The Longest Day

I 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

Expanded Access, What Is It?

Riluzole has been around since 1995 as the only drug to treat ALS. Riluzole is not a cure for ALS. Its purpose, as I understand it, is to prolong the median stage of the disease for a person living with ALS (PALS) by about three months. Some PALS decide not to take the Riluzole for a lot of different reasons. Cost of the drug is one of those reasons. Drugs for rare or orphan diseases typically cost a fortune and can bankrupt a family.

I chose to take Riluzole immediately upon being diagnosed. I don’t remember what my own motivation was at that point, but my husband wanted those extra three months. I had private insurance at the time and the copay was $385 for a month’s supply. I found a coupon online at GoodRX that lowered the cost to $185 per month as long as I did not use my private insurance. Another pharmacy we tried wanted $999 for the same medicine. I can never understand how that works. It seems to me that drugs, like politics, is just another game. Maybe I am just naive.

Not too long ago I took part in a Patient Advisory Panel for ALS patients and caregivers. The panel was designed to get input on a new sublingual form of Riluzole. Biohaven Pharmaceuticals made the drug and was writing a protocol for FDA approval. Imagine that! A pharmaceutical company who wanted to hear from the end user, the ALS patient.

This discussion was being facilitated by Easy Access Care. Their brochure explains how the FDA works with pharmaceutical companies under the Expanded Access Program as follows:

Under its expanded access programs, the US Food and Drug Administration (FDA) works with companies to allow access to investigational drugs outside of a clinical trial to patients with serious or life threatening illness for whom there are no comparable satisfactory alternate therapies (EAC).

According to Biohaven Pharmaceuticals and Early Access Care, (Biohaven, February 17, 2018) expanded access is intended to be a form of treatment for “a patient with a serious disease disease or condition“ (Biohaven, February 17, 2018). EAC’s expertise allows the drug companies to focus on research and development of new treatments and drugs while they take care of the paperwork and red tape. You know there has to be red tape if it involves getting governmental approval.

Biohaven’s sublingual riluzole offers advantages over the usual tablet form. The obvious advantage is that it can be put under the tongue. For patients who have difficulty swallowing this is a huge plus. It does not require any liquid to be taken with it and it dissolves quickly. Fasting is not required in the sublingual form as it is with the tablet form (Biohaven, February 17, 2018).

In order for me to try the new sublingual medicine, my doctor had to complete forms, obtain approval from Duke Health Systems, and apply for the medication from Biohaven. My doctor handled a lot of the paperwork himself because there was no budget that would cover the program cost. This is what your doctor will do for you if he is a true ALS Advocate. I was the first, and to date, the only patient at Duke to make the request for the drug. I was interested in taking sublingual riluzole because under the expanded access program it would be free to me. I am on hospice now and they will not provide a drug that will prolong my life. So even though all of my other medications and supplies are provided free, riluzole is not. If I choose to take it, I pay out of pocket.

I was on the sublingual form of riluzole for about a month before I had to stop. I knew during the panel discussion that I was never a fan of mint, but that was the flavor chosen for the sublingual. I took the initial dose and did not expect such a strong flavor of peppermint. I pretty much hate peppermint. The first dose gave me a coughing and choking fit that lasted for 15-20 minutes. I also had the stinging and numbing that I recognized from my past experience taking the tablet form by mouth. I don’t know if I would have been able to take the sublingual if it came in another flavor. I had even resorted to adding the new form into a slurry of my other meds that are put into my feeding tube. That helped me to avoid the coughing and choking. I realized, however, that a sublingual and transdermal medication shouldn’t be taken this way. It would not be effective.

Those PALS who are peppermint lovers and currently taking riluzole, but have swallowing or economic problems may be interested in trying this sublingual version. If you are a patient at the Duke ALS Clinic will be a little bit ahead in the application process. I hope it works well for you if you decide to try it.

References

Biohaven Pharmaceuticals and Early Access Care, February 17, 2018, “Patient advisory panel: ALS patients and care providers”

Early Access Care (EAC), (n. D.), BHV-0223 “Expanded access protocol: For people with amyotrophic lateral sclerosis”

Hospice At Home

I thought about writing this blog post because I was asked what the difference was between a typical hospice where you are an inpatient and home hospice. Having had no experience with the typical inpatient setting there is not much I can say about it. I did, however, visit two traditional hospices in my area not too long ago to check them out in order to give my husband a brief respite. This is what I discovered.

The first hospice was the Hock Family Pavilion run by Duke University Health Systems. It was not easy from the outside to recognize it as a hospice. The outside looked like an older style home setting in Durham. I liked that it was set back quite a bit from the main road. When my husband and I visited, we did so without an appointment. We were greeted by a volunteer who was sitting at the front desk. She took us to see a typical patient’s room which was vacant. I immediately fell in love with the soothing yellow color scheme. The sun was shining through the window which gave me a welcoming and homey feeling. The room was large and had a sofa for visitors. The bathroom/shower area looked like it would be a tight fit for bathing with an aide. The room we were shown happened to be located adjacent the nurses’ station. A nurse was summoned and she readily answered all of our questions.

The downside to the Hock Family Pavilion is that you cannot schedule your stay. The facility only has 12 rooms available for patients. There is a waiting list for the five-day respite period. You literally are called the day before a room becomes available. I found a video on YouTube that I feel gives a good depiction of the Hock Family Pavilion and you can view it by clicking here

The second hospice we visited that day was located in Raleigh, a bit further from my home. This facility was run by Transitions Lifecare. We had used Transitions when I received palliative care. Their services at that time were excellent.

When we arrived at the Transitions facility there was no one at the front desk to greet us when we arrived. After a while a volunteer coordinator showed up and she took us back to see a room. As I passed through the door separating the front entrance area from the rest of the facility, that little voice in my head was shouting nursing home.  The only difference was that unlike other nursing homes I have been in, there were no patients out in the hallways. But this was a hospice so I would presume that patients do not typically congregate in the hallways. There was a huge nursing station which appeared to be the hub for the three or four hallways jutting out from the station.

The room we were shown depressed me. It was dark without a speck of sunlight to brighten up the room. Perhaps a twist of the window shade might have made a huge difference, but the volunteer did not make a move to do so. There was no one else associated with the facility who was available to speak to us at that time and the volunteer was unable to answer many of our questions. I was not motivated to spend any time in their facility. I was not able to find a video depicting the inside of the facility that would be equivalent to the Hock video.

Hospice at home is the other side of the coin toss. Reading comments on Facebook, it appears that not everyone has the same in-home experience. But this is my blog, so, it will be my experience you read about.

While both facilities seemed competent, my husband and I decided to go with Duke Home and Hospice for reasons of our own which included the fact that they cover most of my medications, work closely with the Duke ALS Clinic, have personnel with actual experience with persons living with ALS (PALS), use the durable medical equipment company and therapist that is at the clinic, and will defer to my doctor on all decisions that deal with my ALS. There was no chance that I would be willing to give up my doctor, a man who has dedicated his entire career to me! (I like to believe that is true, but, in fact, his career in neurology has been devoted to ALS.)

Hospice at home means that I can see a second medical team devoted to me. I can also travel to the ALS Clinic for quarterly appointments as long as I am able to do so. I have a nurse manager who visits weekly and oversees the care I receive. I also have hospice aides who assist with bathing or range of motion exercises to keep my joints from freezing up. Other pluses are that there is a hospice chaplain, social worker, and volunteer whose services I can also utilize. Of course, bereavement services are available as well.

My volunteer is wonderful and I always feel better when she is here. She has a cheerful disposition and leaves me feeling better. Right now we are sorting through photos for my memorial video. If I don’t feel up to the task, we watch TV (we like the same shows!) or do something else.

The social worker helps find resources for the things I want to do while I still can. No matter if it is selling jewelry or planning a trip, she always finds an answer for me.

Now do not misunderstand me, when we initially made our decision to go with the Duke Hospice we had a tough time getting the administration to work with us. We were receiving phone calls for all kinds of services, but we had a difficult time getting a nurse manager assigned to me. My husband finally worked it out. The nurse who was ultimately assigned is very caring and professional. Don’t get me wrong, the nurses I saw previously were equally as competent, caring and professional, but the first had handed in her resignation two weeks earlier, the second had no ALS experience, and the third was an intake nurse and had not been involved with patient care for quite some time. We have not been able to figure out how to get the hospice aides here before noontime, so once a week I have a long morning in bed. It works out okay unless I have to be somewhere that day.

To sum it all up, hospice at home can work for you if you have the right caregiver at home. My husband is my caregiver, best friend and ALS advocate. We are not perfect, and my husband deals with a lot of ALS bullshit, especially because of my issue with pseudo-bulbar affect (PBA). But he’s still here. We do not like the idea that we were given an estimate of six months left for me. That six months could come right on time to coincidence with the holidays. So in the meantime, we deal with the Beast the best we can. We hope to soar past the end of 2018.

Telemedicine

If you are reading this, you may know that I have amyotrophic lateral sclerosis (ALS) also referred to as Lou Gehrig’s Disease. I had my first telemedicine visit with my doctor last month. Telemedicine allows patients to stay at home and still visit their doctor. I had my visit using my iPad. The connection and setup are facilitated before the visit by technicians working with the Duke ALS Clinic. It’s not possible, of course, to see the multitude of other specialists that I would usually see during a visit to the clinic, but it does help to have an awesome doctor like mine.

My doctor is Richard Bedlack, and he comes to the clinic after his morning at the Duke VA Hospital where he is Chief of Neurology. Duke offers telemedicine for patients who have difficulty getting to the office for a visit. While it is not far for me to travel, I am now on hospice so this is another way to check up on me. The telemedicine offered at the Duke ALS clinic is supported by the NC Chapter of the ALS Association. You can learn more about Duke telemedicine by clicking on this link

When I visit the clinic, I am never rushed so that the doctor or any of the other specialists can get to the next patient. Every one takes the time to listen to all my questions and concerns and they take the time to get me an answer. Duke has assembled a great team of specialists to work with the ALS patients. My one concern, however, is that they do not have an ALS team available in the main hospital. As I described in a previous blog post (Blog Interrupted, March 23, 2018), it was a horrible experience in the emergency room that prompted me to write to the President of Duke University Hospital. The real problem seems to be that ALS is not a money maker for the hospital, just like many other rare or orphan diseases are not. The patients diagnosed with ALS are greatly outnumbered by diseases with treatments that are billable and can bring in a lot of cash. Telemedicine does not even have a billing code. My regular in-patient visits to the multidisciplinary clinic take place on Tuesdays. This is typically the only day Duke will allow the clinic to operate. Like I said before, it is a money issue.

I recently found out that the NC ALS Chapter did not reach its goal this year. The clinic and telemedicine services are very important resources for people living with ALS (PALS). Other PALS have organized fundraising events to help support the Chapter in its mission to help PALS and their caregivers (CALS). I am not able to do that. Instead, I am asking those of you who may have missed making a donation to my Team to do so now. If every registered participant raises $60 the goal could be met.

PALS need your help now. The cure for familial ALS is close. This will not help me, but there are so many other things that are supported by the NC ALS Association that helps PALS each and every day.

My Team page will be available until July 31. I did not join the Walk in Northern NJ this year.  We raised a lot of money there last year.  If my Team Kathryn North donates, we can help make this a reality.

Let’s do this! Click here to donate to Team Kathryn.

Bitching & Complaining

Yes, you read that right. This blog is about bitching and complaining. It is mostly about ALS, the bitch in my life, but a few other things may slip in. I am after all a born and bred New Yorker, so it’s a given that I am going to complain. If you don’t like complaining, don’t read any further. I am not about to change now. There are lots of things to complain about with ALS, and I’m taking the opportunity to put it all out there.

Disclaimer: These complaints are not directed toward any specific person, but even so, names will be changed to protect the guilty.

Communication
This is a big one. It may actually be the only real issue because not being able to express even the smallest desire is a huge frustration.

I live with my husband who has hearing loss and wears hearing aids, most of the time. So communication has always been an issue. I was always being reprimanded for talking to him from another room instead of face to face. That was a difficult thing for me to get used to since he was the first hearing impaired person I really knew. Heck, in my childhood home we would yell to each other from one floor to another. So, of course, it’s Murphy’s Law that I should lose the ability to speak and make our communication even more difficult. I spent about six weeks reading 1600 nonsensical phrases so I could have a synthetic voice that would sound like me. I now have a speech device that Medicare paid $16,000 for, and guess what? He can’t understand my synthetic voice on it. Murphy, again.

We used to say that as we got older, we could learn sign language to add to our ability to speak to one another. Damn you, Murphy. I can’t use my arms and hands very well.

I have my speech device which is a Tobii Dynavox. I just call it my Toby. I took it with me to the hospital where no one had the time to wait for me to type my responses. So I was essentially voiceless. I take the effort to practice so I can be faster at communicating and there is always something to annoy me. For example:

1) Toby quits in the middle of a sentence I have been painstakingly writing;
2) People are on to another discussion before I can fully respond;
3) As I type sometimes people will read the screen and try to anticipate what I am saying (sometimes this is OK, but it takes away privacy of prior conversations because of its predictivity function. I really don’t want people to see “I need the bed pan” when I was writing “I need some help.”)
4) I attempt to use the dwell-free keyboard which is faster and doesn’t appear on the screen until I want it to, but if I make an eyegaze mistake, it takes longer to correct and, therefore, longer to speak;
5) People don’t really understand what Toby said, but don’t bother to ask for clarification. To me this seems like they are not interested in communicating.

The worst times for me are when I am asked questions that require more than the nod of my head, which I can still do right now, and Toby is packed away or out of my sight. If I notice something important or need something, I am helpless. These instances occur frequently during bathing and dressing.

Sensations
Constantly feeling itchy is an extreme annoyance.  I cannot reach very far so lots of these feelings must be ignored. Mind over matter, or in this case itches, does not always work. I can feel loose hairs on my skin like bugs crawling on me. My pleas to remove them bring snickers from Barney. A real annoyance.

Burning sensations from pressure occur at times. I feel the burning in my feet at least once again daily. I used to experience burning in the heels of my feet almost every night. Fortunately, this situation seems to be under control with the use of many, many pillows. In fact, our bedroom has become overrun by pillows.

PBA
PBA or pseudo-bulbar affect is the nemesis of my relationships. I know many people are put off by it. It is hard on me and hard on people who are witness to it. I was never an emotional person and now I start crying at the drop of a hat. Sometimes it’s because I’m upset or frustrated. At other times I’m just overwhelmed by the emotion of the moment. Thankfully, medication has helped control it a lot, but a little bit lingers behind. It makes it hard to breathe. I hate it!

Relationships
Where have some of those old relationships gone? I’m dying and you can’t say you’re sorry? Did I piss you off? Let me know if you want to reconnect. I can apologize too. I will be dead a long time and I won’t be back.

Hospice
I have now signed up for hospice. It should begin this week, right in time for my trip to Washington, DC, for ALS Advocacy Day. Hospice isn’t a prison. They actually expect me to live my life before it’s over. How long I will be on hospice will depend on my respiratory system. While on hospice (which is at home by the way) I will get a weekly visit from a hospice nurse and 2-3 CNA visits a week. I will also have a case manager. Medications will be monitored and provided through hospice as well. When I travel, I get information on a local hospice to contact, if necessary. If you have ALS, look into palliative care or hospice, and find out when it is appropriate and how it works with the insurance you have. It can give your caregiver some help.

Getting hospice on a predictable schedule seems like that will be my next complaint. Can’t things be planned in advance? Don’t call at 3 pm to come give me a shower. I like to plan things ahead. Most of my funeral plans are finalized. There’s just one date I can’t schedule. So get with the program, already! Please and dammit!

Blog Interrupted

Things have evolved here at home.  I’m sleeping back in the hospital bed as a concession to my caregivers.  They did not ask me to do it, but since I’m the one dying I did not want to kill them in the process.  I said goodbye to the Sara Stedy for transfers because I was losing the upper body strength necessary to use it.  It was getting too difficult to get out of the lift chair safely.  I’ve transitioned over to the Hoyer lift and it’s not bad, not that I ever thought it would be.  But I know there were people out there who doubted I would willing convert.  As I felt my strength leaving, I was begging for my caregivers to use the Hoyer.

Washing and dressing are the longest part of my day.  Sometimes I need a nap afterwards.  Dressing is done in bed now.  Basketball pants with snaps up the outside of both legs and skirts are becoming my new apparel.  I’m still fighting against going commando so I’m searching for someone who wants to help me adapt clothes.  Since I know how to sew, it may turn out that I will have to teach my husband how to make the adaptations.

My husband is now on a leave of absence from work.  It turns out that he is my only untrained caregiver allowed by law to operate all the equipment I need.  I won’t go into the absurdity of that.  His awesome co-workers have donated almost six weeks of time to him; something I think is amazing.  I am anxiously awaiting warmer weather so we can travel the neighborhood walking trails.

My community has a volunteer organization, Woodlake Cares, that helps neighbors in need.  They do not perform personal care though.  One day while I was out on the trail I met a friendly neighbor Suzanne.  Suzanne  slowed down her pace to walk along with me and my aide.  She wanted to learn about ALS and me.  That chance meeting spawned a new volunteer group that joined with my church community to form Woodlake Cares 4 Kathryn.  Awesome and amazing, isn’t it?

Also amazing to me is the help I have been given by Team Gleason.  As I mentioned in a previous blog (Trick-or-Treat, Nov. 5, 2017), Team Gleason is a charitable foundation formed by Steve Gleason who played professional football for The New Orleans Saints from 2000-2008.  He was diagnosed with ALS in 2011.  The mission of Team Gleason is “to generate public awareness for ALS, raise funding to empower those with ALS to live a rewarding life, and ultimately find a cure” (teamgleason.org, 2016a.).

In the evenings when my husband removes his hearing aids, it is even more impossible to communicate.  I was looking for a floor stand for my eye-tracking device so I could have it bedside to communicate with my husband.  New stands cost upwards of $1,200.  My husband searched the internet for a used stand and found one for $950.  I told him to hold off on purchasing it.  I wanted to post in one of the ALS forums I joined on Facebook to see if anyone there had one to offer.  I quickly received responses.  A few people told me their devices came with both wheelchair and floor stands; something I was told was not possible for me.  Then a message popped up from Team Gleason.  They offered to send me one free.  Two days later it arrived.  Brand spanking new, no red tape, and no strings attached.  Unbelievable!

I was also having trouble transferring to my wheel-in shower chair.  It cost me almost $600 when I bought it.  (Why so pricy?  Duh!  Because you are handicapped and need it.)  The transfer from the Hoyer lift was making it difficult to get seated properly.  My husband was overwhelmed by the multitude of chairs available for sale.  How did we know it would work?  Was it a good quality?  They were not returnable.  He decided to call the experts at Team Gleason for advice.  After all, they organized Team Gleason House which was planned specifically “for Innovative Living is a new residential facility designed to help people diagnosed with incurable neuro-muscular disorders live more independently” (teamgleason.org, 2016b.) so we knew they had the knowledge.  His email was returned with a request for his phone number.  Two minutes later he received a call.  They understood and offered me a custom-built shower chair.  Again, no red tape!  How do you thank someone and their foundation for their generosity?  You SHOUT IT OUT HERE for the world to read.  Thank you Steve Gleason and Team Gleason for your generosity.

Blog interrupted.  I was interrupted writing this blog post on March 15th when I had to suddenly go to the ER.  I had planned to meet with a funeral director that day to arrange  my final plans and continue living.  But my aide noticed how swollen my left leg had become.  A quick call to my doctor sent me to the ER.  Once I left the house anything and everything went wrong.  I was triaged and then called in for a sonogram of my leg to check for a blog clot.  Oops, you’re in a wheelchair!  They didn’t want to inconvenience me with a Hoyer lift, picking me up to be put on a stretcher and then picking me up to go back in the wheelchair.  So it was back to the waiting room.  Waited some more and got called again.  Oh, now it was decided that they could perform a sonogram from my wheelchair.  Amazing!  The wheelchair had the same ability to recline as earlier.  Guess they just figured it out.

We had arrived around 11:30 a.m. before the crowds, but it took several hours to get a room in the ER.  Unlike other hospitals I’ve been in, Duke has actual rooms in the ER for patients.  Once you’re in the room you get the feeling you are locked away from everyone, abandoned.  I hadn’t taken any of my medicine that day and no one was in a bother to get it for me.  I guess the ER  staff was busy, but doing what I have no idea.  It took two requests to get a head pillow.  I was put on the ER stretcher where I was left for 8 or 9 hours.  It was like murder.  My sensitivity due to neurological problems and the inability to move kicked in and it felt like my buttocks was burning. I cried and screamed for relief; my PBA was in full swing and I let it rage.  Instead of simply moving me, I was offered morphine!  Morphine would slow my respiratory system.  How could it be that no one understands ALS in a hospital with a renowned ALS Clinic!  You would think a neurologist would be called in to consult for a patient with a neurological disease.  That only happens on TV.  Finally I got two more pillows placed beneath me.  That solved the problem.  Morphine versus pillow.  Smh.

Around midnight I finally got a hospital room, but only because my husband went home to get my Trilogy machine.  The admitting doctor had never heard of Trilogy and said I would need to go to the respiratory ward which had no bed available until the next day.  Not happening!  So off my husband went to get my machine from home leaving me under the watchful eye of a friend who had joined us and knew her way around the medical nonsense.  About ten minutes after arriving at the valet my husband learned that they had killed our van’s battery by leaving the transmission in neutral and the lights on.  Now he needed a jump at 12:30 a.m.  On his way back to the hospital, our friend had to call to tell him the hospital pharmacy did not carry one of my ALS-related medications.  (Again, this is a hospital with a renowned ALS Clinic.)  So again he went back home.  At 1:30 a.m. we kicked our friend out so she could rest up for her next day at work.  It was an exhausting day.

The good news in all of it was that the staff on the hospital floor are excellent.  The nurses and nursing assistants are great at what they do, although I was their first ALS patient.  The doctors did not know much about ALS so if you cannot speak, you had better have an advocate with you.  Communication for me came down to shaking my head.  No one has the patience to wait for your speech device.

In the ER with my friendly Angel Eileen

My FU to ALS

In the comfy bed

The final result is that I have two blood clots, one in my left leg and one in my lungs.  I will be on blood thinners for life.  FU ALS.

References

teamgleason.org (2016a.).  Team Gleason, Retrieved March 14, 2018 from http://www.teamgleason.org

teamgleason.org (2016b.).  Team Gleason House, Retrieved March 23, 2018 from http://www.teamgleason.org

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

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Trick-or-Treat

Happy 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.

Next 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.

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