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I went to Boston!

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Dave Johnson Find out more about Dave Johnson
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  • I went to Boston!

    Here's the e-mail (in part) that I sent to my friends and family after going. I'd like to get everyone's feedback, particularly about the conclusions I've drawn from my appointment.

    Let me tell you guys - you've GOT to go to an HCM clinic if you haven't yet. Dr. Maron stayed in the room the whole time I was having my echo - and the techincian actually KNEW what HCM was! Dr. Uddelson and Dr. Maron sat in the room with us for about an hour answering every question we had - it was unbelievable. I have a new lease on life - it's amazing what correct information can do. If you haven't gone - GO!!!!

    Here's the e-mail -

    Hello all,
    I'll warn you this e-mail is going to be LONG, so feel free to skim or delete if you don't want the details.

    As many of you know, Christie and I travelled to Tuft's New England Medical Clinic in Boston early this week so that I could be evaluated at an 'HCM' clinic. Let me back up just a little for those who might be unaware of what's been going on, and then I'll tell you what we found out.

    THE TECHNICAL/MEDICAL STUFF:
    Almost 5 years ago I was diagnosed with HCM or 'Hypertrophic Ventricular Cardiomyopathy' (say it five times fast). I was having heart-attack-like symptoms (shortness of breath, chest pain, numbness in the extremities), as well as debilitating fatigue.
    In my case, absence of obstruction means the muscle is still thick (though not horribly thick), but the condition cannot be 'cured' or even bettered by burning of the muscle (septal ethanol oblation), cutting (septal myectomy), or the installment of a pacemaker. We thought for sometime that I would be getting a pacemaker or defibrilator (ICD), but we've since learned that there's very little chance this would help at all.
    That means that the only course for me right now is drug treatment, and possibly a transplant. It's very important for everyone to understand however that drug treatment does not mean treatment of the disease in any way - the drugs simply work to relieve the symptoms caused by HCM (shortness of breath, chest pain, etc.) so that that patient can attempt to lead a somewhat productive and 'normal' life. The drugs do not reverse or 'shrink' the muscle in any way -they only treat the symptoms. I want to be clear about this since many people often ask if I'm 'getting better' - with non-obstructive HCM there really is no such thing in terms of treating the actually disease. It might be more accurate to ask 'are you feeling better?' - which is where our trip to Boston comes in.

    WHAT WE'VE BEEN DOING
    My doctor in Little Rock has been very aggressive and gutsy in his use of drugs to treat my HCM symptoms, and many of you know that I went through a long period of time where meds were being switched around and increased/decreased as we attempted, by trial and error, to find a combination that worked. Since this was a nightmarish time for me (daily vomitting, migraine headaches, constant nausea, fatigue, dizziness, irritability, etc.), we were all happy when we finally struck on a combination that seemed to vastly improve my quality of life by masking and reducing symptoms. So for several years I've been on a combo of three different meds, which has made it possible for me to function without being constantly short of breath or suffering chest pains.
    The downside to these meds (which btw are 'lopressers' 'calcuim channel blockers' 'and 'beta blockers') is that by nature they decrease blood pressure. My blood pressure was low to begin with, so as you can imagine, the side-effects of these meds have been difficult to deal with. I fight with low-blood pressure, nausea, slight headaches, and being *cold* most days (even in the summer!). Over time, I have developed hypo-glaucemic-like problems with blood sugar and food (small portions many times a day). The worst part of these side-effects is that when I've got a cold, or I over-exert myself, or don't get enough sleep, I develop short-term memory loss, and can become easily disoriented, not to mention extremely irritable. Probably the biggest daily obstacle is that I'm distracted very easily and have trouble focusing. This has probably been my largest obstacle, since my life's work is to study and teach.

    WHY NOW?
    Over the last year, these increasing side-effects of the medication have become more difficult to live with (especially with an active, growing, 19 month old son running around!), but we've tried to work our lives around them as best we could. There has also been a rising concern about the HCM symptoms slowly overcoming the resistance of the meds. About 6 months ago when I mentioned this to my local cardiologist, he indicated that I was taking such high doses of the medications, that he would fear increasing the mg's in the event that the HCM symptoms returned with force. Most people on the these doses are virtually bed-ridden -so I've really pushing the limits of what these drugs should be able to accomplish in day-to-day life (HOO-AAHH!!!). Still, this left us debating about evaluation for heart transplant, and many quality vs. quantity of life issues.

    SIDENOTE ABOUT TRANSPLANTS
    Don't believe everything you see in movies and on TV about heart transplants. Hollywood has done a great job (as usual) of obscuring the facts about the procedure. Transplant procedures are 100% successful right now. 90% of all transplant recipients live past the first year. 75-80% live more than 5 years. After that, the information gets a little vague because this is a relatively new procedure. There are stories of some people living for 20-30 years with a transplanted heart, but it seems the more realistic average is 7-10 years. At that point, an individual can receive a 2nd transplant (and possibly even a third) *if they are a good candidate for the organ.* This, again, is very important to understand. Transplants come with very heavy baggage in the form of anti-rejection drugs (or 'immuno-suppressants) to keep the body from rejecting the invading organ. The effects of this battle within the body can be more debilitating than HCM, and the drugs often cause damage to other vital organs, and dissolve bone structure in the body, making the individual extremely in-eligible for a 2nd or 3rd transplant. Immuno-suppressant drugs can also be financially crippling since many times they cost thousands of dollars per month and most insurance (especially Medicare) offers little or no coverage (score another point for our wonderful insurance industry...). Heart transplants are wonderful if you have no other option, but they are very definitely the *last* option on my list. With HCM, life-span is unpredictable, with a transplant, it is most assuredly 7-10 years except in extremely rare cases. I'll take my odds with HCM. Since I'm 32 - a heart transplant would give mean I would live to be 42 at best, longer if extremely lucky. This would be about the time Richard is 12. You can see why a transplant is not a very attractive option until I'm absolutely bedridden and unable to function.

    WHY BOSTON?
    As you can see, Christie and I were not ready to begin down the road to a heart transplant. By way of a friend and fellow HCMer, Mindi Barger, we were able to find out about an online association called the HCMA - a patient advocate group who led us to several different clinics in the U.S. that deal specifically with HCM patients. We had NO idea that such clinics existed (score one more for the Web!). Obviously we wanted to go and have me evaluated at one of these - we picked Tuft's New England at the advice of some online friends, but also because Dr. Marty Maron is on staff there - his father Dr. Barry Maron practically wrote the book on HCM (which we will make available to anyone who's interested shortly). The timing was great considering we'd hit a wall here locally - with loving apologies to my atheist/agnostic/wiccan friends (you know who you are!) I can't help but believe that it was God's providence that brought all of this to a head at this time (coincidence isn't a kosher word!).
    So we travelled to Boston this week and spent all day Tuesday at the clinic - held once a month for HCMers. All I can say is WOW!

    WHAT WE LEARNED
    Much important stuff.
    1) I am not at risk for sudden death, based on family history, monitor readings, and a variety of other factors. So from now on when you tell me to drop dead - no can do. What a relief to us - this alleviated a lot of fears for us about day to day living.
    2) No one can predict the progression of HCM. Theoretically, I could go downhill tomorrow and need a transplant in the next few months. Or I could progressively get worse - either the thickening or just the symptoms. OR I could stay exactly the same. You might think that's not a great bit of information - but it's huge to us, since we were under the impression that the only way to go was down. We were told that there are people who have onset in their mid 20's, find the right meds, and live to be 75 years old. So it's very possible (and hopeful) that I won't progress any further. As you can see, there are still a lot of mysteries about HCM.
    3) I can feel better - they docs made some recommendations about re-arranging meds. In particular, they'd like to see me weaned from the lopressor called Atenolol and increase another called Verapamil. This means I would be on 2 meds rather than 3 (which hopefully means 1/3 of the side-effects). I've often referred to Atenolol as 'the demon' because it has been the most difficult to maintain and deal with in terms of side-effects. I actually take 150 mg's of this per day, but I had to start with 1/2 of a 25mg pill per day and increase by increments of 25 over an 8 month period because the side-effects were so brutal. Needless to say, I won't shed any tears to watch the Atenolol go down the disposal...
    4) This is the biggie - we thought that any time I had chest pains, palpitations, or 'episodes' that it was a tell tale sign of the disease progressing (in other words-exertion=thickening=progression= transplant or sudden death). We were told that this was absolutely NOT true. Pains, shortness of breath, pounding, and the like are *only symptoms* of the disease - not a warning sign of impending death!
    This is important for day to day life. I've restricted myself from a host of activities that caused these symptoms because I thought I was literally taking months or possibly years off of my life. I've stopped playing drums, I'm reluctant to run through the house with Richard, climb stairs, etc. - there are many places I can't go because the incline or walking causes chest pains and shortness of breath. As you can imagine, this has left Christie doing all the shopping, all the housework, all the childcare, etc. and has limited me in countless ways over the last few years.
    The docs told me I still need to take it easy, so I don't intend to start running marathons tomorrow, BUT - my decision to engage in these activities can now be based on a temporary consequence, rather than a permanent (and possibly fatal) one.
    In other words, if I want to run through the backyard with Richard, I have to decide whether it's worth the chest pain (it is) and the shortness of breath (it is) and the fatigue (it is) and possibly collapsing (it is) that would result -rather than deciding whether it's worth taking a year off of my life. If I want to do something cardiovascular, I can do so (within limits) and just choose to suffer the temporary consequence, but it's not going to do permanent heart damage.
    I'm going to buy a six pack of CAFFIENATED Mountain Dew TODAY. Out of the wilderness indeed...
    5) Not so good news - regardless of which parent has HCM there is a 50/50 possibility that the children will have HCM. Richard can be tested at 2 and if it's positive that he has it, it should show up on EKG. It's also possible the EKG will look fine, but he should be tested every 2 years - particularly when he becomes a teenager since HCM can be a little sneaky in younger people. Keep in mind that even if he has it, he may not require meds at all. Obviously we're praying that he doesn't have it!
    6) They gave me the go ahead to take (in moderation) such medications as Ibuprofen, Sudafed, etc. - a major help since every time I've had a sinus cold or headache I basically have to outlast it since we thought most anti-biotics and other drugs would interfere with the HCM meds.
    7) There is ABSOLUTELY NOTHING I can do to either slow or speed up the progression of the disease (it may not ever progress beyond where it is now, it may progress significantly tomorrow). Even more or less thickening may or may not cause 'progression' in the technical sense. Progression is determined by cell structure, which is undetectable. Doctors judge the need for more meds or a transplant by increase in symptoms.

    WHAT NOW?
    Following Passover week, I plan to begin the changes in the medications. It could take some time for my body to adjust, which means we may have to go through the vomitting, headaches, etc. again - but probably not to the same extent as I did before. This means I may be worse before I'm better - so don't worry if I seem more sick in the coming months (remember - the meds have been 'covering' the symptoms, so symptoms are more visible and noticeable when the meds are being re-arranged).
    It's also entirely possible this med switch won't be effective, but I've responded well to these particular drugs already, so switching the doses probably won't be as tough as starting from scratch, and the Boston docs expect this to improve quality of life.

    Now let's get on with life!
    peace
    Dave
    "We come into the world and take our chances, fate is just the weight of circumstances - that's the way the lady luck dances...roll the bones." - Rush

  • #2
    Re: I went to Boston!

    Dear Dave,

    I'm so glad that your appointment went so well. I know it is such a relief to have some solid answers to your biggest questions. Your email was very informative and I'm sure everyone is glad to hear from you. Just a little pointer about Richard being tested. I think you meant that if he has HCM it will show on an echo, not an EKG. Not everyone has funky EKG's. but yes, they will be able to see on echo and yes, he should be tested every couple of years until puberty. Richard aside, I'm glad you're doing so well. Hopefully the changes in the meds will make you feel even better. Now go run through the backyard a time or two. You deserve it!

    Reenie
    Reenie

    ****************
    Husband has HCM.
    3 kids - ages 23, 21, & 19. All presently clear of HCM.

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    • #3
      Re: I went to Boston!

      Pretty awesome Dave.

      I am also a patient of Dr. Maron and speak with him on the phone once a week. He never seems to tire. He always is available to talk and returns my call within minutes. He is probably the single most dedicated physician I have ever had the pleasure (or displeasure) of caring for me. You are in excellent hands. Feels good to have them on your team huh?

      Doug
      NEMC's (Boston) First Myectomy 7-22-2003

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      • #4
        Re: I went to Boston!

        I can't say enough good things about Dr. Maron. To actually feel like you're the only patient in the place was incredible. He's such a nice guy and very compassionate.
        "We come into the world and take our chances, fate is just the weight of circumstances - that's the way the lady luck dances...roll the bones." - Rush

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        • #5
          Re: I went to Boston!

          Dave---Great post! I learned a lot. You may like Verapamil. My first try at the right dose was too high and my ankles became swollen but, other than that, I have had no side effects. Hope it works as well for you. Sue

          Comment


          • #6
            Re: I went to Boston!

            Dave, thanks taking the time to post for us. I'm so glad you had such a positive visit. Hope the med changes go smoothly and the benefits are many - Linda

            Comment


            • #7
              Re: I went to Boston!

              Dave,
              WOW what a wonderful story. You have provides such a great outline for those to follow "the real story of living with HCM".
              I can not tell you how happy I am that you had a good visit and that you are on the road to having a better quality of life!
              I am sorry it took me a while to respond I have been having internet problems (and for the record that is life "withdrawl" for me!)
              Your story will provide encouragement to so many. Thank you for sharing it with us.

              Peace and good health to you!
              Lisa
              Knowledge is power ... Stay informed!
              YOU can make a difference - all you have to do is try!

              Dx age 12 current age 46 and counting!
              lost: 5 family members to HCM (SCD, Stroke, CHF)
              Others diagnosed living with HCM (or gene +) include - daughter, niece, nephew, cousin, sister and many many friends!
              Therapy - ICD (implanted 97, 01, 04 and 11, medication
              Currently not obstructed
              Complications - unnecessary pacemaker and stroke (unrelated to each other)

              Comment


              • #8
                Re: I went to Boston!

                dave,

                i'm new here but want to add my happiness at your good news and (hopeful) continued improvement in quality of life. i am 33 and only recently diagnosed--i also have no obstruction but as of yet i am lucky to have no symptoms or limitations to speak of. nonetheless, i am aware that this is a very variable condition and something that is true today may not be so tomorrow. as such i am also considering visiting a true hcm specialist soon. my question for you is insurance based: did you end up paying for the consultancy with dr. maron out of your own pocket? or did your insurance cover some portion of it? either way, can you (or anyone else with experience) give me a sense of how expensive it would be to see someone like dr. maron for a consultation if one was paying for most or all of it?

                thanks!

                mongo

                Comment


                • #9
                  Re: I went to Boston!

                  Dear Mongo,

                  If you have insurance, you will need to talk to them or read your policy. Many will pay for out-of-network doctors if you follow the correct procedures (whatever theirs are) for the referral, etc.

                  take care,

                  S

                  Comment


                  • #10
                    Re: I went to Boston!

                    yes, sarah--i think mine pays 50% for out of network doctors. i'm just wondering what the 50% i would have to pay for would likely come to with the major hcm specialists (especially since the out of network people likely won't have the negotiated rates).

                    Comment


                    • #11
                      Re: I went to Boston!

                      Hi,

                      Well, pricing is complicated. First you have the doctor's fee, then the tests, and then the travel costs. Travel costs are tax deductible, but they have low caps. See irs.gov for specifics.

                      To really know what costs to expect, call the business office of the center and they will tell you everything you want to know. They will also be familiar with your insurance company (most likely) and can give you an idea about how they handle things.

                      take care,

                      S

                      Comment


                      • #12
                        Re: I went to Boston!

                        hi dave,

                        me again--this time with a response to one of your points. sarah (i think) corrected something you said saying that if richard has it it will show up on an echo not an ekg. i haven't gotten any specialist advice on this but based on my reading my understanding is that unless hcm manifests itself spectacularly in childhood thickening does not usually show up on echoes till puberty. ekg's, on the other hand, may sometimes catch irregularities before this time. so, yeah i think you're right. then again, i could be wrong. could someone who's actually undergone genetic counselling on this score clarify?

                        cheers!

                        mongo

                        Comment


                        • #13
                          Re: I went to Boston!

                          Yes, Mongo, I believe you are correct. HCM can present later in life...its likely to present at adolescence, but if you don't have it then, you are still not in the clear.....

                          I had my son tested in utero, at 15 mos., and will keep retesting every few years until adolescence, when I will do it every year.
                          Daughter of Father with HCM
                          Diagnosed with HCM 1999.
                          Full term pregnancy - Son born 11/01
                          ICD implanted 2/03; generator replaced 2/2005 and 2/2012
                          Myectomy 8/11/06 - Joe Dearani - Mayo Clinic.

                          Comment


                          • #14
                            Re: I went to Boston!

                            Dave,

                            To take it one step further, my son is living proof that echos do not always pick up HCM even during adolescence. Cardiac MRI was the diagnostic tool for him as his echo was completely normal (that's what we were told anyway). So, you see it is even more complex than people think. I would ask for an MRI and not take any chances if and when the time comes.

                            Best of luck to you, and I'm glad to hear Boston was able to make you feel better. They're good at that.

                            Comment


                            • #15
                              Re: I went to Boston!

                              Hi,

                              Well, I need to point out that 99% of HCM is found by echo. And Bert --if I remember correctly, your son was diagnosed by echo correctly and it was only a different echo by someone else that was inconclusive (right?). So technically, it _was_ found by echo.

                              Unless the price comes down exponentially, MRIs are not going to be used for diagnosis for a long time.

                              ECGs of HCM patients may show changes, or not. This one is a bit of a grab bag.

                              An ECG AND an echo are the two tests that need to be done.

                              S

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