Bonnie's journey through MPS (Hurler's) treatment

Follow us on Instagram @bonnierays_journey to stay up to date!

We are the Ray Family - Will, Olivia, Bonnie, and our puppy, Chili. We live in Winston-Salem, NC. Bonnie was born January 10, 2023. When she was 4 months, the pediatrician picked up on her increased head circumference and abnormal curvature in her back. Soon we would know that she had hydrocephalus (increased fluid on her brain) and what is known as a Gibbus deformity in her lumbar spine. This started to raise some red flags. Mucopolysaccharidosis I (MPS I), or also known as Hurler Syndrome, came on our radar and our pediatrician realized it had been added to the Newborn Screen shortly after Bonnie was born. She brought us to the clinic and we repeated the heel stick days before she was 5 months old. 2 weeks later, we were called by UNC with results that were highly suggestive of MPS I, which was later confirmed.

With MPS I, Bonnie lacks an important enzyme that breaks down a particular complex carbohydrate. Without it, that carbohydrate builds up throughout her body and can cause damage wherever it goes. This is what caused the hydrocephalus. To begin her medical journey, she had a shunt to drain the fluid put in on 6/23/23 and has recovered well. The next step is to get Bonnie the enzyme, because without it these children do not survive the first decade of life and more often do not survive beyond 3 or 4 years of age. We can do Enzyme Replacement Therapy, which helps many of her organs, but it does not get to her brain. In order to halt the neurocognitive decline, Bonnie requires a stem cell transplant as well. We are going to the University of Minnesota for her stem cell transplant as they are national leaders in pediatric transplant in MPS I and are leading the country in research.

Transplant will be an intense time of chemotherapy and low blood counts with about a month-long hospital stay and then us being there for at least 100 days from when she gets her stem cells before we can return back to NC. Transplant will not be easy and there are many risks along with it, but we know this gives our sweet Bonnie her best chance at a healthy life. Even after transplant, there will be aspects of the disease that will require lifelong monitoring and care.

We appreciate you coming alongside us financially and prayerfully as we leave our lives in NC for a time and move across the country. Our prayer is that we would abide in Christ and that Bonnie's life would be a testimony for His glory.

"He who dwells in the shelter of the Most High will abide in the shadow of the Almighty. I will say to the Lord. "My refuge and my fortress, my God, in whom I trust." - Psalm 91:1-2