Fibrous dysplasia (FD) is a rare disease, occurring in approximately 1 in 15,000 to 1 in 30,000 individuals. Those with craniofacial fibrous dysplasia (CFD) are the largest subgroup. CFD encompasses FD lesions in the jaws, facial bones, calvaria (skull bones), and/or bones of the skull base and may lead to facial asymmetry and distortion of features, sinus or dental problems, or, rarely, impairment of vision or hearing. Craniofacial surgery may be performed on CFD patients to treat functional problems or for cosmetic purposes. Research shows regrowth of FD lesions after surgery is common and patients frequently require multiple operations, leading to pain and other side effects. In CFD, stigma is a component of the experience of disease—both based on an appearance that is jarring and because others associate a non-normative visage with intellectual deficits. Patients with CFD negotiate others’ negative reactions to their appearance and their own awareness and feelings about deviation from “normal.” Extensive stigma is typically associated with negative mental health outcomes, including depression, low self-esteem, and self-isolation. The Fibrous Dysplasia Foundation, and medical and social science investigators, will pursue development of two comparative effectiveness research proposals that will ultimately provide patients with better guidance about surgical options and the timing of surgery: Do craniofacial FD patients who receive surgery have better quality of life outcomes than those patients who have not had surgery? Do craniofacial FD patients who receive surgery experience less stigma than those patients who have not had surgery?
*All proposed projects, including requested budgets and project periods, are approved subject to a programmatic and budget review by PCORI staff and the negotiation of a formal award contract.