My Trikafta Experience in Australia

On Thursday morning, 11 August 2022, I started Trikafta.

Baseline stats about my health

My lung function FEV1(%) is 55-60 and FEV1(L) 1.8L. In the past year I reached 66% at 2L but I wasn’t able to maintain that. The medication I take is:
- Vit C (once daily)
- Iron (once daily)
- Salt Tablets (2 per day)
- Vit B Complex (once daily)
- Creon 25,000 (4-6 daily)
- Symdeko (morning and night)
- Salbutamol Inhaler (2 morning and night)
- Symbicort Inhaler (2 morning and night)
- Pulmozyme when sick (started Monday 15/08/22)

I also have MRSA however it hasn’t been active in just under a year.

Infections and recent symptoms

For the past month, I had a slight pseudomonas exacerbation with dark thick mucus each morning and night. After starting Trikafta that stopped completely and instantly. It didn’t phase out, it just stopped. I had the purge happen within the first 12-24 hours before my sputum changed to just clear but constant. From Saturday, I was only productive with physio or after having my inhalers.

I have had CMV (Cytomegalovirus. Received from a co-worker who had 3 viruses from his kidney transplant and no physical contact between us) which will never leave and reinfected me for the first year with every infection I had. I have had Haemophilus Influenzae (blood flu then spread to lungs, picked up by another co-worker) which reinfected me over approx. a 2-year period with each infection I had (same crossover period as CMV.)

I had EBV (Epstein-Barr Virus, herpesvirus 4. Picked up by the same first co-worker with a kidney transplant) but has never shown again since the first infection. Safe to say, there was a 2-year or so period where I had constant infections with constant 40% FEV1(%), hospital admission, and struggles to get back up to even 55% when healthy.

A rocky start

My Trikafta experience so far has been overwhelmingly good even with the following things that happened.

• Thursday morning, I took my first dose. My Lung function prior was FEV1% 59 and FEV1L 1.8.
• Thursday night, I coughed up a very small blood clot. Clots are not new to me, but they were something that happened often years ago, which makes it a new symptom.
• Friday morning, I had a minor fever.
• Friday night, I had a severe fever with uncontrollable shaking and no way to calm it, even with NSAIDs. I then went into Emergency and waited 3 hours without being seen. At 3:30 am I left as the fever subsided and there weren’t any other symptoms.
• Saturday, I had only experienced a minor fever all day, I felt uncomfortable in my own skin and that’s all.
• Sunday night, I had a stitch that wouldn’t go away. It was painful to breathe at rest and even worse breathing deeply. If I coughed or had a coughing fit, I would be in agonizing pain that went down the right side of my ribs and stomach.

Something wasn't right

The pain felt like it was under the skin and above the lungs. It felt like it was the lining. After doing a quick Google search, with a grain of salt, I felt as though it was pleurisy that I was experiencing. My coughing fits were dry and tight-chested, yet I had already been taking salbutamol, so it didn’t make sense. Pair it with the fevers and it’s quite simple.

After 1.5 hours I decided to go back to Emergency to have a Chest X-ray done. The X-ray only showed an infection although the scan was completed after a nap and therefore minimal inflammation. I even felt the difference. After being awake the pain got even stronger than before.

A painful hospital stay

I went through all the struggles of attempted cannulation and mid-lines and finally by Tuesday had a Picc-line put in. In 48 hours I had been pricked by 9 needles with only 2 being successful. The others lasted for their purpose or were straight misses. I don’t allow anyone to redirect after entering the skin as the pain it causes is not worth it.

After starting my first dose of IVABs (late Monday night, Cefepime) the pain subsided by 60%. The next morning after my next dose, it was only a 0.5/10 pain. Doctors won’t outright say what the cause for the sharp pain was as there were no definitive results and no clots (confirmed by CT Scan).

Trikafta's role

Throughout this whole time, I kept taking Trikafta and only missed one dose (Sunday night) as that was when I was most worried Trikafta was the cause. I now believe it stirred something up and my lungs reacted to whatever had been uncovered from the deep dark depths of my lungs.

I am now on day 2 of IVABs and day 6 of taking Trikafta. My lung function today is at FEV1% 71 FEV1L 2.11. I’m incredibly mind-blown and confused. How did I go from struggling with my lungs and health, organising my future based on what my limitations will be, always making sure I eat as much as I can and full-fat, full-protein to now having to plan for a longer future that includes a healthy diet?

Where I'm at now

My doctors are currently telling me that they’re sorry, but I need to stay in hospital for 2 weeks as I have a chest infection. Where? I’m confused because my body is telling everyone it’s fighting an infection and yet, my lung function has not only increased by 12% in 6 days, but I have now beaten my highest out of the past 6-10 years. My memory is a blur because as my function went down each year I lost hope and lost count of when in my timeline was a good time. To comprehend such a change is a struggle.

The gift of time

My brother, William, lost his life to CF at 23. I am now 24 and offered the opportunity to live a healthy, stable, and longer life. Life is not fair sometimes, and right now it’s a contradiction.

I know my writing is a mess but I’m not a blogger nor do I write things often. I just wanted to share these past 6 days because I always felt blessed with having Symdeko and now I have this thing called Trikafta and I’m still waiting to wake up.