Quality of Life & Pain Assessment

Doctors and other health care workers routinely measure physiological and biochemical indicators of health status during the patient visit. This information is critically important in the medical decision-making process. It is important that the effects of medical treatment translate into benefits that patients can experience directly.

The most widely used and recognized instrument for measuring functional health and well-being (i.e., what patients are able to do and how they feel) is the Short Form SF-36^®. The Fabry Registry has decided to use this tool to measure the patient’s quality of life.

The Patient Survey Process
Treating physicians will determine the actual frequency of necessary assessments according to a patient's individualized need for medical care and routine follow-up, as well as to published or local guidelines, as appropriate. As a minimum, however, a Minimum Recommended Schedule of Assessments have been developed based on the input of physicians from the international medical community with expertise in the care of patients with Fabry disease. The recommended schedule represents the core assessments that are currently thought to monitor Fabry-related clinical manifestations and to stage disease progression across the life-long course of the disease.

General Guidelines on Health and Pain Assessment Survey Completion
Please be sure the patient is fluent in reading the language used in the survey. If not, do not proceed with administering the survey. The translations are available in the Fabry Registry Materials Section. If the language is not listed please contact the Fabry Registry staff to see if there is a translated survey available in the patient’s native language.

The self-administered surveys may be conducted when the patient is in the clinic/office or through the mail. If conducted in the clinical setting, then administer the survey before the patient sees the physician or is asked about other health questions, to avoid influencing the patient’s answers.

For patients who cannot complete the form themselves, interview the patient reading all questions as written and slowly enough for him or her to consider each statement and respond. Record responses, but avoid extended discussions of the question or response.

After the patient has completed the survey, check to be sure that all questions have been answered, and that only one answer has been provided to each question. If a response is incomplete or unclear, ask the patient to complete the question or clarify his or her choice. If the patient makes an error while completing the survey, the mark must be lined through or completely erased and the correct mark filled in and prominently noted.

For more information on performing Quality of Life and Pain Assessments, please contact the Fabry Registry.