If you have nothing nice to say, don't say anything at all.

My mum was always full of sound advice. And that's kind of how I've been feeling this week. Not really wanting to update until I had something positive to say.

But....  Isaac is still not well. 

During the Gastrograffin explosives of last weekend, he came out with a rash, all over. Our best guess at the moment is that it was due to the new medicine (we hope side effect, rather than allergic reaction, as we'd like to be able to keep this drug in our arsenal for future use). He's also been tested for other possibilities, and we await blood test results for those, none of which would be good news. 

His tummy is much better (lots of other extra drugs to help avoid more DIOS) but the best thing about the last few weeks is that his chest has been great (and sorry guts, but you are a poor distant relation to those regal organs that we call lungs. In other words; Lung health is the booyaka of CF). 

But now he is coughing. Really coughing. And, I can't quite believe this, but it very much looks today like he has tonsillitis again. That in itself is not strange, but the fact that he has had tonsillitis for the past two years at exactly the same time (as in, he breaks up from school, we plan to go away, and boom, he gets tonsillitis, which in turn gives him a crappy cough and we end up in for more IV's). I so don't want the same summer again! Please let me be wrong. Is tonsillitis even seasonal? 

Some happy news. Spent today at a party that Starlight throw every year (read here about our  experience with Starlight, an amazing charity who makes dreams for kids living with illness come true). Was so much fun. Isaac threw up in the car on the way down, coughing too hard, and struggled all day, but still wanted to stay and even played a little football. The girls got the most amazing face paint, and had a ball. 

Tomorrow we were due to go camping for a week, with some of our bestest friends. But after months of good weather, the forecast for the next week is just terrible, so we're not sure what to do. It makes me all the more sad for the kids, as the last few weeks has been difficult enough, for all of them in different ways. And we can't afford to do anything but camp right now. Isaac may not be well enough to anyway (back to the Dr tomorrow), so it may be beside the point, but the saying it never rains but it pours seems annoyingly apt right now. 

I know deep down that none of the above is insurmountable. We can and will cope. He will be OK. Things will get better. But also, I must admit I'm struggling. Missing a lot of work (so spending evenings catching up), feeling worried all the time. It just feels like the breaks in between the problems are getting fewer and far between. 

Merriness will resume shortly, I'm sure x 

ps; People have asked about the clinical trials that I spoke about previously; no news yet. But still hopeful for something to happen this year, it's a question of where right now. It looks likely that we will have to travel outside of Cambridge, and unfortunately not in London either. 

Back in....

Isaac illustrates what his medicine tastes like; 

And how he keeps on smiling anyway; 

And some happy pictures from last weekend; 

Home again soon (we hope) x 

DIOS... Again. Back in hospital? Maybe.

Isaac has this (sorry for the wiki quoting, but kind of sick of people comparing this to normal constipation. It is not!)

Distal intestinal obstruction syndrome (DIOS) involves blockage of the intestines by thickenedstool and occurs in individuals with cystic fibrosis. DIOS was previously known as meconium ileus equivalent, a name which highlights its similarity to the intestinal obstruction seen in newborn infants with cystic fibrosis. DIOS tends to occur in older individuals with pancreatic insufficiency. Individuals with DIOS may be predisposed to constipation. Features; Symptoms of DIOS include crampy abdominal pain, vomiting, and a palpable mass in the abdomen. X-rays of the abdomen may reveal stool in the colon and air-fluid levels in the small bowel. Management; Surgery is often required to relieve the obstruction. However, if there is no sign of bowel rupture, a more conservative approach may be attempted. Restricting oral intake, placement of a nasogastric tube for decompression of the stomach and proximal intestines, and administration of laxatives and enemas may resolve the obstruction without the need for surgery. Individuals prone to DIOS tend to be at risk for repeated episodes and often require maintenance therapy with pancreatic enzyme replacement and stool softeners and administration of laxatives and enemas may resolve the obstruction without the need for surgery. Individuals prone to DIOS tend to be at risk for repeated episodes and often require maintenance therapy with pancreatic enzyme replacement and stool softeners.

There were no beds on the ward, so we're home, with some meds, but may be back on the ward tomorrow if he is no better/they have room for us. 

Need to add, we have the most wonderful CF team. Especially our fantastic CF nurse Caroline, who we caught on her way home, having just been told that the pharmacy could not prescribe us the drugs he needed. She went out of her way and spent the best part of an hour sorting us out, as everyone else went home. We can't thank them all enough. 

Thanks also to Jin, for listening to me moaning today, and my work colleagues who didn't mind Isaac sitting in the office for a bit while we waited for his doctors to call (that's a first!). 

After that I reversed into a large post and scratched all the back of the car. 

And worse, the best Mum in the world is also in hospital overnight with a bad knee, and I can't even go and help her as I need to be here. 

Today can just piss off (sorry for the language Mum) x 

New lungs new life

Hate this paper, but This article is pretty good. Check out the GOSH documentary too (Great Ormond Street: Fight To Breathe will be broadcast on BBC2 at 9pm on Tuesday, July 21). 

Pretty hard to imagine that the only difference between Isaac and this gorgeous, brave and courageous boy is one year and a really horrible infection. 

Thankfully new lungs were found for Charlie. Please consider signing up, which you can do Here. 

Have a great day. 

CF fame

 

I often feel a bit mixed after CF is in the news or on TV. It's either portrayed in drama as a 'terminal illness' where sufferers are pale, poor skinny souls without a hope. Or in the news as the NEXT BIG BREAK THROUGH disease. A cure is imminent! We can all throw out the nebulisers and look back on these days and laugh at the hours of physio endured! 

 

The truth is, it is mostly neither.

 

Gene therapy is ground-breaking science, it really is. And like others in the CF community, I am hopeful that one day this may be a therapeutic treatment for cystic fibrosis. But I also believe we are many years away from this, and after the science, the funding will also delay this further. I'm also not hugely excited about a 3% increase in lung function. Isaac's can vary by 15% from one clinic visit to next. 3% and lung function stable? That would be, promising, but not exactly epic (as Ise would say), is it? I believe this is only the beginning of the long story of gene therapy. It is progress, and I am hopeful, but I'm not yet excited. And people saying to me how fabulous this is doesn't help much - I must look a bit deflated when I am less enthusiastic than they are. Sorry guys. But we've been here before. 

Gene therapy was the buzz phrase when Isaac was a baby. We asked our consultant about it then, and she looked kind of flat in the same way. She surmised it might only work well in healthy lungs, not already destroyed by infection, inflammation and scarring. We asked at what age Isaac might have signs of this, and she replied age 5, maybe. When he way a baby, 2013 was talked about as a time when we might hope gene therapy drugs to be in the clinical setting. 2015 and we're still many years away. And yes, he probably does have all these signs already, but the trial showed positive results for those less well, so it is far from hopeless even though the wait continues.

 

A consultant once told us that they did not believe in a cure for CF. Which sounds like a crushing blow, but I think I agree. Rather, there will be many new therapies (small molecule drugs, new antibiotics….) that put together will hold off decline.

 

So great, the gene therapy results have raised the profile of CF (which is always good, helps raise funds, as well as awareness) and people are excited for us. Thank you. I'm just not sure that Press publicity accurately reflects what we should actually be excited about. Other trials results are a lot less sexy, but no less important. 

 

In other Moly news;

 

One puppy, one less chicken. Oops.

Fibrogel is the evil cousin of Movicol - can anyone really drink that stuff? Back to the Movicol battles.

Summer holidays are fast approaching, and the Moly's will be going camping soon. Obie's first trip. We may be home sooner than we think.

My baby is 3! And starting pre-school soon - where did all the time go?

Isaac won most improved player of the year for his football team, and Anouk lost her first tooth and has her first horse show soon. Not sure which she is most excited about.

 

Have a great day.

Home

We are home. 

Happy Father's Day men folk x 

Hospital survival guide

While we are here, and it's fresh in my mind, here is my official hospital survival guide, should you ever be so unlucky to need it; 

Bring flip flops or slippers. Wearing shoes on the ward feels, just wrong. Pyjamas and shoes? Not a good look, and you don't want to be venturing in toilets bare foot. 

Take your own pillow/blanket. Home comforts to make things feel a lot less cold and sterile. Plus hospital pillows are plastic coated, the crinkley sound as you roll over just reminds you of the thousands of other people who have once dribbled on them too. 

Books, magazines, games, cards. I may remind you here that every hour in the real world, is three hours in hospitaland. You will need entertainment. And at least here at Addenbrookes, the kids wards still have no Wifi. *** note to NHS, this is probably the single biggest thing you could do to improve the hospital experience for kids and parents alike *** No need for toys, the playrooms are great. 

Bring money. We rejoiced when M&S food opened on our concourse, but between that and Costa hot chocolates (complete with cream and marshmallow) it is haemoraging our bank balance. Not to mention the parking and TV/films (... £10 for 24 hours). 

CF families, I suggest you bring all your own medicines from home, everything. You know all the doses, routines, cleaning and preparation required, and just explaining that to the nurses would take as long as doing it. As fantastic as nurses are, if they do all the normal drugs as well, everything will be late, in the wrong order, or somehow, just-not-quite-right. Plus, as parents on the ward, we have jobs too. The nurses have enough to do. 

Invite visitors. Make sure you ask for help, most people (I hope) are happy to be asked. Grown up company for you, but the kids want kids, so ask their best friends/cousins. 

*** Note to visitors *** No need to bring anything, just come ready to play, he wants to be distracted and Mum wants a break from getting her butt kicked at Mario Kart. But don't stay too late - no one sleeps well on the ward, so we need our early nights. Also, best time to come is early evening, the days are mostly filled with doing or waiting for the next treatment and visits from numerous doctors and what feels like hundreds of medical students. If you come in the day, you risk not finding us as we're out at X-ray, lung function, ultrasound..... 

Treat yourself to nice pyjamas. Almost worth having a 'Sunday best' pair just for coming in, if you come in as often as we do. The consultant that you see all the time in clinic is soon going to see you in your nightwear. Would feel a bit odd in your old grey granny pants and your husbands old t-shirt. 

Use the parents kitchen. I used to live on sandwiches and rubbish when Isaac was little on the ward. Now we have M&S I have microwave spinach and ricotta cannelloni, or have soups and salads. Much more fulfilling. Also lots and lots of tea. Helps pass the time. Tea helps everything. 

And a word of warning. Prepare to manage in hospital, but have a meltdown the day after you get home. Happens to me every time. You hold it together when you need to, but it's got to come out sometime. 

Back in hospital

We are in again. This time on the baby ward, not because he has shrunk, but as that was the only ward with a cubicle available. He needs privacy as this time it is tummy related.... CF friends will understand. DIOS hell.

I am feeling tearful as we spent a lot of our first year here together. Many memories of C3.

Hoping this one will be a briefer visit. More soon x 

Third time....

Jayne has had two calls now. You can read about the second Here.

This is no bog standard, call from your Mum, or the local neighbourhood watch to tell you about the latest stolen hanging basket. This is, we may have new lungs for you, so you can like, BREATHE, and be well, and get one with your life with your lovely family.

Can you imagine? Each time, it's been a no-go. This time, the lungs were no good (but thank you donor for at least offering).

Having known others on the waiting list for new lungs, this is; to-be-expected-but-still-bloody-annoying. I know people who have moaned more when their dentist has cancelled an appointment. I know people with CF who have had almost a dozen calls like this before getting new lungs. 

I'll give the lovely, funny, wonderful Jayne a little twist on a saying she will almost definitely be hearing right now; 'third time fucky?'. 

Love you funny lady. It will happen. I can feel it in me creaky old bones x x x 

Our roundabout.

Our week.

1; Wakes up with tummy ache. Pain. Folded over. Vomits. Eats. Feels better. School. 

2; Wakes up with tummy ache. On the loo all morning. Can't eat. Braves school. 

3; Wakes up with tummy ache. I vow to call dietician. Doctor. Start the dreaded 'food diary'. Is it not enough Creon. Too much Creon? Looks pale and sad walking into school. 

4; Wakes up, feels fine! Panic over. 

5; Wakes up, tummy distended. Movicol, Movicol, Movicol. DIOS again? Can't leave toilet. No school. 

6; Wakes up, tummy ache. Vomits. Bug at school. Is it tummy bug? Or side effect of new meds? Or not enough Creon? Too much Creon? Last nights curry? 

7; Wakes up, feels fine! Panic over. 

And repeat. 

Feeling helpless. 

Super bugs are taking over the world.

Or, to put in a slightly less hysteric manner, multi-drug-resistant organisms are spreading — like klebsiella (a bug Isaac was chronically infected with when he was younger for some years) or MRSA, the multi resistant form of staphylococcus aureus, which commonly affects CF lungs. Even infections that used to be easy peasy to treat, like gonorrhoea, are becoming incurable. Tuberculosis alone kills 1.4 million people a year. One reason for this crazily high number is that most people in the world are too poor to pay for medicines, but another is that strains have become resistant to the array of drugs we have. Even after two years of toxic treatment, drug-resistant tuberculosis has a fatality rate of about 50 percent...

Add to this the ease of access, in some parts of the world, to buy antibiotics over the counter – which people are over using, and not finishing courses, which only helps breed super bugs. I saw an experiment once where they spread bacteria on an agar type table, and added the correct amount of antibiotic, which killed the bacteria. Take the same table and add a little antibiotic, but not quite enough, and the bacteria not only overcame the antibiotic, but it mutated and got all the way over the table and had a massive super bug-party. 

Asking your doctor for antibiotics when you don’t really need them is adding to this problem. As is not quite finishing the course she gives you when you do need them…

Old antibiotics are being phased out at twice the rate that new ones are being developed. We are losing the most important kind of medicine ever discovered, and why?

Duh duh dah… MONEY SCHMONEY! Historically, the drug industry loved antibiotics. But if an antibiotic is useful against only one type of bug then relatively few people need it during its patents life (patents run for 10 years from invention, I think?). And if an antibiotic is “broad spectrum,” meaning it works on many different types of bacteria, wider use shortens its life because resistances develop faster. Also antibiotics are designed to cure an acute disease, so only used for short periods. So not a great money spinner compared with drugs that are used for years and years to treat widespread conditions like asthma. 

But CF is one exception…. People with CF are on daily antibiotics, usually numerous ones (oral, nebs and IV’s). It’s the single biggest thing in our extensive arsenal to keep Isaac well. 

The other problem is how blimin CLEVER bacteria are. Alone they are pretty feeble bunch, but collectively they gain intelligence. They learn together how to survive antibiotics. And the more they work out how to survive, the harder it is to find drugs that can beat them.

So how do we get drug makers to want to try? Addressing these two problems might be a start… 

Monopoly protects the ability to set price for profit. Patents allow monopoly – can they not allow longer patents for new antibiotics for a start? 

Like bacteria, who share what they learn and get smart, could drug makers operate in the same way: academic and government scientists and other drug companies working together to share what they learn… collaborate and innovate? I know this is happening in a small way already, thanks to philanthropists, which shows that even competing research teams can share knowledge, risk and avoid costly redundant efforts. 

Or maybe rewarding drug makers for products in proportion to their IMPACT? The lower the cost of a lifesaving antibiotic, the greater the number of people who could use it; the more lives protected, then, the greater the reward. If common infections start to kill again, due to super bugs, then surely the impact of a new antibiotic would be huge? This is already happening people! 

If we don’t create new antibiotics, the super bugs will win. They were around before us, and they will be around after us. Little shits. 

 

Old neb, new neb, family love.

Found Isaac's first ever nebuliser. Look at the size of it.... He is pretending here, he can't even remember doing this now. His is now handheld, silent, and quick(er). This was like a washing-machine-volumed-steam-machine. We had Thomas the Tank Engine on loop, full volume to get him to sit there long enough at 18 months old. Don't miss those days, but funny looking back now. 

And some pictures below of the kids with their cousins, Ben and Ellie, who mean the world to us also. They are all super close. And some Obie love and Rosa dressing up. Got to include those! 

Happy weekend all. 

I hate mostly.

The week started with another sadness. Another life lost to CF. I am so so very sad for him, and his family. Feels so frickin unfair. 

Isaac's tests from his Immunology clinic came back as 'mostly reassuring'. I hate mostly. Why can't we have completely. Or absolutely. Or incredibly? 

Crucially some of the vaccines he has had in the past have not worked sufficiently, so he'll be having a few more jabs this week. I'm so glad that I pushed for the referral to the specialist.... He has CF, but little immunity to pneumonia.... ? Worrying. They are also going to keep an eye on him long term. There is a chance this immune deficiency of sorts will remain subtle. There is also a chance it may not. None of this is linked to his CF, just shitty luck I suppose. 

On the sunny side, he is well. We are all well. We have lots to look forward to. We are the lucky ones. We have new parquet floor, the garden is starting to look lovely and Obie has stopped toileting in the house. And shhhh, I am making secret plans for Dan's 40th.... (Not sure he reads this anyway!). 

Have a great weekend. 

Here comes the sun

Life is pretty sweet right now. Rosa is tucked up in bed. Isaac and Anouk are playing on the trampoline enjoying the evening sun. Those two can be pretty terrible together at times. Whilst they both dote on little Rosa, just the two of them and they become, if I'm honest; a bit of a bully and a whining tell tale. But lately they have been getting on better... Maybe, just maybe they will become better friends. 

Kids are all well. Obie is mad, funny, lovely and infuriating all at once. Work is going well, and right now I'm feeling immensely grateful to have a job at all (cuts, and friends I will miss). Dan is busy too, and has also just finished laying our new parquet flooring. Get us eh! Love it. Love him. 

Him and Isaac at off on a rugby tour soon, we have a holiday in Wales, weekends in Norfolk, Cinematic Orchestra for my birthday as well as Rosa's 3rd, summer parties, BBQ's and a wedding to look forward to.... 

Life may have thrown us a few lemons, but we're sure mixing them up with vodka. 

The sunshine of my life

And so the sun arrives, and with it, some hope. Isaac is doing great right now. Back to a normal routine; normal for us at least. Back to 4 nebs a day, base physio. His chest has been great, and his tummy better. Girls continue to be absolutely gorgeous, growing up fast. I find myself telling people at work that I have a ten, six and two year old, and yet I still can't believe I really do (I'm still a big kid myself at heart). Still waiting for news on the Ataluren trial. You prepare yourself for long and frustrating waits when it come to health, but it never gets easier. Fortunately for us, it's frustrating as we're excited, not frustrating because we need this, and we need it NOW. Long may this continue. 

So for us, spring is lush, and green and wonderful.... Although my thoughts are also with Jayne, still awaiting new lungs, a friend at work, affected by CF also, and a teeny tiny baby born yesterday. Be strong little guy! Let spring be good for all. 

Have a great day. 

A year of blogging.

Isaac is really well. Still having a few issues with nausea, but hoping this will resolve soon. 

March has been jam packed. Spending a lot of time with family and friends. I had a couple of low weeks, if I'm honest, but it has just reminded me how lucky I am to have such fantastic friends. So thank you to all, especially Lemon and Bon for picking me back up, and to my wonderful sister Jo, who celebrated her 40th, and raised over £600 for the CF Trust in lieu of presents. As well as a great party, we had a weekend away in Norfolk with all the family, including our Obie (who is growing up fast, and an absolute joy). Isaac took some friends to Alton Towers to celebrate his birthday, and had a ball. 

Feeling very lucky. 

Thank you for reading.... One year on, and I'm finding this blog is as I'd hoped it would be; a great way to share our news, as well as raising CF awareness and getting people to consider signing up to the organ donor register. My friend Jayne just had her first call.... Sadly the lungs were not for her this time. Wishing her well until the right pair come along x 

Annual review; clinical trials, my heart goes wild.

Annual review today, and some exciting news! 

First the not quite so very good; Lung function was down, but only 5%, so nothing to start worrying about. And he has new medication, another to add to his already long list, but if it solves this tummy pain, we're smiling. 

Now the good news; No further changes on chest X-ray. Diet praised by his dietician. Ultrasound fine. New physio trial to do (he can hook up his Acapella to a game on an iPhone, really cool, and should help focus and entertain him). Glucose tolerance test all groovy. Waiting on blood and dexa scan results. And..... He has no scoliosis (this has been suspected for a while, not necessarily CF related). Phew. 

He was really really well today, lots of physio in clinic, and hardly a cough. The whole team seem really happy with him. He was chatty with our new psychologist too, which makes a change. 

Now the fab news; Our favourite Consultant is very hopeful we can join a clinical trial for Ataluren within this year. Either here in Cambridge, or we transfer to care in London for the duration of the trial (a year). Ataluren is something I have been reading up on for as long as I can remember, and would be the first (and at the moment, only) drug that would work for Isaac that actually treats the underlying cause of CF; 

In short, his gene mutation puts a stop sign on the end of an otherwise good message to produce the CFTR protein. This drug puts a blanket over that stop sign, so the protein is then made. This COULD be a game changer drug. And our boy will be in the trials! Too soon to be getting too excited, but it feels like really positive news. Instead of endless treatments for all his symptoms, imagine a single tablet that *could* halt the progression of his disease.... I'd be lieing if I said I wasn't a teeny bit excited about this. 

So.... Happy happy day. Long old day, but we laughed so much. Then home to Dan, our lovely girls, and Obie shnobie. Love love love! 

Have a great day all x

Happy birthday lovely boy. 10 today.

Dear Isaac....

My wonderous boy. How can it be ten years since we met you? I was 27 when I had you, and you were a planned and much hoped for first baby. Still, it all happened so quickly... Pregnancy was amazing, absorbing, and somewhere along the way, I think I forgot that at the end of it, we would really, truly have a baby to take home with us. My first words to you, after a 3 day labour; delirious in drugs, sleeplessness, and ecstasy 'it's my baby, it's my baby!' As well as overwhelming love, I felt oddly huge surprise - how was it that we could produce something so exquisite, so soft, so perfect. 

You were such a smiley baby, always on the go. I remember holding a friends baby girl one day, and thinking there must be something wrong, she sat still... Was that even possible? We called you a multitude of names (a few which I will list here just to embarrass you; our tinkleberry, Isey bonisey, bonbon, cheekycheekynose, poppychops). We would just look at you, and look at you, and marvel some more. We had a baby monitor with a camera over your cot, which meant we could watch you on our TV at night. With every toss and turn, we ohh'd and ahh'd. 

I knew you, really knew you. I had a new pea in my pod. We laughed at the same things. You loved diggers (your first word 'didder, didder!') and Thomas, and so I did too. I loved humous and butterfly kisses, and so you did too. Dad would rush home from work to lay on your bedroom floor for hours, teaching you to roll over, or playing games under the covers. 

Always on the go. Fidgety. Funny. Active. A water baby, always wanting to swim. So very sociable, always wanting to be with your cousin. You craved fun with friends. Becoming a brother, you were better than we ever hoped you would be. You loved little Shnuks passionately. Like you loved Rosa again years later. 

Your growing independence would have been so hard for me, had I not had Anouk and Rosa to fill that cuddle time. But I still miss you. Luckily at nights I can get my fix, occasionally still crawling into your bed, where you immediately shift over and spoon, like we were never apart. 

I love you my gorgeous boy. I could not be prouder. You are everything I had dreamt of, and more. 

I had a moment after you were born, a sharp intake of breath, and a sinking feeling so hard, instantly winding me; a pain. It was the enormous realisation that I loved you more than I could have ever imagined my heart being capable of, and that whilst I would protect you with my life, there may be some things even a mothers love cannot protect you from. What had I signed myself up for? A life time of BIG LOVE but worlds of worry? Your Dad picked me up, and I knew that having you made me and him a team, for life. Whatever happened, we would always be your parents. We could love and protect you together. 

Three weeks later, we found out you had CF. 

Happy tenth birthday my amazing boy, you make me laugh every day, and a teeny tiny bit of me is still reeling from the shock 'it's my baby!'. 

Always, Mama x 

Tummy aches and Obie shakes

Isaac has been really well lately, chest wise, but he somehow seems to suffer with more tummy problems between chest infections.... I'm not sure if there could be a reason for that or not (I suspect Sod's law). Could be any number of things.... He agrees that it could be that he is either not taking his Creon on time (at the beginning and in the middle of his meals); he has forgotten to take his Creon on occasion (we only know of one instance of this for sure); he has outgrown his Creon dose and needs more (awaiting results of food diary now); he has another blockage (needs more Movicol); he has a tummy bug (probably not, going on too long now); it could be for any of these reasons and a whole lot more that we don't yet know about. So, clear as mud. 

He had a blip like this a while ago, a few days in I go to call his dietician.... but just as I am about to, it gets better for a few days. The mornings are always the worst, he is back and forth to the loo, and curled up in pain at times. It's so frustrating not knowing the cause, and so sad seeing him like that. 

But I'm also feeling really proud right now. His Creon dose is dependant on the fat content of what he eats (plus, rounding up a bit for high protein foods), as its fats and proteins that he cannot digest. He has always needed the full whack of Creon, and had very high doses as a baby, which he seems to have grown into more now (for CF friends, he takes Creon 10,000, anything from 25-50 a day). We know he manages well on about 1 Creon to every 3-4grams of fat. Over the last couple of years we have moved from labelling items in his lunchbox with how much Creon each is, to giving him a set amount and letting him work it out himself. He is so good at this now. Even with homemade food, without wrappers to refer to, his guesses are pretty good. This makes a huge difference to him; it means freedom to just grab a pot of Creon and go. 

Please do feel free to contact me on FB of you're reading, I love questions. In reply to a recent one, Obie is a German Shorthaired Pointer (GSP). He is settling in so well. Few toilets get issues if we don't keep an eye on him, but he is just lovely. Very cuddly and loving, as well as active and a bit bonkers. So he fits in well around here. Loving his little pointer shake when he sees us. He has just started to go out to work with Dan (he's a builder) so he's busy having fun every day now. 

We are fast approaching Isaac's 10th birthday, which is unbelievable, scary, funny and a little sad all in one. Can we really have met him all that time ago? Could he really leave home in less time again than that? How can he only have one more year of Primary school to go? Our little tinkleberry is all pre-teeny and I don't know where all that time has gone. 

Plus, 10 whole years of our life with CF. 

Birthday also means annual review. I usually dread, no, dreads not the right word, but dislike mildly going into clinic these days. It's not because it's a bad day. Actually, it's a rare time to be alone with Isaac, and we have fun playing don't step on the cracks all through the hospital corridors, get an M&S lunch, and see our lovely team. It's that when he is well, and we're on a high, we ALWAYS come away with an almost glowing report, but not quite. There is always a new thing to worry about, another niggeling worry to fit at the back of your mind. Or when he is not well, and we expect bad news, it might be better than we hoped but still, we still go home with the same symptoms, maybe unresolved. Plus, who ever wants to go into hospital? Full of sick people.... and who wants to bring home Noro virus for the weekend? 

Annual reviews are different though. I prepare myself fully. Arm myself with expectation that it won't all go our own way. It is a full day of tests; bloods, X-rays, ultrasound, physio review, exercise tolerance test, in depth lung function, dexa bone scans, doctors review, CF nurse review, dieticians review, psychologist review, and new this year; test for diabetes. 

All in one llllllong day. But although we could find out a whole lot of news we don't want to know, we feel positive that we won't, and if we do, we know that knowing is for the best. 

Have a great day. 

My family

In no particular order, I would like to thank (in the Academy award style); 

Rosa: for so much fun yesterday, playing our made up game, dinner or daughter, in which she was either showered with kisses or eaten. And for saying repeatedly that getting Obie was 'best day ever!'. 

Dan: for everything, always. I'm so sorry that I'm a grumpy lumpy in the mornings sometimes. I love you. 

Anouk: for asking how NOT to have babies, and when I said the easiest way is not to have sex (sexy cuddles she calls it) she replied, 'no way is anything coming near my cha cha'. Age 6. Go girl. 

Isaac: for a fab try at rugby today, and for humming away to music I love in the car, which he pretends to not like in public. He will have great taste! 

Obie: for being so unbelievably gorgeous and fitting into our family so well. It is early days, and maybe I'll be cursing next week, but he just eats, sleeps and poops right now, and as long as one of those is outside, and he keeps giving all these cuddles, I'm happy! Love love love him. 

Obie!

Our gorgeous new boy.... 

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.

A Merry Moly Christmas after all....

Merryness resumes. We are all well for Christmas! At least, we were all well all the way until Boxing Day when Dan woke with a dicky tummy, but we shall blame the cheese for that. We have had a fantastic few days of festivities.

Wishing you all a healthy and merry time too x

A sad film, a happy note

a sad film to watch here

Isaac is feeling better today, hospital put off for a few more days at least. Looking forward to feeling festive now. Goodnight all x

Sick of sucky sickness

I am sat here listening to my boy coughing and I want to cry. 

I am feeling crappy too, just a nasty cold thing, but it doesn't help. How can I be the one looking after him when I can't stop coughing and spluttering myself? 

We have just been away for the weekend with friends, which was great, he managed really well, but home again he looks thinner, pale, and exhausted. He is already on the big guns (Cipro), we have upped his hypertonic saline nebs, physio, and added inhalers. Nothing more we can do right now but hope that he turns a corner very soon. 10 days til Christmas. That should be 10 days of fun filled Christmassy things with his friends and family.... Not home doing more treatments. The mission now is to get him well. And part of that is hoping that we are all well.... It feels like an endless game of pass the bug in our household this winter. Bleughhhhh. 

Merriness to resume shortly. 

Almost festive.

It hasn't been the best of weeks. The Molys need some merriness back! Isaac's chest sounds a bit yuck again, which we dread any time, but all the more so before the holidays (I can't remember a year where we haven't been a teeny bit worried about spending Christmas on the ward). Work has been stressful, but as people who bang on about their jobs are about as entertaining as Ed Milliband, I'll stop there. I will just say that I had to fire someone last week, for the first time ever, which was no fun at all. I know I need to take a step back from all the work stress before I implode... It's just so busy and the pressure is huge. It's not normally this bad, I love my job, it's just.... A LOT right now.

Christmas Break is what we all need. I just can't wait. Dans back is hurting again (he had surgery on it last year) which is always a worry (he is self employed, so bad back means no earning). He works so hard despite it. Went to Anouk's nativity today (our little Elf) and it was surround sound coughing there are so many germs at school right now, it makes me want to keep them at home just to avoid it. Only little Rosa is having a good week, finally getting the whole potty malarkey, woo hoo!

I want to smother myself in family and friends, and yummy food and drink, and loud music, and dancing around the table, and kisses and cuddles, and tickeling fights, and bitter cold walks followed by the fire and hot food.....and and and.....to forget everything that ever worries me. Actually, just writing about this has made me feel better. Roll on Christmas!

Merry Molys

Clinic this week bought good news. His lung function is up another 2%. Doesn't sound like much, but it's going in the right direction, even though he is off the steroids. He is a little rattely again, so we're upping his nebulisers. Five a day instead of four. On a good day this would take him 50 minutes, but in reality, it can take anything up to 2.5 hours, mostly down to his lack of concentration (his neb is breath activated, and it only releases the drug when he has breathed deeply enough for the drug to reach his small airways. Clever things nebs these days). But overall, a good visit, and as always we are so thankful to have such a fantastic team at Addenbrookes.

I'm even beginning to feel Christmassy. We have much to look forward to this month, and beyond. I am one of those people that has to have a project or plan to look forward to.... Be that a baby, an extension, a party, a fundraiser.... and our next plan is something entirely new for us. More to come on that soon! Happy days all x

PS; and just in case she reads, Happy Birthday to the most amazing Mum ever. Last weekend she organised (yet another) fantastic CF fundraiser, and there washing up, I found myself helped not just by her, but also my step dad and my dad. Our family has changed in so many way, but we all come together when it counts. I am very lucky to have them all.

And then there were three...

My children are all playing together. Yes. That's right. All three. And for a whole 42 minutes now there has not been one 'Maaaaammmm' from their den. OK, they have every cover in the house in my room, they have probably stolen some chocolates from the jar, and the den will take a while to deconstruct when they are in bed, but still.... It's so rare these days that they all get on. It's the elder two mainly at each other's throats. Everyone finds Rosa Banosa so funny you just can't get mad with her.

I don't think about it so much these days, but it's moments like this that I do. How lucky are we? How the odds were humongously stacked against us having a second child, let alone a third. So thank you for science, and to a bloody amazing NHS that gave us this chance, our complete family, our everything.

And breathe....

Isaac is doing really well right now, so I have less to blog about. His little moon face is going, and he is back to normal. His kind of normal at least. Clinic next week, and hoping if his lung function is OK, he might be able to stop the hypertonic saline nebs. Four lots a day is taking him a couple of hours, the speed he does them, so one less would make a big difference, and might make getting to school on time more likely. He has appointments in December with an immunologist too, to check out some long standing issues. Hoping for reassuring news before Christmas on that.

I had a lovely weekend break in Norfolk with two of my best friends, eating, drinking, walking, and laughing so much my tummy muscles still ache. I really needed that, I think we all did. Love you guys.

The girls and I are finally getting over our coughs, bit of a bugger that bug, and the toe I think I may have broke stubbing it on the box under the bed whilst ever so slightly under the influence seems to be less purple and throbby. Oh, and I was promoted at work, which makes all the extra hours I am doing because we are so busy that bit sweeter. So all good in merry Moly world right now. Hope you're all good too x

Feeling ill, but not for long.

It's my turn to cough this week. My lungs feel like they are on fire, like a huge itch on the inside. It's funny how when you're well, the idea of a sofa day, while your husband takes the kids out and you have rare custody of the remote, seems quite welcoming. What you forget is just how sucky it feels to be ill. 

For me, this is just a little insight. I slept for 12 hours last night (albeit it coughing the whole time, my body kindly allowed me to sleep in-between). Tonight I will have inactivity induced insomnia even though I still feel tired. But I know others who live this feeling, day in, day out. When climbing up the stairs becomes a struggle, and drug induced nausea makes even the small joy of eating an enemy. 

I think I allow myself this little fantasy where all the love, and treatments, and family, and creativity, and fun, and friends, and action, and fresh air, and hoodies with that soft fleecy inside, and hot chocolate...  all of this and more will somehow really save him from ever feeling that bad. But I guess even I must concede that just occasionally he will feel pretty damn crappy, and what I feel right now is just a fraction of that. 

I know that the chances are I will wake up tomorrow and feel better. I'll get outside and breathe down that bitter cold air into my lungs, and feel cleansed. If not, I'll feel rubbish a couple more days, get down the doctors, who may or may not give me some antibiotics, and I'll recover at a slightly slower rate. But I will recover. Even if this bug leaves me any long term damage, lowering my lung function, I'll probably never know nor notice. Whereas for people with CF, just one more infection might be the one that chips off that extra 10% that they couldn't afford to lose. 

And while they live their lives, and do their treatments, and go to school, or work, and all with the knowledge that their current infection may be irreversibly damaging their lungs, they will be doing all that feeling pretty damn crappy at the same time. 

So no, to those people that have said to me, 'oh I know how you feel, my son/daughter had flu last week and coughed for days' it is not the same (But I still feel for you, flu sucks too). 

On a brighter note, the kids are featured on the Starlight website right now. Which you can read here.

And here  is a link for my CF friends, which has cheered me up today. I hope it does you too.  

Good night all. Stay well x 

Merry Moly World on TV

Did I ever tell you about the time we were on TV? We were contacted through Guys hospital, where we had the IVF and PGD to conceive the girls. Reluctant at first, after meeting Adam, the writer/producer, we agreed. Loved him, and all the questions that he was raising. This was 4 years ago now, around Christmas time, and just before we got married (at home, with the kids and all our family and friends around us) so here you can see a mini Isaac and baby Anouk (make sure you watch to the scrambled egg bit).

To see the full program, search on you tube 'should I test my genes'. It's well worth watching, as well as Adam Wishharts other documentaries.

For the little segment including us, click HERE!

PS; Isaac is doing great right now, thank you to everyone who has asked x

Our crazy lovely boy, our gorgeous girls.

Just sitting here, looking back at our year.

Things have been a bit up and down.

We've had some wobbly moments.

But we're still smiling (with our cousins). 

And we've had some beautiful moments. 

And we've shared these moments with our amazing family and friends. 

Love you guys. 

OK, I may have had a teeny drink tonight and be feeling a bit soppy. So shoot me x