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!

Organ Donation Week

But you don't LOOK ill? Why are you parked in a disabled space? How comes you only work part time? How comes you spent a week in Ibiza if you're ill? The questions I get asked on a regular basis by people who are unaware of what CF is and how it affects me. 

Listen to this video and look at this photo of me and then continue to ask yourself these questions. 

 

 

 

I may look 'well' to you but you couldn't actually be more wrong. Each day my lungs are slowly deteriorating due to a build up of mucus which I cannot clear and it's becoming more of a challenge to keep well. Think of it this way, when you catch a cold/chest infection your body works its hardest to clear that infection and it's gone within about a week. The build-up of mucus in my lungs causes chronic infections as I cannot clear what's stuck down there which leads to a reduced lung function, and I often end up in hospital on iv antibiotics for weeks. To avoid this from happening as regularly I spend hours doing treatments, physiotherapy and nebulisers but they are only to keep my lungs healthier, unfortunately they aren't a cure. 

CF also affects my pancreas as it is blocked with mucus meaning that the enzymes required for digesting food cannot reach my stomach. This also means I cannot absorb fat. Therefore I have to eat lots of fatty foods and take enzymes (tablets) every time I eat and if I don't it will cause more complications. 

So I may not 'look' ill to you but I definitely feel and sound it. 

So, why are you parked in a disabled space you ask? Again, although I look 'well' I can't actually walk more than 20metres before I need to sit down or stop to catch my breath. I often have to remind my family/friends to slow down as I can't keep up! So next time you go to judge someone for being in a disabled space, just remember not all disabilities are visible! 

Why do I only work 20 hours a week? The harsh reality of CF hit me when I started a job, working 40 hours a week around 4 years ago in August. By the December of that year I dropped my working hours to just 16 a week. This was because I physically couldn't do the hours as my body was becoming too tired and I was nearly asleep in the afternoons. I also wasn't finding enough time to fit my treatments in as I was coming home and just sleeping as I was too tired to do anything else. Therefore I work part time now, just mornings I'll add, so I can come home and have an afternoon nap to recharge myself before doing all my afternoon treatments. That way I can try to stay well as I have more time to focus on treatments. 

On to Ibiza now. I spent a week of partying and having fun with my sister where we didn't see a bed until 5am most mornings and didn't wake up until about 4pm. Sounds great right? Not so great when you think you'll be able to keep up with everyone else despite the fact you have CF. Food wasn't really on the top of the list of priorities when in Ibiza so we sort of just ate when we were really hungry and often forgot about it otherwise. This meant probably only eating one meal a day- if you can call a slice of pizza a meal! This is definitely a mistake I won't make again. Remember me saying I don't absorb fat? I came home to find all of my clothes were too big and I'd lost 11lbs in weight in just a week! So I'm back in size 6 clothes and on lots of supplements to try and get my weight back up. Although my lungs felt fine for about a week after getting home, I picked up a cold from somewhere and it's gone straight to my chest. 2017 has been my best year as an adult with CF as I have stayed the most well that I have been in years. However, this week has given me a little reminder of how CF really affects me when my lungs are struggling. Let's just say my lung function has dropped 20% and I am non stop coughing and struggling to clear the mucus even with my physio equipment. I went to the hospital on Friday and was prescribed with some oral antibiotics but they have made me feel groggy and worse! So no doubt it will be a trip to my second home soon (the hospital) for some ivs. 

So why am I writing all of this and why now? It isn't to make anyone feel sorry for me but it is to outline how CF affects people. I am doing this because it is organ donation week and I am urging all of you to sign up to the organ donor register! Luckily, despite all of the above, I am still at a stage where I can live my life with these lungs. However, this may not always be the case and it wasn't for my cousin Sian and many other young people. By signing up to become an organ donor, you could help people with CF get that second chance at life. https://www.organdonation.nhs.uk/register-to-donate/?campaign=1160

Annual Review 2016

Hello!! Look at me.. I've remembered to update my blog! 

I had my annual review yesterday which I think went well. However I don't get the results until the end of July. 

I arrived at 8:30am where Judith took my blood straight away to begin the glucose tolerance test. Gotham then did my obvs and lung function which is still up at 63% so that's good. After this I had to drink the sugary drink for the glucose test. I absolutely hate the drink and it takes me about half an hour to drink it every time! However, they have changed it and it actually has a bit of flavour to it now. I managed to drink the whole glass in less than 4 minutes... Go me! An hour after finishing the drink Judith took my blood and again after two hours and I was then finally allowed to eat! I now let her take my blood without Emla cream which if you know me you'll know how needle phobic I used to be. 

In between the times the blood had to be taken, Judith did the normal annual review talk with me where we talk about having children, transplant, my worries, my future and a few other bits.

Charissa came in with a student dietician and we discussed my weight and how my day usually goes eating wise, such as what I eat and how many enzymes I take a day. My weight has improved since last year and is managing to stay up without any supplements so Charrissa was happy with that. What she wasn't too happy about though was the fact we put my target last year as 'to try a strawberry' and I still haven't done it! I told her I drink strawberry daiquiris but unfortunately she told me that doesn't count haha! So she has told me we are doing a 'strawberry challenge', and at my next appointment she is going to bring strawberries along and make me try one so there is no getting out of it haha! This target was set because I am so fussy and don't eat any fruit. After this, Charissa and the student did a few tests including the grip test, where you have to grip your hand on this handle thing as hard as you can and it gives you a score. Last year my highest score was 18. This year it was 22.4!!  

Once all the bloods had been done and Charissa had finished, Gotham took me to X-ray. Unfortunately, Parkside was closed so I had to go down to the normal X-ray department and ended up waiting over and hour despite the receptionist saying she would 'fast track' me. Once it was finally my turn I had two X-rays; chest and abdominal. 

By the time I got back to the CF unit, I had missed my lunch so had to order something else and it was 1:10pm already. This then made me behind schedule and everything else seemed to be a bit of a rush. 

Lisa, the physiotherapist, came in and saw me and we quickly discussed my lungs and how I have been feeling chest wise over the past year. I am also supposed to do a fitness  test but Lisa had to go somewhere so I didn't get to do it. I missed it last year for some reason too so that's two years with no data! She said hopefully we can do it at my next appointment. 

Alice, the research nurse, then came and saw me. Back in January I saw a new 6 month clinical trial that the CF trust are doing so I asked Dr. Orchard about it who then passed me on to Alice. The trial consists of getting many biometric results daily and uploading them to a smart phone. You have to record your lung function, weight, oxygen levels, steps count, how well you've slept, how well you're feeling, how bad your cough is on a scale of 1-10, and  you also have to do one sputum sample a day. So Alice came to set me up on the trial and gave me all the gadgets and showed me how to use them. The gadgets include; a bluetooth scales, lung function monitor, oxygen levels machine, fitness band (a bit like a Fitbit), a sputum pot for each day which you freeze once doing the sample and then take back to the unit when you can (a bit gross I know) and a smartphone with an app created by the CF trust where you upload all of the data. The trial is for 6 months and you get to keep all of the gadgets at the end. But can drop out whenever you like. I'm looking forward to it as I think it is going to be really interesting to see all of my results. 

Judith and Gotham came and did an ECG test on me which didn't take too long. Then finally I saw the pharmacist and we went through my drug changes over the last year and there has surprisingly been a few more changes than I thought. 

I was then allowed to go home 6 1/2 hours later! 

I didn't go straight home though! For years since before I was 18 I have wanted a 'just breathe' tattoo which is kind of known as the CF tattoo. Miley Cyrus has it because she did a starlight wish for a young girl with CF who sadly passed away not long after. I have also seen a few photos of girls with them and have just really wanted it. The last month I have been really thinking about getting it and contacted the local tattoo studio in Basingstoke and me and Abbi were going to book in to get them done. She wanted 'Jones' and a double infinity. However, last week she text me and told me she had gone elsewhere with a friend as we didn't hear back from the local studio. So yesterday I decided to go and get it done at the 'walk in' studio in Camberley. It took less than 5 minutes and as the man was doing it I thought 'oh it's not actually that bad' but as the needle got closer to my front it started to  really hurt! I love it though and can't wait for it to heal.

Sorry for the really long post, but I guess it makes up for not posting very often! Night night :) 

Vancomycin Nebuliser Trial

Hello!

It’s been a very long time since my last update, nearly a year in fact. I seem to never have to time to sit down and blog like I used to! This last year hasn’t been a brilliant one for me CF wise. It has been a real battle to try and keep my lung function above 60%, so I have constantly been on different oral antibiotics, Iv’s and nebulisers. I recently had some Iv’s at the beginning of March which managed to boost my lung function back up to 65%. However, by the beginning of April I was feeling rubbish again so have just finished a two week course of oral Septrin. I had hospital last Tuesday and even with the Septrin my lung function is still only just at 60%. Dr Orchard has been talking to me about a nebulised antibiotic called Vancomycin for quite some time. But he wanted to do some more research and speak to other consultants before trialling me on it. On Tuesday, I finally got round to doing a test dose of it and am now trialling it for one month. So far, it has been going ok, it seems to make me quite breathless and tight chested but I do Bricanyl inhaler just before the nebuliser so that seems to ease the tightness a little bit. Hopefully this will boost my lung function and keep it stable. Fingers crossed! I am also on Chloramphenicol oral antibiotics for two weeks so hopefully they help out to!

Me doing the Vancomycin Nebuliser

 

Job wise, I now work in a junior school as a teaching assistant and I love it. I had an interview there back in December and found out on the same day as having the interview that I had been successful and got the job! So I have been there since the end of January. It is more hours than I worked at the nursery, but I seem to be managing it so far, so hopefully this continues.

Unfortunately, we lost our dog Maddy back in March as she had a tumour. We all really miss her.

I have to go back to hospital for a follow up of the Vancomycin trial on 13^th May so hopefully all goes well! I then have my annual review on 2^nd June, so if I remember to get round to blogging... I will do an update!

Sorry it was only a quick update!

Night :)
 

Annual Review Follow Up Appointment 2015

I went back to the hospital yesterday afternoon for my annual review follow up. I went to work in the morning and then left at 3 o'clock for my appointment at 3:30 and mum met me there. When I arrived I did my weight and lung function. Unfortunately, my lung function had dropped down again, but not massively. It was 63%. 

Dr. Orchard came and saw me and we spoke about my annual review results. We said this year hasn't been the best for me with my lung function constantly dipping and us battling to keep it up. But he said he wants to be able to find something that will help to improve my lung function and stop me having so many ivs, as I had two courses very close together back at the beginning of this year. He mentioned a drug which can be nebulised, but I can't remember the name, which they have started to use in America and have seen good results so he was going to look further into it and maybe think about trialling me on that. But in the meantime, until he knew more about it, he suggested trialling me on Bronchitol. Bronchitol is a a dry powder inhaler done before physio and is designed to reduce the amount of mucus build up in the lungs. He thinks my lung function is low because there is mucus stuck right down in the bottom of my lungs which he believes can be brought up with the help of Bronchitol and lots of physio. So I have an appointment with Jo next Friday to show me how to use Bronchitol and to make sure I don't have any immediate reactions. The review results were fine except that so there were no more changes and Dr Orchard said to finish the course of Septrin that Dr.Higton gave me last week and to keep on slowly easing off the steroids right down to 1 a day so I'm still on those for another two weeks. Then I have an appointment to see him again on the 24th July.

It's all fun and games in the life of a CFer!

Not The Outcome That I Expected.

On Wednesday, Brenda called back about 12pm and said she had spoken to Dr.Higton and she would see me at 3 o'clock. So I got dressed and ready and packed a bag with some overnight things and clothes for a few days as well as my meds and physio machines as I was expecting to be admitted with how rubbish I had been feeling for the past fortnight. Abbi then drove me to the hospital so if I did have to stay she would stay for a few hours so I wasn't bored.

When we got there, I did the usual routine. I got on the scales and my weight showed the most I have ever weighed and my BMI was finally at 20.0. I guess five weeks of pigging out on food because you're on steroids shows on the scales. After doing my weight, Brenda set up the computer ready to test my lung function. I then went ahead and did the first blow and could not believe the results. 2.23 litres. I don't remember the last time it was that high. We were laughing at the fact it was so high when I was feeling so rubbish. I did two more tests and again they showed higher results. I always read the percentage in the end as I never really understand litres but my FEV1 results were 70%. I checked and it hasn't been that high since September last year and before then it was months ago. Typical. I feel really tight chested and breathless and I'm up all night coughing. Yet from my results you would think I was making it all up!

Dr.Higton came and saw me after and laughed with me about the lung function as we were both so shocked. I explained to her what had been happening over the last month as I saw Dr.Orchard at my last visit at the beginning of June. I told her about the reaction to the Symbicort, the tight chest feeling after doing the Aztreonam, the waking up in the night etc. She said she thinks that actually I am allergic to the Aztreonam judging by how I had told her it made me feel but because my body has had such a kick of different antibiotics, steroids, nebulisers and inhalers my lung function has managed to improve. So as an outcome, she told me to completely stop the Aztreonam and go back to my Promixin in the Ineb. She gave me another two week course of Septrin antibiotics as they had made a difference to my lung function and made me feel better prior to my appointment back at the beginning of June. And she said to carry on easing slowly off the steroids that Dr.Orchard had prescribed a few weeks ago.

So off home again I went. Lets just say it wasn't the outcome I expected! Since stopping the Aztreonam, my chest has been really loose and I have been coughing up a lot more sputum so have had to add in extra physio each day. I explained to Dr.Higton at the appointment on Wednesday that my chest was feeling loose and she said it was probably just because I hadn't had a nebuliser that morning and it should feel fine after the Promixin that evening. But it seems to have been loose for a few days now so I will have to bring that up with her. Wednesday and Thursday night I finally had 2 good night sleeps and didn't wake up coughing! So Friday as I was feeling a little better I went down to Devon to Jane's farm where Grandma and Grandad were staying in their caravan, as Dad was participating in the fishing competition along with Uncle Mark. But Friday night, my chest was at it again and I was up most of the night coughing so I don't know what went on. So Saturday, when they were taking part in the competition I had a nap in the caravan and me and Dad ended up coming home last night instead of today.

My chest has been a bit tight again this afternoon so I did some physio and managed to cough up lots so the Septrin must be loosening it all up.

I have hospital on Tuesday as it's my annual review follow up from May so I will get all the results back from that. Dr.Higton is on another ward that day but she said Dr.Orchard will decide what to do with the steroids and when to see me again after the antibiotics providing my lung function is still high. I hope he stops these steroids those as my face is so chubby where I have been eating so much where they make me constantly hungry haha!

I've finally posted an update after 4 months!!

It's been months since I updated here. After Grandma recently came over and said the exact date I last did a post I thought maybe I should finally get round to updating! Well my health hasn't been great this year. Since my last update in February, I had IV's again in April and have been put on various different antibiotics and steroids and just missed them about 3 more times where my lung function has managed to creep up a little bit. But now I'm at a low point again and think I will probably be admitted to hospital today. 

Back on Monday 8th June, I went up for a hospital appointment following a recent dose of oral antibiotics (I can't remember the name of them now) I had not had before and steroids. I had just been to Disneyland as well and with the help of lots of walking and different antibiotics, my lung function improved and I was feeling a bit better! At my appointment before this, Dr. Higton suggested to trial me on a new nebuliser when I got back from Disney so Dr. Orchard increased the length of time I would be on the steroids but to slowly drop the dose as the weeks pass rather than just put a stop to them all together incase I didn't get on with the new nebuliser. Then Jo came and saw me and got me started on that. The new nebuliser is called Aztreonam and is done in a whole different new nebuliser called an Eflow. It's pretty simple to use and the drug has to be done 3 times a day. Dr Orchard also started me on a new inhaler called Symbicort. So off I went home feeling a bit better and ready to start my new neb and inhaler. Mum went away to Turkey the following day so it was lucky I was feeling better otherwise I would have had to stay in hospital for two weeks where mum isn't at home to help with the home IV's. 

By the Thursday I wasn't right again. A horrible rash had appeared on my hands and feet, it had made them go very dry and itchy. I knew this was an allergic reaction as I am allergic to another drug and get this same reaction if I take it. So I called the hospital on Friday and spoke to Brenda. She spoke to the doctors and they said to stop the Aztreonam over the weekend and see how the rash is and call them back on Monday. After speaking to Brenda, I realised it could also be the Symbicort causing the reaction so I stopped that too. By Monday the rash had gone so I called the hospital and spoke to Emy. I told her I wasn't sure if I it was the Aztreonam or the Symbicort causing the reaction and from reading the side effects to them both I think it was probably the Symbicort so I was happy to try the Aztreonam again. So she said that was fine and to call again in a few days to give her an update. Well by Wednesday I was now feeling very tight chested, had a constant tickly throat so kept coughing and was having sleepless nights. I went to work on Wednesday feeling very unwell so once again had to call the hospital.  I spoke to Brenda on Friday and asked her if she could speak to the doctors and get them to prescribe me a different inhaler because before I had stopped the Symbicort my chest was feeling fine when using the Aztreonam.  So they prescribed me with Seretide inhaler which Abbi went and collected for me and I called into work sick on Friday as I had been up all of Thursday night coughing. 

By Saturday the Seretide inhaler had given me a sore throat and I was now full of cold with the tickly throat still there. I had another sleepless night on Friday so was not feeling good again. Saturday night and Sunday night were the same so I spent all of Monday in bed to try and make sure I would be well for work yesterday. Monday night was once again the same and I was awake at 3am for about two hours just cough cough cough every two seconds. But I went to work yesterday to see how I'd get on. By my lunch break I felt really breathless and tight chested so went and called the hospital and got sent home from work. Hospital didn't answer so I left a message and then went to sleep in the meantime. I woke up at 5:30pm and tried the hospital again and got through to Judith. I told her what had been happening over the past few weeks and she said to stop the Aztreonam and she would leave a note for Brenda to speak to the doctors today and hopefully get me seen. She asked if I think I needed IV's but I said I wasn't sure because I have never felt this ill for this long but then again I don't know what my lung function is doing as it all could just be in my throat. 

I was up again in the night at 1am for over an hour and then again at 4:30am so I haven't gone into work today and I am just waiting to hear from Brenda so I know when to go to the hospital. Hopefully they finally sort me out and I start to feel better soon!  

Mum and dad are back from Turkey tomorrow so hopefully if I'm admitted to hospital I won't be there for too long as mum can help out with home IVs. 

I'm going to try to go back to sleep now before Brenda rings. 

February IVs

Back on the 9^th February I had a hospital appointment following up from my January appointment. You may remember reading from my previous blog post that I had been up in the night coughing quite a lot so Dr. Higton told me to double up my dose of Omeprazle as it was most likely to be acid reflux. Since doing that, the coughing in the night stopped but my cough in the day has been very productive and I have been coughing lots. So at the hospital I was expecting the lung function to be lower than usual.

I arrived about 11:15am and mum met me there straight from work before her afternoon clinic started. When I arrived Jackie did my obs and I did my lung function. As I was expecting, the lung function had dropped to 50% which is quite low for me. Therefore, I was expecting IVs. Judith came in and saw me and asked how I was feeling and I told her I wasn’t feeling great. She said “Do you think you need IVs?” and I said “yes but I’m not staying in” and she told me they didn’t have any beds free anyway. (Thank god! Haha)

Charissa came and saw me first and wasn’t too concerned about my weight as it was stable to last time. However, I explained to her that I had been having some stomach troubles so she gave me a food diary to complete over the next few days where I include what I’m eating and how many Creon I am taking. She said Dr Orchard may want an X-Ray to see if I’m blocked up.

When Dr Orchard came in, we spoke about my stomach troubles first and he said after reading my notes and speaking to Charissa that the Omeprazle change of dosage would have had something to do with it because it stops the Creon dissolving as fast which would explain my stomach pains. But he would like an X-Ray to see if there is a blockage. Charissa also came back in and told me to change my Creon-fat intake to 9g. So to take 1 Creon capsule per every 9 grams of fat I eat.  Dr Orchard and I then spoke about my lung function and agreed that I would need some IVs for two weeks. He also said he didn’t want me going to work because technically I should be staying in hospital where the lung function is so low but he knows I will do all the treatment. But he wanted to see me one week into the IVs and if my lung function hadn’t improved he would then want me to stay in the hospital. So once all this was discussed, Jackie took me down to the X-Ray department to have my X-Ray. In the meantime mum had to go to work.

Once we were back at the CF Unit, Judith came and accessed my port and started up my test doses. Then she went to contact Calea to get the home IVs delivered. However, they don’t deliver until 48 hours after ordering so she had to call pharmacy to order all of the medication so that mum could draw up the IVs for the first 48 hours until the delivery arrived. The test doses took about 2 hours as Dr Orchard prescribed me with 3 different Ivs; Meropenem, Tobramycin and Teicoplanin. Then I waited for the pharmacy to bring the drugs up to the unit.

Since being at home mum and dad have been really helpful, getting up at 6am and doing all of the morning IVs and I have tried really hard with the physio and done it twice a day to cough up as much as I can. Then I went back to hospital last Monday and was so happy with the result..

.

My lung function was 70%!!!! It had improved 20% in just a week! IVs and hard work really do pay off!!! Because the result was so good Dr Orchard said he didn’t need to see me. So I just saw Charissa and Jo and then it was home again.

The IVs finally finish tomorrow evening and the one thing I am excited for is a proper bath!! I haven’t been able to have a proper bath as I can’t get the port wet. So I have been having to shower my hair first and then have a shallow bath afterwards.

I am also back to work on Tuesday which I can’t wait for. You’re probably thinking who wants to go back to work, but when you have been at home bored for 2 weeks and you enjoy your job you will want to go back!

I then have hospital again on Thursday at 2pm so I will wait and see what the result is then. Who knows, maybe the lung function is even higher!!

Hospital Appointment- 9th January

I had hospital on Friday which is my first appointment since 28th November. 6 weeks without having to call them to be seen earlier, that's good for me. 

However, I have had this horrible tickley cough for about 2 weeks now where I have been waking up every night coughing for over an hour but it's just a dry cough and nothing comes up. So annoying! I have been a bit more productive in the day than normal too but have been doing lots of physio to shift it all and cough it up. 

I drove myself up for my appointment at 11:30 as mum was working. So it was my first appointment on my own. I didn't mind though. When I arrived Jackie did my obs and I did my lung function. Surprisingly my lung function was at 68%!!! It hasn't been that good without ivs or oral antibiotics for a ages. So that was a good start despite the cough. Charissa came and saw me after, my weight had dropped a little but she wasn't concerned as it wasn't a huge amount.

Dr.Higton came to see me and I was happy to see her because she's been off work since January last year on maternity leave with her little boy. She said I was looking really well and was very happy that I have stuck at my physio since the last time I saw her. I explained how I had been feeling and the cough had been keeping me up at night. She explained that it is most likely to be acid reflux if nothing is coming up when I cough so told me to double my dose of Omeprazle and to take it just before bed and hopefully that will sort it out but it may take a few weeks. Then to keep up with the physio to carry on bringing up as much sputum as I can. That was a good result for me as I was expecting to be told I would need some Iv's, so I'm glad the hard work with phsyio kept the lung function up.

Jo came and saw me last and was pleased I was keeping at the physio and that the lung function hadn't dropped. Then Emy came and flushed my port and I was ready to go home. 

Hopefully the Omeprazle fixes the cough and it has sorted itself by my next appointment on the 9th February. Otherwise, Dr.Higton said it may be that I need some Iv's.