I regret to inform this Community of the recent death of David Colburn (davec), 76 years old and a couple of months away from retirement, apparently from a severe episode of SCLS. David was a distinguished historian, university professor and senior administrator based at the flagship University of Florida, Gainesville, and you may read more details about his life and career in his obituary at https://news.ufl.edu/2019/09/david-colburn/
David had been compliant with his IVIG therapy since December 2015, taking the recommended dose (1 gr/kg every 2 weeks, rather than 2 gr/kg over 2 consecutive days every month), and was episode-free for over three years. Earlier this year, however, he apparently had two leak episodes which were relatively mild, but then he was hit hard last month (mid-September 2019) and died in the hospital within 24 hours of first feeling ill.
While David is not the first, and probably won't be the last, SCLS patient to fail a prolonged therapy with IVIG -- to my knowledge, he is the second such patient to die in the United States, and several more have died in Europe -- regular infusions of immunoglobulins remain the best treatment option by far for the prevention of episodes of SCLS.