Dear SCLS group: I am currently visiting the Mayo Clinic in Florida to find a diagnosis for what has baffled my doctors since 2005. During my first visit last week it was mentioned that SCLS is a possible diagnosis. I was doubtful about this diagnosis because Dr. Starr told me when it occurs it is dramatic and that I would present with massive swelling all over and would be in ICU. For this reason he felt that I could not possible have SCLS. He said it always presents as a recurrent life threatening event. I did some research over the weekend and found this site. The are so many similarities in what I have experienced and your stories that now I am reconsidering that this (or a variation of this) may be the correct diagnosis. I have to ask, is it possible that many on this site do not really have SCLS, or is my Florida Mayo doctor misinformed? For some background on me, I had a very scary episode in 2005 following routine surgery. My BP suddenly dropped so low it couldn't be measured. I did not have bleeding problems but quickly went downhill. I could not take pain meds for 4 days due to low BP and low 02 saturation. Then extreme swelling began and I grew to an enormous size. I could not even bend my fingers. At a later time the surgeon told me I had a very serious medical problem and I needed to find out what it was. He said that it could be life threatening if I ever needed surgery again. He said I was the only patent he had eveer had that actually scared him. A subsequent surgery in 2007 resulted in bleeding into the muscle and rehospitalization. Snce that time I have had periodic severe abdominal swelling 2 or 3 times a year. In March of this year the swelling became more severe and frequent. By June 17th I was on short term disability and the swelling would not resolve. I was swelling in my right hip and thigh, then eventually both hips. The abdominal swelling is less in the morning but becomes very extreme with movement such as walking. I have to lay flat for several days to get relief. CT scans have not shown distention in bowels, or organs, nor has it shown free fluid or air. I have generalized swelling in face and sometimes my shoes don't fit. However, the extreme swelling is in my abdomen. I have documented the extreme changes in photos. The photos are so extreme that it almost looks unbelievable. I am told that if a CT with contrast does not show free fluid then there is no fluid. Will fluid leaks into tissue or muscle show up clearly on a CT? I have read that others in this group have described similar abdominal swelling without the need to be rushed to the ER. Are these individuals improperly diagnosed with SCLS or should I talk with my Mayo doctor more seriously about this as a possible diagnosis for me? Sorry for such I long post. BPerry7

Hello dear BPerry7, Try to find the laboratory of the episode of 2005. If you had a shock (severe drop in blood pressure), an hemoconcentration (severe elevation of hemoglobin), an hypoalbuminemia (severe decrease in protein) as part of an MGUS (monoclonal gammapathy of undetermined significance), the diagnosis of SCLS is made. Extravasated fluid during an acute capillary leak is sometimes difficult to identify by CT. The liquid spreads between cells of the tissues that appear enlarged. The organs are no longer functional and can lead to disorders such as air and water accumulated in the intestine or water around the lungs or heart. Claude Pfefferlé

Dear Claude: Thank you for your quick response. I have further evaluation today to rule out infectious disease. All other causes have been ruled out. If SCLS is ruled out I may be left with a continuing mystery. I do have a documented case of shock from low blood pressure. I do not have a documented case of hemoconcentration. I will look into obtaining this from past hospital records. I do recall needed IV fluids containing magnesium, potassium, or something else. I just can't remember. I was very weak and foggy at the time. When I think about it there have been hints of hemoconcentration such as several comments from the surgeon and nurses (2007 gallbladder surgery) that is was extremely dehydrated. When I asked how they could tell I was told my blood was very concentrated. I will look into obtaining that record. There have also been changes in the ease of obtaining blood or placing IVs. Now it seems to be a big problem. There have been casual comments from nurses "you must be dehydrated". I have a few findings that do not fit the clinical picture for SCLS and many findings that do. From reading the stories here it appears everyone has a different twist (as in those who take medication for hypertension for example). The two main findings that do not fit the clinical pictu for SCLS are anemia and low grade fevers. Anemia has been a lifelong battle but low grade fevers (between 99 and 100 typically) started 3 months ago. It may simply be that my body has a different reaction or it is something else altogether. The symptoms that do fit the clinical picture are the following: Swelling - general, eye puffiness, round face, extreme abdominal distention (can increase 8 inches) that increases with movement (only relieved by laying flat for days but returns with activity), thigh and hip swelling (more right side than left likely due to right lymph node that was removed in groin in 2007). Bouts of intense thirst and decreased urine output Occasional poor kidney function Racing heart 2005 critical episode of low BP and shock (preceded by flu symptoms without fever Problems with 02 saturation Variant BP Sudden crushing fatigue Bouts of faintness and nausea (with onset of sudden fatigue) Flu symptoms without low grade fever I realize this does not make a definitive diagnosis but I believe it is enough to be suspicious. I am interested in hearing from anyone who has experienced extreme abdominal swelling but only mild extremity swelling. I have read that some on this site have chronic problems with this but have normal blood levels. Kindest regards, Beverly Perry

OMG! Seriously - you could be me! I was evaluated at Mayo in Minn. by Dr. Greipp. He diagnosed me as atypical SCLS. This allowed me to get the IVIg treatments monthly. It was a gradual "recovery" and I am soooo grateful. I would implore you to see him. Misdiagnosis shmisdiagnosis - you sound like you certainly have an immune system out of control. What you said about the abdominal swelling - that is meeee. I am scheduled for this months IVIg today so I have to go but I want to compare notes with you - I will try to get back. I'm very interested in your situation. Carole Thorn

Carol, Thank you so much for responding. You can reach me directly at beverlyperry3@gmail.com . I am very interested in your story as well. I have taken a lot of pictures of the swelling in my feet, hips, thighs and abdomen. I also have pictures shoring the change in my abdominal distention from morning until night. If you don't mind I would like for you to take a look at them to see if they look similar to yours. I meet with Dr. Starr and discussed the possibility of an atypical version of SCLS. He agreed that this is a possibility. He said he would contact Dr. Greipp and see if he would agree to see me. I have not heard back from Dr. Starr yet. I am leaving Florida to go back to Texas today. I will be working on obtaining medical records from past hospital stays to see if there are any clues. I also need to compile all of my symptoms and signs in an undstandable format. There were several things I didn't mention to Dr. Starr because they didn't seem related or important. However, when I read the posts on this site I realized that I could have been omitting important clues. I will compile all of this information before I see Dr. Greipp. Hopefully he will agree to see me and I can get in with Him soon. Bev

Sorry for the typos! I am typing quickly on my iPad!

Dear Bev: I read your posts and we share similar symptoms.I just returned this week from a week long stay at Mayo in Minnesota.This was my second stay there.The first visit was Aug/Sept 2010 and I was run through just about every department , ruling out heart, kidney, allergy, bloodpressure, celiac disease, lupus, rhematology, endocronology, ent, gastrology, etc etc finally ending with a consult with Dr GREIP.Dr GREIP recently retired but he assigned me to Dr Suzannne Hayman, also in Hematology.Dr Hayman prescribed Singulair for me ,however, I could but tolerate the side effects , especially the generalized EDEMA.I unfortunately developed angioedema to the medication Dr GREIP recomended and am allergic to terbutaline. My throat closed up when I tried lasix.I just started edecrine, a loop diurectic to take with my 100 mg of spirnolactone , a potassium sparing diurectic, however, I just broke out in hives.I have a call into Dr Hayman. Exercise, stress, overexertion , air travel and high altitude cause me to swell tremendously.My last attack was the end of January.These episodes are different then the everyday chronic EDEMA from which I suffer because it results in a sudden drop in blood pressure , nausea, chills, weakness, vertigo, fatigue, stomach pain sometimes, fatigue, feeling that your legs are paralyzed, excessive thirst, night sweats, shivering, and my entire body filling up.with massive amounts of fluid and looking like the michelin man.( I'm only 5 ft 1" small frame). Sorry about any typos, as im writing you from my smartphone as I lay in bed ,swelled up .The bottoms of my feet are so swollen it hurts to walk on them ,I currently look like I'm 9 months pregnant, and I am short of breath. I will email you . Susan

Oh no! Really sorry to hear that Dr. Greipp has retired. Not sure if Dr. Starr knows that. I am happy for Dr. Greipp but that is a loss for me. Bev

Hi SLCS Group ! Keep hope ! I think I will be the longest SLCS survival (22 years) after having had all by you the described symptoms and a over 15 years long quest for a diagnosis which was confirmed in the begin of 1989 after several emergency hospitalizations in collapses and very bad vital prognosis where luckily one emergency MD recognized the signs of this disease (first named "Clarkson's disease") after having remove this very similar of angio-oedema. At this time just a few cases were related (all deceased so far I am informed) and we experimented a lot of medicine unsuccesessfully and without benefit for me. In June 1989, an renowned acupunrist MD and surgeon) initiate a long term treatment of me and thank his help I am always alive at time and without any new crises and all the bad effects are alleviate and I am coming back to a nearly normal way of life, without other medication or drugs. But the disease is always present and require to be careful to prevent any relapse. It was a long way and a very close collaboration between this acupunturist and me. And at time 5 or 6 other persons with SLCS in Brasil and USA are alive and in good conditions thanks this therapy. Back to "Angio-oedema" here in France this disease is diagnosticated if "deficit in inhibitive C1" is reduce and is mostly hereditaryare present and treated by "Danatrol" until C1 coming back to the normal figures. (cf. L'AVANCEE MEDICALE - 25 Juin 1990 - n° 296). For more details have a look on my profile or e-mail : MAG.H@wanadoo.fr. Best wishes to all, Maggy

Bev, Try to get to the Mayo clinic in Rochester. That is where my brother was finally diagnosed 9 mos after his first episode. He lives in Washington dc area and was at a very good hospital when it occurred and teams of specialists looked at him and came to no conclusions. He brought a 4 inch notebook of records with him to Mayo and we met with Dr Suzanne Haymons the first day. The doctors in DC said he did not have the mgus protein and that is why they doubted scls. She spent about 5 mins looking thru the records and then easily showed us on the lab report where the mgus protiein was. She found it because she has a lot of experience with scls and knew what to look for. She had worked with Dr Greipp for several yrs at that time which was 7 yrs ago. Dr greipp was semi-retired then, I think, but still came to meet my brother at the end of his visit there and confirm her diagnosis. After she pointed out the protein that first day he went thru a couple days of testing to definatively diagnose scls and we met with her and Dr. Greipp that last day. She is obviously very intelligent,a good communicator,compassionate,and she knows about scls. The Rochester Mayo does a great job as well and because of Dr Greipp they are more familiar with SCLS than any other Mayo. If you think there is a possibility you have it you will get your answer there. Good luck with everything. Linda

Hey people! Again I have to get down to the hosp. this a.m for day 2 of IVIg but I had to take a moment to read the updates... Susanfv - I had to laugh at your description of feeling like The Michelin Man - I have said that about myself so many times. The swelling in the bottom of your feet makes for very painful walking and when you do walk on them (hard not to) it's not too good for your feet. Mine had swollen up so large that I actually fractured a small bone just by walking around my house. And at times my abdomen was so full of fluid that when I did stay put in a chair for a day, I swear the fluid would shift to my ass and upper thighs. Bev - I would like to see your photos and hear more about what is being done for you now. I just read Linda's post above saying that Dr. Greipp was able to consult with her brother and Dr. Haymons - even though Dr. Greipp is retired. I think that what is important is that all the other causes for your swelling are ruled out and that you start the Theophylline regimen and if that fails, you get the documentation you need in order to get insurance coverage for IVIg. Because IVIg is the best treatment for so many otherwise uncontrollable autoimmune conditions - the list is endless, and even though not every one of them calls for the "high dose" treatment - from what you describe - I mean these symptoms are so strange in a way - that when I hear someone else describe them - it's like they are so specific - you couldn't make this stuff up and doctors look at you like you're f---ing nuts! I'm sorry - just venting - I know how frustrated you and everyone else is - who is going and going and just trying to get better - just so they can function.. I will get back to this - I am gonna be late for my apt! Carole Thorn

Linda - Thanks for the info. I will try to get in to see Dr. Haymon. I would like for Dr. Starr to make the referral. I am typically very proactive and take matters into my own hands. However, I am finding that physicians take your case more seriously if you go through a physician referral. Bev

Carol - I sent you and email with pictures of my swelling. Bev

Bev- did you send them to jthorn0606@bellsouth.net? This is the only email I have - try to resend. Thanks Carole

Carol - my bad....I sent to Susan originally. I just forwarded to you. Bev

Bev / Carol / Linda I wrote a long reply to your posts but somehow I must have accidentally deleted it.I'm emailing from my phone. Bev ,I emailed you photos. I will email you Dr. Hayman's email address , phone number and name of her nurse. I'm hoping to speak with Dr. Hayman tomorrow. Take care, Susan

Susan: Thanks for the photos. This gives me a good idea of what you are dealing with. I often look very much like you. The swelling around my eyes is not as severe as the picture you emailed and my abdomen swells much bigger. The pitting edema on my legs and feet has started to look much like yours. My neck also swells. It hurts to walk on my feet. I now get pins and needles feeling in my feet and hands. Is the IViG helping you? I am so miserable. I can't do much. I spend most of my time in bed laying flat. I can't even put my knees up in bed without making the swelling in my feet worse. Bev

Susan: Thanks for the photos. This gives me a good idea of what you are dealing with. I often look very much like you. The swelling around my eyes is not as severe as the picture you emailed and my abdomen swells much bigger. The pitting edema on my legs and feet has started to look much like yours. My neck also swells. It hurts to walk on my feet. I now get pins and needles feeling in my feet and hands. Is the IViG helping you? I am so miserable. I can't do much. I spend most of my time in bed laying flat. I can't even put my knees up in bed without making the swelling in my feet worse. Bev

Bev, I have very similar symptoms to you also. I have a lot of photos of my abdominal swelling. This was the only way for me to get some of my earlier Drs. to take me seriously. I also get a lot of swelling in my arms and thighs. Have pictures of this also. I do get swelling in my face, head, back, hands and feet, but not nearly as bad as the others mentioned. I was also skeptical when I first heard about this disease. I found this sight before my diagnosis, when it was first started and didn't think my episodes were quite as serious as the few people that were communicating on this sight. Once I got the diagnosis I came back on and was amazed at how many more people had joined and how similar many of their stories were. I don't have the typical acute form of this disease. I have a chronic form. My episodes don't send me to the the hospital every time, only 3 times, but I would have episodes every few days that would interfere with my everyday life and make it impossible for me to work for 2 to 3 days most episodes. It has progressively gotten worse over the years and I haven't had a long break in 3 years. Just about all of the symptoms everyone talks about I have or have had at one point or another. My current Dr. is wonderful and whenever I ask him about one of my symptoms he gives me a very detailed explanation of why I feel the way I do. It doesn't make it go away but it makes it a little easier to deal with. I went for so long not knowing what was going on with me (7 1/2 years). I have found a lot of articles about the chronic form of this disease and a lot of similar case studies in articles about the acute form of the disease. I work in a hospital so have access to a lot of articles in our on-line medical library. I started IVIG therapy in May. I had a lot of side effects at first and ended up in the hospital with aseptic meningitis. They have had to increase my dose gradually over the past few months. Last month was my first month on the the full 2g/kg. I had no episodes at all. It was so nice to feel somewhat normal. I started to get extremely tired and felt funny the day before this months infusion but felt much better after the 1st dose. I still get headaches afterwards. I have one right now. Just had my treatments on Wed. and Thurs. but they aren't that bad anymore. I take prednisone before and after my treatments to help with that and a lot of Tylenol. I hope I have another good month and will someday feel more like my old self again. Best wishes, Maria Morris

Maria- Thanks so much for posting. Is it possible to share the articles you have mentioned or tell me how I can have access to them? Would you be willing to share photos of your swelling with me so that I can give my physician an idea of what others are experiencing? I will be glad to share my photos with you as well. my email is beverlyperry3@gmail.com. If you provide your email I will send my photos. I am very interested in your story. I have an oncologist who is championing my case and is asking for as much information as possible. He has been a witness of my struggle because I underwent major open abdomen exploratory surgery in August because a malignancy was expected. My right ovary and appendix were removed due to masses that proved to be completely benign. My symptoms continued after surgery. I have now experienced pulmonary vascular edema. Yesterday I was sent to the ER by my GP for a dramatic drop in BP. I was dizzy and nauseous. In the ER they took one BP measurement when I walked in the door and sent me home with no exam. Today I am very swollen. Without a definitive diagnosis I am at risk for mis-judgement in a potential emergent situation in the future. Thanks, Bev

Maria, I also would appreciate it if you could email me those articles you reference in your post.My email is Susanfv@gmail.com. Thank you, Susan P.s Do you also suffer from vertigo? Tingling and pain in your fingertips when the swelling is severe? Did you go directly to ivig or exhaust a number of meds first like singular, terbulatine, etc?

Allo Bev. je vous ai envoyé des photos par e-mail. j'étais très enflé lors d'une grave crise. maintenant tout va très bien pour moi. bonne chance à vous. Josée

Bonjour Josée, J'ai bien reçu votre e-mail et des photos. Merci beaucoup. Je peux dire une grosse différence de la façon dont vous regardez maintenant. Je lui ai répondu avec des photos de moi-même. Je pense que vous conviendrez que la manière dont je regarde aujourd'hui, c'est assez choquant. Je suis l'espoir d'obtenir un diagnostic d'une façon ou d'une autre bientôt. Merci pour le partage, Bev

bonjour, je vous envoie le lien de l'article dont je vous ai parlé: http://www.mendeley.com/research/systemic-capillary-leak-syndrome/ c'est mon médecin (sepehr javaheri) qui a écrit cette article, la femme de 40 ans, c'est moi. bonne chance Josée

I always thought my low blood pressure was a sign of good health. I have swelling that doctors always thought was fat. I am sure there is some fat under there but if one pinches just above the wrist, it takes slow motion for it to return to normal. Doctors would always think it was weird that I do not have the normal problems of an obese person. I could be on a less than 1000 calories per day and never lose an ounce. One doctor made me bring my calories back up to 1500 calories per day and I felt better. I have help from a naturopath, Dr. Boxer in Playa Del Rey. She put me on horse chestnut. It helps. My family does compression massage to reduce the swelling when it gets our of control. I guess one would look for lymph draining. Sometimes when I swell, it feels like my skin is going to rip. I think this problem is under diagnosed and missed most of the time. I wrote a personal history section in wikipedia.

rivcal, I know what you mean about the skin swelling -it feels and looks like my skin is puffy and any wrinkles are way more pronounced. At the end of the month, as my IVIg appointment is nearing - sure enough, I will wake up and look 15 years older with this horrific distorted face. My NOSE will be fat! And of course the doc's think you're in denial about ageing and need to quit trying to find a medical explanation for fat and lazy. And I can only imagine what they think when you explain that stress and activity makes it worse! Life's little ironies! Carole T.

I sleep with my head well elevated. If I don't I have massive facial swelling in the night. My body is small in the morning and by evening I am at least 2 sizes larger and very uncomfortable with difficulty breathing. It seems the more you leak, the more you can leak as the body just adjusts to the stretch.I have always been very slim to the point of thin - and super fit- so am finding these changes very difficult emotionally plus feeling so nauseous. You are fortunate you are able to have the IVIG. I'm keeping my fingers crossed. The wheels are in motion at least.I can relate to your horrific distortions though- I am unrecognisable sometimes Sarah

Please don't neglect your mental health. I know as well as anyone how a deep depression can set in as these symptoms effect your life. There are many therapies available and I'd strongly suggest looking into them.

Dear Bev and Susan, Sorry it has taken so long for me to get back to you. My computer bit the dust and it took this long to find out they could not fix it and had to get a new one. I don't really use the internet on my phone, I'm not sure I know how. lol . I never have time to figure it out. Bev, I will send you those pictures as soon as I get done typing this and will get the articles around to send to both you and Susan. I have them at work and am off for a few days. Susan, to answer your questions, I do get tingling and pain when I swell. The tingling is all thru my arms,hands,legs and feet. I also get a lot of pain here, but the worst pain is in my abdomen. I can't even stand up straight. I do get vertigo, but only when I have a really bad attack. Also, I went directly to IVIG. We were going to try theophylline and terbutaline first, but he discussed my case with the french doctors and decided to go straight to IVIG. I was very lucky. I have not had a swelling attack since I started the full dose. It's been almost 2 months now. I have some side effects from the IVIG, but am hoping that will get better with time. Maria