A cure? Yes please!

People often say to me "oh, there are so many medical advances, I'm sure a cure is just round the corner" which is lovely, and I certainly hope they're right. I am a total CF geek, reading all sorts of medical journals and following clinical trials, so if there was any wiff of a cure on the horizon, I would know about it. And I'll tell you now why I don't think there will be 'a cure' for us.

The underlying cause of CF is the CFTR gene which normally creates a protein that moves salt and water out of a cell. If the CFTR gene is defective, it results in a build-up of thick, sticky mucus in the bodies passageways.

Up until very recently, treatments for CF were solely focused on the symptoms of the disease. In 1989, when they first identified the defective CF gene they hoped that a cure was just around the corner, but it didn't quite pan out like that. I guess knowing what the fault is, doesn't always mean you can fix it.

Anyway, new treatments are finally being developed which correct the CFTR defect, they can help move or correct the protein and create some normal movement of salt and water out of the cells. The hope is that these treatments will help kids whose lungs are not already damaged by disease, or hold off further damage and stabilise those whose are.

But these new treatments work only for a small minority of people with CF with a certain class of the disease (class 3). They are hoping to develop another drug which will target the class of CF that is most common (class 2) - these are in clinical trials right now.

Unfortunately these drugs will not work for Isaac, who has class 1 gene mutations (associated with more severe disease) because in his case the protein does not need help folding correctly, or getting to the right part of the cell, it's just never made properly in the first place. Bugger.

Have I lost you already? I told you I was a CF geek!

However, that's not to say we are not HUGELY positive about his future. There are some lesser known trials happening on drugs which may help his kind of nonsense mutations, and that's just the small molecule drugs; There is also hope in gene therapy, which is going on in the UK, and is truly groundbreaking - this kind of research is not happening anywhere else in the world.

Plus there is something else we have high hopes for. Since the 1940's median life expectancy has risen from a few months, to the mid thirties. This is largely down to the use of antibiotics, physiotherapy, pancreatic enzymes, and a whole lot of other medication that he takes every day already. I believe that the closest we will come to a 'cure' is a jigsaw of a whole lot of different pieces. Take DNase, which he nebulises daily, this is thought to save 5% lung function. Hypertonic saline, thought to do almost the same... So that's 10% already, and bit by bit, piece by piece, we hope that put together, the myriad of treatments on offer, or to come, this will be our 'cure' - albeit one where he has a lifetime of treatments and medications. It will be a long lifetime.

I hope that gene therapy, or Ataluren, comes along and makes all my dreams come true. Every birthday candle blown out, every Christmas cake stir, every penny in a fountain, wishes come true. But if I'm right, we'll be OK too. Bit by bit we will beat this disease. We just need major pharma's to invest in new antibiotic research - this is a subject that will not just affect the CF population, but us all, if we don't do more about it soon (read more here ). Imagine all those evil little bacteria laughing at us, the big people, at their power over us, to make us ill, to kill.... Forget the Middle East, there are wars going on in our bodies, and we need to help arm against them!

Update on our boy soon x

IV's OV-er.

I am trying to resist the urge to say that it is just one thing after another right now. Clearly I have failed. I also tried to resist the urge for vodka tonight, and this too failed. 

Yesterday his line came out, and the IV's are no more. It's great to get the time back as well as the full use of his bruised and punctured arm. Mixing up the drugs when pharmacy had mistakenly given me 5ml ampules of sodium chloride when we needed 50ml for his Tobi took too long (Being uber sterile means numerous gloves, aprons, needles, syringes, bungs, meds, sharps bins.... The house looks like a dispensary). We get home, he goes straight out on his bike, only to wobble back to me with his chain cog thingy having taken a lump out of his leg. Straight to the doctors for a clean up and steri strips to hold him back together. No tears. It really was quite a chunk - my boy is either mad or very very brave! 

Clinic was OK. We are continuing with the steroids (which have him up and down and everywhere in between) and the hypertonic saline nebs for now. Hoping that the HS might be the reason he still sounds pretty fruity. It would be so good to have a little break from his cough after the truck loads of medication that they are throwing at him, but no (he doesn't cough all the time, mostly with physio right now, so not so bad). Lung function is a little down from last week, but not dramatically so, and that might be down to the steroids working to reduce his inflammation and therefore allowing him to cough up more gunk, which in turn affects his ability to do the tests well. There is still a broncoscopy looming if his chest doesn't dry up soon, but I'll worry about that another day. 

He is also being referred to specialists for two other issues that may or may not be issues at all. I'm not quite ready to talk about this yet, need to get my head around it first*. 

Get home to find that a regular job that Dan and a good friend of ours have been doing for one company for quite a few months now is over, just like that. He is a self employed builder, so it's not his sole job, but it was a good, regular income, and one that he has turned down other work to do. Now it means a last minute scramble for more work, leaving our income, more.... uncertain. Not great timing. 

On the otherhand, Isaac was on great form all day yesterday at the hospital, and we have laughed so much. Rosa did her first number two on the toilet, and black permanent marker does wash out of blonde hair (oh the joys of being a parent), and my Mum managed to fix the big lump of hair that I cut out when I decided I could possibly maybe trim the back of my (short still) hair myself. And it's good to be back at work, school, and some normality. Now time to plan some autumn fun (I love autumn). So lots of Moly merriness too. We're not quite OK, but we're getting there. 

Have a great day x 

*I do always check with Isaac that he is happy for me to blog about his health before doing so. He is old enough to know what he would like known and what he would not, and I absolutely respect that. He absolutely never has a smelly bottom for example. Never. 

Smiling on.

Isaac just about to go into his clinical trial last week. Smiling on. Love that bed hair. 

Nurse Anouk helping Mummy out with the home IV's. 

Hospital schmostimal no more!

Two posts in a row, because I had no wifi earlier.... What a difference a few hours makes! 

We are home! The steroids have done the trick - he is like a fire ball of energy right now, mood swings galore and eating for England (all known side effects), but his lung function is up at last! In fact, it's the best it's been FOR A YEAR! And we are home, woohoo! Continuing the steroids and IV's at home until Tuesday, when we go back for a review and more lung functions tests. They need to see him stabilised, but we are feeling much more positive. His fantastic response to the steroids means he may need to continue you with them long term. (CF friends; Perhaps inflammation rather than infection is his main problem. What do you think? Any experiences of long term steroid use to share?). 

He was so sad and feeling defeated just before the test, it was heart breaking. I am so proud of him, it's not been easy. Now I need to get back to my lovely family for some serious cuddle time. More soon x 

PS: Sorry, got to do a massive shout out to Ben, Wills, Sonny and Matthew for visiting so much (the parentals accompanying them were OK too ;0) it seriously made each day knowing that he had that to look forward to, and the biggest thanks to Auntie Bronte, who came everyday, and sat with him to give me a break. Love you. And again for everyone who helped with our gorgeous girls, who I have missed so so much. We are so lucky to have you all x 

Hospital schmostipal.

We have become super saturated with each other. It's been almost two weeks, and we're still not sure when we'll be home. Today he is grumpy and fed up and angry and frustrated and happy and sad and everything in between. I had a little cry in the garden. One minute he is sweet and cuddly, the next he is refusing to come when the doctors ask him to, and yelling at me. 

Sometimes it just feels so HARD. The day is one long list of things I have to ask him to do (nebulisers X 4, IV's X 4, physio X 4, clean your teeth X 2, go to hospital school X 1, let the doctors examine you X 2, nurses obs X 4, take your meds X 54,  wash your hands properly X 23.....). And I have to do it all with a smile on my face, holding it all together. How can you ask a kid to do all that and not sound like a nag? 

The good news is, he has put on 3kg since coming in, which actually makes me worry that he might actually pop. Isn't that how Elvis died? I checked this with his doctors, but they seem very pleased. His cough is slightly better, in that he coughs less often, and is sleeping better. The long line is lasting well, with no sign of infection. And most importantly, he feels OK. (It is very odd being on the ward where kids are coming in seemingly more poorly than your own, but the next day they are the ones going home, whilst day after day, we stay).

The not so great news is that he is still rattley. With physio he is very productive still, and a huff sounds less like a whoosh of air, and more like a chest full of jelly. This doesn't sound any better to me than when we arrived. He is still breathless with exercise. 

Everything hinges on today's lung function tests. The bottom line is, they won't let him go home until they have seen some kind of improvement in his lung function. On one hand I am so glad that they are committed to seeing is come back up, and not willing to accept that we might have lost that 20%, but on the other I am worried that we might not see an improvement YET (I'm sure we will see it, but listening to his chest, I'm just not sure it's gonna happen today, and if not today, then we'll be in over the weekend at least. It might only sound like a few more days, but it feels like an eternity to us). 

I think now that he feels OK, the only advantage of staying in is the weight gain (not because we feed him less at home, but just that out of boredom and inactivity, he is eating more here). Not wanting to knock anything they are trying to do here, but we were meant to be staying in for intensive physio, but I know he is doing less here than we could do at home. This morning the physio could only give him 5 minutes, and then when I ask him to do more, he argues that he's had all he needs (when I know by his cough, he hasn't). We have half an hour at the gym, but this is nothing compared to the exercise he could be getting at home, even on IV's he could be going on long bike rides. I can do everything at home, IV's, nebs, more physio, that we do here. He is due to finish the IV's in 3 days, but they have mentioned carrying them on for another week. We just really want to see that rise in lung function today so we can do that extra week at home. 

I think I'm sounding a bit desperate now, sorry. Our girly girl is crying before school every day, missing me and having the new school term to cope with at a time when she only ever gets to see one parent at a time, and when she does, they're pooped and worried about everything. We miss our family, our home, our beds. It's hard to be positive all the time. Hospital seems to have zapped it all up. Wish us luck. 

PS; When I started this blog, I promised myself that I would be honest, else what would be the point? So this is an honest account of how I am feeling right now. This blog is about life with CF..... It is not an illustration of our lives as a whole, so if you find it a depressing read, and I sound like I am moaning all the time, maybe find a blog about cats or something. I hope for our family and friends it is a good way of keeping up with how he is, and that it also raises awareness and funds for CF, and names on the organ donar register. We are currently experiencing a CF blip. Happiness will resume shortly. Have a great day x 

Staying in....

We won't be coming home today as we had hoped. There is no improvement in his lung function yet, it's just kind of static, and they want him to gain some 20% before we get out, to get him back to his baseline (really hoping we're not looking at his new baseline).

Started some mega dose steroids to put the heeby jeebies into his inflammation problems, and hope this improves things enough by his next test (Friday) otherwise we could be looking at a broncoscopy (camera down into the lungs for a little scoot around, and a wee hoover out while they're down there, which means theatre, general anaesthetic, and most importantly to him, some time NIL BY MOUTH (his most dreaded phrase, along with 'Hospital School' - oh yes, he is now having a hour a day with a teacher too - he is not impressed!).

Good news is his long line seems to be lasting well, no problems as yet, and he contunues to feel OK(ish), but for the coughing. Chest X-ray showed no pneumothorax.

So again we are thanking all his visitors, family, a friend who kindly sent him a Costa voucher for his hot chocolate addiction (thanks Gabe) and everyone who is helping out with the girls while we stay in. Dan is back to work tomorrow so I am in full time for the next few days, just in time for the steroids, which always have him grumpy and bouncing off the walls. Wish me luck!

Will update when I can (wifi access only in some parts of the hospital). Have a great day x

When the drugs don't work....

I'm not sure I've explained what IV's actually are, have I? In case you're not sure, by IV's we mean a course of intravenous antibiotics. When the drugs don't work (by mouth or nebuliser to his lungs) then IV's are the heavy artillery of bug killing - by feeding the antibiotics directly into his blood. At first he had a normal cannula (a short line, in his hand) but this was then swapped to a PICC line (which is a long line, from his elbow right up to his chest). The advantage of this is that it should last a lot longer, hopefully for the whole two week course, whereas a cannula would only last 1-3 days using it as much as we are. It's also much more comfortable for him. The disadvantage of a PICC line is the risk of infection, which is why have to be so careful when mixing and giving his IV drugs. We have had PICC lines fail before (becoming blocked, or tissued) so we are really hoping that this one lasts the whole course. He was very brave having it inserted on gas and air, but it took two attempts, and it's a long and sometimes painful procedure, so he would be very disappointed to have to do it again. 

For my CF friends reading, he is on Ceftaz and Tobi, for assumed Pseudo again, although his last culture was lost in the lab so we're still waiting to hear what he is currently growing. We've started hypertonic saline again (which he is tolerating much better than when we last tried it), salbutamol, and may start steroids next as his LF is actually down from when we first came in. He is really productive with physio - so we assumes it's just lots to shift. We are just swapping his three times daily Ceftaz to twice daily, in the hope we can get home in a few days and he can get to school some of the time. 

We had some weekend leave today, so took him home for a few hours. I felt OK until then, but crashed in the afternoon. Napped for an hour or so. Think it's all catching up with me. 

It's odd being on the ward when he is outwardly very well. I mean, he is coughing a lot, but is eating fine, and running round with the physios. He tires quickly, but he feels, kind of OK. You have other kids come in with acute problems, or post op, who seem so poorly, but then they're home in a day or two, while we are still here. It's also funny how quickly you become institutionalised. It feels normal now to be popping down to Costa in my slippers, microwaving M&S ready meals for my supper and lying awake at night to the sounds of hushed voices, machines beeping, and babies crying. 

We need to see an improvement in both his cough and his lung function by Tuesday (next test). I don't want to accept that we might slowly be losing some LF with every infection that we're not ever going to get back. He's just too young. 

I'm sorry, I think I'm over tired. Will be back to CF butt kicking positivity in the morning! I'll leave you with a picture of my boy on the beach (can that really just be a couple of weeks ago?) surfing on, despite the thunder clouds looming over. 

Huge thanks you's to our visitors for keeping him going, our parents for helping with the girls, Dan for always being there and coming to watch us sleep (love you) and biggest thanks of all - to the AMAZING NHS Trust with whom we sleep tonight. 

Sweet dreams. 

Hospital stays.

I am home for the night! This is a mixed bag for me, it is the first time I have ever left him on the ward, and the first time his Dad has stayed with him. It's not been an intentional thing, just the way it has always worked out. So I am home, with my gorgeous girls for the night. Little Rosa sang to me as I rocked her to sleep 'let it go, let it go' and now Anouk is staying up late (she needs some special Mama time) and we're watching... Yes, you guessed it, Frozen. I am both delighted to be home, and yet somewhat sad - I think I need to learn to let it go a bit too. 

The good news is he is feeling OK - the physio's have him in the gym daily doing all sorts, we fill our days with giant chess and connect four in the hospital garden, super mario and endless nebs, IV's, and physio. We have both made friends on the ward, and being in hospital is always hugely humbling. We come away feeling very lucky for our lot. 

The bad news is, his cough is about the same, and his lung function has dropped again. Damn it. Early days. He may be started on steroids if there is no improvement soon. 

This admission has been unusual for us. In the past we have stayed just a day or two, before going home, where I complete the two weeks of IV's at home, with community nurses input where needed. It's only been when he is very unwell where we have had to stay in longer. This time they want him to stay in for the first week or so, which we were not expecting. They are trying both intensive physio and hypertonic saline, both of which we can do at home, so hoping we can get him home soon. I have refreshed my IV training (he has a PICC line in, which can be a big infection risk, so we have to be super dooper sterile in mixing up and administering the drugs). We just want to get him home now. Better sleep.. Better food...  Our family all together. 

Being home without him.... Just feels, wrong. 

I made it home from the the hospital in ten minutes. Which tells you two things; we are very lucky to live so close to an amazing (CF Specialist) hospital, and that when I am tense and need to see my lovely girls, I may speed. 

Things that I have learnt (again) this week; My boy is so brave. Hospitals are crazy hot. Every hospital hour = three normal hours. Children's wards are not for light sleepers. People can be so lovely.

When I ask him how he feels about staying in, he said it is OK, painful, fun, tiring and good for snacks (as well as three pretty good NHS meals a day, his dietician drops off extra snacks as he need to up his weight...  This and the Costa hot chocolates and M&S extra breakfasts, which are costing us a small fortune, keep him very happy). He is not unhappy about missing school either. Yet. 

Hoping for better new to report soon. I'm sure we will x 

IV's it is....

Just waiting for a bed on the ward. His cough seems worse. IV antibiotics, steroids and intensive physio. 

More soon x 

Thank you for your support.

Canoe trip went wonderfully - huge thanks to everyone, in particular the Campbell's, who helped come up with the whole idea and made the event such fun. Also to the Fountains, Pledgers and everyone else who joined us, and helped feed and cheer the boys on. Special thanks to my fantastic father in law for setting up camp (and, embarrassingly, saving me when I got the car stuck in the mud miles from anywhere) - don't know what we would do without you.

You can still sponsor the boys by clicking Here

Our family and friends have never failed to completely overwhelm us with their support. However large or small a donation, we know the thought, hopes and generosity behind each one. THANK YOU so much. For this event we are over £1800, and since Isaac was born, up to £45,000 raised for the Cystic Fibrosis Trust. Woo hoo!

PS; Hospital tomorrow. IV's or not IV's... That is the question. Wish us luck.