Patient, caregiver, and provider perceptions of pain and pain management in adolescents and young adults with bleeding disorders.

INTRODUCTION: Recurrent bleeding and associated pain are critical components in the management of bleeding disorders, yet scant data describe perceptions of pain in this patient population. OBJECTIVE: This study assessed perceptions of pain and pain management in adolescents and young adults (AYAs) with haemophilia or von Willebrand disease (VWD) to determine agreement/disagreement between patients, caregivers and health care providers. METHODS: Using an online questionnaire, AYA patients (N=89), their caregivers (N=77), and providers (N=54) reported on pain perception, pain treatment and pain control. Acute and chronic pain was measured in patients via the Faces Pain Scale-Revised (FPS-R). Questionnaires queried about pharmacologic and non-pharmacologic pain management methods and how well providers and caregivers helped to manage pain. RESULTS: Poor agreement existed between patients and caregivers across all pain levels, perception of pain control and effectiveness of pain management. Specifically for chronic pain, poor agreement was noted between patients and caregivers (kappa=0.04; 29% agreement) and patients and providers (kappa=-0.07; 21.4% agreement). Among patients reporting acute or chronic pain, only 67% and 43%, respectively, utilized medication for their specific pain. Patients used more opioid medications than expected by their providers. On average, AYAs reported initial use of pain medications for chronic pain at 11.5 years. CONCLUSIONS: Ongoing research is needed in haemophilia and VWD pain management, and on the differences in pain perception between patients, caregivers and providers. As chronic pain often begins at an early age, optimal pain management should include acknowledging patient complaints, exploring pharmacologic and non-pharmacologic options, and optimizing prophylaxis.

Predictors of quality of life among adolescents and young adults with a bleeding disorder.

BACKGROUND: Health-related quality of life (HRQoL) in adolescents and young adults with bleeding disorders is under-researched. We aimed to describe factors related to HRQoL in adolescents and young adults with hemophilia A or B or von Willebrand disease. METHODS: A convenience sample of volunteers aged 13 to 25 years with hemophilia or von Willebrand disease completed a cross-sectional survey that assessed Physical (PCS) and Mental (MCS) Component Summary scores on the SF-36 questionnaire. Quantile regression models were used to assess factors associated with HRQoL. RESULTS: Of 108 respondents, 79, 7, and 14% had hemophilia A, hemophilia B, and von Willebrand disease, respectively. Most had severe disease (71%), had never developed an inhibitor (65%), and were treated prophylactically (68%). Half of patients were aged 13 to 17 years and most were white (80%) and non-Hispanic (89%). Chronic pain was reported as moderate to severe by 31% of respondents. Median PCS and MCS were 81.3 and 75.5, respectively. Quantile regression showed that the median PCS for women (61% with von Willebrand disease) was 13.1 (95% CI: 2.4, 23.8; p = 0.02) points lower than men. Ever developing an inhibitor (vs never) was associated with a 13.1-point (95% CI: 4.7, 21.5; p < 0.01) PCS reduction. MCS was 10.0 points (95% CI: 0.7, 19.3; p = 0.04) higher for prophylactic infusers versus those using on-demand treatment. Compared with patients with no to mild chronic pain, those with moderate to severe chronic pain had 25.5-point (95% CI: 17.2, 33.8; p < 0.001) and 10.0-point (95% CI: 0.8, 19.2; p = 0.03) reductions in median PCS and MCS, respectively. CONCLUSIONS: Efforts should be made to prevent and manage chronic pain, which was strongly related to physical and mental HRQoL, in adolescents and young adults with hemophilia and von Willebrand disease. Previous research suggests that better clotting factor adherence may be associated with less chronic pain.

Interrelationship between depression, anxiety, pain, and treatment adherence in hemophilia: results from a US cross-sectional survey.

PURPOSE: Depression, anxiety, pain, and treatment adherence have reciprocal effects not characterized extensively in hemophilia. This study explored the relationships between depression, anxiety, chronic pain, and treatment adherence in adults with hemophilia. PATIENTS AND METHODS: Adults with self-reported hemophilia A or B completed the cross-sectional IMPACT QoL II survey. Depression (9-item Patient Health Questionnaire [PHQ-9]), anxiety (7-item Generalized Anxiety Disorder scale [GAD-7]), chronic pain (Faces Pain Scale-Revised [FPS-R]), social support (Duke UNC Functional Social Support questionnaire), level of pain control, clotting factor treatment adherence (VERITAS-Pro or -PRN), and previous depression/anxiety were analyzed. RESULTS: Among 200 participants (male, 77.3%; female, 22.8%), 54% had PHQ-9 and 52% had GAD-7 scores indicating moderate to severe depression or anxiety without diagnosis of either disorder. Participants with PHQ-9 scores ≥10 (moderate to severe depression) were more likely to have lower treatment adherence than those with PHQ-9 scores <10 (P<0.05). Participants with PHQ-9 or GAD-7 scores ≥10 were more likely to report uncontrolled pain and less social support versus PHQ-9 or GAD-7 scores <10 (χ2 P<0.05). Significant correlations were found between PHQ-9 and GAD-7 (P<0.0001), PHQ-9 and FPS-R (P=0.0004), PHQ-9 and VERITAS (P=0.01), GAD-7 and FPS-R (P=0.02), and GAD-7 and VERITAS (P=0.001). CONCLUSION: Depression and anxiety are underdiagnosed in hemophilia. Depression is associated with anxiety, pain, and lower treatment adherence. While treatment providers play an important role in diagnosis, social workers may play a pivotal role in depression and anxiety screening. This study highlights the importance of regular screening and treatment for these disorders.

Racial Differences in Chronic Pain and Quality of Life among Adolescents and Young Adults with Moderate or Severe Hemophilia.

BACKGROUND AND OBJECTIVE: We explored racial differences in adherence to recommended clotting factor treatment regimens, chronic pain, and quality of life (QoL) among adolescents and young adults (AYAs) diagnosed with moderate or severe hemophilia. METHODS: A convenience sample of hemophilia patients aged 13-25 years completed an online cross-sectional survey in 2012. Chronic pain was measured using the revised Faces Pain Scale (FPS-R) and dichotomized as high (FPS-R ≥ 4) or low (FPS-R < 4). QoL was measured with the SF-36. RESULTS: Of 80 AYA participants (79 male), most had severe disease (91 %) and hemophilia A (91 %). Most were white (76 %) and non-Hispanic (88 %). At the univariate level, compared to whites, non-whites were more likely to have produced an inhibitor against clotting factor treatment (74 vs 38 %, p < .01), less likely to have commercial health insurance (16 vs 63 %, p < .001), more likely to report high levels of chronic pain (FPS-R ≥ 4) (63 vs 26 %, p < .01), and had lower SF-36 physical composite summary (PCS) scores. Adjusted logistic and quantile regression modeling, respectively, revealed that non-whites were 5.31 (95 % CI 1.62, 17.4; p < .01) times more likely to report high chronic pain and had median PCS scores that were 26.0 (95 % CI 11.0, 40.9; p < .01) points lower than whites. CONCLUSIONS: Targeted efforts to prevent and manage chronic pain among non-white AYAs with moderate or severe hemophilia are necessary. After accounting for demographic and clinical differences, there were no racial differences in adherence to recommended clotting factor treatment regimens; however, non-whites were more than five times more likely to report high levels of chronic pain, which predicted worse overall physical QoL, bodily pain, physical and social functioning, and greater role limitations due to physical health.