Hello, Everyone:

My name is Amanda and my little brother, Robbie, is 23 and has DMD. We have been very lucky with Robbie as his progression has been much delayed. However, we encountered a VERY scary situation back in December. Robbie was in the ER and they diagnosed him with pneumonia. However, right after they told us this, Robbie went into cardiac arrest twice and we thought we were going to lose him. Thankfully, they revived him and, when they did, they realized they read the xray wrong and that he did not have pneumonia, rather he was in congestive heart failure and had only 5-10% EF.

Since then, Robbie has been in and out of the hospital here in Tampa, Florida. He’s been evaluated for a heart transplant / LVAD but was denied because he has DMD and they are worried about getting him off the vent post-surgery. Since he was denied these things, they put him on a 24/7 infusion of Primacor (milrinone) which, thankfully, he’s reacted VERY well too. Before this medication Robbie’s BNP (measure of heart failure) was over 3000 and now his BNP is at 315. So we are pleased with the results so far. We will also know if his EF has improved soon as he had an echo done yesterday. Hopefully there is an improvement.

Although we have seen great improvement with this medication, the doctors have also warned us that we should also prepare for lung muscle deterioration. So in order to help Robbie keep his lung mobile and active, they have given him a CPAP to wear at night, a spirometer to use during the day, and a cough assist to use as needed to help clear his lungs. He also has Pulmonary Arterial Hypertension (PAH) and is on Ventavis which he has to take every 2 hours, 6 times a day! So, we have a lot of work to do.

So getting to my point now that I have given all the background details…

When Robbie was denied a transplant / lvad I immediately reached out to Ohio to see what our other options were. I heard about the 29 year old, Jason who received an lvad at Ohio last Fall and I wanted to be sure we were covering all of our bases. Since then, I have been in contact with Dr. Lorts at Ohio and we are waiting for insurance approval so Robbie can be evaluated up there. Basically…we need a back-up plan in place if and when the Primacor isn’t effective anymore. We also want to be sure that he’s receiving the correct breathing support and nothing is being overlooked when it comes to anything and everything.

That is what brings me here as well. As I mentioned at the beginning of this post we have been very fortunate with Robbie. We haven’t had any major changes between the time he lost his ability to walk (age 13) up until this heart episode in December. Although this is a blessing, it has also left us a little out of the loop with what is going on in the world of DMD and now we just have so many questions. Are we doing everything we should be doing? What other options are out there? Is this the “normal” thing to do at this point? Are we missing something? Are these medications on the horizon going to benefit those individuals who are in the late stages? What should we be looking for at this point? I feel like all information I read is geared towards families with younger children and it just doesn’t apply to us anymore so it makes it hard to know if we are doing everything we should be doing.

So I guess this is where we are at. We would like to hear from anyone who has any advice and can help provide us any information that could help us. Anything at all really would be much appreciated. We need to be brought back into the loop a little and are looking forward to hearing from people in similar situations!

If I need to post more details, I will. Just ask me and I would be more than happy to.

Thanks so much for your help in advance!

Amanda

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So sorry to hear about Robbie.I can only pray for the best under the circumstances.The only thing I have to say is that our DMD guys are not put on c-pap. The C stands for continuous pressure of airflow in. Older DMD guys cannot breathe out well against the continuous pressure in and this makes CO2 rise. If CO2 is too high it signals the body not to breathe. That is why our guys are but on a bi-pap with 2 levels of pressure. One for breathing in and a lesser pressure setting for breathing out.  My son is 32 and uses a volume vent at night(also 1 setting for inhale/a lesser for exhale, but based on volume rather than pressure) and a sip-vent during the day. I cannot advise you about any other circumstances. My site is AirDuchenne.com if you want further breathing advice. Best wishes.

Thanks, Ann. I appreciate your help. This is exactly the type of guidance we need. My mother is calling the pulm this morning to discuss this. Your site is also very helpful and I appreciate your sharing it! Robbie actually does very well breathing but they are trying to prepare us for it getting worse eventually so he was just started on the CPAP. I'm glad we are finding out about this early enough to ensure we have him on the best path possible.

 

Thanks for your advice and your prayers! Definitely appreciate it!

So 2 things:

1) Robbie's heart is back up to 15 - 20% which is incredible news and they are hopeful it will continue to improve with therapy. Such a relief

2) My mom called the pulm doctor today and he is gettng a bipap tomorrow! When my mom mentioned it to them it was like "oh yea, he should have that instead of cpap." CRAZY!

Thank you thank you thank you, Ann, for providing us this informaiton. We really do appreciate the help!

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