ReviewTreatment of musculo-skeletal pain in haemophilia
Introduction
Haemophilia is a congenital hereditary disorder of blood coagulation characterised by recurrent painful articular bleeding episodes (haemarthrosis). The goal standard of treatment is primary prophylaxis intravenous infusion of the deficient factor: FactorVIII (FVIII) in haemophilia A, Factor IX (FIX) in haemophilia B [1], [2], [3]. However, despite primary prophylaxis subclinocal bleeding episodes may occur. Repeated haemarthrosis will cause joint degeneration (haemophilic arthropathy) accompanied by chronic articular pain in several joints (knees, ankles, elbows) [4], [5]. According to Riley et al. [6], > 50% of adult people with haemophilia have painful joints that cause disability and impair quality of life (QoL). Adequate pain handling is indispensable so as to rise the patient's QoL [7], [8]. Promoting analgesic usage might diminish the effect of pain on functional limitations [9].
In 2014, Young et al. [10] carried out a review of the literature and stated that studies were needed to identify optimal pharmacologic treatment for chronic articular pain in adult haemophiliacs. In another systematic review published in 2015, Humpries and Kessler [11] emphasised the importance of an appropriate pain management in adult haemophiliacs.
Efficacious pain treatment in haemophilia is crucial to lessen the affliction that pain inflicts on patients. The purpose of this review is to analyse the existing conservative strategies for the treatment of acute and chronic musculo-skeletal pain related to haemophilia.
Section snippets
Epidemiology
In 1987, Chiniere and Melzak [12] reported that in adult haemophiliacs ethnocultural factors associated with language affiliation could make a contribution to inter-individual variety in chronic articular pain perception. In 2001, Wallny et al. [7] analysed 71 adult haemophiliacs. On average, there were four articulations with major chronic articular pain. The most common painful joints were the ankles (45%), followed by the knees (39%) and the elbows (7%). In 29% of adult haemophiliacs,
Haemarthrosis (acute pain)
The following five features need to be taken into account if adequate management of acute joint bleeding is to be accomplished [15]: Haematologic treatment (intravenous infusion of FVIII/FIX), in preference within 2 h from the beginning of joint bleeding, till a plasma level not < 30–50% of the insufficient factor is attained, short-run repose of the affected articulation, local criotherapy, joint aspiration (arthrocentesis) of blood, and analgesic medication.
Haemophilic arthropathy (chronic pain)
Three main strategies to alleviate chronic musculo-skeletal pain secondary to haemophilic arthropathy (joint degeneration) exist: pharmacological management, physical medicine and rehabilitation, and intra-articular injections.
Summary and future directions
The following five aspects have to be taken into account if adequate treatment of joint bleeds is to be accomplished: Haematologic management (intravenous infusion of the insufficient coagulation factor, FVIII or FIX), by preference within 2 h from the start of articular bleed, till a plasma level of at least 30–50% of the insufficient factor is obtained; short-run repose of the affected articulation; Local cryotherapy; Joint aspiration (arthrocentesis) of intra-articular blood, recommended in
Practice points
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The following five features have to be taken into account if adequate treatment of haemarthrosis is to be accomplished: Haematologic treatment, short-term rest of the involved joint, cryotherapy, joint aspiration and analgesic medication.
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In about 30% of patients, pain continues after intravenous infusion. of F VIII/IX. Analgesics are used only by 36% of adult people with haemophilia.
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Limitation in activities of daily living is encountered in 89% of adult haemophiliacs and 85% describe a
Research agenda
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Collaborative studies are needed to better understand the topic due to the rarity of presentation.
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The role of analgesia in haemophilia is still based on preliminary studies.
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Improved understanding of treatment of pain in haemophilia.
Disclosures
Nothing to declare.
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