I want to thank you all for your support. Finding this community has been such a lifeline since my daughter's first ISCLS flare a year ago. She has had 7 major flares since, the 2nd on her 30th birthday. We have had a few minor flares as well, and have been using albumin as a stop-gap measure. Ray has been treated in the ED or hospitalized six times, although most of the serious things we manage at home, as I am a nurse practitioner. She has gone from being a super active engineer to almost bed-bound, using a wheelchair outside of the house, with about 5 "good" days a month. I am privileged to be her care person on-site.
We have been fighting for IVIG since the initial diagnosis in September of 2025. Arturo generously sent me a "care package" of resources, and using those and the info here I created a literature review, case study and family appeal. This packet, along with the medical notes, was submitted through 6 appeals over 10 months, and we have just received approval for Panzyga. It will be given in three parts, with a day between; for monthly infusions.
Along the way, many have said pseudo comfortaing things like "well you probably wouldn't have tolerated it anyway." If you have any tips or tricks that is welcome. If you would like a redacted version of the lit review I am happy to offer.
Warmly,
Joanna
Hi Joanna,
I am really grateful for your daugher's treatment getting aproved. My husband got diagonised last September. Since the IVIG started, he is back to normal and stable. Hopefully your daughter can be well soon.
Joanna,
Many thanks for the good news, and I cannot believe that it took so many months and appeals to get the treatment approved, especially if, as I had suggested, you obtained a seconding letter from a local our out-of-town immunologist or internist that had met your daughter and approved of her tentative diagnosis. The physician you had relied upon initially was neither an internal-medicine, or intensivist, or hematologist, or even an immunologist, and he was not affiliated with a major medical center or medical school, so that's usually not a recipe for early success with the insurance companies.
While I'm not a physician, here are some comments and suggestions from long experience -- mine and that of fellow patients.
First, the three-part infusion plan is unusual. Maybe your daughter starts like that, but if it is effective and she doesn't have any adverse reactions other than temporary headaches, encourage her to insist on the usual, back-to-back, two-day infusion cycle, and also to push for progressively faster infusion speeds, that way she only has to commit two sequential mornings or afternoons per month to the treatment and can use the rest of the month to work or whatever else she does.
Second, you didn't mention what dosage she's going to get. Usually, insurance approvals are for the medication or procedure but without specifying dosages, so here too there usually is room for experimentation. The recommended initial dose is 1 gr/kg on Day One and another 1 gr/kg on Day Two, every 4 weeks. If that dosage is effective, then one can always experiment with lower dosages (say, 0.5 gr/kg on Day One and also on Day Two) later on. What sometimes happens is that patients are started on a lower-than-the-recommended high dose, and if it's not effective immediately in terms of preventing the episodes, then some insurance companies refuse to approve additional infusions because of evident lack of effectiveness. That's why it's better for her to start at 2 gr/kg/4 weeks.
And as a reminder, I had asked that you fill out properly your profile page, by adding as much information as possible about your daughter's journey until diagnosis and since then, including her year of birth. You need not mention specific names of treating physicians and hospitals. Moreover, I urged you to encourage your daughter to join our SCLS/RareShare community. It's very informational and therapeutic, and we're very supportive. Most importantly, by joining and reading up on past postings, she'll get a good idea of what to expect going forward.
Arturo