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Gaining Better Understanding of Blood Sugars Problems Early in Cystic Fibrosis

Dr. Larson Ode

Almost 10 years ago, investigators from our Division determined that young kids with cystic fibrosis (CF), less than 5 years of age, often have high blood sugars in response to a standardized sugary drink. However, the long term importance of these findings is unknown. Furthermore, we don’t know if this issue occurs when young kids are eating their usual foods and drink. To address this shortcoming, Dr. Katie Larson Ode of our Division, has partnered with other researchers across the country to create a study using wearable continuous glucose monitors. In one part of the study, they are using these monitors to determine what blood sugars do in young kids with CF in their usual environment (home, school, etc). However, so little is known about what blood sugars do in healthy young kids that it is difficult to know exactly what is normal. To address this, Dr. Larson Ode and her research partners will also study young, healthy kids. Dr. Larson Ode has just received grant funding to conduct the study, entitled “BEGIN Substudy: Continuous Glucose Monitoring in Healthy Children“. We thank Dr. Larson Ode and her research volunteers for their work to help advance knowledge.

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Diminished Growth and Bone Density in Children with Pancreatitis.

Dr. Larson Ode

Potential endocrine problems are under studied in children with pancreatitis. Dr. Larson Ode from our division is part of a multicenter team that is investigating endocrine complications in children with pancreatitis (either chronic or acute recurrent). They have just published results from a study examining height and bone density in children with pancreatitis. Their manuscript is published in the journal Pancreatology (link to manuscript). The found an excess portion of children with pancreatitis had low height and/or low bone mineral density. These results indicate that children with pancreatitis need closer attention to their growth and to their bone health. It would be ideal for such children to be followed in a multidisciplinary clinic devoted to children with pancreatitis, such as the nationally recognized Pancreatitis Clinic at the University of Iowa Stead Family Children’s Hospital (link).

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Dr. Larson Ode Recognized as Ongoing National Leader in Cystic Fibrosis Endocrine Care

Dr. Larson Ode

Cystic fibrosis (CF) increases the risk of several endocrine complications, especially diabetes and bone weakening. The causes and treatments of these conditions are somewhat unique in CF, with some differences from the treatment of diabetes and weak bones in the general population. The Cystic Fibrosis Foundation (CFF) has recognized the need to train and mentor endocrine physicians to provide endocrine care for persons with CF. To help meet this need, the CFF has identified and supported nationally recognized mentor endocrinology physicians with CF-expertise. One of these leaders is Dr. Katie Larson Ode from our division. In recognition of her expertise and outstanding mentoring, the CFF has just renewed her role in this national effort with a grant entitled “EnVision CF III: Emerging Leaders in CF Endocrinology Chair”. Additionally, the CFF has awarded an ongoing research grant to Dr. Larson Ode, entitled “SPECTRUM and CF Endocrine and Diabetes DAta Repository (CEDAR)”. Congratulations to Dr. Larson Ode on these accomplishments and thanks to her for her efforts to help support the health of those with CF.

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Screening for Diabetes in Persons with Cystic Fibrosis: Current Situation on the Ground

Dr. Larson Ode

Many persons who have cystic fibrosis develop diabetes at some point. In its early mild stages, diabetes can have few symptoms. Despite the lack of symptoms, mild diabetes still can damage the eyes, kidneys, and nerves. Furthermore, in those with cystic fibrosis, diabetes can worsen lung disease and increase the risk of mortality. For these reasons, it is important to screen for diabetes in persons who have cystic fibrosis. To accomplish this, the recommendations are to perform an oral glucose tolerance test yearly in those over age 10 with cystic fibrosis. To assess how well this recommendation Is being carried out, a group of physicians, including Dr. Larson Ode from our division, surveyed medical centers. Unfortunately, less than half of centers were screening at least half of persons with cystic fibrosis. These results indicate that further progress is needed in screening persons with cystic fibrosis for diabetes. These results are published in the journal Frontiers in Endocrinology and can be found at the following links (pubmed & doi).

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Setting Research Priorities to Better Understand, Treat, and Prevent Cystic Fibrosis Related Diabetes

  • Dr. Norris
  • Dr. Larson Ode

Potent medications have recently been developed to treat cystic fibrosis. These new therapies dramatically improve lung disease for those with cystic fibrosis. Persons with cystic fibrosis often develop a unique form of diabetes termed cystic fibrosis related diabetes (CFRD). It is not yet clear how the new therapies will impact the propensity of persons with cystic fibrosis to develop CFRD. A group of researchers, clinical experts, and patients/families were assembled by the NIH and the Cystic Fibrosis Foundation to discuss research priorities to better understand treat and prevent CFRD. This group convened in June of 2021 at a workshop open to the public. This group has now written and published a summary describing their collective thoughts on research priorities priorities CFRD, Their writings appear this month in the two journals Diabetes and Diabetes Care. Contributing to the article were two physicians from our division: Dr. Norris and Dr. Larson Ode, with Dr. Norris serving as one of the lead authors of the work. The publication can be found at the following Pubmed link.

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Impact of Highly Effective Modulator Therapy for Cystic Fibrosis on Body Composition

Dr. Larson Ode

The triple combination therapy consisting of elexacaftor/tezacaftor/ivacaftor (ETI) has made a tremendous impact for the health of persons with one of the most serious and most common genetic forms of cystic fibrosis. This medication dramatically improves lung health and helps persons with CF live a longer and healthier life. These medications also help persons with CF regain weight, which is important for health because being underweight in those with CF is associated with a higher risk of worsening lung disease and mortality. However, what is not yet known is whether the weight gain with ETI therapy is healthy weight gain such as muscle mass or less health gain such as excess adipose tissue. To address this knowledge gap, Dr. Katie Larson Ode from our division teamed with a group of investigators at several other hospitals across the country to study this in persons on ETI therapy. They found, perhaps concerningly, that the weight gain occurred mainly as increased fat mass and that the changes were associated with reduced insulin sensitivity. These results indicate a need to closely monitor and further study the metabolic impact of ETI therapy. Their study has been published in the journal Pediatric Pulmonology and is titled “The Impact of elexacaftor/tezacaftor/ivacaftor on Body Composition in a Small Cohort of Youth with Cystic Fibrosis”. It can be found at this pubmed link.

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Patient Choice Award Recipients

We are pleased to report that 6 of the pediatric endocrinology providers 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
  • Julie Osterhaus
  • Liuska Pesce
  • Catherina Pinnaro
  • Mike Tansey

The Award was given to only 174 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.

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New Practice Guidelines for Care of Cystic Fibrosis-Related Diabetes

Dr. Larson Ode

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.

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Highly Effective Modulator Therapy for Cystic Fibrosis Impacts Body Mass Index and Insulin Sensitivity

  • Dr. Larson Ode
  • Dr. Norris

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.

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Identifying Gaps in Reproductive Counseling for Persons with Cystic Fibrosis

Dr. Larson Ode

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.