What is MPS VI?
MPS VI (of Maroteaux-Lamy Syndrom) is a rare progressive genetic condition that affects major organs and systems of the body. MPS VI is a form of mucopolysaccharidosis (mu*co*poly*cha*ri*do*sis)
Enzymes are proteins that perform specific jobs in your body. People with MPS VI are missing an enzyme called arylsulfatases B,or ASB. ASB breaks down and gets rid of waste in your cells. The waste is callled glycosaminoglycans or GAGs.
Over time GAGs can build up in cells, leading to organs or entire bodily systems being harmed.
MPS VI is a recessively inherited condition. This means that both parents must have a genetic mutation in the ARSB gene to pass on MPS VI. When both parents have the ARSB gene, each of their children has a 25% chance of getting MPS VI.
MPS VI has a range of physical characteristics that can look different from person to person, but there are some common patterns. These changes happen because certain substances build up in the body over time, affecting bones, joints, and organs.
MPS VI isn’t just something happening on the inside, it shows up physically too, in ways that can be subtle, noticeable, or somewhere in between. For those living with it, those physical differences are just part of everyday.
At first, the changes might not seem obvious. Maybe it’s stiffness in the joints or feeling less flexible than everyone else. Maybe it’s getting tired faster, or noticing that your body just moves a little differently . The things people don’t always see can be cornea cloudiness, hearing loss, breathing challenges-due to airway differences, a fuller abdomen from organ enlargement.
Living with MPS VI isn’t just about having a rare condition-it’s about living in a body that tells a slightly different story every single day.
What Treatment for MPS VI Looks Like.
When people hear about MPS VI, one of the first questions is : “Is there treatment?”
The answer is-yes. But it’s not a one-time fix or something you do and forget about.
Treatment for MPS VI is a commitment. It’s routine. It’s part of your life in a very real, very personal way.
The truth is, treatment doesn’t cure MPS VI, Instead, it helps manage the condition. It slows progression. It supports the body in ways it can’t do on it’s own.
The main treatment for MPS VI is called enzyme replacement therapy (ERT). It helps replace the enzyme the body is missing. Most people receive a medication known as Neglazyme (Galsulfase) Neglazyme has been shown to improve walking and stair climbing.
Neglazyme is administered Intravenous (IV) I have weekly infusions, that lasts about 4 hours. When I was first told I would have to have these weekly infusions, it sounded awful, but 12 years later- I actually look forward to my appointments. I love my facility I get to go to, my nurses have become friends, and I get to sit and do “nothing” and not feel guilty.
MPS VI reminds me that life doesn’t always follow the path we expect. And while I can’t control everything, I can choose how I show up- for my family, my future, and for the people I love most.
If this topic resonates with you in a deeper way, and you’ve ever found yourself thinking about how to better protect or prepare for life’s unknowns, I would love to grab coffee with you and have a conversation.
Sometimes the most meaningful plans begin with simply sitting down, sharing a story, and talking through what matters most.
Tracy DeWaard
