“But he looks so well”

“But he looks so well”, something every CF parent will have heard numerous times when telling people their child has CF. It’s one of the most frustrating things I hear, because children with this invisible condition don’t look any different, but as I have said before looks can be very deceiving.  Yesterday during Chester’s annual review clinic, for the first time I uttered very similar words “But he’s been so well”.

I have said it many times before that one of the hardest things about CF is not being able to see what is going on inside and not being able to see what damage that is being done.

So yesterday when I was told that despite sticking rigidly to Chester’s treatment plan with physio, meds and exercise, his recent bronchoscopy showed an extremely large amount of secretions in his lungs. I honestly felt like I had been kicked in the stomach, like they would be questioning whether or not I actually do his treatments and like everything we had been doing was not enough and that I should have been doing more. I know deep down I haven’t, but I honestly do feel like I have let Chester down in some way, I am the one trusted with his care and yet here we are with this news. 

When Chester had his first Bronchoscopy last year post pseudomonas eradication treatment, in true Chester style, he was further diagnosed with another condition called “tracheomalacia” which is more commonly known as a “floppy windpipe”, this is where the windpipe collapses, and in Chester’s case it is severe, collapsing by around 80% which is why his breathing can appear noisy, a little like a 40 a day smoker.  It is beilieved that this condition has most likely been the culprit in the pseudomonas growths. With his windpipe the way it is, despite chest percussion physio Chester is not physically able to clear the secretions which in turn allows bugs to settle and grow and for secretions to build up clogging is lungs. 

The team have come up with a plan to trial a clearway ventilator machine which will blow air into Chester’s airways to open them up and allow him to move the secretions easier. This machine is incredibly expensive so will be on loan to trial it first before purchasing to ensure it works for him. They have made the decision to keep him on nebulised antibiotics which I was a little gutted about purely for selfish reasons that it adds another hour or so into our already packed schedule. 

They have also noticed that when asleep, Chester’s oxygen saturation drops significantly and they believe this also to be caused by the floppy windpipe; this may be when Chester has been more susceptible to growing pseudomonas. He will be admitted to undergo a sleep analysis and dependant on the results he may need to be ventilated on a machine overnight to increase his oxygen levels.

In comparison to Chester’s first year where we spent so much time in hospital with various chest infections, this last year was remarkably better with no admissions because he was “unwell” only admissions to treat pseudomonas. In the past 12 months Chester has only required the use of back up antibiotics where we increase his prophylactic antibiotic and add another antibiotic to help beat infection once. This was an achievement for us and I took this to be a good sign, things were starting to get easier, less meds and equipment to take with us on our travels, and now I feel like we may actually never leave the house with all the added kit and meds we are now on.

I was left a little shell shocked by yesterday’s review and angry that CF is doing this to Chester, I hate that I feel completely out of control and that I am powerless to stop it. I should be able to help him, but I can’t, and it’s the most awful feeling.  I can’t help but think that he is too young to have so many complications and I have found myself thinking about what the future holds for him.  I found myself thinking the absolute worst, I started to think about all the younger CF children I have seen lose their battle just in the short space I have been a CF parent and it is terrifying.

Last night I sat watching both boys playing together on the floor with their train set, both oblivious to the news I had been given, I had word with myself about feeling so down and realised I had to put my heartbreak to one side partly because I would hate for the boys to see me so broken, but also because I am determined that if Chester can pick himself up, smile and get on with it despite everything he goes through, then I have to man up and carry on as well. 

Yesterday was a blow and it doesn’t matter how hard it gets, I have to stay positive, I need to show the boys nothing has changed and I need to carry on helping Chester fight the fight.

This boy has had so much thrown at him and CF is much becoming the gift that keeps on giving, it doesn’t matter to Chester though, to him this is normal life, he knows nothing different and no matter how difficult the circumstances he always manages to smile and more often than not blow a kiss.