Evaluate the population rates of eFVC-3%p/month-drop-per-eFVC-per-100 days observation per patient with monthly VC measurement longitudinally in ALS populations with AHT(Helleman 2022,Young 2022)

Consensus assessment of eFVC in ALS patients at quarterly (q3month) intervals has been brought into question by deployment of supervised (Young 2022) and unsupervised (Geronimo 2019, Rutkove 2019, 2020, Shefner 2022, Helleman 2022) AHT. In the context of telemedicine, AHT allows monitoring of ALS patients at home without in-person clinic attendance as well as prior to attending ALS Clinics.

ALS populations[Utrecht-33 patients-120 eFVC measurements; Syracuse-18 patients-72 eFVC measurements] were tabulated according to baseline eFVC-1^st-tertile<60% VC predicted(%vcp)(6/7); 2^nd-tertile 60-80 %vcp (10/2); 3^rd-tertile >80 %vcp (17/9)]measured with portable spirometer at home at monthly intervals.  Occurrence of eFVC 3 %p/month drops per eFVC per 100 days observation per patient were tabulated in total populations and distributed according to eFVC tertile.

The eFVC-3%p/month-drop-per-eFVC-per-100 days-observation per patient at both centers[Utrecht-1 month = 0.2272, 95% CI =  0.0936 to 0.3608[ was not statistically different from [Syracuse-1 month = 0.2272 95% CI =  0.0936 to 0.3608].  In the Utrecht ALS population where 4/33(12.1%)patients were using NIV this measure increased statistically significantly[p= 0.0446;two tailed t test]over 3 months[2 month=0.3029, 95% CI=0.1586 to 0.4427; 3 month=0.4291, 95% CI= 0.2792 to 0.1072 ].  In comparison, in the Syracuse ALS population where  8/18 (44.4 %)patients used NIV, this measure remained stable and did not change over time.

Measurement of eFVC change from baseline in the Utrecht ALS cohort showed stastistically significant increase in eFVC-3%p/month-drop-per-eFVC-per-100 days-observation per patient.  Further utilization of AHT for monthly eFVC measurements to evaluate the potential beneficial effects of NIV on eFVC changes in ALS patients is warranted.