Fostering Disease-Specific Expertise Outside of Pulmonology: The CF/GI Combined Clinic

Columbus, OH — May 2018

The Case for a Cystic Fibrosis and Gastroenterology Combined Clinic

The majority of patients with cystic fibrosis experience gastrointestinal (GI) problems during their lifetime, a common complication of the buildup of thick mucus associated with the disease. The management of GI manifestations in cystic fibrosis is further complicated by the fact that the children are chronically ill and take multiple medications, sometimes prescribed by specialists in several disciplines.

As part of the CF Program at Nationwide Children’s Hospital, pulmonologists and gastroenterologists have teamed up to address these issues with patients in a single visit, with on-the-spot consultation and treatment plans created by the joint clinician team. Patients also have access to registered dieticians familiar with the unique GI complications many patients experience. Many of the pediatric CF patients — and now young adults, as well — seen at Nationwide Children’s receive care in this CF/GI Combined Clinic, for a range of GI complications.

As patients with CF live longer, an increasing number of specialists beyond pulmonologists will be managing GI and other conditions in this population. The combined clinic’s multidisciplinary team offers as much guidance and training as possible to other specialists and fellows so that they will know how to care for patients with CF as they age and develop other health needs.

Supported by the Cystic Fibrosis Foundation

Recognizing the importance of specialized GI care for patients with CF, the Cystic Fibrosis Foundation awarded a $60,000 grant to Ala Shaikhkhalil, MD, attending gastroenterologist in the combined clinic, to support her time in the clinic and further develop her expertise in the unique GI needs of CF patients. As part of this grant, Dr. Shaikhkhalil attends joint weekly meetings with the faculty from the general CF Program at Nationwide Children’s to discuss recent and upcoming cases. She is able to provide input on patient care so that the team can collectively make informed decisions on the best treatment options for all patients.

Dr. Shaikhkhalil’s grant is funded via the CF Foundation’s DIGEST program: Developing Innovative Gastroenterology Specialist Training. She is currently investigating ways to improve nutrient absorption in patients during nighttime tube feedings as part of her grant-related research initiatives.

Common GI Complications in Patients with CF

Historically, CF was considered a disease of malnutrition and the pancreas. As survival improved and the disease’s tie to lung function became apparent, pediatric pulmonologists absorbed the bulk of patient care. Now, physicians must learn to identify and manage a wide range of GI problems common in this population throughout an expanding lifespan.

In some cases, patients with CF are accustomed to the way they feel and do not mention GI symptoms unless they are asked about them directly. The types of GI complications affecting patients include common issues implicated with the disease process, side effects from their multiple CF medications, GI conditions totally unrelated to their CF diagnosis and a combination of the above. This makes the diagnostic and therapeutic approach challenging.

The CF/GI Combined Clinic team sees patients with any GI concern, including patients with severe complications or health problems requiring multiple follow-up visits.

Conditions evaluated include:

  • Abdominal pain
  • Constipation
  • Delayed gastric emptying
  • Distal intestinal obstruction syndrome (DIOS)
  • Functional GI disorders
  • Gallstones
  • Gastroesophageal reflux disease
  • Liver disease
  • Loss of appetite
  • Malnutrition
  • Meconium ileus
  • Medication side effects
  • Nausea
  • Pancreatic insufficiency
  • Rectal prolapse
  • Small bowel bacterial overgrowth

Management of Complex Cases

In some patients, the multiple medications required to treat the primary disease and its symptoms may cause side effects in the GI tract. In addition, medication adherence problems and anxiety about the medications or disease (especially among teens and young adults with CF) can make it challenging to identify the true source of GI issues. In the combined clinic at Nationwide Children’s, diagnosis and management of the GI disturbance is conducted in a manner that is sensitive to the patient’s disease, psychological wellbeing and potential for medication interactions.

CF patients may also experience delayed complications from meconium ileus surgeries performed as infants. These may present several years after the operation, making symptoms difficult to properly attribute. At Nationwide Children’s, patients with this complication are seen by both pulmonary and GI specialists to optimize lung function and maintain overall health prior to and following surgery to correct the concern.

Patients who require complex care for other severe or unique conditions have access to the full breadth of GI, nutrition, transplant and other services at Nationwide Children’s to ensure their needs are met.

A CF Program With the Power of 12 Disciplines

High quality, comprehensive CF care involves more than just pulmonary and GI physicians. In the CF Program at Nationwide Children’s, patients can receive clinical care from experts in a dozen specialties, and have access to many more when needed.

CF clinic days include shared sessions with:

  • Gastroenterology
  • Endocrinology
  • Ear, Nose and Throat
  • Respiratory Therapy
  • Physical Therapy
  • Pharmacy
  • Nurse Specialists
  • Social Work
  • Psychology
  • Child Life
  • Diet and Nutrition

Dr. Shaikhkhalil also networks with adult GI services at The Ohio State University Wexner Medical Center to bring them on board for CF patient care as appropriate. By leveraging shared clinic times, multiple specialists can discuss patient needs, medication and management together to come up with a well-rounded treatment plan before the patient leaves the clinic. This streamlines care for patients, reduces the number of visits required and optimizes multidisciplinary collaboration to help improve the care experience and clinical outcomes. In addition, it allows individuals from each specialty to learn more about the needs of CF patients — a crucial undertaking as patient survival improves.

References:

  1. Katkin JP, Baker RD, Baker SS. Cystic fibrosis: Assessment and management of pancreatic insufficiency. UpToDate, 2017 Jan 04. https://www.uptodate.com/contents/cystic-fibrosis-assessment-and-management-of-pancreatic-insufficiency.
  2. Van der Doef HPJ et al. Intestinal Obstruction Syndromes in Cystic Fibrosis: Meconium Ileus, Distal Intestinal Obstruction Syndrome, and Constipation. Curr Gastroenterol Rep. 2011 Jun; 13(3): 265–270.
  3. Sabharwal S, Borowitz D. Cystic fibrosis: Overview of gastrointestinal disease. UpToDate, 2016 Nov 06. https://www.uptodate.com/contents/cystic-fibrosis-overview-of-gastrointestinal-disease.