A Giant Leap for Me ...

My First Evkeeza Infusion

It’s been a long time coming. I remember hearing about this new drug called Evinacumab (or Evkeeza) sometime in the fall of 2019 when attending The (then) FH Foundation’s yearly Summit (now, The Family Heart Foundation). One of the HoFH advocates at the event was participating in a clinical trial for it. I was terrified, but I think the world of people like her who are willing to put their body through anything, to navigate the unknown or the little known, in the darkness, it seems, to give us all better treatments. My words cannot be enough to express the gratitude for such people! 

In February 2021, Evkeeza was approved by the FDA for use in the USA for adults with HoFH, in addition to other cholesterol-lowering therapies. Two years later, the FDA approved it for children also. 

Around May 2021, I participated in a brainstorming session that Regeneron (the inventor of Evkeeza) organized with HoFH patients and medical professionals to test their marketing materials for Evkeeza on us. Amanda Seef-Charney, a Regeneron patient advocate, said at the meeting: “We must listen to and learn from individuals who so intimately know what it means to live with a rare disease. Only then can we begin to bring meaningful change ...”. This stayed with me. It’s so unusual when we have this almost invisible, rare disease to actually feel like you’re being heard. 

My own cardiologist did not hear me, when I asked whether I could be considered to be treated with this new drug in July 2021 (https://livingwithfh.blogspot.com/2021/07/who-knows-more-about-fh-you-or-your.html). He had no idea what I was talking about and the reason I went to him in the first place (starting with 2018) is because he advertised himself as a cardiologist specializing in lipid-lowering therapies. By November 2021, he started to look more closely into it and researching what we needed to do in order to get me approved. It was not until December 2023 that I actually got him to prescribe it and get it approved with my insurance. My first appointment was set for some time in February 2024, but the infusion center of his medical system had not heard of the drug and did not know how to order it. I knew I had to change course. 

So, I changed cardiologists and medical systems altogether. I started seeing my new cardiologist at the beginning of May 2024. Today, June 11, 2024, I finally got my first infusion of Evkeeza. It’s been such a long time coming (it'll be 3 years next month since I first asked my previous cardiologist if I can be on this) that it feels surreal to even believe I got it! 

The prior authorization was approved in less than two weeks, and the appointment was secured in two more weeks. As it turns out, this new (to me) medical system has an infusion center right here, in my small town - I usually have to travel at least 40 minutes one way to go anywhere from where I live. But only 12 minutes for the infusion center. It’s like Christmas. 

The appointment was early this morning (scheduled for 8AM). It was the first time in my life that  I stepped into an infusion center. Most people there were getting cancer treatments, but there were other folks with iron infusions and some other restorative medicines. The place had about 8 chairs that I could tell and they were all full. It felt packed and tight. No one was wearing a mask, including the staff, but my husband and I were. 

They weigh you just as soon as you get in because the infusion dosage is based on your weight. Then, because I am considered a woman of child-bearing age and with all my reproductive organs intact, I had to take a pregnancy test which is a urine test. They cannot give you the infusion if you are pregnant. They also drew blood for cholesterol levels which my doctor scheduled in advance. 

After all that and after the IV needle was in, they attached the IV bag. I took a picture of my first bag like it was gold! I hope it works! 

The IV lasted exactly one hour. After that, I waited for another 30 minutes to see if I had any reactions to it - mostly because this was my first time and we didn’t know. We scheduled three more appointments after this, as they like to schedule in advance to keep you on the one-month schedule that this drug is given by. 

During the IV, I felt dizzy a couple of times, but it was hard to tell what from. I do get dizzy occasionally, pretty much daily, and this felt no different than my usual dizzy spells. I also had this taste and smell of chlorine in my mouth and nose which was bizarre but it didn’t last too long. I got light-headed a few times, also and this was new, but not unmanageable. 

I came home right after it and I am taking the day off to just take it easy. So far, I have not felt anything unusual except for some light-headedness, or rather heavy-headedness as I feel like my head is too heavy and swimmy ... I also feel random tingling in my lips and palms, but it kind of comes and goes. No hives (they asked me to look for this), no swelling of the mouth or tongue. I also have a strange mild headache at the back of my head. This is unusual (my headaches are usually in my forehead and temples), and I think it’s what gives me the heavy-headedness, if you will. But, again, it’s totally manageable. The side-effects of a Covid shot are 1000 times worse than this! 

My husband came with me today because we didn't know what to expect from this drug. But I am sure that going forward, I will just go in by myself, barring any new developments in my overall health.

The infusion nurse said they will repeat the blood tests (for cholesterol levels) at every appointment. I asked if the liver function will also be monitored and she said she doesn’t believe so, but to call the doctor and ask that they add it. I know that it takes a full course of 6 infusions (6 months) before we see the full effect of Evkeeza, but the first rung on that ladder is now behind me after today. 

During the whole day, I have been hearing my late dad’s voice in my head. When I was 8 and they discovered that I had “a very severe form of familial hypercholesterolemia” (my total cholesterol was 734 mg/dl), I remember him saying: “one day, far, far away into the future, when you’ll probably be old or even after you’re gone, they might discover some kind of a miracle drug that might cure what you have. But it won’t be for a while. And it might be too late for you.”

When I was 8, they also told me I will not make it past my 25th birthday if I was lucky. I have been looking for this drug for only 3 years. I have been looking for an efficient treatment that will stop the progression of this disease for 41. 

Today is a huge milestone for me, in my journey, to add to the string of all the other milestones throughout my life: the first day that they discovered that I had familial hypercholesterolemia (when I was 8), the first day I took my first statin, or the day I learned without a shred of doubt that I have homozygous FH (I was 41), or the day of my open-heart surgery (I was 40). This is yet another milestone. What I hope for with all my heart is that this drug works. That it works so well that we’re done searching for more therapies. I hope that dad’s dream will be fulfilled and that, in fact, it’s not too late for me ...