First time Home Since January 3

I had my breathing crisis on January 3, 2020, which led to my tracheostomy in my throat and PEG feeding tube in my stomach through my abdomen.   Karrie and our son Mark have received training in tracheostomy care and all the many pieces of equipment and supplies are being delivered Monday.  I am going home with Karrie Monday evening.

It will be an adjustment time because Karrie will be doing my trach care and all breathing treatment needed with back up from Mark and our other adult kids all supporting her.  The tube feeding, meaning nothing by mouth, will also be Karrie’s responsibility.  This is a lot. So it is a joyful time, but I know it will be hectic and Karrie asks that for a few weeks at least no visitors outside of the immediate family.  We appreciate your love and support! Dan

Still Going On

January 3, 2020 was a day that changed life as we know it forever.  You will need to go back and read my last entry to have the basic outline of that crisis because I am going to fill in the blanks with this additional narrative. As they were attempting to save my life, and I was on the brink of oxygen deprivation, I looked at Karrie’s loving and concerned face one last time and she at mine.

Since I could not move a single finger or move my mouth to speak, I hoped somehow my eyes would show my need for help and she could convey this to the emergency room staff.  Somehow she knew and when she said “He can’t get air”, they put a large instrument down my throat and then an intubation tube.  Next I went to ICU and discussions ensued.

Karrie and my soon to be surgeon were discussing the conditions that caused my vocal cord paralysis and from Karrie’s explanation, he knew exactly what Multiple System Atrophy was and how it could stop the body’s ability to get proper signals from the brain thus interfering with a person’s ability to breath.  Thank God for this doctor for whom it all clicked.  Also, my own ENT doctor came to see about the situation and contributed a good deal of information about my brain disease affecting my vocal cords.  It was determined the best route would be a tracheostomy with a feeding PEG tube inserted directly in my stomach to prevent the future reoccurrence of the inability to get oxygen or choking on food and liquid which I had been experiencing severely for years.

As I was then put under heavy anesthesia to keep me safe from removing any tubing, I was put on a ventilator and waited out the weekend for a three day stretch before I could be operated on. The surgeon opened a hole in my trachea and inserted a tracheostomy.  During this time I saw and was aware of very little.  I was fighting for life and at times had to be restrained as I unconsciously would attempt to pull what to me were foreign objects from my mouth.

I had pneumonia and fever following my two simultaneous operations, the trach and the feeding tube insertion into my side.  I contracted a staph infection and they had to check my heart function, which through all of this was doing great.  My sons, our daughters in law, Karrie and my brother and his wife all hoped when I made it out of all this that I would still be me and have my full capacities.  Several close friends came by during my hospitalization, for which I am deeply appreciative.

Some great doctors, nurses and staff did and decided so many best practices that led to my successful surgeries.  Now I had my trach in place and my PEG tube, but I wouldn’t be finished fighting.

Next, I will tell you about my dream state from anesthesia and the delusions that followed me for weeks until the medicine all worked it’s way out of my body.  Also, a few weeks later while at the rehab hospital, I had to be sent out to a nearby hospital to have my dislodged PEG tube replaced through surgery.  I will chronicle these and other accounts with my next blog posting.

As I close, I must add that I had no idea how much my family went through until weeks later when through conversations they helped sort out my dreams from their reality of what actually happened.  I don’t write this account lightly thinking it isn’t hard for my dear ones to relive.  They went through so much worry and wondering if they had lost me.  I am so very thankful for all who stood by me, especially Karrie and our beautiful family. — Dan
“I know the plans I have in mind for you, declares the Lord, they are plans for peace, not disaster, to give you a future filled with hope.”— Jeremiah 29:11 (Common English Bible)

Below:  Karrie in my room at the rehab hospital where we learned about caring for and living with a tracheostomy.

Saved by a Trach

I have so much to share but it is not within my power to write all that has occurred.  I have long known my vocal cords, swallowing and breathing risk were big issues with the autonomic failure of MSA.  On Jan. 3, 2020,  I had a breathing crisis and was rushed to the hospital. While there, I struggled until I was near the end.   Karrie helped inform the doctors and nurses of the type of disease I have and they saved my life.  I was under heavy sedation and on a ventilator for several days while the family didn’t know the shape I would be in even if brought back.

I applaud Karrie and the wonderful physicians at Parkview Community who  determined a tracheostomy inserted and a PEG feeding tube placed in my side into my stomach were my best chance of survival and preventing the same breathing crisis from recurring.  I am now in a rehab center full time with experts in learning to use a trach full time and gastric feeding (liquid  only) situation.  Family training is involved and my sons, wives and grandkids have all been so supportive. Thanks to all our closest family, friends and church who have sent love and prayers our way.

I am here by the grace of God, the miracle of science and dedicated medical professionals.  Thank you most sincerely.  Above all, I miss my sweetheart who visits me every single day.  We will go on  in mutual love and shared faith.  Karrie and I would not have pulled through thus far without our great, and I mean great sons, their wives and our grandkids.  We love you all.  Thanks to my brother Matt and wife Lillian for all the visits and caring, as well.   “I can do all things through Christ who gives me strength.”— Philippians 4:13  Dan Brooks (patient-online)

Adapting to Changes

Our clinic now has a neurologist whose specialty is movement disorders like Parkinson’s Plus Syndromes.  We have returned due to the opportunity to see this wonderful doctor without needing referrals and approvals. She agreed with my previous MDS that what I have are two Parkinson’s Plus syndromes but not the same two.

You may remember that I was diagnosed with MSA and CBD. What I am now facing as my diagnoses are close “cousins” to these previously diagnosed conditions: Lewy Body Dementia and Progressive Supranuclear Palsy.  Lewy Body Dementia includes movement symptoms as well as autonomic issues, as does MSA.   Progressive Supranuclear Palsy involves falls as well as serious eye control issues. As I look back, I remember my PET scan results indicated findings consistent with Lewy Body Dementia.

So as I return to write after a hiatus lasting months, I am revealing what has been happening and why Karrie and I have been overwhelmed.  I am self conscious about the nature of my diagnosis and what it means.  In the meantime, I am not sure how this fits with an existence that is more and more limited. I choose to live each day to its fullest no matter how limited I am.  Adapting to the changing picture of my diagnosis isn’t for the faint hearted, but it is doable.

I will end by encouraging you to go to the icon on the right and click on my song, I Will Go On, which says how it feels to be in this situation and wanting to find a way to make these adjustments. — Patient-Online

New Drug for MSA in Final Stages?

Today we were given some hope.  I have never had the thought that there is a treatment for some of the symptoms that I have, let alone one of the two syndromes with which I have been diagnosed.  We were visiting my neurologist, who is a Movement Disorder Specialist (a neurologist who specializes in degenerative brain diseases that cause movement abnormalities, such as Parkinson's, MSA, PSP, CBD, etc.), and she explained that there is a new treatment in the final stages of approval and will soon be available for Multiple System Atrophy patients.  She indicated that I am going to be eligible for its use and I feel very excited.

I will do my best to give an idea of what it is, how it was developed and the process that is being utilized to bring it to fruition.  At the conclusion of this brief summary, I will provide a link so that you may read about it further. In the studies utilizing animals, the agent PBT434 was used to target accumulations of iron found in the brains of animals.  This iron builds up and contributes to the clumping of alpha synuclein, the substance that misfolds and destroys brain cells.  This process of misfolding proteins causes clumps known as Lewy Bodies that bring on oxidative stress in Multiple System Atrophy, leading to movement issues that are disabling and contribute to severe disease progression.

The substance has shown the ability to stop neuron loss and improve movement disorder symptoms.  As a result of the studies that have taken place, human trials ensued and are leading toward the potential release of the drug for the treatment of MSA.  As you know I have MSA and Corticobasal Syndrome, two forms of Atypical Parkinsonian disorders that are brain diseases leading to severe disability.

If you would like more information, I have the following links that I have referenced in making this post:

https://www.parkinsonsmovement.com/pbt434-iron/

https://www.empr.com/home/news/drugs-in-the-pipeline/investigational-treatment-for-multiple-system-atrophy-gets-orphan-drug-status/

This is new hope that I have seldom experienced the 13 years I have had the MSA and CBS diagnosis. Thanks for visiting and for reading. -- Patient-Online

I have done some more looking, and a friend of mine let me know that the above drug may be a little further away from realization that I thought.  The one my physician was referring to may have been a different treatment.  I am going to be following up on this blog with more information.  In the meantime, the above looks like it holds promise.  Another being studied and that is further along in the pipeline is found here:
https://www.biohavenpharma.com/investors/news-events/press-releases/02-19-2019

Where Does the MSA, PSP or CBD patient fit?

Karrie looks out over the Back Bay in Los Osos.  Without her I would not be able to live at home.

I have been an outlier for years.  It is the nature of the beast.  I have an Atypical Parkinsonian Disorder, namely Corticobasal Syndrome with Multiple System Atrophy.  These syndromes are so similar to one another that they can be paired in my brain, and are hard to tell a part in another case where it would be considered one or the other.  On this site I have defined and summarized these syndromes, so this post will not consider those questions.

The issue I am discussing today is support. Support groups are a major factor in seeking support and education in a group where trust is established and fostered by the leaders and group members at large.

Let's take a few minutes and step back to look at my diagnostic history.  Those who have kept up with this blog are aware that "Dr. N" was my original neurologist who diagnosed me with a Parkinson's Plus disorder.  He conducted tests and examined me, concluding that what I had was "So much more than Parkinson's Disease."  That was in February 2006.  He said Progressive Supranuclear Palsy or Shy Drager were the likely diagnoses.  Over the years he sharpened the focus somewhat, but eventually he decided my case was unique enough that Parkinson's Plus was the most closely defined he could confidently label my case.  In 2012, Dr. N referred me to "Dr. S", who conducted Deep Brain Stimulation Brain Surgery in order to make two implants in my brain and one in my chest.  This treatment, though risky and hard to undergo, was a success in the way it has increased my overall health, lengthening my life.  This surgery was not a cure and is still benefiting me, though less and less over time.

Three years ago, I was fortunate to be referred to neurology at a respected clinic near our home and I was paired with a movement disorder specialist.  She ran tests, including DAT and PET scans, along with video taping my condition as I walked, moved my limbs and eyes.  She spent a great deal of time with us and we also met with a team of doctors who entered the exam room and contributed their opinions.

"Dr. M" determined that what I have is Corticobasal Syndrome with Multiple System Atrophy.  I have a very long list of symptoms, which, again, are well covered on this blog other places.  Among those symptoms are several movement issues that fall under the description of parkinsonism.  Parkinsonism describes symptoms that are found in Parkinson's Disease-- walking trouble, shaking, balance problems and slow movements.  Because I have parkinsonism, along with autonomic disorders, fronto temporal issues, neurologically based eye aiming struggles and swallowing issues that cause pneumonia, I have always tended to gather with Parkinson's Disease patients. Parkinson's Disease affects 1% of the population over 60 years old.  Atypical parkinsonian syndromes are rare and only represent 3% of the total Parkinson's Disease population.  The size of the group that is represented by PD means that there are Support Groups available in great prevalence.

Karrie and I were attending a Parkinson's Disease support group in Orange County as early as January 2006.  We enjoyed that group for a year and were recruited by the Riverside Parkinson's Support Group in November 2006.  We joined that group, stating from the beginning that I had a different disorder that shared some of the same symptoms.  We have developed many friendships in the RPSG over these 12-plus years, and Karrie and I led the group for most of four years between 2007-2011.  After all this time, it has become clear that my Atypical Parkinsonian disorder is not relevant enough to the needs of the patients, caregivers and leaders that are found in that group.  We love and care for these folks and their leaders, but feel that it is best that we not attend the sessions knowing that my diseases, which have some overlap with Parkinson's but are arguably faster progressing and more life threatening in the immediate sense, are frightening to the general group.

This Support Group issue is not peculiar to me, but is a universal problem that needs to be addressed by the Movement Disorder and Neurodegenerative Brain Disease community.  I have been fortunate to be welcomed and cared for by the Orange County group we initially joined, and grateful for all the years that we attended and supported the Riverside group.  We have been involved in online groups concurrently such as CurePSP, MSA Coalition, Brain Support Network, and a number of other organizations.  With these, we have had the chance to participate in online chats, but no face to face meetings.  There was a movement that Vera James, and Lorreta Mazorra provided leadership for as groups were gathered in Los Angeles at UCLA and in Orange County at UCI.  Robin Riddle of the Brain Support Network has organized an atypical parkinsonian syndrome support group in the San Francisco Bay area and she has made a number of us outside of that region honorary members.

I couldn't be more thankful to have had the opportunity to know so many of the people in these groups and so many have unselfishly listened, provided resources and taken time to voluntarily support families like ours, with a rare disease that overlaps somewhat with Parkinson's.

The point of this post is that those of us with Corticobasal Syndrome, Multiple System Atrophy, Progressive Supranuclear Palsy and Lewy Body Dementia are not frequently able to find a group in which we fit.  Typically, it is helpful if the Parkinson's Disease Support groups are able to recognize the reality that a small but important percentage of the PD support group participants will eventually be diagnosed with an atypical parkinsonian disorder.  It is because of this fact, that years ago I would lead discussions at the Riverside PSG in order to inform the patients, caregivers and their families about the signs of such a diagnosis so they would be able to recognize the onset of such a syndrome.

I am an outlier as a patient that does not specifically have Parkinson's Disease. There are people throughout the US and across the world that have this same distinction.  All of us could need support groups, if we choose to avail ourselves of them. I am advocating for more atypical parkinsonian groups to be formed within a reasonable driving distance so that patients and caregivers are able to support each other and learn from one another's experiences. When this can't be accomplished, Parkinson's Disease groups need to be better informed about the facts concerning MSA, CBS, PSP, and LBD and open their doors to theses conditions.  All atypical parkinsonian patients will benefit, as will Parkinson's Disease patients and their families.  -- Patient-Online

Reviving My Writing

Thanks for hanging in there with me.  I have been somewhat overwhelmed with both good, err, great things in our family and other physical and brain related things that have been challenging.  I plan to get my cyber pen warmed up and start to express my thoughts and communicate with you, my readers, friends and family that faithfully stand with us. 

Please watch for my next expression of questions, hope, faith and wonder at the surprises of this life we all live.  Regards,  Dan