Walking in Support of Huntington’s Disease Research and Awareness – May 8

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 LAST of 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.

Please visit my fundraising page at the Huntington Society of Canada