Table 2 Considerations for the creation of consensus-based guidelines regarding pregnancy management in patients with TSC

1. All pregnancies in patients with TSC should be considered “high-risk” and patients should be counseled on the increased risk of both new onset and worsening of TSC manifestations as well as pregnancy specific complications

2. Preconception genetic counseling and MFM consultation should be provided

3. Medical evaluations for TSC specific manifestations should be done prior to and/or during pregnancy:

 i.Pulmonary evaluation (PFT and/or CT)

 ii. Renal evaluation (MRI)

 iii. Neurologic evaluation, especially as related to seizure control

 iv. TAND/Psychological evaluation

4. Providers should provide careful consideration and review of medications prior to and during pregnancy. These decisions, especially concerning mTORi use, should be made via employing shared decision making with the patient and should be individualized to each patient after taking into account the indication(s) for the use of mTORi, underlying disease severity, consequences of treatment interruption, and level of concern regarding potential teratogenic effect to the fetus

 i. Circumstances informing the need to stop mTORis and the recommended medication free interval prior to pregnancy

 ii. Circumstances that would warrant either maintenance or reinstatement of mTORi use during pregnancy

 iii. Seizure medication adjustment during pregnancy including recommendations for the interval of medication blood level checks