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

Trick-or-Treat

IMG_6625Happy Halloween!  Tuesday was Halloween and the day I chose to follow-up at the ALS clinic.  I had also requested a speech evaluation and it wound up being that morning.  It was a full and exhausting day.  It was also fun and sobering as far as my journey with ALS is concerned.

I was up at a bleary-eyed 6 A.M.  After being helped with showering, dressing, and breakfast, we were off.  We were well aware that the ALS clinic would be filled with costumed medical personnel so we packed our costumes in the back of the van in anticipation of our afternoon appointment there.

First up was my 9:30 A.M. appointment at the Duke Speech Pathology Clinic.  We waited in reception for my name to be called along with costumed kids who were trick-or-treating while also waiting for their own appointments.  After a short wait, I was summoned to a small room in the back of the clinic to meet with the IT technician and speech pathologist along with their respective interns.  I handed over my email from Team Gleason¹ whom I had previously contacted for advice on speech technology.  The technician politely handed it over to his intern.  A minute or two into my evaluation it was apparent that this Duke team did not need any advice.

I practiced using eye tracking on three different devices.  Eye tracking measures the activity of your eyes and allows data to be collected from a computer screen allowing you to type hands free (eyetracking.com, 2011).  I found the different eyetracking devices easy to understand and use.  I guess I have my 30+ years working as an intellectual property legal assistant to thank for that.  I will be getting a speech device that will use a synthetic voice to speak for me.  Right now, I can still use my arms and hands but they want me to be proactive in learning how to use the eye tracking technology.  It will be 2-3 months before I get my own equipment.

At home, I have a MacBook Pro and I use the track pad.  I was told to practice using a mouse and an on-screen keyboard in advance of an in-home trial on the three devices.  (Of course, I just realized I am not following that advice as I type this.)  It seems that everything is about saving energy.  Using my fingers to type and edit takes energy that I need to save in order to avoid fatigue.  I can also learn how to use the technology to talk on the phone.  Unfortunately, I will most likely have to give up my iPhone.

IMG_6630Next up was a trip to the ALS clinic to meet with the research nurse prior to my quarterly follow-up visit.  On arrival at the ALS Clinic, my husband and I quickly donned our Mr. and Mrs. Potato Head apparel in the parking lot.  The friendly witch at the reception desk checked me in.  While the costume fun was just about to begin, the sobering wake-up call to the reality of ALS was hiding somewhere amongst the gaiety.

 

The ALS Clinic is multidisciplinary.  This means I don’t have to travel around to see the various members of the ALS team every time I need a follow-up.  They are all located at the clinic, and I have the convenience of having them come to see me and not the other way around.  I see so many people it’s hard to remember who I saw and what we did.  Thankfully, I have the benefit of an excellent caregiver who pays attention and knows what is going on.  Electronic medical records also help.  I can go home and log into Duke MyChart to download a copy of my visit summary.

The sobering part of the visit meant I left with information on a chopped diet and pureed foods along with information on feeding tubes.  Because my ability to chew and swallow food is becoming more difficult and my forced vital capacity (having to do with lung capacity and breathing level) is slowly decreasing, it is time to consider a feeding tube.  The tube is a proactive move and does not mean that I am dying.  It means that while I can still eat a lot of foods by mouth, it takes a long time and a lot of energy.  Ironically, the energy I get from the food is already burned while I’m eating.  That means I have no caloric intake.  The tube is meant to supplement meals.  The best part is I can save my energy with tube feedings and later eat fun stuff like ice cream or Italian food.

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Staff at the ALS Multidisciplinary Clinic dressed to mimic the clinic’s director, Dr. Richard Bedlack (front center)

So in between visits by various costumed (Halloween vs. medical) garbed personnel, I also earned about oral hygiene (another problem), got my wheelchair adjusted, and discussed physical therapy.  There was probably some other important stuff I missed.  I can only thank God that between my doctor and my husband, someone is looking out for my well-being.  I’m too busy living to dwell on dying.  The trick is on you, ALS.

¹Steve Gleason played professional football for The New Orleans Saints from 2000-2008.  He was diagnosed with ALS in 2011.  His foundation is a charitable 501(c)(3) non-profit corporation.

References

eyetracking.com (2011).  About Us: What is Eyetracking?  Retrieved November 4, 2017 from http://www.eyetracking.com/About-Us/What-Is-Eye-Tracking