Jonathan Simms was a man from Belfast, Northern Ireland, who contracted the variant Creutzfeldt-Jakob Disease in his late teens. His life expectancy post-diagnosis was given as being only one year which is the same as other young people who were diagnosed in the same age bracket. However, due to an experimental treatment, he lived for another ten years after diagnosis, although his higher functions were severely limited.
Life and diagnosis
Simms was born in Belfast in 1984 and was, by all accounts, an athletic teenager who excelled at football and had undergone trials with the Northern Ireland International squad. He initially displayed symptoms between May and September 2001 of a disease which was consistent with either multiple sclerosis or vCJD. The doctor who first examined him, Dr. Mark McClean, later said:
It was either multiple sclerosis or variant C.J.D.; I told them that I thought it was M.S., because I hoped to God that's what it was.
From diagnosis to death, patients with vCJD are given a life-expectancy of between six months and two years. Simms was given a year to live, however, two years after diagnosis he was the first person with vCJD to be treated with an experimental drug that was known to slow the onset of similar diseases in animals.
Treatment
In December 2002, Simms' parents won a legal battle in the High Court in the United Kingdom for their son to receive an experimental drug called pentosan polysulphate. The legal challenge centred around the fact that whilst the drug had been shown to decelerate the onset of the disease in animals, it had not been tested on humans. Eventually, Dame Butler-Sloss ruled in the family's favour stating: The medical community's objection to the use of the drug was that it has traditionally been indicated for its anti-coagulant and anti-inflammatory properties, which meant using it in high doses on Simms ran the risk of haemorrhage. This was also clouded by the fact that an effective dose and lethal dose were very close to each other when tested on animals, so there was an inherent risk of killing Simms just by administering the drug. The family were forced to go back to court when the UK ruling was found to have no validity in Northern Ireland, but in January 2003, the High Court in Northern Ireland ruled in favour of the treatment. Whilst the High Court ruling allowed the administering of the drug, the NHS were not legally bound to be the framework by which it was given to Simms. After the ruling, a neurosurgeon was found who would undertake the procedure, but the NHS trust that he worked for would not allow it. Because the molecules of PPS are so large, there was no way that an intravenous or pill ingestion would work as the drug would not be able to pass safely between the bloodstream and the brain. Eventually a method of delivery was established with a catheter that went up his body from his stomach directly into his brain. Injections were then administered by this shunt straight to the folds in the patient's brain. By September 2003, it was reported by his primary care team that he had regained the ability to swallow, his body weight had stabilized and returned to normal and that his anxiety levels had decreased. His family were critical that when they first wanted to apply the drug in March 2002, Simms was able to walk and talk for himself. By the time the case had been heard by the courts system, 8 months had elapsed and Simms' condition had deteriorated. In 2007, the treatment appeared to have stabilized Simms and it was announced that he was no longer terminally ill. Don Simms said that his son was aware of his surroundings and sometimes "made attempts at vocalization, and on occasion, we can make out the words". By 2004, PPS had also been administered to 12 people who also had vCJD, There was one death, some who continued deteriorating and six patients where the disease appeared to have stabilized.
Death
In the latter stages of his illness, Simms needed intensive care. He was cared for by his family at their home in the Highfield Estate of West Belfast. Simms died on 5 March 2011, almost ten years after his first diagnosis. He was buried on 10 March 2011 at Carnmoney Cemetery just outside north Belfast.
