Combating sickle cell anaemia in Chhattisgarh

In the fight against the genetic disorder in the State’s Jashpur district, local interventions to take healthcare services to the last mile and introduce peer-supported patient networks are bringing hope amid challenges in overcoming stigma

For 22-year-old Anshul*, pain has been an unwelcome companion ever since he was diagnosed at a tender age with sickle cell anaemia, or sickle cell disease (SCD), a genetic disorder that causes red blood cells to become rigid and sickle-shaped, which can block blood flow and lead to health complications. The discomfort often clutters his thought process but his mind invariably travels to his maternal grandmother, who awaits his return to their village in Chhattisgarh’s Jashpur district.

Anshul had to quit studies after school due to frequent visits to hospital and lack of financial support after his parents died. His deteriorating health made it impossible for him to hold on to the odd jobs he did for sustenance. “Neglect by my father, who consumed liquor possibly to deal with the sickle cell anaemia he was afflicted with, marked my past. On this hospital bed, I dread my future but am more worried about what will happen to my grandmother if I cannot go home again,” he says at the Jashpur District Hospital.

Facing similar struggles is a 13-year-old girl at the community health centre (CHC) in Bagicha, 90 km from Jashpur Nagar, the district headquarters, 480 km north-east of the State capital, Raipur. With a protruding cranial bone and swollen eyes, she has lived with SCD since the age of six. “I stay indoors when I am at home as I am embarrassed to be with other children,” the girl says, adding that she had to drop out of school due to the pain and inability to concentrate.

Over 62% of the district’s population is constituted by two communities — Pahadi Korva and Birhor, categorised as particularly vulnerable tribal groups (PVTGs). With SCD or its trait often carried by people from rural and impoverished backgrounds, the PVTGs were the main reason why Jashpur became the first district in the State to screen more than its targeted population of 6,93,393 under 40 years for SCD. District authorities screened 7,30,110 people out of the total population of 9,54,561 (2011 Census). While 5,873 people were found with sickle cell trait, 507 were diagnosed with SCD, of which 270 were women.

According to official data, 179 of those suffering from SCD are undergoing treatment with hydroxyurea, a drug that reduces the need for blood transfusion. The Chhattisgarh government undertook large-scale screening in other districts too. A recent official statement stated, “Against the targeted 1,77,69,535 sickle cell screenings in 33 districts of Chhattisgarh, 1,11,06,561 have been done. During the process, 2,90,663 carriers were found and 22,672 diagnosed with the disease.”

Officials said the “unprecedented mass exercise” would help the government maintain periodic data and document the battle against SCD.

Replicating the Surguja model

Chhattisgarh’s Surguja district, adjoining Jashpur, designed a strategy to combat SCD a year before Prime Minister Narendra Modi launched the National Sickle Cell Anaemia Elimination Mission (NSCAEM) at Shahdol in Madhya Pradesh in 2023. The national programme was aimed at making India SCD-free by 2047.

The NSCAEM broadly defines sickle cell anaemia as a disease that “not only causes anaemia but also pain, reduced growth, and affects many organs like lungs, heart, kidneys, eyes, bones, and the brain”. The most common symptoms are joint pain and fatigue, but they can vary. Necrosis, osteoporosis, and paralysis also set in as the disease spreads.

The Jashpur administration adopted the Surguja model to take healthcare services to the last mile and introduce peer-supported patient networks to tackle SCD. It also roped in Sangwari, an NGO that has been coordinating with the Surguja administration for sickle cell management.

“While there are no national studies to estimate the number of patients, a field study near Raipur suggests there is at least one or at most three SCD patients per 1,000 people in Chhattisgarh. This translates into 30,000 patients among 3 crore people,” says Dr. Yogesh Kalkonde, a public health expert associated with Sangwari.

If the findings of the study are extrapolated, about 10% of the State’s population is AS, the genotype for sickle cell trait, Dr. Kalkonde explains. AS is the genetic condition that occurs when a person inherits a gene for normal haemoglobin (A) and a gene for sickle haemoglobin (S). Post-screening confirmatory tests establish whether a person is sickle cell negative, has sickle cell trait (AS), or is afflicted with sickle cell (SS).

A team of coordinators from Sangwari has been working in Jashpur since May, acting as a bridge between the government and patients. In the process, it is discovering new challenges and complexities of the disease. The interactions between patients and counsellors reveal how deeply the disease impacts people and how combating SCD goes beyond medical care.

Biswajeet Singh, a Sangwari coordinator from Surguja, shares a unique case. “This involved a man experiencing prolonged penile erection caused by abnormal blood flow due to SCD. Doctors, unaware of the symptoms, were stumped by the severity and even recommended surgery. It was only when we intervened that his sickle cell tests were conducted and treatment began.”

Counselling strategy

According to Biswajeet, calling people for counselling to the CHCs instead of the district hospital was a major step in the fight against SCD. “People earn very little here. A visit to the district hospital nearly 100 km away costs ₹200 and means missing out on the daily wage of about ₹300. If they reach after the out-patient departments have closed, the whole day is wasted, and they lose motivation.”

The strategy of drawing patients to the CHCs has helped create a peer-supported community network. “We keep telling them that we won’t be here always and they will have to look after themselves. In Surguja, we conducted a conclave for about 400 patients. They now exchange notes and everyone gains,” Biswajeet says.

Dr. Kalkonde says doctors cannot do the heavy lifting in cases like SCD, where patients have to live with the condition, but a little nudge helps. “Our observation is that people are much more receptive when they find some help from the government system. It motivates them,” he says.

Another significant intervention Dr. Kalkonde speaks of is the use of hydroxyurea in districts like Jashpur and Surguja. “In Chhattisgarh, there is a good supply of hydroxyurea, a mild agent with minimal side effects. Though doctors were scared of using it in the past, it reduced patient admission and blood transfusion by nearly 50% in Surguja. We are replicating that model in Jashpur. We have created charts that will help doctors detect symptoms and recommend hydroxyurea.”

The drug, widely known for its use among cancer patients, has been in use for SCD only for the past year or so, says Dr. R.N. Dubey, a paediatrician at the Bagicha CHC for the past 30 years.

The patient base has increased due to awareness and the number of people “asking for blood transfusion” has decreased, says Laxmikant Apat, a senior doctor at the Jashpur District Hospital. “Earlier, people would ask for blood transfusion even if they felt the slightest discomfort. In Jashpur, availability of blood is low in the blood bank and frequent transfusions will prevent proper diagnosis and even lead to side effects.”

‘Stigma: a double-edged sword’

Mohan*, 21, a patient at the Jashpur District Hospital, says using hydroxyurea has helped him prevent transfusion. Although he feels healthier, he notes the stigma surrounding SCD doesn’t disappear. “Unlike my friends and most people my age, I cannot think of marriage and starting a family because I am a sickle cell patient,” he says.

Devesh Singh, a Sangwari coordinator from Jashpur, explains that stigma is a double-edged sword for counsellors. “On the one hand, we have to actively dissuade carriers from getting married [to other carriers], but on the other, we have to create awareness among healthy people that they can marry carriers,” he says.

The NSCAEM guidelines suggest “accepting the reality” and ensuring that two people getting married are neither sickle cell carriers nor have SCD. “If one of them is a sickle cell carrier, or has SCD, then their children won’t have the disease. But there is a possibility of them being sickle cell carriers. So, it would be appropriate to get one’s blood tested at a hospital and choose a suitable life partner,” the guidelines state.

During counselling sessions, newly married couples, older couples with children, teenagers — all come in, some carrying the trait, some suffering from the disease, and some feeling its after-effects more severely than others. What is common though is that most patients are from the Other Backward Classes, Scheduled Castes, or Scheduled Tribes, lending credence to claims that SCD predominantly affects marginalised groups.

A staff nurse at the CHC, however, points out that this could also be due to Bagicha development block’s demographics. Later, during a visit to the district hospital, it becomes clear that SCD is not limited to vulnerable groups.

Despite claims that screening accelerates the fight against SCD, a visit to the CHC reveals a mixed picture. While many patients were already receiving treatment, others sought care after being tracked down by Health Department officials and counsellors. Significant shortcomings in the health system also came to the fore. A critical gap is the unavailability of reagents for blood solubility tests, a crucial confirmation test post-screening, at both the CHC and the district hospital, effectively impacting the entire district. Such gaps result in delayed diagnosis and treatment.

While sickle cell anaemia may have originated in central India, it wasn’t before 2016 that an effort to combat it in an organised manner was carried out in Chhattisgarh by the Jan Swasthya Sahyog (JSS) — a voluntary, non-profit, registered society of health professionals running a low-cost health programme in the rural areas of Bilaspur.

JSS caters to a large number of patients, including those with SCD, from districts in Madhya Pradesh such as Shahdol and Anuppur. According to Dr. Kalkonde, even if district hospitals in areas like Bastar and Surguja tried to tackle SCD, lack of accessibility kept patients away.

Yogesh Jain, founder of Sangwari and co-founder of JSS, points out that NSCAEM has disproportionate emphasis on preventing new patients by stopping marriages rather than providing existing patients what they need, and often these resources are available within the State’s healthcare system but not properly utilised.

Call for quick action

Jashpur District Collector Ravi Mittal acknowledges the challenges but emphasises the need for quick action. “Sickle cell anaemia remains undetected especially among remote communities in a district like Jashpur. This increases the chances of transmission and lack of treatment. We wanted to complete screening fast to gauge the problem’s extent, ensure marriage counselling, and provide personalised support to patients and carriers. Challenges of resources remain, but a multi-pronged strategy involving patients and the larger community will help optimise existing infrastructure and produce better results,” he says.

(*Names changed to protect privacy.)

Published - October 12, 2024 07:01 pm IST