Deccan Chronicle

Early diagnosis can treat sickle cell disease effectively

Deccan Chronicle. | DC Correspondent

Published on: March 10, 2023 | Updated on: March 11, 2023
Prof. Dr. Tulika Seth, Professor at Dept. of Hematology at AIIMS, New Delhi     By Arrangement

Prof. Dr. Tulika Seth, Professor at Dept. of Hematology at AIIMS, New Delhi By Arrangement

The Hon’ble Prime Minister Narendra Modi has shined a spotlight of hope for the large population of people suffering from sickle cell disease in India. The recent budget announcement by Smt. Nirmala Sitharaman, our hon’ble 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, this occurs 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 are affected to some extent. The situation for patients has been steadily progressing, largely due to the NHM programs 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 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 the new opportunities of correct diagnosis. Oral medicines are available which modify the disease, immunizations 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 centers for necessary therapy is feasible. 

The question is, can the disease be prevented? The reply is 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 are sickle cell trait, 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 Centers 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 Dept. of Hematology at AIIMS, New Delhi.

Latest News
Most Popular