COMMENT
Supporting adherence and improving quality of life in haemophilia care
692
enhances treatment uptake (Khair et al, 2012) and quality of life (QoL). Health-related QoL is a term used to represent self-reported (subjective) views of health, clinical signs or symptoms, morbidity and mortality. Assessment of QoL in haemophilia is a relatively new area of research interest that is increasingly considered a relevant health outcome. QoL assesses functional, physical, social and emotional wellbeing. Generic QoL measures such as EQ5D or SF36 can be used to measure QoL in haemophilia and are useful for comparing QoL in haemophilia against other illnesses.There are, however, many haemophiliaspecific tools such as HaemoQoL, HaemAQoL and CHO-KLAT that can be used to measure QoL specific to haemophilia. On a day-to-day basis these tools are not routinely used as they require complex statistical analysis and scoring.
‘
The haemophilia nurse is the pivotal person in the team to facilitate communication with other specialist services
haemophilia population ages, this is perhaps more complex as patients now require other specialist hospital services. The haemophilia nurse is the pivotal person in the team to facilitate communication with other specialist services ensuring basic haemophilia knowledge is imparted (Forrester et al, 2013) and that the patients come to no harm. This patient advocacy role is one which promotes best outcomes for patients by ensuring communication, troubleshooting, coordinated care, monitoring and auditing outcomes, and sharing best practice. A community of practice, www.haemnet.com enables nurses to discuss cases and outcomes and share best practice via a secure online educational platform. Nurses are key members of the haemophilia team; they interface between patients and the remainder of the multidisciplinary team, promoting communication and coordinating care and outcomes. Their role in supporting patients with treatment issues, day-to-day concerns and managing episodes of care is often overlooked, yet they are perfectly placed to: ensure appropriate treatment programmes, monitor outcomes, promote PROs and QoL and provide evidence-based care for those with BJN haemophilia.
’
However, assessment of patient-related outcome measures (PRO) remains an important part of the haemophilia nurse’s role to justify treatment expenditure to commissioners of haemophilia care. PROs can be seen as ‘critical measures of care quality and outcome and may define individual benefit to each patient’ (Aledort et al, 2012) as well as benchmarking tools across health providers.
What is the nurse’s role? Haemophilia nurses are ideally placed to support patients in many ways; educating about the importance of treatment, keeping treatment records—particularly at the time of any bleeding, evaluating response to treatment and the impact of haemophilia on day-to-day life.The majority of care is delivered outside haemophilia centres, so the nurse’s key role is in supporting patients at a distance from hospital, by phone or internet (Mulders et al, 2012), home visits or liaison with local services. Patients with haemophilia who are admitted to hospital should be looked after where there is some haemophilia expertise. As the
Aledort L, Bullinger M, von Mackensen S, Wasserman J, Young NL, Globe D (2012) Why should we care about quality of life in persons with haemophilia? Haemophilia 18(3): e154-7 Forrester C, Bielby H, Johns S, Efford J, Khair K (2013) Potential for development of haemophilia link nurse roles within UK hospitals. Haemophilia [Epub ahead of print] Khair K (2006) Managing haemophilia at home. BJHH 1:10-11 Khair K, Lawrence K, Butler R, O’Shea E, Christie BE (2008) Assessment of treatment practice patterns for severe haemophilia: a global nurse perspective. Acta Haematol 119(2): 115-23 Khair K, Gibson F, Meerabeau L (2012) The benefits of prophylaxis: views of adolescents with severe haemophilia. Haemophilia 18(3): e286-9 Khair K, Meerabeau L, Gibson F (2013) Self-management and skills acquisition in boys with haemophilia. Health Expect [Epub ahead of print] Lindvall K, Colstrup L,Wollter IM et al (2006) Compliance with treatment and understanding of own disease in patients with severe and moderate haemophilia. Haemophilia 12(1): 47-51 Lindvall K, Colstrup L, Loogna K, Wollter IM, Gronhaug S (2010) Knowledge of disease and adherence in adult patients with haemophilia. Haemophilia 16(4): 592-96 Mulders G, De Wee EM, Vahedi Nikbakht-Van De Sande MCVM et al (2012) E-learning improves knowledge and practical skills in haemophilia patients on home treatment: a randomized controlled trial. Haemophilia 18(5): 693-8 Schrijvers LH, Uitslager N, Schuurmans MJ, Fischer K (2013) Barriers and motivators of adherence to prophylactic treatment in haemophilia: a systematic review. Haemophilia 19(3): 355-61 Vidler V (1999) Teaching parents advanced clinical skills. Haemophilia 5(5): 349-53
Kate Khair
Nurse Consultant, Haemophilia, Great Ormond Street Hospital for Children NHS Foundation Trust, London
© 2013 MA Healthcare Ltd
T
reatment of haemophilia demands regular replacement of the missing coagulation factors via intravenous infusions administered at home (Khair, 2006) initially by parents (Vidler, 1999) and then, once the necessary knowledge and dexterity skills have developed, by patients themselves. While parents are usually very good at adhering to prescribed treatment (Khair et al, 2008), during adolescence there may be a waning of treatment adherence due to poor knowledge and disease understanding of young adults (Schrijvers et al, Lindvall et al, 2006). The nurse’s role is key at this stage in supporting and re-educating patients about their disease and its treatment. Nurses are an integral part of the haemophilia multidisciplinary team. In the UK, haemophilia care is delivered through accredited haemophilia services in local and specialist centres, where dedicated haemophilia nursing is mandatory for centre designation. Lindvall et al (2010) suggest that during regular haemophilia reviews with a doctor, patients should also see a nurse to discuss: type and severity of haemophilia, treatment issues including adherence, bleed management, education and work and genetic inheritance knowledge. They suggest that this additional systematic support and education improves the ‘nature of haemophilia, the risks of complications, benefits of treatment and the importance of self-care’ (Lindvall, 2010). Many children and young adults in the UK have grown up receiving only prophylactic therapy, experiencing very few, if any, joint bleeds and are fitter and healthier than any generation with haemophilia before them. Boys as young as five recognise the importance of treatment, with many aged over ten years vocalising the importance of treatment and protection from unpredictable bleeding, reduced mobility and enabling more normal lifestyles to be adopted (Khair et al, 2012). Treatment regimens are now less rigid, with prescribing patterns likely to engage the patient in deciding timing and frequency of infusions; individualised treatment regimens around ‘risky’ activities such as sport; and issues with selfinfusion, which leads to greater concordance with treatment (Khair et al, 2013). Understanding their own treatment and therapy decisionmaking enables boys to plan treatment around their normal day-to-day activities, which in turn
British Journal of Nursing, 2013, Vol 22, No 12
British Journal of Nursing.Downloaded from magonlinelibrary.com by 130.113.076.006 on February 10, 2015. For personal use only. No other uses without permission. . All rights reserved.
Supporting adherence and improving quality of life in haemophilia care
692
enhances treatment uptake (Khair et al, 2012) and quality of life (QoL). Health-related QoL is a term used to represent self-reported (subjective) views of health, clinical signs or symptoms, morbidity and mortality. Assessment of QoL in haemophilia is a relatively new area of research interest that is increasingly considered a relevant health outcome. QoL assesses functional, physical, social and emotional wellbeing. Generic QoL measures such as EQ5D or SF36 can be used to measure QoL in haemophilia and are useful for comparing QoL in haemophilia against other illnesses.There are, however, many haemophiliaspecific tools such as HaemoQoL, HaemAQoL and CHO-KLAT that can be used to measure QoL specific to haemophilia. On a day-to-day basis these tools are not routinely used as they require complex statistical analysis and scoring.
‘
The haemophilia nurse is the pivotal person in the team to facilitate communication with other specialist services
haemophilia population ages, this is perhaps more complex as patients now require other specialist hospital services. The haemophilia nurse is the pivotal person in the team to facilitate communication with other specialist services ensuring basic haemophilia knowledge is imparted (Forrester et al, 2013) and that the patients come to no harm. This patient advocacy role is one which promotes best outcomes for patients by ensuring communication, troubleshooting, coordinated care, monitoring and auditing outcomes, and sharing best practice. A community of practice, www.haemnet.com enables nurses to discuss cases and outcomes and share best practice via a secure online educational platform. Nurses are key members of the haemophilia team; they interface between patients and the remainder of the multidisciplinary team, promoting communication and coordinating care and outcomes. Their role in supporting patients with treatment issues, day-to-day concerns and managing episodes of care is often overlooked, yet they are perfectly placed to: ensure appropriate treatment programmes, monitor outcomes, promote PROs and QoL and provide evidence-based care for those with BJN haemophilia.
’
However, assessment of patient-related outcome measures (PRO) remains an important part of the haemophilia nurse’s role to justify treatment expenditure to commissioners of haemophilia care. PROs can be seen as ‘critical measures of care quality and outcome and may define individual benefit to each patient’ (Aledort et al, 2012) as well as benchmarking tools across health providers.
What is the nurse’s role? Haemophilia nurses are ideally placed to support patients in many ways; educating about the importance of treatment, keeping treatment records—particularly at the time of any bleeding, evaluating response to treatment and the impact of haemophilia on day-to-day life.The majority of care is delivered outside haemophilia centres, so the nurse’s key role is in supporting patients at a distance from hospital, by phone or internet (Mulders et al, 2012), home visits or liaison with local services. Patients with haemophilia who are admitted to hospital should be looked after where there is some haemophilia expertise. As the
Aledort L, Bullinger M, von Mackensen S, Wasserman J, Young NL, Globe D (2012) Why should we care about quality of life in persons with haemophilia? Haemophilia 18(3): e154-7 Forrester C, Bielby H, Johns S, Efford J, Khair K (2013) Potential for development of haemophilia link nurse roles within UK hospitals. Haemophilia [Epub ahead of print] Khair K (2006) Managing haemophilia at home. BJHH 1:10-11 Khair K, Lawrence K, Butler R, O’Shea E, Christie BE (2008) Assessment of treatment practice patterns for severe haemophilia: a global nurse perspective. Acta Haematol 119(2): 115-23 Khair K, Gibson F, Meerabeau L (2012) The benefits of prophylaxis: views of adolescents with severe haemophilia. Haemophilia 18(3): e286-9 Khair K, Meerabeau L, Gibson F (2013) Self-management and skills acquisition in boys with haemophilia. Health Expect [Epub ahead of print] Lindvall K, Colstrup L,Wollter IM et al (2006) Compliance with treatment and understanding of own disease in patients with severe and moderate haemophilia. Haemophilia 12(1): 47-51 Lindvall K, Colstrup L, Loogna K, Wollter IM, Gronhaug S (2010) Knowledge of disease and adherence in adult patients with haemophilia. Haemophilia 16(4): 592-96 Mulders G, De Wee EM, Vahedi Nikbakht-Van De Sande MCVM et al (2012) E-learning improves knowledge and practical skills in haemophilia patients on home treatment: a randomized controlled trial. Haemophilia 18(5): 693-8 Schrijvers LH, Uitslager N, Schuurmans MJ, Fischer K (2013) Barriers and motivators of adherence to prophylactic treatment in haemophilia: a systematic review. Haemophilia 19(3): 355-61 Vidler V (1999) Teaching parents advanced clinical skills. Haemophilia 5(5): 349-53
Kate Khair
Nurse Consultant, Haemophilia, Great Ormond Street Hospital for Children NHS Foundation Trust, London
© 2013 MA Healthcare Ltd
T
reatment of haemophilia demands regular replacement of the missing coagulation factors via intravenous infusions administered at home (Khair, 2006) initially by parents (Vidler, 1999) and then, once the necessary knowledge and dexterity skills have developed, by patients themselves. While parents are usually very good at adhering to prescribed treatment (Khair et al, 2008), during adolescence there may be a waning of treatment adherence due to poor knowledge and disease understanding of young adults (Schrijvers et al, Lindvall et al, 2006). The nurse’s role is key at this stage in supporting and re-educating patients about their disease and its treatment. Nurses are an integral part of the haemophilia multidisciplinary team. In the UK, haemophilia care is delivered through accredited haemophilia services in local and specialist centres, where dedicated haemophilia nursing is mandatory for centre designation. Lindvall et al (2010) suggest that during regular haemophilia reviews with a doctor, patients should also see a nurse to discuss: type and severity of haemophilia, treatment issues including adherence, bleed management, education and work and genetic inheritance knowledge. They suggest that this additional systematic support and education improves the ‘nature of haemophilia, the risks of complications, benefits of treatment and the importance of self-care’ (Lindvall, 2010). Many children and young adults in the UK have grown up receiving only prophylactic therapy, experiencing very few, if any, joint bleeds and are fitter and healthier than any generation with haemophilia before them. Boys as young as five recognise the importance of treatment, with many aged over ten years vocalising the importance of treatment and protection from unpredictable bleeding, reduced mobility and enabling more normal lifestyles to be adopted (Khair et al, 2012). Treatment regimens are now less rigid, with prescribing patterns likely to engage the patient in deciding timing and frequency of infusions; individualised treatment regimens around ‘risky’ activities such as sport; and issues with selfinfusion, which leads to greater concordance with treatment (Khair et al, 2013). Understanding their own treatment and therapy decisionmaking enables boys to plan treatment around their normal day-to-day activities, which in turn
British Journal of Nursing, 2013, Vol 22, No 12
British Journal of Nursing.Downloaded from magonlinelibrary.com by 130.113.076.006 on February 10, 2015. For personal use only. No other uses without permission. . All rights reserved.
