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.
Ben -
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.)
Thanks for sharing all this info. I’m so sorry Ben is going through this. Will be praying hard for you all. Hugs!
Thank you for the prayers! Monday was a really hard day. I’m feeling more optimistic now, but it just sucks. There’s no other way to say.*
I’m sorry to hear that he is facing chronic rejection. I know it must be hard on you and your husband to be so far away while his brother is sick. I’ll be praying for your family and that they find him a new match quickly.
It is the pits! I obviously haven’t been there for all his CF ups and downs, but I was there for a lot of it. I hate not being with everyone now. And with Stephen off at training, we can’t even really lean on each other. I’ve been in kind of a haze this week, but I feel like it’s starting to lift.*
Sorry to hear about this turn. I am signed up as an organ donor. Hopefully my running will keep them all in good shape and when I do not need them anymore someone else will be able to make good us of them.
I think most states let you sign up as an organ donor at the DMV when you renew your license.
High five for being an organ donor! I love what you said about taking good care of your body so that your parts can benefit someone else! That’s exactly how I feel!*
Oh Amy, I’m so sorry! That’s a lot for him and your family to go through. Sending good thoughts your way!
Thank you for your kind words!*
I’m so sorry to hear about Ben’s health. I know it’s extra hard for you and Stephen to deal with this because you’re so far away. I hope and pray he’ll stay healthy while he waits for a new set of lungs.
Yes – the distance is so sucky! I’ve spent a few holidays in the hospital with Ben and my mother-in-law. I was there when Ben got his transplant. I was there in November. I wish I could be there now. The time difference sucks, too. For most of my day, people are asleep or it’s very early. When I go to bed, the day is really just getting started back home. It’s makes the sharing of information frustrating and painful at times.*
Thank you for the courage to share this story.sending prayers your way
Thank you!*
Amy, I am so sorry to hear about Ben. It takes tremendous courage to share this story on your blog and I can only imagine what you and your family must be going through right now. I am praying for all of you. Hang in there.
CF is a lesser-known disease (I didn’t know a thing about it until I met Stephen), so I’m happy to spread the word. I think that if more people know, they’ll be more compelled to take action. I mean, how can you hear about an amazing guy like Ben and not want to do something, even if it’s something small! I just wish the circumstances were happier these days. Thanks for the prayers!*
Amy Amy Amy. What am I gonna do with you? You’re so freakin brilliant and kind. It gives me hope in the world that you guys have so much love for each other and you’re willing to put it all out there and spread the word about CF. You are simply amazing! Ben too, obviously
And the whole fam! Thinking bout all you guys daily 
I am an organ donor and have been since I was 16-ish. It’s a no-brainer for me. As my mama says “it’s not like you can use ‘em after you’re gone!” I donated to Josh’s walk. Does the fam have something set up to help cover Ben’s medical expenses or get Sorelly a stress-reducing massage? I’d donate to that too
Janelle! If the transporter was working, I’d beam into your teal room and give you a big ol’ hug! Thank you for making that donation! That’s so incredibly generous of you! Josh says thanks, too. (Well, he said it in his Josh way when I told him that you donated.)
Thankfully, Ben has great insurance coverage through Stephen’s dad, I think. I’ve seen people set up webpages via Caringbridge to help raise funds for that kind of thing. It’ll definitely be something to consider if the need arises. (You’d think when someone has a disease like CF they could just get fixed up for free, right?)
Thank you thank you thank you!*
haha Josh was probably like “That’s weird. I don’t even know her.”
Praying for you and your family, Amy! I’m so sorry you guys are going through this.
Thanks, Jamie!*
Great post, CF foundation should put u to work for them!
Oh Amy, It makes me so sad to learn hear this. Organ donation is also near and dear to us, my husbands best friend in the whole world was blessed with a new liver a few years ago, I can’t imagine how life would be if he hadn’t gotten it. I will be praying for you and your family.
I saw you say on twitter his BFF had a transplant, and I wondered what organ it was. (Is that a creepy thing to wonder?) I hope that liver lasts him many, many years! Thank you for your prayers.*
Wow, such heartbreaking news y’all got! So glad that it turned out to not be that way! We’ll be hoping to see a report soon that Ben is getting a transplant! We both are organ donors!
They’ve had a few possible lungs become available that weren’t healthy enough. I’m keeping my fingers crossed that a healthy set comes their way. Sad as it is to say, this is the time of year when transplants are more common. (The bad weather = more accidents = more organs.)*
I’m an organ donor.
So sorry to hear this. I would be flipping out. If he does get another transplant, then it’s back to the waiting game to see if these lungs are a good match all over again? Poor guy. He’s in my thoughts.
Yeah, Stephen was pretty wound up when he heard the news. I was trying to stay calm, but definitely cried it out for a while. Getting new lungs would improve Ben’s quality of life and give him a few more years, but there’s no real permanent solution. Blarg. So shitty.*
Sorry to hear Ben’s condition has gotten so bad. In Germany you can get little cards where you can state your opinion on donating your organs so anyone concerned knows how you feel about it. There have been quite a few scandals in Germany lately where doctors made the patient’s health status look worse so they would be higher on the list. Apparently that made quite a few people reconsider their opinion about organ donation. While I absolutely do not approve of this kind of thing, it doesn’t change my mind about donation. It’s not like they pronounced somebody dead who was not to get their organs.
Wow! It’s good that you are still an organ donor despite the shady actions of those doctors!*
I’m sorry to hear that you guys are going through this. If you decide to become an organ donor, it is so, so important to make your family aware. I’ve also signed up for the bone marrow program too, since it’s so easy!
Oh, bone marrow list. I need to get on that!*
I’ll be keeping you all in my prayers! I did a presentation on organ donation back in college and I remember reading the statistics about how many people don’t even consider it, it made me so sad. (Don’t remember the specifics anymore..) to me it’s a no brainer. I don’t need them once I die, give them to someone who does!
I think we’d be so much better off if we instituted an opt-out approach. (People would automatically be considered organ donors unless they took steps to get themselves off the list. It’d basically be the opposite of what we have now.) Some countries do it this way and have higher rates of organ donation as a result. It wouldn’t completely close the gap between the need and the supply, but even if it went up 5 or 10%, that’d be so many lives saved!*
It sucks to be so far away when all you probably want to do is be there! I hope he gets the call that they have a match soon and things will work out. Prayers and hugs and hoping for the best!
Thank you so much! Prayers, hugs, and hopes are greatly appreciated!*
I can only imagine how hard it must be for you two so far away. I’m keeping y’all in my thoughts and prayers, and hope that Ben can continue to fight. He seems like such a strong guy, already overcoming so much. I’m thinking positive!
Pingback: Great Strides for Ben and CF | the20what
Pingback: Our First Great Strides | the20what