LOVE

I have decided to continue on with this blog for Kathryn. Although the blog name has a singular reference the “ME” for Kathryn in all honesty the human reference “ME” is really “US”. Most that knew us would attest to the fact that although we are two beings we operated as one in the true meaning of team or couple or whatever adjective you care to use. I chose today to write my first post because July, 8 2019 would have been our 19^th wedding anniversary.

Our story needs to continue on, my story of our life together and what life is now like being a widower. Since Kathryn left us all just about 8 months ago I embarked on a new unplanned journey in my life. To be quite frank Life Sucks alone. The wonderful group of friends we had before Kathryn left seems to have disappeared. So to keep in touch with those of you that may think “gee I wonder how Joe is making out”. I will put it all out there the same way Kathryn did. After all she had so many people following this blog that I already have a captive audience.

This will be a short post since it’s my first. There is much to be written about so be prepared and please don’t be offended by some of the things I will say.

Coming soon my next post. See you soon.

Joe

Celebrate the Life of Kathryn M Manginelli

For Kathryn’s Memorial celebration of life, she planned a New Orleans’ style celebration, and requested we bring noisemakers, Disneyland Mickey-Mouse-ears-hats, ect.

Please dress in something festive or wear something pink.

When: this Saturday, December 1, at 1 p.m.

Where: The Episcopal Church of the Advocate — 8410 Merin Rd, Chapel Hill, NC 27516

All are welcome.

Remember the fight against ALS for so many others is real and continues. Please consider helping by donating to http://www.teamgleason.org

 

 

http://www.hallwynne.com/kathryn-mary-manginelli/

 

The End of the Journey

If you are reading this blog post, it means that I have passed. I wrote it before I passed and a friend posted it for me. It was read during my memorial service.

Well, I thought that I was being smart and had my jokes all ready for this occasion. I planned to open with the following: This is a first for me, giving a “sermonesque-type” of speech, at least in a church. Well, it turns out that this is not a first. I did it once before, when I asked our Vicar, Lisa, if I could introduce myself to the new people in the congregation. And next thing I knew, there I was smack in the middle of the sermon.

I think many of you will agree that I have had strong opinions on things my entire life. Well, it’s over. This is definitely my last sermon.

Some may say that ALS has finally won. I disagree. I think I gave it a good fight, just like everything else in my life. ALS, or the Beast, as my husband always referred to it, certainly took a toll on our emotions and threw our lifestyle and future dreams for a loop. I naively thought I could handle most of what it threw my way.

I was interviewed before the ALS Walk in 2017 and I said that the loss of independence was the worst part of this disease, but I was wrong. As I write these words, I have already lost the use of my legs, arms, hands, fingers, and the ability to eat food by mouth and speak. I was willing to accept these changes.

As my ALS progressed, I realized that my fear of losing independence only meant that I was unwilling to compromise and accept help from others. I ultimately realized that there were really two things that destroyed me. The first was losing my ability to communicate with the world. Try it for a day and you will begin to understand the frustrations that many other voice-less people experience, whether it is due to illness, politics, or language. Oh, and you cannot use arms and legs to point either, because I can’t do it. I can still shake my head and move my shoulders a bit at this point.

During my journey with ALS, I met many Clarences, if you have ever watched the movie It’s a Wonderful Life. (Later on today you will have to look it up, along with all the Roman Catholics who are trying to figure out what a Vicar is.)  

The second thing that ALS destroyed in me was having to watch loved ones grieve for you while you are sitting there in front of them. I could not even offer a hug. I could not help them with their own feelings; I could only sit there and watch.

I lost my 21-year-old sister when I was 14 years old.  My father died 10 years later. The first death we all knew was coming, but it was not something I could talk about with my 21-year-old sister. What can a 14-year-old say to give comfort? My parents didn’t even want to let my sister know her own diagnosis. I am more realistic and want to know the truth. My father died suddenly in a construction accident. I was the first to arrive at the hospital because I was working in Manhattan and the accident took place in the subway tunnel being built under the East River. They say he couldn’t wait one more day.  He had to be in Heaven because the next day was Saint Patrick’s Day. My dad was proud of his heritage and loved children.

My biggest fear is dying on a relative’s birthday. If I could ask God for one favor, it would be not ruining someone’s birthday or holiday. I know that this is difficult for some of you to have to listen to, it was just as difficult for me to write. I do hope that this “sermon” will reach my siblings in particular, and have the intent I want it to have, even if it will embarrass some of you into action. It is not something I planned to add to this narration, but perhaps this setting will be a reminder of the Christian lessons we learned so long ago. I remember sitting in the church of my childhood and reading the words printed on the walls surrounding the altar. As I remember them, they read:

“They will know you are my disciples if you have love for one another.”

And to the right:

“Love thy neighbor as thyself.”

Grieving can last a lifetime – so can love. Love is a four-letter word that, in my opinion, too many people are afraid to say to each other, but I have heard it a lot lately.

I had two sisters and three brothers. We had many get-togethers, and then something happened. I believe it was dad’s death that transformed our family. I would like to suggest that we bring that four-letter word LOVE back into action. It works on my husband’s side of the family and it would be a nice thing to copy. I have tried endlessly to solve the rift between my siblings. Unfortunately, as I write this in October 2018, I have not succeeded. ALS did not cause this rift, but it might as well have, because it has the same symptoms. Incurable, untreatable, progressive and fatal. I am sorry for any pain I caused, and I hope you are, too. I think I told you that I was sorry, either in person or in writing. If I missed you somehow, please accept my apology now.

In a spirit of forgiveness and renewal, would you all please turn to a person next to you who is not related to you, and tell them that you love them.  And please join together in one of my favorite prayers:

Lord, make me an instrument of thy peace;

where there is hatred; let me sow love;

where there is injury, pardon;

where there is discord, union;

where there is doubt, faith;

where there is despair, hope;

where there is darkness, light;

and where there is sadness, joy.

– Prayer of Saint Francis 

 

Please support ALS Research.

 

The Minutaie

There are a million little things you can do for yourself every second of every day. You don’t need to ask; you simply get up and do it. But what if there were times you couldn’t get up or if you were disabled and dependent on someone else? How long is it okay to wait? I mean, think of a baby. They are as helpless as I am, and most people come to their aid quickly. But when you are an adult with a paralyzing disease like ALS, it feels to me as if I can wait for any amount of time. After all, they know that my disease is incurable and fatal. So why spend time and money on something that progresses onward to my death? It’s pennies on the dollar in healthcare.

There are times when my caregiver is right in front of me and I cannot even make a sound to let them know that I am in pain or if they themselves are hurting me. So a reasonable adult waits in pain. Babies can cry for some of the same reasons I do. It might be my only form of communication at the time.

I do have a question I would like to ask though. If we protect babies at all costs, what about adults? I have good respiratory numbers to keep me alive. So the next move is to slowly decrease my food and to give me intravenous liquids. Does it sound like I am being replaced by an infant? I mean, we are all going to die. I just didn’t realize that I would be dying by an involuntary food strike or I could have joined the IRA years ago (OK, that is the author’s interpretation of what I heard from a hospice representative.)

At what point do we decide a person’s value and who makes the decision? What makes a person worth saving? Is it the number of years they live? Is it because they do not have a rare disease and more money can be earned on their illness (WWJD)?

On April 1, 2018, I wrote a letter to the President of Duke Hospital. I didn’t ask for, nor did I receive a reply, not even a form letter. But all the surveys I get from Duke have had his name on them. Do you really think he cares about an individual response, or just tallies up the responses his survey company gets? I bet I know the answer. After all isn’t Duke a for-profit hospital?

Got onto my soapbox again and off topic – the minutiae. So who scratches your itches? Who drives your power wheelchair when you can no longer move your hands?

Who opens the door when there is no handicap button, or you can’t push it? Who moves the trash can so conveniently placed in front of that button?

Do you care? Do you notice? The Americans with Disabilities Act has been a law for more than 25 years. Do you know any part of the law? I bet you know about driving with a cell phone, though.

Minutaie…

 

(This blog  was  written  with eye-tracking)

The 10%

This is the blog I mentioned in Wonder Woman (Sept. 8, 2018), when I promised to tell you about the other 10% that I have not written about before. It may probably the give you the willies, but why bother writing if you don’t tell the truth? Wouldn’t that make you really mad. Like fake news?I asked for input from other PALS and CALS and will try to include it if I got any input or I may decide that I need more than one blog to put it out there.

When I finally stopped working, I stayed home without an aide. It was OK for a few months then I needed an aide. It was a little strange getting naked and having a stranger give you a shower. We did fine. All of my aides were professionals and most all did a great job for me. There were some clashes in personalities and I had to get rid of at least two aides who got on my nerves.

Then there is the indignity of using the toilet. Your body is the test ground. You either accept the way the aide cleans you or you have to direct them about what you want. I had to ask one aide to change gloves after I used the toilet and before my shower. I now have an aide who goes through at least eight pair of gloves in three hours. But now Medicare pays because I am on hospice. Constipation, diarrhea, and uncontrollable cramping and quick diarrhea are some other fun parts of the disease that I have will leave to your imagination, or have I already ruined the best part for later? There is also the constant hacking after eating and the constant phlegm ball you feel in your throat. PALS you may want to ask your doctor about a nebulizer if you have this problem.

I go commando because it is easier for me. Putting on pants is impossible. Skirts are now my new form of clothing. I put them on over my head once I am in the wheelchair, then the blouse is next over the head. Works for me. I also had some wonderful friends and neighbors who altered pants for the winter and made me an adaptable jumper.

I went through several types of beds and devices in order to attempt to sleep in my own bed. I already had a hospital bed waiting. I could not get comfortable and I woke my husband almost every night for some annoyance. He was still working at the time so this was a major inconvenience for him. I eventually gave up and slept in the living room in my lift chair. This worked OK for a while. I would occasionally have to call my husband on the home phone in order to wake him if I needed something. You see, I lost my voice and my husband is hard of hearing. Karma.  Several times I have had to use my iPhone call to wake my husband. Now I have a Tobii that allows me to call, text or set off an alarm.

My husband did not like the idea of me sleeping in another room so he went out and bought me a second lift chair. That is the most comfortable lift chair and it was on sale! I would recommend it to anyone. It reclines fully like a bed and gave me the most comfortable sleep.

I had to give it up when I lost a lot of upper body strength and I could no longer use the sara stedy to pull myself up. I was now begging to use the Hoyer lift to put me in and out of that hospital bed. I decided to give it a try again for the sake of my caregiver, my husband. The last straw came when I had a fill-in aide and I slid out of the chair and onto the floor. To my surprise, EMTs are not trained to use a Hoyer lift. Luckily my aide knew how to use it and helped get me off the floor. EMTs are also not in a hurry if you don’t appear injured. I still wonder how they would have gotten me off the floor if it had been an emergency.

I now sleep in the hospital bed with lots of pillows and an air pressure mattress provided by hospice. It works wonders. No more pressure pains waking me up in the middle of the night. My hubby loves that part a lot. I don’t have too many problems now that I have become the pillow lady. Pillows under my arms has also solved the neuropathy in my hands so far.

But it is a problem when I can’t activate my speech device to wake my husband. We still have problems positioning the Tobii at night. Part of the problem is that I can no longer use the remote control for the bed. I don’t have enough strength in one finger to change the bed’s positioning. Is there not one hospital bed company that can make a remote control with infrared (IR) technology? Having IR technology would allow me to and all eyegaze users to use their eye-tracking devices to change their bed positions.

Losing arm strength was a huge fear. I cannot comb my hair, brush my own teeth, clean my ears, or scratch, pick, rub, blow, or clean my nose by any methods. Forget about itching anything; it’s an impossibility. You have to depend on some one else to do these things. Wedgies are a painful blessing when you go commando and I find that a sanitary pad works wonders when properly placed. So are there any volunteers out there?

I have learned to live with a lot of annoyances. I play a game that I call mind over matter to get past a lot of these things. I want my caregivers to have lives too. I just imagine the pain away. It used to work when I had a leg cast with an itch I could not reach. There was no way I was putting something down the cast that would cause further problems.

I  am not sure if you got the whole 10%. I will need to make another review tomorrow to see if I left out any goodies you just have to know about. And I still have the cough from my 17 hour Florida nightmare trek home. Hospice nurse and priest visiting today; seems appropriate somehow.

Have a Magical Day

Hurricane Florence tried to screw with my bucket list. She caused the closure of Disney’s Hilton Head Resort. Related or unrelated to the trip, I had a PBA episode over communication issues so we drove back home ready to let ALS ruin another part of our lives. But instead of ruining the trip, Disney and our hospice representatives managed to move our trip up a few days and now I am comfortably situated in Disney’s Old Key West resort. Those lemons make for a great lemonade, thank you Disney and Duke Hospice for taking care of the big and small details.

We were able to make a short visit with friends from our former church in Hasbrouck Heights, NJ. My PBA kicked in so I didn’t have much fun and I couldn’t get in the house because as all PALs know, a flat entry is never really flat and no one wants to take off the door moulding trying to get in someone’s home.

I spent the first day hanging around the hotel. It’s harder for me to drive the wheelchair now, but we are looking at a possible solution. The second day was a great day. We met with some friends who relocated to Florida. Of course, we had to move inside because Tobii Dynavox does not work outside. Funny how that is is not part of the sales pitch.
Have any PALS come up with a solution for that?

Besides being able to visit with friends, the rest of the trip has been a nightmare for me. I am now stranded in Florida because part of I-95 have been shut down. This was supposed to be my Goodbye trip to Mickey and Minnie and it was not a memorable one. The trip was a nightmare that will not be repeated by me. I know that I should have stayed home. They say you should go with your gut feeling. I guess I should start listening to my gut and stop being so agreeable to other people’s idea of what is fun for me. I only have myself to blame for not being more forceful.

So the lesson to myself is just say no. I would prefer doing something else.

Wonder Woman

So the other night I posted on Facebook that I felt like this could be my last blog. Things are getting difficult now. Can’t get my Toby to do what I want. Can’t find a bed to operate by eyegaze (a/k/a eye tracking) or some other person who can change the remote so I can sync it with bed to make it work with eyegaze technology. I feel more tired lately, I don’t know if it is the new medication, dissatisfaction with getting my device to work according to instructions (which is frustrating), or just ALS hitting on me now.

So, I have been wondering about a lot of things lately.

1) I wonder why some people visit one time, promise to come back, and are never heard from again. When you make a promise to a dying person, they don’t forget.

2) I wonder how many people actually read my blog and how many just click like on Facebook.

3) I wonder how many blog readers have taken the time to find out what ALS really is all about.

4) I wonder sometimes how a family can be so F’ed up that they can’t say “I’m sorry,” or tell you why they are angry.

5) I wonder what it takes to have to deal with these issues when you are dying. When you are looking death in the face, you can wonder about a lot of things. Not all of it is good.

I once read a sign on someone’s property that said Friends are our True Family,. At the time it shocked me to see the sign, especially when you are leaving a relative’s home. But I saw the true meaning behind that message when my mom passed and those relatives did not show up. I know all families have their own horror stories and that mine are not
unique, but I could probably write a book about it and it would be a bestseller.

So I don’t know how often you will see a blog post from me or if they will just be shorter. I believe I wrote on Facebook that I was really only telling you 90% of my true story. So I will throw in a few teasers about my full 100% story:

A) The hospice nurse just left. We called because I was very uncomfortable and felt like I had to take a wicked pee. I felt that something was wrong with my Foley catheter. There was. She worked her magic and the urine came flowing out. If you have ever popped a zit, you will understand the feeling of relief you get. Well, that might not be the best example, but it’s all I got right now.

B) The other day I had diarrhea so bad that I had to cancel a friend’s visit. They give you some really strong laxatives when you can’t go. The laxative is better than the alternative method and believe me I’ve had it done. My husband had to do it, and he was pretty good. It put me on the right track. And that laxative – wow. It filled the bed pan to the top.  the clean up was a long ordeal.

 

C) I have never smoked but recently took up vaping! Actually it’s a nebulizer to help me get all the crap out of me that you can simply cough up

So how do you like hearing what it is really like to have ALS? It’s about the same amount of fun my husband and caregiver has everyday along with his usual daily breakdown watching me slowly slip away.

 

AML. AML, Joseph

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.