Elsevier

Blood Reviews

Volume 32, Issue 2, March 2018, Pages 116-121
Blood Reviews

Review
Treatment of musculo-skeletal pain in haemophilia

https://doi.org/10.1016/j.blre.2017.09.004Get rights and content

Abstract

Musculo-skeletal pain treatment is inadequate in many haemophilic patients. Analgesics are used only by 36% of adult patients. FVIII/FIX intravenous infusion is mainly used to lessen pain, followed in frequency by usage of NSAIDS (primarily COX-2 inhibitors). In about 30% of patients, pain continues after infusion of F VIII/IX. In acute haemarthroses pain treatment must continue until total disappearance (checked by ultrasonography) and include haematologic treatment, short-term rest of the involved joint, cryotherapy, joint aspiration and analgesic medication (paracetamol in mild pain, metamizole for more intense pain, and in a few precise patients, soft opioids such as codeine or tramadol). In the circumstance of intolerable pain we should use morphine hydrochloride either by continual infusion or a patient-controlled analgesia (PCA) pump, determined by the age, mental condition and grade of observance of the patient. Epidural blocks utilizing bupivacaine and fentanyl may be very efficacious as well. Three main strategies to alleviate chronic musculo-skeletal pain secondary to haemophilic arthropathy (joint degeneration) exist: pharmacologic management, physical medicine and rehabilitation, and intra-articular injections. As for pharmacologic management, NSAIDs (ibuprofen, diclofenac, celecoxib, robecoxib) are better than paracetamol. The advantages of tramadol or tramadol/paracetamol and non-tramadol opioids are scanty. With respect to physical medicine and rehabilitation, there is insufficient confirmation that a brace has supplementary favourable effect compared with isolated pharmacologic management. Land-based curative exercise and watery exercise have at the minimum a tiny short-run benefit. Curative ultrasound can be helpful (poor quality of evidence). The efficacy of transcutaneous electrostimulation (TENS) for pain mitigation has not been proved. Electrical stimulation treatment can procure notable ameliorations. With respect to intra-articular injections, viscosupplementation appears to be a useful method for pain alleviation in the short-run (months). The short-run (weeks) advantage of intra-articular corticosteroids in the treatment of joint pain has been shown.

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

  • 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.

  • 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.

  • Limitation in activities of daily living is encountered in 89% of adult haemophiliacs and 85% describe a

Research agenda

  • Collaborative studies are needed to better understand the topic due to the rarity of presentation.

  • The role of analgesia in haemophilia is still based on preliminary studies.

  • Improved understanding of treatment of pain in haemophilia.

Disclosures

Nothing to declare.

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