Objective:
Treatment of bleeding disorders consists of factor replacement on-demand in response to acute bleeding or prophylactically to prevent bleeding. Venous access is a critical aspect of hemophilia care. Placement of Arteriovenous Fistulae (AVF) has previously been reported (Urgo, J et al 2008). We are reporting the long-term use of the AVF and an additional 3 patients.
Methods:
All patients who received an AVF had their records reviewed and they were evaluated for their AVF usage patterns, perceived appearance, longevity of use, and overall satisfaction. The insertion protocol has been previously reported. Each patient’s AVF was assessed routinely at each clinic visit.
Summary:
There were 17 AVF insertions in 16 patients: two von Willebrand disease, 12 Hemophilia A (3 inhibitor), and 3 Hemophilia B (1 inhibitor). Mean follow-up was 5 years (1- 13 years). 15 patients had excellent results with adequate flow and patients/caregivers were able to easily access the AVF for treatment. 1 patient, who underwent 2 procedures, had a poor surgical result with inadequate blood flow to the AVF. No patients had bleeding complications from AVF creation. No patients have an AVF related infection over 5 years. Patients have not experienced any difficulty accessing the AVF for administration of factor. Four patients have reported dissatisfaction with the appearance of the AVF. All report embarrassment over appearance, self-consciousness, wear clothing to hide the AVF, and limited participation in activities where others may question the AVF. All report the AVF works well, no issues with access, and increased confidence in self-infusion. These patients all had enlarged AVF with increased blood flow as demonstrated by fistulogram. 1 patient had revision with banding that had excellent results as well as improved appearance and continued excellent intravenous (IV) access. Two more patients are scheduled for revision. The 4th patient had removal of the AVF due to increased availability of peripheral access.
Conclusion:
AVF continues to be a viable option in patients who do not have IV access and have had repeated complications with other methods of IV access. The complication rate for insertion is 3/17 (18%). Excellent IV access was achieved in 15/16 (94%) patients. Overall satisfaction is good with 9/13 (69%) patients reporting excellent function, ease of access, and satisfaction of the cosmetic appearance of the AVF.
Objective:
To investigate the effect of modified pull-up exercises on elbow range of motion (ROM) and function for people with arthropathy secondary to hemophilia and recurrent bleeding
Methods:
B) Design and procedure: This pilot study was a prospective case series. Subjects were asked to perform a home exercise program consisting of modified pull ups three times per week for 8 weeks. Data was collected prior to start of program, at 3-5 weeks and at 8-10 weeks. Outcome measures included elbow ROM, pain, upper arm girth and activities of daily living (ADL) related reaching tasks. Information on how often the exercises were being performed, as well as episodes of bleeding was collected each week.
C) Analyses: A paired t-test was used to compare pre and post intervention measurements.
Summary:
Ten subjects have been recruited with ages ranging from 26-45 years. All have severe hemophilia A. Six subjects have completed the 8 week program to date. Those who completed the program demonstrated a mean increase of 5.3° of elbow flexion ROM (p=0.007). There was a trend toward increase in supination ROM between the two time points (p=0.058). No significant difference was seen in pre and post measurements for extension and pronation ROM. The only ADL related reaching task that demonstrated change between the two time points was palm of hand to occiput. Distance between the palm of hand to the occiput decreased 2.2 inches (p=0.009). Only 2 of the 6 subjects reported having pain in their elbow prior to the start of the program and it was unchanged during the course of the study. Arm girth data only existed for 5 of the 6 subjects and was measured at 3-5 week and 8-10 weeks. There was a trend toward increase in upper arm girth (p=0.078). There have been three reported episodes of elbow bleeding; only one was attributed to the exercises according to subject’s report.
Conclusion:
Preliminary results suggest that an eight week exercise program consisting of modified pull- ups may increase elbow flexion ROM in men with elbow joint contracture due to recurrent joint bleeding from hemophilia. Continuation of this study as well as development of others is needed to further determine if strength training is beneficial to improving elbow movement and function in those with hemophilic arthropathy.
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:
To assess the dental experiences of patients with von Willebrand disease for the purpose of developing guidelines for screening and dental management.
Methods:
A 13-question survey was administered to individuals at the National Outreach von Willebrand Conference, held in Phoenix, AZ in February 2012. A total of 55 respondents answered questions regarding oral hygiene habits, frequency and types of prior dental visits, dentists’ attitudes and knowledge of the disease, adverse bleeding events and quality of communication between dentist and haematologist.
Results:
Eighteen percent of respondents reported being refused dental treatment upon disclosure of von Willebrand disease history, while 81% of respondents reported that their dentist did not consult their haematologist prior to rendering treatment. More than half of those surveyed (56%) reported adverse bleeding events following dental procedures. Finally, 37 respondents reported gingival bleeding and 21 had not visited a dentist in the past six months.
Conclusions:
The results of this pilot study indicate that there is a need to educate the dental profession about von Willebrand Disease, especially its oral manifestations. Simultaneously, patients with von Willebrand Disease need to be educated as to the importance of maintaining oral health. Much more research needs to be done on the effects of poor oral health on the severity of bleeding disorders.
