Good news.

We do have good news sometimes, forgive me for not always sharing this. I know I post more when he is less well. I'm too busy enjoying him being well to post! He seems better, a lot better, and the lab results were good this week. He will continue on the extra antibiotics this week; annual review next week (heaps of extra tests he has yearly). Let the Birthday celebrations continue (I say continue as we started early at the weekend)...

Our handsome, funny, lovely boy is almost 12. And we couldn't love him more x 

Invisible disease.

I hope I'm wrong, but it feels suspiciously like we're hurtling towards another course of IV antibiotics (which inevitably involves an admission to hospital, of various lengths). 

The typical reasons for IV's are.... 

He is more symptomatic than usual (coughing, wheezing, rattley chest). 

His sputum has been sent to the lab and has grown a new bug, or a heavy growth of his usual suspects (Pseudomonas and Acinetobacter). 

His extra oral antibiotics (Cipro) to which these bugs should be sensitive to, has not reduced his symptoms. 

His lung function drops (FEV/FVC). 

Me saying this might seem mighty weird to people around him.... I think if you bumped into Isaac today, or most other days, the only thing that would cross your mind was what an unbelievably handsome kid he is.... not how he is fighting chronic lung infections every day. It's not inconceivable for him to come from the football pitch, trying to give it his all for 60 minutes, but then go straight onto the ward. 

To me, it's not so weird. I developed an uber sensitive 'cough radar' soon after his diagnosis at three weeks old; Give me a whole bunch of kids in a mile radius, and I'll be able to say if it's him coughing (I get right funny looks as I wince when I hear him coughing from three rooms away - And I do literally wince, it pains me so).  

He certainly doesn't fit the 'horribly ill' description you might think CF means. Typically when we get to hospital, he looks like the healthiest kid on the ward. But he's not. Plus he's very unlikely to tell you that he feels unwell, because that just not his style, and this, frustratingly, is his norm, so he wouldn't think to. 

It's his 12th Birthday next week, so I'm very much hoping that I am wrong. If I'm not, this would be his shortest gap between IV courses ever, which hints of a disease progression that I am not ready to accept. 

Big fat pharmaceuticals and the depleted NHS.

Picked up some of Isaac's drugs from clinic today. Due to crazy NHS budget issues his inhaled drugs have to be dispensed at the hospital, while the rest we can get locally. Here, lovely readers, is £4,500 worth of medications. Seriously; 

 

Crazy I know. Two of these replace two of the drugs he currently inhales through a nebuliser (an electronic device which aerosolises drugs into smaller particles to better deliver them into his lungs). Instead these are dry powder inhalers, although not exactly the kind you see for asthma. With these he cracks open each capsule in a device and breathes it in until empty, which can take a few goes, and he needs 8 capsules a day. But still, much quicker than the 20 minutes per neb that he does currently. Test dose went really well, so hoping this is something he can tolerate long term. This reduces his nebs from five to three a day, so he is delighted. 

But what of these profiteering pharmaceutical companies? Who regularly make a profit margin of 20% or more? With one drug costing £10m per patient, and a drug that targets the underlying cause of CF in a minority of CF pateints (Kalydeco) costing £180,000 pp, per annum, with a manufacturing cost a tiny fraction of this, you can see how. Drug companies justify the prices they charge by arguing that their research and development costs are huge. On average, only three in 10 drugs launched are profitable. Many more do not even make it to market. The industry argues that the overall value of the drug needs to be considered; some high cost drugs do save the NHS money over all, as they reduce other needs for intervention. But just because you can charge a high price for something does not mean you should, especially when it comes to health. 

Big pharma companies then argue they only have a limited time in which to make profits. Patents are generally awarded for 20 years, but 10-12 of those are typically spent developing the drug. This leaves eight to 10 years to make money before the formula can be taken up by other pharma's, which sell the medicines for a fraction of the price. Sales then fall dramatically because there is no brand loyalty, but how can there be when the NHS struggles as it does? How could they continue to pay more for the same drug? A drug which might be made for just pennies? 

Ultimately the profit line speaks for itself...in the news they regularly bemoan energy companies making a hell of a lot less profit at our expense, and this is about health. People's lives. It sickens me. 

The WHO has talked of the "inherent conflict" between the legitimate business goals of the drug companies and the medical and social needs of the wider public, which is also rife with bribery. Isaac is prescribed one drug which costs the NHS more than an identical drug because they give the patient the incentive of a free nebuliser (worth £3,000) - if he stops that drug, but we still need a nebuliser (as is the case if this trial on the Tobi podhaler and Colobreathe goes well) will they demand the neb back? Another drug he takes is preferred by the NHS because they throw in free filters which protect pregnant women from the drugs which the patient is taking. One of his drugs costs £500 per week. Another £8,000 per year. Another still, about £8 per year. Seriously. 

In other news..... Sinus surgery sounds more invasive than we first thought; Difficult to read and moving article here about life on O2; I am still appreciating my own functional internal organs, but not altogether right just yet; And frustratingly Isaac is more symptomatic towards the end of his course of Cipro (extra course of Pseudo fighting anti-bots) than when he started, and will continue on them for another week. Being no better after Cipro usually means IV's, so hoping for a better week to come; He was cheerful at clinic today and his sinus pain means he is actually quite keen for the surgery, and extra marsh mellows on his hot chocolate had its usual positive effect on my brave and handsome boy x 

The good, the bad, and the damn right annoying.

The good; 

Isaac trialling two dry powder inhalers again (Colobreathe and Tobi Podhaler). If he can tolerate them this time, this would mean two less nebulisers a day and much quicker treatment times. 

Having my dream job and fantastic colleagues. Off to Madrid again soon - one of my favourite cities. 

Rosa performing the Gingerbread Man with her class, and not having a paddy about it. So cute. 

Dan and I going to Croatia for a weekend to celebrate a 40th birthday with some very old friends. Thank you GP x

Planning a joint party for my own 40th this year too. Be warned friends, there will be fancy dress shenanigans! 

The bad;

Pseudo-bloody-monas infection exacerbation. More antibiotics. Poor kid.

Isaac likely needing sinus surgery. But waiting on ENT appointment...

....Over stretched NHS ☹️

Angry Rosa (Nice Rosa's nemesis, who throws glasses of water on the floor, rips all the pages from a book, and runs away from doting grandparents). Scary.

Annual review looming, tests aplenty, but feeling less and less confident that they will deliver good news this year. 

The annoying; 

Pre-teeniness, and trouble in school. Uh oh. 

Unhelpful pharmacy dispensary staff (yes, I really do mean 14 boxes of Creon per month, it is not a mistake. Oh look, despite having this conversation three days ago, you have still given me just four, and yet you're insistent that this is my fault? Wonderful). 

Relentless treatment regime, but always worrying you could do more. There is always more. 

Him trying harder at treatments and being more compliant, and yet being more symptomatic rather than less. DRRRR! 

My mojo seems to have moseyed off track a little today.... but who knows what tomorrow will bring (summer would be nice). If I believed in luck I'd be expecting a truck load for my boy to arrive; I wish I could give him all of mine. He complains a hell of a lot less than I do. 

Have a great day x