Good afternoon, I was looking for a unique way to introduce myself to this fine community as I have also been recently diagnosed with SCLS.  Hopefully this is light at the end of the tunnel after five years of mystery attacks and hospitalizations. I've been told in the past I've possibly had septic shock, heart failure, kidney failure, severe dehydration, polycythemia vera, pneumonia, and many other things I cannot remember at the moment. About six months ago someone in the ER mentioned SCLS and when I read up on it, the symptoms, blood tests, etc. matched up closer than anything before. None of the dr's in my area (a major metropolitan city in the US) would agree to an evaluation, and I eventually ended up at the Mayo clinic. They ruled a few other things out, and here I am, now waiting for my first IVig treatment.

Being a rare disease, I have begun to start to question my diagnosis of Meniere's disease from 30 years ago. To date, I've had two otolaryngologists say "Yes it's Meniere's" and two say "It's definitely NOT Meniere's".  I have issues in both ears, one started when I was 18, the other three years later. The doctors say it's extremely rare to have it in both ears. So did I hit the jackpot of rare diseases or what? 

Some of the recent blood tests I've had indicate problems in my immune system, and one otolaryngologist mentioned it could be an autoimmune disorder causing the Meniere-like symptoms. 

So I guess once I get things stabilized with the SCLS I can re-evaluate on the Meniere's 

Has anyone here had anything like Meniere’s ?  i.e. sudden hearing loss, sudden vertigo, "drop attacks" where you fall due to loss of balance and can't stand up?

Kevin,

Welcome to our community! So sorry to hear about your saga! I have two ideas for you.

First, have your main physician request a consultation on your case with Dr. Kirk Druey at the National Institutes of Health in Bethesda, MD, right outside Washington DC, see phone and email at https://irp.nih.gov/pi/kirk-druey  He’s the leading authority on SCLS in the USA, and if your medical history suggests a possible diagnosis of SCLS, he will arrange for you to visit there for a couple of days to be tested and evaluated not just by him, but also by experts in other diseases that might fit your bill. It’s basically like getting a second opinion from another Mayo-type Clinic – except that this one is free of charge!

Second, I can see where there could be some confusion between SCLS and Meniere’s, because at the onset of a typical episode of SCLS, which features a massive leak of plasma from the circulatory system, we too tend to feel dizzy and often pass out – but in our case it’s because our brains are not receiving their usual delivery of oxygen and nutrients, not because there’s something wrong in our inner ears.

Anyway, take advantage of our many pages of tips, resources, and discussion topics to get acquainted with SCLS, just in case you do belong here.

Arturo

Arturo,

Thanks for the information and for the welcome. Based on the messages I've been able to read so far, you seem very knowledgeable on the subject. 

I just wanted to clarify, I wasn't really confusing the SCLS and Meniere's, just curious if anyone else in this group might have had similar experiences. I've had the Meniere's symptoms since my late teens, and the SCLS stuff just started in my late 40's.

And just curious, is it usually only an official SCLS diagnosis if you visit Dr. Druey at the NIH?  I've currently been evaluated by two Hematologists, and two Immunologists, two locally, and two at the Mayo Clinic in MN who are all in agreement with a SCLS diagnosis.  I already have the recommendation to the NIH study and have received the paperwork. My first IVig treatment is scheduled in one week pending insurance coverage.

When you mention the consultation with Dr. Druey, is it a 1 on 1 clinical evaluation similar to a physician?  Or are you referring to the SCLS study at the NIH and four day evaluation?

Thanks!

Kevin,

Hi! Welcome to the community. No, I don't think Dr. Druey must diagnose you. I was diagnosed in the ICU by an infectious disease doctor at a Mayo Clinic affilated hospital. Dr. Druey later confirmed the diagnosis when I met with him. (I live near NIH so that was easy for me to arrange.) He is an incredible resource and very responsive. He pretty much tells the doctor who prescribes my IVIG how to treat me and he always answers my emails within minutes. I had an issue getting IVIG after I was exposed to covid back in January and he helped me sort that out with the infusion center. I am curious if you're doing the four day evaluation. I'd gotten the impression that that aspect of the study was over. I only met with Dr. Druey for about 2-3 hours on that one day. They did take 18 vials of blood, though!

Jen

Jen,

Thanks for that information! Good to hear.

The consent form I received described the study as "Studies in the Pathogenesis of Systemic Capillary Leak Syndrome" and has a "version" dated December 3, 2020.

I'm seriously considering participation, and I'm fairly sure my doctors will support the referral letter.

I'm not too keen on the bone marrow biopsy procedure described in the consent form, is that something you were required to do?  How bad was it?

Kevin, Hi! Sorry, I didn't do a good job explaining before. I didn't participate in the four-day study. I spent about an hour or two with a resident or intern who took a detailed history and about an hour with Dr. Druey who answered my questions. They took blood and that was it. I wonder if the study was put on hold due to covid (I was there in April 2021) or if they've started it up again for some reason. Keep us posted! Jen

Kevin, so no, you don't get "an official SCLS diagnosis if you visit Dr. Druey at the NIH," you get a confirmation or alternative diagnosis, but you achieve 3 important things.

First, you get peace of mind that you have been properly diagnosed, which you and your loved ones should value highly after having gone through so many years without a proper diagnosis. 

Second, it's good for Dr. Druey to get to know you in person because, as many of us have discovered, his telephone and email advice, plus any formal letters on NIH stationery (e.g., to insurance companies and Medicare and its equivalents around the world to cover treatments) are considered authoritative -- even better than letters from the Mayo Clinic, as several of us can attest. 

(In my case, I filed two lawsuits against Medicare and was involved in litigation for over six years to get them to cover IVIG treatments for SCLS retirees, and trust me, the judges cared mostly about what NIH [namely, Druey] had to say on the issues to guide their rulings.)

And third, going to NIH and becoming a part of Dr. Druey's cohort of SCLS patients enables him to advance his evolving research agenda into this exceedingly rare disease, including by the blood, tissue, and other in-kind donations that we make when we go there.  Having said the latter, I too would want him to persuade me face-to-face before I would subject myself to a bone-marrow biopsy.  (In fact, I didn't know that he was asking for permission to do them.)

As to the timing and nature of a consultation with Dr. Druey, you get to meet with him 1-on-1 plus 1-to-1 with other relevant experts, much as you might have done at the Mayo Clinic, and you can negotiate with him ahead of time how long you will stay and what body samples you're willing to donate. 

I hope this guidance helps.

Jen,   I for sure will update with more information.

Arturo, I just came across your court case while doing a Google search on "ivig insurance denial for SCLS", small world ;)   My first infusion(scheduled for today) was canceled because of insurance. The doctor claims he gives IVig for various things and it usually requires two appeals and a peer-to-peer call before they approve. Hopefully it will apply for SCLS treatments as well.

Your reasoning about the NIH visit was just the push I needed to put things in motion for the visit. Thanks!