Author Archives: Kathryn Puzzanghera

Accessibility hacks, mobility aids, and tips (Part 1 of 3)

Written by Paul Shay
Groton, Massachusetts

I am one of the lucky, late-onset FSHers: diagnosed at age 57, now 71. As my progression has been relatively slow over those 14 years, I have incorporated a number of adaptive devices and techniques that I can share with you. Since my wife and I are still working, we have been fortunate enough to have the resources to try out a number of products.

As I am mostly affected in my shoulders, biceps, and quads, my issues revolve primarily around climbing stairs, reaching high and low, and getting up from a seated position.

For those challenges, here are a number of things and tricks I’ve found useful: Continue reading

George Pollock shows off his racing form

Team FSHD Cycling eyes epic race in June 2017

by June Kinoshita

During the month of June of this year, the FSHD community will enjoy the thrill of having one of its own lead an eight-person team in the Race Across America (RAAM), a bicycle race from Oceanside, California, to Annapolis, Maryland. The team, recruited by George Pollock, plans to compete in the Open Relay Division. The team expects to complete the race in under seven days and cover more than 450 miles a day.

The team has a personal connection to the disease. Pollock has FSHD. He started to have muscle weakness in his lower right leg while in his late twenties. Ten years later, when he experienced muscle weakness in his left leg, he was finally diagnosed with FSHD. Fortunately for him, the disease is progressing slowly. He was recently elected to the Board of Directors of the FSH Society and has made a commitment to promote awareness to improve diagnosis, support patients, and raise money to fund the Society’s mission. Continue reading

Upcoming Denver FSHers Meeting: March 25th, 2017

Written by Beth Johnston, Development Officer at the FSH Society

When my husband, Jeff, was diagnosed with FSHD in 2001 (after years of wondering what was going on with his body), we couldn’t pronounce the disease, let alone understand what it all meant for our family.  I discovered the FSH Society several years later (thank goodness) after moving from Denver to New York – and began the journey of discovery and hope.

Fast forward 16 years, 2 kids later, and we are back in Denver. One thing that has kept me hopeful and sane during these years is the camaraderie of patients and their families and friends – all working together to raise funds, raise awareness, and share helpful information with one another. Continue reading

Johns Hopkins Studies

Johns Hopkins and the Kennedy Krieger Institute are currently recruiting for two studies!  Volunteering for studies like this helps provide researchers with the information they need to provide better treatments, understand the mechanisms of the disease, and search for a cure.

For Family Members (no travel necessary!):

The Johns Hopkins Hospital and the Kennedy Krieger Institute are looking for first-degree relatives of FSHD patients ages 35 and older who do not currently show symptoms.  Volunteers will be asked to give a blood draw, which can be performed at any local lab.  The blood draw, the genetic test, and shipping will be covered by the study.

Interested individuals should contact Pegah Dehghan: dehghan@kennedykrieger.org.

Study Protocol Number: NA-00019985. 

For Patients: Continue reading

FSH Society’s first Open House!

The FSH Society is looking forward to seeing you!

 

If you live in the New England area or even just happen to be visiting, we’d love to have you stop by our office on March 7, 2017, between 5 and 7pm for our Spring Open House!

We’re very excited to show you our new office space.  We have made lots of improvements with our move: cabinets for all the brochures we send you, a fridge that’s larger than the microwave, a conference room with a projector so we can talk to Beth in Colorado, and full desks for Kate and Robyn who were previously working off folding tables.  This new space is part of a new chapter for the Society, as we strive to grow as an organization to better serve you and reach our goal of finding a cure for FSHD. Continue reading

Imaging study shows positive effects of exercise and CBT on muscle degeneration

In 2014, a Dutch team reported that aerobic exercise training (AET) and cognitive behavioral therapy (CBT) decreased fatigue and improved the quality of life significantly in FSHD patients. Now, the same group has published a study demonstrating that not only did patients given AET or CBT feel more energized and active, but that their muscles degenerated more slowly than in patients who received standard care.

Strikingly, the effect was largest in the CBT group. CBT often focuses on how your thoughts can influence your behaviors and the choices you make. It is often used to treat patients with chronic illness to improve their functioning in their daily life. Continue reading

Ask the Physical Therapist: Inversion tables, trigger points, and chronic pain management

The following is a transcript of a question-and-answer session, conducted over the FSH Society’s Facebook page, with Julie Hershberg, PT, DPT, NCS. Hershberg is a physical therapist who is a Board Certified Neurologic Specialist.  She practices at [re+active] physical therapy & wellness and is an instructor in Doctor of Physical Therapy program at USC.

I recently was examined by a physiatrist. Her report has recommended ongoing therapy plus she’s suggested an evaluation at a pain clinic and possible destroxe prolotherapy and/or trigger point injections. Do you know of any studies/reports about this type of treatment for someone with FSHD (or related conditions)?      Here’s the link to their website: http://www.bowlermedical.org/. The physician page and FAQ page provide information/articles relevant to their treatments.

First of all—so glad that you are working with a physiatrist and specialty clinic for pain—these are great steps toward better health.  There is not specific research regarding FSHD and trigger point or prolotherapy injections. However, there is also not evidence that either of these would be particularly detrimental to people with FSHD.  Trigger point injections are usually an anesthetic and therefore the mechanism of action is at the level of the nervous system rather than the muscle.  The prolotherapy injections are typically also not done directly into muscle and include concentrated dextrose and an anesthetic.   In regards to management of chronic pain, there is evidence that  biopyschosocial factors should be considered in the management of chronic pain for people with FSHD (Miro et al, 2009).  In fact, there is evidence for this approach for all people with chronic pain. I would recommend you inquire about incorporating addressing biopsychosocial factors  as part of your comprehensive pain management program. 

 

I’m watching a program on an inversion table. I was wondering if that would help or hurt us FSHers? It looks like a great way to twist and stretch the back out (which feels SO good) and helps with respiratory by opening the lungs up–which is hard to do by myself! Any idea if an inversion table would be good for us or have negative effects?

My first question is: have you tried one?  Most people either have a strong aversion or a love of the inversion table just based on personal preferences.   Inversion tables are a form of spinal traction.  Spinal traction most likely stretches the muscles around the spine and can temporarily relieve muscles spasm.  While spinal traction makes us temporarily feel very good, it does not provide long term relief (a Cochrane review in 2006 concluded that there was not evidence to recommend it for the treatment of low back pain).   There are some risks to be aware of with use of an inversion table:  it raises blood pressure, lowers heart rate and increases pressure in the eye.  It is recommended to not use an inversion table if you’re pregnant, have high BP, heart disease, glaucoma or any other eye disease. 

Overall I would recommend that you might try it with a physical therapist under supervision and incorporate it as part of a comprehensive program for low back pain. 

 

Ask the Physical Therapist: Car Accidents and FSHD

The following is a transcript of a question-and-answer session, conducted over the FSH Society’s Facebook page, with Julie Hershberg, PT, DPT, NCS. Hershberg is a physical therapist who is a Board Certified Neurologic Specialist.  She practices at [re+active] physical therapy & wellness and is an instructor in Doctor of Physical Therapy program at USC.

I have FSHD and have been in two vehicle accidents—-one in 2001 and one in 2014. Both accidents resulted in soft tissue/whiplash injuries affecting neck, shoulder, arm, spine and back areas. I am wondering if you know of any articles related to soft tissue/whiplash injury and recovery in people who have FSHD (or a similar conditions)? I am currently receiving physiotherapy, massage therapy, exercise and pool therapy (the latter two provided by a kinesiologist). I am the first client with FSHD that any of the therapists have seen and I’ve given them some articles about FSHD and exercising with FSHD but none address accident injury or treatment.

I reviewed the literature in this area and there has not been research regarding FSHD or similar disorders post whiplash injury.  In looking at the research of whiplash in general, there is also not conclusive evidence that pre-existing muscle weakness or postural deformities contribute to pain or disability post injury.    In that case, I don’t think there would be anything recommended from research for the therapists to do differently in managing your whiplash.  I would just guess that it will likely take longer for you to heal due to potential pre-existing trunk and shoulder weakness.  Continue reading

Ask the Physical Therapist: Zero-Gravity Treadmills, Strength Training, and Braces for Lordosis

The following is a transcript of a question-and-answer session, conducted over the FSH Society’s Facebook page, with Julie Hershberg, PT, DPT, NCS. Hershberg is a physical therapist who is a Board Certified Neurologic Specialist.  She practices at [re+active] physical therapy & wellness and is an instructor in Doctor of Physical Therapy program at USC.

I have been recently diagnosed. I have always been quite active. However, I have been told to stop my kettlebell class, as well as using any free weights, and go swimming instead. Is this something you agree with?

The general thought in the past is that strength training was harmful for FSHD—that is not the case now. Moderate-intensity strength and aerobic exercises have been found to be safe. So the answer is not a universal recommendation to stop strength training! However, it would be important to know how you are performing the exercises so that you are using the appropriate amount of resistance and not compensating or hurting yourself. I recommend a physical therapist to help give you guidance in this area! Continue reading

Ask the Physical Therapist: Running a Marathon and Customized Workouts

The following is a transcript of a question-and-answer session, conducted over the FSH Society’s Facebook page, with Julie Hershberg, PT, DPT, NCS. Hershberg is a physical therapist who is a Board Certified Neurologic Specialist.  She practices at [re+active] physical therapy & wellness and is an instructor in Doctor of Physical Therapy program at USC.

I am a 48-year-old man who is asymptomatic. I am still fairly active. Prior to my genetic diagnosis of being positive for FSHD in 2011, I ran two NYC marathons in 2008 and 2009. I am thinking about running another one next year or the year after at a slower pace than I did six years ago. I ran a sub 4:15 in those, and this one would probably be somewhere between 5 and 5.5 hours.

Do you foresee any issues or have any concerns if I take up this endeavor? If not, what precautions could or should I take prior to and during the training for the race?

Awesome! I think this is an excellent goal! My advice is to proceed with awareness and just an ounce more of caution with your training in general. I would say the same thing to anyone who has not run for a while. Something in particular to think about is that while you may not be aware of any symptoms right now, there may be very subtle areas of weakness or tightness that can predispose you to injury (this is true of the general population). This video, put out by the American Physical Therapy Association, is an excellent resource for things to consider when returning to running: http://www.moveforwardpt.com/…/VideoLibrary/detail.aspx. Continue reading