1. Screening (CBC, upper and lower endoscopy) should begin in the mid-teens (15 years) or at the time of symptoms, whichever is earlier.
2. If these screening tests are negative, then repeat in three years.
3. If the screening tests are positive, and only one or a few polyps were found, you should have the polyps removed. You should have a CBC and endoscopy done annually until no more polyps are found. At this time, screening intervals may return to every three years.
4. If the screening tests are positive, and many polyps are found, removal of all or part of the colon or stomach may be necessary. You should have a CBC and endoscopy should be done annually until no more polyps are found, at which time screening intervals may return to every three years.
5. If your family was found to have JP as a result of a PTEN gene change, surveillance should also be performed for breast, endometrial, and thyroid. Please see Appendix 1 for additional screening recommendations.

Negative gene test (for a known mutation) **

1. You should have a CBC and lower endoscopy beginning at 15 years as a baseline screening. If the baseline screening is negative, then repeat screening every ten years until the age of 45 years. After 45 years, the standard American Cancer Society recommendations for colon cancer screening should be followed.
2. If polyps are found, they need to be removed: The screening should be repeated in one year. You may wish to consider repeating the gene test or perhaps a different gene test may be warranted.** These screening recommendations are precautionary as gene testing for JP is still very new and we are still learning about the functions of these genes and the significance of the changes that can occur.

Surveillance guidelines for at-risk relatives
When the family has chosen not to have genetic testing or the genetic testing was not helpful for the family (not informative):

1. Baseline screening (CBC, upper and lower endoscopy) should begin in the mid-teens (15 years).
2. If the screening tests are negative, then repeat them in three years.
3. If the screening tests arc positive, and only one or a few polyps were found, you should have the polyps removed. You should have a CBC and endoscopy done annually until no more polyps are found. At this time, screening intervals may return to every three years.
4. If the screening tests are positive, and many polyps are found, removal of all or part of the colon or stomach may be necessary. You should have a CBC and endoscopy should be done annually until no more polyps are found, at which time screening intervals may return to every

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