Developing Formal Guidelines for the Diagnosis and Treatment of CVS in Adults

Update October 2018:
Clinical practice guidelines on the management of cyclic vomiting syndrome in adults.

A note about progress with the CVS guidelines for adults – The four articles below have been completed and submitted to the governing Council of the American Neurogastroenterology and Motility Society (ANMS) – the sponsoring body. There are to appear in a special supplement to the journal Neurogastroenterology and Motility. Hopefully this set of articles will be published in open access in early 2019. The submitted draft will be circulated within the ANMS Council for comment. Requests for edits may be submitted back to the developing committee. On completion of the final draft, the documents will be submitted to the publisher of the journal.

  1. Guidelines on Management of Cyclic Vomiting Syndrome in Adults by the American Neurogastroenterology and Motility Society and the Cyclic Vomiting Syndrome Association.
  2. Technical Review on Pharmacologic Agents Used as Therapy in the Management of CVS
  3. Role of cannabis use: Cyclic Vomiting Syndrome vs. Cannabinoid Hyperemesis Syndrome.
  4. Pathophysiology of CVS: Current knowledge, gaps in understanding, and future directions

Update July 2018
A “State of the Art” Compilation of Articles on CVS in Adults
Clinical practice guidelines on the management of
cyclic vomiting syndrome in adults.
Kathleen Adams – CVSA – project coordinator
With assistance from Thangam Venkatesan, MD project lead 8-17

Over the last nearly 3 years, you may have been following the progress of this landmark project for adult patients with CVS worldwide. American Neurogastroenterology and Motility Society (ANMS) with scientific sponsorship and CVSA with financial and logistical sponsorship have teamed up. The result will soon be available as a special supplement to the journal Neurogastroenerology and Motility. The contents of the supplement:

  1. Guidelines on Management of Cyclic Vomiting Syndrome in Adults
  2. Technical Review on Pharmacologic Agents Used as Therapy in the Management of CVS
  3. Role of cannabis use: Cyclic Vomiting Syndrome vs. Cannabinoid Hyperemesis Syndrome.
  4. Pathophysiology of CVS: Current knowledge, gaps in understanding, and future directions

This extremely important and useful set of documents is dedicated to honor David R. Fleisher, M.D. who has spent his entire career of over 50 years and still counting working passionately and tirelessly on behalf of patients with CVS. He will be honored in print within the document.

In 1991 when CVSA was founded and for a number of years thereafter, it was thought that CVS was a condition of childhood and adolescence. Now we know, all too well, that adults suffer from CVS as well. There is CVS that begins in childhood and may extend into adulthood and adult onset CVS. As so many adults can attest to, the condition causes untold suffering, disability, family, school, and career upheaval. Because CVSA is devoted to increasing awareness and quality of treatment of CVS, we, along with our medical advisory board, proposed this project – development of a standard of care for the diagnosis and treatment of CVS in adults. We are making good progress with this partnership and work that will have a ripple effect worldwide. This will make a difference in the lives of many – patients, families and medical professionals.

Background
After years of discussion, in March of 2002, it was decided that CVSA would partner with the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) to development and publish formal guidelines for the diagnosis and treatment of CVS in children and adolescents. Six years later the result was published in the Journal of Pediatric Gastroenterology and Nutrition. In spite of the fact that the document is now 10 years old, it is still widely referred to by medical professionals around the world. Discussions have started to work on a much-needed update of this document.

Li BUK [ed], Boles R, Chelimsky G, Issenman R, Lefevre F, Lewis D, Linder S, Nelson S, Rudolph C: North American Society for Pediatric Gastroenterology, Hepatology and Nutrition diagnosis and management of cyclic vomiting syndrome, a consensus statement. JPGN July 47 (1), 2008.

Yes, it took 6 years of steady medical committee work from beginning to when the following publication was ready to circulate. That gives you an idea of the level of detail, research, committee collaboration and time needed to publish it takes to produce a consensus statement or guideline. The current project is taking 3 ½ years – with the publication to be available for distribution in early 2019.

As with the pediatric guideline project, the need for adult guidelines has been awaited for too many years. When a variety of people and circumstances coalesced in the fall of 2014, Thangam Venkatesan, MD – CVSA Primary Advisor and Director of the Program for CVS in Adults at the Medical College of Wisconsin, started the ball rolling. She spoke several times with Benson Massey, MD – ANMS Councilman and faculty member of the Medical College of Wisconsin. Dr. Massey welcomed Dr. Venkatesan’s proposal for partnership. Approval followed by the ANMS Council in early 2015. Historically, CVS has been such a difficult illness to diagnose and treat, it was very clear that having management guided by a standard of care that would be published in a widely circulated and highly respected journal would be a great advantage for patients and families.

Members of CVSA Medical Advisory Board and Dr. Massey (ANMS council member) vetted and finalized committee members for this task after deliberating on individual expertise, unique talents, diversity and ability to work in groups. This was to ensure that we had a multidisciplinary, diverse group of individuals with unique areas of expertise in different fields such as emergency department protocols in CVS, migraine and development of clinical practice guidelines to name a few. For instance, Dr. Safwan Jaradeh from Stanford University is a neurologist with experience in management of CVS, dysautonomia and migraine. Dr. Robert Issenman from McMaster in Canada has developed emergency department protocols for CVS. This committee also includes members with expertise in the GRADE methodology and a librarian who has extensive experience and expertise in systematic reviews and guidelines development. Another unique feature of the composition of this committee, as the ANMS recognizes, is the active participation of a patient representative taking into consideration patient values and preferences in the management care.

Goals of the committee
The ANMS is providing oversight of the process and publication of the resulting paper. The committee of 13 physicians and PhDs was charged with the goal of developing clinical practice guidelines (CPG) that are intended to assist the medical community including gastroenterologists, primary care physicians and other health care personnel that are involved in the care of adult CVS patients. The methodology used in development is based on the GRADE (Grading of Recommendations Assessment, Development and Evaluation). The committee has developed a technical review to accompany the CPG, which will provide readers with details about the review process.

Aside from providing a framework for management, this guideline is also intended to identify knowledge gaps in CVS, which will provide an impetus for future multi-center research and federal funding.

We have worked together via frequent conference calls and 4 in-person meetings. Pittsburgh – October 2015, Denver – December 2017, Milwaukee – April 2017, Chicago – January 2018. There has been extensive literature search and analysis working up to the actual document writing. The committee is completing the writing this month. Article #3 has required extra time. Cannabinoid Hyperemesis Syndrome (CHS) has come to the attention of the medical and lay audience of late. Article 3 will inform the discussion on the role of cannabis in CVS/CHS which is very relevant to the diagnosis and management of CVS. We have been informed that there is a “clamoring for this supplement” by clinicians who are identifying CVS more frequently all the time. There is an obvious need for this set of articles to help define and manage CVS in the midst of unclear discussion about the relationship of CHS and CVS.

During the month of September the committee will be submitting the manuscripts to the ANMS Governing Council for initial review and comment. After the usual process of review and revision, the articles will be submitted for publication with hopes of open access circulation in early 2019. Open access publication allows free worldwide access via the internet to anyone. The cost for open access alone to CVSA is about $16,000, very well worth it as this is the only way to assure the document will be fully available.

Like the consensus statement for the diagnosis and treatment for CVS in children, this resulting journal supplement will provide an excellent resource for clinicians diagnosing and treating CVS in adults around the world. Because CVSA is the sole financial sponsor for this landmark project. We thank those of you who have made this possible with generous donations. Additional donations are needed as costs are ramping up for publication and circulation which is right around the corner.

Even though the diagnosis of CVS has been emerging out of the cracks in the floorboard of medicine for the last 2 1/2 decades, there is still much to do about the lingering dearth of information about CVS that is available to physicians, school nurses, psychologists and others that are asked to provide care. Having another formal standard of diagnosis and treatment will go a long way to ensure that professionals out in the field will be better equipped to care for patients who are still struggling with CVS. So we are wrapping up after forging ahead with dogged determination. Without the tireless work of each committee member, the hopes for an adult standard of care would be for naught. Gratitude for the work is endless.

Committee members
Chair:
Thangam Venkatesan, MD. adult GI, Medical College of Wisconsin; William Hasler, M.D., adult GI
University of Michigan; Robert Issenman, M.D., pediatric GI, McMaster’s University, Ontario, Canada; Safwan Jaradeh, M.D., adult neurology, Stanford University; David Levinthal M.D., Ph.D. adult GI, University of Pittsburg; B Li, M.D., pediatric GI, Medical College of Wisconsin; Benson Massey, M.D., adult GI, Medical College of Wisconsin; Irene Sarosiek, MD., adult GI, Texas Tech Health University ; Raj Shah, M.D. – GRADE methodology, University of Missouri, Kansas; Ravi Sharaf, M.D. , M.S., methodology – GI, North Shore Long Island Jewish Health System, New York; Shahnaz Sultan, M.D., M.S., methodology – GI, University of Minnesota; Sally Tarbell, Ph.D., psychology, Lurie Childrens Hospital, Chicago; Kathleen Adams, B.S., R.N., Cyclic Vomiting Syndrome Association

Update October 2017:
Once or twice a month conference calls have been held along with in-person meetings. Tireless work has been completed on the available literature search and evaluation of relevant articles. This process must be completed before the work of writing the document can commence. Yet to be published relevant articles are being considered as they become available.

The process used in evidence-based practice to frame and answer a clinical or health care related question and background questions have been completed. This framework is also used to develop literature search strategies. The first draft of the main manuscript incorporating background questions is being completed by the committee.

Because of an emerging decision by the publication board of the journal of choice for the final document, Neurogastroenterology and Motility, there may be good news coming of an agreement between the committee and the publisher that would expand the usefulness of this document beyond what we originally expected. Stay tuned for that news.

Again, like the consensus statement for the diagnosis and treatment for CVS in children, this resulting document will provide an excellent resource for clinicians diagnosing and treating CVS in adults around the world. We thank the financial donors, for this project, for their generosity making it all possible for the benefit of many. Also, the tireless efforts of all the committee members.

When CVSA  was founded in 1993, it was thought that CVS was a condition of childhood and adolescence. Now we know that adults also suffer from CVS. There is CVS that begins in childhood and may extend into adulthood and adult onset CVS. As so many adults can attest, the condition causes untold suffering and disability, and often leads to family, school, and career upheaval.

Doctor showing tablet to her patient in hospital room

With children’s guidelines already in place thanks to CVSA fundraising and our strong medical team, we are embarking on a partnership project that will help adult sufferers around the world. Partnering with the American Neurogastroenterology and Motility Society (ANMS) www.motilitysociety.org, a strong medical committee of ten medical professionals has been formed to develop guidelines for the diagnosis and treatment of CVS in adults. When completed, these guidelines will help doctors and ER personnel around the world understand how to diagnose and treat adult CVS patients more effectively.

Developing medical guidelines is a rigorous and expensive process, estimated to take two years. This is where you come in. CVSA is actively seeking financial sponsorship for this project through our membership and beyond. You can be part of this long overdue work of CVSA, the product of which will reach people still suffering in isolation with CVS. Please consider sending in a donation through our website.