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Factors Influencing Motivation and Engagement in mHealth Amongst People with Sickle-Cell Disease in Low-Prevalence, High-Income Countries: Qualitative Exploration of Patient Requirements (Preprint)

  • University of Tromsø - The Arctic University of Norway

Abstract and Figures

BACKGROUND Sickle-Cell Disease (SCD) is a hematological genetic disease affecting over 25 million people worldwide. The clinical manifestations of SCD are related to hemolytic anemia and vaso-occlusion, which lead to acute and chronic pain symptoms and organ infarction. With recent advances in childhood care, high-income countries have seen SCD drift from a disease of early childhood mortality to a neglected chronic disease of adulthood. In particular, coordinated, preventive and comprehensive care for adults with SCD are largely under-resourced. Consequently, patients are left to self-manage. Mobile health (mHealth) apps for chronic diseases’ self-management are now flooding app stores. However, evidence remains unclear about the effectiveness of mHealth apps and literature indicates low user engagement and poor adoption rates. Finally, few apps have been developed for SCD patients and none encompass their numerous and complex self-management needs. OBJECTIVE This study aims at identifying factors that may influence the long-term engagement and user adoption of mHealth among the particularly isolated community of adults SCD patients living in low-prevalence, high-income countries. METHODS Semi-structured interviews were conducted. Interviews were audiotaped, transcribed verbatim and analyzed using thematic analysis. Analysis was informed by Braun and Clarke framework and mapped to the COM-B model (capability, opportunity, motivation, and behavior). Interview results were translated into high level functional requirements (FR) and non-functional requirements (NFR) to guide the development of future mHealth interventions. RESULTS 6 males and 4 females were interviewed. Patients were aged between 21 and 55 years old. 30 FR and 31 NFR were extracted from the thematic analysis. The majority of participants (8/10) was concerned about increasing their physical capabilities through the automated regulation of their blood parameters and by becoming able to stop pain symptoms quickly. Regarding the psychological capability aspects, all interviewees desired to receive trustworthy feedback on their self-care management practices. About their physical opportunities, most of respondents (7/10) reflected a strong desire to receive alerts when they would reach their own physiological limitations (i.e. during physical activity). Concerning social opportunity, most of respondents (9/10) reported wanting to learn about the self-care practices of other patients. Relating to motivational aspects, many interviewees (6/10) stressed their need to learn how to avoid the symptoms and underlined their desire to live a normal life. Finally, NFRs included inconspicuousness and customizability of user experience, automatic data collection, data shareability and data privacy. CONCLUSIONS Our findings suggest that motivation and engagement with mHealth technologies among SCD patients living in low-prevalence, high-income countries could be increased by providing features that clearly benefits them. Self-management support and self-care decision aid are patients’ major demands. Since SCD self-management requires a high cognitive load due to complex and multiple components self-care practices, natural user interfaces such as mixed reality or speech recognition should be explored. Some of the required technologies already exist but must be integrated, adapted or improved to meet SCD patients’ specific needs.
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JMIR Preprints Issom et al
Factors Influencing Motivation and Engagement in
mHealth Amongst People with Sickle-Cell Disease in
Low-Prevalence, High-Income Countries: Qualitative
Exploration of Patient Requirements
David-Zacharie Issom, André Henriksen, Ashenafi Zebene Woldaregay, Jessica
Rochat, Christian Lovis, Gunnar Hartvigsen
Submitted to: JMIR Human Factors
on: May 04, 2019
Disclaimer: © The authors. All rights reserved. This is a privileged document currently under peer-review/community
review. Authors have provided JMIR Publications with an exclusive license to publish this preprint on it's website for
review purposes only. While the final peer-reviewed paper may be licensed under a CC BY license on publication, at this
stage authors and publisher expressively prohibit redistribution of this draft paper other than for review purposes. [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
Table of Contents
Original Manuscript ....................................................................................................................................................................... 5
Supplementary Files ..................................................................................................................................................................... 39
Multimedia Appendixes ................................................................................................................................................................. 40
Multimedia Appendix 1
.................................................................................................................................................................. 40 [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
Factors Influencing Motivation and Engagement in mHealth Amongst
People with Sickle-Cell Disease in Low-Prevalence, High-Income
Countries: Qualitative Exploration of Patient Requirements
David-Zacharie IssomBSc, MSc, ; André HenriksenBSc, MSc, ; Ashenafi Zebene WoldaregayBSc, MSc, ; Jessica
RochatBSc, MSc, ; Christian LovisMD, MPH, ; Gunnar HartvigsenBSc, MSc, PhD,
Corresponding Author:
David-Zacharie IssomBSc, MSc,
Phone: +41223722601
Background: Sickle-Cell Disease (SCD) is a hematological genetic disease affecting over 25 million people worldwide. The
clinical manifestations of SCD are related to hemolytic anemia and vaso-occlusion, which lead to acute and chronic pain
symptoms and organ infarction. With recent advances in childhood care, high-income countries have seen SCD drift from a
disease of early childhood mortality to a neglected chronic disease of adulthood. In particular, coordinated, preventive and
comprehensive care for adults with SCD are largely under-resourced. Consequently, patients are left to self-manage. Mobile
health (mHealth) apps for chronic diseases’ self-management are now flooding app stores. However, evidence remains unclear
about the effectiveness of mHealth apps and literature indicates low user engagement and poor adoption rates. Finally, few apps
have been developed for SCD patients and none encompass their numerous and complex self-management needs.
Objective: This study aims at identifying factors that may influence the long-term engagement and user adoption of mHealth
among the particularly isolated community of adults SCD patients living in low-prevalence, high-income countries.
Methods: Semi-structured interviews were conducted. Interviews were audiotaped, transcribed verbatim and analyzed using
thematic analysis. Analysis was informed by Braun and Clarke framework and mapped to the COM-B model (capability,
opportunity, motivation, and behavior). Interview results were translated into high level functional requirements (FR) and non-
functional requirements (NFR) to guide the development of future mHealth interventions.
Results: 6 males and 4 females were interviewed. Patients were aged between 21 and 55 years old. 30 FR and 31 NFR were
extracted from the thematic analysis. The majority of participants (8/10) was concerned about increasing their physical
capabilities through the automated regulation of their blood parameters and by becoming able to stop pain symptoms quickly.
Regarding the psychological capability aspects, all interviewees desired to receive trustworthy feedback on their self-care
management practices. About their physical opportunities, most of respondents (7/10) reflected a strong desire to receive alerts
when they would reach their own physiological limitations (i.e. during physical activity). Concerning social opportunity, most of
respondents (9/10) reported wanting to learn about the self-care practices of other patients. Relating to motivational aspects,
many interviewees (6/10) stressed their need to learn how to avoid the symptoms and underlined their desire to live a normal life.
Finally, NFRs included inconspicuousness and customizability of user experience, automatic data collection, data shareability
and data privacy.
Conclusions: Our findings suggest that motivation and engagement with mHealth technologies among SCD patients living in
low-prevalence, high-income countries could be increased by providing features that clearly benefits them. Self-management
support and self-care decision aid are patients’ major demands. Since SCD self-management requires a high cognitive load due
to complex and multiple components self-care practices, natural user interfaces such as mixed reality or speech recognition
should be explored. Some of the required technologies already exist but must be integrated, adapted or improved to meet SCD
patients’ specific needs.
(JMIR Preprints 04/05/2019:14599)
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JMIR Preprints Issom et al
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JMIR Preprints Issom et al
Original Manuscript [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
Original Paper
Factors Influencing Motivation and Engagement in mHealth
Amongst People with Sickle-Cell Disease in Low-Prevalence,
High-Income Countries: Qualitative Exploration of Patient
David-Zacharie ISSOM, MSc1,2, André HENRIKSEN, MSc3, Ashenafi Zebene WOLDAREGAY,
MSc4, Jessica ROCHAT, MSc1,2, Christian LOVIS, MD, MPH1,2, Gunnar HARTVIGSEN, MSc,
Corresponding Author:
David-Zacharie Issom, BSc, MSc
Division of Medical Information Sciences
Geneva University Hospitals, Switzerland
Rue Gabrielle-Perret-Gentil 4
1205 Geneva, Switzerland
Phone: +41 22 379 08 16
E-mail: [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
Background: Sickle-Cell Disease (SCD) is a hematological genetic disease affecting over 25 million
people worldwide. The clinical manifestations of SCD are related to hemolytic anemia and vaso-
occlusion, which leads to acute and chronic pain symptoms and organ infarction. With recent
advances in childhood care, high-income countries have seen SCD drift from a disease of early
childhood mortality to a neglected chronic disease of adulthood. In particular, coordinated,
preventive, and comprehensive care for adults with SCD are largely under-resourced. Consequently,
patients are left to self-manage. Mobile health (mHealth) apps for chronic disease self-management
are now flooding app stores. However, evidence remains unclear about the effectiveness of mHealth
apps, and the literature indicates low user engagement and poor adoption rates. Finally, few apps
have been developed for SCD patients and none encompasses their numerous and complex self-care
management needs.
Objective: This study aims at identifying factors that may influence the long-term engagement and
user adoption of mHealth among the particularly isolated community of adult SCD patients living in
low-prevalence, high-income countries.
Methods: Semi-structured interviews were conducted. Interviews were audiotaped, transcribed
verbatim, and analyzed using thematic analysis. Analysis was informed by Braun and Clarke
framework and mapped to the COM-B model (capability, opportunity, motivation, and behavior).
Interview results were classified into high level functional requirements (FR) and non-functional
requirements (NFR) to guide the development of future mHealth interventions.
Results: Six males and four females were interviewed. Patients were aged between 21 and 55 years
old. We extracted 30 FR and 31 NFR from the thematic analysis. The majority of participants (8/10)
were concerned about increasing their physical capabilities through the automated regulation of their
blood parameters and by being able to stop pain symptoms quickly. Regarding the psychological
capability aspects, all interviewees desired to receive trustworthy feedback on their self-care
management practices. About their physical opportunities, most of respondents (7/10) reflected a
strong desire to receive alerts when they would reach their own physiological limitations (i.e. during
physical activity). Concerning social opportunity, most of respondents (9/10) reported wanting to
learn about the self-care practices of other patients. Relating to motivational aspects, many
interviewees (6/10) stressed their need to learn how to avoid the symptoms and underlined their
desire to live a normal life. Finally, NFRs included inconspicuousness and customizability of user
experience, automatic data collection, data shareability, and data privacy.
Conclusions: Our findings suggest that motivation and engagement with mHealth technologies
among SCD patients living in low-prevalence high-income countries could be increased by providing
features that clearly benefits them. Self-management support and self-care decision aid are patients’
major demands. Since SCD self-management requires a high cognitive load due to complex and
multiple component daily practices, pervasive health technologies such as wearable sensors,
implantable devices or inconspicuous conversational user interfaces should be explored to ease their
load. Some of the required technologies already exist but must be integrated, bundled, adapted or
improved to meet SCD patients’ specific needs.
Keywords: mHealth; wearables; self-management; sickle-cell disease; user engagement; adoption;
motivation; natural user interfaces; persuasive technologies
A tsunami of mHealth apps for chronic disease self-management
Mobile health (mHealth) apps are flooding the app stores, with 200 new apps each day [1]. Many
can significantly improving health outcomes [2,3] by supporting people with diverse medical [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
conditions [4]. However, little is known about app usage frequency and long-term engagement
amongst chronic patients [5,6]. Indeed, Robbins et al. [7] underlined that people who would most
benefit from such apps under-use them. To promote mHealth usage, app developers need to
comprehend what could motivate patients engagement [8]. The Theory of Reasoned Action [9,10]
demonstrates that the likelihood to engage in a specific behavior is a function of the motivation to
perform it. Authors such as Coa et al. [11] confirmed that calculating baseline motivation levels
could predict app retention rates. To influence people’s motivation through persuasion rather than
coercion, the field of persuasive technology offers novel user-centered approaches (i.e. co-creation)
The case of Sickle-Cell Disease, one of the world’s most neglected
chronic diseases
In this paper, we focus on Sickle-Cell disease (SCD). SCD is the most common monogenic blood
disorder in the world. Studies approximate 400’000 neonates per year [19,20] and between 25 and
100 million patients worldwide living with the disease [21,22]. In this study, we specifically target
populations from low-prevalence areas of high-income countries. In these settings, the disease is
under-resourced, research-derived evidence is lacking, and patients are particularly isolated [23].
Yet, SCD remains a serious illness. Hemolytic anemia and vaso-occlusive pain crises (VOCs) are the
hallmarks [24]. Patients may suffer severe and potentially lethal complications [25]. Hydroxyurea,
the preferred disease modifying treatment, is under-utilized and not effective for every patient [26].
Furthermore, the only curative option, bone marrow transplant, is largely inaccessible [27].
However, public health interventions such as regional screening programs, preventive care,
coordinated care and comprehensive care plans have been introduced in the major regions of high-
prevalence, high-income countries [28]. These interventions drastically reduced early childhood
mortality and made SCD shift to a chronic disease of adulthood [29].
Nevertheless, such programs have not been implemented widely and remain virtually absent in most
low-prevalence, high-income countries [20,30]. In addition, trained physicians are lacking and
access to specialized healthcare is suboptimal [31–33]. Indeed, SCD patients are particularly prone
to be confronted to stigmatization, to suffer unequal treatment, and to experience healthcare injustice
(i.e. perception as drug seekers) [33–35]. This often leads to mistrust between patients and
healthcare providers [36,37]. As several studies demonstrated [38–44], when patients arrive in
emergency departments, the lack of objective hematological findings and little awareness of them
makes healthcare providers suspicious of the veracity of a VOC. This distrust makes the SCD
community infamously difficult to recruit in research initiatives, hard to engage in interactions with
medical providers and lowers adherence to medical recommendations [36,45–48].
Consequently, most patients are left to self-manage, rely on poor quality healthcare, and report low
levels of Quality of Life [49,50].
Persuasive mHealth interventions to support an under-supported
To make matters worse, as several studies demonstrated [51,52], self-care management is
challenging for people with SCD. Indeed, managing the numerous potential precipitating factors of
VOCs requires high levels of self-efficacy [52–55]. Well-known triggers [56] include inadequate
diet, stress (i.e. exertional, oxidative, and psychological), infections, inflammations, acidosis,
dehydration, fatigue, chronic hemolysis, hypoxia, smoke inhalation, alcohol intoxication, pregnancy,
and environmental factors (i.e. altitude, pollution, extremes of temperature, climate, and wind speed).
In consequence, SCD self-care management practices requires patients to pay special attention to
everything. In an usual day, extreme temperatures changes, bad weather, high altitudes should be
avoided, eating behaviors should be optimized and hydration regular, exercise should be moderate [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
and not exhausting, rest should be sufficient, prescribed drugs taken, and stress managed and
avoided as much as possible [57–60]. Lastly, as in all diseases, socio-economic factors such as
education levels, occupation, or income play an important role in empowering patients [61,62].
Mobile health apps, because of their relatively low cost and wide reach, could offer a potential route
to support patients’ numerous self-management tasks [63]. Little work has been done to design
tailored mHealth interventions for the comprehensive self-management needs of people with SCD
patients [64,65]. Today, most existing apps and research focuses on medication adherence [66].
Consequently, solutions encompassing the multiple-component of SCD self-management are absent.
However, Shah et al. [67] suggested that people with SCD could be interested in such tools.
However, as with other chronic diseases, little is known about people with SCD’s mHealth apps
adoption and long-term engagement.
This paper is the last component of a study from which preliminary results have already been
published [68]. This prior publication was the first to elaborate on mHealth long-term engagement
among SCD patients. The authors explored common motivational patterns for mHealth use between
people with SCD, diabetes, and “healthy” people.
This current paper focuses on adults with SCD living in low-prevalence areas of high-income
countries. It ambitions to assess patients’ requirements in terms of value adding digital health tools
and aims at guiding the development of future mHealth interventions that people with SCD would
want to use.
Inclusion criteria
To be part of the study, applicants had to be diagnosed with SCD or be the caregiver of a person with
SCD . Participants had to be at least 18 years old and able to understand French or English. People
who had been cured (i.e. bone marrow transplantation) were excluded.
The sample for this study was a convenience sample from Switzerland and
Norway, two very low-prevalence, high-income countries. Indeed, compared to an
average in the European Union of 2.5 cases in 10’000 people [69], these two
countries have less than around 1-4 cases per 100’000 people [70–72] and
totalize approximately 100 adult SCD patients. We recruited participants through
the national patient associations’ online support groups. 64 individuals with SCD
were invited to participate. One week after the initial invitation, non-responders
were sent a reminder.
The first author (DZI), an expert-patient, conducted most semi-structured interviews using the guide
presented in Table 1. The choice of an “insider” interviewer had been done in order to build a trusted,
warm, and open rapport with the interviewees and to maximize the reception of honest and open
responses. Additionally, the interviewer was already familiar with some participants. JR conducted
the test interviews. Both interviewers have years-long interview experiences. Interviews were
conducted in locations convenient and comfortable for the participants (i.e. university, private
The interview guide was developed previously as a joint effort by all co-authors [68]. It was divided
into five themes: 1) Preliminary questions, 2) Goals, expectations and attitudes, 3) eHealth Literacy
and Data Integration 4) Wearables and sensors, and 5) Data sharing. Data saturation was reached and [unpublished, non-peer-reviewed preprint]
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determined by no new information emerging after conducting interviews with all participants.
Interviews were audiotaped and lasted around 60 minutes. Questions were open-ended and
discussions conducted flexibly. Questions were ignored or adapted relatively to the context. [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
Preliminary questions
What is most important for you in your life?
If you had access to a new health technology, which purpose or features should it have?
Goals, expectations and attitudes
What motivates/demotivates you to access online health information?
What are the most difficult things about your self-care?
What could help you become more autonomous with your self-care?
eHealth Literacy and Data Integration
Have you ever used an app that collects health data?
What factors would demotivate you from using such an app?
Wearables and sensors
What indication would you expect from wearable sensors for health self-monitoring?
What is the most valuable indication you would want from devices collecting your data?
Data Sharing
What would you share with other patients, caregivers and doctors and why?
What feedback should be provided by the system?
Data Analysis
Firstly, transcriptions of the resulting interviews were de-identified. Secondly, an inductive thematic
analysis was conducted using the guidelines and checklist from Braun et al. [73]. Codes were
extracted by reading the interviews recursively. Thirdly, emerging patterns were clustered together
and checked for variability and consistency. Themes were interpreted by reading the codes back-and-
forth. Once saturation reached, themes were mapped across the Capability, Opportunity, Motivation
and Behavior model (COM-B), hub of the Behavior Change Wheel (BCW) framework [74]. The
BCW is a fairly recent theory-driven approach that helps to design health interventions for
preventive care [74]. With the specific reading grid it provides, it allows us to identify barriers and
enablers of engagement in any intervention and in our particular case, to identify factors that, if
implemented together, may elicit the long-term engagement and user adoption of mHealth apps [75–
77]. Transcripts were organized and coded using ATLAS.ti software version (ATLAS.ti
Scientific Software Development GmBH).
Finally, in order to make it easier for software developers to comprehend, themes were categorized
into functional requirements (FRs) and non-functional requirements (NFR) [78]. In software
engineering, FRs are descriptions of the specific behaviors and functions of an information system.
They make explicit the features a software should offer to the end-user. In other words it describes [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
what the system should do [79]. NFR are descriptions of how the system should operate, which is
not linked to the functionalities. To put simply, it defines how the system should be (e.g. responsive,
intuitive, fast, secure).
Ethics and study approvals
The Norwegian Regional Committees for Medical and Health Research Ethics (REC) and the
Swiss Regional Research Ethics Committee (CCER) approved the study protocol and interview
questions. As required by committees, all participants gave informed consent before the
interview and responses were anonymized.
Participant characteristics
A total of 11 participants (7 males and 4 females) agreed to join the study. Eight patients and three
caregivers took part in the study, but one patient withdrew because of a VOC. This led to a total of 7
patients participating and 3 caregivers. Patients were between 21 and 55 years old. Lastly, 7
participants were residing in Switzerland, 3 in Norway and 1 was partly residing in Congo and in
Switzerland. Table 2 presents the demographics of the population studied.
Male 50% (n=5)
Age, years
Mean (SD)
35.6 (9.41)
Country of residence
Interview results
This section shows extracted themes from the interview data. The themes were classified into 31 FR
and 30 NFR. We organized them with the COM-B framework and illustrated them with quotations
from interviewees. Table 3 presents some of the most frequent themes that appeared during
interviews. Each theme is a FR or an NFR and belongs to a COM-B system category. In the table,
themes are sorted by COM-B system attribute, type of requirement and then by number of quotes.
The complete list is available in Multimedia Appendix 1.
"( -)$,$$!)./
Requirement Quotes COM-B system Type
Prevent crises by avoiding symptoms 12 Automatic motivation Functional
Family / Social Community Support
15 Automatic motivation Non-functional
Gain more control on disease through
daily self-care support
28 Physical Capability Functional [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
Limit management 9 Physical Capability Functional
Importance of information
17 Physical Opportunity Non-functional
Invisibility / inconspicuousness 9 Physical Opportunity Non-functional
Similarity with messaging apps 8 Physical Opportunity Non-Functional
Automatic reminders (Automatability) 5 Physical Opportunity Non-Functional
Simplicity 3 Physical Opportunity Non-Functional
Learn trigger factors 14 Psychological Capability Functional
Predict health outcomes 11 Psychological Capability Functional
Receive threshold alerts 10 Psychological Capability Functional
Feedback on self-care practices 17 Psychological Capability Non-Functional
Customizable 8 Reflective Motivation Non-Functional
Privacy 4 Reflective Motivation Non-Functional
Learn what other patients do 14 Social Opportunity Functional
Physical capability
All participants were concerned about not being able to better predict the onset of VOCs or avoid
chronic complications. Another point to consider is that many differentiated general daily self-
management skills (i.e. pain management) from preventive care (i.e. symptoms prevention). As one
patient said:
In addition, a couple of interviewees said they would wish novel technologies that automatically
regulates their hematologic parameters (i.e. hemoglobin concentration, leucocyte adherence to
vascular endothelium) [80]. Accordingly, a participant proposed a very innovative solution:
568 :9
Psychological capability
The majority of participants desired to receive feedback about SCD self-care tasks. Most emphasized
the difficulty of managing their own limits. Some wished to be warned before exceeding their
physical limitations. Namely, an interviewee proposed:
Importantly, most of participants suggested that such warnings should be detected with wearable [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
sensors, releasing their cognitive load. This can be illustrated by the following quote:
568 :9
Social opportunity
For most participants, learning about other patients’ self-care practices was a very important concern.
A majority of participants said they would like to share their own experiences on digital platforms,
like one participant said:
5!, >9
Many participants stressed the importance of social support. Some stated that mHealth could help
them communicate their needs. As one participant suggested:
5!, '89
A majority of participants reported using social media for information sharing, communication or
entertainment. Only a minority used social media to get health information. Many participants
deplored the absence of mechanisms to easily access and control the quality of information. As one
participant said:
56 >9
Physical opportunity
For several interviewees, it was crucial to receive trustworthy information. Many suggested that
wearable sensors could support this. As one participant highlighted:
$,(!A6$,$<,$$($13 4
568 :9
Several participants stated that information overload would demotivate their long-term engagement,
precising that mHealth apps should be as discreet as possible. Furthermore, most participants
recognized the potential usefulness of notifications, but only if discrete and not disturbing. As one
participant stressed:
Notwithstanding, majority of participants preferred user interfaces that are simple to use and do not
require a high learning curve. Some participants believed messaging software were the best
inspiration because of their intuitive interface. As one caregiver said:
5!, >9 [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
Reecve movaon
Importantly, a majority of participants were not using mHealth apps. One (1/10) participant reported
using Apple Health for menstruation management, hydration, and physical activity management.
Two (2/10) participants reported using Samsung Health for blood oxygen recording and physical
activity management. Another point to consider is that all participants expressed a lack of specificity
and adaptability to SCD particularities in the usual health apps (i.e. normal values for people with
SCD are diseased values for healthy people). As one participant stressed:
56E 79
Privacy issues were also a fundamental concern for most interviewees. Many feared to lose control
on their data. As one participant said:
56 >9
Finally, most participants preferred customizable information systems. As one interviewee said:
03($+$$-$-3 4
Automac movaon
The three main motivators identified were: 1) strengthen social support, 2) prevent VOC, and 3)
reduce the limitations to functioning and independent living. Regarding the first motivator,
participants wished to be able to enjoy their families and give back to their communities. As one
participant said:
Regarding the second motivator, all participants stated their desire to prevent the excruciating pain
crises. As one participant said:
More importantly, most participants stressed the importance of living as normal a life as possible and
stay in good health. This can be illustrated by the following quote:
Principal findings
To the best of our knowledge, this is the first study to explore factors that could influence the long-
term engagement with mHealth interventions of adults with SCD living in low-prevalence, high-
income countries. As a result, interviewees described how mHealth could benefit their life and
detailed what could increase their long-term engagement and motivation towards mHealth apps
usage. [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
Functional requirements in the prism of the COM-B framework
In order to maximize the chances of eliciting behavior change and engagement, as many as possible
patient requirements should be implemented. In other words, providing patients with information or
alerts is not sufficient to lead to behavior change. However, implementing simultaneously other
features such as therapeutic education (e.g. lessons, accompaniment), specifically designed wearable
devices could maximize engagement. In the following sections, we propose potential solutions and
summarize what needs to be done before being able to develop them.
Increasing Physical Capability: Regulate hematological parameters
Patients suggested how pioneering technologies such as blood regulating nanorobots could inspire
future pharmaceutical research or stimulate patient-led research initiatives [81]. By altering
hematological parameters, some innovative pharmaceutical compounds are already aligned with
patients’ suggestions [56,82]. For instance, recent clinical trials of crizanlizumab, an antibody acting
against endothelial adhesion, have shown to significantly reduce the frequency of VOCs [83].
However, this substance requires frequent intra-venous injections. Therefore, knowing the current
under-utilization of orally administrated therapies such as hydroxyurea, intra-venous therapies bring
a supplementary barrier.
In the meantime, non-pharmaceutical strategies based on information technologies could inform
patients on how to adapt their behaviors (i.e. dietary change) to alter blood parameters (i.e.
hemoglobin levels, oxygen levels, and inflammation) [84–87] while novel devices, inspired from
other clinical populations, could be created. This can be illustrated by what is happening in the
Diabetes Do It Yourself (DIY) community [88] (i.e. #WeAreNotWaiting and #DIYPS). Here,
impatient patients self-organized to hacked blood glucose monitoring system and insulin pump. They
proceed to create a system that can, after clever calculation, automatically inject the needed dose of
insulin. In comparison, one could foresee similar initiatives of DIY solutions supporting SCD self-
care practices. In particular, As a reminder, anemia and oxygen desaturation are common
complications amongst people with SCD. Since oxygen delivery by hemoglobin increases when the
amount of red blood cell and hemoglobin increases, one could imagine to create a closed-loop
system using a wearable hemoglobin meter or a blood oxygen meter. This could subsequently be
combined with an auto-injector filled with ultra-short term anemia reducing treatments [89] (e.g.
erythropoietin alpha, vitamin B12, oral folic acid, and Voxelotor [90] ).
Nonetheless, it is important to consider that a significant amount of work is required before such a
system could be created and made accurate or safe. Even if bypassing any approval from a health
authority (e.g. Federal Drugs Administration), implantable auto-injectors and specific bio-monitoring
devices would have to be engineered. Then adequate software would have to be created and be able
to analyze significant quantity of patient-generated data. Indeed, in order to be accurate and effective,
such algorithm should be trained on a high quantity of data. Afterwards, the data-driven algorithm
should be able to actuate injection of individualized doses of the adequate substances. All of this
requires a tremendous amount of skilled bio-engineering work. Another point to consider is the lower
socio-economic status of members of the SCD Community [91] and the low awareness of SCD
amongst the general population, especially in high-income, low-prevalence countries. This
mechanically leads to a scarcity of patient innovators and interested independent researchers. In
consequence, few people would be skilled to build such a specialized system.
Therefore, the SCD community could start with less invasive, less complex, but also less cognitively
unloading solutions. A simpler system could propose patients to ingest relevant drugs or dietary
supplements (e.g. anti-inflammatory, anti-oxidants), after a bio-sensor [92] (e.g. blood oxygen,
inflammation) detects a threshold. In addition, a mHealth app could send an alert. On one hand,
medication adherence would then become an issue to overcome. Additionally, if taken, the effect of
the substance would be delayed compared to a direct subcutaneous injection. [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
To summarize, the most effective solution using today’s technology would be a closed loop system
with an auto-injector and smart algorithm, functioning without any patient intervention, but, there is
still a long and challenging way to go before such a system could be built.
Increasing Physical Capability: Stop pain fast
Quick pain relief was a very important concern for most interviewees. In current VOCs self-
management, pain crises are treated at home with oral painkillers [93,94]. When oral drugs are no
longer sufficient or when complications surge, patients need to visit emergency departments to
receive acute care [38]. However, aware of the several challenges they would face in emergency
departments (i.e. long waiting times, recurrent stigma, unrelieved pain, prolonged hospitalizations),
many patients choose to postpone admission until pain becomes totally unbearable [95,96].
These challenges could partly explain why interviewees were highly motivated by mHealth
interventions that could help them relieve their pain as fast as possible. To this end, novel sensors and
software measuring pain levels through physiological signs or electrical signals [97] could be
combined with implantable pumps for intrathecal opioid therapy or subcutaneous injections [98].
However, in order to release cognitive load and avoid patients to calculate themselves how much
they should inject, smart, accurate, safe and individualized algorithms would have to be developed.
In the USA, one mHealth app helps children and adolescents with SCD to inform family, physicians
or friends about their health status [99]. Recently, innovative digital health interventions have been
deployed to facilitate emergency care process [100]. We could also imagine apps that allow sufferers
to support and motivate each other or to come together to share their experience of treatment
efficacies [101,102]. Existing mHealth apps for pain management could be adapted to SCD [103].
Finally, Virtual Reality could be used has a new option for pain relief through patient distraction
Increasing Psychological Capability: Quality feedback on self-care
The complexity of SCD self-care tasks demands various skills (e.g. high cognitive capabilities, good
disease-specific knowledge) [105]. Literature has shown that only one percent of SCD patients were
able to master them [106]. Therefore, it is easy to understand why most interviewees desired to
receive feedback on their self-care practices.
For instance, a release on the cognitive load could be partially solved by using natural user interface
(e.g. text, voice, mixed reality, augmented reality), gamification items [107] and simple data
visualization [108–111] when providing patients with targeted information. The technology exists
but needs to be integrated, adapted, bundled and improved [112–114]. Inspiration could already be
taken from existing systems for people with diabetes [115,116].
Furthermore, studies have shown that behaviors change techniques for self-management such as
health coaching could improve patient-important outcomes like self-efficacy, self-management or
medication adherence by 80% [117–120]. For instance, collecting physiological data and behavioral
data such as eating behaviors and oxygen levels then automatically reporting them to a SCD expert,
could allow the latter to provide advices directly inside the mHealth app. This would not require to
overcome many barriers since data could already be collected through fitness and wellness apps and
smartwatches. However, the biggest challenge reside in the development of smart algorithms and
methods to allow the automated interpretation of such individualized and heterogenous data[121]. In
the meantime, coaching and high quality electronic patient decision aids could be created to structure
information and help make patients make informed decisions [122–124].
Regarding disease-specific knowledge, studies have reported that many patients did not follow
medical recommendations [45], but may still search for health information online. When the quality
of information is poor, disparities in health information accessibility are created [125,126]. Frost et [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
al. [127] have already discussed the promises of online health information for people with SCD, and
Breakey et al. [128] argued that information was not always adequate nor of good quality.
Consequently, the use of artificial intelligence [129] with evaluation criteria such as Health On the
Net code [130] or the DISCERN [131] framework could facilitate provision of quality material to
patients. Finally, content constructed with the help of SCD patients and using consumer health
vocabulary, simple patient language and thesauri could improve the communication of health
information and improve adequacy [132,133].
Increasing Physical Opportunity: Receive alerts when reaching own
physical limits
Already struggling with normal life challenges, several interviewees strongly desired help to manage
the potential triggers of VOC. However, objective laboratory, clinical, hematological, biochemical,
and rheological data are not easy to self-monitor [41–43,134–136]. However, existing non-invasive
sport-related wearable devices technologies could help to monitor some markers of hematological
parameters [97]. For instance, sport watches with pulse oximeters [137], connected bottles,
oxidative stress monitor, and pH meters [138,139] could be used. To this end, smart algorithms
could be developed to provide patients with individualized feedback.
Studies have shown that poor physical functioning was frequent amongst people with SCD, making
their participation in sports difficult [140,141]. mHealth interventions specifically tailored for
physical activity support could be developed to assist people with SCD. Accurate sensors could be
integrated during the practice of physical activity (i.e. exhaustion) or after exercise (i.e. recovery)
Increase Social Opportunity: Learn what other patients do
One of the most reported motivational factors was the desire to learn from other patients. This
consideration is coherent with the stigma and isolation faced by SCD patients living in low-endemic
areas [144]. This concern is also consistent with the general lack of educational interventions [145]
and the scarcity of specialized health care providers [146]. It is well known that chronic patients
hold non-negligible experiential knowledge (e.g. effective dietary supplements and where to find
them, tips) and often share it on social networks [147–149]. However, the knowledge available on
these platforms is difficult to extract, the quality is difficult to guarantee, and the information often
unstructured, hence difficult to mine [150].
Well-funded and organized social networks such as Patients Like Me or Diabetes online communities
(e.g. TuDiabetes, glu, and Diabetes Daily) could serve as inspiration to structure patients’
experiential knowledge [151]. However, in comparison to the total SCD population, few SCD
patients use online networks. Studies from Ragnedda et al. [152] have demonstrated that socially
disadvantaged groups (e.g. gender, ethnicity, and disability) tended to use the internet less than more
advantaged groups. Conversely, a study from Issom et al. [153] suggests that SCD patients would be
willing to use such online platforms if the quality of information is ensured and if it is specifically
tailored for people with SCD.
Consequently, SCD-specific online communities such as OneSCDVoice [154] could be turned into
persuasive Q&A Social Networks ensuring the medical accuracy of patients’ shared experiences
[155]. In addition, artificial intelligence techniques could be used to help detect bad quality
information [156]. Another issue is the multitude of SCD online communities (e.g. hundreds of
Facebook groups, various websites). This spreads the information, fragments messages and
complicates access to experiential knowledge. Additionally, since people with SCD and their
caregivers have lower educational levels [62], their organizational skills and digital literacy are
reduced, subsequently hindering their access to online health information. [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
Main motivations: Live a normal life and learn to avoid symptoms
A majority of participants stated that their highest motivation would be to be asymptomatic. When
bone marrow transplant is not possible, or when hydroxyurea does not significantly reduce
symptoms frequency, alternative treatment options such as self-management interventions, chronic
transfusions or red blood cells exchange could be proposed [157]. Transfusions have shown to
significantly reduce the frequency of VOCs but require high quantities of rare phenotype blood. In
addition, phenotype matching is difficult in high-income, low-prevalence areas. Self-management
interventions require high level of self-efficacy. Electronic patient decisions aids (ePtDAs) could be
helpful. Indeed, ePtDAs have proven effective in engaging patients in self-care processes and helping
patients choose alternative treatment options [158]. To date, there are no such tools for SCD.
However, a study form Kulandaivelu et al. [159] showed that people with SCD asked for such help.
In order to push for the development of such systems, patients would require to be more aware of
novel treatment options, innovative solutions or self-management support possibilities. As a result,
they could better organize to raise awareness amongst potential payers (e.g. philanthropists and
pharmaceutical companies) who could help finance the development of such advanced solutions.
However, this remains a challenge for the socio-economically disadvantaged majority of people with
SCD [62].
Non-functional requirements
This section discusses the most frequently NFRs reported by the interviewees. Patient-important
NFRs are crucial when developing software [160]. A mismatch between them and the final product
could lead to low adoption rates and discourage app usage.
Participants clearly preferred automatic health data acquisition rather than manual data entry. To
date, there is no SCD specific mHealth intervention using automated data capture. However, data
from Electronic Health Records (EHRs), smartphone sensors, or wearable devices [161] could be
automatically collected in future mHealth interventions for people with SCD [162].
Invisibility or inconspicuousness is the ability of a system not to attract attention. As many
interviewees reported and consistently with existing literature [163], mHealth interventions are more
likely to be adopted if they clearly reduce the inconvenience and burden of self-management tasks,
while being discreet. Indeed, bulky and inelegant wearable devices could indicate to other people
that wearer has a disease. Implantable devices, discreet patches, wearables with subtle design or
integrable into daily life objects (e.g.. contact lenses, implants, and bottles) could be preferred [164–
Similarity with messaging apps
More than half of respondents called for a mHealth intervention that provides a similar user
experience than the apps they are using the most (i.e. messaging apps). Only few studies have
explored conversational designs for mHealth interventions. However, some conversational user
interfaces have been successfully used to reduce obesity or as support for mental health interventions
[168,169]. Such systems have already proven to encourage behavior changes and have high levels
of acceptability. This could encourage similar designs in future studies for SCD patients.
In the context of online health information, shareability [170] is the capacity of a patient-generated
health data to be shared with third parties. By allowing patients to share their knowledge, mHealth [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
apps could highlight valuable information that clinicians cannot offer [171] and ease social support
[172]. As for people with rare diseases, Q&A social networks [173] could be leveraged [174].
In the mHealth context, privacy is the ability of a patient to seclude information about their medical
condition [175]. Being aware of the rarity of SCD in their countries, interviewees were particularly
attentive to this issue. Participants emphasized that control should be given on what they share and to
whom. Studies for other chronic diseases showed that privacy is a very important aspect [176].
Answering this concern by taking into account regulations (i.e. GDPR and HIPAA) when designing
mHealth interventions would be key to reduce poor adoption [177]. Here, existing compliant data
management platforms with dynamic consent management [178] or personal data cooperatives
[179] could be used. As well, novel de-identification approaches could help implement this patient-
important NFR [180].
In order to maximize acceptability and inconspicuousness, most participants proposed to customize
the timing of delivery of push notifications. This finding is aligned with a study from Morrison et al.
[181] suggesting that notifications with tailored timing could enhance exposure to mHealth
interventions. However, several patients stressed their dislike of recurrent notifications (i.e. water
intake), saying it would remind them of their disease. This is aligned with a study from Bidargaddi et
al. [182] suggesting that notifications should be sent at mid-day or on week-ends. The truth probably
lies in between, where a system could propose patients to choose from a range of predefined settings.
Finally, as the interviews demonstrated, patients did not think that existing health apps where
adapted to their needs. As a result, any new digital health solution for people with SCD should
include patients in every phase of the development and focus on SCD particularities. Lastly, the
developed system should be strongly marketed as a patient-centered solution.
Lessons learned
Our findings highlighted participants’ very clear expectations towards mHealth apps. Respondents
seemed undeniably motivated to use “an invisible technology” that would accompany their self-care
practices (i.e. personalized feedback). Participants were very critical towards privacy issues and
information quality. Given the numerous and complex day-to-day self-care management tasks that
SCD patients face, and taking into account the isolation of living in low-prevalence, high-income
countries, it appears that, in order to get long-term engagement and adoption, mHealth apps must add
clear value and be particularly tailored to patients’ needs.
From requirements to successful implementation
For such a mHealth solution to have an impact once implemented, quality and perceived value must
be distinguished. However, the Digital Health world still lacks a standardized mechanism for health
app quality evaluation and certification [183]. There is no consensus for guidelines, nor clear criteria
to help recognize what a quality mHealth app is. For instance, people with Diabetes, with more than
300 apps on the Google Play Store, can easily struggle to find what apps they should download.
Although, some app curation websites [184] help end-users to find health apps ranked by quality.
However, the analytic methods vary for each website. For instance in the UK, the NHS Digital and
National Institute for Health and Care Excellence have chosen clinical effectiveness, regulatory
approval, clinical safety, privacy and confidentiality, security, usability and accessibility,
interoperability, technical stability, and change management as criteria. Other websites could use
other criteria, for instance apps which there is published medical evidence.
As a result, app developers should spend energy to meet patient-important requirements and thrive to [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
meet as many quality indicators as possible. This can be summarize by the following steps:
-Take a patient-centered approach:
oMake sure the app improve patients outcomes;
oBeing validated in terms of clinical outcomes.
-Offer a real solution to self-management problems faced by patients:
oMeet patients requirements;
oDo better than any alternative and cheaper;
-Be compatible with the existing healthcare information systems infrastructure:
oImplement interoperability standards by design;
oAnswer privacy and safety requirements;
Key points
Nevertheless, it is clear that there is no “invisible technology” yet to support SCD patients’ self-
care practices. However, the various technological pieces needed to build such an ubiquitous
system are largely available today. Nonetheless, they are scattered, not bundled nor adapted to the
specificity of SCD. The following steps summarize what needs to be done prior implementing the
key requirements:
Disease-modifying functionalities (i.e. pain relief, regulation of hematological variables)
to reduce poor adoption rates by providing patients with:
ocontinuous blood oxygen meters;
ospecific auto-injectors;
osmart software that includes algorithms able to manage and make sense of the big
datasets generated.
Targeted information (e.g. alerts when approaching limits, access to other patients’ self-
care practices) could influence long-term engagement by providing patients with:
oknowledge adapted to their health literacy levels;
ouser interface matching their digital literacy levels;
oinformation adapted to their disease-specific knowledge and if necessary offer
assistance to interpret the information.
Controlling data flows (e.g. shareability, privacy, quality information) could influence
patients’ motivation to start using apps by:
ostoring data on personal data cooperatives;
oimplementing existing interoperable standards;
ousing algorithms and hire content moderators (e.g. physicians, expert patients) to
monitor content creation and the quality of information.
Future plans
Given the complexity of SCD self-management, supporting patient-important self-care needs with
mHealth interventions will be challenging. However, such systems will be key to fill the gaps in
healthcare delivery service. Further work is needed to implement patient requirements. Prioritization
could be done using participatory approaches. Tools such as the APEASE criteria, a set of
benchmarks from the BCW framework, can be helpful to decide what content should be included and
what intervention delivery strategies should be used. In addition to proven added value with the
effective implementation of patient-important requirements, other non-functional requirements
include affordability, practicability, effectiveness and cost-effectiveness, acceptability, side-effects,
safety and equity.
In addition, DELPHI surveys [185], a technique consisting of seeking experts’ views to obtain a [unpublished, non-peer-reviewed preprint]
JMIR Preprints Issom et al
level of agreement by transforming opinion into group consensus, could be sent to expert-patients
Finally, motivational factors will have to be assessed in the long run to maintain high user
engagement levels. This could be done using tailored frameworks for mHealth engagement analysis
such as the AMUsED framework [187].
Strengths and limitations
The study has a number of strengths and limitations. Using the COM-B model to identify
motivational factors is a relatively recent approach in the field of mHealth. However, in this
example, the lens offered by the model was helpful to gain a full picture of patients’ motivations.
Additionally, this model allowed us to classify patient requirements into explicit categories and
helped us to discuss potentially useful technologies to meet patients’ unmet needs.
The age range of the sample size was large, consequently, younger patients may feel more
comfortable using digital health interventions than older ones. Additionally, our sample size
approximates 10% of the adults with SCD living in the selected low-prevalence, high-income
countries. Also, because of the notorious difficulty to enroll SCD patients in studies, we were
surprised by this relatively high response rate. The fact that the main interviewer was an expert-
patient may have eased enrollment and facilitated trust building but also added a bias.
Finally, the study results could be affected by the recruitment criteria. Since participants were
selected inside an active SCD community, it is possible that those who volunteered to be interviewed
were more in search for new coping solutions and had more positive views about the disease than
those who declined or did not replied.
Since interviewees were particularly explicit in what could benefit them, this study provides initial
insights on how to build mHealth apps that could engage particularly isolated SCD populations. The
use of qualitative methods enabled in-depth exploration of interviewees responses. As well, the BCW
and its hub, the COM-B model could be used as a robust framework to inform the development of
future persuasive technologies for people with SCD. As patients highlighted, future research should
focus on supporting their self-care decisions. Exploring the integration, adaption or improvement of
highly adopted mHealth interventions for other chronic diseases could be helpful.
This study would not have been possible without the support of the Swiss Sickle-Cell Association
and the Norwegian Sickle-Cell Association. The authors thanks Henna Martilla, Gerit Pfühl, Martin
Mikalsen and Keiichi Sato for their input in the earlier phases.
Authors contributions
DZI, AH, AZW, CHL and GH conceived the qualitative study. DZI conducted the interviews with the
support from JR. DZI wrote the manuscript and subsequent revisions were undertaken with the
support and input from all authors. DZI, AH, and AZW created the interview guide, with support
from all authors. DZI conducted the interviews. DZI coded the data and co-analyzed them with the
support of AZW and AH. All authors approved the final manuscript.
Conflicts of Interest
None declared. [unpublished, non-peer-reviewed preprint]
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BCW: Behavior Change Wheel
COM-B: Capability, Opportunity, Motivation, Behavior
ePtDA: Electronic Patient Decision Aid
SCD: Sickle-Cell Disease
VOC: Vaso-occlusive crisis
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Background Deoxygenated sickle hemoglobin (HbS) polymerization drives the pathophysiology of sickle cell disease. Therefore, direct inhibition of HbS polymerization has potential to favorably modify disease outcomes. Voxelotor is an HbS polymerization inhibitor. Methods In a multicenter, phase 3, double-blind, randomized, placebo-controlled trial, we compared the efficacy and safety of two dose levels of voxelotor (1500 mg and 900 mg, administered orally once daily) with placebo in persons with sickle cell disease. The primary end point was the percentage of participants who had a hemoglobin response, which was defined as an increase of more than 1.0 g per deciliter from baseline at week 24 in the intention-to-treat analysis. Results A total of 274 participants were randomly assigned in a 1:1:1 ratio to receive a once-daily oral dose of 1500 mg of voxelotor, 900 mg of voxelotor, or placebo. Most participants had sickle cell anemia (homozygous hemoglobin S or hemoglobin Sβ⁰-thalassemia), and approximately two thirds were receiving hydroxyurea at baseline. In the intention-to-treat analysis, a significantly higher percentage of participants had a hemoglobin response in the 1500-mg voxelotor group (51%; 95% confidence interval [CI], 41 to 61) than in the placebo group (7%; 95% CI, 1 to 12). Anemia worsened between baseline and week 24 in fewer participants in each voxelotor dose group than in those receiving placebo. At week 24, the 1500-mg voxelotor group had significantly greater reductions from baseline in the indirect bilirubin level and percentage of reticulocytes than the placebo group. The percentage of participants with an adverse event that occurred or worsened during the treatment period was similar across the trial groups. Adverse events of at least grade 3 occurred in 26% of the participants in the 1500-mg voxelotor group, 23% in the 900-mg voxelotor group, and 26% in the placebo group. Most adverse events were not related to the trial drug or placebo, as determined by the investigators. Conclusions In this phase 3 randomized, placebo-controlled trial involving participants with sickle cell disease, voxelotor significantly increased hemoglobin levels and reduced markers of hemolysis. These findings are consistent with inhibition of HbS polymerization and indicate a disease-modifying potential. (Funded by Global Blood Therapeutics; HOPE number, NCT03036813.)
Full-text available
Digital health solutions continue to grow in both number and capabilities. Despite these advances, the confidence of the various stakeholders — from patients and clinicians to payers, industry and regulators — in medicine remains quite low. As a result, there is a need for objective, transparent, and standards-based evaluation of digital health products that can bring greater clarity to the digital health marketplace. We believe an approach that is guided by end-user requirements and formal assessment across technical, clinical, usability, and cost domains is one possible solution. For digital health solutions to have greater impact, quality and value must be easier to distinguish. To that end, we review the existing landscape and gaps, highlight the evolving responses and approaches, and detail one pragmatic framework that addresses the current limitations in the marketplace with a path toward implementation.
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Background: Although traditional forms of therapy for anxiety-related disorders (eg, cognitive behavioral therapy, CBT) have been effective, there have been long-standing issues with these therapies that largely center around the costs and risks associated with the components comprising the therapeutic process. To treat certain types of specific phobias, sessions may need to be held in public, therefore risking patient confidentiality and the occurrence of uncontrollable circumstances (eg, weather and bystander behavior) or additional expenses such as travel to reach a destination. To address these issues, past studies have implemented virtual reality (VR) technologies for virtual reality exposure therapy (VRET) to provide an immersive, interactive experience that can be conducted privately and inexpensively. The versatility of VR allows various environments and scenarios to be generated while giving therapists control over variables that would otherwise be impossible in a natural setting. Although the outcomes from these studies have been generally positive despite the limitations of legacy VR systems, it is necessary to review these studies to identify how modern VR systems can and should improve to treat disorders in which anxiety is a key symptom, including specific phobias, posttraumatic stress disorder and acute stress disorder, generalized anxiety disorder, and paranoid ideations. Objective: The aim of this review was to establish the efficacy of VR-based treatment for anxiety-related disorders as well as to outline how modern VR systems need to address the shortcomings of legacy VR systems. Methods: A systematic search was conducted for any VR-related, peer-reviewed articles focused on the treatment or assessment of anxiety-based disorders published before August 31, 2017, within the ProQuest Central, PsycINFO, and PsycARTICLES databases. References from these articles were also evaluated. Results: A total of 49 studies met the inclusion criteria from an initial pool of 2419 studies. These studies were a mix of case studies focused solely on VRET, experimental studies comparing the efficacy of VRET with various forms of CBT (eg, in vivo exposure, imaginal exposure, and exposure group therapy), and studies evaluating the usefulness of VR technology as a diagnostic tool for paranoid ideations. The majority of studies reported positive findings in favor of VRET despite the VR technology's limitations. Conclusions: Although past studies have demonstrated promising and emerging efficacy for the use of VR as a treatment and diagnostic tool for anxiety-related disorders, it is clear that VR technology as a whole needs to improve to provide a completely immersive and interactive experience that is capable of blurring the lines between the real and virtual world.
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Background: Pain is a common condition with a significant physical, psychosocial, and economic impact. Due to enormous progress in mobile device technology as well as the increase in smartphone ownership in the general population, mobile apps can be used to monitor patients with pain and support them in pain management. Objective: The aim of this review was to assess the efficacy of smartphone or computer tablet apps in the management of patients with pain. Methods: In December 2017, a literature search was performed in the following databases: MEDLINE, EMBASE, CINAHL, Cochrane, and PsycINFO. In addition, a bibliography search was conducted. We included studies with at least 20 participants per arm that evaluated the effects of apps on smartphones or computer tablets on improvement in pain. Results: A total of 15 studies with 1962 patients met the inclusion criteria. Of these, 4 studies examined the effect of mobile apps on pain management in an in-clinic setting and 11 in an out-clinic setting. The majority of the original studies reported beneficial effects of the use of a pain app. Severity of pain decreased in most studies where patients were using an app compared with patients not using an app. Other outcomes, such as worst pain or quality of life showed improvements in patients using an app. Due to heterogeneity between the original studies-patient characteristics, app content, and study setting-a synthesis of the results by statistical methods was not performed. Conclusions: Apps for pain management may be beneficial for patients, particularly in an out-clinic setting. Studies have shown that pain apps are workable and well liked by patients and health care professionals. There is no doubt that in the near future, mobile technologies will develop further. Medicine could profit from this development as indicated by our results, but there is a need for more scientific inputs. It is desirable to know which elements of apps or additional devices and tools may improve usability and help patients in pain management.
2070 BACKGROUND Patients with sickle cell disease (SCD) often complain of long wait times in the emergency department (ED) when they present for treatment of pain. It is known that African-Americans in general often have longer ED wait times than other patients. Because patients with SCD in the US are much more likely to be African-Americans, it can be difficult to separate the effects of disease vs. race on SCD patient wait times. We attempted to disentangle these effects by examining a national sample of ED visits in the US. METHODS We examined data from the National Hospital Ambulatory Medical Care Survey (NHAMCS), which is conducted annually by the National Center for Health Statistics. Weights are provided to allow for the estimation of national level statistics. We examined NHAMCS data from 2003 through 2008. Our outcome variable was waiting time (in minutes) from ED arrival to being seen by a physician. Our primary independent variable was disease status, comparing patients with SCD to those with long bone fracture (LBF) and all other patients. We used a two-part approach for our analyses. First, we examined the association of disease status with wait times among all patients in the sample. We then restricted all analyses to the African-American sample of patients. Because of the highly right-skewed nature of the outcome variable, multivariable regressions were conducted using generalized linear models assuming a gamma distribution and a log link function. All analyses accounted for the complex design of the survey. RESULTS An estimated 553,943,439 ED visits occurred over the study period, with LBF patients accounting for 1.1% of those visits (n = 5,929,085), and SCD patients accounting for 0.2% of those visits (n = 1,142,078). SCD patients were significantly younger than all non-SCD patients (27.6 vs. 36.9, p < 0.001), and were more likely than LBF patients and all other patients to be male (55% vs. 51% vs. 46%, p < 0.001), and to have Medicaid (55% vs. 16% vs. 24%, p < 0.001). SCD visits were more likely than LBF and all other visits to have severe pain (scores of 7 to 10) at triage (54% vs. 32% vs. 19%, p < 0.001), and more SCD visits (70%) compared to LBF visits (58%) and all other visits (56%) received high priority triage recommendations of level 1 or 2 (<15 minutes to 1 hour) (p = 0.005). Bivariate analyses found that SCD patient wait times were 25 minutes longer than LBF patients, and 13 minutes longer than all other patients (mean wait = 67 minutes vs. 42 minutes vs. 54 minutes, p < 0.0001). Accounting for the skewness in the data, waiting times for SCD patients were found to be 59% longer than LBF patients (p < 0.001), and 25% longer than all other patients (p = 0.03). After adjustment for age, sex, insurance, and race, SCD wait times remained 32% longer than LBF patients (p = 0.007), and 8% longer than all other patients, though the latter result was no longer significant (p = 0.463). SCD wait times were 38% longer than LBF patients after additional adjustment for assigned triage level and presenting level of pain (p = 0.001). After restricting all analyses to the African-American patient sample, SCD patients were still found to wait 51% longer than LBF patients after adjustment for age, sex, insurance, assigned triage level, and presenting level of pain (p = 0.001). CONCLUSIONS Compared to patients with LBF, and all other patients, SCD patients were consistently found to have longer wait times to see physicians in the ED. The disparity between SCD patients and all other patients appeared to be explained by patient race. Nevertheless, analyses restricted to African-American patients still found a significant disparity between SCD patients and patients with LBF. Our findings suggests that both the black race of SCD patients, and their status as SCD patients, contribute to longer wait times in the ED compared to other patient populations. Disclosures No relevant conflicts of interest to declare.
A gap in trust between patients with sickle cell disease (SCD) and medical providers is well recognized, largely originating from repetitive requests for opioid analgesics. Although expert clinical care guidelines in sickle cell disease are available, they rarely address measures by which this endemic gap in trust may be narrowed to fulfill the goals of medicine. We hypothesized that an increased familiarity with the patient as an individual through exposure of physicians to first-person narratives of the life-world of SCD may allow reformulation of perceptions and narrow the gap between physicians and patients. In a pilot study, extended first-person narratives of the illness experience elicited from patients with SCD with a history of recurrent hospitalizations (n=10) point to the individualized impact of pain, illness and stigmatizing disruptions in life-building efforts imposed by SCD together with the recurrent conflicts with medical caregivers, particularly after transition from pediatric to adult medicine. Patient-elicited narrative fragments such as "You are constantly fighting with people who are supposed to be making us feel better", "You don't know me, I am a church boy!" and "Put down your microscopes and talk to us" reveal the yearning by patients for individual integrity to be acknowledged, absorbed and interpreted by physicians. Additional narrative fragments such as "I hate myself. I hate my life", "Why am I broken?", "you don't want to be the girl with jaundice", "I had to leave work for 2 months because I was hospitalized, it killed me, it killed me", "you never know when it's going to be the last day, the last moment", exhibit the woundedness and fragility of existence experienced by patients. Narratives elicited separately from physicians caring for SCD (n=5) reveal anxiety, fear and distrust of the reported pain experience and opioid requests, and point toward deeper schisms that disparities may define: "I was very scared when I took care of my first sickle cell patient", "I came in with a bias already", "a similar frequent flier who had the same kind of behavior", "you have to be careful with empathy, people can take advantage of it", "I am not an enabler", "My grandfather told me about these people". Yet when the same physicians were invited to read and reflect on transcripts of the life-world stories of patients, reshaped perceptions of the stigmatized patient are revealed; "without knowing the story, you can't put it all together", "this would change the way I view treating them", "I will have more patience now" as examples. However, "Why can't they listen to our story? It could help them to be better patients," suggests that additional studies of the mutual intersections of patient and physician narratives are warranted and that these can offer insights and pathways toward mitigating distrust and conflict between medical care providers and patients with SCD. Disclosures No relevant conflicts of interest to declare.
Introduction: Sickle cell disease (SCD) is a chronic disease that can cause significant complications including acute chest syndrome, recurrent pain and stroke. Current guidelines for the use of chronic transfusions include primary and secondary prevention of stroke. Although there is currently limited support for the routine use of transfusions for acute vaso-occlusive crisis (VOC), there has been increasing use of chronic transfusions as an alternative treatment for recurrent VOC. Moreover, there is evidence that patients on chronic transfusions have less VOC. We sought to review the outcomes of patients at our institution placed on chronic transfusions for non-stroke related indications. Methods: We performed a retrospective cohort study to summarize clinical and nonclinical features of sickle cell patients on transfusions for non-stroke related complications. Demographic, clinical, and laboratory information were summarized. Acute care events per month were calculated for both the year prior and up to one year following initiation of chronic transfusions. Acute care events were defined as emergency department visits or hospitalization. Results: Of the 378 patients with SCD treated in the pediatric specialty clinic, there were 21 patients being either chronically transfused or exchange transfused. Six (20%) of these patients were initiated on chronic blood transfusions (CBT) for recurrent pain crisis (median age = 12, range 8 to 17). One of these patients also had suspected hepatic sequestration. All patients were type SS and had been treated with hydroxyurea (HU) for an average length of 6.5 years (range 1 to 12 years) at a mean dose of 25 mg/kg (SD 4) prior to initiation of CBT. All patients continued on HU during chronic blood transfusions. Patients were on chronic transfusions for a median of 11 months (range 3 to 58 months) with mean %S while on transfusions of 39.6% (SD 10). Patients were transfused on average every 5 weeks (range 4 to 6 weeks). Following initiation of transfusions, 50% were started on chelation based on criteria of having a ferritin >1000 ng/mL. Mean peak ferritin was not significantly increased in the year following the start of CBT (513 ng/mL ± 343 vs. 1260 ± 934, p=0.13). There was one new alloantibodies (anti-Jk) reported following initiation of CBT, which developed within 3 months. Acute care visits per month were significantly higher in the year prior as compared to after initiation of chronic blood transfusions (1.04 ± 0.45 vs. 0.28 ± 0.22, respectively; p=0.006) (Figure 1). Discussion: We found that patients started on chronic transfusions for pain crisis had a non-significant increase in peak ferritin and a significant reduction in acute care visits. Prior to CBT, all patients had been initiated on hydroxurea (mean dose of 25mg/kg) in an attempt to treat recurrent VOC. However, following therapy for an average of 6.5 years, patients were placed on CBT to prevent further acute care visits and reduce morbidity. All patients were continued on HU while on CBT with no dose adjustment or effort to titrate to maximum tolerated dose. While on CBT, patients had a mean %S of 40%, which is higher than the recommended goal of 30% for stroke related indications. Importantly, despite the higher mean %S, there was a drastic and significant decrease in acute care visits. It should be noted that although only three patients (50%) of patients were placed on chelation, the remaining three had been on transfusions for less than or equal to 6 months and likely to require chelation with continued therapy. The expected elevated ferritin highlights the difficulty in long-term treatment with chronic transfusion and risk for eventual iron overload. The balance between the clinical benefit and potential long-term complications leads to individual assessment of the risks and benefits prior to initiation of chronic transfusions for VOC. These results advocate for the use of prospective studies to evaluate the role for chronic transfusions for non-stroke related indications in SCD. Figure 1 Figure 1. Disclosures Shah: Novartis: Speakers Bureau.
The complexity and rise of data in healthcare means that artificial intelligence (AI) will increasingly be applied within the field. Several types of AI are already being employed by payers and providers of care, and life sciences companies. The key categories of applications involve diagnosis and treatment recommendations, patient engagement and adherence, and administrative activities. Although there are many instances in which AI can perform healthcare tasks as well or better than humans, implementation factors will prevent large-scale automation of healthcare professional jobs for a considerable period. Ethical issues in the application of AI to healthcare are also discussed.
Personalized health research depends on aggregated sets of personal data from millions of people. Given that personal data can be copied, individuals are entitled to copies of their data and individuals are the ultimate aggregators of all their personal data, citizens are elevated to new roles at the center of health research and a novel personal data economy. There, citizens, not some multinational company, control the use of and benefit from the intellectual and economic value of these data. Here, I show that democratically controlled nonprofit personal data cooperatives provide a governance and trust framework for data sharing and data donation. They also provide a means of attaining improved precision health and a digital society in which socio-economic asymmetries can be balanced.