The Waiting List

There is a boy (well technically he is a man now) that we know in the US whom 11 years ago had a lung (and liver) transplant because of Langerhans Cell Histiocytosis. The LCH was in his lungs, amongst other places, and had caused cysts like Dylan's. His mum has once told me herself that his lungs were 'nowhere near as bad' as Dylan's were. So why, you may be wondering did he have a transplant?..

Because the doctors did not know what was wrong with him. They had no idea what he was suffering from was LCH, and like with Dylan last January, they thought lung transplant was the only option. As I mentioned previously he also had a liver transplant for the same reasons.

Great problem solved!! Erm well actually no.. because the rogue Langerhans cells were never treated so it returned in the new lungs. This time around they diagnosed him and treated the problem with Chemotherapy. Fabulous, happy days right?!

There is a common misconception that transplant solves all issues. It is a misconception that I once had myself.

When I was originally told Dylan would need a double lung transplant I naively thought that would fix the problem, that he would have a new healthy set of lungs. Taking away for a second the chance of recurring disease.. I knew about the anti rejection drugs that transplant patients need to take but I also knew of people living full happy lives after liver and kidney transplants. I thought as long as the disease couldn't come back we'd be fine. Then I got hit with the bombshell that double lung transplants don't come with a long life expectancy.. the average is 5-10 years before patients suffer Chronic Rejection of the organ.

That's right. 5-10 years. Some people don't last a year, others make it 20+ but the average is between 5-10 years. Ok so then you just have another transplant right? That's what we commonly get asked when we talk about transplant. The answer is usually a resounding no..

The wait time for a double lung transplant isn't as long for a child as an adult as they do tend to get priority now adult lungs can be made smaller and used. Also there aren't many children waiting for double lung transplants compared to adults. But the problem lies with how do you keep a patient alive, adult or child, when their lungs are failing?!

I am by no means a medical expert but I do have some knowledge so I can outline the basics..

When organs fail, certain things can keep people alive whilst they wait for a donor such as Dialysis for example. Even heart transplant patients can be kept alive whilst they are on the waiting list. Just recently there were articles shared about a young boy in London being on an artificial Berlin Heart for 3 years whilst waiting.

This isn't always an option for the lungs. If you can survive with a ventilator breathing for you without deteriorating then great.. you can have a Tracheotomy and wait it out that way. But if your lungs are failing even a ventilator won't help. When Chronic Rejection occurs in the lungs they become stiff and difficult to move up and down even for the ventilator and a person will deteriorate.

There is a machine called ECMO which effectively oxygenates your blood and organs without the need of the lungs, but it is only a temporary option. The average time ECMO is recommended for is 5 days but it can be used for up to 25-30 days. Any longer and the body starts to suffer adverse effects such as brain damage. Yes you read that right.. DAYS.

The average lung transplant waiting time is currently 3-6 months but it can be up to 3 years plus if you have a rare blood or tissue type.

This is why lung transplant is a last resort and why you'll often hear us say we don't want Dylan to ever need to have one. Yes his lungs will always be damaged but if he can survive and live a fairly normal life with what he has (yes he may never be an athlete) it is a better option than putting a timer on his life expectancy..

THIS is why we support Organ Donation as we believe this waiting list should be shorter giving people more chance of survival and secondary transplant. Certain countries actually have an 'opt out' system now rather than 'opt in' so patients are automatically candidates for transplant unless they state otherwise. Whilst I do not think this is the answer, I do think more people should be willing to give if they'd be willing to receive. Same goes for Blood Donation and we are advocates for that also.

This is also why we raise awareness of LCH and particularly Pulmonary Langerhans Cell Histiocytosis.. so doctors know what to look for and recognise the symptoms and characteristics of the disease maybe avoiding transplants in some cases and providing quicker access treatment. Especially PLCH because it can become life threatening very quickly. We literally need our lungs to survive.. so be thankful for every breath you take today!🎗💙