Investigating How Patients With Hemophilia Manage Pain

As the life expectancy of people with hemophilia has increased, patients are living longer with disease-associated pain, particularly in their joints. According to the results of a national survey of patients with hemophilia published in Haemophilia, patients use a variety of strategies to deal with their acute and chronic pain, while also experiencing psychological issues, functional disability, and reduced health-related quality of life.

The variable use of pain management strategies among the survey population suggests that clinical pain assessment tools are insufficient for developing better pain management programs, noted the authors, led by Michelle Witkop, DNP, from the Munson Medical Center in Traverse City, Michigan.

The investigators reported results from the Pain, Functional Impairment, and Quality of life (P-FiQ) study, which assessed the impact of pain on functional impairment and quality of life in patients with hemophilia A or B with or without inhibitors who had a history of joint pain or bleeding.

For this non-interventional, cross-sectional study, the researchers recruited adult male patients who were attending an annual or other routine comprehensive care visit at a hemophilia treatment center (HTC). Between October 2013 and October 2014, 381 patients (median age = 34 years; range = 26.3-47.2 years; 77% with hemophilia A, 23% with hemophilia B, and 9% with inhibitors) completed a survey that assessed:

  • sociodemographic information
  • hemophilia treatment information
  • bleeding history in the six months prior to enrollment
  • functional status (using the Centers for Disease Control and Prevention-Universal Data Collection scale and questions)
  • acute hemophilia/joint-related pain characteristics/descriptors and treatments
  • chronic (persistent) hemophilia/joint-related pain characteristics/descriptors and treatments
  • five patient-reported outcome instruments that assessed pain, functional impairment, and quality of life

Investigators also completed case reports during the comprehensive care visit.

The researchers noted discrepancies between patient- and site-reported measures of functional disability (such as restricted work, school, and recreational activities because of pain, loss of motion, and weakness), “suggesting a potential underestimation of patients’ functional status by HTC staff, or potential discrepancies in the perception of function between patients and investigators.”

Many patients (56%) reported that they did not receive routine infusions, with some (30%) having never received routine infusions.

“A low rate of prophylaxis was observed, considering the prevalence of severe hemophilia (71%),” the authors reported. Thirty percent never received prophylaxis, 32 percent received prophylaxis for less than half of their lives, and only 47 percent of those with both acute and chronic pain were currently receiving prophylaxis. “The lack of prophylaxis, particularly if it occurred during childhood and adolescence, may have affected participants’ development of joint damage and chronic pain,” they observed.

During the six months prior to completing the survey, most patients (85%) experienced pain. Twenty percent reported experiencing acute pain only, 34 percent reported experiencing chronic pain, and 32 percent reported experiencing both acute and chronic pain. The most frequently reported and most painful joints included ankles (37%), knees (24%), and elbows (19%).

Most patients who reported experiencing acute and chronic pain used acetaminophen (62% and 55%, respectively), followed by non-steroidal anti-inflammatory drugs (34% and 49%). Many patients also relied on non-pharmacologic strategies, including ice (65% and 33%) and rest (51% and 33%).

Hydrocodone-acetaminophen was the most common opioid used for both acute and chronic pain (27% and 30%, respectively); other long-acting opioids were infrequently used, specifically for chronic pain (morphine = 7%; methadone = 6%; fentanyl patch = 2%).

Patients who reported no pain in the past six months appeared to be younger than those who reported acute pain only or any chronic pain (median age = 27.3, 32.9, and 35.3 years, respectively; p values not reported). Employment status did not appear to affect the incidence of chronic pain, with similar rates of chronic pain between unemployed and employed participants (31% and 33%, respectively; p value not reported), but did affect the incidence of acute and chronic pain (39% vs. 28%; p value not reported).

In addition, patients with both acute and chronic pain were more often married or with a long-term partner and more often lived with others. Compared with those reporting no pain, only acute pain, or only chronic pain, patients with both acute and chronic pain also were more likely to have (p values not reported):

  • depression (30% vs. 0-15%)
  • obesity (35% vs. 20-29%)
  • lower health-related quality-of-life scores (mean EQ-5D visual analog scale = 69 vs. 83-86)
  • decreased function (median overall Hemophilia Activities List score = 60 vs. 88-99)

Patients described acute and chronic pain similarly: Acute pain was “sharp” (77%), “aching” (65%), “throbbing” (58%), and “shooting” (54%), while chronic pain was “aching” (80%), “nagging” (50%), “throbbing” (45%), and “sharp” (39%). “[This] lack of discrimination between acute and chronic pain suggests that refined clinical assessments and focused dialogue may be needed to personalize pain management for patients with hemophilia,” the researchers noted.

However, they added, “while the description of pain and management strategies may be similar … the impact of pain on quality of life differs. … These differences suggest that a deeper understanding of pain associated with hemophilia may help guide more effective pain management strategies for people with hemophilia.”

The study’s inclusion criteria may have limited the findings through potential sampling bias because requiring a history of joint pain or bleeding “limits any assessment of the epidemiology of pain prevalence, enrollment and participation of patients at routine or comprehensive care visits in a non-bleeding state, and potential use of prescription and non-prescription medications ahead of the visit,” the authors noted. In addition, the study was not powered for statistical analysis.

The authors report funding from Pfizer, Baxter BioScience, CSL Behring, Novo Nordisk, Octapharma, Kedrion, Baxalta, and Genentech.


Witkop M, Neff A, Buckner TW, et al. Self-reported prevalence, description and management of pain in adults with haemophilia: methods, demographics and results from the Pain, Functional Impairment, and Quality of life (P-FiQ) study. Haemophilia. 2017;23:556-65.