|(Top) Sagar Guin when he was 17 years old; and now as a patient. Picture by Pranab Debnath
A neuro-degenerative disorder with genetic roots has taken the lives of nine persons in a family in a span of 50 years and at least four other members are struggling with the ailment at present.
Doctors mentioned two names of diseases with similar symptoms and said no cure was available for either, a fact that the family of 35-year-old Sagar Guin rues.
Sagar’s mother died of the disorder and three of her cousins are afflicted at present.
Sagar, who lives in Krishnagar, cannot speak or walk. Nor can he see clearly or chew food. The disease has robbed his body and limbs of muscular strength and co-ordination. Once a cricketer and an employee of the Krishnagar district judges’ court, Sagar was forced to take voluntary retirement because of the “incapacitation”.
Sagar was taken to Christian Medical College, Vellore, by his father Mukul Guin first. There he was diagnosed with hereditary spastic paraparesis. Later, doctors at the National Institute of Mental Health and Neuro Sciences (Nimhans) in Bangalore suggested that Sagar suffers from spino cerebellar ataxia: type III (SCA T-III), a neuro disorder that is also genetic.
Mukul Guin, a retired deputy commissioner of sales tax, said Sagar showed the symptoms of the disease when he was 20.
“My wife Anju had suffered from the same disease and died an untimely death. Many members of my wife’s family, who lived in Rabindra Nath Tagore Road (High Street) in Krishnagar town, died after showing similar symptoms of the disease in the past 50 years,” Mukul Guin said.
L.N. Tripathy, the director of Calcutta’s Medica Institute of Neurological Diseases who was asked about both the disorders, said the genetic disorders that are neuro-degenerative have no treatment.
“Such patients can undergo physiotherapy. These diseases are progressive and the rate of progression varies from patient to patient and in the end, patients become completely immobile and unable to move or speak. There is no prevention. Detection can be done by the analysis of chromosome,” said Tripathy.
Both disorders are rare.
Sagar’s mother’s sister Iva Dutta, a retired clerk of the state finance department, has been witness to a procession of deaths.
“My grandfather was the first person to have been noticed with the symptoms of this disease. All his four sons, but one, died from the same disease. I am the daughter of his eldest son. In my generation, six persons, including my three sisters, have succumbed to the disease. One of them was Sagar’s mother Anju. At present, three of my cousins are suffering from the disease. They seem to be approaching death. In the next generation, Sagar is the first victim,” she says.
The disease strikes early, she says. “By the time they reach 40, they are incapacitated and eventually die by 50,” says Iva.
Her cousin Dipankar Dutta, 49, a clerk with Krishnagar municipality, has shown the early symptoms of the disease. “He often collapses on the ground,” says Iva.
The family feels helpless also because they cannot afford an endless diagnosis.
“I have realised what awaits me in the near future. But, like others, I want to live a normal life. I am worried about my son Subhankar’s future. He is a BA first-year student,” says Dipankar.
Mukul Guin has run from pillar to post to get treatment for Sagar.
“Doctors and researchers could not find a way to fight it. Now at 65, I feel depressed and have lost the hope to live,” he says.
“I have lost my wife and many of my in-laws and now I am on the verge of losing my son. Recently, I came to know about stem cell therapy which is being experimented at the School of Tropical Medicine in Calcutta, but later found out that it can provide little relief to stiff muscles,” he says.
Professor Anup Kumar Bhattacharjee, a neurosurgeon and the head of the department of neurology at Mata Gujri Memorial Medical in Kishenganj (Bihar), thinks the disease said the “symptoms of the disease indicate Sagar and members of his family have been suffering from a degenerative neurological disorder in which, the function or structure of the affected tissues or organs deteriorates increasingly over time”.
He said it was “commonly identified as spino-cerebellar ataxia”.
Bhattacharjee said ataxia was of several types, quite different in nature, and no way of treatment had been found for it so far.
“I have personally analysed the chromosomes of such patients, but failed to make any breakthrough. As of now, no evidence of remedial treatment of such diseases could be found in the medical journals.”
He advises rehabilitative treatment and counselling for such patients. Bhattacharjee, like some other neurologists, thinks that such patients shouldn’t have biological offspring and should adopt children.