Laryngological Complaint Prevalence in Hypermobile Ehlers-Danlos or Hypermobility Spectrum Disorders.

OBJECTIVE: The aim was to study laryngological complaints in patients with hypermobile Ehlers-Danlos syndrome (hEDS) or hypermobility spectrum disorders (HSD). METHODS: A total of 363 patients met inclusion for the study by completing questions related to voice, upper airway, and swallowing between July 7, 2020 and July 13, 2022. Demographic data, voice-related questions, and hypermobility diagnosis were analyzed retrospectively. From those, 289 patients were diagnosed with hEDS or HSD with 74 that did not meet the diagnostic criteria for either diagnosis serving as controls. RESULTS: There were no statistically significant differences between patients with hEDS and HSD regarding Voice Handicap Index (VHI-10) scores, voice, upper airway, or swallow complaints. However, more hEDS/HSD patients answered positively to the laryngeal dysfunction question versus controls (p = 0.031). 22.5% of hEDS/HSD patients (n = 65) reported hoarseness, of which 52.3% reported hoarseness >2 days/month. 33.9% (n = 98) with hEDS/HSD reported symptoms of dysphagia, and 27.0% (n = 78) reported laryngeal dysfunction symptoms. Controls demonstrated 20.3% prevalence of hoarseness, of which 46.7% reported hoarseness >2 days/month. 24.3% of controls had dysphagia and 14.9% laryngeal dysfunction symptoms. Of the 363 patients, VHI-10 scores >11 were more likely in patients reporting >2 days of hoarseness/month (p = 0.001) versus those with <2 days of hoarseness/month. There was an increased prevalence of voice, upper airway, and dysphagia symptoms in hEDS/HSD patients compared with previously reported prevalence data in the general population. CONCLUSION: A significant proportion of patients diagnosed with hypermobility due to hEDS or HSD were found to have voice, upper airway, and dysphagia symptoms. These rates are higher than those previously reported in the general population. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:773-778, 2024.

High overlap in patients diagnosed with hypermobile Ehlers-Danlos syndrome or hypermobile spectrum disorders with fibromyalgia and 40 self-reported symptoms and comorbidities.

Background: Joint pain is a common symptom in patients with hypermobile Ehlers-Danlos Syndrome (hEDS), hypermobility spectrum disorders (HSD) and fibromyalgia. The goal of this study was to determine whether symptoms and comorbidities overlap in patients diagnosed with hEDS/HSD and/or fibromyalgia. Methods: We retrospectively examined self-reported data from an EDS Clinic intake questionnaire in patients diagnosed with hEDS/HSD, fibromyalgia, or both vs. controls with an emphasis on joint issues. Results: From 733 patients seen at the EDS Clinic, 56.5% (n = 414) were diagnosed with hEDS/HSD and fibromyalgia (Fibro), 23.8% (n = 167) hEDS/HSD, 13.3% (n = 98) fibromyalgia, or 7.4% (n = 54) none of these diagnoses. More patients were diagnosed with HSD (76.6%) than hEDS (23.4%). Patients were primarily White (95%) and female (90%) with a median age in their 30s (controls 36.7 [18.0, 70.0], fibromyalgia 39.7 [18.0, 75.0], hEDS/HSD 35.0 [18.0, 71.0], hEDS/HSD&Fibro 31.0 [18.0, 63.0]). There was high overlap in all 40 symptoms/comorbidities that we examined in patients diagnosed with fibromyalgia only or hEDS/HSD&Fibro, regardless of whether they had hEDS or HSD. Patients that only had hEDS/HSD without fibromyalgia had far fewer symptoms/comorbidities than patients with hEDS/HSD&Fibro. The top self-reported issues in patients that only had fibromyalgia were joint pain, hand pain when writing or typing, brain fog, joint pain keeping from daily activities, allergy/atopy and headache. Five issues that significantly and uniquely characterized patients diagnosed with hEDS/HSD&Fibro were subluxations (dislocations in hEDS patients), joint issues like sprains, the need to stop sports due to injuries, poor wound healing, and migraine. Conclusion: The majority of patients seen at the EDS Clinic had a diagnosis of hEDS/HSD plus fibromyalgia that was associated with more severe disease. Our findings indicate that fibromyalgia should be routinely assessed in patients with hEDS/HSD and vis-a-versa to improve patient care.

The Impact of a Group Telemedicine Program for Chronic Disease: A Retrospective Cohort Survey Study on Hypermobile Ehlers-Danlos Syndrome and Hypermobility Spectrum Disorder.

Background: We present an innovative care model for telehealth by creating a video conference group telemedicine program for patients with chronic disease and discuss findings from a post-program survey that was instrumental in understanding the response to telemedicine in a group setting. Methods: All patients who attended the group telemedicine program had a diagnosis of Hypermobile Ehlers-Danlos Syndrome or Hypermobility Spectrum Disorder and were requested to complete survey responses at the close of the program. Surveys were completed anonymously and electronically by REDCap. Elements of the Press Ganey, Consumer Assessment of Healthcare Providers and Systems, and Utah Telehealth Network patient satisfaction surveys were modified to construct the survey. Results: A total of 102 patients completed the post-telehealth program survey between August 20, 2021, and February 11, 2022. Around 93.1% stated that they gained a better understanding of the chronic condition, 88.3% stated that the program gave them the tools to improve, and 76.5% indicated the program addressed their specific needs. Approximately 92.1% found it easy to interact with the program facilitator and 79.4% found it easy to interact with program members. Around 93.1% said they would recommend the program to others. Discussion: We created a group telemedicine program for a complex chronic medical condition. The foundation of knowledge provided by the telemedicine program allowed more time during face-to-face encounters for individual assessment of the patient, and increased access to care. Overall, the program has improved the treatment process by reducing treatment burden and empowering patients with self-management skills to help reach our fundamental treatment goal of improving quality of life.

Establishing an Ehlers-Danlos Syndrome Clinic: Lessons Learned.

In a large academic medical center, patient requests from the community and internal referrals for evaluation of suspected hypermobility conditions were being denied consultation because services specific to this condition were not available. We identified this gap and developed a comprehensive evaluation for this unique patient population. The objective of this paper is to demonstrate a solution for improving outcomes in a neglected patient population by establishing an innovative outpatient clinic specifically tailored for patients with EDS. We describe the lessons learned on establishing a specialty clinic for treating patients with hypermobility syndromes including hypermobile Ehlers-Danlos syndrome (hEDS) and hypermobile syndrome disorder (HSD). Findings were collected from a patient focus group that was instrumental in understanding common care gaps. We document the firsthand perspective of three patients presenting with hypermobility accompanied by joint pain and denote the complicated state of healthcare in recognizing and treating this condition. A summary of patient demographics and characteristics was collected from patients seen in the clinic from November 14, 2019 to April 13, 2021. The firsthand accounts illustrate the challenges faced in treating this condition and the need for, and success of, this clinic using a coordinated care model. Demographics reveal a primarily white female population under the age of 50 with many comorbidities. Genetic testing was largely negative, with more patients diagnosed with HSD than hEDS. Our shared experience of launching a successful EDS clinic may assist other clinicians in establishing similar care models.

Opioid Use in Fibromyalgia Continues Despite Guidelines That Do Not Support Its Efficacy or Risk.

BACKGROUND/OBJECTIVE: The aim of this cross-sectional study is to determine the prevalence of opioid use in a large sample of fibromyalgia (FM) patients and examine the factors associated with opioid prescription/use despite multiple clinical guidelines that do not recommend opioid use in this population. METHODS: Data were collected from a convenience sample of 698 patients admitted from August 2017 to May 2019 into an intensive 2-day Fibromyalgia Treatment Program at a tertiary medical center in the United States after FM diagnosis. Patients were administered the Fibromyalgia Impact Questionnaire-Revised, the Center for Epidemiologic Study of Depression Scale, and the Pain Catastrophizing Scale upon admission to the program. Demographic information and opioid use were self-reported. Logistic regression analysis was utilized to determine associations between patient-related variables and opioid use in this prospective study. RESULTS: Of 698 patients, 27.1% (n = 189) were taking opioids at intake. Extended duration of symptoms (>3 years), increased age, higher degree of functional impairment, and increased pain catastrophizing were significantly associated with opioid use. CONCLUSIONS: Opioids are not recommended for the treatment of FM under current guidelines. Greater burden of illness appeared to be associated with the prescription and use of opioids in this population. These findings suggest that some providers may not be aware of current recommendations that have been found to be effective in the management of FM that are contained in guidelines. Alternative approaches to the management of FM that do not involve opioids are reviewed in an effort to improve care.

Opioid Tapering in Fibromyalgia Patients: Experience from an Interdisciplinary Pain Rehabilitation Program.

OBJECTIVE: Despite current guideline recommendations against the use of opioids for the treatment of fibromyalgia pain, opioid use is reported in approximately 30% of the patient population. There is a lack of information describing the process and results of tapering of chronic opioids. The purpose of this study is to describe opioid tapering and withdrawal symptoms in fibromyalgia patients on opioids. DESIGN, SETTING, AND SUBJECTS: This retrospective research study included a baseline analysis of 159 patients consecutively admitted to the Mayo Clinic Pain Rehabilitation Center from 2006 through 2012 with a pain diagnosis of fibromyalgia completing a 3-week outpatient interdisciplinary pain rehabilitation program. Opioid tapering analysis included 55 (35%) patients using daily opioids. METHODS: Opioid tapering was individualized to each patient based on interdisciplinary pain rehabilitation team determination. Opioid withdrawal symptoms were assessed daily, utilizing the Clinical Opioid Withdrawal Scale. RESULTS: Patients taking daily opioids had a morphine equivalent mean dose of 99 mg/day. Patients on < 100 mg/day were tapered off over a mean of 10 days compared with patients on > 200 mg/day over a mean of 28 days (P < 0.001). Differences in peak withdrawal symptoms were not statistically significant based on the mean equivalent dose (P = 22). Patients taking opioids for <2 years did not differ in length of tapering (P =0.63) or peak COWS score (P =0.80) compared with >2 years duration. Patients had significant improvements in pain-related measures including numeric pain scores, depression catastrophizing, health perception, interference with life, and perceived life control at program completion. CONCLUSION: Fibromyalgia patients on higher doses of opioids were tapered off over a longer period of time but no differences in withdrawal symptoms were seen based on opioid dose. Duration of opioid use did not affect the time to complete opioid taper or withdrawal symptoms. Despite opioid tapering, pain-related measures improved at the completion of the rehabilitation program.

A cognitive behavioral smoking abstinence intervention for adults with chronic pain: a randomized controlled pilot trial.

Current evidence suggests it may be difficult for patients with chronic pain to quit smoking and, based on previous formative work, a 7-session individual and group-based cognitive behavioral (CB) intervention was developed. The primary aim of this randomized controlled pilot trial was to test the hypothesis that abstinence at month 6 would be greater among patients with chronic pain who received the CB intervention compared to a control condition. Upon admission to a 3-week interdisciplinary pain treatment (IPT) program, patients were randomized to receive the CB intervention (n=30) or the control condition (n=30). The 7-day point prevalence of self-reported smoking status was assessed at week 3 (upon completion of the 3-week IPT program) and at month 6 in an intent-to-treat analysis. At week 3, 30% (n=9) of patients in the CB condition were abstinent from smoking compared to 10% (n=3) in the control group (P=.104). At month 6, 20% (n=6) of patients who received the CB intervention were abstinent compared to none in the control group (P=.024). At week 3, a significant group by time interaction effect was found where the CB patients experienced greater improvements in self-efficacy from baseline compared to the control group (P=.002). A greater proportion of patients randomized to the CB group completed the IPT program (P=.052). The findings of this pilot trial suggest that integration of a CB-based smoking abstinence intervention into ongoing pain therapy may be an effective treatment for smokers with chronic pain.

A psychometric investigation of the Suicide Status Form II with a psychiatric inpatient sample.

We investigated the psychometric validity and reliability of the Suicide Status Form-II (SSF-II) developed by Jobes, Jacoby, Cimbolic, and Hustead (1997). Participants were 149 psychiatric inpatients (108 suicidal; 41 nonsuicidal) at the Mayo Clinic. Each participant completed assessment measures within 24 hours of admission and 48-72 hours later. Factor analyses of the SSF core assessment produced a robust two-factor solution reflecting chronic and acute response styles. The SSF core assessment had good to excellent convergent and criterion validity; pre-post SSF ratings also demonstrated moderate test-retest reliability. The results replicated previous research and show that the SSF-II is psychometrically sound with a high-risk suicidal inpatient sample.