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360-degree plan for persons suffering from sickle cell disease

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By Prof Dr Tulika Seth

The Prime Minister has cast a spotlight of hope for the large population of people suffering from sickle cell disease in India. The recent budget announcement by Nirmala Sitharaman, our Finance Minister, and the new proposed scheme for Sickle Cell Disease awareness and screening has given a huge boost to the patients suffering from this disease. Sickle cell anemia is a type of anemia which is not due to deficiency of iron, but is caused by a genetic mutation. In this blood disorder, the usual rounded red blood cells get distorted into a “sickle” shape under times of stress. This change makes the red blood cells rigid and leads to blocking of small blood vessels causing severe pain crises. The blood cells also become fragile and break easily. This results in anemia and an inflammatory state due to the free hemoglobin from the broken blood cells. All of these factors contribute to the problems seen in the sickle cell patients, like stroke, which can sometimes occur even in young children. Children and adults with sickle cell frequently may suffer from painful crises. These occur at any time but can be precipitated during examinations, or other stressful events. These attacks of pain are the hallmark of the disease. The painful episode is due to blockage of blood vessels and contributes to the disability seen in such patients.  Persons with sickle cell disease are also more prone to infections.

Many sickle cell patients live in tribal and remote areas and remain undiagnosed and untreated. Even the non-tribal community is affected to some extent. The situation for patients has been steadily progressing, largely due to the NHM programmes for hemoglobinopathy. Now, with increased awareness and the important steps taken by the Ministry of Tribal Affairs and the Ministry of Health and Family Welfare, the scenario is further improving rapidly. The convergence of governance to tackle this problem is heartening. This has provided a boost to doctors and healthcare providers caring for patients with sickle cell.

Early diagnosis ensures that effective treatment can be started to control and manage the problems caused by sickle cell disease. The need of the hour are multi-pronged awareness activities to ensure that patients, their families and health care providers can avail of the new opportunities of correct diagnosis. Oral medicines are available which modify the disease and immunisations are available which reduce the risk of infections. Now, access to treatment needs to be provided at the very last mile to ensure compliance.  Augmentation of the blood banks and clear guidelines for the indications for different types of transfusion support along with adequate training will be effective. All the different levels of our health care system are well equipped to deliver the recommended medical care needed. A hub and spoke model with clear referral guidelines can ensure better patient care and safe, timely referrals to higher centres for necessary therapy are feasible.

The question is, can the disease be prevented? The reply is in the affirmative. Sickle cell disease can be prevented easily if screening is done in time and the couples are counselled about their options. Basically, Sickle Cell is a genetic condition. If both parents have sickle cell traits, also known as sickle cell carriers, they themselves are healthy but they have a 25 percent chance of having a baby who has sickle cell disease. Timely screening and counselling can provide couples with risk factors, the correct information and a choice. Awareness about a person’s carrier status is a vital piece of medical information, just like knowing if you have a family history of diabetes or high blood pressure. This information can be obtained by a simple blood test.

The problem of sickle cell disease is present all over India with 17 states reporting a large magnitude of population affected and additional states with pockets of affected patients. Additional Centres of excellence in all the states will be crucial to provide support for training, molecular labs and prenatal testing of couples who choose testing. The Department of Hematology, AIIMS, New Delhi, and other AIIMS can play an important role in delivering expert medical care for sickle cell disease patients requiring tertiary care management of complication and can support training. The rapid innovations in the field of sickle cell disease need to reach our patients and this will require concerted and dedicated efforts of not only the Ministry of Health but also Ministry of Tribal Affairs and other Ministries as well. The care of sickle cell disease in tribal patients is an important and much needed step towards health equity.

(Prof Dr Tulika Seth is a Professor at Department of Hematology at AIIMS, New Delhi.)