On July 13th my friend James Dingle, his band SUGARBEAT and some very special guest stars will be playing at the Portland Arms, Cambridge, in aid of the Cystic Fibrosis Trust, and to celebrate a special birthday. To date, thanks to this event, and others that my friends, family and I have undertaken (...treks, raffles, marathons, hair shaves, skydives, events) we have helped raise almost £98,000 for this cause.

Why CF? Well, this is about an issue that is very close to my heart. In fact, it's directly over, next to, and surrounding my sons heart: in his lungs. And it's also in the lungs of approximately 10,800 people in the UK, and some 70,000 worldwide, who have Cystic Fibrosis (CF).

The CF Trust fund research that has led to major developments in the treatment of CF; Since the 80's the median life expectancy for a person with CF has risen from 16 to about 35 years, but while there have been amazing improvements in CF care, the focus of that care was solely on treating the symptoms of the disease, rather than the underlying defect; For Isaac we have drugs that help thin and hydrate the sticky mucous that collects and pools in the CF lungs; drugs that help compensate for ineffective pancreases by giving synthetic enzymes to digest food; we have drugs that reduce inflammation in the airways; and drugs that attack the many different strains of bacteria that inevitably find their way into diseased lungs, causing recurrent infections that in turn lead to irreversible scarring; and, for a lucky few, we have the final option of transplant.... trading in CF lungs for the lungs of a generous stranger. But even transplant is no cure.

But CF treatment has begun to fundamentally change. It’s been 30 years since the CF gene was first identified, but only now do we finally have drugs available that treat that actual genetic defect. These new drugs have the potential to change the entire approach to CF treatment (allowing for less use of the symptom-treating drugs that often lead to other, secondary issues). They are not a cure, but the hope is that they might halt the progression of the disease.

However, these new drugs only work for 90% of CF sufferers, and Isaac is not one of them. He is in that unfortunate 10% due to a severe and rare mutation of the gene, which needs a different kind of fix.

We desperately need research to continue, to help him and others in this group. In the meantime, as CF is a progressive disease, we see his health continue to decline. These new treatments cannot undo the damage to his lungs that CF brings, so to benefit from future advances, such as gene therapy, Isaac continues on an aggressive treatment plan, including two hours a day of physio and treatments, and two week courses of IV antibiotics frequently throughout the year, to keep him as well as we can. He never complains, and he never stops smiling.

It costs close to 800 million USD (can't find this fact in GBP, but you get the idea) to bring a single drug to market, from research and development through to testing and approval. Because of this, it is ONLY through the support of people like yourselves that any of these drugs ever reaches the CF patient population. And, despite the seemingly astronomical sum required to make a difference, let me assure you that it's the little donations that add up to the bulk of this life-changing number.

To read more about Cystic Fibrosis, you can find my blog at https://mymerrymolyworld.blogspot.com/