Addressing D.R.E.S.S. Podcast

For 16-year-old Izzy McKinney, a course of treatment with the antibiotic Bactrim first led to fevers and a bad rash.

But that was just the beginning.

What no one realized was that Izzy was suffering from a severe case of D.R.E.S.S: Drug Reaction with Eosinophilia and Systemic Symptoms.

This introductory episode tells the tragic story of how DRESS syndrome took Izzy's life, leaving behind a devastated family. 

It is a cautionary tale that illustrates the importance of understanding and making a prompt diagnosis of this potentially fatal condition.

Interviewed: Tasha Tolliver, Izzy’s Mother, (President, Executive Director the DRESS Syndrome Foundation), July,  2019 in Richmond, VA.

Making Drugs Safer: Izzy’s Story – In-Depth Physician Interview: https://www.ivanhoe.com/interview/making-drugs-safer-izzys-story-depth-doctor-interview/
https://www.ksat.com/health/2017/07/14/making-drugs-safer-izzys-story/
Medical Paper Highlighting Izzy's Case -- HHV-6 encoded small non-coding RNAs define an intermediate and early stage in viral reactivation: https://www.nature.com/articles/s41525-018-0064-5
The DRESS Syndrome Foundation:
https://www.dresssyndromefoundation.org

Medication-induced drug disorders have been evident for nearly a century and labeled with a variety of names. French dermatologist Dr. Vincent Descamps, who has been at the forefront of DRESS syndrome since the mid-1990s, joins us to review this historically misunderstood and under-reported illness. We'll also discuss the history of how an international team of researchers and physicians identified the clinical condition known as DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms). 

Interview: Dr. Vincent Descamps (Professor of Dermatology at the Bichat Claude Bernard Hospital, University Denis Diderot in Paris, France) June, 2019 in Quebec, Canada.

PUBLICATIONS/RESOURCES:

DRESS syndrome: https://docs.wixstatic.com/ugd/cac731_643993b7381f4c85b7d4b62dce42087b.pdf
Diagnosis of DRESS (Drug Reaction With Eosinophilia and Systemic Symptoms) in the Intensive Care Unit: Essential But Challenging: https://journals.lww.com/shockjournal/Fulltext/2013/11000/Diagnosis_of_DRESS__Drug_Reaction_With.14.aspx
French dermatologists call for antiviral therapy in DRESS: https://hhv-6foundation.org/drug-hypersensitivity/french-dermatologists-call-for-antiviral-therapy-in-dress
Saliva polymerase chain reaction assay for detection and follow-up of herpesvirus reactivation in patients with drug reaction with eosinophilia and systemic symptoms (DRESS): https://pubmed.ncbi.nlm.nih.gov/23426332/
Drug rash with eosinophilia and systemic symptoms (DRESS) in patients receiving strontium ranelatez: https://pubmed.ncbi.nlm.nih.gov/23361875/
Human herpesvirus 6 involvement in paediatric drug hypersensitivity syndrome: https://onlinelibrary.wiley.com/doi/abs/10.1111/bjd.13592
The DRESS Syndrome: A Literature Review: https://www.amjmed.com/article/S0002-9343(11)00258-0/fulltext
Chapter 11 - HHV-6A and HHV-6B in Drug-Induced Hypersensitivity Syndrome/Drug Reaction with Eosinophilia and Systemic Symptoms: https://www.sciencedirect.com/science/article/pii/B9780444627032000112
Service de Dermatologie/Hôpital Bichat - Claude-Bernard: https://www.aphp.fr/service/service-16-011
Chronic persistent HHV‐6B infection after sulfasalazine‐induced DRESS with demonstration of HHV‐6 encoded small noncoding RNAs (sncRNAs) in Crohn’s‐like colitis: Case report: https://onlinelibrary.wiley.com/doi/10.1002/ccr3.3680#.X90Agxjv1sI.twitter

Hannah's mother and long-time DRESS advocate, Nancy Szakacsy tells of her daughter's struggle against a fatal case of DRESS syndrome that was triggered by the antibiotic Minocycline.

The devastation of losing Hannah, and the obvious lack of information and resources for families like hers inspired Nancy to write a book about her daughter's illness. She now leads what has become the largest network of support for those affected by DRESS, and is currently Vice-President of the DRESS Syndrome Foundation where her tireless work continues.

Interview: Nancy Szakacsy, Hannah’s mother, (Author, “Hannah Was Here. D.R.E.S.S an Alarm That Must Be Heard” and Vice President of the DRESS Syndrome Foundation). August 2019, in Bethesda, MD

RESOURCES:

Facebook Support Group: https://www.facebook.com/D.R.E.S.S.SyndromeCommunity
Hannah Was Here: D.R.E.S.S. an alarm that must be heard: https://www.amazon.com/Hannah-Was-Here-D-R-S-S/dp/1496923138
DRESS Syndrome Foundation: https://www.dresssyndromefoundation.org

International severe adverse drug reaction experts, Dr. David Ostrov and Dr. Vincent Descamps, talk to us about the immune response that occurs in DRESS syndrome as well as how antiviral and steroid therapies are often indicated in fighting this condition.

We also hear from Kent Van Donge about the importance of a team approach in the treatment of DRESS.

Interview: Dr.David A.Ostrov (Associate Professor of Experimental Pathology/University of Florida), Dr. Vincent Descamps (Professor of Dermatology at Bichat Claude Bernard Hospital), Kent Van Donge (Intern/Student and EMT) at the HHV-6 Foundation 2019 Conference in Quebec, Canada.

PUBLICATIONS/RESOURCES:

Dr. David Ostrov:

David A. Ostrov Laboratory: https://ostrovlab.pathology.ufl.edu/about-dr-ostrov/
The structural basis of HLA-associated drug hypersensitivity syndromes: https://onlinelibrary.wiley.com/doi/10.1002/ccr3.3680#.X90Agxjv1sI.twitter
Drug hypersensitivity caused by alteration of the MHC-presented self-peptide repertoire:https://www.pnas.org/content/109/25/9959.long
Full List of Publications: https://ostrovlab.pathology.ufl.edu/about-dr-ostrov/select-publications/full-list-of-publications/

Dr. Vincent Descamps:

DRESS syndrome:https://docs.wixstatic.com/ugd/cac731_643993b7381f4c85b7d4b62dce42087b.pdf
Diagnosis of DRESS (Drug Reaction With Eosinophilia and Systemic Symptoms) in the Intensive Care Unit: Essential But Challenging: https://journals.lww.com/shockjournal/Fulltext/2013/11000/Diagnosis_of_DRESS__Drug_Reaction_With.14.aspx
French dermatologists call for antiviral therapy in DRESS:https://hhv-6foundation.org/drug-hypersensitivity/french-dermatologists-call-for-antiviral-therapy-in-dress
Saliva polymerase chain reaction assay for detection and follow-up of herpesvirus reactivation in patients with drug reaction with eosinophilia and systemic symptoms (DRESS): https://pubmed.ncbi.nlm.nih.gov/23426332/
Drug rash with eosinophilia and systemic symptoms (DRESS) in patients receiving strontium ranelatez: https://pubmed.ncbi.nlm.nih.gov/23361875/
Human herpesvirus 6 involvement in paediatric drug hypersensitivity syndrome: https://onlinelibrary.wiley.com/doi/abs/10.1111/bjd.13592
The DRESS Syndrome: A Literature Review: https://www.amjmed.com/article/S0002-9343(11)00258-0/fulltext
Service de Dermatologie/Hôpital Bichat - Claude-Bernard:https://www.aphp.fr/service/service-16-011
Chronic persistent HHV‐6B infection after sulfasalazine‐induced DRESS with demonstration of HHV‐6 encoded small noncoding RNAs (sncRNAs) in Crohn’s-like colitis. Case report:
https://onlinelibrary.wiley.com/doi/full/10.1002/ccr3.3680?af=R

In this episode, we talk with world-renowned DRESS Syndrome expert, Dr. Elizabeth Phillips. She and her colleagues at Vanderbilt University Medical Center are working to better understand the genetic factors that increase a patient's risk for developing  life-threatening, immune-mediated drug reactions - with a focus on simple, inexpensive diagnostic tests can save countless lives.

Interview: Dr. Elizabeth J. Phillips (Professor of Medicine, Pharmacology, Pathology, Microbiology and Immunology and is Director of Personalized Immunology at the Oates Institute for Experimental Therapeutics at Vanderbilt University Medical Center. Lead Scientific Advisor for DRESS Syndrome Foundation) August, 2019 in Bethesda, MD

PUBLICATIONS/RESOURCES:

Vanderbilt Institute for Infection, Immunology and Inflammation: https://www.vumc.org/viiii/person/elizabeth-j-phillips-md
HLA-A*32:01 is strongly associated with vancomycin-induced drug reaction with eosinophilia and systemic symptoms: https://www.sciencedirect.com/science/article/pii/S0091674919302106
A Rapid Allele-Specific Assay for HLA-A*32:01 to Identify Patients at Risk for Vancomycin-Induced Drug Reaction with Eosinophilia and Systemic Symptoms: https://pubmed.ncbi.nlm.nih.gov/31158526/
Severe delayed cutaneous and systemic reactions to drugs: a global perspective on the science and art of current practice: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5424615/
Applications of Immunopharmacogenomics: Predicting, Preventing, and Understanding Immune-Mediated Adverse Drug Reactions: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6409210/
Successful translation of pharmacogenetics into the clinic: the abacavir example: https://pubmed.ncbi.nlm.nih.gov/19351209/
HLA and pharmacogenetics of drug hypersensitivity: https://pubmed.ncbi.nlm.nih.gov/22920398/
Fever, rash, and systemic symptoms: understanding the role of virus and HLA in severe cutaneous drug allergy:  https://pubmed.ncbi.nlm.nih.gov/24565765/
Recent advances in the understanding of severe cutaneous adverse reactions: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5582023/
Penicillin Allergy:
https://www.nejm.org/doi/full/10.1056/NEJMra1807761

Theresa Stickney shares the story of her long and complicated course of illness with DRESS Syndrome, triggered by the drug Vancomycin. 

Since recovering, she has been inspired to share what she knows, both as a nurse and a patient - helping other patients, caregivers, doctors and researchers better understand this often misdiagnosed and difficult to treat condition.

Interview: Theresa McDonnell Stickney (Mother, Registered Nurse, DRESS patient and advocate), August, 2019 in Bethesda, MD 

From opposite ends of the U.S., two mothers share a common bond. They both know what it’s like to sit at a child’s hospital bedside, desperately searching the world over for the medical information that could save their daughter’s life.

When the resources they needed weren’t available, they took matters into their own hands to create them.

While working through their grief, these mothers have bonded in their mission to create better awareness of DRESS syndrome and support others who suffer from it –beginning  with a foundation, social media, and public outreach projects.

Interview: Tasha Tolliver (President/Executive Director the DRESS Syndrome Foundation),Nancy Szakacsy, (Vice President the DRESS Syndrome Foundation), August 2019, in Bethesda, MD

RESOURCES:

DRESS Syndrome Foundation: http://www.dresssyndromefoundation.org
https://www.facebook.com/DRESSSyndromeFoundation
Facebook Support Group: https://www.facebook.com/D.R.E.S.S.SyndromeCommunity

Life-threatening drug reactions often present with a rash, so dermatologists may be the first line of defense for treatment.  Dr. Arash Mostaghimi, a dermatologist at Harvard Medical School’s Brigham & Women’s Hospital, draws on his experience with DRESS patients in teaching medical students about this complex and often misunderstood condition. 

Interview: Dr. Arash Mostaghimi (Assistant professor of Dermatology, Director of Inpatient Consultation Services, co-director of the  Complex Medical Dermatology Fellowship at Brigham & Women’s Hospital) March, 2019 in Boston, MA

RESOURCES:

Harvard University/Arash Mostaghimi: https://scholar.harvard.edu/mostaghimi/home
Topical Podcast: https://topicalpod.libsyn.com
Inpatient Dermatology Best Practice Strategies for Educating and Relaying Findings to Colleagues:
https://pubmed.ncbi.nlm.nih.gov/33133770/
Published Articles: https://scholar.google.com/citations?user=0VaCfdkAAAAJ&hl=en

When Linda Wondrack was diagnosed with rheumatoid disease in 2015, she never imagined that her treatment with Sulfasalazine would end up leaving her even more severely ill. In the wake of a complicated recovery from DRESS, she shares the physical and psychological toll this delayed drug reaction can take... and the importance of self-advocacy and self-care.

Linda's Website: Surviving DRESS
https://www.survivingdress.org

Viruses from previous infection may stay dormant in our bodies for years - sleeping away the time, never causing any trouble. However, in immunosuppressed conditions like DRESS, the virus may awaken and wreak havoc. Viral reactivation of  human herpesviruses, in particular HHV-6, are a known phenomenon of DRESS, occurring in up to 50% of patients. While the exact role of HHV-6 reactivation in DRESS has been a controversial topic, many experts believe that it is not taken seriously enough.

Co-founder, president and executive director of the HHV-6 Foundation, Kristin Loomis discusses the importance of identifying HHV-6 viral reactivation  in DRESS syndrome and the existing roadblocks to treating infected patients with antiviral medication. 

Interview: Kristin Loomis (Founder and Executive Director of the HHV-6 Foundation), June, 2019 in Quebec, Canada

https://hhv-6foundation.org
https://hhv-6foundation.org/category/drug-hypersensitivity