The Longest Day

lopdayI titled one of my previous blogs “Clinic Day and Advocacy” and then only wrote a short paragraph on the clinic. That was not very nice of me, don’t you think? Some of you may never have the need to visit an ALS Clinic, and that would be a good thing, in my opinion.

You probably have read some of my blogs where I have talked about what goes on there. This time I would like to take you through my visit as best as I can. As you may know I had been diagnosed with ALS on April 29, 2016, by a neurologist at Duke’s neurology clinic. It just so happened that the doctor had previously been a resident working with my current doctor whose area of expertise is ALS. It may sound funny, but I believe I thanked him for the diagnosis. You see, there is no test for ALS. I had been running around to all sorts of doctors, physical therapists, and I had even gone back to my acupuncturist for relief.

So to finally have a diagnosis meant the running around was over. I was then referred to the multidisciplinary ALS Clinic at Duke. I did not yet understand the full impact of such a diagnosis. I googled it on the ride home. When I suddenly stopped reading out loud, my husband pulled over and made me tell him what it was that made me stop. It was that one tiny detail – – life expectancy.

I have been a patient at the Duke ALS Clinic since July 5, 2016. At that April visit the doctor forewarned me that it could take up to seven months to get an appointment in the ALS Clinic. I only got in to the clinic so early by calling every other day and getting on the waiting list. My original appointment was September 20! Since then, I have participated in many different types of research, not all involved drugs. The longest drug study that I participated in was a year-long. It was a year full of clinic visits to see the research nurse.

 

 

 

I thought that the drug worked. I even went to open label on the drug which meant I would get it free for life at the end of the study. Then about two months into open label, the drug was pulled. We were told that the study failed. Later I found out that the study failed because too many patients had dropped out of the study which would skew the data and make it meaningless. In order to find a cure or even a treatment, we need more patients to take part in research.

So getting back to the subject, July 31st was my most recent clinic visit. First up was the usual intake where a nurse checks on any changes since my last visit. They always want to know if I have fallen in the last three months and check on all the medications I am currently taking. Used to be they would also weigh me at this time, but since I cannot stand and the roll-on scale does not work for me, it has to be done differently.

 

 

 

 

Prior to the actual visit, I met with a research assistant regarding the latest research I am taking part in. It is a genetic study. In this new research, I donate blood and answer questions about my family‘s medical history and jobs I have held, where I have lived, and things like that. They also test my cognitive abilities by asking questions to test my memory. I am given groups of numbers up to five digits long and I must repeat them backwards. I had been tested previously to see which form of the disease I had, familial or sporadic. Familial would have meant that I have an inherited form of ALS from which 10 to 20 percent of patients suffer. I have sporadic ALS so it appears that I am taking one for the team.

 

 

 

 

Since I am now on hospice at home, there are not as many specialists that I need to see at the clinic anymore, but they stop by any way just to be sure. Being on hospice means that the doctor feels that I am in the end stage of the disease and have only six months left to live.

The first person I saw was the respiratory therapist. This time I took the breathing test with a mask. I don’t know if that made a difference in the results, but my forced vital capacity (FVC) fell another 8 points. This is not a good thing. It is difficult for me to expel carbon dioxide. This is an important fact for first responders. It is necessary for them to know that giving an ALS patient full oxygen could kill them.

 

 

 

The physical therapist dropped in but I did not have issues that needed to be addressed. I just try to do my range of motion exercises at home with my aide. I do have neuropathy that kicks in every now and then. Mostly I feel it in my hands. I get a feeling like pins and needles or numbness. I can usually get rid of the feeling by asking someone to massage or rub my hands. I also take medication for the neuropathy pain.

A new development at this visit was that I was seen by a medical student. He checked all my symptoms just like my doctor does. ALS patients are rated on a scale, the ALSFRS. FRS is short for functional rating scale. I rated pretty low at 7. This test measures my muscle strength. Some of the questions ask about my ability to climb stairs, grip a pen and sign my name, ability to dress myself, etc. You get the point. I fail in almost all categories. Then, of course, my doctor comes in to conduct his examination and to answer any questions I or my family might have.

 

 

 

There was a visit from the occupational therapist. In the past she has been very helpful coming up with solutions to problems I have had making things work for me at home. She was also the first person to demonstrate the Hoyer lift to my husband and me. There is not much she can help me with any more because of my limited abilities. But I was very happy to show her the adaptable jumper my friend made for me. She was interested enough to ask for the pattern number and construction information. I was happy to be able to pass along the information if it might help someone else.

The speech therapist, however, has become a VIP in my clinic visits. Even though I have a speech device, the Tobii Dynavox, which I have nicknamed Toby, there is a lot to learn about it. The speech therapist works closely with the IT technician when a PALS has a device. An evaluation by the therapist is necessary if medicare is paying for the speech device. This time around the IT specialist was not available, but I did have questions about other ways to communicate when I don’t have access to Toby. I have made some phrase boards with help from the speech therapist and asked her to demonstrate how to use them. She also came in with an oversized alphabet chart that is a huge help. There are many things you can learn about and get help with if you are lucky enough to go to a multidisciplinary clinic. This is just one example. The huge advantage of the clinic is that you get to see all the specialists in one day at one location and you don’t have to change rooms, they come to you.

 

 

 

I also had blood drawn, blood pressure and oxygen levels checked, and my weight was taken by Hoyer lift. As I mentioned previously, the roll on scale does not work for me. According to the measurements taken that day, I lost 17 pounds. I thought I lost weight because my legs looked thinner, but I never thought it would be that much. I still say that I have enough fat to survive on a deserted island for a long time, but that was before ALS entered my life.

 

 

 

Other people I have seen at clinic in the past are the wheelchair technician and a representative from the ALS Association. This time out I missed the wheelchair tech, but I already had a visit scheduled at his shop for some needed adjustments. The nutritionist was not in the clinic and my weight was taken after the doctor examined me so I don’t know what the verdict will be on my weight loss until I have my telemedicine visit later in the month. The ALS Association sends representatives to clinics to see if they can offer any services to patients or caregivers.  The Association supports the ALS Clinic and has knowledge of other resources to help families affected by the disease.

Even though I did not see all the specialists, we managed to be the last ones in the clinic, as usual.  The social worker is the last person you see in the clinic. Stacey is the social worker in the clinic and she can work miracles. Nothing slips past her watchful eye. She is known as the quarterback of the clinic. She is the first person to talk to if you are having any problems connected to your condition, insurance forms, or any other needs. She can run interference for you.

This was the first clinic visit my son attended. I have told him before how much time we spend at a regular clinic visit, but I know that he was very happy to be out of there after five hours. This is how you spend your time when you have a progressive, untreatable, and fatal disease.

Some photos courtesy of and copyright by Matthew R. Reis 2018

ALS Advocacy Day

IMG_0270As my husband and I prepare for a trip to Washington, DC, for the annual ALS Advocacy Conference, I sit here and contemplate all the things that had to be planned in advance to make the trip comfortable and enjoyable for both of us. The things that used to be taken for granted are much more complex.

The conference itself is pretty expensive. Last year it cost us more than $3,000. This time we decided to forego staying at the hotel where the conference is being held. We also rejected two other nearby hotels that are accommodating conference guests. We opted for a hotel nearby that was less expensive and listed as one of the best ADA rated hotels in DC. It included a hot cooked made-to-order breakfast; my husband will appreciate not having to run around the city in the mornings. There was an evening cocktail reception which was nice.

The hotel change resulted from the reluctance of the three recommended hotels to accommodate my request for a hospital bed. Oh, my mistake, the conference hotel would allow the bed as long as my caregiver, that would be my husband, had his own room. Makes sense for a semi-paralyzed PALS (Persons Living with ALS) to be alone in their room all night, right? Maybe that was because the rooms cost $399 a night – the reduced rate!

So now that my chosen hotel was four blocks away, I knew I would have a problem when it was time to use the bathroom. So I asked the National ALS Association if they could ensure the availability of a Hoyer lift for me and other PALS. That did not happen for a variety of reasons, none of which I fully understand. So, for the comfort of my caregiver and myself, I decided to go ahead and get a Foley catheter. I am not really thrilled with another tube coming out of me. The ALS Association graciously provided an aide for the duration of my stay.

We left Friday, May 11th for Washington, DC with the van packed like the cargo bay on an aircraft. We packed the van with all of my machinery (cough assist, suction, trilogy, and Hoyer lift) and my custom shower chair. My husband also took along the pile of pillows I sleep with every night. The drive started off with me feeling uncomfortable seated in my power wheelchair (PWC) which required a quick stop to shove a pillow under my right side and remove my leg braces and shoes. We made it a little over halfway when I needed to escape the van. I took a break to tilt my PWC back and shift weight off my back side and raise my legs. A very necessary break for comfort and to keep any more blood clots from forming.

Soon we arrived at our hotel and it was time to unpack the gypsy van. The hotel staff were super friendly. Our handicap room had double beds. Unfortunately, the beds were on platforms and the Hoyer would not be able to get beneath the bed. The floor was carpeted, too, which makes moving the Hoyer very difficult. (The Americans with Disabilities Act really needs updating.) My bed solution was the pull out sofa in the other room. Hubby’s addition to the solution was to drag the mattress from the bedroom and put it on top of the sofa bed. I felt like a princess in search of a pea. The next day we rented a hospital bed for the rest of the stay. Minimum rental is a month; the cost is not pro-rated.

Saturday, two of our besties traveled from New Jersey via Greyhound to visit for almost three hours before turning around for another four and a half hour ride home. Part of our hearts went with them but it was so worth it! Just before they arrived we were surprised by a call from my cousin Mike. He drove up from Virginia for a visit just like the year before. We are blessed by friends and family.

Sunday we got down to business – the National ALS Convention. We had a meet and greet all day up until the welcoming dinner. The following day we received a lot of information relating to current research. There was a panel with a researcher, doctor, and clinician. It was exciting to hear researchers say that they feel they are close to solving the riddle of familial ALS, maybe even by 2019! Ten percent of people diagnosed with ALS have the familial form. The rest, myself included, have the sporadic form of the disease.

I spent a lot of time during the lunch break with the convention exhibitors, two in particular. They were Biohaven and Tobii Dynavox. My husband and I had traveled to DC in March to take part in a patient advisory panel for Biohaven. They were writing a protocol for FDA approval of a new sublingual form of riluzole. I am happy to tell you that I wrote a letter to the FDA in support, and it has been approved for expanded access prior to clinical trials. This means that it is available to PALS now, before final FDA approval, but PALS must ask their doctor to complete the necessary paperwork.

Later in the afternoon, we met with representatives of our local ALS chapters for a strategy session. We went over what we would be discussing Tuesday on Capitol Hill with our senators and representatives. I had a script prepared in advance that I would read with my Tobii.

Tuesday morning traffic was a nightmare on the Hill. It was law enforcement week and the President was addressing the police. There were police walking, biking, and riding motorcycles everywhere. It was a hot day and we had a long walk from our shuttle bus to the Senate office building. Every year walkers are advised to wear comfortable shoes.

We met with staffers for Senators Burr and Tillis for about fifteen minutes. Senator Tillis was tied up in committee meetings most of the day and could not meet with us. When Senator Burr arrived, we told him what our “asks” were and then the individual PALS told their stories. I was first. I spoke about my ALS journey so far and asked the Senator to support legislation eliminating the five-month waiting period for social security disability benefits. He assured us that he was in favor of the legislation and was a co-sponsor. Next we visited the House office building.

 

 

The House and Senate offices are connected by an underground tunnel. It is a bit of a walk between buildings. If you are lucky enough not to be in a wheelchair, you can ride a tram between buildings. We visited the offices of four Congressional Representatives and met with their staff people. In addition to presenting our four asks, we requested that the representatives and senators vote to approve a bill that would award the Congressional Gold Medal to Steve Gleason, a fierce advocate for ALS rights.

It was a long and exhausting day. And I violated one of my own ALS cardinal rules, not to do stupid stuff (thereby risking my own personal safety) by agreeing to walk, and not ride the shuttle, back to the hotel. Okay, I don’t really walk, but the trip back was the longest half mile for my PWC and me. When I entered the hotel lobby, my battery was at 3%. I prayed that I would make it from the lobby to my room on the 12th floor. As I entered the room, my battery read 0%. That was the same way I felt, drained. I was so tired that my husband got me undressed and put me to bed before the aide arrived at 6 pm. I slept straight through to the next morning.

ALS Advocacy is important. Next year there will be new PALS fighting for a whole community of people who are either dying or being diagnosed every 90 seconds. Please give us your support.

IMG_0284

Research without drugs

Recently I responded to an email from the ALS Association that requested volunteers to work with graduate students studying motor speech disorders.  Last week I met with four students from UNC in my home.  The students are studying speech irregularities in ALS patients.  The initial meeting was to get to know me and to learn my ALS story.  I was a little concerned that they would not be able to understand me even though they study speech disorders.  I played a couple of videos for the students so they could compare my voice in August 2016, April 2017, and currently.  We chatted for a while and then the students conducted some speech testing.  In addition to meeting at my home, there will be an in-class meeting where I will be joined by another PALS (person with ALS).  The two of us will take part in a panel discussion about our life experiences and communication issues relating to ALS.  Details of that meeting will follow.

I have also volunteered to work with a Duke student who is gathering photos for a project in her Documentary Studies class.  She hopes to show the side of ALS that only PALS and CALS (Caregivers of person with ALS) know.  She is accepting picture submissions and is also willing to visit PALS at home to take photos.  She was also looking for ALS patients willing to be photographed during a typical visit to the ALS clinic.  I volunteered and we will meet at my next clinic visit.  It should be interesting; my visit is scheduled for Halloween!

Segueing now to my personal ALS UPDATE, I can report that my arms are losing their flexibility.  It is hard to hold them over my head and it is becoming a challenge to cut some foods.  What about my hands, you ask?  Typing is pretty much the same – two hands on the keyboard, but it’s much slower.  Discrete finger movements are hard and I drop a lot of things.  Holding my electric toothbrush is OK but turning it on sometimes requires two fingers instead of just one.  Squeezing a tube of toothpaste is not always easy either.  Picking up a full glass of water is a tricky task and drinking with a straw is a necessity.

I stopped walking about 9 months ago.  My power wheelchair is my means of transport.  It gets me almost everywhere.  I was given a few exercises by my PT that I try to do more often than not.  The exercises are really for endurance because it’s not really possible to build strength.  And I am still fighting so that I can stand up and bear weight.  All things considered, I’m not feeling too bad.  I get tired faster and like to take naps.  One thing I will be working on in the near future is the Walk to Defeat ALS.

SAVE THE DATE
April 21, 2018