Hi all, My brother ( Allen overland) had been diagnosed with scls over 10 years ago. He had visited the mayo clinic in Rochester,mn and dr greipp and dr haymons are who diagnosed him and gave him the recommendation to use theophylline to prevent his leaks. This was prior to the use of ivig and at the time they told us there were only about 10'people living that had been diagnosed with scls. The prognosis was not good but the advice was to live each day to the fullest which he certainly did because eventually he would have another episode. A few years ago we had the pleasure of being acquainted with arturo who is the scls community expert and also dr Druey from NIH. My brother lived in the DC area. Fortunately he only had a few minor leaks but they were always there to provide support and knowledge to me and his medical team during these episodes including the last one that claimed his life. This last episode which began 1/14/15 and ended with him passing on 1/23/15 was the only other major episode he had. Unlike the first episode to my knowledge every guideline was followed and they gave ivig and consulted the experts giving judicious fluids, ivig, prednisone,etc. He had fasciotomies on 4 limbs and was on a vent for 7 days. His leak and recruitment phases were not as clearcut mainly because he also developed a severe bleeding problem which we believe caused strokes and significant brain damage. Dr Druey said the bleeding could happen with scls lile with ebola but I don't remember reading much about it so if anyone has experienced this please write in. Also they finally gave something called factor 7 which stopped the bleeding so that is available although his team did not find out about it until the bleeding persisted and another expert was contacted. Perhaps if they had given that sooner we would have had a different outcome? The episode also seemed to last longer than it should have with the leak phase persisting longer than the 48 to 72 hrs. I wanted to share this with the community and to let everyone know that dr Druey and arturo were there and followed this episode so that it may be helpful to others in addition to the obvious reason of so generously giving their time and expertise in an effort of trying to save his life. I am eternally grateful to both of them. Also I wanted to tell anyone that is not on ivig that u may want to seriously reconsider. Arturo said it has a 98 percent success rate which I knew and had been trying to get my brother to have it for a couple years now.The theophylline was working in preventing big leaks but recently he had more frequent minor leaks. He had also had an increase of side effects with theoplhylline. He had an appointment on 1/26/15 with dr Druey and I believe he was going to ask to be put on ivig however he never made that appointment. I am in mourning at this time and devastated at losing my brother. I am still in shock that the protocol was followed but with no success. I don't feel like doing anything because I am exhausted from this last episode as some of u know it takes a front row seat in your life for days with no sleep,etc.. However I feel compelled to immediately encourage any of u with scls that are not on ivig to seriously reconsider and to act quickly!!! It is a matter of life and death. Initially we thought theophylline was doing ok and ivig seemed like a hassle when he was doing so well. Maybe he was different as so many people are??? There are some that do not take anything and r still alive so at least he was doing what mayo clinic recommended and when he asked his primary care they thought the theophylline as fine or at least that is what I was told. It seemed that his minor leaks were under control using the correct protocol sonwaht was the rush in changing? Some of u do not have a choice because your episodes r frequent and all life and limb threatening so it is an easy choice. For the others like my brother please call your doctor today and ask to be put on ivig as soon as possible. You are playing a game of russion roulette with your life. I know there are side effects, costs( although most insurance covers it now) and inconvenience and maybe some cannot have it for what ever reason. But if it has been offered as a choice please take it. In closing I am so very grateful to arturo, dr Druey and the members of this community that have shared their experience. I am hoping that my brother passing will somehow help others. Dr Druey has first hand knowledge of the autopsy and as I said was there taking samples during this last leak and we will see to it that arturo and he have a copy of the medical record to use for research after all even if ivig works there are still others that will have to go thru an episode to be diagnosed and we need to know why the protocol did not work this time. God bless u all and be well and I hope my brother's is the last name to be added to the list of those lost to this disease. Warm regards, Linda

Dear Linda, I address my deepest sympathies to you and your family. I fully agree with your comment about the usefulness of IVIG. I recommend all Patients with SCLS to discuss with their doctor the best prophylactic treatment adapted to each case. In my opinion, if there is a doubt about treatment, the precautionary principle would lead to use IVIG. In my case, the treatment with IVIG clearly kept me alive until now. Claude Pfefferlé

Dear Linda, Like Claude I sent you my condoleances. I'm sorry for you and your family to hear about the loss of your brother Allen. like Allen I'm using theofylline, besides terbutaline (and Glivec, but no IVIG) thanks for telling us the story about Allen, and your emotional call for treatment. I would like to know if your brother was using terbutaline in combimation with the theofylline? Hiltjo

Linda, So very sorry to hear about the loss of your brother Allen. I too was on a therapy regimen of Terbutaline and Theophylline and had another severe attack. I had the good fortune and guidance from my specialist, Arturo and Dr. Druey to recommend that I switch to IVIG. Thankfully my insurance authorized the treatments. Fast forward two years. No episodes of any leaks. For those still pondering the treatments, my advice is don't delay, get it approved from your insurance and begin getting the treatments. The inconvenience is minor. It saves lives. How can that be inconvenient?

Thank you for the condolences and more importantly weighing in on the topic of ivig/ theoplhylline. Hiltjo, My brother only took the theophylline and did not take the terbutaline. It was recommended to take when he was first diagnosed but he had some trouble with tolerance and he only took it for a short time 10 years ago. Interestingly when all else was failing during this final episode they did give him some terbutaline along with the theophylline that he usually took but it did not work. I hope u will think about what I and the other ivig users have written and consider ivig if u have not already done so. God bless and be well! Linda

This obituary was published in The Washington Blade on February 10, 2015: *Guy Allen Overland dies at 54* Guy Allen Overland, a librarian who managed libraries and resource centers in Washington for the National Endowment for Democracy and the U.S. Export-Import Bank for more than 20 years, died Jan. 23 at Virginia Hospital Center in Arlington. He was 54. Jim Houser, his partner of 16 years, said the cause of death was complications stemming from Systemic Capillary Leak Syndrome, a rare disease marked by severe body swelling and organ failure. Overland most recently served as Supervisory Librarian for the Research Library and Archives of the Export-Import Bank, where he worked from October 2013 until early January of this year, when he was hospitalized for his illness. He served for 19 years, from 1994 to October 2013, as director of the National Endowment for Democracy’s Democracy Resource Center. Houser said Overland helped start the resource center and oversaw its growth from a traditional library to a technologically advanced resource facility. Houser said the National Endowment for Democracy plans to add Overland’s name to the official name of its Democracy Resource Center in recognition of his distinguished tenure and service to the center. An aide to the organization’s president, Carl Gershman, confirmed plans for the name change. Prior to joining the National Endowment for Democracy, Overland worked as a law librarian for the law firm Fulbright and Jaworski and, prior to that position, served as a program officer for the United Nations Institute for Training and Research. “He was active in library and international organizations in Washington, D.C., and was the originator and coordinator for the Washington International Library Group,” Houser said in a statement. “He overcame obstacles with the illness he had,” said longtime friend Patrick Kalk. “He was just a very positive person, always in a good mood, hardworking, intelligent, a good sense of humor — all of those good things.” Overland received a master’s degree from Catholic University in Library and Information Science and a separate master’s degree from American University in International Affairs. He received his bachelor’s degree in history and political science from Florida State University, where he was president of Phi Gamma Delta Fraternity. According to Houser, Overland was born in Bellefonte, Pa. He is survived by his mother, Cynthia Overland, of Fairfax, Va.; his three siblings, John Overland, Laura Maun Garcia and Linda Stewart, all of the Tampa Bay, Fla., area; and his partner Jim Houser of Arlington, Va. A celebration of his life is scheduled for 12:45 p.m. Sunday, Feb. 22, at Foundry United Methodist Church, 1500 16th St., N.W., Washington, D.C. A reception is scheduled to follow the service at the church’s Fellowship Hall. In lieu of flowers, loved ones request that a donation be made in his memory to either the National Organization for Rare Disorders, which funds research and treatment of patients with rare diseases, or Doorways for Women and Families, which provides services for people encountering domestic violence and homelessness.

Linda, My condolences as well. Thank you for sharing and for your encouragement. IVIG has been my course of treatment since July of 2011 thanks to Arturo, D, Druey and Dr. Greipp. Regards, Marc

Linda, I send my sincere condolences to you and your family.

Marc and josephite, Thank you for your kind words. Hoping all is well with both of u and the rest of the rareshare family! Warm regards, Linda

I'm so sorry, Linda. I hope you are doing well, finding comfort, and that the love in your heart for your brother is helping you to heal. peace, Cathy

Thank you Cathy! I hope u continue to do well! Be well Linda

I am sorry for your loss

Thank you ritz . I hope ur ivig is working better for u now!! Be well!!!

I am so deeply sorry for your loss. This bleeding thing really concerns me. I know of others on this site with anemia issues which I also suffer from. The doctors were looking for an internal bleed because we can't stabilize my hemoglobin level or my iron level. I had a colonoscopy and an endoscope looking for a bleed because my hemoglobin was 6 a month ago and it was around the same in January. I have been transfused 3 times this year and the doctors are unable to find a bleed. They have given up looking for a bleed. Were the doctors able to tell where the bleeding was coming from? I will ask about Factor 7 at my next appointment. I am receiving IVIG infusions once every three weeks. Thank you for your heartfelt advice. Once again I am so sorry for your loss

In regard to the bleeding that occurred during my brother's final episode this was the only time that had happened. He did not have any problems with anemia or low hemoglobin when not in an episode. He was not a chronic leaker and only had a couple major episodes but mostly lived symptom free.From what I understand when the capillaries start to leak in someone with scls it is possibly because the body thinks it has been exposed to some sort of toxin. Just like in Ebola or poisoning the body tries to get rid of the " toxin" by leaking it out . Many people report flulike symptoms and so they may possibly be exposed to something that causes the immune system to go " haywire" and cause this excessive leak. Most likely scls is a defect with the autoimmune system and eventually the body self corrects if u can live thru the episode. My brothers bleeding started after he had leaked all the other fluid out approximately 2 to 3 days into the episode. The bleeding was from the capillaries and was extreme. The physician had to hand pump blood in as fast as it was leaking out for a few hours and he was given alot of blood. We think they could not replace the blood fast enough and that it when he suffered the strokes leaving him brain damaged. Because I did not remember this type of bleeding during an episode I asked dr. Druey and that is when he mentioned that it was similar to exposure to Ebola. The factor 7 was given because of the amount of blood that was being lost and I don't know if it is given except in emergency type situations. I also did not know that others had problems with chronic anemia so it is good u wrote. I wonder if u r a chronic leaker if that could contribute to ur anemia although from what I know it is usually the other fluids in the blood that initially leak out.. The red blood cells with the hemoglobin are larger and are generally left in the capillaries causing hemoconcentration during an episode which is the opposite of anemia. This also puts u at risk for stroke because of the thickness of the viscosity of the blood which is why judicious fluids are given. I hope I have helped answer some questions. I am battling back from aml leukemia and a stem cell transplant and have chronic anemia so I know how tired u feel! Thank you for your condolences and best of luck. If anyone else has more info please share as I don't remember anemia or excessive bleeding during an episode being discussed and it may help others. As I said had they known about and given the factor 7 sooner perhaps he could have been saved. Thank you again and be well!! Linda