Long‐term outcome of multiple joint procedures in haemophilia

In elderly people with haemophilia (PWH), surgery of more than one joint of the lower extremities might be needed. Multiple joint procedures (MJP) were introduced in 1995, defined as any combination of Total Knee or Total Hip Arthroplasty or Ankle Arthrodesis during one in‐hospital stay. The expectation is that by means of such procedures this specific population is able to physically function better for an extended period of time. Thus, they will participate in their society in an optimal way. In this study, we tried to describe an outcome after MJP, including pre‐ and post‐operative pain and range of motion (ROM), and recommend measurement tools. 22 of 37 PWH who underwent MJP between 1995 and 2012 were available for assessment. Pain (WFH score) and range of motion were compared pre and postoperatively. Current outcome was described by VAS per joint, nocturnal and overall pain, MACTAR, Hemophilia Activity List, SF36, and EQ‐5D. Mean age at surgery was 50, 3 years (SD 8, 3); mean follow‐up 12 years (1–18 years). Pain (VAS) decreased post‐surgery (Median 1 – 1, 5), but moderate pain remained. Extension of knees slightly increased, but both knee flexion and ankle plantar and dorsal flexion decreased. PWH reported the ability to stand longer but also pointed at specific problems, e.g. riding a bike (MACTAR). The HAL showed limited activities (functional domains), especially in the ‘complex lower extremity’ (22, 8/100). The SF36 and EQ‐5D showed a mix of physical problems of our population, while experiencing moderate pain and reasonable physical functioning. This led us to the conclusion that adequate follow‐up is needed: ROM of all joints, VAS of all joints as well as nocturnal and overall pain, HAL, SF36 and EQ5D. Performance based activities and participation need further attention.     

Rehabilitation of patients with haemophilia after orthopaedic surgery: a case study

Summary. Preoperative and postoperative rehabilitation may be useful for improving the recovery of patients undergoing orthopaedic surgery, particularly in those with co‐morbidity or special requirements. This case study, of a patient with haemophilia and inhibitors to factor VIII undergoing total knee replacement, demonstrates the benefits of 6 weeks' preoperative physiotherapy (‘prehabilitation’) combined with 6 weeks' postoperative rehabilitation. The supervised physiotherapy regimen was individually tailored to specifically increase range of motion and muscle strength, enabling rapid mobilization and recovery of function, whilst minimizing the risk of bleeding.     

Rehabilitation of patients with haemophilia after orthopaedic surgery: a case study

Summary.  Preoperative and postoperative rehabilitation may be useful for improving the recovery of patients undergoing orthopaedic surgery, particularly in those with co-morbidity or special requirements. This case study, of a patient with haemophilia and inhibitors to factor VIII undergoing total knee replacement, demonstrates the benefits of 6 weeks' preoperative physiotherapy ('prehabilitation') combined with 6 weeks' postoperative rehabilitation. The supervised physiotherapy regimen was individually tailored to specifically increase range of motion and muscle strength, enabling rapid mobilization and recovery of function, whilst minimizing the risk of bleeding.     

Rehabilitation in chronic respiratory diseases: In‐hospital and post‐exacerbation pulmonary rehabilitation

Exacerbations of chronic obstructive pulmonary disease (COPD) that require hospitalization are important events for patients. Functional impairment and skeletal muscle dysfunction can increase the risk of hospitalization and readmission, independent of lung function. In addition, once a patient is admitted, multiple factors can lead to worsening outcome including immobility, systemic inflammation and nutritional depletion. These non‐pulmonary factors are potentially amenable to exercise therapy, as part of pulmonary rehabilitation (PR). Peri‐exacerbation PR has an important role in the management of exacerbations of COPD. In this review, we explore how functional limitation and skeletal muscle dysfunction affect patients having a severe exacerbation of COPD, the systemic impact of hospitalization on patients including potential aetiologies and the role of PR around the time of an exacerbation. This includes rehabilitation during the inpatient phase, post‐exacerbation rehabilitation and rehabilitation bridging hospital discharge. We also describe potential future developments in peri‐exacerbation PR.     

Expert opinion and key recommendations for the physical therapy and rehabilitation of patients with ankylosing spondylitis

Aim: Physiotherapy is an integral part of the management of ankylosing spondylitis (AS) and there is a need for recommendations which focus on the rehabilitation of patients with AS. We aimed to develop recommendations for the physical therapy and rehabilitation of patients with AS based on the evidence and expertise. Methods: The Anatolian Group for the Assessment in Rheumatic Diseases (ANGARD) is a scientific group of Turkish academicians (physiatrists and rheumatologists) who are experts in the rehabilitation of patients with AS. A systematic literature search summarizing the current available physiotherapy and rehabilitation trials in AS were presented to the experts before a special 2‐day meeting. Experts attending this meeting first defined a framework based on the main principles and thereafter collectively constructed six major recommendations on physiotherapy and rehabilitation in AS. After the meeting an email survey was conducted to rate the strength of the recommendations. Results: Six key recommendations which cover the general principles of rehabilitation in AS in terms of early intervention, initial and follow‐up assessments and monitoring, contraindications and precautions, key advice for physiotherapy methods and exercise were constructed. Conclusion: These recommendations were developed using evidence‐based data and expert opinion. The implementation of these recommendations should encourage a more comprehensive and methodical approach in the rehabilitation of patients with AS. Regular lifelong exercise is the mainstay of rehabilitation and there is a considerable need for well‐designed studies which will enlighten the role of physical therapy in the management of AS.     

The risk of elective orthopaedic surgery for haemophilia patients: Japanese single‐centre experience

Haemophilic arthropathy causes pain and a severely restricted range of motion, and results in a significant reduction in quality of life. When conservative treatments have failed, orthopaedic surgery is recommended for these patients with severe haemophilic arthropathy. However, surgery for haemophilia patients is challenging due to high complication rate such as infection, delayed wound healing and mortality. The aim of this study was to evaluate the incidence of early complications and identify preoperative risk factors of surgery for haemophilia patients. We report a series of haemophilia patients undergoing elective orthopaedic surgery between 2006 and 2012. During this period, 119 surgeries in 81 patients were prepared and 118 surgeries in 80 patients were actually performed. Four deep bacterial infections and four delayed wound healings occurred within 6 months postoperatively. One patient died preoperatively and four patients died postoperatively. Only the presence of inhibitor was a significant risk factor for infection. We found no risk factor related to delayed wound healing. Our data revealed alkaline phosphatase, albumin, platelet, alpha‐fetoprotein, presence of ascites and child classification C as predictors of perioperative mortality following elective orthopaedic surgery. Our role is to identify potential patients who present with risk factors for complications and attempt to seek the best determination of treatment strategy for these people.     

Recombinant activated factor VII for haemophilia patients with inhibitors undergoing orthopaedic surgery: a review of the literature

Summary.  Arthropathy is prevalent in patients with haemophilia and inhibitors and is a major source of pain and disability, significantly reducing quality of life. Recombinant activated factor VII (rFVIIa; NovoSeven^®) is one of the treatments available for acute life-threatening bleeding episodes in haemophilia patients with inhibitors. It has also been used successfully in a range of orthopaedic surgical procedures in these patients. This is a review of published data on elective orthopaedic procedures in haemophilia patients with inhibitors under cover of rFVIIa from January 2002 to November 2006. Articles were retrieved from MEDLINE using specified search parameters. Twelve articles covering a total of 80 orthopaedic procedures were identified. In the vast majority of cases, rFVIIa provided safe and effective haemostatic cover during orthopaedic surgery with no bleeding complications. There was variation in the administered dose, although the majority of patients were treated with 90 μg kg⁻¹ bolus followed by either continuous infusion or bolus infusion. Of those cases reporting bleeding complications, most were considered to be related to an inadequate amount of rFVIIa. The cumulative experience presented here suggests that rFVIIa is safe and effective for providing adequate haemostatic cover for haemophilia patients with inhibitors undergoing orthopaedic surgery. The optimal dosing regimen and mode of administration has yet to be identified. Further controlled trials are needed to confirm these experiences.     

Quality of life before and after surgery: mobility issues, the fear of surgery, inpatient recovery and outpatient rehabilitation

Summary.  A positive life-changing experience of joint replacement surgery from the point of view of a patient with Factor VII inhibitor.     

Continuous infusion during total joint arthroplasty in Japanese haemophilia A patients: comparison study among two recombinants and one plasma‐derived factor VIII

Summary. As for the available factor VIII (FVIII) concentrates in Japan, there are two recombinant FVIII concentrates (Kogenate‐FS and Advate) and one highly purified plasma‐derived FVIII concentrate (Cross‐Eight M). To evaluate the inter‐product variability, the differences in the continuous infusion rates and total consumption of the above three concentrates were compared when continuous infusion was used as the administration mode to control bleeding during 28 total joint arthroplasties (TJAs) for 17 patients. There were no significant differences among the FVIII plasma levels during surgery, except day 0. Advate needed to be given at a significantly higher infusion rate (4.2–2.1 IU kg^?1 h^?1) than the other two concentrates (Kogenate‐FS: 1.0–2.9 IU kg^?1 h^?1, P < 0.01 and P < 0.05; Cross‐Eight M: 3.2–1.8 IU kg^?1 h^?1, P < 0.01); however, their infusion rates were within the rates which were previously reported. The total consumption of Advate (652.1 IU kg^?1) was also significantly greater than either of the other concentrates (Kogenate‐FS: 395.1 IU kg^?1, P < 0.01; Cross‐Eight M: 519.1 IU kg^?1, P < 0.05). The results of this study showed that the continuous infusion of three FVIII concentrates is effective and safe during TJA, and also showed the differences in the continuous infusion rates and total consumption among concentrates when continuous infusion was used to control bleeding during surgery. These two results suggested that the continuous infusion of FVIII concentrate is a good administration mode, but there is still room for further investigation to use it as a more cost‐effective and safer administration mode.     

Perceived self‐efficacy gained from,and health effects of,a rehabilitation program after hip joint replacement

Objective

To examine whether a routine multidisciplinary inpatient rehabilitation program can increase patient self‐efficacy, and to investigate the effects of high self‐efficacy at admission, and increases in self‐efficacy, on health changes in patients who undergo such rehabilitation after hip joint replacement.

Methods

Participants in this longitudinal study were 1,065 patients who underwent inpatient rehabilitation after hip joint replacement. Questionnaires were administered at admission, discharge, and 6‐month followup. The main outcome variables were disability, pain, depressive symptomatology, and self‐efficacy to cope with disability and pain.

Results

Significant improvements from admission to discharge from the inpatient rehabilitation program in disability, pain, depressive symptoms, and self‐efficacy were found. In addition, higher levels of self‐efficacy at admission and larger increases in self‐efficacy over the course of the program predicted larger health changes (i.e., greater decreases in disability, pain, and depressive symptoms). Results were generally similar for health changes from discharge to 6‐month followup.

Conclusion

A routine multidisciplinary inpatient rehabilitation program after hip joint replacement can result in enhanced self‐efficacy.

     

Elective orthopaedic surgery for haemophilia patients with inhibitors: single centre experience of 40 procedures and review of the literature

Summary. With the introduction of safe and effective factor VIII/IX‐bypassing agents – recombinant activated factor VII (rFVIIa) and plasma‐derived activated prothrombin complex concentrates (pd‐APCC) – elective orthopaedic surgery (EOS) is a viable option for haemophilia patients with inhibitors. We report a series of patients with haemophilia and inhibitors undergoing EOS between 1997 and 2008 using bypassing agents to provide haemostatic cover. All inhibitor patients undergoing EOS and receiving rFVIIa, plasma‐derived prothrombin complex concentrates (pd‐PCC) or pd‐APCC as haemostatic cover were included. Patients were operated on by the same surgeon and were managed by the same haemophilia treatment centre. Forty procedures (25 minor and 15 major) were conducted in 18 patients. Twenty‐one minor cases were covered using rFVIIa, three with pd‐PCC, and one with pd‐APCC; all major cases were covered using rFVIIa. Bleeding was no greater than expected compared with a non‐haemophilic population in all 25 minor procedures. In the major procedure group, there was no excessive bleeding in 40% of cases (6/15) and bleeding completely stopped in response to rFVIIa. For the remaining nine cases, bleeding response to rFVIIa was described as ‘markedly decreased’ or ‘decreased’ in 4/15 cases and ‘unchanged’ in 5/15 cases. Overall, efficacy of rFVIIa, based on final patient outcome, was 85%. One death occurred as a result of sepsis secondary to necrotizing fasciitis. Good control of haemostasis can be achieved with bypassing agents in haemophilia patients with inhibitors undergoing minor EOS; rFVIIa was used as an effective bypassing agent, enabling EOS in patients undergoing minor and major procedures.     

Impacts of the perioperative fast track surgery concept on the physical and psychological rehabilitation of total hip arthroplasty: A prospective cohort study of 348 patients

Studies have shown that rapid rehabilitation surgery has a positive effect on recovery after major orthopedic surgery. However, very few studies have examined the impact of fast track surgery on physical and psychological rehabilitation in patients who have undergone total hip replacement.This study aimed to investigate the value of the rapid rehabilitation surgical model for patients undergoing total hip arthroplasty during the perioperative period.We conducted a prospective cohort study that included patients who underwent total hip arthroplasty at our hospital from January 2015 to December 2018. We divided the patients into 2 groups – the rapid rehabilitation group and the conventional rehabilitation group – and compared their length of hospital stay, time to off-bed activity, pain score, Self-Rating Anxiety Scale scores, Self-Rating Depression Scale scores, complication rate, and rate of satisfaction during hospitalization.A total of 348 patients were included in the study. Of these, 180 received rapid rehabilitation nursing and 168 patients received conventional nursing. Compared with the patients in the conventional rehabilitation group, those in the rapid rehabilitation group had shorter hospital stays (11.5 ± 1.2 day vs 15.5 ± 2.3 day, P = .021), resumed off-bed activities sooner (20.5 ± 3.4 hours vs 61.8 ± 4.7 hours, P = .001, had less postoperative pain (4.0 ± 1.2 vs 6.5 ± 1.1, P < .001), and lower anxiety and depression scores (anxiety score: 24.4 ± 2.1 vs 47.9 ± 2.9; depression score: 25.8 ± 1.8 vs 43.7 ± 1.7, P < .001).The application of rapid rehabilitation surgery in total hip arthroplasty can accelerate patients’ postoperative recovery, relieve anxiety and depression, and increase the patient''s satisfaction with the treatment.     

Variability in clinical phenotype of severe haemophilia: the role of the first joint bleed

To quantify variation in clinical phenotype of severe haemophilia we performed a single centre cohort study among 171 severe haemophilia patients. Age at first joint bleed, treatment requirement (i.e. annual clotting factor use), annual bleeding frequency and arthropathy were documented. Because treatment strategies intensified during follow-up, patients were stratified in two age groups: patients born 1968-1985 (n = 91), or 1985-2002 (n = 80). A total of 2166 patient-years of follow-up were available (median 12.0 years per patient). Age at first joint bleed ranged from 0.2 to 5.8 years. Patients who had their first joint bleed later needed less treatment and developed less arthropathy. In patients born 1968-1985 during both on-demand and prophylactic treatment, the 75th percentile of annual joint bleed frequency was consistently four times as high as the 25th percentile. In both age groups variation in annual clotting factor use between 25th and 75th percentiles was 1.4-1.5 times for prophylaxis and 3.8 times for on-demand treatment. To conclude, the onset of joint bleeding is inversely related with treatment requirement and arthropathy and may serve as an indicator of clinical phenotype. Thus, providing a starting point for aetiological research and individualization of treatment.