Amyotrophic lateral sclerosis care in Saudi Arabia: A survey of providers’ perceptions

Abstract Objective Provision of care for patients with amyotrophic lateral sclerosis (ALS) is complex and requires the contribution of multiple healthcare professionals. Several international ALS care measures were developed to ensure optimal care for ALS patients. We looked at the rate of inconsistency in providing standard ALS care measures in Saudi Arabia (SA). Methods A 5‐point response survey was distributed to practicing neurologists in SA. They were asked to grade their perceived consistency of accessibility for 19 items of ALS care measures at their center. The list of ALS care measures items was derived from international ALS guidelines. Results The response rate from neurologists was 47.3% (62/131), and the responses of 39 neurologists who follow ALS cases were included. Most of the selected ALS care measure items, 63.1% (12/19), were perceived by 50% or more of the ALS care providers to be not consistently accessible to their patients. The perception of ALS care providers of the inconsistent accessibility for ALS patients to ALS care measures was high for communication devices (92.3%), supportive equipment such as motorized wheelchairs (76.9%), end‐of‐life discussion (74.4%), and respiratory monitoring (66.7%). Conclusion Our data show that ALS patients in SA do not have consistent access to the recommended ALS care measures.


| Method
We created a questionnaire based on a 5-point frequency scale ranging from "never" to "always" and confidence scale ranging from "not confident" to "extremely confident", with a few items using "yes" or "no" answers, as appropriate. The questionnaire items were derived from the published guidelines of ALS management (Miller et al., 2009a(Miller et al., , 2009b. Four neuromuscular neurologists were asked to independently review the provisional version of the survey in order to ensure content validity and clarity, and their input was incorporated into the final version (Supporting information). The survey was designed (using https://surve ymonk ey.com) and distributed to participants through email and private messages during the months of October 2018 to February 2019. We included all board-certified neurologists who are registered with the Saudi Commission for Health Specialties (SCFHS), including neuromuscular neurologists and general neurologists. Two additional reminders were sent to nonresponders two months apart. The study was approved by the institutional review board of King Abdulaziz University. The participants provided their consent at the beginning of the survey.

| Survey reliability and validity
The internal consistency of the survey was evaluated by using Cronbach α coefficients. The content validity was achieved mainly through agreement between four neuromuscular specialists on the survey content. Additionally, the validity was evaluated through exploratory factor analysis. Cronbach α > 0.8 and appropriate factor loading (eigenvalues > 1) explaining >50% of the variance were considered sufficient.

| Objectives
The primary objective was to identify the ALS care measure items that are perceived by ALS care providers to be inconsistently available to ALS patients. The secondary objective was to compare the ALS care measure accessibility between different cities in Saudi Arabia (the cities of Jeddah and Riyadh as the two large metropolitan areas, and other cities). We investigated the wait time to receive timely needed services such as bi-level positive airway pressure ventilators (BiPAP) and percutaneous endoscopic gastrostomy (PEG) tube insertion. Additionally, we looked at the confidence and comfort level among ALS care providers in certain aspects of ALS care.

| Statistical analysis
The demographics of the participants were described using frequencies. The responses of participants regarding their perceived frequency of access to ALS care measure items were dichotomized into inconsistent (never, rarely, and sometimes) and consistent (often and always). Similarly, the confidence level was dichotomized into confident (very confident and extremely confident) and suboptimal confidence (not confident, not so confident, and somewhat confident).
The chi-square test was employed to assess whether the provision of ALS care measure items was different across different cities.

| RE SULTS
The questionnaire was sent to 131 neurologists from 14 cities in SA; 62 of them (47.3%) responded. There were 36 (58.1%) neurologists practicing in tertiary hospitals, 17 (27.4%) in secondary hospitals, and 9 (14.5%) in private hospitals. There were 43 (32.8%) neurologists who follow at least one ALS case per year. Of these, four did not finish the survey; therefore, the responses from only 39 (29.7%) neurologists were included in the analysis (referred to as ALS care providers hereafter). There were 23 (37.1%) neurologists from Jeddah, 13 (20.9%) from Riyadh, and the remainder were from other cities (Table 1).

| Survey reliability and validity
Cronbach α was 0.88 which indicated a satisfactory reliability. Four neuromuscular specialists agreed on the survey content indicating satisfactory content validity. Exploratory factor analysis revealed six factors with eigenvalues >1 and explaining 75% of the variance (Tables S1 and S2).

| Items of ALS care perceived to be not consistently accessible by ALS care providers
Twelve out of the 19 items included in the questionnaire were perceived by 50% or more of the ALS care providers to be not consistently accessible to their patients ( Figure 1). These items were riluzole, respiratory and vital capacity assessment every 3 months, end-of-life discussion, palliative care, communication devices, visually controlled communication devices, motorized wheelchairs, head collars, home lifts, and cough assist devices. There were additional six items perceived by 33%-49% of the ALS care providers to be not consistently accessible to their patients, which included monitoring of riluzole blood work, physiotherapy access, occupational therapy access, BiPAP access, speech and language pathology access, and dietitian access.
There were no differences between different cities in accessibility to items of ALS care measures, except for more accessibility to motorized wheelchairs in Riyadh and other cities, as compared to Jeddah (Table 2).
The time required to arrange for PEG tube insertion was reported to be 4 weeks or less by 79.5% of ALS care providers, 1-2 months by 15.4%, and 2-4 months by 5.1%.
Confidence and comfort levels among ALS care providers in certain aspects of ALS care are detailed in Table 3.
A minority of ALS providers feel uncomfortable with making an ALS diagnosis (11.6%); however, almost a quarter (23.3%) of ALS care providers feel uncomfortable breaking the news of an ALS diagnosis.

| D ISCUSS I ON
Our study shows that more than half of ALS care measures are provided inconsistently to ALS patients in Saudi Arabia. An important factor related to this low accessibility is the lack of multidisciplinary clinics in which ALS care can be coordinated. As ALS progresses, it affects multiple systems that require specialized healthcare professionals to address. Timely coordination between healthcare professionals reduces the rate of hospitalization of ALS patients from 79% to 37% (Cordesse et al., 2015). The lack of multidisciplinary clinics makes such coordination slower; this is supported by our data, especially in the delay in access to BiPAP, for instance. vital capacity (FVC) should be performed every 3 months; however, our data show that this is perceived to be done inconsistently by approximately two-thirds of ALS care providers. Noninvasive ventilation, such as BiPAP, should be considered early, with onset of signs or symptoms of respiratory insufficiency (Bourke et al., 2006). The perceived inconsistency of accessing BiPAP in our survey was 46% implications on the disease, it should be considered for palliative