff and sticky and become trapped in the body’s tiny blood veins as they circulate. This interferes with the flow of oxygen to various body areas and causes symptoms including anaemia, flare-ups of pain from bone and joint degeneration, swelling in the extremities, an elevated risk of infection, inhibition of normal growth, and eyesight issues.”

He elaborated, “Sickle cell disease is a hereditary blood disorder that can be detected through newborn screening or prenatal testing if there is a family history. Symptoms may appear as early as 2-5 months of age and, if left untreated, can lead to serious health problems and even death. Effective management and treatment are crucial for reducing complications and improving quality of life. The only cure for sickle cell anemia is a stem cell transplant, which is a high-cost and complex treatment in India. Screening is a crucial tool in preventing this disease, and the government has a critical role to play in increasing awareness and providing access to screening.”

According to him, one of the biggest challenges in addressing sickle cell anemia in India is the lack of awareness among the general population. Dr Santanu Sen suggested, “If the disease is prevented in the current generation, it can be prevented from passing on to the next generation. This highlights the need for increased education and awareness about the disease, especially in tribal areas where the burden is highest. Sickle cell anemia is a pressing health issue in India that requires immediate attention. The Union Budget of 2023 also focused on the elimination of Sickle Cell Disease potentially by 2047. To tackle this issue, the government and private hospitals must work together to make this treatment more accessible, affordable and available to those in need.”

Bringing his expertise to the same, Dr Vipin Khandelwal, Consultant Hematology, Hemato-Oncology, Pediatric Bone Marrow Transplant Physician at Apollo Cancer Centres in Navi Mumbai, shared, “Sickle cell disease (SCD) is a hereditary blood disorder that primarily affects children. It is characterized by the presence of abnormal hemoglobin molecules in red blood cells, causing them to become rigid and sickle-shaped. This can lead to a range of complications, including chronic pain, organ damage, and increased susceptibility to infections. Early detection, intervention, and long-term management are crucial in improving the quality of life for children with SCD.”

He revealed, “Early detection plays a vital role in managing SCD in children. Newborn screening programs have been implemented in many countries, allowing for the identification of affected infants shortly after birth. This early diagnosis enables healthcare providers to initiate appropriate interventions promptly. Regular screenings throughout childhood help monitor the progression of the disease and identify any complications or coexisting conditions that may arise. Interventions for children with SCD involve a multidisciplinary approach. Pediatric hematologists, nurses, psychologists, and other healthcare professionals work together to provide comprehensive care. One of the primary interventions is ensuring proper hydration to prevent dehydration, which can trigger sickling of red blood cells. Children are encouraged to drink plenty of fluids, particularly water and avoid excessive physical exertion or exposure to extreme temperatures.”

Calling pain management another critical aspect of SCD intervention, Dr Vipin Khandelwal said, “Children with SCD often experience acute and chronic pain episodes known as vaso-occlusive crises. Prompt administration of pain medications, such as nonsteroidal anti-inflammatory drugs (NSAIDs) or opioids, helps alleviate pain and improve the child’s overall well-being. Additionally, the use of heat packs, massage therapy, and distraction techniques can complement pharmacological interventions in managing pain. Long-term management of SCD in children focuses on preventing complications and maintaining overall health. This involves regular check-ups with healthcare providers, including hematological assessments and monitoring of organ function. Children may also receive vaccinations to protect against infections, as they are more susceptible due to functional asplenia, a condition where the spleen does not work properly.”

He pointed out, “Hydroxyurea, a medication that increases the production of fetal hemoglobin, has shown significant benefits in reducing the frequency and severity of vaso-occlusive crises in children with SCD. It is often prescribed in conjunction with other treatments and requires careful monitoring of blood counts and potential side effects. Education and psychosocial support are essential components of long-term management. Children and their families should receive education about SCD, including understanding the disease process, recognizing symptoms of complications, and adhering to treatment plans. Psychosocial support, such as counseling or support groups, can help children and their families cope with the emotional and social challenges that come with living with a chronic illness.”


Source link