I know that I'm not the only one who has these feelings. Numerous blogs and books have been written about the mourning and grief that accompanies having a child with special needs and/or medical complexities; "How to Cope with Recurring Grief," "The Unique Grief of Special Needs Parents," and "The Perpetual State of Losing," are just a few.
On Friday as I walked and talked and laughed and cried with another mom who "gets it," one of the things that we discussed was the use of the word "terminal" vs "life-limiting" when we talk about PBD-ZSD and how these words that in many ways mean the same thing, can carry very different connotations. Here are just a few possible definitions of these words that I found online.
- Leading ultimately to death; fatal; approaching or close to death; being in the final stages of a fatal disease; causing death eventually; leading finally to death; having an illness that cannot be cured and that will soon lead to death [Merriam Webster]
- An incurable and irreversible condition caused by injury, disease, or illness that would cause death within a reasonable period of time in accordance with accepted medical standards, and where the application of life-sustaining treatment would serve only to prolong the process of dying. [Duhaime's Law Dictionary]
- Defined in UK Social Security legislation as: "a progressive disease where death as a consequence of that disease can reasonably be expected within 6 months." [The Free Dictionary]
- Conditions for which there is currently no cure and the condition is likely to lead to a child dying prematurely; Any chronic condition (longer than three months) developed in childhood that without major intervention is likely to result in premature death; Recognition and diagnosis of the condition must occur before the age of 16 years. There is no cure for the condition and a likelihood that the child will die prematurely as a young person (25 years or under). [Centre for Palliative Care Research & Education - Children with a Life Limiting Illness]
- Diseases with no reasonable hope of cure that will ultimately be fatal. [Rising National Prevalence of Life-Limiting Conditions in Children in England]
My friend's son is 2 years old. Ethan is 5 years old. My friend frequently uses the term "terminal" when explaining her son's illness to others. While I still use "terminal" at times, more recently, I more frequently describe Ethan's illness as "life limiting with no cure." Why have I changed how I describe Ethan's condition? To be honest, I think it has a lot to do with grief and mourning. Both mine and the general understanding of if within today's society. When most people hear the word terminal they think that there must be an "expiration date" or a given life expectancy. We have been in mourning since we first learned about PBD-ZSD on August 20, 2010 when we held our beautiful 28 day old baby boy and the world collapsed around us. We were told the truth about the disease (50% chance of dying in the first year, etc.) and began to prepare for the worst but hope for the best. Fast forward more than 5 years later, Ethan is still alive. He is severely disabled and although we don't think he is likely to die tomorrow, he still hasn't "beat" PBD-ZSD. He still has a "terminal" disease and his health could deteriorate at any given time without much warning or he could continue to trudge along day by day fighting PBD-ZSD with all his might yet not making a lot of progress. The honest truth is that God is the only one who knows my son's future and while we have looked, we have yet to discover an expiration date stamped anywhere on his little body.
While there is some variation in the definitions of "terminal" and "life-limiting," depending on the source, the overlap is undeniable. Let me be clear --- PBD-ZSD is a terminal life-limiting condition with no known cure. However, PBD-ZSD is a disease that has an extremely wide range of symptoms, so wide in fact that prior to recent medical advancements/discoveries the spectrum was actually thought to be four separate diseases (ZS, NALD, IRD, Heimler Syndrome). The sad reality is that approximately half of all children born with PBD-ZSD have the most severe presentation of the disease and typically die before their first birthday and the majority of children with PBD-ZSD do not survive into their teens. Although there are individuals with PBD-ZSD living into their twenties and thirties with varying degrees of disability, this is not the norm. This is a nasty disease and while there are amazing physicians and scientists working on discovering ways to improve the quality of life for individuals with PBD-ZSD, there is still a lot that is unknown. [For more information about PBD-ZSD please visit the Global Foundation for Peroxisomal Disorders (GFPD).]
I also draw strength from "reminders" like these from Proverbs 31 Ministries:
|I want to live a life that reflects this. I hope you do too.|