Discussion

For our purposes, we have considered patients outside of Regina or Saskatoon to be “rural”. We feel this is justified given the fact that these smaller centers in Saskatchewan would be effectively considered remote with respect to access to neurology, since there are neurologists only in Regina and Saskatoon. In our study, there was no difference in diagnostic delay for rural patients, and despite the large remote population in Saskatchewan, linear regression modeling did not uncover a relationship between the distance from an urban center and diagnostic delay.

While some attention has been paid to the relationship between geospatial factors and incidence, prevalence, and survival in ALS,^{Reference Rooney, Heverin and Vajda9–Reference Traynor, Codd and Corr16} to our knowledge, the relationship between diagnostic delay in ALS and urban versus rural dwelling is largely unstudied, aside from one paper origininating from the Cantabria region of Northern Spain.^{Reference Riancho, Lozano-Cuesta and Santurtun13} While not the focus of this study, the median time to diagnosis did differ between groups (4.8 months for urban vs 9.1 months for rural dwellers); however, there are some limitations with interpreting the significance of this finding; while the total number of patients was 53, there is no documentation of the actual number of rural patients included in the study nor is there any information regarding their distance from the tertiary center. Our study, while still small, presents more than three times the number of patients included in the Spanish study. Saskatchewan’s geography and demographics also differs significantly from the region described in this article. Cantabria’s area is 5289 km² with a reported catchment population of 300,000,^{Reference Riancho, Lozano-Cuesta and Santurtun13,Reference Lopez-Vega, Calleja and Combarros14} and Saskatchewan’s area is 651,036 km² with a population of 1.1 million.⁷ It is difficult to compare such a small and comparatively densely populated region with Saskatchewan’s large territory and sparse population.

Internationally, rural dwelling has been cited as a cause for diagnostic delay in diseases, such as tuberculosis, cancers, congenital hearing loss, and dementia.^{Reference Ngwira, Dowdy and Khundi17–Reference Williams and Thompson20} Explanations have included travel distance, a higher likelihood that patients would seek alternative/traditional medical care first,^{Reference Yimer, Bjune and Holm-Hansen19} and lack of education.^{Reference Kisiangani, Baliddawa and Marinda18} In Saskatchewan, rural patients with dementia experienced diagnostic delay because caregivers had a lack of confidence in their local healthcare center and felt that their privacy may not be maintained.^{Reference Kisiangani, Baliddawa and Marinda18} Consequently, formal support services were utilized only after the patient could not be cared for adequately at home.^{Reference Kisiangani, Baliddawa and Marinda18} This is congruent with a Canadian systematic review that reported many rural dwellers with chronic disease in Canada are more hesitant to seek healthcare services until their disease is prohibitive to their ability to work.^{Reference Riancho, Lozano-Cuesta and Santurtun13} The lack of difference in diagnostic delay for our rural cohort might be explained by the rapid neurologic decline of ALS resulting in significant disability, and the loss of physical independence prompting medical attention.

An additional barrier to receiving adequate specialist care is the distance needed to travel. A study evaluating the usefulness of telehealth for dementia care in Saskatchewan showed that such a system would save patients an average of 426 km per round trip – a distance that may be prohibitive to some patients from attending follow-up care or utilizing a multidisciplinary clinic.^{Reference Dal Bello-Haas, Cammer, Morgan, Stewart and Kosteniuk21} Compounding this issue, travel in the winter in Canada can be particularly challenging and has been cited as a factor preventing rural dwellers from accessing healthcare.^{Reference Brundisini, Giacomini, DeJean, Vanstone, Winsor and Smith22} A study of patients and caregivers who attended the recently developed multidisciplinary ALS clinic in Saskatchewan cited travel (including inclement weather) as the only barrier to clinic attendance.^{Reference Schellenberg and Hansen23} These barriers considered it is somewhat surprising that even the most remote dwellers did not incur a difference in diagnostic delay.

The system of healthcare delivery may be a factor mitigating the rural versus urban discrepancy in diagnostic delay. In 2014, a Spanish study found that the greatest delay occurred between the first consultation with a non-neurologist and the assessment by a neurologist (with referral delay influenced by the patient’s symptoms and age). Patients assessed by a neurologist at the first or second consultation had a significantly shorter diagnostic delay.^{Reference Nzwalo, de Abreu, Swash, Pinto and de Carvalho3} Furthermore, it has been shown that a shortage of neurologists in rural areas can create diagnostic delay.^{Reference Sato, Morimoto, Deguchi, Ikeda, Matsuura and Abe24} However, the lack of discrepancy between urban and rural residents in Saskatchewan may be partly due to the existing healthcare system. Primary care physicians are the gatekeepers to secondary and tertiary care available in urban centers; this is consistent for both rural and urban dwellers. Furthermore, both rural and urban patients are placed on waitlists for the same specialists in urban centers; therefore, there is little discrepancy between the waitlist times of urban and rural patients.

Another important consideration is differences in attitude toward formal medical treatment and management. Studies of diagnostic delay in Kenya, Ethiopia, and Morocco show that some rural patients sought alternative, traditional treatments that were better understood by the community.^{Reference Kisiangani, Baliddawa and Marinda18,Reference Yimer, Bjune and Holm-Hansen19,Reference Maghous, Rais and Ahid25} Although recognizing that the urban/rural discrepancies in these countries may not be applicable to a Saskatchewan population, it might be assumed that rural patients find interventions, such as the insertion of feeding tubes, NIV, riluzole, or use of communication devices less palatable than their urban counterparts. However, our study found that these medical interventions were utilized equally in both rural and urban populations. Of note, both rural and urban patients attended an equal number of clinic visits, which may speak to the engagement of rural patients in the clinic processes. This may also partly explain why geospatial factors in Saskatchewan were inconsequential to patient life span after first symptom onset – a finding consistent with the 2016 study in Ireland that found that distance from dwelling to the ALS multidisciplinary clinic had no bearing on survival.^{Reference Rooney, Heverin and Vajda9}

Lastly, while a recent study on provincial differences in ALS care in Canada found Saskatchewan to have the longest diagnostic delay at 27 months,^{Reference Hodgkinson, Lounsberry and Mirian6} our study shows Saskatchewan’s diagnostic delay to be an average of 16.5 months – a timeline more consistent with the previously reported provincial averages (mean time to diagnosis 21 months; range 15.1–27.0 months). It has been previously noted that slower progressors may be more likely to be referred to multidisciplinary clinics,^{Reference Hutchinson, Galvin, Sweeney, Lynch, Murphy and Redmond26,Reference Lee, Annegers and Appel27} and slower progression is also associated with diagnostic delay.^{Reference Paganoni, Macklin and Lee1,Reference Hardiman, Van Den Berg and Kiernan2} Since the previous Canadian study enrolled a small number from Saskatchewan (n = 22), a recruitment bias is possible whereby the patients attending the clinic were slower progressors thus spending more time in clinic and therefore more likely to be enrolled.