The story of our dad and his battle with Progressive Bulbar Palsy (PBP)

PBP is a motor neuron disease in which there is no cure or standard treatment. The nerves controlling the muscles of chewing, swallowing, and talking are affected in people suffering from PBP. Because swallowing is difficult, food and/or saliva is often inhaled into the lungs, causing choking or gagging and increasing the risk of pneumonia. Death, which is often due to pneumonia, usually occurs 1 to 3 years after the disorder begins.

The story about my dad and his battle with PBP can be found at the following link: (click here). I hope the story provides some value and insight into the disease and my prayers are with anyone that suffers from this terrible disease. One thing that I talked about in our story was how helpless we felt during our 2 year battle with the disease. There were many times that a symptom would come up (e.g. not being able to sleep at night or the feeling of suffocation), and we'd struggle to find a solution. Anyway, I hope that some comments and suggestions on this site will be helpful to someone. Also, I'd like to mention that the doctors and nurses at OSU were very good to us - kudos to them!

Friday, June 22, 2012

Dola's story

Dola, thank you for sending me this story. My thoughts and prayers go out to you and your family. -- Ken


Hello

I lost my father very recently after he fought for about 2.5 yrs with PBP. The story of his sufferring is all the same and its very painful in my part to explain, especially when I was totally unware of the days ahead. On several occasions I treated him badly. We took him almost everywhere possible to get him cured, visited best doctors in the country. My dad was so active throughout his life, I dont remember any moment when I saw him actually resting. He was only 59 when he left us in May 2012.
I am writing to you not just because I wanted to share my feelings but also to tell you that we were never informed by our doctors that my dad was sufferring from PBP, we did not know what to expect with the progression of this disorder. We were kept in dark untill the day he passed away when the doctor who treated him at the end explained to us about PBP. Doctor said that he never explained and kept the facts of PBP concealled so as not to scare us. But what we feel now is that we would have treated him (psychologically) in a better way, getting him everything he wished for and helped him a bit to have lived a quality life, should we know about PBP earlier. It is important for the doctors to be straightforward and kind with the patients and their family. Hope that someone soon finds a cure...
Thank you so much for creating the blogspot for PBP, it is helpful for those who are progressing through it and their dear ones.
My prayers go out to them.
---
Heartbroken from India!
Dola

Tuesday, February 7, 2012

Reflecting upon the first six months

It's been over a year since my father died from motor neuron disease (progressive bulbar palsy specifically). Over the course of the year, my family and I have spent plenty of time reflecting upon the issues that occurred during his illness. We've had some very nice comments from people that have made it to our web site, and we've appreciated them very much. One question that has came up a couple of times has been 'what were the initial symptoms'. I believe that we had answered this in our story, but I thought that I'd reflect upon it again.

The initial complaint from my father was a slight slur in his speech. What specific letters (if any) were an issue, I do not remember. With that, he pursued talking with his family practitioner and then later, a neurologist. I attended the initial neurologist appointment with him and the only symptom mentioned was 'slurred speech'. The neurologist tested his muscle tone/strength in his arms and legs, tested for any lack of feeling in his extremities, etc. He ordered him to get an MRI, an EMG, and some blood work. Afterward, the neurologist didn't want to see him back for a few months.

During the wait for the next neurologist appointment (still less than 6 months since the initial appointment), my father did have some subtle choking episodes. I don't remember saliva being a problem at this point; however, by about 6 months it was probably adding to some of the subtle choking. To answer a few questions that we've had directly, he did not have any issues with biting his tongue or teeth pain. By six months, the speech was considerably worse and it never got any better from that point forward. Also, walking and arm strength was not a problem up to 6 months. His neck muscles were strong and turning his head and nodding was not an issue.

Remember, if you're concerned that you or a loved one may have PBP, don't draw any conclusions by what you read here. Go see your neurologist. Good luck and god bless.

Saturday, August 20, 2011

Walk to defeat ALS

The ALS Association is sponsoring many Walk to defeat ALS events throughout the country. If you are looking for ways to show your support or are interested in raising money, get active! We formed a team in honor of my dad. If anyone has interest in walking with us, please feel free to come and join us in Dayton, Ohio on September 18th 2011. There will be other walks that we plan on attending (e.g. Columbus,Cincinnati, etc). For donations to anyone on our team, use the following link (see below). Thanks and god bless.

http://web.alsa.org/site/TR/Walks/CentralandSouthernOhio?team_id=204851&pg=team&fr_id=7428

ALS and trichostatin A

I've been looking for recent experimental findings that may benefit ALS patients. One recent article suggests that it's possible trichostatin A (TSA) may have some ability to help prolong lifespan (at least it seems to in mouse models). The article is published in Experimental Neurology 2011 147-159 by Young-Eun Yoo and Chien-Ping Ko.

One interesting premise for their work is that progressive motor neuron death in ALS may involve the participation of non-neural cells (glial cells, etc.). Most research that I've read seems to target motor neuron cells specifically, so this seems like a good approach. Their idea was to target not only motor neuron cells but neighboring non-neural cells. What they had found was TSA shows a protective effect in different cell types implicated by ALS. How did they test this? They used a mouse model and at day 90 and after disease symptoms appeared, injected TSA into the body cavity (intraperitoneally) and continued to do so until day 120 or end of life. What did they find? It seems that TSA reduced motor neuron death and axonal degeneration in SOD1-G93A mice. TSA also helped muscle wasting and neuromuscular junction loss (denervation) and enhanced motor functions. TSA increased survival duration by 18% and lifespan by 7%.

Conclusion, their findings may tell us that TSA may be a theureputic agent to help slow the progressive of ALS.

References
Y.-E. Yoo, C.-P. Ko / Experimental Neurology 231 (2011) 147–159


Friday, July 1, 2011

Heavy metal detox

My goal is not to advise you here; however, my goal is to document an approach used by my father and myself to slow the progression (or stop the progression) of his progressive bulbar palsy. Our story doesn't end well, but I do think it has value. There were certain things that we waited too long to do in our approach and we were obviously uncertain if anything would have helped.

7 months into the progression of the disease, we had decided to try a heavy metal detox regiment. My father was already a vegetarian and was a proponent of juicing which he tried to do at least once a day. When we began to realize how serious his situation was, we researched everything we could. Regarding PBP specifically, we found very little information. Note that the doctors had yet to give us a diagnosis, but the symptoms were leading us to progressive bulbar palsy (or possibly (but not likely) a stroke or myasthenia gravis). In addition to not receiving a diagnosis, we weren't getting advise or 'next steps' or 'things to do' which was nerve racking and stressful for dad and our entire family.

Back to our approach, many years prior, dad was suffering from consistent heart problems. He had undergone open heart surgery in addition to 4 prior stents. In the end, it was a holistic approach that finally allowed him to survive. Yes - the stents and open heart allowed him to survive to a certain point; however, there was a point in which the clogged arteries couldn't be bypassed and it was his holistic approach that let him survive for many more years. He was a firm believer in holistic health and holistic approaches to healing. At the same time, he was practical and also realistic.

At this point, we had felt that we only had a few options to try - increasing antioxidants, introducing a heavy metal detox regiment, and avoiding toxins. (On a side note: a few months later we had picked up the book 'Eric is Winning' and we started down the road of removing amalgam -- more on this some other time). After some investigation and at 7 months into the disease we ordered a product called NDF plus (Note: i'm not promoting this product). This product was one of the only products that seemed to have good science behind it and seemed to prove to be a good heavy metal detoxification agent. I won't go through the marketing pitch with you; however, it does seem to be a good heavy metal detox remedy. Studies with this product show elevated levels of mercury, aluminum, cadmium, etc. Dad took this product consistently for 3-4 months but not in the full dose since he was on a high blood pressure medication and was worried about the 2 interfering. He increased his juicing, increased his antioxidant intake, and avoided toxins.

The story doesn't end well as i mentioned. My father died at 25 months after the first symptom. Some interesting points to anyone who wants to take a similar approach. My father couldn't take the full doses of NDF and he didn't start it very early in the progression. We also waited too long to remove any amalgam fillings - this was a big disappointment for dad. Furthermore, he felt that he needed to stay on various blood pressure medications through the entire disease progression which didn't allow him to detox as aggressively as he could have. We both felt that juicing helped delay the start of the disease - my father was 74 when the symptoms started (which is older than the average PBP patient). Last thing, if you or a loved one has symptoms and if I can answer any empirical questions - please feel free to ask. God bless.

Saturday, June 18, 2011

Progressive bulbar palsy, nutrition, and toxins

I had picked up the book 'Eric is Winning' back in May 2010. This book is written about a guy with ALS who believes that he has prolonged his life through diet and supplementation. When I picked up the book, I wasn't sure how my dad would feel about it. My dad was always a 'stick to the facts' kindof guy, and this book is mostly the observations and opinions of one person. Anyway, to my surprise, after a week or 2 my dad had read this book cover to cover, had it book marked, had an action item list from it, and was ready to move forward with many of the suggestions in it. Did I think that was crazy? At the time, I did think it was a little crazy.

The main points that Eric, the author, makes through the book are that toxins are a core cause of many diseases and ailments (a scientific fact - I'll write more about this later). If you look around you, it's obvious how many toxic substances we expose ourselves to everyday. Think about the chemicals that we put on our lawns (even though mine still looks bad), or the chemicals in our foods (dyes, preservatives, etc), our hair sprays, cooking sprays, etc. Now, here is the point that I didn't appreciate when I had picked up the book. There really is some good, valid research that shows toxins (e.g. Copper, Zinc, Manganese, Magnesium, etc) are a good possibility for leading to AlS/PBP and other neurological diseases. In particular, I just read an article Patterns of levels of biological metals in CSF differ among neurological diseases from the Journal of Neurological Sciences 2011. The findings suggest that copper and zinc play an important role in the onset and/or progression of ALS and that copper chelation agents and modulators of copper and zinc can be candidates for treatment.

So, back to Eric's point about toxins causing neurological diseases, in many cases he would be correct. So, if you follow his logic, the next logical thing to do would be to avoid all toxins and detoxify your body. He has some good suggestions on dieting and provides us some insight into his supplementation regiment. Anyway, if you think that you possibly have PBP or ALS, this book may be a good read for you. Best wishes.

Monday, May 9, 2011

Progressive bulbar palsy and power wheel chairs

It was just six months ago that my dad and I were looking at power wheel chairs. He was beginning to lose his ability to stand up and sit down, and he was losing strength in his legs and right hand. We really needed help. It was incredibly difficult to find the right medical devices for the symptoms that we needed to deal with through this entire process. This goes back a few years when speech and swallowing started to become a problem, and it seemed like no one could really offer us any good help. Just 4 months before dad passed, we had a recommendation to get a power wheel chair - a really nice, really expensive power wheel chair. Being the conservative people that we are, it was mind boggling to spend $20,000.00+ dollars on a chair that we weren't confident would help. Unfortunately, when you are frustrated and at wits end, you begin to do 'whatever it takes' or whatever you think it will take. We bought this wheel chair and it was delivered about a month before dad died.
Due to the rapid progression of the disease, dad never had a chance to use the chair. It was too big for the house and I doubt it could have been manipulated by him as the disease progressed. Now that my dad has passed, we've been looking for good places to donate the chair to. From our investigation, it seems that many associations have 'loaner closets'. These 'loaner closets' allow groups (MDA, ALS Association, etc.) to loan medical devices to patients as the need arises. The picture that I've included here is a picture of our Quantum Q6 that we donated to the MDA lone closet. Our hopes and prayers are that it gets used by someone that can really use it.