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Introduction: The therapeutic approach to pain in hemophilia should be multimodal. Intra-articular injections are a good option when joint lesions do not respond to hematological treatment or rehabilitation and orthopedic surgery is not yet indicated. Performing these procedures under ultrasound guidance has been shown to improve their accuracy and efficacy., Areas Covered: This article provides a practical overview of the most frequently employed ultrasound-guided intra-articular procedures on the joints of people with hemophilia. The article describes the key elements for performing the technique on the elbow, knee and ankle as the most affected joints. The particularities of the most frequent indications, arthrocentesis, synoviorthesis and analgesic injections with various products are detailed., Expert Opinion: Current hematological treatments have made it possible to incorporate new therapeutic tools for pain relief for people with hemophilia, including ultrasound-guided joint procedures, which offer excellent results.

Background: Guidelines for congenital coagulopathies recommend that patients record treatment administrations and bleeding episodes to help healthcare professionals monitor the disease., Research Design and Methods: We studied over two years which patient profiles (age, treatment regimen, treatment compliance) were most likely to accept the use of an app to collect this information. We validated the quality of patient-reported data by comparing it with data obtained from hospital electronic records, pharmacy dispensing records and patient interview, collected in an access database used as a reference. Patient and professional opinions were solicited through open-ended interviews., Results: The app was used by 52% of 315 patients studied. Younger patients were the most frequent users. Patients with better treatment compliance used the app more, although data collection was incomplete for most patients. The best rated by patients were the reminders of days of administration and the minimum stock alerts at home. Healthcare professionals rated the app positively., Conclusions: Healthcare professionals valued the app as useful for managing treatment of congenital coagulopathies. Patients need support and time to use the app and improve the quality of the data entered. Patients who used the app rated it positively. The treatment compliance improved.

Introduction: The purpose of this article has been to review the literature on total knee arthroplasty (TKA) in people with hemophilia (PWH), to mention the lessons we have learned from our own experience and to try to find out what the future of this type of surgery will be., Areas Covered: A Cochrane Library and PubMed (MEDLINE) search of studies related to TKA PWH was analyzed. In PWH, the complication rate after TKA can be up to 31.5%. These include infection (7.1%) and bleeding in the form of hematoma, hemarthrosis or popliteal artery injury (8.9%). In a meta-analysis the revision arthroplasty rate was 6.3%. One-stage or two-stage revision arthroplasty due to infection (septic loosening) is not always successful despite providing correct treatment (both hematological and surgical). In fact, the risk of prosthetic re-infection is about 10%. It is necessary to perform a re-revision arthroplasty, which is a high-risk and technically difficult surgery that can sometimes end in knee arthrodesis or above-the-knee amputation of the limb., Expert Opinion: TKA (both primary and revision) should be performed in centers specialized in orthopedic surgery and rehabilitation (knee) and hematology (hemophilia), and with optimal coordination between the medical team.

Introduction : People with hemophilia (PWH) commonly experience acute and chronic musculoskeletal pain during childhood and young adulthood, but their treatment is often inadequate. Areas covered : From 1 September 2020 to 15 April 2021, authors performed a literature search in PubMed and the Cochrane Library using 'hemophilia AND pain' as keywords. Authors found 1082 articles, 51 of which were chosen because we considered them to be intimately connected with the topic of this report. Multimodal pain treatment, including multimodal analgesia, physical and rehabilitation medicine (PRM), and psychological therapies (imagery or relaxation, hypnosis), is currently the most recommended treatment for PWH. In acute hemarthrosis, in addition to hematologic treatment and joint aspiration, paracetamol/acetaminophen should be prescribed if the pain is mild, metamizole if the pain is moderate, and soft opioids (codeine or tramadol) if the pain is severe. In cases of chronic musculoskeletal pain due to hemophilic arthropathy, paracetamol/acetaminophen, COX-2 inhibitors, PRM, intra-articular injections of some drugs (corticosteroids, hyaluronic acid, platelet-rich plasma, mesenchymal stem cells), radiosynovectomy and behavioral therapies are advised. Expert opinion : Management of musculoskeletal pain in children and young adults with hemophilia should employ multimodal pain treatment (multimodal analgesia, PRM, and psychological strategies).

Introduction : The new World Health Organization (WHO) guidelines on physical activity incorporate new concepts, such as sedentary behavior, recommendations for specific age groups and for people living with chronic conditions and disability. This could be extrapolated for the first time to people with hemophilia (PWH) within the framework in which these recommendations are applicable. The benefits of physical activity for PWH include physical, psychological and social benefits. Areas covered : This is a narrative review of the literature. We searched the MEDLINE and the Cochrane Library for WHO guidelines and articles related to physical activity in PWH. For all population groups, the benefits of performing exercise outweigh the possible harms. In PWH, there is a perceived increased risk of bleeding associated with physical activity. However, research suggests that this risk is associated with trauma during activity rather than intensity of physical activity, which offers the most health benefits. Expert opinion : Given the hematological, physical and functional conditions of PWH are highly variable, individualized counseling is needed based on age, therapeutic strategy, degree of arthropathy and level of disability. In this article, we analyze the applicability of the WHO recommendations to PWH, which is important to further promote healthy lifestyle motivation.

Introduction: The coronavirus disease 2019 (COVID-19) pandemic represents an unprecedented global health crisis. To combat its effects, many governments have opted for strategies of social isolation that involve a radical change in people's behavior., Areas Covered: For patients with hemophilia, the negative consequences of these measures can be greater, given they modify aspects of health care and lifestyles needed to counteract the adverse effects of hemophilia. The long-term consequences of the pandemic on patients with hemophilia are not well known. The aim of this special report is to show what COVID-19 could mean for this population, beyond the risk of infection., Expert Opinion: Considerations of the clinical, care, therapeutic, physical, nutritional, mental health, pain, and disability aspects that might be affected are included. Strategies are also suggested to minimize the effects that these issues can have on patients' lives. Patients, health professionals, and society as a whole must work together to mitigate the effects of the pandemic on people with hemophilia.

Introduction: After the ankle and knee, the elbow is the most commonly impaired joint in patients with hemophilia., Areas Covered: A Cochrane Library and PubMed (MEDLINE) search related to elbow problems in hemophilia was conducted. Early and continuous primary hematological prophylaxis (ideally starting in infancy) is essential, given that the juvenile elbow is highly prone to the musculoskeletal complications of hemophilia. If primary hematological prophylaxis is not administered (due either to lack of affordability or lack of venous access), persistent hemarthrosis can occur in the elbow. Hemarthroses will then require aggressive treatment (through arthrocentesis under factor factor coverage) to prevent progression to synovitis (requiring prompt radiosynovectomy or arthroscopic synovectomy) and ultimately hemophilic arthropathy (requiring rehabilitation and orthopedic surgery). Between the second and fourth decade of life, many patients with hemophilia present elbow joint destruction., Expert Opinion: In the initial stage of joint destruction, rehabilitation and intraarticular injections of hyaluronic acid can relieve the symptoms. In the advanced stage of hemophilic arthropathy, however, surgery is necessary. The main surgical procedures currently available are arthroscopic debridement, radial head resection, and total elbow arthroplasty. Transposition of the ulnar nerve and removal of heterotopic ossifications might also be necessary.

Introduction: Iliopsoas muscle hemorrhage in patients with hemophilia is a serious complication with relevant related morbidity., Areas Covered: A Cochrane Library and PubMed (MEDLINE) search of studies related to iliopsoas hematomas in hemophilia was analyzed. Pain in the hip joint area and femoral nerve involvement in people with hemophilia should alert physicians to the likelihood of a hemorrhage of the iliopsoas muscle. Clinically, reduced hip range of motion and deficient motor and sensory function of the femoral nerve can be found. Imaging studies, such as ultrasonography (US), CT scan, and MRI will confirm the diagnosis. The first-line therapy for iliopsoas hematoma must be conservative (hematological treatment using high-dose replacement therapy until full resorption monitored by means of imaging studies). Related femoral nerve involvement typically resolves with conservative treatment (rehabilitation). Pelvic hemophilic pseudotumors can occur in patients with hemophilia due to recurrent iliopsoas muscle bleeding, often related to late diagnosis and poor hemostasis., Expert Opinion: Long-term clotting factor replacement is the pillar of the management of iliopsoas hematomas. Percutaneous US-guided aspiration of blood can be performed in hematomas of large size. However, the rate of failed aspirations and hematoma repetition is substantial (13%).

Introduction : The purpose of this study is to evaluate the degree of education and empowerment of the patients of the Haemophilia Unit at 'La Paz' University Hospital (Madrid, Spain). Materials and methods : Haemophilic patients attending routine haemophilia consultations were asked to complete a questionnaire with a view to determining the patient´s perceived knowledge about the disease; their ability to make individual and group decisions; and whether the frequency of their hospital visits could be reduced. Results : Patients were shown to have ample knowledge about the disease. Knowledge had been acquired chiefly through personal experience and from the interaction with healthcare providers. Nearly 70% of patients felt capable of making decisions on matters affecting their health. Over half of the patients considered that their hospital visits could be reduced if they had more information. Patients experiencing the largest number of bleeding episodes were those showing the highest levels of empowerment. Over half the patients were satisfied with the institutional support they received. Conclusion : These patients with haemophilia are in general reasonably empowered on account of their personal experiences and their interaction with the healthcare providers. Current widespread access to information makes it easier to step up educational interventions in patients at lower haemorrhagic risk.

Background: Prevention of hemarthrosis is the key factor in the adequate management of people with hemophilia (PWH). If hemarthrosis occurs, early diagnosis of joint damage is essential to make personalized treatments. This study is aimed at gaining an understanding of the ability of point-of-care ultrasound (US) using the `Hemophilia Early Arthropathy Detection with Ultrasound´ (HEAD-US) protocol to detect abnormalities in joints without history of hemarthrosis and clinically asymptomatic joints of PWH., Methods: The sample included 976 joints from 167 PWH (mean age 24.86 years). Data were collected from routine practice over a 3-year period and analyzed based on history of hemarthrosis and results of clinical (HJHS 2.1) and HEAD-US examinations., Results: In our series, 14% of patients exhibited HEAD-US signs of incipient arthropathy in joints with no history of bleeding and with a HJHS 2.1 score of 0. The most severely involved joint was the right ankle. Synovitis, articular cartilage and subchondral bone damage scores in joints with subclinical findings were slower than in joints with previous hemarthroses or HJHS 2.1 > 1 Conclusions: Our study demonstrates that HEAD-US is better than hemarthrosis records and the HJHS 2.1 scale in detecting the early signs of joint damage in PWH.

Introduction: The management of acute hemophilic hemarthrosis (AHH) classically has consisted of factor replacement therapy and RICE (rest, ice, compression and elevation). However, there is still a controversy in the literature regarding the role of local cryotherapy (LC) in AHH. Areas covered: The aim of this article is to review the potential role of LC in AHH. Expert commentary: Some authors have reported that LC attenuates joint inflammation and has a positive influence on controlling articular swelling and temperature. However, other authors have stated that low temperature is associated with impairment of coagulation enzyme activity and platelet function. Although some reports have mentioned the benefits of LC in AHH and other have mentioned its adverse events, the data on the topic are still scant and preliminary. Literature of LC in the management of AHH is controversial. Whilst LC has always been considered as an unquestionable necessity in the management of AHH, it should be utilized with caution until a randomized study can solve the dilemma.

Introduction: Knee flexion contracture (KFC) is a common complication of recurrent hemarthrosis in children and young adults with hemophilia. If the KFC is not prevented (by means of primary prophylaxis) and treated properly and early (be means of physical medicine and rehabilitation), it will become fixed. Areas covered: The aim of this article is to review the potential role of botulinum toxin type A (BTX-A) intramuscular injections for the treatment of KFC in people with hemophilia (PWH). Expert commentary: Although two recent reports have mentioned the benefits of intramuscular injections of BTX-A in PWH with KFC, the data are still scant and too preliminary. The use of intramuscular injections of BTX-A in PWH today should not be recommended until more case studies/small series (ideally well-designed clinical trials) fully demonstrate that this is safe and effective. The risks of intramuscular injections to a hemophilia patient cannot be underestimated (iatrogenic muscle hematomas and pseudotumors). This paper calls the attention of hemophilia treaters on the potential risks of this apparently interesting technique. The current use of BTX-A intramuscular injections in KFC of PWH could make no sense. Raising false expectations in these patients should be avoided.