L. H. Schrijvers

Learning intravenous infusion in haemophilia: experience from the Netherlands

Summary.  Nowadays, nearly all severe haemophilia patients in the Netherlands practice self infusion at home. Learning intravenous administration of clotting factor requires time and effort. In order to inform patients about the burden and time‐investment needed to learn intravenous infusion, we performed a two‐centre retrospective study. All data on the learning processes, involving haemophilia patients born between 1980 and 2010 treated in Utrecht or Amsterdam, were extracted from patient files. A total of 154 patients and their parents were analysed (168 learning processes). Almost all patients had severe haemophilia and started prophylaxis at a median age of 2.7 years. 152/154 patients successfully learned intravenous infusion, including nine patients who temporally stopped and succeeded later. Overall, parents or patients needed a median of eight visits (IQR 4.3–14) in a median of 7 weeks (IQR 4–14.8) to learn home treatment. Parents who began to infuse by CVAD started at a median age of 1.9 years and succeeded within a median of 12 visits during 7.5 weeks. Parents who learned to perform intravenous infusion started at a median age of 4 years and needed 11 visits during 9 weeks. In 77% of cases, the mother was the first who started learning to infuse the child. Patients started with self infusion at a median age of 12.9 years, requiring a median of five visits in 12 weeks. The majority of patients and parents were able to learn intravenous infusion, with 50% of all parents and patients succeeding within eight visits during 7 weeks.

Unravelling adherence to prophylaxis in haemophilia: a patients’ perspective

Given the lifelong therapy in haemophilia patients, insight in non‐adherence behaviour from a patient perspective is important to understand patients’ difficulties with the following treatment recommendations. The aim of this study was to clarify the process underlying adherence (behaviour) to prophylactic treatment, from a patients’ perspective. To develop a grounded theory, a qualitative study using individual in‐depth interviews was performed to understand experiences, perceptions and beliefs concerning adherence to prophylaxis. From two Dutch treatment centres, 21 adults with haemophilia using prophylaxis were interviewed. Patients were asked how they experience their task to administer prophylaxis and how they adhere to this. The interviews were transcribed, coded and analysed in an iterative process, leading to the development of the grounded theory. Adherence was determined by the position of prophylaxis in life. The position of prophylaxis was determined by the perception of prophylaxis and the ability to exert prophylaxis. Patients’ perception was influenced by two main factors: acceptance of haemophilia and feeling/fearing symptoms. The ability to exert prophylaxis was influenced by understanding haemophilia and prophylaxis and planning/infusion skills. The combination of different perceptions and skills led to four main positions of prophylaxis in life: (i) prophylaxis integrated in life, (ii) prophylaxis according to doctors’ advice, struggling with irregular situations, (iii) prophylaxis is too much to handle, (iv) prophylaxis is a confrontation with illness. The adherence level gradually decreased from position 1 to 4. This information can be used to design tailored interventions to promote adherence.

Promoting self‐management and adherence during prophylaxis: evidence‐based recommendations for haemophilia professionals

Throughout life, a patient with severe haemophilia is confronted with many treatment‐related challenges. Insight into self‐management and non‐adherence could improve the quality of care for these patients. The aim of this study was to provide an overview of the current evidence on self‐management and adherence to prophylaxis in haemophilia.

Adherence to prophylaxis and bleeding outcome in haemophilia: a multicentre study

Prevention of bleeding and joint damage in severe haemophilia is dependent on adherence to prophylactic replacement therapy. The aim of this study was to assess adherence to prophylaxis, including associations with age, bleeding and clotting factor consumption (CFC). In three Dutch haemophilia centres, semi‐structured interviews about adherence to prophylaxis in the previous 2 weeks were conducted with patients or parents of a child with haemophilia. Patients were classified, according to pre‐specified definitions, as adherent, sub‐optimally adherent or non‐adherent based on missing, timing, and dose of infusions. Association of annual bleeding rates, mean CFC, person performing the infusion (parents verus patients) with adherence categories were analysed. Overall, 241 patients with haemophilia using prophylaxis were studied. Parents were more adherent (66%; n = 48/73) than patients (43%; n = 72/168). Sub‐optimal adherence occurred in 29% of parents and 37% of patients and was characterized by changes in timing of infusion (mostly from morning to evening), while missing <6% of infusions. Non‐adherence occurred less often: in 5% of parents and 20% of patients. Reduced adherence was associated with lower CFC, but not with joint bleeding. In conclusion, non‐adherence in haemophilia was relatively rare, yet 1/3 of patients struggled to administer prophylaxis at the appropriate time of day.

Achieving self-management of prophylactic treatment in adolescents: The case of haemophilia

OBJECTIVE Adolescents with a chronic disorder, such as haemophilia, need to attain responsibility for their disease. The aim was to gain insight into how adolescents achieve self-management of prophylactic treatment. METHODS In three Dutch Haemophilia Treatment Centres, adolescents (10-25 years) received structured questions on treatment responsibility and self-management (pre-specified definitions) during routine nursing consultation. RESULTS In total, 155 interviews were performed in 100 patients (median age 14.4 years). Self-infusion was initiated at a median age of 12.3 years (IQR 11.5-13.0) and self-management was achieved 9.6 years later, at a median age of 22.6 years. This process included three phases coinciding with known stages of adolescence. In early adolescence, patients acquired the technique of self-infusion (12.3 years) leading to independent self-infusion in middle adolescence (17.2 years). In late adolescence, patients demonstrated an increase in more complex skills, such as bleeding management and communication with the haemophilia physician (19.9-22.6 years). CONCLUSION Although, the first steps in self-management with regard to self-infusion are taken in early adolescence, complete self-management was achieved in late adolescence after almost 10 years. PRACTICE IMPLICATIONS Insight in this transitional process helps to provide individualized support and emphasizes the need for continued education with regard to self-management skills.

A European curriculum for nurses working in haemophilia

Currently, there is no consensus on education required to develop haemophilia nursing. The aim was to develop a curriculum for haemophilia nurses that could be used as a resource in Europe. This could form a basis for continuous professional development and used in the preparation of specialized educational programmes.

Defining adherence to prophylaxis in haemophilia

ized by the qualitative presence of VWF. This is the first documented case of a female patient with hereditary EDS and VWD. Only a limited number of studies are available that describe an association of acquired VWD and EDS [7,8]. The results indicate that genetic analysis of EDS is important especially in women with bleeding diathesis, to clarify the EDS type as the case of bleeding diathesis did not entirely fit the primary diagnosis. Moreover, a normal aPTT does not exclude a possible VWD. The same is true for factor VIII as it was in normal range in this case. VWF should then be taken into account to accurately identify the underlying disorder so that this group of patients is appropriately diagnosed. Acknowledgements

Self‐infusion of prophylaxis: evaluating the quality of its performance and time needed

Prophylactic replacement therapy is the cornerstone of treatment in severe haemophilia. Regular infusions with clotting factor concentrate have been proven effective to prevent bleeding, subsequent (joint) damage, and positively affect the impact of haemophilia on daily life [1]. Patients or parents of younger patients learn to infuse clotting factor concentrate in a peripheral vein (i.v.) or a central venous access device (CVAD) [2].