My name is Marilyn. I am a 44 year old mother of 7 children(ages 10-25) My first SCLS episode occurred Jan.9th,2008. It began with symptoms like the beginnings of a head cold, skin crawling, etc. I upped my zinc, and airborne for two days. On the third day, nausea began, so i figured i had caught a mild stomach virus on top of the cold. That night, i awoke nauseated with muscle weakness, fatigue. I had been diagnosed with high blood pressure, and all i could think of was to check my bp. In the process, i collapsed and began throwing up all over myself. I checked my bp which was very low like 80 over 40. I didn't want to wake my husband, but when i saw these numbers i knew i was in trouble. Even so, i took some promethazine, and laid back down. Sat morning i continued to get worse. I began to become confused and sicker. If i tried to sit up, i would begin vomiting. i had to stay horizontal. My husband rushed me to er. They actually left me waiting for three hours, before getting me a room. I was in ER for thirteen hours before being admitted to the floor and then immediately to ICU. No one had a clue what was going on, i had 7 different doctors trying to figure out what i had. In the meantime, i was pushed an enormous amount of fluids(i gained 60 pds.) and dopamine. This was the worst episode out of the total four i have had. (i was in ICU two weeks, one week on the floor, and the last in physical rehab-due to severe neuropathy). The second was Thanksgiving08,(to which i was being treated with sterioids for inflammation in my neck). Looking back i realized i had thwarted off an episode. My belly had swollen up, i had muscle weakness, and bp was on the low side. The third was Dec. 29th,08. This time my husband called for an ambulance, again low bp, dehydration, had initial cold-like symptoms, and so forth. It was then that we realized the first time was not some rare infection but a syndrome of sorts. My sister is a physician and she remembered reading of a syndrome similar to this. She along with her husband(critical care pulmonoligist), and my husband had me moved to St. Luke's in Houston, TX. There i stayed only two weeks but with a 40lb weight gain. They didn't use dopamine, but some sort of vaso-restricter instead. Less than two weeks after being released, i went to the mayo clinic and was seen by Dr. Greippe. I began the terabutaline and theophylline as soon as i returned home. Before i reached the theoph. therapeutic level, i had a fourth episode in Feb09. I gained only 20lbs., was treated with albumin and steroids, and left 5 days later. Since that time, i have stopped the terabutaline, but continued the theoph. When i reached the level during my recovery, i never seemed to bounce back. and this was the least traumatic of my hospitalizations. My original weight was 120-122 lbs. i now fluctuate daily from 125-131. i feel sluggish, bloated, and depressed every day. despite the fact i am on cymbalta I originally was on welbutrin, but switched prior to fourth episode because of the theoph. interraction. My belly is still bloated. The other three times it went down. I even had my OBGYN do a sonogram to check for ascites,(spell check that one). It came back negative. My husband keeps insisting if i exercised i would get better, but the problem is i don't have much energy. Occasionally, i will have a good day, maybe once or twice a month and i am up and moving with all the energy i had before. Those are the days that help me to remember what "normal" is. My question is do you think i am struggling with the SCLS on a daily basis and the theophylline is suppressing it? Or, the theophylline in the therapeutic zone ,is actually making me this way? Any suggestions would be a lifesaver since it has been 4 months of this, and i feel like i am no longer living. It's unfair to my children and my husband. i live with guilt for not being a participant, but rather a spectator. And yet, there is no magic pill for this. i do pray, though. And this has definitely drawn my family and my husband and i closer. I write down plans for the future(things i want to do again, or plans with my family)-- when i will be well. This is something positive i do when the present gets to be too depressing. It has helped. sincerely, marilyn

Marilyn-I can really relate to what you wrote. I am headed out to a birthday celebration and will write more later. Please feel free to email me. judithdavis3@hotmail.com. I would love to talk to you. Judy

Hi Marilyn, My name is Kim and I am 42, just diagnosed last year. I went to see Dr. Greipp in May, basically to ask what I coud do to cope daily, and to get permission to exercise. I climb one flight of stairs and have to rest because it wipes me out - I feel like my 5'2" frame is carrying 300 pounds - and I weigh 145. I used to work in an office 5 days a week, then on my feet all weekend and evernings - very physical. Dr. Greipp said everything looked fine in my tests, but he would put me in touch with a cardiologist there at Mayo, just to be sure. After 2 stress tests, a cardiac catheterization & biopsy, and an MRI, it was discovered (with another, more obscure, test on the biopsy) that I have protein deposition in my heart muscle, and this causes my heart not to relax as it should to fill up with blood. I believe this is on the molecular level (?), and this was something the other tests would not have shown. I am on an ACE inhibitor for 6-8 weeks to see if this works to 'loosen up' the muscle (with exercise, too). If that doesn't work, we are going to try another, stronger drug. I, too, have no energy, wear out quickly, have a good day (or morning) once in a while, but am at about 1/5 of the level of energy that I used to have - you may want to get checked out for this issue. We weren't sure if one may have caused the other - as you know, it is all pretty mysterious.

Marilyn, I have had four episodes of SCLS in the last 20 years and was not diagnosed until July of 2008. My episodes were similar to yours, low bp, disoriented, nausea,etc. Each episode was associated with some type of cold or flu like symptoms. Each episode required hospital stays of 7-14 days including time in the ICU. Fortunately, after large infusions of fluids my vital signs stabilized and I eventually got better. I gained weight due to large amounts of fluids, I was then given diuretics any my weight slowly returned to normal. It took several weeks to regain most of my strength. After my first episode I started exercising (easily) and have been exercising regularly ever since. The doctors (including Dr. Greipp) all feel that my physical condition have allowed me to recuperate from these episodes with very little long term side affects. I continue to now aggressively exercise 3-5 times per week. Ask Dr. Greipp about me (I am his cyclist patient). I would encourage you to begin an exercise plan, very modestly at first. Please don't over exert yourself. If you can do a few minutes at first, great. You must be aware of how you feel and when you can go on or stop. Eventually, you may be able to regain some of your strength. I am not a doctor, but only can speak from my personal experiences. Good luck. It does get depressing at times, but we must press on. Robert

marilyn mother of 7 just makes me tired thinking about it!! my brother has SCLS and has had one episode in the last 5 yrs. the doctors said one of the reasons he survived his attack is because he was ( and still is ) in great physical shape. he has always worked out and now continues to do so even though he does get tired easier. he travels extensively for work and play and just does not seem to let it get him down. he probably does just as much now if not more than he did before his diagnosis. we are both in our forties and he just wears us all out at times:) ( he has no kids so that may be the difference ) but i just want to cheer you on and encourage you to keep active as this seems to have helped my brother. i know how hard it is and i hope you do well. it is good to have others to help you through the tough times.. take care linda

kmiclon, Your protein deposition sounds to me like a decease called Amylodosis. Check it out on this website. I have a friend suffering from it right now and his symptoms sound like yours. The walls of his heart are thickening to the point that his heart can not beat strong. There are some similarities between SCLS and Amylodosis and they can be mistaken for each other. It is worth a try. These are the drugs they are trying on him as of today.Cytoxan, Lenalidomide,( trade name Revlimid ) and Dexamethasome which is a corticosteroid. The Lenalidomide is a form of the old drug Thelidomide which caused all those birth defects several years ago. My husband was on it for three weeks and he was like a walking dead man. Could not tolerate the drug. He found out that reaction is very common but it is the only drug his doctor knows of that will stop the production of the protein. Good luck.

Good questions and suggestions from everyone. I have a few comments: First, exercise is great for some but not for all - you just have to try and see. Second theophylline and terbutaline can sure drag some folks down - it is a balance for many - between side effects like fatigue and benefit - fewer less severe attacks for many also. Third Kim's experience with the heart problem is probably not unique and may be related to the capillary leak - fortunately she does not have amyloidosis. I am happy to talk to any of you and do my best to answer questions. Dr Kirk Druey and I both appreciate all this group does. I think we can lick this with a bit more knowledge and understanding. Dr Phil Greipp.

I am having cardiac issues as well. I have had transient congestive heart failure a few times during recruitment phase the past few years and have been diagnosed with pulmonary hypertension March 2008 (maybe had PH 3/2007) probably secondary to sleep apnea. My exercise tolerance has gradually been worsening since I was diagnosed 5 years ago. Can you explain the protein deposits in the heart muscle related to SCLS? Could this be effecting my heart?

Please send me an email Judith Phil

This is so exciting to have new info all the time!! This site has been a tremendous source of encouragement, and a huge thanks to everyone for taking the time & energy to get this information out. I have been on lisinopril (80 mg daily) for 3 weeks, hoping it would 'soften' my heart, but doesn't appear to be working. We are going to try rituximab, hopefully starting next week, for 4 weekly treatments. This is the recommendation of Drs. Lapeyre & Cooper at Mayo in Minnesota. Apparently there isn't much known yet about this heart issue, and I myself am a bit unclear on the specifics, but Dr. Lapeyre explained it in simpler terms. (hope I get this right) - My immune system is fighting the cells of my heart (as in an organ transplant) and hopefully this rituximab will stop that process and modulate my immune system. And no one is sure how or if this is related to scls. I will keep updating here how things are going.

Thanks. Rituximab is something Dr Druey is considering maybe to try for SCLS so I find this very interesting. Please update how the rituximab works for you-cardiac and SCLS.

RItuximab is a monoclonal antibody prepared from a plasma cell line developed to make the antibody. It is commerically available and used to treat B-cell lymphoma, chronic lymphocytic leukemia and various 'autoimmune' disorders. Ironically it is helpful only in a minority of patients with myeloma - the blood cancer associated with a monoclonal protein - like the one in SCLS. Does this help? Dr Phil Greipp

To patient who calls himself scls, I am the wife of a Clarkson's patient and we live in north Alabama. My husband sees Dr. Nancy Brown at Vanderbilt University Hospital in Nashville, Tennessee and we thought that might be closer for you to go see a doctor. Remember that you will be making multiple trips, not just one. Dr. Brown took our case over from Dr. John Oats. He was an intern working under Dr. Clarkson when Dr. Clarkson diagnosed his first case of SCLS. Dr. Oats was the thirteenth doctor we had seen and he was the only one who knew what my husband had. He does not see patients anymore. He confines himself to the research lab. Dr.Nancy Brown has been in touch with Dr. Kirk Druey at NIH and they are collaborating on my husbands case. I am not trying to dictate who you see and seeing Dr. Greipp is probably a good idea. I just wanted you to know that there is another option and it may be closer to home. Good luck with whatever you choose. (My husbands profile name on this site is dannycfi in case you want to look up his "about you" section.

My four year old was just diagnosed (December 22, 2009) with this syndrome. Looking back, we realized his first attack came in March 2009 when diagnosed with the flu. Because of his dehydration, they pumped him full of IV's. His protein levels dropped and then we referred to Connecticut Children's for kidney and GI folow-ups. He had recovered from the attack, and left with no answers or reason for what happened. This attack (December 18, 2009) began with a runny nose and upper leg pain. Then began the vomitting and severe drop in blood pressure. He was transported to Connecticut Children's with a blood pressure of 67/32. Again, to raise his blood pressure he was pumped full of fluid only to have his proteins drop to 2.1. After numerous GI work ups, we came across this syndrome. Our Dr. at children's email Dr. Greipp and thank goodness he reads emails over vacation and helped confrim the diagnosis. Now we are looking on ways to "prevent" future attacks and ways to help him lead a life like any other four year old.

I am a 43 year old female who was diagnosed with adult onset asthma and Raynaud's Syndrome 12 years ago. I have been taking 400 mg of Theophylline 2x per day for the asthma since that time. During the last three years, I have been diagnosed with pneumonia 3 times. I was hospitalized after ER visit for abdominal pain to be told that I had fluid in my lungs ... but nothing was wrong with my appendix, gallbladder, etc. I was told the pain was from the pleuresy. My health seems to be rapidly declining for no known reason during the last year and a half ... last year, family friend/physician was afraid I was experiencing congestive heart failure because of the swelling in my ankles/lower legs and my inability to get a full breath ... cardiac stress test revealed no abnormalities. My SED rate was sky high and my hormone levels were weird but nothing else was out of the ordinary. My ob-gyn took me off birth control. After one month of misery, my condition improved dramatically after the change in my birth control. When my health normalized, I was fitted with a Mirena IUD for birth control with no negative side effects. However, I now seem to be in a 3 month cycle where I am feeling normal and then start to feel as if I have an upper respiratory,my ankles are swollen, my skin feels tingly on my upper arms, my chest is tight and I can't get a full breath,my head pounds, I feel confused and my vision is sometimes wonky. I often wake feeling like I am drowning and am actually gasping. I try to sleep in a half-sitting position. Last week, a Raynaud's attack was especially severe and my hands turned purple from the wrist to the tips of the fingers for FOUR HOURS. After circulation returned, the pain was intense and my hands were swollen for two days. A rheumatologist now has me on a calcium channel blocker to prevent the vaso-constriction. I have been on a medical mystery tour visiting every specialist in my town and a local neurologist mentioned that he had heard of this disorder and is going to investigate it as it relates to me. However, after reading this forum, I am not so certain that this idea is applicable ... I have not had any "severe" attacks as described in most of these posts. I am wondering if it is possible that I am having mini-attacks and that my long-term usage of Theophylline has mitigated the symptoms of an active episode??? Did any of you start off on a "slow" ramp up in severity of these attacks? Also, my Raynaud's seems to be contraindicative (capillary constriction) to the presence of a leaky capillary .... my doc theorized that my body is somehow compensating for the drop in blood pressure by denying blood flow to my extremeties. Do any of you have Raynaud's too? I am now on a variety of medications for my breathing issues but have long noted that the nebulizer/inhaler components always seemed like a waste ... I have never obtained any relief from these meds. In addition to the Theophylline, I am currently taking morning dosages of zyrtek, advair 500, omnaris, restasis (dry eyes ... my tear ducts are plugged to treat this ailment as well), bupoprion (generic for wellbutrin) for generalized depression, nifedipine (Raynauds) and the occassional xanax. At night, I take another Theophylline as well as a 10 mg singulair and another puff of advair 500. As you can imagine, I often need to take an ambien CR to sleep given the stimulants swirling in my system. I am desperate for a direction and believe that I need to be proactive in my research and treatment. Thank you in advance for reading this lenghty text and for any ideas that you may be able to offer. Sincerely, Sabrina

Dear Sabrina: Welcome to this SCLS Community -- and I say that even though I hope you don't suffer from SCLS, because you seem to have enough other health problems already. As to one of your questions, yes, it is within the realm of the possible that you are having mini (so-called sub-clinical) episodes of SCLS which have been mitigated by your long-term use of Theophylline. And yes, many of us have "progressed" from having occasional episodes to having frequent episodes of SCLS. My recommendation is that, if you can afford it, you should go to a top-ranked medical complex like the Mayo or Cleveland Clinic, where they are used to patients flying in from all over the world for several days of top-to-bottom evaluation -- because their home-town specialists, like yours, have been unable to see the whole picture and thus they have been unable to put "two and two together." At the Mayo Clinic, in particular, there resides the top authority on SCLS, Dr. Philip Greipp, and he can then schedule you to see all the other MDs that are required to come up with a comprehensive assesssment of your health situation. It's probably the best investment you can make.

Dear Arturo, Thank you for your reply. It is probably no coincidence that my husband and I were discussing a potential visit to Mayo Clinic even before I stumbled upon the possiblity of SCLS. We have decided to await the results of the latest round of tests to evaluate what, if any, new information is obtained. This data should be received within the next two to three weeks. Thereafter, we will most likely be scheduling a trip to Mayo. Thanks again, Sabrina

sabrina, I know you said you are probably going to the mayo clinic and i agree with arturo/s advice.. My brother has SCLC and like most went undiagnosed for 8 months after he nearly died when his first attack took place 6 years ago at age 43. He was extremely healthy up till that time and with theophylline has only had one other episode (when he tried to reduce his dosage). I was with him at the mayo clinic and i can not say enough good about dr. greipp, dr haymons etc.. As a health care provider i can tell you they handle diagnosing difficult cases very efficiently and leave no stone unturned. One doctor oversees all of the testing and reviews what the other specialists find. I told my brother kiddingly that It reminded me of a very organized scavenger hunt.. You just plan on being there for a couple days to a week.. The first doctor you see orders tests and for you to see specialists (all done at mayo based on his suspicion). They give you a list and you go from station to station ( ie neuro, blood,xray) and get all these things checked off. Then they send all the results back to the first doctor and the doctors consult and by the time you leave you have your diagnosis (hopefully). One stop shopping and they are very organized and thorough. Most everyone they see has a complicated or difficult case so that is what they do best. They doctors take a lot of time listerning and explaining and do not seem to run on such tight schedules as many doctors on the "outside" do. They are research based and appear to be really dedicated to the medical profession and the good of mankind. I hope you do not have SCLS but understand your frustration in just wanting answers and a plan..Good luck with it all and press on till you get your answers. linda