Before I set out to see this province on my hike for Huntington Disease awareness and research, I thought I would like to profile my hometown of Vanguard, Saskatchewan by doing a little walking tour.
We are celebrating Canada’s 150 this Saturday, July 8. There will be a social, dance, inflatable battle rings, and a huge fireworks display.
I have worked out a tentative schedule for my walk across the province of Saskatchewan in support of Huntington Disease research and awareness. I totally expect the schedule to be ruined by noon of the first day but if all goes as planned (which it never does) I should be on the road from July 10-27. Here is the breakdown:
July 9 – Sunday
Leave from my hometown of Vanguard SK. Drive to Macklin.
July 10 – Monday
7:00 a.m. – Leave the Alberta/Saskatchewan border.
8:00 a.m. – Arrive in Macklin SK – The rest of the towns are all in Saskatchewan.
I am 10 days away from starting my walk across Saskatchewan! My school year is all wrapped up. It is always a little sad at the end of the year knowing that another year is gone but there is also a sense of accomplishment and fulfilment.
My campaign is going very well. I am over 70% towards my stated goal and still haven’t started the walk yet. Also, there have been a couple of interesting developments. Some folks are concerned about my safety and well-being and as a result, have been working towards potential sponsorship so that I have a pilot vehicle, places to rest at night, and proper nutrition. I will have a formal announcement with details once it is all in place, but isn’t that cool?
What is Huntington Disease (6)?
Quick Facts:
1 in 7000 people in Canada has HD.
1 in 5500 Canadians is at risk of developing HD.
1 in 1000 Canadians is affected by HD in some way.
A direct genetic test to determine if an individual has inherited the HD gene was developed in 1993. Most people at risk do not get tested.
Huntington Disease is an autosomal dominant inheritance. This means if you inherit the HD gene you will get the disease.
Unity is a town with a population of 2389 people. It started as a small settlement in 1904 and was the subject of a 2001 award-winning play about the 1918 flu pandemic. It is 59 kilometres from Macklin, meaning that I will likely be in Unity on July 11.
I have started to limit the kilometres that I am putting on in training. I might as well save them for July 10.
One very large upside of just walking is being able to see the countryside. I used to run the same routes when I would be training for running events or just jogging, but I never enjoyed the scenery as much when I ran as opposed to when I now walk. Here are some pictures:
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Did You Know:
At my height (5 feet, 11 inches) on flat terrain, the horizon will be about 5 kilometres away. Therefore, in order to make it across Saskatchewan along my route, I will have to “walk to the horizon” about 140 times.
Most Listened to Song on My Playlist:
Nothing Compares – Third Day
I am going back to Third Day for this one. For some songs, you immediately want to pick up a guitar and play along. This is one of those songs.
I did, in fact, pick up the guitar to play this song, but instead of playing the original A, E, F#m chord progression, I switched to G, D, and Em (In fact my computer even knows I don’t like playing any other chords so it just automatically transposes for me now.) As I started playing, it reminded me of this 1998 Semisonic song.
Three weeks, that is all I have left before setting out on my 700-kilometre hike across Saskatchewan in order to raise funds and awareness for the Huntington Society of Canada and Huntington Disease research.
It is has been some time since my last post. Life has a way taking you on rabbit trails before allowing you back on the path again. My last post was before the May long weekend. That weekend I took my son to play in his club volleyball national tournament in Calgary, Alberta. I went to sleep that Friday night and woke up with a sprained ankle in the morning. Apparently, although no believes me, spraining your ankle while sleeping is actually a “thing; ” Yet something else to look forward to in your forties.
I was also involved in two graduations, one at my own school in Vanguard on the 26th of May and then my daughter graduated from Athol Murray College of Notre Dame on June 17th. I am proud of Vanguard Community School and our graduating class and I am proud of my “little girl” Jessalyn.
I did have to rest for about 10 days while my ankle healed, but eventually, I did get back out on the road and have since put in a few hikes. June has been just too crazy though and I have not been able to put on as many kilometres as I would have liked. I will have to train as I walk somewhat, starting on July 10.
My school had a surprise for me on June 14. Some of the senior boys asked if I could go out to the woodshop with them while they built a box for another class. I thought it was a strange request but went anyways. While out in the shop I was called on the intercom to come to the gym. I thought a student had gotten hurt. When I got to the gym, the entire school was waiting for me. Without me knowing, the students and staff had planned their own little “walk” and were sitting in the gym with backpacks on waiting for me to join them. In total, they also raised nearly 2000 dollars for my campaign. This, plus donations that I have already received, has put me over halfway to my goal and I’m still three weeks to the starting line. Cool!
What is Huntington Disease (5)?
You will often hear the analogy that Huntington Disease is like having ALS, Parkinson’s Disease, and Alzheimer’s at the same time. This is mostly correct but doesn’t help much unless you know what those diseases are, and what attributes of those diseases can be found in a person that suffers from Huntington Disease. Here is the breakdown:
The full name for ALS is Amyotrophic Lateral Sclerosis. When you see that word trophic, it comes from the Greek trophikos for food or nourishment. Sclerosis means hardening or stiffening (dying). In the case of ALS, neurons in the brain responsible for voluntary muscle movement die. With nothing controlling them anymore, the muscles stiffen and eventually waste away. This is the common element with HD. Although not caused by the same neuron malfunction, in late stage HD, you will see muscle wasting to the point where HD patients in the latter stage of their life look extremely thin, fragile, and malnourished.
Parkinson’s Disease happens when brain cells stop producing dopamine. Dopamine is necessary to carry signals between nerves in the brain. Without it, people start to exhibit the hallmark signs of Parkinson’s: tremors, rigidity of muscles and impaired balance. Again but for different reasons, someone with HD will begin to display these same characteristics. This is the chorea or “dance” movements associated with HD.
Alzheimer’s or more specifically dementia caused by Alzheimer’s is also very HD like. This is where an individual with HD suffers from a host of cognitive and emotional issues; everything from memory loss to forgetfulness to irritability.
So, yes, in a sense Huntington Disease can be described as having ALS, Alzheimer’s and Parkinson’s all at once but I would even go further than that. It is more like a person starts with Alzheimer’s (or dementia or even schizophrenia), then develops Parkinson’s, and then finishes off with ALS. You could even use another analogy and say that HD is like someone having a very prolonged and slow developing case of the dreaded Creutzfeldt–Jakob disease (people with this disease often die within six months, whereas an individual with HD can have symptoms for 20 years or more).
All of these diseases are just plain and simple nasty!
Featured Town:
Evesham, SK
The next place I walk through after Macklin is a hamlet called Evesham. I looked it up and it is a hamlet of 35 people. It used to be a village up until the year 2000. That is about all I could find on Evesham. Here is a picture of a church in Evesham:
I will look to see if it still there when passing through.
This Day in Training:
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It was a great day for hiking. The temperature was a perfect 21 degrees and there were no bugs. My ankle held up well as I went about 8 kilometres. On this one stretch of highway, this one hawk keeps diving at me. At first, I didn’t think anything of it, but she definitely does not want me around!
Most Listened to Song on My Playlist:
Cry Out to Jesus – Third Day
Upon hearing Third Day’s music, they became my third favourite band (Triumph and The Tragically Hip will always be my favourites). Third Day’s music always makes me feel better. Their lyrics mean so much, and their sound is like a Christian version of Lynyrd Skynyrd. I can’t imagine a scenario where you feel worse after listening to this song.
I put on 25 more kilometres since my last post. The hikes are getting easier and I am feeling more confident that I can reach my goal of hiking 50 kilometres per day and 700 kilometres across the province of Saskatchewan in July for Huntington’s Disease research and awareness.
Today, my hike became official. The Huntington Society of Canada has helped me with planning and promotional materials. Check these out:
Canada 150 – I will be visiting some great places this summer of Canada’s 150th year so I will use this opportunity to promote some of our wonderful Saskatchewan towns and cities.
Macklin is a town of about 1500 people and is most famous for the game of Bunnock. The object of this game is to throw the ankle bone of a horse towards a row of someone else’s ankle bones. It is kind of like playing horseshoes or lawn bowling.
I had a close call today. I was hiking down a dirt road west of Vanguard with my headphones on and head down. Expecting no traffic, I wasn’t particularly paying attention when I looked up to see this:
That tail is up and he is pointed at me! I think another if I had walked another 10 feet, I would have been in for a major surprise!
I did an exit stage left, walked well around and left him behind me.
Disaster averted.
What is Huntington’s Disease (4)?
Huntington’s Disease used to be known Huntington Chorea. The two names are synonymous. Chorea is Greek for dance. It is the sporadic, random and never-ending movements that have become symbolic of the disease although, as I mentioned in a previous post, cognitive and behavioural symptoms appear much earlier than the Chorea stage. Here is an example:
Another 16 kilometres hiked since the last time I posted. Yesterday was cut short by rain. I know this summer, it won’t matter if it is rain or shine, but at this point, I don’t want to push it and risk not being able to train. I was hoping to get in 20 kilometres before the rain hit. Today I went 10 kilometres after school. It was an easy hike but I do get a sore back after about 8k on the road from carrying the backpack.
Again, my plan is to walk 700 kilometers across Saskatchewan this summer beginning on July 10. In the meantime, I am tracking my training days and writing about HD.
What is Huntington’s Disease (3)?
Today, I want to write a little about the early symptoms of this disease, since this an area of increased focus lately. In the past, an HD diagnosis usually coincided with the onset of chorea (Greek for dance). Chorea is the name for the random and sporadic movement that plagues someone with HD. From this point on, the disease is fairly predictable in terms of its progress towards eventual death. The pre-diagnosis stage of the disease, however, is often the most misunderstood and most difficult stage of the disease to deal with for HD sufferers and the people closest to them. The reason is that you don’t see it coming; especially if you haven’t been tested for the HD positive gene or if you didn’t know that HD was in your family history.
We now know that up to 10 and sometimes even 20 years before the physical manifestation of the disease, subtle changes in the HD positive individual start to occur. This happens because the malfunction in the HTT gene starts to destroy the brain. As you likely know, different parts of the brain control a multitude of different cognitive, personality, physical and emotional functions, so prediagnosis HD symptoms will vary from individual to individual depending on what part of the brain starts to die first. Generally speaking, however, most prediagnosis HD individuals will notice (or more likely people around them will notice) a slight but progressive decrease in cognition accompanied by personality changes.
Decreased attention span, short-term memory lapses and an inability to learn new tasks are all hallmarks of early onset HD. In addition, procedural knowledge starts to deteriorate a little later, but still relatively early in one’s battle with Huntington’s disease. Our brain stores two types of knowledge, declarative and procedural. Declarative knowledge is factual and dependent upon our ability to process information and later recall it. Procedural knowledge is simply our knowledge of how to do something. This includes everything from tying our shoes to driving a car or doing long division. Now imagine getting into your car one day and not remembering how to get it into “drive” or, imagine waking up one morning and not having a clue how to get dressed. That piece of the brain responsible for that particular procedure just disappeared. This happens in an HD-positive individual and usually well before any diagnosis is made.
Perhaps even more unsettling is the personality change that usually happens in a prediagnosis HD individual. Hair trigger temper, high anxiety, depression, irritability, apathy, impulsiveness; any of these or all of them can plague someone with HD in the prediagnosis stage. Once again, imagine having a certain predictable personality for 40 years and then, one episode at a time, changing into someone completely different. Imagine the effect on the people around you as they “wonder what the heck is going on?”
All of this happens in a person with Huntington’s disease and this is before they have even had a diagnosis!
Did You Know?
Just last week on May 4, Bill S201 received Royal Assent making Canada the LASTof the G7 nations to protect its citizens from genetic discrimination. In March, Justin Trudeau ordered his backbenchers to vote against Bill S201 but in an act of defiance they ended up voting against their own Liberal government and with the Conservatives and NDP to pass Bill S201 into law.
This Day in Training:
Distance: 10 kilometers hiked; 16 over the last two days.
Backpack: 20 lbs
Temperature: 17 degrees
Conditions: Rain on Sunday. No rain or wind on Monday.
Most listened to song on my playlist:
Yer Not the Ocean – The Tragically Hip – Even people that don’t “get” The Hip still like this 2006 song. It is my favourite song to play drums to.
Today was another training day for me. I didn’t get as many Ks in as would have liked, but still had a decent hike; 8.8 kilometers for the backpack hike and 12.85 kilometers total for the day.
As per my format, I will try and update some information about Huntington’s disease in order to build awareness as well as journal my activity in preparation for my walk across Saskatchewan this summer.
What is Huntington’s Disease (2)?
Previously, I posted that Huntington’s disease (HD) is an inherited brain disorder. The problem is in a little DNA segment known as CAG trinucleotide repeat. Here, the building blocks cytosine, adenine, and guanine (CAG) repeat 10-35 times in the normal HTT gene. In a person with Huntington’s disease, the CAG repeat in this gene is greater than 36. If the repeat is from 36-39 a person may not develop symptoms of the disease but can still pass it on to the next generation (whose CAG repeat is likely higher). If a person inherits this gene and the repeat is over 40, it will almost always lurk around until a person is 30-50 years old and then systematically destroy the frontal lobe of the brain over the next 10 to 20 years. Early symptoms will vary depending on what part of the brain is affected and when, but the disease will always result in uncontrollable, jerky movements, total impairment, and death (prognosis is this 10-20 years after becoming symptomatic) in its advanced stages. Some people will show symptoms earlier than 30 and likewise develop symptoms after 50, but the vast majority of people with this abnormal HTT (ie. Huntington) gene will become symptomatic between ages 30 and 50. Unfortunately, the faulty gene is dominant, meaning if you inherit it, you get Huntington’s disease; there’s simply no way around it.
Very nasty stuff and it is all because of a tiny hiccup on one gene.
This video explains in more medical terminology what goes on with the Huntington gene and its effect on the brain:
Did You Know?
Huntington’s disease is named after Dr. George Huntington who published a paper on his studies of the disease in 1872. Huntington came from a family of doctors who lived in East Hampton, New York and who over generations had observed several residents of East Hampton suffering from a condition known then as “St. Vitus’s Dance.” This included Phebe Hedges who also suffered from St. Vitus’s Dance and who famously committed suicide by walking into the sea in 1806. Huntington made the connection that this condition was passed on through generations and thereafter, the disease bore his name rather than the previous incorrect label (which actually refers to a childhood disease that produces similar bodily movements but goes away after a few months).
This Day in Training:
Distance: 8.8 kilometers walked
Backpack: 20 lbs
Temperature: 21 degrees
Conditions: No wind but bugs were out for the first time. I hope the mosquitoes are not like they were last summer where they basically took over the yard in mid-June.
Most listened to song on my playlist: I basically listened to two songs over and over today.
Here Without You – 3 Doors Down – Just a beautiful classic song from 2003. Also, it is a great road song.
Tell Me Ma – Rankin Family – This song really works when you need to pick up the pace!
May is Huntington Disease Awareness month and also a time for me to step up my training. I started in April with about 150 kilometers of walking and I want to double that in May. I also want to inform people about Huntington’s Disease
What is Huntington’s Disease?
Huntington’s disease (HD) is an inherited brain disorder. HD causes cells in parts of the brain to die: specifically the caudate, the putamen and, as the disease progresses, the cerebral cortex. As the brain cells die, a person with Huntington’s becomes less able to control movements, recall events, make decisions and control emotions. The disease leads to incapacitation and, eventually, death. There is no cure.
Huntington’s disease is a genetic disorder. The HD gene is dominant, which means that each child of a parent with HD has a 50% chance of inheriting the disease and is said to be “at-risk”. Males and females have the same risk of inheriting the disease. Huntington’s occurs in all races. Symptoms usually appear between the ages of 30 and 50, but the disease can appear in children or seniors.
Most listened to song on my playlist: Today by the Smashing Pumpkins – Interesting story: I never really got the name of this band, until I lived in Cochrane, Alberta. I awoke on November 1, 1997, to find that all of the Jack-o-Lanterns on our street had been stolen and smashed on the street. Later in 2006, when teaching in Vanguard, Saskatchewan, I told this story to my Grade 12 English class on Halloween. The next morning the streets of Vanguard were littered with smashed pumpkins. Several townspeople blamed me for the incident.
I actually like this acoustic version of the song:
Just Learn 