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:
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.
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: