And I'll tell it and think it and speak it and breathe it.

Isaac is never one exactly keen to go to school. His mornings are mired with nausea and pain way too often, not to mention always being on catch up work from the days he misses. But today was his school sponsored walk, and he wanted to get up early, eager to go. But his chest was audible with crackles before he even entered the room. Cough after cough after cough. Productive enough to get a juicy sample for the lab (dropped off first thing today. Results won't be ready before Monday earliest). Until then, we have to rely on his usual antibiotics, extra physio and Cipro (his most powerful back up anti-bot). Clinic had advised last week to delay Cipro as it is too soon after his IVs, but he is now too poorly to put it off. We will give this a few days, and if no better, take him in. He is feeling so bad, he actually asked to go to hospital (which never happens). But he made it through all 16 miles of the walk. How amazing is that? Battling through with crappy, angry lungs, coughing all the way, he still won his own personal battle. 

CF is all consuming. It affects him in countless, immeasurable ways. Laying awake at night, listening to my boy go through this, is utterly heartbreaking. I can't describe it. I wrap my hands round his chest for physio (we do chest percussion when he is coughing this much, on top of his physio he does with his Aerobika device, as he gets so tired) and I don't just hear his rattle, his wheeze, his crackles, I feel them. 

At times like this, CF is all I can think about. I like to think I do all the worrying so he doesn't have to, but as he gets older, I'm not sure that's always true. 

A stormy autumn.

The kids are back to school, but already Isaac is coming home sick. Yesterday was not a good day. Today, Rosa was sent home after spontaneous vomiting in class too. This time of year is often not good for us. A new round of viruses to pick up and pass round the family. At the moment I'm hopeful that we can stay home and send in samples to the lab, which they grow to show any new or rampant bugs. All being well we stay home and put off CF clinic longer. He has had too many rounds of Cipro (the most powerful oral antibiotic that his bugs are sensitive to) this year to begin another so soon after IVs. Although he has other daily oral and nebuliser antibots, we have to reserve Cipro to occasional use on top of these, else his bugs will fast develop cunning ways to overcome it. He is still waiting on a date for his nasal surgery (which will involve removing CF related polyps he has grown, and widening his airways.... not nice, but very necessary) but his consultant is on the case. It's likely he will have more IVs at the time too, as the surgery and associated aneastetic will leave him open to infection rampage. I don't want to say the 'C' word in September, but I bloody hope it's well before that busy season, and we have him well and home for the holidays. 

The other night, unwell in bed in the early hours, he said 'Why me?'. 

It's times like those where I feel so helpless. 

And so I try and turn that helplessness round and start thinking about my next fundraiser..... core team (you know who you all are!)... anyone up for a skydive? 

This blog and home IV's.

I am often asked why I write this blog, and do I find it cathartic? The answer is absolutely yes, it feels so good to get things down in words, and I am a very open person generally, so happy to share (although of course I keep some things back that are not mine to share). 

I began this blog to raise awareness of Cystic Fibrosis, and Organ Donation. Although 1 in 25 people in the U.K carry the CF gene mutation (unknowingly usually, as carriers are healthy) only 1 in 2500 babies born have CF, and this amounts to around ten thousand people in the U.K. The most common, genetic, life threatening disease is still pretty rare. And increased awareness really does help, not only in raising more funds for research, but also to dispel myths, and reduce the number of crazy questions sufferers deal with. The organ donation part is linked as people with CF make up a large proportion of people on the waiting list for organs. The chances are, at some point, Isaac's lung health will be so low, transplantation will be his best option. Although this does mean swapping one disease, for another in a way (a lifetime of suppressed immune system and other drugs, along with their own special side effects). Currently half of those people on the waiting list for new lungs die waiting. 

The one thing I never wanted this blog to be about was pity, or to attract sympathy. I really hope that it comes across in the way I intend - to share, inform and give a glimpse into family life affected by a serious genetic disease, in a positive way, while still being very real. One thing to emphasise is that the treatment regime for CF is to treat infections very aggressively. This is why Isaac takes daily antibiotics, and occasionally has these courses of high dose (treatment doses in CF are much higher than for other people) IV antibiotics, which kick some serious bug butt. So whilst the treatments sound, and really are, pretty full on, this is all aimed at keeping him well, rather than because he is currently very un-well. As I am often told, you'd never know Isaac has CF to look at him. 

We talk very openly at home about CF, we never wanted to make Isaac feel it was something he should be secretive about, and he knows he can ask us anything freely, and without worrying about upsetting us. Anouk and Rosa talk quite proudly about their brothers CF, and that he has medicines into the 'wiggly' in his arm. They all understand that the many treatments that Isaac does, very much part of our everyday life, are doing just that; keeping him well, as such, we never refer to CF as being a sickness (although you will regularly find us all agreeing; CF sucks!). 

Some observations about home IV's; 

We very much prefer home IV's to staying in hospital (this study supports the idea too). It is no doubt easier for our family as a whole, and protects Isaac from the risk of picking up extra bugs in hospital. And whilst the physiotherapists do an amazing job, trying to increase his exercise and clear his chest, we can do more at home. He can't swim or do contact sports with a line in, but he can do most other things. 

If we do this more often (many kids with CF have regular courses of IV's every 12 weeks, rather than adhoc as Isaac does, maybe one or two times a year) we will need another blue bin. The amount of discarded packaging is immense! 

I am happy and willing to mix up and administer the IV drugs at home, which enable Isaac to spend less time in hospital. That said, I would be lying if I said it was easy, and I always felt relaxed doing it. Mixing up the drugs to give him includes many vials of drugs, various mixers, sterile wipes, needles, bungs, syringes, and making sure various parts don't ever make contact with other parts.... in hospital each dose drawn up by one nurse has to be checked and signed off by another; the patient, the dose, the use-by dates, whereas at home it's just me (thankfully with just a single patient mind). After all, I am pushing these powerful drugs up a line directly into his bloodstream. Problems can include spontaneous anaphylaxis, infection and the vein popping and the drugs going into his tissue instead of his blood. Scary stuff. 

My son is amazing. 

And so, we have come to the end of yet another course of IV's (have lost count how many he has had now), feeling pretty lucky. A single long-line, which came out today, has lasted the whole time; He's had very few side effects; We got to spend half the time at home, rather than the full two weeks in hospital; and most of all, he is feeling better. Plus I am the reigning queen of our new card game, Exploding Kittens. 

Huge thank you's to our parents for all their help, our visitors in hospital, Dan for surviving the long weekend on the ward while I got to spend some much needed time with our girls, and for all your lovely comments on FB. Isaac 1 - 0 CF x