Hello, like Arturo,I have recounted the number of SLCS patients, we are now 86 adults and 11 children known in the world. I noticed that about 45 adults and 3 children who have not logged in for more than one year and more ! I wish they didn't die from an attack. How to know if they are still alive? I APEAL TO ALL TO GIVE US SOME NEWS of your condition. I think is very important to know if all goes well !!!!! you are 66 adults and 7 children in the USA 6 adults and 2 child in canada 1 in new zeland 1 in australia and one child 1 in Ireland 3 in uk 9 in europe and one child PLEASE DO IT !!!! Anne laure.

Hello Anne Laure, I had my first attack of SCLS on May 04 2003. I am doing well and I hope the same prognosis for all members of this communitiy. Claude Pfefferlé 1953, Switzerland.

Hi I had my first attack of SCLS in October 2005 and apart from two minor attacks and several operations to correct my feet due to extensive fasciotomies to my legs I keep very good health. I continue to be on no medications for SCLS however I do take pain relief medications and a selection of natural medications Zinc, Magnesium and Cherryvite. Best wishes to all of you out there coming from Ruth Nolan 1960 Christchurch New Zealand.

Hello, My first attack dates back to June 1954 (I was then 11 years old) and reappeared about every 7 years. I was hospitalized the first time on April 5,1988 in severe collapse and "saved" by the assistant Chef de clinique of the C.H.U.NICE which suspected then an angio-neurotic pathology (not subsequently demonstrated). Four other hospitalizations followed and the diagnosis was established in April 1989 (in my 46th year). Various experiments have taken place during these hospitalizations, including IG to which I had very bad reacted what excludes me from this treatment). Through treatment initiated from my case by an acupuncturist renamed in June 1989 (followed to this day) I'm still alive 25 years after under acceptable conditions without another attack since. Although, for a long time,, the familial (various cases by the maternal side) and the very young age of appearance of the symptoms, have not taken into account by the medical profession, but the advancement of the knowledge of this disease permit to detect other cases by children with more recently a family case which was described in Israel (American academy of Pediatrics(, 2010, vol.125, no. 3 March, 2010, pp.e687 - e692). Best whishes to all ! Keep hope. God bless you. Maggy (from France).

I was first diagnosed in 2011 but attacks date back over 10 years. I am on IVIG and in generally good health.

My first acute attack was in December of 2011, resulting in faciotomies on all four extremities, induced coma, and 12 days( 5 in ICU) in the hospital. Extensive physical and occupational therapy helped to alleviate some of the damage.I had no prior evidence of SCLS before this.I began T&T, visited Mayo Clinic, and NIH. I began IVIGG in March of 2012 but was only getting treatments every 3 months while continuing T&T. I had a minor episode following the flu shot, but had just received my IVIGG the day prior. The attack did not amount to much. I had a second acute attack in November of 2012. During this attack I did not end up in a coma or have to have faciotomies. I spent 5 days in the hospital and recovered in a week due to the doctors knowledge this time around. I no longer take T&T but do IVIGG treatments every two weeks. I get 4g/kg and do 30g of IVIGG one treatment and 35g of IVIGG the following. I have had no other attacks or symptoms. I will be curious to see what happens this year around NOV- Dec with flu season though. That seems to be my trigger (upper respiratory flu like symptoms). I still battle nerve damage in my legs and feet, but I am able to exercise and keep up with my children. Best wishes to all! Cristina

I had my first and only attack of SCLS in February 2005 and I am in good health apart from some permanent tiredness. I am on no medications for SCLS for I didn't ask for as long as I am in good health. Sosotout

hi i just writen 2 weeks ago about my last visit to dr .Amoura in paris he conferm IVIG 1 gr/kg monthely he sed if the stiuation stell like that he well tray 0.5 gr/kg from next year after my meeting with him next april 2014 , i hope that our Brothers and sisters whisch sign in are still in a good health god bless oll of us thank you yaser roma italy

Hello Anne Laure. My husband's profile is one of the Canada accounts (monkeyracing). He is indeed alive. Still having episodes, but alive. He hasn't logged in for a very long time, but you can get his updates from my profile page.

Josephite, My heart goes out to you both for all that you have been through -- even after your husband was finally put on monthly IVIG in January 2012. I have a question: Have your doctors tried increasing the dose, or the frequency of the infusions? You write that he used to have adverse reactions to the infusions, and maybe that (and the expense) has kept the doctors from trying upping the dose (say, to 3 gr/kg) or the frequency (say, every 3 weeks). I've discussed a scenario like your husband's with some of our doctors, and they told me that they would try such an approach if the patient were willing. There is nothing sacrosanct about 2 gr/kg/month; it's the recommended *minimum* that works for most patients, that's all.

Dear Josephite, I have just read your update, I am sorry that your husband doesn't go well in spite of IVIG. Arturo has certainly right, why not to increase the dose? I hope things start to improve to him.I wish you some better days ,be brave !!! Anne laure.

Hi all, My wife elaine and I have a 5 year old little boy named Noah, he was diagnosed with SCLS about two and a half years ago, my profile is prima2007 and my wife is spidermans mom. We have been fairly quiet on the forums as it has been a very challenging couple of years especially about a year and a half ago when we just about lost Noah to an episode. His diagnosis as most I am sure we're very perplexing and came with lengthy visits in the hospital and "pokes" as Noah calls them. When he was first diagnosed he had seen every specialist except ear and brain and potential diagnosis were allergies, twisted scrotum and others. At this time we met our team of doctors and one being Dr. Anne Junker and is one of the foremost experts and has vast experience with the disease in Vancouver Canada where we live and where we are treated at BC Children's Hospitals and as far as we are concerned one of the best hospital's and care facilities in the world. His most recent episode that was severe started as a visit to emergency due to swelling and quickly escalated to organ failure where he had to be manually pumped full of fluid to save his life but was also harming him as it was filling him full of excess fluid which then led to compartmentalized syndrome and 6 fasciotimies on all 4 limbs. He then spent a number of days in ICU and with treatment of IVIG as well as other transfusions as he wasn't producing red blood cells on his own. Then there was weeks of physical therapy as he needed to learn to walk again after his surgery but before long he was up and around and now is a happy and healthy little almost 6 year old. IVIG for the most part has saved his life and or avoiding us having to put him in a plastic bubble to protect him as the littlest cold or flu can send him into an episode and to emergency. We are very fortunate as we have specific orders at the hospital and we carry a one page protocol from our doctor so there is no confusion on what needs to be done and we are treated and admitted essentially as soon as we arrive. In the fall and winter Noah is in medical day unit once a month for treatment for nearly the whole day and spring and summer that is moved to every 6 weeks and this will continue. Beyond this Noah again is a happy and healthy rambunctious, playful, inquisitive, loving and intelligent little boy you could ever meet. He has taught us so much through this very tough couple of years. From what we know there is another child in BC with the condition being treated at children's hospital. We are also being blessed by another situation where the Miracle Weekend at BC Children's Hospitals is being done June 1st and 2nd and rare disease is being highlighted and Noah was picked to do a spot on the telethon with one of our specialists as well as my wife and I are being interviewed for the hospital magazine to discuss Noah's condition. This is a huge opportunity for not only our sons condition to be recognized and brought to the forefront but rare disease in general as there are so many diseases that are life threatening and just as scary as some of the more mainstream diseases that are talked about on a more regular basis. We are very excited to share our story and experiences and hope that if we can help just one family then what we have been through will have been worth it. Please feel free to ask questions or share what you are willing as together hopefully we can help others overcome further challenge and bring further recognition to this disease. Take care. Mark

Hi Anne Laure, As you can read on the profile I updated it recently. I'm trying to get in contact with another patient living in The Netherlands, who is not active on this site. So I suspect two living SCLS patients in my country. Hiltjo

thank you Hiltjo, I am going to add at my list two people ! take care,Anne laure.

Thanks for your concern Arturo and Anne Laure. When you get as sick from IVIg as my husband does, the idea of increasing the dose is not an easy decision to make. We expect that such increases would actually reduce the amount of days in a month that my husband feels good, or somewhat "normal." It's not expected that reducing the time between treatments (3 weeks vs. 4 weeks) would do much good, because my husband has had several episodes that happened mere days after a transfusion. So, it becomes a quality of life issue. At this time, we feel an increase would simply reduce quality of life...and probably wouldn't provide us with much benefit. I think what we have now is the best we can expect for now. I think my husband would be more willing to consider it if he had a specialist with experience with this syndrome, but we just don't have that in our province. Edit: And I don't mean to be critical of our current health providers. They have lots of experience with my husband and know exactly what to do when he is ill. It would be nice though to work with someone that has a few cases of this under their belt. I think they would understand us better.

My daughter Ela was diagnosed with SCLS in September 2011 (she was 3 years old then). Her last episode was severe and resulted in a pericardial effusion and a couple of surgeries to save her life. I'm so glad to report that since her first infusion of IVIG in 2011, she has not had another episode. She is a normal, healthy and happy kid!

Hi ! My name is Dawn. My first attack was major on March 15, 2000. 4 months in hospital, died & rescitated 2x. Rt AK amputee, left foot nerve damage now supported with AFO brace & nerve damage in both hands. Second attack 4/2005. Third attack 9/2010..Then off to Mayo & diagnosed with SCLS by Dr Greipp. Tried T & T, but bad side effects & 6 more attacks in 8 weeks 9/2011. Have been on IVIG ever since. I go every 2 weeks & feel much better. If I suspect any symptom, I sit down to take it easy. It just isnt worth another attack-even a mild one. Take care everyone - we can fight this disease together !!

Hi Started having attacks in 2007 and was diagnosis in 2009. I think I have had SCLS since I was a child. I would get very sick from the "flu". I was having attacks every three months before I started IVIG about 2.5 years ago. I think I would have died by now without it. Since going on IVIG I have gone back o work full time and have completed my M.B.A. which I had given up on before IVIG. I hope other have been as lucky as me, Ritz

Hello, I am new here. My 7 year old has SCLS, I saw that more than one other child is known to have SCLS. I thought my daughter was the only child in the USA. She has had , 3 severe attacks, 1st was at age 3 in june 2009, 2nd / and the worst was Oct. 2011, and 3rd September 2012. She is now on Sub Q igg as she could not tolerate monthly IVIG. We are seen by Dr. Druey at the NIH. I finally decided to give Rareshare a visit. Thank you all for Sharing Kristy

Welcome to our SCLS community, Kristy! Yes, we all start out thinking, or we are erroneously told, that we or our child are just about the only ones afflicted with this one-in-a-million illness, but then most of us are actually comforted to find out that we are not alone. With you on board we now have 11 parents of children mainly in the 5-8-year-old range: 6 from the USA, 2 from Australia, 2 from Canada, and 1 from Italy. Check out the others: alapenna cmanmommy erintaylor jessclark kbas719 lcb042012 Maccy80 nairp sclsusr1 supermansmom

Hello leilasmom31, I am sorry to hear that your child has SCLS,fortunatly you had chosen doctor Druey!!! I am wondering if with so young patients this illness can vanish during the adolescence or not? This forum is very helpful,stay in touch with ! Anne Laure.

Kristy, The truly accurate way to check whether and to what extent anybody is having an episode of SCLS is to measure the concentration of red cells in their blood (because of a leakage of plasma out of the circulatory system). This can be done at home or anywhere with a portable device called a Hemocue, which requires pricking a finger and getting a good drop of blood for the equipment to analyze, and gives you an instantaneous result. Read all about it in the tab "Disorder Details," under "Diagnostic Tests," click on "Portable, instantaneous test for hemoconcentration" and also elsewhere in this site, e.g., in the Discussion Forum topic "Hemocue machine." If you're interested, talk it over with your doctors. You can buy it online.

Thank you , I will look into that . Josephine - maybe your husband would benefit from sub q igg therapy , its basically Ivig given very couple days in very small amounts . My 7 year could not tolerate monthly Ivig , she was terribly ill afterwards, but has almost no side effects from sub q ! Just a thought . When isay terribly ill , I mean headache , fever , dizzyness and vomiting and high bp.. And steroids would only push the reaction out by however many days the steroid was prescribed . Honestly I was hesitant about doing the transfusions at home but over time , it is so easy and so much better than spending one full day a month in hospital and a week of torturous side effects.

Hi everyone, Cara from Ireland here. Thank you for checking up on those of us who have not been in touch for a while. I have been mostly well since my two chronic attacks in 2008, and continue to manage my SCLS with salbutomol and theophylin. I do still get symptoms when I suffer a bad cold or dose of the flu but no further hospitalisations to date. I have noticed that the symptoms with a cold have become stronger though in the last 12 months so the leaks may be increasing in severity, it is hard to tell yet. But as IVIG may be a next step at some point I very much appreciate you all sharing your IVIG experiences. Wishing you all good health. Cara

It's good to hear from you Cara, after such a long time! Frankly, I feared that you might have died. Even Dr. Druey at NIH asked me a couple of years ago about you, because apparently you had reached out to him, and then he never heard back. I suggest you contact him ASAP, and make arrangements to ship him blood samples from your episodes, because he's looking for SCLS patients who are still having episodes. Most of us have since moved on to an IVIG-based therapy and have stopped having episodes, as you will read in these pages. It's been 3 1/2 years of normality for me, and less for most others. Dr. Druey is very happy for us, but the irony is that the success of IVIG treatments has meant a shortage of patients who can donate the episode samples that he needs for his laboratory experiments to determine what causes the episodes and why IVIG or other therapies work or do not.

Hello Arturo, I am wondering if it is possible to send to Dr. Druey some copies of my blood tests from Paris? thank you, Anne laure.

A new case of SCLS (in Berkshire UK) was just published in the British Medical Journal. i am contacting the author of the publication

Connor's first attack was in March 2009 and misdiagnosed- even though they knew something was wrong with albumin levels and other electrolyte numbers off. Nine months later he had his most severe attack that almost took his life twice. That attack had a blood pressure of 67/32 on the way to the hospital. That stay led to his diagnosis. After numerous trips to Children's Hospital Boston he has been given a standing order for Prednisone when we know he is leaking. His last attack was in October 2010. He has small episodes since but nothing requiring a trip to the hospital- just a round of steroids. He does not receive IVIG and is living a normal healthy life of an eight year old boy.

I've not been active in the community for a long time. I am doing well now. I had a hysterectomy for uterine cancer three years ago. I think that set off an episode of SCLS. I was hospitalized with pneumonia ( I don't think it was pneumonia, but SCLS) and very low BP. I ended up getting more and more swollen, and could not convince the doctors to give me prednisone. I spent some time in intensive care, then in a nursing home. After leaving on my own accord, we convinced our family doctor to try the prednisone. She did, and I recovered quickly. Now, I see a hematologist, who has given me a prescription to have on hand if I have another episode. So far, so good. I've not had to take the prednisone.

My first attack was in 1989, finally diagnosed in 2008. I have seen Dr. Druey at NIH and have been to the Mayo Clinic in Rochester, MN and they agreed that the diagnosis was correct. I had been on Terbutaline and Theophylline until recently. Three months ago began IVIG infusions with no side effects. Leading a healthy, normally life, thank god. Only side effect from SCLS is a drop foot, but it has not inhibited my lifestyle. This was caused by too much IV fluids administered during one of my early attacks.

Well I am one of the ones that has "fallen through the cracks" I am sure I have SCLS, chronic in my case, and had the IVIG for several months empirically last year and felt so much better, but had frightening liver failure (due to the IVIG?) and had to discontinue it. My quality of life has degraded since then and the slightest trigger mainly anything that causes my blood pressure to rise such as heat, stress, exercise causes symptoms like lowered blood pressure, nausea, vomiting, massive swelling, extreme muscle cramps etc. but I have yet to have had a definitive diagnosis. I have had this for about 12 years now and it is becoming worse each year. I have been trialled all other current treatments.

Hello everybody, It has been some time since I have wriitten in. To date, I have trialed: theophylline, turbutaline, singular, zocor, revlimid and decadron, then IVIG and now remicade. None of the drugs mentioned stopped my attacks. The next drug that Dr, Druey suggested that I trial is Gleevec, an anti-cancer drug that has been used on two patients successfully in Europe, Fortunately the Gleevec is taken PO. Hopefully my imsurance will approve it. I will try to keep everyone posted. Thanks Steve (heptagona)

Hi Heptagona. You are probably in the same category as I am. I trialled IVIG for 6 months with some success (I think) and am now not on anything and am still ill with the chronic version. Last summer, although it was hot, and I was active, I did not have the chronic leaking all summer. It was a dry heat and I have no explanation for it as I have had this condition solidly for about 13 years. Like you, I have trialled all the usual drugs that you mentioned with limited or no success.My quality of life is gradually slipping away I hope the Gleevec works for you. Please keep us informed as your input is very valuable. All the best!

I hope you have some answers on Wednesday to this Tolleya. I do not think people realise how debilitating this condition is. I also have Ehlers Danlos Type 1V, What type of EDS do you have ?I wonder if there is a link somehow? There are so many parts of this puzzle unsolved as yet although much progress has been made. All the best for Wenesday

Checking in for my son, Andrew, now 12. Although one of the doctors at AIduPont Nemours still believes he has SCLS, another specialist there and one at Johns Hopkins have diagnosed him with idiopathic anaphylaxis, not capillary leak. We have stopped his terbutaline since October, with no adverse effects to his health. Since the idiopathic anaphylaxis is something that he can grow out of, we are releived. He has been 2 1/2 years without an attack (he has had 3 that put him in the hospital for observation each time). He still carries an EpiPen everywhere, but we have greatly reduced his meds. I still follow all of the posts on here, concerned for all of you. God bless you all - reading your posts the last two years has given me comfort and support during what has been a very very scary time in our lives.

Hi all, just checking in to let you know I still follow to hope you are well and keep up with any progress in cause and cure. My SCLS patient had his fort major episode 2/2003 and passed away 3/2006. I used to talk to Judy on the phone when we thought it was just us...I continue to keep all the families suffering with the uncertainty of when the next episode will come and how bad it may be in my thoughts.

Hi! My name is Shannon. I was diagnoised back i Aug 2011. The first episode I nearly died . THankfully to a complitent ICU staff and Kidney Doc he saved my kidneys from dialysis. I remained in hospital for 7 or 9 days. After the first event there were 3 or so just as serious events the nevt 6 months. after that about 1 ever other month i was going to the hospital for what I thought was confusion and couldnt pee but was CLS due to the fact of 4+ edema to legs aand creatine at 10. this continued until last fall were I started going every month for events. I was diagnoised by my renal doc and also the mayo clinic through lay tests an skin and muscle samples. I did not tolerate tubuertline (sp), THeophine (sp) or Sudaphed. I am lookig for a doctor to try thr IVIG. I was living in FL and my doc there was going to try it then my husband got trasfered to KY. I am on my scond kidney doctor in the last year. :( I did talk to the doctor in the NIH study and hope to be excepted and find out what els is wrong. I tested postive for carcinoid tumors and the last 3 years the levels have only grown. last value doubled from lasst time checked. prior to this I was a healthy stay at home mom or twins that I home schooled. shannon

Hi everyone, I am new to this forum. My daughter was first diagnosed with Capillary Leak Syndrome in 2006. At first, doctors could not figure out what was wrong with her. She was in NICU for nearly 2 weeks when her body somehow repaired itself. Because her symptoms were not consistent with just one condition, she was discharged with primary diagnosis of CLS and 7 secondary diagnosis. She was a perfectly healthy child before that and for the next 2 year years after that episode. Then in October 2009, she wasn't feeling well. The symptoms were nothing like that of 2 years ago so I honestly did not think much of it. Thought she was just not feeling good. It wasn't until she complained that her knees and arms hurt (joints). That was the first symptom she showed that was the same as 2 years prior. I rushed her to the emergency room at Stanford Hospital. 3 and half hours later, my little 6 year old passed away. To this day, I regret not doing more research on capillary leak syndrome. I had never even heard of Mayo Clinic until I started doing research. I knew absolutely nothing about this condition until it claimed my daughter's life. I wish I had "met" you all prior to October 2009 - there's a possibility my babygirl would be with me today :(

mclcrouch, I'm very, very sorry to hear of the loss of your daughter back in October 2009! If it will help you deal with your regrets, consider the following: 1) Back in 2009, we knew very little about how to prevent and manage episodes of SCLS -- especially in children, and that's a fact that your having done research on SCLS would not have changed. In November 2009, I lost a RareShare friend to a severe episode of SCLS, and she was a nurse who had done her research on this illness and thus knew everything that was known at the time. 2) Your daughter may not have died from an episode of SCLS, but rather from something else which may or may not have been related to SCLS. Children are more vulnerable than adults. 3) If your daughter was in fact having an episode of SCLS, the ER doctors at Stanford Hospital probably did not know what to do or how to do it. Even if you had known something than they didn't, they probably wouldn't have listened to you, anyway.

Josephite I have been on IVIGG since 2011 and at one point went on every two weeks. As with your husband it seems for a week after the infusion . I am tired and spend most of the week in bed. So I was in touch with Dr. Pecker and Dr. Druey and put me back on every 28 days once a month . It is better because Now I have one bad week a month. I think it may be due to the fact that I sleep 2 to 3 hr a night after the infusion and am tired all that week.

Hello, this is Mary Jane, or mjanie . Problems with my computer, had to create someone new. I was diagnosed at Mayo Clinic in 2004 with SCLS. Was on cocktail meds, had attack in July, 2012. I am currently doing IVIG fusions every month. Will be two years in July and I have been attack free!! Amen! So very thankful. Best wishes for good health to us all!:) One more thing, while on cocktail meds I had many attacks, since fusing IVIG, NONE!:)

Hi Custom. I agree, it's easier to deal with one bad week rather than being sick all the time. Unfortunately, my husband's reaction to IVIG can cause more than just feeling bad. He can also get severe rigors and has had to go back into hospital mere days after receiving an infusion. For these reasons, we can't increase the dosage or experiment with the protocol too much. :(

Hi aporzeca, because they could not determine the cause of death, the Santa Clara county coroners office performed an autopsy. They reissued her death certificate from pending, to systemic capillary leak syndrome as the cause. I am grateful to have found this site. The wealth of information is amazing...bittersweet indeed.

I am so terribly sorry. There is nothing worse than to lose a child.

Agreed. I'm so sorry to hear of your loss.

My son Connor was first diagnosed in December 2009. Since then he has had two minor attacks and is doing well with no medication.

Hi, I thought I would give an update on my status. I was diagnosed in January 2012. I spent all of 2012 basically ill mostly on and off. By 2013 I was always sick and constantly swollen. I was bedridden. Taking a shower was exhausting. My patient and loving family would keep track of all my medicines, give them to me on time and force me to wake up to eat a little and drag me to all of my different Dr. appointments. Around March 2013 my patient and loving family had decided it was time for me to make some changes. I posted my first comment on this site at about that time. I had been following but never posted anything. With the help and support I found on this site and the encouragement my family gave me , I finally got up enough nerve to consult with my doctor. That consultation with my physician, along with some very good advice I received from Arturo and others on this site lead me on a journey to the Mayo clinic in Rochester, MN. I actually just had to fill out a form online (having the talk with my Dr. was the toughest part). And I was fortunate to receive a phone call the next day in response to that online form. They telephoned me to set up a schedule of appointments with Doctors at the clinic. My first appointment was with Dr. Kapoor, who is a hematologist at Mayo and very familiar with SCLS. I was supposed to spend a week there consulting with experts to hopefully determine my best course of treatment. I ended up staying for three weeks. Four of those days I spent in St. Mary's Hospital with what was diagnosed as cellulitis. The rest of my extended stay was spent consulting with other doctors in other departments. I saw these other doctors because a few suspicious items showed up in my first blood draw. I wrapped up almost all of the tests that were ordered, but I have to return for an endoscope and a colonoscopy and a few other procedures depending on what they find on the first two. I don't believe these are related to SCLS. All in all it was a fantastic trip! I am currently receiving IVIG infusions every three weeks. It is Flebogamma 5% DIF solution. I am getting three bottles over six hours, or one bottle every two hours. I had an emergency cholecystectomy in September of 2013 for an enlarged and bleeding gallbladder. I received my first IVIG infusion prior to that surgery and have continued to receive one every three weeks since then. * And just a side note, I had several abdominal CT scans with and with out contrast beginning in 2005. My doctors were looking for Ascites. Each time I had an SCLS episode, my abdomen would swell until I looked like I was nine months in my "pregnancy", so each time my doctors would order abdominal CT scan. The first CT scan report in 2005 noted that my gallbladder contained gall stones and suggested a follow up ultrasound. Each CT scan report following the first one, noted changes and listed concerns about the condition of my gallbladder. The last CT scan I had, at that doctors office, was in 2009. The report from that final scan noted that my gallbladder was now septic and deteriorating or decaying. I don't recall which adjective was used to describe the sad state of my gallbladder. I recently reviewed all of these reports with my new primary care physician who was able to conclude that the reason for my off the charts white blood cell count and my low hemoglobin count was due to my bleeding and infected gallbladder. I learned I had a systemic infection for over three years! Combine a systemic infection with my SCLS and no wonder I was bedridden and so very ill. I am amazed that I am still alive.* But thanks to my bad gallbladder I was able to receive much needed IV antibiotics and my first IVIG treatment. I am thankful to report I am doing well.

Wow what an extraordinary story. Who diagnosed your scls in the first instance? You are indeed, lucky you are still alive:) You are also lucky you found the right doctor to help you. I have not even had a definite diagnosis but I am suffering terribly with all the symptoms of chronic SCLS and have been for years. I am now very anaemic and nobody knows why. I am seeing yet another specialist next week but don't hold out too much hope. I hope you continue to get better and better. You are one very fortunate lady. All the best!

*I am sorry I have two profiles, I need to fix this. I am the same person that posted just above your reply.* *I have been fortunate to have some amazing professionals who cared for me. I was told I was crazy by so many doctors I sought psychiatric care. On the second visit to my psychiatrist I was in the middle of a very bad attack. She took one look at me and told me you do not have a mental condition causing your illness, you have a very real physical condition causing your illness. She then spent he entire session on the phone trying to reach the very doctor I had been trying to get an appointment with for several years. She would not take no for an answer and informed his receptionist if she did not hear from this specialist by 5pm that day she would begin calling his office every hour on the hour the next morning beginning at 7 am. I left her office and she phoned my home at 5:30 pm explaining all of the information I would need to gather and bring with me to my appointment with Dr. Carl Lauter which she scheduled on my behalf in exactly three weeks from the date of that phone call.* My illness wouldn't wait three weeks, so I met Dr. Lauter in the hospital. He had been called in by the ER doctors who admitted me the night before due to another SCLS attack. The big coincidence that led to my diagnosis happens to be that the very day Dr. Lauter met me in the hospital he had been contacted by a nephrologist from the Mayo Clinic to consult on a suspected SCLS diagnosis on a woman who was being seen at Mayo clinic. He later explained that after meeting me in the hospital the same day he was asked to consult on the other suspected SCLS case he was able to see the similarities and made the connection during his car ride home that evening. I have a very high threshold for pain. I didn't experience any of the usual pain associated with gallbladder attacks. So it wasn't just doctors ignoring the CT scan reports that caused my gallbladder/infection/anemia to go on for so long. While at Mayo I discovered that I had a few broken ribs. The doctors asked me about my cracked ribs after reviewing my x-ray. At the start of any SCLS attack I would fall asleep on my feet or while sitting down and even in the middle of dinner at a noisy restaurant. So I was used to hitting objects with my body. I would notice bruising, but not broken bones. Just recently my doctor discovered I had a nasty case of Shingles on the left side of my torso. I didn't experience pain, just noticed the rash. Don't give up hope. Go over test results with your doctors. Look for tell tale signs like low Albumin levels. Keep a record of the first symptoms you notice before an attack. Have an allergy test. Keep a food diary. Maybe you have already done all of these things, I am no expert that is for sure. I can assure you that I will keep you in my prayers. I still have a long way to go. I have other physical problems that I need to address as well as a few surgeries in my future. Please don't get me wrong I am very thankful that I have come this far. I am hopeful that you will soon receive your diagnosis and also be well.

Thank you for your compassion and encouragement! I was at the point of giving up but your kind comments have spurred me on again. All the best with your own issues

Kim: The woman to whom you refer in your post , is me . I've been a patient of the amazing, brilliant , Dr. Lauter for approximately 2 1/2 years . He saved my life as far as I'm concerned . I receive 60 grams/ month of Flebogamma 5%. I receive 30 grams every 2 weeks and also a bag of fluids for 45 minutes at the end . It takes around 8 -8 1/2 hours total . We are very fortunate to be under Dr. Lauter's care . I'm sure you know how thorough he is ! He even suspected another medical condition missed by Mayo and referred me to the appropriate Dr .( His personal Doctor ) . Turned out he was right . I'm glad to hear you are doing well . Wishing all our members good health.

Hi - I'm 51 years old. My first attack was Dec. '12, second was Jan. '14, and third was 3/14, which is when I got a diagnosis. Made it through the first 2 attacks without medical intervention, but the 3rd was worse and ended up in ER and then ICU, 34 liters of IV fluids, compartment syndrome, myofasciotomies, skin grafts to repair that surgery, and a total of 15 days in the hospital. All in all, I've had a very easy history compared to many. i am grateful and my heart goes out to you all. For what it's worth, I used eucalyptus essential oil during my first 2 attacks because it's a bronchial dilator, and I do believe it helped. I applied it topically to my chest. It is possible to overdose with strong essential oils, and some people may have reactions to it, so do be cautious if you choose to try it. I also dealt with the swelling by pumping my lymphatic system, because one of it's big jobs is to pick up interstitial fluid. It's not pumped by the heart like the circulatory system, it's pumped by the motion of the body, especially walking, and specifically by pumping the calf muscles (the gastrocnemius and soleus). For me, when the dizziness and faintness got too bad, it worked to elevate my feet to balance out all the fluids, and then point and flex the feet to pump the calf muscle, and then when I felt good enough, walk around very deliberately focusing on the calf muscles. THat third attack I didn't use eucalyptus because I'd become convinced that my weird symptoms were a reaction to the eucalyptus. Once I got the diagnosis, I knew better and have been so relieved to understand what's been going on. I'm in Maryland, so I get to see Dr. Druey. WIll be going in to NIH at the end of this month. Got IViG before leaving the hospital. Suppose I'll continue with that therapy once we get all the kinks worked out with who's treating me and whatnot. What I"m really excited about is that once I see Dr. Druey and he gets a baseline for where I'm at, I'm going to start working with a naturpathic physician and a nutritional therapist who uses EAV technology. My hope is that Dr. Druey will agree to see me 6 months or a year after that and we'll see if a natural approach using nutrition, herbs, homeopathy, and etc. will make a difference. THanks to all of you who have shared your stories and experience. It's helped me a lot. cheers, Cathy

hi i like to aske all of you about the ivig dos that every one riceve every month becouse i have a probleme now with my dos i stell have 1gr/kg a monuth i writ also to Artouro and he answer so if posibal to have some information about all of you alsso if any have a sidefect after recived the ivig thank you yaser

What sort of problems do you have elganzory? Sometimes I used to get headaches, mainly if the rate of flow was too fast, with the infusions and skin rashes too. I believe this is not uncommon.

I had just some headeches but i ask about how many gr every boudy have in one mounth to dised what i have to do becouse Dr Amoura now ask me to reduce to 0.5 gr/kg so After i toaik with Arturo and My doctores at rome and with Dr Dueary i like to bear from all of you Thankes

Hello Everyone My name is Andrew Davis. I'm a 49 years old living with my wife and two teenage sons in the Inner City Sydney Australia. In Dec last year I started to get sick with this unexplained illness. Renal Failure aches and pains and my body being flooded with Ascites. For four months whilst I was in hospital the only diagnosis they could come up with was Mylio Fibrosis, which they apparently found after extensive bone marrow biopsies, but only in cellular form. Subsequently they started me on a course of Hydroxia, which is a Chemo drug. My condition seem to worsen and fluid started to invade my lungs as well as my heart and abdomen. My breathing became harder and harder. After my second Flash Pulmonary Adema attack, which had me on life support the immunologist, which was one of the five specialist treating decided it look more like a Autoimmune disease and started me on a course of Panafcortelone and IVIG. My condition improved almost immediately and has done since. Seems that the SCLS is the most probable diagnosis that seems to fit. I was reasonably fit and up until Dec was cycling between 2-300 kms on my rode bike Think this was what help me get through this. I'd be very interested to see if any SCLS sufferers have problems with their voices. I have had problems with my vocal chords for some time and wonder if there is any connection Kind Regards Andrew Davis

Hi Andrew, My name is Melanie, my son Ryan who is 10 was diagnosed with SCLS in February 2013. We live at Seven Hills in Sydney, and visit the Children's Hospital at Westmead every 3 weeks for 30mg IVig infusions. He has not had any severe leaks since starting the treatments, but he does continue to have minor leaks, and does get severely sick from the infusions. He was very fit and healthy and never got sick pre Feb 2013. He has put on 15kgs in 15 months and although he does complain of a sore throat I can't say he has problems with his vocal chords. Were you sick with any type of virus before December? Where are you treated? If you would like to contact me my email address is mel.mcf@bigpond.net.au. Cheers Mel

Answering Elganzory, I am not sure how to read the dose but I am on Ocatagam, and on my chart they give me after my treatments; it states 1 g/kg Daily x 2 doses month.

To Andrew, My voice is a leading indicator when I get symptoms. It gets scratchy, and rough. now I'm on IVIG its a lot rarer than it used to be. Jeff

To Andrew, I had problems with my vocal cords when I first became sick. This was years before I was diagnosed with SCLS. It would get so bad that I would have to struggle to speak. They had no explanation for why it was happening. They had me try 6 months of speech therapy which did nothing to improve it and I actually ended up developing lesions on my vocal cords. I had to have surgery in 2005 to have them removed. Within a few months after surgery everything went back to normal and I haven't had any problems since. I would often feel like I had swelling in my throat but it never affected my voice like it did that 1st time. Maria

I have also had similar problems which is very frustrating given that I have previously always had a strong voice and that I have to speak to large groups regularly as part of my job. Andrew I live in Canberra and had my first attack around 13 years ago, diagnosed around 3 years ago and have been treated with IVIG since. My immunologist and I wrote the submissions which resulted in IVIG being supplied by the NBA. This is not permanent and we need to keep data in front of the NBA to keep this treatment approved.

Hi Wazza & Maria This is all very interesting. I am a keen bike rider and in the months before my initial sickness was cycling between 2-300kms per month. Interesting three people with SCLS have reported having voice issues. I was told 5 years ago I had "Spasmodic Dysphonia" of the vocal chords. Which meant that starting to talk was the issue. Almost had to start growling before I started talking to get the vocal chords moving. I was having Botox injected into them. This would initially take my voice for ten days then give me a clear strong voice for up to three-four months at a time. Wondering if it all could be connected. Are you into exercise Maria ? Cheers Andrew

Dear Andrew, and others The latest study on SCLS suggest a divers biologic pattern in patientes with the disease. However it is boord te Search for similarities within the group. My problems were initiated through a 8 days lastig cycling tour in the mountains, as well during comparable circumstances. I used to cycle tours up to 200 km. Nowadays all kind of exercise can be a trigger. I know this is not the case wit all people on this forum, but still I'm curious If anybody do recognise it. Greetings to all. Hiltjo

Interesting. I have had some trouble choking on food and was told my throat swells a little during minor attacks. I have had people ask me if I have a sore throat because of my voice but blamed it on allergies. I do know that many of my major attacks happened after exercise or intense activity before I was on IVIG. Since I had been on IVIG my attacks have been minor but I don't have a good way to monitor them, I just go my degree of swelling. I also seem to be have TIAs once in a what. I am a little afraid that the IVIG is not doing as much for me as before but I am probably worrying about nothing. Rita

Fortunately I have not experienced any problems with vocal chords as several people have mentioned. Occasionally, I notice my voice becomes raspy but I think it may be associated to allergies. Like apdavis and Hiltjo, I too cycle regularly, up to 400 miles each month. Extreme exercise has not caused me to have any new attacks. Prolonged exercise in heat does affect me, but drinking electrolytes helps regulate me. I have been on monthly IVIG infusions since June last year-with not attacks,thank god.

Dear all, I wondered about the different patterns within this group, like mentioned above. As well concerning the triggers, and patterns of disease (beside the major attacks). Maybe the patterns differ because of biologic variation in the origin of disease, but maybe an explanation can be found in: - Were you put the strain (longer time or regularly) , the problems might occur most clearly- Do you recognise anything or not at all?

I was also wondering on rereading this thread if everyone had allergies of some sort or the other? I see quite a few members here do.Is this a common link perhaps?

Hi Windows (?) Indeed it could be a common link, I suggest you to start a new topic on the item 'allergy' . For me personally the answer is yes (allergy, anaphylaxis in the past) but I do not experience problems with allergies recently. By the way, I read your personal comment about taking salbutamol in stead of terbutaline, which is not available as a tablet in New Zealand. I'm using terbutaline as inhaler 4 times a day. I think it is avalable at your place as well. Maybe something to consider. Hiltjo

Yes, Windows is my strange name! Sarah really. I used to use an inhaler as I have always had asthma. The strange thing is the asthma went and the swelling began immediately...... I still have an inhaler somewhere. Maybe I will try it again. Nothing to lose for sure as all other options so far are closed to me. Thanks for the suggestion Hiltjo. I will start another thread.... Exercise is also a trigger and anything which causes my blood pressure to rise. Is this the same for you?

Hi Sarah, Sorry I didn't reply, to give an answer to your question: exercise is indeed a trigger, and a kinds of similar influences are too. In steep contrast of the years before. So: yes I recognise your statement. The link with blood pressure is not that simple to answer. Sometimes exercise did lower my blood pressure on the very moment in stead of the expected: rising it. on the other hand exercise (Max)tests (with-> BP) all had a negative influence afterwards in general. it varies in relation to the degree of stability at a soecific period I guess. Greetings to you

Greetings to you too! Do you find just prior to having an attack that you also feel very nauseated? I always do and it is a signal for me to lie down

I am not aware of any allergies but have not been tested. However, exercise lowers my heart rate and BP. My periodic swelling is mild to pitting, but has only been super quick and dramatic twice. I dehydrate every ten days to few weeks even with sufficient oral hydration. I do not get nauseous per se, but I do feel epigastric squeezing and sometime mild queasiness.

I have no knowledge of any connection between allergies and SCLS. I, for one, never have had any allergies.

I didn't see my name on here so I am not sure if I am on the list for USA adults, I began having severe dehydration attacks with some minor swelling in 2008. After many trips to the ER, I was diagnosed with SCLS in Dec. 2010 after a very severe attack where I nearly succumbed to kidney failure. I have been on IVIG therapy since April of 2011 and have not experienced any further symptoms related to SCLS. I receive 35grams over a three hour infusion once a month thanks to the VA. The only side effects I have noticed from the IVIG is headaches and am usually given Benadryl and Tylenol at the start of the infusion and that seems to help but does make me sleepy. Photodonn

Hi Hiltjo , wazza, and others. I have been training extremely long hours, and couldn't work out why my voice 'went' when I knew I was not mouth breathing. This started about 9 months before my first apparent attack. One of the other features of my syndrome is the extreme muscle pain needing morphine ( which I never take.. Not even paracetamol). The onset of the attack takes minutes, resulting in hct of 65%, with sats <70%,. BPAP rapidly brings my sats up, and the whole process lasts about 4 hours, and goes as quickly as it came. I can literally sit up, take off the mask, and be ready for home. Arturo's idea of a home haematocrit is the go for me. I will also get onto daily weighing as my retention is up to 6kg. WAZZA, I got my first approval for octagam (IVIG) today, delivered from CSL. Interestingly, I also exercise a lot... Last year I walked >1000 km, my vertical ascent was >9,000 m; and I have maintained my fitness except for limitations caused by my SHREK ALTER EGO (scls). I reckon my first attack of severe myalgia happened on the first night on the KOKODA track last Sept. Cheers John John

I feel so frustrated as I had to stop the IVIG after 5 months as I developed hepatitis. I now am having NO treatment and my quality of life is diminishing daily. I have daily swelling, nausea and severe myalgias and the worst muscle cramps imaginable after even the most minor of exercise. I have had no definitive diagnosis after 12 years. and not one doctor has ever asked me to go on a treadmill or exercycle and show them what happens when I exercise despite my suggesting it!! So I don't even know if I have SCLS but each time I come on this board, I know that is what I have. I did try acupuncture as Maggie on this board has had some success with it and it made me swell up so badly immediately afterwards, I was afraid to continue! Maybe I should try it again. I had 2 sessions and swelled up both times and felt quite unwell. The damage to my body is extensive and visible with pockets of stretched skin. I think the more one swells, the more one can swell as the tissues just get even more extended. Even though I have been hospitalised with this three times, nobody seems to take it seriously. So where to go from here? I don't really know..''

Wow Windows! Wow! I certainly empathise. What brand/ batch IVIG gave you hepatitis? Have you tried compression stockings, or "skins" which come in both upper body and lower body models? John. (Alter ego Shrek)

Goodness Iit was the end of the year before last going into last year when I had the IVIG. I think it was Intragram P if I remember clearly. Yes I have compression stockings which help a bit and I had a custom made full body compression garment (it was awfully uncomfortable!) but even then, the swelling went through it and I had to take it off as I felt so miserable with the tightness as if I would explode. If I lie in a cool room 24/7 I am OK. but who can live like that?? I developed the hepatitis (drug induced they say) about 4 months into the treatment and it was so bad, I had to undergo a liver biopsy go on large doses of prednisone and I was about to be put on a liver transplant list so it was not a small event. I do believe the IVIG was helping too but it was just experimental, not science based and of course, very expensive. My insurance company covered the treatment costs and the drug was subsidised by the govt. My blood work a that time was shocking - all sorts of strange things appearing.

I am so sorry about Window's experience. My swelling is certainly not as severe. My last critical event was August 2012. However, I was in the Emergency Department again yesterday and last night with marked dehydration and moderate edema. It was the 8th time this calendar year and I am getting both frustrated and worried. My primary care physician and hospitalists in Maine are uncomfortable trying IVIG due to the risk of general anaphylaxis and continue to prescribe IV saline and hydrocortisone, which hold me only a few to several days. They are excellent physicians and former colleagues who care for me deeply and personally but don't want to introduce further risk. They have recommended Mayo Clinic and I am in the queue for a January 2015 work up. So, I am praying that I hang in there until that time. My dear friend and former husband, a retired emergency physician, is also frustrated and worried about my mortality, and has recommended that I relocate close to Mayo Jacksonville so that I can get definitive treatment in the event of a critical event. Any suggestions from Arturo or the group? Fours years ago I was back-packing in rural Zambia and volunteering in a free school and orphanage there. Now, I don't tolerate air travel very well and will need to hire someone to help me get to Florida by car or train. Life certainly changes. Susan

Hi, I'm not sure if I'm counted in that 66. It turned out that my 5 episodes over 2 years, 2010-2012, were not SCLS. I was experiencing anaphylactic shock due to an acquired allergy to taking psyllium fiber followed by exercise. I did inform Arturo & I updated my info page. All of you were so wonderful as I endeavored to learn more about SCLS because a doctor diagnosed me with it. It was through a process of trying to get on IVIG that the immunologist in Denver who actually gave lectures at NIH & knew Dr. Drury, was most hesitant to give me the SCLS label. Over a period of several months & many tests, we discovered the actual cause. Again I want to thank each & everyone of you that was so thoughtful & helpful. My heart truly goes out to you folks who have SCLS. Having been in ICU 3-4 days at a time was very frightening so I can slightly identify with what happens when one has an episode. May God bless you all. Ann

Hello Stedrick, Regarding the allergy to IVIG. I have heard that it is more likely if your IgA levels are raised, and that the converse is true. Mine were tested prior to starting, ( of course I needed immune electrophoresis as part of my work up). Nevertheless, I still had to ask the question during the infusion as to whether the pretty standard reaction (headache chills fever) were allergic or not, and Yes, it boils down to faith in your physicians, an element you are lucky enough to possess. However this faith does not preclude you from asking if there is a correlation between allergic response and IgA . John

Thank you, John. This is very helpful. Susan

Diagnosed with SCLS in January 2014. Louisiana USA

Hell Stedrick, I am interested in your autonomic dysfunction. For the months leading to my first attack, and still now, after my first IVIG, I have an extreme intolerance to temp change, and in the prodromal stage, I would often complain that I had 'run out of adrenalin'. This is to suggest an adrenal medullary problem, not a cortical problem.. And having said that, I believe my 24 hr urine was normal. Doesn't resting phase normality irritate you? John

Re Autonomic Dysfunction Before the SCLS was taken seriously, my blood pressure, heart rate and body temperature would drop precipitously. At one point my heart rate dropped to 30, I was placed in ICU and transported to a tertiary care center and a pacemaker was implanted even though all arrhythmia, heart disease and coronary disease were ruled out. The pacer only fires when I am sliding into an SCLS episode. Florinef supports my blood pressure fairly well. Also before SCLS was recognized [except by my former spouse, who is a physician], my adrenal cortisol levels were a tiny bit low but I was wildly symptomatic per above, so I was diagnosed with secondary adrenal insufficiency [good adrenals; questionable pituitary] and placed on steroids. This, of course put my adrenals to sleep and made me steroid-dependent. However, they were unable to do an MRI of my pituitary due to the pacemaker. These days, I am fairly autonomically stable except when I am dehydrating and swelling, the prodromal phase of SCLS. During that time I shudder with goose bumps, then flush and perspire; my blood pressure goes up and down but the Florinef helps prevent a drop below the low 80s over 50s. The pacemaker supports the fluctuating heart rate. My big question for Mayo Clinic Jacksonville is whether the stabilization of my SCLS may mitigate the autonomic dysfunction and, in turn, enable them to determine whether the pituitary-adrenal issue was a red herring. Hope this helps, Susan

Thanks Susan, Many similar symptoms which I can only theorise on. I too had increasingly severe palpitations which preceded my first severe attack by months. 6 months before, I had a normal Holter and echo, and was fobbed off. During the first attack I swung in and out of bradycardia from 130 down to 30. The bradycardia was a problem. Was it due to a vagal hyper response, or did it bring on the vomiting and urge to defaecate? The ambulance ecg suggested a junctional rhythm. However an echo in ED suggested a Bezold Jarisch syndrome where the empty left ventricle ( from dehydration) went into a paradoxical bradycardia. I have resisted a pacemaker. Indeed, the electrophysiologists agree it is not needed at this stage, yet I still get heaps of trigeminy. The Trigeminy appears to occur when I am either overhydrated or under. I too get the goosebumps, flush, perspire and become hypotensive. I manage these for the main part by heaps of Gatorade. I guess we will never really get to the bottom of the autonomic instability, but sure as God made little apples, I am determined to get back on my bike.

Dear Sarah, (Windows) Reading your frustration about not being sure about diagnosis and the hepatitis which urged to stop with IVIG I feel sorry for you. It is hard to have a lot of limitations without knowing how to escape, or knowing If there will be any kind of improvement later. you wrote not to be taken seriously because no. exercise test was performed. But take care, it could induce more problems! If they would perform such a test please ask for simultanously testing of acidosis ( and lactate). When ever testing: insist on it. I can give more information on that item. as you are a chronic sweller as well, discuss about pro's and contra's of mightbe using imatinib tablets. At least three SCLS persons (world wide) are treated with it, with more or less succes as far as I know. partly publicated. Should be done in an Academic setting (!!) because of the potential disadventages, costs, and evaluation, your liver dysfunction could be a contraindication, so a open discussion ( balancing) and medicall advise should be the first step. Stay carefull. You can contact me for more details. Hiltjo

Thank you for your post. I looked up Imatineb. It sounds a bit scary! How do I contact you please? The last specialist I saw said there was nothing he could do except he would see me in the emergency room next time I was admitted!! Not very comforting at all..... I would just like to provoke a leak in a hospital setting which I can do easily by exercising or getting very hot and have bloods taken whilst in this state to try to prove or disprove what is happening with my body chemistry. My latest blood tests show I am quite anaemic with no known cause. Is this common with SCLS? Question: Can one take albumin orally to help with oedema?

Dear Sarah, Please send me a message on my mailaccount which you can find in my profile. I would be very hesitating by provoking an attack. Anaemia is not specific for SCLS as far as I know, except for the recruitment period because of haemodilution ( more fluid returning to your bloodvessels). Taking orally albumin will problably not be of any use, because the problem is not a deficit of albumin, but more the albumin leaking with the fluid out of your vessels, and vice versa. Hiltjo

Hello Sarah, While the thought is good re albumin, there are several unknowns. First, albumin is generally not absorbed well at all from the bowel.. The suggestion is that approx 2% of ingested albumin is absorbed as such, but most is denatured by the bowel into amino acids and peptides before absorption. Secondly, of course that is no predictor of what actually happens during a CLS attack. There is indeed the possibility that the bowel may well LOSE albumin during the attack, although I am not aware of this being demonstrated. Thirdly, much of the way food is prepared results in denaturing albumin. On a positive note, if you wished to attempt albumin ingestion, this would be easy, inexpensive, and, I suspect, unlikely to be in any way harmful. I.e. ingested egg white... I personally am quite partial to an egg flip! On a practical note, albumin is synthesised in the liver, and it is important to check liver health with your physicians, and avoid liver injury with e.g. Alcohol, or paracetamol excess. Cheers John

Thank you John. It was just a stab in the dark for self help! I believe my liver has recovered fully from the hepatitis I had. I will never know whether it was the IVIG itself or something in one particular batch of IVIG I had as I was sitting there clutching a bowl that time, feeling really sick. However the doctors are too nervous to try it again understandably. I don't have alcohol excess nor take much paracetamol. I have been asked to go into the local hospital for further testing in about 5 weeks so hopefully other blood tests will performed in a safe environment. I will certainly be taking My ICU 5 minute assessment with me and will also leave a copy there. (Thank you Arturo!). I am not sure what is planned for me and will update afterwards Sarah

Sarah, you are a legend. A real trooper! John

No, not me John but it is nice of you to say so! The real legends here are those that have survived so many major attacks and live to tell the tale and share their experiences on this board to help others. John I read your profile and was interested to see that you had previously been 'very' fit. I have noticed that quite a few SCLS patients have been super fit including myself and wonder if there is any commonality there? Sarah

http://www.ncbi.nlm.nih.gov/pubmed/18190880 Hi Sarah, I am quite convinced that my form of the Shrek disorder started with extreme physical stress.. But that's me. The role of physical and emotional stress in auto immune disease forms the basis of huge areas of study. http://www.ncbi.nlm.nih.gov/pubmed/18190880 This also applies to heart disease, viral susceptibility and others. Another point of interest is why some endure severe compartment oedema, while others (like myself ) suffer pulmonary oedema mainly. Others have major hypo albuminemia, whereas some are only borderline. My facial oedema seemed to be due to the treatment (recruitment phase). Does IVIG work because of its osmotic effect? Or is it an immune mediated effect? When it all boils down, we each have to tread our own path, all the while keeping an open mind about the view. Cheers John

Yes I agree about an 'event' being the possible trigger. I had pneumonia and was ill for 6 weeks. I think, but cannot be 100% sure, that was my trigger. Your questions about the IVIG effects are fascinating! I had not thought of those different options. I just assumed it was immune mediated....

Interesting. I had a series of viral illnesses that culminated in a superimposed bacterial pneumonia. I had had mild symptoms before that time and a major crash thereafter, followed by many episodes of dehydration and edema. Although this and the co-occurrences above are circumstantial, it will be interesting to see whether they are considered in future studies.

Hello Susan, have you been tested for autoimmune autonomic ganglionopathy by measuring the g-AchR antibody? Cheers John

Just a quick update on my scls,my first attack was 07,a major attack in 09,amputation and paralyzed.i happy to report no other attacks.Im on theophylline,so far so good.

Oh I am so sorry to hear you had such bad attacks saladboy but good to hear the theophylline is working. What dose do you take and do you just take this alone?

My dose is 100mg 4x a day.That is all I take.I have made a remarkable recovery,I am to live life the best I can,my bionic leg(lol) works great for me,my hands are 85% paralyzed,which is kinda a bummer.I just make them do what I want them to do.Positive attitude,prayer,and taking advantage of every minute of the 2nd chance at life is my best defense.During my 2nd attack,i cant tell you how many times they counted me out.Most doctors are amazed when they see me today.

You have a wonderful attitude:) I am so glad the treatment is working for you! So have you been on the Theophylline since 2009?

Yes that is correct.

Do you suffer any side effects from the drug?

JCarson, Re autoimmune autonomic ganglionopathy and measurement ofg-AchR antibody. I am not aware of being tested for this condition. I will raise the question during my Mayo Clinic work up, which is not scheduled until late December.. As an aside, I made it to North Florida, but not without a bit of adventure. I experienced edema and significant dehydration in a hotel room in Philadelphia and was transported to a teaching hospital for steroids and rehydration. After a few extra days of resting in the hotel room, I put my car and myself on the auto train to lessen the fatigue of travel.

Hi Susan, Aaah! The trials and tribulations of this whole thing. At least you made it to Florida! Roll on Dec for you!

Hi, everyone. I hope I am included in the numbers for Canada. I am in Owen Sound Ontario. My case sounds mild compared to most. Only 1 or 2 episodes a year that have always (for the last 30 years) resolved on their own within about 2 weeks. Just coming out of one now. I would love to hear from other Canadians about how they are doing. Mike1949

Hello Mike 1949, I hope you do not mind me asking you a few questions. I am curious about those with SCLS who are able to navigate an attack alone without hospital care. I myself have had two acute attacks and am now on monthly IVIGG. My first episode was made worse by my visit to the hospital. They treated me for dehydration by administering fluid which caused me to balloon rapidly, and I needed fasciotomies in all four extremities. The second attack though, I received no fluid and was able to leave the hospital after 5 days. My questions are : Do you monitor your hemoglobin levels when you are having an attack? Are you able to move about with the swelling? Is your swelling localized to one part of your body? My swelling always begins in my legs. During the second attack it remained only in my legs. Have you ever lost consciousness during an attack? I ask these questions only because I am concerned about being able to maintain IVIGG care for the rest of my life. I appreciate you taking the time to answer my questions. Cristina Burns