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
