Timely referrals for augmentative and alternative communication (AAC) assessments are crucial for people with amyotrophic lateral sclerosis (ALS) in order to continue social participation, decision-making, and become proficient in using AAC strategies to communicate. The purposes of this study were (1) to identify people with ALS for whom the AAC assessment was delayed and (2) to document the factors that result in a late AAC assessment. The Nebraska ALS Database was reviewed to identify people with ALS for whom AAC assessment was delayed. The reasons leading to these delays were investigated by interviewing the AAC specialists who provided intervention services, as well as surviving family members when necessary. According to referral guidelines outlined by Ball, Beukelman, and Bardach (2007), 12% of people included in the Database received a late AAC assessment. Ninety-three percent of the assessments were delayed because of late referral, and 7% were delayed because of factors related to the individuals with ALS. Suggestions are made to assist medical personnel to make timely AAC assessment referrals.
People with amyotrophic lateral sclerosis (ALS) suffer a debilitating degenerative disease that leads to loss of movement throughout their bodies and eventually death. Ninety-five percent experience such severe loss of speech that they are unable to meet their communication needs through natural speech and must rely on some form of augmentative and alternative communication (AAC) support. If timely completion of the (a) referral for the AAC assessment, (b) AAC assessment, and (c) receipt of the AAC system is accomplished, people with bulbar ALS use their AAC (speech generating) technology until within two weeks of their death, an average of 23.1 months and those with spinal ALS used their AAC technology for 25.9 months (Ball, Beukelman, Anderson, Bilyeu, Robertson, & Pattee, 2007).
Benefits of a speech generating device
Use of a speech generating device (SGD) to supplement or replace speech can improve an individual's quality of life by fostering independence, improving self-esteem, helping to maintain relationships, reducing anxiety and frustration, allowing for discussions about medical procedures and financial concerns, and participating in the decision-making process (Brownlee & Palovcak, 2007). However, decisions about an SGD need to be made early to allow individuals with ALS to become familiar with the device while they can optimally manipulate the system. Brownlee and Palovcak (2007) report a lengthy time period needed for individuals with ALS to accept initially supplementing and eventually replacing their speech with another means. This reinforces the need to start the process of obtaining a speech generating device early.
AAC Referral Guidelines
AAC referral guidelines are available (Ball, Beukelman, & Bardach, 2007; Ball, Beukelman, & Pattee, 2002) to identify the appropriate timing for an AAC assessment referral. This allows for technology to be prescribed and purchased and for training to occur while people with ALS are still able to communicate using their residual, natural speech. These guidelines are based on habitual speaking rate, a parameter that is easy to measure in clinical settings. Ball, Beukelman, and Pattee (2002) identified a dramatic drop in speech intelligibility for persons with ALS when their speaking rate reached 100 wpm. In order to prepare for the upcoming decline in intelligibility, an AAC evaluation referral should be pursued when a person's speaking rate reaches approximately 125 wpm (65% of habitual rate), to allow for time to complete the evaluation, funding processing, setup, and initial instruction (Ball, Beukelman, & Bardach, 2007). Ball, Beukelman, and Pattee (2004) monitored the acceptance rate of persons with ALS for whom an AAC referral and an assessment was completed, and they reported that 96% accepted the recommendation for AAC technology.
Referral guidelines are not always followed and many people with ALS receive late AAC assessments resulting in a period when their natural speech is unintelligible and their participation in life and decision-making is limited. For some, an AAC assessment is not conducted until they are unable to meet any of their communication needs with their natural speech. At that point, they must rely on AAC technology from an AAC loan bank or wait even longer while the assessment is completed, the technology ordered, funding is approved, and training is completed. To date, there is little empirical information in the literature that documents the reasons for late AAC assessments of people with ALS. In the following sections, potential reasons for untimely AAC assessments are introduced.
A late diagnosis of the disease can occur and can impact the timeliness of referral for an AAC assessment. Often, the neurologist may be the second, third, or fourth professional to evaluate the patient (Belsh, 2000). Referrals to general practitioners, orthopedists, rheumatologists, physiotherapists, and otolaryngologists (Chio, 1999; Hillel et al., 1999; Househam & Swash, 2000; Pongratz, 1999; Swash, 1998), patient delay (Pongratz, 1999), and waiting for additional tests (Househam & Swash, 2000) often delays the referral to the neurologist. Prior to seeing the neurologist, a patient may also be misdiagnosed, which can significantly lengthen the time to an accurate diagnosis (Househam & Swash, 2000).
Patterns of Medical Decision-making
Even when diagnoses are timely, other factors related to medical decision-making can delay a referral for an AAC assessment. Medical care for adults is often based on two general strategies--prevention and response to symptoms. Of course, the speech limitations resulting from ALS cannot be prevented. People with ALS, their families, and even some physicians have a tendency to wait for an AAC assessment referral until speech becomes difficult to understand or relatively unintelligible. Unfortunately for those with ALS, once speech intelligibility begins to deteriorate, they typically become unable to meet their communication needs through speech after a relatively brief period of time. Therefore, intervention guidelines suggest that referrals for an AAC assessment be based on speaking rate rather than speech intelligibility (Ball, Beukelman, & Pattee, 2002).
Availability of AAC services
Timeliness of AAC assessment may also be related to the availability of AAC services. If personnel within a medical office or within a specialty clinic are not familiar with AAC intervention strategies, they may be insensitive to the need for timely referral. Even if the referral is made in a timely manner, some people with ALS live in geographic areas without accessible AAC services, and they may delay the assessment because it is inconvenient or expensive to travel.
Compliance with the Referral Recommendation
A final reason for delayed AAC assessment is that the person with ALS or family members are unwilling or unable to comply with the referral and the AAC assessment is not initiated until the individual is in communication crisis. These compliance related delays can occur for several different reasons. For example, the person with ALS and family members may have difficulty with decision-making in general and the failure to obtain an AAC assessment fits into a pattern of compliance problems. At times, decision-making may be delayed due to limited information about the financial obligations involved in AAC intervention. They may not be aware that Medicaid, Medicare, and many insurance policies frequently cover AAC related services. Finally, there may be unwillingness by the individual with ALS or other important decision-makers to move forward with the assessment. Through the years, a range of factors have been reported that have interfered with AAC referral compliance, such as decreasing self-image, cost, and fear of losing speech faster (Ball, Beukelman, & Bardach, 2007; Mathy, Yorkston, & Gutmann, 2000).
Despite the potential reasons for late AAC assessment referrals, there is little empirical evidence that documents current practice. The purposes of this study are (1) to identify people with ALS for whom the AAC assessment was delayed and (2) to document the factors that result in a late AAC assessment.
Data from 28 individuals with ALS were included in this report. The research team identified these individuals from the Nebraska ALS Database, which included 240 individuals. Each individual's habitual speaking rate was below 125 words per minute on the Speech Intelligibility Test (Beukelman, Yorkston, Hakel, & Dorsey, 2007). These individuals were primarily served through specialized ALS clinics where they are referred early in the progression of their disease. However, others received their care though independent medical providers and were entered into the Database when they came to AAC specialty centers for assessment. Although the participants resided in Nebraska, Iowa, South Dakota, and Kansas, their demographic information in terms of age, gender, and socio-economic status was similar to national statistics for persons diagnosed with ALS.
This study included a questionnaire development phase and an implementation phase. The lead author tallied the results.
The researchers developed an eight-item questionnaire (Appendix A), including a few with subcomponents, to identify information about the AAC assessment process. Questions were developed by a group of three AAC specialists to obtain information regarding the decision to make an AAC referral. All three specialists were experienced in completing AAC assessment and training for adults in medical settings. Each specialist reported information they collected during routine appointments to help determine the need for an AAC referral. The questionnaire reflected a comprehensive summary of this information.
The pilot implementation of the questionnaire included a select group of local speech-language pathologists to ensure ease of the questionnaire process, fine tune questions, and determine the most appropriate implementation procedures. Feedback received led to additional answer options for frequently listed items and provided more detailed instructions on how to answer questions.
The research team reviewed the Nebraska ALS Database and identified 28 people with ALS who received a late AAC assessment since the year 2000. A late AAC assessment was defined as an AAC assessment being conducted when the individuals' speaking rate was below 125 words per minute, as recommended by Ball, Beukelman, and Bardach (2007). Since patients could not be contacted directly, general information was solicited through speech-language pathologists working in the clinics. First, the authors completed each questionnaire database review to document the month and year of diagnosis, onset of symptoms, referral, and completion of the AAC assessment. The remaining information was obtained through a more extensive chart review or a personal interview with the AAC specialists who had completed the assessment, depending on the respondent's preference, to obtain information about the type of ALS, referral source, reasons for a delayed referral, and the speech characteristics and communication effectiveness at the time of the AAC evaluation.
The questionnaires were completed during a sixteen-week time period from March to July of 2008, for each person with ALS that was referred for a late AAC evaluation.
The researcher assessed content validity by having AAC experts review the survey to determine if the questions measured what it was proposed to measure and identify questions that may have been left out and questions that may not be needed. There were three AAC specialists working in universities and hospitals in Nebraska that work with clients with ALS that agreed to review the survey.
Timeliness of AAC Assessment
Twenty-eight participants were identified from the Nebraska ALS Database as receiving a delayed AAC assessment according to the guidelines of Ball, Beukelman, and Bardach (2007). This number represents approximately 12% of people who have been entered into the Database during the past 10 years. People with ALS are enrolled in the Database when they participate in an AAC assessment by four affiliated AAC clinics.
A review of the demographic information about participants who did not receive a timely AAC assessment did not differ in type of ALS and age of onset compared to the individuals in the Database who received timely assessment. Of those who received a late AAC assessment, 59% presented with bulbar onset, 33% with spinal onset, and 7% with mixed (bulbar-spinal) onset. The mean age of onset of 59.87 years (range 41-79 years) for those receiving a late AAC assessment was similar to the national median of 60 years (range 20-90 years) (Centers for Outcomes Research, 2009). However, they did differ in terms of gender. Individuals with ALS who received a late referral were more likely to be women (60%) than men (40%), which is opposite of the Database population and national demographics that report a higher incidence in men (59%) compared to women (41%) (Center for Outcomes Research, 2009). Additionally, 14 (50%) lived in rural areas.
Reasons for Delayed AAC Assessment
Of the 28 ALS participants identified as receiving a late AAC assessment, 93% (N = 26) were late because of difficulty obtaining a referral, while 7% (N = 2) received a delayed assessment because of factors related to the participants' acceptance.
Of the 93% who received a late AAC assessment because of a delayed referral, several factors were reported (Figure 1). Most physicians who did not refer in a timely manner were general practice physicians (32%), neurologists not associated with multi-disciplinary neuromuscular clinics (29%), or medical staff of long-term care facilities (7%). In time, these participants were referred to or sought out services in specialty clinics, at which time they were immediately referred for an AAC assessment. According to a spouse report, one neurologist initially refused to write an order for an AAC assessment for a participant because of his misperception that people with ALS were abusing AAC interventions because they had a terminal disease. Another participant had a history of developmental cognitive disability and resided in a residential facility in a very rural location. He had no living family member to advocate for him, and facility staff members were unfamiliar with the ALS diagnosis or available services. A third participant lived in a nursing facility located at a distance from her adult children. They eventually transferred her to a facility near them; however, this resulted in a delay of a range of services, including the AAC assessment.
Figure 1. Reasons for a Late AAC Assessment. Reasons for a Late AAC Assessment ASSESSMENT WAS DELAYED DUE TO ... General Practitioner 32% Non-clinic Neurologist 29% Clinic Neurologist 4% Skilled Nursing Facility/Group Home 7% Other Health Impairment 14% Family Issues 18% Travel 11% Language Barrier 7% Unaware of Services 4% Personal Acceptance 7% Note: Table made from bar graph.
Other factors, combined with a late referral from a physician, exacerbated the AAC assessment delay for some individuals. The timing of an AAC referral was also influenced by other health impairments (14%), such as frontotemporal dementia, aphasia, and cancer. One man was diagnosed with cancer the same week that he received his definitive ALS diagnosis. Although he was referred for an AAC assessment in a timely manner, he decided to focus on cancer treatment and was therefore delayed in making arrangements for his AAC assessment. The timing of AAC assessment was also influenced by decision-making patterns of the family (18%), travel difficulties (11%), language barriers (7%), and being unaware of services locally (4%).
Location of Residence
Many of those who received late assessments tended to reside in rural areas (50%) either in their own homes or in care facilities. Because of location, they did not have ready access to specialty neuromuscular clinics or AAC specialists. They tended to be served by general practice physicians or by medical specialists at a distance. Several of these participants were unable to travel the distances needed to receive specialized AAC services, and staff from the clinical affiliation had to travel to them to provide these specialized services.
Preparation of AAC Finders
The results of this project revealed that about 12% of people with ALS were delayed in their access to an AAC assessment. The authors are unaware of published data related to AAC assessment referral patterns for people with ALS. So, comparison to other studies or geographic regions cannot be made at this time. The most common reason for this delay was a delay in referral by medical personnel. Typically, these physicians or physician assistants practiced in relatively rural areas. As was described in a recent article by Beukelman, Ball, and Fager (2008), there is a need for "AAC Finders." For people with ALS, these individuals usually are medical personnel with an awareness of the communication limitations associated with ALS, the intervention options, financial support for AAC intervention services, and service provides. These Finders can work with those diagnosed with ALS, their families, and caregivers to access communication services in a timely manner. Especially in rural areas, these Finders are critical to timely interventions of all types. The results reveal the need for further education of AAC Finders about the importance of a timely AAC referral and assessment for individuals with ALS. The authors have found most medical personnel to be appreciative of information that the affiliated clinics provide to them either on a personal basis, through state medical societies, or at professional meetings. It is important for primary medical providers, the Finders, to be aware of the data related to AAC decision-making, as people with ALS and their families often rely on their primary doctor to assist them with these important medical decisions.
In order for people with ALS to have the time needed to complete an AAC assessment, purchase AAC technology, and learn to effectively operate this technology, they need to make decisions related to AAC options while they are still able to speak. In other words, they need to adopt a proactive decision-making strategy that might be quite different than what they and their families have used before. Rather than wait for the symptoms of speech disorder to become so severe that they can no longer communicate effectively, they need to make AAC related decisions based on speaking rate reduction as a predictor of future reductions in speech intelligibility and speaking effectiveness (Ball, Beukelman, & Bardach, 2007; Ball et al., 2002). AAC Finders and general practice speech language pathologists play an important role in preparing people with ALS to make these anticipatory decisions.
Integrating AAC and Other Medical Decisions
When discussing a late AAC referral with a referring physician, the physician asked why they were expected to introduce communication limitations so soon after the diagnosis of primary bulbar ALS. The physician was concerned that these individuals have just learned that they have a terminal illness and realize that they will not be able to work, they will not be ready to learn they will be unable to speak. This statement indicates how important it is to provide medical personnel and people with ALS a strategy for predicting the natural course of their communication decline.
We find that when speaking rate is monitored as a predictor of speech decline, people with ALS can be introduced to future communication limitations and intervention choices when their rate is well above 125 words per minute, and they can be assured that their natural speech will support them for the foreseeable future. Then, as speaking rate gradually declines they can prepare themselves for an eventual AAC assessment.
It is also important to have a timely diagnosis when an individual with ALS experiences co-occurring deficits. McCluskey (2007) revealed that frontotemporal dementia (FTD) is now considered a component of neurological degeneration in ALS. Although only 10% of the population will experience functionally debilitating FTD, deficits may be identified in up to 50% of the population. Since FTD can affect decision-making, important decisions, such as artificial nutrition (percutaneous endoscopic gastrostomy tube), mechanical ventilation, and speech generating technology (AAC) to replace speech, must be discussed early before FTD may interfere (McCluskey, 2007).
Given that 95% of people with ALS are unable to meet daily communication needs using their natural speech and that they require AAC strategies to support their communication efforts, it is critical that people with ALS receive timely AAC intervention services. The results of this investigation document the important roles that medical personnel play in timely referral. In addition, the importance of AAC decision-making by people with ALS, their family, caregivers, and medical personnel is highlighted. In addition, the field must continue to investigate the AAC referral patterns for women compared to men. The percentage of women who received a late referral was much greater than would be expected, given the gender demographic patterns reported for the ALS population. The AAC field must continue to find ways to prepare all decision-makers for their anticipated roles, such that people with ALS can communicate effectively throughout their lives.
Acknowledgments This research was supported in part by the Munroe-Meyer Foundation, the Barkley Trust, and the Muscular Dystrophy Association. The authors especially wish to thank the participants with ALS and their families who have supported this research project.
Address Corresponds to Amy S. Nordness, Ph.C, Speech Language Pathology, Munroe-Meyer Institute for Genetics and Rehabilitation, University of Nebraska Medical Center Omaha, NE 68198-5450, USA PHONE: 402-559-6460.
Ball, L. J., Beukelman, D. R., Anderson, E., Bilyeu, D. V., Robertson, J., & Pattee, G. L. (2007). Duration of AAC technology use by persons with ALS. Journal of Medical Speech-Language Pathology, 15, 371-381.
Ball, L. J., Beukelman, D. R., & Bardach, L. (2007). Amyotrophic lateral sclerosis. In D. R. Beukelman, K. L. Garrett, & K. M. Yorkston (Eds.), Augmentative communication strategies for adults with acute or chronic medical conditions (pp. 287-316). Baltimore, MD: Paul H. Brooks Publishing Co.
Ball, L., Beukelman, D., & Pattee, G. (2002). Timing of speech deterioration in people with amyotrophic lateral sclerosis. Journal of Medical Speech-Language Pathology, 10(4), 231-235.
Ball, L. J., Beukelman, D. R., & Pattee, G. L. (2004). Acceptance of augmentative and alternative communication technology by persons with amyotrophic lateral sclerosis. Augmentative and Alternative Communication, 20(2), 113-122.
Belsh, J. M. (2000). ALS diagnostic criteria of el escorial revisited: Do they meet the needs of clinicians as well as researchers? ALS and Other Motor Neuron Disorders, 1(Suppl. 1), 557-560.
Beukelman, D., Ball, L., & Fager, S. (2008). A personnel framework for adults with acquired complex communication needs. Augmentative and Alternative Communication, 24, 255-267.
Beukelman, D. R., Yorkston, K., Hakel, M., & Dorsey, M. (2007). Speech Intelligibility Test (SIT) [Computer Software]. Lincoln, NE: Madonna Rehabilitation Hospital.
Brownlee, A., & Palovcak, M. (2007). The role of augmentative communication devices in the medical management of ALS. NeuroRehabilitation, 22(6), 445-450.
Center for Outcomes Research (2009, February 23). The ALS C-A-R-E program. Retrieved February 23, 2009, from http://www.outcomes-umassmed.org/ALS/over-view.cfm
Chio, A. (1999). ISIS survey: An international study on the diagnostic process and its implications in amyotrophic lateral sclerosis. Journal of Neurology, 246 (Suppl. 3), III/1-III/5.
Hillel, A., Dray, T., Miller, R., Yorkston, K., Konikow, N., Strand, E., et al. (1999). Presentation of ALS to the otolaryngologist/head and neck surgeon: Getting to the neurologist. Neurology, 53(8), S22-S25.
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Appendix A: Questionnaire
The purpose of this study is to evaluate a subgroup of individuals with Amyotrophic Lateral Sclerosis (ALS) who received an augmentative and alternative communication (AAC) evaluation late, compared to the speaking rate guidelines developed by Ball, Beukelman, and Bardach (2007).
Please review your records, beginning in 2000, for AAC evaluations you have conducted for individuals with ALS who received a late AAC assessment (i.e., those with a speaking rate of less than 125 wpm). Please answer the questions as specifically as you can. We are not trying to intervene in professionals' referral process and do not need to know the specifics about who made the referral. In addition, please do not send this questionnaire home with your clients. You are intended to complete this questionnaire as their speech-language pathologist.
* Please complete the following information for each late referral.
1. Indicate the month and year of the AAC assessment referral.
2. Indicate the month and year of the onset of speech symptoms.
3. Indicate the month and year of the diagnosis of ALS.
4. Indicate the month and year of the AAC assessment.
5. What type of onset did the patient display?
6. Who made the AAC assessment referral?
* ALS/MDA Clinic
* Family Doctor
* Other allied health personnel (e.g., OT, PT)
* Private practice
* Family Member
7. Why was there a delay in initiating the AAC assessment referral?
* Referral was delayed (if checked, answer 8a)
* Referral was timely, but the patient delayed (if checked, answer 8b)
7a. If the referral was delayed, what interfered?
* Problem obtaining a referral
* Problem finding an AAC specialist
* Problem getting an appointment with an AAC specialist
* Compliance problem with the individual
* Individual had other interfering health issues
* Family issues
* Travel demands
* Funding difficulties for the assessment
* Funding difficulties for the device
7b. If the referral was timely, but the patient
delayed, what interfered?
* Travel demands
* Other health issues
8. Describe any difficulties encountered with the AAC referral that will help further explain the circumstance.
Amy S. Nordness, Ph.C.
University of Nebraska Medical Center
Laura J. Ball, Ph.D.
East Carolina University
Susan Fager, Ph.D.
Institute for Rehabilitation Science and Engineering at Madonna Rehabilitation Hospital
David R. Beukelman, Ph.D.
University of Nebraska, Lincoln
University of Nebraska Medical Center
Gary L. Pattee, M.D.
University of Nebraska Medical Center