Fabry Disease: Clinical Proof of Concept
FACTS Trial (NCT02800070) — 5-Year Phase 1 Results
Live-cel Autologous Lentiviral HSC Gene Therapy Platform · GT-GLA-S03
(5-year follow-up)
(p < 0.0001, 99% power)
Trial Registration & Design — FACTS (NCT02800070)
| NCT | NCT02800070 |
| Health Canada CTA | Approved April 26, 2016 |
| Phase | Phase 1 (Safety pilot) |
| Design | Multi-center, single-arm, open-label |
| Screened / Treated | 7 screened → 5 treated (2 failed screening) |
| Enrollment | September 2016 – October 2018 |
| Follow-up | 5 years (End-of-Study February 2024) |
| Sites | Calgary AB · Toronto (UHN/Princess Margaret) · Halifax NS |
| Safety gating | Sequential enrolment; CTSC + DSMC review required before each successive patient authorized |
- Alpha-Gal A enzyme activity — plasma, peripheral blood leukocytes, and bone marrow
- Lyso-Gb3 and Gb3 levels in plasma and urine
- VCN persistence in peripheral blood (qPCR)
- Anti-alpha-Gal A IgG antibody titres
5 Patients Treated — Baseline & Drug Product Characteristics
All five patients were male with confirmed classical Fabry disease, on ERT prior to enrollment. VCN values reflect vector copies per cell genome measured in the drug product prior to infusion. CD34+ cell doses reflect the per-kg infused cell count.
Safety Profile — 5 Years / 5 Patients
(2 Grade 1–2 product-related AEs only: nausea, cough)
(neutrophil/platelet recovery Days 11–13)
(same-day discharge: Patients 1, 3, 4, 5)
| Safety Category | Finding at 18-Month Interim | Finding at 5-Year End-of-Study |
|---|---|---|
| Product-attributable SAEs | 0 (5/5 patients) | 0 (5/5 patients) |
| Product-related AEs | 2 Grade 1–2 events: nausea (P4), cough (P2) | No new product-related AEs beyond 18 months |
| Cardiac (LVMI) | Stable in all patients; no new LGE or T1 mapping changes | Patients 1, 2, 3 transient LVMI increase then reduction by Year 5; no LGE change in any patient |
| Renal function (eGFR) | Stable in all patients; Patient 2 progressive CKD (pre-existing Stage 3a) | Patients 3–5 near 90+ mL/min/1.73m²; Patient 2 stabilized Year 3 (slope 0.11) |
| Genotoxicity / ISA | Polyclonal integration profile; no clonal dominance at 18 months | Polyclonal reconstitution confirmed at 5 years; no clonal dominance; VCN mirrors enzyme activity (R² > 0.80) |
| Anti-alpha-Gal A IgG | Pre-existing antibodies declined in 3/4 affected patients without resurgence | All antibody titres at or near baseline beyond 18 months; no sustained elevation in any patient at 5 years |
| Health economics | N/A | Estimated $4.8M CAD health system savings from ERT pause in 3 patients through last visit |
Source: Khan et al., Nat Commun 2021 (PMC7907075); Foley et al., Clin Transl Med 2025 (PMC11726700)
Summary Charts — Engraftment, Substrate Reduction & Renal Function
Interim vs. 5-Year End-of-Study Outcomes
| Endpoint | Phase 1 Interim (18 months, 2021) | 5-Year End-of-Study (2025) |
|---|---|---|
| Plasma lyso-Gb3 | 40–86% reduction from baseline within 3 months; reduced in most patients | Significantly lower in 4/5 patients; stable from Years 1–5 |
| Alpha-Gal A activity | Detected Days 6–8 in all 5 patients; plasma reached reference range levels; leukocyte activity supranormal | Stabilized above Fabry disease baseline in all patients; not returned to pre-treatment levels; Patient 5 supranormal throughout |
| Vector copy number | Highest at 30–60 days; declining but durable (Patient 1: >0.05 copies/genome at ~3 years) | Polyclonal; no clonal dominance at 5 years; VCN mirrors enzyme activity (R² > 0.80) |
| Renal function (eGFR) | Stable in all patients; Patient 2 progressive CKD (pre-existing) | Patients 3–5 near 90+ mL/min/1.73m²; Patient 2 stabilized Year 3 (slope 0.11); Patient 1 slope 0.09 |
| Cardiac (LVMI) | Stable in all patients; no new LGE or T1 mapping changes | Patients 1, 2, 3 transient LVMI increase then reduction by Year 5; no LGE change in any patient |
| Product-attributable SAEs | 0 (5/5 patients) | 0 (5/5 patients, 5 years) |
| ERT discontinuation | 3/5 patients qualified and elected to stop ERT; Patient 3 never resumed | Estimated $4.8M CAD health system savings from ERT pause in 3 patients through last visit |
| Anti-alpha-Gal A antibodies | Pre-existing antibodies declined in 3/4 affected patients without resurgence | All antibody titres at or near baseline beyond 18 months; no sustained elevation in any patient at 5 years |
Sources: Khan et al., Nat Commun 2021 (PMC7907075); Foley et al., Clin Transl Med 2025 (PMC11726700)
Longitudinal Biomarker Data — 5-Year Per-Patient Follow-up
Data from the Canadian FACTS trial, reported across two publications: Khan et al. 2021 (PMC7907075) covering the first 2–3 years of follow-up, and Foley et al. 2025 (PMC11726700) reporting the full 5-year end-of-study results. Where available, early timepoint values (Year 0 through approximately Year 2–2.7) are taken directly from machine-readable supplementary Excel tables published alongside Khan et al. 2021. For biomarkers where supplementary tabular data was not available, or for the Year 2–5 extension period covered only in the 2025 publication, values were digitized by visual inspection of the published figures in PMC11726700.
Each point represents a single measured value per patient. Year 0 = infusion date. Grey shaded bands indicate the published normal reference range for the relevant biomarker. Charts use a compressed x-axis beyond Year 2 to show early kinetics with precision while displaying the full 5-year window.
Fig. 1 — Plasma Alpha-Gal A Activity Over Time (5-Year). All five patients began below the normal reference range (grey band, 5.1–9.2 nmol/min/mL). Enzyme activity rose rapidly within weeks of infusion, reaching or exceeding the reference range in all patients by Month 3. Patient 5 (purple) remained at or above the upper reference range through Year 3.5. Patients 2–4 stabilized in the 2.8–4 nmol/min/mL range, detectably above the Fabry disease baseline, through Year 5. Early timepoint data (Year 0–2.7) from exact values in Khan et al. 2021 (PMC7907075); Year 3–5 extension digitized from Foley et al. 2025 Fig 2A (PMC11726700).
Fig. 2 — Leukocyte Alpha-Gal A Activity Over Time (5-Year). Leukocyte enzyme activity (nmol/hr/mg protein) provides a complementary engraftment readout to plasma activity. Grey dashed reference band marks the normal range (22–57 nmol/hr/mg protein). Patient 5 (purple) showed a dramatically elevated early response peaking at ~330 nmol/hr/mg protein at Month 6, then declining to ~40–60 nmol/hr/mg by Year 4–5, near the upper reference range. All data digitized from Foley et al. 2025 (PMC11726700, Fig. 2B, ±10 nmol/hr/mg protein precision).
Fig. 3 — Plasma Lyso-Gb3 Over Time (5-Year). Lyso-Gb3 (nM) is the primary pharmacodynamic endpoint planned for Glafabra's FDA trials. The dashed grey line marks the published reference range upper limit (~3 nM). All patients reduced from pre-treatment baseline by Month 3. Patient 5 (purple) is the standout result: starting at 16.6 nM, lyso-Gb3 dropped below 10 nM by Year 0.5 and remained 5–7 nM through all 5 years — approaching normal reference range. Patients 1 and 2 stabilized in the 14–19 nM range through Year 5 (>40% sustained reduction from no-ERT baseline). Early timepoint data from Khan et al. 2021 (PMC7907075); Year 1.5–5 extension digitized from Foley et al. 2025 Fig 3B (PMC11726700).
Fig. 4 — Vector Copy Number (VCN) in Peripheral Blood Over Time. VCN (copies/genome) reflects durable engraftment of gene-corrected HSCs. Exact values from Year 0–2 derive from supplementary data in Khan et al. 2021 (PMC7907075); Year 2–5 extension digitized from Foley et al. 2025 (PMC11726700, Fig. 4B). All patients show a peak engraftment phase at ~1–3 months post-infusion followed by stabilization at a durable plateau, consistent with polyclonal long-term repopulating HSCs. VCN correlated strongly with plasma enzyme activity (R² > 0.80).
Fig. 5 — eGFR Over Time (Years Post-Infusion). Early values (Year 0–2.7) from Khan et al. 2021 supplementary data; Year 1.5–5 extension digitized from Foley et al. 2025 (PMC11726700, Fig. 4A, ±2 mL precision). Patients 3, 4, and 5 maintained eGFR above 110 mL/min/1.73m² through 5 years. Patient 1 oscillated around 75–82 mL/min/1.73m² (stable CKD Stage 2). Patient 2 enrolled with pre-existing Stage 3a CKD and stabilized at ~25 mL/min/1.73m² from Year 3–5. Horizontal dashed line marks the CKD Stage 3a/3b threshold (60 mL/min/1.73m²).
Fig. 6 — IgG Antibody Titre Against Alpha-Gal A Over Time. All values digitized from Foley et al. 2025 (PMC11726700, Fig. 5A, ±15% precision on log scale). Y-axis is logarithmic. Patients 3 (gold) and 4 (red) mounted the highest early humoral responses (peak ~60,000 and ~30,000, respectively) then declined to baseline by Year 2–3. Patient 5 (purple) maintained near-background IgG levels throughout follow-up, consistent with the superior sustained enzyme activity observed. All antibody titres at or near background beyond 18 months in all patients.