EDS ECHO Summit: Fatigue Virtual Summit, October 21, 2023

Debilitating, chronic fatigue is common among people with hypermobility spectrum disorder (HSD) and Ehlers-Danlos syndrome (EDS) types. To tackle fatigue, it is important to recognize the role of persistent inflammation from conditions that travel with HSD or EDS, including mast cell activation (MCA) disease. Breathing difficulties including rhinosinusitis and asthma, some neuropsychiatric syndromes, hypersensitivity gastroenteritis, and bladder pain syndromes are frequently under-diagnosed and consequently under-treated in people with HSD and EDS.

These unchecked MCA disorders perpetuate unnecessary immune activation, which interferes with the normal function of affected organs and leads to diminished resources - nutrition, sleep, and hydration, e.g. First steps to treating EDS and HSD-associated fatigue is to screen for the presence of MCAD, with validated questionnaires and appropriate testing. After ruling out syndromes that mimic MCAD, such as POTS and Hereditary Angioedema, it is important to recognize that mast cells are not "one-trick ponies".

There is a spectrum of mast cell activation events, including MCA disorders that may not involve degranulation events or release histamine. Therefore, medications such as ketotifen, cromolyn, or histamine blockers will offer little or no relief, respectively. Moreover, it is important to (1) apply solid diagnostic criteria when considering the diagnosis of MCAD; (2) define the triggers/drivers of aberrant or excessive mast cell activation (MCAD phenotypes); and, (3) consider unrelated, overlooked disorders that can masquerade as MCAD.

In people with fatigue, these efforts will prevent delays to appropriate care, avoid unnecessary procedures, reduce the risk of adverse reactions to medications, and prevent waste of the affected person’s time and money.

Anne L. Maitland, MD, PhD is:

• an Assistant Professor in the Department of Medicine, Allergy and Clinical Immunology at the Icahn School of Medicine at Mount Sinai, in New York
• Director of Allergy/Immunology Services at Metrodora Institute in Salt Lake City, UT
• Consultant, Clinical Paradigms Dr. Maitland serves on committees, addressing mast cell activation disease (MCAD), Health Care Disparities, and Integrative Medicine of the American Academy of Allergy, Asthma, and Immunology. She also serves on the scientific faculty for the Mast Cell Disease Society, the Ehlers-Danlos International Consortium, and the Chiari-Syringomyelia Foundation.

She is:

• a Fellow of the American College of Allergy, Asthma and Immunology
• a member of the American Academy of Allergy, Asthma and Immunology
• a Vice Chair of the Allergy/Immunology Work Group of the National Medical Association.

Her clinical and research efforts focus on increasing access to Allergy/Immunology Specialty care and diagnosing and managing immune-mediated disorders, including MCAD. Recent publications highlight immune dysfunction in patients with Ehlers-Danlos syndrome and dysautonomia, including chapters in the book, Transforming Ehlers-Danlos syndrome, and the recent publication, from the National Academy of Sciences Engineering, and Medicine, entitled Selected Heritable Disorders of Tissue and Disability.

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