Kaftrio Updates 2020

(Post from 7th September 2020)

I have been taking Symkevi and Kalydeco since December last year. Together, they were the second precision drug to be used in Cystic Fibrosis, but the first for the F508del mutation. They are a therapy drug made up of Lumacaftor and Ivacaftor and together they help to keep a balance of salt and water in the organs, especially the lungs, by causing the chloride channels in the cells to operate more effectively. Since then I have had 0 hospital admissions and only one 2 week course of intravenous antibiotics, when I would have usually had about 4 or 5 courses in that time.

Earlier this year, the NHS signed a deal to make a triple therapy drug, Kaftrio, available to CF patients in the UK. In Cystic Fibrosis, the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein is damaged due to the mutation in the gene. Unlike Symkevi, Kaftrio is more effective as it is made up of Ivacaftor, Tezacaftor and Elexacaftor which all work together to target the proteins that are damaged by increasing the amount of CFTR protein at the cell surface to help it to work better. Therefore the Kaftrio will replace the Symkevi doses and I hope to see even more improvements in the upcoming months. I can’t believe this day has come after 24 years of kicking CF’s butt.

Although this won’t mean I’m cured... but maybe one day when we’ve seen such a change in just a year

The last 10 months (and 24 years of living with CF) have been bloody tough but I am pleased to say I got through everything that was thrown my way and I have come out better and stronger than ever! Never did I think in November last year this is where I would be. Last month I secured and moved into my own home. The second time around is better than the first, right? When you've loved and failed, it can be painful. But with this pain came a newfound appreciation for myself. Which I never believed would be possible. Today I returned to work after 23 weeks off and I return to University in a few weeks time to complete my final year of my Primary Teaching Degree. Those knockbacks were a blessing in disguise and the grass is definitely greener where I’ve watered it.

(Post from 29th September 2020)

(Post from 10th October 2020)

The last 11 days have been very overwhelming, to say the least! But as promised, here is the Kaftrio update for you all (see below). I’ve been flooded with loving messages and calls and it’s crazy to know that this many people are invested in my Kaftrio journey. This video’s a long one so get your popcorn ready and congratulations if you make it to the end of the video! Sorry, the quality is not great, I’d given up after 40 minutes of listening to my own voice.

As many of you are aware, my beautiful cousin Sian passed away 14 years ago due to Cystic Fibrosis and I wish these drugs had come around sooner. Therefore, this ones for you Sian, love and miss you always.

(Post from 13th October 2020)

OMG I DID IT, 81%!!!!! Two weeks on Kaftrio and the wonder drug is really working!!!!

This time last year my lung function was 63% (2.06 litres).

Self Isolation: Day 21

I am now approaching week four of self isolation and have managed to complete nearly 21 days... just! It has been very tough waking up every day to do the bare minimum, but the sun is shining which puts everyone in a good mood! Despite the fact I can't go out and walk, at least I am able to sit in the garden. I have to think of the positives throughout this as some CFers will be stuck inside a flat without a garden!

Mum and dad arrived back from Costa Rica earlier than planned but at least they were able to get a flight back! It's been nice to have some company in the house, although we are all keeping our distance. 

I have been keeping up with the indoor home workouts, using the Beam website. These have helped out with my chest clearance and have stopped my chest feeling so tight. I have been following up with my physiotherapy after each workout. So hopefully when all of this blows over, I will see an improvement on the lung function graph! Fingers crossed!

If you're a Disney fan, like me, I would recommend downloading Disney+. I already have all of the 56 classics on DVD, but the Disney+ app has all Disney movies, series and programmes ever created. There's even some films only available on the app such as the new live-action Lady and the Tramp movie. I've never watched so much Disney in my life haha.

I have managed to continue to Facetime family and friends which is keeping me sane! My friend Dom and I were supposed to be in Manchester this weekend, so instead we had an indoor virtual party via the Marco Polo app. 

So, I have managed 3 weeks of total isolation. If I can do that long, it's proof that this is possible for those who are not vulnerable to complete 14 days. Please follow the guidelines and STAY AT HOME and hopefully this will be over quicker.

Noni x

Self Isolation: Day 14

This morning I woke up and my chest was feeling tight due to not being able to go out and walk. A few days ago I tried the Jo Wicks Kids PE workouts. However, I failed and had to keep stopping due to my amateur pair of lungs! Then I remembered about a website called Beam, which has workouts designed especially for CF sufferers. The instructor I chose, Natalie, has CF herself and has designed workouts that she finds manageable. So this morning I gave them a go! Before starting the workouts I did my DNase nebuliser which is used to break down excess DNA in the pulmonary secretions, making mucus thinner and easier to cough up. I started with a warm up routine. Followed by a body strengthening routine, using dumbbells, and a core muscle fitness test. Then ended with a cool down and stretch routine. Now my workouts are finished I am going to do my chest physiotherapy using a BiPAP. This is a continuous positive airway pressure machine that helps keep the upper airways of the lungs open, by providing a flow of air to the lungs. I do 10 cycles of 8 breaths on the machine and end each cycle with a huff and a cough. This technique helps to move the mucus and cough it up which also helps to keep my lung function up.

Noni x

Self Isolation: Day 7

The past few days have been extremely tough, especially on my mental health. I've had three, what I'd describe as 'anxiety attacks', where I suddenly feel short of breath and cry a lot due to the stress I am under. I have experienced these a lot over the past few months but yesterday was a particularly bad one. The current stress of the 'unknown' and the stress I was already under before this pandemic has been quite scary. I never used to experience this at all in my life so it's been a difficult time, but I just have to keep reminding myself I am strong and I can get through this.

Living with CF, I am used to 'flare ups' involving infections, struggling to breathe, high fevers, sickness bugs you name it. Over the years, this has also involved ambulance rides, IVs, blood cultures and countless hospital stays. But Coronavirus can cause a lot more damage for someone like me and my body wouldn't be able to handle it. So I have followed the government guidelines over the last week, to keep myself safe, and have only left the house to go for a walk. I have been trying to walk as far as my lungs will let me and walking for as long as possible so I am not sat at home, alone, feeling down. Yesterday I managed 4 miles, which has got to be my biggest achievement with CF yet. It made me smile a little that through all of this, physically my body is well and the precision drugs are doing their job. It's just a shame I can't say the same about my mental health.

I also haven't managed to see anyone face to face in seven days, except my sister Abbi when she comes home from work in the evenings. But she is keeping her distance by not sitting in the same room as me. Regular facetimes with family/friends are the only contact I can have with people close to me currently. I am literally counting down the days until mum and dad arrive back from Costa Rica!

However, now the latest government guidelines is that those 'at high risk' (including CF), should not leave their house AT ALL for 3 months. Which sadly means no more walks and not even answering the door to the postman! Therefore, even if you are not at risk, please comply with social distancing and stay home! You can still go for a walk or pop to the supermarket, so be grateful that you can do those two things, unlike me.


Just another 11 weeks to go :(…

Noni x

Wonder Drugs

Having Cystic Fibrosis (CF) means life is not always an easy road. For nearly 24 years, I have been fighting a battle to try to stay well. This means taking endless antibiotics and medication, completing lots of physiotherapy and battling to try to stay out of my second home: the hospital.

The lead up to each hospital check-up is spent fighting to try to stay well and hoping for the best result to show on the dreaded lung function graph each visit. The smallest change on the graph can mean a two-week course of IV antibiotics and a complete pause to your normal lifestyle. This means cancelling all social plans with friends and calling in to work to explain why you wont be in yet again. Over the last few years, I have seen a decline in my lung function which can be scary at times. January 2019 for example, when it dropped to 37% after a particularly unwell episode.

In October 2019 some exciting news for the CF community was announced...

After 4 years, the CF trust fought for access of life saving precision drugs Orkambi and Symkevi in the UK, costing £104,000 a year per patient and NHS England finally AGREED! I received an email to say that my genotype meant I was eligible for Symkevi. It is the second precision drug to be used in people with Cystic Fibrosis. However, it treats the Deletion of Phenylalanine 508 (F508del) mutation that many people with CF have, including me. Its purpose is to slow the decline in lung function, which is the main cause of death among CF sufferers. It is a medication made up of Lumacaftor and Ivacaftor and together they help to keep a balance of salt and water in the organs, especially the lungs, by causing the chloride channels in the cells to operate more effectively. If you know much about CF, you’ll understand why this is a benefit. But if you don’t, the basics is that an effective Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leads the chloride ions (or protein) in and out of cells in the body. However, a defective CFTR gene causes mutations in how the protein passes across cell membranes. This interrupts the balance of salt and water needed, creating a thick mucus in the lungs, pancreas and other organs.

I booked in an appointment, with my CF Team, to review everything to find out when I could begin Symkevi. Symkevi is in addition to all of my usual therapy so there were lots of important things to think about before starting it such as; when to take the tablet and what to eat with it to ensure it is absorbed correctly, adjusting my current medications to ensure they don’t interrupt the process and all of the side effects that will come with taking it. At the appointment I found out that it would be two precision drugs; Symkevi and Kalydeco and I would have to take them 12 hours apart, every day. Because it is such a new drug, the long-term evidence and reported side effects were limited.

The beginning of December I received the wonder drugs. I had a very upsetting end to the year, with my boyfriend sadly breaking up with me. This caused a fluctuation with my CF, as I couldn’t eat, which resulted in a 3kg (6 pound) weight loss in a week. But I decided it was time to begin the drugs.

After 6 weeks on Symkevi and Kalydeco it was difficult to tell if the drugs were actually making any difference as I was still very stressed and down. However, I got through my first November/December period without needing any IV antibiotics!! One thing I did notice though was that I could breathe (haha)! I managed a walk of 1.5 miles  without stopping to catch my breath. Previously I couldn’t even walk up the stairs without stopping to breathe, before completing what I went up there for. 1.5 miles may be nothing to someone with normal lungs, but it’s a big thing when you have lungs that function on 60% when well!

I have now been on the drugs for 15 weeks and the side effects are still changing every day!

For the first few weeks, I feel like I coughed up 23 years’ worth of phlegm, it was gross. I felt like I was drowning in my own lungs, more so than ever before.

I had insomnia and was awake almost all night, which made me extremely tired in the day. This has only just settled over the past few weeks. I have also felt extremely down and somewhat depressed. Although I assume this is due to heartbreak and having to move back in with my parents after thinking I had found my forever home, rather than a side effect. It has been horrible as I haven’t felt myself at all. Onwards and upwards now though!

I completely lost my appetite and was sick every time I put food near my mouth. However, speaking with the doctors, we were unsure if this was due to the drugs or the other added stress I was going through. This also meant I was on the toilet constantly, when I wasn’t being sick, it was the other end (sorry if too much info haha). When the sickness settled and I was back to eating normally, my bowels still weren’t normal. This has meant adjusting my Creon intake (see CF dictionary for what this does).


If you didn’t already know I also have Cystic Fibrosis Related Diabetes (CFRD) and throughout December/January my blood sugars were spiking some highs such as 15.9 and then randomly dipping very low to 2.9. Which was frustrating as it made me feel very faint and sweat uncontrollably. Normal range should be between 5-7.  Luckily, my wonderful diabetes team gave me some techniques and tried me on two different insulins to my normal one. I now take a faster acting insulin called Fiasp which seems to be doing the job.

I also experienced a horrible itchy rash all over my feet after about 2 weeks on the drug. This cleared up pretty quickly after taking some anti histamine and I haven’t seen a reappearance. However, I have had an itchy rash on my back that has been there for around 13 weeks. It is irritating but I have had to decide whats worse; an itchy back or shit lungs!


I have also seen a change to my skin, it feels much more oily than normal. At first I thought this may have just been because I changed my makeup brand. But since trying my usual brand again I have noticed it’s not the makeup.

Onto the most important bit… my lungs! Since starting these drugs, I wasn’t hoping for a massive change in my lung function numbers. The findings from clinical trials was based more on stability to health and lifestyle, than huge increases in lung function. In February, I was still feeling down in myself and knew my numbers would be low. However, despite the fact I felt unwell, my lung function hadn’t dipped as much as it normally does during an unwell episode. The doctors said that this was probably because the drugs had helped to keep it up. But we decided a course of IVs was due anyway as I had missed my usual end of year ones due to not needing them at the time. I didn’t feel as though the course of IVs made a drastic difference but that was over a month ago now. So hopefully when I next go back to the hospital I see a difference to the numbers on the lung function graph!

Although who knows when my next appointment is going to be due to this Covid-19 pandemic!!!?? I spoke to my CF team on the phone and they have supplied me with an emergency batch of antibiotics just in case. But until this all blows over, I am self isolating away from it all. The CF Trust have recommended limited social contact for 12 weeks, I think I may go crazy if they stick to this! All my upcoming plans (clubbing, theatre trips and weekends away) have all been cancelled which absolutely sucks but I guess it’s better to be safe. So, if anyone is up for a Facetime chat or phone call, I’d appreciate it! Mum and dad are away on holiday so I’m already feeling bored and alone! I am hoping they can fly back safely in a few weeks. All UK schools are closing tomorrow too so I definitely won’t be able to go to work. The one thing my CF team have suggested and said is safe to do so, is going out for daily walks. So, I managed that yesterday and wasn’t breathless… RESULT! I’m planning to do the same again later today.


If you managed to get to the end of this post, firstly congratulations, I’ve just written an Inclusion essay the same length as this and it took me two months! Secondly, thank you for reading.

Stay safe, look after yourselves and your loved ones. Hopefully the world will be back to normal soon!!

 

Noni x

IV Time, Zzzz...

So my predictions were correct... My lung function is still down after 4 weeks of oral antibiotics so we’ve decided it’s time for some IVs. So the two week ‘refuel’ begins. I am currently sat in Treatment Room 1 having my IV test doses to check that I don't react to any of the antibiotics and can begin the two week course. I am on Meropenem, Teicoplanin and Tobramycin this time so will be 'hooked up' 5 times a day. This is because Meropenem is 3 times a day, Teicoplanin once a day and Tobramycin once a day. But 6 times tomorrow as the Teicoplanin is twice on the first day. Then when I’m not attached to an antibiotic, I have to do my physiotherapy treatments, which increase to 2-3 times a day when I'm unwell. This involves breathing on a Bipap machine- see CF dictionary page above for what it does.


Wish me luck!

A little update and CF Week 2019

Wow it’s been nearly two years since my last post. Life’s been pretty crazy in those two years which is why I guess I haven’t updated the blog in all this time, I’ve even moved out of my Mum and Dad’s house, which I thought I’d never do! However, after reading Five Feet Apart book by Rachael Lippincott and then watching the movie I thought about writing a little diary about my life with CF (but again haven’t yet got around to doing so) and now with it being Cystic Fibrosis Awareness Week I thought what better time to do an update. I clearly had the same idea almost two years ago during Organ Donation Week haha.

A little more about me, just in case you’ve forgotten who I am. I’m 23 years young, have Cystic Fibrosis and now Cystic Fibrosis Related Diabetes too. So, life’s all fun and games whenever you want to eat to remember that first you have to:

1. Check your blood sugar by pricking your finger
2. Inject yourself the right amount of insulin based on what your sugar levels are and what meal you’re eating.
3. Take your Creon throughout your meal (check CF Dictionary above if you’re not sure what these are). If you take all of the required dose before eating and then struggle to eat all of your meal, you’re basically screwed and risk spending a few hours later on the toilet, sorry for the honesty!

I now live with my boyfriend Craig who has been my partner, best friend, nurse, physiotherapist, you name it, over the last year. I knew he was the one when I arrived on our first date with an IV drug hanging from my portacath and he asked if he could flush it off instead of running a mile. I’ve known of Craig for many years as we went to the same school, although he is five years older than me. But we got talking many years later when we bumped into each other in a nightclub, classy eh? Anyway, I’ll leave that there as Craig is probably cringing as he reads this and will moan about it later.

I work in a Primary school that I attended myself as a child. I am also slowly completely a Foundation Degree in Teaching too but have had to defer until next year after a scare to my health that I had in January.

So back to my life and the joys of Cystic Fibrosis.

At my recent annual review, my lung function was down slightly from its best and my sputum has still grown MRSA and Pseudomonas, so I’ve been on oral antibiotics for four weeks now. I did two weeks of Septrin but my lung function stayed exactly the same percentage and just made my chest a lot more productive. So, I then started a two week course of Ciprofloxacin and Linezolid which again I think has only helped to some degree so it could be IVs again when I go back to the CF Unit on 2^nd July.  Although I haven’t been in hospital now since January when I picked up a sickness bug and ended up with tonsillitis too. That was a scary time as I couldn’t eat a single thing for a week and ended up coming into hospital via A&E. I would say that has been my worst time ever. My lung function dropped right down to 37%. Being on antibiotics that aren’t my usual tablets has its side effects and when I say side effects, I mean ALL of them. Whenever my pharmacist prescribes me with medication he always gives me the side effects booklet despite if I have taken them before as he knows I literally get all of the listed ones. So, I’m now on a week of more antibiotics to cure the side effects I got.

In the last two weeks I’ve also had other problems occur too. Such as my blood sugars dipping to 3.4 or going high to 17.6! The diabetic team told me there isn’t really an explanation to blood sugars with CF Related Diabetes and it takes a few years for your body to ‘get used’ to it after diagnosis. For example, on Monday I had 1 unit of insulin with breakfast and my Calshake at 8:15am and by 10:30am my levels had dropped to 3.4. So yesterday I had no insulin with breakfast and found myself having sweats to then realise my levels were 17.6. There’s arthritis and joint pain down to the CF too which randomly comes on. This week it’s been coming on each day at around 2pm, so by the time Craig is home from work I’m walking like an old lady if I haven’t taken any pain relief. It usually goes again after a few days though so hopefully by Friday the pain has eased.

Anyway, there’s just a little update of how my week is going! My real reason for writing this blog is because it is CF Week 2019 which is a week dedicated to raising awareness of Cystic Fibrosis by kindly donating and wearing yellow to secure a brighter future for people like me with CF. You can find out more on the Cystic Fibrosis Trust website. However, I am hoping to raise a small amount towards the fantastic Cystic Fibrosis Unit I attend at Frimley Park, who are currently campaigning to expand the CF Unit to a 10 bed in-patient unit with 10 treatment rooms so they can continue to support and care for people like me.  As you may know with Cystic Fibrosis, there is a segregation policy in place where I cannot be around other patients with CF due to risk of infection. Therefore, with your support, myself and other patients can receive the treatments needed and feel less stressed about missing home during two week hospital admissions.

Below is my link..

https://justgiving.com/fundraising/naomijones1805

Many Thanks!