How to Make an Invalid

I see I have not posted anything on my progression of my tangled nerves since September 2018. There are changes, such as to my sinking face. Do you think I'm cute? Look at these dimples, except these aren't dimples, they're sink holes.

I mentioned this to my wife not long ago saying my cheeks were sunken. She claimed she couldn't see this, but my friend, Ronald, noticed right away as we connected on FaceTime. Not that this particularly bothers me. I just don't want to see my bottom four teeth pultruding out over my upper lip as Stephen Hawking's teeth did.

The reason I m posting again is that last Friday (May 3, 2019) I was back to the ALS Clinic in Philadelphia. Actually, I hadn't been to clinic for a while. I was originally scheduled way back in February, but I canceled that one because I didn't want to chance driving into Philadelphia in bad weather. I then changed the date again until March, then April, , but the hospital rescheduled me to the May date.

At least I figured I escaped any snow or ice, but it still proved a miserable date for he drive up I-95.

The weather people had claimed all week that Friday would be nice, warm and dry, so I dressed in an outfit of shorts and T-shirt. This proved less than an ideal choice. The morning was in the 50s, gray and drizzly. Fortunately for me that exam room wasn't cold.

If you have forgotten how this clinic works, there are doctors of differing disciplines that parade through over a three hour period to question, prod and test me. All they are doing is measuring me against my prior clinics to see if the ALS has progressed. There is no treatment they can give me, no cure, just my progress report and off-hand advice.

I know the disease has progressed because I know my body. I've lived closely with it for a long time. It will be 78 years in June. I can tell I have weakened, that I grow fatigued quicker and I drop things more, and there is less and less physical chores I can do. But I am still around for them to thump and thud. When I was first told what I had I read that the usual life expency was two years. I've beat that. It has been three years at this point. This is why I resist what the doctors tell me to do because I am not ready to be turned into an invalid quite yet.

So let's look at some of the tests and the advice.

The first doctor to knock on the door and enter was Brittany, the pulmonary person. Brittany is very nice for a person carrying a couple of torture devices in her bag.

You can see her holding one test instrument. In her plastic bag is the other. I am not sure if both are called Spirometers or not.

The one she is holding works thus. You take in a huge breath, then wrap your lips over this thing and blow as hard and long as you can.

The other instrument is similar, except this time you exhale all the air in you, pop it in your mouth and suck as hard as you can. You do each test three times. I don't know what anyone standing outside the door is thinking as she roots you on for each test, shouting, "Blow, blow blow"; then "suck, suck, suck."

Oh the most uncomfortable things about this is the great nose clamp she puts on your nose. Also,
when you blow there is no feeling of resistance. It is a very strange feeling.

On the first test I was the same as last time, but on the second I was below last time, so I think this means I can not suck in as much air as before. She again is pushing I use my Trilogy Ventilator more, and I promise I will try.


Others who came in either asked questions. The Dietician was happy because my weight had stayed consistent at 178 pounds. I wasn't. Three years ago I weighted 160 pounds and had worked very hard to get down to that. I had also build up up muscles back then. Now I can't do anything to build up my muscles, but I can keep my weight up and develop a "Buddha Belly".

Two social workers talked with me. They will follow up and see what kind of help Lois and I can get.  Lois wasn't thrilled with this, but we need some kind of help.

Near the end came the Physical Therapist, Occupational Therapist and I am not certain about the other guy. I didn't see the speech therapist this time around. It is these Therapists I resist. I don't know why the rush to turn me into an invalid, that status could come later.  This trio do a lot of pressing and pushing of my arms, hands, legs and feet.. The one does a bunch of tapping with his little hammer as well. They seem to have not found a lot of change in my strength, yet they want to alter me a lot.

First, they want me to forsake my trusty walking stick and go to a walker. They may want it, practically demand it, but I don't. It is awkward. It is harder to put in the car, since you fold it up and have to find space in the trunk, then pull it from there, open up and hobble on like an elderly man. My walking stick I can just toss in the back seat with ease. When we go to a restaurant I put the walking stick across the bench and sit on it. It is then out of everyone's way. This walker can't be handled like that. It can be folded up, but will sit along the isle in people's way.

It is also more difficult to maneuver steps with it. I am more likely to trip on the walker and fall than anything.

Then they said I should continue my walks, but only on the paved paths. What fun is that?  I am irked enough I had to stop walking in Brandywine Creek State Park because I can't do the hills and trails there; I can't see not walking up along the meadows at Bellevue, and of course I wouldn't be able to do that with a walker.

I failed the push test. In this one of them places their hands on my chest and I am to press against them, then they release and I stumble forward. They do the same with my back. I told him on release I might have ended up out in the hall when he released. Whoops, should not have said that, although it was blatantly obvious. He pointed out if he had did these presses with Gabby (the other therapist) she would have only taken one step in each case and been in balance.

They said the last time I was at clinic I told them  I had fallen three times. I don't think so. Unfortunately, I had told about the two falls I had this year. Now they began talking of putting me in leg braces. I have one leg brace that was foisted upon way back in the beginning and I've never used it. It was called a toe-lift brace, but I am lifting my toes so far, not dragging hem.

The worst of all was when the main guy suggested I take a Senior Supplementary Driving Test. I

managed to get them to wait three months until the next clinic to decide. They asked if I thought I could still drive safely. I said, "I drove here on I-95 at rush hour in a drizzle. I made it. I think I can still drive."  I don't want them to take away my driving. I don't know what we would do then.

Now one of the final things I did Friday was join in ALS research. Every time I go now they will take urine and my blood. As much as I hate needles, I'm willing to do it. I also signed the paperwork to donate my body to the research department. There are things they can only examined on the deceased, such as biopsies of the spinal cord and the brain. If I can help them find the cause or cure, great. I'll be done with my body anyway. Better they have it than it get buried somewhere and my family get stuck with funeral costs.

Now to go on and still try to out live my dad. He died at age 94. I still think I can beat that.

Visit of an Old Nuisence During the Week of My Clinic

Every three months I go to the ALS Clinic where a slew of Doctors and medical practicioners come to question and prod me. It is at Jefferson Hospital on Walnut Street. That is in Philadelphia in case you don't know. There is a parking garage a block away on 9th Street that belongs to the Neurology Division.

For some reason, perhaps related to my condition or perhaps related to an accident my wife had witht he car last month, I was very nervous about the drive. I don't know exactly why. I worked in the city for many years and we lived there in the University City area for a while. I have driven the city streets many, many times. In recent times I even delivered my friend Ronald to downtown when he stayed for a week.

I couldn't get a driver so I drove my wife and I there. We had no problems.

The photo was taken of Walnut Street from the examination room I was in while I waited between doctors. I just found it humorous to see a police vehicle that was a Smart Car.

For some reason, about a week before my appointment, an old adversary reappeared. My psoriasis

flared up something fierse. I have had this scourge since I was in junior high school, but in 2012 when I began taking Methodrexate for my arthritis it basically disappeared. They use the same medicine for treating psoriasis. It is also used in chemotherapy of cancer, but it stronger doses.

When I saw flared up, I mean expoded. I haven't had it this bad in 30 years. My whole body was covered and it itched something awful the first couple of days.I'm praying it will fad away soon.It's disgusting. The itching has generally calmed down to tolerable.

I was supposed to have an allergy test. I have been having problems with sinusitis. I generally got some sinus troubles each spring, but then it would clear up by summer. Last year it didn't clear up and was pretty miserable. That was when I was at the doctor's in June and he gave me the antibiotic causing me to have C. Diff and a couple hospital stays. The stuffed up head and runny nose and constant cough returned for an encore this spring and persisted all summer. It also made me very hard of hearing, so I finally went to an ear, nose, throat clinic.

At first they perscribed the same antibiotic that did me in a year ago. I refused to take it, so they gave

me a compound I mix in saline solution and squire up my nose. It goes up one nostril and comes out the other. It did help. I am not hacking or sneezing and my hearing improved. The doctor there also cleaned a lot wax out of my ears. I was given a hearing test and I do have some hearing loss, but frankly, I feel I'm hearing fine now. It was there an alergy test was suggested. However, when I came for it I couldn't take it. I didn't have enough clear skin to administer it upon. Instead I must get a blood test, which I keep forgetting to get.

Hard for me to get up and go these days because of the progression of the ALS. They say I'm doing well, but I have so much fatigue. It is difficult to keep my Blogs up, including this one, because of the fatigue. I am getting very tired already just typing this. Some of this is probably due to my breathing capacity. My FVC is at 48% and my NIF is -17.

FVC stands for forced vital capacity and NIF is negative inspiratory force. They are primary measures of respiratory muscle strength, capacity of air sucked in and then the ability to blow it out again.

Measure forced vital capacity (FVC) and negative inspiratory force (NIF) immediately and every two to six hours thereafter. FVC and NIF are primary parameters to measure respiratory muscle strength, especially in patients without obvious respiratory distress. They have a device for checking these and when i took the tests it sounded very pornagraphic. The Respiratory Doctor, a female, was cheering me on. The first phase is done three times and she stood over me yelling, "Suck, suck, suck." The second phase is also done three times and now she was shouting, "Blow, blow, blow". This time I had dropped in lung intake capacity from 53% to 48%. The NIF at -17 was higher than last time, but that also means my muscles ability to push air out has decreased. I said, "What happens when I reach -1, I die?" I have a sick sense of humor I guess.

Anyway, this decrease ability of my muscles to pull air in and push it out again is probably part of my increasing tireness. With this disease, most patients die from respiratory failure because the muscles, such as the diaphragm, become to weak to make the lungs work. My lungs are actually in very good shape.

The Physical Therapists do a lot of measuring of muscle strength in all parts of the extremities. They also check the walking gait. I'm still walking with just the aid of a walking stick, but the doctors want me to cut the distance I walk each morning by half. That morning walk is a long established routine with me, I really don't want to give that up. I have cut back some on the distance anyway, because my body tells me when I need to head to the car. I will contune to listen to my body.

I am getting more unsteady. My balance has declined. In fact, if I walked on a decline I might fall. (Joke!)

My weakest parts are my hands. It is why I make so many messes. They said I didn't have much less under the skin but bones and tendons. The thing I didn't care to hear and scares me the most is they told me if my hands get much weaker I will not be able to drive.

As far as diet, they were happy I weighed in at 188. To think, a year and half ago I weighted 165. I do not enjoy getting a belly, speaking of which, I did tacitly agree that I'd get the feeding tube after the holidays. I would go up to jefferson overnight and they would sent a edoscope kind of thing down my throat and then punch a hole through my stomach to outside and insert the tube. You get your nourishment with a syringe through the tube. They did say I could still eat what I liked as long as I could swallow without difficulty. So far I don't have any problem swallowing.I am still a bit leary.

I spent a long session with the Pallitive Doctor. That is a polite way of saying Doctor Death. She will oversee my comfort as I gradually fade into the Big Sleep. She is very nice. Her name is Doctor Parks and I found that ironic and humorous. The doctor who delived me into this world was a Doctor Parks and now the one who will escort me out is a Doctor Parks as well.

We discussed the Advanced Directive, or Living Will. I haven't done this yet. It is not easy. You are making life and death decisions for yourself as well as assigning some awesome responsibilities to somebody to make medical decisions for you when you can't. I decided my oldest daughter, Laurel, will be my agent and my next daughter, Noelle, will be her backup. 

I now have to tick off what artificial life supports I don't want. I already changed my mind on the feeding tube. I also told the Doctor I don't was a trechnomy. That is a burdenous thing. She told me most people choose against it.

There are some blocks to mark if you want organs donated. I want to check the one for donating my entire body. I think funerals are a waste of money. I need to find out how I manage to give myself away. What does it matter what they do with the body; I won't be home anyway. 

Huffing and Puffing Through Another Month

I don't let things lie. I looked it up. The description I read said, "ALS: an incurable, untreatable, progressive, ultimately fatal disorder".

'Tis like a line of poetry.

What is this poet supposed to do with that?

Doctors told me on December 1, 2016. Merry Christmas !

Knew it months before they told me. Oh, no, not what it was, not by name, but knew it wasn't good. I knew it on a beautiful May morning when such things should be unthinkable. Sun was up to my left. The air was already warm  though it wasn't much after 6:00 AM. I was taking my daily walk.

Every morning, nearly 365 days a day I took that walk. I think I missed only 6 mornings summer to summer 2015 to 2016.  At least five miles when I went and often more.

My favorite park was the Brandywine Creek State Park. This morning I was in Rockwood Museum Park. It would ever more become my home base. I'll probably never walk Brandywine Creek again. I don't even know how long I'll manage Rockwood.

Coming down a hill, not steep, there started a strange feeling in both thighs. I had never felt anything like that before. I can't even describe it. This was part of the problem, I never could describe it to anyone. Closest I can come is this:

I use to play guitar. Don't see me doing that again.  My hands are pretty wasted, not delicate instruments any more. Anyway, this feeling was like the low E string, the big fat one on the bottom, came loose. When plucked there is nothing but a dull thud, no note. That was my legs, not in the sense of sound, but a feeling like a dull thud, over and over.

It was in my forearms, too, but I didn't notice yet. Wasn't thinking about my arms, just my legs. Here I stood in the middle of a path unsure if I could walk.

I had to.

 I took a few steps with this strange gait, my toes pointing out, especially on the left. I moved in mincing steps.

I thought it would go away, whatever it was. First my belief was it could be walked off, you know, push through the pain. Except there was no pain. My legs did not hurt, they just felt like they weren't connected. I decided to make it to my car and go home. Maybe with some rest it would go away.

It didn't.

It took six months for doctors to figure out what it was; six months of many specialists, many tests, of  much money spent, and nobody had a clue.

I had a runny nose and cough. Oh, yeah, doctor could take care of a runny nose and cough. Whipped off a prescription for an antibiotic. Bad move.

 I got clostridium difficile colitis or C. Diff. It was terrible. You never want to have C. Diff. I nearly died right then and there. They told me I was septic. Septic means full of infection. Might mean full of death. There is another definition of septic, as in septic tank. I was that, too. How can I put this delicately? I can't. I was a septic tank full and overflowing.

No, you never want C. Diff.

But I  had four bouts  and two stays in the hospital. I also drank a lot of down tasting medicine, expensive medicine that none of my insurances covered.

After my C. Diff. was over; summer was waning. I was now sent from pillar to post, like a pinball, bounced from doctor to doctor for explorations and hopefully an explanation. There were no explanations.


Finally, on the 1st of December, six months after the strangeness began,  a diagnosis came. I had ALS and it was incurable, untreatable, progressive and fatal.

INCURABLE

Nothing I can do about that, so forget it. No one can cure something when they can't even figure out why someone has it. We'll just move on then. Maybe all that money collected from people dumping ice water over their heads will lead to a cure.

UNTREATABLE

Oh, good one. Most illnesses that come along have some form of treatment. An icky tasting medicine. A neetle in the arm. A voodoo dance. Blood letting, at the very least. Not this thing. In some ways this is a blessing. I'm not being prodded or poked or stuck with any sharp objects. I'm not running for tests every third day. I'm not living between doctor appointments. I'm kind of free to live what living is left me.  The only sensible approach is to keep on doing the things you like to do...as long as you can.

PROGRESSIVE

That is where I live now, in the progression. Progression is the part where you do as long as you can do. Progression is where you better be adjustable.

So, where am I this one-year-plus since the E string went flat?

My legs and arms have gotten weaker. I still do a morning walk with the support of my trusty walking stick. I walk short, really short, distances without any aid, but if I start wandering off far without that stick I am in trouble. I will get off-kilter and look drunk. I might fall. I do not want to fall.

If I fall I may not rise to my feet because my arms have gotten too weak to push me up. I can pull myself up if there is something to grasp. I can't carry anything of much weight.

I also drop a lot of things because of my hands atrophying along with the arthritis. When I sit or get in bed I do not lower myself gently. At some point I just drop, kerplunk. Getting into bed, or out of a bathtub for that matter, is pretty funny to behold. Sadly, I may even be beyond able to take a bath. Showers are scary due to weakness of leg and unsure balance.

Fasciculations can be fascinating. These are muscle twitches. I was looking at my thigh last night and there was a lot of jitterbugging going on under the skin.

I am getting many more muscle cramps now. They hurt. I get them everywhere and anywhere, I had them in my fingers earlier as I tried to type this piece. Had to stop for a while.

Been lucky so far. I can still talk. I can still swallow. I can still breathe, but I get winded quicker. I have a respirator I use at night while asleep. It is supposed to help strength my diaphragm. Most people with ALS die of respiratory failure.

DEATH

Which brings us to that term, "Ultimately death". You can say that about us all, can't you? They say the average life expectancy is 2 to 5 years after diagnosis. I don't think about death. I believe in Jesus as Savior; I know where I am going. I don't fear that ultimate. I'm still shooting for 100.

You know, I have a double whammy. I've been my wife's caretaker for years. She has Type II Bipolar Disorder. Sometimes I wonder which is worse, her disorder or mine. I say her's. It is a horrible thing that affects her mind. I just have a deteriorating body. My brain, my eyes, my sexual equipment, my inner organs all are unaffected. I am cognizant of what is going on with me. I would not want the horrors of Bipolar on top of this.

Anyway, here we are. Much lies ahead. Pray for us.

That Grand New Gang of Mine

What awaited me at the hospital once they proclaim I have Amyotrophic Lateral Sclerosis was quite different from what my previous experiences had been. I was not admitted or committed to some uncomfortable bed  and no one was coming about sticking needles in me here and there or forcing pills down my throat. Quite the contrary, for my decisions about my fate have been placed pretty much in my own hands. It is not that I have been cast out by the medical profession to fend for myself, no not at all. I find myself part of a cast of thousands who seem genuinely concerned about my well-being and prepared to aid me in having as good a quality of life as can be managed under the circumstances.  There really isn't any treatment, not in the usual sense for this thing, but they provide a clinic presumedly to help me transition through the progressing of the disease. It is a combination efforts of hospital staff and members of the ALS Association.

Friday was my first such clinic.

I was finally diagnosis as having ALS on December 1. I could have attended my first extensive clinic in December, but because of the holidays and all the fuss and feathers of the season, I put it off until January 6. I awaited this with much anticipation, then, for just over a full month. It was going to require me to drive into Philadelphia, which was one part I wasn't looking forward to at all. My appointment was at 8:45 AM and this meant my travel time would fall during rush hour, o joy, o joy.

And of course, we had to get our first snow fall of the year over night.  Fortunately the roads were fairly good.

It does not have to be difficult from here in this section of Delaware to Philadelphia. Straight up I-95 North should have you there in about a half-hour, unless there is an accident or road construction upon the highway. This time there was neither and even traffic wasn't as brutal as is common that time of day. Still, I left at 7:30 to allow for any hiccoughs and thus arrived on the 900 block of Walnut Street with plenty of time to spare. I parked at a garage at 925 Walnut.

Philadelphia parking can be expensive when compared with Wilmington parking. These garages seem to be manned with attendants who do not speak English with great skill. The person who gave me the ticket totally confused me, but I gave him my key and trusted the car would be there for us when we returned. The clinic was at 909 Walnut, juts a short walk from the garage and we signed in a half-hour early.

I expected we would be sitting in the main waiting room for that half-hour, but like before, someone took us back almost immediately, the first of a parade of people. This one weighed me and then escorted Lois and I into an exam room. I weighted 171 pounds with my clothes and shoes upon me, so let's guess I am probably 168 pounds give or take some ounces.

We had been initially greeted by Kate, ALS Program Manager of the Jefferson Weinberg Center. I'd be hard pressed to tell all those who followed over the next three hours plus. I know that Goran Rakocevic, my main ALS doctor and Judy Guarnieri, the Led Nurse Practitioner visited with us several times during the day, but there were a ton of people, dietitians, nurse coordinator from the ALS Association, Social Workers, Speech Therapists, Occupational Therapists, Physical Therapists, I-am-not even-sure-what-all therapists. They stretched me, pulled me, poked me, struck me with a hammer, watched me walked and asked a million questions.

I will get an assessment of all this sometime next week.  Meanwhile, I was given some physical exercises to stretch my muscles. I was also given the gadgets in the photo, consisting of nose clips and a tube I am to breath through for several minutes a day to strengthen my breathing. I was also given the tube in my hand to make handle utensils such as forks and spoons easier for me. It was discussed me getting a mask to wear when I sleep to also help breathing and a leg brace for my right shin and foot to help me not drag my toes to close to the round. There was also discussion of grab bars and ramps and other alterations I might need in my not-very-handicap friendly house.

I will go back for another such clinic on February 7 and so the adventure has begun. I feel I am in the hands of caring folk.

The hospital will validate parking; however, I had picked a garage not one of theirs. They gave me a $15 voucher towrad parking. The fee for four hours was $30, so I had to pay $15 out of pocket. Next time I will drive about the block and park at the neurology Garage at 9th and Locust.