end stages

[Daisy]

I would like to know, What are the end stages of HCM? A person can be stable for years and then all of a sudden it changes or is it a gradual process?

[Sarah]

Re: end stages

Dear Daisy,

I think that it is typically gradual for most people, however, HCM is really different for everyone and it is usually impossible to say "this happens like this" for most of it.

Sorry this isn't really an answer. Perhaps someone with end stage will chime in.

S

[mjesaitis]

Re: end stages

Daisy,

I am 39 y/o and in end-stage HCM. For me it was pretty gradual over the last 10 years, but for the past year it has been more of a downward spiral. For the previous years, my EF has been pretty steady at about 30-40%, but in August it dropped to 20%. In March, I was placed on the transplant list. There were a lot of things I probably should have or shouldn't have been doing, but because of my lack of knowledge on the disease, I didn't know better. But please remember that every single person is different. I thank God I found the HCMA a year ago.

Hope this helps. Matt

[mjesaitis]

Re: end stages

To answer your question better...

End-stage HCM for me was going into chronic A-Fib, then about 5 years later going into chronic CHF, then 3-4 years after that my EF dropping way too low. Over this 10 year period, my symptoms of shortness of breath, tiredness, dizzyness, sleeplessness, headaches, joint pain, water retention, have slowly been getting worse.

Matt

[Midge Rollins]

Re: end stages

Daisy, as Matt says, end stage is usually gradual. I had my myectomy 22 years ago. Now at the age of 57 I am on the transplant list. Having gotten worse mainly over the last 2 years. I have less exercise tolerance, CHF, shortness of breath. You did not mention if you are in the end stage. I assume you must be or you would not be asking. Feel free to email me and I can answer more specifically.

[Lisa Salberg]

Re: end stages

For most people it is very gradual. I do know of a few people who did turn rather quickly (several months to one year). End stage is a really BAD name and I would like to change the name as it is misleading. Some docs call it "burnt out" - not to thrilled with that one either but I think it is a little better.
"End Stage" can last for 10-15 even 20 years in some people. So please do not hear the word and think...OK I give up!
Transplant is an option and there are also many many meds now available that can be of great help.
My dad is in "End Stage" and honesly 2 years ago it was not looking good. BUT here we are 2 years later and he is doing very well. In fact his EF went up nearly 20 points after managing his meds better.

Be well,
Lisa

[pickaback]

Re: end stages

What does EF stand for?

[Karen P]

Re: end stages

EF stands for ejection fraction - which is the measurement of the amount of blood ejected from the heart into the body in a single beat. I believe the norm is anything above 50/55............please someone correct if I am wrong.

[pickaback]

Re: end stages

Is EF the same as the lower number on a blood pressure reading? Thanks again.
pickaback

[Sarah]

Re: end stages

Hi,

No. EF is a number that you will find on an echo report.

* Blood pressure is the force of blood against the walls of the artery.

* Systolic pressure is the highest pressure in an artery when your heart is pumping blood to your body.
* Diastolic pressure is the lowest pressure in an artery when your heart is at rest.
* Blood pressure measurement is made up of both the systolic and the diastolic pressure. It is normally written like this: 120/80, with the systolic number first.

Your bp is measuring the pressure in your artery where as the EF is measuring the percentage of blood leaving the ventricle.

S

[kthanki]

Re: end stages

I would like to open this topic again...
Some clarification will be helpful please for new questions:

1) Is end stage inevitable for every HCMer?

2) Does this stage apply to both obstructive and non-obstructive HCM?

3) What kind of medications are used for "end stage" or "burn out" condition?

4) What are the indications, just the symptom of SOB or change in EF?

5) For non-obstructive people seems like options are very few to nothing since A.abl or myectomy is not applicable. What are the course of actions as the condition progresses?

Info please..???

[Lisa Salberg]

Re: end stages

1) Is end stage inevitable for every HCMer?
Answer: NO it is in fact rare and only happens in about 10% of cases.

2) Does this stage apply to both obstructive and non-obstructive HCM?
Answer: Yes it appears so.

3) What kind of medications are used for "end stage" or "burn out" condition?
Answer: Funny thing is that the drugs are the drugs we avoid in HCM - Ace, diruetics, and other drugs that help the heart beat harder.

4) What are the indications, just the symptom of SOB or change in EF?
Answer: The EF drops as the walls thin - SOB and fatigue are big symptoms fluid retention it a big one too.

5) For non-obstructive people seems like options are very few to nothing since A.abl or myectomy is not applicable. What are the course of actions as the condition progresses?
Answer: Medication changes, pacemakers and ICD's in some rare cases transplant (about 2-3% of cases are transplanted)

Hope this helps.
Lisa

[Midge Rollins]

Re: end stages

Lisa, thanks for the information. I was wondering what percent of us get to my stage and now I know. Thank God it is not a higher percentage.

[Tommy Mac]

Re: end stages

There were a lot of things I probably should have or shouldn't have been doing, but because of my lack of knowledge on the disease, I didn't know better.

What are some of those things? Sure, some of them may be obvious but maybe you'll have some input for those of us still trying to do the right thing.

Thanks so much and good luck to you...

[Dolly W]

Re: What About Dobutrex Treatments?

I was told that sometimes in end stage HCM, dobutrex infusions are used to help the heart beat more efficiently. Lisa, have you heard of this? Can you explain?

Thanks.