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  • Measure Summary
  • NQMC:010269
  • Jul 2012

Amyotrophic lateral sclerosis (ALS): percentage of patients diagnosed with ALS who were offered at least once annually assistance in planning for end of life issues.

American Academy of Neurology (AAN). Amyotrophic lateral sclerosis performance measurement set. St. Paul (MN): American Academy of Neurology (AAN); 2012 Jul 31. 75 p.

View the original measure documentation External Web Site Policy

This is the current release of the measure.

Primary Measure Domain

Clinical Quality Measures: Process

Secondary Measure Domain

Does not apply to this measure

Description

This measure is used to assess the percentage of patients diagnosed with amyotrophic lateral sclerosis (ALS) who were offered at least once annually assistance in planning for end of life issues (e.g., advance directives, invasive ventilation, hospice).

Rationale

Referral to palliative services varies considerably across different countries (Bede et al., 2011). End of life discussions will improve patient decision making with respect to disease management (Bede et al., 2011; Oliver et al., 2011; Albert et al., 1999; Mitsumoto et al., 2005; Nolan et al., 2008; Albert et al., 2005; Albert et al., 2009). Offering assistance in formulating an advanced care directive can initiate this discussion and re-discussion of the patient's preferences for life-sustaining treatments every 6 months (Albert et al., 1999; Mitsumoto et al., 2005; Nolan et al., 2008). Pain in amyotrophic lateral sclerosis (ALS) should be treated following accepted guidelines (Chiò et al., 2012; Handy et al., 2011; Brettschneider et al., 2008). Physical therapy will aid in treating spasticity and pain (Morris et al., 2006). Discussion of respiratory support if the patient has dyspnea, other symptoms of hypoventilation or vital capacity (VC) less than 50% will allow patient to choose intervention or hospice (Morris et al., 2006; Tripodoro & De Vito, 2008). Early referral to hospice or home care teams well in advance of the terminal phase of ALS will facilitate the work of the hospice team and improve patient transition to hospice (McCluskey & Houseman, 2004; Krivickas, Shockley, & Mitsumoto, 1997). A medical social worker can help with financial issues. Medications for terminal dyspnea, pain and/or anxiety will improve quality of life (McCluskey & Houseman, 2004; Oliver et al., 2010; Dand & Sakel, 2010; Rabkin et al., 2005; Krivickas, Shockley, & Mitsumoto, 1997).

The following clinical recommendation statements are quoted verbatim from the referenced clinical guidelines and represent the evidence base for the measure:

  • Advance directives for palliative end-of-life care should be discussed early with the patient and carers, respecting the patient's social and cultural background (EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis et al., 2012).
  • Offer assistance in formulating an advance care directive (Andersen et al., 2005).
  • Review the patients' wishes regarding their care and advance directives regularly (Heffernan et al., 2006).
  • Re-discuss the patient's preferences for life-sustaining treatments every 6 months (Andersen et al., 2005).
  • Initiate discussions on end-of-life issues whenever the patient asks or "opens the door" for end-of-life information and/or interventions (GPP) (Andersen et al., 2005). Treat pain in ALS following accepted guidelines (Andersen et al., 2005).
  • Initiate early referral to hospice or home care teams well in advance of the terminal phase of ALS to facilitate the work of the hospice team (Andersen et al., 2005).
  • Discuss options for respiratory support and end-of-life issues if the patient has dyspnea, other symptoms of hypoventilation or VC less than 50% (Andersen et al., 2005).
  • Treat terminal dyspnea and/or pain with opioids alone or in combination with benzodiazepines if anxiety is present (Andersen et al., 2005).

Evidence for Rationale

Albert SM, Murphy PL, Del Bene ML, Rowland LP. Prospective study of palliative care in ALS: choice, timing, outcomes. J Neurol Sci. 1999 Oct 31;169(1-2):108-13.

Albert SM, Rabkin JG, Del Bene ML, Tider T, O'Sullivan I, Rowland LP, Mitsumoto H. Wish to die in end-stage ALS. Neurology. 2005 Jul 12;65(1):68-74. PubMed External Web Site Policy

Albert SM, Whitaker A, Rabkin JG, del Bene M, Tider T, O'Sullivan I, Mitsumoto H. Medical and supportive care among people with ALS in the months before death or tracheostomy. J Pain Symptom Manage. 2009 Oct;38(4):546-53. PubMed External Web Site Policy

American Academy of Neurology (AAN). Amyotrophic lateral sclerosis performance measurement set. St. Paul (MN): American Academy of Neurology (AAN); 2012 Jul 31. 75 p.

Andersen PM, Borasio GD, Dengler R, Hardiman O, Kollewe K, Leigh PN, Pradat PF, Silani V, Tomik B, EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral. EFNS task force on management of amyotrophic lateral sclerosis: guidelines for diagnosing and clinical care of patients and relatives. Eur J Neurol. 2005 Dec;12(12):921-38. [122 references] PubMed External Web Site Policy

Bede P, Oliver D, Stodart J, van den Berg L, Simmons Z, O Brannagáin D, Borasio GD, Hardiman O. Palliative care in amyotrophic lateral sclerosis: a review of current international guidelines and initiatives. J Neurol Neurosurg Psychiatry. 2011 Apr;82(4):413-8. PubMed External Web Site Policy

Brettschneider J, Kurent J, Ludolph A, Mitchell JD. Drug therapy for pain in amyotrophic lateral sclerosis or motor neuron disease. Cochrane Database Syst Rev. 2008;(3):CD005226. [42 references]

Chiò A, Canosa A, Gallo S, Moglia C, Ilardi A, Cammarosano S, Papurello D, Calvo A. Pain in amyotrophic lateral sclerosis: a population-based controlled study. Eur J Neurol. 2012 Apr;19(4):551-5. PubMed External Web Site Policy

Dand P, Sakel M. The management of drooling in motor neurone disease. Int J Palliat Nurs. 2010 Nov;16(11):560-4. PubMed External Web Site Policy

EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis, Andersen PM, Abrahams S, Borasio GD, de Carvalho M, Chio A, Van Damme P, Hardiman O, Kollewe K, Morrison KE, Petri S, Pradat PF, Silani V, Tomik B, Wasner M, Weber M. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS) -- revised report of an EFNS task force. Eur J Neurol. 2012 Mar;19(3):360-75. [186 references] PubMed External Web Site Policy

Handy CR, Krudy C, Boulis N, Federici T. Pain in amyotrophic lateral sclerosis: a neglected aspect of disease. Neurol Res Int. 2011;2011:403808. PubMed External Web Site Policy

Heffernan C, Jenkinson C, Holmes T, Macleod H, Kinnear W, Oliver D, Leigh N, Ampong MA. Management of respiration in MND/ALS patients: an evidence based review. Amyotrophic Lateral Sclerosis. 2006 Mar;7(1):5-15. PubMed External Web Site Policy

Krivickas LS, Shockley L, Mitsumoto H. Home care of patients with amyotrophic lateral sclerosis (ALS). J Neurol Sci. 1997 Oct;152 Suppl 1:S82-9. PubMed External Web Site Policy

McCluskey L, Houseman G. Medicare hospice referral criteria for patients with amyotrophic lateral sclerosis: a need for improvement. J Palliat Med. 2004 Feb;7(1):47-53. PubMed External Web Site Policy

Mitsumoto H, Bromberg M, Johnston W, Tandan R, Byock I, Lyon M, Miller RG, Appel SH, Benditt J, Bernat JL, Borasio GD, Carver AC, Clawson L, Del Bene ML, Kasarskis EJ, LeGrand SB, Mandler R, McCarthy J, Munsat T, Newman D, Sufit RL, Versenyi A. Promoting excellence in end-of-life care in ALS. Amyotroph Lateral Scler Other Motor Neuron Disord. 2005 Sep;6(3):145-54. PubMed External Web Site Policy

Morris ME, Perry A, Bilney B, Curran A, Dodd K, Wittwer JE, Dalton GW. Outcomes of physical therapy, speech pathology, and occupational therapy for people with motor neuron disease: a systematic review. Neurorehabil Neural Repair. 2006 Sep;20(3):424-34. PubMed External Web Site Policy

Nolan MT, Kub J, Hughes MT, Terry PB, Astrow AB, Carbo CA, Thompson RE, Clawson L, Texeira K, Sulmasy DP. Family health care decision making and self-efficacy with patients with ALS at the end of life. Palliat Support Care. 2008 Sep;6(3):273-80. PubMed External Web Site Policy

Oliver D, Campbell C, Sykes N, Tallon C, Edwards A. Decision-making for gastrostomy and ventilatory support for people with motor neurone disease: variations across UK hospices. J Palliat Care. 2011;27(3):198-201. PubMed External Web Site Policy

Oliver DJ, Campbell C, O'Brien T, Sloan R, Sykes N, Tallon C, Taylor-Horan J, Udoma M. Medication in the last days of life for motor neuron disease/amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2010 Dec;11(6):562-4. PubMed External Web Site Policy

Rabkin JG, Albert SM, Del Bene ML, O'Sullivan I, Tider T, Rowland LP, Mitsumoto H. Prevalence of depressive disorders and change over time in late-stage ALS. Neurology. 2005 Jul 12;65(1):62-7. PubMed External Web Site Policy

Tripodoro VA, De Vito EL. Management of dyspnea in advanced motor neuron diseases. Curr Opin Support Palliat Care. 2008 Sep;2(3):173-9. PubMed External Web Site Policy

Primary Health Components

Amyotrophic lateral sclerosis (ALS); advance directives; invasive ventilation; hospice

Denominator Description

All patients with a diagnosis of amyotrophic lateral sclerosis (ALS) (see the related "Denominator Inclusions/Exclusions" field)

Numerator Description

Patients who were offered at least once annually assistance in planning for end of life issues (e.g., advance directives, invasive ventilation, or hospice)

Type of Evidence Supporting the Criterion of Quality for the Measure

  • A clinical practice guideline or other peer-reviewed synthesis of the clinical research evidence
  • A formal consensus procedure, involving experts in relevant clinical, methodological, public health and organizational sciences
  • A systematic review of the clinical research literature (e.g., Cochrane Review)
  • One or more research studies published in a National Library of Medicine (NLM) indexed, peer-reviewed journal

Additional Information Supporting Need for the Measure

Importance of Topic

Prevalence and Incidence

  • Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is a type of motor neuron disease that is a rapidly progressive and fatal neurological disease (National Institute of Neurological Disorders and Stroke [NINDS], 2013).
  • Twenty thousand to 30,000 people in the United States (U.S.) have ALS (NINDS, 2013).
  • Five thousand people are diagnosed with ALS in the U.S. annually (NINDS, 2013).
  • ALS is one of the most common neuromuscular diseases worldwide (NINDS, 2013).
  • In 90% to 95% of all ALS cases the disease occurs apparently at random with no clearly associated risk factors (NINDS, 2013).
  • Five percent to 10% of all ALS cases are inherited (NINDS, 2013).
  • Twenty percent of all familial cases result from a specific genetic defect that leads to mutation of the enzyme known as superoxide dismutase 1 (SOD1) (NINDS, 2013).
  • No cure exists for ALS. Newer pharmacotherapy agents have been found to reduce the progression, but not halt the disease development (NINDS, 2013).
  • The prevalence of ALS is said to be between six and eight cases per 100,000 in the population. Using the higher prevalence estimate and data from the 2000 U.S. census, nearly 22,600 Americans are living with ALS at any one time. Since ALS is a disease of aging, as the U.S. population increases and ages, an increase in the prevalence of ALS can be anticipated (ALS Association, 2012)
  • Cognitive dysfunction is seen in 20% to 50%, while only 3% to 5% develop dementia that is usually of frontotemporal type (Strong et al., 2009). Consensus criteria for diagnosis have recently been reported (Strong et al., 2009).
  • Death due to respiratory failure follows on average 2 to 4 years after onset, but a small group may survive for a decade or more (Haverkamp, Appel, & Appel, 1995).
  • The mean age of onset is 47 to 52 years in familial cases (FALS) and 58 to 63 years in sporadic (SALS) cases (Bobowick & Brody, 1973).
  • The lifetime risk for developing ALS for individuals aged 18 years has been estimated to be 1 in 350 for men and 1 in 420 for women (Armon, 2007) with male sex, increasing age and hereditary disposition being the main risk factors (Heffernan et al., 2006).

Mortality and Morbidity

  • Most patients with ALS die within 2 to 5 years of onset (Lechtzin et al., 2002). Only 10% of ALS patients survive for 10 years or more (Miller et al., "Drug, nutritional," 2009).
  • Treatment of respiratory insufficiency improves survival, quality of life and respiratory symptoms (Lechtzin et al., 2002; Miller et al., "Drug, nutritional," 2009). The diagnosis and management of respiratory insufficiency is critical because most deaths from ALS are due to respiratory failure (Lechtzin et al., 2002; Miller et al., "Drug, nutritional," 2009; EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis et al., 2012; Laird et al., 2001).
  • Falls surveillance will lead to interventions to prevent falls and decrease fall related deaths in ALS patients. Falls are an independent predictor of adverse health outcomes (Gil et al., 2008). Fall related deaths occur in 1.7% of ALS patients (Rubenstein & Josephson, 2002). Several specific risk factors for falls have been identified, including muscle weakness, deficits in gait or balance, visual deficits, arthritis, impairments in activities of daily living, depression, and cognitive impairment (Ringholz et al., 2005).
  • Studies confirm the presence of cognitive impairment in 50% of patients with ALS and particularly implicate executive dysfunction and mild memory decline in the disease process (Laird et al., 2001). More severe impairment occurs in a subset of patients with ALS and has features consistent with frontal temporal dementia (FTD) (Phukan, Pender, & Hardiman, 2007; Gordon et al., 2007). Recent studies have demonstrated the feasibility of screening patients in a busy specialized ALS clinic (Flaherty-Craig et al., 2009; Woolley & Katz, 2011), but this is still not routinely practiced. A fuller characterization of the extent of cognitive and behavioral dysfunction in ALS has important implications given that it shortens survival (Elamin et al., 2011), and the burden and stress for carers of patients with FTD is very great. It also has relevance to effective communication, legal issues and end-of-life decision making by patients with motor neuron disease (MND) (Elamin et al., 2011).
  • Pseudobulbar affect (PBA), excessive laughing or crying, or involuntary emotional expression disorder affects 20% to 50% of patients with ALS, especially in pseudobulbar palsy (McCullagh et al., 1999). Patients are embarrassed and isolated by these symptoms, which in turn greatly diminishes the patients' quality of life.
  • Sialorrhea, or drooling, is embarrassing, socially isolating, and is associated with aspiration pneumonia. The prevalence is estimated at 50%, and 70% of patients receiving oral medications for treatment reported benefit (Laird et al., 2001; Miller et al., "Multi-disciplinary," 2009)
  • Fatigue may be a symptom of depression, poor sleep, abnormal muscle activation, immobility, or respiratory dysfunction. Fatigue diminishes quality of life for patients with ALS. Fatigue was a side effect of therapy in 26% of patients taking riluzole vs. 13% taking placebo (Bensimon, Lacomblez, & Meininger, 1994). Asthenia occurred in 18% of patients taking riluzole vs. 12% of patients taking placebo in a larger study (Lacomblez et al., 1996).
  • The prevalence of depression in ALS ranges from 0% to 44%, although systematic studies suggest 10% in advanced ALS (Laird et al., 2001; Wicks et al., 2007). Depression shortens survival and lowers quality of life for patients with ALS (Phukan, Pender, & Hardiman, 2007). There is consensus among experts that depression should be treated in patients with ALS (Laird et al., 2001); however, there are no controlled studies of benefit or harm.
  • Insomnia is common in ALS and may be a symptom of early respiratory weakness, underlying anxiety, depression, or pain (Hetta & Jansson, 1997). There is a concern that sedative/hypnotic agents may suppress the respiratory drive in patients with ALS.
  • Weight loss is a key prognostic indicator for ALS with the risk of death increased 7-fold when body mass index is less than 18.5 kg/m2 (Marin et al., 2011; Lehéricey et al., 2012; Spataro et al., 2011; Desport et al., 1999; Vaisman et al., 2009; Dupuis et al., 2008).
  • ALS patients have dysarthria in nearly all bulbar onset patients and nearly 40% of ALS patients with spinal onset. More than 95% of ALS patients cannot speak before death and patients who accept gastrostomy tube, non-invasive ventilation or tracheostomy-ventilation have a greater need for augmentative alternative communication as the disease progresses (Ball, Beukelman, & Pattee, "Communication," 2004; Ball, Beukelman, & Pattee, "Acceptance," 2004; Mathy, Yorkston, & Gutmann, 2000; Beukelman, Fager, & Nordness, 2011).
  • End of life discussions will improve patient decision making with respect to disease management (NINDS, 2013; ALS Association, 2012; Strong et al., 2009; Haverkamp, Appel, & Appel, 1995; Bobowick & Brody, 1973; Heffernan et al., 2006). Pain in ALS should be treated following accepted guidelines (Oliver et al., 2011; Albert et al., 1999, Mitsumoto et al., 2005; Nolan et al., 2008; Albert et al., 2005; Albert et al., 2009).

Office Visits and Hospital Stays

  • One study's significant findings were that common morbidities increased over time (pneumonia [38.1% to 47.3%], respiratory failure [26.9% to 35.5%], and nutritional deficiency [43.0% to 56.3%]); the median length of stay dropped from 6 to 4 days; mean hospital charges increased from $21,574 to $24,314; the proportion of hospital deaths decreased over time (17.6% to 14.6%), whereas the proportion discharged to home health/hospice care (14.0% to 18.2%) and to long-term care facilities (13.2% to 27.9%) increased. The odds ratio (OR) of death was 5.03 (95% CI: 4.57 to 5.54) for those admitted with respiratory failure, 1.36 (1.24 to 1.50) for those with pneumonia, and 0.84 (0.77 to 0.92) for those with nutritional deficiency. The high OR of death in patients admitted for pneumonia or respiratory failure is likely associated with more advanced disease, whereas the protective effect of admission for nutritional deficiency is consistent with the predominance of bulbar symptoms and admission earlier in the disease. The trends during the 15 years of this administrative data set were for increasing comorbidities and higher utilization of end-of-life care (Dubinsky, Chen, & Lai, 2006).

Family Caregiving

  • Caregiver burden was correlated to their level of depression and quality of life and, differently from other chronic disorders, increased with the worsening of patients' disability. ALS patients have a good objective perception of their impact on caregivers (Chiò et al., 2005).
  • Recent studies assessing caregivers' burden in chronic neurologic disorders have found some features shared by caregivers: the perceived burden exceeds the objective measures of patients' impairment, the amount of burden is independent of diagnosis, and the patients' cognitive functioning is an important factor in determining the level of burden (Thommessen et al., 2002).

Cost

  • ALS is a difficult to diagnose, fatal, progressive degenerative disease with an average survival time of 2 to 5 years. Percutaneous endoscopic gastrostomy (PEG) and bi-level intermittent positive pressure (BIPAP) ventilation may be the major interventions leading to longer survival of patients with ALS. Riluzole has been shown to have modest effects on survival (as opposed to functional) gains and is currently the only drug approved for the treatment of ALS. Mechanical ventilation (via a tracheostomy tube) is expensive, but is widely used in later stage patients with ALS in the U.S. A review of nine cost-effectiveness studies of riluzole found the following: drug costs and survival gains are the major drivers of cost effectiveness; survival gains are estimated from truncated databases with a high degree of uncertainty; more accurate stage-specific utility weights based on patients who agreed to treatment are needed; case incidence-based evaluations should be carried out; cost-effectiveness ratios are insensitive to discount rates; employment and caregiver issues or externalities have been widely ignored; threshold acceptance cost-effectiveness values are ill-defined and evaluations are not generalizable to other countries because of cost and treatment style differences. On account of the high degree of uncertainty pertaining to survival gains and the relatively high costs per life years or quality-adjusted life-years gained, and while acknowledging that not every therapy has to be cost effective (e.g., orphan drugs), it is still inconclusive as to whether or not riluzole can be considered as cost-effective therapy for ALS (Ginsberg & Lowe, 2002).

Disparities

  • All races and ethnic backgrounds are affected by ALS (NINDS, 2013).
  • ALS most common in individuals 40 to 60 years old, but younger and older people can develop the disease (NINDS, 2013).
  • Men are more likely to develop ALS than women. Studies suggest an overall ratio of about 1.5 men to every woman who develops ALS in Western countries (ALS Association, 2012).

Opportunity for Improvement

  • Palliative care should be adopted from the time of diagnosis (Bede et al., 2011). Many patients are not adequately informed about advance directives and end of life decision making and many hospice workers are not familiar with ALS (Mandler et al., 2001; Miller et al., "Outcomes," 2009; Bradley et al., 2004). Management of the terminal phase of ALS is unsatisfactory in 33% to 61% of cases in Europe (Borasio et al., 2001) and only 8% of palliative care units are involved from the time of diagnosis (Oliver & Webb, 2000). The current system of palliative care in the United States is highly decentralized (National Hospice and Palliative Care Organisation [NHPCO], 2009). Between 60% and 88% of patients die in a medical facility in some countries and not at home, while over 58% of seriously ill ALS patients do not have hospice care (Spataro et al., 2010; Gil et al., 2008; Krivickas, Shockley, & Mitsumoto, 1997). Approaches to end of life care vary widely and are not standardized either in timing or content (Bede et al., 2011; Elman et al., 2006).

Evidence for Additional Information Supporting Need for the Measure

Albert SM, Murphy PL, Del Bene ML, Rowland LP. Prospective study of palliative care in ALS: choice, timing, outcomes. J Neurol Sci. 1999 Oct 31;169(1-2):108-13.

Albert SM, Rabkin JG, Del Bene ML, Tider T, O'Sullivan I, Rowland LP, Mitsumoto H. Wish to die in end-stage ALS. Neurology. 2005 Jul 12;65(1):68-74. PubMed External Web Site Policy

Albert SM, Whitaker A, Rabkin JG, del Bene M, Tider T, O'Sullivan I, Mitsumoto H. Medical and supportive care among people with ALS in the months before death or tracheostomy. J Pain Symptom Manage. 2009 Oct;38(4):546-53. PubMed External Web Site Policy

ALS Association. Epidemiology of ALS and suspected clusters. [internet]. Washington (DC): ALS Association; [accessed 2012 Apr 06].

American Academy of Neurology (AAN). Amyotrophic lateral sclerosis performance measurement set. St. Paul (MN): American Academy of Neurology (AAN); 2012 Jul 31. 75 p.

Armon C. Sports and trauma in amyotrophic lateral sclerosis revisited. J Neurol Sci. 2007 Nov 15;262(1-2):45-53. PubMed External Web Site Policy

Ball LJ, Beukelman DR, Pattee GL. Acceptance of augmentative and alternative communication technology by persons with amyotrophic lateral sclerosis. Augmentative Altern Commun. 2004;20(2):113-22.

Ball LJ, Beukelman DR, Pattee GL. Communication effectiveness of individuals with amyotrophic lateral sclerosis. J Commun Disord. 2004 May-Jun;37(3):197-215. PubMed External Web Site Policy

Bede P, Oliver D, Stodart J, van den Berg L, Simmons Z, O Brannagáin D, Borasio GD, Hardiman O. Palliative care in amyotrophic lateral sclerosis: a review of current international guidelines and initiatives. J Neurol Neurosurg Psychiatry. 2011 Apr;82(4):413-8. PubMed External Web Site Policy

Bensimon G, Lacomblez L, Meininger V. A controlled trial of riluzole in amyotrophic lateral sclerosis. ALS/Riluzole Study Group. N Engl J Med. 1994 Mar 3;330(9):585-91. PubMed External Web Site Policy

Beukelman D, Fager S, Nordness A. Communication support for people with ALS. Neurol Res Int. 2011;2011:714693. PubMed External Web Site Policy

Bobowick AR, Brody JA. Epidemiology of motor-neuron diseases. N Engl J Med. 1973 May 17;288(20):1047-55. PubMed External Web Site Policy

Borasio GD, Shaw PJ, Hardiman O, Ludolph AC, Sales Luis ML, Silani V, European ALS Study Group. Standards of palliative care for patients with amyotrophic lateral sclerosis: results of a European survey. Amyotroph Lateral Scler Other Motor Neuron Disord. 2001 Sep;2(3):159-64. PubMed External Web Site Policy

Bradley WG, Anderson F, Gowda N, Miller RG, ALS CARE Study Group. Changes in the management of ALS since the publication of the AAN ALS practice parameter 1999. Amyotroph Lateral Scler Other Motor Neuron Disord. 2004 Dec;5(4):240-4. PubMed External Web Site Policy

Chiò A, Gauthier A, Calvo A, Ghiglione P, Mutani R. Caregiver burden and patients' perception of being a burden in ALS. Neurology. 2005 May 24;64(10):1780-2. PubMed External Web Site Policy

Desport JC, Preux PM, Truong TC, Vallat JM, Sautereau D, Couratier P. Nutritional status is a prognostic factor for survival in ALS patients. Neurology. 1999 Sep 22;53(5):1059-63. PubMed External Web Site Policy

Dubinsky R, Chen J, Lai SM. Trends in hospital utilization and outcome for patients with ALS: analysis of a large U.S. cohort. Neurology. 2006 Sep 12;67(5):777-80. PubMed External Web Site Policy

Dupuis L, Corcia P, Fergani A, Gonzalez De Aguilar JL, Bonnefont-Rousselot D, Bittar R, Seilhean D, Hauw JJ, Lacomblez L, Loeffler JP, Meininger V. Dyslipidemia is a protective factor in amyotrophic lateral sclerosis. Neurology. 2008 Mar 25;70(13):1004-9. PubMed External Web Site Policy

EFNS Task Force on Diagnosis and Management of Amyotrophic Lateral Sclerosis, Andersen PM, Abrahams S, Borasio GD, de Carvalho M, Chio A, Van Damme P, Hardiman O, Kollewe K, Morrison KE, Petri S, Pradat PF, Silani V, Tomik B, Wasner M, Weber M. EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS) -- revised report of an EFNS task force. Eur J Neurol. 2012 Mar;19(3):360-75. [186 references] PubMed External Web Site Policy

Elamin M, Phukan J, Bede P, Jordan N, Byrne S, Pender N, Hardiman O. Executive dysfunction is a negative prognostic indicator in patients with ALS without dementia. Neurology. 2011 Apr 5;76(14):1263-9. PubMed External Web Site Policy

Elman LB, Stanley L, Gibbons P, McCluskey L. A cost comparison of hospice care in amyotrophic lateral sclerosis and lung cancer. Am J Hosp Palliat Care. 2006 Jun-Jul;23(3):212-6. PubMed External Web Site Policy

Flaherty-Craig C, Brothers A, Dearman B, Eslinger P, Simmons Z. Penn State screen exam for the detection of frontal and temporal dysfunction syndromes: application to ALS. Amyotroph Lateral Scler. 2009 Apr;10(2):107-12. PubMed External Web Site Policy

Gil J, Funalot B, Verschueren A, Danel-Brunaud V, Camu W, Vandenberghe N, Desnuelle C, Guy N, Camdessanche JP, Cintas P, Carluer L, Pittion S, Nicolas G, Corcia P, Fleury MC, Maugras C, Besson G, Le Masson G, Couratier P. Causes of death amongst French patients with amyotrophic lateral sclerosis: a prospective study. Eur J Neurol. 2008 Nov;15(11):1245-51. PubMed External Web Site Policy

Ginsberg G, Lowe S. Cost effectiveness of treatments for amyotrophic lateral sclerosis: a review of the literature. PharmacoEconomics. 2002;20(6):367-87. PubMed External Web Site Policy

Gordon PH, Wang Y, Doorish C, Lewis M, Battista V, Mitsumoto H, Marder K. A screening assessment of cognitive impairment in patients with ALS. Amyotroph Lateral Scler. 2007 Dec;8(6):362-5. PubMed External Web Site Policy

Haverkamp LJ, Appel V, Appel SH. Natural history of amyotrophic lateral sclerosis in a database population. Validation of a scoring system and a model for survival prediction. Brain. 1995 Jun;118 (Pt 3):707-19. PubMed External Web Site Policy

Heffernan C, Jenkinson C, Holmes T, Macleod H, Kinnear W, Oliver D, Leigh N, Ampong MA. Management of respiration in MND/ALS patients: an evidence based review. Amyotrophic Lateral Sclerosis. 2006 Mar;7(1):5-15. PubMed External Web Site Policy

Hetta J, Jansson I. Sleep in patients with amyotrophic lateral sclerosis. J Neurol. 1997 Apr;244(4 Suppl 1):S7-9. PubMed External Web Site Policy

Krivickas LS, Shockley L, Mitsumoto H. Home care of patients with amyotrophic lateral sclerosis (ALS). J Neurol Sci. 1997 Oct;152 Suppl 1:S82-9. PubMed External Web Site Policy

Lacomblez L, Bensimon G, Leigh PN, Guillet P, Meininger V. Dose-ranging study of riluzole in amyotrophic lateral sclerosis. Amyotrophic Lateral Sclerosis/Riluzole Study Group II. Lancet. 1996 May 25;347(9013):1425-31. PubMed External Web Site Policy

Laird RD, Studenski S, Perera S, Wallace D. Fall history is an independent predictor of adverse health outcomes and utilization in the elderly. Am J Manag Care. 2001 Dec;7(12):1133-8. PubMed External Web Site Policy

Lechtzin N, Rothstein J, Clawson L, Diette GB, Wiener CM. Amyotrophic lateral sclerosis: evaluation and treatment of respiratory impairment. Amyotroph Lateral Scler Other Motor Neuron Disord. 2002 Mar;3(1):5-13. PubMed External Web Site Policy

Lehéricey G, Le Forestier N, Dupuis L, Gonzalez-Bermejo J, Meininger V, Pradat PF. [Nutritional management in amyotrophic lateral sclerosis: A medical and ethical stake]. Presse Med. 2012 Jun;41(6 Pt 1):560-74. PubMed External Web Site Policy

Mandler RN, Anderson FA, Miller RG, Clawson L, Cudkowicz M, Del Bene M, ALS C.A.R.E. Study Group. The ALS Patient Care Database: insights into end-of-life care in ALS. Amyotroph Lateral Scler Other Motor Neuron Disord. 2001 Dec;2(4):203-8. PubMed External Web Site Policy

Marin B, Desport JC, Kajeu P, Jesus P, Nicolaud B, Nicol M, Preux PM, Couratier P. Alteration of nutritional status at diagnosis is a prognostic factor for survival of amyotrophic lateral sclerosis patients. J Neurol Neurosurg Psychiatry. 2011 Jun;82(6):628-34. PubMed External Web Site Policy

Mathy P, Yorkston K, Gutmann M. AAC for individuals with amyotrophic lateral sclerosis. Augment Disord. 2000;:183-231.

McCullagh S, Moore M, Gawel M, Feinstein A. Pathological laughing and crying in amyotrophic lateral sclerosis: an association with prefrontal cognitive dysfunction. J Neurol Sci. 1999 Oct 31;169(1-2):43-8. PubMed External Web Site Policy

Miller RG, Anderson F, Brooks BR, Mitsumoto H, Bradley WG, Ringel SP, ALS CARE Study Group. Outcomes research in amyotrophic lateral sclerosis: lessons learned from the amyotrophic lateral sclerosis clinical assessment, research, and education database. Ann Neurol. 2009 Jan;65 Suppl 1:S24-8. PubMed External Web Site Policy

Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, Kalra S, Katz JS, Mitsumoto H, Rosenfeld J, Shoesmith C, Strong MJ, Woolley SC, Quality Standards Subcommittee of the American Academy of Neurology. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009 Oct 13;73(15):1218-26. [40 references] PubMed External Web Site Policy

Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, Kalra S, Katz JS, Mitsumoto H, Rosenfeld J, Shoesmith C, Strong MJ, Woolley SC, Quality Standards Subcommittee of the American Academy of Neurology. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: multi-disciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review): report of the Quality Standards Subcommittee [TRUNC]. Neurology. 2009 Oct 13;73(15):1227-33. [40 references] PubMed External Web Site Policy

Mitsumoto H, Bromberg M, Johnston W, Tandan R, Byock I, Lyon M, Miller RG, Appel SH, Benditt J, Bernat JL, Borasio GD, Carver AC, Clawson L, Del Bene ML, Kasarskis EJ, LeGrand SB, Mandler R, McCarthy J, Munsat T, Newman D, Sufit RL, Versenyi A. Promoting excellence in end-of-life care in ALS. Amyotroph Lateral Scler Other Motor Neuron Disord. 2005 Sep;6(3):145-54. PubMed External Web Site Policy

National Hospice and Palliative Care Organisation (NHPCO). NHPCO facts and figures: hospice care in America. Alexandria (VA): National Hospice and Palliative Care Organisation (NHPCO); 2009.

National Institute of Neurological Disorders and Stroke (NINDS). Amyotrophic lateral sclerosis (ALS) fact sheet. [internet]. Bethesda (MD): National Institute of Neurological Disorders and Stroke (NINDS), National Institutes of Health (NIH); 2013 Jun [6 p].

Nolan MT, Kub J, Hughes MT, Terry PB, Astrow AB, Carbo CA, Thompson RE, Clawson L, Texeira K, Sulmasy DP. Family health care decision making and self-efficacy with patients with ALS at the end of life. Palliat Support Care. 2008 Sep;6(3):273-80. PubMed External Web Site Policy

Oliver D, Campbell C, Sykes N, Tallon C, Edwards A. Decision-making for gastrostomy and ventilatory support for people with motor neurone disease: variations across UK hospices. J Palliat Care. 2011;27(3):198-201. PubMed External Web Site Policy

Oliver D, Webb S. The involvement of specialist palliative care in the care of people with motor neurone disease. Palliat Med. 2000 Sep;14(5):427-8. PubMed External Web Site Policy

Phukan J, Pender NP, Hardiman O. Cognitive impairment in amyotrophic lateral sclerosis. Lancet Neurol. 2007 Nov;6(11):994-1003. PubMed External Web Site Policy

Ringholz GM, Appel SH, Bradshaw M, Cooke NA, Mosnik DM, Schulz PE. Prevalence and patterns of cognitive impairment in sporadic ALS. Neurology. 2005 Aug 23;65(4):586-90. PubMed External Web Site Policy

Rubenstein LZ, Josephson KR. The epidemiology of falls and syncope. Clin Geriatr Med. 2002 May;18(2):141-58. PubMed External Web Site Policy

Spataro R, Ficano L, Piccoli F, La Bella V. Percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis: effect on survival. J Neurol Sci. 2011 May 15;304(1-2):44-8. PubMed External Web Site Policy

Spataro R, Lo Re M, Piccoli T, Piccoli F, La Bella V. Causes and place of death in Italian patients with amyotrophic lateral sclerosis. Acta Neurol Scand. 2010 Sep;122(3):217-23. PubMed External Web Site Policy

Strong MJ, Grace GM, Freedman M, Lomen-Hoerth C, Woolley S, Goldstein LH, Murphy J, Shoesmith C, Rosenfeld J, Leigh PN, Bruijn L, Ince P, Figlewicz D. Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis. Amyotroph Lateral Scler. 2009 Jun;10(3):131-46. PubMed External Web Site Policy

Thommessen B, Aarsland D, Braekhus A, Oksengaard AR, Engedal K, Laake K. The psychosocial burden on spouses of the elderly with stroke, dementia and Parkinson's disease. Int J Geriatr Psychiatry. 2002 Jan;17(1):78-84. PubMed External Web Site Policy

Vaisman N, Lusaus M, Nefussy B, Niv E, Comaneshter D, Hallack R, Drory VE. Do patients with amyotrophic lateral sclerosis (ALS) have increased energy needs?. J Neurol Sci. 2009 Apr 15;279(1-2):26-9. PubMed External Web Site Policy

Wicks P, Abrahams S, Masi D, Hejda-Forde S, Leigh PN, Goldstein LH. Prevalence of depression in a 12-month consecutive sample of patients with ALS. Eur J Neurol. 2007 Sep;14(9):993-1001. PubMed External Web Site Policy

Woolley SC, Katz JS. Review article: utility of the ALS Cognitive Behavioral Screen. Neurodegener Dis Manag. 2011;1(6):473-9.

Extent of Measure Testing

This measure is being made available without any prior testing. The American Academy of Neurology (AAN) recognizes the importance of testing of all of its measures and encourages testing of the amyotrophic lateral sclerosis (ALS) measurement set for feasibility and reliability by organizations or individuals positioned to do so. The AAN welcomes the opportunity to promote the initial testing of these measures and to ensure that any results available from testing are used to refine the measures before implementation.

Evidence for Extent of Measure Testing

American Academy of Neurology (AAN). Amyotrophic lateral sclerosis performance measurement set. St. Paul (MN): American Academy of Neurology (AAN); 2012 Jul 31. 75 p.

State of Use

Current routine use

Current Use

Internal quality improvement

Professional certification

Measurement Setting

Ambulatory/Office-based Care

Home Care

Hospital Outpatient

Skilled Nursing Facilities/Nursing Homes

Professionals Involved in Delivery of Health Services

Physicians

Least Aggregated Level of Services Delivery Addressed

Individual Clinicians or Public Health Professionals

Statement of Acceptable Minimum Sample Size

Does not apply to this measure

Target Population Age

Unspecified

Target Population Gender

Either male or female

National Quality Strategy Aim

Better Care

National Quality Strategy Priority

Person- and Family-centered Care
Prevention and Treatment of Leading Causes of Mortality

IOM Care Need

End of Life Care

Living with Illness

IOM Domain

Effectiveness

Patient-centeredness

Case Finding Period

Unspecified

Denominator Sampling Frame

Patients associated with provider

Denominator (Index) Event or Characteristic

Clinical Condition

Denominator Time Window

Does not apply to this measure

Denominator Inclusions/Exclusions

Inclusions
All patients with a diagnosis of amyotrophic lateral sclerosis (ALS)

Note: Refer to the original measure documentation for International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) diagnosis codes and Current Procedural Terminology (CPT) Evaluation and Management (E/M) service codes.

Exclusions
Documentation of a medical reason for not offering at least once annually assistance in planning for end of life issues (e.g., patient in hospice and already in terminal phase)

Exclusions/Exceptions

Medical factors addressed

Numerator Inclusions/Exclusions

Inclusions
Patients who were offered at least once annually assistance in planning for end of life issues (e.g., advance directives, invasive ventilation, or hospice)

Exclusions
Unspecified

Numerator Search Strategy

Fixed time period or point in time

Data Source

Administrative clinical data

Electronic health/medical record

Paper medical record

Type of Health State

Does not apply to this measure

Instruments Used and/or Associated with the Measure

Unspecified

Measure Specifies Disaggregation

Does not apply to this measure

Scoring

Rate/Proportion

Interpretation of Score

Desired value is a higher score

Allowance for Patient or Population Factors

Unspecified

Standard of Comparison

Internal time comparison

Original Title

Measure #10: ALS end of life planning assistance.

Measure Collection Name

Amyotrophic Lateral Sclerosis Performance Measurement Set

Submitter

American Academy of Neurology - Medical Specialty Society

Developer

American Academy of Neurology - Medical Specialty Society

Funding Source(s)

Unspecified

Composition of the Group that Developed the Measure

Work Group Members Amyotrophic Lateral Sclerosis

Co-Chairs: Robert G. Miller, MD; Benjamin Rix Brooks, MD

American Academy of Neurology Representatives: Steven Ringel, MD; Hiroshi Mitsumoto, MD; Carlayne Jackson, MD; Christen Shoesmith, MD, BSc; Edward Kasarskis, MD, PhD

Pulmonologist: Robert C. Basner, MD

Gastroenterologist: Nicholas Procaccini, MD

American Academy of Physical Medicine and Rehabilitation: Gregory Carter, MD

Nurse: Dallas Forshew, RN, BSN

Physical Therapy/Occupational Therapy: Mohammed Sanjak, PhD, PT, MBA; Pat Casey, MS, CRCC

Hospice/Palliative Care Specialist: Bob Osborne, RN

Muscular Dystrophy Association: Valerie Cwik, MD

Patient Representative: Christine Jasch, OTR/L

Insurance Representatives: Fredrik Tolin, MD, MBA (Humana)

American Academy of Neurology Staff: Rebecca J. Swain-Eng, MS; Gina Gjorvad

Methodologist: Rebecca Kresowik

Quality Measurement and Reporting Subcommittee Facilitators & Liaisons: Richard Dubinsky, MD (Facilitator); Joel Kaufman, MD (Facilitator); Adam Cohen, MD (Facilitator); Christopher Bever, MD (QMR Chair); Eric Cheng, MD, MS (QMR Vice-Chair)

Financial Disclosures/Other Potential Conflicts of Interest

Unspecified

Adaptation

This measure was not adapted from another source.

Date of Most Current Version in NQMC

2012 Jul

Measure Maintenance

Unspecified

Date of Next Anticipated Revision

Unspecified

Measure Status

This is the current release of the measure.

Source(s)

American Academy of Neurology (AAN). Amyotrophic lateral sclerosis performance measurement set. St. Paul (MN): American Academy of Neurology (AAN); 2012 Jul 31. 75 p.

Measure Availability

Source available from the American Academy of Neurology (AAN) Web site External Web Site Policy.

For more information, contact AAN at 201 Chicago Avenue, Minneapolis, MN 55415; Phone: 800-879-1960; Fax: 612-454-2746; Web site: www.aan.com External Web Site Policy.

NQMC Status

This NQMC summary was completed by ECRI Institute on March 8, 2016. The information was not verified by the measure developer.

Copyright Statement

This NQMC summary is based on the original measure, which is subject to the measure developer's copyright restrictions.

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