Objective:
Demonstrate the success of collaborative efforts between the specialized multidisciplinary Infusion Pharmacy Provider (IPP), the prescriber, patient and payer, in achieving improved outcomes.
Methods:
A Case Study including chart review, cost analysis, and interviews with patient and prescriber.
Summary:
Patient is a 23-year-old male with severe hemophilia A and an inhibitor, followed by a Hemophilia Treatment Center (HTC). Patient developed a high titer inhibitor with a Bethesda Titre of 1000 BU/ml as a child. Several complex treatment plans including: Immune Tolerance Therapy (ITT) utilizing plasma derived and recombinant factor products, immunosuppressive therapy, and prophylaxis with bypassing agents failed. Complications with implanted ports resulted in hospitalizations and replacement of approximately twenty ports. Numerous hospitalizations for uncontrolled bleeding episodes and pain management contributed to a disruptive childhood/adolescence and suboptimal quality of life for the patient and family.
Patient was unable to attend school regularly, develop socially, or participate in normal age-appropriate activities. Repeated uncontrollable bleeding episodes led to the development of target joints and hemarthrosis. The complex nature of the patient’s treatment regimen, his psychosocial issues, bleed history, and cost of therapy resulted in frequent communication and collaboration between all stakeholders to maximize therapy outcomes.
Inhibitors presents a significant management challenge.2 Emicizumab (Hemlibra®) was approved for the treatment of hemophilia A with inhibitors in November 2017. Well in advance of the transition, the IPP and prescriber discussed the benefits with the patient. Although understandably reluctant due to his history of failed therapies, the patient agreed to try Emicizumab. Initial doses were administered at the IPP’s Alternate Infusion Suite (AIS) under clinical observation, per prescriber’s request. The patient and caregiver received extensive education regarding potential adverse events, self- administration, and bleed treatment regimen during these visits.
Conclusion:
The coordination of care, communication, and goal alignment by all stakeholders resulted in positive outcomes for this patient. Following eighteen months of therapy with Emicizumab, the patient reports improved over-all quality of life as evidenced by his ability to maintain employment, attend college, and engage in social events/ activities. Twenty-two hospitalizations in the twelve months prior to changing therapies decreased to one in the eighteen months after transitioning. His bleeding events decreased from six to eight bleeds per month to one bleed in the past eighteen months and this bleed was attributed to a missed dose. Education on the importance of adhering to prescribed dosing schedule was reinforced by both the IPP and HTC. His port has been removed. Along with his significant increase in quality of life, the dramatic decrease in overall cost of care will be highlighted.
Objectives:
To streamline and standardize the transition process of care through improved collaborations with staff and for persons with hemophilia transferring from a pediatric to an adult HTC. Processes were developed to provide patients with documented transition skills in order to foster medical independence in a complex healthcare environment.
Methods:
Continuous quality improvement tools were used to develop, implement and test a standardized transition tool for patients diagnosed with hemophilia A or B, ages fifteen to nineteen years of age. The transition tool was designed to assess the knowledge and skills of the adolescent in preparation for transition to adult care. Adherence to the administration of the tool in the pediatric HTC was the initial outcome measure. Key drivers included 1) improving communication between staff at the HTCs 2) transition tool development 3) educational resource content identification and 4) education of staff and families regarding the transition project. Communication was fostered through weekly team meetings to discuss and develop the transition tool in collaboration with the adult HTC. The adult HTC social worker would then attend the comprehensive clinic appointment at the pediatric HTC for those identified patients to assist in the preparation of transferring care. An excel spreadsheet, along with the ATHN database, was utilized to track patients to provide continuity of care during the transfer. Additionally, quarterly meetings were implemented with both HTC teams to discuss transferring patients and the continuum of the transition process. The transition tool was developed after review of available transition tools and was designed to provide systematic assessment of patient knowledge for transition readiness. The tool was refined through a series of PDSAs and implemented at each comprehensive clinic. The patient responses to the transition tool highlighted educational opportunities and led to the development of a resource cart to provide readily accessible targeted educational tools. Family and staff were educated about the value of transition readiness through team meetings, community outreach and during clinic visits.
Summary:
Use of the transition tool began in March 2016. Data was available through May 17, 2017. Thirty of 31 (97%) eligible patients completing the tool. Communication was improved between HTC teams. Educational tools were identified, obtained and provided to patients.
Conclusions:
We have successfully streamlined and standardized the transition process, identified educational opportunities and improved communication with staff at our HTCs utilizing established quality improvement techniques. Next steps include measurement of answered transition tool questions to further enhance patient/family knowledge and promote successful transition, as well as expansion to other age appropriate transition tools, to facilitate the journey to medical independence.
Aim:
The goal of this project was to gather data and identify factors affecting access to dental care for people with bleeding disorders in the United States.
Methods:
The Arizona School of Dentistry and Oral Health and the National Hemophilia Foundation conducted a joint survey. The survey was completed by 102 of the 147 hemophilia treatment centers (HTC) in the USA. This represents 69% of the HTC’s in the country. Each HTC provided specific information concerning the dental services and education provided for patients.
Summary:
Survey results revealed inconsistent levels of oral health services available to patients. Major factors limiting access to care include finances, patient anxiety and a lack of providers with the skills to treat this population.
Conclusion:
Improvement in oral health for persons with bleeding disorders requires appropriate education for providers, patients and families. Additionally, both public and private health funding must be re-evaluated as it relates to people with bleeding disorders.
Objective:
Highlight the importance of collaboration in addition to dose, frequency, and PK data to personalize regimens in order to minimize bleed rates in Hemophilia A patients on prophylaxis.
Methods:
Dose and bleed data was collected and reviewed from January 1, 2015 through March 31, 2015 for all moderate to severe hemophilia A patients on prophylaxis regimens with traditional acting products in 2 regional bleeding disorder hubs. Units per kilogram per dose, units per kilogram per week, and spontaneous bleed rate were calculated from data. If reporter was unsure of bleed origin, it was counted as a spontaneous bleed.
Results:
Region X had 34 and Region Y had 40 patients that met the inclusion criteria for review. The average dispensed dose for Region X was 43.89 units per kg per dose. For Region Y, it was 35.47 units per kg per dose; a difference of 8.42 units per kg per dose. Due to varying frequency orders, regimens were further calculated in units per kg per week. Region X’s average was 121.38 units per kg per week and Region Y’s was 96.98; a difference of 24.4 units per kg per week. Patients in Region X reported zero spontaneous bleeds during the study period. Patients in Region Y reported 16 spontaneous bleeds from 12 unique patients. Of these patients, target joints were cited as contributing factors with 8 of the 16 bleeds. When appropriate, prescribers were notified and collaboration on regimen adjustments was made. Pharmacokinetic data was inconsistently available.
Conclusion:
Zero spontaneous bleeds should be the goal for hemophilia A patients on prophylaxis. Small changes in dose or frequency can make significant changes in outcomes. Many variables impact bleed rates and doses vary widely. There is the potential for refining dosing algorithms based on a more personalized approach that includes close and careful monitoring of trough levels, patients activity level, bleed pattern and response to changes in treatment plans. This individualized approach would require collaboration between patients, prescribers, and pharmacies. Such an approach can have huge and long-term beneficial effects on pharmacoeconomic, clinical, and quality of life outcomes. Data collected from this study may further assist pharmacists with influencing prescribers to consider pharmacist obtained bleed data and/or obtain and provide PK data so they can assist with regimen adjustment recommendations based on their assessments. With increasing payer pressure and the introduction of longer acting products, this collaboration will become even more imperative.
Objective:
Promote awareness of and an opportunity for dialogue regarding variability among prescribed regimens to enhance care for hemophilia A patients with inhibitors undergoing immune tolerance.
Methods:
Retrospective chart review of all severe hemophilia A patients receiving interdisciplinary inhibitor management home infusion support between March of 2013 and March of 2015. Excluded mild patients developing inhibitors postoperatively and those for which insufficient titer information was provided/available from prescribers. 14 patients met the inclusion criteria. We attempted to further categorize these regimens in to low and high dosing regimens as outlined in the International Immune Tolerance Study.
Summary:
We identified 14 patients on 7 different ITI regimens, none of whom expressly followed the “low or high dose regimens” of 50 units per kg 3 times a week or 200 units per kg daily. They were on either recombinant FVIII products (11) or vWF containing products (3). The reviewed population was followed by 11 different HTC’s which included 13 different prescribers. We noted time to tolerization (when information available), bleed rate, as well as the interventions and support offered patients by the homecare inhibitor team. Although the sample was small, a notable increase in bleeds was seen in those patients on regimens below100 units/kg/day. Time to tolerization was unavailable for 3 of the 14. Of the remaining 11, time to tolerization ranged from 1-45 months and there was no significant difference seen amongst regimens.
Conclusions:
Seven different regimens for ITI were prescribed for 14 unique patients across the country. All achieved successful immune tolerance, but there was variability in the frequency of spontaneous bleeds and time to tolerance. Exact time to tolerance was limited by both the inability to obtain lab values (titers) from the prescriber or long periods between titer levels. Immune tolerance induction dosing regimens have been long debated and several studies continue to attempt to provide clarity and guidance. A missing component of the research published to date is the importance of patient adherence and the benefit of prescriber/pharmacist/payer collaboration. Economic influences further complicate this as many HTC’s perceive pharmacies as competitors, rather than collaborators in care. Additionally, payers may limit networks or implement other barriers to refills. The authors wish to work collaboratively to remove these barriers and enhance outcomes.
Objective:
To examine the causes for spontaneous bleeds in severe hemophilia A patients on prophylaxis in an effort to increase care collaboration, decrease these incidents and optimize care.
Methods:
From our company’s bleeding disorders patients’ prescriptions and assessment records databases between April 1,2011 and March 31, 2012 (12 months period), prescriptions and assessment records were analyzed for prophylaxis (factor VIII) patients with severe hemophilia A who did not have inhibitors and who had at least one bleed (self- reported) requiring extra factor in the last 12 months. Due to limitations related to data retrieval from different databases, we eliminated all the patients where accuracy of match was doubtful resulting in a reduced “N”. Out of the 52 patients, 17 were identified with at least one spontaneous bleed. To identify details of psychosocial environment that might have contributed to the bleeds, chart reviews were done on five among them who reported two or more spontaneous bleeds.
Results:
Patients with four bleeds:
Patient A developed a left ankle target joint. Patient has not consistently reported bleeds to HTC. Specialty RPh notified HTC for evaluation of dose and to report bleed pattern.
Patient B frequently missed doses resulting in spontaneous bleeds.
Patients with three bleeds:
Patient C communicated well with home care and HTC. This collaboration contributed to care plan personalization and an increase in dose and frequency resulting in cessation of spontaneous bleeds.
Patients with two bleeds:
Patient D is a toddler making it difficult to identify spontaneous versus trauma induced bleeding. Homecare nurse communicated frequently with HTC leading to dosing adjustments resulting in rare spontaneous bleeds.
Patient E is a non-compliant teen and at times does not adhere to his prophy regimen.
Conclusion:
The goal of prophylaxis should be zero spontaneous bleeds. There are a variety of factors that might contribute to the bleeds such as lack of compliance, development of target joints, age, growth spurts, or improper dosing frequency and amount. Collaborating with a home infusion company or specialty pharmacy can afford an opportunity to identify and address some of these factors to take corrective actions wherever possible and hence to optimize outcomes.
Objective:
When a surgical procedure is required in a patient with hemophilia, continuous infusion of factor (CIF) is a safe and effective alternative to bolus dosing.1-5 However, when cultural values collide with best practices, a patient-centered collaborative care plan is necessary to help ensure a positive outcome while respecting the core values of the patient.
Method:
Our team collaborated with our local Hemophilia Treatment Center (HTC) physicians and nurses to plan CIF for an Amish patient who required a total knee replacement. After interviewing the patient, the care team recognized when the patient transitioned on CIF to the home, we would need to respect the cultural beliefs of the patient without compromising the care.
The HTC physician and nurse ordered continuous infusion of factor for the patient with goal factor levels to remain between 70-100% on post-op days 1-7 and between 50-70% on post- op days 8-14. The home infusion team collaborated with the family and HTC team to finalize the care plan. The patient-centered decision prompted the use of a battery powered ambulatory infusion pump and the use of pre-approved sliding scale factor orders (ACAT protocol) with daily factor levels. Our biggest barrier was in respect to communication with the patient. In our local Amish community, telephones are not accessible. Our brainstorming lead to our COO suggesting we include a battery operated phone that would be attached to the pump and only be used for pump emergencies.
Summary:
The patient was discharged to home post-op day three with recombinant factor VIII running at 2.5 units/kg/hour via a battery operated ambulatory pump. Levels on post-op day four were below 70, prompting the nurse to call on the “pump phone” and return for a visit that night to increase the rate to 2.7 units/kg/hour. The rate remained the same throughout the remainder of the therapy and the levels stayed within the range designated by the physician.
Conclusion:
The patient-centered multidisciplinary care plan allowed for a positive outcome while respecting the patient’s culture.
