To what extent is pain associated with HME?
Sandra Darilek, MS and Jacqueline T. Hecht, PhD
To gain a better understanding of pain in HME and its impact on the lives of people with HME.
Study Design
A questionnaire was developed to obtain information about:
How many individuals with HME experience pain.
Type of pain.
Frequency of pain.
Location of pain on the body.
Medical history.
Number of surgeries.
Types and amount of pain medications taken.
Days of school/work lost to pain.
Results
A total of 293 (43%) people returned the questionnaire that was sent to 700 people/families with HME.
Pain is significantly associated with HME in this study population.
Interference
Of those people who have pain, 55.1% have generalized pain. Generalized pain is defined as having throughout the body and not just localized to an exostosis.
One-third of people with pain (37.3%) also have depression.
Pain also affects different aspects of an individuals life. The following table is based only on the responses of those participants who had pain (n=244). Fifteen to 31% found that pain did not detract from general activity level, sleep, interactions with others or mood.
Rating
General Activity
with Sleep
Interactions with
Others
with Mood
* Age younger individuals are less likely to report having pain
* HME-related complications individuals with complications, such as compression of tendons, muscles, ligaments, blood vessels or nerves, are more likely to report having pain
* Surgery individuals who have had surgery are more likely to report having pain
* Support group membership individuals who are members of the HME support group are more likely to report having pain
HME-related complications and surgery were the factors found have the greatest relationship to pain.
* Individuals with HME-related complications were 5 times more likely to report having pain.
* Individuals who have had surgery were 3.8 times more likely to report having pain.
Future Directions
Long-term natural history studies that follow people with HME over the course of their lives are needed to determine when and where they start to develop HME-related complications, when they start to develop pain, and whether their pain experience changes over their lifetime.
Genotype-phenotype studies that add pain assessment variables to the analysis are needed to determine if there is a correlation between the genetic mutation and pain.
Defining the psychological and personality characteristics of HME individuals with pain are needed to determine if there are other factors that make certain individuals more likely to report having pain.
Conclusions
The results of this study indicate that pain in HME has been underappreciated.
Pain is an important aspect of HME that must be addressed when caring for people with this condition.
The results of this study apply to those people who took part in the study and may not be true of all people with HME. It is possible that individuals with pain may have been more likely to take part in the study than individuals without pain. This could have biased the study making the results more representative of individuals with pain than the overall HME population.
We would once again like to stress the need for participation. In this, as well as other studies, lack of response can raise more questions than answers. Your MHE experiences, medical history, pain (or lack of) are of value to the many who suffer, and to generations to come. You hold a piece of the puzzle in your hands, and it is only by working together that we will be able to find the answers.
