Background: Sickle cell disease is the most prevalent inherited
blood disorder in the world. It can lead to many life-threatening
chronic issues and comorbidities. The prevalence of the disease
is more important in developing countries. In developed countries,
self-help solutions for chronic patients are rising and are
increasingly used. E-Health tools become companions for selfmanaging
patients.
Problem: The use of mobile applications to support the selfmanagement
of chronic diseases has proved to improve the
quality of life of patients. We try to identify the opportunities for
people with Sickle Cell disease created by the use of such tools
and investigate what are the patients’ main concerns.
Method: We have performed a literature review to under-stand
the main challenges of the disease. Additionally, we sent a questionnaire
to patients to identify their needs. Finally we researched
how the potential use of eHealth tools and Information
and Communication Technologies based solutions
could resolve some issues.
Results: The literature review has shown a low number of contributions
but these studies had mainly positive outcomes. The
results of the questionnaire have shown a significant dissatisfaction
from patients about the health care system taking care
of them. Patients’ responses also showed their will to improve
their general knowledge about the disease and their interest in
using e-Health tools for improving their quality of life.
Sickle cell disease; eHealth; mHealth; self-care; self-management; self-monitoring; self-help devices; wearable devices; quantified self; public health informatics; global health; uHealth
[1] Roberts I, de Montalembert M. Sickle cell disease as a par-adigm of immigration hematology: new challenges for hematologists in Europe. Haematologica 2007;92:865–71.
[2] France E, Noire EA. La drépanocytose 2011.
[3] Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet 2010;376:2018–31.
[4] Prevalence of rare diseases : Bibliographic data n.d. Or-phanet :1–29.
[5] de-Graft Aikins A, Unwin N, Agyemang C, Allotey P, Campbell C, Arhinful D. Tackling Africa’s chronic disease burden: from the local to the global. Global Health 2010;6:5.
[6] Chakravorty S, Williams TN. Sickle cell disease: a neglected chronic disease of increasing global health importance. Arch Dis Child 2014.
[7] Gustafson SL. Knowledge and Health Beliefs of Sickle Cell Disease and Sickle Cell Trait: The Influence on Acceptance of Genetic Screening for Sickle Cell Trait 2006.
[8] Njamnshi AK, Mbong EN, Wonkam A, Ongolo-Zogo P, Djientcheu V-P, Sunjoh FL, et al. The epidemiology of stroke in sickle cell patients in Yaounde, Cameroon. J Neurol Sci 2006;250:79–84.
[9] Okwi AL, Byarugaba W, Ndugwa CM, Parkes A, Ocaido M, Tumwine JK. Knowledge gaps, attitude and beliefs of the communities about sickle cell disease in Eastern and Western Uganda. East Afr Med J 2009;86:442–9.
[10] Shahine R, Badr LK, Karam D, Abboud M. Educational Intervention to Improve the Health Outcomes of Children With Sickle Cell Disease. J Pediatr Health Care 2014.
[11] Yusuf HR, Lloyd-Puryear M a., Grant AM, Parker CS, Creary MS, Atrash HK. Sickle cell disease: The need for a public health agenda. Am J Prev Med 2011;41:S376–83.
[12] Grosse SD, Odame I, Atrash HK, Amendah DD, Piel FB, Williams TN. Sickle cell disease in Africa: A neglected cause of early childhood mortality. Am J Prev Med 2011;41:S398–405.
[13] States U, Americans A. Sickle Cell Trait — Neglected Opportunities in the Era of Genomic Medicine 2014;02118.
[14] Meijer WJ, Ragetlie PL. Empowering the patient with ICT-tools: the unfulfilled promise. Stud Health Technol In-form 2007;127:199–218.
[15] Ballas SK. Current issues in sickle cell pain and its management. Hematology Am Soc Hematol Educ Program 2007:97–105.
[16] Amertil NP. Self-management in Adult Clients with Sickle Cell Disease (SCD). 1997.
[17] Derlega VJ, Janda LH, Miranda J, Chen IA, Goodman BM, Smith W. How Patients’ Self-Disclosure about Sickle Cell Pain Episodes to Significant Others Relates to Living with Sickle Cell Disease. Pain Med 2014;15:1496–507.
[18] Vij R, Machado RF. Pulmonary complications of hemoglobinopathies. Chest 2010;138:973–83.
[19] Smith WR, Bovbjerg VE, Penberthy LT, McClish DK, Levenson JL, Roberts JD, et al. Understanding pain and im-proving management of sickle cell disease: the PiSCES study. J Natl Med Assoc 2005;97:183–93.
[20] DeBaun MR. The challenge of creating an evidence-based guideline for sickle cell disease. JAMA 2014;312:1004–5.
[21] Lionnet F, Arlet J-B, Bartolucci P, Habibi a, Ribeil J, Stankovic K. [Guidelines for management of adult sickle cell disease]. Rev Med Interne 2009;30 Suppl 3:S162–223.
[22] Cecchini J, Lionnet F, Djibré M, Parrot A, Stojanovic KS, Girot R, et al. Outcomes of adult patients with sickle cell disease admitted to the ICU: a case series*. Crit Care Med 2014;42:1629–39.
[23] Piel FB, Hay SI, Gupta S, Weatherall DJ, Williams TN. Global burden of sickle cell anaemia in children under five, 2010-2050: modelling based on demographics, excess mor-tality, and interventions. PLoS Med 2013;10:e1001484.
[24] Toolkit S. Living Well With Sickle Cell Disease: Self-Care Toolkit n.d. http://www.cdc.gov/ncbddd/sicklecell/documents/liv-ingwell-with-sickle-cell-disease_self-caretoolkit.pdf (accessed December 22, 2014).
[25] Aloni MN, Nkee L. Challenge of managing sickle cell disease in a pediatric population living in kinshasa, democratic republic of congo: a sickle cell center experience. Hemoglo-bin 2014;38:196–200.
[26] Swan M. The Quantified Self: Fundamental Disruption in Big Data Science and Biological Discovery. Big Data 2013;1:85–99.
[27] Banaee H, Ahmed MU, Loutfi A. Data mining for wearable sensors in health monitoring systems: a review of recent trends and challenges. Sensors (Basel) 2013;13:17472–500.
[28] Shaywitz D. Do Data From Wearables Belong In The Medical Record? n.d. http://www.forbes.com/sites/davidshay-witz/2014/09/07/do-data-from-wearables-belong-in-the-medical-record/ (accessed December 22, 2014).
[29] Lee J, Finkelstein J. Activity trackers: a critical review. Stud Health Technol Inform 2014;205:558–62.
[30] Silva IV, Reis AF, Palaré MJ, Ferrão A, Rodrigues T, Mo-rais A. Sickle cell disease in children: chronic complications and search of predictive factors for adverse outcomes. Eur J Haematol 2014.
[31] DeBaun MR, Rodeghier M, Cohen R, Kirkham FJ, Rosen CL, Roberts I, et al. Factors predicting future ACS episodes in children with sickle cell anemia. Am J Hematol 2014.
[32] Altini M, Penders JF, Amft O. Estimating Oxygen Uptake during Non-Steady-State Activities and Transitions Using Wearable Sensors. IEEE J Biomed Heal Informatics 2015;PP:1.
[33] Shmueli G, Koppius O. Predictive Analytics in Information Systems Research. M I S Q 2011;35:553–72.
[34] Diaw M, samb A, Diop S, Sall ND, Ba A, Cissé F, et al. Effects of hydration and water deprivation on blood viscosity during a soccer game in sickle cell trait carriers. Br J Sports Med 2014;48:326–31.
[35] Jin W, Wu L, Song Y, Jiang J, Zhu X, Yang D, et al. Continuous intraarterial blood pH monitoring by a fiber-optic fluorosensor. IEEE Trans Biomed Eng 2011;58:1232–8.
[36] Carrara S, Bolomey L, Boero C, Cavallini A, Meurville E, De Micheli G, et al. Single-metabolite Bio-Nano-Sensors and system for remote monitoring in animal models. 2011 IEEE SENSORS Proc., IEEE; 2011, p. 716–9.
[37] Gedde MM, Yang E, Huestis WH. Shape response of human erythrocytes to altered cell pH. Blood 1995;86:1595–9.
[38] Prototype electrolyte sensor to provide immediate read-outs | EurekAlert! Science News n.d. http://www.eure-kalert.org/pub_releases/2014-06/dnl-pes060314.php (accessed December 22, 2014).
[39] Nagalla S, Ballas SK. Drugs for preventing red blood cell dehydration in people with sickle cell disease. Cochrane Database Syst Rev 2010:CD003426.
[40] Durán A, Martín P, Runkle I, Pérez N, Abad R, Fernández M, et al. Benefits of self-monitoring blood glucose in the management of new-onset Type 2 diabetes mellitus: the St Carlos Study, a prospective randomized clinic-based interventional study with parallel groups. J Diabetes 2010;2:203–11.
[41] Haywood C, Beach MC, Lanzkron S, Strouse JJ, Wilson R, Park H, et al. A systematic review of barriers and interventions to improve appropriate use of therapies for sickle cell disease. J Natl Med Assoc 2009;101:1022–33.
[42] Boyd JH, Watkins AR, Price CL, Fleming F, DeBaun MR. Inadequate community knowledge about sickle cell disease among African-American women. J Natl Med Assoc 2005;97:62–7.
[43] Pegelow CH, Colangelo L, Steinberg M, Wright EC, Smith J, Phillips G, et al. Natural history of blood pressure in sickle cell disease: risks for stroke and death associated with relative hypertension in sickle cell anemia. Am J Med 1997;102:171–7.
[44] McManus RJ, Mant J, Bray EP, Holder R, Jones MI, Greenfield S, et al. Telemonitoring and self-management in the control of hypertension (TASMINH2): a randomised controlled trial. Lancet 2010;376:163–72.
[45] Panepinto JA, Torres S, Bendo CB, McCavit TL, Dinu B, Sherman-Bien S, et al. PedsQLTM Multidimensional Fatigue Scale in sickle cell disease: feasibility, reliability, and validity. Pediatr Blood Cancer 2014;61:171–7.
[46] Pinnock H, Hanley J, McCloughan L, Todd A, Krishan A, Lewis S, et al. Effectiveness of telemonitoring integrated into existing clinical services on hospital admission for ex-acerbation of chronic obstructive pulmonary disease: re-searcher blind, multicentre, randomised controlled trial. BMJ 2013;347:f6070.
[47] Yale SH, Nagib N, Guthrie T. Approach to the vaso-occlusive crisis in adults with sickle cell disease. Am Fam Physician 2000;61:1349–56, 1363–4.
[48] Hm CS; PC; SS; RB; MJ; JA; K. Telemonitoring for pa-tients with chronic heart failure: a systematic review. J Card Fail (J CARD Fail 2007;13:56–62.
[49] Paré G, Jaana M, Sicotte C. Systematic Review of Home Telemonitoring for Chronic Diseases: The Evidence Base. J Am Med Informatics Assoc 2007;14:269–77.
[50] Giokas K, Kouris I, Koutsouris D. Autonomy, motivation and individual self-management for COPD patients, the Amica project. Lect Notes Inst Comput Sci Soc Telecom-mun Eng 2011;55 LNICST:49–53.
[51] Pinna GD, Maestri R, Andrews D, Witkowski T, Capomolla S, Scanferlato JL, et al. Home telemonitoring of vital signs and cardiorespiratory signals in heart failure patients: System architecture and feasibility of the HHH model. Int J Cardiol 2007;120:371–9.
[52] Vanagas G, Umbrasiene J, Slapikas R. Effectiveness of telemedicine and distance learning applications for patients with chronic heart failure. A protocol for prospective paral-lel group non-randomised open label study. BMJ Open 2012;2.
[53] McGann PT, Nero AC, Ware RE. Current management of sickle cell anemia. Cold Spring Harb Perspect Med 2013;3.
[54] Ofovwe C, Omoti C, Ofovwe G, Akenzua G. Perception, attitude and knowledge of sickle cell anaemia among students of a Nigerian University. J Coll Med 2012;16:1–8.
[55] Wonkam A, de Vries J, Royal CD, Ramesar R, Angwafo FF. Would you terminate a pregnancy affected by sickle cell disease? Analysis of views of patients in Cameroon. J Med Ethics 2014;40:615–20.
[56] Tanabe P, Porter J, Creary M, Kirkwood E, Miller S, Ah-med-Williams E, et al. A qualitative analysis of best self-management practices: sickle cell disease. J Natl Med As-soc 2010;102:1033–41.
[57] Amid A, Odame I. Improving outcomes in children with sickle cell disease: treatment considerations and strategies. Paediatr Drugs 2014;16:255–66.
[58] Karunakar Y, Kuwadekar A. An Unparagoned Application for Red Blood Cell Counting using Marker Controlled Watershed Algorithm for Android Mobile. 2011 Fifth Int. Conf. Next Gener. Mob. Appl. Serv. Technol., IEEE; 2011, p. 100–4.
[59] Angastiniotis M, Vives Corrons JL, Soteriades ES, Eleftheriou A. The impact of migrations on the health services for rare diseases in Europe: The example of haemo-globin disorders. Sci World J 2013;2013.
[60] Telen MJ, Wun T, McCavit TL, De Castro LM, Krishna-murti L, Lanzkron S, et al. Randomized phase 2 study of GMI-1070 in SCD: reduction in time to resolution of vaso-occlusive events and decreased opioid use. Blood 2015:blood – 2014–06 – 583351.
[61] Oniyangi O, Cohall DH. Phytomedicines (medicines de-rived from plants) for sickle cell disease. Cochrane Database Syst Rev 2010:CD004448.
[62] Odame I. Developing a global agenda for sickle cell disease: report of an international symposium and workshop in Cotonou, Republic of Benin. Am J Prev Med 2010;38:S571–5.
[63] Network GSCD. Our Network n.d.
[64] Orphanet: Sickle cell anemia n.d. http://www.orpha.net/con-sor/cgi-bin/OC_Exp.php?Lng=EN&Expert=232 (accessed December 22, 2014).
[65] Bediang G, Perrin C, Ruiz de Castañeda R, Kamga Y, Sawadogo A, Bagayoko CO, et al. The RAFT Telemedicine Network: Lessons Learnt and Perspectives from a Decade of Educational and Clinical Services in Low- and Middle-Incomes Countries. Front Public Heal 2014;2:180.
[66] Epstein RM, Siegel DJ, Silberman J. Self-monitoring in clinical practice: A challenge for medical educators. J Contin Educ Health Prof 2008;28:5–13.
[67] Shah N, Jonassaint J, De Castro L. Patients welcome the Sickle Cell Disease Mobile Application to Record Symp-toms via Technology (SMART). Hemoglobin 2014;38:99–103.
[68] Breslauer DN, Maamari RN, Switz NA, Lam WA, Fletcher DA. Mobile phone based clinical microscopy for global health applications. PLoS One 2009;4:e6320.
[69] Gonzalez-Hidalgo M, Guerrero-Pena FA, Herold-Garcia S, Jaume-I-Capo A, Marrero-Fernandez PD. Red Blood Cell Cluster Separation from Digital Images or use in Sickle Cell Disease. IEEE J Biomed Heal Informatics 2014;PP:1.
[70] Youngchan Kim, YongKeun Park. Quantitative phase im-aging and spectroscopy techniques for the study of sickle cell diseases 2012:1–2.
[71] Linguraru MG, Orandi BJ, Van Uitert RL, Mukherjee N, Summers RM, Gladwin MT, et al. CT and image processing non-invasive indicators of sickle cell secondary pulmonary hypertension. Conf Proc . Annu Int Conf IEEE Eng Med Biol Soc IEEE Eng Med Biol Soc Annu Conf 2008;2008:859–62.
[72] Shah S, Dhameliya V, Roy AK. ImPatho - an image processing based pathological decision support system for disease identification and a novel tool for overall health gov-ernance. 2014 IEEE Reg. 10 Humanit. Technol. Conf. (R10 HTC), IEEE; 2014, p. 64–9.
[73] Cheng C, Brown RC, Cohen LL, Venugopalan J, Stokes TH, Wang MD. iACT--an interactive mHealth monitoring system to enhance psychotherapy for adolescents with sickle cell disease. Conf Proc . Annu Int Conf IEEE Eng Med Biol Soc IEEE Eng Med Biol Soc Annu Conf 2013;2013:2279–82.
[74] Brown C, New T, Stokes TH, Dampier C, Wang MD. Sick-leREMOTE: A two-way text messaging system for pediatric sickle cell disease patients. Proc. 2012 IEEE-EMBS Int. Conf. Biomed. Heal. Informatics, IEEE; 2012, p. 408–11.
[75] Jacob E, Duran J, Stinson J, Lewis MA, Zeltzer L. Remote monitoring of pain and symptoms using wireless technology in children and adolescents with sickle cell disease. J Am Assoc Nurse Pract 2013;25:42–54.
[76] Jacob E, Stinson J, Duran J, Gupta A, Gerla M, Ann Lewis M, et al. Usability testing of a Smartphone for accessing a web-based e-diary for self-monitoring of pain and symptoms in sickle cell disease. J Pediatr Hematol Oncol 2012;34:326–35.
[77] Jacob E, Gerla M. Web-based mobile e-Diary for youth with Sickle Cell Disease. 2012 IEEE Consum. Commun. Netw. Conf., IEEE; 2012, p. 385–9.
[78] Jacob E, Pavlish C, Duran J, Stinson J, Lewis MA, Zeltzer L. Facilitating Pediatric Patient-Provider Communications Using Wireless Technology in Children and Adolescents With Sickle Cell Disease. J Pediatr Heal Care 2013;27:284–92.
[79] Venugopalan J, Brown C, Cheng C, Stokes TH, Wang MD. Activity and school attendance monitoring system for adolescents with sickle cell disease. Conf Proc . Annu Int Conf IEEE Eng Med Biol Soc IEEE Eng Med Biol Soc Annu Conf 2012;2012:2456–9.
[80] Schwartz LA, Radcliffe J, Barakat LP. Associates of school absenteeism in adolescents with sickle cell disease. Pediatr Blood Cancer 2009;52:92–6.
[81] Woods K, Kutlar A, Grigsby RK, Adams L, Stachura ME. Primary-care delivery for sickle cell patients in rural Georgia using telemedicine. Telemed J 1998;4:353–61.
[82] Woods KF, Kutlar A, Johnson JA, Waller JL, Grigsby RK, Stachura ME, et al. Sickle cell telemedicine and standard clinical encounters: a comparison of patient satisfaction. Telemed J 1999;5:349–56.
[83] Woods KF, Johnson JA, Kutlar A, Daitch L, Stachura ME. Sickle cell disease telemedicine network for rural outreach. J Telemed Telecare 2000;6:285–90.
[84] Medical Home for Sickle Cell Disease : A New Paradigm for Health Care Delivery n.d.
[85] Ballas SK, Vichinsky EP. Is the medical home for adult patients with sickle cell disease a reality or an illusion? Hemoglobin 2015;39:130–3.
[86] McClellan CB, Schatz JC, Puffer E, Sanchez CE, Stancil MT, Roberts CW. Use of handheld wireless technology for a home-based sickle cell pain management protocol. J Pedi-atr Psychol 2009;34:564–73.
[87] Panneerselvam P. Application of embedded system for a genetic disease, sickle cell anemia. 2014 Int. Conf. Adv. Electr. Eng., IEEE; 2014, p. 1–4.
[88] Ameringer S, Elswick RK, Smith W. Fatigue in adolescents and young adults with sickle cell disease: biological and behavioral correlates and health-related quality of life. J Pediatr Oncol Nurs 2014;31:6–17.
[89] Lopez G, Liles DK, Knupp CL. Edmonton Symptom Assessment System for outpatient symptom monitoring of sickle cell disease. South Med J 2014;107:768–72.
[90] Steinhubl SR, Muse ED, Topol EJ. The emerging field of mobile health. Sci Transl Med 2015;7:283rv3.
[91] Andreu Perez J, Leff D, Ip H, Yang G-Z. From Wearable Sensors to Smart Implants - Towards Pervasive and Person-alised Healthcare. IEEE Trans Biomed Eng 2015.
[92] Arsand E, Frøisland DH, Skrøvseth SO, Chomutare T, Tatara N, Hartvigsen G, et al. Mobile Health Applications to Assist Patients with Diabetes: Lessons Learned and Design Implications. J Diabetes Sci Technol 2012;6:1197–206.
[93] VOICE Crisis Alert App n.d. http://crisisvoice.com/ (accessed April 21, 2015).
[94] Årsand E, Muzny M, Bradway M, Muzik J, Hartvigsen G. Performance of the First Combined Smartwatch and Smartphone Diabetes Diary Application Study. J Diabetes Sci Technol 2015.
[95] Thomas VJ, Taylor LM. The psychosocial experience of people with sickle cell disease and its impact on quality of life: Qualitative findings from focus groups. Br J Health Psychol 2002;7:345–63.
[96] Constantinou C, Payne N, Inusa B. Assessing the quality of life of children with sickle cell anaemia using self-, parent-proxy, and health care professional-proxy reports. Br J Health Psychol 2014.
[97] Kelly AD, Egan AM, Reiter-Purtill J, Gerhardt CA, Van-natta K, Noll RB. A controlled study of internalizing symp-toms in older adolescents with sickle cell disease. Pediatr Blood Cancer 2015;62:637–42.
[98] Wonkam A, Mba CZ, Mbanya D, Ngogang J, Ramesar R, Angwafo FF. Psychosocial Stressors of Sickle Cell Disease on Adult Patients in Cameroon. J Genet Couns 2014.
[99] Tatara N, Årsand E, Skrovseth SO, Hartvigsen G. Long-term engagement with a mobile self-management system for people with type 2 diabetes. J Med Internet Res 2013;15.
[100] Glasemann M, Kanstrup AM, Ryberg T. Making Chocolate-covered Broccoli : Designing a Mobile Learning Game about Food for Young People with Diabetes. Communica-tion 2010:262–71.
[101] Arsand E, Tufano JT, Ralston JD, Hjortdahl P. Designing mobile dietary management support technologies for people with diabetes. J Telemed Telecare 2008;14:329–32.
[102] Miller AS, Cafazzo JA, Seto E. A game plan: Gamification design principles in mHealth applications for chronic disease management. Health Informatics J 2014. doi: 10.1177/1460458214537511.
[103] Cafazzo J a., Casselman M, Hamming N, Katzman DK, Palmert MR. Design of an mHealth app for the self-management of adolescent type 1 diabetes: A pilot study. J Med Internet Res 2012;14:1–20.
[104] Whitson JR. Gaming the quantified self. Surveill Soc 2013;11:163–76.
[105] Mobile Usability | Book by Nielsen Norman Group n.d. http://www.nngroup.com/books/mobile-usability/ (ac-cessed April 21, 2015).
[106] Carnets de santé n.d. http://www.carnetsdesante.fr/Tcher-nia-Gil (accessed April 21, 2015).
