According to a story from the Manchester Evening News, Tom Miller turned 30 years old in January 2019. For Tom, that age was more of a milestone than it was for most other people, because it was never certain that he would actually live that long. That’s because Tom was born with cystic fibrosis, a disorder that affects breathing and can cause an early death. Tom is a realist; when he turned 30, he began to prepare himself for death.
Cystic fibrosis is a type of genetic disorder which can have impacts throughout the body, but it is most characterized by the build up of abnormally thick, sticky mucus in the lungs. This mucus becomes a fertile breeding ground and habitat for potentially infectious bacteria. Many patients must take antibiotics for much of their lives. This disorder is caused by mutations of the CFTR gene. Symptoms of cystic fibrosis include progressive decline in lung function, lung and sinus infections, coughing up mucus, fatty stool, poor growth, infertility in males, clubbed digits, and digestive problems. Treatment includes antibiotics and medications or procedures intended to maintain lung function. Lung transplant is an option when lung function declines severely. Life expectancy ranges into the 40s and 50s with good care. To learn more about cystic fibrosis, click here.
In general, 30 years is pretty old for a patient with this disorder, so Tom’s decision to accept an imminent death were pretty reasonable:
“I was never specifically told I wouldn’t live past 30, but it was implied due to the low life expectancy. That’s the age I always had my thoughts set on.” – Tom Miller
Life with cystic fibrosis means getting used to frequent admissions in and out of the hospital, often because of severe infections. For Tom, the two-week hospital visit has been something of an annual affair, even a routine of sorts. The disease has also affected Tom’s mental health as well; at times he turned to alcohol in order to cope.
However, his last hospital visit has been a cause for celebration. Since Tom was very sick, he was given access to a recently introduced triple-action drug called Trikafta. The drug isn’t approved in the UK and he was only able to get it through compassionate use from Vertex, the manufacturer.
The treatment has caused major improvements for Tom; when before a flight of steps would leave him winded, he was able to get up one faster than his unaffected sister without much effort. Its no exaggeration to say that the medication has given him a chance to live a new kind of life, and Tom’s story highlights why this new drug should be covered on the NHS as soon as possible.
