Mazidul Islam told the Daily Star that he had to watch his 14-year-old son Farid die and could do nothing to save him. Among other things, he regrets the fact that the family could not afford a comfortable life for Farid.
Nine-year-old Anisur Rahman’s parents are living with agonizing feelings of helplessness. Their son, Anisur, is suspected of having an exceptionally rare disease called progeria (Hutchison-Gilford syndrome) which causes rapid aging in children.
Children with progeria eventually develop strokes and heart disease, resulting in early death. There is no cure.
Anisur’s parents have been told of a treatment that costs Tk 6.68 crore and could extend their son’s life. However, they are not able to afford the medication. (1 crore=10 million Rupees =160, 000 USD)
At their poverty level, Anisur’s parents are not even able to travel to Lalmonirhat from Dhaka for an appointment with a specialist where they could receive a definitive diagnosis of their son’s illness.
Anisur’s father acknowledges that the family cannot even afford their daily ration of food. Yet he and his wife still have hope that his son may receive treatment and that he will eventually recover.
Calling on Divine Intervention
Due to extreme poverty, does Farid Islam’s fate also await Anisur? Farid’s death at the age of fourteen was caused by a rare disease called fibrodysplasia ossificans progressiva (FOP) which is a genetic disease whereby the connective tissue and skeletal muscle are replaced by bone. The bone forms outside the skeleton and gradually restricts movement.
There is no cure but if patients can afford medical attention, they may survive for an average of 40 to 56 years.
In 2014, a doctor at a well-known neuroscience hospital in Dhaka administered a sedative to Iraboti Roy. Iraboti, age 40, died the following day.
Her brother, Shashanka Baran Roy, does not blame the doctor, but said that Iraboti would still be alive had she not received that injection. He had taken Iraboti to a neurologist after suspecting a problem with her hypothalamus.
Iraboti had been diagnosed with myasthenia gravis (MG) which is a rare condition usually causing muscle weakness. It is often undetected, misdiagnosed, and thereby treated with the wrong medication.
Lack of Awareness and Adequate Medications
With 7,000 rare diseases affecting approximately 300 million individuals worldwide, according to the US-based advocacy organization Global Genes, a serious lack of awareness exists among physicians as well as a lack of adequate medications.
Going back to Bangladesh and its problematic healthcare infrastructure, out-of-pocket expenditure is 68.5 percent of the total cost of treating a rare disease. With no government policies or state funds covering rare diseases, only wealthy citizens can afford treatment.
Jakia Abedin was diagnosed with myasthenia gravis (MG) in 1999. In 2004, Jakia’s doctor advised that if her thymus gland were removed, it would alleviate some of her pain.
Unfortunately, this was not an option for Jakia. The cost of the procedure was between Tk 2 and Tk 10 lakh rupees or ($2,000 to $10,000 US).
Since then, Jakia’s symptoms have worsened, and she is no longer able to manage the pain. She tells the Daily Star that she is unable to swallow her food. At times she cannot move her arms and legs as she becomes temporarily paralyzed.
Jakia’s medication costs Tk 4,000 a month. If she were able to afford a full dose it would keep her muscles from becoming rigid and fend off spasms. Jakia explains that since she can only afford to take her medicine occasionally, she saves it for times when she is unable to get out of her bed.
The feeling of helplessness by rare disease patients and their families is evident throughout Bangladesh. Physicians are mostly unaware of these rare diseases but when they are confronted with a known rare disorder, the doctors usually discover that there is no approved medication available.
101 Interesting Cases
The co-author of “101 Interesting Cases in Clinical Medicine” Ahmedul Kabir suggested a government policy for rare diseases. He noted the high cost of treatment for a rare disease and suggested that a fund should be set up to support these patients.
Further to Author Kabir’s suggestion, the NIH conducted a separate study of the Bangladesh government’s growing burden of non-communicable diseases (NCD). Their findings indicate that existing programs lack adequate planning, implementation, and monitoring and were therefore somewhat unsuccessful. The NIH suggests that in the future, research should assess NCD policies with careful monitoring of their implementation and the level of population coverage.