Sib Sankar Chowdhury and his wife Debjani Chowdhury are worried about their 12 year old son Arian Chowdhury who is suffering from an almost incurable disease known as Mucopolysaccharidosis type II (Hunter Syndrome). Arian was diagnosed with Hunter Syndrome at the tender age of four. It is a rare genetic disorder and around 2000 people worldwide have been diagnosed with this disease. This disease slowly affects the entire body and life expectancy is drastically reduced.
The treatment for this disease is called Enzyme Replacement Therapy which will be given as Intravenous injection and needs the drug ELAPRASE which is available with only one company – Shire HGT, an American firm.
Speaking to AV, Sib Sankar Chowdhury said, “We are a middle class family and it is impossible for us to afford the treatment for my son’s disorder as it is prohibitively expensive. Approximately, 88 lakhs to 1 crore is the estimated annual expenditure for my son’s treatment.”
Arian was nearly four years old when he was diagnosed with Hunter Syndrome. At that time, there was no recognised treatment available for this disease.
Around 2006, Elaprase was approved by FDA for ERT in Hunter Syndrome patients. However, Arian’s parents have not been able to procure those medicines as it is very expensive. Sib Sankar Chowdhury has started a petition to save his son on the website www.change.org. Netizens can sign this petition and support this cause.
Elaprase can prove to be highly beneficial for Hunter Syndome patients. It has in fact been called as one of the most expensive medicines ever created. This medicine is the only hope for Arian now.
Developed by Shire Human Genetic Therapies Ltd, the medicine Elaprase (Idursulfase), was approved by the US Food and Drug Administration as an enzyme replacement treatment for Hunter syndrome. This life-saving drug is at present unavailable in India. It can be imported by seeking prior permission from Drug Controller General of India. A weekly dose of this drug will cost around 2 lakhs. Considering that the drug is to be administered on a regular basis and for a long-term, the cost is estimated to run into several lakhs, probably even running into a crore.
There is no assurance that Arian’s life can be rescued but it definitely increases his chances of survival and that’s what his parents are expecting.
What is Hunter Syndrome?
Hunter syndrome is a lysosomal storage disease caused by a deficient (or missing) enzyme. In simple words, it is a serious genetic metabolic disorder.
It interferes with the body’s ability to break down and recycle specific mucopolysaccharides, also known as glycosaminoglycans or GAG. Thus, GAG builds up in cells throughout the body and interferes with the way certain cells and organs in the body function, eventually leading to a number of serious symptoms.
Hunter syndrome is always severe, progressive, and life-limiting.
Symptoms of Hunter syndrome may not be visible immediately at birth, but start becoming noticeable after the first year of life or even later. In Arian’s case, symptoms emerged after he turned four. What started as a casual checkup for a mild bout of wheezing turned out to be a nightmare for his parents.On a planet with 7 billion people, around 2000 people are afflicted with Hunter Syndrome. Of these approximately 500 are in the United States, 30 in Canada, 2 in New Zealand, and few others exist in various countries.
You may contact Arian’s father Sib Sankar Chowdhury on this number:
Mobile: +91 9831361116
Email: sibsankar.chowdhury@gmail.com
Mother’s name: Debjani Chowdhury
Address: 19, Dharmadas Kundu Lane, Shibpur,
Howrah – 711 102
West Bengal, India
