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Subject :Love to hear from heart transplant patients..
2007-03-22 05:22:56
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| kgoodfellow |
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| All Star |
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Joined: 2007-02-20 17:21:41
Posts: 679
Location: Denver |
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Hey
I have BMD caused heart problems, that will probably result in a heart transplant somewhere down the road. Hopefully it isn't anytime soon.
I would love to hear first hand experiences with BMD heart issues, the road to the transplant, and of course how life has changed after the transplant.
I haven't really started studying the whole process, but I would love to hear from those that know best.
Kevin |
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K. Goodfellow |
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Subject :..
2007-04-10 07:06:30
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| Rhonda Bliss |
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Joined: 2007-02-27 08:21:26
Posts: 35
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Hi Kevin,
My son Aaron age 8 was diagnosed with a dystrophinopathy- Calling it Becker because he has many of the symptoms (enlarged calf muscles, pain and muscle fatigue upon exercise, exercise intolerance etc.) Our biopsy, Western Blot and MRI were normal however so it is speculated that Aarons form will manifest in his heart muscle. I am a confirmed carrier as is my mother, and we believe that her uncle was affected as he died very suddenly at 41 of heart attack. I was told that Aaron may also need a transplant at some point.
What is your deletion.
Our is Exon 48 which is in the area that has been related to heart problems.
Thanks
Rhonda |
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Rhonda
Mom to Aaron-(exon 48 deletion) age 10 Dirtbike rider & video game whiz! |
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Subject :..
2007-04-11 00:20:21
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| kgoodfellow |
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| All Star |
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Joined: 2007-02-20 17:21:41
Posts: 679
Location: Denver |
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Hey
My deletion has been stated as 40 & 41, but there is going to be a repeat of the testing by Kevin Flanigan in Sept to insure that the whole gene has been sequenced properly. Like your son, most of my tests are fairly normal and the fact that I was completely normal until I was 30 shows that somewhere I am getting back in frame. They suspect I have another deletion of 1+ further downstream that is causing me to produce fairly normal dystrophin, but just a bit less of it.
Anyhow, it is affecting my heart more seriously than my muscles. I only started having any significant symptoms with my muscles when I started taking the meds for my heart.
Has your son had a repeat genetic test to confirm the entire gene? He seems to have a similar situation. Kevin Flanigan at the University of Utah is the guy to see for it. I have his email if you're interested. What type of cardiac care is your son getting?
Kevin |
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K. Goodfellow |
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Subject :..
2007-04-11 00:51:42
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| Rhonda Bliss |
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Joined: 2007-02-27 08:21:26
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Hi Kevin,
We stumbled onto an elevated cpk completely on accident. My son had been struggling to recover from mono, and to date there is still nothing going on that would have made us think MD, if we hadn't found the CPK. He does struggle with cramps and fatigue- always has- but walked and talked on time, rode a bike without training wheels at 2.5 years old. People always commented on how muscular he is both arms and legs. We spent months here in NH trying to figure out what was causing the liver and ck counts to be elevated- nobody thought it was DBMD, finally Dr Brenda Wong figured it all out for us. Dr Linda Cripe did a full heart eval and right now everything is normal. Dr. KEvin Flanagan has repeated our testing and Aaron is a participant in the Utah study- which confirmed our exon 48 deletion. It has been speculated that although there is a disruption to the dystrophin gene it seems to not be in an area not necessary for binding together the protein for the skeletal muscles(although there are cases of DMD with an exon 48 deletion). There have been 5 other reported cases of an exon 48 deletion and no weakness, hence they beleive it is not a exon critical for the protein so long as it keep the reading frame intact.
From this point moving forward we will be working with Dr. Steven Colan at Boston Childrens- he is known frm his work with DBMD related cardiomyopathy- we have not met him yet.
I am very happy this site it here.
Thanks for all the work you do for it.
Rhonda |
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Rhonda
Mom to Aaron-(exon 48 deletion) age 10 Dirtbike rider & video game whiz! |
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Subject :..
2007-04-11 10:47:36
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| kgoodfellow |
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| All Star |
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Joined: 2007-02-20 17:21:41
Posts: 679
Location: Denver |
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Hey Rhonda
Funny, I had elevated SGOT enzyme levels since high school, about 2x normal, but nobody ever figured out why. I even had two liver biopsies when I was younger, thinking it was a liver problem (that was 20 years ago). Nobody ever tested my CK. I'm not sure what it would have shown then...even now it's only 1500. I was doing normal things, so I bet it would have been only slightly above normal.
Have your son's doctors talked to you about Diovan or Losartan? They are both ACE inhibitors and recent MD mouse studies show that it helps alleviate some of the muscle damage. With genetic issues, there are usually more than a single problem causing muscle damage, and therefore soreness etc. This study showed that in mice these drugs reduced one of these causes.
Before this study came out, I had been telling my doctor that Diovan has been the only drug that makes me feel almost normal in terms of muscle strength and lack of soreness. These drugs are mostly prescribed to healthy people with high blood pressure (they lower blood pressure) and to people with heart damage (to prevent further damage).
I know a BMD patient that have been prescribed them as a preventive measure for many years, since they have few side effects and some potential benefits. He hasn't developed any serious heart issues and is the same age as me. It might be worth discussing with your son's cardiologist/doctor. My personal experience with Diovan has been very positive. I'm thinking about trying out Losartan at some point to see if it works any better/worse.
Check out the diovan thread for more details. I have a copy of the study if you're interested.
Kevin |
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K. Goodfellow |
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Subject :..
2007-04-12 05:50:56
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| Rhonda Bliss |
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Joined: 2007-02-27 08:21:26
Posts: 35
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Hi Kevin,
The elevated SGOT is appearently always present in DBMD during the younger years. I was told many healthy livers get biopsied becasue of it.
My son is not on meds now, but I have spoken to a woman whose 12 year old son was put on Losartan as a precaution- They also see Dr. Colan- Aaron is 8 so they may want to wait a few years, They told her they like to get the boys on it as they approach puberty as boys have pretty erradic hormonal changes then (There's a news flash ha-ha!)
Can I ask how old you are now?
Are men sensative about that question?
Thanks
Rhonda |
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Rhonda
Mom to Aaron-(exon 48 deletion) age 10 Dirtbike rider & video game whiz! |
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Subject :..
2007-04-12 07:41:14
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| kgoodfellow |
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| All Star |
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Joined: 2007-02-20 17:21:41
Posts: 679
Location: Denver |
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Hey Rhona
Interesting stuff.
I'm 37 and was diagnosed with BMD just last year when doctors detected an abnormal heart rhythm when I had my appendix removed. I had 3 opinions from the best heart hospitals in the US until Mayo Clinic figured it out.
Kevin |
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K. Goodfellow |
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Subject :..
2007-04-13 03:35:09
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| Rhonda Bliss |
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Joined: 2007-02-27 08:21:26
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Hey Kevin,
It is pretty interesting-
It sounds like my sons case might be similar to yours.
Can I ask you, thinking back now is there anything in your childhood that you now say 'that was probably BMD related' Did you have muscle cramps of unexplained fatigue?
Just to be fair I am 36!
Are you on transplant list, or do you still have time? Not the same but if it helps at all my husband had a kidney transplant 20 years ago and is doing great.
Take care,
Rhonda |
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Rhonda
Mom to Aaron-(exon 48 deletion) age 10 Dirtbike rider & video game whiz! |
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Subject :..
2007-04-13 03:53:09
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| kgoodfellow |
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| All Star |
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Joined: 2007-02-20 17:21:41
Posts: 679
Location: Denver |
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Hey Rhonda
Other than my SGOT levels, there was nothing majorly different about me. I do have larger calves and always have, but that was the only physical sign.
I played lots of soccer when I was a kid and it was clear that I wasn't built for long distance marathon type running, but I was good enough to play competitively up until high school. My calves would tighten up during soccer games, but a few minutes of massage at halftime and I was ready to go again.
When I was in grad school in '96 (about 26 years old) I was running 2-3 miles a day to keep in shape, with no soreness or noticible issues.
Looking back, I think that any serious 'signs' started when I was about 30 and have very slowly gotten worse. I still look and feel normal, but climbing stairs is more difficult. Other than that, I can't complain.
I am not sure if/when a heart transplant is on the horizon. My current cardiologist is very familiar with MD heart issues, thinks that I may not need one for 10-15 years or more. Others think it will be sooner....but they are not MD cardiologists.
I would encourage you to make sure you have a cardiologist that has MD experience (especially BMD). They are hard to find, but worth the effort, since traditional cardiologists advice can be misleading, due to the fact they aren't familiar with MD causes, but more familiar with heart attacks and viral causes.....which are different. The heart drug therapies are good, but most have not been tested specifically on MD patients....so be an informed patient and question what they advise. Most non MD cardiologists are just winging it, but will not tell you.
If I had to do it all over again...I would take an ACE inhibitor like Diovan when I was younger. I feel that something 'triggered' this acceleration when I was about 30 and these Alpha/Beta and ACE inhibitors are proven to help stop cascading heart damage. I have a hunch that they would have helped me be perfectly normal for longer.
Anyhow, that's my 2 cents. Hope it is helpful.
Kevin |
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K. Goodfellow |
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Subject :..
2007-04-13 22:54:44
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| Rhonda Bliss |
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Joined: 2007-02-27 08:21:26
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That does help. I think we were extremely fortunate to figure this out so early on. I often think if our PCP hadn't followed up on the mono, as most don't, we still would not have a clue- I have always know something wasn't quite right- but honestly I would have put money that it was an immune system problem, never would I have guessed this.
We will see Dr. Steven Colan from Boston Children's Hospital later this summer. He'll do our annual heart eval while Aaron is young. I have not met him, but have heard good things, and Dr. Linda Cripe out at Cincinnati said we'd be in good hands with him. Thanks for your help.
Rhonda |
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Rhonda
Mom to Aaron-(exon 48 deletion) age 10 Dirtbike rider & video game whiz! |
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Subject :Re: Heart Transplant..
2008-05-07 15:26:24
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| srbrent |
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Joined: 2008-05-07 15:14:04
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This is for Kevin:
Hi my name is Brent and I am 36yrs old with BMD. I had a heart transplant 12years ago. I feel great with a new heart.
I was diagnosed with a cardiomyopathy when I was 24. 2 years later I had a transplant at TGH in Tampa. Transplantation is a new begining not the end of the road. Feel free to email if you have any questions.. Thanks
Brent Z :D |
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Subject :..
2008-05-07 18:39:53
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| kgoodfellow |
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| All Star |
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Joined: 2007-02-20 17:21:41
Posts: 679
Location: Denver |
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Hey Brent
Did you notice any 'increase' in muscle strength after the procedure? Part of the weakness I have may just be related to the lack of normal blood flow to my muscles etc.
I've met one other BMD patient that had a transplant 10 years ago and he is doing very well. He's getting old enough that he's having some mobility issues. I do see that it can be a new beginning. At the moment I feel pretty 'normal' and am not limited yet by my heart issues, but that may change over time.
Are you still getting around ok?
Thanks
Kevin |
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K. Goodfellow |
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Subject :Re: to kevins answer..
2008-05-29 07:41:03
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| srbrent |
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Joined: 2008-05-07 15:14:04
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Hi, sorry it has taken so long to respond. Anyway to answer your question, after you are transplanted you will be like you were before you had problems and you will get your strength back. It will take a while though, 6months or longer to start feeling normal again. When I got sick with my cardiomyopathy, I lost a lot of weight (I am about 5'11"and weighed 110 pounds right before my transplant). I became very weak and lost the ability to walk so did use a wheelchair. After I was transplanted I could not walk but with physcial therapy and time I was able to walk again !! However, some of the meds you take after transplant may cause some weakness. Please be sure to talk to your doctor about statin drugs. I was on Pravachol and was fine for a few years. Then one day my CPK shot up to 11,000 and I could not even muster up the strength to call for help. I was hospitalized and after that seemed to have lost some muscle strength. About 2 years ago, 10 years post transplant, I started using a scooter just to be on the safe side. It was my shoulders that suffered the most. Another thing to be sure and tell your doctor is that people with Becker's MD are more prone to develop a condition called malignant hyperthermia with certain anesthesia. I did not know about that at all pre-transplant. I hope that this information helps you. If you ever need to talk to someone, please don't hesitate to email me since I have been there done that. I will be happy to answer any questions that you might have. |
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Subject :Hey..
2008-09-24 09:21:07
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| Gyimadu |
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Joined: 2008-09-24 08:46:20
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Yes, I'm one of those unlucky people who needed a heart transplant. I needed a heart transplant because my heart which is a muscle was damaged by muscular dystrophy. It was a long ordeal and I spent two months in hospital then the doctors sent me home to die but I was on the recipient list and then I went to my church and got baptized thinking i was going to die. But by a miracle the next day they called and said i got the heart. I would like to know if anyone else had one and I would really like to hear your stories. |
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Subject :..
2009-05-22 12:53:46
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| JEFF |
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Joined: 2009-05-22 12:37:25
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My son just turned 15 in Feb and has BMD. He is missing 3 and 4 exon. He is 8 weeks post-op of his heart transplant and doing great. He was on the list for 12 days when we got the phone call. He is making it look like a walk in the park. There was only a few set backs, so far, with his transplant. The first was he was hyper sensitive to the med they use to get the new heart of the pacer, and he had to miss that step. The other set back was he formed blood clots in his new heart which they haven't figured out why. But he is on twice daily lovenox for the clots. They also have a hard time getting into his juggler for his heart biopsy and they have to go through his groin. All in all thou he is doing great! I thought this was going to be far more difficult than it has been on him. |
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Subject :..
2009-06-14 13:30:54
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| MistyGee |
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My son had his heart transplant before he was diagnosed with BMD. When he was 12 he got a virus that destroyed his heart (viral myocarditis). Having the underlying BMD contributed to the devastating damage to his heart. The only reason we found out he has BMD is that he was taking a "statin" drug (lowers cholesterol) that sometimes has a side effect of muscle damage, so they regularly check CK levels. When his levels came back a bit higher than normal, they stopped the drug, but his CK remained a bit high. It was then that we were referred to neuromuscular and the rest is history. He also has a deletion of exons 3 and 4 (in-frame).
He was transplanted when he was 13 years old and will be turning 16 this summer. For any BMD patient facing the prospect of a heart transplant, I say "Go for it!!" My son was on life support the day he was transplanted and on death's dorstep. Once he got his new heart, he was discharged from the hospital just 2 short weeks later and was competing in the World Transplant Games (swimming) only 9 months later in Bangkok, Thailand (where he won a Gold medal and a Bronze medal for Canada!!).
The transplant was truly miraculous- what a difference - we feel like we sent one very sick child into the operating room and got one very healthy kid back. The miracle of organ transplatation cannot be truly appreciated until you see it for yourself. Our donor family are the first people I think of first thing in the morning and the last people I think of before I go to sleep at night. Without these incredible people, who selflessly decided to help complete strangers in their darkest moment, our son would not be with us today! |
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Subject :..
2009-06-15 00:10:28
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| Roxanne |
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Joined: 2007-04-13 22:09:01
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Hi Mistygee, reading your story about your son and how well he's doing after his transplant brings tears to my eyes. I'm so very happy for your son to hear he's doing so well. God Bless! :) |
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Roxanne Mom to Nicholas (10) with BMD |
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Subject :..
2009-06-15 03:42:56
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| kgoodfellow |
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Joined: 2007-02-20 17:21:41
Posts: 679
Location: Denver |
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It is amazing the difference better heart function can make.
I was diagnosed with heart failure about three years ago. I recently had a procedure to eliminate a non-dangerous arrythmia (PVCs) in hopes it would help alleviate load on my heart and that heart tune up caused my ejection fraction to go from 25% to 45%. I do feel better and am able to exercise more and gain back some strength. I'm almost feeling like I did 5 years ago.
It is extremely important for BMD patients to closely monitor their heart function. There are some drugs that have shown to help slow down or prevent the heart breakdown. Steroids have also recently been shown to help.
BMD patients can and do get heart transplants and others that I know have also made a great recovery. |
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K. Goodfellow |
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Subject :Re:Love to hear from heart transplant patients..
2009-12-31 09:00:17
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| shrenp |
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Joined: 2009-07-12 03:18:04
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Hello Kevin,
I am a recent heart transplant recepient. I am 36 and has been diagnosed with axon 45-47 deletion of BMD since 2000. My heart function took the turn for worse about a year ago and the last few months it was around 10%. I went to mayo for a transplant eval and was put on milrinone infusion and received the transplant on 11/28/2009. I was on the list abour 2.5 months. I feel very lucky to get a heart in such a short time. Currently, I am doing ok. Rehabbing and staying here at Mayo since they check for rejection every week with a biopsy. I can tell you, it is the best place. If you have to have a transplant do not go anywhere else but Mayo Clinic. It was scary before I got it, because you don't know what would happen. Yes there are some side effects from all the immunosuppressants but looking at the long term picture, it is well worth it. I feel more energetic, enthusiastic toward life and feel like a new person. I think the biggest change is you have to have a positive mindset going in. Don't let yourself feel down or depressed, think positive and all the fear goes away.
I will keep you guys up to date on my progress
shrenik |
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