CF Conference

Yesterday I went into London for the yearly CF Conference. I was excited to hear about the developments of the gene therapy clinical trial that is just wrapping up, but I was also very nervous as I was attending alone.

I'm unsure how to write this blog entry as my emotions are still very mixed about the day. The best way I know how to deliver the news is by starting with the positive things. So, what is it that left me feeling good?

1. The statistics. In 1960, those living with CF were children 16 and younger: 100%. Today, children only make up 43%. Adult care centers are bursting at the seams and this is the CF Trusts biggest problem - how to give sufficient care to adults. The oldest man with CF is 79 and there are many well into their 40s and 50s. The outlook is promising.

2. Gene Therapy. The good news is that the run-in study is nearly finished and it seems to be working. Gene therapy involves putting a virus into the body (through nebulizers) and having it reach the cells in the small airway in order to replace one of the faulty gene. Once this happens, chloride is produced, reducing the amount of sticky mucus on the small airways. The lungs become normal. The problem is that this works once, but then the body wises up and immunity kicks in. However, the UK team is working with a Japanese team which has found a way to put viruses into the body without the body rejecting it again. Anyway, next up is a multi-dose trial in the UK, which is the biggest trial being done in the world. If that is successful, the UK will team up with Europe and the US for the last trial in 2012. By 2013 we should know if this will be available to all CF patients. So, the hard thing is the wait. Jeb will be 6 by then, so it's just keeping him healthy until then. A mum looked at me and said, "you are so lucky, you have time. my children are 28 and 31 and I've already lost one to CF." This is the reality I faced yesterday, which leads to the emotional result (trying to keep negative out of this!).

I think we've been living in a bit of a bubble lately. Jeb has been healthy and giving daily doses of his antibiotics and physical therapy seems like no big deal now. But, when I sat down and talked with a few parents I realized that we may be in for more in the future. One family talked about a port that is surgically placed in the body making it easier to give IV doses of meds to their 4 year old. They have to get up at 5am to mix the meds to be given at 6am. They were so positive, but my heart sank as I heard this. They told me they almost lost their son at 15 weeks before he was diagnosed. Another family was told a 20% chance of survival at birth; their son is now 23.

As the day wore on, I heard about pancreatic sufficient kids like Jeb becoming insufficient by age 3. Hospital visits, taking off from work because of sickness, etc. It was heartbreaking and I pray that we are lucky and Jeb never has to endure this. CF is such a funny disease with no two cases being the same. It's hard to know what to expect because with every bad case you hear about another person who has never been hospitalized and has had only one or two IVs. We've been blessed with a mild case and I pray that this remains.

So, as I left and sat on the tube I held back the tears and tried to think positively. I remembered the words of a woman who had invited me over for tea when we first found out. She said, "this will work." Her daughter is 17, healthy and has the same gene mutations as Jeb. I'm holding on to her words and the trust that I have in God. He knows our every need.

By the time I got off the train in Beaconsfield and had slipped out of reality into the pages of a very expensive, imported People Magazine, the Conference felt like a lifetime away. So, now I'm back in my "bubble" with the faint memories and a little bit more knowledge. But I'm still grasping for hope.