Hello all-

My wife was diagnosed with SCLS about 3 weeks ago, based on an incident of extremely low blood pressure, hemoconcentration, and fluids 3rd spacing into every part of her body...we are still in the hospital's inpatient rehab facility at this point as she recovers from the edema issues that were created from the leaking, and from being bed-bound for nearly a month (requiring extensive PT to get to walking again).

We are in a unique situation, in that we travel full-time in an RV, and therefore, can take her anywhere for treatments, but don't know which path to choose.

When her first "attack" began, we were 7 hours from home, down in far south Texas, and had intended to stay in this are for about 2 months - which has turned out to be a good thing, as it will likely be pretty close to that by the time we get out of rehab, and get our ducks in a row for the next steps.

As for next steps, we have become well aware that monthly IVIG treatments are the best preventative course of action to try to minimize and/or eliminate future attacks, but again, are not sure where to go to start those treatments - our options seem to be:

1)  Even though we are now discharged from the hospital, and in rehab now, we'd try to individually meet with one of the doctors we saw here, and try to set up an initial dose of IVIG while still here, and figure out the ongoing doses with another doctor.

2)  Return to the Houston area (7 hours by RV, or 1 hour by plane), to try to locate an expert in the SCLS area there.

3)  Visit the Mayo Clinic in MN, in order to confer with 2 doctors that responded to family emails, regarding providing guidance as to if this was ultimately SCLS.

4)  Visit the NIH in MD, in order to confer with Dr. Druey, who did speak directly with her doctors, and did state that her presentation seemed to be a "classic case of SCLS".

Again, because we have an RV, and travel full-time, we consider any of these options to be possible - although for further away destinations, we would likely put the wife on a plane (with family), and I would drive the RV to the destination...thus allowing us to easily stay weeks, or months, in a given area.

My initial thoughts on these various choices are:

Choice 1 seems promising, but there are also concerns as to if insurance would be more likely to balk at covering the IVIG treatments, without more of an expert treating her for the SCLS.  This choice would likely give the most peace of mind, as it has been nearly a month since her initial attack.

Choice 2 is closer to some family, but not knowing of a specific doctor with experience with SCLS in the Houston area makes it a bit of an unknown.  If anyone here is seeing a doctor in the Houston area specifically for SCLS, I would appreciate if you could share their name.  Or, even if you are seeing a doctor for your SCLS anywhere in Texas, and you would highly recommend them, please share!

Choice 3 seems promising, just because it is the Mayo Clinic, but we also don't want to forever be in MN (especially not for a winter in a thin-skinned RV), and wonder if we need to try to establish more of an Internal Medical doctor in the Houston area first, then go to Mayo - although the hospital here in south Texas, is supposed to be getting us referral appointments to Mayo now.

Choice 4 seems like it is something to later, becoming part of Dr. Druey's study, but that he would not be someone that would start her on, or be able to oversee, ongoing IVIG treatments.  For anyone that has participated in Dr. Druey's study, can you speak to your experiences in general?

I'm happy to hear anyone else's thoughts on the choices above, or additional choices you can imagine!

In addition, if there is anyone out there that has travelled extensively while also receiving the monthly IVIG treatments, can you provide any thoughts on your experiences?

I would think that there would be a network of infusion centers throughout the U.S. that could handle the treatments (although it might require lots of extra legwork from whomever our doctor finally becomes)...plus, I had seen something in 1 publication that spoke of a patient, who after doing 2-3 monthly IVIG treatments, was giving the option to just inject herself every day or 2 with smaller doses, in order to get the same amount of IVIG over time - not sure if that was in the states, or over in Europe though.

Thanks again in advance for any thoughts, opinions, guidance, or advice that anyone has to share!

Quantumleap,

Welcome to our Community!  We hope to also have your wife as a member before long, so that you may climb the learning curve together, thus being better positioned to support one another going forward.  You are both very lucky that she survived the episode and didn't suffer (much) organ or limb damage.

You are the first member of our Community who has been leading a nomadic lifestyle, so I don't think there is anybody else I can recommend for you to speak with about what's in store for the two of you.  The rest of us do some business or pleasure travel, but we generally return to base for our treatments and schedule all travel around them.

Until about a decade ago, being given a diagnosis of SCLS used to come close to being issued a death sentence, but we've made quite a bit of progress since then in terms of saving lives and restoring a semblance of normality.

To make a long story short, and to be brutally frank, dealing constructively with this diagnosis is likely to mean putting an end to your nomadic lifestyle. And the reason is that the best way to avoid going down the slippery slope of having more limb- and life-threatening episodes is (1) to have the constant support of at least one competent and compassionate physician -- and forget about finding experts in SCLS in Texas or wherever else you’d like to roam, because they pretty much don't exist -- and (2) to receive IVIG like clockwork during two consecutive mornings or two afternoons every 4 weeks. 

And because IVIG is one of the most expensive medications in the planet -- her likely dose may well have a street value in excess of $40,000/month -- and while it's an FDA-approved blood product (a so-called biological), IVIG for SCLS is not yet an FDA-approved indication for SCLS, so you will be very lucky to have your medical insurance company cover the treatments in any one fixed location – never mind in two or more. The problem is not the absence of infusion centers around the country; the problem is getting your insurance to pay for the treatment in all kinds of out-of-network places, because they lose control of the cost.

To give you an idea, I spend most of the year in one major East Coast city and the remainder in another, and while I’ve managed to get my infusions covered every 4 weeks for the past 8½ years, it’s been such a hassle getting the pre-authorizations and bills paid in the two locations that starting this year I’m going to resort to getting them in the same city year-round, even if I have to travel back and forth for 4 hours solely for that purpose. So this is not at all like having kidney problems and needing to get, say, dialysis wherever you go, which is something achievable nowadays.

Here are my comments on your 4 options:

Option #1: The key thing is to get this doctor to write a “To Whom It May Concern” letter explaining what led him/her to come up with a tentative diagnosis of SCLS for your wife; mentioning his/her consultations with Dr. Kirk Druey in NIH; and stating his recommendation that your wife be put on as preventive therapy of IVIG infusions of 1 gr/kg/day for two consecutive days every 4 weeks. Keep the original, because you’ll be needing to make many copies of it.  In addition, request 2 copies of the entire hospitalization records for your wife: one for you to keep and another for you to send to Dr. Druey, see more on this below.

Option #2: There is no point in your returning to Houston for the stated purpose.  The city has wonderful, renowned hospitals, but I don't recall them having published anything on SCLS.

Option #3: You may visit the Mayo Clinic for the purpose of them reviewing her medical records, confirming her diagnosis, and issuing an authoritative opinion – namely, writing a letter on Mayo stationery recommending that your wife be put on as preventive therapy of IVIG infusions of 1 gr/kg/day for two consecutive days every 4 weeks. Keep the original, because you’ll be needing to make many copies of it. Usually it works in terms of persuading private insurance companies, but I know of at least one member of this community who went to Mayo and got such a letter, but his insurance company – Medicare – wasn’t sufficiently impressed as to authorize and cover his infusions. Hopefully, your medical insurance will cover the cost of your stay at Mayo.

Option #4: You should make an appointment to visit Dr. Druey and team at NIH as soon as possible, because he is the leading expert in the USA on this exceedingly rare disease, for the purpose of him reviewing her medical records, confirming her diagnosis, and issuing an authoritative opinion – namely, him writing a letter on NIH stationery recommending that your wife be put on as preventive therapy of IVIG infusions of 1 gr/kg/day for two consecutive days every 4 weeks. Keep the original, because you’ll be needing to make many copies of it. Usually it works in terms of persuading private insurance companies, but I know of at least two members of this community who went to NIH and got such a letter, but their insurance company – Medicare – wasn’t sufficiently impressed as to authorize and cover the infusions. The good thing is that NIH doesn’t charge you for your visit.

Good luck and keep us posted of your progress,

Arturo

Quantum Leap, I manage a bit of medical snow-birding, but not without challenges. I was first diagnosed by my former husband, an emergency physician who had seen a patient being followed by Mayo Clinic. In addition, I served on the executive team of a hospital in Maine, which may have increased my credibility when I woke up one day in our ICU. I consulted by email with Dr. Druey and journeyed to Mayo Clinic in Jacksonville, Florida for a confirmation of the diagnosis, finally gaining access to IViGG at Shands Teaching Hospital in Gainesville, Florida.

Being woefully homesick for my home state of Maine, I have been able to arrange infusions at my former hospital with emergency backup in Augusta.

The challenges:

1. I only go to Maine in the summer because medical transport in snowy climes can be treacherous and sometimes impossible. To wit, after a not uncommon 13 inch snowfall, an ambulance was unable to reach my home righ in town.

2. The brand of IViGG may vary. I limit my stay to 6-7 weeks as I twice had moderate breakthrough edema when I stayed longer. Both episodes required >24 monitoring and urgent infusions. There is no way to confirm any causation brand or other factors, but I'm sufficiently nervous that I limit my stay.

3. Providers change, even if you are rotating among only a few locations. Most walk in saying something like, "What works best for you? We've never seen this before and we need your input." However, it's very alarming when a physician or PA state, "I know everything and I'm ignoring anything you say." You're less likely to encounter this if you can tell them to please consult your prior medical records at that hospital.

4. SCLS is clinically counterintuitive. Excellent ED providers and hematologists will forget from visit to visit that your dehydration and edema mustn't be treated with high speed fluids or Lasix. It's essential to have your emergency protocol loaded into the ED electronic medical record, or at least, stapled to the wall of the physicians' and nurses' seating areas. Obviously, the more places in which you receive care, the hardest this step is to achieve.

Hoping this helps,

Susan

Arturo and Susan-

I very much appreciate your replies and opinions!  And Arturo, a special thanks to you for all that you have done to bring this community, and many SCLS resources, together!!  

It does seem as though the monitary aspect of the IVIG treatments is going to be the biggest hurdle to deal with, as we move forward in facing this health concern, but it is our hope that we can find a balance between what insurance might require and continuing our nomadic lifestyle....time obviously will tell how that works out.  

It sounds like you both (and probably most here) are having very structured treatments at (at most) 2 infusion centers, but I am still hopeful there are other options...I will have to re-dig throgh my internet search results, to try to find the case that mentioned a patient being given the option to do home injections of Ig - my gut tells me that might have been a European patient, and if so, I suspect that Ig treatments in Europe would be a REALLY hard sell for my current U.S. based insurer!  

I will continue to report on our progress, and I can say at this point, that we have already secured an appointment with a Mayo doctor that has authored a number of papers on SCLS, and we are still working on getting an appointment set up with Dr. Druey as well - hopefully we can accomplish both of those in the next month (once we are out of physical rehab)!

Thanks again for everyone's support, and I hope to hear from any others in the community that have had experiences (successes or failures) with getting the IVIG treatments in multiple locations!  

Take care,

-Will