From Ruth Nolan in Christchurch, New Zealand: My major systemic capillary leak (scl) episode happened in October 2005. This resulted in bilateral lower limb compartment syndrome and similar compartment syndrome in both my arms. As a result of this scl episode I ended up in the intensive care unit (icu) at Christchurch Hospital and was placed in an induced coma for 9 days. During my stay in icu I underwent fasciotomies on both my right and left legs (3 on each leg) and fasciotomies on both arms; along with this I underwent serial debridements of necrotic muscle tissue. The icu doctors believe I was one hour short of death (through heart or kidney failure) when the leak stopped. So I am very much blessed to be writing this! The result of the fasciotomies and debridements meant that I had 18 months of being in and out of hospital (in the plastic surgery unit) having my fasciotomies closed through plastic surgery … along with numerous infections and operations to remove more necrotic muscle tissue. The result of all this was that I was left with foot drop and was unable to walk. The next set of operations involved bilateral posteromedial releases and lengthening of my achilles tendon to turn my feet back around to a straight and flat position. These were partially successful but over a 12-month period my feet began to turn back inwards and rotate; and this meant I was putting great pressure on my little toes. The result of this was the amputation of my 5th left toe and then the amputation of my 5th right toe. Following these amputations I underwent bilateral cavovarus foot reconstructions – my right foot was reconstructed December 2008 and in January 2009 my left foot was reonstructed. So my feet are almost straight and flat and I’m back walking again; still suffering a lot of pain and my ongoing battle with oedema continues. Has anyone else out their “walked” a similar path as a result of their scl?

Dear Ruth, I am so sorry to hear about your capillary leak episode and your struggle to walk again. I had an episode very similar to yours in Nov. 03 and have bi-lateral foot drop though fortunately I didn't have any infections. I wear "Walk On" splint made by Otto Boch, a German company and I find they help a lot and of course I go to phyical therapy for gait training. I'm glad to hear you're walking again...don't give up! I'll tell you I've have hiked part of the way down the Grand Canyon and can even jog 50- 100 ft or though its not pretty to see me try so there is hope over time... And good luck managing your Capilary Leak. I have been symptom free for 5+ year. So keep your spirits up, I belive that positive thinking is key. Best, Allen

Dear Ruth: Welcome to this SCLS virtual community, and I am terribly sorry to hear about all the problems you've had with your legs and feet in the wake of an episode of SCLS that led to compartment syndrome. In case your misery loves company, I should tell you that in my first episode in November 2005, the muscles and nerves in my arms were damaged, such that I am weak and my hands have trouble grasping and doing fine manipulation (like buttoning buttons and tying shoelaces). In addition, the muscles and nerves in my lower legs were completely destroyed, such that I have no feelings or movement below both of my knees. Luckily, my lower-leg and foot circulation are good and my feet fused naturally with my legs at a 90-degree angle, such that I can walk quite well without a foot drop problem with the help of compression stockings and braces that go from below my feet to below my knees (see http://www.trulife.com/product.html?product_id=729) to give me flexibility and a natural gait. I also use a cane, because I cannot balance and stand still without leaning on something. To get to this stage, and including my second life- and limb-threatening episode (April 2007), I have had to survive more than 20 operations requiring general anesthesia, including fasciotomies, muscle debridements, skin transplants and other procedures. And yet, I find comfort in the realization that I could have had a much worse outcome, including limb amputation or death. As a matter of fact, I just heard that another SCLS patient here in the United States has had a leg amputated, so I thank my family, doctors and God for the relatively good quality of life I enjoy. Please let us know if you are having and how your are coping with other episodes of SCLS, and what medications and procedures seem to be working for you.

Thanks to those of you who have replied, it is comforting to know others out there can understand what it is like to live with this syndrome. While I have not had any recurring attacks I believe that I have probably had 2 previous attacks however for what ever reason they righted themselves and I was in the dark to explain what was wrong with me other than very low blood pressure. How the doctors in ICU, who I am ever so grateful to for saving my life, discover what was wrong with me was through the MGUS protein I have and was aware of. My father died from bone cancer and had the same protein in his system. Am I correct in saying that all of the cases of SCLS have this protein present? As for medication I am not on any thing other than pain relief however have an arrangement here with the hospital that I am to ring Haematology and be admitted as soon as if another attack occurs. This is why I am so grateful to this site as I am due to see my haematologist next month for a check up and will be able to share the information gained from this site. Ruth

Ruth Amazingly I was to be in New Zealand this week for a conference in Wellington. I have been to NZ 4 times now. Unfortunately I got the flu and have had to stay home. We visited Christ Church while there - It was a memorable visit. Your doctors might want to consider using theophylline and terbutaline to reduce the frequency and severity of attacks.Our last publication was in 1999 and another review of 25 bona fide cases is being submitted to the literature. T&T helpful in prevention not during the acute attack. It is important that therapeutic blood levels of theophylline be measured by blood tests. I would be glad to talk with your doctors. New reports on Intravenous Immunoglobulin show that might be helpful during the attack and as prevention but it is hard to say it works for sure with few cases reported. Numerous other treatments have been offered. Steroids are used in the acute attacks and by some to head off an attack. You always need to weight the pros and cons of any treatment with your doctors. Yes most patients with SCLS do have a monoclonal gammopathy. Stay tuned with the website - patients and others participating are sharing a lot of information. Thanks to Arturo we have another weapon against SCLS - knowledge sharing is key to better outcomes. Dr. Phil Greipp

Thanks so much DR. Phil Greipp I will pass on your information to my doctors at my next appointment which is April 30th 09. I am in good health at present, recovering from foot reconstruction. I Have finally got the pain under control ( to some degree) and heading to the pool for a physio session this afternoon. I am sorry you could not make your trip to New Zealand and if you find yourself back here especially in Christchurch itself we would love to meet and talk with you. I am ever so grateful to this site as this has been an interesting journey and at time a lonely journey. Being able to find out more is a powerful experience. The doctors here I am sure will be very interested in your expertise. I also have an amazing supportive husband who did a Zoology degree many years ago but should have been a doctor. He has been my strength at times when I have been too exhausted to understand what is happening to me. His interest and ability to comprehend medical terms has helped to explain to me at a later date what the doctors have said. Thanks again. Ruth Nolan

Discharged from hospital today after my second acute attack in just on four years. A huge thanks to this site for all the information it help save my life and my trip this time was less invasive than my time before. The doctors here followed the information off this site and I was delighted they listened to me! Feeling good only problem now is I seem to be still filled up with fluid? Weighing in at 65kgs should be 62kgs any suggestions as to how to get rid off excess fluid? Doctors here not happy about giving me diuretics due to them lowering my blood pressure too much? Any help would be great-fully appreciated. Best wishes to all Ruth (Christchurch New Zealand)

Dear Ruth: Once your capillaries stop leaking, a day or two after admission to the hospital, during which time Saline and similar fluids are to be administered very judiciously (less than 2 liters/day, to minimize leakages into the muscle compartments, even if your blood pressure remains abnormally low), and steroids, vasopressors and albumin are to be front-loaded and maximized, doctors have a duty to switch gears and are to help you eliminate previously extravasated and administered excess fluids by giving you diuretics (like Lasix). They should not fear that your blood pressure will drop. On the contrary, it is during this "recruitment" phase of SCLS that patients are at greatest risk of death because, unless evacuated, the extra fluids have a tendency to back into the lungs and other organs, causing pulmonary edema and other organ-threatening complications. Dont' take it just from me; have your doctors check out the information on SCLS posted on the Mayo Clinic website or elsewhere here.

Dear Arturo Thanks a million for your reply. I'm feeling comfortable that my doctors did all the right things. My weight is coming down today, so the excess fluid appears to be being excreted. Here's hoping! I've printed off the Mayo Clinic information and I will have this with me if there is a next time. Best wishes from New Zealand Ruth PS I'm sure this current episode was triggered by a chest infection that I contracted a few days before this episode ... it was the first one I have had in 4 years.

Question please Can any body who suffers from ISCLS tell me if they have red spots on their torso? I discover them after my very first acute attack and during my second acute attack however now I am noticing I am getting more red spots and existing ones are getting bigger in size and more pronounced? I had an appointment with my hematologist last week and he was unsure what was causing them and how to treat them. He is looking into what would be the best way to investigate them and I am waiting his reply. In the mean time I was wondering if any body else has noticed the same skin changes or any other skin changes? Walt, I have just been lucky enough to have been sent a copy of the DVD The Man Who Tripled in Size, thank-you to you and your family for sharing your story. Best wishes from New Zealand Ruth

I've not had any skin issues and eerily enough my first attack was in 2005 as well. That might not mean much however since there are so few of us and individual variation in symptoms could account for it. Jeff

rnolan I was diagnosed with SCLS 1995. As you can understand, I have a long disease history. I feel very alone with my illness SCLS. I have searched but have not found anyone with a diagnosis of SCLS in my country. For the first time I read SCLS patients' own words about their symptoms and medical history of treatments and experiences. I read everything written here. I've also read about Mayo Clinic and Dr. Phil Greipp's amazing knowledge and helpfulness in many years. Found Rareshar in 2008 became a member in 2010. My English is not so good so I can´t participate in your discussions, which was my greatest wish. But I want to tell you about my red spots with the help of translation software. Now to your question. It is first time I read about the red spots. I often had red spots on arms and shoulders for eight years. My doctor did not understand why. In 2003 I received treatment for another disease (Borrelia) with Antibiotics fifteen days. After two months I needed more treatment. To my surprise even my red spots vanished, have not got them back but the scars remain. I hope you understand what I wrote and that someone can help you with your red spots. Best wishes from irerolper.

Hi irerolper, your translation was very readable and I would encourage you to contribute to the discussion forums the best you can. Then you will not feel so alone. Thank you for you advise regarding my/your red spots, I am waiting for some tests to be done at our public hospital here in Christchurch New Zealand and will post the results. Keep in touch I hate to think you feel you are on your own through this lonely illness ISCLS. I find this site my life line along with my supportive family, doctors and friends. Best wishes Ruth Nolan Christchurch New Zealand

HI Ruth: I just read your above post after reading your most recent post to "windows" referring him to your" journey". Interestingly enough, I too get red spots on my torso!!! But I only seem to get them with heat. Especially after taking a hot shower. Also, with regards to skin issues, I don't know if this is related to scls, but since my illness, I have had MRSA twice on my face (looked like boils) and I have had skin issues with acne on my chin and itchy rashes on my body. I have seen the dermatologist numerous times as recently as this past Friday. I have ALWAYS had the most beautiful complexion and NEVER had a problem with skin issues, acne or otherwise until the onset of this mysterious illness. I thought maybe it had to do with hormones ? I am 45 and maybe entering perimenipause? I know this may sound like an unusual request, but I wonder if you would be interested in emailing me a photo of your red spots or If you like, I can take a photo from my camera phone and email you a photo of my red spots so that we can compare and see if they are similiar. Dr.Greipp may be interested if there is a correlation of these red spots to scls. My email is : susanfv@gmail.com. I hope you are feeling well, Susan

Hi Susan, firstly thank-you for your reply. I have just been to a dermatologist in the past week and she has diagnosed them as Campbell de Morgan spots and she also seems to think that they have been activated by the acute attacks I have had in the past. They start really small like pin pricks and get as big as a small finger nail. They have now appeared on my upper arms and a few on my feet. At present there is nothing she can do so looks like I just have to live with them, thankfully they are mostly covered. You could google Campbell de Morgan however don't be to alarmed by what you see as mine are not that alarming. I hope this helps take care Ruth Nolan :-)