The Diagnosis

Hi, this is Steve’s daughter Deb writing.  

It was very important to my dad to donate his brain for research.  He also wanted us to have a definitive diagnosis – MSA is not typically hereditary, unlike some very similar diseases.  He would have been relieved to know that the brain autopsy results indicated a definite diagnosis of MSA.

Here is the statement we received from the University of Miami Brain Endowment Bank:

The section shows dense a-synuclein-positive glial cytoplasmic inclusions (GCIs) throughout in the putamen, brainstem, and cerebellum with lesser amounts in the caudate nucleus and thalamus.  Rare a-synuclein-positive neuronal inclusions and scattered a-synuclein-positive processes were observed.  Gliosis was most evident in regions with abundant GCIs.  The clinical history together with the widespread a-synuclein-positive warrants a definite neuropathological diagnosis of multiple system atrophy.  

A little bit of googling tells us that this is typical for MSA – basically, a specific type of lesion throughout certain parts of the brain.

We are glad to have the diagnosis, but there’s a certain finality to it that is saddening to us.  My mom is doing well, but we all miss my dad every single day.

My dad worked so hard on this blog - it was a way for him to deal with what was happening to him, and to share his experiences so that other people with this disease might benefit.

It’s unlikely that we will add more entries to this blog, but will keep it alive in the hope that it will help other people who are facing MSA.

The very best to all of you.

Post 78 - STEVE STEIDEL 1944-2014

I'm so sad.  We lost Steve on June 28.
 
He was doing ok.   We were managing all his symptoms and problems. 
We quietly celebrated our 49th anniversary on June 12.
Then, the following week he started with a low fever.  It climbed to 102.8 even though he was being given a strong antibiotic through an IV. 
Our family doctor sent his nurse practitioner every day.  One day she came 4 times. She was so caring and compassionate.

Steve gradually got worse. He could no longer move his limbs but his eyes stayed on me all the time.  The worse for me was that he could not communicate. We tried everything but his brain wouldn't let him blink his answers and he could no longer squeeze my hand.  He managed a big smile when our two daughters came. We stayed by him around the clock.  He knew what was happening, he could hear and understand until the very end.
After a week of this the nurse practitioner brought in Hospice.  What a wonderful organization.  His lungs then began to fill up and his larynx was closing. The end was near.  The girls and I whispered our love to him and hopefully he left this earth without pain.

I have been inundated with beautiful notes from so many people.  I realize how many lives he has impacted.
I also realized that the hospice nurses are more informed about MSA than most doctors, especially the ones in the ER. 

Steve put so much of himself into writing this journal, especially in the beginning when he wanted to get the word out.  I have tried my best to keep it going for him.  This will be the first post that he is not "editing". 
Will I continue the posts?  I do not know but I will let you know the results of the brain donation.  I understand it takes months before they contact you.

I am including the link to his obituary and a slide show for those that did not know him personally but faithfully followed his blog.

http://www.kays-ponger.com/obits/obituaries.php/obitID/755589/obit/Stephen-E-Steidel

http://youtu.be/BcKlu-pVPH8

Thank you, everyone for your support.

Fran Steidel

Post 77 - New Month, New Message

I'm glad to say that things are going along pretty well.

I am irrigating Steve's catheter every morning and that seems to be keeping the sediment buildup in check.
Steve has finally acclimated to the bi-pap machine and we only need to adjust it once or twice a night because it moves and causes an air leak.  When there is a leak the noises it makes sounds like Steve is a one man band.  It's entertainment while we sleep.

Caregiving is a full time job that is both exhausting and physical and we are concerned about the toll it is taking on me. Steve had a regular doctor's appointment this month and the doctor saw the progression in Steve and also how hard it is for me to manage him physically. Therefore he contacted hospice and arranged for a house visit.
Although Steve's disease has progressed he did not qualify for hospice yet. I guess I should be happy but I guess I was hoping for some help. The hospice organization is wonderful.  We are now in "Transition Hospice" which means a volunteer will come and stay with Steve for 4 hours a week to allow me some respite.  The only problem is that most of the volunteers are snow birds and they have flown the coop.  We are hoping that they will recruit some nice, new folks and we can start the program soon.
Meanwhile I have contracted with a home health agency to come in several mornings a week to help with Steve's personal needs.  That starts tomorrow.

In several months Hospice will evaluate him again.  The criteria they look at is weight loss,  loss of appetite, bed sores, pain and several other things.
So, yes, I am happy that Steve has not reached that stage.

We are looking forward to an uneventful summer.  We have learned to accept the hot and humid days of Florida.  Hopefully there will not be any hurricanes anywhere nearby.
Both girls and our granddaughter will be visiting soon so that will definitely brighten our days.

Fran

Post 76 - Ouch! Ouch! Ouch!

Ouch! Ouch! Ouch!

When things get ironed out on one end something else gets wrinkled on the other.

Several new happenings this month.
First the bi-pap machine.  It was back to it's old self with dozens of alarms throughout the night.  These are alerting us to Apnea episodes.  Steve finally decided to have them disarmed.  This took a summit meeting between doctor, technician and the medical supply company.  Finally all agreed that Steve would need to sign an affidavit taking full responsibility for this decision. OK, done deal and what a difference it made. We BOTH now have a restful sleep.

Second the catheter change.  The nurse comes every three weeks to do this. About 4 months ago the doctor changed the type of catheter from latex to silicone to see if it would help with the sediment buildup.  It did but it caused another problem.  The silicone has a tendency to adhere to the inside wall of the bladder.   The nurses have been having a hard time getting it out.  Last time it really hurt him and there was blood in his urine for several days.  This time the nurse tried but decided not to yank it out like last time.  A trip to the urologist was in order to solve the problem.  But his nurse could't do it either.  So now the doctor was going to take charge.  Steve and I both thought he would deaden the area or inject some novacaine.  Not to be.  Doctor walked in, took hold of the catherer and yanked it out.  I guess it's like removing a bandaid, the faster you do it, the less pain you feel.
Of course Steve does not agree with that analogy.

Things  are now fine.  We are back to using the latex catheter but now I am irrigating Steve every morning to try and keep the sediment in check.
Hopefully next month will be uneventful.

Happy Easter
Fran

Post 75 - Odds and Ends (no pun intended)

It has been a while, so I (Fran) thought I would let everyone know that the Bi-Pap machine is doing it's job.  The technician decided we needed a new machine. It took a while for the supply company, technician, and doctor to get on the same page but finally tweaking some numbers solved the problems.  I think it took so long because I was trying to be nice and not my usual impatient self. Well sometimes vinegar works better than sugar.  Yes, the alarm still goes off but only several times a night. Steve is getting a restful sleep and wakes up refreshed.  He will be trying a new full face mask in April because the smaller mask irritates the bridge of his nose.
For those who asked, the machine itself is whisper soft. If you use an older machine and it is noisy,  get yourself a newer model.

Life never stays uneventful for long.  For several days Steve complained of an odd sensation on one side of his buttocks. He was worried he was developing a bed sore.

So what did it turn out to be......SHINGLES!  On day 3 I could see the signs of small blisters in a linear pattern.  We went immediately to the doctor.  Funny thing is that Steve had the shingles vaccination three years ago.  We are finding out that it is only 67% effective.  Between the vaccination and the prompt dosing of an anti viral drug he has a mild case.  Thank goodness. Hopefully it will be all cleared up by our next posting.

Some good news is that the FDA has finally approved the drug droxidopa.  This drug has been used successfully in Japan for decades for orthostatic hypotension.
People with OH have a sudden severe drop of  blood pressure when they stand up, causing dizziness and sometimes fainting. It will be available in 6 months.  We will see our GP about this.  If he does not agree to prescribe and monitor it then we will  have to deal with our neurologist. We will keep you posted.

We hope you are all seeing some signs of Sping wherever you may be.

Fran and Steve.

Post 74 - A Note from Fran

Several of our friends have been inquiring as to how Steve is doing with his new Bi-Pap machine. I've decided to post an entry to let you know about our trials and tribulations with this BOX.
Eventually it's going to be good, but until then, it has been frustrating and I am completely sleep deprived.  The machine has a modem and SIM card so that the doctor and Medicare can read the results. The doctor wants to make sure it is working and Medicare wants to make sure that Steve really needs this expensive gadget.  According to the technician that has come to the house twice now, this high tech machine is learning Steve's sleeping and breathing patterns ???? 

Steve has obstructive apnea and also the more dangerous type called Central Apnea.  The brain of an MSA patient neglects to tell the lungs to push air out to start breathing when sleeping.  This machine is suppose to "read" this and push a bigger burst of air out of his lungs. ( I think, or at least this is what I understand). This very smart machine has an alarm to warn you that you are having an apnea episode.  The technician neglected to tell us about this alarm.  The first night when this alarm sounded I jumped out of bed thinking the condo fire alarm went off. It then proceeded to go off another dozen times that night and every night thereafter.  Steve has learned to reach over and silence it. If he doesn't then I get up and gently wake him.  BUT remember this machine has it's own brain and agenda.  It only stays quiet for 90 seconds and then automatically re-activates itself.

I will regress to tell you we go to bed earlier because it takes time to get the straps, mask and tubing attached and plugged in correctly.  There is a small humidifier inside this box that gets filled with distilled water every night. Every morning the mask, tubing and filter has to be washed and dried.

I hope this does not sound as if I am complaining because nothing is further from the truth.  I am thankful that all these medical advances are available to us.

Steve is still in good spirits but he is getting more tired and exhausted with time. Our Christmas was delightful with our children here  but it took a toll on him.  He has not wanted to go out to a restaurant now for over a month.

Hopefully he will be in good form soon and write the next post himself.

Post 73 - Finally!

After weeks of waiting, I finally have taken possession of a sleep machine; specifically called a BiPA autoSV Advanced System One by Philips or, as it’s more commonly known, a new version of a BiPAP machine which blows a steady stream of air into the sleeping victim, er, I mean patient. The delay, I have since learned was due to the senior doctor taking an abrupt leave of absence, the closing of one of two offices, the re-organization of the remaining three doctors into a new group, and the failure of my doctor to completely fill out my correct machine orders. And the Holidays. Other than that, no problem!

 

Wait – there is one slight problem. Do they really expect you to sleep soundly with both a chinstrap to keep your mouth closed and a bunch of straps to hold the mask in place? Looks like I’ll need another visit from the talkative technician to show me how to make this thing work as intended.