McVey boys fundraiser for wheelchair van. Duchenne

The boys story!  June 22nd 2016 a word that I wasn't too familiar with, Duchenne, became an every day apart of my life. At first I wasn't really sure what it meant, but I started reading about it and watching YouTube videos and I was heart broken. In March of 2016, Connor's teachers brought to our attention Connor's falling down and falling behind when walking distances. I had noticed it, but thought it was just Connor goofing off. I saw his calf's were very tone for a 4 year old and he was having trouble walking up and down the stairs. We finally, after hearing it from his teachers, realized we were right, Connor was not able to keep up with other kids his age and we wanted a Doctor to tell us why. We started off in Beaver Dam with his primary doctor and didn't get an answer, but two referrals. The first was a waste and didn't help, but the second was at UW Health in Madison with a Neurologist. Connor pulled a move called the Gower's maneuver and that is all he had to see. https://youtu.be/0htikR11nU8

Our next step was a test where the CK levels are checked in the blood, the levels were around 30,000 IU/L and normal levels are 30 - 200 IU/L. This made it all but certain, but we still needed to do a genetic test to prove it.

We were heart broken, but got thrown a curve ball when the doctor told us, b/ Connor has Duchenne the other two boys had a 50% chance of having it also. I couldn't see it, "no way all three could have it" I told Kelly. Then we got the call again and I was devastated and felt like I got the biggest kick in the groin ever.

We hope and pray for a cure every day and anxiously await someone to figure this puzzle out. We ask everyone willing to pray for a cure for all the boys with Duchenne.

Connor turned 10 in November 2020 and we have tried getting into two different trials to slow his progression, but he didn't qualify for either. Kian is now 8, and in a trial, while Finnegan is 6 as of December 3rd and the trial he was in has ended. The trial was not seeing the results they were looking for and shut down without warning. Connor and Kian are both on steroids and will most likely be for the rest of their lives. Finnegan will now go on steroids, like his brothers, as the steroid naive trial has fallen through.

I try and stay as strong as I can for these amazing boys and keep a smile on my face about this whole situation. I show them my smile and up beat attitude about their situation b/ I need them to be positive and always keep a smile on their face and fight this with everything they have. I want them to be as upbeat and happy as possible. I want them to find as much happiness in their lives, where ever their lives take them.

We don't know how long we have in this world and we'll keep fighting to try and extend their lives as much as possible. No parent should ever have to bury their child and I pray the Lord helps them beat this disease and makes them stronger than they've ever been.

Below are a few facts about the Duchenne but with your prayers, advocacy and willingness to fundraise along with us – we are hopeful to change the predicted path of this disease.

1. Duchenne is classed as a rare disease, affecting 1 in 3600 boys. The odds of a female having Duchenne of the same severity as boys is 1 in 50 million. Extremely rare, but it does happen.

2. Duchenne is a disorder of the Dystrophin gene, the largest protein-coding gene in the entire human genome. There are 79 exons on the Dystrophin gene, just one mutation in any one of these exons is all it takes to cause Duchenne. Hence World Muscle Wasting Disease Day is on September 7th.

3. Those with Duchenne are unable to produce Dystrophin, a protein essential for the stability and repair of muscle cells. Without it, every muscle in the body will eventually waste away.

4. Those with Duchenne usually appear healthy at birth, but often struggle to meet developmental milestones. They struggle to run and climb stairs like healthy children and fall often. The typical age of diagnosis is around 5 years old.

5. Walking ability is typically lost between 8-12, with a strong decrease in arm function generally occurring between 10-16. Those with Duchenne are not expected to reach their 30th birthday, in fact some don't even reach their 20th. For some this decline may occur sooner, while some children with Duchenne may never walk at all.

6. Unlike most disorders, Duchenne occurs across ALL races and nationalities.

7. Not all cases of Duchenne are passed on by carrier females. Around a third of cases are caused by a new genetic mutation with no previous family history. Quite simply, it could happen to any family.

8. Females can get Duchenne too. Carriers can be manifesting, meaning they display some symptoms of Duchenne, varying from muscle pain and fatigue to Duchenne of the same severity as seen in males.

9. Those with Duchenne often have unusually large calves, this is known as pseudo-hypertrophy. It's caused by dying muscle cells being replaced by fat and scar tissue.

10. There is currently NO effective treatment for the majority of those with Duchenne, it is 100% FATAL at a very young age.

Again, with your prayers, advocacy and willingness to fundraise along with us – we are hopeful to change the predicted path of this disease for Connor, Kian, Finnegan (The McVey Trinity), and others fighting this disease.

https://youtu.be/6wLnR7GJakY