UP: This family has 3 kids with rare genetic disorder

The challenge: Finances are a major problem for Dinesh of Ismailpur village as Rs 4 lakh per month is required for each child’s treatment

lucknow Updated: Dec 07, 2018 14:02 IST
Anupam Srivastava
Anupam Srivastava
Hindustan Times, Lucknow
Dinesh Kumar,Lucknow,Uttar Pradesh
Dinesh’s ordeal began when his first daughter, Ranjana, started developing certain bone and joint issues. Her condition could not be diagnosed for around 5-6 years. (Image for representation)

Life is a challenge for Dinesh Kumar of Ismailpur village near Lucknow. His three children – Ranjana, 12, Kalpana, 9, and Vivek, 6, are suffering from MPS I – a rare genetic disorder.

MPSI stands for mucopolysaccharidosis type I. Bodies of people with MPS I can’t produce a specific protein called alpha-L iduronidase, which is needed to break down sugars. These sugars build up in cells and cause damage throughout the body.

Govt funds allocated, more required
  • “Initially, we received Rs 9 lakh from the CM’s fund under the previous government. Now, under the present government Rs 15 lakh (Rs 5 lakh for each child) has been allocated to us for treatment. However, the amount is sufficient for only three weeks of treatment. The fund relief for two cycles of treatment has already been exhausted and the amount is left for one more week of ERT,” said Dinesh.
  • “We are sure that the treatment will benefit our children and thus, given this, we would like to request the UP government and other donors for support till the National Policy for Treatment of Rare Diseases is implemented, he added.

The condition of patients afflicted with this disease is often debilitating and its treatment is beyond the means of most patients.

Since years now, Dinesh, a tailor by profession, has been fighting to save his children. Finances are his major problem as Rs 4 lakh per month is required for each child’s treatment. Being a tailor, he only manages to earn two square meals for himself.

Dinesh’s ordeal began when his first daughter, Ranjana, started developing certain bone and joint issues. Her condition could not be diagnosed for around 5-6 years.

During this time, his other children showed similar symptoms as Ranjana’s. They were finally referred to the Sanjay Gandhi Post Graduate Institute of Medical Sciences (SGPGI) in Lucknow, where it was detected that they were suffering from MPS-I.

“Me and my wife were running from pillar to post to find out the root cause of our children’s health issues. Had it been diagnosed earlier, we could have taken timely steps and at least begun some supportive treatment,” said Dinesh Kumar.

Dr Kaushik Mandal (medical geneticist, SGPGI) said enzyme replacement therapy (ERT) was the treatment available for MPS and had proven very effective in having a positive impact on the life of such patients.

“The disease leaves the patients unable to do even simple daily tasks. With the progression of the disease, the condition of the patient deteriorates as damage is caused throughout the body including the joints, bones, heart and respiratory system. ERT, if started at the right time, enables a patient to lead an almost normal life. Thus, it is very important to provide the treatment on time,” he said.

Unfortunately, the cost of treatment for three children was beyond the means of Dinesh and his family. This is when they approached Lysosomal Storage Disorders Society (LSDSS), a patient group for LSDs, which helped them send a letter to the local MLA requesting for funds for treatment for their children. The letter was then forwarded to the Chief Minister’s Office.

“Initially, we received an amount of Rs 9 lakh from the CM’s fund under the previous government. Now, under the present government Rs 15 lakh (Rs 5 lakh for each child) has been allocated to us for treatment. However, the amount is sufficient for only three weeks of treatment. The fund relief for two cycles of treatment has already been exhausted and the amount is left for one more week of ERT,” said Dinesh.

ENZYME REPLACEMENT THERAPY
  • Dr Kaushik Mandal (medical geneticist, SGPGI) said enzyme replacement therapy (ERT) was the treatment available for MPS and had proven very effective in having a positive impact on the life of such patients.

“We are sure that the treatment will benefit our children and thus, given this, we would like to request the Uttar Pradesh government and other donors for support till the National Policy for Treatment of Rare Diseases is implemented. The condition of my eldest daughter is very critical, wherein she is not able to do anything on her own. I do not want the same for my other two children. Therefore, the government’s intervention is our only hope,” he added.

First Published: Dec 07, 2018 14:02 IST