Hospital and hoping for holidays...

Isaac was admitted to hospital today. He is exhausted and coughing, but otherwise OK. His sputum is noticeably ickier than his norm. As usual, he amazes us with his chilled and accepting attitude, but this time he is worried as we go on holiday soon and so have a time limit to get him better, or risk missing out. Kick arse IVs and intensive physio is our plan A to get him there. I can’t tell you how crushed he would be (our first big holiday in two years) not to mention the girls if they had to go ahead without him. As we are driving (South of France) our plan B would be for me and him to fly down and catch them up when he’s well enough, if that’s even affordable/possible. 

Sometimes I feel like I’m not, not coping, but neither am I coping. Reading that sentence back, I’m thinking this is a sign of some pretty fucking muddled thinking! It’s certainly pretty hard not to get angry that he has to go through so much. On admission today I went through his list of medicines with a doctor, a nurse and then a pharmacist (we always have to do this, I’m never quite sure why so many times). The pharmacist placed her hand on my arm as I listed them off; so many drugs (and that’s without the IVs) why aren’t they keeping him well??? It’s been just three weeks since his last IVs. He needs a break. 

We went to clinic this morning for lung function tests, but as there was no doctor available there, we then had to be admitted via A&E, where we stood in a queue for 45 minutes with a guy in serious chest pain, a drunk woman who vomited on the floor, and a woman with a teeny tiny cut on the top of her head. Thank god for triage. In the end, it was all pointless anyway as the doctor never even came, they just phoned down to A&E to say Isaac was to be admitted as his lung function had fallen significantly and he so obviously needed treatment. NHS I love you, but the crazy bureaucracy less so.

Please send well wishes x 

Sick sick....

The first week of the school holidays, and come with it... a fucking CF exacerbation. 

Coughing. Wheezing. Walking around in a dressing gown, gripping a hot water bottle, in 30 degree heat. Napping. Stairs suddenly feel like a challenge, and huffs with physio sound like they traverse through treacle..... 

This disease holds my son hostage, and I hate it. 

We’re only three weeks clear of his last IVs, I don’t think he’s ever sounded so bad, so quickly. Plus he’s trying to hide how bad he feels as he knows that we go on holiday soon, and he fears another admission. 

We need him better. 

Rothko blue, because I’m blue, and a Miro, because, everyone needs a Miro. 

Cystic Fibrosis FAQs

What is a portacath? Isaac had a portacath surgically implanted in his chest wall earlier this year, the aim being easy venous access for regular IV treatment (IV being intravenous, as in, drugs delivered directly into his blood stream). 

Regular cannula/long line/PICC line access over the years has wrecked his veins, and it became harder and harder to find a viable vein each time (veins repeatedly used would just collapse resulting in multiple needles, and often, delayed treatment when he needed it most). We had an agonising wait in HDU (high dependency unit) once, and delayed antibiotics give bugs a chance to fight back. Not good. 

The port meant surgery, always advisable to avoid, but the hope is that this will last him 5-10 years before replacement. Cancer patients often have a port for for chemotherapy. 

He still requires a needle to access it for use, but the key difference is trying to guide a line up a long, thin, windy and collapsible vein, usually blind (they can use ultrasound, but this is unusual) which often fails and needs repeated attempts, compared to a needle plopped right into a kind of rubber bung with a hole in the centre, and ta dah... you have accessed the (pre-accessed) vein. 

What does having a port mean to Isaac? On the whole, it’s great. When his port is not accessed you can see only a smallish bump under his skin in his chest (with one scar nearby and a second by his neck, from the surgery). When it is accessed for treatment, he has a needle in for the whole course (usually 2-3 weeks at a time) and cannot get this wet, so no swimming or showers. The rest of the time, it’s life as normal, aside from avoiding full contact sports like rugby, which might damage the port. 

In between treatments, the port is accessed every month for a flush to keep it clear. So it does still involve regular needles, but compared to cannulas, long lines and PICC lines, overall, it is a helluva lot less stabs! 

What would a Transplant mean to Isaac? CF is a multi factor disease, it affects mainly his lungs, pancreas, liver, sinuses, and digestive system. A lung transplant would only cure the disease in his lungs. But this is also where the biggest risk to life is (90% of people with CF die from lung disease). 

After transplant, people with CF no longer have CF lungs. However, infections in their sinuses may re-infect their lungs, meaning they need to carry on with some traditional CF lung treatments (but not all, and maybe very few). 

Digestive health is unaffected by lung transplant, for example, Isaac will always need to take Creon in order to digest fats and proteins (pancreatic enzyme replacement meds, of which he takes about 50 capsules a day) and will continue to be at risk of CF related osteoporosis, diabetes, some cancers and liver disease. 

Why not transplant now? You will hear me shout loudly on this blog about organ donation and the fact that we have chronic shortage of donors. 

1 in 3 people on the waiting list for lungs dies waiting

I have often been asked why Isaac cannot be put forward for transplant now? The good news is; he is too well. The main test of wellness is a lung function test. His FEV (forced expiratory volume) is measured regularly and forms part of the picture on which his team plan his treatment. I don’t like to post much about his FEV, as I feel it becomes a focus on a number which is only part of a much bigger picture. Plus it fluctuates widely, at his worst (while in intensive care) he blew a 17%. Needless to say, his baseline is never as high as I would like it to be, but also, it’s not near the point where transplantation would be considered (regularly less than 30%). 

More importantly, transplantation is not a cure; the chances of surviving the surgery for a year is only 80% and surviving 5 years 60%. This is a fast changing statistic, as development in anti-rejection therapies improve. Transplant is truly amazing, and life changing for many, but it’s also like swapping one disease for another, and further, a whole new set of not so fun side effects. 

More FAQ’s to follow. Any questions, because medical jargon slips into my vocabulary pretty quickly, please do ask x 

A squash and a squeeze

For anyone who has read this book, the way the old lady feels when she gets her house back? Sums up how we feel post home IVs...

His daily treatments (tablets, nebulisers, physio) which usually feel half defeating to get done, suddenly feel SO simple after two weeks of very early starts, gloves, aprons, mixing up antibiotics and syringe driver pumps....  So here is to feeling thankful, de-accessed, hardly coughing, and our boy getting his summer fun back on track. 

I’m going to post some FAQ’s soon, about CF. All questions welcome. Have a great day x 

NHS England deny Orkambi access

Today NHS England stated, again, that Orkambi is too expensive to fund for sufferers of Cystic Fibrosis, despite a promising pipeline deal proposed which would mean earlier access for people with CF to drugs still under development. See here for more details, and here for how a young girl with CF eloquently puts this frustration across. 

Last year, half of all people who died with CF were under the age of 31. Sorry to be so blunt. Really. If I could avoid the statistics, I would. But in the last year Isaac has had 5 courses of IV antibiotics for 2-4 weeks at a time, 10 weeks in hospital, 4 operations, countless tests and clinic visits, and continues to take nebulised drugs every single day which cost the NHS thousands of pounds every year. I am not saying this is a moany way, we are so so grateful to the NHS for the treatment that he receives, and the life it allows him to live. We really do consider ourselves lucky. 

Orkambi will not work for Isaac’s mutation, but I hope one day there will be a precision drug that will, and while the cost for these initially seems astronomical (Orkambi costs around £100K per annum, per patient), I do believe there is a saving for the NHS in terms of keeping people with CF well and out of hospital, which these drugs really have the capacity to do, and the pipeline deal would guarantee access to future drugs hopefully even more effective than Orkambi. 

I’ve long been gutted that Isaac’s rare mutation means we need to wait longer for these kind of treatments (they are also an oral drug, no IVs, no nebs, Isaac’s dream come true!) in a sad way I’m glad that Orkambi won’t work for him, because knowing that a treatment was there, approved, and waiting on a shelf for him, and not being able to reach it would drive me beyond crazy. I honestly don’t know how I would cope. 

If you are in England, and could find the time to help, please download the template found here and send it to your MP, which you can find here. It would mean so much to me if you could. 

Things here are OK. We had a wobble last night as he had a mild fever and vomited. We have to be very cautious about infection risk in his port on IVs. Thankfully this morning he was back to his usual self. Despite home IVs, we’re just really thankful to not be in hospital for this course, which we hope will end on Tuesday (with a cool swim for him) all going well with lung function tests. 

Have a great day x 

Heat wave and wild woods

Isaac is doing really well, frustrated in the heat, port accessed and unable to swim, but coughing much less. Bloods have been OK (the blood fairies come every three days to check his Tobi levels, as his kidneys can suffer from the toxicity). Angry earlier, he asked why he can’t have a transplant already. He is sick and tired of the treatments; as soon as his hour hooked up to the IVs is done... he still needs to do his nebulisers and physio. There is no holiday from CF treatments. In this heat he walks round, top off, port accessed and IV line dangling on his chest, a very visible sign of disease, but I’m proud he doesn’t care. 

This weekend a little baby in our family gained his angel wings, far too soon. Little lion Leo. We are thinking of you all, Sian, heartbroken. 

Some pictures from Wild Wood last night, basically a rave in the woods. A very welcome blow out, with some beautiful friends, without whom I would cope a lot less well. Thank you lovelies x