A personal look at starting Sotatercept with PAH and HHT — what surprised me, what was harder than expected, and what’s changed in 100 days.

For a long time, Sotatercept was something that came up at almost every doctor’s appointment.

It was always part of the conversation — something on the horizon, something promising, but also something uncertain.

During the clinical trials, I wasn’t eligible because of my six-minute walk distance. And even once it became available, there was hesitation from my doctors and parents. With HHT, there were still so many unknowns of how this medicine would interact with this condition. No one could fully say, do the potential risks outweigh the gain, or what the experience would look like. There are not many HHT and PAH patients on this medicine to know more data or have a clear answer on what to expect.

So, I cautiously waited. Until November 2025 when I made the decision that I did not want to wait anymore. I have been on the same treatment for 8 years, since my diagnosis, and while stable, I knew life could potentially be better with this medication.

Also, honestly, some of it was FOMO... I didn’t want to miss out on something that could have a meaningful impact, after seeing so many with PH doing well on this medicine.

With doctor's approval, in December, I had my first shot.

And now, 100 days later, I can honestly say: it’s hard to fully put into words what starting Sotatercept is like.

Because it’s not one thing.

It’s not instant.

It’s not always easy.

But there is progress, even if small.

WHAT I WISH I KNEW BEFORE STARTING SOTATERCEPT

Finding the balance is harder than you think

If you’re on Remodulin, the beginning of starting Sotatercept is not simple.

Finding the right balance between your Remodulin rate and Sotatercept is no joke. One of the hardest parts is trying to figure out what’s causing what.

Is something a side effect of Sotatercept?

Is it from Remodulin?

Or is it the adjustment between the two?

At the same time, you’re trying to figure out how much you can safely adjust your Remodulin rate to help manage symptoms — without going too far. Because what if it’s not the Remodulin causing it, and you’ve lowered your rate more than you should have?

There isn’t a clear formula for this. You’re not following a structured dosing schedule like when you first start Remodulin titration. It becomes much more of a day-to-day process of monitoring, adjusting, and reassessing.

And especially in the beginning, that can feel really uncertain and overwhelming.

“What’s tolerable?” isn’t always a simple question

Along with adjusting your rate, you’re also in constant communication with your doctors — which is reassuring. You’re not doing this alone.

But at the same time, there aren’t clear rules.

My doctors would often ask what side effects felt “tolerable.” If something was tolerable, then there might not be a need to adjust my rate or make any changes.

But what does “tolerable” actually mean?

That’s where it gets hard to define.

For example, one of my side effects has been headaches. Some days they’re debilitating — clearly not tolerable.

But other days, it’s more of a constant pounding. You can still get out of bed, still go about your day — but it’s there the entire time.

So is that tolerable?

That line isn’t always clear, and it can be difficult to know when something is manageable versus when it’s something that needs to be addressed, and adjusted.

Don’t focus only on the numbers

This was a big one for me.

After my first bubble echo since starting Sotatercept, I felt really discouraged. My pressure numbers weren’t showing a decrease, and it was hard not to fixate on that.

But what I learned — and what other doctors with more experience treating patients on Sotatercept emphasized — is that one number doesn’t always tell the full story, especially early on.

There are other measurements and indicators that can show progress in ways I wasn’t aware of. It’s easy to get hung up on one number, or even the overall appearance of the heart, but that doesn’t always reflect what’s actually improving.

Some changes take longer to show up in those specific metrics, while other signs of progress happen first.

Over time, I had to shift my focus away from just the numbers and pay more attention to how I was actually feeling day to day.

Because progress isn’t always immediately visible on a test — but that doesn’t mean it’s not happening.

The time and coordination it takes

Another thing I wish I had understood going into this is how much time and coordination it requires to be on this medicine.

It’s not just taking a dose and moving on — there’s a whole process around it.

You need blood work done within a specific window so your doctor can send in the referral. You have to stay on top of scheduling labs, making sure results come back on time, and confirming everything is sent to your doctors so you can receive the medication without missing your injection window.

And that window matters — there’s not a lot of flexibility.

On top of that, there are more doctor appointments and check-ins to monitor side effects, plus coordinating with a specialty pharmacy — which isn’t always the easiest process to begin with, but now you are having more to coordinate.

It ends up being a lot to manage, especially in the beginning when everything is new. Luckily I have a great team of doctors and caretakers who help me stay on track, but it truly takes a village on this medicine.

....But on the other side of that, there were also things I didn’t expect.

WHAT I DIDN’T EXPECT

Going into this, I tried not to have expectations. I didn’t want to set myself up for disappointment. But within these 100 days I have noticed a change in aspects of my life that may not have been possible without this medicine.

Subtle but meaningful physical changes

Over time, I started noticing small changes in how I was feeling.

My oxygen is no longer dropping below 90% at night.

My heart rate is staying consistently under 100.

My oxygen during the day is 98% and above.

And... I’ve also been able to titrate down over 20% on my Remodulin rate.

Doing the unexpected

It wasn’t anything dramatic or all at once, but over time progress showed up in what felt doable day to day. With the subtle changes noted above, I noticed I was able to be more physically active.

I could go on longer walks with my dog.

Stay on an exercise bike for 10 minutes.

Go up a flight of stairs (or 2!) without stopping.

Do a 2-mile walk

and even go up a hill with a 8% grade without stopping!

Not because it suddenly felt easy — but because it felt possible in a way it hadn’t before.

That’s the kind of progress that doesn’t always show up in numbers, but shows up in real life.

Reflection After 100 Days

At 100 days, this hasn’t been perfect.

Side effects are part of it, and there are still things I’m figuring out.

But there is progress — and that’s enough for right now, and something worth celebrating.

This is a lifelong condition, and while treatment has come a long way, there’s still a lot we don’t know — especially when it comes to HHT and PAH. That’s part of why sharing experiences like this matters.

Not because sotatercept is a solution for everyone, but because if this helps build a clearer picture of what’s possible and what to expect — and helps someone have that conversation about whether this medication is the right next step — that’s the goal.

I wish I had something like this, and that’s why I’m sharing what 100 days in has looked like.

Here’s to the next 100 days — and seeing what’s to come!