December 22, 2014
by marfmom


2014 Marfan/RD Gift & Resource Guide

It’s that time of year! You’re looking for last minute gifts for the Marf in your life, and you’re stumped. Never fear! My (mostly!) annual gift guide is here! Let’s get crackin’!

Britax B-Agile
Stephanie found the B-Agile stroller to be a perfect fit for her son! It holds kids up to 55 lbs, has a seat tall enough for a Marf (so that their heads don’t hit the sun shade), and lots of leg room. It works well for older kids who may not be able to walk yet, or who tire easily. It’s also lightweight (16.5 lbs), which is great for Marf parents.

Keen shoes
Brie loves Keen shoes and says they help with back pain. They make both kid and adult size shoes. Brie says they have great arch support and although they don’t specifically say narrow, they do fit narrow sizes. They make boots, casual shoes, and sandals. Women’s sizes go to an American 12, and men’s to an American 15. keen-logo-tilt

Cascade DAFOs
We use these for J. Cascade makes both ready-to-fit orthotics and custom orthotics. We use the Cricket ready fit ones (pictured). AFOs/SMOs/DAFOs are controversial with Marfan specialists. The ones I’ve talked to expressed concerns about dependence on them due to muscle atrophy, as well as the higher braces causing additional issues due to the foot not being able to flex at the ankle (so the child is learning to walk improperly). Obviously it’s a risk-benefit decision that parents have to make. With the cricket, J’s right foot is totally corrected and his left foot is partially corrected. I’m not sure it’s made a huge difference with pain, but I’m hoping it’ll be helpful long-term. I also noticed that in the custom section, Cascade makes knee-high DAFOs with a hinge at the ankle. Cricket@2x

Wave Cruise Shoe
This is a Clark’s brand shoe for women, recommended by Michelle, who has Loeys-Dietz. She said all Clark’s shoes are great, but the Wave Cruise has been the best, and fits AFOs well. They go up to an American size 12. wave cruise

Pain Management:
Kinesio tape
Do you or your child have pain? J was waking up regularly sobbing at night complaining of foot and leg pain. I had the same pain. It’s common with connective tissue disorders, especially little kids. His physical therapist started using kinesio tape and it’s made a HUGE difference. I can count the number of times he’s had pain while wearing the tape over the past 6 months on one hand. My mom (non-Marf) has also had success with this taping. Kinesio

Foam Rollers
Johannes recommends foam rollers as a way to relax muscles after a workout or long day. It’s like “a good and strong massage.” When used properly, he says they increase blood flow. He suggests starting out with a softer roller and working your way up to a more firm one.

This suggestion comes by way of Brie. I’ve used one for my fibromyalgia pain and it definitely took the edge off. TENS works by sending little electrical impulses to your skin to, as my physical therapist explained, try to teach your brain to recognize that instead of pain. TENS doesn’t really hurt….it’s just focused, rapid vibrations. Although you don’t need a prescription to purchase one (see the site I linked to), it is still medical equipment so it would be a good idea to consult with your/your child’s doctor before purchasing.

Freeflow Waterbed Mattress
Valerie’s daughter swears by these! She says her mattress is the best thing to help her chronic pain.

Leg Pillows
Faith says that sleeping with leg pillows help her back pain and have been a “lifesaver.” Waiting to hear back about a particular brand suggestion from her, but she did send me this picture. leg pillows

USB handwarmers
Sarah suggests these for those with Raynaud’s in particular, but anyone who is constantly cold (meeeee!!!!!) could appreciate these! This one warms to 113 degrees F (45 C). handwarmer

Exercise Ball Chair
Faith uses one of these to help with her posture, trunk strength, and back pain. Exercise ball chair

Gotta put in a plug for MarfMom’s Marfan Merch, my CafePress store. I’ve got customized clothes for newborns through adults, and there are plenty of non-clothing items too. I’ve got items for those with Marfan in the family, as well as Loeys-Dietz, Ehlers-Danlos, and Beals. I’m happy to make custom orders! superpower_tshirt

Are there must-have products or life-changing toys I’ve missed? Fill us in in the comments! Past gift guides: 2011, 2012 p1, 2012 p2

* I did not receive any compensation at all from any of these companies for reviewing or otherwise mentioning their products.

** The opinions offered at Musings of a Marfan Mom are for informational purposes only and are not intended to be a substitute for professional medical advice, diagnosis or treatment. Always seek the advice of a qualified healthcare provider with any questions you may have regarding Marfan syndrome and any medical condition. Never disregard professional medical advice or delay in seeking care because of something you have read here.

November 19, 2014
by marfmom


The Losartan Trial Results Are Out…Now What?!

By now you’ve likely read the synopsis of the atenolol vs losartan trial results. Perhaps you’ve read them from multiple sources and are a little confused. Maybe the results are what you expected and you’re happy, or maybe you’re feeling disappointed. Was the trial a success or a failure? Where do we go from here?

Here are my thoughts!

Does losartan work? Yes! The trial showed that both losartan and atenolol work. It seems like people are disappointed because the trial showed that they work the same, and their expectation was that losartan would work better. However, there was a dosing issue. Losartan was compared to a very high dose of atenolol (up to 2x the FDA recommended dose commonly used for people with Marfan syndrome). The losartan was dosed at up to the FDA maximum dose for treating people with hypertension (so not everyone in the study received the maximum dose). In the past, most people with Marfan were prescribed much less atenolol than was used in the trial. And, the amount of losartan prescribed to patients in clinics over the last few years has varied greatly.

Why is this important? If the atenolol had been dosed at a more clinical level, and losartan where it is, or at a higher level (more in line with what some doctors are using clinically), the results might have been different. All we can say from this trial is that losartan and atenolol work equally well at the particular doses from the trial. The results can’t be generalized to other doses. Another important takeaway is that for those on beta blockers, this higher dose is potentially necessary for the best effects.

Why was the atenolol dose so high? My understanding is that because we’d never had a double blind trial (where patients and the trial site administrators don’t know which drug the patients are taking) to prove the effectiveness of beta blockers before (though there were other studies!), these researchers also wanted to prove beyond a shadow of a doubt whether beta blockers were effective at managing aortic growth in Marfan patients. They definitely accomplished this, though the remaining question is whether this might have muddied the comparative effects of losartan at all.

Kids vs adults: The researchers discovered that optimum results occurred in young kids. This provides evidence for what a lot of us have been thinking: it’s important to start our kids on medication early. This makes me feel even more confident in our decision to start J on medication when he was 2.5 years old.

Placebo: The New York Times article (which, I want to point out, neglected to explain the dosing issue with this study) talked about the fact there wasn’t a placebo in this trial and seemed to question the efficacy of the results because of this. In light of the article, I’ve seen a few people asking why there wasn’t a placebo. There was no placebo because it wouldn’t have been ethical. Prior to this study, there was a standard of care for treatment: beta blockers. It’s generally frowned upon to do a high-risk study (in terms of risk to the participants; in this case, the risk of dissection) with a placebo when a standard of care exists already. And let’s be honest: this is a trial looking mostly at children. Would you have taken your child off treatment to enter a study where there was a 50% chance they’d have had NO treatment for their existing aortic aneurysm for 3 years, thereby increasing the risk of needing surgery or having a dissection during that time? No way! This study was designed to be as safe as possible for the patients enrolled and therefore it was set up to compare two drugs against each other. While some researchers might have argued a placebo would have been “better science,” it wouldn’t have been in the best interest for our community.

This study doesn’t have all the answers. It was never going to have all the answers…no single study can! But because of this, other studies are going on all over the world. Some have already been published. These studies ask other questions. They look at whether other ARBs, like irbersartan, might be better than losartan. They see whether losartan + atenolol is better than either drug alone. They study different age groups. There are also ancillary studies to the US trial, which are looking at whether or not losartan affects other body systems.

I am glad to know that losartan is a valid “tool in the Marfan toolbox.” This is an option for people who can’t tolerate beta blockers for whatever reason, and now we know a little more about dosing beta blockers. Over the next few years, we’ll learn even more about what ARBs can and can not do for us, and I am confident that other drug options will be brought forward to be tested as well. Every step forward in finding preventive treatments for aortic aneurysms is a success!

If you haven’t already, consider signing up for the interactive webinar with the study’s principal investigator, Dr. Ron Lacro of Boston Children’s Hospital. He’ll explain more about the study and take your questions. It’s a great opportunity! In addition, the Marfan Foundation will be planning another webinar with Dr. Hal Dietz shortly. The Foundation has also put together a Q&A, which you can find here.


The opinions offered at Musings of a Marfan Mom are for informational purposes only and are not intended to be a substitute for professional medical advice, diagnosis or treatment. Always seek the advice of a qualified healthcare provider with any questions you may have regarding Marfan syndrome and any medical condition. Never disregard professional medical advice or delay in seeking care because of something you have read here.

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September 10, 2014
by marfmom


Approaching Strangers: Do You, or Don’t You?

In continuing my series on conversations we have about Marfan syndrome with our kids, today’s topic is on approaching strangers who look like they might have Marfan.

The topic comes from reader Jamie, who writes:

Once you know about Marfan, you can’t help noticing people you meet in public with great height, long fingers, obviously corrected eyesight, thin limbs, etc. The condition is not *that* rare and if you live in a populated area, the odds are good that you’ll encounter someone else with Marfan — and, you might be the only person in their lives who has learned to recognize it.

Of course, a lot of people have these traits without having Marfan. Even so, warning people seems like an ethical no-brainer since their health might be in danger without them knowing it, and the costs of ruling that possibility out seem small in comparison. On the other hand, it really *feels* wrong; health is personal, and you could be stepping into a stranger’s life in a very intimate way to add a serious health concern and possibly life-changing news to whatever else they have to worry about.

I’d be curious about others’ reactions to this situation.

I’ve only asked a stranger once. I was at a party that one of my brother’s friends was hosting. I had never met the guy before, but he was so tall I couldn’t pass up the chance to ask. I followed him into the room to get more snacks, struck up a short conversation, then said something to the effect of “hey, so this is a little weird to ask, but have you heard of Marfan syndrome? I ask because I have it, and you’re tall like me so I wondered if you might have it too,” or something like that….this was when I was pregnant with the Menininho, so it’s been a few years. He ended up not having Marfan, but he wasn’t offended that I’d brought it up, and I was glad that I’d played it safe and asked.

If you have Marfan, have you approached other people you think you might have it too? What about if you’re a sibling/parent/partner? If you are a parent, have you talked to your child about approaching strangers who fit the Marfan or a related disorder phenotype?

I wanna hold your hand

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