Anyone take new drug Camzyos (mavacamten) for HCM?

Thank you for your kindness. She is still on the drug. It has reduced her gradient to 0 at times, yet the gradient keeps going up and down. It’s all over the place. She has echos monthly. She got diagnosed 5 years ago after a pregnancy and something happened after year 4 where the gradient was at 100. Today, it is at 80. Last month it was at 4. In other words, it hasn’t held at 0. Yet, her left ventricular has remained think and so even when the LVOT was at 0, she still felt no relief. Her entire body aches, but she just came out of the hospital today. Her potassium was at 1.9. We don’t know if ivs of potassium will reduce the pain. For my daughter, who has been on camzyous for 8 months, there has not been an improvement. Her cardiologist has a patient who is currently doing better. It may be a journey each person has to decide to take. There is a small feeling that we wasted time and her condition has worsened and maybe the septal myectomy should have been done months ago. We will never know and of course, I do not know about her results with the surgery. She is being measured next week to see if she qualifies. Her thickness may be too deep to qualify for surgery, then that is a whole other terrifying topic.
I hope your journey goes well. Thank you.

Your daughter and you will be in my prayers. My heart aches for you both. I see you are a spiritual person. So, try to maintain your faith. I know that is easier said than done right now.

Has your daughter stopped taking Camzyos? I don’t know what her condition was prior to taking the drug; but, I hope it did not make her worse.

Try to get her the very best medical treatment possible. I too live in a major metropolitan city. But, if I do have to have surgery I intend to go to a Center of Excellence, a place where they do hundreds of the type of surgery that is needed not a dozen a year.

I have not been part of this support group very long. But it has given me a lot of comfort. I am still afraid of Camzyos. But, I decided I will try it first before pursuing surgery. I encourage you to use the group to ask questions. It helps.

I will. Thanks for your wonderful support!

Oh gosh... she really has a lot going, what a terrible time she's having. And you too. It sounds like you will have a better sense of what's going on next week. It must be so difficult to see her like this. I will be praying for you and her. I know He hears our prayers. Will you give your daughter a hug from me? And tell her I am praying?

Day 9
5mg @ 0756 am
Weight 267
Breakfast 10oz coffee
16oz sugar free monster energy drink
1 slice of pizza

Lunch a Diet Coke
Some more pizza
500mg metformin
10/325 oxycodone
My back was really hurting this morning I was up late doing laundry and folding clothes and cleaning floors.,

Last night I intended to have baked chicken but .. I had pizza and Murphy had the chicken lol. No side effects, the 1 episode of dizziness seems to have abated itself. I was up early today. Dinner tonight will unfortunately have to be a healthy salad and a Diet Coke with 24oz of water.
Until tomorrow unless something changes..
See y’all then
Dave
For those that don’t know.. I’ve posted a pic of Murphy:)

Imaging is next week. Surgery may be the following week. She is sick of her life. In January she went in for a heart catherization and ended up with septic shock. They are also talking heart transplant. Very scary stuff. I have put it into the Lord’s hands because that’s all I can do, other than babysit every day!

You are in a good place then! Thank goodness for that. She's so young and having a special needs child, being single, having this awful condition...her stress level must be so high! And yours too. It is hard not to fear the septal myectomy. When I learned I needed to have it done I was stunned. Shocked. In denial. No way not me! Knowing I was in the best hands in the world, having the Lord, and trusting Him, I can say yes, yes it is very scary, and the fear is real. I got my life back and that was worth the fear, the pain, the 9" scar and two 'stab wounds'.
I think having faith in her doctor, trusting the medical team, and having a positive attitude will be so valuable.
Thank you for asking. Yes I am so much better. I no longer gasp for air, have chest pain, burning pain in my neck, dizziness, head rushes etc. I still have the disease, so there will be life long symptoms, but the obstruction is gone. That is the positive take away from the septal myectomy. It is permanent and I am not on any medications except a 81mg aspirin and vitamins and minerals. I walk every day. I can tie my shoes now without getting short of breath. I do not have disabling fatigue. Feel free to reach out with any questions about the myectomy if you want...if I can help one person by putting their mind at ease, I feel good. It's not fun, but it is not as horrible as I thought it would be. Pain, sure. Any surgery has pain. But if your daughter is healthy prior to surgery, she will do much better and her age is in her favor. She's got her mom, thank goodness. Aside from that what is she doing to cope with the stress?

Thank you. I am at Texas Heart Institute. I think the doctors here have wonderful reputations. Her diagnosis started at the Mayo Clinic in Phoenix. It is a wonderful facility. She is receiving weekly meetings with her cardiologist who also calls her multiple times per week. I am terrified of the septal myectomy as she is divorced with a 5 year old son with autism who needs his mom. I have no rights as a grandma if anything goes wrong. Do you feel healthier now?

Oh my goodness @dbrima, my heart goes out to you. Thank you for sharing your story here on Connect. I suppose there is always a downside to trying new drugs...and this sounds like it started out with good results but now the bad outweighs the good. There certainly are no long term studies on a drug so new, and it sounds like your precious daughter is not a candidate any longer. I'm relieved to hear your daughter has a doctor experienced in HOCM. There truly aren't many, so that is a blessing.
I had a septal and papillary myectomy a little over two years ago at the Mayo in Rochester. Naturally, anyone would have fear of undergoing a major, major surgery. I can tell you that the care I received at Mayo was world class and the doctors, nurses, lab techs, dietary aids, housekeeping, etc are the reason I did so well. You mention you live in a major city, do you know if the hospital you are going to is a Center of Excellence (COE)? That is super important as the septal myectomy should only be performed by the top surgeons at a COE. There are only a few places in America that are considered COE for HOCM surgery, naturally, Mayo is one.
I am so sad for you, I can tell this is terribly hard on you too. Watching your child suffer so much. You came to the right place for information. Please feel free to reach out anytime with any questions. When do you believe they plan to schedule surgery? Do you know if they need to let her heart get stronger before going through it? What does your daughter think about all this?

My adult daughter started Camzyos 8 months ago. Her gradient was 100 and now it’s 0. However, it has exhausted her, she never has enough to drink, her potassium level is at 1.9. She is always needing diuretics. She is in excruciating pain from the lack of potassium. It has relaxed her heart so much that she has no quality of life and is starting imaging next week for a septal myectomy. There is too much water to drink, so many supplements, her eyes are hollow (she was beautiful). This may not happen to everyone, but that’s been our experience with a top cardiologist in one of the largest cities in America.