The International Collaborative Gaucher Group (ICGG) is comprised of physicians from around the world who are experts on Gaucher disease. Together, they have established a set of minimum recommended guidelines for monitoring patients with Type 1 Gaucher disease.
In addition to a complete patient and family history, as well as a thorough physical examination, the table below outlines the various assessments that should be included in the initial work-up for Gaucher disease.
All patients should be monitored on an ongoing basis to assess their disease status and/or response to treatment. The recommended schedule of assessments varies depending on whether or not a patient is receiving therapy.
- Patients Not on Therapy: For those who are not receiving therapy, laboratory studies (including hemoglobin levels, platelet counts, and biochemical markers) and a thorough physical examination should be performed at least once a year, while visceral and skeletal evaluations should be completed every 1224 months. All patients who exhibited borderline or above normal pulmonary pressures at baseline are advised to undergo reevaluation every 1224 months.
- Patients on Therapy: For those who are receiving therapy, the frequency of recommended evaluations is dependent on whether or not a particular patient has achieved his or her therapeutic goals. (For more info regarding therapeutic goals, click here)
- Not Achieved Therapeutic Goals: Until the therapeutic goals have been met, it is advisable to have hemoglobin levels, platelet counts, and biochemical markers checked at least every 3 months. A thorough physical examination, as well as visceral and skeletal evaluations, should be completed annually.
- Achieved Therapeutic Goals: Once a clear and sustainable response to treatment has been established, the recommended frequency for checking lab values, visceral response, and skeletal disease diminishes, allowing for routine monitoring every 1224 months (at a minimum). However, a thorough physical examination should be conducted annually.
All patients who exhibited borderline or above normal pulmonary pressures at baseline are advised to undergo reevaluation every 1224 months.
For a more comprehensive explanation of the recommended monitoring guidelines, please feel free to download this informative brochure.