Anyone take new drug Camzyos (mavacamten) for HCM?

From what I understand, Sotalol is a rhythm regulator. It is a safer alternative to Amiodarone from what I understand. I had my ablation on April 11th, and will spend 3 days in the hospital on May 30th to get “loaded” with Sotalol.
To me, this is going to be the indicator of how well things have worked. Along with that aspect, this will be the stage at which I’m most vulnerable to a defibrillation.
As far as the defibrillation itself, it is like getting kicked in the chest and head by a mule at the same time. I developed a case of PTSD after the second of five shocks. It’s been a year and a half since my last one and things have settled down. I’m just really hoping not to reawaken that demon.

Sounds like day 6 went well @starbuck41. Glad to hear you have more energy, are sleeping better, and breathing better.
Enough more energy in fact to make a late night pizza run. Stuffed crust Pepperoni pizza?
Does your doctor know about your late night pizza fest, or your double bacon cheese burger/onion rings? Just wondering 🙂 I had many occasions before my surgery, when my "dietary indiscretion" got the best of me. It would just knock me for a loop, and I slowly learned I could not eat like that if I didn't want to pay for it. I was a slow learner though 😉
Maybe Murphy needs to keep a closer eye on you, and steer you to the unprocessed, lean protein, fresh fruit and vegetable section of your local grocery store!
Seriously though, glad you are tolerating the Camzyos like a champ. Besides Murphy, do you have a support person in your life that you can share with?

Thank you for sharing that @irishpeaks. I wonder if the odds are similar to HCM, 50/50 that it is passed to another relative.
It sounds like you are doing much better after the ablation. I know that amiodarone has a lot of side effects that are not pleasant. I am not familiar with Sotalol, is it a rhythm regulator?
I cringe for you, knowing you have a defibrillator 😮
I can't imagine how frightening it must be to live with one of those. Like walking on eggshells, never knowing when it may go off. I've heard it described as feeling like a mule kicked you. I've been kicked by horses, maybe mule kicks are stronger. They talked about a defibrillator in my case too, but I dodged that thank goodness. I would not like living in fear of the thing firing off when you least expect it.
How long before you know the ablation/Sotalol worked for you?

My apologies for the delayed response.
I do have some uncles with the same diagnosis. But, not everyone of my dad’s siblings have it.
The testing was done by an independent lab that came recommended by Mayo. My brother had his transplant at the Mayo, so his was certainly seen by them, and they asked me to release my test results, so I absolutely agreed if it meant helping others.
I just had an ablation and stopped taking amiodarone. It will be substituted with Sotalol in a couple of weeks. I feel better, and have been working hard enough to sweat most of the day. All signs are pointing towards a successful procedure so far. If it doesn’t work, my ICD will defibrillate me, and there is nothing I hate more in this world than that. I’ve had it happen 5 times early on, and I’d be cool with it never happening again.

You have a lot to consider @mckenzie541! Colleen laid out some great questions for you to write down and take with you. I'm happy to hear that your new cardiologist is specialized in HOCM. That is so very important.
If you don't mind, I would add another question to your list, in addition to what Colleen already listed. I would ask if he/she would recommend genetic testing for your kids, siblings and other first degree relatives. From what I have learned, if you have HCM you have a 50/50% chance of passing it down to your children. That also means one of your parents passed it to you and potentially other siblings if you have them. Those are big percentages! The sooner you know, like you at such a young age, the better off you are. This way you will be aware of the big scary stuff, as they say knowledge is power.
Your plan so far sounds very reasonable. It's probably something that can give you time to think about things and decide later if it is something you want to do long term. I see you mentioned that you wish you could visit the Mayo Clinic in Rochester. If the plan you have chosen, Camzyos, doesn't pan out and surgery eventually becomes the option, I would encourage you once again to be sure whomever you are sent to for surgery is from a Center of Excellence (COE). This surgery is so precise, only the best of the best should be performing it. There are a handful of places in the Country that specialize in septal myectomy. You know now that Mayo Clinic is a COE, and they have amazing people working there in the patient service/ business office/insurance that can check to see if your insurance will cover the visit. I was shocked and thrilled at the same time when I found out that because Mayo was a COE, my insurance (BC/BS of Texas) approved me to be seen there. Same thing with Cedars-Saini in Beverly Hills, CA. I went to both places for opinions after being misdiagnosed for several years and once again, my local cardiologist diagnosed me with Sub aortic membrane not HCM. I chose the Mayo, even though it was a bit more difficult to travel to, because when I first met the HCM doctor (Dr. Evans) he did two things not one doctor had ever done. He ordered a chest x-ray and a pro BNP lab. Two simple routine tests that had never been done. The x-ray showed my heart was enlarged and the BNP showed I was developing heart failure. That was the moment I knew I was in the right place. I thanked God for that, even though it meant open heart surgery. Your life is worth a plane ticket to Rochester if it comes down it it!
It sounds like you have some good coping skills, and little ones to distract you from over thinking. Trust me...I know I was a huge over-thinker when I found out. I was afraid I was going to die! I know now I made the right choice once I got the right information. It just took a long time to get that information! Feel free to keep coming back for virtual support, virtual hand-holding, virtual venting! You are in the right place now. I hope you will keep the HCM group posted on your progress.

Yes you are absolutely correct..that’s why I drink the peach flavored.. but I have had a couple original Fresca.. but I didn’t think it had enough juice to matter.? …. I should have been more specific;)

Thank you Colleen!
I appreciate all your helpful questions! When I met with my cardiologist last week I asked very similar questions, he wants me to try the medicine first but said if with my age it’s too much on me taking medicine and doing the echos and I don’t want to do it the rest of my life I can opt in to surgery still. He says the pros and cons are very similar, so it’s whatever I prefer. Surgery is going to be safer at my age then if I wait and need it at 70, but still even later in life it seems to be pretty safe despite being open heart surgery. It’s all scary and I feel kind of at a loss, but I figured I’d try the medicine and then after giving it a proper go I’ll decide what I prefer long term.
That’s the hard thing with such a new medicine, they don’t have long term studies available so they weren’t able to answer that for me
Yes they have, I was originally going to be seen in the pediatric dorm because most people are usually children or older that know they have it, so I feel like the odd one out with it. But it seems to supposedly be promising to have treatment done at my age so hopefully that holds true
Thank you! I will look over those discussions as well.
My doctor is specialized now with it, I’m being seen at OHSU on the HOCM floor I just transferred! Before my cardiologist knew almost nothing haha.
Thank you for the time you’ve spent on here!

That is amazing!! Hearing stories like this is very inspiring and makes me excited. I hope I have the same effects, I’d love to feel better and have more capacity to do things. I’m so happy camzyos is working well for you and giving you your life back. Reading all these is helping me fear and giving me the motivation to try it 🙂 it seems I’ll start with the free trial next week. I get the medicine tomorrow but have to wait for my first echo to be scheduled before I can start it. It’s good they’re monitoring people so closely, that gives me some comfort also

Hello! Thank you for your response and insight. I have been looking into all options and doing a lot of research, I’m prepared for either option. My cardiologist wants me to try the medicine first, and said if I have bad effects or if I just decide it’s too much of a hassle with my age we can do surgery instead. So I think I’m going to try that route, but I’m still having them get me prepared for surgery as it’ll take around 4 months they said. So I figured in the meantime I could try the medication out at least.
Surgery is terrifying, but I do like the peace of mind of having it done and taken care of and it feeling permanent. So I’m definitely not casting out the option either!
My new cardiologist specializes in HOCM, I am currently being seen at OHSU but my previous didn’t and was local to me and I think that was a big reason I wasn’t getting the care I needed. I do wish I could be seen at the Mayo Clinic but my resources are limited.
I will see my cardiologist in one month for a check up and then 3 months for a full visit again! I do have some questions but always ask people what I should be asking because in the moment I space it all haha,
Right now I try to do “normal” activities with my kids like camping, park, beach etc without overdoing myself and that helps take my stress away, otherwise now joining these forums is a step for me and I may see a psychologist if it gets bad. I hope with treatment it’ll get better.
Thank you for all your guys responses and helpful comments!
I’m glad you got treatment even if it wasn’t the medication, it seems the surgery is very successful and a great option! I hope you are doing well now.

Hello Dave, I’ve been reading I can’t have Fresca with camzyos because of the bad interactions with grapefruit. Has anyone talked to you about the potential effects? I got a whole list of things that contain grapefruit and told I cannot have them at all 🥲
Im excited to see how the medicine works for you. I’m hoping to have good effects too. I’m the same way if I eat much my condition comes out really bad, I’m glad you’re feeling better already!