Cystic Fibrosis was first labelled as a disease in the 1930s but certain aspects of the illness were referred to in medical literature dating back to the 1800s.
The early descriptions included a line that wouldn’t seem out of place in a horror movie.
It said: “woe to the child who tastes salty from a kiss on the brow, for he is cursed and soon must die,” recognising the first medical association between the salt loss in CF and illness.
At this stage everyone in Ireland should be aware that we have the highest rate of the disease in the world.
A massive 1200 Irish people currently have Cystic Fibrosis and one in 19 of us is a carrier. If one carrier meets another and has a baby the chances of it having CF is one in four.
Cystic Fibrosis National Awareness Week takes place between 11 April and 17 April
What people seem ignorant of is the fact that Cystic Fibrosis affects far more than the lungs. The genetic defect in CF causes secretions, which are normally thin and watery in healthy people but for someone with the defective CF gene it becomes very thick and sticky.
The thick secretions clog up organs and prevent them from working properly.
It damages the pancreas, meaning patients need to take pancreatic enzymes every time they eat, otherwise they would gain no nutrition from the food they eat.
A damaged pancreas also means a large proportion of people will get CFRD (cystic fibrosis related diabetes).
It leaves 97% of men infertile but not sterile and 20% of women with problems having children requiring fertility treatment.
The first sign of Cystic Fibrosis for me was liver disease. At the age of 12 I vomited massive amounts of blood.
The first liver transplant surgeon Thomas Starzl described this symptom in his autobiography by saying, “There is no more terrifying sight in medicine than an ashen and panic-stricken patient, bleeding internally into the oesophagus and stomach and then vomiting his life’s blood onto the floor before anything can be done to help. Many patients do not survive the first such incident.”
The doctors could find no reason why and labelled my cirrhosis cryptogenic, meaning cause unknown.
Yet four years previously my lung problems were labelled as asthma. Babies are now routinely tested upon birth for CF but this test is a relatively new one.
In 1995 I had undergone two liver transplants.
Incredibly on the night of my first, which was almost cancelled due to lung problems, a respiratory doctor was called in to decide if the transplant needed to be cancelled.
His first words were “Did anyone ever tell you that you might have cystic fibrosis?” Yet I was never tested.
Five years after a liver transplant I got diagnosed with another classic cystic fibrosis-related lung complication. Even at the age of 17 I had told doctors before I was ever transplanted that I was coughing up blood. Another classic CF manifestation.
Yet it took me until the age of 37 to get the diagnosis. The extra time my transplant gave me saw me fulfill a list of life’s ambitions.
I visited Graceland, as I am a huge Elvis fan. I graduated from DCU with a degree in journalism and worked until my health deteriorated.
I went to Philadelphia and ran the Rocky steps and while working in London got to meet the man at the press conference for the 2006 release of Rocky Balboa.
I saw Liverpool achieve the miracle of Istanbul and saw another great Kerry team emerge after the famine, post-Mick O’Dwyer. I have one sister and I lived long enough to see her have four girls and a little boy. Having no kids of my own I spoil them incessantly and adore them.
Despite all the publicity CF gets every year the government ineptitude on the issue is staggering
I attend the new CF unit in St Vincent’s and that only came about because of a campaign on radio and TV.
Previously, patients were placed in six bedded wards and not isolated. This is a necessity because we pick up infections that, while harmless to others, can prove life threatening to us.
A few weeks ago my nephew, who is only 15 months old had a mild case of the sniffles. One hug had me in hospital for 18 days. I was chronically ill.
Without the Irish CF community fighting so ferociously I’m pretty sure the government would have remained intransigent. Most of the other centres around Ireland are almost entirely self funded.
CF West in Castlebar raised €900,000 for a daycare centre, while the government funded a paltry €400,000.
Build4Life is perhaps the most extraordinary story. Set up by Joe Browne, whose own son has CF, he raised €2.3m for a dedicated 20 bed CF unit which finally opened last December.
Click here for the Cystic Fibrosis Ireland website >
They will fund the Children’s Cystic Fibrosis Outpatient Clinic and Inpatient Bedrooms in Cork University Hospital at a cost of €1,000,000, and have already raised €800,000.
Already they have deemed the new cystic fibrosis drug not financially viable. While not a cure it’s the first drug that addresses the root cause and will lessen hospital stays and lessen the number of days sick.
Grants we get, such as a yearly exercise grant, to join a gym or help toward an exercise bike or treadmill come from funds raised by the CF community.
Drug company Vertex are asking for astronomical figures too, so they need to reduce the price and be more humane and not just see the drug as a licence to print money.
The NHS in the UK have also turned the drug down based on cost. And the understaffing in CF centres is appalling.
A few weeks ago I turned 42 and I have lived well past my expiration date. It gets harder every day to keep fighting.
It’s often difficult now to see any positive path. It’s like a slow death. My lungs are not fit for purpose. 45% function leaves me breathless constantly. I have MRSA in my lungs.
My kidneys are damaged from 21 years of toxic anti-rejection drugs. My liver, while still functioning well, is damaged again. The fight within me to battle all these obstacles is still resolute.
Today the moments I feel well are near extinct. In days when things are really bad I’d just like to go to sleep and not wake up. On days I feel good I’m planning another yearly holiday, to New York or somewhere I love.
But I will fight on. I’d rather die fighting for life because I’m a fighter and I feel I owe it to the two donors who gave me the gift of life.
Trevor’s Twitter account can be found @elvisrockysly
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