[Nancy L Worden]

When my calendar finally completely crashed in early March,  I made the decision that I was going to take this time off to rest and heal. Last fall I moved my business into the bottom level of our house. I was so focused on the move that I forgot to do my physical therapy exercises. The repetitive motion of getting up out of chairs and off the toilet created a weakness in my pronator muscles in my arms. I went to physical therapy for a few months and then it happened again. It wasn’t until my calendar was completely blank that I had time to figure out what was going on. I’m sharing this because someone else out there might be having similar struggles.

The most important part of the healing process is figuring out what activity is causing the problem, and it may be more than one. I can pull myself off of the toilet without my shoes and leg braces on, but when I add the leg braces and shoes it adds a lot of work for my arms. I realized that we needed to elevate the toilet seat, which is part of a bidet. My husband found a commercially available bidet riser and we ordered it but when it came, it was too tall for my ADA toilet (4”). Fortunately I had some 3/4inch plastic stock that we could cut strips from. Little by little we raised the seat one, two, and finally stacked three strips for a total of 2 1/4 inches. Then my husband drew a pattern that fit each side of the bowl, shaped like parentheses, and sent the design to a machinist we know. He is now producing more strips and hopefully the toilet problem is solved. If you want to bypass the machinist, try cutting hard foam with an electric knife to the shape and thickness you need.

The next thing I did was begin my exercises that were given to me by a physical therapist when I broke my arm 18 months ago. Fortunately my husband came with me on my appointments and recorded the exercises on my phone. I had digital recordings of my exercises for my broken arm and for the pronator problem. I reviewed and organized all of them and labeled each exercise with a name and a number. I am now giving each exercise a nickname because the names my therapist gave me are often redundant. I put them in a file on my desktop and when my husband helps me with the exercises, we can pull it up on my iPad. This is so much better than trying to interpret handouts they give you at physical therapy. The little recording shows me what I look like when I’m doing the exercise and I also get the benefit of hearing the coaching my therapist is giving at the same time.

I am now into my fourth week of the exercises, increasing them in baby steps. I need to work up my strength gradually. To keep track of what I do I started a PT Journal of the exercises every day. I’ve also been taking a lot of hot baths to warm up my muscles before I start the exercises and I skip a day between workouts. This process has required a lot of patience and trial and error and there’ve been times when I’ve been very frustrated. But the gift of the quarantine was the quiet time to sit down and think through what was going on and how to fix it. When the shelter in place order is over I will also go see my massage therapist and maybe my physical therapist. In the meantime I am  gaining better understanding of each exercise and improving on my own.

[Nancy L Worden]

Hi Dagmar; In response your suggestion  of contacting ALSA about accessible hotels, that might be a good start but they will only have a list of hotels with accessible rooms. What they won’t have is a customer review of those rooms. I think what we need is a yelp- type app where we encourage PALS to review the places where they stay so that other disabled people can read the review. It is possible something like this already exists, does anyone know of one?

[Nancy L Worden]

Hi Cate; I travel with my rollator a lot and I have learned that it is best to check it at the gate not with your baggage. This means that when you get to the gate you need to tell the ticketing agent to put a  claim tag on the rollator. They will give you a claim check and the important part is to remember to pick up the rollator near the door of the plane as you’re getting off. I usually ask the wheelchair pusher to take me into the airport and then  send them back for the rollator. Hopefully your rollator has big wheels so you can use it on uneven terrain. I use the Volaris rollator because the brakes are easy to engage and disengage. Most PALS do not have strong fingers and so the kind of hand brakes that are common on the Drive rollators don’t work for me.  Also  the Volaris is made out of plastic and aluminum and will double as a shower chair.

[Nancy L Worden]

 

What exactly does accessible mean?

Those of us who have some physical limitations are often looking for an accessible hotel room or restaurant. What I have learned over the years is there is no standard for this term and no policing of its use. The ADA has been federal law for almost 30 years and many public facilities are not in compliance. The best way for me to describe what noncompliant means is to tell you a few stories from my personal experience.

I have been participating in a drug trial in San Diego for going on 4 years now. When I first started I was one of the first patients and so they gave me a list of hotels in the area of the clinic. One by one I tried all of them. I found that the rooms at a very fancy Hilton Hotel had a disability height toilet but the handles for the sink were so stiff I couldn’t move them to turn on the water. The door to the room was very heavy and had one of those springs that makes it very difficult to open. When I complained to the management, they told me that I seem to be managing just fine. They would not remove the spring and the maintenance engineer would not address the handles to the faucet. This same hotel, the Hilton at Torrey Pines, once put me in a regular room and brought in shower stool thinking that would suffice in making it accessible. There was nothing to hang onto in the shower. When I complained they gave me a coupon for a free breakfast. I don’t stay with them anymore.

The hotel where I stay now is an Embassy Suites Hilton, La Jolla and the staff is very accommodating. There is no spring on the door. There is only one chair in the suite that’s high enough for me to get in and out of but at least there is one. When I check-in someone always helps me drag my bag up to my room and make sure that the shower is pointed in the right direction. The microwave is too high for me to reach but if I call the engineer they will move it for me.

A couple times I slipped out of bed and called down to the front desk and they sent somebody up to pull me up. I feel safe in this hotel and they listen. Unfortunately they only have two accessible rooms with two beds. One of the rooms has a bathtub with some bars and they call that accessible. I cannot get in and out of the bathtub.  The other room has a roll in shower but it’s always reserved because there’s only one. That means when someone comes with me they either have to sleep on the couch or in a child size rollaway bed or with me and some people are not comfortable sharing a bed. It is one reason that I traveled by myself to San Diego for so many years.

Another example of a supposedly accessible place is IslandWood where my daughter’s wedding was last summer. She intentionally chose a venue with accessible accommodations. Knowing the variations of the term accessible, my husband and I went there to check it out several months before the wedding. What we found was a toilet in our accessible room that was too low, beds that were too high, many gravel paths, some of them steep, and door openers only at the welcoming center. They had also recommended that I use their golf carts. I tried to get into one of their golf carts and it was impossible as there was nothing to hold on to.  I walked all over the paths, many of them hilly, with my rollator to make sure that I could walk it. My rollator was also absolutely necessary where the ceremony was performed in the grass.. We decided we would bring my toilet riser but when we got there I was too tired to walk all the way back to the welcome center to find someone to take off the toilet seat so we could use the riser and my husband was not inclined to do so. So, every time that I used the toilet that weekend, any of their toilets, I had to have someone go with me to help pull me off. I couldn’t get into bed by myself and the floor was so slippery in our room that I slipped and fell on concrete. I made a special trip out there and was very clear about the things that would be difficult for me and the only change they made was to prop the door to our lodge open so I could get in and out. However there was a ramp to the lower level of the dining room, one good thing.

The typical disability bathroom in a public facility is a wall mount toilet mounted very low to the floor where they have added bars on either side or on the side and in the back. This is normal even though many people cannot push themselves up from 10 inches off the floor. My favorite example of accessible gone wrong is the accessible toilet on the D concourse near gate D3 at SeaTac international Airport. Some brilliant designer decided to mount a diaper changing table right over the accessible toilet with very little room for fingers between the table and the bar at the back of the toilet. When I got stuck in that one I had to call for someone to come and get me out. Afterwards I was so angry I asked a ticketing agent who to complain to and I was told to complain to the Seattle Port Authority. I sent them an email and I had a couple of responses and nothing’s happened.

My favorite experience at SeaTac was with Horizon Airlines. As usual when I booked my flight I asked for help from the gate to the plane. When I got there on a cold February morning, the first red flag was that the wheelchair operators were all young people and they were looking at their phones. I made a point of connecting with the ticketing agent to make sure he knew what I needed. When they called my flight, a young woman came to get me and she took me outside to an elevator and then out on the tarmac in 38° weather and rain. She pushed me to the base of a long ramp with switchbacks and told me okay get out and walk up. I said no, I can’t walk up there and so she hailed a baggage handler and he was kind enough to take me up this complicated and very steep ramp. When we got to the plane there was an even steeper short ramp into the plane. At this point he grabbed me under my armpits and dragged me backwards into the plane.

After I composed myself, and had something to eat, I went to find the restroom. The seat look pretty low but I really needed to use it. Then I tried to get off of it and there was no way. The only thing I could do was kick the door and yell help and fortunately the flight attendant came and pulled me off the seat. By this time I was in tears and very grateful to the guy sitting next to me who leaned over and said enthusiastically, “You made it!:” I survived the flight and when we got to San Diego, the flight attendant told me to wait to get off last even though I had a first-class bulkhead ticket. So I waited for everyone to get off and when all the weight was off of the plane there was a substantial distance between the plane and the gate so once again they wrestled me off the plane into the wheelchair.

I was so angry after that trip that I called Alaska Airlines and complained to customer service. I talked to a very nice guy who listened but couldn’t do anything else except give me some bonus miles. The moral to the story is even if they say it’s an accessible flight that doesn’t mean that it is. Avoid anything smaller than a 737 and drain your bladder before you get to the airport.

 

 

 

[Nancy L Worden]

Five or six days a week I do a 10 minute morning workout. This includes leg lifts, breathing exercises, and other  stretches I’ve learned in physical therapy over the years. Two to 3 times a week I ride my reclining trike either outside or inside on a trainer. This helps to keep my legs on lifting. Some days I do  standing balance exercises and at least twice a week I do stretches  and strength building with my arms. I’m really careful with this and if my arms are fatigued I only do one or two at a time. Walking is also important for me and I use a Volaris rollator. The walking helps with balance and stamina.  The more I walk the better I walk. I find if I sit around too much my muscles get stiff and weak but at least one day a week, I try to have a complete day of rest. On that day I take a hot bath and sit in my chair. If I skip my one day of complete rest, I really get worn out the following week.

I take some supplements and I eat a high-protein diet. I sleep with a BiPAP. I am also participating in the  Biogen Tofersen drug trial for the SOD1 mutation. I have been in the drug trial going on 4  years now and recently received permission to be dosed every two months instead of every month as a travel to and from the clinic was wearing me out. I have seen some improvement with this drug, primarily with my breathing.

[Nancy L Worden]

Hi Amanda; I  have the SOD1 mutation and I also have the slow kind. Since May of 2017 I have been participating in the BIIB067 (originally  IONIS-SOD1 Rx) drug trial in San Diego at UCSD. I completed the phase 1 part of the trial in 2017 which was a double-blind and I’ve been participating in the continuing trial since February 2018. The continuing trial is open label which means that I know I’m getting the drug. It took them several months to ramp up my dosage from 40 mL to 60 mL and finally 100 mL of the drug which I receive once a month in a spinal tap. Since I live in Seattle, it is a journey for me to go to San Diego every month, though it’s better than going every week which I did for seven weeks during phase 1. Mostly I go down there by myself and I am not going to say that it’s easy. I have enormous respect for John Ravits and his staff and that is what has kept me going. Ravits is the principal investigator for the drug trial and the anti-sense technology was developed at UCSD. Ionis and Biogen combined their efforts for phase 1 and last December, Biogen had seen results significant enough to buy the technology from Ionis. The drug is now being  referred to as Tofersen.  UCSD is also participating in an anti-sense trial for the C9 mutation and gearing up for the same with Huntington’s disease.

The anti-sense technology is a manufactured mRNA designed to bond to the mutated gene and prevent it from producing the protein that causes the symptoms. That’s a very simplified explanation.  Last December when Biogen decided to exercise their option to buy the SOD1, drug I was told by Ravits that the drug was working best with patients who have the fast kind of SOD1 mutation. However, he also said that almost all the patients participating in the trial had shown some improvement. Drug companies never share the individual data they collect during the drug trial. I will never know if I got the drug or the placebo in phase 1. However, when they discovered the advantage to the people with the fast kind, they immediately started putting together a special trial targeted  for those patients.

The good news perked me up even though I didn’t know if it was helping me personally. What I did notice was that the travel was hard on my body. As far as the data that they collected from me I didn’t notice any improvements but I also noticed that their equipment for collecting pulmonary information was not as sensitive as what my pulmonologist in Seattle works with. This drug trial is taking place in 17 venues around the world and they all use the same equipment. If there’s one thing I have learned these past three years, it is that a drug trial is about collecting data,  not treatment. Quite frankly, I quit worrying about the data but I do share the scores from my pulmonary tests that I get in Seattle.

Recently I saw my pulmonologist, the one who prescribes my BiPAP, and it was his opinion that my speech and breathing had showed more improvement than just what I would normally get from using the BiPAP. In other words he was telling me that the drug was working for me.

I was ready to quit the drug trial but now I am rethinking it. Participating in the trial has taken a lot of my time and energy and it has cost me financially because I can’t work very much on the weeks that I go to San Diego. The symptoms of the drug aren’t too bad but the symptoms from the spinal tap and the travel are exhausting.  It would be better if I had someone to take care of me while I’m down there and I have hired someone to help me the day after my dosage as I am often too tired to pack my bag and dress myself. I do not have a family member that can travel with me but my nephew lives in Los Angeles and he has come down several times to help. The drug company compensates patients for travel, hotel and food however they  enforce limits and I pay whatever costs exceed the limits.

I would be happy to answer any questions that anyone has about this particular drug trial. I have never regretted participating in it  and I have learned so much more about ALS and SOD1 than I knew before.  It has been hard to stay with it and 31 spinal taps is no small deal. What I like is being so close to but it’s happening and things are exploding in ALS research. It is a very exciting time.