Cecelia’s Story Posted on February 21, 2019 By Kara Satalino, Cecelia’s mom Photo credit: Through My Lens Photography My daughter, Cecelia, looks like any normal 3-year-old, and she is definitely the sassiest little girl I’ve ever met. She’s wild and smart. She loves to run and play sports with her three older brothers, especially soccer. At the boys’ games, we have to play with her on the sidelines to keep her energy occupied, and she will try to join in on the game. She’s too young to play on our town teams, but we’re hoping to get her involved in sports early because she loves to play so much. Because of Cecelia’s vibrancy and energy, you’d likely never know that she has cystic fibrosis (CF). As a family, it was so hard learning her diagnosis. But we didn’t have to face it alone. We’ve had tremendous help and support from Dr. Melanie Sue Collins and the rest of the pulmonary medicine team at Connecticut Children’s Medical Center. They’ve been there for all of us as we deal with her disease together. They are part of our extended family now, whether they like it or not! The first 12 days Every child in Connecticut gets the heel-prick test when they’re a few days old. The doctors are looking for various genetic diseases that could be present. Cecelia was no different, but we were so caught up in the joy of our new little girl’s birth that we didn’t think much about it. You just don’t think about your child having anything seriously wrong. For the first few days of Cecelia’s life, I treated her just like I did my three boys: Gabriel, who’s now 11; Luke, 9; and Samuel, 5. But there were a couple of times when I would wonder if something was a little off; for example, I’d taken a photo of Cecelia while the boys were playing in the pool and she was in her baby carriage. “My gosh, her belly looks so big,” I thought to myself. It was obviously bloated then, but at the time, I didn’t realize anything was wrong. A few days before we got the call from the doctor’s office, Cecelia was up all night filling dirty diapers, and she wasn’t able to go to sleep because of it. The boys had never had anything like this happen to them, but I just thought she had a fast metabolism. I thought that maybe I should mention it to the pediatrician when we went in. Then, when Cecelia was 12 days old, the doctor’s office called. My husband, George, took the call, because I had run to pick up the boys. When I got back, George told me the doctors wanted to see us that afternoon at 5. I asked why, and he didn’t know. I wondered to myself, “We have Cecelia’s regular appointment this coming Monday, so why is it so important that they see us the Tuesday before?” I told George to call them back and tell them we were coming over right then. I knew something was wrong. The doctors told us Cecelia’s heel-prick test had come back with a strong chance of her having CF, and that we needed to schedule a sweat test for her right away. A sweat test is when a patient’s sweat is measured for high levels of chloride, which is a telltale sign of CF. Cecelia’s sweat test came back positive, and she was diagnosed with CF. CF care from the Connecticut Children’s team I didn’t know it at the time, but when Cecelia’s heel-prick test was flagged for CF, the pulmonary medicine team at Connecticut Children’s had received a heads-up. The sweat test results went right to them, and they started coordinating Cecelia’s care. On the day of Cecelia’s sweat test results, I got a call from R.B. Curtis, the coordinator of the pulmonary medicine department. She told me we were set up for a family meeting with the doctors and dietitians so we could learn about CF and what would be involved with Cecelia’s care. Going into that meeting, I was terrified. All I knew about CF was that there’s no cure and people die from it way before they’re supposed to. I recalled a book I had read when I was a child about a little girl who had CF and had passed away when she was 8 years old. This was a nightmare. During the meeting, the doctors asked about us and took our family history. They also shared with us the current state of CF care and research — how treatments have come a long way; that patients can live long, healthy, full lives with the disease; and that research continues into improving treatments and outcomes for patients. After we left, I felt so much better. Dr. Collins is amazing. After our first appointment with her, she called us to make sure we were doing OK and to find out if we had any additional questions for her. She didn’t have to do that, but she went out of her way to check on us. We see Dr. Collins every month or two, depending on whether Cecelia is sick or well. That’s a huge improvement from where we started, which involved seeing Dr. Collins every week. Cecelia has made great gains under her care. Dr. Collins always listens to what I think we should try as part of Cecelia’s treatment. We’ve been doing this so long that I often have an idea of what she needs, and it makes me feel like I’m really part of her care team. At every visit, Dr. Collins always asks Cecelia how she’s feeling, she typically replies with something silly, but she loves to be included in her care. Gabe and Sam are also under Dr. Collins’ care for severe persistent asthma, which Cecelia has as well. We underwent genetic testing as a family, and it turns out that Gabe and Sam are carriers for CF. They each have one of the two genetic mutations that Cecelia has. No matter how late we call, the pulmonary doctors always take time to answer our questions and let us know what we should do. Drs. Lapin, McLaughlin and Simoneau often work with us when Dr. Collins is unavailable, and they’re terrific. They make us feel like Cecelia is their No. 1 concern, even though they see and take care of so many children. And, of course, the pulmonary medicine staff members are great. Sinead Rodriguez at the front desk is so friendly, and Anita Cruz knows all my kids’ birthdays because we call in all the time. Raisa Quinones, Jessica Parker and Debby Foster are always so friendly and make us feel welcome. Every person in the pulmonary office is amazing. They’re like family to us. The hospital is really lucky to have them. Coordination and communication are crucial to a successful treatment plan, and my husband and I truly appreciate how the pulmonary team works so closely with Cecelia’s other doctors. It helps us keep her care well-managed. We see a number of other specialists at Connecticut Children’s for Cecelia’s related medical conditions, such as: Otolaryngology (ear, nose and throat) Gastroenterology Endocrinology Infectious diseases A day in the life: Cecelia’s home CF care There’s a lot involved in taking care of a child with CF. Every day, like clockwork, Cecelia has to get up an hour before her brothers. She begins with one nebulized medication for about 15 to 20 minutes. Next, my husband does chest physical therapy (PT) on Cecelia. For a half-hour, he claps on her chest, sides and back with two handheld percussors. Chest PT helps break up mucus and lets Cecelia cough it up more easily. This takes about 10 minutes for each stage: Two parts on the front of her chest Under both arms Two parts on the back of her chest After chest PT, Cecelia has another nebulizer treatment. Next are her enzymes, which she needs as part of her feeding through her gastronomy tube (G-tube). Patients with CF often are underweight and have trouble gaining weight, so Cecelia’s G-tube helps her get much-needed calories. Next, Cecelia receives medication for a clinical trial she participates in, followed by her various allergy medications, nasal sprays and vitamins. Then it’s off to pre-kindergarten, where she visits the nurse for her enzymes (at meal and snack times) and her inhaler in case her asthma acts up. On weekends, we do two more hourlong breathing treatments every four hours when she is on her sick plan. All of this is to help keep Cecelia as healthy as possible. It mostly works, but there have been a few times in the past year when she’s had to be admitted to the hospital because she’s gotten sick. We try to keep Cecelia home as much as possible when we know there are viruses and infections going around. While you or I likely could fight off a cold in a week or so, as I write this in February 2019, Cecelia is on her third month and fourth antibiotic for treating a cold. Thankfully, Cecelia doesn’t know anything different. This is just her normal routine. Two of her brothers have nebulizer therapy too, and they sometimes ask for chest PT if they need help breaking up some mucus in their lungs, so it’s nothing too out of the ordinary for her. She does her treatments every day, and she knows skipping them isn’t an option. A family affair with CF Our older two boys had a tough time with Cecelia’s CF diagnosis. We were taken away from them so much in the beginning to go to her doctor’s appointments. They felt left out and jealous of the baby that they started acting out, and they showed serious signs of anxiety. We now see a family therapist on a bimonthly basis. These visits help keep us all in check and make sure that our feelings are normal. They also remind us that we’re all going to be OK. I really recommend therapy to families who are facing CF. It can be overwhelming to keep everything together, and it’s good to have someone to talk to. Sometimes I go by myself if I’m struggling, or sometimes my husband will take the boys if they need help. It’s been a hard adjustment for us as a family as we’ve learned to cope with Cecelia’s CF. In our situation, we went from having two active parents for three kids to having one active parent for three kids and one parent for Cecelia because her care involves so much. We’re working hard to make her treatments work around our busy schedules now. For example, if the boys have sports on the weekends, we try to time Cecelia’s treatments so they don’t have to be done during the boys’ sports. As she gets older, she is able to join us more and more at these events. We are just extremely cautious about washing hands, wiping down surfaces and sanitizing places she may be playing on or around. Gabe, my oldest son, read an article about CF at school last year and got really scared. He came home and asked me in front of everyone if Cecelia was going to die. We’ve explained that times are different now, and Cecelia’s going to live a healthy, happy life as long as we keep up with her care. But it’s tough to explain that to a little boy who’s just scared for his sister. It’s taken a lot of work and finagling, but in the last year, the kids have started to ease off of the anxiety they were feeling for the first couple years of Cecelia’s life. We talk to them about it in child-friendly terms, but we try to not hide anything from them about it. Stronger together My message to other parents and families facing CF is this: You are not alone. I find strength and comfort by reaching out to other CF parents on social media and through email. Even though each patient is different, there are similarities between cases and situations, and I often find that other people’s stories help me through their shared experiences. I also try to get involved with area activities provided by the Connecticut chapter of the Cystic Fibrosis Foundation, such as Parent Nights and walks for CF research. CF can feel like an isolating illness, but it’s not as scary when you connect with others. I’m grateful for the care and support we’ve gotten along the way from the Connecticut Children’s pulmonary team. I’ve recommended Connecticut Children’s and the pulmonary medicine division in particular to so many other people and families since we’ve been with them. As a family, we appreciate how amazing the team is for us. I don’t know where we’d be without them. We — our boys and Cecelia — are much better off for having them in our lives. Contact our pulmonary medicine team for more information about how we can help if your child has CF.