ALS Clinic #3 - December 10, 2014

Today was a long and exhausting day.  Kim and I got up at 5:30 this morning and were at Hershey Med Center Neurology at 8AM for my third quarterly ALS Clinic.  Fortunately the snow passed us by last night.  There was some good news and some not so good news.

My Forced Vital Capacity score was 84, down from 89 in September and 96 in May.  To be honest, I expected it to be worse because of the difficulty I have breathing at night, which is related to my chronic sinus condition.  While it was disappointing that it went down, I am happy that it wasn't worse.

The reflex tests were exaggerated indicating nerve and muscle damage.  Occupational therapy evaluated my arm and hand strength.  My right hand (dominant) grip strength was about 60 pounds, compared to 80 pounds for my left hand.  That explains why my handwriting is getting worse, it takes me longer to do almost everything, why I have some trouble shaving in the morning, have trouble opening things, and am dropping things frequently.  My weight was down several pounds also, which they have been cautioning me about.  It is hard to eat when I am at work, and it takes me a long time to eat a large dinner - about 45 minutes.

They didn't find a major loss of strength in my legs, but they feel very spastic to me and I cannot walk as smoothly or as quickly as I used to.  When they tested my ankles they found some catching, which may explain part of my walking slowdown.  However, I can still do 30 minutes on the treadmill without getting out of breath or feeling like I am going to fall, just at a slower speed than I used to walk.

The good news - swallow is still good, not much change since September.  Surprised everyone since my speech is completely gone for all practical purposes.  My blood pressure was good.  The physical therapist walked me down to the PT room observing me as I walked and then had me walk up and down 4 steps 3 times and didn't notice any balance, strength, or wobbliness issues on the steps or on the walk down the hall to and back from PT.  She did say that I walked with my head forward, but we think that has been the case for a while.  She suggested I should work on my posture and try to get my keyboard and computer screen raised more to my eye level to keep my head up.

The push is on for me to agree to let them install a PEG feeding tube to ensure that I am getting enough food and fluids and to help prevent me from aspirating food into my lungs.  The feeding tube would be directly inserted into my stomach and it would be fairly inconspicuous, although it might be noticeable if I wear a tight shirt.  The doctors, nurses, and nutritionist indicated it wouldn't limit my ability to travel or do any physical activities.  They suggested that I might want to do this sooner rather than later.

We also talked about a diaphragm pacer that may help me long term before I would need to use a non-invasive ventilation system such as a BiPAP.  Can often delay the onset of using an NIV by a year.  The diaphragm pacer system requires an external power pack that must be carried everywhere, which may be more limiting in terms of travel and normal activities.

Both devices could be installed at the same time on at outpatient basis.  Lots to think about and I am not in a hurry to make a decision on such important topics.

I also asked to be tested for mercury toxicity, and Dr. Simmons agreed to write the lab order for me, although he indicated that he didn't feel that we would find anything.  My first symptoms started a week after I received my flu shot last year.  I didn't realize that most flu shots contain Thimerosal, which is a preservative that has organic mercury in it. 

I have been severely grinding my teeth for more than 6 years, and I have a lot of large amalgam fillings in my molars.  I knew amalgam had mercury in it, but I was very surprised to learn recently that amalgam contains 50% mercury!  Mercury is one of the most toxic substances to the human body, and can cause sinus issues and brain and motor neuron damage.  My sinuses and gag reflex have gotten worse every year that I have been grinding my teeth at night.  I have several mouth guards, but my terrible gag reflex means that I often cannot tolerate one in my mouth at night.

These days, I grind my teeth 24x7 due to the muscle spasms in my face and jaws, and I can feel my fillings disintegrating, which means that I am ingesting more mercury into my body in addition to the mercury vapors that leach from the fillings.  Since the teeth are so close to the brain, sinuses, and eyes, amalgam fillings can cause significant bodily damage.  I am anxious for the tests and to see what the results are.  I am educating myself on amalgam mercury toxicity and treatments.

I gave three vials of blood for two ALS research projects today.  Heidi had trouble finding a vein in my right arm so we tried my left arm and eventually found a vein, but it came out as a slow drip, and filling 3 vials took more than 10 minutes.

We finally got out of the ALS clinic around 12:30 and headed to Panera's for a bite of lunch before driving over to Nyes Road for my annual dermatology checkup at the Hershey Med offices at 2PM.  No major issues again this year, but about a dozen sun damage places frozen off my face and head.  Finally got home around 3:30.  Kim and I were both exhausted!

The day was emotionally and physically draining, but all in all it was a pretty good clinic report.  So it's early to bed tonight after a full day missed from work and back to the office and PA Turnpike tomorrow.

Grateful!

I have a lot to be thankful for!  I have felt really good since my appointment at the Hershey ALS Clinic on Wednesday morning.  I cut down my caffeinated coffee to one each morning, but no headache.  I feel less stressed, stronger, and am definitely less worried.  I have had fewer gagging/choking events the past few days, and I am very grateful to have some relief!  I don't know if the Riluzole can act that quickly, if I am experiencing a placebo effect, all of your prayers are being answered, or if my body has finally relaxed a little bit, but regardless of the source/sources, I am very relieved to not be suffering the same effects! 

Thank you for your continued prayers!

We have already exceeded our team goal for the ALS Walk for the Cure on June 7 at the Hershey Med Center!  Three people have signed up to walk with Kim and me.  A sincere thanks to everyone that has donated or signed up to walk with us!  The Team JFK page is open and accepting new walkers and donations.  Kim and I will match the total donations up to $1000 total to thank you for your generosity.

Reading about the Brainstorm Clinical Trial in Boston, where they will be testing the safety and efficacy of a stem cell therapy that has had excellent results outside of the U.S. for a product called NurOwn, based on harvesting the individual's mesenchymal stem cells, treating them, and then reintroducing them into the individual's body again.  There is another stem cell research project in Atlanta, called Neuralstem.  Since ALS is a brain/spinal column/motor neuron disease, it makes sense to me that stem cells may hold promise for a future therapy or a cure.

Enjoying the long weekend so far!  I got up at 2:45AM to try to see the meteor shower, but it was completely overcast.  Oh, well!  Hope that some of you were able to see it.  They were predicting between 500-1000 meteors/hour, which would be phenomenal.

Doing a little bit of work, interspersed with reading my book, sitting and holding Kim's hand, talking to Jean.  I brought Jean some of my purple Columbine, which has been spreading at our house over the past 10 years.  She planted them this morning - I hope that they survive.  They are a beautiful color!

I learned that my Uncle Frank in Grand Junction, CO fell this week and may have had a minor stroke.  He is at the VA hospital for now and my Aunt Geneva is living alone in their apartment.  Our prayers for both of them and I hope that Frank recovers quickly, regains his strength, and gets home soon.

For our traditional Memorial Day weekend cookout tonight we are having hamburgers, hot dogs and Kim's famous hot dog sauce, Pasta salad, baked beans, and deviled eggs.  YUM!  I'll cook them on the grill shortly.  Have a nice bottle of red wine I am going to open in a minute to let it breathe before Kelly and his dog Liam come over.

I hope that all of you have an awesome weekend, spend quality time with your family and friends, and make some good memories.

Hershey ALS Walk, Saturday, June 7

Dear friends,

If you are interested in joining me Saturday morning, June 7, at the Hershey Med Center East Campus for the Walk for ALS, please join Team JFK at the following website:

Team JFK

The walk is about 2 miles on gently rolling paved walkways and sidewalks on the Hershey Med Center East Campus and the fundraising goes toward ALS research and supports the Philadelphia ALS chapter, which the Hershey ALS Clinic is part of.

If you can't walk with me but would like to sponsor the Team, you can contribute on the page also.

Registration starts at 9AM, and the walk starts at 10AM.  It usually takes about 2 hours as each team is photographed not too far from the beginning of the walk.

Thank you for your support!
Jim

Work/Life Balance

I am still crazy at work with multiple PennDOT and PA Turnpike projects in addition to a big project with the City of Charleston, WV.  I worked late tonight onsite at PennDOT, leaving at 6:15. 

My inbox is as full as it ever has been in my current job, and I really struggle to keep up with it.  Hard to believe that for most of my 8 years at the commonwealth I tried to keep the number of emails in my inbox to 100 or less every day.  How did I do that?

The work onsite at PennDOT has been quite rewarding, although very challenging.  I am rewriting the entire backend for the Oracle Highway Performance Monitoring System (HPMS).  I get to use my technical database skills and writing Oracle Stored Procedures to create the 2M record HPMS data set that has to be reported to the FHWA in June. 

I made some major changes to the program yesterday because I realized I wasn't splitting the records properly so that I would get the correct mileage numbers for the Urban Areas.  That set me back a few days and I inadvertently caused some errors that I am now trying to fix so that I can give the PennDOT management team the updated road mileages for the State Mileage Certification Letter that is due June 1.  All of the data that we deliver 2 weeks later has to be within 1 mile of that number, which is not an easy thing to accomplish.  Unfortunately I was out of the office at the Turnpike today twice for Asset Management meetings, which took away from my ability to get the programs debugged.  That's where I start again tomorrow morning.

Kim and I had planned to work outside around the house this weekend, but sounds like we're going to get lots of rain.  May be time to clean up the basement - it is a mess, and I need to start going through things and pitching them so that Kim won't have to some day.  That sounds a bit morbid, but I am a packrat, and have boxes of college books and notes downstairs plus many hundreds of books from the authors I read and collect.  FYI, I just added 60+ new books last weekend at the Penn State AAUW Used Book Sale. :)  I'm getting ready to turn in for the night and (re)read The Talisman by King/Straub.

I may lose my ability to speak in the next year if the progress continues at the same pace and will be forced to depend on a computer to help me with verbal communication.  The nurse at Hershey assured me that "There is an app for that!"  My ability to speak clearly comes and goes - today was a pretty good day altogether for speaking, but my capabilities are definitely declining due to my partially paralyzed tongue, vocal cords, and face.

My family and friends have been wonderful and amazing.  The unfortunate thing with my disease, Bulbar ALS, is that it affects your emotional state quite severely, so I am often so moved by the smallest kindness that I am moved to tears and become verklempt (thanks Mike Myers).  Same with sappy TV programs.  I really struggle to communicate.  I told PennDOT today of my situation and they were very kind and compassionate. 

My dear friends and family, thank you for your prayers, emotional and physical support over the past months and especially over the past few weeks as I have struggled to communicate and arrive at a diagnosis. 

I have received a lot of spiritual guidance and support from many people.  Kim and I have not been active in church, but I am still a believer.  I think it will be helpful to find a church and become a more spiritual person in the days and months ahead.  Lord knows I need a lot of help to be a better husband, brother, son, coworker and friend.

Please forgive my ramblings, I'm tired.   But it was a good day, and I know that there is a lot of work for me at PennDOT and the PTC in the next 12-24 months if I am capable of working that long.

We moved my meeting with the Hershey team to next week.  I thought it was just with the speech pathologist, and Kim told me it was with the ALS Clinic team - SP, doctor, nurses, social worker, nutrionist, etc. and would take several hours.  Kim was right, and I was wrong, of course.  Maureen was kind enough to move it to next week so that I could take care of my work commitments this morning.

Thanks for reading, and I hope that some of this information is useful to you as writing it is helpful and cathartic to me.