Pain management in patients with haemophilia: a European survey

Abstract

There are no evidence-based guidelines on pain management in people with haemophilia (PWH), who may suffer acute, disabling pain from haemarthroses and chronic arthropathic pain. To review evidence and to investigate current clinical practice in pain assessment and management in PWH the European Haemophilia Therapy Standardisation Board undertook a literature review and a survey in 22 Haemophilia Treatment Centres (HTC), using a questionnaire and seven clinical scenarios. Consensus was sought on pain assessment and management in PWH. Few clinical studies on pain management in PWH were identified. The HTCs care for 1678 children (47% severe haemophilia, 84% on prophylaxis, 17% with arthropathy and 8% with chronic pain) and 5103 adults (44% severe haemophilia, 40% on prophylaxis, 67% with arthropathy and 35% with chronic pain). Analgesics are prescribed by HTCs in 80% of cases (median; range 0-100%) and in 10% (median; range 0-80%) are bought over the counter. Pain and analgesic use are assessed when reported by patients and at check-ups. Only eight centres use a specific pain scale and/or have specific pain guidelines. Two HTCs arrange regular consultations with pain specialists. For acute pain, the preferred first-line drug is paracetamol for children, and paracetamol or non-steroidal anti-inflammatory drugs (NSAIDs) for adults. Children with chronic pain are treated with paracetamol or NSAIDs, whereas adults usually receive Cox-2 inhibitors. Second-line therapy is heterogeneous. There is little published evidence to guide pain assessment and management in PWH, and clinical practice varies considerably across Europe. General and specific recommendations are needed.

Delphi approach to select rare diseases for a European representative survey. The BURQOL-RD study

“…Only 5 diseases from our selection were previously surveyed by Eurordis [23]. Other RDs have been studied on European level by other research organisations [29,30].…”

Pain and pain management in haemophilia

“…Chronic pain in haemophilia is long lasting, recurrent, and usually results from arthropathy and/or other long-term complications of haemophilia (such as synovitis and arthritis) [6,19,20]. Chronic pain is more complicated than acute pain and is associated with neurobiological, psychological, and social changes that can maintain the pain [10].…”

“…Little evidence exists on pharmacological pain management of patients with haemophilia [19,20,36,37], and clinical practice is largely empirical and varies widely [19,20]. From available data, however, it appears that there are varied pain management practices in current use in haemophilia.…”

Comparación de la conducta de enfermedad en pacientes colombianos con hemofilia A, en una muestra con dolor crónico y sin dolor crónico

“…El dolor en la hemofilia es un efecto secundario de los episodios de hemorragias, que comienza con un dolor agudo y ante su complicación se convierte en un dolor crónico (13,14). La intensidad del dolor es el principal factor influyente en el nivel de calidad de vida física y mental de los pacientes con hemofilia, por lo que en pacientes con hemofilia severa existe mayor afectación ya que suelen presentar más episodios de dolor recurrente en comparación a pacientes con hemofilia leve o moderada (15).…”

“…La definición más completa del dolor crónica se resume en una experiencia sensorial y emocional desagradable que se fija de manera arbitraria mínimo durante tres meses a pesar de los intentos terapéuticos curativos, y está asociada a un daño en los tejidos de forma continua o intermitente (17,18), requiriendo del manejo integral de quien lo padece abarcando factores de corte biológico, conductual, emocional y social. Los episodios de dolor deben ocurrir al menos una vez por semana, y su causa debe ser de difícil o nulo control (13). De este modo, y recordando el modelo biopsicosocial, el dolor crónico se asume como un complejo multidimensional donde aspectos valorativos y motivacionales empiezan a hacer parte de los factores influyentes frente a la experiencia del dolor (18,19).…”