Objective:
To educate teens and their siblings on building skills related to self-care, medical independence and living a healthy lifestyle so that they are better prepared to be on their own as they transition to the next stage in life: adulthood.
Methods:
A collaborative, multidisciplinary team consisting of team members from the Cincinnati Children’s Hospital Medical Center Hemophilia Treatment Center (HTC) and the Tri-State Bleeding Disorder Foundation lead 14 teens attending a family bleeding disorder educational conference in an interactive, educational program.
Participants were teens (ages 12-17) with a bleeding disorder and their siblings. Participants worked in teams and visited six different booths where they engaged in interactive activities such as role playing, exercising and games. Booths were staffed by the HTC staff that included nurse care managers, pharmacists, data manager, social worker, health educator, and a physical therapist. Each booth focused on: genetics of a bleeding disorder, digital citizenship, managing home treatment independently, understanding bleeding disorders, insurance/career planning and fitness /healthy joints.
This evidenced-based educational content was based on the Medical and Scientific Advisory Council (MASAC) transition guidelines for people with a bleeding disorder (Belling e al., 2003). The content and program design was also rooted in the social cognitive theory with elements of vicarious learning, behavioral capability, and self-control.
Summary:
An interactive and evidenced-based teen transition program allowed for teens to gain knowledge in various topics related to gaining in medical independence and self-care skills. All teens that participated in the program demonstrated an increase in knowledge at the program’s end.
Conclusions:
All participants showed an increase in scores from pre-test to post-test. The average scores increased by 20%. The team plans to implement this program again in the future.
References:
Belling L., Harrop M., Kocik S., Obstein, L., Standish, D., Vlasich, W., & Zappa, S. (2003). Transition Guidelines for People with Bleeding Disorders. New York, NY: M.A.S.A.C, 147, 1.
The inherited bleeding disorders (IBD) community has witnessed significant advances in recent years thanks to novel therapeutic advances and technologies and improved diagnostic proficiency.
Yet important gaps persistent, particularly for those with rare disorders and underserved populations, including women with IBD.
A new initiative led by the National Hemophilia Foundation (NHF) and shaped by the voices of the patient community is underway to address this gap.
Our goal: to design and implement a national research blueprint that outlines actionable strategies to address the most important needs within the community and opportunities to accelerate progress through coordinated collaboration.
People with bleeding disorders are often connected to and with various community members such as local and national foundations/associations, their home health company, nurses , pharmaceutical representatives, etc. While people are often encouraged to wear medic alert ID, they do not know anyone in the EMS system who may be caring for them in case of an emergency. Through this project, EMS providers will receive education regarding bleeding disorders and the treatment required in an emergency. This project will seek to connect the HTC's with the EMS systems locally and regionally to coordinate the education with the EMS professionals to provide care.
Background:
There are no reported cases of acute lymphoblastic leukemia (ALL) in patients with hemophilia B. There is one case report of a young adult with hemophilia B and acute myeloid leukemia (1). Currently, there is no best practice recommendation for the management of patients with hemophilia B and ALL.
Objectives:
To report our experience in managing a pediatric patient with congenital hemophilia B and ALL, which presents a rare and unique clinical challenge.
Design/Method:
Retrospective chart review
Results:
This is a 2y/o male with hemophilia B diagnosed at birth from a cord blood sample showing a factor IX level <1%. Mother is a known carrier and maternal grandfather has severe hemophilia B. Patient started prophylaxis with a standard half-life product through central venous access around 8months of age, following a spontaneous wrist bleed. The schedule was 35 units/kg twice a week. He had no spontaneous joint or soft tissue bleed on this regimen. 3mo ago he presented with pain and swelling of the right wrist. He fell on an outstretched hand the day before and received 100% factor infusion. X-ray showed metaphyseal lucencies with overlying soft tissue swelling. No evidence of fracture. Due to this finding, additional labs were done. WBC 4.8K, hemoglobin 7.1 g/dL, platelets 18K and 31% blasts. Flow cytometry confirmed the diagnosis of preB-ALL. On exam, he had pallor, scattered petechiae and cervical lymphadenopathy.
Based on recovery studies and thrombocytopenia, the prophylaxis was changed to 50 units/kg every third day. The platelets are kept above 30K at baseline. For lumbar punctures, he has been corrected to 100% factor level and platelets kept above 50K. However, due to the risk of port infection with frequent accessing, he was switched to long-acting albumin fusion factor IX product on day 22 of induction. The current prophylaxis regimen is 75units/kg weekly and the schedule is adjusted to coincide with lumbar puncture days whenever needed. He has tolerated all his procedures well without increased bleeding, including end of induction bone marrow aspiration, biopsy and lumbar puncture with intrathecal chemotherapy. He is currently in remission and is in interim maintenance phase of treatment per COG protocol AALL0932.
Conclusion:
Long-acting factor IX products could potentially decrease the number of infusions and need for frequent central venous access in immunocompromised patients with hemophilia B. In addition, a higher trough level with a weekly schedule could provide better bleed control in patients with severe thrombocytopenia due to underlying malignancy. A baseline platelet count of at least 30K is recommended during treatment. More treatment guidelines need to be established.
(1) Clark C et al, Pediatric Blood and Cancer Jan 2011
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.
Hemophilia causes repetitive bleeding episodes throughout the musculoskeletal system, primarily into joints, such as knees and ankles. This leads to significant joint damage resulting in increased pain reproduction, decreased functional abilities, such as walking, and negatively impacts quality of life. Traditionally the extend of joint damage has been examined via clinical assessments, such as the Hemophilia Joint Health Scores, x-rays, MRIs, and more recently musculoskeletal ultrasound (MSKUS). However, these modalities fail to establish the global impact of joint damage on the entire body of a person with hemophilia and their functional abilities. Analyzing joint motion and forces acting upon the joint during walking has been a widely established technique to gain understanding of abnormal three-dimensional movements and is a key factor in clinical decision making-processes. With the overall goal of establishing better treatment approaches for persons with hemophilia it is vital to understand the underlying functional joint limitations. Therefore, the purpose of this study is to investigate characteristics of damaged joints, joint motion and control as well as forces acting upon the joint during walking in persons with hemophilia.
Objective:
To examine the frequency and methods used to screen patients for substance use and behavioral health disorders in Hemophilia Treatment Centers (HTC). We hypothesized that inconsistencies in methods utilized and frequency of utilization exist.
Methods:
Marshall University (MU) Physical Therapy faculty along with MU addiction education staff developed a 26-question survey using Qualtrics. The survey included questions on demographics, validated screening tools utilized, screening frequency, and team member responsible for screening. The HTC email addresses were obtained from the Hemophilia Treatment Center Directory on the CDC’s website. Following approval from MU IRB, the survey was disseminated via an online link. Descriptive analysis was performed on the data.
Summary:
Health professionals from 19 HTCs, representing 8 different regions, completed the survey. The overall response rate was 13.6%. Social workers (12, 63.2%), nurses (6, 31.6%)) and counselors/psychologists (1, 0.05%) submitted responses. On average HTCs reported 34.5% (0-92%) of their patients experience chronic pain with an average 22.4% (0-56%) receiving prescription opioids for pain management. Adverse consequences related to opioid use existed in all of the HTCs including overdose (31.5%), withdrawal symptoms (42.1%), increased dose due to tolerance (63.2%), and increased bleeding episodes (26.3%). The majority of HTCs (57.9%) reported being the primary provider of pain management for people with hemophilia (PWH). Standardized screening for substance use disorders is occurring 31.6% of the time with marijuana and illicit drugs (100%) being most commonly screened followed by alcohol and prescription drugs (83%) and tobacco at 33%. Frequency of screening for substance use varied widely from every comprehensive visit to initiation of an opiate contract to suspicion of misuse. Screening for behavioral health is more common (81.3%) with a variety of validated screening tools being utilized. Over 60% of the time, screening for anxiety and depression occurs either annually or every visit.
Conclusions:
PWH often develop chronic pain related to joint arthropathy.
Based on our findings, the incidence of chronic pain in PWH is relatively equal to the national average. HTCs are often the primary provider of pain management and are challenged to find safe treatment methods. PWH are often prescribed opioids which may place them at increased risk for potentially developing an opioid use disorder.
The presence of a behavioral health disorder may further enhance one’s risk. Although behavioral health screenings appear to be more consistently utilized in HTCs, substance use screenings are rare. Our research suggests that universal screening for substance use and behavioral health conditions should be considered, as a standard of care in HTCs, to better inform healthcare providers of patient risk, need for referral and to guide prescriber’s decision making with regard to pain management options.
Objective:
Persons with bleeding disorders experience pain in association with needle pokes, joint and muscle bleeds and permanent tissue damage. The impact of this pain on patients can include time off school or work, a change in career, income, stress, mental health concerns and change in relationships. Comprehensive pain management includes strategies from the “Four P’s of Pain Management” which include pharmacological, physical, psychological and prevention.
The aim of the project was to examine current psychological knowledge and management of pain within our patient population. This study asked the following research questions: 1) What is currently understood about pain and bleeding disorder care among social workers (CSWHC)? 2) What specific pain knowledge and training is prioritized by social workers in Hemophilia Treatment Centres?
A scoping review was conducted concurrently with the qualitative study. Medline and SocIndex were searched with the terms “social work” and “pain management” and a second search was conducted with the term “social work and hemophilia/von willebrand's or platelet disorders”. A total of 105 articles were examined by three independent reviewers. Eleven articles have been included for the purpose of examining the role of social work in pain management.
Methods:
Qualitative interviews were conducted and recorded with 12 social workers from the CSWHC between September 2018 and February 2019. Five provinces were represented. Social work participants were deployed within paediatric, adult or within combined clinics. The interviews were approximately 20-45 minutes. Transcribed interviews were coded with NVivo by two independent reviewers with Thematic Analysis.
Summary:
Social workers identified the roles of social work to include completion of psychosocial assessments and meeting the practical needs of patients, while supporting patients in medical decisions. Barriers to pain management and the impact of pain on patients were described as having an impact on individuals and families. Social workers also discussed their understanding of acute and chronic pain in patients, which has indicated an increase of knowledge is required. Skills development in multi-dimensional nature of pain and pain assessment were determined to be most likely to produce positive impact on practice outcomes. Initial themes include hope, relationship of trust, stigma (diagnosis vs. pain), defining multidisciplinary roles.
Conclusion:
Study results, first, will contribute to the literature supporting the need for social work education for those practicing in bleeding disorder care. Secondly, they will provide recommendations for an educational pain curriculum for social workers in bleeding disorder care. This education will reflect the need for pain knowledge in acute and chronic pain dimensions which will facilitate dialog with other professionals in pain management. Pain assessment will also be a focus in order for social workers to be able to support and provide appropriate referrals for pain management.
