I had written about a year and a half ago how I had been diagnosed with SCLS.  My Mayo doctor firmly believed I had it, he sent my records to Dr. Druey at the NIH and he concurred, EXCEPT I need to have two blood tests during my episode to be 100% sure of my diagnosis.  So just recently my blood pressure started dropping, and I was taken to the hospital by a friend and sat in the waiting room for over an hour.  By this time I was in excruciating pain and felt like I was having another major episode.  By the time I got into a hospital room my bp was coming back up past normaI and I still had the pain. I have outstanding lab orders to have my blood drawn right away and I cannot have any fluids until then. The er doctor barely looked at my paperwork, drew my blood and told me I had hypertension and I should see a doctor.  When I was released from the hospital a few hours later on my discharge paper my bp was 95/58. Later that same night I took my bp and it had dropped down to the low 80's, I didn't have the mental stamina to go back to the hospital and be treated like a 5 year old with a runny nose.  I have had several "small episodes" like the one I described and one major one that put me in the icu for 4 days.  The major one was my first and it was the one that sent me to Mayo in the first place.  I was hoping that maybe some of you could give me some advice....first of all is any one else in limbo regarding a diagnosis? Also I don't know where to turn to find a doctor who will be there if I have another major episode. The doctor I am seeing now does not do hospital work...just her office.  Needless to say i am at a loss.

Rubeanie-While you may be comfortable with your physician, it is imperative that you find a specialist (internist, hematologist, etc) who has hospital priveleges.  They can expediate your admittance and begin the proper treatments necessary for SCLS.  Futhermore, ask the doctors for their cell phone numbers. I have found that many doctors are willing to share that with individuals who have life threatening conditions.  In addition, make sure you bring with you the SCLS Protocol that Arturo posted on our website.  The emergency room doctors have never encountered someone with SCLS and they do not the proper protocol for treatment. Too many times they insticively follow their medical training and attempt to infuse too much fluids over a very brief time. As you probably know, this can be most dangerous with serious complications.  In addition, you must be your own vocal advocate.  You or your immediate family must ensure that you get admitted quickly, as this is serious life threatening condition. You must absolutely insist that they follow the protocol as this is the most effective manner to treat a SCLS attack.  Finally, the best treatment to prevent or minimize future attacks is to begin IVIG treatments.  Find a doctor who is willing to do so.

The diagnosis of SCLS is made partly by exclusion, namely, by eliminating the possibility of other more common diseases, and is based on measurable, clinical symptoms such as hypotension, hemoconcentration, hypoalbuminemia, and the presence of a protein called Monoclonal Gammopathy of Unknown Significance (MGUS).  Every time you went to the ER and they drew blood and monitored your blood pressure was an opportunity to have the diagnosis of SCLS confirmed or rejected.  The periodic drop in blood pressure you cite is not sufficient evidence.  You and your existing doctor should have the ability to get you out of limbo, by gathering your results, examining them for tale-tale evidence, and sending them to Dr. Druey at NIH for evaluation.

With regard to your question, it is highly unlikely that you will ever "find a doctor who will be there if I have another major episode."  Your best bet is to print out the Wikepedia write-up on SCLS and hand it over to the triage nurse who will screen you upon your next arrival at the ER.  Have the ER measure you for hypotension, hemoconcentration, hypoalbuminemia, and the presence of a Monoclonal Gammopathy of Unknown Significance (MGUS).  If you test positive, then they should continue to follow the instructions provided there, in order to treat you appropriately.  If you do not, then return to Mayo for a thorough evaluation.

I have had the frustrating experience of ED staff disregarding my documentation. My latest strategy has been to work with my hematologist at University of Florida Shands Teaching Hosptital to place a suggested protocol in the EMR. The key is to place the information in the system in a field/function that pops up in the early stages of ED activity. Often, the ED is on a separate module or system than the rest of the hospital. This takes a willing and knowledgeable provider, but can be life-saving.

I also have had the experience of languishing in the ED until my labs no longer show the flags of SCLS. Excellent hospitals have very good EDs that just don't recognize SCLS due to its rarity. It may be helpful to carry multiple copies of the single page of the medical record that confirms your diagnosis, preferably a physician's dictated note. A challenge for me is to remember to follow my own advice when I have leaking and resulting cerebral edema. A preloaded satchel of info can help.

Susan

In the beginning, we had similar challenges getting ED staff to recognize this as a life-threatening condition that needed quick treatment and attention. 

To counter this, my husband's internal medicine specialist wrote a detailed letter outlining the condition as life-threatening, how it presents, advice on how to treat, that my husband has good knowledge of his condition and how to contact him for advice (he is often on call). It's not as vital now, as my husband has been to that same hospital so many times that his medical history can now be seen by the ED staff rather quickly. However, in those earlier days, that letter was vital to get taken seriously.

Also, the hospital my husband goes to is associated with a local University and therefore has a Medical Training Unit (MTU) of Internal Medicine. The MTU residents are often eager to treat something rare, so the letter also asks ED staff to consult MTU quickly when my husband comes in. That entire teaching unit now knows my husband very well. They come down to the ED and admit my husband as their patient every time. Not only does my husband get some level of consistent care from a department that has experienced his episodes multiple times, but the new residents also get exposure to a very rare medical condition.

ED almost killed me as the Doctor would not listen or read the information we have to provide them.

Thank you all for your input.  It sounds like so many of you have the same experience with the ED as I do.   I had thought my doctor had put a red flag across my chart, as the ED and my doc are all on the same network.  Evidently they paid no attention to it.  After my last visit with my doctor, and a discussion with my husband, I have decided to find a doctor who is willing and able to learn about SCLS.