The fight against sickle cell disease: how one hospital in rural Maharashtra is making a difference

Sickle cell disease shortens your life by at least twenty years. Slow-progressing organ failure is common in older patients, and acute chest syndrome, a form of lung injury, is a significant cause of death among the young. The disease’s symptoms vary widely among patients, leading to a wide range of complications

I know sickle cell disease (SCD) less from the medical textbooks and more from the suffering of patients I’ve treated. Over time, this disease has ceased to be just a diagnosis on a chart. It has become something I deeply despise from the core of my being. It has drawn many of my young, tender-looking patients into painful crises, causing me to cry out in desperation, hoping to see it eradicated — though I know that’s just a daydream. Because it’s a genetic illness with no definitive cure to date.

But desperation and hopelessness cannot be the final word. In Agatha Christie’s words, “I like living. I have sometimes been wildly, despairingly, acutely miserable, racked with sorrow, but through it all, I still know quite certainly that just to be alive is a grand thing.” For the sake of patients, the fight continues, no matter the adversity. We cannot lose.

The brutality of the disease

I have witnessed first hand the plight of young people afflicted by sickle cell disease at three mission hospitals. Patients come in with intense pain, high fever, chest infections, or severe anaemia, needing blood transfusions. Whether in southern Odisha, north Maharashtra, or western Uttar Pradesh, the patients are almost always poor, young, rural, and from the same family.

Sickle cell disease shortens your life by at least twenty years. Slow-progressing organ failure is common in older patients, and acute chest syndrome, a form of lung injury, is a significant cause of death among the young. The disease’s symptoms vary widely among patients, leading to a wide range of complications from anaemia due to the destruction of blood cells to blockage of blood vessels in limbs, causing both severe pain and death due to organ failure. 

Practising medicine is generally hard, but over time, you learn to trust in your skills, hoping that drugs and interventions will relieve suffering. However, sickle cell disease has the reputation of defeating even the most courageous healthcare professionals. The disease often laughs at our best efforts and stops at nothing short of taking precious young lives with its painful and choking grasp.

Doctors on the frontlines

I attribute much of my knowledge about managing this disease to Dr. Ashita Singh, with whom I worked at Chinchpada Christian Hospital in Maharashtra. She has seen this dreaded disease up close and has not shrunk back in horror, but has loved her patients, looking for ways to relieve the suffering of patients and improve their quality of life.

She has dealt with several practical challenges in the management of the disease. 

Since sickle cell disease is a genetic condition, its treatment is lifelong. Only gene therapy or bone marrow transplant can cure it, but these treatments are out of reach for many in India because of the costs. But Dr. Ashita, who works in a resource-poor setting, has moved mountains to introduce measures that can help control the disease’s symptoms, making it bearable for the poor patients who receive treatment from her. Her hard work has upheld the health and dignity of many of her patients through various hospital initiatives that deal with the reality of living with sickle cell disease.

Chinchpada Hospital’s initiatives

One of Chinchpada Hospital’s most significant initiatives is that the hospital secured State Blood Transfusion Council (SBTC) cards for around 250 of the 350 sickle cell disease patients. These took two years to procure. The cards guarantee that patients will receive free blood transfusions across Maharashtra, a vital service that can be lifesaving for those suffering from severe anaemia.

Recognising that sickle cell disease patients are highly prone to infections like bacterial pneumonia, influenza, and meningitis, the hospital has also provided these patients free preventive vaccinations for all these diseases. This challenging but crucial task will prevent patients from developing life-threatening infections.

The hospital has also subsidised the cost of hydroxyurea, a drug that reduces the defective haemoglobin entity (Hb S) and increases normal haemoglobin by using hospital funds and donations. Hydroxyurea is a drug that reduces the number of times a patient has worsening symptoms or life-threatening crises. It also minimises the need for blood transfusions. Subsidising this drug has significantly improved patient compliance, as patients can afford to take it for as long as their disease requires, increasing their quality of life. The government, too, should recognise hydroxyurea’s benefits and make it cost-free.

The hospital also works to enrol SCD patients in government disability schemes, ensuring they receive regular pensions and other essential benefits. With the help of donors and well-wishers, the hospital treats life-threatening infections and anaemia episodes at low costs or even writes off bills. They also provide free food to patients and their families, thus meeting their nutritional needs during treatment, especially during extended hospital stays. The hospital’s experience in palliative care has been instrumental in providing effective pain relief and symptom management for SCD patients.

The hospital organises events like World Sickle Cell Day and sends personalised birthday cards to patients. These efforts help patients feel valued and supported, reinforcing the hospital’s commitment to their well-being.

On a community basis, the hospital conducts awareness programs in villages and schools to educate the community about SCD, its symptoms, and the importance of early diagnosis and treatment. Teachers, some of whom are patients of the disease, play a vital role in identifying children with symptoms and ensuring they receive appropriate care.

The hospital opposes policies promoting the diagnosis of sickle cell disease in a child during pregnancy, which might lead to abortions.

A battle that is far from over

Despite these remarkable efforts, the battle against sickle cell disease is far from over. The disease remains a significant challenge, mainly where medical students and young doctors need adequate training to recognise and treat it. The lack of awareness and testing facilities further exacerbates the problem, leading to many undiagnosed and untreated cases.

My experiences in Uttar Pradesh highlight the widespread ignorance about SCD. Many patients had been receiving iron supplements for years before being correctly diagnosed with SCD. We must work hard to educate, manage, and give hope to these lives because it’s our collective pain.

This message Dr. Ashita sent me after losing a young lady to a recent SCD crisis reminds us why we can’t lose. “Twenty-year-old J had been off hydroxyurea treatment for the last few years and came to the hospital with acute chest syndrome following a severe vaso-occlusive crisis. Despite our best efforts, she passed away, joining her sister, who had died from a sickle cell crisis a few years earlier. This loss, like so many others, compels us to continue our fight against this terrible disease.”

In conclusion, our experience from Chinchpada Hospital shows that our approach to tackling SCD must be comprehensive, addressing not just the medical aspects but also the socio-economic factors perpetuating suffering. We must continue to fight for the lives of those suffering from SCD because every life is worth saving, and we can’t afford to lose this battle.

(With inputs from Christianez Ratna Kiruba)

(Dr. Dawn Kuruvilla is a physician working in a secondary mission hospital at UP-Jiwan Jyoti Christian Hospital at Sonbhadra. He is passionate about bedside clinical medicine and bringing hope and clarity to patients in despair and senseless suffering.)

(Dr. Christianez Ratna Kiruba is an internal medicine doctor with a passion for patient rights advocacy. christianezdennis@gmail.com)

Published - October 04, 2024 06:00 am IST