Father spends monthly salary on medicines for son with rare disease

Over one million Indians are believed to be living with primary immunodeficiency diseases (PID). Yet there is rampant lack of awareness and diagnostic facilities for the disease in the country. Patients afflicted with the condition need life-long treatment, and in the absence of any state-sponsored programme, their families struggle with the exorbitant treatment costs.

Four-year-old Aditya (seen with father Manoj) was admitted to Wadia hospital on March 29 in a critical state of pneumonia. The family has spent over Rs 50,000 in less than a week on his treatment. Pic/Naveen Nair

MiD DAY spoke to a Vikhroli-based family where the sole breadwinner has a monthly income utterly insufficient for the medical expenses for his four-year-old son who has PID. Manoj Singh (30), an employee of a private company, earns Rs 15,000 a month and has to support a family of four. His son Aditya Singh (4) has to be administered five grams of IVIG (see box) once every four weeks.

The dose itself costs Rs 15,000 while other medicines required round the month cost another Rs 3,000. On top of that, Manoj has to get his son admitted to a hospital each time his health deteriorates owing to lack of sufficient antibodies in his system. Aditya is now admitted in the ICU at B J Wadia Hospital for Children, battling a severe case of pneumonia. He was admitted on March 29 after his breathlessness escalated critically. Manoj has spent over Rs 50,000 in less than a week on his treatment.

"My son requires five-gram dosage of IVIG every fourth week and if it is delayed even by a couple of days he suffers from several infections. I risk my son's life every month by skipping timely dosage. I get paid only every fifth week and hence give my son the dosage after a week's delay," Manoj said. When Aditya gets admitted, it is mandatory for him to get one gram of IVIG per kilogram of his body weight. This escalates the medical expenses as his body requires a dosage of 15 grams, which costs Rs 45,000.u00a0At his present age, Aditya requires only five grams of IVIG a month, but soon the dosage has to be increased.

"My son was born healthy. But two years ago, he started showing symptoms of cough and cold all around the year and complained of stomach ache. When none of the medicines prescribed by local doctors helped we got him tested by a senior doctor from a Parel-based hospital and the reports revealed that he was suffering from PID." Aditya frequently suffers from debilitating stomach pain for hours at a time. The only option left with the Singhs is a bone marrow transplant, which costs around Rs 20 lakh and does not guarantee a cure.

Hope floats
There are families working hard to support others in similar situations. In 2008, Andrea Vanristell, mother of 14-year-old Ashley from Andheri started the Ashley Hypogammaglob-ulinemia Foundation Trust in 2008, to support children in the city with PID. Ashley has been on IVIG for almost nine years and has to receive her entire life. Ashley's foundation also raises money for four other kids with PID whose families are unable to afford the treatment.

Doctors speak
Doctors across the nation are urging that the government come forward and bear the expenses of PID patients from the economically weaker sections. In the United Kingdom, the National Health Scheme provides lifetime support to PID patients. Though any medical condition prevalent in the ratio of 1 in a population of 10,000 is declared a national issue, PID is yet to be recognised as one by the government, say doctors.

Dr Surjit Singh, professor at department of paediatrics, PGIMER, Chandigarh, said, "Approximately 250 kids are receiving IVIG treatment at our facility. Most of these children are from families that cannot afford their treatment, and the institute raises money for the same. A few of them are close to 20 years of age, and without the IVIG treatment they could not have survived."

Dr Mukesh Desai, chief of immunology at B J Wadia Hospital for Children said, "Approximately one in 1,200 people have a potentially life threatening PID but over 99% of the cases go undiagnosed or misdiagnosed due to lack of awareness among the medical fraternity and the general public. Ninety percent of the diagnosed children eventually die due to lack of funds." Dr Desai is part of the team that works at the PID diagnosis centre based in Mumbai.

"In a year around 250-350 cases of suspected PID are referred to us. Earlier, 12 out of 100 cases referred to us were diagnosed, but in 2012 the number of positive cases went up to 30. We suspect that most of the other referred cases are also some form of PID but we are yet to identify that type." The central government is planning to set up advanced centres in Chandigarh and Mumbai to facilitate diagnosis and treatment of PID. At present, there are only two referral diagnostic centres in the country, one of which is at Parel's KEM Hospital.

What is PID?
Primary immunodeficiency disease (PID) refers to a group of diseases afflicting those who are born with a weak immune system. There are more than 120 different PIDs described in the medical literature and over 200 genes have been linked to be the cause of PID, say experts. Victims of PID require lifelong therapies including intravenous immunoglobulin (IVIG) infusion, aggressive antibiotics or bone marrow transplantation (BMT).
PID occurs in persons born with an immune system that is either absent or hampered in its ability to function. While not contagious, these diseases are caused by hereditary or genetic defects and can affect anyone, regardless of age or sex.

Help
Anybody willing to help Aditya Singh's family may contact: Manoj Singh (Aditya's father): 9702420044 or 9619972053u00a0