The hemophilia and thrombosis centers in Tucson and Phoenix would like to join efforts to make a meaningful contribution to our understanding of the mental health profile of our pediatric population. This knowledge could contribute to a more tailored approach when designing clinics and programming, and, by identifying mental health issues, inform the development of targeted interventions. We hope to look at the prevalence of depression and anxiety in children with bleeding disorders including hemophilia, von Willebrand disease, and other congenital coagulopathies. We expect that the existence of a chronic health condition could affect a child's psychological development. We're also aware of the critical impact of family culture so our survey will include data on the mental health of the participants’ primary caretakers as well as other significant socioeconomic markers.
Objective:
To investigate how bleeding disorder characteristics influence patient perceived challenges and management strategies.
Methods:
This is a mixed-method, retrospective, cross-sectional continuation of a pilot study identifying themes of self-perceived challenges and management strategies for persons with bleeding disorders. Sixty-one participants with a bleeding disorder (BD), either hemophilia (PWH) or Von Willebrand disease (PWVWD), were asked what their most significant challenge was in managing their BD and how they managed that challenge. Data were collected from March, 2017 through December, 2018, coded for themes and uploaded to NVivo. Similar themes were grouped for analysis. Subject-level data was extracted from the electronic medical record including demographics, disease type, severity and presence of joint disease (JD). Pain interference was determined from participant response to the Brief Pain Inventory (BPI).
Results:
Conclusions:
Gaining insight to patient-perceived challenges and management strategies is important to be able to tailor an effective treatment approach that is individualized and meets the changing needs of PWBD across circumstance and life-course.
Dr. Satish Nandakumar is currently a postdoctoral fellow in the laboratory of Dr. Vijay Sankaran at the Boston Children's Hospital. Previously, he did his graduate work at the St. Jude's Children's Research Hospital in Memphis, Tennessee. In his JGP Fellowship project, Dr. Nandakumar aims to develop a novel gene therapy approach for hemophilia that involves activation of the endogenous factor VIII or IX genes within hematopoietic stem cells by taking advantage of the CRISPR/Cas9 gene activation system. This work has the potential to benefit patients with mild hemophilia mutations.
Objective:
Acute bleeds in patients with rare bleeding disorders (RBDs), including congenital hemophilia with inhibitors (CHwI), acquired hemophilia, congenital factor VII deficiency, and Glanzmann’s thrombasthenia (GT) must be treated as quickly as possible. This study evaluated the obstacles and experiences of patients with CHwI, or their caregivers, for the early treatment of acute bleeds in non-hemophilia treatment centers (HTCs).
Methods:
Patients in the United States (aged 18–65 years [or caregivers of patients <18 years]) with CHwI, who currently have or have had inhibitors in the last 3–4 years, and who sought treatment in a non-HTC, underwent an interactive online qualitative discussion over 7 days.
Summary:
The survey was completed by 23 respondents (seven patients and 16 caregivers; all patients with CHwI). Respondents were aware of the need to treat bleeds quickly, which had been taught to them by physicians and learned from experience. Delays in respondents initiating their treatment were typically due to: technical issues (e.g., 7/23 respondents had difficulty gaining access to a vein or port); delay in diagnosis (e.g., 5/23 respondents’ child does not inform caregiver of the bleed); convenience (e.g., 3/23 respondents were unwilling/unable to take treatment out of the home); or financial issues (e.g., one respondent had inadequate insurance). Respondents tended to visit a non-HTC as a last resort, often due to the long distance to an HTC when emergency treatment was needed, unsuccessful pain management, or unsuccessful factor administration at home. Most patients/caregivers (20/23) reported treatment delays in emergency departments (EDs). Delays in EDs were often due to healthcare professional’s (HCP) lack of knowledge (16/23 respondents; 4 hours average wait until treatment) and four reported delays due to lack of available treatment (14 hours average wait for treatment). All patients/caregivers reported that they had dealt with uneducated/unaware HCPs, having to spend significant time educating the ED staff. Three respondents reported not waiting for treatment—partly because they chose hospitals very carefully, and because they had educated their closest hospital prior to needing an emergency service. Patients/caregivers with negative experiences reported that HCPs were unwilling to listen to them, did not seek consultation quickly, dismissed their instructions, and directed care that forced an outcome. When patients had satisfactory experiences, HCPs listened intently, immediately called an HTC/patient’s physician, and provided care in consultation. Respondents highlighted the need for HCPs education on hemophilia.
Conclusions:
Patients/caregivers are aware of the need to treat an acute bleed fast, but sometimes delay their treatment, and experience delays when attending non-HTCs. The lack of experience of HCPs in managing acute bleeds contributes to these delays. Improved education of HCPs at non-HTCs and provision of protocols or guidelines would be beneficial for patients with CHwI.
Objective:
Report on the utilization of a multi-point videoconferencing platform, Extensions for Community Healthcare Outcomes (ECHO), in providing a clinical learning opportunity to physical therapists (PTs) involved with people with bleeding disorders (PWBD) within Western States Region Hemophilia Treatment Centers (WSR HTC).
Methods:
WSR HTC includes thirteen HTC’s located in California, Nevada, Hawaii, and Guam. Monthly one-hour evidence based case presentations with a facilitated discussion were conducted using the ECHO platform. Each session was recorded, so all the therapists invited to participate have access to the information.
Data were collected from the WSR participating PTs by using anonymous on-line surveys, Qualtrics software (Qualtrics, Provo, UT), and prior to the start of the physical therapy ECHO session and upon completion of each session.
Descriptive statistics were calculated to evaluate the educational value of presentations.
Results:
Thirteen PTs, surveyed prior to the first PT ECHO session had reported > 6 years of experience as a PT. Twenty-three percent reported < 5 years of experience working with PWBD and over half of PT surveyed had > 16 years of experience working with PWBD.
Eight topics were presented in 2018 included musculoskeletal ultrasound imaging, invasive surgery rehab outcomes for patients with inhibitors, kinesiology taping, knee arthroplasty and stiffness, iliopsoas bleeding, myofascial decompression, chronic pain, knee bleed, and ankle joint impact from bleeding. An average of nine HTC PTs attended each session (range 4 to 18). Ten (11.2%) non-HTC PTs (outpatient PTs, HTC nurse, HTC Nurse Practitioner) attended some of the PT ECHO sessions. Table 1.
Ninety-five percent of respondents reported strong agreement with the program’s educational value and appropriateness for a practicing PT. Thirty-seven (94.9%) of responses reported agreement that the PT ECHO program improved their knowledge of physical therapy and bleeding disorders. Table 2.
Conclusion:
Videoconferencing platforms such as ECHO allows PTs in the WSR HTC, who are geographically separated to successfully share clinical knowledge to facilitate best practice in the area of specialty care for PWBD.
Please see files attached for tables and figures.
