Quality improvement in neurology: Amyotrophic Lateral Sclerosis Quality Measures

Amyotrophic lateral sclerosis (ALS) is a lethal, progressive, neurodegenerative disease characterized by loss of motor neurons (1). Most patients die from respiratory failure 2 to 5 years after onset of symptoms. Cognitive dysfunction is seen in 20–50% of patients (2). The disease burden for patients and caregivers is enormous. The average cost of care in the US has been estimated at $50,000 per patient per year (3).

In 1999, the American Academy of Neurology (AAN) published an evidence-based practice parameter for managing patients with ALS, including recommendations for the only disease-modifying drug, riluzole, which modestly lengthens survival (4). The publication was updated in 2009, and similar practice parameters were published by the EFNS in 2005 and 2012 (5). Important advances include the benefits of multidisciplinary clinics, noninvasive ventilation, enteral feeding, and symptomatic treatments for pseudobulbar affect and sialorrhea (5–15).

To further identify gaps in care, specific aspects of management of patients with ALS in the US have been evaluated serially using a national Amyotrophic Lateral Sclerosis Clinical Assessment, Research, and Education (ALS CARE) database to encourage compliance with evidence-based recommendations and to measure continuing quality improvement (14,19). Although awareness of therapeutic opportunities has increased, many evidence-based treatment recommendations are still underutilized (14). These gaps in care led the American Academy of Neurology (AAN) to develop a new ALS quality measurement set to improve the quality of care for patients with ALS. The expert work group represented physician organizations, patient advocacy groups, and other relevant stakeholder groups with the collective aim of improving care beyond that of traditional quality assessment methods for ALS. Details of the overall AAN measure development process are available (24) and additional details on the ALS Measure Development are available in the full manuscript found in appendix e-1 and the full measurement set in appendix e-2 online on Neurology's website at www.neurology.org.

In brief, a literature review and evidence search identified 378 recommendation statements from twenty guidelines and consensus papers (5–7,16,18,25,26) and many supporting evidence papers(references in appendix e-1). Review of the recommendations resulted in 13 recommendation statements that were rated highest on clinical importance, link to desired outcomes, evidence base, the level of evidence, gaps in care associated with the recommendation, and validity and feasibility to implement the recommendation in practice in a quality measure. Following extensive consultation, 11 final measures were approved by the AAN ALS quality measure work group and other appropriate AAN committees. These are listed in Table 1, and will be revised periodically with an extensive review every 3 years.

Table 1. ALS quality measures approved by the American Academy of Neurology.

Measure Title and Description

1. ALS Multidisciplinary Care Plan Developed or Updated

Percentage of patients diagnosed with ALS for whom a multi-disciplinary care plan was developed, if not done previously, and the plan was updated at least once annually.

2. Disease Modifying Pharmacotherapy for ALS Discussed

Percentage of patients with a diagnosis of amyotrophic lateral sclerosis with whom the clinician discussed disease-modifying pharmacotherapy (riluzole) to slow ALS disease progression at least once annually.

3. ALS Cognitive and Behavioral Impairment Screening

Percentage of patients diagnosed with ALS who are screened at least once annually for cognitive impairment (eg frontotemporal dementia screening or ALS Cognitive Behavioral Screen (CBS)) and behavioral impairment (eg ALS CBS).

4. ALS Symptomatic Therapy Treatment Offered

Percentage of visits for patients with a diagnosis of ALS with patient offered treatment for pseudobulbar affect, sialorrhea, and ALS related symptoms.

5. ALS Respiratory Insufficiency Querying and Referral for Pulmonary Function Testing

Percentage of patients with a diagnosis of amyotrophic lateral sclerosis who were queried about symptoms of respiratory insufficiency (awake or associated with sleep) and referred for pulmonary function testing (eg vital capacity (VC), maximum inspiratory pressure (MIP), sniff nasal pressure (SNP), or peak cough expiratory flow (PCEF)), at least every three months.

6. ALS Noninvasive Ventilation Treatment for Respiratory Insufficiency Discussed

Percentage of patients diagnosed with ALS and respiratory insufficiency with whom the clinician discussed at least once annually treatment options for noninvasive respiratory support (eg noninvasive ventilation (NIV), assisted cough).

7. ALS Screening for Dysphagia, Weight Loss and Impaired Nutrition

Percentage of patients diagnosed with ALS who were screened at least every 3 months for dysphagia, weight loss or impaired nutrition and the result(s) of the screening(s) was documented in the medical record.

8. ALS Nutritional Support Offered

Percentage of patients diagnosed with ALS and dysphagia, weight loss, or impaired nutrition who were offered at least once annually dietary or enteral nutrition support via percutaneous endoscopic gastrostomy or radiographic inserted gastrostomy.

9. ALS Communication Support Referral

Percentage of patients diagnosed with amyotrophic lateral sclerosis who are dysarthric who were offered a referral at least once annually to a speech language pathologist for an augmentative/alternative communication evaluation.

10. ALS End of Life Planning Assistance

Percentage of patients diagnosed with ALS who were offered at least once annually assistance in planning for end of life issues (eg advance directives, invasive ventilation, hospice).

11. ALS Falls Querying

Percentage of visits for patients with a diagnosis of amyotrophic lateral sclerosis with patient queried about falls within the past 12 months.

The measurement set addresses accurate and appropriate evaluation/monitoring of disease status and associated symptoms to guide treatment options (measures 1, 3, 4, 5, 7, 8, and 9), effective therapeutic options in eligible patients (measures 2,4,6 and 8), increasing patient awareness of advanced planning (measure 10), and patient safety (measure 11). For the full measure specifications and exceptions see appendix e-2 on the Neurology^® website at www.neurology.org.

The appropriate exceptions for each measure can be found in the full measure specifications (appendix e-2).

The deployment of this AAN ALS quality measurement set culminates a progression from an individual formulation (Ten Commandments for Optimal Motor Neuron Disease Patient Care) (27) through the demands for setting standards, (28) to addressing the gaps for quality standard-based clinical care (29). This quality evaluation also reflects similar initiatives in Europe, including France (30) and the United Kingdom (31). and as part of the recent European Commission Joint Programme in Neurodegeneration (ALS-CarE) (32).

In conclusion, the diagnosis of ALS has profound implications for the patient and their family. In a recent analysis, too few patients received evidence-based treatment that can ease the disease burden. The AAN ALS quality measures seek to encourage multidisciplinary care plans, treatments for respiratory and nutritional dysfunction, use of the single disease modifying agent available, and plans for a smooth transition to palliative care. Multidisciplinary clinics are available to some patients, and where available, referral is encouraged. For those who cannot access such expert services, the availability of practical and meaningful quality measures for the care of patients with ALS may raise the standard of care and lead to the desired outcome, an increased life expectancy with an enhanced quality of life for all with ALS.

Disclosures

Dr. Miller has received research support from the Muscular Dystrophy, and is a consultant for Cytokinetics, Neuraltus, and Asubio pharmaceuticals. Dr. Brooks has received funding and/or research grants from AB Science, Acorda, ALS Therapy Alliance, Asubio, Avanir., Biogen Idec, Bristol-Myers Squibb, Cytokinetics, GlaxoSmithKline, Knopp Bioscience, Muscular Dystrophy Association, Neuraltus, NINDS Clinical Research Consortium, QuestCor, and Teva. Ms. Swain-Eng is a full-time employee of the American Academy of Neurology. Dr. Basner is Principal Investigator of a Muscular Dystrophy Association grant, Opto-Electronic Plethysmography (OEP): A novel non-invasive quantitative assessment of lower rib cage paradox and use of accessory muscles in patients with amyotrophic lateral sclerosis; Site Investigator and Sleep Protocol Subcommittee member of an NHLBI/NIH grant, Sleep-Disordered Breathing During Pregnancy and Risks to Cardiovascular Health; and co-investigator of the following: R01HL103676-01A1 NIH/NHLBI; Subclinical Interstitial Lung Disease in MESA; R01HL093081 NIH/NHLBI; Pulmonary Vascular Changes in Early COPD; and InterMune PIPF-016: a randomized, double-blind, placebo-controlled, phase 3 study of the efficacy and safety of Pirfenidone in patients with Idiopathic Pulmonary Fibrosis (IPF). Dr. Carter reports no disclosures. Ms. Casey is a full-time employee of Northwestern Medical Faculty Foundation, Department of Neurology. Dr. Cohen reports no disclosures. Dr. Dubinsky reports disclosures for relationships with Allergan Pharmaceuticals, NIH, and AHRQ. Ms. Forshew is a consultant for Questcor and Asubio Pharmaceuticals. Dr. Jackson has received research support from Knopp Pharmaceuticals, Biogen Idec, and Cytokinetics. Dr. Kasarskis is a consultant for Asubio Pharmaceuticals and ATSDR. Dr. Procaccini reports no disclosures. Dr. Sanjak reports no disclosures. Dr. Tolin reports no disclosures.

Acknowledgements

The authors would like to thank all the ALS Measure Development Work Group Members for their contributions and work that supported the development of this manuscript: Robert G. Miller, MD (neurology); Benjamin Rix Brooks, MD (neurology); Steven Ringel, MD (neurology); Hiroshi Mitsumoto, MD (neurology); Carlayne Jackson, MD (neurology); Christen Shoesmith, MD, BSc (neurology); Edward Kasarskis, MD, PhD (neurology); Robert C. Basner, MD (pulmonology); Nicholas Procaccini, MD (gastroenterology); Gregory Carter, MD (physical medicine and rehabilitation); Dallas Forshew, RN, BSN (nursing); Mohammed Sanjak, PhD, PT, MBA (physical therapy/occupational therapy); Pat Casey, MS, CRCC (physical therapy/occupational therapy); Bob Osborne RN (hospice/palliative care); Valerie Cwik, MD (Muscular Dystrophy Association); Christine Jasch OTR/L)(patient representative); Fredrik Tolin, MD, MBA (Humana); Rebecca J. Swain-Eng, MS, CAE (AAN staff); Gina Gjorvad (AAN staff); Rebecca Kresowik (methodologist); Richard Dubinsky, MD (facilitator); Joel Kaufman, MD (facilitator); Adam Cohen, MD (facilitator); Christopher Bever, MD (AAN QMR); Eric Cheng, MD, MS (AAN QMR). The authors would also like to thank the Muscular Dystrophy Association for their support in the development of the measures.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.