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
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