Spring.

The sun started to shine, which for me means padding around barefoot, and reading in the garden. 

I am utterly obsessed with my new book, My Absolute Darling (Gabriel Tallent); be warned, this is DARK (I love my books, music and art quite dark)... but unputdownable; in a jeez, this is frickin sick kind of way. But the sun seems to have gone back into hiding these last few days. Still, in the small window of early summer the kids spent a weekend in the paddling pool, which is pretty damn good for April in the U.K., and Isaac is doing..... OK. The results from the bronch trickle in... Pseudomonas infection; check. No surprise there, but symptomatically he is doing better, so we’re feeling good. Roll on summer for real... (and a few hospital free weeks please). 

Just the small matter of throwing myself out of a plane first....on Saturday. To say I am nervous is an understatement, but needs must. Everyone with CF deserves the chance of a longer, healthier life, so there is not much I wouldn’t do to help this cause. Please, please support our skydiving fundraising by clicking here.  We are so close to our target now! Post to follow on the amazing friends jumping with me, and all the support we have received. THANK YOU! x 

Bronchoscopy results....

.... were mixed. While they look at his lungs with a camera, they also flush the mucous out with saline, and these samples are sent to the lab. Results for these are outstanding (normal bacteria is grown in 3-7 days, but the NTM ones take up to 6 weeks), so no news on these yet. But the good news is that infection wise, his lungs looked good. This is in stark contrast to his last bronch in Oct/Nov last year, where we were told his lungs looked like ‘one of the worst CF lungs’ that the consultant had ever seen. Sorry if I repeat this often, I’m still haunted by it, and if I’m honest, a little pissed off. He was so sick back then that I think this was merely a snapshot of his lungs at that horrible time, rather than an indicator of his lung health more generally, but that was not what was implied (a later CT looked better also). 

Anyway... things look better now, infection/inflammation wise. But with good news, often comes bad... the bronch was performed by our favourite doctor, and she came straight from theatre to tell me that he has areas of ‘malacia’ which basically means some of his airways are too floppy. This is normal for little ones, but not for a teen. For a more medical description; 

“TM (Tracheomalacia) is significantly more common in infants and children with CF than in the general population and is associated with airway obstruction (lower FEV) and earlier Pseudomonas acquisition. TM is identified as a common co-morbidity of infants and children with CF. It appeared most commonly in children with other severe manifestations of CF such as meconium ileus and was not observed in pancreatic sufficient patients. TM was associated with some features of disease progression.” And “These disorders both tended to occur in children with more severe CFTRalleles, and our most severely affected patient with TM had meconium ileus and two Class I CFTRmutations (G542X and 3659delC).”

Kind of fits what we know already (Pseudo infection: check. Those exact class 1 mutations: check) and not great news. Only reassuring part is that the kind of physio that Isaac has at home, he uses BiPAP, is ideal in this condition as these kind of interventions stent open proximal airways and help relieve respiratory distress. 

So... Another worry to add to the list (to be discussed further with his team next clinic). On the positive side, he is recovering well from the bronch, his cough is calming, the sun is shining, and we’re hoping for a stretch of coughing-less, hospital-free time. 

For me, I am getting increasing scared by the idea of throwing myself out of a plane.... in less than two weeks! If you could sponsor me here I will be so grateful. I’m also coping with an Isaac-fiddled auto correct which means when I type ‘Hi’ it turns to ‘Daddy’, ‘love’ is ‘hate’, and ‘Hello’ is ‘nipples’. I have sent some seriously dubious text messages of late. Got to love that kid x 

Obie’s latest sleep pose. 

A closer look at his lungs.

Less than two weeks since his IV’s ended, and Isaac is coughing. His consultant rang me at work today to discuss this (she is so brilliant) and has suggested we hold on more antibiotics, and instead do a bronchoscopy to get a better picture of his lung health, and hopefully figure what the hell is going on (they hold on the extra antibiotics now because they want to suction off a mucous sample for the lab, and giving him Cipro now might just mask the problem). 

There are numerous ways in which they piece together a picture of his lungs; lung function tests, exercise tests, chest X-rays, listening to his lungs, sputum samples which they grow in the lab to see what infections he has, how he feels, CT scans, and bronchoscopies (a camera inserted into his lungs, and they flush out secretions with saline while they are down there). Each give a slightly different illustration of what is wrong. As his last bronch was so bad (“one of the worst CF lungs I have ever seen” the doctor said. Still haunts me) we are feeling both apprehensive, but also hopeful that this time, things will be different. Thankfully they can fit him in on Monday, so not long to wait, and it is our favourite consultant performing it this time. Yet another anaesthetic and theatre trip, but hoping this will be a fleeting visit to hospital unlike last time. 

He is not super unwell. He’s just not quite right either. 

In the meantime, have I mentioned lately that I’m throwing myself out of a plane soon? Everyone with CF deserves the chance of a longer, healthier life. Please support our skydiving fundraising by clicking here. Thank you x 

In Bristol. Cousin love 💕 Anouk and Bodhi.