When genes wield a sickle

Kumar was travelling for work in 2016 when he received a call from his daughter’s school. An urgent voice on the other end informed him that the six-year-old had fell unconscious.

Far away from his home in Araku Valley, he frantically called his wife and asked her to rush to the school. It took him a few hours to reach the school, with worry gnawing through his heart.

He found that his daughter’s legs had swelled and lumps had formed on several parts of her body. With no major medical facilities available in the tribal area, a frightened Kumar decided to take his daughter to King George Hospital (KGH) in Visakhapatnam, about 120 km away. It was at the hospital that he first came to know about the malady afflicting his child, a name that has not left his mind since: Sickle Cell Anaemia.

“We were unaware of the disorder then, but I have since enquired about it and learnt that there are some more cases in Ananthagiri, Pedabayalu and Paderu. In some cases, children have even died,” says Mr. Kumar, stress and fatigue weighing him down. He later took his daughter to a retired paediatric doctor in Visakhapatnam, who, he says, had a vast experience treating such children. The child underwent treatment for over two weeks, and Kumar, his wife and the rest of the family were told to get tested. The results showed that Kumar and his wife were ‘traits’, which means they are in safe zone and asymptomatic.

“Jaundice is the most common health issue she suffers. If she gets stressed owing to studies, games or any other reason, she develops swelling on her body, joint ache and fever among other issues. Sometimes, there is a drop in platelets, and we need to monitor blood levels regularly,” Kumar explains.

Even a change in temperature triggers health complications in a person with Sickle Cell Anaemia, he says. During November-February, when temperature drops to single digits in the Agency areas, his now 15-year-old daughter battles with breathing difficulties in the mornings.

“Every month, we go to Visakhapatnam from Araku Valley for health check-up. We had even tried taking her for advanced treatment in Hyderabad. Once one develops this disorder, the patient needs lifelong treatment,” says Kumar, who is a pastor.

According to sources, a number of children in the Agency areas of Alluri Sitharama Raju district suffer from Sickle Cell Anaemia, a genetic blood disorder in individuals who inherit mutated haemoglobin genes from their parents. It causes the red blood cells to take the form of a crescent or a sickle. These misshapen cells have a shorter survival rate and can clog small blood vessels, leading to anaemia, severe pain and other major health complications.

Kumar says he and his wife are constantly worried about their daughter. “We are tense till she returns home from school. We had even submitted a representation to the school, requesting the management to call us even if it’s a small health issue,” he says.

K. Praveen, another resident of Araku Valley mandal, recalls how he and his sister were diagnosed with Sickle Cell Anaemia in 2010 when they were around 15 years old. He becomes emotional when he talks about how she suffered for 15 years before passing away in September last. “It cost us about ₹25,000 a month for her treatment and diet. She often suffered joint pains, high fever and jaundice; PMS added to her health problems every month,” he recalls.

The disorder has become a challenge faced by several tribal communities in the district, and each year, a significant number of new cases are diagnosed.

Blood relation

District Medical and Health Officer Jamal Basha says consanguineous marriages are the primary reason why the condition is prevalent among tribespeople. Individuals in the communities continue to marry their blood relatives as part of their tradition. This leads to persistence of the disorder in the next generation. Nutritional deficiencies, especially a lack of essential vitamins, minerals and iron in their diets, exacerbate the condition, making it harder for the affected children to fight the symptoms, he adds.

Jamal Basha says tests are being taken up throughout the year to find the prevalence of Sickle Cell Anaemia among schoolchildren and pregnant women. A massive drive was organised a year ago to find adolescents with the condition. “Over the past two years, we screened about 4.01 lakh cases, of which 12,595 cases came out as ‘traits’. As of now, there are about 1,372 positive cases [individuals with Sickle Cell Anaemia],” the DMHO says.

For mild and moderate cases of anaemia, the department provide the patients with iron-and-folic acid tablets, and their condition is monitored time to time through tests. Severe cases should be treated more carefully. They are given iron sucrose and monitored regularly. Some times, the patients require blood transfusion, he says.

“Earlier, severe cases used to be handled by the KGH in Visakhapatnam. Now, however, the government has made diagnosis available at Government Paderu Hospital. We have physicians and testing equipment such as HPLC at the hospital. However, we shift critical patients who require blood transfusion to the KGH,” he adds.

Poverty loop

About two years ago, the State government introduced a scheme to provide a financial assistance of ₹10,000 to the families of patients, which, according to many parents, is insufficient.

Anil, a poultry farmer from Pedabayalu, says it costs him around ₹20,000 a month to take care of his 12-year-old daughter, who suffers from the condition. The money goes into buying nutritious food, medication and travel.

Anil, who gets his daughter treated at a private hospital in Visakhapatnam, says he spends two days every month for her treatment. “We include a lot of vegetables, mutton and dry fruits in her diet. Daily medicine should never be missed,” Anil explains, adding that his income from the farm is insufficient to meet the expenses and that he often borrows money.

Many parents also turn to various doctors in Telangana and Maharashtra, seeking advanced care, the costs of which are making them poorer by the day.

Impact on childhood

Satish Kumar, a resident of Hukumpeta who has a son suffering from the disease, throws light on the devastating effect the disorder has on childhood. “Children are under a lot pressure from a young age… My son wants to play and have fun with his friends but is unable to do any of that. He is not fully aware of his condition but knows he has some health issue,” he says.

Intergenerational problem

“Sickle Cell Anaemia has been ravaging tribal communities for decades. Though the government has conducted tests and provided medication, no proper research has been taken up. There has to be a permanent solution, now that ASR district has a medical college,” says Killo Surendra, a member of A.P. Girijana Sangham and a resident of Araku Valley.

“Though the government has conducted tests and provided medication, no proper research has been taken up. There has to be a permanent solution, now that ASR district has a medical college”Killo SurendraA member of A.P. Girijana Sangham

Surendra also opines that more awareness should be spread among the tribespeople about the disease. It is vital that the communities that bear the brunt of the disorder receive more support so that they can fight it and ensure a better future for their children.

Jamal Basha says the department has been spreading awareness about the disorder through posters and pictures on RTC buses, at weekly shandies and through ANMs and ASHA workers. This apart, awareness programmes are held at students hostels as well.

A Senior Health Assistant from Alluri Sitharama Raju district, who did not want to be quoted, claims that many tribal families in the interior areas of the district are unaware of the condition and lack access to proper healthcare. Owing to a delayed diagnosis and treatment, the consequences are often devastating. “In some cases, early deaths happen, but this does not come to light,” he adds.

Rama Rao Dora, a tribal activist from Paderu, says Sickle Cell Anaemia deals a crushing blow to many tribal families who already live in poverty. “There have been cases wherein people ignored it, leading to fatalities. The government should regard those with the condition as special cases and come up with more measures to help them. Testing centres and doctors who can treat the condition should be made available in the interior areas as well,” he adds.