CF Myths, part three

This follows on from my previous CF myth blogs.... Which you can see here and from there to there. 

I probably don't want to talk about it, so you don't ask: Not at all. I am always happy that people ask how our boy is getting on.... It shows people care, so please ask away. I find writing this blog cathartic in some way. It gets it out if my head somewhat, reordering things in my mind so I can focus on what really matters. 

But I can't talk for Isaac; it is for him to decide how much or how little he likes to disclose about his health, and for now, that is not much (mostly he just talks to me about it). If things are really not great, he might say more (and oddly I go quiet and say a lot less). 

But please never ask me about him when he is there; you wouldn't believe the comments I have had in the past, serious questions about his health, all whilst he is stood right in front of me. He has CF; he's definitely not stupid, deaf, or ignorant to the fact that you're talking about him. 

Remember, he is the only person with CF that he has ever properly met.... 

A lung transplant is a cure for CF: Sadly no. A lung transplant is only ever offered as a last resort scenario, where living more than two years is unlikely with your current CF lungs. It CAN give people years of good health that they may not have experienced since childhood. It can also bring more complicationso (problems due to being immune suppressed, and chronic rejection) than it solves. It will mean you would no longer have CF in your lungs, but you would still have CF in the rest of your body, meaning the digestive issues remain and the risks of liver disease, CF related diabetes, cancer, sinusitis, osteoporosis all persist, and can affect your new lungs. That said, transplantation is still a relatively new science, if average life expectancy after transplant is five years now.... but some are living twenty years or more, we can have high hopes for the future.

He looks so well, so he must be fine, right?: I wish. Like my friend Jayne says 'It's my innards that are fecked, not my outards'. Isaac looks great, most of the time. But he is one handsome little dude which may dazzle you to not notice that he can look kinda tired and pale at times. He can have relatively cough free days, but with physio sound like his chest is full of jelly. All the same, it's good to know he looks well, just don't forget how hard he works to stay that way. 

Wow, he takes how many tablets? OK, not a myth as such, but I never get how on CF documentaries, or when you have similar diseases on t'telly, they focus on how many tablets that person takes. Isaac takes around 40-50 tablets a day (for a start, he needs some with everything he eats just to digest fats and proteins), and yes, it's a bit of a pain if you've left them in one room and you're eating in the other and you have to go find them, or like the one time we forgot them, got the train to London, then spent ages in Boots trying to get some dispensed (they did, without a prescription, which we were eternally grateful for), but it's not like he spends ages swallowing down each one in painful gulps... On the contrary, he scares me by swallowing 10 at a time. Taking tablets is not hard (OK, granted, it was more of an issue when he was three, and some people are tablet phobic). But apart from 3 years olds and fuss pots, it's really not that hard. 

What they never explain though, is how the SIDE EFFECTS of those drugs, and drug interactions, might effect those people. Now that really can be a bummer. Isaac can barely stand near a low voltage table lamp before getting sunburnt when he is on Cipro. He gets a rash on Ceftaz. He gets grumpy and aggressive on Prednisolone. He gets a tummy ache from too much Creon. He gets a tummy ache from too little Creon. He has some drugs that he can't take one hour either side of milk. Or food, and others he has to have with food, otherwise his stomach burns...... Taking tablets is great, it's the side effects that suck. 

CF is a terminal illness: No way Jose! I once heard a parent describe CF as such, and was appalled. I believe the term 'terminal' describes a disease where you are expected to live less than one year. CF was later described by the Trust as 'Life threatening' and now 'Life limiting', which is more accurate in my opinion. No one knows what is round the corner. Science changes things so painfully slowly when you are full of hope, but it is changing things. We were told when he was a baby that his lungs would be irreversibly scarred by the time he was 5 years old, and we hoped that gene therapy would become a clinical option by then, before it was too late. He's almost ten now, and gene therapy is still a dream, but you know what, he's still doing OK. The life expectancy quoted now doesn't take into account new therapies. I don't believe in one single cure, but as our consultant once put it, rather there would be many small pieces that put together will improve outcomes greatly, and then one day, we will look back and think, wow, CF is a treatable 'condition' rather than a life limiting disease. We truly believe that all these small pieces we are doing now will keep him well enough to benefit for all those others to come. 

Have a great day.

It's a class thing, init.

So here is some science for you. There are 5 classes of CF mutations, class I being seen as the most severe and five as the least, but with lots of variance in each (you can have kids with a supposedly severe mutation who do really well, and others with "mild mutations" do quite badly, as well as twins with the same mutation who have very different outcomes...). Best analogy I can think of to describe these classes is a baking one I'm afraid, but I'm sure you'll all agree, a very realistic, factual account of the problem (CF friends may well want to correct me!); 

You really need to make a cake and get it to a party.... 

Class I; you've got some ingredients, but not all of them. You're fecked basically. 

Class II; the most common problem bakers have, you have all the ingredients, you make your cake, you just don't put it together too well, it flops in the middle. 

Class III; you make the cake, woohoo, it looks OK, but it tastes, well, not great. Maybe the slightly out of date butter wasn't such a good idea. 

Class IV; you make the cake, it's pretty much OK, but on the way out the door you trip, fall, and the cake goes everywhere. 

Class V; you bake a lovely cake, but just before you get to the party to share it, you realise the dog has eaten it in the car. The little shit. 

So, all pretty annoying, and maybe if you were in class five, you'd wish you had never started making the bloody thing in the first place. But in CF terms, the worst position to be in is class I, which means the correct (CFTR) protein is never correctly made, which in turn means a fault in the way water and salt move between cells, resulting in the sticky mucous that those blimin bacteria love to holiday in. 

There are some amazing things happening in CF research right now, and in the last couple of years, the first drug was licensed that actually treats the underlying cause of CF (until now, all treatment has been symptom treating). This is aimed only at people with a certain kind of (class three) mutation, of which there are very few (about 5% of the CF pop). Coming up soon, assuming it gets the astronomical funding that it needs (...which if it does, as the rumoured price stands, would cripple the NHS.... but that is another blog) will help the vast majority (who have class two mutations). This really is exciting times, and makes all the fundraising that we, and other families like ours do, feel so worthwhile. 

These new drugs, if they make it to the people who need it (and I have every faith that they will, eventually) will leave only a small minority of people with other mutations that won't benefit from them because of the different problem they have in the baking of that cake. I just hope that the pharmaceuticals (hopeful) success with the majority won't mean a lack of funding on other research for the minority left behind. 

... Like Isaac - who has two class one mutations. 

In other news, we had an appointment this week to discuss his rather rubbish immunology results, and the news was.... mostly reassuring. Waiting on further blood results now, but every reason to think that this requires no further treatment (which if it had, would mean monthly IVIG transfusions, which we REALLY don't want to add to an already full regime!). And Isaac is feeling great right now and was fab for his bloods. He celebrated with an M&S cottage pie (the one thing he misses from being on the ward). 

And just two weeks until puppy Obie joins us! So excited. In the meantime, one ex chicken this morning, resulted in a trip to get three new ones. Welcome Goldie 2, Floella and as yet unnamed white one. 

Have a great day x

Farewell friends

CF, it is said, is an isolating disease. Cross infection means that people with CF are excluded from things like conferences, where I might meet up with other parents from time to time. It's one of the harder things about CF, much more so for those suffering with it. 

Technology means that we can still share our stories though, and when you have a query about that bloody plastic part in your iNeb which pops out all the time, or you have that sinking feeling when the sputum is distinctly green one morning, these are exactly the people I need to talk to. So there are forums, and these tend to be either for people with CF, or parents of those with CF (I never ever thought I would be a forum kinda person!). Although the two can mix.... they don't always do so nicely; There are parents who write things like 'We could cope with another child with CF, so we're trying for a baby!' Or entitle posts 'My child's preschool allowed them to kick through autumn leaves!' shock horror (see previous blogs on these subjects). This tends to piss off some people with CF, as they are better positioned to recognise that having CF is about them, not their parents, and that physically and psychologically being able to enjoy life (particularly as a child when they are well enough to) is so important (just using some common sense about infections risks, as you would with any child). Equally, someone with CF might post about how annoying their Mum is about making them do their nebs (I'm sure Isaac will be soon...). 

I don't know why, but I often share the views of more adults with CF, than I do some other parents. I have tried to learn from their experiences with CF, and ask them for advice on how best to handle things at times. And while I haven't met many of them, I do count many as my friends.

But having friends with CF comes with some significant problems, in that they get sick; And sometimes they struggle for a really long time. Sometimes for a short time. And sometimes.... they don't make it.

Emily was one such person. She was a great character on the forums years ago (when I was terrified mother of a poorly baby and needed them more). She was very very sick at that time, and had a double lung transplant at age 22. She did amazingly well after that, and co-founded the charity Live Life Then Give Life (read more here) with her friend Emmie, to raise awareness of the serious lack of organ donors. She was a funny, bubbly and caring lady, and I am so pleased that she got to experience the joy of motherhood (through surrogacy) before she died. She suffered at the end from chronic rejection, pneumonia, and a second transplant came too late.

If you want to help at all, sign up to the organ donor register. Spread the word that you are more likely to need a organ transplant than you ever are to give one. And to make sure you tell your family your wishes before it's too late. Emily had 7 great years thanks to her donor, her legacy is that she has raised awareness so that others might too (as well as her lovely baby daughter). 

An absolutely amazing blog.

On the edge of sleep last night I thought of an amazing blog to share with you all. 

Forgotten all about it by morning. Sorry about that. 

All OK in Moly world right now x 

Happy New Year, but not for all.

Isaac is well. We are all well! Christmas has been pretty wonderful after all. I know it sounds cheesy, but I have spent much of it thinking about how lucky we are. We have three amazing children. A supportive family. Fun friends. A house that we have worked hard on to make beautiful (and it is our kind of beautiful). Jobs that we enjoy. Things to look forward to....

At the moment, it is a new addition to our household. That's right, we are having another boy! Albeit a four legged version this time. His name is Obie, and we are in love already... Pictures to follow. He is a German Shorthaired Pointer, and we will have him home in a few weeks. Kids are UBER excited. Of the litter of ten, he was the one that came right over to Isaac and sat on his lap the whole time... Like it was meant to be. We are all looking forward to another someone to love and to running around with him having fun.

And while we have been enjoying our festivities, presents, food, friends and family, all soaked in wine and love, my thoughts have also been with the daughter, friends and family of Emily Thackray, who has been so kind as to advise me about life with CF when Isaac was young, and has done so much for both the CF community and in raising awareness of organ donation for all who benefit from it (see here). I will miss you Emily. It all seems so fucking unfair.

Good night.