Does anyone have info on the cumulative effects of either chronic or episodic type SCLS ? Both anecdotal and statistical would be greatly appreciated. Thanks Carole T.

Besides the worst-case scenario of death from an episode or from the medical mismanagement of an episode of SCLS, I have witnessed or heard about -- and suffered from two of -- the following cumulative effects: permanent damage to muscles and nerves in all four limbs; chronic pain from said damage to nerves; permanent damage to vital organs such as the brain, heart and kidney; anxiety and depression; and sharp deterioration in personal and family quality of life.

What kind of permanent damage does it have on your internal organs? I can see the exterior damage to the skin being stretched constantly but do not know of the type of internal damage suffered. I also agree one's life's quality deteriorates due to not being able to join in those activities that you have always enjoyed such as sports, flying, getting hot, drinking alcohol etc.

Hi Windows. During a major attack, the heavy capillary leakage actually interrupts your blood flow. With no blood flowing to all of your major organs and muscles, they become damaged. If the capillary leak does not stop soon enough, the patient can go into multi-organ failure. I would guess that if they manage to bring you back, there is probably a lot of cellular damage...in addition to a lot of muscle and nerve damage. My husband's first recorded episode resulted in multiple organ failure and acute respiratory distress syndrome. He also experienced coma, encephalopathy, myocardial infarction, acute kidney failure, critical illness neuropathy/nerve damage, bilateral drop foot, and the low blood pressure knocked out enough nerves to cause partial blindness and deafness. The nerve damage and surgeries have crippled his hands and left him with giant skin grafts and scars on his arms and legs. The neuropathy also prevents him from standing up straight, and he is permanently dependent on crutches. It's kind of hard to imagine for many. When we are out, lots of people ask us what happened to him. They assume it's a car accident or something like that. They are always confused when we say he has a dysfunctional immune system.

In addition to Arturo's list I've had one bowel resection, and my abdomen has been opened up several times to prevent compression damage due to the leakage.

Thanks you guys. That's hard to read. But certainly not surprising. And it is very helpful - so, thank you. The more info, the better. Carole T.

Yes thank you guys too. I am so sorry to hear how bad it is for you. I am fortunate I guess in that I am a chronic leaker and my more acute attacks are nothing compared to yours. It is very hard to read, as Carole said. It is a foul disease

hello thank s for all alsso for me i have a hard problemes in my four limbs spcialy inmy right arm that i had thre operation just to reteren to us it in40% like was befor for the rest i git my ivig every month and every thing is ok just i must be carfuly to stell near any boudy ill thanks agine for all of you have a nice day god bless us yaser

Hello I to have had much of these issues. I have done so much research trying to find answers. I just contacted my doctor by email to refer me to NIH. I was diagnosed by Dr. Griepp in June 2011. I started IVIG in Aug 2011. There has been some improvement but I suffer in much pain with swelling in my hands that never has gone back to normal. It has become difficult to walk with the pain in the muscles and my skin feel as if will rip open at any time. My husband feels I may be loosing my hearing at times. There are times I have some ringing in them. As for depression who dose't have some when you have this ugly disease. My friends are shocked that this happened so quick and how my life has changed. I have gone on medical leave from work because I just can't function on a full day. It helps to read what everyone writes because it makes me feel that I am not the only one in this world suffering from this. I feel for each and everyone one of you who fight this ever day. I try to keep my faith and hope that some day research will help us. I pray for all!! Annamarie

I posted in another topic to parents with kids, but my son Connor needs Phyiscal therapy and sensory integration in an attempt to regain muscle strength and in an effort to minimize the nerve damage he has in his legs and core muscles.

In the last year of her life, my sister experienced extreme discomfort from what seemed like blistering in her mouth and throat, along with frequent nausea and diarrhea. It is worth noting, in fact, that her autopsy lists the cause of death not as the result of a severe SCLS episode but as intestinal ischemia, i.e., damage inflicted on her gastrointestinal tract by repeated / sustained interruptions of blood supply over the course of the five years she lived with SCLS.

I am so sorry to hear of your sister, Thomas. Was she a chronic leaker or did she have severe episodes? I am at present trying to get IVIG treatment and working my way through our system. Did your sister have IVIG or was her death prior to this treatment modality?

I am afraid I'm not sure about the distinction between chronic leakers and those with severe episodes. I think maybe Judy fell into both categories? But actually Arturo is probably better able than I to characterize her condition on the chronic - acute spectrum. Sadly, she died just weeks after a visit to NIH that she had hoped might allow her to participate in a study of IVIG's efficacy.

Thomas and all who responded to my inquiry, Thank you for this info. It is exactly what I need. And Thomas - you mention the documented diagnosis of ischemia vs. SCLS. This distinction is precisely what I'm looking for. I am so sorry about your sister. I'm curious about how long she had been ill and how long in to her illness did she start IVIG? I'd like to see stats on this. My hypothesis is obviously the sooner you're diagnosed and started on IVIg, the better the outcome or a return to previously enjoyed health. Thanks again people! Carole Thorn

Dear Carole, We know of at least one fairly serious episode some months before the very serious episode when she was diagnosed. She lived five years (nearly to the day) after her diagnosis. I should perhaps mention that we find the ischemia attribution absurd: she was hospitalized with a serious leak, her hematocrit was through the roof, and the blood flow to her brain was interrupted long enough that her eeg flatlined for a couple days before her heart finally stopped. Everything about the episode screamed SCLS. I'm no doctor, but I believe the post-mortem ischemia dx simply means that the damage was reaching the point where it would soon have killed her if the SCLS had not. Dr. Druey received tissue samples and a copy of her autopsy report. Judy's husband authorized the autopsy specifically because he knew that Judy would have wanted in passing to do anything possible to help surviving members of this community. Perhaps Dr. Druey could comment here on whether useful information came of that?

No doctor may make public comments on individual patients without breaking professional rules and even laws that shield patients' confidentiality -- even after they are deceased. Thom: I've let Dr. Druey know of the questions you have raised here. I suggest you contact him directly at _kdruey@niaid.nih.gov_, phone 301-435-8875.

Thom - I'm no doctor but I will venture a guess as to why "intestinal ischemia..." was documented as the cause of death. Specifically, the lack of blood flow to the intestine that developed as a result of the SCLS was the actual physiological cause of death. I can imagine however, that unless it was somewhere specified that the ischemia was directly caused by, for instance, shock related to SCLS verses a random blood clot that happened to an otherwise "healthy" person - it would seem to lack the impact that your dear sister would have hoped for. Surely this can't be the case. (?) If you do learn anything about their findings that you wish to share - please do. This is very interesting. Thank you for passing on this info. Carole T.

I used to be so horribly swollen. My only relief was compression massage by my daughter. Now, I am taking horse chestnut. My swelling is down. I started Food Grade Diatomaceous Earth. It is a natural diuretic and good for the veins. For the first time, in a long time, my hand can bend normally. The swelling used to feel like I had inflated gloves on my hands preventing me from making a full fist. Under the natural cure topic, no cure was given but we found that swimming especially in cold water, helps the swelling go down. Many people said that they take magnesium. Infrared saunas sort-of work. Ginkgo Biloba and Alpha-Lipoic Acid help nerve damage. Nerve damage can be caused by antibiotics. Garlic milk is a natural remedies for Nerve Pain. Cook two crushed raw garlic cloves in about half a cup of milk and drink this milk to reduce Nerve Pain effectively over a period of time. Celery and carrot juice (mostly celery) is another home remedy for nerve pain. Not cures, but people say it lessens the pain. Capsicum improves the capillaries and lessen pain. I have not (myself) tried the capsium or magnesium, but I plan to try both of them soon.

I fall into the category of severe leaks. I've only had three which hospitalized me in 3/05, 1/07 and 2/08. The second one, my kidneys shut down, but now function normally. The last one I had compartment syndrome in both legs, and had double fasciotomy on each of them. Have not had an attack since 2/08, but am on disability because of nerve and muscle damage, as well as fatigue. No way could I work a full day,and I also had to give up a lot of activities like athletic things and flying. For the past several weeks I've had severe fatigue, I also seem struggle for enough air. My sclc doctor has sent me to all kinds of specialists, yet they can't find anything. One of my lab results showed a higher than normal level of protien in my blood, which sends up the myloma antanae, yet they don't think Ihave myloma. I go back to the Hemo/oncologist in January for another blood and urine test. Hopefully it will be normal. I do think these attacks have each taken a toll on my organs and muscles

I have been somewhat remiss in my participation in our community, but here is my personal experience with long-term or cumulative effects of repeated leaks. While I believe I started having leaks prior to July 6, 2010, this was the first and most severe episode that put me in the ICU with compartment syndrome in both legs, failing kidneys and heart irregularities. I received a fasciotomy in my right leg, went through several months of physical therapy and have sustained permanent nerve damage in both feet, although not so severe as to impose major limitations on my mobility. All my other symptoms from that experience resolved once the episode was over. Between that first episode and my final episode, I had 5 or 6 hospital stays, one of which included a return to the ICU, and numerous minor episodes that I managed at home with lots of hydration. Toward the end of my last hospital stay in August, I was having episodes every 4-5 days, a permanent cycle of hemoconcentration that would resolve and then start all over again despite taking Theophylline and Terbutaline. Almost thirteen months to the day -- August 5 and 6 of 2011, I received my first IVIG treatment thanks to Arturo's generous kindness and have not experienced any leaks since then. In fact, I feel better than I ever have...but there is one health issue that has come up that may be related in some way, although this is purely speculative on my part and in no way scientific. I have developed dupuytren's contracture in both hands (I never had much of any swelling of my arms or hands during episodes). It is generally thought to be an inherited condition, but it can be worsened by hypoxia and chronic ischemia (http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1743313/pdf/v081p00425.pdf). This discussion of ischemia's involvement in others who share this disease has strengthened my suspicion that my SCLS may have caused the contracture to worsen as a result of the repeated episodes. Otherwise, the good news is that with IVIG treatment, my life has returned to normal and other than the few lingering issues I've mentioned, I am healthy again.

I have been on IVIG for one year and today will add another month. Before IVIG I was having attacks that required hospitalization every three month. I had a port installed as it was taking over an hour to start a central line. I had two attack that almost resulted in death before I was diagnosed. The good news is my employer is great about my condidtion. I started working full time and work form the infusion center two days a month. It helps me keep a good aditude. I have fun with my grandkids,camp, bike ride (three wheel recumbent with 18 speeds) and travel with the students at the college I work for. I am taking classes toward my MBA and hope to graduate in May of 2013. I am slower than before and studying is harder, however doing well. I guess I should count my blessing, I do a lot, just a lot slower than before, but I am fine with that. Rita