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!
Please visit my fundraising page at the Huntington Society of Canada