Life As an MPS 1 Adult: Walking In The Valley Of The Shadow Of Death

silhouette of man with shadow of death and sickle

This topic is something that MPS families and survivors have to come to terms with early on in a diagnosis. It is especially hard to prepare for when there is no standard to which we ultimately pass. It could be six, it could be 46. Not even the supposed experts know.

While it could be said that no one really knows how or when their life is going to end, ultimately MPS individuals are affected daily by this mysterious "end" because it is a progressive illness that requires us to actively stave off symptoms that could be our undoing. This is something I think makes this a different experience than the average.

Especially within the last three years I have been having to ask myself questions that most individuals don't have to ask themselves until they're in their 70s, and I'm only 31. One of which is "When is enough, enough, and is that reasonable to myself and my family?"

For example, as it stands my muscles are getting weaker, leading to carbon dioxide building up in my blood. Which means there will come a point where I need a tracheotomy and to be placed on a standard or portable ventilator, or else my brain will suffocate to death like it almost did earlier this month. But for me I feel that this is not an option I am willing to take. My quality of life is barely tolerable to me as it is. Don't get me wrong, I have a lot of great things going for me now, but because of finances I spend the majority of my time in a box, communicating to the world through a box. It is difficult to get out for various reasons as it is; untethered to machines, I do not feel it is reasonable to be strapped to another box that will maybe extend the quantity of my life for a couple months or years but reduce the quality of it even further than it already is. Adding to the complexity and maintenance of the different aspects of my life.

There are many types of decisions like this that have to be made, and believe me it's even harder when doctors don't understand your choice. It's not only our lives we have to think about but the emotional and physical strain our life and death imposes upon those around us. I may be depriving those who love me the blessing of taking care of me in my time of need, but I would rather live out my short days in happiness leaving behind memories of joy, then stretch them out to long days of misery. This I feel is not fair to myself or my family and friends.

During my last stint in the ICU, my infection and carbon levels had risen to the point of two days of delirium and bouts of unconsciousness. I to this day don't recall anything I said or did, and had to be informed after the fact. By what I've been told, I spent that time moaning and saying "help me", all the while there wasn't anything my family could do but stand and watch. Even in my delirium I wanted my suffering to end. Coming out of it, this is something I don't want me or the ones I love to experience again. To ensure it never reaches that point, it means making the proverbial red line - which is a tough choice to make. The line that says I realize I have explored my options and I have peace with what is coming, and to spend my time surrounded by love.

I think this is why I chose the particular title the way I did because I believe this psalm properly illustrate what it's like living with a progressive terminal illness like MPS. We are constantly walking through the valley of life under the ever looming mountainous shadow of death. But when we live the life we should be living however – with family and friends – it will be lived joyfully without fear of the end.


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