Reasons Patients With Primary Progressive Multiple Sclerosis Contact Their Specialist Nurses

Multiple sclerosis (MS) can cause a large variety of symptoms and is highly heterogeneous across individual patients.¹  In the Netherlands, approximately 26,700 people are living with MS.²  Although exact numbers for the Netherlands are unavailable, approximately 15% of patients worldwide have the primary progressive disease course,¹  and there is no reason to assume that this percentage is different in the Netherlands. The variety of symptoms and differences in disease course can make it challenging for health care providers to tailor counseling and treatment according to patients’ needs.^3,4  Frequently, various medical specialists are involved, sometimes causing fragmented care.³ 

In the early 1990s, MS specialist nurses (hereafter, MS nurses) were first introduced in several countries. The exact role of MS nurses may vary between countries and even between hospitals within a country, but in general, MS nurses are trained to offer medical, social, and psychological support for patients with MS and to reduce fragmented care.^3,5,6  In our teaching hospital in the Netherlands, Albert Schweitzer Hospital, MS nurses are the first point of contact for patients with MS with questions about their physical and mental health. They refer patients to the appropriate health care providers in consultation with a neurologist. Multiple sclerosis nurses serve as the liaison between various health care professionals in primary and secondary care for individuals with MS.^5,7 

Previous studies about the unmet health care and personal needs of patients with MS have focused mainly on those with relapsing-remitting MS (RRMS).^8,9  However, it is likely that patients with primary progressive MS (PPMS) have needs that differ from patients with RRMS due to differences in disease course.³  Moreover, only 1 modestly effective disease-modifying therapy is currently available for patients with PPMS (ocrelizumab, available in the Netherlands since 2018) compared with several relatively effective disease-modifying therapies available for RRMS.^1,10,11  In addition, fampridine can be prescribed to certain patients with MS, including certain patients with PPMS, to improve walking speed.¹²  Knowledge of the needs of patients with PPMS is important to be able to tailor their care. The importance of studying the PPMS subgroup was already acknowledged by Holland et al³  in 2011; the present study adds insights into the needs of this group.

Multiple sclerosis nurses are approachable, have sufficient time for extensive and frequent consultations, and usually create an environment wherein patients feel no barriers to asking personal and delicate questions.^5,6,8,13  Insights into MS nurse consultations, therefore, provide a unique resource to investigate the needs of patients with PPMS, which can help tailor counseling and treatment. To gain insight into those needs, 3 interrelated questions were investigated: (1) What questions and issues for patients with PPMS result in MS nurse consultations? (2) Which patient-related factors make patients with PPMS more likely to consult an MS nurse? (3) What subsequent actions are performed by MS nurses to meet the patients’ needs?

This was a retrospective cohort study of all patients diagnosed with PPMS by their treating neurologist in the MS center of Albert Schweitzer Hospital, a large teaching hospital in the Netherlands. Patients were included if they visited the MS center between January 1, 2007, and January 1, 2021. Due to the retrospective character of the study and the disproportionate effort that would be needed to request explicit consent, all patients were included unless patients objected to the use of their data in general. The institutional review board of the Erasmus University Medical Center decided that this study was not subject to the Medical Research Involving Human Subjects Act.

In our MS center, standard PPMS care consists of 2 visits per year: an annual visit to the MS nurse and an annual visit to a neurologist who specializes in MS. During scheduled visits, the MS nurse uses a topic list that includes pain, spasms, and micturition and defecation issues, as well as more delicate topics such as sexuality. Clinic multidisciplinary meetings with 2 neurologists specialized in MS, 2 MS nurses, 2 rehabilitation specialists, a urologist, a neuropsychologist, a clinical psychologist, a psychologist-sexologist, a nursing home doctor, and a nursing home physiotherapist take place every 4 to 6 weeks. By indication, a gastroenterologist, an ophthalmologist, a gynecologist, and a hematologist can be present during multidisciplinary meetings. In case of defecation problems, patients are initially referred to a urologist. Patients are invited to our annual MS patient day, and a caregiver support program is offered.

Patient and disease characteristics were retrieved from patient files. Missing data on the presence of signs and symptoms were interpreted as negative. Start of follow-up was the date of diagnosis, unless it was before January 1, 2007, because MS nurses were employed by our hospital beginning January 2007. In that case, start of follow-up was defined as January 1, 2007. Follow-up ended at the last registered contact with the hospital within the study period defined previously herein.

Level of disability is expressed as the Expanded Disability Status Scale (EDSS) score (from 0 [no disability] to 10 [death due to MS]).¹⁴  The EDSS score was typically obtained at least once a year by the treating neurologist. In the case of a missing EDSS score, an expert neurologist (J.d.B.) estimated it retrospectively when possible.

The number of consultations (phone or live) with the neurologist and the MS nurse was registered, as well as whether a consultation was scheduled or patient initiated. To determine the reasons for patient-initiated consultations, patient files were searched for topics that were discussed during patient-initiated consultations and were categorized according to prespecified categories: car driving, care at home, cognition, comorbidities, daily activities (including work), fatigue, housing, medical devices, medication, micturition and defecation, mobility, mood, nutrition, pain, physical therapy/occupational therapy, progression, arm function, sleep, spasms, substance use, vision, and other. All questions by and quotes from patients that were found in the patient files were copied into our database word-for-word.

To gain insight into subsequent actions, the frequency of referrals to medical specialists or other health care providers (ie, as noted previously herein) was retrieved from the patient files.

The study population is described using medians and interquartile ranges (IQRs) for continuous variables and frequencies for categorical variables. Reasons for patient-initiated consultation (research question 1) and subsequent referrals (research question 3) are described using frequencies.

For research question 2, we determined which patient-related factors were associated with the number of patient-initiated consultations. Because the frequency of patient-initiated consultations is a count variable, an appropriate regression model for count data was used. Because the analysis indicated evidence of overdispersion (ie, the variance of the outcome was higher than the mean), a negative binomial regression model, which can handle overdispersion, was used. After consideration of the available literature, the following variables were selected as potentially relevant predictors: living with a partner (yes/no), EDSS score at diagnosis, comorbidities (yes/no), age, and sex.^4,15 

First, all variables of interest were included as independent variables in univariable negative binomial regression models. A multivariable negative binomial model was constructed using a stepwise backward approach to select the most relevant independent variables. All candidate variables were added in the multivariable model and were removed 1 at a time until all remaining variables had P < .20 in the final model. All negative binomial regression models were adjusted for follow-up duration by including the natural logarithm of this variable as an offset (ie, an independent variable with a fixed coefficient of 1) in the model. The associations between independent variables in the negative binomial regression models and the number of patient-initiated consultations are reported as incidence rate ratios (IRR). To assess whether patient characteristics were also associated with the different reasons for patient-initiated consultation, univariable logistic regression models were run with the predictors remaining in the multivariable negative binomial regression model as independent variables, and the reasons/topics as dependent variables. These logistic regression models were adjusted for within-patient correlations due to repeated measurements using a generalized estimating equation model specification with an exchangeable working correlation matrix. Statistical analyses were performed using Stata software, release 14 (StataCorp). All statistical tests were 2-sided with a significance level of .05.

In total, 98 patients with PPMS were included. Median age at diagnosis was 50 years (IQR, 44-58 years), and 61 of the patients were women (62%) (TABLE 1). During follow-up (median duration, 8.1 years; IQR, 4.4-16.3 years), these patients had a total of 720 consultations with MS nurses (median, 0 per year; IQR, 0-1 per year), of which 274 (38%) were patient initiated (median, 1 per year; IQR, 0-4 per year).

Overall, patients consulted the MS nurse with a wide variety of questions (FIGURE 1). For approximately half of the patient-initiated consultations, patients had more than 1 reason to contact the MS nurse. The most common category was treatment (n = 101, 37%), which included any questions regarding disease-modifying treatments as well as symptomatic medications. Patients frequently asked for new treatment options and expressed their need for effective disease-modifying treatments. Patients also contacted the MS nurse with questions about treatment options mentioned in the media. Illustrative quotes and questions from patients that were extracted from the patient files are as follows: “Is there currently a running clinical trial in which I could participate?”; “I have the feeling I haven’t tried everything in my power to beat MS; what can I do?”; “During the night, I wake up because my legs are cramping, and they are itching and burning. Previous treatments gave me side effects. Is there any new treatment for this?” Patients also asked questions about fampridine: “A few weeks ago I started using fampridine. The first 2 weeks went great, yet the last weeks my walking speed is declining. Should I continue using fampridine?”

The second most common category that patients consulted the MS nurse about was micturition and defecation (n = 85, 31%), including questions regarding concerning incontinence (both urine and feces), obstipation, and urinary tract infections (Figure 1). “Last week I was at the supermarket and suddenly I lost stool. I’ve never felt so embarrassed before in my life. I don’t dare to go shopping anymore”. “I have to go to the toilet so frequently during the night, and sometimes I cannot make it on time”. “I am not feeling well; I am having cold shivers. Could it be that I have a urinary tract infection again?”

Third, patients contacted the MS nurse expressing dark feelings (n = 54, 20%). “I am so worried about the future. Who is going to take care of my kids and partner when I will not be able to do so anymore?” “I could not sleep due to stress at my new job. Therefore, I have visited the company doctor, and he advised me to report fully sick for work. That makes me so sad and I cannot stop thinking about this. Now I cannot sleep anymore due to these sad feelings.”

Impaired mobility was also a reason for patients to contact the MS nurse (n = 37, 14%), often in combination with questions regarding spasms (n = 32, 12%), pain (n = 32, 12%), or medical devices (n = 15, 5%). Other topics that were discussed varied widely (Figure 1). The category “other” (n = 46, 17%) included questions regarding life expectancy, financial s ituation, t ransportation, and sexuality. One patient wanted to discuss euthanasia during a patient-initiated visit.

Figure 1 also shows the multiple topics discussed during scheduled visits. Some topics were discussed as frequently during scheduled visits as during patient-initiated visits. Yet there was a statistically significant increase in the discussion frequency during scheduled visits for the topics mobility (P < .001), medical devices (P = .003), care at home (P = .003), daily activities (including work) (P = .003), nutrition (P = .009), physical therapy (P = .010), fatigue (P = .013), vision (P = .021), and micturition and defecation (P = .028), probably due to the use of the topic list.

Based on the univariable negative binomial regression analyses, male sex (IRR, 2.227; P = .025), older age (IRR, 1.046; P = .020), and living without a partner (IRR, 2.751; P = .013) had a significant association with more frequent consults with the MS nurse (adjusted for variation in the duration of follow-up). The variables living without a partner and sex were retained in the final multivariable model (TABLE S1, available online at IJMSC.org). Patients living without a partner consulted the MS nurse 2.340 times (95% CI, 1.057-5.178 times) more frequently than patients living with a partner, corrected for sex and variation in the duration of follow-up. Male patients consulted the MS nurse 1.890 times (95% CI, 0.925-3.861 times) more frequently than female patients, also corrected for living with a partner and variation in the duration of follow-up. There was no significant difference in topics brought up by patients with a partner vs patients without a partner. Male and female patients brought up different topics. Female patients had a significant increase in questions regarding care at home (P = .037), nutrition (P = .041), physical therapy (P = .047), and spasms (P = .047) compared with male patients.

The third research question concerned what MS nurses did to meet the patients’ needs. In most consultations, no referral was deemed necessary. The MS nurses attended to the patients’ needs by providing information (verbally or by leaflet) and advice concerning diet, exercise, and defecation and micturition. In 22% of patient-initiated consultations (n = 59; 20% of all contacts [n = 146]), MS nurses referred a patient. The most frequent referrals after patient-initiated visits were to the neurologist (n = 18, 31%), followed by the urologist (n = 7, 12%), rehabilitation specialist (n = 7, 12%), psychologist (n = 6, 10%), physical therapist (n = 3, 5%), speech therapist (n = 3, 5%), and occupational therapist (n = 1, 2%). The remaining referrals (n = 14, 24%) were to other medical specialists and the general practitioner.

In this study, we investigated the needs of patients with PPMS using data on patient-initiated consultations with MS nurses, and we found that they consulted the MS nurse for a variety of reasons. Patients living without a partner and male patients were more likely to contact their MS nurse. In one-fifth of the patient-initiated consultations, the MS nurse consultation resulted in a referral to a specialist, most often a neurologist, a urologist, or a rehabilitation specialist.

The main reason patients contacted the MS nurse was for treatment-related questions. The present results are in line with previous results described in 2011: because there is no cure for PPMS, patients are still searching for treatments to lower symptom burden and frequently have questions about treatment options mentioned in the media.³  Because treatment is a recurrent topic in patient-initiated consultations, it is important that MS nurses are highly accessible to answer questions concerning treatment accurately and in a compassionate matter. Moreover, it seems important to repetitively provide detailed information to patients about new treatment options, not only if a treatment would be an option for a particular patient but also if it is not or not yet an option for a particular patient to manage patient expectations.

Micturition and defecation problems remain a major concern for patients with PPMS and, thus, a reason to contact the MS nurse despite the fact that MS nurses and neurologists also ask about these problems during scheduled visits. Zecca et al¹⁶  described the negative impact of micturition problems on the quality of life of patients with MS. Wang et al¹⁷  reported on the low treatment satisfaction of patients with MS and bladder, bowel, or sexual problems. The recent report by Alvino et al¹⁸  shows that moderate/severe bowel dysfunction occurred more frequently in patients with progressive MS phenotypes, higher disability, older age, and longer disease duration. The present results underline the importance of repetitively providing detailed information regarding micturition and defecation problems, on top of strictly and proactively monitoring such problems over time.

The present results indicate that male patients with PPMS and those without a partner contact the MS nurse more frequently. Previous findings by Lonergan et al¹⁹  indicated that patients with MS, but not specifically those with PPMS, without a partner perceive more unmet health care needs. Most patients with PPMS need supportive assistance at home, frequently provided by informal caregivers such as the partner.^3,4,20  Informal caregivers enable people with MS to remain in their homes as their functional limitations become more permanent and their need for personal assistance increases.^21,22  Sex differences in MS are well-known, especially concerning epidemiology, clinical aspects, and prognosis. Generally, male patients have a poorer prognosis, with a disease course that more severely restricts their mobility. Moreover, male patients with MS tend to experience lower social support than female patients with MS.^23-25  These findings might explain our observation of more frequent patient-initiated consultations by male patients with PPMS. On the contrary, men in the general population are a smaller proportion of health care consumers than are women.²³  Also, in a recent study, female patients with MS, but not specifically PPMS, had significantly higher direct medical costs than male patients with MS, due to ambulant consultations among other things.²⁶  More research into sex-related differences in the health care needs of patients with MS is needed to further individualize care. For now, the present findings provide justification for additional visits to meet the needs of male patients with PPMS or those living without a partner.

An interpretation of the fact that only 20% of all MS nurse consultations and 22% of all patient-initiated MS nurse consultations resulted in a referral is that the MS nurses provided tailored care that met the needs of their patients. In 20% of the consultations, the care needs were not able to be immediately addressed. In those cases, MS nurses referred patients to the appropriate providers. The diversity of referrals to other specialists that we found in this study clearly emphasizes the key role that MS nurses play in guiding patients with PPMS to the appropriate care, as has been reported in other studies as well.^5,6 

One interesting finding: in contrast to the findings of Holland et al,³  MS nurses did not receive questions about caregiver support in the present study. This might be explained by the fact that a caregiver support program is offered at our MS center, perhaps pointing to the need for more MS centers to consider such a program.

To our knowledge, this is the first retrospective study to specifically describe the needs of patients with PPMS by studying the topics brought up by the patients. Previously, questionnaires were frequently used in studies focusing on patient needs.^4,13,19  Using questionnaires has several well-known and important advantages; potential disadvantages are the loss of relevant information and context due to closed-ended questions, receiving socially desirable responses, questionnaire fatigue, and recall bias when discussing past events.²⁷  Furthermore, a prospective study design may have the potential disadvantage of individuals modifying their behavior in response to their awareness of being observed (ie, the Hawthorne effect)^28,29  when told that the help-seeking behavior will be studied. A retrospective design can overcome the Hawthorne effect^28,29  and provide insights into the actual behavior of patients. This study provides qualitative information based on quantitative data, thereby contributing more in-depth knowledge about what matters to patients with PPMS.

In conclusion, patients with PPMS consult the MS nurse with a variety of questions, emphasizing the pivotal role of MS nurses. The recurring questions about treatment options illustrate the pressing need for highly effective treatments. The present findings imply that strictly monitoring micturition and defecation problems and providing detailed information on symptomatic treatment options is of great importance to patients with PPMS. In addition, these results suggest that male patients with PPMS and those living without a partner would benefit from more frequent visits with the MS nurse.

We thank MS specialist nurses Teuni Boon and Yvonne van Vuuren and neurologist Désirée Zemel, MD, PhD (Department of Neurology, Multiple Sclerosis Center, Albert Schweitzer Hospital, Dordrecht, the Netherlands), for providing MS care; MS trial nurses Celine Schippers and Suze Roodenburg-Kooij for selecting the study participants; and neurologist Peter Sillevis Smitt, MD, PhD (head of the Department of Neurology, Erasmus University Medical Center, Rotterdam, the Netherlands), for his critical review of the manuscript.