The treatments available for persons with cystic fibrosis and for persons with diabetes have both been advancing rapidly over the past half-decade. It is no surprise therefore that care for persons with both conditions, termed cystic fibrosis-related diabetes (CFRD), is advancing as well. An updated set of treatment guidelines for CFRD have been issued by the International Society for Pediatric and Adolescent Diabetes. The guidelines have been indexed in Pubmed (pubmed link) and can be found here (doi: link). The lead author of the guidelines is Dr. Katie Larson Ode from our division. Her co-authors include CFRD experts from Minnesota, Colorado, Indiana, Philadelphia, Australia, Canada, Italy, and Germany. We are fortunate to have Dr. Larson Ode’s expertise and leadership in this field.
Cystic fibrosis is a genetic disease that causes dysfunction in multiple systems, but especially in the lungs which progressively deteriorate. The past few years have seen massive progress in the medical treatment of cystic fibrosis. Drugs have come to market that correct the basic molecular defects that cause cystic fibrosis. These drugs are classified as “highly effective modulator therapies”. These therapies must be tailored to each person, by matching to the different mutations that cause cystic fibrosis. In 2019, a blend of three modulators was approved for treatment of the most common form of cystic fibrosis involving the “F508del” mutation. This therapy combines elexacaftor, tezacaftor, and ivacaftor (“ETI”). This therapy dramatically improves lung dysfunction in persons with cystic fibrosis due to F508del mutation. Persons with cystic fibrosis are at very high risk to develop diabetes. For example, those who have only have F508del mutation have an over 80% chance of developing diabetes by middle age. It is currently not known if ETI-therapy for cystic fibrosis will impact diabetes risk. To address this knowledge gap, investigators from 5 institutions conducted a study of twenty persons with cystic fibrosis. Each person underwent an oral glucose tolerance test before and roughly 10 months after starting ETI-therapy. Interestingly, there was not a significant change in glucose levels after starting ETI. However, C-peptide levels increased with ETI therapy, consistent increased insulin secretion. Accordingly, an insulin resistance index significantly increased as did body mass index. Taken together, these results suggest that ETI therapy produces a degree of insulin resistance, likely related to an increase in body mass index. The longer term impact of ETI and related therapies on diabetes risk and body weight will need careful ongoing study. The faculty investigators involved in the study from our division were Dr. Larson Ode and Dr. Norris. The publication describing the study and results can be found at this Pubmed link.
Therapies for cystic fibrosis are becoming far more effective, improving health and extending life for those with this genetic disease. Decades ago, most persons with cystic fibrosis often died before reaching reproductive capacity. Thankfully, this is no longer the case. For this reason, counseling about reproduction is thus more important than ever in this population. However, little is known about how often health care teams provide reproductive counseling for these patients. This is even more important, because pregnancy can have a highly adverse impact on health in those with cystic fibrosis. To better understand the issue, Dr. Katie Larson Ode and colleagues have reviewed medical charts of persons with cystic fibrosis. They found that most patients did not receive documented reproductive counseling. This highlights a potential gap in care that could be readily addressed. The results from the study have now been published in the journal Pediatric Pulmonology, and indexed in PubMed at this link.
Please join me in congratulating Dr. Katie Larson Ode for her well earned promotion to full professor!! In brief, Dr. Larson Ode has been promoted in recognition of her clinical mastery, her teaching enthusiasm, her compassion as a physician, and her international recognition as a leader in the clinical research field of cystic fibrosis-related diabetes. She joined the University of Iowa Hospitals and Clinics in 2011, having just completed a pediatric endocrine fellowship at the University of Minnesota. During fellowship she simultaneously obtained a Master’s in Clinical Research. She has spearheaded several new clinical initiatives at the University of Iowa, including initiating the Pediatric Endocrinology outreach services in the Quad Cities and serving as the inaugural LGBTQ-clinic endocrinologist. To her peers and trainees, she is highly esteemed for her enthusiasm. Her international reputation stems from clinical studies she directed relating to diabetes in persons with cystic fibrosis. She was chosen by the Cystic Fibrosis Foundation to mentor a cadre of physicians across the country in the endocrine care of persons with cystic fibrosis and related clinical research. She has published multiple manuscripts in this area as well and is regularly invited to talk across the country and even internationally on this subject matter. Once again, congratulations Dr. Larson Ode!
Each June, the American Diabetes Association hosts its annual scientific meeting. This meeting is the world’s largest and most important gathering focused on diabetes research, attracting over 10,000 attendees who come from across the world to hear the latest cutting edge research. This year, the University of Iowa was featured in a short video film shown at the meeting. The video focused on how the Fraternal Order of Eagles Diabetes Research Center (FOE-DRC) is advancing diabetes research . The FOE-DRC was created in 2008 when the Fraternal Order of Eagles pledged $25 million to establish a diabetes research center at the University of Iowa. With this gift, the FOE-DRC has grown to include over 100 faculty researchers from across the University. Collectively, these faculty conduct over $30 million of NIH-funded research annually. Several members of our Division are faculty in the FOE-DRC: Drs. Curtis, Larson Ode, Norris, Pinnaro, Tansey, and Tsalikian. Earlier this year, the American Diabetes Association requested that investigators at the University of Iowa help create a short video highlighting the work of the FOE-DRC. The video is now available on youtube (link to video here). The video highlights work by two members of our Division: Dr. Larson Ode and Dr. Norris, as well as several colleagues in the Division of (Adult) Metabolism and Diabetes and in the FOE-DRC.
We are pleased to report that 6 of the pediatric endocrinology physicians in our division have received Patient Choice Awards. These awards are given out by UI Health Care to recognize physicians for consistently providing patients with an excellent healthcare experience. The recipient physicians were:
- Lauren Kanner
- Katie Larson Ode
- Liuska Pesce
- Catherina Pinnaro
- Mike Tansey
- Eva Tsalikian
The Award was given to only 156 providers across the entire institution. The Award recognizes those who scored in the top 10% nationally in response to patient surveys asking whether the physician showed concern for patient questions or worries, gave explanations about problem or condition, made efforts to include the patient in care decisions, discussed proposed treatments (options, risks, benefits, etc), and whether they would be likely to recommend the care provider to others. Our division is fortunate to have these Award winning physicians on our team. We thank each of them for their wonderful work. Find more about the awards at this link.
Cystic fibrosis is an inherited disease that leads to progressive lung dysfunction. Persons with cystic fibrosis are also at high risk to develop diabetes. Unfortunately, cystic fibrosis plus diabetes is a dangerous combination, further worsening lung function and increasing risk of death. Recently, over the past decade, several new very effective medications for cystic fibrosis have been developed. Collective, these new medications are termed modulators. The modulators work by restoring function to the mutated proteins that cause cystic fibrosis. Thus, the specific modulator therapy used must be matched to the specific mutations that each person with cystic fibrosis has inherited. Although the modulators are very effective at improving lung function, their impact on diabetes risk for persons with cystic fibrosis is not yet clear. Dr. Larson Ode has co-authored a new, peer-reviewed article (PubMed link) summarizing current knowledge about how modulators might impact diabetes risk in persons with cystic fibrosis. The article highlights mechanisms and data suggesting that modulators might reduce risk of diabetes, but also notes potential mechanisms by which the modulators might increase diabetes risk.
We are proud to note that the University of Iowa Stead Family Children’s Hospital has maintained its recognition by the National Pancreas Foundation as a Center of Excellence for treatment of youth with pancreatitis. Pancreatitis is uncommon in children, but requires expert multidisciplinary care including specialists from gastroenterology, endocrinology, radiology, and surgery. Diabetes is a common complication of recurrent pancreatitis. Pancreatitis-related diabetes is not the same as type 1 or type 2 diabetes, and can have specific treatment considerations. As such, it requires expertise from an experienced pediatric endocrinologist, such as Dr. Katie Larson Ode, who is the lead pediatric endocrinologist who works with the pancreatitis clinic here. You can read more about the designation at the original press release here.
Congratulations to Dr. Katie Larson Ode, who was just named the recipient of a research grant award. The award comes from the joint Minnesota-Iowa Diabetes Research Center (MIDRC) as part of an initiative to foster collaborative diabetes research between the two institutions. For the research project, Dr. Larson Ode has teamed up with Univ of Minnesota physician Dr. Melena Bellin, whom is also a pediatric endocrinologist. A portion of children who develop chronic or recurrent acute pancreatitis will develop diabetes. However, the reasons for this are poorly understood. To better understand why, and hopefully delineate preventative strategies, Drs. Larson Ode and Bellin will enroll children with pancreatitis into a study in which glucose monitors and meal tests will be used to determine how well their pancreases are functioning to produce insulin and control blood sugar.
The National Institutes of Health and the Cystic Fibrosis Foundation held a 3-day workshop devoted to cystic fibrosis related diabetes from June 23-25 (workshop link). The workshop was attended by interested physicians, scientists, and affected families and persons, and also was open to the public. The purpose of the workshop was to discuss the current state of knowledge about this form of diabetes, and to help inform future research directions. Dr. Larson Ode and Dr. Norris from our division both spoke on their areas of related expertise, with talks entitled “Glycemic Abnormalities in Young Children” and “Innervation of the CF Pancreas” respectively. The University of Iowa was also represented by two other speakers, gene therapy expert John Engelhardt PhD and pediatric gastroenterologist Aliye Uc MD. Drs. Engelhardt and Norris were also part of the workshop planning committee, along with other experts from Children’s Hospital of Philadelphia and Boston Children’s Hospital.