Article Text
Abstract
Objective Neurofibromatosis type 1 (NF1) is a genetic disorder in which chronic pain commonly occurs. The study sought to understand the needs of individuals with NF1 and pain management experts when adapting a pain self-management mobile health application (app) for individuals with NF1.
Design We conducted a series of online, audio-recorded focus groups that were then thematically analysed.
Setting Online focus groups with adults currently residing in the USA.
Participants Two types of participants were included: individuals with NF1 (n=32 across six focus groups) and pain management experts (n=10 across three focus groups).
Results Six themes across two levels were identified. The individual level included lifestyle, reasons for using the mobile app and concerns regarding its use. The app level included desired content, desired features and format considerations. Findings included recommendations to grant free access to the app and include a community support feature for individuals to relate and validate one another’s experience with pain from NF1. In addition, participants noted the importance of providing clear instructions on navigating the app, the use of an upbeat, hopeful tone and appropriate visuals.
Conclusions Both participant groups endorsed the use of iCanCope (iCC) as an NF1 pain self-management mobile app. Differences between groups were noted, however. The NF1 group appeared interested in detailed and nuanced pain tracking capabilities; the expert group prioritised tracking information such as mood, nutrition and activity to identify potential associations with pain. In tailoring the existing iCC app for individuals with NF1, attention should be paid to creating a community support group feature and to tailoring content, features and format to potential users’ specific needs.
- COMPLEMENTARY MEDICINE
- Adult neurology
- Neurogenetics
- PAIN MANAGEMENT
- QUALITATIVE RESEARCH
- Neurological pain
Data availability statement
Data are available upon reasonable request. Data may be made available upon request to the senior author.
This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/.
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- COMPLEMENTARY MEDICINE
- Adult neurology
- Neurogenetics
- PAIN MANAGEMENT
- QUALITATIVE RESEARCH
- Neurological pain
Strengths and limitations of this study
Achieved data saturation, ensuring a deep understanding of the stated research question.
Online focus group format permitted a time-efficient and cost-efficient opportunity to enable wide geographical representation among participants.
Unverified neurofibromatosis type 1 status and low representation among members of minority communities may have limited the credibility and transferability of the findings.
Online focus groups limit the ability to observe in-person dynamics or subtle reactions to the various discussion topics.
Introduction
Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder affecting an estimated 1 in 3000 individuals worldwide.1 Common presentations of NF1 can include plexiform neurofibroma tumours, café au lait macules2 3 and extensive dermal neurofibromas.4 Over 50% of individuals with NF1 report significant pain and discomfort,5 often stemming from the plexiform neurofibroma tumours.6 In addition, frequent headaches and orthopaedic and other tumour-related pain4 may also impair NF1 patients’ quality of life.7
Current treatment recommendations for chronic pain related to NF1 are limited to pharmacological and surgical methods due to the complexity of the tumours.8 It is common for individuals with NF1 to employ complementary pain management approaches (eg, yoga, physical therapy and massage therapy) to manage their pain symptoms.9 However, there are also challenges to receiving treatment for NF1-related chronic pain such as healthcare system barriers and limited access to self-management techniques,10 creating a need for innovative, effective and accessible treatment options.
Mobile health applications (apps), with over 325 000 currently available, have become increasingly popular,11 including some for self-management of many chronic illnesses.12 13 A systematic review concluded that mobile health interventions improved clinical outcomes across varying chronic diseases and increased access to pain management resources at low cost,14 but once established, they can also be tailored to specific illnesses and conditions.
iCanCope (iCC) is an innovative mobile app originally developed to address pain self-management needs of adolescents and young adults with chronic pain.15 16 The iCC platform was created through a user-centred design approach that involved end users at every stage and is founded on the principles of cognitive–behavioural therapy and the use of mind–body approaches. The core iCC features include daily symptom tracking, personalised goal setting to improve pain and functioning, and a toolbox of disease education and pain self-management strategies. A pilot study of iCC demonstrated feasibility of deploying the app among adolescents with chronic pain17 and has since been expanded to support pain self-management in youth and young adults with juvenile idiopathic arthritis,18 sickle cell disease19 and postoperative pain,20 with each adaptation reporting clinically meaningful reductions in pain intensity.
The use of mobile technology to address the needs of patients with NF1 is a promising strategy to expand access to pain self-management interventions. We therefore proposed to adapt the iCC platform. As a first step, we sought recommendations about the desired features and content that adults with NF1 and pain management experts considered important to include when adapting the iCC mobile health app as well as the perceived acceptability and barriers/facilitators to its use. We reasoned that the multiple perspectives of these individuals would yield a richer and more nuanced assessment of the desired features and content to include in the adaptation.
Methods
Study sample
The study involved two participant groups: adults with NF1 (n=32) and experts in the treatment and management of NF1-associated pain (n=10). Inclusion criteria for the NF1 group were (1) age 18–45 years with an NF1 diagnosis, (2) pain severity aggregate score of ≥3 on the Brief Pain Inventory–Short Form (BPI-SF)21 22 for pain experienced in the previous 2 weeks, (3) being proficient in English at the fifth grade level and competent to provide informed consent, (4) agreeing to audio-recording of the focus group session and (5) residing in the USA. The exclusion criteria consisted of (1) moderate to severe cognitive deficits or (2) evidence of moderate to severe depression or anxiety, as assessed by the Patient Health Questionnaire (PHQ),23 SCID-Self-report (SCID-SR)24 and Generalised Anxiety Disorder 7-Item (GAD-7) scale.25 Inclusion criteria for the expert group were (1) providing healthcare to patients with NF1 (eg, physicians, nurse practitioners and psychologists) and (2) agreeing to audio-recording of the focus group session.
Patient and public involvement
Patients or the public were not involved in the design, conduct, reporting or dissemination plans of this study.
Recruitment procedures
Recruitment for the NF1 group consisted of posting an announcement briefly describing the study on the Facebook pages for two NF advocacy groups (NF Northeast and NF Network) and emailing all members of both group registries. Membership in both registries requires self-report of having received an NF diagnosis. Individuals interested in participating were directed to an online survey (www.qualtrics.com) that collected demographic and contact information as well as responses to the BPI-SF, PHQ, GAD-7 and SCID-SR. The research team reviewed the results of these clinical measures, and individuals meeting the inclusion requirements then received a list of focus group discussion (FGD) dates/times from which to select. Recruitment for the expert group occurred via website postings about the study on the US Association for the Study of Pain and the NF Network clinician pages. Prior to beginning each FGD, the participants were provided with a brief description of the study and were informed of the voluntary nature of their participation. All participants provided verbal informed consent prior to participating in the FGD session.
After completing the FGD, participants in the NF1 and expert groups received $25 and a $100 gift card, respectively.
Data collection and analysis
Each FGD session lasted approximately 1 hour and occurred via a secure, password-enforced video conference (www.zoom.com). Two members of the research team (FDB and KL) with prior training in conducting focus groups jointly facilitated the sessions.
An interview guide was developed specifically for this study (see the online supplemental file 1). Only the NF1 group sessions began by asking whether participants owned smartphones. All participants were then asked their opinions about using a phone-based app to address NF1 pain management. A brief presentation then occurred that described the existing iCC mobile app and included four screenshots from the app. The remaining session time was guided by that description, the four screenshots and the four domains included in the FGD guide: (1) perceived barriers and facilitators to using a mobile app to manage pain, (2) perceived acceptability and benefits of the proposed app, (3) contexts within which the app would most likely be used, and (4) recommendations for possible additions or modifications to the app. Demographic information for the NF1 group included age and gender, collected at eligibility screening, and that for the expert group included professional training/title, years in practice and gender, collected at the beginning of each FGD. The audio-recorded transcripts were professionally transcribed verbatim.
Supplemental material
An existing codebook from a prior iCC study26 was modified for use in the current study. Members of the data analysis team (FDB, KL, LEG, JNS and CL) met regularly to review transcripts and discuss codebook development. Interviews continued until data saturation had been achieved.27 28 The final codebook included 29 codes, 3 of which were new codes from the expert group sessions. Acceptable intercoder agreement was established after independently coding two transcripts. The remaining transcripts were then independently coded by both coders (FDB and KL). The coding team continued to meet periodically to resolve coding discrepancies (through consensus), address coding questions and further refine code definitions as needed. The coded transcripts were then entered into Dedoose V.8.3.47 (www.dedoose.com) for analysis. The coding and analysis process continued in an iterative fashion using thematic analysis.29 30 Negative instances where the data did not fit the existing themes were identified as part of the confirmability process.31 Descriptive statistics were calculated for the demographic data. The entire research team reviewed all analytical findings.
Results
A total of nine FGDs—six NF1 groups (n=32) and three pain management expert groups (n=10)—occurred between January and May 2020. Previous research has shown intrinsic motivation32 to be the critical, first step in promoting future goal-oriented behaviours.33 We therefore sought to identify themes that shaped motivation to use the proposed mobile app and generated a total of six themes across two levels (ie, individual and app) as seen in the conceptual model in figure 1. The themes of lifestyle, reasons for using the mobile app and concerns regarding use of the mobile app were identified at the individual level. The app level included the themes of desired content, desired features and format considerations.
Description of the study sample
A total of 42 individuals participated in the study (table 1). Overall, the NF1 group was generally young and predominantly female. Except for one participant, all owned smartphones. Most within the NF1 group were experiencing moderate pain at screening. The expert group consisted entirely of individuals with postgraduate training in their respective fields and had, on average, over 19 years of professional experience. Similar to the NF1 group, the majority of expert participants were female.
Individual-level themes
The individual level focused on the life situations of individuals with NF1 that were thought to influence motivation to use the proposed mobile app.
Theme 1: lifestyle (individual level)
Two subthemes were identified within the lifestyle theme (table 2): affordability/accessibility and time. The former focused on financial or other considerations that could influence ease of access. Both groups recognised that the mobile app needed to be ‘very cheap’ or free and not consume much data (ie, bottlenecking). Access issues related to geographical location or app use that depended on external factors such as internet signal strength or workplace access policies were also mentioned but were beyond what could be addressed within the app.
Time, the second subtheme, focused on the busy lifestyles of most NF1 group members, usually involving parenting or work responsibilities. Both participant groups recognised that use of the app needed to easily fit into users’ schedules. Barriers to its use included forgetting to access the app during busy times or when confronted with unexpected outside demands.
Theme 2: reasons for using the mobile app (individual level)
This theme concerned potential factors thought to directly increase motivation to use the mobile app, primarily through rapid user engagement with programme content and activities. It included two subthemes: engagement and hope (table 2).
The engagement subtheme focused on the fact that users would be more likely to use the app if they achieved improvements or successes early on. As one expert noted, ‘The ultimate aim is showing progress’. Both participant groups also believed that earning rewards (eg, badges and points) can promote engagement with and continued use of the app. Participants often referred to this as ‘buy-in’ and viewed it as critical to programme uptake.
Similarly, instilling a sense of hope was seen as essential to promoting engagement and continued use of the programme. Instilling hope was not considered to be limited to explicit messages of hope but could also result from individuals with NF1 recognising improvements in function, quality of life or having their NF1 pain validated by others.
Theme 3: concerns regarding use of the mobile APP (individual level)
The NF1 group seemed particularly concerned about privacy and access. They worried about who would have access to their health data and whether access to such data could jeopardise health insurance coverage or pose an unforeseen risk (table 2). As previously mentioned in the affordability/accessibility subtheme, participants were also concerned that internet coverage or work restrictions could limit use of the app. Members of the expert group were concerned that if goals were not realistic and achievable within an appropriate timeframe, some users might discontinue using the mobile app.
Application-level themes
The three themes included at the app level focused on elements in the proposed mobile app that could increase users’ motivation to use the product. The themes primarily concerned the content to be included and the functionality and features of the proposed product.
Theme 4: desired content (app level)
Participants identified the important types of information about chronic pain and its management that are needed by individuals with NF1. It included the subthemes of ‘pain 101’, self-care and clinical care (table 3).
The experts believed that accurate knowledge about the topic of pain was important in motivating individuals to use the mobile app—‘a very essential piece of this work, not sufficient, but necessary’ and referred to it as ‘pain 101’ information. This included information on the physiological basis of pain, neuropathic and nociplastic pain, the difference between acute and chronic pain, and NF1-specific pain. They also noted that users should receive instruction about how to successfully navigate the healthcare system and effectively interact with their healthcare providers.
By contrast, the NF1 group appeared most interested in learning about symptom recognition, recommended analgesics and strategies for targeting specific types of pain.
The self-care subtheme dealt with pain treatment approaches potential users could learn and then do independently or in group settings. Both participant groups offered many suggestions about possible pain mitigation strategies. These could be divided into two types of self-care strategies: physical and psychobehavioral. The former, endorsed by both groups, included movement-based activities such as martial arts, yoga or ‘grounding exercises’ and pacing. Only the expert group suggested improvement of sleep hygiene and use of heat and ice as physical approaches to self-management of NF1 pain. Both groups suggested psychobehavioral approaches (ie, those requiring only brief training with a qualified professional) such as deep, circular or meditational breathing and music or aroma therapy.
The final subtheme, clinical care, focused on pain treatments that required meeting with a trained professional for longer periods than typically required in self-care approaches. Yet, similar to the self-care subtheme, the treatments could be divided into physical and psychobehavioral approaches. Only the expert group identified physical approaches such as physical therapy or nutrition care. Longer-term psychobehavioral approaches included cognitive–behavioral therapy, acceptance and commitment therapy, guided imagery, biofeedback and mindfulness training.
Theme 5: desired features (app level)
The desired features theme included four subthemes: (1) tracking capabilities, (2) community, (3) external resources and (4) future features. This theme focused on the functionality of the mobile app and the desire for links to external resources (table 3).
Tracking of various events and information was considered by both participant groups to be an important feature of the mobile app. It included tracking and monitoring pain level, mood, activity, sleep and stress, all considered to be important potential pain triggers. Both groups also believed that maintaining notes or a diary was important but for somewhat different reasons. The NF1 group viewed this feature as a potential memory aid that could enable patients to ‘talk about (pain symptoms) more intelligently to a provider’. It could serve as a reminder for exercising, logging information in the app, or tracking meditation and physical activity. The NF1 group also expressed interest in recording their surgical and medication histories and having the capability of tracking pain details such as onset, location, quality and level of pain.
By contrast, the expert group appeared to be interested in a less detailed approach to tracking pain, although sufficiently detailed to be able to identify associations between pain and such things as mood, activity or treatment efficacy.
Both participant groups were enthusiastic about creating an online community that could serve as a resource for socioemotional support and NF1-relevant information (eg, local events and providers). Members of the expert group cautioned that it should not be a venue for complaining about having the disease, however. The NF1 group offered many suggestions about the format (eg, group size, age restrictions, creation of a buddy system), although none emerged as the preferred format. Both groups also agreed that sharing experiences could help normalise having NF1 and help individuals feel less isolated.
Participants believed that external resources such as links to other mobile apps could be helpful to patients with NF1. The NF1 group was interested in links to relevant articles and treatment guidelines. The expert group suggested links that could assist individuals with NF1 in self-management of their disease, including relaxation and pain sites.
Both groups identified some features that were currently not feasible to include in the mobile app, as they would require extensive resources, negotiations with other apps or lengthy modifications to the current iCC. They can be considered for future modifications to the app. Suggestions included automatic data upload at the next sign-in to make it more convenient for users whose internet access is blocked at work or in certain geographical locations, printable reports, 24/7 access to medical advice and a visual graphical representation of progress toward a specific goal.
Other suggestions, primarily from the NF1 group, were currently unrealistic to implement because they required formalising agreements with healthcare institutions. For example, they mentioned wanting the capability to automatically share information with healthcare providers or access existing X-rays when at another location.
Theme 6: format considerations (app level)
The final theme included two subthemes (table 4): user interface and presentation/tone. The first addressed issues that would increase users’ ease of use, and the second focused on the visual appeal and tone of the content presented.
Both groups understood the importance of user interface, that motivation to use the mobile app could be heavily influenced by its ease of use. The expert group suggested having explicit instructions on how to navigate and use the app. Participants from both groups recommended tailoring the app to users’ specific preferences on pain-tracking measures and format (eg, numeric vs figural, pain descriptors) and including an ‘other’ option so users could further qualify their responses to forced-choice questions. They also identified features to enhance time efficiency such as autopopulation of key information, use of drop-down menus, and consistency in format and content when updating.
Critical elements in motivating individuals to use the mobile app were tone and presentation, the final subtheme. Participants described the ideal app as being ‘approachable and digestible’ and ‘simple and fun’. Visual appeal was important, noting that the font and use of colour could influence use. Careful selection of NF1 photographs and language could also be used to set an appropriate tone. Members of the NF1 group believed that daily tips or inspiring quotes could enhance engagement, and experts recommended varying the messages to keep the content fresh. Both groups agreed that music could help set a positive tone as well.
Discussion
Few studies have examined the perceived benefits, desired content and features, or efficacy of mobile health apps on coping with NF1-related chronic pain. To our knowledge, the current study is the only one to examine some of these issues from the perspectives of the targeted end users and pain management experts. We believe that this is essential information to ensure that the product is scientifically accurate and relevant while also satisfying targeted end users’ needs and preferences.
Both groups were enthusiastic about the prospect of adapting the iCC mobile app for individuals with NF1 with chronic pain. They believed that tailoring the programme to accommodate users’ lifestyle needs and affordability, incentivising its use, fostering an NF1 community support group, and using appropriate and acceptable tone and format features will encourage uptake and continued use of the mobile app. In addition, offering NF1 and coping skills information and an online support community in a single mobile app renders it both convenient and comprehensive in scope.
Participants believed that flexibility in fitting into potential users’ current situation was important. They noted that the app should be affordable, easy to use and convenient; it should fit users’ schedules, efficiently capture the desired data and be modifiable as users’ life situations change. These findings support previous research on features needed to ensure uptake and ease of use of the mobile app34 35 as well as health-related mobile app users’ privacy and security concerns.36 37
With respect to the issue of promoting sustained use of the app, participants from both groups noted the importance of including realistic and achievable goals. Early improvements might involve pain reduction or changes in pain attitudes that could improve mood, function or quality of life. Consistent with the notion of contingency management,38 39 participants identified strategies involving points, levels, prizes or well-established reinforcement schedules to incentivise continued use. These findings suggest the importance of considering contingency management when developing an app to promote health behaviours.40 41
Regarding the pain symptom tracking feature, the two groups had somewhat different views on what type of pain data are most desired. The NF1 group appeared interested in gathering as detailed and nuanced pain information as possible. By contrast, the expert group appeared more interested in tracking information that might identify potential associations between pain, mood, nutrition, activity and stress levels; they believed that noting these patterns would be more useful than tracking pain intensity, duration, quality and directionality. Consistent with previous research,42 the two participant groups expressed different perspectives on and desires for tracking chronic pain. The data suggest that to accommodate these disparate views, the pain tracking feature should permit simultaneous tracking of several constructs (eg, pain, mood and activity) while also providing a flexible format so that patients can select the level of detail and pain metrics they prefer. One cautionary note, however, would be to avoid the potential for users to become fixated on their pain symptoms to the detriment of recognising the associations between their pain and the other constructs. Based on input from our expert group, we plan to develop an app that is engaging without overfocusing on tracking or complaining about symptoms and subsequently assess its effectiveness and potential positive/negative effects.
Community support, particularly for individuals with rare diseases such as NF1, can be an important coping resource.43 44 Previous research on online community support platforms for individuals with rare diseases has noted that they are used for both informational and socioemotional support purposes.45 46 The value of community support has been identified within the context of cultural and racial socialisation,47 48 and the study corroborates these findings. Both groups noted how the creation of an online NF1 community would provide users the opportunity to share and thereby validate their experiences with the disease and chronic pain, provide socioemotional encouragement and potentially motivate continued use of the mobile app.
The study supported previous findings about visual appeal, tone, ease of use, instructions on effectively navigating app options, tailoring mHealth interventions to personal needs and preferences and the use of appropriate visuals.49 50 This last feature is particularly important with a potentially disfiguring disease such as NF1. A novel finding from this study, however, is the importance of establishing an upbeat tone that instils hope.
We note several study limitations. First, the findings may have limited transferability, given the modest racial and ethnic diversity of the study sample. Second, lack of external confirmation of self-reported NF1 diagnoses may have limited the credibility of the results. Third, the myriad of suggestions proffered may be attributed to social desirability wherein participants sought to identify as many options as possible in order to ‘please’ the group facilitators. Nonetheless, these options made possible a deeper analysis of potential format and content considerations when adapting the iCC app. Finally, virtual FGDs limit the ability to observe subtle, in-person dynamics during discussions. We chose this format, however, prior to the onset of the COVID-19 pandemic, based on our desire to collect data in a time-efficient and cost-efficient manner across diverse geographical regions and living situations.
Conclusions
The study findings suggest that adapting the iCC mobile app for use in the self-management of NF1-associated chronic pain was seen by adults with NF1 and chronic pain experts as a welcome addition to the armamentarium of NF1 treatment resources. Careful attention should be paid to the creation of a community support group option as well as programme content, format and ability to tailor it to users’ specific needs.
Data availability statement
Data are available upon reasonable request. Data may be made available upon request to the senior author.
Ethics statements
Patient consent for publication
Ethics approval
This study involves human participants and was approved by the human subjects committee of the Yale Human Research Protection Program of institutional review boards at Yale University (protocol #2000025437). The participants gave informed consent to participate in the study before taking part. The institutional review board at Yale University approved all study materials and waived the need to obtain written consent.
Acknowledgments
We are indebted to Karen Peluso, director of NF Northeast and Kim Bischoff, Director of NF Network for their help and commitment. We also thank the study participants for sharing with us their time and insights.
References
Supplementary materials
Supplementary Data
This web only file has been produced by the BMJ Publishing Group from an electronic file supplied by the author(s) and has not been edited for content.
Footnotes
Contributors LEG: manuscript preparation and review, study design and data analysis and development of interview guide. KL: daily oversight of project, focus group facilitation, data management and analysis, manuscript preparation and review. CL and JNS: development of interview guide, manuscript preparation and review. WTZ and SAB: supervision of the project and manuscript review. FDB: guarantor, study design and data analysis, led the focus groups, development of interview guide, manuscript preparation and review and oversight for the general conduct of this study.
Funding This work was supported by the US Army Medical Research Materiel Command endorsed by the US Army, through the Congressionally Directed Medical Research Programs’ Neurofibromatosis Research Program (award number W81XWH-19-1-0618).
Competing interests None declared.
Patient and public involvement Patients and/or the public were not involved in the design, conduct, reporting or dissemination plans of this research.
Provenance and peer review Not commissioned; externally peer reviewed.
Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.