We’re asking for lots of prayers, positivity, and strength for Bryce, Kyle, and Jenna this week as they prepare for his surgery, which they’ve scheduled for Friday morning. The multi-part procedure will take place at the UW-Children’s Hospital in Madison, and will include the following:
- Nissen Fundoplication: The surgeon will create an anti-reflux valve at the bottom of Bryce’s esophagus so he will be able to keep his meals down; because Bryce can no longer swallow, this will also prevent the risk of aspiration.
- G-Tube Replacement: A surgeon will be replacing Bryce’s current g-tube button with a more modern version (when Jenna and Kyle had the surgery consultation at the UW, the surgeons told them they hadn’t used the kind Bryce currently has in nearly eight years …)
- Fixing Bryce’s floppy epiglottis: A surgeon will be tightening Bryce’s epiglottis, which should help him breathe a little easier and reduce the amount of energy he’s expending by simply breathing.
Because there are three different surgeons involved (I think), it took quite a bit of coordination to get this scheduled, but we’re all relieved that they were able to get it scheduled for this week. Bryce has been vomiting about once a day, and the sooner they can do this and keep all of his food and medicine in him, the better. If everything goes as planned, Bryce may only have to be in the hospital for one night. He’ll have lots of company when he’s there in Mommy and Daddy, and his Grandmas and Grandpas.
In preparation for the surgery, Kyle and Jenna took Bryce in last week for a couple of pre-op appointments. They had his oxygen levels checked as his doctor in Dodgeville was a little concerned with his breathing patterns. When he’s relaxed, his O2 as at 98%, which is great; when he gets upset and agitated, however, his O2 drops to about 70% — it’s like he’s holding his breath. Because his levels are fine the majority of the time, they are not at the point of putting him on any additional oxygen support.
I think that’s it for now … I will be keeping everyone posted on the surgery here on Friday and throughout the weekend, so please be sure to check here for updates. Thank you so much for all the love and support 🙂
In like a lion, out like a lamb … it’s hard to believe we’re through March already. I hope the first day of April is being kind to all of you so far, especially after this long, hard winter.
Bryce had his consult at the UW-Children’s hospital yesterday about the Nissen Fundoplication procedure (it was a week later than I originally posted — my apologies for any confusion or if you’ve been checking back for an update).
Jenna told me yesterday that they’re going to move forward with surgery and that it will likely happen late next week. They will also change out Bryce’s g-tube at the same time: The one he currently has hasn’t been used by surgeons at the UW hospital in about eight years. The hospital stay will not be as long as they expected; in fact, if all goes well, it may only be one night.
Bryce has been a bit more awake since the last time I posted. Jenna talked to Tara, the assistant with Dr. Escolar’s program, and it sounds like his sleepiness may have been his way of fighting off an infection. This makes sense as both Jenna and Kyle were fighting nasty colds right around this time; Bryce never ended up getting a cold.
He also went a whole week without getting sick after his meals, which was really good: He needs to keep down all the calories he can get as he’s lost a pound since his last appointment. Once he has the Nissen, that will prevent reflux and he should put on a little weight. He’s a peanut.
Here’s what Bryce-ster has been up to lately:
- Spent the weekend of March 22-23rd at the farm, and got to see lots of extended family.
- Spent some quality time with Grandpa Bart and Grandma Wendy last Wednesday–Friday when they came to stay with them at home in Dodgeville.
- Made another trip to the farm and got to see cousins Maddy and Lizzy. They all went on their first tractor rides: As you can see, Papa Tom was very proud!
Kyle, Jenna, and Bryce have a few different appointments this week. Please keep them in your thoughts as prayers as they continue to make these difficult decisions about Bryce’s care. And please know how grateful they, and all of us, are for all of your continued support.
Krabbe Disease … we wish more than anything you didn’t exist. These past few weeks have been very tough on Kyle and Jenna, but especially on sweet Bryce. It’s become very clear that the disease is progressing, though how much exactly we don’t know.
Once they returned from Pittsburgh in January, Bryce plateaued once they got his medications in place, which is what the team out there predicted would happen. His movement has been limited since then: He really only moves his arms and legs when he’s experiencing a nerve or muscle spasm: It’s like he’s paralyzed from the neck down.
Since about the first week of March, though, he has been a bit more irritable and has started vomiting after many of his meals. They’ve speculated that it could be because they’re feeding him too much or too fast; that it could be about how he’s positioned and how soon they lay him down after he eats, etc. But there isn’t much consistency to when it happens. That’s what makes them think the disease progressing.
With this in mind, they are anxious to move forward with the Nissen Fundoplication procedure, which will prevent reflux and aspiration: Because Bryce can no longer swallow on his own, there is a greater chance of this happening. Also, due to Bryce’s floppy epiglottis, there’s a greater chance of him getting pneumonia as a result of the vomiting: Because his epiglottis doesn’t seal, every time he gets sick, the bacteria builds up on the epiglottis and could cause infection in his lungs. They are meeting with a doctor at the UW-Children’s Hospital on Monday and hope to move forward with the procedure within a week or so.
Bryce’s vision has also been affected, something that Kyle and Jenna began to notice a couple of weeks ago. He is no longer tracking people across the room or focusing. It’s almost as if his vision is delayed – like it takes a while for things to become visible to him. This change is devastating …
They’re not sure if it’s because of the medication or just the disease, but Bryce is sleeping a lot more now. His digestion is also slow, a result of his lack of movement; they are giving him a supplement with two of his feedings to help him have more regular bowel movements.
In spite of all these things, Kyle and Jenna have been experiencing life with Bryce: They spent a week in Mosinee with the Heckendorf family; they spent the weekend here in Minnesota and went to the Sea Life Aquarium at the Mall of America; yesterday, he laid in his first snow pile 🙂 These among many other things …
We ask for your continued prayers for Bryce, Jenna, and Kyle. Your love, strength, and positivity are what is carrying them, and all of us, through this … Thank you!
For those of you who are not on Facebook, a note from Kyle and Jenna:
We thought we would get to teach our son about the world. It turns out we get to teach the world about our son. Without ever saying a word, we know he will continue to touch the lives of many in so many different ways.
This is not how our story was supposed unfold. This is not what we had planned. Up until November 19th, we were planning our future with Bryce and talking nonstop about all the fun things we were going to do with him and all the things we couldn’t wait to teach him. We had big dreams, like all parents do, until suddenly we were blindsided with the saddest news that would forever change our family and the people we were. We thought, until that day, that this kind of stuff doesn’t happen to people who are healthy and happy and well, just not to people like us. We had everything we needed and wanted until that day…
And since that day, we have witnessed some of the most amazing things and have learned so much. THE SKY IS BLUE. People are amazing. We have been blown away by everyone’s generosity and support and it’s hard to put into words how thankful we are for everything everyone has done. I write this post to say THANK YOU! We left Saturday night speechless. We couldn’t wrap our heads around what had just happened. It was amazing and I still don’t know what to say other thank you, thank you, and thank you again.
We’d especially like to thank my aunts Nikki, Peg, and Cindy and my cousin Trisha for spending hours and hours organizing the event. You ladies are AMAZING! We love you all so much! To everyone who made donations, who volunteered their time, and who attended the benefit…thank you! We are so touched by the generosity and support that family, friends, coworkers, community members, and complete strangers have given us. Your love and support reminds us during this extremely difficult time that the Sky is Blue even when it doesn’t appear that way to us.
As always, we ask that you please continue to pray for Bryce and our family and for strength to get us through each day. Thank you for helping us B.Strong and for helping us teach the world about our son. We will remain with HOPE for a cure. We will continue to PRAY for a miracle. We will always be THANKFUL for each moment.
All our love, Jenna and Kyle
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
First off, thank you for your patience since our last entry here: I know it’s been a while and you’re all very concerned about Bryce, and wondering how he’s doing. The good news (if there can be any in this whole situation) is that Kyle and Jenna have not seen any major changes in Bryce since the last time I wrote: He has been holding steady these last few weeks.
His medications are helping to control his nerve pain and he seems more content. Some days are better than others for him, of course, and it’s really hard to see him in those moments of discomfort/pain. I don’t know how Jenna and Kyle are doing it, but they are truly amazing parents. Please keep them in your prayers, too — the physical and especially emotional strength they’re gathering to get through each day is unbelievable.
My family and I got to spend some quality time with him (and Jenna, Kyle and Grandma Sue) the first weekend in February as they were en route to and from St. Cloud for Uncle Kole and Aunt Kristen’s wedding. They arrived Thursday night, the 6th, and Bryce got to hang out with cousins Maddy and Elizabeth, which is always so special. I especially loved getting to hold my Godson for long stretches — I haven’t been able to do that since shortly after his diagnosis. I must have kissed his fuzzy blonde head and soft cheeks a thousand times over the three days I saw him.
I know all of his Heckendorf and Kutz family enjoyed their time with him in St. Cloud, too. They celebrated Kole and Kristen’s marriage on Saturday, February 8th, and Bryce was their handsome ring-bearer (he couldn’t have been any cuter in his little brown suit — be sure to check out the photo area here to catch a glimpse).
Kyle, Jenna, and Bryce then spent some time at home alone in Dodgeville, and then with Grandpa Bart and Grandma Wendy. They’ve been at the farm for a few days now, staying off the roads and avoiding the crummy weather. Jenna sent me this update earlier this evening about what they’ve been discussing about Bryce’s care:
We have been consulting with doctors on whether or not to proceed with some of the surgeries that were recommended by the team in Pittsburgh. We have one more consultation on the 26th and after that we will make our final decision. The procedures involve tightening Bryce’s “loose” epiglottis, which will allow for easier breathing, and having a nissen done. The purpose of the nissen is to prevent reflux and aspiration. This is more serious surgery than we originally thought, our stay would be four to five days in the hospital.
You may be wondering why we may not consider going through with the procedures. Quality vs. quantity of life for Bryce is our main focus and it will be the deciding factor on everything from here on out.
Whether or not we decide to do these two procedures, we will for sure be replacing Bryce’s current G-tube button with a similar one that has a “lock” system to it. Everything in terms of feeding will remain the same.
Other things coming up:
- Kira at KJB Photography will be coming to our house this week to take photos
- Regular physical therapy
- Consult with a doctor at the UW Children’s Hospital on the 26th
- Meeting with a home health representative about a stander/seat for Bryce for when he gets closer to 12 months
- “The Sky Is Blue Benefit” on Saturday, March 1st.
Thank you again for checking back here regularly, and for keeping Bryce, Kyle and Jenna close to your hearts. We hope to see many of you at the benefit in Lancaster next weekend! –Auntie Jamie