Drugs and rivers.

A week into IVs and his lung function had increased a measly 2%. Enough to not be admitted (for now) but much less than we had hoped for one week in. This means an increased course - instead of finishing on day 14, his next test will be on day 17, and if we don’t see a significant increase by then, I imagine we’ll be looking at third week. He is still coughing (a lot at times) so either we’re looking at something new, these drugs are just taking their time, or his inflammatory response is on overdrive. He is mostly in good spirits still, going to school when he can. He sleeps through the morning dose (at sparrows fart) and I get to count his beautiful freckles while I push high dose toxic drugs directly into his bloodstream. He is so handsome. 

Today by the river with my gorgeous girls.

Hello spring, we are very glad to see you x 

In this world nothing can be said to be certain, except death and taxes...

.... and getting a super itchy nose when you’re mixing up IV drugs and need to stay sterile. Fact.

Port accessed. Bloods done. Lung function tested (about the same as last week). Four days in and he is feeling....well, ropey...this is pretty standard, he tends to get more symptomatic before he starts to feel better. He feels generally pants, headachy, has low grade fevers and was violently sick again last night. No idea if these are side effects or his chest infection being disturbed by these killer antibiotics, or just something new. Hoping he will feel better tomorrow and improve enough in the next three days to not have to be admitted for the second week.

It is very, very hard to see him suffering like this - but what makes it bearable is him; his ‘let’s crack on’ attitude, his lack of complaining, his cheeky smile, his ability to vomit and then immediately ask ‘what’s for dinner?’ - I swing from tears, to laughter, to utter sadness that it’s him and not me, to feeling like I might burst with with pride and immense love - a thousand times a day. 

To sponsor us for our latest fundraiser, in which I will be walking 67km in a single day (by current training standard, will likely take me 14ish hours) alongside some amazing friends (shout out to my team Holywalkamolies!) raising funds for the CF Trust who fund groundbreaking research into CF treatments, please follow this link. Thank you so much for everyone’s support so far, donations large or small, every penny counts and means so much to us all.

Some say CF is the most curable incurable disease - let’s prove that to be true x

Kandinsky ❤️

Crackles and coughing

We’ve gone from resigned acceptance that he needs to start IVs this week to step up his treatment against Pseudomonas, to a much increased crackle and cough, and a desire to bring it on as quickly as possible. He needed percussion physio last night (on top of the physio he does independently) which he doesn’t ask for often. The Cipro is having no effect, despite his Psuedo seemingly appearing sensitive to it. It’s looking like Tuesday before they can fit us in for his port access and for the first dose (he always has to have the first dose in hospital, in case of a reaction).

When he is like this I simply have to listen, to hold my hands round his ribs to feel the vibrations, the crackles, to look at his eyes, to hear the wheeze... and I just know that his daily nebulised and oral antibiotics, the mucous thinners, the lung shaking physio (his everyday treatments), they’re just not going to cut it this time. He needs these IVs. 

In true Isaac style, he is taking it all in his stride.

To support our fundraising for the Cystic Fibrosis Trust who fund seriously groundbreaking research, please click here. CF is actually one of the areas of medicine where a cure is a realistic idea, but they lack the funding to achieve this potential. As such, myself and a bunch of my amazing friends will walking the Great Strides walk in the Surrey Hills in June. For me, a bonkers 67km in a single day. 

Man Ray love ❤️

No place like home/Brexshit.

We will be starting IVs again early next week. 

Isaac is feeling no better, no worse, but a review of his lung function, xrays, bloods and symptoms have led his team to this decision, which makes sense to us. His last IVs were actually 12 weeks ago, which is a pretty good run for him of late. We will go in, pick up mountains of drugs, access his port, have the first dose, and (all going well) come home. I will do his IVs here (home, glorious home) and we only need be admitted if he gets much less well/his lung function has not increased after the first week (nothing like a deadline to get him to do his physio super well!). 

For that chance.... to stay home and snuggly.... we are super grateful. 

Have a great day x 

PS: I half wanted to write a ranty brexshit post about how Brexit/no deal may affect our availability to medications/and not attract talent/resource to meet recruitment needs in the NHS/and further negatively impact medical research (the UK is very successful at conducting clinical trials, sponsoring around 1,500 trials right now in collaboration with other EU countries – half of which will happening this year. Or won’t be happening). Sigh. But right now I think we have enough Brexshit. 

MOT

Today was Isaac’s annual review (a kind of CF MOT) and as we find every year, the immediate results are (*sigh*) mixed. Most other results are still pending - meaning days or weeks of wonder, but amongst those immediate results; 

Chest X-ray, not so great. 

Lung function down, again. 

Blood glucose test (for CF related diabetes) was.... normal (a MASSIVE relief after a borderline test last year). 

He started the day in hospital at 8.30am feeling pretty good. Port was accessed early and bloods all went well. But later, the lung function tests themselves stirred up his congested and inflamed lungs, and we still had X-rays (chest and abdomen this time, for his GI issues) and ultrasound after that, before returning to the main clinic, by which times he was tired, coughing and looking pretty peaky and ill. He coughed up some pretty spectacular looking blood streaked sputum for the lab just as we left. The blood is indicative of inflammation, not good, but it is good to have a juicy specimen for the lab - we cannot treat what we cannot see. 

As we wait for the team to review this news, and await the sputum and many blood results, we’re back on Cipro for a few days, with the view of probably starting IVs next week. We’re also working with his dieticians and doctors to find a way of helping with his GI problems (abdo X-ray not good either). 

After a long wait in pharmacy (brightened massively by bumping into a wonderful friend; love you Laura!) we got home just past 6pm. Isaac spent the journey home with his face in a bag, nausea taking over his weariness. Annual review is something we both dread but feel hugely reassured by. He absolutely needs all of these tests, and taking a step back and looking at the changes from one annual review to the next is a useful (but at times painful) exercise. Isaac is never enthusiastic about talking to the CF team psychologist, but we keep trying. We are so grateful for the amazing service the NHS provides us - but still, days like this can be very hard. 

Lucky for me, I have the best company x 

Fourteen and five feet apart.

‘Five Feet Apart’ is a Hollywood film due out this spring starring some young famous hunk (the fact that I still use the word hunk explains perfectly why I have never heard of him). I don’t know much about it yet, I’ve not seen the movie much less read the book, but the trailer looks a lot ‘the fault in our stars’ish, but this time around the subject of two Cystic Fibrosis patients who fall in love, ultimately risking the cross infection of bugs which could prove detrimental to their health - unless they stay five feet apart at all times (as hospital guidelines dictate about limiting cross infection risks in CF). 

A number of CF bloggers/vloggers have responded to this already.... and it’s proving pretty divisive. I can relate to the negative attitudes to the film; seeing CF depicted in medical dramas again and again in the past has always left a bitter taste in my mouth - too inaccurate, too cringy, and you’d need an Oscar worthy performance to get that CF cough right (they never do). Why can’t they do their homework, actually meet a CF patient and ask decent questions? 

On the other hand - this is Hollywood, and with that comes big time awareness. People will google CF. People will learn about CF; that alone can be invaluable to a sufferer. Hell, they may even donate to fund research.... so that has to be good, right? And, I also know Clare Wineland was involved in the film before her death, so maybe this time we can be hopeful for a more accurate depiction of the disease too. 

From our own perspective it worries me in terms of the attention Isaac may receive from his friends at school...

All of them will know he misses a lot of school. 

Some will know that is because he is in hospital a lot. 

A few will know that is because he has CF (his life, his choice). 

But right now, even fewer will really understand what that might mean for him, and a film aimed at his exact demographic might change that. 

More on this later as I’ve been asked (via the CF Trust) by the film makers to provide a response to the film (waiting to see what this entails exactly). 

Today Isaac turned 14. My darling boy. Every one of Isaac’s birthdays have been emotional for me (today was part celebration, part hugging baby pictures and snotily weeping about how time goes too quickly, part producing a soggy banana cake which cemented my reputation as the terrible birthday cake baker that I am). As my first born, perhaps I am not just celebrating him, but also my leap into motherhood fourteen years ago (best decision I ever made).... x 

Little Isaac.