174 – Jeremy Knakmuhs of Colorado Springs, CO Who Is The Father Of Three Including A Daughter With PFIC Special Fathers Network Dad to Dad Podcas

Transcript:

Tom Couch: Special thanks to Horizon Therapeutics for sponsoring today’s Special Fathers Network Dad to Dad Podcast. Working tirelessly to research, develop and bring forward medicines for people living with rare and rheumatic diseases. Discover more about Horizon Therapeutics’ mission at horizontherapeutics.com

Jeremy Knakmuhs: My wife and I are always available for new families that are just getting diagnosed, if they have questions. You know, “Hey, what can we expect with this disease?” Because it’s so much different than the diseases you’ve heard of, because it is a rare disease.

Tom Couch: That’s our guest this week, Jeremy Knakmuhs, a structural project engineer and a father of three, including three year old twins, Remy and Kennedy, who has PFIC, a rare genetic liver disorder. We’ll hear the Knakmuh’s family story and how Jeremy and his wife Emily have navigated the ups and downs of raising a young child with special needs.

That’s all on this Special Fathers Network Dad to Dad Podcast. Say hello to David Hirsch.

David Hirsch: Hi, and thanks for listening to the Dad to Dad Podcast, fathers mentoring fathers of children with special needs, presented by the Special Fathers Network.

Tom Couch: The Special Fathers Network is a dad to dad mentoring program for fathers raising children with special needs. Through our personalized matching process, new fathers with special needs children connect with mentor fathers in a similar situation. It’s a great way for dads to support dads. To find out more, go to 21stcenturydads.org.

And if you’re a dad looking for help or would like to offer help, we’d be honored to have you join our closed Facebook group. Please go to facebook.com/groups and search Dad to Dad. And now let’s hear this compelling conversation between Jeremy Knakmuhs and David Hirsch.

David Hirsch: I am thrilled to be talking today with Jeremy Knakmuhs of Parker, Colorado, who’s the father of three, and a structural project manager at CTL Thompson, a 50-year-old engineering and consulting firm. Jeremy, thank you for taking the time to do a podcast interview for the Special Fathers Network.

Jeremy Knakmuhs: Thanks for having me.

David Hirsch: You and your wife Emily, have been married for eight years and are the proud parents of three children, Colton, six, and three-year-old twins, Remy and Kennedy, who has PFIC, which is Progressive Familial Intrahepatic Cholestasis, a rare genetic liver disorder that affects infants and children. Let’s start with some background. Where did you grow up? Tell me something about your family.

Jeremy Knakmuhs: My family grew up in southwest Minnesota. My dad was a farmer for a lot of years, then switched to construction, which ultimately led to our moving out to Colorado with the housing boom back in the nineties. I have an older sister and a younger sister, and then my mom has since remarried, and I have an older stepsister and older stepbrother as well.

David Hirsch: Excellent. You mentioned that your dad was a farmer and then a contractor. Did you spend more time with your dad, or with your mom and your stepdad, when you were growing up?

Jeremy Knakmuhs: Mom and stepdad for the most part—they had more custody—and then with my dad for a couple nights and weekends here and there. I was able to get a lot closer with him during my college years. He had moved back to Minnesota, and I was in South Dakota. So we spent a lot more time together during those couple years.

David Hirsch: Okay. And out of curiosity, what did your stepdad do for a living?

Jeremy Knakmuhs: He was also a contractor. He worked with home builders, but more on the customer service side of things. But still in the same line of work.

David Hirsch: Okay. And how would you describe your relationship with your dad, and then separately your stepdad?

Jeremy Knakmuhs: Very close with my dad. I get together with him quite often. I’m close with my stepdad as well, but not as much over the past few years. He and my mom moved back to South Dakota, so we don’t see them as often as we used to.

David Hirsch: Okay. Any important takeaways from either of those relationships, things that you think about today as a dad yourself that have been instructive?

Jeremy Knakmuhs: No. I had good role models, and I think that’s all carried through to how I am as an adult and as a dad.

David Hirsch: Okay. Well, from what I remember, you went to South Dakota State University. You took a BS in civil engineering, and you’ve been working as a structural engineer for 11 years. I’m sort of curious to know what motivated you to go into engineering?

Jeremy Knakmuhs: I’ve just always had that mindset as a kid. You know, playing with Legos and Erector Sets, things like that. I always had that mindset. My dad being a builder, he basically told me, “Don’t go into building. It doesn’t make good money.” So they wanted me to get an education and do things on the other side of it. And it’s definitely something I really enjoy doing.

David Hirsch: That’s excellent. So I’m sort of curious to know, how did you and Emily meet?

Jeremy Knakmuhs: So originally we met back in seventh grade math class. We were friends. Never dated, anything like that, all through middle school and high school. Both went our separate ways for college. And after college, I moved back to Colorado from South Dakota, and just happened to reconnect on Facebook. We met up a couple times, started dating—and here we are now married and three kids later.

David Hirsch: And the rest is history. So I don’t know if you’d give attribution to Facebook for getting married, but a lot of people are reconnecting online, whether it’s through dating websites or social media like you were just talking about.

So let’s talk about special needs, first on a personal level and then perhaps beyond. I’m sort of curious to know, before Kennedy’s diagnosis, did you or Emily have any experience with the special needs community?

Jeremy Knakmuhs: Not a ton firsthand. My brother-in-law and my sister-in-law both work in the school district with special needs. So we’ve seen how they interact with it. But other than that, that’s the only connection we have. Nothing we’ve ever had to deal with on a personal level before.

David Hirsch: Okay. So how did this PFIC diagnosis come about? What is it, and what was your first reaction?

Jeremy Knakmuhs: So with Kennedy being a twin, it was very easy to compare how she was growing compared to her brother. She was not growing at the same rate. She was having feeding intolerance issues. So we began just seeing the pediatrician. They thought it was reflux and treated her for that, but it continued to get worse.

And one day our daycare lady recommended she go to urgent care, because she was completely out of it, not herself. So we took her to urgent care, and they agreed something wasn’t right. They recommended she go to the ER so that blood work and other types of testing could be done at a faster rate.

I took her there that night. They did tests quickly and found that one of the big factors was her vitamin K levels were extremely low, which caused her to have a spontaneous brain bleed. This ultimately led to her being sent by ambulance up to the Children’s Hospital Center in Denver.

We were there the first time, I think for three or four weeks. Each day was just test after test. They were basically ruling things out. Okay, it’s not this disease, it’s not this disease, it’s not this disease. And when they started getting to the bottom of the list, the GI specialists there who specialized in liver were pretty positive that this is what it was.

So then they did a liver biopsy. They took a sample and sent it off for testing somewhere on the east coast. I’m not sure where exactly, but they were the only lab in the nation that could do the test. That took, I think, two months to get the results back. They basically started treating her for what they thought it was. That was in March or April, and then I think June or July is when we got the official diagnosis of PFIC.

David Hirsch: And what is that? Just so our listeners know.

Jeremy Knakmuhs: So PFIC affects the liver’s ability to process bile. And when your liver processes bile, that is how your body absorbs fat soluble vitamins. That’s your vitamin K, your calcium, vitamin D, you know, very critical things. So because of that, she couldn’t absorb those type of vitamins and minerals on her own.

So part of what she was on in the beginning was she got extra vitamin D, she got vitamin K shots, she got extra calcium, and a medication to help with all of that, since her body wasn’t able to process it on her own. And then due to that, one of the side effects was she got rickets, because she lacked the vitamin D and calcium in her bones.

David Hirsch: Wow. It sounds like quite a journey. Did I remember you telling me in a prior conversation that she’s also had a liver transplant?

Jeremy Knakmuhs: Yep. So right now, the only true cure for PFIC is a liver transplant. Some kids are able to treat it with certain medications, and it can prolong it. Others are rushed to transplant right away. It was just about a year from diagnosis when she got her liver transplant. She was 18 months old. So that’s basically what it ultimately led to for her. 

What it took to get there was when the bile backs up in the liver, one of the biggest side effects is itching. The kind of slogan for PFIC is “Stop the Itch.” So kids itch all the time. They itch until they bleed all over their body. No amount of lotion or anything helps. Prior to the transplant, she was on three anti-itch medications. I don’t know if they helped or not. But that was part of it.

And then the final straw for Kennedy needing the transplant was that her spleen was starting to enlarge. So basically there was so much damage done to the liver that it was going to make its way to the spleen. By doing the transplant, they basically saved everything else.

And it’s not like there’s available livers all over. So we just went to Facebook again. My older sister did a Facebook blast, looking for liver donors, because it’s an organ that you can give just a portion of, and for the donor, the liver will regenerate. There would also be no issues for Kennedy. That piece of the liver grows and becomes a fully functioning liver.

So we had responses from all over the country. It was just insane how big that network became. We read through dozens of emails, if not more, from people that responded who wanted to help. And we ultimately chose Caitlin Roth, mainly because of how she reached out.

She and her husband had tried for kids but were unsuccessful, and she had always wanted to become a nurse. Her husband at the time was active duty in the Army, and basically she took it as a sign that she was meant to do something else other than be a mom, at least at that time. And so that one really spoke to us.

At that time they lived in Texas. They’ve since moved to Colorado, and we get together with them as much as possible. They’ve definitely become a part of our family as well.

David Hirsch: What an inspiring story. Thank you for sharing. I was unaware that there were living liver donors, as opposed to organs that are donated when somebody loses their life, and their organs are used to save another—or others for that matter. Very inspiring to learn that. And I’m wondering if you think of Caitlin as being one of the angels.

Jeremy Knakmuhs: Yep, definitely.

David Hirsch: You know, without people like Caitlin, where would you be today? Right?

Jeremy Knakmuhs: Yeah. I mean, maybe we would’ve found another one, or maybe not. I don’t know. But there’s no doubt that she saved Kennedy’s life.

David Hirsch: Yeah. Well, thank you for sharing. I’m curious to know, is it like water under the bridge and all blue sky from here, or are there some other implications to having this disease?

Jeremy Knakmuhs: There have certainly been further complications. A transplant is not the cure, and the next day everything’s gone. Kennedy continues to have issues with the bile duct. Where her old bile duct and the bile duct from Caitlin were sewn together, she’s had issues there. Basically there’s a stricture, and it causes the bile to back up into the liver again.

I would say she has had probably five or six different operations to put in stents. Or maybe even higher than that. They go in, they place a stent to open that back up, but those can only be in place for about eight weeks. Then they remove them or put a new one in. Most recently she got to have it removed, and the bile ducts stayed open after they removed the stent.

So right now things are going well. That is, at least maybe that part of this is going to finally be behind us. But it’s something that they always keep an eye out for. We still go in for routine blood work, and when certain numbers elevate, they kind of have an idea whether this has come back or something like that.

With the lack of vitamins and all of that growing up, Kennedy is very behind on her motor skills. Vocally, she is far advanced. She’s always been that way. She can have a pretty clear conversation at her young age, even better than her older brother at times. But she struggled with her motor skills. She didn’t walk until she was almost two years old. She didn’t crawl until after she was one. She really didn’t even crawl before the transplant.

With her feeding intolerance prior to transplant, she also was tube fed. We started with the nasal tube and then, with either her or her brother pulling that out, that got switched to a gastric tube through her stomach wall, mainly for safety reasons. So she had her little pump that she was connected to, and I built her a little cart so it would roll around the floor next to her.

And at one point when she was was actually starting to lose weight, they switched her to IV nutrients through a central PICC line. Then she got TPN for several weeks at least, basically until she started kind of making that curve back into the growing process. So all of that was after transplant. She still had a long way to go.

She still goes to physical therapy about once a week. She also went to occupational and speech therapy to help with her feeding after the transplant and with her fine motor skills. Right now, the biggest thing we’re working on is just getting lower body strength. She always favors her left side, for whatever reason. But now we’re really trying to strengthen her whole body so she can be more of a kid.

David Hirsch: Yeah. Well, with all that you’ve described, it’s hard to remember that she’s only three years old. I’m sort of curious to know, if you look back over the last three years, can you pinpoint any meaningful advice that in hindsight seemed to be instrumental along the way?

Jeremy Knakmuhs: I think for us it was trusting our gut. We might have been able to catch this sooner, before the brain bleed happened and all that. We knew something wasn’t right with her, the way she was growing versus her brother. Something just seemed off. We kept asking, and we kept getting the runaround from our pediatrician.

So in hindsight, looking at it now, I wish I would’ve pushed harder to get to a specialist, and maybe we could have caught some things earlier. Because of how her disease progressed, I don’t think that it would’ve saved her the transplant. But maybe we would’ve known about it sooner, and she could have gotten the help she needed sooner. We had it in our gut that something wasn’t right. So trust your gut on a lot of things.

David Hirsch: Yeah. Well, the way I’ve heard it phrased before is that you know your kids better than anybody else.

Jeremy Knakmuhs: Mm-hmm.

David Hirsch: And I say this with no disrespect to any professionals in the medical profession otherwise. You need to listen to what’s on your heart. Maybe that’s just another message for moms as well as dads to lean into, right? Don’t be passive. Don’t take this wait and see attitude. If it doesn’t feel right or look right, be more intentional, more proactive.

Jeremy Knakmuhs: Yeah, absolutely.

David Hirsch: So I’m sort of curious to know what impact Kennedy’s situations has had on her siblings, your marriage, or the rest of your family for that matter.

Jeremy Knakmuhs: It’s affected everything. As parents, Emily and I try to make sure our boys also get the attention they deserve. It was often hard to give them that attention when Kennedy needed so much. There were times where we had a strict medicine regimen. It was this time she gets this, this time she gets this, and the multiple doctors visits. We were counting it up at one point. She’s had over 200 nights in the hospital as well. So that’s nights that one parent isn’t home, so one of us is here taking care of two kids.

Emily was forced to leave her job because of how much care Kennedy needed in the beginning. She ended up leaving her job because I made more money. If she had made more, I would’ve been the stay-at-home dad. We just had to do what our family needed.

That’s definitely taken a toll financially, and on everything. She was a legal assistant at a law firm, and she was very successful. But it’s something that she gave up to take care of our kids, and Kennedy specifically. So that’s definitely been a strain.

We can tell our boys will act out when there’s something big going on with Kennedy, and they’re not getting as much attention as maybe they do on other days. So we’ve definitely tried to give them one-on-one time when we can. I take the boys camping. And they really love Monster trucks, so we’ve taken them to Monster Jam when we can. Obviously the last year with Covid we couldn’t go to those. But you know, we try to do those things just with them so that they get some one-on-one time, and that definitely helps with them.

With our marriage, it’s definitely been tough. We were fortunate. My company had an employee assistance program that provided counseling, so for a long time, Emily and I were going to counseling. Not necessarily marriage counseling. It was more how do we learn to cope with a child with a rare disease, and how do we still be good parents to our other kids? And how are we there for each other?

That definitely helped. For anybody going through this, I would highly recommend getting the help like that. Even Colton, our oldest, has gone to a couple of those, because they can’t always communicate with us what they feel as well. But it’s definitely put a strain on all of that.

Because of tube feeding and the all of Kennedy’s issues, she was in our room until she was just about two years old. So that was two years of not having any privacy with my wife. And it made things a lot more difficult. But it’s something that we worked on.

If it weren’t for our extended family, we also wouldn’t have gotten through all this. Emily’s mom Kathy has been our savior. She’s retired, but she drops everything she’s doing to help watch kids if Kennedy’s in the hospital, or she will even come sit in the hospital with Kennedy so we can just go outside for a walk, go get lunch, and things like that. She’s been amazing.

My sister-in-law Courtney will watch our boys any time we have to run to the hospital or things like that. Between her and my mother-in-law, they watched our boys for the first week we were in the hospital, because we were in the ICU, and neither one of us was leaving. We were staying there with Kennedy.

Then my brother-in-law Doug, he’s always the go-to, to bring lunch or dinner. He works near the hospital, so I don’t know how much he’s spent on food for us, but I’m sure it hasn’t been a little amount. He’s always good to help out. And my brother and sister-in-law have brought our family a lot of dinners. And my younger sisters helped out, sending DoorDash. Without them, I don’t know how we would’ve gotten through this. They’ve all been tremendously helpful.

David Hirsch: Well, thanks for sharing. And you know, you’re fortunate that there are extended family members that can be there. Because if it was just the two of you, like you said, the story would be quite a bit different, right? It’s stressful enough just based on what you’ve described. It certainly takes some of the stress out of it, if you’ve got that support network, doing this, doing that, and just allowing you to have little bits of normalcy here and there.

So have there been any supporting organizations that you’ve relied on, either for Kennedy or for yourselves, that we haven’t talked about?

Jeremy Knakmuhs: So when we first found out about PFIC, we just Googled it. There were not a lot of things. There’s pfic.org, but that was just barely being created. Since then it’s gotten a lot bigger, and there’s a lot more information available to us and to other families.

And it’s another one of those places where Facebook has really helped. There’s a PFIC page. We use it to meet new families. My wife and I are always available for new families if they have questions. “Hey, what can we expect with this disease?” For the families that are just getting diagnosed, because it’s so much different than the diseases you’ve heard of, it’s a lot to process. So we try to stay in contact with that.

My wife has been on a lot of support teams and stuff like that with other moms. And we’ve also worked with Donate Life Colorado since Kennedy is a donor recipient. We’ve gone to different events that they’ve held and things like that. There are organizations through the hospital that have helped us. Some helped get us meal vouchers at the hospital. Some have even helped pay some bills while we’re there.

It’s the hospital that helped us get onto Medicaid for Kennedy as well, so they at least help with some of the expenses that our basic insurance doesn’t cover. And they even helped get it so that Emily gets paid a small portion to take care of Kennedy. Since she’s unable to work, she qualified for that as well. So that at least helps supplement some of the income we lost when she had to leave her job.

David Hirsch: Excellent. Well, thanks for mentioning that. And I’m wondering, what relationship you’ve developed with this Albireo Pharma? What’s the connection there?

Jeremy Knakmuhs: They’re a pharmaceutical company that’s working on more treatments for PFIC that would hopefully eventually lead to kids not needing a transplant. Whether it’s medication for life or something like that, it’s still better than a transplant. Kennedy will still always be on medication for life with her anti-rejection meds.

I’ve recently started interacting with them. My wife has done quite a few meetings with them to just try spreading the word. What they’re very much interested in doing is spreading the word about this rare disease and other rare diseases as well. Because people only know about them if there’s more information brought forward. So they’ve done a very good job of marketing it and bringing this disease and treatment to light.

David Hirsch: Okay. So given the fact that you have had pretty significant experience, I’m wondering if there’s any advice you can share with other dads who might be listening. Not as relates specifically to PFIC, but just as a dad raising a child with a rare disease.  

Jeremy Knakmuhs: It’s probably to just be there. Seek the help that you need,  for yourself, for your family and for your child. Basically fight for everything you possibly can. One of the things we also had to fight for with Kennedy was our insurance. At the time they would only pay for a transplant in certain hospitals in the U.S. For us, that would’ve meant we had to travel to Los Angeles with two other kids, and a career that was…it was going to put a huge complication into things.

So we fought really hard to get the insurance company to change their policy on that. We wrote an extremely long letter. We went into the statistics of what the success rates were in different hospitals across the country. The children’s hospital here in Colorado actually had a higher success rate than the one they were willing to pay for in LA. And once we found that, it was really hard for them to kind of continue.

My employer also got involved. They got our HR department and everybody involved. And we ultimately got the decision we wanted, to have her transplant done here. So we were here with our support network. We were here with each other.

And so, yeah, my best advice is fight. If it means fighting an insurance company, it’s not easy, but you can do it. I’ve done it several times over things. Push for as much as you can. Push your doctors for the answers you’re looking for. You know, push as much as you can. And for us, it’s helped.

David Hirsch: Yeah. Well, thanks for sharing. And I think what I heard you saying again is that you know your situation or your child better than anybody else, and you need to be the one that speaks out. You need to be the one that is intentional about the situation, and you don’t want to get pushed around, to the extent that you can avoid it.

So let’s give a special shout out to Caitlin Gallagher at Barry & Company on behalf of Albireo Pharma for helping connect us. Is there anything else you’d like to say before we wrap up?

Jeremy Knakmuhs: No, no. I just thank you for letting me be a part of this. Thank you, Caitlin, for continuing to expand the PFIC network, how you’re doing it. I’m just glad to be a part of this, to help tell Kennedy’s story and help other dads out there that might be going through something similar.

David Hirsch: If somebody wants to learn more about the PFIC network, Albireo Pharma, or to contact you, how would they go about doing that?

Jeremy Knakmuhs: Best way for PFIC is just pfic.org. And yeah, you can feel free to post my email address if you want to on the podcast, and anyone is always welcome to reach out. I’m always willing to talk.

David Hirsch: Excellent. Well, we’ll include those in the show notes, so it’ll make it as easy as possible for somebody to follow up with you. Jeremy, thank you for taking the time and your many insights. As a reminder, Jeremy is just one of the dads who’s part of the Special Fathers Network, a mentoring program for fathers raising a child with special needs. If you’d like to be a mentor father, or are seeking advice from a mentor father with a similar situation to your own, please go to 21stcenturydads.org.

Thank you for listening to the latest episode of the Special Fathers Network  Dad to Dad Podcast. I hope you enjoyed the conversation as much as I did. As you probably know, the 21st Century Dads Foundation is a 501(c)3 not-for-profit organization, which means we need your help to keep our content free to all concerned. Would you please consider making a tax deductible contribution? I would really appreciate your support. Jeremy, thanks again.

Jeremy Knakmuhs: Thank you.

Tom Couch: And thank you for listening to the Dad to Dad Podcast presented by the Special Fathers Network. The Special Fathers Network is a dad to dad mentoring program for fathers raising children with special needs. Through our personalized matching process, new fathers with special needs children connect with mentor fathers in a similar situation. It’s a great way for fathers to support fathers. Go to 21stcenturydads.org.

David Hirsch: And if you’re a dad looking for help or would like to offer help, we would be honored to have you join our closed Facebook group. Please go to facebook.com/groups and search dad to dad. Also, please be sure to register for the Special Fathers Network biweekly Zoom calls held on the first and third Tuesdays of every month.

Lastly, we’re always looking to share interesting stories. If you’d like to share your story, or know of a compelling story, please send an email to David@21stcenturydads.org.

Tom Couch: The Dad to Dad podcast was produced by Couch Audio for the Special Fathers Network. Thanks again to Horizon Therapeutics who believe that science and compassion must work together to transform lives. That’s why they work tirelessly to research, develop, and bring forward medicines for people living with rare and rheumatic diseases. Discover more about Horizon Therapeutics at horizontherapeutics.com.