Two moms, 2 stories: What it's like raising a child with Cystic fibrosis
Posted May 11, 2022 11:20 a.m. EDT
Updated May 11, 2022 11:25 a.m. EDT
Cystic fibrosis, an inherited disease that impacts multiple body systems, is rare. Only about 1 in 2,500 to 1 in 3,400 infants are diagnosed with CF, but two moms at Capitol Broadcasting Company have connected because their children are living with it.
Holly Shaw, a radio account executive at CBC's Sunrise Broadcasting in Wilmington, lives with her husband, 12-year-old daughter Ani and her son, Clyde, or "IV," who is 10. They also have three pugs. IV, now in fifth grade, was diagnosed with CF when he was six months old.
Katie Fulp, director of operations for WRAL Digital Solutions, lives with her husband, 4-year-old son Eli and daughter Virginia, who will turn one this month. Virginia was diagnosed with CF two weeks after she was born.
Q: Describe when you found out your child had CF. What was that like for you?
Holly's story: CF is usually detected at birth, but Holly didn't learn IV was sick until around six months.
Feeding a newborn is already stressful, but Holly noticed IV wasn't gaining weight.
"He was kind of skinny and, anytime he would eat, he would just go to the bathroom immediately," she said.
Pediatricians told Holly, who was breastfeeding at the time, to try to supplement formula, but that didn't help, so Holly began researching IV's symptoms and looking at his bloodwork.
"I kind of became this detective and went and checked out all his newborn screens and his blood work and everything," she said. "Everything was normal."
She knew something wasn't right, so Holly requested a new full panel of bloodwork. IV's liver levels were off, so the family was referred to UNC Hospital, where doctors suspected CF. Holly said she will always remember that phone call. She was picking up her daughter from daycare when she learned her infant had CF.
"It's definitely ingrained in my memory forever," she said. "We jumped feet first into the world of CF. There's a lot of crying and tears and hard times and struggles, but we made it, and he's just like any other average kid. On the outside, you wouldn't even know he has CF."
Katie's story: Katie experienced a healthy pregnancy with her second child, and everything seemed normal. She had to grieve the loss of her mother toward the end of her pregnancy and then a surprising CF diagnosis two weeks after her daughter, Virginia, was born.
The couple was home with their new baby when the pediatrician called and said, based on her bloodwork, there was a good chance Virginia had CF. The family was referred to a specialist at Duke Children's Hospital, who confirmed the diagnosis.
"It took the wind out of me," Katie said. "There was a day where my husband and I didn't really know how to proceed because we just thought everything was okay. There's a part of you that thinks the testing is going to come back [normal]. You try to give yourself a little bit of hope, but at the end of the day, we didn't really know what we were getting into."
Katie and Holly have a lot in common, but some aspects of their stories differ. The 10 years between when Virginia and IV were diagnosed with CF made a lot of difference.
"We likely got a very different conversation," Katie said. "Our conversation was very filled with hope ... about the facts and the research and what has happened in the last 10 years, whether it be medication, research or treatment in general. Being a parent of a child with Cystic fibrosis in 2022 or 2021 is very different than those before us."
Q: What is life like for your child?
Holly's story: At the age of 10, IV is on a lot of medication -- around 40 pills a day, including enzymes to help him digest food and modulators, which are meant to target specific components of his CF. The Shaw family drives to Chapel Hill at least four times a year for checkups and treatments.
To help keep his chest clear, IV wears a lung clearance vest for almost an hour each day -- 25 minutes before school and 25 minutes after school. When he is sick, the treatments take longer, 45 to 50 minutes each.
IV was recently diagnosed with CF-related diabetes, so he has to check his insulin and keep up with what he eats, especially at school, when his parents aren't there.
Although his life is more complicated than most children, IV is doing so well, Holly said. He skateboards, plays soccer and is in a running club, and he walked with his family in the Great Strides for CF fundraiser in Wilmington on May 7.
"On the outside, he's just your average active kid," Holy said. "But behind the scenes, in a month's time, he's taken over 1,000 pills, and he's done over 1,500 minutes of vest treatments."
It's difficult for IV to sleepover at a friend's house, because there is so much to pack and explain to the other parents, Holly said. Their family does travel, but with dozens of bags full of breathing treatment equipment and sterilizing equipment.
"We have to be very, very careful, but we don't keep them in a bubble," Holly said.
Katie's story: Virginia is in a small percentage of children with CF who is pancreatic sufficient, so she is able to digest her food and get adequate nutrition without taking oral enzymes. Still, she takes medication morning and night.
"Virginia was eligible for medication starting at four months," Katie said. "It's a very small percentage of people living with CF that are eligible for these modulators at such a young age."
She was also just fitted for her first airway clearance vest. Vests are usually introduced to CF patients around age 2, but Virginia was able to get one early. Her parents put the vest on her often to practice the routine she'll likely need as she gets older, but they'll also perform pats on her back, chest and sides to help clear her lungs.
Despite Virginia's diagnosis, doctors told the family that the best thing for their daughter would be to treat her like any baby. That's part of the reason Katie and her husband, who both work, decided to put Virginia in daycare.
Just like other babies who attend daycare, Virginia gets sick often from all the germs she encounters. Even though this isn’t Katie’s first time with a child who is constantly sick from so-called “daycare crud,” Virginia’s CF makes your average cold a little more complex.
"I feel like I have gone to the pediatrician faster than I would have with my son -- but maybe not, because I feel like when you're a first-time parent, anything new is scary, like even a cough or a sniffle," Katie said. "I just try to trust my gut, and I definitely seek help, even if it's just help for myself, you know, like to help me sleep better at night."
When Virginia goes to the doctor, it's a little more complicated for Katie and her husband.
"We love her pediatrician, but a lot of the time I have to fill them in of her care at Duke," Katie said. "She goes to Duke once a quarter, and we go to the pediatrician very frequently throughout the first year of life."
Katie is ready at each doctor's appointment to make sure everyone is on the same page.
"Anytime someone comes in, I have my five bullet points," she said, explaining how she'll tell doctors Virginia's CF protocol and ask them to swab the back of her throat to make sure she isn't harboring bacteria. She'll also ask them to notify Duke that Virginia was seen for an illness, although Katie already notifies the specialist anyway.
"That is one of the biggest parts about being a mom and a parent in general, is that you are the advocate for your child, whether or not they can communicate," she said.
Q: What has been the hardest part for you?
Holly's story: The first six months of IV's life, when he was undiagnosed were the hardest for Holly, who was juggling work and caring for two young children, including her newborn, who wasn't gaining weight.
"It was really hard," she said. "Honestly, I never slept those six months before the diagnosis. I had gone back to work and he would get up [all night]. I mean, he was up all the time because the minute he would eat, he'd go to the bathroom. He'd be crying all the time. I think I blocked out half of that six months because I had to. I had to show up and come to work every day. I would be on one hour of sleep trying to hold meetings and stuff. It was probably the roughest time in my life. You know, it breaks your heart when your child is crying all the time."
Once IV was diagnosed with CF and began treatments, he started to gain weight and life became just a little bit easier.
Katie's story: As Virginia's advocates, Katie jokes with her husband that they are the CEOs of their daughter's care plan. For Katie, who is extremely organized, it's both comforting and stressful to make sure all Virginia's doctors and teachers are informed.
Virginia gets sick like all other babies but, because of her CF, she may need to be treated sooner to ensure she doesn't develop a lung infection. Katie fought very hard early in her daughter's life to make her eligible for a specialized Synagis injection that gives her antibodies to better fight off respiratory syncytial virus, or RSV, during the winter months.
"I was just nervous across the board, and it's a very expensive medication that insurance didn't cover much of," Katie said. "Thankfully the drug company was willing to work with us and give us a really good deal."
According to Katie, without insurance, the cost of Virginia's ongoing CF medication is $30,000 per month, and the cost of the monthly Synagis injection, which must be administered each month between October and March, was close to $6,000 to $8,000.
"The cost of doctor's visits, clinic visits, medication and treatment has been incredibly eye-opening for my husband and me as we navigate everything this first year," Katie said. "Both drug manufacturers have programs set up to assist families, regardless of their household income, which was hugely helpful for us. Even being financially 'comfortable,' the costs of medication can make you question everything when really all you want to focus on is ensuring your child has everything they need and more to fight this disease and thrive."
Q: How has a CF diagnosis impacted your family?
Holly's story: Holly is so thankful for her husband, who supports her and encourages IV to be independent.
"He picks up the pieces when I fall apart, and he's very calm, so he's always there to help out," Holly said. "I think the best thing about my husband is he encourages IV to be just like be a normal kid, giving him responsibilities."
She also is amazed by her daughter, Ani, who reminds her brother to do his treatments and has taken it upon herself to raise money and awareness for CF in a creative way. For years, 12-year-old Ani has made bracelets or painted pictures to sell at the Great Strides event to raise money for research.
"She is like a little mama, she knows CF inside and out," Holly said. "I mean, it can be hard on siblings because the child with CF gets a lot of attention, but she's never been like that."
Katie's story: Since Virginia is still a baby, in addition to managing her care, Katie and her husband work so hard to make sure their daughter grows up being informed about her CF.
"Especially as these children get more hobbies and go to friend's houses and all of these things, I just want to create that habit and help it feel more normal and less like something that has to be a burden or something to be embarrassed of," Katie said, adding she will do whatever it takes to help her daughter thrive as her CF progresses.
"You've got to do your best," she said. "You've got to step up. Even when it gets really challenging, you've got to do more. You know what it's like to be a mom, like when you feel like you've been stretched to the furthest, to the furthest point. And you're like, I gotta keep stretching farther, you know?"
With an amazing mother, father and big brother by her side, Virginia has a lot of people to look out for her. Katie said 4-year-old Eli is very caring toward his sister.
"He feels very responsible for Virginia, so he loves to help and help her not cry, "Katie said. "He'll give her things, play with her. He's been so wonderful throughout the process."
Q: How are you doing?
Holly: "I am doing great. There are definitely days and moments when it's way more overwhelming than others, where, you know, I don't want to fundraise anymore or do all the treatments, but those days are few and far between."
Katie: "At the end of the day, if we can make this diagnosis less scary or less abnormal than it is, then that's a win for us and it's a win for Virginia too."
Q: What do you want others to know?
Holly's story: Supporting the Cystic Fibrosis Foundation and getting support from it is precious to families living with CF. Although their children are nine years apart and live in different cities, Holly and Katie rely on each other for support. Holly has joined a Facebook group for parents with children living with CF, which also helps.
"I want to encourage anybody who is new to this lifestyle or who has a loved one with CF that there's just a big community of people who can support you," Holly said. "The Cystic Fibrosis Foundation is amazing -- they stepped up and were there for me when we got diagnosed. There's a lot of people who care out there, and it's evident with the money we raised with our fundraisers and just people stepping up to help wherever they can."
Katie's story: When it comes to social media and being in public, Katie and her husband first wondered if they should keep Virginia's CF relatively private. They quickly realized they wanted to help spread awareness and open up conversation.
"We want it to be more normal for people that are struggling," Katie said. "Instagram and Facebook and all these social media channels give you a little blip of what life is and what it really isn't. Our goal is just to raise awareness just to live our lives as normally as we can, but at the same time, help people understand that it's okay to not be okay."