Lessons from the MH Hotline

Lessons from the MH Hotline by MHAUS President Henry Rosenberg, MD

I would like to wish all of our friends and supporters a happy and joyous holiday season. Although there are numerous and very threatening problems in the world today, we also have a lot to be thankful for as well. Not the least of which is the astounding progress that has been made in medical care in recent years and the accelerating pace of applying lessons from the laboratory to care of the patient. As mentioned previously, 2012 marks the thirtieth anniversary of the MH hotline.

The MH Hotline is one of the signature services of MHAUS. The hotline provides expert advice when and where it is needed. I have previously discussed the origins of the hotline and how it functions. In this blog I would like to share with you some of comments from the consultants in response to my request that they share some of the things that they have learned from the hotline and general comments about their service on the hotline.

I would also like to also acknowledge MH Hotline Consultant Dr. Sheila Muldoon who is retiring from Uniformed Services University.

The consultants have provided many and varied comments, here are some that I think are especially significant:

• Virtually every anesthesiologist, other physicians, CRNAs and anesthesia residents aiding in the management of a real MH crisis were extremely grateful for the assistance from the hotline.
• Early in my hotline tenure, most anesthesiologists knew little about MH. This has changed over time and by the end of my tenure with the hotline, most seemed to have a more than adequate understanding of MH and, at least, its initial treatment.
• Many clinical problems in anesthesia thought to be MH to those not familiar with the syndrome are really not MH.
• The range of presentations of MH, even if one considers only the OR presentations, is very wide.
• I learn something from almost every hotline call.
• Callers are mostly unaware of the molecular genetics of MH.
• The hotline kept me in touch with MH and all the new findings and stay up-to-date with ongoing information related to MH.
• It encouraged me to do research
• It has encouraged me to bring awareness of MH and promote the availability of dantrolene to India.
• MHAUS is a great organization; great support staff and volunteers that include professionals and non-professionals.
• Each event is unique.
• From one of the first members of the hotline: It was evident that anesthesia providers were crying for assistance. Here's an impossible situation in a patient that resembles nothing in my education or practice and no one can help. In those times (early 1980s) MH experts didn't exist and those in their early development were hampered by stories of sudden death not explained by usual rational approaches.
• Dantrolene is a great drug to reverse hyperthermia. I have not encountered significant complications from its use.
• The presentations of MH are often complex and influenced by variables we don't understand.
• MH or MH like signs can occur without anesthesia in some cases.
• Temperature monitoring should be done in all general anesthetic cases.
• Episodes of muscle breakdown can be "missed" clinically and present later with renal failure.
• Sometimes it seems that having a label is more important than knowing if it's the correct label.
• Clinicians often have trouble telling patients and their families that things can go wrong with grave consequences.
• There are many astute anesthesiologists who just want to make sure they have done the right thing in treating an MH episode.
• I like to make a follow up call on all cases just to make sure that all has gone well.
• There is a large range of variability in knowledge about MH. Some providers just call for confirmation that they are doing the right thing, others need more support. Especially for these latter calls, the hotline provides critical support
• It is a privilege to share my knowledge and expertise with other clinicians.

I will add that the Board and staff of MHAUS is extremely grateful and impressed by the knowledge and dedication of the hotline consultants to not only give advice, but to learn from others about MH and how best to recognize and treat the condition.

The MH hotline has been a unique and effective way for the medical community to learn about a condition that an individual provider is likely to see only a few times in his/her career.

Now I would like to turn to a different subject.

This past October, Dr. Sheila Muldoon Director of the MH Diagnostic Laboratory at the Uniformed Services University and Board member of MHAUS for many years, officially retired from her position on the faculty of the Uniformed Services University of the Health Sciences. Although she is no longer employed by the University, this does not mean that she will not continue to make contributions to the understanding and management of Malignant Hyperthermia.

The community of patients and clinicians and scientists interested in the problems of MH owe Sheila a huge debt of gratitude for her work on MH for more than 25 years. She and her colleagues at Uniformed Services have contributed enormously to our understanding of the molecular genetics of MH. She has guided, supported and trained many clinicians and scientists who themselves have made contributions to medicine and to MH. She has been on the Board of MHAUS for more than 20 years, has served as the Vice President for Scientific Affairs for most of that time and also served on the MH Hotline for 20 years. She has been a strong supporter of the activities and goals of the North American MH Registry and helped guide many of the important studies that have emanated from the Registry.

The laboratory for the study of MH at Uniformed Services has not only sponsored investigations in the molecular genetics of MH and described the relationship between unusual clinical presentations of the disorder and the pathophysiology of the disorder, but the center has been performing muscle biopsy diagnostic testing for MH for those in the military since the early 1980s. The laboratory she established has been one of only a few in North America that worked on perfecting the muscle biopsy diagnostic test, performed original research in animals who are MH susceptible, explored the molecular genetics of MH, as well as the cellular biochemistry of the disorder. In addition she has authored close to 100 original publications in the peer reviewed literature and has organized educational symposia and has herself lectured widely on MH.

Dr. Muldoon, however did not start her career with MH in mind. Rather, her first interests were in the physiology of vascular smooth muscle. It was only after she moved from Mayo Clinic in Rochester, Minnesota where she was performing clinical work as well as conducting research in smooth muscle, to the Uniformed Services University in 1977 that her interest in MH developed. In part, this was because the military was concerned about the implications of the diagnosis of MH in military personnel. In 1987 she was appointed Chairperson of Anesthesiology at the University. Despite clinical and administrative activities, in addition to her research in MH, she continued her active research in the pharmacology and physiology of smooth muscle. I played a part in her introduction to MH diagnostic testing when she visited my laboratory at the Hospital of the University of Pennsylvania, and later at Hahnemann University, to become familiar with the contracture test. We became fast friends and colleagues since that time. Later, in order to understand genetic testing, she spent several months in Wurzburg, Germany learning the techniques of working with DNA. A steady stream of original investigations demonstrating the relationship between molecular changes in MH susceptible persons and clinical manifestations of the disorder followed.

Dr. Muldoon established a cadre of excellent clinical and laboratory scientists at Uniformed Services to work with her on the problems of MH at every level. I hesitate to list them all for fear of omitting some of the names. Many were military anesthesiologists who worked with her for a few years and then moved on to other posts. A few however, have been collaborators for many years, such as Drs. Said Bina and Nyamkishig Sambuughin. One of her trainees who also has worked for many years on the problem of MH, Dr. John Capacchione has now taken over the leadership of the MH laboratory. In the early 1980's she established a collaboration with the Anesthesiology Department at the Children's National Medical Center (CNMC ) in Washington D.C. that offered diagnostic MH testing to non -military families in the Washington area. This collaboration continues to this day under the direction of Dr. Richard Kaplan and Dr. John Capacchione. Dr. Muldoon stepped down from the Chairmanship position in 2001, but continued to devote her time to the problems of MH and to support the North American MH Registry and MHAUS.

In recognition of her contributions to the Uniformed Services University and to the promotion of excellence in medical education and research, Dr. Muldoon was awarded the prestigious Carol J. Johns medal by the University.