Table 2:

Checklist of management and healthcare maintenance tasks for patients diagnosed with LAM

Category	Tasks
Disease monitoring	Screening HRCT at age 18 for patients with TSC
	PFTs every 3–6 months (in patients with confirmed LAM) to establish trajectory of disease progression
	Baseline serum VEGF-D. Consider repeating at annual intervals, especially in patients who can’t perform PFTs
	Start mTOR inhibitors for patients with abnormal lung function (FEV1 < 70%), problematic chylous effusions, rapidly declining patients, and patients with significant disease burden as assessed by cyst profusion on HRCT chest, need for supplemental oxygen, abnormal DLCO and/or air trapping on PFTs.
Drug monitoring	PFTs every 3–6 months post drug initiation to monitor treatment response
	Baseline CBC, CMP, Lipid panel, and urinalysis
	Repeat safety labs every month for the first 3 months followed by every 3 month lab checks
	Caution about sun protection
	Monitor for signs and symptoms of pneumonitis
Other healthcare maintenance	Pneumococcal vaccination and annual Influenza vaccination
	Avoid live vaccines in patients taking mTOR inhibitors
	Pneumothorax action plan (symptoms to recognize, pleurodesis after first event)
	Reassure regarding safety of air travel, counsel to avoid scuba diving

Abbreviations: See Table 1 plus, CBC = complete blood count, CMP = comprehensive metabolic panel, DLCO = diffusing capacity of the lung for carbon monoxide, FEV1 = forced expiratory volume in one-second, HRCT = high-resolution computed tomography, mTOR = mechanistic target of rapamycin, PFTs = pulmonary function tests.