22/05/2023
It's me banging on about Organ Donation again... 🙂
Helen is currently not on the active list for a lung transplant after benefiting from Trikafta,but was on the list for 4 years. & shares her story during this our lung month;
“I was diagnosed with cystic fibrosis (CF) in the early 80s at three years old. I was very unwell at this point and the majority of my lungs had collapsed.
Once I was on a treatment regime, my parents managed to get my health on track again. I had yearly IV's roughly until the age of 11 or 12 where I started to need them every three months or so.
When I transitioned to the adult Cf team, the consultant there suggested most of my problems had come from my right lower lobe which had never reinflated. He suggested that I have a low back to me. At the age of 18 I had this operation. It was quite traumatic at the time and I spent a lot of time off school (six months).
However, this did seem to benefit my health and I was even able to go to uni the following year. Uni was a great experience for me and I certainly made the most of my time, even managing to fit in some study amongst all the socialising.
I was still having regular IVs and would have them at home in & around my activities. I worked full time after uni in a range of jobs settling on a career in marketing, which my degree was in.
As I got older my IVs became increasingly frequent and fitting them, and hospital appointments, in alongside a full time job & it became too difficult so reduced my hours at work.
In 2016, my health took a massive decline and I spent 10 weeks in hospital, and was extremely lucky to make it home again. During this episode, the team and I discussed how i really needed to be on the transplant list as it was obvious how ill I was becoming.
After many assessments I was told I was too well for the list but my CF team asked them to reconsider especially as id just experienced another difficult infection. The second time, the transplant team agreed to accept me onto the list.
I had mentally prepared to wait two years and not have any calls. After that as I was getting progressively worse, things were very difficult.
I wasn't well enough to do my IVs at home and needed the intensive physio at hospital. This meant at points I was spending more time in hospital than at home. And even if I was at home, I couldn't plan anything as Id usually be too unwell to attend. It really was a case of just getting through the day.
I had spent most of Jan to March 2020 in hospital and things weren't looking good. I was in hospital when I found out my transplant centre was closing their unit and wouldn't be doing transplants until further notice. This felt like an absolute blow as it was the only hope.
As it turned out, lockdown was very pleasant for me. Warm days in the garden when felt able to, and many rooms and the security of a covid free area at home with the company of my boyfriend and dog.
A few months later, during another hospital stint, I was given compassionate use of Trikafta (Kaftrio), a drug to help correct the faulty gene. The effects on the lungs were almost immediate and astounding. I had just finished four weeks of iv’s and felt like my chest was clear, yet was coughing copious quantities of sputum up and such delights such as fungal balls which kept appearing in the following days and weeks.
However all was not plain sailing. I have balance issues caused by Gentamycin toxicology,& moving onto Trikafta caused my balance to go completely. To the point where I needed the help of 2 others just to go the few metres to the bathroom. We tried various doses, but nothing seemed to help. On top of this, I completely lost my appetite and was experiencing a lot of nausea and vomiting.
I needed to make a decision - to stay on the drug and try and cope with these issues or to stop the drug completely. I was so poorly before I started. I knew realistically wouldn't be long for this world if I stopped the drug.
So I went home and tried to cope as best as I could... which wasn't very well.
About 8 months later, my consultant started to suspect I had Adrenal Insufficiency as I perked up so much when on steroids. A test showed this to be the case and started on artificial steroids to replace the cortisol my body couldn't make. I felt better quite quickly but the biggest surprise was my balance, nausea and sickness all improved dramatically.
By this point the transplant centre was starting to see if other transplant centres would take on some of their patients, and I ended up going to Harefield. I had CF friends who were very complimentary about the hospital
By the time had my appointment, it was clear the issues we had thought were due to Trikafta were as a result of the Adrenal Insufficiency, and could now stay on the drug and benefit from it.
My lung function improved from around 27% to 40%. I felt so much better. Things are still difficult but I am able to do so much more and have a better quality of life. I am seen by Harefield every six months as they want to keep a close eye on me considering how ill I was.
Whilst spending a lot of time in hospital, I decided to try and raise awareness of organ donation and the need for people to share their wishes. I was interviewed in different local media including TV, newspapers and even a live radio broadcast.
I started a blog (www.butyoulooksowell.com) and Instagram (www.instagram.com/butyoulooksowell) account to show people what life was life waiting for a transplant and encourage conversations around the subject.
I am still closely involved with transplant community and am well aware that at some point in the future I may go back on the active waiting list.”
Thanks to NHS Organ Donation for working with us on this piece.
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