All: During my recent visit to Mayo Florida I was told I would be referred to Dr. Greipp for a consultation at Mayo north. I have not heard back and discovered that Dr. Greipp just retired. I then read on this forum that Dr. Haymon had worked with him for some 7 years and was taking over his patients. I called to get and appointment today and was told that Dr. Haymon was no longer seeing new patients nor was she seeing SCLS patients. It was explained that she was moving to a new position at the hospital. Her nurse "Fern" said that no one else there is very familiar with SCLS. She said that there was a Fellow that had an interest in SCLS but had only seen a couple of patients. She said his first opening was Nov. 10th but that she could not give me an appointment with him due to some rule (which she did not go into detail about). She said I could make an appointment for a general hematology appointment if I would like. I told her the only reason I would travel there from Texas would be to rule out (or in) SCLS. I have already been through hematology and other departments at Mayo Florida. United Health Care is contacting NIH on my behalf. I am perplexed on what to do next. Do I have to travel to Paris for an expert, or even someone who is familiar with this disease??? Help!

Dr. Druey at the NIH is very knowledgeable about SCLS. I'm sure Arturo, of this forum, will know of some resources as well.

I was dealing with SCLS for 2 1/2 years before it was diagnosed. I found out that I was pretty much on my own as far as getting any treatment. I was able to supply my hemotologist with info on the IVIG therapy and the VA agreed to it. I have been getting monthly infusions for 6 months now and it has made a big difference in my energy level and I have not had any symptoms of SCLS. This is a great site to get info and support. Good Luck

My insurance will not pay for IViG without a definitive diagnosis. Arturo - Do you have any ideas on where I can receive a consult from a physician who has experience with SCLS? I have only had one life threatening episode and seem to be a chronic leaker. I have reached the point of spending most days laying flat due to the edema that takes place when standing or walking. I need to be aggressive with finding an answer. Thanks, Bev

Bev - I know you must feel like you've entered the twilight zone. Other than this website - some of us really are dealing with this "condition" alone. That's kind of discouraging that you got that response from Mayo. However, when I first went to Rochester - I had no idea what they would find. I had only briefly seen an article on SCLS during my own research of the medical literature. (I didn't even read it - I didn't even want to consider the possibility that my symptoms weren't simply secondary to a more "mainstream" diagnosis.) So when I first went up to Mayo in Minn. (I did NOT want to go) - I saw Internal Med first (Dr. Mikhail) and went from there - ruled out a lot. It was very valuable. And also got a good work up of the "collateral damage" to other systems caused by the years of relentless inflammation and fluid expansion. The Pulmonary doctor, (Dr. Patel) was the first to suggest SCLS. Then the Allergy / Immunology doctor (Dr. Butterfield) ruled out more things and then he agreed with Patel about possible SCLS. I went home for a month first and then flew back for my apt. with Dr. Greipp. I was in Rochester for 8-10 days the first time and maybe 3-4 the next. (I loved the town - so different from where I live in Florida so it was a treat for me -the campus was so beautiful and the buildings were fantastic with a great glass art exhibit while I was there - oh! Sorry - got off track) Anyway - I saw the photos you forwarded. You really do have the same sort of fluid retention, swelling and so forth. I don't think you can go wrong as long as you get either to NIH or Mayo, Rochester. Take your photos with you absolutely. Oh yea - when I saw Butterfield (Allergy and Immun.) before I saw Greipp - he went ahead and wrote me prescriptions for Theophylline and Terbutaline so I had a month of "trial" on that before I saw Greipp. I think the doctors there will be open to what you're suggesting and if further tests fail to uncover a different cause - would help you get on the right track (IVIg perhaps). I hate the idea that Dr. Greipp has retired but I think you will still get what you need in Rochester. (I've been told that the atmosphere in Rochester is very different from Jacksonville.) You're still on the right track, I'm sure! Carole Thorn

I would contact Dr. Druey at the NIH. Arturo will have other contacts as well.

Dear Beverly: Welcome to our SCLS community! Thank you for bringing to our attention the fact that Dr. Greipp's recent retirement from the Mayo Clinic has created a vacuum there in terms of having someone take over from him as the "anchor doctor" for patients with, or suspected of having, SCLS. I had very much hoped that this problem would not arise. I have been in touch with Dr. Greipp and one of his Mayo colleagues today, Dr. Prashant Kapoor, and I'm told that they are going to sort this problem out very shortly now that they realize it does exist. In the meantime, Dr. Kapoor suggests that you have your Mayo Florida doctor send him an email requesting a consultation. Your doctor may email him at _Kapoor.Prashant@mayo.edu_ If you provide access to your medical records -- including the records from your severe episodes in 2005 and 2007 -- he said it may not be necessary for you to come to the Mayo Clinic in Rochester MN, because they may be able to confirm the diagnosis with the clinical and laboratory history that they get from their Florida facility. As one of our community members (Dr. Claude Pfefferlé) has already advised you, if you had cases of shock (severe drop in blood pressure) with hemoconcentration (severe elevation of hemoglobin) and hypoalbuminemia (severe decrease in protein) as part of an MGUS (monoclonal gammopathy of undetermined significance), the diagnosis of SCLS can usually be confirmed.

Bev, I actually had written a response to you the other day on this forum that I accidentally deleted. Dr Hayman called me the other day and left a voice mail message that she was out of the office until Oct 19 .studying , taking a test , vacation, etc.She is transitioning into a hospital position .She is no longer accepting new patients but I was told by her nurse, Jessica , who is wonderful, that she would see me again if needed since SCLS is such a rare disease .When I was in Minnesota a couple of weeks ago, I was originally assigned to a Hematologist ( whose name I will not mention) on that Monday who didn't know anything about SCLS.So I had to return to Hematology on Wednesday to see Dr Hayman who I was told was an expert in SCLS..Dr Hayman ,who incidentally, is a very warm, compassionate person, advised us that she had only seen ONE other SCLS patient several years ago..She told us about that patient from Washington.That patient is Linda's brother. I did speak to Dr Hayman's nurse , Jessica about your case.She said Dr Hayman will not see new patients.She said you would be assigned to.someone else and that you should have your Dr at the Mayo in Florida call as you would have a lot better chance of getting in.I am also mailing Dr Hayman regarding my concerns. The medicine Dr Hayman prescribed me for SCLS the day I left Mayo, I had an allergic reaction to , but when I called to report what was going on I didn't get a call back until over a week later.The message from Dr Hayman was if you think you are allergic, then you should probably stip taking it. uh do u think??? Dr Hayman also told us several other things she would do, calls she was going to.make to the NIH , etc and again, no follow through. Jessica told me to email all of my concerns to the email address Dr Hayman provided me..I'm extremely disappointed .I took 10 days off from work, (we drove to Minnesota from Michigan) pulled my 3 young kids out of school because this was the only time we could be seen, and I Feel it was all in vain.We my Doctor and I had to wait until Dr Greip's next and last scheduled day to come in (we were told to clear out his desk ) which was August 29th , to sign the order of admission. Sorry to vent but I'm miserable.I'm suffering daily from generalized edema.Bev you have seen some of the photos of my legs and feet and bottom of my feet and ankles and eyes .My face and neck are so swollen now its difficult to swallow.I'll send you before and after photos of my abdomen. Dr Hayman and other doctors saud the reason I stop breathing in my sleep and wake up.gasping for air is the fluid is backing up into my lungs.My cardiologist at Mayo as,well as Dr Hayman said something is "seriously wrong".They saw the photos.They both said this is NOT idiopathic edema as originally diagnosed last year by my Doctor. Yet, I can't seem to get the help I need.Bev if u email me your phone number I will call you. Sorry for typos I'm typing on my Android phone.

Bev, Sounds like Arturo has got the wheels turning(thank you Arturo!)at Mayo and hopefully they can diagnose you without a trip there. I agree about the thoroughness of mayo but really sorry Dr Haymons is not seeing scls patients especially since Dr.Greipp is retired. Several people from this website have been diagnosed with scls at the rochester mayo and the process requires you to several specialists so I am sure they are somewhat familiar with it and not everyone is retired. A lot of it is ruling out other things so it is several days of testing which you may have done already in Florida. I think a letter of referral from the Florida doctor will really help. Also I agree that Dr. Druey at NIH would be able to diagnose whether you have scls or not. Not sure if he Is willing to do that but it is worth a call. If you do have scls a trip there would allow you to participate in a scls study he is doing. One of the hardest things for most scls patients is getting a diagnosis. For some it took months or even years. Hang in there and be persistant. You have to be your own advocate. If you do have scls you will need these skills in obtaining the correct emergency room treatment and if you need ivig that will be a battle as well. Good luck and hang in there! Linda

I'm still in the process of getting a diagnosis. I have seen a doctor at Penn in Philadelphia. He thinks it may not be SCLS, as some of my tests don't support that diagnosis. He ordered labs yesterday, and is looking into angioedema. In looking at the information, I'm not sure that's what I have. However I'm open to all suggestions and tests. You may want to go to www.haea.org, which I found on this site. It mentions specifically the belly and neck swelling you've mentioned. Good luck to all of you. It is very frustrating to have a rare disorder. This group has helped me so much with information and support. Eileen

Arturo: Thank you very much for being so proactive on my behalf. I have contacted Dr. Starr's office and am waiting on a call back. I will give him Dr. Prashant's email as a contact. I believe a direct consult is needed in my case. I am atypical in that my episodes have become chronic. From many posts here I see that chronic cases typically don't show hemoconcentration, and is sated in one of the literature reports. Albumin was not tested in my 2005 episode. Hemoconcentration was not a factor as my hemoglobin eventually dipped to 5.5 requiring 2 blood transfusions and subsequent IV magnesium. However, I had no bleeding from the surgery site nor was there unusual blood loss in this routine surgery. The days following this event I gained 50 to 60 pounds of edema requiring diurectics. No one had a clue as to why I suddenly became critical. This does not meet the definition of SCLS. This appeared to be a full on leak of plasma and cells that happened spontaneously. All clotting and blood disorders were ruled out. I find it curious that one of the SCLS members on this site reported a prolonged nosebleed prior to her first SCLS attack. The second episode was following surgery in 2007 at which time I was told I had severe hemoconcentration (I am working to obtain those hospital records). There were no problems with bleeding during the procedure. However, in the following days I had a a small internal bleed in the surgery location. Strangely there was also a simultaneous large bleed of capillaries into the muscle in an area totally unrelated to the surgery location??? I was rehospitalized and the bleed resolved. Within a week of this I began having recurrent extreme abdominal distention with no known cause. Episodes lasted a few days and recurred about 3 times a year until March 2011 when they became monthly then somewhat chronic in June 2011 at which time my feet ankles and general edema became obvious. Everything has been ruled out. I believe at this point it is obvious that I either have Idiopathic Capillary Leak, or a version of Atypical Systemic Capillary Leak Syndrome which may have initially been triggered by the events mentioned above. As i have been reading through posts here it is obvious everyone has a slightly different twist on the disease. Dr. Starr mentioned they it is also possible that I may have something not yet described in medicine thus far. My local DO would like to try IViG as she believes if it works the success may prove diagnostic. However, as we all know, without a diagnosis insurance will not pay. As an FYI, my triggers are physical activity. Many in this group have posted the same trigger. Three days of bed rest (seems to be the magic number) will buy me a day or two of activity. The difference in size from morning until night is measurable and can be extreme. I find it interesting that some of the SCLS patients on this site can exercise while others have been hospitalized after taking a simple walk. It is frustrating when we know movement improves the lymphatic flow, but capillary leak is triggered by movement. That is for sure the case for me. It took a very long time for me to isolate it as movement is tied to so many things we do each day. Sorry for such a long post. Thanks, Bev

Susan - My number is 817-808-6565. Bev

Bev - I'm glad to see you're making some progress! I wondered about the "exercise trigger" as well. I absolutely swell very quickly with exertion of any kind. Taking a half mile leisurely walk, half way through, my abdomen and lower legs get heavy and I feel as though I've just drunk a quart of water. It feels like my guts are just sloshing back and forth with every step. My legs stay bruised and sometimes red and very tight. UNLESS I've been completely still for a few days. (not possible) Another huge trigger for me is heat. I keep my house really cold - not easy in the hot, humid south east. Stress causes an immediate reaction also. Anything that seems to get the adrenaline flowing. Which makes me think that really - anything that increases my pulse rate will aggravate the problem. After IVIg treatments - for a couple of weeks - It seems like I'm less "reactive". Oh yea - as you mentioned - you first noted you would have the swelling etc. a few times a year and then it became cyclical and now chronic. This is exactly how it progressed with me as well. And every acute episode (hospital) followed a virus or flu and once after dental work. Carole Thorn

Bev I am now quite certain that exercise and air travel, especially in combination, are absolutely triggers for me which is pretty disappointing since I used to travel a lot for work and I have been a very keen fitness enthusiast since my early 20's. Warren

Warren: You bring up a good point about air travel. I have read a few posts on this site about air travel. It seems that many in this group are professionals in a field requiring extensive travel, or have required travel in the past. I find it interesting as I have worked for a major pharma/medical device company over 15 years. I currently work as a scientist in R&D which does not require extensive travel. However, I traveled to over 50 countries and most US states in my previous position. Could it be that extensive air travel is an initial trigger for the onset of SCLS in susceptible individuals? My role as a scientist researching adverse events has taught me to look at AEs from every angle and never ever assume a certain cause and effect relationship is not possible. For this reason I believe there is no dumb question. I will ask this question in a separate post. Bev