My Symkevi Experience: Am I actually having to work harder?

I started taking my first CFTR modulator drug - Symkevi - on 31st January 2020, after it was made available on the NHS in the UK.

For someone with cystic fibrosis (CF), I consider myself really healthy. But, with each year that’s passed until now, maintaining good health has taken more and more work. My hope for starting Symkevi was that I would have to work a little less hard to maintain the health that I have now. But the changes that I saw in myself have actually been very different than that… Am I actually working harder?

My first month on Symkevi

The first thing that I realised was that I really wanted to take advantage of the opportunity presented to me. I wanted to do everything I could to make sure that I had the best chance of Symkevi working effectively. My motivation changed. I felt so driven to do more, to switch things up, to go beyond what I usually do on a daily basis.

Because being on my first modulator drug gave me hope and really inspired change in me, I’m not sure whether the physical changes that I’ve seen are due more to Symkevi or to the changes that Symkevi inspired me to make in other areas... But, to be honest, I’m kind of accepting them as one and the same.

Maybe the first thing to change was my motivation to get active. And I’m very active anyway. I go to the gym several days a week, I walk, I do occasional classes: yoga, spin, dance. But I became more dedicated. I stuck to my training plan more.

Plus, I started running. Well, I use that term loosely. It’s a jog. And a slow one at that. But that doesn’t matter. For the first time in over 10 years, I’ve jogged for a solid 20 minutes and found that it was actually easy. Part of this was down to really slowing my pace. I’d generally prefer to race through 30 second intervals than to slow down to a plod, but it was so worth it. It feels so good to see that my body can do this. And the airway clearance that I get from jogging is like nothing else. The combination of increased breathing and the ‘shaking’ from it being high impact does wonders for my lungs. I don’t know if my ability to jog like this was down to Symkevi. I actually kind of doubt it. I think it’s more to do with my improved awareness of pace and improved determination to try. But then again, the latter was as a result of Symkevi...

The next big change? Airway clearance. I’ve been dedicated to taking all of my nebs and inhalers every day. I even make a checklist on my phone. Airway clearance is on that list too.

Mainly I’ve been trying new techniques. Some of them, I learned or had reinforced by Pamela. Pamela is Beam’s co-founder, physiotherapist and yoga instructor, and I also consider her a cherished friend. She explained to me how to do assessment breaths and gave me a refresher on autogenic drainage. And they really, really work!

Another change to airway clearance was that it started to feel easier to cough. Less abrasive.

There have been days while, doing this increased exercise and increased airway clearance, that I felt really drained. So tired from the extra effort. I’d schedule my weeks so that I could take evenings and afternoons off work to lay in bed and watch Netflix (I haven’t done this since being on IVs in 2018!). That feeling of exhaustion is hard. But I’ve never been so sure that it’s worth it.

And here’s why.

Another change. I got more vigilant with my home spirometry testing. And my other health monitoring. I was keen to make sure that my transition into starting Symkevi was captured. I wanted to try and establish a baseline before starting, and to monitor changes to my numbers from that first dose onward. If nothing else, I feel really good about the fact that I’ve been better at capturing all of the data on my Project Breathe app each day. But what’s really awesome is that, just over 3 weeks in, I was noticing an upward trend in my spirometry results.

Then, at 4 weeks, I had my CF Clinic appointment. First thing I do when I get to clinic is do a lung function test using the hospital spirometry equipment. After my first attempt - blow number 1 - I nearly cried.

Usually, I really try hard with my spirometry. I do as many tests at they will let me do. Usually that's a full 8 attempts. The first few are usually a 'warm up' and then I notice that around test 4, my results creep up a little. But it takes real effort and determination. And there's always a number in mind that I'm shooting for. Sometimes I hit it, sometimes I don't. But this time, on my first blow, I hit 1% over where I was aiming.

The exercise, the airway clearance, the drug adherence… while it’s still early days, I already feel like it’s all paying off!

3 months on Symkevi. Cause or effect?

A little over 3-months on from me taking my first dose of Symkevi, I had a phone consultation with my CF clinic consultant (I obviously wasn’t taking my usual trips to clinic due to COVID-19).

She asked how I was getting on with Symkevi. What did I tell her?

On the whole, I’m doing great. I’ve had no major side effects from Symkevi but several positive things have happened. The only thing is, I don’t know if they’re down to Symkevi, or due to other changes that have come about because of Symkevi.

Here’s what I mean:

Lung function upward trend

The results on my project breathe app from my home spirometer show an upward trend to my lung function tests.

Is this due to Symkevi? Or is this due to my increased vigilance with airway clearance and exercise?

Airway clearance is easier

I’ll spare you the details. But in essence, I never used to be very ‘productive’ when I coughed. Now, well, it’s absolutely no problem to get a sample, that’s for sure.

I wondered for a while if this was a good thing or not. As anyone with cystic fibrosis might know, an increased cough could be really good...or really bad… It’s either a sign of great airway clearance (really good). Or it’s a sign of an exacerbated infection (really bad).

But, since my lung function was increasing and my coughing was pretty localised to deliberate airway clearance, I figured it was probably good.

The only downside is that with increased samples for my clinic, bugs that we thought may have been eradicated started to appear on my cultures again. I can only assume that they were lurking deep down there somewhere. And I can only be hopeful and optimistic that all of this increased clearance will rid my lungs of them entirely, sooner or later.

Insulin dosage reduced

I have CF-related diabetes and take two types of insulin: Novorapid (which I dose with individual meals and snacks) and Levemir (which I administer once daily, as it’s longer-lasting). My diabetes nurse suggested that, based on my FreeStyle Libre data, I could reduce my Levemir dose from 4 units to 3. Not huge, but it’s a little something.

Creon dosing

The dose of digestive enzymes that I take with meals has changed. Or has it?

If I’m being totally honest, I have no idea what correct Creon dosage looks like for me. Every year, when I was at my paediatric clinic, my dosage was increased along with my age. It wasn’t until I got to adult clinic that my dosage was reviewed and reduced. I’d since been taking between 1 and 4 Creon 10,000 capsules with a meal. When my dietician asked me how I dosed it, I assumed I needed around 1 Creon 10,000 for 15g of fat.

However, when I started Symkevi, I wanted to be sure I was taking the drug - as instructed - with fat-containing foods. At least 10g with each dose. So, to make sure I was doing this, I started to track my fat intake more diligently than ever. And I realised that snacks or meals that I was only taking 1 Creon 10,000 with contained perhaps 30 or 40grams of fat!

Now, was I needing less Creon? Or had I just never paid full and diligent attention to every gram of fat in my meals? Had I always been taking a little more than I needed? Who knows!

Working harder on Symkevi

It turns out that taking a CFTR-modulator hasn’t led to me working a little less hard, as I first hoped and thought. Actually, I’m working harder in almost every area, but the benefits are way bigger and more immediate than I thought that they’d be, too.