{
  "abstract": "Background Myotonic dystrophy (MD) is a neuromuscular disease that may affect the respiratory muscles contributing to the development of chronic hypercapnic respiratory failure. Long-term non-invasive ventilation (LT-NIV) is indicated for these patients; however the adherence to LT-NIV has been commonly described to be suboptimal. The aim of this project was to investigate the factors associated with adherence to LT-NIV in an attempt to provide interventions to improve LT-NIV use.Methods We analysed the clinical data of patients with MD referred to two large tertiary long-term ventilation centres in Liverpool and Manchester. Factors associated with adherence were analysed with Cox-regression and Cox-Mantel test.Results Out of 46 patients identified in this project, 26 were selected for the analysis (10 were not on positive airway pressure, 6 were on CPAP and in 4 patients no long-term follow up data was available). 20% of patients completely stopped LT-NIV by 28 days, 31% by 6 months, 40% by 1 year and 61% by 5 years. Patients with bulbar symptoms had significantly worse adherence to LT-NIV (p=0.02); whilst patients with atrial fibrillation were more likely using LT-NIV (p=0.03). Adherence was not related to age, sex, body-mass index, other comorbidities, the presence of respiratory symptoms, pre-setup nocturnal oximetry results, pre-setup blood gases, the mode of NIV or NIV settings (all p>0.05).Conclusions The adherence to LT-NIV is variable in MD with a significantly high percent of patients stopping LT-NIV early. Bulbar symptoms may prevent effective NIV use. Further studies are warranted to identify treatable traits to improve adherence to LT-NIV.",
  "authors": [
    {
      "affiliations": [
        "Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK"
      ],
      "name": "F Fyles"
    },
    {
      "affiliations": [
        "Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK"
      ],
      "name": "H Clay"
    },
    {
      "affiliations": [
        "Wythenshawe Hospital, Manchester University NHS Foundation Trust, Manchester, UK"
      ],
      "name": "J Wood"
    },
    {
      "affiliations": [
        "Wythenshawe Hospital, Manchester University NHS Foundation Trust, Manchester, UK"
      ],
      "name": "C Mason"
    },
    {
      "affiliations": [
        "Wythenshawe Hospital, Manchester University NHS Foundation Trust, Manchester, UK"
      ],
      "name": "A Bentley"
    },
    {
      "affiliations": [
        "Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK"
      ],
      "name": "B Chakrabati"
    },
    {
      "affiliations": [
        "Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK"
      ],
      "name": "R Parker"
    },
    {
      "affiliations": [
        "Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK"
      ],
      "name": "P Plant"
    },
    {
      "affiliations": [
        "Wythenshawe Hospital, Manchester University NHS Foundation Trust, Manchester, UK"
      ],
      "name": "A Bikov"
    },
    {
      "affiliations": [
        "Liverpool University Hospitals NHS Foundation Trust, Liverpool, UK"
      ],
      "name": "A Manuel"
    }
  ],
  "title": "P223 Long-term adherence to non-invasive ventilation in patients with myotonic dystrophy",
  "uid": "fc4b2fe4-f4f9-5f9c-a8f2-11dd82248f2a"
}