Portacaths and ports

Hospital tomorrow for this lineogram to check his portacath is working OK. 

Best case scenario; it bleeds back for bloods and takes in IVs perfectly.

Worst case; it’s not working properly for bloods or IVs, and he’ll need more surgery for a new port (also not good because that is one less place to site a port (you can’t use the same vein twice and there are only so many places on a body that you can have a port fitted). Since the life span of a port can be 5-10 years, we could do with this one lasting more than just less than 2! 

Likely outcome is midfield... it will continue to not bleed back, but will happily take IVs, in which case we accept he needs a separate needle for bloods, but happily stick with this port for his much needed IVs.

Isaac is feeling pretty good this week, which feels pretty weird as we will kick off IV antibiotics next week... but knowing this should set him up with the bug-arse-kicking lung health that we want for winter... bring it on! 

No-deal Brexit-shit-lit: What Yellowhammer says:

• Supply chains for medicines and medical products are "particularly vulnerable" to disruption at the Channel ports
• While some products can be stockpiled, others cannot because they have a short shelf life. It will not be practical to stockpile products to cover expected delays of up to six months. 

So just really super reassuring. Thanks. 

Not seeing the woods for the trees.... 

© Pierre Pellegrini

Weekend with little and Obie.

All is good in the woods and sea. 

My heart, it sings. 

Rosa. 

Planning IVs

Lots of great news today at clinic. Lung function is wonderfully, delightfully.... UP. 

Best it has been in months. His team are pleased with his height, his weight, how his chest sounds, his sinuses... pretty much everything! 

We spoke about transition into adult care (this kicks off around now, so he is ready for the big transfer a little over age 16). He will meet his new team soon and have the chance to see the new Papworth Hospital building where his care will be managed. The Royal Papworth has recently moved to the Addenbrookes site where we are treated now; a brand spanking new building with individual rooms for CF patients (hurray!) all the uber mod-cons (state of the art tech, e.g. air changes every minute to reduce infection risks, lifts segregated by bugs that patients grow...). But still, the idea of leaving peads after our long journey together feels HUGE. We have known many of his team from the start and I’ve had more than a few tear stained hugs in that time with them - but Isaac’s attitude to transition today was fantastic (Isaac very much lives for today, worrying very little about the what if’s or the maybe’s). The biggest thing for Isaac, and me, is that from transition onwards, he doesn’t need to invite me. Mind you, he says there is no way he is going without me, simply because he’s too lazy to remember all the details for the many many questions we have in clinic (When did this symptom start again? When did you last have a CT? When did you come off that treatment? What did you eat for dinner last night, and how many Creon did you take for it? Is your cough more of a wheeze or a fruity one today?....)! 

He also had a port flush, and for the third time, his port is refusing to bleed back. He will have a line-o-gram (inject it with dye and X-Ray) soon to check that the port is still working as it should. We can accept it not bleeding back, it means he will need more needles for bloods, but still means it will work for IV drugs going in, which is the main thing. 

The plan is for IVs in October, all going well, which we have scheduled to coincide with school holidays (as he is starting GCSE years, really hoping to up his (circa 80%) attendance record if we can). 

We spoke about the sensitive balance between wanting an IV free quality of life (and limiting the risk of building up drug resistance and the kidney/liver and hearing damage that using toxic IV drugs so frequently can incur) with not leaving it too long that he is so unwell that it means he’ll need to have IVs as an inpatient rather than at home, or worse it will result in long term lung damage and recovery will be so much harder. 

Vigilance is key. Thankfully it is not just Dan and I with an active interest in this now; Isaac too has a much greater understanding and appreciation of staying well and out of hospital as much as we can (the one silver lining of intensive care memories?). This is the first time since 2017 when we first talked about planned IVs that we have actually planned them! Until now he has never lasted the full 12 weeks between courses before needing the extra treatment. 

Today was a good day. Thank you for reading, whoever you are. Have some random pictures! 

Georgia O’Keefe, wave. 

Nan Goldin. 

Persian rug love (a secret obsession of mine!).

What would no deal Brexit mean to us?

Concerns about the supply of medicines in the case of a no-deal-Brexit led me email the pharmacist at our hospital. I got a reply, that while understandable, was about as helpful as an itchy arse when you suspect the kids might have worms; 

An evening dose of meds. 

‘Unfortunately we would not supply above and beyond normal supplies of medicines. If we started to do that, then the current supply chain would fall down quite quickly before we get to Brexit. NHS England are monitoring the current situation to ensure additional supplies are not being made and stockpiles are not being held. The Department of Health / NHS England are working up strategies for supply of medicines in the event of a no deal Brexit’. 

Weekly tablet organiser (add on inhalers, nasal sprays, nebuliser and IV drugs). 

According to a recent white paper published by the Business, Energy and Industrial Strategy Committee, 73 per cent of pharmaceutical imports in the UK come from the EU. This equates to around 37 million packets of medicines every month from the EUworth £18.3 billion.

This is SCARY shit. 

Now, there are some medications people with CF could manage without short-term (the high dose vitamins which they cannot absorb from their diets as healthy people can, the slow release sodium maybe, the acid inhibitors at a push?)

But the Creon (enzyme replacement therapy, which allows the breakdown of fats and proteins which cannot otherwise be absorbed, of which he takes 15-30 a day, and would have agonising tummy aches and no weight gain/growth without) the oral, nebulised and IV antibiotics, the anti-fungals (in Isaac’s case right now, very important) and the steroids....being without these, even for a short time, could be catastrophic. 

Scarier still, delay to flu vaccines (due around the time of Brexit and which have a super short shelf-life), have already been indicated as a nationwide crisis waiting to happen this year, likely to kill thousands at high risk. Not to mention the risk to diabetics and others with chronic illnesses or cancer. 

Our dry meds cupboard. We have a whole shelf in the fridge too. 

Anyway, there is enough catastrophising already (not only in my own head) - what we need now are answers and assurances that our most vulnerable in society will be protected. Assurances that we can actually believe in. Where is that??? 

On a positive note; we are well over our fundraising target for our epic 43 mile trek in June. Check it out here! Thank you so much, again, to my incredible team for walking over 17 gruelling hours, laughing most of the way, and for all the amazing support we have had. This now means our family and friends have raised over £80,000 for the Cystic Fibrosis Trust since Isaac was born. To all our lovely family and friends, this is all down to you, Love, love, love x 

Isaac said to me tonight...

“I can’t believe I’ve been well this long”.

Which is both something to celebrate, but also to recognise. He was last on IV drugs to calm his lung infections down in June. When did two months become a good run? 

Summer.

This summer we are home (having holidayed in May quite expensively). A mix of work and home, day trips, the pool in the garden (we have an over ground pool we set up each summer which Dan has built an amazing deck around it, meaning the kids jump in and out all day) and long dog walks. Yesterday we had a family wedding (thank you so much to Alex and Becky for not only sharing their special day with us, but also for holding a raffle which raised an amazing £560 for the CF Trust). It’s actually been really good to be home. Dan is likely to have three lots of surgery later this year (hernia, vascular surgery due to repeated thrombosis and a knee op for a torn ligament; my poor broken husband) - he’s OK, but it all needs doing, so it’s good to squeeze in some fun before all of that.  Isaac is doing really well.... the anti-fungal drugs really are reducing his symptoms overall (we think), and right now, the only troublesome side effects are his poor cracked lips which bleed. Having last had IVs in June, we’re feeling pretty smug that he is still so well.... if he can last until at least September (or beyond?!) before his next IVs - that would be fabulous. 

Some rare pictures of my lesser spotted teen, and his gorgeous sisters (and cousins Ben and Bodhi). 

Wishing you all a wonderful summer x 

A bloody mess.

Our lovely community nurses visited yesterday to take a blood level for the anti fungals Isaac has been on the last month or so. This is to check the level of the drug in his bloodstream is high enough to be at a therapeutic dose. Kind of different to his normal antibiotic levels which he has on IVs, which are to check for that, but also more importantly that the level is not so high it fries his liver and kidneys. For antibiotic levels, blood is taken from a finger prick as taking blood from his port might mean we have an abnormal level of residual drugs in his port line (in case you are new to this blog, Isaac has a portacath fitted in his chest, which is a little device connected to a major vein in his chest, and implanted under his skin - this enables us to easily access his vein when we need it, by putting in a short (1 inch) gripper needle through the skin directly into the port - making venous access much easier than inserting a cannula or other kind of line, as after years of IV treatment, his veins are shot). However, for a Posaconazole level, he only needed to have blood taken from his port, which needed flushing anyway (every 4 weeks). Great. Only, blood was not forthcoming from his port. It simply wouldn’t bleed back. This is not terrible (for the Posaconazole level, he ended up having a finger prick instead) but it is frustrating, as it’s possible his port has given up bleeding back for good (it happens) meaning he’ll always need needles for outputs (bloods) and his port will only be good for inputs (drugs). 

Anyway, that was quite a long ramble when really what I wanted to say was that he ended up two needles and two finger pricks in, and a bit of a bloody mess at the end of it. But did he complain once? Nope. 

He’s been really well these last few weeks - but his cough is beginning to increase. I had hoped the antifungals might be the magical factor in him lasting longer between courses of IVs, but now I’m not so sure. At least I can be sure of his fantastic let’s get on with it attitude. 

I wish I were half as strong as my fourteen year old son. 

Have a great day x 

Georgia O Keefe 

Life is beautiful.

Clinic this week. Anti fungal treatment will continue for another two months. Lung function was up 3%. Not exactly party time, but a hope filled step in the right direction. Right now, Isaac is feeling well. 

I pass this boat every time I walk Obie down by our river. It always makes me smile. 

Happy summer everyone x 

Cracked lips, home, and well.

Isaac is home and well. The anti-fungal side effects continue to be quite evil, his bottom lip is so cracked it’s almost a crater. The treatment course is three long months. He complains hardly at all, as per his norm. God, I love this kid. His monosyllabic responses to ‘How was the trip?’ ‘Was CERN great?’ ‘What was it like at the UN?’ indicated a typical teenage blah enjoyment of anything vaguely educational. I’m taking that as a positive. 

Have a great day x

Nicola Davison-Reed

Away Away.

Isaac is in Switzerland. Our fears of him not being well enough to go on this school trip were joyfully dispelled - this week he is visiting CERN, the UN, St Pierre Cathedral, the Natural History Museum and enjoying a boat trip on lake Geneva. School trips have changed somewhat since our day, when a dusty day trip to Calais, to order une baguette ci vous plait was seen as a cultural peak.

While away, he is on physio, tablets and inhalers only, as agreed with his doctors. They were keen he take only what they could trust him to do independently, rather than send all the nebuliser drugs, chargers, part and machines that he might not do, and would need sterilising, that he could not easily do. He has back up antibiotics if he becomes less well to hand, and a whole load of sun block to put on for the side effects of his anti-fungals which make him uber photosensitive. His lips continue to crack and bleed on this toxic drug too. That aside, he left feeling good right now (at least, Isaac good). 

I on the other hand feel anxious. The logical 5% of my brain tells me it’s just a few days! He is fine! He can call! If he comes home less well, we can just increase physio! I’m being neurotic! Unfortunately for me 95% of my brain is made up of irrational fear, love and anxiety when it comes to Isaac. I have spent 15 years now, in utero and beyond, trying everything to keep him well. It’s hard to start to let go and trust him to take up the reins. 

But it will be worth it - what an amazing chance, an opportunity we would not want him to miss (but that we would like to see him home well from after) x 

Post IV lung function

Nothing to celebrate here. 

Nothing to cry about either. 

An increase of 4%. 

Not the 20, 30% of my dreams. 

Not the 10% of my reasonable hopes. 

But no stalemate. 

No decrease.

After another two week course we are relishing in IV free times - more sleep, less schedule. Suddenly, everyday treatments feel like a breeze! 

When I deaccessed him, his port site was sore, open and goey (it may be he needs a new needle size, as it looks like a pressure wound). It’s now healing nicely, TF (infection in the port would be very bad news). 

He has started on a three month course of treatment to try and clear this fungal infection. 

We’ve swapped the drug to one which is a little less toxic than the first week. 

He needs to have blood levels taken to check we have the level of the drug at a therapeutic one, but not so high it fries his liver and kidneys. 

The side effects still required an A3 sized drug pamphlet. 

So far, so good. 

New changes to his physio plan to try and shift the mucous better from his lower airways. 

He continues to amaze me - don’t get me wrong, he is a moody teen like the best of them, but how he takes this all in his stride without complaining is incredible. 

He is going away with school for three days soon - he’d lost a lot of confidence about being well enough to go, but these last few days, seems keen, more hopeful. 

Daring to look forward to something. 

He is better. He is. 

Please let him stay well for this, he’s already missed out on too much.

Thank you for reading x 

From our epic trek in Surrey - almost over £5000 now! 

From Rosa. Age 6.

Isaac sometimes is mean to Anouk and me. 

And he asks Anouk to go and get him sweets at the shop. 

It’s hard for Isaac because he doesn’t like doing his nebs, they taste funny. 

Sometimes it is hard for isaac and we try and make him do it. 

Isaac is brave because he doesn’t do anything when he’s in pain. 

I hope that they will get a potion for Isaac to not have CF. 

I love Isaac to death. 

I feel bad for him. 

I hope his IVs will go away. 

Isaac makes me laugh and he plays with me all the time. He always wants to pick me up. It’s annoying sometime, but I like it. 

I hope the Doctors on his birthday give him a potion to make his CF go away. 

I hope everything goes away that he worries about. 

By Rosa Banosa (of the cheeky cheeky nosa) x 

Robin Williams offering toilet paper to The Thinker, ca. 1990s.

Bronchoscopy, IVs and subsequent results

The Bronchoscopy went as well as we could have hoped. It was a long day of nil by mouth (always a frustration point for my ever hungry boy) with delays in going to theatre. But having been bumped off the list by emergencies many, many times before, we were very glad to only be delayed, not cancelled (please never under estimate the huge, debilitating pressure on our beloved NHS). General anaesthetic was fine, he tolerated it really well this time with no big de-saturation’s (his oxygen levels did not dip badly while he was ventilated). I was on my own this time, so spent a nervous hour pacing the corridors (never gets easier) remembering that last look of him, confused, glassy eyed and unreal before the anaesthetic took hold and he fell ‘asleep’ (under GA looks nothing like sleeping by the way, it is very hard for a parent to see). Recovery was OK; usual sore throat, grumpy, disorientated. All IV access was through his port this time, which was great, meaning he didn’t need a cannula as well. He slept and coughed a lot all the rest of the day as his body recovered. 

We kicked off IVs on the ward, but we’re now happily at home. I administer the IV drugs, and the lovely community nurses come every 2-3 days to do bloods (they need to check the levels of one of his antibiotics in his blood stream regularly to check that it’s not too high. Too high could be very hard for his kidneys/liver to process, causing potential and irreversible damage). All good so far. 

That said, Isaac has been sick almost every day since starting IVs, and experiencing chronic nausea despite lots of anti-sickness drugs. Right now, the doctors think this is an unfortunate concurrent viral thing. I’m not so sure, it’s so very hard to know in CF what causes what; it could be his current exacerbation, viral infection, drug side effects, or CF tummy related - and his tummy wasn’t right pre-IVs either. 

Today he managed his first whole day at school in a week or so, so hopefully this is coming to an end. 

While all this is going on, my anxieties build as I know the lab have taken the mucous they washed out of his lungs during the bronch (called a lavage) and are waiting to see what bugs grow on their agar plates to see what is going on in his lungs. Early results (some take days, some weeks) reveal more fungal infection. This is mega frustrating news as he only very recently had treatment for this (early in May). To have it back, again, so soon may mean he will need longer term treatment to attempt to kick this out for good. We meet with his team next week, but I’m aware treatments may include 1) long term anti fungals and 2) steroids - both of which have pretty terrible side effects in his case. And this would be alongside his usual treatment for his chronic bacterial infection (PseudomonasA). Right now, I’m trying not to worry (trying, ha! But not always succeeding). 

The positives: We’re on acute treatment already; We know now what we’re battling (which is much better than not knowing, not treating, and him continuing to lose lung function); This is exactly why the bronch was a good idea - making it all worthwhile. 

We don’t know enough yet about what this reoccurrence means and what his options are. So until next week, we carry on with IVs, we do the new treatments, and we very much hope for improvement. Plus, whilst his tummy has been horrible, his chest is sounding a little better these last couple of days. 

Have a great day x x x 

PS; Fundraising update: We are just £345 away from our £5000 target. Thank you so much! If you’d still like to support us, there is a link on the picture of Isaac to the right (unless you’re viewing on a mobile). Some of my hero team are still wearing medical boots while they recover! x 

Robert Mapplethorpe ❤️

We made it (and hospital)

It was not only 67km.... but 69 (some 43 miles) due to some wee detours along the way. Starting at 6am, with torrential rain and injury time we made it to the end at nearly midnight. During this epic walk I know we all hit walls, felt pain, weariness, love and laughter, but to the end, we were together - guiding each other in the darkness. Thank you so much for supporting us, it means so much to all of us (if you have not yet, you can still sponsor us here) We’re almost at our target (£5000). 

To Jo’s team, Michelle, Jo, Jenny, Cassie, Erika and Tracy, incredible job ladies, and you beat us too! I can’t thank you all enough for joining this bonkers mission and raising much needed funds for the CF Trust. 

To my Holywalkamolies team - I hope you know how much I love you all. 

Shell, Matt and Sarah, it has been so incredibly special to spend so many walk and training hours with you - you were all incredible! 

To Bon, Jennifer, Bodhi and Anouk, you made this all happen. I’m not sure I’ve ever been so happy to see someone as when we saw you all at the checkpoints!

Gem and Annie, you lied! You said you couldn’t possibly walk that far. But you did. And your singing and dancing saved me from despair. Thank you for all the laughter. 

To Gavin, I hope your feet are not permanently damaged. You mad old lovely fool! 

Some pictures for you below of some very special people and some pretty awesome memories. 

We are going into hospital on Thursday for a bronchoscopy, followed by two weeks of IVs, and then possibly a CT scan too. Isaac is home sick right now and feeling pretty rubbish, so we’re hoping he will be well enough for theatre. Sunny side up? It’s good to have a plan. More soon x 

If the sun refused to shine 
I would still be loving you 
When mountains crumble to the sea
There will still be you and me 
Kind woman, I give you my all 
Kind woman, nothing more
Little drops of rain whisper of the pain 
Tears of loves lost in the days gone by 
My love is strong, with you there is no wrong
Together we shall go until we die 
My, my, my inspiration is what you are to me 
Inspiration look, see
And so today, my world it smiles 
Your hand in mine, we walk the miles 
Thanks to you it will be done 
For you to me are the only one
Happiness, no more be sad 
Happiness, I'm glad
If the sun refused to shine 
I would still be loving you 
Mountains crumble to the sea 

There will still be you and me

Zeppelin. 

Antibiotics, bronchoscopies and CT scans.

Thursday we popped into CF clinic for an emergency review, as his cough is building. We saw the registrar as many of the team are away at the European CF Conference, but he was great, really took the time to listen and understand where we are right now. The relationship we have built with our MDT (multi disciplinary team), which is made up of CF/Respiratory doctors, CF specialist nurses, physios, dieticians, pharmacists and psychologists, is an essential part of Isaac’s healthcare, and it’s both important and valuable for Isaac and I to feel part of the decision making, not simply passive participants in his care. Anyway, his lung function was (as predicted) down, and he had more bloods to check his infection markers (TBC). 

We discussed that for six months now his LF has been creeping down steadily (as lung function is considered a vital measure of wellness in CF, this is major concern). The doctor suggested that as Isaac had been so poorly in Oct 17 (read more here) this may have contributed to his overall decline, but he also agreed with me that we don’t want to simply accept that this is his new baseline, without really understanding why this might be, and nor will we stop trying to regain lost LF. 

It’s also clear that he is exacerbating right now (despite recent IVs, anti-fungals, and steroids) and we need to deal with this now. Therefore, tentatively the plan is a week on Cipro (extra strong oral antibiotics, started last night), with a review in one week. If his LF has not improved by then, IV antibiotics will be kicked off in hospital, with the idea of doing a bronchoscopy (a looksee in the lungs with a camera, plus a flush out of his mucous to test in the lab) at the beginning of the course, and potentially another CT scan following the course (to have a more detailed look at his entire lungs; beyond the point they can bronch, essentially). It’s a tentative plan for now, his MDT will discuss at their meeting next week. It’s incredibly important to know we’re not missing anything, and bloods, xrays, medical reviews and culturing sputum samples can only give us a partial picture; a bronch and CT will ensure nothing is escaping us that might explain why he’s declining. 

Not great news by any means, but a reassuring plan of action, and in true Isaac style, he took it all in his stride. There is good news scattered in each clinic visit too... his weight and growth are great (something many people with CF struggle with), his exercise tolerance is amazing (he does CrossFit and is in a football team) and his gastro problems are much, much less frequent and severe than they were a few years ago (a cycle of DIOS and problems with nausea that for a while I thought we would never get out of). Lots to be thankful for, heaps to hope for. 

Have a great day - and please think of us, walking for some 13,14 hours tomorrow!

Picasso ❤️

Blisters, love, and gratitude.

Holiday was wonderful. The kids had a ball, swimming dawn until dusk. I’m not sure if I explained about the holiday? If you’ve been following this blog for a while you may remember that Isaac had a wish granted 5 years ago by the wonderful charity Starlight. His wish was to go to the biggest water park in the world (he is part fish) and amazingly they really, truly did this! We spent 5 breathtaking days in Dubai at Atlantis (he is still featured on the Starlight website here if you want to read about his dream holiday). At that time, our little Rosa was just one, so we made the difficult decision to leave her at home with the grandparents as it would be too hot, and having her there would mean we could do less with Isaac and Anouk. So ever since then, we have promised Rosa that we would go again, and revisit everything we did then - as a family of five. A good deal gave us our chance - only four days this time, but every second counted, and our fears that Isaac would not be well enough to go were proved gladly wrong. 

Following the Voriconazole (to treat his evil lung munching fungus) Isaac was really well for a couple of weeks. Side effects made his poor lips blister so much they bled at nights (he looked kinda goth in the morning, lips black with dried blood) - but his chest cleared. 

Now? Coughing again. A low, deep, rumbling cough. Clinic have squeezed us in this week on an emergency list. It’s a worry+ situation; an increase in symptoms is always a worry, but gets a plus whenever ‘other life’ factors (Christmas, birthdays, holidays, epic fundraising walks....) are imminent, as I’m away this weekend for our walk. In fact, the walk is a worry generally as I’ve hurt my hip (for a couple of years now I’ve been able to click my hip in and out of the joint - true and, yes; yuck) and the last training broke me slightly... but I’m still very much planning to make it round (even if my wonderful friends joining me have to wheelbarrow me!). 

Thank you SO MUCH for all the support so far - it really means the world to me, our family, and our whole Holywalkamolies team. You can still sponsor us here - I hate to ask (at least, as often as I do), but CF research absolutely needs these funds, and Isaac is sadly not one of the 90% of people with CF who will benefit from drugs which are soon to be licensed which treat the underlying cause of his disease. He unfortunately has a rare, severe mutation. This means a longer wait - but I’m confident a cure will come, in time. Thank you for helping in any way you can x 

Huge thanks to my friends and family Jo Cayley, Gavin Dytham, Michele Dytham-Ward, Jennifer Dytham, Sarah Cater, Matt East, Gem Wilkins, Annie Marriot and Bonny Fountain. Michelle kavanagh, Jo Elliot, Jenny Hays, Cassie Flack, Erika Pyne, and Tracy Coulton-weir for joining me on this epic 67km trek.

And then smiles cover your heart, again.

Isaac is doing really well. 

His cough is better. 

He hasn’t been sick in a couple of weeks. 

Thank you, our toxic friend, Voriconazole. 

Descripti

DescriptiVoriconazole

We love you. 

Hope you’re having a great day x 

Lee Miller love x