Tortoises

Isaac finished IVs today, feeling so much better. The last couple of weeks have been really rocky; firstly coughing SO much more, then so exhausted from the double treatment of anti-fungals and IV antibiotics at the same time. Then feeling better, but followed up with a couple of days of nausea, chills, and sickness. He has bounced back miraculously and his lung function today was good. Hopefully he is set up now for a summer of good health. He really deserves a good break.

If you would like to support the amazing work the CF Trust do in funding research into life changing treatments AND have a chance of winning money back, please enter this online raffle - it really only takes two minutes. Thanks so much for reading, and happy summer all. We don’t have many plans, since we thought we would still be shielding, but now feel like tortoises, slowly poking out our heads to see what is what. There is so much fun to be had. Life is beautiful x 

Gerhard Richter, one of my favourites. 

PPE, exacerbations, and the NHS

Yesterday Isaac had an emergency admission to hospital. He is sick, cough, sick, cough, sick, sick now. My poor boy. 

We were met at the hospital doors by a nurse and escorted the back way to lung function labs. After that, we were led to a triage COVID Ward. We know our way around the hospital more than most, but this is a precaution due to the pandemic. Last week, when we came in for just an X-ray, we were offered a security escort, had we not known the way. It is so well controlled. 

We never thought it was COVID, but with any respiratory illness, they have to assume it could be. On the COVID triage ward, we sat in a sealed room. Everyone we saw had to wear full PPE and discard it as they left the room, sometimes the same people, changing multiple times, each and every time they leave and come back in the room. Forget one thing for his port access? De-gown/glove, and put a whole new set on just to collect what you missed. It must be exhausting. 

We were both tested for COVID (me, only as I was with him). Isaac had bloods, and sputum was already in the lab (we now know he’s growing aspergillus again, so a fungal infection on top of his live in PseudomonasA bacterial infection). To cover all bases and to avoid coming into hospital again soon (despite all the amazing measures they take, and really, all they do to protect the spread of the virus is frickin spectacular) they decided to start the full shebang immediately; two weeks of IV antibiotics AND anti-fungals. This is perhaps more than they would usually do at this stage (at this point, we were still pending the blood results, so it was a good guess), but it helps us avoid going back in for a second assessment, tests... and further exposure. So all good with us. 

Isaac has had a very hard and sleepless week, but we’re hoping that this plan will have him bouncing back very soon. I have to acknowledge the irony that as soon as we are told we can unshield, and he can return to school, that this latest exacerbation has kicked off. Just bad timing. He started lockdown before us (due to sinus surgery and an exacerbation) and now coming out of the most stringent rules, he hits another CF bump. Anyway, we’re getting back into the rhythm of home IVs, and he is sleeping lots to regain his energy. 

We are so, so thankful; to be home rather than on the ward; to our fantastic CF team for enabling this; for our community nurse team that come for his bloods every few days; and for our amazing NHS as a whole, for all that they do, especially at this difficult time x 

Trikafta/Kaftrio tears and X-rays

This week has been an emotional one. 

 

Four years of campaigning, protesting outside Westminster and writing to MPs about the immense and immediate need for these new small molecule drugs (that treat the underlying cause of CF, rather than only its symptoms, as Isaac does) that have been licensed but remained unavailable to patients across the UK, due to COST. From Kalydeco, Orkambi, Symkevi and now Trikafta (the UK brand will be called Kaftrio) - yesterday a deal for the whole portfolio of drugs has been agreed with NHS England. 

This will prolong lives; this will improve the quality of lives; and for young children with CF, this should prevent the lung scarring, colonised infection and chronic inflammation the CF brings about. 

I am so proud of the CF community and the CF Trust that have helped bring this about. There is no doubt that the campaign and publicity have driven this decision in a way that the government and NHS England have not. 

So many happy tears for those who can now start this amazing treatment. But also tears for those that this drug comes too late for. There are too many. 

For us in that last 10% that these drugs will not work for (I explain about this here) we hope not to be forgotten, and that these breakthroughs in medical research will open up new avenues for treatments that will work for the minority too. When Isaac was born, we were told that gene therapy was our best hope, but that it may come too late to help him. Still waiting on that! That these treatments have come to fruition at all gives us incredible hope for a better future. 

Isaac is not well right now. Chest full of treacle. Hoping oral antibiotics may help, if not, will need to kick off IVs shortly. Tomorrow we head into hospital for the first time since lockdown began (which is an amazing stretch for him!). Mask up. Drop off sputum. X-ray, and get out of there. Follow up by telephone. So thankful for our incredible CF team that have enabled us to stay safe at home so long x 

Local art. Life is beautiful.