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Thal Happy! A thalassemia coach in your pocket
Background and Problem Statement
The management of beta thalassemia major is complex. It includes ensuring adequate
transfusions, monitoring of organ iron load, ensuring regular iron chelation therapy, and
monitoring and management of complications. Optimization of a transfusion regimen and iron
chelation therapy based on organ iron load significantly reduces complications and improves
survival. A comprehensive care team consisting of multiple healthcare providers collaborate with
the patient with the goal of achieving best outcomes. Thus optimal care is a complex set of
appointments, laboratory testing, radiological investigations, and, especially important,
adherence to medications and appropriate drugspecific monitoring. Coordination of these key
tasks, lack of communication, and the ever increasing amount of information can be
overwhelming and are significant stressors for the thalassemia patient.
In order to live healthy and full lives, people with thalassemia major must find a way to manage
chronic transfusion appointments, multiple specialist appointments and multiple medication
regimens over the course of a lifetime. Since research on chelation medications and
management of this chronic illness is ongoing, it is also in a patient’s best interest to stay
informed of the newest updates in thalassemia care. Patients are expected to remember a large
number of details about their medication adherence, appointments attended, and symptoms
experienced in order to report this information to multiple medical providers who may or may not
communicate with each other. In order to reduce this burden on the patient it is important for a
tool to be developed that can streamline the process of recording data and which allows
thalassemia patients to consistently communicate accurate information to all of their medical
Currently, there is no smartphone app that is personalized to the experience of people living with
thalassemia. Many medication management apps do not even list common chelators in their
medication lists. And most of the medication management apps only focus on helping patients to
remember to take their medications only one aspect of managing a chronic illness such as
thalassemia. Many medical providers are still unfamiliar with best practices in caring for
thalassemia patients, thus it is even more important for patients of all ages to be well informed
about their medical care and to be able to communicate their medical information effectively.
The proposed app, Thal Happy, aims to empower thalassemia patients by giving them the tools
to record all information about their thalassemia management in one place and to then easily
share this information with their providers. Another important purpose of this app is to continue to
inform patients about the newest updates in thalassemia care. Existing thalassemia programs
have found that as thalassemia patients move through childhood and into adolescence and
adulthood they often have gaps in knowledge about their medical care and therefore take a less
active role in the management of their health 1
. Patients who are uninformed about why providers
prescribe certain medications, for example, and require certain procedures are less adherent
because they do not see the value in these recommendations. It is our hope that Thal Happy will
empower people living with thalassemia to become or remain adherent to their medications,
appointments and procedures by making it easier to record medical information, share this
information with providers and remain informed about medical advances in the field of
Goals for Patient Outcomes
● Improving Adherence to Treatment Plans: Poor adherence to chelation therapy is a
significant barrier to good clinical outcomes. Poor adherence to monitoring of chelator
toxicity has the potential for adverse outcome. By providing a log and reminders for
medications, laboratory investigations, and appointments we aim to improve patient
● Educating and Empowering Patients: By providing a tool for patients to enter their
medical information, schedule appointments, and track their medical progress, we hope
to empower patients to take control of their disease and their life. Education is power, and
by facilitating transfer of information and education on thalassemia, we aim to improve
patient engagement and involvement in their care. The Media & Resource section of this
app allows patients to keep up to date on the most current developments in the field of
thalassemia. This will empower patients by giving them access to the most current
research on chelation medications, gene therapy trials, etc which will give them the
knowledge they need to make decisions about their care with their providers. It is clear
that patients are more adherent to courses of care that they have an active role in
choosing. Many thalassemia patients find it empowering to develop relationships with
other people living with thalassemia. The app will give patients the opportunity to easily
connect with thalassemia organizations, receive updates from these organizations and
become an active member of the thalassemia community.
● Facilitating Caregiver Involvement: Thal Happy can be used by a parent on behalf of a
child, later transitioning so that the patient may use it directly. Caregivers can also be
kept informed via the data reporting feature which sends reports by email in PDF. These
data include adherence tracking, test result scores, and symptom reporting.
● Promoting Communication with Clinical Teams: The app allows patients to keep all
the information necessary for the management of their thalassemia care in one place.
This will make it possible for patients to easily pull information from the app during
appointments with providers instead of relying on their memory to communicate
symptoms, adherence, appointments attended, and questions. It is common for patients
to feel overwhelmed in a clinical environment and when communicating with medical
providers, especially providers they are not familiar with. This can lead to unproductive
medical appointments because patients can forget questions they had prior to the
appointment or feel uncomfortable asking questions. If patients feel prepared for
appointments and have accurate answers to providers’ questions about their adherence
and symptoms this will empower them to become active members of their care team.
● Serving Transitions of Care: The app helps to coordinate information around transitions
between providers. One particular transition is that of pediatric care to adult care. The
app acts as a tailored Personal Health Record (PHR) which can export data to EHR
systems. This app allows adolescents and young adults to gain independence in
managing their chronic illness in a simple and rewarding way. The “Ask an Expert”
feature also gives adolescents and young adults the opportunity to ask questions about
their care that they may not be comfortable asking providers in person or with caregivers
present during appointments. By housing all of their medical information in one place, this
app will allow young adults to walk into new adult provider offices with confidence,
knowing they have answers to their provider’s questions.
● Accessibility: Thal Happy is not targeted to any specific community, age group, or
country. Rather, it aims to serve the spectrum of populations with the disease. It
supports multiple languages and platforms, and strikes a balance of fun and efficiency
that is appreciated by all ages. Dates and phone number formats are internationally
● Interoperability: Data can be copied to PHR and EHR systems using the recently
recognized BlueButton+ format. Or it can be saved as a PDF and emailed to a family
member or provider.
● Game Design Principles: The app breaks a complex set of self management tasks into
small, achievable chunks and provides reinforcement at that level. It also shows
progress, and allows the user to take control over their regimen.
● Behavior Theory: The app applies some aspects of the Health Belief Model (HBM): to
educate the user in order to promote proper perceived risks and severity; and to use
cues to remind users of when behavior is needed.
● Privacy of Data: Data is stored locally on devices, not on centralized storage, so the
app remains fast and responsive and does not rely on network connectivity or speed.
Furthermore the data is confined to the local device.
Product Development & Business Development
● CoDesign Approach: Several prototype versions will be created and put in the hands
of volunteer patients and providers in order to collect feedback. The formation of the
group of focus group participants will use the team’s connections in the academic health
system and thalassemia patient support groups all team members work with Penn
Medicine (the University of Pennsylvania Health System including 3 hospitals and many
● Lean Development Processes: The app uses an iterative, rapid development process
to develop multiple prototypes to be tested and refined.
● Distributed and Cloud Based Infrastructure: Prototypes are developed on Amazon
Web Services for availability at very low cost. And the app is distributed to app stores
which are able to handle fluctuation in demand.
● Business Model for Sustainability: Due to the distributed and lowtouch design of Thal
Happy the cost of maintaining is kept low. This cost can be covered by charging patients
$5 each to install via app stores.
● First Marketing Strategy: The position of the team as academically based will make
Thal Happy attractive to the various providers for these patient populations across the
world. If, through an effectiveness study, we demonstrate that this app improves patient
engagement and adherence, then providers have an incentive to “prescribe” the app to
all thalassemia patients.
● Second Marketing Strategy: If demonstrating effectiveness for improved patient
outcomes, this app is also suitable for recognition by nonprofit organizations who work
with thal patients. They would then help to publicize the app and include it in educational
and outreach programs.
● Next Step: We propose evaluating effectiveness through a pilot study of thalassemia
patients at multiple Philadelphia health systems randomized to use this app.
Features in Initial Prototype
● Home Screen: One quick place to view all important reminders and To Do items. This
view also gives uptodate medication adherence progress and serves as a quick way to
access the most important information to communicate during ED visits.
● Medication Adherence: Text message reminders customized to each patient's
medication schedule, visual weekly medication adherence tracking, the ability to record
symptoms along with medication adherence data.
● Info Card for Emergency Department: With one click patients or anyone accompanying a
patient to the ED is able to communicate all of the most important information necessary
to getting the best care for Thalassemia patients even when providers are not familiar
with treating Thalassemia patients. This feature will include information such as fever
precautions in Thalassemia patients and custom information about each patient’s
transfusion requirements, antibodies, medication dosage and frequency.
● My Log: Thal Happy is designed to accommodate all of the medications, lab tests and
procedures that Thalassemia patients typically need. Thal Happy organizes this
information in a way that makes it easy to document changes in medications, symptoms
and results of procedures and tests.
● Appointments: Thal Happy gives patients the opportunity to easily schedule
appointments and communicate with providers because patients are able to contact
providers with the click of a button on one screen. The app also allows patients to send
custom appointment reminders through text messages and link appointments to their
existing phone calendar.
● Care Team: Thal Happy stores all of a patient’s Care Team information in one place,
serving as a reminder to regularly schedule visits with all providers and making it easier
to access provider contact information to schedule, cancel and reschedule appointments
as well as communicate important information.
● Media & Resources: Thal Happy houses the most uptodate and reliable information
about developments in Thalassemia care and serves as a connection to the
Thalassemia community. Patients are able to find answers to their questions through the
Ask an Expert feature and connect with the Cooley's Anemia Foundation all in one place.
● Thalassemia Center Locator: This feature gives contact info and video introductions to
each of the Comprehensive Thalassemia Centers. This allows patients to plan in
advance when moving from one location to another or when transitioning care from
pediatric to adult providers.
● Download: Data Download or PDF snapshot allows patients to easily pull information
from Thal Happy to share with providers and caregivers so that all members of their Care
Team receive accurate and uptodate information about their medication adherence, lab
results, appointment outcomes and future appointments, procedures and tests.
● Language Selection: Thal Happy gives patients the ability to select from a list of
languages, which makes it possible for Thalassemia patients around the world to use this
● Damien Leri, MS.Ed, MPH [c] is the CEO and lead developer for Big Yellow Star
(http://bigyellowstar.com). This health information technology company provides
consulting and application development and integration services for Penn Medicine,
Children’s Hospital of Philadelphia, and the Camden Health Coalition, among others.
● Genevieve Cattanea, MSW, LSW is currently the Social Worker for the HeartStrong
Program (Automated Hovering to Improve Medication Adherence among Myocardial
Infarction Patients) at the University of Pennsylvania’s Center for Health Incentives and
Behavioral Economics at the Leonard Davis Institute. Past experience in health care
includes her work as the Thalassemia Program Social Worker in the Division of
Hematology at the Children’s Hospital of Philadelphia. Prior to joining CHOP Hematology
in December 2010, she worked as the Social Work Intern at CHOP’s Regional Autism
Center. She holds a Master’s of Social Work degree from the University of Pennsylvania.
● Farzana Sayani, MD MSc is an adult hematologist with experience in the care and
management of patients with thalassemia. She is currently the program director of the
adult comprehensive thalassemia clinic at the Penn–CHOP Blood Center for Patient
Care and Discovery at the Hospital of the University of Pennsylvania. Her training
includes a clinical fellowship in Hemoglobinopathies from the Hospital for Sick Children
(Toronto, Canada) and a Master’s in Haemoglobinopathies (University College London,
UK). She was the lead author for the ‘Guidelines for the Clinical Care of Patients with
Thalassemia in Canada’. Her areas of interest include iron overload, complications of iron
overload, and pediatricadult transition care for patients with thalassemia.
● Lauren HalldenAbberton is the Program Coordinator at the Center for Public Health
Initiatives at the University of Pennsylvania, where she manages communications and
marketing in support of the center’s programming. Her work includes print and web
design, data visualization, and presentation design in a variety of media. She received
her BFA from the Moore College of Art and Design and is currently a Graphic Design
Certificate student at the University of Pennsylvania.
1. Levine L, Levine M. Health care transition in thalassemia: pediatric to adultoriented care. Ann
N Y Acad Sci. 2010. 1202: 2447.