Pain management in hemophilia: expert recommendations

Summary of recommendations

• Prophylactic treatment either with replacement of factor VIII or IX or non-factor therapies prevents bleedings, pain, and protects joint function. Beyond that, targeted pain therapy can be necessary.
• Physical and rehabilitative measures are used to complement therapy to treat acute pain and to prevent and treat chronic hemophilic arthropathy and the pain it causes. Physiotherapy and adequate pain therapy should be organised to go hand in hand. During acute phases the RICE principle (Rest, Ice, Compression, Elevation) is used, mobilisation should follow as soon as possible; however, in order to be able to initiate physical measures, adequate pharmacological analgesia is paramount and sufficient factor protection is equally important.
• Systematic analgesic therapy at an early stage can effectively ease pain, following the concepts of modern pain medicine, but adapted to the specific situation of hemophilia patients.
• Pain treatment should always focus on the underlying mechanisms of pain and not just its intensity; it should follow a multimodal approach and focus on the patient’s overall psychosocial status.
• Because of its effect on coagulation, acetylsalicylic acid is contraindicated for the treatment of hemophilia patients. If an NSAID is chosen, the substance’s affinity to COX‑1 should be as low as possible. Coxibs (selective COX‑2 inhibitors) constitute a valid alternative.
• Paracetamol (acetaminophen) is indicated for low to medium intensity of pain. Due to its low analgesic potency, paracetamol should only be used if a patient’s specific constellation does not allow for coxibs or the analgesically more potent non-opioid analgesic dipyrone (metamizole). In order to safely administer paracetamol, the patient’s age, body weight, duration of therapy, maximum daily dosage, and dosage intervals need to be taken into account.
• Compared to paracetamol (acetaminophen), dipyrone (metamizole) has a relatively high analgesic potency and a low potential for interactions and adverse effects.
• A combination of two non-opioids with a different mode of action, such as a coxib and dipyrone (metamizole), leads to a potentiation of the analgesic efficacy, therefore allowing for an up to 40% reduction in opioid dosage.
• If non-opioids fail to reach the desired analgesic effect or if pain intensity is high, the therapy should be complemented with opioids as soon as possible during an acute phase.
• When non-opioids in the case of chronic pain do not produce the desired analgesic effect, weak and strong opioids should be used complementarily. The choice of opioid depends on the pain category, the intensity of the pain, the character and rhythm of the pain, the receptors involved as well as possible contraindications.
• Adequate dosage and appropriate intervals are important to ensure almost complete pain reduction.
• Pain specialists should—insofar as they are available—be consulted when there are indications of pain chronification risk or when the treatment does not permit sufficient pain control.
• Since hemophilia patients are in constant contact with hemophilia centres, good interdisciplinary cooperation within the hospitals with hemophilia centres facilitates cooperation between hemophilia specialists, pain therapists, psychologists, and specialists for physical medicine as well as psychotherapists.