In all the jobs I did around the house growing up, I never had to take out the garbage. But that's what I want to talk to you about. My husband has a rare disease called Hunter Syndrome (not to be confused with Jim Kelly's son Hunter who also had a very different rare disease). It is part of the MPS family of diseases - and it has to do with garbage and recycling...in the human body.
MPS stands for mucopolysaccharides. You can see why they abbreviate it! Mucopolysaccharides are long chains of sugar molecules that are found throughout the body. When someone has MPS, they lack an enzyme which breaks down these sugars. So, the sugars do not get broken down. In other words, the "garbage" doesn't get taken out. Even worse, the body is not recycling. Enzymes break things down, very similar to recycling a material.
There are anywhere from 1000 to 1300 different enzymes in a single cell, and a staggering 75,000 enzymes in the human body. It is amazing that by lacking just one of those enzymes, this disease can occur. Even though I never took the garbage out growing up, someone always did. But with MPS, that one little enzyme that is lacking is the garbage taker outer (or recycler) for, well, MPS sugar molecules. He is the only one who can take out or recycle that specific molecule. When that enzyme is missing, those specific molecules don't get recycled. They accumulate in every cell and cause damage over time.
There are 7 known types of MPS today. Each type lacks a different enzyme which breaks down a different type of sugar molecule.
My husband and my nephew have the attenuated (mild) form of MPS II. Research has been able to identify the specific enzyme they lack, and develop a replacement enzyme. This didn't cure the disease, but it has helped to slow down the progression and extend MPS II patient's lives.
You can go to the this secure link to give support and learn more about MPS.
My husband was also a pastor for 40 years in the Christian and Missionary Alliance. Recently we started to podcast his sermons from the last 10 years of his ministry. You can go to Hope for the Journey to listen.
Our family in 2017. Wayne, pictured in the green sweatshirt, has MPS II |
Thank you for this glimpse into some of the challenges your family faces. I appreciate your transparency.
ReplyDeletePart of my reason for including this is to help promote awareness since it is such a rare disease. Thank you for reading it.
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