Cystic Fibrosis (or CF) – A genetic disease that causes the body to produce thick mucus making it hard to breathe. The lungs and pancreas are the primary organs impacted by CF. In the United States, somewhere in the neighborhood of 30,000 people are living with CF. Another 10 million people (1 in 31 Americans) carry the gene.
Life expectancy – As of 2009, the median predicted age of survival was estimated to be in the mid-30’s. 45% of those with CF are over the age of 18. The is a vast improvement over decades past. In the 1950’s, CF was known as a “child’s disease” as most of those who had it were not expected to live long enough to even attend school. Unfortunately, even with these improvements, life expectancy is still tragically low.
Cure – there is no known cure. There are drugs and treatments that can improve the quality of life and lengthen the life expectancy of those with CF, but even a lung transplant is not a cure.
Standing in order from oldest to youngest: Stephen, Ben, Josh. Taken in September.
Those who have followed my blog for a while have heard me talk about Ben before. He is my brother-in-law, and he was born with CF. He has dealt with it his entire life, always with grace, a good attitude, and a fighting spirit.
In 2011, Ben was fortunate to be the recipient of a double lung transplant. (1,700 lung transplants were performed that year.) The surgery went without a hitch, and Ben recovered with miraculous speed.
At the one year-mark, it seemed like Ben was doing better than ever. (80% of CF patients to receive lung transplants live to see the 1 year anniversary, 50% make it 5 years post-transplant.) In the months since then, his health has declined. Last fall it was determined that he is facing chronic rejection.
Ben a few hours after his lung transplant.
Chronic rejection – long-term loss of function in a transplanted organ. In the case of the lungs, it may take the form of scarring, inflammation, respiratory tract infections, and overall decreased lung function. Chronic rejection is more common in lung transplants recipients than those of other major organs.
There was in incident in November when Ben suddenly went downhill. It was very touch-and-go for a few hours. Stephen took emergency leave so we could fly home to be with the family. That incident might have been related to rejection, but it might not. (Our best guess is that it was related to a port that was improperly flushed by a nurse.) At the end of our time home in November, Ben was doing well, and in a matter of days he was discharged. It seemed like he was on the up and up.
But he struggled to gain weight, was still suffering shortness of breath, feeling weak, and returned to the hospital in December. On Monday, we were told that his oxygen numbers were so low, that if he didn’t get a transplant in the next few days, that he probably wouldn’t live much longer.
Talk about a kick to the gut.
He has been intubated and is breathing with the aid of a ventilator. The doctors took a chest x-ray, and it appears now that things are not as bad as they initially thought. (It was a terrible 24 hours waiting to hear the prognosis.) The “must get a transplant in two days” timeline was determined to be incorrect. So it seems that Ben has more time, but he is still fighting rejection and his health is still not good.
Josh, Me, Ben at Passover last year. They aren’t my brothers by blood, but I love them just as much.
Ben is back on the transplant list. New lungs are his best chance for improvement. It is likely that he’ll remain in the hospital until he gets another transplant.
So where does that leave us? We hope and we pray and we wait.
What can you do? If you aren’t already, consider becoming an organ donor. I don’t expect Ben to get your lungs, but 1,700 lung transplants a year seems dismally low. More people need to become donors if we want our loved ones to have the chance they deserve in their time of need. If you are already an organ donor, make sure your family members know your wishes so they can carry them out. (Organdonor.gov has more information if you are interested.)
If you want to help CF patients in particular, think about donating to Great Strides. Josh (Stephen’s youngest brother) is participating this year and raising money for the Cystic Fibrosis Foundation. (I participated last year; you can read about it here.) This is a fantastic organization to put your money towards since almost every single penny goes directly to support the development of new drugs to fight the disease, improve the quality of life for those with CF, and ultimately to find a cure.*
(All statistics are from the Cystic Fibrosis Foundation and Second Wind.)