Neurologynewsletter fall 2015

Volume 1, Issue IV

October 2015

Neurology HOSPITAL FOR SPECIAL SURGERY

Neuromuscular News Letter from the Director

In This Issue ● Ask the Experts ● Hot Topics ● Patient Spotlight

● Research Buzz ● Caregiver Corner

Share your story!

If interested in writing in our patient blog, sharing your experiences and thoughts, please contact Mona or Shara.

Resources for Your Needs Rebuilding Together: http://rebuildingtogethernyc.org/access-to-home/ Rehabilitation Engineering and Assistive Technology Society of North America: http://resnaprojects.org/ Ability List: https://abilitylist.org/ (download the app!)

American Association of People with Disabilities http://www.aapd.com/ & www.dcu.org Wheels for the World http://www.joniandfriends.org/wheels-for-the-world/

Dear Friends, Welcome to our Fall issue of Neuromuscular News. This issue includes information that we hope you will find of interest based on

feedback we have received. As always, our commitment remains providing the best available care to our patients and families.

Your thoughts, insights and suggestions are welcomed and always help us grow.

American Academy of Neurology, American Neurological Association, North East ALS Association and the International MND meeting are all taking place over the next several months. We hope to bring you any breakthrough research or new treatments that will be unveiled during these sessions.

Regards, Dale J. Lange, M.D. Director, ALS program at Hospital for Special Surgery

Meet Our Newest Member Dr. Pantelis Pavlakis

Team Gleason http://www.teamgleason.org/ Neighborhood Workshttp://www.neighborworks.org/

Please join us in welcoming our new fellow, Dr. Pantelis Pavlakis.

He joins us this year as the neuromuscular fellow, focusing on neuromuscular diseases and electrodiagnostic studies. His research interests include motor neuron disease, immune-mediated neuropathies, and neurological syndromes associated with systemic autoimmune diseases.

United Spinal Resource Center: http://www.spinalcord.org/resource-center/askus/ index.php?pg=kb.printer.friendly&id=3

He has previously trained in Boston University and is looking forward to joining our team this year. In his free time he enjoys reading, cooking and hiking.

National AgrAbility Project: http://www.agrability.org/

ASK THE EXPERTS Making Bathrooms More Accessible NARROW DOORWAYS: A simple solution is to take off the doors completely! If you’d like to keep your doors, try an offset hinge.

It can add 2” to the width of a doorway (this can work for any doorway!) It replaces the existing hinge.

Same holes, same location, same purpose, swinging the door in and out of the doorframe but the unique design of an offset hinge is that it opens the door past the doorframe, not inside the doorframe. You gain 2” of clearance, and have access to the entire width of the doorway. Offset hinges may be found in some hardware stores, though some may not know what you are referring to. You can also search online for offset hinge or swing clear hinge.

Two things to remember when considering an offset hinge: There needs to be 2-3” from the existing hinge to the next wall. If the existing doorway is right up against the wall, there is no room to place the offset hinge.

An offset hinged door will swing 2″ further into the room. If there is open space in the room, the larger door swing is unimpeded. But if the current door swings very close to a sink or other fixed item in the room (less than 2″), using an offset hinge will cause the door to bump into that object, and not open fully.

When building a new doorway, make sure the measurement you get is the doorway clearance (the unblocked space of the doorway), not the width of the doorway itself. When you open a door, the edge of the door right along the hinge, blocks part of the doorway. A door edge can take up 2” so depending on the wide of the wheelchair, it may not make it through.

TOILET A raised toilet seat. It’s a sturdy plastic cylinder placed on the toilet bowl to enable one to sit higher than the standard 15” toilet. They generally add 2” – 6” of height. They come with or without handles.

Standing from a higher seated position, requires less effort than from a lower position. It also makes it easier to sit back down!

So this is something to consider if standing up is difficult. For a raised toilet seat to be safe, it must be secured, which is easily done by tightening a bracket under the lip of the toilet. Without this bracket, a raised toilet seat can tilt or tip.

A standing or rolling commode. These provide a separate layer to sit on above the toilet., but make sure toilet seat is lifted up! The commode can also be free-standing alone in the bedroom for safe transfers at night when you’re groggy and sleepy. Look for models that allow you to adjust the height. There are ‘up-lift’ commodes as well.

These have seats with a mechanism that actually rise to assist you in getting to a standing position.

ASK THE EXPERTS GRAB BARS There are different kinds of grab bars that can further help to lift yourself off the toilet. The options below are specific to this kind of use and provide the needed safety.

1) There are easy to install grab bars that attach to the back of the toilet seat itself where the handles are on either side of the toilet.

This is called a Versa-frame.

2) There are grab bars that rest on both sides of the toilet.

3) There are grab bars that secure directly to the studs in a wall, which can be placed next to and behind a toilet. These come in varying lengths to accommodate the space available.

No matter how handy and convenient, please do not use a towel rack that happens to be in close proximity. It is not designed to accommodate the grip or pressure when standing up.. Speak with your therapist about placement of bars and height from floor. The bars can be installed vertically, horizontally, or on a diagonal.

WATER WATER EVERYWHERE: Both sliding and hinged doors prevent water from going outside the shower area. but sliding doors (which have a track on which they roll) can create an obstruction to the wheels of a walker, wheelchair or shower chair. Hinged doors swing open, but in many bathrooms, the swing of the door hits the sink or toilet when opened and it decreases the accessible space to roll into the shower.

Possible solutions?

1) A collapsible water stopper. Is a soft, rubberized strip that is glued to the floor. It’s about 1� high and flexible. It will push down when a wheelchair rolls over it and will bounce back up when the pressure is removed. This stopper can reduce excess water from going beyond the shower area.

2) A weighted shower curtain can be easily drawn away from the shower entrance and closed snuggly once drawn shut. This also provides easy access, while maintaining a water tight barrier.

3) A hand-held shower allows the water to be positioned where you need it and helps prevent the water from splashing all over.

HOT TOPICS Medicare exempts SGDs from capped-rental Medicare willprovisions deduct previous payments under the On July 30, 2015, the President signed the Steve Gleason Act of 2015 into law. This new law helps protect patient access to medically necessary Speech Generating Devices (SGDs) for individuals with communication disabilities, including ALS, Cerebral Palsy, spinal cord injuries, and Rett Syndrome. SGDs will be exempt from the cappedrental provisions from October 1, 2015 through September 30, 2018. Specifically, the new law will remove SGDs from the Medicare Durable Medical Equipment (DME) payment category as a “CappedRental” and provide coverage of eye-tracking technology for patients who rely on this method to access and operate covered SGDs A revised Final Decision Memorandum was also released, which allows SGDs to connect to the Internet to generate e-mail, text, or phone messages. Rented devices under the new definition of SGDs can perform additional functions paid for by the beneficiary as long as the device is primarily used for generating speech. Devices are able to be “unlocked” and—with clarification regarding the capped-rental policies—will now be owned by the beneficiary upon request, effective October 1, 2015.

For devices that were rented prior to the October1, 2015, changes will be allowed for purchase and transfer of ownership to the Medicare patient.

capped-rental policy from the Medicare allowed amount, not to exceed the amount allowed for the purchase of a new item. Responsibility falls on patients with SGD to contact the manufacturers of the equipment for further instructions regarding payment and the transfer of ownership. Personal devices, such as computers and tablets, remain uncovered items for the Medicare program.

The bill is called the Steve Gleason Act of 2015, in honor of the former Saints player who was diagnosed with ALS in 2011.The passage of The Steve Gleason Act is a monumental step for Medicare patients who rely on assistive technology to communicate with their loved ones and caregivers.

Sen. David Vitter, R-La., said the equipment is critical to giving these patients more independence. “Living with ALS is a tremendous challenge for the patients and their families. Our bill will help empower ALS patients to live and work more independently, and give them an opportunity to communicate with their loved ones” Vitter said. “Speech generating devices, like the equipment Steve Gleason uses, are a necessary part of everyday life for patients with ALS and similar diseases. Vitter said the bill will help “change the lives of patients across the country by giving them their voices back.”

SOURCES: http://www.asha.org/News/2015/Medicare-Releases-Instructions-Regarding-New-Speech-Generating-Device-Policy/; http://www.tobiidynavox.com/stevegleasonact/; http://www.nola.com/politics/index.ssf/2015/04/senate_approves_steve_gleason.html

Like what you see? You can join the fight!

Our program is largely built on support of donors. Your support gives us the foundation to provide the best patient care under the direction of leaders in the field in a multidisciplinary care model. We are dedicated to finding answers, treatments and therapies for neuromuscular disease.

If interested in supporting our program or volunteering your time, please contact Douglas Williams: williamsDou@hss.edu

RESEARCH BUZZ Updates On The Dps Study As some of you may know, the DPS study was started by the North East ALS Association, to determine if surgical implantation of the NeuRx® Diaphragm Pacing System™ (DPS) improves survival or diaphragm function in individuals with ALS and breathing muscle weakness.

Recent data has been published over the last few weeks regarding two studies in Europe that are also investigating use of DPS, albeit with different inclusion criteria than the American version, one in Great Bitain and another in France. Both studies have closed after announcements that suggested potential harm could come to patients who had received the diaphragm pacer system.

The American Data Safety monitoring Board (DSMB) has reviewed all this data and issued a recommendation that new enrolment in the study be halted at this time. At the same time, the DSMB recommended that patients already randomized to the diaphragm pacing arm continue to be actively stimulated and all active patients continue to be followed according to the protocol. The DSMB also reviewed data from a third open label study in the US that found much longer survival in paced patients than was observed in the British study.

Finally, data from the current American study were analyzed. While the results are still preliminary, the DSMB did not see evidence of the risks to patients that was found in the other trials mentioned above.

The Principle investigators of the study, Dr. Sheffner and Dr. Katz, state “ While we are cautious, we continue to believe that the utility of diaphragm pacing in ALS patients remains undetermined given the mixed data from the different studies.

We think that data from continued follow up of our patients will be extremely important. We plan to ask the DSMB to conduct a safety review of our ongoing data in 3 months, and to completely reassess all available data in 6 months. If our data still indicates the lack of risk and possible benefit to patients, we will ask the DSMB to reconsider its recommendation to suspend enrollment. In six months we should have further details on both the French trial and the open label experience in the United States. We continue to believe that if there is a role for diaphragm pacing in the care of ALS patients, it is critical that this is appropriately identified. “ If you have any questions regarding this issue, contact your clinic team.

ENROLLING NOW @ HSS: Fingolimid Evaluating the efficacy and safety of oral fingolimod versus placebo in patients with CIPD. Retigabine Assessing the effects of Ezogabine on neuronal excitability in patients with ALS. Subcutaneous IVIG in CIDP Investigates the efficacy of two different IVIG doses given subQi n the maintenance treatment of CIDP Cytokinetics Evaluates the safety and effectiveness of Tirasemtiv on muscle strength and muscle fatigue in patients with ALS NeuroNx Assessing Rituximab’s effectiveness and safety in patients with myasthenia gravis (MG) who are also taking

Patient Spotlight

by Joan Broder

MY STORY IS LIKE MANY OTHERS OUT THERE. THE DATE, NAME AND LOCATION ARE DIFFERENT. OUTCOME THE SAME. In 2013 I realized that there were THREE initials I did not want to be associated with. How harmful could the alphabet be?

ALS are the letters that not only changed my life but that of my family. It was at that time I realized life can change in a second. I am a stubborn person, one of my inherited traits, and not a quitter. I have things to do and many moments to share with my husband, children and most important my grandchildren. My family keeps my heart beating and they are the force behind me. I must put one foot in front of the other. If I fall, I must pick myself up. Don’t get me wrong.

There are days when I want to yell out to the world WHY ME? What did I do to deserve this life sentence.

Fast forward and now it is August 2015. I go for infusion every two weeks and stay for about four hours. I have the routine down. Pack my goodies the night before. Up early to sit in traffic. If it’s not the Major Deegan, definitely the FDR. First to greet is Leah, the infusion nurse. Get my smile, my hug and sit in my assigned chair. Considering my veins are practically non-existent, Leah finds one hiding on the first try and we are in. After Leah sets up her other patients, we discuss lunch. I became educated on what to order from where. Ask for utensils and repeat the order. Mona, nurse practitioner, is never too busy to answer a question or an email. If a response is not there within 24 hours it means she’s away or off. Mona has the answer before I ask the My journey began in 2012 when I had trouble zipping the question. She knows me well. I always marvel how she sits zipper, buttoning the button, sewing camp labels, on the floor and gets up in a single, graceful move. I opening jars. I made my own educated guess…. would need to call AAA for a tow truck. Lauren, arthritis. Why not? It sounded right. After a social worker, is always on the go. I thought Everyone in few weeks of discomfort, I went to a hand I was smart when it came to insurance. I the medical field specialist who said I had arthritis and to am now mentally challenged and I know take Advil PRN. Like an ostrich, I put my Lauren will have the answer in her head my children knew head in the sand for almost eight months or make the right contact. She knows was called. Every when things got worse. I did what I told my by looking at my face the mood I am in. favor called in to daughter never to do and went online.

Always has a smile on her face and ready get appointments. to help. Shara, speech therapist, is making I looked up diseases I never knew existed. sure that I am updated on the latest. Her I read up on MS, diabetes, Lyme disease, smile is bright, her concern is genuine and she muscular dystrophy, etc. Then like a flash of has that extra minute. She squeezes my hand lightening I remembered 60 minutes had a segment in a way that she understands the frustration with on ALS. I typed ALS and there I was in disbelief. When I my verbalizing. Dr. Lange walks back and forth in deep mumbled the letters to my family the ball began to roll. thought. I lose weight watching him. I never feel like I am Everyone in the medical field my children knew were called. overstepping my boundaries. I can ask questions and know Every favor called in to get appointments. I sat paralyzed the answer will be direct and honest. He always has time for and my husband frozen. I met with a few doctors but I that last question, which with my family has several parts. was merely a number like in the bakery. The doctors were surprised when six people, including myself, came to the Let me be straight forward. ALS is challenging and no one consultation. One doctor said his office was too small for wants to be a member of the club. However, one thing that all of us. No problem. We will sit on each other’s laps. My makes the disease digestible for me is Dr. Lange and his staff. children and their spouses were there. We are family. Don’t They are my extended family. His staff is young, ambitious, start. We have each other’s back. cheerful, caring and most important genuine. You can wear any emotion on your sleeve and no one questions you or tell A breath of fresh air. A new start. I have an appointment you that the behavior is not proper. There’s always a hand with Dr. Dale Lange. We were greeted and ushered into the to hold. Someone to rub your shoulders. Someone with a main conference room. I felt like a celebrity and a sigh of tissue (I’ve used many boxes) or just a wave of the hand. relief came over me. I knew this is where I belong. I was in You are never alone. I thank every member of Dr. Lange’s good hands. There was immediate trust from within. It was staff for allowing me to be me and being there for my family a LONG clinic day but worth it. We were all in the conference unconditionally. room: Dr. Lange — the judge. Me — the guilty party. My family — the jury. Every question got answered. Time meant nothing. We came at noon and left after sunset.

Caregiver’s Corner

by Ami Broder Hoffman

MY STORY IS LIKE MANY OTHERS OUT THERE. THE DATE, NAME AND LOCATION ARE DIFFERENT. OUTCOME THE SAME. In the early summer of 2013 I caught my mother cheating for the fist time on her grandmotherly duties. She set out to sew hundreds of camp labels on her grandchildren's clothes like she had done in years before. However, this year would be different; this year she would bring the labels to a tailor because her hands weren't working well. Like always, she blamed it on the weather, the food she ate, or way she slept.

I quickly diagnosed her as having carpal tunnel. An easy diagnosis — one that I could handle. As the months went on, I noticed her getting on and off a chair saying "oh" and "ah I'm stiff”. She would often ask for a hand when getting off the couch or out of the car.

I cried on the phone to the receptionist real tears of agony as if she were my family. We made an immediate real connection. She said how is Friday?

It was love at first sight — they became extended family.

We marched in like an army with color coded folders, stapled blood work, scans, X-rays, you name it. They gave us a conference room, we moved in and haven't moved out.

Instead, we have upgraded to an IVIG suite where we gossip, discuss where to order lunch, what clothes we like and what are the most fashionable shoes to wear with leg braces.

Lauren ,the amazing social worker, particularly knows when to call me out of the room as she sees my eyes well up at times. There are not enough words to express the gratitude we have for Dr. Lange and his staff .... from Lauren, to Mona, to Shara to Dr. Manning...one is smarter, more patient, and nicer than the next.

On October 1, 2013 I turned 40! As I sat there getting my hair done for my birthday luncheon I was fumbling through my phone on Facebook. Somebody friended me and she was smiling holding a I was on big check that said ALS charity. I zoomed [the phone] with in because I knew the face as I asked myself the question "what is ALS again?" every personal

Here we are two years later. I am no longer the same person or daughter that I was on October 1 2013. I am stronger. I care less contact and every I did something that day I will never forget about some of the things I used to worry as long as I live. I surfed the web and it was doctor that specializes about. I have different stress. If there is one in ALS. from that moment on when I knew as I saw thing I have learned its that my mother is the my mother’s story unfold in front of me. She ultimate rock star!! She is brave, determined, was really sick. I left the hair salon got in my funny as ever and a true fighter. There might car and called my best friend. I said " I can't make not be a cure (which is another article I would like my 40th my mother has ALS. " As usual, my friend's to write) but we are her cure. Her five grandchildren response was you are insane, she is fine, now stop. You are her daily medicine; her husband is her biggest pair of have a lot of people coming. She doesn't have ALS; don't hands, biggest worrier, with a heart of gold; my husband and be ridiculous. I hung up the phone and cried so hard that sister in law the voice of reason. And as for my brother and I felt paralyzed. I became part of the club the one that no I, we are the managers of her ALS team. We conquer and one imagines belonging. It wasn't the 40 club of getting divide as all siblings should in times like these. wrinkles, getting older. It was "I'm 40 and I know my mother is seriously ill and I can't handle this kind of club.

Although one person might suffer the physical symptoms, it truly is a family disease. Dr. Lange said it best from day one I had allowed myself 24 hours to be depressed and then — nobody with ALS is the same, everyone has a different pulled an all-nighter on the computer. What would take most course of the disease. We can try and do what we can to people months, I accomplished in only a day. I was on with slow down the progression and get ahead of the disease as every personal contact and every doctor that specializes in best as we can. So, we have taken the Dr's orders and are ALS. Every person told me that we had to wait until February living with it one day at a time. We are a team, a force to be for an apt---Unacceptable! By the end of the same week we reckoned with. Mom, thank you for fighting everyday, and had been to Mt Sinai, Columbia and eventually got to the pot NEVER give up. We will be your legs, your voice and your of gold at the end of the Dr. search rainbow and are now in biggest cheerleaders in the stadium of life. Love you always. the hands of Dr. Dale Lange and his amazing staff. They too Your daughter, best friend, and biggest fan! initially told me months for an appointment. However, as I told my story to Dr Lange’s office , I calmly tried to explain to them this isn't just my mom or any mom. She is special, she is funny, she is my life.

Neurology HOSPITAL FOR SPECIAL SURGERY

Thank you for reading! Feedback is welcomed & appreciated!

Sincerely, Your Team

If you are interested in more information about our ALS Research Program, or supporting our work, please contact Dr. Dale Lange at 646-797-8917 Upcoming Events 14th Annual NEALS Meeting will be open to patients and caregivers November 6-November 7 in Clearwater Beach, Florida.

Click here for more details: http://www.alsconsortium.org/annual_neals_meeting.php 26th International Symposium on ALS/MND from December 11 – 13 in Orlando, Florida http://www.mndassociation.org/research/international-symposium/

Meet Your Clinic Team Dale J. Lange, MD, Attending Neurologist Program Director

Shara Holzberg, MS CCC-SLP Speech-Language Pathologist and Research Coordinator

Mona Shahbazi, NP, MSN Assistant Program Director

Devany Tiedeman, MS, PT Physical Therapist

Alex Shtilbans, MD, PhD Attending Neurologist

Laura Manfredo, MS, PT Physical Therapist

Dora Leung, MD Attending Neurologist

Gioia Ciani, CMT, MS, OTR/L Occupational Therapist

Lauren Langford, LCSW Social Worker Clinic Coordinator

Lorene Janowski, DPS, OTR/L, MS, C/NDT Occupational Therapist

Melissa Kaiser RN Clinical Nurse Manager,Infusion therapy

Jessica Cording, RD, MS, RD, CDN Dietician

Lindsay Kaplan, MA Clinical Research Coordinator

Connie Zayas MDA Health Care Services Coordinator

Kayan Freeman, RN ALSA Patient Services Coordinator Neuromuscular Program at Hospital for Special Surgery Department of Neurology, Belaire Building 5th floor 525 East 71st Street, New York, NY 10021