Pulmonary Medicine. Shaping the Future of Cystic Fibrosis Care

Transcription

1 Pulmonary Medicine Shaping the Future of Cystic Fibrosis Care

2 2 Nationwide Children s Hospital Pulmonary Medicine

3 In the Section of Pulmonary Medicine at Nationwide Children s Hospital, our innovations and research are shaping future care for patients with cystic fibrosis. From quality improvement projects aimed at improving access to cystic fibrosis medications to discovering correlations between ischemic time and transplant outcomes, our team is devoted to improving outcomes for all patients with cystic fibrosis. We are leading the way in clinical trial participation, adolescent to adult care transition programming and education, and transplant research. From bench to bedside, quality improvement to clinical drug trials, we are actively involved in helping children with CF and their families achieve their best possible outcomes and improving outcomes for generations to come ,108 Clinical Trials Unique Inpatients Unique Outpatients Multidisciplinary Clinics Transition Program Completions Faculty Members 63 Publications Nationwide Children s Hospital Pulmonary Medicine 3

4 Light therapy avoids many of the typical problems related to treating depression during hospitalization. It works quickly it s inexpensive, and it does not interact with other medications that these patients may be taking. Benjamin Kopp, MD Light Therapy Associated with Shorter Length of Stay for Hospitalized Cystic Fibrosis Patients Clinician-researchers at Nationwide Children s have shown that hospitalized patients with CF have a high incidence of depressive symptoms associated with longer lengths of stay. Those patients also have reduced levels of light exposure while hospitalized, according to a 2013 study led by Benjamin Kopp, MD, pulmonologist and principal investigator in the Center for Microbial Pathogenesis in The Research Institute at Nationwide Children s. So could light therapy, an inexpensive and widely-available treatment for depression, have a positive effect on these patients? A pilot trial led by Dr. Kopp showed such a benefit that Nationwide Children s now plans to offer the therapy to all of its hospitalized CF patients. Thirty CF patients were enrolled in the study upon hospital admission. The Quick Inventory of Depressive Symptomatology self-report and clinician-administered survey found at least mild depression in 80 percent of the subjects. Approximately 58 percent were already taking antidepressant medication. The subjects had a shorter length of hospital stay compared to a historical cohort of hospitalized CF patients from the year prior. The two-day length of stay decrease for 30 patients amounts to a cost savings of nearly $300,000, according to the researchers. The average scores on the depressive symptom survey also decreased significantly; only two patients who were admitted with depressive symptoms showed no improvement after light therapy. The Cystic Fibrosis Questionnaire- Revised showed that six out of 12 quality of life measures physical, vitality, emotion, health perceptions, body image and respiratory significantly improved during light therapy. Kopp BT, Hayes D Jr, Ghera P, Patel A, Kirkby S, Kowatch RA, Splaingard M. Pilot trial of light therapy for depression in hospitalized patients with cystic fibrosis. Journal of Affective Disorders Jan 1;189: Kopp BT, Hayes D Jr, Ratkiewicz M, Baron N, Splaingard M. Light exposure and depression in hospitalized adult patients with cystic fibrosis. Journal of Affective Disorders Sep 5;150(2): Nationwide Children s Hospital Pulmonary Medicine

5 Specialty Pharmacy for Cystic Fibrosis Improves Access to Medication For most patients with cystic fibrosis, getting all of their prescriptions filled is a monthly ordeal that results in many phone calls and some level of frustration and aggravation. Many patients with CF need to use three to five pharmacies to get all of their medications, explains Emily Middleton, PharmD, advanced patient care pharmacist in the CF Specialty Pharmacy. Not all specialty pharmacies carry all of the medications these patients need, and insurance rules vary by plan and by medication. Depending on the insurer, it could be dramatically more affordable to use multiple pharmacies. The Nationwide Children s Specialty Pharmacy is the one of the few pharmacies in the United States that has access to all CF medication, says Dr. Middleton. We are working with insurers to get more contracts in place so that we can truly be the only specialty pharmacy our patients with CF need. Even when an insurer won t allow the prescription to be filled at Nationwide Children s CF Specialty Pharmacy, Dr. Middleton and her team offer themselves as a resource to families. We know how to navigate the system, Dr. Middleton says. I have spent hours on the phone with other specialty pharmacists advocating for my patients and educating others about billing and insurance issues. Karen McCoy, MD, chief of Pulmonary Medicine and Cystic Fibrosis at Nationwide Children s, and her team in the clinic are monitoring the impact the specialty pharmacy is making on patients. We have early signs of increased adherence, and anecdotal evidence of reduced frustration and difficulty for families to get the medications they need, says Dr. McCoy, who is also a professor of Pediatrics at The Ohio State University College of Medicine. As we move forward, I expect to find that the specialty pharmacy leads to a quantifiable improvement on the outcomes for some patients. Nationwide Children s Hospital Pulmonary Medicine 5

6 A Graft Ischemic Time Sweet Spot in Lung Transplants for Children with CF It didn t surprise me that the lung could tolerate a longer period of ischemia and still do well after lung transplantation. What was surprising was the fact that we found a sweet spot in which children with cystic fibrosis seem to do better. Don Hayes Jr., MD While common practice in lung transplantation is to limit graft ischemic time as much as possible, some evidence in adults suggests that times even longer than six hours do not necessarily result in worse outcomes. It has been unclear, though, how ischemia times affect survival in children with CF who require a lung transplant. A recent study shows that an ischemia time of four to six hours is associated with better long-term survival than either a shorter or longer ischemic period. The study retrospectively evaluated data from the United Network for Organ Sharing (UNOS) registry on 199 pediatric patients with CF who were first-time recipients of a lung transplant from a cadaveric donor between May 2005 and September Using univariate and multivariate analysis, the groups with graft ischemic times of less than four hours and greater than six hours were comparable both had significantly worse survival after lung transplantation compared to the group with graft ischemic times between four and six hours. Further research is needed to identify the mechanisms involved in this process, but the inferior outcomes associated with shorter ischemic times suggest that a certain level of hypothermia, as organs are placed in ice during this time of ischemia, suppresses certain cellular metabolisms and biomolecular pathways involved in the ischemia-reperfusion process that occurs in solid organ transplantation, says Dr. Hayes, who is also a professor of Pediatrics, Internal Medicine and Surgery at The Ohio State College of Medicine and professor of Epidemiology at The Ohio State College of Public Health. The study has potential implications for transplant centers, as it suggests that they may lengthen the distance that acceptable lungs can travel from a donor to a pediatric recipient with CF. Hayes D Jr, Tumin D, Kopp BT, Tobias JD, Sheikh SI, Kirkby SE. Influence of graft ischemic time on survival in children with cystic fibrosis after lung transplantation. Pediatric Pulmonology Apr 29. [Epub ahead of print] 6 Nationwide Children s Hospital Pulmonary Medicine

7 An Easier Way to Calculate Sinus Disease Severity in Patients with Cystic Fibrosis Sinus disease is common in patients with CF, and computed tomography (CT) is often used to assess the condition. But the most widely used systems for scoring CT scans do a poor job of determining sinus disease severity in CF patients; those systems don t take into account several CF-specific findings. Beyond that issue, the scoring systems are often complicated, requiring a neuroradiologist to interpret scores and even then, clinical and radiological findings do not correlate well. Clinician-researchers at Nationwide Children s have created and validated a new scoring system that uses parameters applicable to CF-related sinus disease and is easily understandable for most clinicians. A recent publication describes the system for use in adults, but an upcoming paper will apply it to children as well. In the past, we have really just looked at CT scans or X-rays and tried to determine severity based on clinical experience because scoring systems don t work for us, says Shahid I. Sheikh, MD, lead author of the publication, and a member of the Section of Pulmonary Medicine and Cystic Fibrosis Center at Nationwide Children s. We thought it would be important to develop a scoring system that does work in CF patients. The Sheikh-Lind scoring system takes its name from its two creators: Dr. Sheikh, and Meredith Merz Lind, MD, a member of the Department of Otolaryngology and senior author of the publication. Five CT findings are considered on each side of the sinuses, for a total of ten: maxillary opacification, nasal cavity obstruction, displacement of lateral nasal wall in the middle meatus, expansion of any sinuses (mucocele) and uncinated process absence or demineralization. Maxillary opacification and nasal cavity obstruction are scored on a 0-3 scale based on severity; the others are scored 0 or 1 based on absence or presence. We believe that this system can be used by any specialty with equal ease, says Dr. Sheikh, who is also an associate professor of Clinical Pediatrics at The Ohio State University College of Medicine. This can give us the opportunity to look at changes in sinus disease severity over time, especially before and after lung transplant and other surgeries when sinus disease is of great significance. Sheikh SI, Handly B, Ryan-Wenger NA, Hayes D Jr, Kirkby SE, McCoy KS, Lind M. Novel computed tomography scoring system for sinus disease in adults with cystic fibrosis. Annals of Otology, Rhinology & Laryngology Jun 29. [Epub ahead of print] Nationwide Children s Hospital Pulmonary Medicine 7

8 Referrals and Consultations Online: NationwideChildrens.org Phone: (614) or (877) Fax: (614) Physician Direct Connect Line for 24-hour urgent physician consultations: (614) or (877) For information on all of our Pulmonary Medicine services, visit: NationwideChildrens.org/Pulmonary Mailing address: Nationwide Children s Hospital, 700 Children s Drive Columbus, Ohio