Jen’s whole life changed when her daughter was diagnosed with cystic fibrosis. She shares valuable advice on how to find joy and hope even with diagnosis.
Jennifer Hepworth is a wonderful woman who was born in Southern California, but calls Gilbert, Arizona home. She currently lives in Davis County, Utah. She married her husband seven years ago and they have four awesome children. Jen spends her free time volunteering for the Cystic Fibrosis Foundation and sitting on the Intermountain’s Cystic Fibrosis Parent Advisory Council and working the women in her church organization. She also is a volunteer in her children’s classroom and in the PTO. She spends a lot of her time driving her children around to all of their activities.
I met Jen a couple of years ago and she has been blessed and challenged with a daughter who has cystic fibrosis. I will be the first to admit that when her daughter was diagnosed, I didn’t really know what it was. And so I watched her go through those early years of diagnosis and figuring stuff out, and I was on inspired by her.
Cystic Fibrosis (abbreviated CF) is a genetic disease which affects most organs in your body, but primarily the respiratory and digestive systems. The genetic mutation causes the CFTR protein not to function correctly. So your salt doesn’t go the places it’s supposed to go. So people with CF end up with very sticky mucus which lines their lungs, intestines, stomach, and it wraps around their pancreas.
If you are a healthy person, your mucus has the consistency of water. With CF, the mucus is more like peanut butter and so it’s very easy for bacteria to stick in there. So, if a person with CF gets a cold, that bacteria can overgrow and cause a lung infection and they can go into the hospital for two weeks. It’s usually 10 to 14 days for IV antibiotics after doses at home and trying to get it kicked at home. That hospital stays are often part of the treatment for people with CF.
Jen had been married for 8 months when she got pregnant with their first daughter. Jen had a really hard pregnancy and was on bed rest and had to stop working. So Jen and her husband John actually moved in with her in-laws who were wonderful and let them take over their basement. Finally Penny was born and they were in love with this darling baby.
Penny kept doing this “snort” like she was trying to gasp for air and snort. Jen took the problem to her doctor, and they passed it off as Jen being a crazy first time mom. Penny was learning how to breathe correctly.
So, the third time she had Penny in, they told Jen they needed to talk to her about something. They then told Jen that Penny’s newborn screening had flagged for cystic fibrosis. This was shock to Jen. The only thing she knew about cystic fibrosis was that John’s cousin passed away from it when she was 14 years-old. So, all Jen knew was that CF was life limiting.
The doctor’s office told her they had an appointment for her the next day at Primary Children’s Hospital, to do a sweat test. So, they went and had to wrap Penny’s arm they put gauze and then make her sweat. And three weeks old babies don’t really sweat. So she held Penny and had a space blanket wrapped around her trying to get a little bit of sweat so they could see what her sweat chloride was. And while they were waiting, there was a genetic counselor who came to talk to them. She explained that if Penny got a mutation from John and a mutation from her that it was a one in four chance.
Jen felt horrible when they told her this. She thought, “Cool. Great. Thanks. I did this to my baby.”
In the hours between when the pediatrician told her this is a possibility and the results coming in, she had convinced herself it was just a false positive. Jen thought maybe Penny is a carrier because she got one mutation from my husband, but she knew there was no history of CF in her family.
The genetic counselor called her back with the results of the test and Jen remembers that so clearly. She was sitting on my mother in law’s couch and nursing Penny. They told Jen, “The results came back and she definitely has cystic fibrosis.” Jen said, “Okay” and then hung up. She couldn’t say anything else to the genetic counselor.
When Jen was told her daughter had cystic fibrosis she felt like, “Every dream I had for this child just like fell to the floor. She told her family, “She has CF.” and then she started bawling. Her family just hugged her. She felt like she had such a perfect baby, but now she found out that, “no, she’s not.”
The next day they had an appointment at Primary Children’s Hospital, where they have an amazing CF clinic. They started talking to them about CF, and told them, “Don’t Google it, because that’s terrible and has a lot of outdated information.”
Jen found out Penny’s life expectancy was 37 years old. Her husband is 38 now, so to Jen that’s barely grown up. That’s not acceptable. Jen didn’t realize when she went in to talk to them that they were going to tell her a life expectancy for her child. Jen began to wonder, “How do I just go about raising this child with this limit or this time clock in my head?” She was glad her husband and mom were there with her because it was so overwhelming. They began telling her how they are now going to be feeding their three week old applesauce because that’s the only way to deliver the pills that she has to take to be able to digest her food.
Unfortunately Penny was dropping weight quickly because her pancreas was clogged with mucus, so it could not release the digestive enzymes she needed to break down the food and absorb the fat and the proteins, the carbohydrates. So with CF you have to have to take pancreatic enzymes.
They also told her she might not be able to breastfeed because it was important that Penny get enough calories and enough fat. So they might have to fortify. As they told Jen this she thought, “Oh another dream. Like there it is on the floor.”
Luckily for Jen, she was able to breastfeed.
Later they had Jen come in so they could teach her chest therapy and how to do nebulizers for Penny. With chest percussion or percussive therapy they give you these little like rubber cups and you have to pound on eight different spots on her body for three minutes each. Jen said that with a tiny baby it kind of looks like you’re beating them because it’s on the front of her chest, the back of her chest, the lower lobes of the lungs and her sides.
After that you give Penny an inhaler and do a nebulized medication, which, at five weeks, was okay but for an eight month old (who doesn’t want something pressed on their face for 20 minutes) it was challenging. So since Penny was five weeks old, she has done chest percussion therapy a minimum of twice a day for 30 minutes. It can be up to four times a day when she’s sick with more nebulizers. Now Penny is six years old and has a vest to do the percussions instead of having to have Jen do it by hand.
A typical day for Jen looked something like this: first they woke up, hen she gave Penny her medicine and her vitamins. Next, she fed her. Then they did the inhaler, the nebulizer and then her chest percussion therapy. After that they usually went to the CF clinic or Jen was on the phone with the pharmacy or the insurance company trying to get Penny’ $4,000 medication covered. More medicine, more chest percussion, and then she’d repeat that the next day.
The whole first year of Penny’s life was such a blur to Jen with learning all these treatments and therapies just to keep her baby alive. So, one of the things that she advises most new parents is, “don’t let CF take away that year because it took my year.” Jen doesn’t remember Penny’s first crawl, her first step – nothing, because she was so focused on what she was doing to keep her child healthy. Jen confesses, “I just don’t remember the first year very well. And it breaks my heart that I don’t remember because I was drowning that whole year.”
Jen is very thankful for the friends, family and her church group. She was constantly getting texts like, “Hey, we love you and we’re going to bring you dinner,” or “I’m sending you some cookies.” This helped Jen know she wasn’t all alone. She sometimes felt that way because her husband was at work and she was staying home with Penny so she wouldn’t be exposed to more germs than necessary.
“Really find your people, whoever it is, if it’s a parent, a spouse, a sibling, a stranger who you met at a parents night activity, who knows what you’re going through. Find your people and lean on them and let them lean on you. Because I have grown more as a person with their help than I would have otherwise and I would have been in so much more despair.”
Getting time away from being a full-time caregiver is hard for Jen because she likes to be in control.
But she knows it is important for her to take care of herself. She has a group of mom friends who all have children with CF. They have started going out to dinner together a few years ago. For Jen, this is her lifeline. “Sometimes because I can go out and be away from that role and talk to other people that 100% understand what it is I’m going through. And we can laugh about things and cry about things and tell funny poop stories (because so much of CF is like how is her poop today). It’s just amazing to have those women because it is my best self care to go and talk to somebody that is right there with me.”
Jen also has a Groupme Chat and so anytime they need anything, they have 10 other moms that are right there ready to give advice or comfort. Jen explains, “The best thing I could’ve done is find this group of women who I love so much and who are right there with me every step of the way. They’re my battle warrior sisters.”
“Find your people who you can talk to, who understands you and if you have to pay that person and go to a therapist, do that. Because it is so important for your mental health, especially if you’re a caregiver. Your mental health is so important because you get so bogged down in who you are caring for and everything that they need. So find your people.”
One of the most important messages Jen tells new CF parents is that, “Hope is the most important thing.” The reason for this is because there are so many new treatments and new medications coming out all the time. CF is so rare that the Cystic Fibrosis Foundation is invested in giving grants to pharmaceutical companies to come up with medications. There’s only 30,000 people in the US with cystic fibrosis, and 70,000 worldwide.
And, so Jen tell them, “Just have hope. There’s so much coming. Your child’s life will not look like a child’s life 20 years ago. It is not the same.”
For example, just as Penny was ready to turn 2-years-old, a medication called Kalydeco was FDA approved for 2-year-olds. Penny started that and has been doing better than Jen could have ever imagined.
Jen explains that every child with CF is going to look different depending on the gene mutation. But everyone can find joy in something. Jen suggests, “You can find joy in doing something you love as a family, even though you thought you wouldn’t be able to with CF.” For example, children with CF can’t be around smoke so they shouldn’t be around campfires. Maybe your family loves camping. Jen’s family still takes Penny camping and have just taught her to move when the smoke shifts.
So Jen recommends that parents not let CF steal their joy because you can, “Get so wrapped up in everything you do to keep your child alive that you forget that this is a child and she needs to go and run and jump and play and wrestle with her big brothers and play with friends and go to preschool.”
Jen continues that sometimes her child wants to go play in the mud because there’s some amazing mud. All Jen can think about is the, “Bacteria lurking in that soil that she’s going to inhale and now she’s going to have a pseudomonas infection and she’s going to be on inhaled Tobramycin for 28 days off and on for a year minimum.” So, Jen says it is hard trying to compartmentalize the risk vs. the reward.
Kids with CF can’t associate with other kids with CF. Jen explains that because different kids with CF harbor different bacterias in the mucus in their lungs, they can pass it to each other. So there’s a six foot rule because that is what would be most likely to protect them from passing germs to each other.
So when they go to the CF clinic, they stagger all of the check-in times because they can’t be in the same area together. They can’t wait together. They can’t be at the elevators together. It’s so hard because it’s so isolating when you don’t have a best buddy that you could go and do something with.
So Penny has CF friends she can talk to on Skype or Facetime. Penny has a CF “bestie.” They each had a half of a “best friend forever” necklaces. They Facetime all the time. They do Marco Polo messages, and they send each other videos. This is helpful because they can help each other with taking a new medicine. For example, Penny’s bestie had to start doing sinus rinses. It’s a scary thing for a kid. So, Penny filmed these little videos for her bestie and walked her through all the steps to do the sinus rinse. Since then they have posted them on Facebook for other CF moms to show their kids
It’s so cool to see the technology that can bring two kiddos with CF together, because otherwise they wouldn’t see each other. Penny has told her, “Mom, I just wish I could play with my CF friends and not just see them on the phone.” So they muddle through as best as they can.
Penny knows what all her medicines do, which is quite impressive for a 6 year old girl. She also has learned to hauck a loogie (or expectorate in medical terminology) so they don’t have to put a swab down her throat to test the mucus in her lungs.
Penny also has a 504 to help her with accommodation in school. She knows if someone in her class is coughing, she needs to ask the teacher to move away. Penny also knows she can’t share her school supplies because she picks up germs that way. She also doesn’t gain weight very easily so she has to have a high calorie snack during school. So CF kids are very aware that they have a lot of differences. So, Penny has had to start advocating for her own care so early.
She has been swallowing pills since she was eight months old with the applesauce pouch. Penny is still six and doesn’t want to be different than her friends, but she knows she has to do her chest percussion therapy and her nebulizer. The neighborhood kids all know that she does this and they now ask if they can come sit with Penny and watch a show with her while she does it.
Jen and her husband John grew up in the Church of Jesus Christ of Latter-day Saints. Ever since Penny’s diagnosis John has had a really hard time with God. It is devastating to hear that your child has a life limiting disease. John really struggled with wondering, “How could a loving God let this happen to my tiny newborn? What purpose is there in suffering? Why does Penny have to go through this? What is this trial going to teach her?”
But it has been very difficult for John rectify those questions. He is a very analytical engineer and he wants things to line up in a row. Sometimes faith doesn’t line up.
So Jen has been trying to support John as he deals with this faith crisis that’s ongoing. It has affected their marriage a great deal because at first they didn’t understand where each other was coming from. With Penny’s diagnosis, Jen didn’t know that if John really cared because he was so stoic about it all.
Finally Jen opened up to him and said, “This really bothers me. I never see you talking about [the diagnosis].” Then John was able to explain that he was just trying to be strong for Jen so she could feel like she could come to him. But he was dying inside. Jen was able to explain that she wanted him to know it was okay to be emotional about it. He didn’t have to be the strong stoic husband all the time. Since then John has been able to be more comfortable with talking about it. Their communication has gotten so much better and our marriage has been so much better for that.
Jen says that having Penny has taught her a lot. Jen explains, “I don’t have the same faith struggles that he does and I am very much someone who thinks, I don’t understand now and that’s okay because one day I will. And if it’s not in this life, then when I meet God, I will understand what the purpose of this was and why we watched these children suffer.”
Of course John doesn’t have Jen’s “wait and see attitude towards anything in life.” The key to keeping their marriage strong through all of this has been learning to better communicate where they each are spiritually. Jen says, “And so we’ve had to learn to communicate a lot better to try and work through this.”
Jen says that having Penny have CF has strengthened her relationship with God. She explains that she has had, “to rely on my Heavenly Father so much, throughout this whole journey because I don’t think I would’ve made it through that first year without having someone to just cry out to and tell how much I’m struggling and have that kind of warm feeling that He knows that I’m struggling.”
She also explains that having a child with CF has helped her, “Understand God a lot better.” As she has watched Penny suffer, she wondered, “How [God] had to let such awful things happen to His Son and that He was scourged and crucified and had to go through so much pain. And growing up you’re like, oh man, that that was so hard for Christ. Yes, 100%, He went through that. And now with this perspective, I see, Heavenly Father stepping back to allow that to happen. I don’t know how He could do that and how He couldn’t just snatch Jesus up and just take away that pain. He needed to go through for us. Right? And so as I have gone through this, I have learned more of the nature of God. I’ve always had faith in God and in Jesus Christ but it’s been very passive.”
But now Jen has a relationship with God which she didn’t have before. “I can more fully understand the nature of God and us as his children. That, no, he doesn’t want my child to go through this. He doesn’t want this for her. He still loves her even though this has happened. It is taken a while to see all of that. And my husband does not see those things now. I hope that one day he will, and one day he’ll understand that this wasn’t done to her. It’s not a punishment to her or to us. And that it is something that happened. And we have to have faith that one day we’ll understand, but today isn’t that day.”
“So keep loving. Keep trying. Keep trusting. Keep believing. Keep growing. Heaven is cheering you on today, tomorrow, and forever.”–Jeffrey R. Holland, “Tomorrow the Lord Will Do Wonders among You”
When Jen read this quote, it really struck her. You have to keep trusting and you have to keep believing. Heaven is there for you there. It’s behind you and God is with you. You may not see it while you’re mucking through and it’s awful and it’s hard. But God still loves us and He is cheering for us.
“Even if you cannot always see that silver lining on your clouds, God can, for He is the very source of the light you seek. He does love you, and He knows your fears. He hears your prayers. He is your Heavenly Father, and surely He matches with His own the tears His children shed. . . Don’t give up, boy. Don’t you quit. You keep walking. You keep trying. There is help and happiness ahead—a lot of it. You keep your chin up. It will be all right in the end. Trust God and believe in good things to come.” –Jeffrey R. Holland, “An High Priest of Good Things to Come”
Jen loves watching him give this talk on the website as he shares a discouraging experience he had as a young father. “It’s so powerful to me and it brings me a lot of peace and to know that I’m not alone in struggling.”
Jen’s final bit of advice is to caregivers, “You have to take care of yourself. You still have to go to the dentist every six months; You still have to get your mammogram; You still have to put yourself first in some areas–otherwise you’re not going to be the best caregiver you can be.”
Jen also points out that, “If you are not taking care of you, you cannot take care of someone else. And however that looks for you, if that’s, you need to go for a drive, go for a drive. If you need to stop and get a diet coke, you get that diet coke, like whatever it is that you need to give yourself a little bit of self care, do it because you need it.”
After taking a break and taking care of yourself, Jen reminds us that we come back and your “Batteries are recharged and you’re like, okay, I can, I can face life again.”
So it, if you need permission to take care of yourself, here’s your permission. Jen and Tamara say, “Go do it. Take care of yourself. You need to take care of yourself so that you can be a better caregiver in whatever area that is. You are in the quicksand with little kids and you’re just trying to keep your head up. You do whatever it can to give you that step up. And if that’s going to get a pedicure, you get that pedicure.”
Facebook: Jennifer Harris Hepworth