High use of pain, depression, and anxiety drugs in hemophilia: more than 3000 people with hemophilia in an 11-year Nordic registry study.

Background: Pain is a common feature of hemophilia, but prevalence of depression and anxiety is less studied. Registry data on prescription drugs can provide an objective measure of the magnitude of these complications. Objectives: To identify treatment patterns of prescribed pain, antidepressant, and antianxiety medications compared with those of matched controls in 4 Nordic countries. Methods: The MIND study (NCT03276130) analyzed longitudinal individual-level national data during 2007-2017. People with hemophilia (PwH) were identified from National Health Data Registers by diagnosis or factor replacement treatment and compared with population controls. Three subgroups were defined by the use of factor concentrates and sex (moderate-to-high factor consumption (factor VIII [FVIII] use of ≥40 IU/kg/week or FIX use of ≥10 IU/kg/week), low factor consumption, and women including carriers). Results: Data of 3246 PwH, representing 30,184 person-years, were analyzed. PwH (including children and adults) used more pain, depression, and anxiety medications compared with controls. This was most accentuated in the moderate-to-high factor consumption group and notably also observed in men with low factor consumption and women including carriers, usually representing a milder phenotype. A higher opioid use was observed across all age groups: 4- to 6-fold higher in the moderate-to-high factor consumption group and 2- to 4-fold higher in the low factor consumption group. Conclusion: The consistent higher use of pain, depression, and anxiety medications among PwH compared with population controls, regardless of age, sex, or factor consumption, in broad national data suggests a need for improved bleed protection and hemophilia care for all severities including mild hemophilia.

Pain, depression and anxiety in people with haemophilia from three Nordic countries: Cross-sectional survey data from the MIND study.

INTRODUCTION: People with haemophilia (PwH) may experience symptoms of haemophilia-related pain, depression or anxiety, which can negatively impact health-related quality of life. AIM: To obtain the perspective of PwH and treaters from Sweden, Finland and Denmark on the management of haemophilia-related pain, depression and anxiety using cross-sectional survey data from the MIND study (NCT03276130). METHODS: PwH or their caregivers completed a survey about experiences of pain, depression and anxiety related to haemophilia, and the standard EQ-5D-5L instrument. Five investigators at haemophilia treatment centres (HTC) were sent a complementary survey containing questions about the management of pain and depression/anxiety. RESULTS: There were 343 PwH (mild: 103; moderate: 53; severe: 180; seven lacking severity information) and 71 caregiver responses. Experience of pain in the last 6 months was reported by 50% of PwH respondents and 46% of caregiver respondents. Anxiety/depression was reported by 28% of PwH respondents. Reporting of pain and anxiety/depression was associated with disease severity. Whilst 62% of PwH who had experienced pain at any time point (n = 242) felt this was adequately addressed and treated at their HTC, only 24% of those who had experienced depression/anxiety (n = 127) felt this was adequately addressed. Disease severity was negatively associated with EQ-5D-5L utility value (p < .001). In the HTC survey, 4/5 and 2/5 agreed that pain and depression/anxiety, respectively, are adequately addressed. CONCLUSIONS: Pain and depression/anxiety occur more frequently with increasing haemophilia severity, with negative impacts on health-related quality of life. PwH with depression/anxiety or unaddressed pain could benefit from improved management strategies.

Self-reported activity of Swedish persons with haemophilia: Change over 2.5 years.

OBJECTIVES: To describe self-reported activity using the Haemophilia Activity List (HAL) for Swedish adults with haemophilia and to detect any changes over time. METHOD: The HAL was sent to the adult population with haemophilia A and B, moderate and severe form, living in Sweden (n = 260). Participants completed the HAL and a questionnaire on sociodemographic and medical information. From a previous study cohort, 61 persons had responded twice to the HAL. The investigated group was divided into early and later treatment onset groups with regard to access to medication. RESULTS: The response rate was 50%. There was a significant difference (p < 0.001) between the early and later treatment groups in all domains in HAL. When analysing HAL "question by question" from the 2 reported time-points, the most prominent outcome was that the reported ability in activities was stable over time at the group level, except for participants who had no access to the clotting factor early in life. They reported greater limitations in some of the activities in the challenging domain "leisure activities and sport". CONCLUSION: The early treatment group reported a significantly better ability in all activities compared with the late treatment group.

The effect of Nordic Walking on joint status, quality of life, physical ability, exercise capacity and pain in adult persons with haemophilia.

Nordic Walking is an exercise form requiring significant energy consumption, but where the use of poles minimizes the risk of injury. The aim of this pilot study was to examine the effect of 3 months of Nordic Walking on males (>40 years of age) with haemophilia, regarding joint function (Haemophilia Joint Health Score), physical ability (Haemophilia Exercise Project - Test-Questionnaire), exercise capacity (6-min walking test), pain (visual analogue scale) and quality of life (the Swedish version of The Short Form Health Survey, SF-36). Pre-interventional and post-interventional scores of above-mentioned parameters were analysed, using Wilcoxon Signed Ranks Test. Eleven participants were recruited to the study. Statistically significant improvements were observed in physical ability (P value: 0.01) and body perception (P value: 0.02). The intervention did not increase number of bleedings or factor consumption. This is the first study ever evaluating Nordic Walking in persons with haemophilia. Our results suggest that Nordic Walking is safe and efficient, also in patients with haemophilic arthropathy.

Persons with Haemophilia in Sweden- Experiences and Strategies in Everyday Life. A Single Centre Study.

INTRODUCTION/AIM: Haemophilia is caused by deficiency in coagulation factor VIII or IX. Treatment with the missing coagulation factors has been available in most developed countries for several decades. The aim was to explore the experiences of adults living with severe or moderate haemophilia and their coping strategies at a single centre in Sweden. METHOD: The interview study had a qualitative empirical approach and was analyzed on the basis of the method empirical phenomenological psychology. The sample included 14 participants, mean age 42 (19-80 y), who met the inclusion criteria and to saturation of information. RESULTS: General characteristics were; All were satisfied with and grateful for access to medication. An acceptance of the disorder and willingness to live a normal life was identified among all participants. They were all content with the care provided by Haemophilia Treatment Centre (HTC) and felt supported by its multidisciplinary team. Four typologies were identified; Protective adults and assertive children during up-bringing, finding a role in social context, symptoms and treatments, fear of limited resources in the future. Task-, emotional- and avoidance coping strategies were seen in the interviews. The most prominent coping strategy was task oriented. CONCLUSION: This interview study with Swedish PWH shows that they strive for normality and adaptation in social activities throughout life finding their own niche. The PWH expressed the importance of knowledge and support from the comprehensive medical team at HTC and therefore it seems important to continue comprehensive medical care at HTC in order to follow-up the haemophilia persons regularly.

Self-reported activity and functioning in daily life; the perspective of persons with haemophilia living in Sweden.

OBJECTIVES: To investigate the self-estimated function, activity and participation in daily life of persons with severe or moderate haemophilia A and B (PWH) living in Sweden. The secondary aim was to explore the differences between participants with early treatment onset and later. METHODS: The Haemophilia Activity List (HAL), Arthritis Impact Measurement Scales (AIMS2) and Impact on Participation and Autonomy (IPA-S) were distributed by mail to 225 PWH with an invitation to participate in the study. The median and min-max are given for the different domains; a higher value indicates more disability. RESULTS: Eighty-four PWH (18-80 years) participated. The HAL indicated more problems in the legs [40 (0-100)] than in self-care [4 (0-92)] and household tasks [3 (0-87)]. The AIMS2 scores confirmed this (physical activity 12 (0-64.5) as well as somewhat reduced autonomy outdoors [IPA-S 17.5 (0-90)]. More limitations in daily life were reported by participants with later treatment onset. CONCLUSION: The PWH reported greater disability in the domains related to activities using the lower extremities compared to domains related to upper extremity. This study pointed out the need of using different questionnaires to capture patients own perspective and as useful supplement to other clinical assessment instruments.

Rising from a chair: a simple screening test for physical function in predialysis patients.

OBJECTIVES: Patients with chronic end-stage renal diseases have decreased physical fitness. This physical deconditioning is relative but the consequences in daily life are related to functional thresholds. The main purpose of this study was to gather information about physical performance, physical fitness and activity in predialysis patients. A second aim was to investigate the possibility of identifying patients at risk of physical deconditioning using clinical tests. MATERIAL AND METHODS: Fifty-five predialysis patients with a glomerular filtration rate (GFR) of