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.