Swings, roundabouts, and plummeting slides

The CF cough is different. Hard to explain if you’ve not heard it. I would say guttural, but I just looked it up and it says harsh and from the throat. This is deeply from the chest. From every muscle and fibre of his being. Violent. Vomit inducing. Loud. Rumbling then escalating. Exhausting. 

These last weeks have been tricky. As a Mum, the stress of a CF exacerbation, plus issues with blood sugars, unknown seizures and tests, culminated in physical, mental and pretty weird ways. Minuscule in comparison to what he has to go through, but hard nonetheless - not only for me, but for those around me. I wept for an hour at a petrol station recently, unable to fill up because the hire car we had was like an escape room puzzle that I could not fathom for opening the fucking petrol flap. This is not normal for me. Then a an old Irish tune that Dan used to sing Isaac as a baby crawled in as an ear worm and I found my cheeks wet with tears before I realised it. 

CF is not only a disease of the lungs. It affects most of his organs, most notable his digestive system, kidneys, liver, pancreas and his fertility. As a child he experienced the gastric issue’s extensively (DIOS) but these have gladly lessened over time. His liver and kidneys suffer predominantly because of the aggressive antibiotic treatments that are essential for his lungs. His pancreas suffers because the enzymes which should be released via a duct to digest food are unable to, as that duct never formed in utero, blocked by mucus, and so slowly erode his pancreatic function from within. This, in turn, impacts the pancreases other job; to produce insulin. Leading to issues with blood sugars or CF diabetes. Seizures, they are not part of the CF package. That’s all new and confusing. 

He was not accepted onto the clinical trial, as his health is not deemed stable enough. Disappointed doesn’t quite cover it. As we approach his birthday, we focus on getting him well and looking forward to easier times, and as this post describes, we enjoy the life amid the noise. Love x

Hazy lush nights with amazing friends ❤️

To think is not always to see.

We had a fantastic Christmas, all home and well. At times like that, it’s easier to shut CF into the back room and ignore it for a while. Even better, Isaac enjoyed his first solo trip (to Germany, to visit a girlfriend). This felt like no small win - CF so often scuppers plans at the worst possible time it can feel like the whole worlds shitty fate is against you. He had a brilliant time, came home loved up, and is planning his next trip back in the Spring. Despite the rampancy of flu and other viruses going around, he has escaped most of this, but his lungs are starting to protest more now. He’s also experiencing more of the confusing episodes that he had late last year. For that, we’re waiting on consultations with other teams and further test results. If I dwell on this new challenge, it’s easy to feel bitter, he already has too much to contend with - but my sunny side says we will work it out. 

I’ve said before, that while we have a pretty good idea what his lungs are like at any given time now (fruity, rumbling, wet, dry, wheezy, clearing, plugged…) we still occasionally surprised by either good lung function results when he is symptomatically much worse, or vice versa. Right now, we have the former, and so wait and hope he can avoid another course of IVs as we’re only a few weeks out from the last lot. Excitingly, he has been invited to screen for the next phase of a gene editing clinical trial, only one of four patients in the UK. I can’t say too much due to confidentiality- but suffice to say we are keen as custard. Hoping to start next month, which is another reason to hope he won’t need IVs anytime soon (he cannot start a trial within 30 days of extra treatment, that is, beyond his daily routine). 

Organ donation is something I’ve always felt strongly about, but even more so since I unwittingly joined the CF community. I have lost friends waiting on the list, and one shortly after (her body likely too weak after end stage CF to survive other complications). I’ve also known friends to be enjoying a full and healthy life many years post transplant. This can and does truly change lives, and yet despite changes to the law that were supposed to increase the number of donations, the reverse is happening https://www.organdonation.nhs.uk/get-involved/news/international-experts-advise-on-action-to-support-more-families-to-say-yes-to-organ-donation/ Please don’t only sign up, but tell your next of kin of your choice, as whatever you say now, it becomes their choice after your death x 

Lee Miller ❤️