Educational and fundraiser
- jjj0urney
- Nov 20, 2017
- 10 min read
So this blog came about today (November 8) after some information I posted on a fundraising site was explained well enough that they wanted me to share with ALL. It has been posted before in different ways, but I don't think everyone reads all the posts unless I post on Facebook sometimes. So, I have decided that everything I post for a a little information session on the fundraiser, I will post it here too. I plan to do 5-6 between November 1 and Thanksgiving, so this blog date will change and be updated accordingly. Fundraiser information is up top and the education posts for the fundraiser are below.
The Rodan and Fields fundraiser:
A friend and coworker of mine from when I worked at Emory had been thinking about doing a fund raiser for Jason and I for some time. As a consultant of the Rodan and Fields skin care products, she gets compensated for her orders and recruits and plans to donate her November work to us. In the facebook group, Gerrie Thomas is giving all the product details about Rodan and Fields November 1-Thanksgiving. She became my consultant more than a year ago when I decided to try it out myself! Although I am terrible at keeping the regimen up, I have enjoyed the products and I always say that it is hard to take care of others if you don't take care of yourself! Gerrie is amazing, so easy to talk to, and just the SWEETEST for doing this for us. She is super woman herself taking care of 3 little girls at home. While Gerrie is posting information on the products, I have been posting education information sporadically about ALS.
****If you have been interested in these products, I can add you to the group, but my purpose of this blog was really to just share the education (or re-share so it is all in one place), plus some of the items are new and updated since whatever post I might have pulled from! For example, the November 8 was pulled from a September 2016 post.
Education items:
November 4:
What is ALS and where is Jason in disease progression?
It is also known as Lou Gehrig's disease, named after a Yankee baseball first baseman who was diagnosed with the disease in 1939. He played for the Yankees for 17 seasons from 1923-1939. The disease became in the spotlight a few years back with the Ice Bucket Challenge as well, a way to raise money AND AWARENESS about the disease. I am big on awareness, if you couldn't tell! ALS is a progressive nervous system disease where nerve cells gradually break down and die, leading to disability and the inability to control muscles needed to move, speak, eat and breathe. ALS doesn't usually affect bowel or bladder control or ability to think. Doctors do not know what causes the disease, although 5-10% of case are hereditary and caused by gene mutation. There is no cure for the disease and life expectancy is 2-5 years. There are only 2 FDA approved drugs that are supposed to slow the progression, rilutek (approved in December 1995) and endaravone (approved in May 2017)
Jason's disease is not hereditary. Muscle weakness just started happening one day and we do not know why. Today, he has little movement that he can control although he can feel everything. His skin is actually more sensitive than before. He can feel aches from being in one position just like the rest of us. He can push his left leg into a straightened position from bent and he can moved 3 fingers slightly on his left hand. He can make small head movements and move some toes too :) He can no longer speak (this has worsened over time since he got the trach), but can mouth words to me and alert me to when he needs something. He got a trach back on May 1 of this year and his breathing is dependent on it-the machine makes sure he breathes 14 breaths/minute. This saves so much energy and calories because he doesn't have to think about breathing. He got a PEG tube for eating back on April 21 of this year after trouble swallowing and 25%+ weight loss. Doctors say he has "advanced" ALS, but his spirits are way ABOVE average. All activity is taxing, so although we live each day to the fullest, we spend the majority of time at home watching sports and spending time together. Jason is still working, so the computer is right beside us all day long.
We are SO EXCITED today because just yesterday his eye gaze communication device was delivered! This means a full weekend of getting it set up. (see post from November 4)
November 8:
We get a lot of questions about research, why there is no treatment, and why we aren't doing any studies right now. I hope this answers some of those questions...
The reason there isn't a good treatment is because all the different forms of ALS are grouped together, instead of treated separately. In comparison, cancers are all treated completely different. 15 breast cancer patients may have 15 different treatment plans according to the expression of proteins and look of their cancer (as an oncology nurse, there are more than 50 treatment plans-I kid you not!). Then there are treatment plans for colon cancer, lung cancer, renal cancer, skin cancer (you get my point!) In ALS, no matter spontaneous or familial, no matter how it presents, no matter the degree of progress, no matter what genes, are ALL grouped into the same category. So, it may make sense why there has been little progress in treatment.
Treating mice in research studies helps a VERY LITTLE percentage of the ALS population. And guess what? They use mice. Bear with me....I'll go back to cancer, because this is what I know! You can give a mouse cancer by inserting cancer cells into the mouse and then see what works. You cannot give a mouse ALS. You can give a mouse a gene and figure out how to treat that. The majority of research has been based on the SOD1 gene discovered back in 1993. However, the SOD1 gene only accounts for about 10-20% of familial ALS cases and 1-2% of sporadic ALS cases (no family history).
These are the numbers...
-There are approximately 5000 new diagnosed ALS patients in a year
-approximately 500 patients will have familial disease (SOD1 gene would be in only 50-100 patients)
-approximately 4500 patients will have sporadic disease (only 45-90 will have the SOD1 gene)
-With these numbers, the SOD1 gene studied since 1993 is only present in 95-190 patients....of 5000 diagnosed yearly
-it is heartbreaking to look at it this way, but the only FDA approved drugs are based off the study of this 1 gene.
Riluzole was FDA approved in 1995 and Jason has taken it since March 2016. In May of this year endaravone was FDA approved. Endaravone isn't always covered by insurance (you actually have to apply for it) and is not readily available yet. If we did this treatment, Jason would get IV infusions nearly 1/2 the days of the year, the chances of effectiveness are close to zero, and it is not a great quality of life. Lets not be too disappointed though. While doing 190 days of IV therapy a year is not for us because it has only proven to slow progression is such a small number of sporadic cases, it may be an option for some and it is good that research is moving! Research is happening and Jason made choices to live with the disease because we are hopeful something will look good for us in the NEAR future!
We love the ALS association and did a walk back on October 7 to raise funds. This is why I feel awareness is key-awareness leads to support of groups such as ALS association. Awareness leads to funding. Funding leads to treatments, cures, and support of patients.
November 12:
Diseases always seem to come with risk factors, and ALS is no different BUT there seems to be only commonalities among patients involved, not necessarily risk factors (but this is just my opinion!) in the cases of sporadic disease. Over the past 20 years, knowledge on genetics and the way ALS happens in the body has been gathered and most doctors and researchers agree that non-genetic factors exist, but there has been little progress made in identifying the factors.
Risk factors that I've collected from various places:
-ALS chances increase with ago, with most cases are diagnosed between 40 and 60 years old
-Men are slightly more likely to get the disease before the age of 65. At the age of 70, the sex of the person does not matter
-Being a veteran or athlete increases risk because they are shown to be exposed to psychological exertion and trauma
-High lead levels in the blood (FYI-in the very beginning, we did do lead and metal testing on Jason)
-Exposure to lead, pesticides, BMAA, or enteroviruses (oh…..the list goes on and on, so not very helpful)
-History of head trauma, metabolic disease, or neuroinflammation
-Higher intake of antioxidants and vitamin E show to decrease the risk
-“electrical” occupations increase your risk (but the list of occupations is LONG....athletes, carpenters, cockpit workers, construction workers, electrical workers, farm workers, hairdressers, house painters, laboratory technicians, leather workers, machine assemblers, medical service workers, military workers, nurses, power production plant workers, precision metal workers, programmers, rubber workers, shepherds, tobacco workers, veterinarians, and welders) so, does working increase your risk?
Specifically regarding Jason and what we’ve experienced:
There is a strong clinical impression that patients with ALS have a higher level of physical fitness and lower body mass index than the average person. Dr. Glass, our doctor at the Emory clinic, said when he first met Jason something to the effect of "let me guess, you are an athlete with no other health issues" He states that when he meets a young ALS patient, they are very athletic and tall. When diagnosed at the age of 32, Jason was very stereotypical of what doctors see. He is athletic, smart, tall, handsome (OK-this I added!), and otherwise healthy. It just happened one day. As a matter of fact, Jason tri-lettered in high school, was valedictorian of his high school, played collegiate soccer, and is an engineer. He has a trunk full of awards, trophies, plaques, and medals at his parent's house. After moving to Atlanta, he played both indoor and outdoor soccer and had to stop one month after diagnosis. Every weekend, he did his own yard work and was my own personal handy man. We bought a house that did not need fixing up, but every few months we made improvements-it’s just who Jason is! There was nothing he wasn’t good at (well, he wasn’t good at yoga or flexibility, so he just didn’t do it!) I found this article and for me, it maybe gives me an explanation of how it developed in Jason: “Researchers say they aren’t sure why there’s a higher concentration of motor neuron disease among top athletes. But they suggest that vigorous physical activity might somehow increase exposure to environmental toxins or make it easier for the brain and body to absorb these toxins. Another possible explanation may be that the added physical stress of being an elite athlete might make them more susceptible to disease.” (”Athletes and Lou Gehrig’s Disease,” 2002)
References:
-Athletes and Lou Gehrig’s Disease (2002, September 9) Retrieved from https://www.webmd.com/fitness-exercise/news/20020909/athletes-lou-gehrigs-disease
-https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4334292/
-https://www.mayoclinic.org/diseases-conditions/amyotrophic-lateral-sclerosis/symptoms-causes/syc-20354022
Below are a few picture of Jason in all his athletic glory
November 19: Choices and ALS
There are so many things that ALS takes away from a person and their loved ones that cannot be controlled. Phrases such as “loss of the ability to walk, eat, talk, and breath,” “independence,” and “takes away a person’s dignity” are common when referring to what ALS takes away. I cannot begin to tell you all the things it can take away from what I’ve seen in the day in and day out. How many times does someone with no mobility issues look at the evenness of sidewalks or pathways, accessibility of entrances or the bathroom when making plans to go anywhere or do anything? Or even personally related to us and our marriage, it took away our care-free honeymoon and our 1-year anniversary gift to one another. It can be financially devastating. It can be emotionally devastating. It really changes all plans and thoughts from that point forward.
I could go on and on, but instead of choosing to focus on what it takes away on a daily basis, I want to talk about what ALS cannot take away. It has not taken away Jason’s choices. If you’ll remember, while physical function gets taken, mental capacity stays the same. Every patient is given the choice to decide interventions to be made versus not. It is not an automatic process to go through everything that has happened for us in the year 2017. The doctors give us the information, we do research and discuss what we (more importantly JASON) wants, and have made those decisions as needed. In medical terms, this is often called “life extending treatment” versus “palliative care.” These interventions specifically refer to PEG tube placement for nutrition and ventilation via tracheostomy to sustain life. As many as 50% of patients choose to get a PEG and as many as 30% of patient choose to go on a ventilator. This REALLY depends on the study or article that you are looking at AND the country though (I challenge anyone to find an article with good data-I just haven't found it!). It seems that with the studies I’ve looked at, patients that wanted the interventions were likely to be newly diagnosed, were younger, had a lot of involvement and activity in life (I’ve actually specifically read ‘expressed greater attachment to life’), and showed very quick changes in pulmonary function or weight decline. I think this refers to all the choices made throughout the journey though….from job choices…to staying in Georgia…to wheelchair, lift, van purchase, bathroom remodel, house accessibility, eye gaze communication…travel choices…everyday choices... I mentioned in a previous post that Jason chose to LIVE with ALS, and this is what I mean. He has been through so much and is yet to go through so much in order to live with us longer. He has chosen to adapt to a new way of life with support and science with hopes of maintaining the best possible quality of life.
This Thanksgiving, I am so thankful for his bravery in acceptance of a somewhat unknown and trust in me to be his "person." I am thankful that he has chosen to live with ALS.
As a cancer nurse, I’ve seen patients get so much hope from the poem below. I am putting it here because I think it would be appropriate to replace “cancer” with “ALS”
What Cancer Cannot Do
by Dr. Robert L. Lynn
Cancer is so limited…
It cannot cripple love.
It cannot shatter hope.
It cannot corrode faith.
It cannot eat away peach.
It cannot destroy confidence.
It cannot kill friendship.
It cannot shut out memories.
It cannot silence courage.
It cannot reduce eternal life.
It cannot quench the Spirit.
Grant me the serenity to accept the things I cannot change,
The courage to change the things I can,
And the wisdom to know the difference.
Reinhold Niebuhr
Articles of interest:
***This article is about the thought processes that need to happen prior to getting a tracheostomy. It may show why only about 30% of people decide it for themselves-NOT an easy decision and we shed some tears in the days leading up to it, but I am positive that Jason would not be with us for the holidays without it.
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