BANGALORE: It’s nothing but the triumph of the will to live in the face of a debilitating morbidity. Fourteen-year-old Nidhi goes to school not just with her schoolbag. She carries along a portable ventilator and a suction machine by her wheelchair. It’s after a long struggle that she has managed to get into a regular school and then get them to install a lift there for her.
Nidhi has one of the rarest genetic disorders which also happens to be one of the costliest. She has been diagnosed with Pompe disease that results in debilitating and progressive muscle weakness because of a defective gene she has received from one of the parents. The disease is life threatening and the treatment is ERT (enzyme replacement therapy), an expensive procedure.
Nidhi is not alone in her struggle. Her mother, Sharada Shirol, sits beside her in the classroom through the day as her daughter listens to the teacher intently. Her father, Prasanna Kumar Shirol, has quit his job at Reliance to attend to his daughter’s needs.
The girl was normal when she was born. The first symptoms started when her parents realized that she could not crawl or stand up on her own. The initial diagnosis was myopathy, a muscular disease. Her parents took her to almost 45 hospitals in India before she was diagnosed with Pompe was in Davanagere in 2007.
It has been a struggle since then. She is wheelchair bound, has to be on a ventilator at night and has tracheostomy where an artificial airway has been done to help her breathe. She developed resistance to enzyme therapy and had to go through an immuno-suppression costing Rs 12 lakh.
For Prasanna and Sharada it has been a relentless fight to keep their precious one alive. “Why should we give up when she’s fighting? Every time she goes to hospital, she exercises and finds a nerve for the injection. She has never cribbed. Many people have told me that I am fighting nature. But when she wants to live, how can we sit back?” asks Prasanna.
The couple’s social life is nil. They have limited visitors at home, as Nidhi’s immunity levels are low. Hangouts are restricted to malls, that too on rare occasions. “She has heard so much about Wonderla that she wants to visit it. We have promised her a visit this vacation,” he said. Nidhi’s only pastime is painting and she can do it endlessly.
It has been one disaster after another that the Shirols are handling. Nidhi has now developed Scoliosis because of being continuously confined to the wheelchair. Her spine is S shaped, and requires 35 screws to be re-aligned – that too at the risk of losing her post this procedure.
OUT OF RESOURCES
And the parents have run out of resources. “The medicines cost Rs 85 lakh per annum for her body weight of 25 kg. Thankfully it is being sponsored by a pharmaceutical company under their Charity Access Programme. But that is not all. We spend around Rs 30,000 to Rs 40,000 per month for her supportive care. The Sciolosis surgery will cost a minimum of Rs 10 lakh and we are running short of funds,” he said.
Nidhi is the couple’s only child. Sharada had conceived again but the pregnancy was terminated after pre-natal tests showed that the second baby too was affected by the disorder.