Rivista e Edizione

Volume 9 (2022): Edizione 1 (January 2022)

Volume 8 (2021): Edizione 1 (January 2021)

Volume 7 (2020): Edizione 1 (January 2020)

Volume 6 (2019): Edizione 2 (January 2019)

Volume 6 (2019): Edizione 1 (January 2019)

Volume 5 (2018): Edizione 1 (April 2018)

Volume 4 (2017): Edizione 1 (January 2017)

Volume 3 (2016): Edizione 2 (July 2016)

Volume 3 (2016): Edizione 1 (January 2016)

Volume 2 (2015): Edizione 2 (July 2015)

Volume 2 (2015): Edizione 1 (March 2015)

Volume 1 (2014): Edizione 3 (October 2014)

Volume 1 (2013): Edizione 2 (May 2013)

Volume 1 (2013): Edizione 1 (January 2013)

Dettagli della rivista
Formato
Rivista
eISSN
2055-3390
Pubblicato per la prima volta
22 Dec 2017
Periodo di pubblicazione
1 volta all'anno
Lingue
Inglese

Cerca

Volume 6 (2019): Edizione 1 (January 2019)

Dettagli della rivista
Formato
Rivista
eISSN
2055-3390
Pubblicato per la prima volta
22 Dec 2017
Periodo di pubblicazione
1 volta all'anno
Lingue
Inglese

Cerca

14 Articoli

Case Study

Accesso libero

Management of severe haemophilia A during surgery: case report

Pubblicato online: 17 Apr 2019
Pagine: 1 - 6

Astratto

Abstract

We describe the case of a patient with severe haemophilia A and significant comorbidities who underwent surgery to remove a large intra-abdominal haematoma first diagnosed 12 years previously. The haemophilia team was instrumental in coordinating care, building a strong rapport with surgical and other medical teams to manage bleeding risk with continuous infusion of factor VIII (FVIII). Medical teams adjusted their working hours according to clinical need. Haemophilia nurses were available to offer support at all times, and developed management procedures and educated staff on haemophilia and its treatment. Perioperative complications included a thrombus occluding the right internal jugular vein, infection and paraesthesia. However, surgery was effective and the patient was pleased with the overall outcome. Haemophilia nurses established strong and rewarding relationships with other teams that will enhance the delivery of care in the future.

Parole chiave

  • haematoma
  • haemophilia
  • nurses
  • surgery
Accesso libero

Parathyroidectomy in a patient treated with emicizumab

Pubblicato online: 23 Dec 2019
Pagine: 51 - 53

Astratto

Abstract

Experience of surgery during prophylaxis with emicizumab is currently limited, but the information available suggests that it is associated with a low risk of complications. This case study describes the surgical management of a patient with haemophilia A and inhibitors, managed with emicizumab prophylaxis, who underwent parathyroidectomy. The plan to manage bleeding risk during surgery involved prophylaxis with oral tranexamic acid 1g six-hourly and recombinant Factor VIIa (rFVIIa), prescribed at the discretion of the consultant haematologist. Preoperatively, rFVIIa 45 mcg/kg (3 mg) was administered immediately, and repeated every three to four hours after surgery depending on clinical presentation. There was no unexpected or excessive bleeding during surgery and no clinical need for additional haemostatic medication. Postoperatively, rFVIIa 3 mg was administered at three and ten hours after the first dose. Two further doses were administered on the morning and evening of the first postoperative day. There was no unexpected or excessive bleeding requiring additional treatment, and satisfactory haemostasis resulted in optimal wound healing. The patient reported no bleeding episodes and also an improved quality of life. This case study demonstrates the successful use of emicizumab in conjunction with rFVIIa.

Parole chiave

  • Emicizumab
  • surgery
  • Factor VIIa
  • tranexamic acid
  • parathyroidectomy

Evidence Review

Accesso libero

Complementary and alternative therapy (CAM) in haemophilia pain management: a review of published literature

Pubblicato online: 17 Apr 2019
Pagine: 7 - 18

Astratto

Abstract

Pain continues to be a significant issue for people with haemophilia (PWH), whether it is acute pain from a joint bleed, a needle stick when receiving clotting factor treatment, or chronic pain from chronic synovitis and arthritic changes. In the US, there has been controversy and uncertainty regarding the use of oral medications and opioids for the management of chronic pain, including haemophilia pain. Both PWH and their clinicians have started to explore nonpharmacologic options for managing pain, with many PWH trying to limit their use of oral pain medication and using alternative methods. There is little research around the use of complementary and alternative medicine (CAM) for pain management in the haemophilia community. This paper aims to provide a comprehensive literature review of current English language research on the use of therapies that fall into four distinctive domains of CAM in PWH. English language articles were obtained via searches in Medline, Embase and CINAHL using the target search words “haemophilia” or “bleeding disorders” in combination with other search terms relating to CAM. All references were considered regardless of year. Twenty-three CAM articles that included PWH or other bleeding disorders were identified; ten were evidence based while the remainder provided case reports and descriptions of CAM use in PWH. This indicates that there is limited current research in the area of CAM use for pain management in PWH. Evidence of the benefit of CAM is seen in studies of its use to treat pain in other conditions and health-related circumstances. Additional evidenced-based CAM research is needed so that alternative pain management therapies can be utilised in a safe and beneficial manner in PWH.

Parole chiave

  • Acute pain
  • bleeding disorders
  • chronic pain
  • complementary alternative medicine (CAM)
  • haemophilia
  • nonpharmacological
Accesso libero

Opioid dependence among people with haemophilia in a low-resource tropical setting: prevalence and risk factors in northern Nigeria

Pubblicato online: 18 Jun 2019
Pagine: 19 - 28

Astratto

AbstractBackground

In tropical countries such as Nigeria, where factor VIII (FVIII) is scarce, severe pain due to musculoskeletal bleeding complications, leading to frequent opioid prescription, is not uncommon in poorly managed people with haemophilia (PWH). The relationship between opioid use and dependence is intensively studied in other painful diseases, such as cancer and rheumatoid arthritis, but surprisingly little is known about opioid dependence in haemophilia. We hypothesise that the risk of opioid dependence among PWH in tropical countries like Nigeria is multi-factorial, encompassing demographic (age), clinical (haemophilia severity and chronic arthropathy) and biological (ABO blood groups and haemoglobin (Hb) phenotypes) factors that may directly or indirectly increase incidence of bleeding and/or pain.

Aims

To determine the prevalence of opioid dependence and relative risks (RR) associated with age, haemophilia severity, chronic arthropathy, ABO blood groups and Hb phenotypes, and to elucidate the pathophysiological roles of each risk factor in the development of opioid dependence among haemophilia-A patients in five hospitals in northern Nigeria.

Methodology

A retrospective review of the medical records of 88 PWH seen between 1996 and 2012 was used to collate data on age, sex, haemophilia severity, painful chronic haemophilic arthropathy, ABO blood group, haemoglobin phenotypes, presence or absence of opioid dependence, and the types of opioids on which the patients were dependent. The prevalence of opioid dependence among the cohort was expressed as a percentage. The frequency of each putative risk factor for opioid dependence in patients with and without opioid dependence were compared using Fisher’s exact test; RR associated with each risk factor was determined by regression analysis. P<0.05 was taken as significant.

Results

Of the 88 PWH studied,15 (17%) were shown to be opioid-dependent. Compared with PWH who were not opioid-dependent, this group had higher frequencies of severe haemophilia (86.7% vs. 49.3%: RR= 5.2, p=0.02), survival to adulthood (73.3% vs. 12.3%: RR= 9.5, p=0.0001), chronic arthropathy in one or more joints (86.7% vs. 21.9%: RR= 13.2, p=0.0004), blood group-O (80% vs. 49.3%: RR= 3.3, p=0.04), and HbAA phenotype (86.7% vs. 54.8%: RR= 4.3, p=0.04).

Conclusion

Prevalence of opioid dependence among PWH treated at five hospitals in northern Nigeria was 17% during the study period. Significant risk factors were directly or indirectly associated with increased rates of bleeding and/or pain, which can only be prevented or treated through optimal application of FVIII. There is a need for the Nigerian government to establish standard haemophilia care centres with adequate FVIII for optimal prophylaxis and treatment in order to minimise painful complications, thereby helping to prevent undue opioid use and dependence.

Parole chiave

  • ABO blood groups
  • arthropathy
  • haemoglobin phenotypes
  • opioid dependence
  • pain
  • severe haemophilia

Clinical Research

Accesso libero

Barriers to the implementation of point-of-care ultrasonography by physiotherapists in haemophilia treatment centres in Canada: a modified Delphi approach

Pubblicato online: 01 Nov 2019
Pagine: 29 - 41

Astratto

AbstractBackground

In patients with haemophilia, evidence suggests that the physical examination alone is not sensitive enough to detect small amounts of blood within a joint. Attention has shifted to methods of improving the sensitivity of the physical examination through adding diagnostic modalities such as point-of-care ultrasonography (POC-US). Proficiency with the physical examination and understanding of the role of POC-US are important competencies for physiotherapists. Despite training, implementation of POC-US by physiotherapists in haemophilia treatment centres in Canada has been mixed.

Aim

Using a theory-based approach, the aim of the current study is to achieve expert consensus regarding the barriers to physiotherapy performed POC-US in haemophilia treatment centres in Canada using a modified Delphi approach.

Materials and Methods

Using the Knowledge-to-Action Framework and the Consolidated Framework for Implementation Research (CFIR), a modified Delphi approach was completed using the Modified BARRIERS Scale (MBS). Participants were blinded and consensus was reached over three rounds at the Canadian Hemophilia Society’s annual three-day conference.

Results

Twenty-two physiotherapists participated; 20 participants completed Round 1, and 21 completed Rounds 2 and 3. Four items of the MBS reached consensus: 1) The physiotherapist does not have time to read research related to POC-US; 2) The physiotherapist is isolated from knowledgeable colleagues with whom to discuss POC-US; 3) Administration will not allow POC-US implementation; 4) There is insufficient time on the job to implement new ideas. All four consensus items can be mapped to one domain of the CFIR: the inner setting.

Conclusion

The haemophilia treatment centre within a healthcare organisation appears to be an important target for addressing barriers to the implementation of physiotherapy performed POC-US.

Parole chiave

  • point-of-care systems
  • ultrasonography
  • haemophilia A
  • physiotherapy
  • implementation science
  • Delphi method
Accesso libero

Blood pressure in haemophilia and its relation to clotting factor usage

Pubblicato online: 23 Dec 2019
Pagine: 42 - 50

Astratto

AbstractBackground

Patients with haemophilia have a higher prevalence of hypertension than the general population that cannot be explained by traditional cardiovascular risk factors such as age, race, diabetes or obesity. Patients with severe haemophilia, who are on clotting factor prophylaxis, have a higher prevalence of hypertension compared to patients with milder forms of haemophilia, who infuse clotting factor less frequently. This raises the question of whether there is a link between clotting factor usage and blood pressure in haemophilia patients.

Methods

Data was collected from 193 patients with severe haemophilia presenting to three haemophilia treatment centres in the United States and Canada, including age, body mass index (BMI), blood pressure (BP), Hepatitis C (HCV) and Human Immunodeficiency Virus (HIV) infection status, and clotting factor usage from pharmacy prescriptions (units/kg/year). The correlation between BP and factor usage was examined using quantile regression models.

Results

Systolic and diastolic BP plotted against factor use showed a cone-shaped scatter of points. There was no association between clotting factor usage and higher systolic or diastolic BP.

Conclusion

Our observations provide no evidence for an association between increased clotting factor usage and high BP.

Parole chiave

  • Haemophilia
  • hypertension
  • clotting factor use
  • blood pressure

Supplement: Community Focus

Accesso libero

”Take your shots and take what comes”: Rex Jepson

Pubblicato online: 17 Apr 2019
Pagine: s1 - s9

Astratto

Accesso libero

Redressing the balance: Bruce Norval

Pubblicato online: 17 Apr 2019
Pagine: s10 - s14

Astratto

Accesso libero

Doubly rare: Evelyn Grimberg and Minette van der Ven

Pubblicato online: 17 Apr 2019
Pagine: s15 - s19

Astratto

Accesso libero

Kin Kam: resilience and enlightenment

Pubblicato online: 17 Apr 2019
Pagine: s20 - s23

Astratto

Accesso libero

Patrick de Smet: Why change a winning team?

Pubblicato online: 20 Apr 2019
Pagine: s24 - s28

Astratto

Accesso libero

Keeping a positive outlook: Margaret Monk

Pubblicato online: 18 Jun 2019
Pagine: s29 - s32

Astratto

Accesso libero

The Ugandan Treatment Gap – Peter Nangalu

Pubblicato online: 18 Sep 2019
Pagine: s33 - s35

Astratto

Accesso libero

Now and then, and half a world away

Pubblicato online: 01 Nov 2019
Pagine: s36 - s38

Astratto

14 Articoli

Case Study

Accesso libero

Management of severe haemophilia A during surgery: case report

Pubblicato online: 17 Apr 2019
Pagine: 1 - 6

Astratto

Abstract

We describe the case of a patient with severe haemophilia A and significant comorbidities who underwent surgery to remove a large intra-abdominal haematoma first diagnosed 12 years previously. The haemophilia team was instrumental in coordinating care, building a strong rapport with surgical and other medical teams to manage bleeding risk with continuous infusion of factor VIII (FVIII). Medical teams adjusted their working hours according to clinical need. Haemophilia nurses were available to offer support at all times, and developed management procedures and educated staff on haemophilia and its treatment. Perioperative complications included a thrombus occluding the right internal jugular vein, infection and paraesthesia. However, surgery was effective and the patient was pleased with the overall outcome. Haemophilia nurses established strong and rewarding relationships with other teams that will enhance the delivery of care in the future.

Parole chiave

  • haematoma
  • haemophilia
  • nurses
  • surgery
Accesso libero

Parathyroidectomy in a patient treated with emicizumab

Pubblicato online: 23 Dec 2019
Pagine: 51 - 53

Astratto

Abstract

Experience of surgery during prophylaxis with emicizumab is currently limited, but the information available suggests that it is associated with a low risk of complications. This case study describes the surgical management of a patient with haemophilia A and inhibitors, managed with emicizumab prophylaxis, who underwent parathyroidectomy. The plan to manage bleeding risk during surgery involved prophylaxis with oral tranexamic acid 1g six-hourly and recombinant Factor VIIa (rFVIIa), prescribed at the discretion of the consultant haematologist. Preoperatively, rFVIIa 45 mcg/kg (3 mg) was administered immediately, and repeated every three to four hours after surgery depending on clinical presentation. There was no unexpected or excessive bleeding during surgery and no clinical need for additional haemostatic medication. Postoperatively, rFVIIa 3 mg was administered at three and ten hours after the first dose. Two further doses were administered on the morning and evening of the first postoperative day. There was no unexpected or excessive bleeding requiring additional treatment, and satisfactory haemostasis resulted in optimal wound healing. The patient reported no bleeding episodes and also an improved quality of life. This case study demonstrates the successful use of emicizumab in conjunction with rFVIIa.

Parole chiave

  • Emicizumab
  • surgery
  • Factor VIIa
  • tranexamic acid
  • parathyroidectomy

Evidence Review

Accesso libero

Complementary and alternative therapy (CAM) in haemophilia pain management: a review of published literature

Pubblicato online: 17 Apr 2019
Pagine: 7 - 18

Astratto

Abstract

Pain continues to be a significant issue for people with haemophilia (PWH), whether it is acute pain from a joint bleed, a needle stick when receiving clotting factor treatment, or chronic pain from chronic synovitis and arthritic changes. In the US, there has been controversy and uncertainty regarding the use of oral medications and opioids for the management of chronic pain, including haemophilia pain. Both PWH and their clinicians have started to explore nonpharmacologic options for managing pain, with many PWH trying to limit their use of oral pain medication and using alternative methods. There is little research around the use of complementary and alternative medicine (CAM) for pain management in the haemophilia community. This paper aims to provide a comprehensive literature review of current English language research on the use of therapies that fall into four distinctive domains of CAM in PWH. English language articles were obtained via searches in Medline, Embase and CINAHL using the target search words “haemophilia” or “bleeding disorders” in combination with other search terms relating to CAM. All references were considered regardless of year. Twenty-three CAM articles that included PWH or other bleeding disorders were identified; ten were evidence based while the remainder provided case reports and descriptions of CAM use in PWH. This indicates that there is limited current research in the area of CAM use for pain management in PWH. Evidence of the benefit of CAM is seen in studies of its use to treat pain in other conditions and health-related circumstances. Additional evidenced-based CAM research is needed so that alternative pain management therapies can be utilised in a safe and beneficial manner in PWH.

Parole chiave

  • Acute pain
  • bleeding disorders
  • chronic pain
  • complementary alternative medicine (CAM)
  • haemophilia
  • nonpharmacological
Accesso libero

Opioid dependence among people with haemophilia in a low-resource tropical setting: prevalence and risk factors in northern Nigeria

Pubblicato online: 18 Jun 2019
Pagine: 19 - 28

Astratto

AbstractBackground

In tropical countries such as Nigeria, where factor VIII (FVIII) is scarce, severe pain due to musculoskeletal bleeding complications, leading to frequent opioid prescription, is not uncommon in poorly managed people with haemophilia (PWH). The relationship between opioid use and dependence is intensively studied in other painful diseases, such as cancer and rheumatoid arthritis, but surprisingly little is known about opioid dependence in haemophilia. We hypothesise that the risk of opioid dependence among PWH in tropical countries like Nigeria is multi-factorial, encompassing demographic (age), clinical (haemophilia severity and chronic arthropathy) and biological (ABO blood groups and haemoglobin (Hb) phenotypes) factors that may directly or indirectly increase incidence of bleeding and/or pain.

Aims

To determine the prevalence of opioid dependence and relative risks (RR) associated with age, haemophilia severity, chronic arthropathy, ABO blood groups and Hb phenotypes, and to elucidate the pathophysiological roles of each risk factor in the development of opioid dependence among haemophilia-A patients in five hospitals in northern Nigeria.

Methodology

A retrospective review of the medical records of 88 PWH seen between 1996 and 2012 was used to collate data on age, sex, haemophilia severity, painful chronic haemophilic arthropathy, ABO blood group, haemoglobin phenotypes, presence or absence of opioid dependence, and the types of opioids on which the patients were dependent. The prevalence of opioid dependence among the cohort was expressed as a percentage. The frequency of each putative risk factor for opioid dependence in patients with and without opioid dependence were compared using Fisher’s exact test; RR associated with each risk factor was determined by regression analysis. P<0.05 was taken as significant.

Results

Of the 88 PWH studied,15 (17%) were shown to be opioid-dependent. Compared with PWH who were not opioid-dependent, this group had higher frequencies of severe haemophilia (86.7% vs. 49.3%: RR= 5.2, p=0.02), survival to adulthood (73.3% vs. 12.3%: RR= 9.5, p=0.0001), chronic arthropathy in one or more joints (86.7% vs. 21.9%: RR= 13.2, p=0.0004), blood group-O (80% vs. 49.3%: RR= 3.3, p=0.04), and HbAA phenotype (86.7% vs. 54.8%: RR= 4.3, p=0.04).

Conclusion

Prevalence of opioid dependence among PWH treated at five hospitals in northern Nigeria was 17% during the study period. Significant risk factors were directly or indirectly associated with increased rates of bleeding and/or pain, which can only be prevented or treated through optimal application of FVIII. There is a need for the Nigerian government to establish standard haemophilia care centres with adequate FVIII for optimal prophylaxis and treatment in order to minimise painful complications, thereby helping to prevent undue opioid use and dependence.

Parole chiave

  • ABO blood groups
  • arthropathy
  • haemoglobin phenotypes
  • opioid dependence
  • pain
  • severe haemophilia

Clinical Research

Accesso libero

Barriers to the implementation of point-of-care ultrasonography by physiotherapists in haemophilia treatment centres in Canada: a modified Delphi approach

Pubblicato online: 01 Nov 2019
Pagine: 29 - 41

Astratto

AbstractBackground

In patients with haemophilia, evidence suggests that the physical examination alone is not sensitive enough to detect small amounts of blood within a joint. Attention has shifted to methods of improving the sensitivity of the physical examination through adding diagnostic modalities such as point-of-care ultrasonography (POC-US). Proficiency with the physical examination and understanding of the role of POC-US are important competencies for physiotherapists. Despite training, implementation of POC-US by physiotherapists in haemophilia treatment centres in Canada has been mixed.

Aim

Using a theory-based approach, the aim of the current study is to achieve expert consensus regarding the barriers to physiotherapy performed POC-US in haemophilia treatment centres in Canada using a modified Delphi approach.

Materials and Methods

Using the Knowledge-to-Action Framework and the Consolidated Framework for Implementation Research (CFIR), a modified Delphi approach was completed using the Modified BARRIERS Scale (MBS). Participants were blinded and consensus was reached over three rounds at the Canadian Hemophilia Society’s annual three-day conference.

Results

Twenty-two physiotherapists participated; 20 participants completed Round 1, and 21 completed Rounds 2 and 3. Four items of the MBS reached consensus: 1) The physiotherapist does not have time to read research related to POC-US; 2) The physiotherapist is isolated from knowledgeable colleagues with whom to discuss POC-US; 3) Administration will not allow POC-US implementation; 4) There is insufficient time on the job to implement new ideas. All four consensus items can be mapped to one domain of the CFIR: the inner setting.

Conclusion

The haemophilia treatment centre within a healthcare organisation appears to be an important target for addressing barriers to the implementation of physiotherapy performed POC-US.

Parole chiave

  • point-of-care systems
  • ultrasonography
  • haemophilia A
  • physiotherapy
  • implementation science
  • Delphi method
Accesso libero

Blood pressure in haemophilia and its relation to clotting factor usage

Pubblicato online: 23 Dec 2019
Pagine: 42 - 50

Astratto

AbstractBackground

Patients with haemophilia have a higher prevalence of hypertension than the general population that cannot be explained by traditional cardiovascular risk factors such as age, race, diabetes or obesity. Patients with severe haemophilia, who are on clotting factor prophylaxis, have a higher prevalence of hypertension compared to patients with milder forms of haemophilia, who infuse clotting factor less frequently. This raises the question of whether there is a link between clotting factor usage and blood pressure in haemophilia patients.

Methods

Data was collected from 193 patients with severe haemophilia presenting to three haemophilia treatment centres in the United States and Canada, including age, body mass index (BMI), blood pressure (BP), Hepatitis C (HCV) and Human Immunodeficiency Virus (HIV) infection status, and clotting factor usage from pharmacy prescriptions (units/kg/year). The correlation between BP and factor usage was examined using quantile regression models.

Results

Systolic and diastolic BP plotted against factor use showed a cone-shaped scatter of points. There was no association between clotting factor usage and higher systolic or diastolic BP.

Conclusion

Our observations provide no evidence for an association between increased clotting factor usage and high BP.

Parole chiave

  • Haemophilia
  • hypertension
  • clotting factor use
  • blood pressure

Supplement: Community Focus

Accesso libero

”Take your shots and take what comes”: Rex Jepson

Pubblicato online: 17 Apr 2019
Pagine: s1 - s9

Astratto

Accesso libero

Redressing the balance: Bruce Norval

Pubblicato online: 17 Apr 2019
Pagine: s10 - s14

Astratto

Accesso libero

Doubly rare: Evelyn Grimberg and Minette van der Ven

Pubblicato online: 17 Apr 2019
Pagine: s15 - s19

Astratto

Accesso libero

Kin Kam: resilience and enlightenment

Pubblicato online: 17 Apr 2019
Pagine: s20 - s23

Astratto

Accesso libero

Patrick de Smet: Why change a winning team?

Pubblicato online: 20 Apr 2019
Pagine: s24 - s28

Astratto

Accesso libero

Keeping a positive outlook: Margaret Monk

Pubblicato online: 18 Jun 2019
Pagine: s29 - s32

Astratto

Accesso libero

The Ugandan Treatment Gap – Peter Nangalu

Pubblicato online: 18 Sep 2019
Pagine: s33 - s35

Astratto

Accesso libero

Now and then, and half a world away

Pubblicato online: 01 Nov 2019
Pagine: s36 - s38

Astratto

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