I was diagnosed with Chronic Systemic Capillary Leak this Summer. I continue to gain weight and have all the symptoms but have now moved into an acute attack. I've been on Theophylline and Terbutaline with minimal response which appeared to just slow things down but I continued to swell. My Albumin has been dropping and my H&H and CK have been rising. If I'm on my feet or exercise too much everything ramps up quickly and I pass out. IVIG has been approved and attempted, but it was in an infusion wing with no doctor present. They gave me GammaGard with Saline. I started screaming after 30 minutes when it reached 90. It felt like I was being electrocuted-with severe paresthesia symptoms. Because no doctor was there to ask what to do they stopped it after trying again. I'm having massive myalgia, and paresthesia and feel like someone has been beating my legs and arms. I keep cramping severely in my limbs, which has been excruciating. I went to the ER and the doctor was extremely uncomfortable with trying to treat, and my immunologists were out of the country and the other vacationing. Neither can find a facility that feels comfortable treating but I continue to get worse. The swelling is now in my abdomen and pelvis. I'm trying to find another doctor or patient in New England for advice on who and where to go that has seen this. Also-can someone tell me if they get severe parasthesia and compartment syndrome and what it feels like? The nausea is also killing me, but the zapping and electricity feeling is painful and disturbing. All the docs tell me this is getting unsafe, but don't know where to send me or who so I don't die being treated. DrDruey said they don't see patients for treatment. Dr Pecker of Yale is out of the country.

I have nerve damage due to compartment syndrome. When I swell I get tingling much like when your arm or leg falls "asleep". I take gabapentin to help with the sensation. Mayo clinic in Rochester MN has a Dr that works with CLS patients. I can no longer use IVIG so we are trying an ACE inhibitor and Singulair. I am not sure it is doing much good as I am having minor attacks on a two-week cycle. I hope things start looking up for you.

My husband has had compartment syndrome in all four limbs. He often gets cramps in his limbs and has a lot of nerve damage which he takes Gabapentin for. Anytime my husband presents in the ER, they call on the hospital's internal medicine team to come treat him. Treating this syndrome is a tricky balance that unnerves most doctors who haven't dealt with it before, so I feel much empathy for your situation. I hope you find good medical guidance soon.

Ritz, would you mind emailing me at liesaevans@live.com My husband also takes diuretics and I wanted to pick your brain a bit about your experience with them. Thank. :)

Has anyone seen a doctor in New England? I'm getting worse fast. My glucose and WBC are high, protein in urine. My local immunologist asked my mast cell doc at the Brigham to infuse so I could be at a tertiary care facility. She said she wanted to only treat my mast cell and asked him to treat. Then my doc asked oncologist/hematologist in closest hospital to infuse in the MGH/Cancer suite. He asked I be referred to Tertiary care to treat which was already attempted by my local, and he told him it had. This leaves my local trying to treat in an observation ward. Because there's no doc after 4p he waited to try and set up for Monday. He sent me to ER because of pain, swelling and lab work. The ER doc sent me home last night because he didn't know how to treat. Which puts me right back at home. My feel, legs and pelvic/hip area really hurt. Even my toes hurt. This is crazy!!! There must be someone in New England that has seen this and is comfortable treating. I feel like my liver and kidneys have something really wrong. My right side hurts so badly. My muscles feel like they are dying and it's hard to walk. I'm going to call Dr Druey again. He said he doesn't treat and only consults. This leaves with me no safe place to get the IVIG. I have no idea what the heck to do? Also is there imaging that can be seen of this? Perhaps T2 weighted MRI?

I am from New England [Maine]. Have you tried Lahey Clinic in Burlington, MA? I'm from Maine and I started my journey there. I ended up being diagnosed and treated by Mayo Clinic Jacksonville before getting past Endocrinology to Hematology. However, it is an excellent research hospital with a well-staffed and informed ED.

Hello mntnhiker Reading The excellent literature on this strange condition reveals that muscle pain is a dominant and common occurence. Personally, each episode failed to settle until I had received a shot of morphine? Fortunately that was 2 years ago... None since! The pain could either be inflammatory in nature, or due to compartment syndrome, or possibly another cause I have not considered. Whichever way my leg muscle mass has shrunk grossly since my days (2 years ago) of hiking the jungle mountains of Papua New Guinea. The pains stopped after IVIG. John

My husband has had SCLS for about 10 years. He had operations on both legs for Compartment Syndrome to relieve the pain. At the time, it was an emergency and he would have had a double amputation if the doctors had not operated. If you could get to Nashville, Tennessee to the Vanderbilt Medical Center to see Dr. Nancy Brown, she may be able to help you. She is a research doctor and she is his doctor. She and her staff have been able to save his life on more than one occasion and he has been symptom free for about seven years. I wish you the best of luck.

Currently have signs of rhabdho and diagnosed with leg and abdominal compartment syndrome. Receiving IVIG right now and desperate for this to work. My kidneys are killing me and I feel like I'm going to burst. Crossing my fingers.

Hi, I am from Massachusetts and have SCLS. My Dr is out of Mass General and her name is Dr. Banerji. After 3 attacks she was the only one who knew what I had when she saw me on my 3rd attack. I am only on Theophylline and Terbutaline and luckly have not had another attack since 2006. Hope this helps.

I saw Dr Banerji when it first started and she thought it was Mast Cell Disorder which I have. She kind of blew me off. I had already been started on Theophylline and Terbutaline and it really effected my arrythymia, and it only slowed the swelling down. I asked my immunologist to run a 24 urine for Creatinine, protein and Creatinine Clearance because my kidneys are killing me. Just picked up lab today, sends out weren't back yet though. Results so far show High-Creatinine Clearance, High-Total Urine Protein, High-Creatinine, Low Albumi, High Alpha2 Globulin, High Platelet and clumping on Smear. H an H elevated. My legs are taut today. I'm up 65#. The doctors have been afraid to treat so far. Afraid of either killing me or trashing my renals. Not sure what to do. Doc that ordered labs is away until Monday. Hematologists wants me to be seen at Yale or Boston but no physician specifically.

I had Rocky Mountain Fever in 2012. I was the fist inland case in Massachusetts so I was dying by the time they figured it out. Ever since then I've had heath problems relating to my bone marrow, Gi tract, pancreas and liver. In the late winter (Feb 2016), the swelling began. Really frustrating as this seems to be induced by a dam tick!

Hi, I'm from NJ and have a chronic form of the condition like yourself. Been dealiing with it for severel years and have become quite disabled over time from it. I was wondering if you have any GI symptoms along with the swelling? Also, do you experince hypovolemia (low blood volume/dehydration)? That's been one of my biggest issues since this happened to me. I'm completely dried out internally even though I have fluid trapped beneath my skin (edema). I barely urinate when I'm swollen. My docs are stumped and also uncertain about how to treat it, as all of the usual causes of generalzied edema have been ruled out already. I also was given a tentative diagnosis of mast cell disoder, but they haven't performed work up for it yet. Lots of infammation as well. I developed pancreatitis, which fortunately was mild, but it all appears related to this condition, whatever it is. I'm not wholly conviced that chronic cases of capilllary leak are due to the same underelying pathology as the acute life threatening cases. Seems to me that some of these chronic cases are just getting lumped in with SCLS when in fact they might be something else, perhaps a more common condtion that is simply presenting in an uncommon way, and therefore confusing doctors. Have you been worked up for hereditary angioedema?

Hi

     I have been tested for hereditary angioedema,  Mast cell disorder, allergic shock and many more diseases related to SCLS.  I did not test positive for any of the related disorders.

       I have chronically suffered the symptoms of the first phase.  I had high Hgb, Hct, low albumin and low BP for weeks at a time with out entering into the second phase.  Just recently my albumin level has been on the rise it's now at the low end of normal and my BP is hovering around 92/58, but my Hgb and Hct are still elevated.  I have gained 33 lbs in 14 weeks.  I continue to swell, sweat profusely, urinate once a day, suffer nausea, flu like symptoms, a burning feeling in my legs and feet, redness, trouble breathing  and constant fatigue.  I have not been able to leave my bed for the last three weeks, not even to shower or brush my teeth.  

      I  experienced  issues with my liver during 2016.  My liver enzymes ALT AST and ALP were severely elevated.    My last blood result in June showed that my liver enzymes were beginning to decrease, they're hovering around the high end of normal.  

I recieve 30 ml of Flebogamma 5% solution twice a month. The IVIG treatments seemed to work for about a year and now I am back to suffering from these  " almost "  first stage SCLS symptoms for weeks  without ever entering into the second stage.  My albumin is at the low end of normal and so is my BP, but not low enough to be considered a full capillary leak.  

I have experienced higher than normal BP off and on this year.  Does anyone else suffer from high blood pressure during, after or in between an episode? 

Kimberly

Hi,

I did have a severe attack without high HMC 8,5 Months ago back In Miami, Florida. .

Doctors were not able to diagnose me until I returned to Spain and finally got diagnosed by a well known Internal Med doctor here in Barcelona.

I do experience GI symptoms when I leak: acute and severe pancreatic pain, Bowel movement and soft stool.

My urine also diminishes considerably.

I had red spots in my face every now and then, flushing sensation, burning sensation and red swollen hands, myalgia, low pressure and general weakness (as I had ran 3 marathons in a row), in addition to runny nose and slight depression or irritability.

I did have hypovolemia at the severe attack (Gained 25lbs in less than 2 days), which caused a secondary hyper aldosteronism.

My current treatment is 30mg of Terbutaline (10mg/8hrs) and 200mg of spironolactone for the aldosterone imbalance (100/50/50), also 20mg of Bilastine in the morning.

So far I am happy to tell it is working. All the fluid went away after three months.

In addition to that, I lost body weight upon starting the treatment. (11lbs)

Presently, I weigh every single day as this is one of my tools to control the symptoms.

Every 15 days, there's a 2 to 3-pound gain when I leak, which remits in 1 to 2 days.

My docs say it's because I already had a mild chronic and silent presentation of scls since years ago ( Now I understand why I used to gain 4 pounds every summer when my calorie intake was absolutely diminished).

Heat, humidity and stress trigger the symptoms, and certain veggies (tomatoes) and fruit (melon, peach) that contain Cucumisine -a protein that elevates histamine-.

Which factors trigger yours?

Big hug for y'all.

#strongforlife

 

Valeria