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Remembering the Power of Stories in Pediatric Research

2019; Elsevier BV; Volume: 207; Linguagem: Inglês

10.1016/j.jpeds.2018.12.001

1097-6833

Jennifer Degl, Deborah Discenza, Keira Sorrells,

Ethics and Legal Issues in Pediatric Healthcare

See related article, p 117Pediatric research is detailed, exacting, and scientific. It provides a clear picture, a freeze frame of the study of a particular issue related to children in their infancy, toddlerhood, childhood, and adolescence. Within studies, the clinical statistics automatically mask faces, remove stories, and keep the focus on sterile data in hopes of educating colleagues on important health topics such as the quality of life for infants with bronchopulmonary dysplasia, as reported by Brady et al1Brady J. Zhang H. Kirpalani H. DeMauro S. Living with severe bronchopulmonary dysplasia - parent views of their child's quality of life.J Pediatr. 2019; 207: 117-122Abstract Full Text Full Text PDF Scopus (12) Google Scholar in this volume of The Journal. But although Brady's report focused on the parental perspective, we suggest that stories provide a bigger picture on the day-to-day complications these infants face, especially when looking across a set of experiences. We feel it is important to add faces and stories to the data to provide pediatric investigators and professionals an honest look into the lives of patients that have struggled with pulmonary sequelae of prematurity and how chronic respiratory morbidity affects the life of the entire family. With this in mind, the stories of Becky, Joy, and Zoe are told. See related article, p 117 I always look at my daughter Becky as an outlier from your average preemie. Born at 30 weeks, weighing 2 lbs 15.5 oz, she was deemed early on as the, "She's doing great!" baby. Starting her life with a tiny kitten cry with the fierceness of a mountain lion, Becky went from continuous positive airway pressure to a nasal cannula within 24 hours of her birth. Yet sitting by her incubator I could not reconcile the rising dread within me about her future. Would she come home with us? Do I put the name down on the birth certificate that I had decided on more than a decade earlier? Will she walk? Will she talk? Becky's stay in the neonatal intensive care unit (NICU) went from strength to strength that first week, but then shortly thereafter it seemed it was one issue after another. Three heart defects but avoiding surgery, severe reflux but getting under control bit by bit, complex feeding issues but with patient nurses trying to help her overcome them and a blood transfusion to help her gain strength. All along I was pumping breastmilk, kangarooing my daughter and starting to feel more and more like her mother. Then the infection hit, although at the time I did not know what was going on with my daughter. She was very sick and we came into the unit one day and were bluntly told "not to touch her or interact with her." She was a splotchy green/gray in color and in the end we were lucky she survived. With feeding issues still rattling her homecoming at the 38-day mark, Becky was prescribed oxygen and a monitor at discharge. At home, I felt less like a mother and more like a medical professional. Between the chaos of follow-up appointments, a visiting nurse, the delivery of medical equipment supplies, and team members doing data downloads, we were unsteady in trying to find a proper rhythm to our days. By the fifth day, the instability grew worse as Becky started to sleep through her feedings and the monitor alarms grew more frequent. We called the NICU, then the pulmonologist, and had an emergency appointment that afternoon. After telling us that Becky had lost weight and was struggling, the doctor took one look at us and realized we had barely slept in 5 days. He gave us a choice—do tests as an outpatient or admit Becky to the hospital for those same tests and potential medication and equipment changes. I felt like a complete failure and it was hard to not break down in tears in the doctor's office. But we knew what we needed to do and Becky was admitted that afternoon. A couple of days later, she came home on full-time oxygen and a new regimen that included a new medication, rice cereal in her mix of breast milk and preemie formula and more. After that and a bottle change on our part, life started to progress in the right direction. Yet life never seemed to truly let us relax. A month out from that hospitalization we had the scariest episode on the alarm ever at 4 a.m. As the alarm sounded, I did not actually wake up until my husband and I were already halfway down the hallway running toward my daughter's nursery. With my heart thumping wildly I tried to quickly clear the fog from my brain as we assessed Becky's situation. Seeing her turn blue, we quickly stimulated her, all the while yelling out her name. I tried to remember the infant CPR steps and panicked. Thankfully, she came out of the spell and after a while all was normal again. But we were shaken to the core. I remember my hands shook as I completed the alarm log from the durable medical equipment company. And I wondered right then if we would ever have any type of normalcy in our lives ever again. It was always 2 steps forward and a full step back for us. But I hoped it would get better, and it did. Becky shed the oxygen and monitor within a couple of months and thrived. We were hopeful, but by now knew our lives were anything but normal. A little over a year later, Becky's cold of the winter season resulted in a cough that would not go away. It took 3 months of calls and visits before the pediatrician finally diagnosed her with reactive airways disease. And oddly enough it came on the very same day Becky qualified for Early Intervention due to global delays. With an inhaler prescribed for our toddler, we headed home. I was in shock. Despite slowly shedding specialists over the last year, our "She's doing great!" baby continued to have challenges. I knew then that we had not left the NICU behind. With each cold, I worried that this would be the time Becky would need an emergency room (ER) visit. Each time I woke up to her coughing in bed at night, I immediately assessed whether the inhaler was necessary, or if an ER visit was needed. Becky's persistent feeding issues had weakened her immune system, and she was frequently sick. At 4 years of age, Becky's pediatrician was concerned she might have pneumonia; luckily, it did not proceed to that point. We were also on a constant journey to figure out how to help my very selective eater eat a decent diet. At one point, I consulted a pediatric nutritionist who prescribed a compounded multivitamin that worked, but was not covered by insurance and had to be overnighted from a compounding pharmacy in another state. At 12 years of age Becky entered a solid feeding therapy program and she succeeded beyond our wildest dreams. Her immune system is still weak but improving thanks to a more varied and healthier diet. Today, Becky is 15 years old and we still have the inhaler on hand. The illnesses have started to lessen, but even now I am always listening for that cough during cold/flu season, during allergy season and more. We have never had to go to the ER for asthma, but I am fully aware of that with each new illness this could change at any time. With childhood diagnoses of autism, attention deficit hyperactivity disorder, and then at 13.5 years old mild spastic diplegia and scoliosis, I know all too well that our "She's doing great!" preemie has had her share of difficulties. We are grateful she is as healthy as possible, but we know that this is still not normal. My daughter Joy was born at 23 weeks gestation in 2012, as a result of preterm labor caused by my severe case of placenta percreta. She weighed only 1 pound and 4 ounces at birth and was not even as long as a ruler. I did not get to meet my daughter for several days and our first meeting lasted only a few minutes due to both of our complications. She was not expected to survive. Most parents get to feed, comfort, and hold their babies soon after birth. Joy was in such critical condition that I could not hold her for more than a month. The closest I was able to get to her included sticking my freshly sanitized finger inside a hole in her incubator for a few minutes a day. After suffering a myriad of complications caused by her premature birth, Joy was discharged from the NICU after 121 days and sent home with a diagnosis of bronchopulmonary dysplasia, retinopathy of prematurity, and severe reflux. She was put on oxygen and nebulizer treatments. Because of her severe reflux, Joy would choke after every feed, causing us to worry if she would aspirate while she slept. I put a bed in her nursery and slept with her for almost 1 year. At 8 months of age, Joy was admitted to the hospital because of pneumonia caused by the human metapneumovirus. She was put back on breathing support in the pediatric intensive care unit (PICU) and almost died. Two weeks later, just before she was going to be discharged, her fever spiked again, her breathing got worse, and she was diagnosed with the rhinovirus. She almost lost her life again and remained in the hospital for another week. Joy has had pneumonia 8 times. Most of these times required hospital stays or long days in the ER. We tried to put her in a regular school when she was 4 years old, but she got sick too often and she missed a great deal of school, compromising her education and putting her at a greater disadvantage—so she has been in a small private school for the past 2 years. Her teacher will call me if another student comes to school with symptoms of a respiratory virus and I will keep her home from school for a few days. This is the only way to keep her from developing pneumonia and being readmitted into the PICU. Our family has had to change our whole way of life to keep Joy safe. Her 3 brothers must always change their clothes and wash their hands when they return home from school or activities and we must isolate Joy if one of the boys gets sick, so she is not exposed to it. We cannot host play dates at our home. We have had to skip many holiday and family parties if another guest shows up feeling unwell. We cannot sit around the fire as a family or camp like we used to because the smoke from the fire causes Joy to experience breathing difficulties. We have had to turn away friends and family from our home if they show up and say that they have a runny nose. Joy is now 6 years old and in Kindergarten. We had to hold her back a year because of a combination of her delays and missed time at school. Although she gets short winded and out of breath when doing endurance activities, she loves to dance and do gymnastics. Joy has been taking inhaled steroids twice a day for her entire life and still needs breathing assistance and bronchodilators when she is ill. Respiratory illnesses continue to last for several weeks, with coughing lasting the longest; bronchopulmonary dysplasia is a complication of prematurity that many parents have to deal with after NICU discharge and there is not enough support for them. Parents are not educated enough on raising a child with a chronic lung disease and what is required to best protect them from respiratory illnesses. If we can figure out some way to protect the lungs of premature babies so that these tiny babies do not have to deal with lung complications and rehospitalizations and reliance on oxygen, and families do not have to live in fear of their babies being catching a cold, then we would be making progress. My daughter, Zoe, spent the first 291 days of her life in the NICU. Of my 3 girls, Zoe was the most fragile from the moment she was born. It was as if she bore the brunt of their prematurity, most notably through her underdeveloped and damaged lungs. In fact, the last 4 months of her NICU stay were solely due to the level of oxygen she required. We waited day after day to be able to wean her to a flow on her nasal cannula that we could manage at home. Zoe spent 14 weeks of her NICU stay on either the ventilator or the oscillator, which, coupled with severe reflux, rendered her unable to take any feeds by mouth due to oral aversion. When Zoe did join her sisters at home, she was discharged on low-flow oxygen, a gastrostomy tube, and with tracheomalacia as a result of damage caused by the endotracheal tubes. Zoe also had profound hearing loss, required hearing aids, and was dependent on oral steroids and inhaled steroids, requiring breathing treatments delivered via nebulizer up to 8 times per day. Although we had home nursing care, the delivery and consistency of care was poor. In fact, it became more of a burden to work with the team instead of lessening the hardship we felt caring for Zoe and her 2 sisters (both of whom had their own medical issues and delays). I often reflect on the mini-NICU we set up in our home and how opposite the "décor" was compared with what I had envisioned when I first became pregnant. Instead of 3 cribs lined up around the room, Zoe had to be separated from her sisters. We simply did not have enough room in the nursery with the added oxygen tanks, concentrator and tubing, pulse oximeter monitor, intravenous pole for the feeding bags and pump, and all of the medical supplies that came with each piece of equipment. We even had our own charting system to make sure Zoe's medications were given correctly, her feeding was fortified correctly, and a breathing treatment was never missed. It was what I call our season of controlled chaos. Life would have been chaotic with healthy triplets, but with the unique needs of our 3 girls and the added fragility of Zoe, we were in a constant state of fear and uncertainty, where every hour was documented in some way. I expected a baby like Zoe to be rehospitalized in her first year post NICU discharge. From everything I read about NICU graduates and the peer mentors I spoke with, a baby with lungs as fragile and an immune system as weak as hers, no amount of hand sanitizer, isolation, or other precaution would ensure she would not find herself back inside the hospital. Added to the fact that she was discharged in October, the middle of cold/flu/respiratory syncytial virus season, and required weekly visits to numerous specialists, it was no surprise when she came down with strep throat in January followed shortly by flu and pneumonia in February. Although the 5-day hospital stay was not a surprise, what I did not expect was to be back in the same hospital just a day later. Throughout the night of February 15, 2008, Zoe had difficulty holding her oxygen saturation level despite increasing her flow of oxygen and doing nebulizer treatments every hour, on the hour. As soon as the sun rose, we rushed her to the hospital which was over an hour away from our home. During that drive, she maxed out on her oxygen tank at 4 L and could only hold her pulse ox sats around 80. Upon admission to the ER, she was stabilized on high flow and received a chest radiograph, which showed pneumonia. I assumed we were in for another week-long stay since her previous stay was due to pneumonia but did not require her oxygen requirements to go as high. After waiting more than 12 hours in the ER, Zoe was finally settled into the PICU. But she did not last much longer on high flow and required bi-level positive airway pressure, where she stabilized for the next 5 hours. Unfortunately, that stabilization would also be short lived. We were asked to move to the waiting room so Zoe could be intubated. My anxiety and fear boiled over and I screamed at the nurse that they absolutely could not intubate her. Knowing how long it took to get her off the ventilator as a newborn, I feared she might never breathe without a machine again and the thought of that crushed me. Of course, medically, she required the intervention and we consented. Before they sedated her, I was able to lean over the side of her bed and look into her big green eyes. A lock of my hair fell from behind my ear and she reached up and twirled it in between her chubby fingers, like she always did when I held her in my arms. I found a single spot of her forehead not covered by the BIPAP mask and gave her a kiss. I whispered in her ear how much I loved her, how strong she was, and that we would be back very soon. It was the last time I would see Zoe's eyes open. My daughter died at 9:30 a.m. on February 16, 2008. After 5 codes, two of which we witnessed at her bedside, the PICU doctor simply asked us if we wanted her to keep working or if we wanted to hold our daughter. Without a discussion, my husband and I quickly stated we wanted to hold her. The tubes and lines were disconnected and her bloated, cold, lifeless body was placed in our arms. We lived every parent's worst fear. A fear that we thought we had left behind us in the NICU. We always thought being in the NICU was the hardest part of our experience with a medically fragile child, but we were mistaken. On Zoe's death certificate the following causes are listed: influenza A, influenza B, pneumonia, bronchopulmonary dysplasia, sepsis, complications of prematurity, and methicillin-resistant Staphylococcus aureus. Her body was attacked by illness and she had no reserve after fighting to live for 14 months. Becky. Joy. Zoe. These names round out the statistical data and add to the research findings on postdischarge outcomes after bronchopulmonary dysplasia. Just as oral histories have carried wisdom from one generation to another over the ages, so do the stories of these 3 girls, these daughters, these human beings. Practical quality of life meets emotion and complications, and these complex realities would never be stated in traditional statistical analyses. Zoe's green eyes are a memory that evokes a stronger picture of what bronchopulmonary dysplasia does to a baby, a child, and her entire family. This disease affects more than just lungs; it affects full lives. We ask that you always seek those human stories to add meaning to your research. It will impact your conclusions in an even more powerful fashion. This article has been written in loving memory of Zoe Rose Sorrells, daughter of Richard and Keira Sorrells, triplet sister of Avery Sorrells and Lily Sorrells. Jennifer Degl is the Founder of Speaking for Moms and Babies, Inc. (www.speakingformomsandbabies.com). She is the author of two books, a member of The International Neonatal Consortium, NIDCAP, the Preemie Parent Alliance and she serves on The Board of Directors of The Morgan Leary Vaughan Fund. Jennifer is also a writer for Huffington Post Parents and The Mighty and has spoken on Capitol Hill on two different occasions to promote legislation on behalf of premature babies. Deb Discenza is the mother to Becky, a 30-week premature infant born in 2003. Formerly the founder of Preemie Magazine, Deb continued on with PreemieWorld, LLC, (www.PreemieWorld.com) a company focused on educational products for parents and professionals. She runs the 46,000+ member Inspire Preemie Community (https://preemie.inspire.com), is a member of boards and committees of various organizations including the Preemie Parent Alliance and the National Coalition for Infant Health and the International Neonatal Consortium and is a columnist with the Neonatal Network Journal, Neonatal Intensive Care Magazine and the COINN newsletter. Keira Sorrells is Co-founder and President of the Preemie Parent Alliance (www.PreemieParentAlliance.org), and has been leading the movement to elevate the NICU Parent Leader as professional in maternal infant health. Together, with a dedicated team of NICU Parent Leaders, Keira has grown the Preemie Parent Alliance to be a sought-after resource and collaborative partner for providers, researchers, industry and other stakeholders in this niche of healthcare and provides unified representation for NICU parents everywhere. Living with Severe Bronchopulmonary Dysplasia—Parental Views of Their Child's Quality of LifeThe Journal of PediatricsVol. 207PreviewTo assess parents' views of their children's health-related quality of life (HRQoL) and the association between neonatal morbidities and HRQoL in children with severe bronchopulmonary dysplasia (BPD) who survived to 18-36 months of corrected age. Full-Text PDF End-of-Life Decisions 20 Years after EURONIC: Neonatologists' Self-Reported Practices, Attitudes, and Treatment Choices in Germany, Switzerland, and AustriaThe Journal of PediatricsVol. 207PreviewTo assess changes in attitudes of neonatologists regarding the care of extremely preterm infants and parental involvement over the last 20 years. Full-Text PDF