Meet Zachary, Amelia, and Luke

This month’s “Do Small Things with Great Love” t-shirt design is inspired by Zachary, Amelia, and Luke who share a special bond but also a rare medical diagnosis. Every purchase in June will be used to help cover the cost of travel from Alabama to Minnesota where Zachary, Amelia and Luke receive treatment.

We invite you to watch their video, read their story, and subscribe to our podcast to listen to a conversation with their mom & dad and a second episode with just the kids.

In a small coastal town in Alabama, the Thomas family stands as a beacon of courage and strength, defying the odds and inspiring all who encounter their remarkable journey.

Meet Brooke and Mich, the loving and resilient parents whose unwavering commitment to their children knows no bounds. Their three incredible kids, Zachary (14 years old), Amelia (11 years old), and Luke (7 years old), are living with unyielding spirit despite the challenges posed by a rare genetic disease called mucopolysaccharidosis type I (MPS 1). Together, this extraordinary family weaves a tapestry of love and resilience that transcends the boundaries of hardship and shines a light of hope for others.

This is their story.
In 2009, Brooke and Mich Thomas were living in Arkansas when they welcomed their first son, Zachary, into the world. However, Zachary’s birth was accompanied by complications, including vascular malformations and failed hearing screenings. It was a challenging time for the young family as Zachary spent two weeks in the NICU battling severe respiratory distress.

During Zachary’s one-year visit to the pediatrician, their doctor, who had personal experience with MPS 1, urged them to seek immediate genetic evaluation without resorting to online research. Despite their doctor’s warning, Brooke and Mich couldn’t resist searching for information about MPS 1. Unfortunately, they encountered outdated and disheartening details, including references to “gargoilism” and a limited life expectancy. Seeking specialized care, they swiftly consulted a genetics specialist who confirmed Zachary’s MPS-1 diagnosis.

Realizing that their local facility wasn’t equipped for the necessary treatment, the family heeded their geneticist’s recommendation to seek treatment at the University of Minnesota. With unwavering love and determination, they embarked on a new chapter, leaving behind their familiar lives.

About MPS 1 (Hurler Syndrome)
Mucopolysaccharidosis 1 is a rare genetic disorder that occurs in approximately 1 in 100,000 newborns.

Mucopolysaccharides are chains of sugar molecules used to build connective tissues in the body. “Muco” refers to the thick jelly-like consistency of these molecules, “poly” means many, and “saccharide” is a general term for a sugar molecule. In MPS I, patients lack the enzyme alpha-L-iduronidase, which is crucial for breaking down the mucopolysaccharides dermatan sulfate and heparan sulfate. As a result, these substances accumulate within the body’s cells, causing progressive damage. While babies may initially show minimal signs of the disease, symptoms emerge as cells suffer increasing harm.

“The best analogy I heard is that lysosomes in the cell work like your recycling or waste factory. Imagine that one day the recycling truck stops coming to your home. You’ll save your recycling for a bit but eventually, your garbage is going to fill up with waste. So, you put it somewhere else: the garage, the closets, anywhere! Eventually, your home is going to fill up with garbage and waste will be everywhere,” Brooke explains.

When the cellular waste begins to turn into sludge in their body, it affects every cell in the system, which is known as glycosaminoglycans (GAGs). The most common features of the disease are short stature and coarse facial features. Most kids at birth experience a lot of hernia issues, cardiac involvement, frontal embossing, hearing loss, cornea clouding, and orthopedic issues.

While there is no cure for MPS 1, the most common form of treatment involves bone marrow transplantation and/or enzyme replacement therapy (ERT) which can help make MPS I a more manageable disease.

Seeking Treatment
Zachary was eighteen months old when he had his stem cell transplant. Leading up to Transplant Day, Zachary had a month of work-up visits to see all fourteen of his specialists while also receiving weekly infusions to mitigate the damage done to the body. Zachary also received chemotherapy to completely eradicate his immune system, ensuring that when he received the stem cells, his immune system would not respond and attack.

Following Transplant Day, Zachary was required to stay for a minimum of one hundred days to ensure there would be no complications. While in the midst of Zachary’s treatment, Brooke discovered she was pregnant only three days after the transplant day.

Applying the knowledge gained from their journey with Zachary, Brooke and Mich underwent prenatal testing and discovered that Amelia also had MPS 1.
Amelia was born on September 16th, and started enzyme replacement therapy six weeks later. “We have noticed a significant difference in how MPS 1 presents itself in Amelia. She is our least affected child. She received her stem cell transplant when she was eight months old, and it went really smoothly.”

While the stem cell transplant helps make the disease more manageable, it does not address the orthopedic issues.

“Once a year, we continued to go to Minnesota for week-long visits to see all the specialists. Both kids have carpal tunnel syndrome and had to undergo trigger finger release surgery. Zachary also had hip reconstruction surgery where they cut the femur and repositioned it to fit better in his hip socket,” Brooke explains.

As the family settled into the routine of navigating life with two kids on rare medical journeys, life surprised them with the birth of their youngest, Luke.
“We didn’t diagnose him prenatally, but once we found out, we started him on the same journey as his siblings. However, we encountered more complications along the way,” Brooke explains. “During the transplant, you are essentially giving their body new DNA so the body can naturally produce the enzyme. In Luke’s case, his white blood cells saw his new hemoglobin as a foreign object and attacked it, preventing him from producing enough hemoglobin. This led to the development of Autoimmune Hemolytic Anemia (AIHA).”

While Brooke was in Minnesota, waiting out the 100 post-transplant days with Luke, Mich called her with an interesting offer. “Mich called and asked if I wanted to move to the Gulf Coast, which, of course, I did. So he accepted the position, and we moved the family to Alabama while Luke and I waited to be released. It was quite a time,” Brooke chuckles.

Following Luke’s transplant, he continued to experience many complications and ended up receiving a GJ tube until he was three years old. Reflecting on that time, Brooke shares, “This was in the midst of COVID, so we had to take triple the precautions since Luke’s immune system was still compromised for a year, just like a newborn baby. The isolation was hard. COVID postponed a lot of what we needed to do for the kids, especially traveling back and forth from coastal Alabama to Minnesota. Finally we are having Zachary’s hardware removed that was placed in 2017, and he’ll undergo the same hip reconstruction on the other side, along with guided growth plates in his knees and screws in both ankles. Later this year, we’ll be returning so Amelia can have the same procedures.”

Reflecting on all they have been through and all that is yet to come, the family holds onto hope that treatment for MPS 1 will continue to improve.

Without treatment, children with MPS 1 typically stop developing between the ages of two and three and soon pass away. While the transplant carries a 30% mortality rate, it ultimately extends our children’s life expectancy to approximately thirty years. Nevertheless, a lot has changed in the medical field, even between Zachary and Luke. The reality is that we don’t know for certain because the children who have undergone similar treatment aren’t significantly older than my kids,” Brooke explains.

While the Thomas family’s life may appear different from most, at the end of the day, they are still a regular family with typical sibling relationships.
Zachary embodies the stereotypical first child—organized, responsible, and outgoing. He has a passion for history, a remarkable memory for flags, and exhibits natural leadership qualities.

“Since Zachary was eleven years old, he has been assisting me with a lot of the legislative work we are undertaking to include MPS 1 in newborn screenings. He definitely wants to continue advocating as he grows older and has expressed interest in working with veterans,” Brooke proudly shares.

Amelia is affectionately known for being a mirror image of her father. She is creative, artistic, and musically inclined. On any given day, you can find Amelia dancing, painting, or singing while strumming her ukulele. “Amelia has taught me to pause and appreciate beauty, and ultimately focus on what brings me happiness. At present, she aspires to become an artist or a baker,” Brooke beams.

Luke claims the title of life’s party-goer and also has a penchant for taking charge. He adores playing with Legos and is deeply engrossed in the world of Pokémon. “There is no such thing as a stranger to Luke. Everyone is invited to his birthday party,” Brooke chuckles.

As the family continues to navigate their new normal, they are also paving the way to raise awareness for MPS 1. They continuously raise awareness for newborn screening at the Federal level and are currently advocating for the state of Alabama to update its screening process.

Time to get loud shouters!

Throughout the month of June we will be shouting loud for Zachary, Amelia and Luke!

So click here to shop now where every purchase in June will be used to help cover the cost of travel from Alabama to Minnesota where Zachary, Amelia, and Luke receive treatment.

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Every purchase in June will be used to help cover the cost of travel from Alabama to Minnesota where Zachary, Amelia and Luke receive treatment.

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