To My Newest HD Angel

After years of my watching her progression from a front row seat, my oldest sister finally passed away almost two months ago now. I have been dragging my feet at writing this final chapter of her life as I knew it was going to make me sad all over again and stir up all the grief I still feel in my heart for her.

In the five weeks before her death she had a stroke and as her sister, seeing her in diapers and then having to be fed minced food as she had lost the ability to chew with the stroke, I was saddened at the further hit to her pride.

After five weeks she had a second stroke but because she was marked with the wrong DNR rating she was revived and put on oxygen at the hospital which never ever should have happened as she had specifically said she wanted no life-saving or prolonging measures.  But apparently because she lived in a care home her DNR order was level system instead of a straight DNR.   They said she would not last the night even with the oxygen on but she did.

So once she was on oxygen it gave me and my other sister no other choice but to pull the oxygen, knowing it was the last thing she wanted.  With that second stroke, she could not even swallow food or water now.  So we pulled her off the oxygen and it took her another four days after that to finally pass away. Her death was nothing short of horrible and they really need to change the right to die laws so people like my sister are not forced by stupid laws to suffer for longer than they ever needed to.  If your pet is sick of dying you can do the humane thing but if a human being is they are made to suffer; how can anyone say that is best for anyone let alone every one?

I was a home support worker for many years and the DNR orders back then were Do Not Recussitate.  Period.  There were no different levels of DNR on the orders.  It meant no heroic measures AT ALL. But once someone goes into a care home, it is a numbered level of DNR. It turned out that she was mistakenly classified as a DNR 3 not a DNR 1 which would have saved her being revived and us having to make the heart breaking decision to pull the plug.

This is why I really feel the need to add it to this written piece as many other people out there both in Canada and other countries may also have no idea there are now different levels of DNR; it is no longer a one size fits all.  My sister would have chosen to opt out of her life years ago if she could have to have been saved all this suffering and humiliation she endured.  I don't understand how the government has the right to choose a slow death for her. The last three years of her life was brutal on her. The unfairness of this whole situation can really make me mad at times.

Her death was a blessing when she finally did leave this world.  I like to picture her with our mother and father in heaven standing on either side of her. She is no longer trapped in a body and mind that had turned against her.  I had hoped and prayed that the cure would come in time for her but it did not but it will come in time for me and for her sons if it turns out they have inherited HD from her.

There have been a lot of breakthroughs in HD in the last 5-10 years for sure but the greatest one of those is the gene silencing. Initially gene therapy researchers were focused on gene replacement for people with recessive genetic disorders, transplanting a healthy gene into people with two defective genes. However, HD is a dominant disorder.  Although most people who will eventually develop Huntington’s disease have a healthy gene as well as a defective one, both produce the protein and the one HD gene is enough to cause the disease.   The strategy had to be to shut off the bad gene while allowing the healthy gene to work.  Researchers are able in principle to selectively silence the HD gene by using genetic variations on the HD gene to distinguish it from the healthy.  The problem is that this approach would vary from individual to individual and each treatment would have to be tested separately, prolonging the time to a cure.  The current strategy being pursued by Isis Pharmaceuticals is to use antisense oligonucleotides to shut off both genes temporarily.  The idea is to do this long enough for the cells to recover from the HD protein but not so long that the reduction of the healthy protein causes damage.   They have now cured HD mice by doing this and next they will be doing it in HD people as the ISIS trials are set to begin this spring and next I believe they will prove it works in HD as well.  

The Isis scientists are already doing clinical trials in people to silence the ALS gene.  ALS is a single gene dominant disease which is exactly the same as HD and a handful of other neurological conditions as well.   So far the trials are going well which is very encouraging.

This is such an exciting time for all of us who have HD or have it in our families. I also understand they cannot say it works for HD people until they have actually done the study and proved it. It is going to take a bit more time and then they will be offering it to all of us.

I hope it flies through the trials and is available as soon as possible to those people who need it the most and are at the end stages of their HD.

This may seem too good to be true but it is not; this is really going to happen for us right around the corner so take good care of yourselves to make sure you better to chances of making it to the cure that is just down the road on the horizon. This is really real and we have waited generations for this cure.

Time is like a river.  You cannot touch the water in the same place twice because the flow that has passed will never pass again.