Posted by Wordsmith on 27 October, 2008 at 1:44 pm. 4 comments already!

1967-2008

He had been admitted to ICU with a serious cystic fibrosis attack about 18 days ago.

Something Dean Barnett wrote a year ago:


As I mentioned yesterday and long-time readers know, I have Cystic Fibrosis. CF is a genetic disease, the number one genetic killer in the country. The average age of death is 36. I’m now 39; when I was born in 1967, the life expectancy for a newborn with CF was 8 years.

CF is a strange disease inasmuch as there are 1500 genetic variations of it. That means in an American patient population of 40,000, most “Cystics” are battling essentially different diseases. With the advances in genetic understanding made over the past two decades, it has become clear that some CF patients draw a much better lot than others.

Until I was a teenager, I was mostly asymptomatic. Even after I was a teenager, I enjoyed what is considered good health in the CF community. I’ve certainly been luckier than most CF patients.

About five years ago, my condition deteriorated suddenly and rapidly. One thing about a lung disease that I’m not sure many people know is that because of the scarring of the lungs that occurs during exacerbations, ground once lost is often impossible to reclaim. In other words, a serious exacerbation causes a new lower baseline. My initial deterioration five years ago reset my baseline, and many of the activities that I had once loved like running and racquetball became memories.

Over the next few years, I continued to get sicker. That’s one of the reasons I decided to remake myself as a pundit. I needed to find an activity that I’d find satisfying but didn’t require 50 hours a week of work or travel or any of the other demanding accoutrements that my business life had featured.

A couple of years ago, I went on the lung transplant list. A lung transplant is what’s known as a treatment of last resort. The survival expectations are rather grim. 30% of recipients don’t make it one year; 50% don’t make it three years. Obviously you don’t go on the list unless your time is winding down.

IN THE SUMMER OF 2005 I made the top of the list. That meant that the next set of lungs that came available in New England and matched my relatively rare blood type belonged to me. I got a new cell phone whose number only the hospital had and waited for a call that could come in a week or might take years.

Shortly thereafter, something that I consider a miracle occurred. Some clever CF doctors in Australia noted that several of their patients who were avid surfers enjoyed much better pulmonary health than their non-surfing compadres. Although the CF gene was discovered almost two decades ago, the way the disease functions is not well understood. Still, the medical community has reached a consensus that CF’s problems originate with sodium transfer issues at the cellular level.

Those of you who remember your high school chemistry no doubt recall that sodium is a key component of salt. The Australian CF doctors figured that maybe the time their surfing patients spent inhaling the salt water was having a salubrious effect on their pulmonary systems.

The doctors wanted to run a study seeing how patients fared inhaling a hypertonic saline solution. (“Hypertonic saline solution” is basically a scientific way of saying sea-water without mud.) They sought funding, they got funding and the results were amazing. The patients in the program showed a remarkable level of increased lung function and a remarkably decreased frequency of exacerbations.

When I heard about the results of this study, I wanted to try the hypertonic saline even though the study’s sample size was tiny. From my past experiences inhaling other drugs, I sensed the treatment would be successful and was at the very least unlikely to be harmful. About nine months ago, I became the first patient at the Massachusetts General Hospital CF clinic to go on the hypertonic saline solution.

As was the case with the Aussie surfer dudes, my results were amazing. My lung function improved to the best it had been in five years. I dropped off the transplant list. I had a shot at a future. Who says miracles don’t happen?

I KNOW THE preceding is a familiar story to some of you, and for that I apologize. But I had to bring everyone up to speed so what follows will make sense.

A couple of months ago, I bumped into an old friend of mine who I’ve known for decades, since I was a small child. His son Joey had CF, and was seven years younger than me. Remember what I said about their being a range of CF cases? These run from the relatively benign like mine to the truly vicious. Joey had a vicious case; he was an incredible fighter, and it was amazing that he made it as long as he did. Joey succumbed when he was 12 years old.

Joey’s father is Joe O’Donnell. For those of you in the Boston area, “Joe O’Donnell” is probably an instantly recognizable name. Joe’s an extremely prominent businessman, a member of Harvard’s board of overseers, an almost-purchaser of both the Patriots and the Red Sox, and was recently coronated by Boston Magazine as Boston’s most powerful citizen.

When Joey was born, my father was head of the local Cystic Fibrosis chapter. He called Joe and said, “I understand your son has CF.” Joe said, “What of it?” My father said that Joe had to get involved. I’m not sure how much (if any) convincing Joe needed, but regardless my father doesn’t take no for an answer. Joe almost instantly became a pillar of the CF community.

When Joey O’Donnell died, it would have been completely understandable if Joe and his family turned away from CF and never looked back. Indeed, that perhaps would have been the normal thing to do. There is no agony like losing a child, and every time the O’Donnells are reminded of Cystic Fibrosis, there must be a measure of a pain involved.

But the O’Donnells didn’t go that way. In their son’s memory, Joe and his wife Kathy formed The Joey Fund to raise funds for research, treatment and support for the Cystic Fibrosis community. As Joe’s business and public profiles have grown over the past decades, so too has his commitment to the Cystic Fibrosis community.

When I bumped into him a couple of months ago, he asked me how I was doing. I told him I was doing amazingly, much better than the recent past and that I felt like I had a new lease on life. He said that was great. He then asked if I was on the hypertonic saline inhalant. I told him I was, and that’s what had made all the difference. He made a little fist-pump, and told me that it had been the Joey Fund that had financially supported the doctors in Australia and that had then made the product available to the CF community. My eyes welled up a bit as did my wife’s and I told my old friend, “You saved my life.”

THE SIGNATURE annual event of the Joey Fund is a Boston area film premiere held in Joey’s honor. Unlike most charity events, this one is a blast. The food is great, the movie is usually good, and since it’s a premiere you get to see it before anyone else on your block. But the most impressive thing about the event is the list of attendees. Politicians, athletes, business tycoons – they’re all there.

Last week, I got a call from the local CF chapter asking if I would participate in the making of a little film that would run before the movie. Of course I agreed, and yesterday I went down to Joe’s office to talk with a camera crew about my experiences with CF and with the hypertonic saline. The point of the film is to tell the audience at the film premiere that their generosity has made a difference. While the search for a cure for CF remains a frustrating one, the Joey Fund and the CF Foundation have produced treatments that have made a difference. I’m living proof of that.

At one point during my interview, the questioner asked me if I expected to see a cure to CF in my lifetime. I answered no, but that it doesn’t really matter. When you see death up close, a couple of things become clear. One is that we all die, and that death is just part of the deal. The other is that life is such a blessing, that’s it just so great, even though you know the inevitable might be near you still want as many bites of the apple as possible.

None of us know what the future of the salt water treatment might be. My health will maintain its current state indefinitely in the truest sense of the term. The good times could continue for years, or it could all crash tomorrow.

But regardless, this treatment has given me time – time to spend with my wife and family and friends. Time to hit golf balls (usually sideways, but even that’s alright). Time to chase my dogs around the house. Time that frankly I didn’t expect to have. There could be no greater gift, and it’s a miracle in so many ways.

The miracle has its roots in my persistent father who got Joe O’Donnell involved in the fight against CF. It continues through the incredible courage shown by Joey O’Donnell, who fought CF with such bravery that he inspired his family to fight the disease long after Joey succumbed. And it finishes with Joe O’Donnell and the rest of the amazing O’Donnell family who have given so much of themselves in so many ways and to such great effect.

There are indeed heroes out there. And miracles, too.

He will be missed. I followed his guest hosting on the Hugh Hewitt Show and read his writings. His family are in my prayers.

*UPDATE*
Hugh Hewitt’s Farewell post

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