joint pain
Woman having knee pain in medical office

Medicine traditionally has recognized 4 vital signs: body temperature, pulse, respiratory rate, and blood pressure. These indicators can be quickly measured and help physicians assess a patient’s status in minutes. There is nothing subjective about these signs; they can be measured without any input from the patient (which is useful if the patient is unconscious).

In recent years, there has been an increasing movement to recognize pain as the fifth vital sign. In the past, the prevalent view was that pain revealed little information in critical settings. However, we now know it can help physicians better understand what is happening in the patient physiologically, considering that pain is one of the manifestations of inflammation. 

“The assessment of pain as the fifth vital sign . . . is of paramount importance since it leads to the management of undertreated pain, consequently reducing suffering, readmissions and emergency department visits after hospital discharge,” Pozza and colleagues wrote in a recently published research article.  


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The Prevalance of Pain in Hemophilia 

In patients with hemophilia, the management of pain is crucial, even in patients who are treated according to the latest protocols. In the past, when factor replacement therapy was unavailable, hemophilia was characterized as a disease that condemned patients to a (short) lifetime of pain. Boys, often playful and active in nature, would often suffer from injuries that would lead to prolonged bleeding and extreme pain.

Read more about hemophilia patient education 

Stromer and colleagues studied pain management in hemophilia and concluded, “Pain management in patients with hemophilia is often insufficient, a fact that not only influences the patients’ quality of life but also implies the risk of difficult to manage chronic pain.” 

Here were some of their findings: 

  • Around 66% of pediatric hemophilia patients reported experiencing pain. 
  • More than 80% of all bleeds affect joints, most frequently the knee. 
  • Approximately 32% to 50% of hemophilia patients experience arthropathy-related pain. 
  • As pediatric patients grow into adulthood, they are more likely to seek medical assistance due to pain. 
  • Approximately 40% of them seek pain management help from a hemophilia specialist. 
  • For hemophilia patients seeking specialist pain treatment, 31% were referred to a physiotherapist and 46% to an orthopedist. 

Managing Pain in Patients With Hemophilia 

In hemophilia, a vicious cycle of pain can be observed: acute bleeding in the joints leads to acute pain, which over time leads to chronic arthropathy, which leads to chronic pain, which then makes acute bleeds more serious and typically more painful. 

Therefore, it is no surprise that pain assessment features heavily in most quality of life (QoL) assessments for hemophilia patients. Even though hemophilia specialists are well-aware of the importance of pain management in hemophilia care, pain management is often inadequate.

This is because the general strategy for pain management—ie, the ascending of the pain ladder—is frequently insufficient for patients experiencing hemophilic arthropathy. Guidelines issued by the 2020 World Federation of Hemophilia do not contain information about pain management for hemophilic arthropathy. This is due to 2 main factors: the lack of supporting evidence regarding pain care recommendations and the lack of consistency among pain assessment tools.

Forming a Consensus on Pain and Hemophilia

A group of Italian physicians from multiple hemophilia treatment centers recently came together to establish a study group in order to create a consensus around pain management in hemophilia. Santoro and colleagues, the authors of the study, wrote with prescience, “Appropriate pain assessment and effective management strategies are essential to improve the functionality and QoL of patients with hemophilia . . . Thus, there is an urgent need to improve and standardize both pain assessment and pain management in patients with hemophilia.” 

Read more about hemophilia treatment 

The fruit of their work is the “Italian Delphi Consensus Statement (2021),” which has made a few brilliant recommendations for hemophilia pain management. The document is extensive, but we will highlight a few key points from their consensus statement here: 

  • Clinicians at hemophilia centers are encouraged to assess pain during each patient consultation. 
  • When assessing pain, it is important to distinguish between nociceptive and neuropathic pain, and between acute and chronic pain. 
  • The first-line pharmacological treatment for acute pain is paracetamol (acetominophen) 1000 mg every 8 hours, combined with an opioid if the pain is severe or continuous. 
  • Nonpharmacological treatment for acute pain should be based on the RICE protocol (rest, ice, compression, and elevation). 
  • For chronic arthropathic pain, oral nonsteroidal anti-inflammatory drugs (NSAIDs), proton-pump inhibitors (PPIs), and cyclooxygenase-2 inhibitors (COXIBs) may be used as first-line therapy if paracetamol is ineffective or contraindicated. 
  • The primary nonpharmacological treatment of chronic arthropathic pain is physical therapy. 

As physicians, we need to recognize the vital role of pain management in healthcare. To the patient, the lack of proper pain management is equivalent to the lack of proper health care. 

The authors of the Italian Delphi Consensus Statement concluded, “The management of acute and chronic pain in patients with hemophilia requires an individualized approach based on multimodal nonpharmacologic and pharmacologic therapies delivered by a multidisciplinary team, which should include hematologists, pain specialists, and physiatrists.” 

References

Pozza DH, Azevedo LF, Castro Lopes JM. Pain as the fifth vital sign—a comparison between public and private healthcare systems. PLoS One. 2021;16(11):e0259535. doi:10.1371/journal.pone.0259535 

Stromer W, Pabinger I, Ay C, et al. Pain management in hemophilia: expert recommendationsWien Klin Wochenschr. 2021;133(19-20):1042-1056. doi:10.1007/s00508-020-01798-4

Santoro C, Di Minno MND, Corcione A, et al. Improving assessment and management of pain in hemophilia: an Italian Delphi consensus statementBlood Rev. 2022;51:100885. doi:10.1016/j.blre.2021.100885