Individuals with hemophilia undergoing hip or knee arthroplasty are at risk for complications such as bleeding and infection. However, data on hospital length of stay (LOS) and readmission rates compared with nonhemophilic controls are lacking. This study compared the complication rates, LOS, and unplanned 30-day readmission rates between patients with hemophilia and nonhemophilic controls.This retrospective cohort study used the Pennsylvania Health Care Cost Containment Council (PHC4) database from 2007 to 2015 to compare outcomes in patients with hemophilia and nonhemophilic controls undergoing partial and total hip arthroplasty, knee arthroplasty, and revision knee arthroplasty.A total of 118 patients with hemophilia and 3,811 controls were identified. Compared with controls, patients with hemophilia had a higher risk of bleeding complications after hip procedures (38.7% versus 16.1%, p = 0.003), a higher risk of surgical site infection after knee procedures (8.1% versus 1.1%, p < 0.001), longer median LOS after hip (6 versus 3 days, p < 0.001) and knee (5 versus 3 days, p < 0.001) procedures, and higher rates of unplanned 30-day readmission after hip (22.6% versus 4.1%, p < 0.001) and knee (10.3% versus 4.5%, p = 0.018) procedures. The most common reason for unplanned 30-day readmission in patients with hemophilia was bleeding or the patient's underlying coagulopathy (25.1%).Patients with hemophilia undergoing hip or knee arthroplasty had a higher incidence of postoperative bleeding (hip procedures) and surgical site infections (knee procedures), longer LOS, and higher rates of unplanned 30-day readmission compared with nonhemophilic controls. Key limitations of our study include the potential for inaccurate coding, the relatively small number of patients in the hemophilia cohort, and the uneven distribution of procedure type in the hemophilia and control cohorts.Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.
Background: The Pain, Functional Impairment, and Quality of Life (P-FiQ) study was an observational, cross-sectional assessment of the impact of pain on functional impairment and quality of life in adults with hemophilia in the United States who experience joint pain or bleeding. Objective: To describe known-groups validity of assessment tools used in the P-FiQ study. Patients and methods: Participants completed 5 patient-reported outcome (PRO) instruments (5-level EuroQoL 5-dimensional questionnaire [EQ-5D-5L] with visual analog scale [VAS], Brief Pain Inventory v2 Short Form [BPI], International Physical Activity Questionnaire [IPAQ], Short-Form Health Survey [SF-36v2], and Hemophilia Activities List [HAL]) and underwent a musculoskeletal examination (Hemophilia Joint Health Score [HJHS]) during a routine clinical visit. Results: P-FiQ enrolled 381 adults with hemophilia (median age, 34 years). Participants were predominantly white/non-Hispanic (69.2%), 75% had congenital hemophilia A, and 70.5% had severe hemophilia. Most (n=310) reported bleeding within the past 6 months (mean [SD] number of bleeds, 7.1 [13.00]). All instruments discriminated between relevant known (site- or self-reported) participant groups. Domains related to pain on EQ-5D-5L, BPI, and SF-36v2 discriminated self-reported pain (acute/chronic/both; P <0.05), domains related to functional impairment on IPAQ, SF-36v2, and HAL discriminated self-reported functional impairment (restricted/unrestricted; P <0.05), and domains related to mental health on the EQ-5D-5L and SF-36v2 discriminated self-reported anxiety/depression (yes/no; P <0.01). HJHS ankle and global gait domains and global score discriminated self-reported arthritis/bone/joint problems, percentage of lifetime on prophylaxis, current treatment regimen, and hemophilia severity ( P <0.01); knee and elbow domains discriminated all of these ( P <0.01) except for current treatment regimen. Conclusion: All assessment tools demonstrated known-group validity and may have practical applicability in evaluating adults with hemophilia in clinical and research settings in the United States. Keywords: hemophilia, pain, functional impairment, quality of life, patient-reported outcome, joint health
Estimates of the size and characteristics of the US haemophilia population are needed for healthcare planning and resource needs assessment. A network of comprehensive haemophilia treatment centres (HTCs) located throughout the United States receives federal support for diagnosis and management of haemophilia and other rare bleeding disorders.Estimate the incidence and prevalence of haemophilia among US males using the HTC network.During the period 2012-2018, de-identified surveillance data were collected on all males who visited an HTC that included year of birth, gender, race, Hispanic ethnicity, residence zip code, haemophilia type and severity. Data from all patients were used to calculate period prevalence by haemophilia type, severity and state of residence. Data from a subset of patients born 1995-2014 were used to estimate incidence rates over the 20-year period.During the period, 21 748 males with haemophilia visited the HTCs resulting in an age-adjusted prevalence of 15.7 cases per 100 000 males (12 for haemophilia A and 3.7 for haemophilia B). Prevalence was higher among whites (15.1) than blacks (12.4) or Hispanics of either race (12.4). State-specific prevalence varied from 1.6 to 23.3 cases per 100 000. Based on 9587 males born during the index period, the average haemophilia incidence was 1 case per 4334 live male births.Based on these data, we estimate that there are between 29 761 and 32 985 males with haemophilia living in the United States today, the majority of whom receive comprehensive care in specialized clinical centres.
In chronic diseases, disease-related distress can impact disease outcomes. Distress and haemophilia-related distress has been demonstrated in people with haemophilia (PwH). The association of haemophilia-related distress on disease outcomes among PwH is unknown.To study the association of haemophilia-related distress with haemophilia specific outcomes, including adherence to prophylactic therapy, the presence of a target joint, healthcare utilization and work-impairment.In a cross-sectional study, adults with haemophilia A or B were enrolled in a study to validate the haemophilia-related distress questionnaire (HRDq). In this planned analysis, univariate and multivariate associations between the HRDq total score and disease outcomes were explored.The 114 participants in this analysis were male, mostly with haemophilia A (92%) and severe disease (52%) with a median age of 31.9 years. On univariate analysis, HRDq total score (5-point change) was associated with the presence of a target joint (P = .002), high healthcare utilization (P = .011), poor adherence (P = .033) and work-impairment (P ≤ .001). After adjustment for age, race, severity and other potential confounders, adherence (aβ 0.33, 95% CI .17, .49) and work-impairment (aβ 4.69, 95% CI 3.27-6.1) remained statistically significantly associated with HRDq total score.Haemophilia-related distress is associated with poor adherence to factor prophylaxis and work-impairment. The direction of the association (causation) is yet to be determined and requires future study.
