Everything Working in Concert

     My favorite music group is Dave Matthews Band. They are the band I requested to be played as I entered the OR prior to my transplant. I have seen them five times in concert, and starting three years ago, I'd made it my vow to see them every time they rolled through Michigan on their annual summer tour.  However, in 2013, I was still adjusting to being on oxygen, which I either had to carry or drag behind me on its little luggage cart style stand.  I was in the process of getting a scooter when the tickets went on sale, and I decided it was just too much to try to go to a venue where I wasn't even sure about the accommodations they have for disabled people.  I remembered vividly that when I went in 2012, the walk to the entry gate was long, the stairs were extremely steep, and the bathroom was down another huge flight of stairs.  So, after missing the concert in 2013, I was committed to making it work the following summer.

How Could I Forget Green Family Robinson?

     In all the excitement and mishegoss of returning from Disney World and the start of the summer school, I left out one of my favorite moments from our family vacation.  In all of my previous trips to Disney World with my wife and son, there is one thing I skipped each and every time.  There was barely any conversation about it; the large structure loomed, with the promise of twists, turns and stairs.  On the first few trips, I knew I could have made the climb, but it would have required stops and rests, and probably triggered a few productive coughs-- none of which are a pleasant public experience when alone, not to mention in front of one's child.  It was the safe decision, but with all the other fun experiences, it was small potatoes, and a logical choice so I wasn't worn out for the other parts of our journey.

Video of my Speech at CF Family Education Night 2015

     I am very happy to share the video of my speech at the CF Family Education Night.  U of M created a social work focused website with a Cystic Fibrosis subsection, and they have linked the video there.  By going to this link and clicking Part 2, you can see my entire speech, including the question and answer session which followed.  As far as I can tell, the speech will play on mobile devices as well as regular operating systems.  If you already read the speech when I posted it, you will see that I pretty much stuck to the script, with a few notable exceptions.  I am so grateful for having had the chance to speak at this even and for all of the great questions people asked after I spoke.  I also can't thank Dr. Simon enough.  He was my pulmonologist for 14 years prior to my transplant and gave me a better introduction than I could have asked for.  Feel free to let me know your thoughts or ask any additional questions in the comments section, or by contacting me through Gmail.  

Medical Trials Without the Tribulations

     I have been fortunate enough to qualify for a variety of Cystic Fibrosis medical studies over the years.  All of them were done through the University of Michigan, and though not all of them went smoothly, they were a fascinating window into how new medicine is approved and the level of commitment that is needed to make strides in finding new and better ways to treat CF.

Beautiful Explosion of Hope

     On July 2nd, the FDA announced that it had approved Orkambi, the Vertex-made drug that offers an unprecedented treatment option for people with the most common CF mutation (which happens to be the one I have).  This article contains all of the relevant details from the press release.  The words in black and white represent a level of hope for almost one third of Cystic Fibrosis patients that surpasses what many ever dreamed possible.

     Innumerable people whose lives have been touched by this disease view Tuesday's announcement as a long awaited trumpet which will sally forth a new era in CF care.  With the medicine being shipped to pharmacies as I type these words, I can't imagine or express how it would feel to be the parent of a child with CF, for whom this announcement must seem tantamount to a redefined future.  But as always, I am mindful of the other perspective:  the parents of the ten year old whose child has the mutation (double delta F508) this medicine treats, but is two years away from being allowed to take Orkambi, since it is currently only approved for those 12 and older.  Or the parents of the pre-teen who doesn't have the CF mutation treated by this new drug or Kalydeco, which works in the same way and was approved in 2012.  The gaps in who these incredible medicines can benefit within the CF population, along with the efficacy of the drug, are preventing everyone from using the word "cure," but a quick glance at recent posts here at the CF Blogroll demonstrate how positively most people are reacting to last week's wonderful news.  This one in particular captures the sentiment of a person with CF very well...

     But what about someone like me, whose lungs do not have and will never have the genetic flaw of Cystic Fibrosis lungs?  I have no clue.  The day the announcement was made, I was seeing my pulmonologist, blowing my highest FEV1 yet (95% Wo0t wOot!) and totally unaware that the approval was happening that day.  When I'd asked about Orkambi a few weeks ago, I was given a relatively canned answer:  that they don't like to theorize on giving people medicines that aren't approved by the FDA yet.  Which I can understand.  I also realize that the odds are very slim that anyone with transplanted lungs took part in any sort of trial leading up to the approval of Orkambi.  I will inquire next week about what the drug might mean for transplanted CF people such as myself, and happily volunteer to be in any studies, which I've done before and always had a positive experience with. 

     For now, though, the weekend's fireworks were, in the lives of a large number of CFers and their families, as much about the prospect of breaking free from the restrictions imposed by Cystic Fibrosis as they were about America's enduring freedom.  We continue to hope that Orkambi is one step on the road toward every single person with CF celebrating his or her independence from pills, puffers, and pulmonary problems.  

     

Spidey Swings by Camp Michitanki

    The time I spent last Friday at Camp Michitanki, a week long sleep-away camp for kids who have received an organ transplant, was the most enjoyable volunteer experience I have ever been a part of.  Probably because I was dressed as Spider-Man for most of the time.  I'm going to go into a lot more detail than would ever be necessary for the book, but that's okay, because I want to have a record of every amazing moment that I was able to witness and be a part of last week.

A Closer Look in the Magic Mirror

     Going to Disney World (and Land) is unlike any other vacation experience for a variety of reasons.  One of the most visible is that people are encouraged to make a public declaration of their reason for being there.  The parks distribute buttons which indicate what a person is celebrating... first visit, birthday, family reunion, anniversary, or honeymoon.  The last of these is also frequently made apparent by mouse ears or a shirt indicating "bride" and "groom."  All of these proclamations are encouraged by Disney, and if you are celebrating something they don't have a specific button for, their generic one allows guests to write on the button to explain what the special occasion is.  So, my wife, son, and I each made a button that said something about my lung transplant.