The Sky Is Blue Benefit
On behalf of Kyle, Jenna, Bryce, and the Heckendorf and Kuenster families, THANK YOU for coming out yesterday and making The Sky Is Blue Benefit a HUGE success. What an event for sweet Bryce … you all showed such love, support, strength, and generosity. You lifted us up in a way that none of us could have expected: It was overwhelming and amazing.
I have to start with the organizers: Aunt Nikki Nemitz and cousin Trisha Gage drove out to my parents’ home shortly after Bryce’s diagnosis back in November and ran the idea of a benefit passed Jenna and Kyle. They then rallied the troops — Aunt Peg Rewey, Aunt Cindy Ritter, Aunt Angie Redman and so many others — and they were able to create a meaningful, beautiful event yesterday, one that brought hundreds of us together around one little boy and his family. What would we do without your unwavering love, commitment, and support??
To all the sponsors, volunteers, and donors — and there were MANY of you: Thank you for giving so selflessly and thoughtfully, whether it was food, your time, or of one of HUNDREDS of beautiful treasures that raised proceeds to help cover Bryce’s care and raise awareness about this awful disease. You made things run smoothly, and all of us went home with some pretty amazing memories, not to mention items, that will forever be remembered as part of The Sky Is Blue for Bryce …
To Tim Slack and crew: You helped build energy and generosity of spirit in that building last night. Thank you for that, and for making the auction portion of the event run so smoothly. I kept thinking there was no way it wouldn’t be chaotic — but it was darn efficient.
To all of our family, friends, and the community at large: We are blessed to be surrounded by such amazing people. Thank you for coming out yesterday — despite the weather, despite basketball games and wrestling matches, despite all the other things happening in your own lives. You came from near and far to be there for Kyle, Jenna, and Bryce, and they are lucky to have you in their lives …
We don’t know what the weeks and months ahead hold for Bryce, but we are forever grateful to have each other and all of you to lean on in this most challenging and heartbreaking of times. Thank you for just being there.
“Life is brutal. But it’s also beautiful. Brutiful, I call it. Life’s brutal and beautiful are woven together so tightly that they can’t be separated. Reject the brutal, reject the beauty. So now I embrace both, and I live well and hard and real.” —Glennon Melton, Momastery.com
There are a few common questions that Kyle and Jenna have been asked about Bryce’s condition, and I thought I’d share the answers to those here.
Q: Do Jenna and Kyle also have Krabbe Disease?
A: No, Jenna and Kyle are both carriers of the mutated GALC gene that can cause Krabbe Disease when passed on to their children. (Genetics 101: We all have two copies of all of our genes — one from mom and one from dad. When you’re a “carrier,” it means you have one good copy and one bad copy.) For the GALC gene, you only need one good copy to be healthy since it creates enough of the GALC enzyme to maintain myelin in the brain.
It’s estimated that every 1 out of 125 people in the United States carries a mutated GALC gene. The likelihood of meeting someone with this same mutated gene is slim. Obviously, it does happen, as we know all too well.
There are hundreds (and probably thousands) of different mutations to this gene — and all of our genes, really. In order to help further the research about Krabbe, Kyle and Jenna underwent genetic counseling to determine their specific mutations: The version Kyle carries has been documented; the version Jenna carries has not. What this means for Bryce is that they don’t know how the disease will progress since these two versions together have never been seen.
An interesting fact: All of us likely carry five to 10 mutated gene that could cause disease. The breast cancer genes, BRCA1 and BRCA2, are examples that more people are probably familiar with: Mutations of these genes in carriers significantly increase the odds of breast and ovarian cancers.
Q: How did Bryce get Krabbe Disease?
A: Krabbe Disease is an inherited disorder that is passed on in the “autosomal recessive manner” — meaning that both parents must have a mutated copy of the gene in order for the disease to present. Any of Kyle and Jenna’s children has a 25 percent chance of having Krabbe; a 50 percent chance of being a carrier; and a 25 percent chance of not being a carrier or having Krabbe.
Yes, there was a 75 percent chance that Bryce wouldn’t get it …
Q: Since the risk is 1/4, what are their plans for future children?
A: Kyle and Jenna cannot fathom going through this again, yet they know they want to have more children — they love being parents to sweet Bryce. They are researching their options, and discussing them with the team in Pittsburgh and other families of Krabbe babies. The great news is that many of the families that Jenna and Kyle have connected with have been blessed with healthy children.
Q: What about other family members — are they carriers too?
A: One of each of their parents is definitely a carrier of a mutated copy of the Krabbe gene (GALC) — something that’s likely been passed down for generations. Both sets of grandparents are planning to have genetic counseling to determine which sides of the family the mutated gene is from. Once we know those results, extended family members can decide if they want to get tested.
All of Jenna’s and Kyle’s siblings will also be getting tested to see if we’re carriers. If I, for instance, would test positive for the mutation (which would be the same as Jenna’s), then Brady will get tested. If he should also test positive (which is HIGHLY unlikely) we will immediately get our daughters tested. If not, then we’ll wait until they’re older to have them tested (it will be important that they know prior to having children of their own).
Q: How do you manage Bryce’s pain?
A: Bryce is currently on three medications, which he gets during his feedings (every four hours or so):
- Baclofen: To control muscle spasms and spasticity (tightness)
- Gabapentin: To control nerve pain and seizures (they don’t think he’s having the latter yet).
- Baby Ibuprofen: To prevent/control inflammation in the brain.
Do you have questions? Feel free to post them here and I’ll do my best to get them answered for you.
In the meantime, just a reminder that “The Sky Is Blue” Benefit for Bryce is this Saturday, March 1, at the Youth and Ag Building in Lancaster, Wis., from 3–8pm (please visit TheBStrongFund.org for more details). We’re looking forward to seeing many of you there, and know that those who can’t be there will be in spirit.
Thank you all for the continued prayers, love, and support. –Auntie Jamie