Annual review

We had Isaac’s annual review today. While we go to CF clinic regularly throughout the year, this is the one day in which we do a whole bunch of tests/reviews and look in more detail at how his baseline is changing with progressive disease. Some results we get are immediate, some, like the vitamin levels in his bloods, take several weeks. What we know now: 

• Most blood results are outstanding; but we do know the glucose fasting test (looking for CF related diabetes) was fine. 
• Lung function; down significantly, but as expected since he has a current exacerbation, and he struggled to do the tests while coughing (they were better later in the day when repeated; mornings are not friends with his lungs). 
• Psychology review; yep, confirmed that he gets pretty cross whenever talking about CF. 
• DEXA bone density scan; results outstanding.
• Sputum lab results; outstanding. 
• Dietician review; his diet is great, but he needs to up his creon dose (pancreatic enzyme replacement) and to take it more reliably.
• Chest x-ray; not too bad, considering current infection. 
• Portacath (his implanted venous access device); looked OK on X-ray (there were fears that it might be broken, as he’s had pain during flushes) but he was accessed today fine. We think the pain may be related to the scar tissue he has around the site from a while back when it got quite sore. 
• Physiotherapy review; all fine, but his adherence to treatments when he’s left alone to do them is lacking (e.g. lower last week, while on half term). We will also drop one of his six nebulisers a day, as they would rather he did five really well, than six badly. Plus the sixth is using DNAse twice daily, which it is not licensed for, but he had prescribed after black October (read more here) as his secretions were so ridiculously thick. 
• Ultrasound; enlarged spleen and fatty liver, indicating CF liver disease. He will start a new treatment for the liver, the spleen we already knew about, and is associated with the number of infections he has had. 
• Doctor review; the fact that his lung function has picked up as much as it has after black October, is great, and this year has been better than the previous year. They praised his hard work to stay well. Despite his current exacerbation, they are really pleased with him. 
• He may be referred to Orthopaedics to check what appears to be a curvature of his spine. He was scanned for possible scoliosis a few years ago, and if memory serves me right, it was said then that he may have a very mild case (no action required) but apparently this is something that can become more significant in teenagers. His consultant will refer his X-rays to see what they think. 

Plan; We’ve started IVs today and he had the first dose in clinic. Bloods tomorrow (and twice more next week) to check antibiotic levels in his blood. He will start treatment for the liver issue next week (just tablets). They hope he will be well enough for his sinus surgery next week, and they will do a BAL while he is under GA (hoover up some more sputum from further down, to test in the lab for infection). They may also do a bronchoscopy at the same time, subject to availability on the day. Await further test results. 

Sorry, nothing particularly poetic or insightful here. The day was full on, and he came home feeling sick and has gone to bed. I feel like I have no edges; I’m all blurry. Like most years, it was a mixed bag, but it wasn’t awful either. So proud of him. 

Have a great day, if not today, then tomorrow x 

Two weeks of IV drugs, a stocked meds cupboard, and a dogs bottom. 

Smells like smelly teen spirit

His chest is sounding worse day by day. While we had hoped to do his next IVs post sinus surgery, it’s looking increasingly likely that he can’t wait that long. He coughs all night, and while he is sleeping through most of it, I’m not. The worry in the night is always amplified to screaming point. Him, coughing his lungs out, me, silently screaming mine. 

Last Thursday we arranged for an emergency appointment to have lung function tests and see his doctor. Dan and I were both working, so Dan’s Dad kindly offered to pick Isaac up from home and drive him into Dan, who would take him in to clinic. Only Isaac refused to open his bedroom door, insisting that he felt fine and that he could wait a week until his full annual review appointment instead. I went home mad, knowing his team had gone out of their way to squeeze him into clinic, and worse, that if he leaves it too long, he risks not only getting worse, but also delaying surgery as they won’t anaesthetise him if he’s that unwell. Despite his pleading that he felt better, he then coughed all night long. 

Friday his consultant phoned to speak to him (this never happens) and explained the same thing... that if he needs IVs, he needs them ASAP to have any chance of not delaying the op that he so badly needs. He grumbled a bit, but then conceded that his decision to delay might not have been the right one. As it was then too late to take him in, we’ve started extra orals instead in the hope they tide him over to this week. If he can make it to Thursday, we can kick off IVs while we’re in for his annual review. This would give him 6 days of IVs pre-op and (at least) 8 post-op (post-op infection is a risk, so as long as it takes). If he gets any worse before Thursday, we’ll be in sooner. We’ve been warned already that they may want to keep him in a few days, considering his chest and the significant surgery, but they are balancing up that decision with the fact that they have flu on the wards right now, and that is the last thing he needs. 

More than the delay, the not knowing just how unwell his lungs are right now and the worry about delaying the op, it’s that he is increasingly taking the reins in terms of how and when he wants treatment, and not always for the right reasons; Refusing to go on Thursday was much more about the fact that it is half term holidays and he wanted to stay home and play PS4 than because he really didn’t want to start IVs. Had it been a school day, he would have happily taken the morning off school to go. 

For fifteen years now we have put his health above everything, and knowing that he might not do the same is heartbreaking. He’s going to make a lot of wrong decisions in the next few years I fear, and sitting back to watch that, as a parent, is really hard. When he was grumpy with me for being mad with him (after refusing to go to hospital) I told him that unfortunately for him, I will always love him way more than he can ever hate me for wanting him to to stay well. So ner ner, ner, ner ner (because we are that childish) x 

Isaac, 2019. 

Complaining and coughing.

After a CT scan, the follow up appointment shortly after, and then pre-op the very next day, we were pretty confident of the surgery date being very soon. After a week of waiting, I called, only to find that yes, he is on the urgent list, but this would still mean AT LEAST a two month wait. 

When I explained to the kind but slightly ineffective lady in admissions that he has constant headaches and nausea, is off school a lot because of this, that he also has CF, and that his infected sinuses cause issues in his already infected lungs, she struggled to reply (I was very polite, I always am, I get that this is a strained NHS, and that they do their very best), the only reassurance she could offer was that she would schedule a reminder for herself to check with the doctors again in TWO WEEKS. We cannot sit back and wait that long while he suffers this much. He has days where he walks round with either an ice pack or a hot flannel on his head all day, despite 24/7 pain relief. 

So I reluctantly called PALS and explained the situation. Within 24 hours, we had a surgery date two weeks from now. 

It’s so sad that we have to do this, yet again - we had the same with his last sinus surgery; after months of waiting our CF consultant actually advised us to complain through PALS to have him bumped up the list, as it was the only thing that would work. It seems being under the care of different teams creates a breakdown in care for the individual (at times Isaac has been under the care of both his CF Team (which includes his Doctors, Specialist nurses, Dietician, Physiotherapy, Psychology and Pharmacy as standard) but also Gastro, ENT and Immunology, and it feels like all these teams see only the aspect that they treat, without looking at him holistically; it doesn’t take a brain surgeon to get that blocked and infected sinuses will further hinder someone with lung disease, right? PALS help see the bigger picture across all of an individuals needs and prioritise appropriately. At least I hope that’s what it is, because otherwise it’s just about ‘he who shouts loudest’ and that would be sad too. Anyway, we have the date. 

A few days before surgery, he is also having his annual review (his full CF MOT; chest X-ray, bloods, bones density scan, ultrasound, full suite of lung function tests, diabetes test, dietician, physio, psychology, CF nurse and doctor reviews, all in a single day) and since he is coughing significantly more each day, a likely action plan will be to have two weeks of IVs post surgery.

But our worry is twofold; one that his lungs will need these IVs sooner - which could delay surgery, and two that because he is having surgery and his chest sounds so shitty that they will admit him to the ward, rather than allow home IVs. Corona* fears aside, the hospital is already amidst a flu outbreak, and we would much rather keep him safe at home. Plus, you know, being in hospital kind of sucks.

*Despite the very small rate of Corona infection rates here in the U.K, they are predicting that this could still unfold into a full epidemic here, and if that happens, up to 50% of people may be affected. Stay safe people! x 

Above, pictures of Rosa bean (her, a willing model and me, playing about with my camera). 

Below, little Isey. This was us festivaling it up, almost ten years ago. He will turn 15 very soon. How can that even be? ❤️

Corona virus

If Isaac were to contract this kind of virus, he would be at such a disadvantage from the off, it would be a significant risk to his life. 

We have already seen a virus take him from being quite well, having IV antibiotics on the ward, to intensive care needing breathing assistance - in just a matter of hours. That illness necessitated the use of a wheel chair, 24/7 oxygen, and a physical recovery which took many weeks (I’m not sure our mental recovery is even complete now). Mostly we just feel lucky that he recovered at all. 

However you feel about the risk (or not) of Corona virus, and wherever you are in the world, please, please practice good hand hygiene (this is much more powerful than wearing masks alone) - like having the flu jab, this is more about protecting those more vulnerable around you, than protecting yourself. 

If this does become a pandemic, and it sounds like it will, every one of us must take responsibility in trying to limit the spread of the virus in any way we can. If this means working at home more, if you can; do. If this means battening down the hatches and avoiding all non-essential socialising, if you can; do. If this means avoiding your GP surgery or A&E, if you possibly can; do. 

If you feel unwell, call for advice instead and keep the vulnerable (the very young, the elderly, and those with pre-existing conditions) in mind. Cough into your elbow, not your hand. Use disposable tissues and dispose of them well. As well as washing hands well, use alcohol gels in-between. Avoid public transport. Stay home. What might feel like a mild flu to you, may kill someone else. Take to the sofa, watch films, get well! 

My thoughts are with those in the eye of this storm, and those that fight everyday to help them x 

Sinus pain, surgery, and miracle drugs

CT scan done. The doctor said it was as he expected (since he’s already poked a camera up there anyway). Our CF nurse gave a less guarded review ‘looks ghastly, no wonder he’s feeling so bad’. 

The follow up which was to be a month later, I had moved to 3 days later (happy kick arse Mama moment), and the ENT team have been great, seeing him for pre-op the very next day, so we are all set for surgery, just waiting for the date now (we will keep the pressure on). 

His CF team will liaise with ENT, as while he is under anaesthetic they will do a lavage (hoover up some sputum from his lungs, to test in the lab), and depending on lung function test results, he will likely start a course of IV antibiotics at the same time. He is coughing more each day, and his last course was in late November, so the timing is no great surprise. He will need two weeks off post-op due to infection risks anyway, so tying in IVs to this time off school is no bad thing. 

Our hope now is that the op comes first, and the IVs second. If he becomes any less well, and needs IVs sooner, that could mean he wouldn’t be well enough for surgery, but at the same time, the sinus pain/infections continue to impact his lungs too. Bit chicken and egg that. 

Our beautiful, spirited, Rosa bean.

In typical Isaac style, he is taking it all in his stride. He is not able to exercise much; he is in pain and sick often; he is missing a lot of school - but he still laughs with us everyday. We got his school report, and despite his low attendance (about 80%, so a day a week on average.... and shall we say, a rather lack lustre attitude to homework in general) he is still doing well. I don’t know how he does it. 

In other news, myself and some very good friends are beginning to plan our next big fundraiser... so excited about this! No trekking, hair shaving or throwing ourselves out of planes this time... but an EVENT. More details to follow. 

Best of all, the CF community is awash with incredible stories about how Trikafta (the third generation of these new *small molecule drugs) is having such a crazy positive impact in the quality of lives of the 90% of CF sufferers for whom this will work.... in some cases, it’s mind blowing (e.g. being listed for a lung transplant due to end stage CF, to coming off the waiting list entirely with a 20% increase in lung function - in a matter of weeks!). Many happy tears hearing all of this news, but with it, I’m reminded of all those we have already lost, for whom this comes too late. I can’t help wondering, if it could have come in time for Jayne, would she still be here? For those most recently lost, this seems all the more cruel. In some countries, these new drugs had been developed, even manufactured, but they sat unlicensed in pharmacy warehouses due to cost and frustratingly slow negotiations and process. This must end. Trikafta needs to be made available in the UK now, before more lives are lost. Please sign the petition here.

Have a lovely weekend all x 

*I am asked about these new developments often, so just to reiterate, the current drugs that are licensed will not work for Isaac who is in that last 10% who have a class of mutation where the protein cannot be fixed (as it can be for the majority) as in his case, the protein is never formed in the first place, so we need a whole different approach. But it brings us a lot of hope too. 

Bassin de lumieres ❤️ (need to go):