This isn't flying, it's falling with style...

On May 5th 2018 I will be jumping out of a plane along with my amazingly supportive and slightly crazy friends and family, Bonny, Jess and Shelley. Luckily I am good with heights, however I do have a mortal fear of rickety old flying machines, and, well, death generally, so this will be no small feat. 

We are doing this to raise funds for the Cystic Fibrosis Trust. Our family and friends have now raised over £55,000 for the CF Trust since Isaac was diagnosed.... such incredible support, we can’t thank everyone enough! 

In 2005 when Isaac was born, he could only be prescribed medications to treat the symptoms of his disease, and sadly, despite dreams of gene therapy, that is still true today. But we are on a brink of a whole new way of treating CF...tackling the underlying cause of the disease, which is all about abnormal movement of salt and water between cells. One drug (Kalydeco) is already available to those few with a CF mutation it will work for, and another (Orkambi, which will treat many more people with CF) is available in other countries, but not yet on NHS England, due to cost (thanks to an overwhelming response to the petition the CF community have been sharing on every social media platform going, we hope this will be debated in parliament very soon). 

Unfortunately, neither of these drugs will work for our boy (he has two class one mutations, which is really crappy) but it fills us with hope for the future; however a huge amount of research needs to happen in order to deliver this kind of treatment and ensure that everyone with CF has access to these groundbreaking small molecule drugs. 

We can personally vouch for just how shitty CF is: 

It means my son has to spend 1-2 hours a day doing treatments, even when he is well; 

It is the reason he has a bacterial chest infection all of the time, and why daily antibiotics only help keep the bugs at bay; 

It is why he has spent two of the last six months in hospital; 

Which means his attendance at school this term has been less than 50%; 

CF is why my daughters worry, lash out and have to cope with us prioritising their brothers health over everything; 

It is the reason he has had two painful operations recently;

....And why that he ended up in intensive care for six awful days; 

It is why he takes dozens of tablets each day, five nebulised treatments, along with rounds of physiotherapy to shift the mucous from his lungs; 

It is why we worry about diabetes, liver disease, deafness, osteoporosis and cancer, all of which can be linked to the disease or its treatments;

CF has resulted in his lack of venous access, after years of blood tests and IV treatments, and is why he now has a permenant IV device in his chest for easier access;

Cystic fucking Fibrosis explains how he was left wheel chair bound, on oxygen 24/7 and unable to even stand without becoming breathless for a time.... 

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

Petition, for young lives!

Please, please take one minute to sign & share this petition. It has received more than 10,000 signatures in 24 hours and we need it to reach 100,000.... 60K and counting! 

You could help save the lives of thousands of young people with cystic fibrosis. Thank you.

Making our own sunshine.

Isaac is home from hospital. All is healing well. All is as it should be. A happy video for you here!

Have a great day, and thank you for reading x 

Some of my favourite from Man Ray; 

Operation.

I used to love that game, growing up in the eighties.... we never had it, so it forever remained an elusive and fun thing that we only ever played at family and friends. Ditto my neighbours barbie oven which had lights and actually hummed like an oven. Oh how I lusted over that oven aged six! 

Isaac is now the proud owner of a shiny new portacath in his chest, which will make blood draws and IV antibiotic treatment so much easier. He has been in a fair amount of pain post-op, the incisions are larger than we expected, and he’s had all the usual fun stuff post anaesthetic (throwing up, massively sore throat from the intubation, lips so dry they crack and peel). But it is in, and it is working! He remains in hospital, and his cough is still worrying me, especially as we are coming to the end of two weeks of IVs, the point at which we’d expect his chest to be the best it can be. Hopefully this is just post-anaethetic-cilia-need-to-wake-up-effects. This is an actual thing, I didn’t just make that up. We have lined up samples for the lab, so have the usual wait now for all the test results. But for now, we just want him home. His sisters need us all home. 

Welldone and thank you to all those that will or have been attending NICE meetings around the U.K, campaigning for the right for people with CF to access Orkambi, a life changing drug; 96-week data shows that Orkambi can slow decline in lung function – the main cause of death among people with cystic fibrosis - by 42%. There are 2,834 people in England, 243 people in Scotland, 118 people in Wales and 101 people in Northern Ireland who could benefit from Orkambi. Sadly Isaac is not one of those (due to his rare and quite shitty mutation) but I can completely relate to just how maddeningly painful and frustrating it must be for other parents and adults with CF to know that this drug is there, it’s bottled up, ready to go, and your doctor wants to prescribe it for you..... but can’t. What price can you put on a life? 

Thank you for reading x

Floods, Op’s and home IVs

We are home.... still on IVs, but home for much needed family time for the weekend. I am back into the swing of administering the IV drugs; it’s all aprons, gloves, endless alcohol wipes and checking the measurements and dilutants obsessively, but my confidence grows quickly each time. His chest is  sounding much better (the sound and feel of his lungs is a great measure of wellness, from a crackle to a wheeze, to a vibration or a gurgle). 

Tomorrow we return to hospital for his portacath Op, subsequent recovery, and to hopefully finish off the course of IV antibiotics (they will also use the opportunity under anaesthetic to suction up more sputum from his lungs to test for New Twattish Microbastard), Thursday marks the two week point, the usual length of the course, and unlike last time, I’m confident his lung function will have increased enough to finish on time. 

This admission has been nothing like last time. Still a little way to go, but we are feeling fortunate and pretty damn chipper; We need this operation to go just as well, I’m sure it will. Have a great day x 

My flooded fen dog walk at dusk: 

Sleeping with other people.

As chirpy as we try and be about hospital, there is one thing that rarely gets any easier, and that is sleeping with other people. Because, let’s face it, a thin blue curtain does nothing to change that fact that you are sleeping mere feet from others, and while I enjoy my own pretty hippy and carefree attitude co-sleeping, life on a hospital ward is something else, and we’ve had some mixed experiences... 

The whole light etiquette: come evening, we try always retire early; knowing our night will be interrupted in a hundred different ways, we have to go for quantity rather than quality. But when is it polite to turn off the main lights when you’re sharing? I’m all for bed at 8pm on the ward me (so frickin rock’n’roll!). We once slept next to a family who every night angled their bed lamp so it was actually touching our curtain, facing our direction, giving them a lovely dimmed light, and us, the full beam. I silently wept and tried to knock it away, as if by accident, with an outstretched yawn. 

Space: The children’s ward we frequent is split into a few individual rooms (which we only get if he is contagious, or if there are a number of CF kids on the ward all of whom need to be kept apart), and other rooms with three, four or six beds. The more beds, the less comfortable you will be. Fact. On the six bed ward, I once had a parent push their chair so far up against the curtain dividing us, his chair was touching my back as I laid on my side. We were next to each other for two whole weeks. 

Crying: The worst was the sweet baby that came in for cleft palate surgery, who cried all night; a shrill, gargling, piercing, and hungry cry. I honestly think Isaac had about an hours sleep. I assumed the babe was nil by mouth pre-op, and having been there myself, it wasn’t hard to empathise with the poor mother. But when the dad came in the morning after, I heard him ask the mum, ‘have you tried feeding her?’ And the mum replied ‘no, I thought she was just tired’. I’m telling you.... ALL BLOODY NIGHT, and she stopped as soon as she was fed - I think it’s sometimes possible people see the illness first, and the baby second. If that makes sense. 

Night arrivals: There are the families that come up in the middle of the night from A&E, who have probably have had some kind of BIG LIFE EVENT of sorts, and it is a known fact that people talk around 22% louder during such episodes than they normally do. Kids often come in with parents, sometimes with siblings and grandparents, maybe aunts and uncles too. And all of these worried and loud people are right next to us as we try and sleep. You understand, but sometimes it doesn’t make it much easier when you’re tired. 

Privacy: This week, we had the 14 year old who was on her phone all the time and tried to have her 18 year old boyfriend stay over (the nurses kicked him out). I felt like I knew her by the time she left without hardly talking to her, we’d accidentally overheard stories of snapchat screenshots going viral round school, her social worker, going back into care, her mother calling social services on her father, her sister being in the pub all day, her warning friends to back up her story to the police (really).... how can you not listen when you’re close enough to hear each other roll over in bed? As she kept rolling back in on her wheelchair smelling of fags, I desperately wanted her to go, but I also wanted to give her a big hug. Equally everyone knows exactly what is going on with us, be it an intentional or accidental invasion of privacy, I get it, but sometimes that is hard to deal with (even for me, who writes a blog about this life. Kind of ironic I suppose). 

Environment: Then there is the inevitable snoring; the heat (wards are always so hot); the hospital hum (the noise which I imagine comes from his generators, boilers, air conditioning units? I hope it’s not the incinerator, I don’t like to think about those); the wondrous smells of all varieties; and of course, the forever, incessant beeping of machines. 

Anyway, I could go on and on, but feel bad for moaning. There are positives... occasionally at least. You can meet amazingly interesting families, and make friends even. We are definitely in the regulars club now; We know all the nurses, and the nurses know what Isaac needs: space, more than most, only because we have to bring in so much medical equipment with us (two nebulisers, bi-pap machine, all of our usual drugs) so where possible, they do try and get us a good bed. And while the majority of people we meet here we only ever see that one time, there are others regulars like us. Sadly I can’t meet up with a good friend of mine, whose daughter also has CF. We are often in at the same time, and text each other from our respective beds, but cross infection is even possible from *patient to person to person to patient* (if that makes sense!). So we support each other from afar and wave if we see each other (good luck for today A&G! Much love to you both). 

Today has been a little difficult for Isaac. His port op has been delayed again, and should happen next week now, unless his longline fails sooner (in which case he should get on an emergency list). He had quite a sleepless night worrying about the op, I think mostly because he had two very recent general anaesthetics, and for both, his experience in recovery was rough (initially that is), so he is understandably frustrated for it to be delayed a second time. Then he had his hopes up to go home for a few days, on home IVs, as his lung function today had increased nicely. However the doctors are not quite ready for him to come home, and will review him on a daily basis until they are. That said, things are otherwise going well. 

I, on the otherhand, have come home for a good nights sleep and a cuddle with the girly ones while Dan stays with Isaac. It’s always bittersweet for me though, I feel like I am missing a limb when I’m not there with him (despite the fact that he hit me round the back of the head with a football earlier in the gym. Little shit). Love him x 

The long escape route from hospital....

Chronic illness.

Chronic illness is very hard to explain sometimes. Isaac’s baseline ‘well’ is almost definitely not the same as your ‘well’, but life is no competition on suffering. I honestly don’t mind when people have a grumble about a particularly bad cold they have been suffering with for a while, but people often apologise for doing so. This last couple of weeks I have had a broken or bruised coccyx (I say broken or bruised because I decided not to have the X-ray to check, as either way, the treatment is the same; try not to sit on it, anti-inflammatories and painkillers). And jeez, did I moan! Only the diazepam/tramadol/naproxen cocktail shut me up (mostly because I was an incoherent wreck crawling around on the floor...I’ll stick to the mojitos in future).

When someone expresses concern about a blood test while Isaac has had more needles than hot dinners in his lifetime, I don’t compare their situation to ours. When people have effortless pregnancy after pregnancy, I don’t bemoan the fact that we had to have IVF. Coping with chronic illness in the family does not diminish your empathy for others. For someone with near perfect health, a new health experience can be a very scary thing, whereas for Isaac, we are used to tests and subsequent results on a rolling basis (and some will always bring bad news) and I guess in some ways this desensitises us to some aspects of hospital life. 

This week we met a particularly useless surgeon who proceeded to inform us that Isaac would have his port fitted in the side of his chest, rather than at the front, because it would leave less of a scar and be less visible. Isaac protested that he wanted it at the front because he doesn’t give two hoots about a) the scar or b) the visibility, as it would still be below his T-shirt line, and that for him, accessibility was his priority. The surgeon just really couldn’t get it (he also called him ‘James’ throughout the appointment, which never fills you with confidence). When we explained that depending on what IV drugs Isaac takes, he might be hooked up to drugs for some hours each day, so to pull down his T-shirt, and have the line pop out his neck, leaving his arms free to go wherever, is a lot better than having it on his side where he would need the line to come up, down or out his sleeve. Plus, one day we hope he’ll be independent and able to do his own IVs, this is only possible (I think, or at least it’s a lot easier) if the port is on your front. The surgeon still looked at us, mouth agape, before finally conceding that Isaac was right, and then proceeding to talk us through the surgery; 

Dr Useless; And we make one incision here (pointing to childish drawing he is doing for us). 

Me; Really, Just one incision? 

Dr Useless; Yes, that’s right. 

Me; But don’t you also need an incision in his neck? 

Dr Useless; Oh yes, that’s right. Two incisions. 

Me; But you’re not the one doing the surgery on Isaac, right? 

The flip side of chronic illness is that yet another admission to hospital, when it is expected, even planned, is a very different context to a first or emergency admission. This is our reality, and for the most part, that’s OK. Last night I stayed in with Isaac, and we gossiped and giggled about the people on the ward (I have a lifetime of shocking stories about the families we see on hospital wards, and please know, this is not us snooping, we are in a four bed ward, and last night, this meant a room full of maybe 15 patients, visitors and nurses - only divided by curtains, you can’t help overhear, and all I can say is that last night was like an episode of Jeremy Kyle!). We stayed up too late, we ate chocolate after cleaning our teeth, we overslept. Went to the gym for physio as soon as we woke up and raced each other on the bikes. Then I swapped over with Dan to spend some time with the girls. We shopped, went to a funfair, and I laughed my head off on the dodgems. Then we snuggled up with the dog in front of the fire and watched Dumbo. 

I think sometimes, because we do carry on, we laugh a lot, we throw a lot of parties, and we make the most of every day, and yet we still prioritise Isaac’s health, we can be labelled as (eek!) inspirational. And this is NOT a word I have come up with, people say it to me all the time. One CF blog I read said this: 

“Frankly, many people treat the chronically ill as inspiration porn. So when we express any type of grief, they act as though we’ve rudely interrupted their televised programming for something unscheduled and unwanted”

But before I very publicly fall off any hypothetical pedestal you might put us on, can I tell you that we do moan, we do cry, we do have tantrums, throw weetabix on the wall and slump against the fridge crying. I can certainly tell you that I myself feel anything BUT inspirational when I’m tired and usher the girls up to bed with their iPads because I JUST WANT TO HAVE A GIN IN PEACE. And Isaac has his little paddies too. 

Sorry, this post has no real outline, no conclusion. Things are going well in hospital. Isaac is coughing more, but that is usual for early on IVs. The physios are pushing him hard, which is great. And best of all, we think his port surgery will be bought forward to Tuesday now, so we just need his line to last until then. Depending on his recovery, we hope to get home on home IVs soon after if we can. I guess what I am trying to say, is that if it was your child, you’d be doing the same too (only probably better, or at least with less gin), and that mostly, life is not so bad. It was a completely different ball game last time, when he was so sick, but right now, we’re doing OK. 

Thank you for all the kind wishes x 

Georgia O’Keefe love tonight. 

The year of the chest.

While 2015 and well into 2016 were the years the digestive problems, with repeated admissions for DIOS (distal intestinal obstructive syndrome) and continued problems with nausea and vomiting, 2017 was most definitely the year of the shitty chest. While he has grown the bacterial infection he has in his lungs (Pseudomonas) for many years now, we used to be better able to keep it at bay by just occasional courses of IV antibiotics and a whole heap of oral and nebulised ones, but we are now much more reliant on the toxic IV drugs to battle this bug. 

His surgery to implant a portacath (permenant IV device, in his chest) was scheduled for tomorrow, and we had pre-op today. However the surgery was postponed (Addenbrookes is one of the hospitals in the news today, declared as in the midst of a major incident, as they battle wards full of flu and the noro virus). We now hope to have it scheduled for next week or the week after, so still headed in for the pre-op chat today. Because we were going in anyway, I spoke to our CF nurse and as he is still coughing despite four weeks of cipro, she asked us to pop in for lung function tests. The news was neither surprising nor good. 

The plan over the last few weeks was to hold off IVs until the port was in to avoid the pain and hassle of trying to get a line in again, and as we seemed so close to this date (and so wanted a Christmas at home) the doctors wanted to wait. But today, with him still so symptomatic and knowing the port is postponed (and could be even further, because let’s face it, the NHS is in crisis) we can no longer delay. So armed with only the clothes we came in wearing, we found ourselves back on ward D2. Isaac was pleased as we got one of his favourite beds, overlooking the garden, and despite his venous access being so pants, he already has a longline in - first time! We very much need this one to last until the surgery now, as I can’t believe we’d be so lucky a second time. 

So we are back in a day earlier than we had thought we would be going in, and a frustrating delay for the port, but it’s certainly been better knowing that we were coming in for planned IVs anyway; the bags were already half packed, the time off work was booked, and the girls were prepared for the news (no tears at all today - I can’t tell you how different that is). Isaac is in a much much better place with his health right now than for his last admission - despite the coughing he has a great appetite, he is fit again, happy and rested after a lovely break at home. 

Our hopes are; IVs this weekend, lung function starts to improve, surgery goes ahead.... and then we go home to finish his two weeks of IVs at home, through his shiny new port. 

I will let you know how we get on, but so far, so good, still smiling x 

Pictures from just a few days ago, out on his Christmas present.