Orkambi.

Exhausted but finally home after a day at Westminster to protest about Orkambi - a ground-breaking drug that could save the lives of thousands of young people with Cystic Fibrosis. It's available in the USA, France, Germany & Ireland, but not in the U.K. This has to change. Read more here. 

Today was great, excellent company, and something I am proud to be part of, and whilst it's not a drug that will directly help Isaac (due to his rare mutation) I believe it will open other doors. Orkambi's UK list price is some £104K per patient, per annum, which NHS England simply cannot afford, but these protests (we hope) will restart negotiations with the pharmaceutical company. 

Many young people who could benefit from this drug will lose valuable lung function the longer this goes on, or worse still, die waiting. 

 

Crashes and Coughs.

After a really good spell with his chest, we've hit another rough patch. It's hard to explain, but his cough sounds and behaves differently to ours. Deep and reverberating. Coming in waves. Infection without fever. This means extra physio, samples to the lab, and CF clinic this week. He is not far off his school camp, so really hoping his lung function tests go well. 

He is also cut up and bruised from a bike accident. On the way home today from a 10m ride to a nearby town for a drink and pasty by the river, he flipped my fixie bike over. It's all elbows and knees, but as I saw it all play out in front of me, hell my heart burst out of my chest! In his usual good humour he picked himself up and carried on. 

I absolutely love our time alone together, he is the best company. His CF dictates that we spent more time together than most sons and mums, but I can't think of anyone more wonderful to do this with. Bloody love that boy. 

This weekend was also our little Rosa's fifth birthday. We surprised her with a disco dome bouncy castle in the garden for her party, lights and music included. Such fun, and some pretty cool physio for Ise (bouncing is great for clearing mucous). She had the best time, our little Banosa, and Anouk made it the best party by mothering all the littlies, she is so wonderful. We partied until late. 

I am off to the Orkambi protest at Westminster tomorrow. Read more about this here. I am looking forward to venting some energy in this great cause, hooking up with other CF parents, and fitting in a swift Tate Modern visit on my way. 

Have a great day x 

All the worlds troubles.

I'm sure I'm not alone in feeling like the news everyday, both political and tragedy filled, is overwhelming at times. Feeling sombre seems like the national BAU these days. In Moly world, we can't help but be humbled and reminded of how lucky we are. Isaac has been well, despite his ongoing problems with his nose (for which we are still waiting for a surgery date) and tummy (including some pretty spectacular projectile vomiting when the kids and I were home alone last week - think sick on the walls, skirting boards, rugs, dog.... It's both a little sad and also a point of pride that the kids all knew their roles in this, little Rosa got towels, Anouk got the water, and Isaac managed it in his usual good humour). But the best news is his chest is great, which is probably the one thing keeping my glass the right side of half filled right now. 

I'm working with a teacher at his school and his CF team to work out a way that he can go away for 4 nights in July without me (a first for us), hopefully on a reduced treatment regime that he can manage on his own, with supervision. I know my anxiety about this will be far higher than his, which is how it should be. 

Today marks the first day of the UK CF week. I have always supported and promoted this, as I can say for sure that more awareness of CF would benefit people with CF, it is still so often confused with other conditions and misunderstood, and this is really difficult at times. I've even had a GP ask me how long Isaac has had CF....! So please, share my posts and encourage people to sign up for organ donation. Some relevant ones can be found here and here about CF myths and here about giving life. Thank you. 

Colour run and other stuff

Isaac, along with his Dad, cousin Ben and Auntie Jo completed his first 5km colour run today, and had a great time. This is a run with 20 obstacles and a whole lot of colour. Sounds easy, but not so much when you have CF and his current nose/tummy problems. Thank you so much for the sponsors so far, and if you'd still like to, click on the image of him on the right in the red t-shirt. 

 

Isaac and Ben, best friends and cousins. 

 

 

Ben. First one over the line. 

 

My lovely and ever supportive sister, Jo. 

I have not been feeling so great lately, which is weird, as Isaac has been more stable. When I was a teenager myself I swore that if I were ever a parent I'd remember exactly how it felt, and therefore I would totally ace the teenage/parent relationship, right? I thought I'd be SO much better at this than I am. Most of all I never wanted to be a nag. But the hand dealt to us by the genetic lottery means our son has so much more to do each day than most, and at times that feels like all I do...

Even when well his day consists of physio therapy, nebuliser one, nebuliser two, nasal sprays, inhalers, medicines he hates but has to drink, tablets, even more tablets, more physio, nebuliser 3, nebuliser 4, nebuliser 5 (hell yeah, CF includes a LOT of breathing treatments!) more tablets, medicines he hates but has to drink, and many more tablets whenever he eats, all interspersed with a Mum checking he feels OK, requests for coughing up sputum samples for the lab, a good 'hands round the chest' huff to check his lungs, O2 saturations and temperature checking, plus all the usual homework, teeth brushing, eating both vertically and with cutlery (this request still surprises him) that we have to ask of him. 

Now we can ensure he does his treatments every single day, 100% of the time, but what we cannot ensure is to what effect he does these, as he needs to put the effort into his breath to do breathing treatments effectively, and we can't measure that at home. Therefore, he insists he has done enough (the required number of breaths, huffs and coughs) but with effort of a geriatric mouse, and so we can't be sure that his chest is cleared of the infected, inflammation causing mucous that clogs his airways. The teenage years are about giving our kids the responsibility for their own lives little by little, and we have to allow them to make their own decisions, however good or bad. The only difference for us is the potential impact of those bad decisions. We have fought hard to keep his lungs as well as we possibly can for 12 years, and seeing him resist, cheat, and lie through treatments leaves us heartbroken. 

People often ask me how we cope, with three kids, both working full time, and with CF to boot. And the truth is, I don't always, at least not very well. And just lately I have felt the balance between coping and not has been kind of, well.... wonky. You know how they say people think about sex every 7 seconds or something? I doubt people look back on their day and think, wow, I thought about sex 376 times today! It's a back of the mind kind of thing, and that's how I feel about CF too. It doesn't dominate our lives exactly, but we sure as hell live alongside it. It is always there. (edit; actually, the whole 7 second thing I might have confused with a goldfishes memory? Either way, hopefully you catch my drift). 

In a crisis, I am a lioness for my cubs; I generally do OK in hospital, even when it has been very hard. And at times, it has. It's when I get home that I crumble (typically by popping open the gin and listen to The Cure or Patti Smith on my headphones very loud and having a little cry). It might surprise a lot of people who know me that I take antidepressants for anxiety,  and have done for many years, and I don't mind saying that, as I feel passionately about mental health awareness. Why should I hide this fact, but openly talk about physical problems? (And everyone knows that I'm a chronic oversharer on health generally (see previous blogs from earlier this year about living with a catheter bag!). It's all the rage anyway.... Prince Harry is talking about mental health, as are other celebs, so we must too, I think its great and does go some way to dispelling some perceived shame about feeling a bit shit. 

I asked my best friends to describe me in three words, and they said; positive, loving and empathetic; creative, honest and generous; bubbly, fun-loving and thoughtful; Spirited, loving and imaginative. This was not me fishing for compliments (although it did cheer me up some, as I like to think I am all of those things to my friends, as I love them dearly) I'm trying to illustrate the point that you can suffer from anxiety, in truck-load of worry proportions, which at times can feel quite debilitating, but also be optimistic, happy, and be a good Mum. And I really am happy, and I do feel like a really lucky person. It's just OK to be anxious, sad, and admit that you're not coping so well at times too. For me, this means focussing more on doing what I like most, hugging my kids until they squirm, spending more time with those closest to me, and figuring out how to get that balance better between work and play. And since many people with CF suffer from poor mental health at times, due to symptoms, limited life expectancy or the burden of treatments, I hope I am showing Isaac that it's OK to not be quite so OK always too. 

Have a great day, but if you don't, that's OK too x