Needles and big fat fails.

After two weeks of IV antibiotics, Isaac was blowing better for his lung function tests, but not quite well enough to finish the IVs while his chest still sounded wet with huffing. And so the course was continued for an extra four days to top him up, bringing us up to the bank holiday weekend. This meant no clinic, and no community nurses, and although we have open access to the ward should we need it, we agreed that I could remove the needle from his port myself. Simple I thought....

Only, delivering drugs through a line into his blood stream is one thing, but actually inserting or removing that line (and gripper needle, which is in his port for the duration of the course of antibiotics) is kind of something else - his skin grows over his port, so it’s not like simply removing something from his port alone..... it’s pulling a needle through his flesh, my closest experience is home piercing ears as a teenager (not something I would recommend). 

So I braced myself; all I needed to do was flush the line with saline, followed by an extra strong Hepsal (like a very strong plug unblocker, to ensure the line in his chest remains clear for next time). I got my gauze and plasters ready for the bleeding. Isaac laid down. I gloved up, and got ready to pull. I anchored his port with one hand, and with the other pulled up hard. The needle made a popping, suctiony sound as released, and I stemmed the bleeding. I’d done it! My first time from his port....

Only as soon as it was out I realised that I’d forgotten the bloody Hepsal flush!  

Now I can tell you that there is not much worse than feeling that you have failed your child like this, especially one who endures so much already. I had been so focussed on the needle part, I had forgotten the simple flush. 

In tears I called the ward.... who were hugely reassuring. His line still has Hepsal in it, from the last dose of IVs I had given, only in a weaker solution. This means his line will be fine, only it’s likely he will need to be reaccessed on Tuesday now (the needle put back in) only to give him the correct flush, to ensure his line will be clear for next time. It’s not the end of the world, and Isaac is laughing about it, but kicking myself is an understatement x 

Drug mix up

This is tonight’s mixing up of drugs for Isaac’s IV antibiotics. Excuse my feck (attractive face/neck merger 😂).  If only it were this quick in real life! 

We are so so thankful to be doing home IV’s rather than in hospital again. It is day 11 of 14 (all going well with his tests on Tuesday, hoping it won’t be more than a two week course). Blood tests every three days show his kidneys are coping well with the toxicity of the drugs. We had one dash to hospital as his gripper needle dislodged, but this is most likely due to his port being new, we’re still sussing out what size needle is best for him. It’s all going well otherwise. Today he is coughing a lot more again, but I’m hoping this is just a big clear out (no more blood tf). 

Look at me, my first VLOG! I am totz down with dis, FML. 

Have a great weekend! 

Parliamentary debate.

Watching the parliamentary debate (here) tonight on Orkambi, a life changing precision drug which is only the second drug made available that treats the underlying cause of Cystic Fibrosis rather than just the symptoms. The stories are heart wrenching and all too familiar. Feeling super proud of the CF community who have petitioned and campaigned relentlessly for this debate, and have been protesting outside Westminster today. This CF community and the Cystic Fibrosis Trust together are well known to punch way above their weight politically. I so wanted to be there today, but as Isaac is currently on home IV’s, and I am the only one trained to give these, I couldn’t.

The debate raises so many interesting points, not least the question of what price you can put on a life? It also highlighted that middle ground that CF exists in; it is neither so rare that a high cost drug in a small population would be affordable, nor so common, like acute conditions, where a large population drives down the cost per patient. 

What is apparent, is that NICE must change the way in which they perform cost benefit analysis on drugs for chronic conditions: Every night we spend on the ward costs the NHS more than £400, and in PICU, more than £2000; more than one of Isaac’s daily drugs cost £8,000+ per annum... and at every single clinic visit he requires the care not only of his CF nurse, his CF consultants, but also a dietician, physiotherapist, psychologist and the pharmacist. He has also at various times been under the care of ENT, the Gastro team and Immunology, all related to his CF symptoms. He has multiple tests each year, including X-rays, CT’s, ultrasounds and bone scans, not to mention the regular bloods and sputum samples the lab deals with..... what this adds up to I can’t imagine. 

.... Orkambi has been proven to reduce admissions to hospital and reduce the decline in lung function by 42%. NICE agree the drug is effective, but not cost effective for the NHS (the original offer was over £100k per patient, per annum). I know none of this is simple, but the debate suggests to me that NICE is not fit to make that decision as they don’t fully understand the bigger picture in chronic conditions. 

There are around 70,000 people with CF in the world, and the U.K. and Ireland have a much higher prevelance of the condition than normal (10,400 in the U.K. alone). Orkambi is available in Ireland, but not in England. Government intervention between NHS England and the manufacturer, Vertex, is urgently required. I have no doubt an agreement will now be found, but everyday it is delayed, children are losing their lives. Half of sufferers will lose their life to CF before the age of 31 and imagining how those at that end stage of the spectrum feel about this delay is simply crushing. Yes, the cost is high, and it’s easy to blame that on the pharmaceutical company, but the portfolio offer to NHS England (Vertex have newer, more effective options to Orkambi already in development, and this portfolio would guarantee future access to these drugs also) is better than deals made in other counties that are already prescribing Orkambi. 

I’ve said before, Orkambi will not work for Isaac, nor will the next drug in the pipeline, Symdeko, nor even the one after that, as he has a rare and shitty mutuation of CF which will require a different kind of precision medicine to enable the correct movement of salt and water through his cells (the root problem in CF) but that doesn’t mean I’m not hugely passionate about access to the drug, and we are super excited about the paths it will open for further research that may one day help him, and move CF into a category like diabetes; a condition that you live with, rather than one that you die from. 

In the meantime, have I mentioned lately that I’m throwing myself out of a plane soon? Everyone with CF deserves the chance of a longer, healthier life. Please support our skydiving fundraising by clicking here. Thank you x 

That long black cloud is comin’ down...

“The friends and family who care for someone with a rare disease really become experts and the ‘voice of care,'” said Nicole Boice, the founder and CEO of Global Genes. Ninety-four percent become involved in care communication and advocacy work. Eighty-nine percent educate healthcare professionals about their care recipient’s condition, and 84 percent help with medical or nursing tasks. Caring for those with a rare disease comes with a lot of stresses and strains. Seventy-four percent of caregivers report struggling with a sense of loss about what their care recipient’s life could have been. And 67 percent report emotional stress —  twice as high as that for those caring for people with other conditions.”

Taken from an interesting article here. CF represents 9% of those rare diseases. 

Today we picked up his IV drugs and he had his port accessed again; a small needle called a gripper is pushed into his port, which the drugs will go through. This needle will stay in place for the whole course (hopefully just two weeks, depending on how he responds). 

Silver linings: at least we are home and not in hospital this time; he is sick, but not too sick; and not having to wait for a long-line thanks to the port is brilliant. 

Need to keep thinking of these positives. I miss Isaac being well. I miss Jayne, I have been thinking about her a lot lately. I admit I’m struggling a little more than usual right now...  the last six months have been tough and I’m not sure my muddled mind has fully caught up with everything. Working full time and raising three kids doesn’t allow a lot of time for contemplation. I’ll be OK. What I’d really like, is when friends ask how Isaac is, is to be able to say ‘He’s doing fine. Thank you for asking’. It feels like its been a LONG time since I’ve been able to do that. Things have got to get better. 

Picking up two weeks of IV drugs today. I bought my huge (and really damn cool) old lady shopper, thinking that would fit everything in. So wrong; 

BD, 1966.

Mama put my guns in the ground
I can't shoot them anymore
That cold black cloud is comin' down
Feels like I'm knockin' on heaven's door.

With a lot of tests, come lots of results.

Today was ‘not the best’ (my favourite of Isaac’s quotes, however hard things get). 

His lung function had dropped some 10-15% since his last test (January) and so he needs his fourth course of IV antibiotics in the last 8 months, his worst run yet. After the really shitty time he had in October last year, the plan was to switch from ‘IVs as required’ to ‘planned IVs every 12 weeks’.... only he hasn’t been well enough to last more than 8 weeks since then before needing more courses to help battle his infections. His doctor thinks this may be because he is still recovering from the October exacerbation and hopefully this will improve and we can start to stretch out the good bits inbetween over time. 

That wasn’t the only bad news: The data from his nebuliser was downloaded in clinic, and disappointingly showed that while he is doing all his treatments, he is not always completing them. His ineb beeps when it’s finished and shows a smiley face screen; sounds straightforward to monitor, right? But as the duration varies depending on how well he is (how deeply he can breathe) and how much he focuses (the data showed that each neb was taking anything between 2 and 41 minutes, and he does six nebs a day) with noisy family life, trying to cook dinner, check homework and pick random bits of school uniform off the floor, we don’t always, 100%, definitely hear the beep ourselves, even if we’re in the room; we rely on Isaac being honest, only it seems he is not always. 

; Cue some pretty emotional and honest conversations about his treatments, his health, and his future health. He has promised to do better, and I think he understood how serious this was. In the meantime, we’re reverting to old school ‘you sit in front of us until it’s done’ method. Cruel to be kind. It breaks my heart at times, we do so much to keep him well, but we need his buy in. 

His liver ultrasound was also worrying (further results and bloods to follow) and demonstrating his physio in clinic today, he coughed up the biggest bit of sticky blood streaked sputum* I have ever seen..... CF truly sucks. 

Anyway, there are no beds available on the ward, and there are no signs of the winter bugs abating. Our beloved NHS is cracking at the seams. This doesn’t sound ideal, but does mean I can administer his IVs at home, instead of spending two weeks on the ward. When he was younger we used to do more home IVs (I trained to do them when he was a toddler) but over the last few years they seem keen to do at least the first week in hospital, arguing that intensive physio will help. I get this, but also know that wards are rife with.... like, sick people (who knew?!) which is not ideal for him to be exposed to. That is no criticism about cleanliness on the ward, the cleaners are amazing, but there is no doubt that people cough all over the place, and we are using shared bathrooms, doors and communal food areas. 

We’re confident that we can keep him active here, do the extra physio, avoid further bugs, and have a more normal family life, which helps us all and keeps him safe. He will continue with his personal trainer (three times a week) and can go to school, even with the needle in. Only missing the football. This will be my first time doing his IVs through his port rather than a long line, but his flush today went well, so I’m sure it will be fine. It just means being even more careful (sterile) than before, as the port is a permenant IV device and so close to his heart, infection in the line could prove fatal. 

Plenty more results from todays tests still to come over the coming weeks, but for now we have a plan, and we will fight for every single percentage of lost lung function that we can. There was good news too, he is growing well, his dietician was very happy with his diet, and they have a new Clinical Trials nurse starting, and a joint venture with Cambridge University, which we hope will result in more access to clinical trials, which are hard to come by for his rare mutation (for my CF friends, he has two Class 1 mutations). Thanks for reading. 

Everyone with CF deserves the chance of a longer, healthier life. Please support our skydiving fundraising by clicking here. x 

*Blood in his sputum is a sign of lung inflammation, and not the same as hemoptysis, where you cough up fresh blood. 

A muddy and knackered Isaac walking the dog. We are so lucky, these lakes are just minutes from where we live. 

This weeks tests.

Glucose tolerance test (for CF related diabetes). 
Bone density scan (for osteoporosis and other bone density diseases associated with CF). 
Chest X-ray (to look for lung damage and areas of infection). 
Ultrasound of abdomen (to check his pancreas, kidneys, bowels, and most importantly, for CF related liver disease). 
Lung function tests (main measure of health in CF). 
Sputum tests (what bugs he has growing in his lungs right now). 
Blood tests (for infection markers and vitamin levels, as he needs daily super high dose vitamin supplements (not the kind we buy over the counter) as his CF prevents his body gaining these from his diet alone). 

Followed by... 

Dietician analysis (reports on what his diet may be lacking). 
Equipment tests (his nebulisers and other equipment are tested at least yearly). 
Physio review. 
CF nurse review. 
Psychology review
Pharmacy review. 
And finally, the doctor review.

All in a day, thanks to a wonderful NHS. 

Please support this petition to have a voice against the privatisation of the health service we so greatly rely on in the U.K. x