I was wondering if any of the self-described "chronic" leakers on this board deal with edema every day. Is the edema always present or does it come and go with short breaks between leaks?

Also, does anyone experience extreme abdominal distention and subsequent loss of appetite during swells?

I'm asking these question because I've been systemically third-spacing fluids for over a decade and have become very disabled from the condtion. I haven't experinced an attack that required an ICU stay but I have been to the ER many times for dehydration and swelling and have devloped many ongoing health problems as a result of the condtion. Six months ago, I made a trip to the Mayo Clinic in MN where doctors there diagnosed me with a chronic form of SCLS.

Unfortunately, aside from one or two other members of this community, I have yet to encounter or read about anyone who leaks as often as I do. I've been screened for every possible disease that could possibly cause generalized swelling like mine so SCLS is my current diagnosis and I will be starting a trial of high-dose IVIG shortly. My edema does wax and wane over the course of several days but it never resolves completely. Due to the leaking/edema, my weight usually flucates between 10 to 20 pounds from week to week with subsequent drops in BP. I also experience many of the same symptoms mentioned in both the literature on SCLS and by others on this board, particularly the symptoms associated with hypovolemia (lack of sweat, very dry mucous membranes, dry mouth, thirst, myalgia, extreme lethargy/exhastion, cold and pale extremities, etc) when my swelling is at it's worst.

Any input from others will be much appreciated.

Sorry about the double post. I made too many typos on the other one

Anthony

hi

I have many of issues you do. I have started get IVIG very two weeks, which seem to keep my leaks under control better. I can handle most of the more aggressive leak by drinking gateraid. I am dealing with a disability claim right now as working full time under current conditions would be impossible. 

I wish Ish you the best.

How is your appetite during flares, Rita? Mine is non-existent. I primarily exist on shakes/liquids due to intestinal swelling/abdominal distension

Hello Anthony,

the abdominal swelling and loss of aperite is entirely consistent with 3rd space leakage... Fortunately not pericardial, pleural, pulmonary, cerebral or optic etc.

i cannot urge sufferers enough to get onto IVIG.

good luck!

John

Dear Anthony,

Like you I have  a pattern of continously persistent swelling with a waxing and waining pattern. I recognize gaining weight, abdominal swelling and discomfort, moderate loss of apetite, lethargy and exhaustion, which have been present even for long periods (weeks/months), beside other problems, like a swollen puffy face and discomfort on the breast and neck. When I started medication all of it resolved in the end (first theofylline and terbutaline, later on imatinib=glivec)

Note: In the last years it has been improving gradually, so I was nowadays in a good condition after all.The amount of Imatinib was graduallly diminished, and a month ago imatinib was stopped. But after a couple of weeks the pattern of suddenly gaining weight and the complains mentioned above re-occurred.

good luck

Hiltjo

AnthonyL,

I see that when you joined this community in April 2017, you wrote that "my current doc, who is an infectious disease specialist, has communicated with Dr Druey, and may start me on Theophylline soon."  Did you ever try it, and if so with what results?  Also, I urge you to make arrangements through your main physician to be seen by Dr. Druey and his colleagues at NIH, because chronic cases of what could be SCLS are tough to diagnose and treat.  I mean, if you've already been to the Mayo Clinic, going to and being evaluated at NIH is much closer -- and free of charge.  There's also the contribution to knowledge you can make by going to NIH and agreeing to donate some blood and possibly tissues to advance their research projects, which I don't think Mayo has on edema-related maladies.  And I wish you good luck with your upcoming trial of immunoglobulins.  John Carson is absolutely correct: while it doesn't always work for chronic leakers, you have little to lose by trying it -- and it may end up saving your life by preventing the kind of full-fledged episode of SCLS that will damage your heart, brain or other organs.

Arturo

PS: Would you please add your birthdate and role (patient) to your profile?

Hi Anthony. In addition to acute, full-fledged episodes my husband also has the chronic third-spacing you describe. His doctors think he is leaking all of the time and as edema builds it eventually triggers a faster leak episode (which for him means the ER or the ICU). As far as I can tell there aren't a lot of people who experience both the chronic swelling and the full-fledged leak episodes (it's like we won the worst lottery ever).

For him, it starts with swelling in the legs & feet that gets worse as the day progresses. If he's lucky, it will be reduced in the morning, but it never really goes away 100%. He also builds up edema in his face and neck over time. By the time he is swelling in the abdomen, arms or anywhere else he's already at the emergency level and in the hospital with some of the symptoms you describe (cold extremities, etc). In the past nine years he has been hospitalized over 100 times. His weight does fluctuate quite a bit similar to what you mention. Once he hits a certain weight gain, we consider taking him in for IVIG to try and head off a serious episode. He can't have monthly IVIG as that also can trigger episodes for him. It's complicated. :)

Hi I’m going to try to give a more complete answer. I have had numerous checks on my colon to see if there are ismore issuesHowever all we have found was one polyp and some diverticuli. The polyp has been removed and it was the kind that could become cancerous but was benign at the present time. However all we have found was one polyp and some diverticuli. The polyp has been removed and it was the kind that could become cancerous but was benign at the present time.

 I am beginning to think my intestinal problems are caused by bloating in the stomach. This perhaps could be due to the variation in weeks. It seems there once every three or four months I get uncomfortable and it takes me almost a month to get back to myself. I have not had any acute attacks requiring hospitalization Since I have started the IV I G every two weeks

I hope all goes well for you 

Thanks for all of the replies. Truly appreciated. I've been a long time lurker and occasional poster on these boards, so I already know about some of your cases, as I read through many of the posts/profiles when I first signed on to rareshare about a year or so ago.....I am also in touch regularly via phone with two other members of the rareshare SCLS community who have been diagnosed with a chronic form of SCLS, one of them by the same doctor who saw me at the Mayo clinic

Anthony

JCarson: Regarding IVIG, easier said than done, John. I've been home from the Mayo for six months and despite a clear-cut treatment plan for trial of IVIG recommended by Dr Kapoor, a hematologist at Mayo who has published on SCLS, I have yet to receive IVIG. In fact, my current physician just informed me a few days ago via email that he believes it will be impossible for me to get approved by Medicare for IVIG because according to him they don't cover it, which I think is absurd, considering that the reason I am disabled and on Medicare in the first place is because of the capillary leak. All other possible conditions that could cause third spacing like mine have been exhaustively ruled out, so my only hope for relief at this point is a trial of IVIG to see if it works.

I was hoping that the Mayo dx and treatment plan would put an end to the ambiguity regarding my medical treatment thus far but no dice. I do have a local hematologist who may be able to take over my case and submit to Medicare but no guarantees just yet. I've been struggling with this condition for over a decade now and it has reduced my quality of life markedly. I am not working, I live on liquids exclusively due to abdominal distention and am exhausted almost 24/7. I could deal with all of that, though, were it not for the breathing issues. The symptom that's most devastating and restricts my ability to function is respiratory issues. My lung function becomes severely restricted thanks to massive abdominal distention that puts pressure on my diaphragm restricting my breathing to the point now that I register lower 60s on digit oximeter reading while lying in the supine position at the doctor's office. I become hypoxic essentially. While standing, my readings return to the normal range, but if it's a bad swelling day and I lie on my back, I have the pulmonary profile of COPD patient. Same goes for spirometry readings on a bad day. They're terrible. But the problem is not stemming from my lungs per se. It's due to pressure on my lungs from the abdominal distention. Feels like a boa constrictor is wrapped around my upper abdomen. When the abdomen deflates, however, and I urinate off some of the fluid, I can breathe perfectly fine and can even run up a flight of stairs. Lung function tests are fine too when I'm deflated. 

Thanks for the input, John

Anthony

Arturo: Thanks for the info, Arturo.......Everything you mentioned all sounds fine in theory, but as you know, I have been at this for a while now and no luck with NIH. Dr. Druey is empathetic toward my situation and is aware of my case but has not shown much interest in it in terms of research, primarily because I do not demonstrate obvious markers for SCLS, namely hemoconcentration--- though my hematocrit, RBC count, hemoglobin levels are all at the upper end of acceptable range and much higher than they were when compared with my CBC reports prior to onset of illness.

My BP drops, I've had two documented episodes of hypoalbuminia recorded at the ER and have such severe pitting edema (3+) in my lower legs that they look like the limbs of a person in end stage heart failure. According to Dr Kapoor at Mayo, my systemic third spacing and hypovolemia symptoms are "classic" symptoms of SCLS (his words, not mine), which is how he describes my case in his notes in the Mayo patient portal. I live on liquids due to intestinal swelling, have permanent damage to my vision thanks to inflamed capillaries in my left retina, abnormal EKGs now (not due to ischemic heart disease or hypertrophy as echo and angiogram have ruled them out as possible causes), documented episodes of hypoxia from restricted respiration second to abdominal swelling, bout of acute pancreatitis, rashes, duodonitis, intestinal wall swelling, etc,....all deemed "idiopathic" because up until very recently physicians had no idea what was happening to me. 

I've just received word today that Medicare has rejected my doctor's petition for trial of IVIG and my doctor tells me that there is no ICD code for capillary leak. I can go to three different doctors and get three different hypothetical ideas about what might be causing these symptoms. At this point, I am not so much interested in hypotheticals as I am in what we know so far about my case and what can or cannot be done to alleviate the leaking/swelling. We know that I am third spacing fluids systemically and all other possible causes have been exhaustively and thoroughly ruled out. There are no other known diseases, syndromes or conditions, both common and obscure, that could be causing symptoms like mine; and since capillary leak syndrome is a diagnosis of exclusion, that's the diagnosis that we're going with at the moment.

Hi Josephite: I'm very sorry to hear that your husband is dealing with this awful condition in both it's acute and a chronic forms. That must a nightmarte and my sympathies go out to the both of you as I realize that this illness takes a heavy toll on both patients and caregivers.

I think that what a lot of physicians have trouble wrapping their heads around is a condition that causes symptoms of hypovolemic shock despite negative blood cultures and without a definitive underlying cause. In the case of chronic SLCS, the presentation is even stranger and diagnosis/management even more elusive because the patient is essentially exhibiting symptoms of low grade shock in chronic form. Chronic low grade shock doesn't exist in the minds of most physicians, unless they are familiar with SCLS and related disorders. It's an impossibility as far as they're concerned. They've neither seen it nor heard of it before, so far as they're aware, it doesn't or shouldn't exist. In my own case, I've found this to be a real problem and major barrier when it comes to proper diagnosis and effective treatment. As a patient, you wind up feeling like you're up against a lot of dogma and preconceived notions about what should and shouldn't be possible in terms of the human body.. I am living proof, however,  and I am sure that your husband is as well, that a person can survive long term with a good portion of their vascular fluid sequestered into interstitial spaces where it doesn't belong...The lack of knowledge about SCLS and related conditions as well as the misdiagnosis by physician has been every bit as devastating to my psychological well being as the illness itself. Feels as though I am trapped inside a never-ending episode of the Twilight Zone sometimes

I am touch via phone with two other patients from rareshare who have been definitively diagnosed with chronic form of SCLS (they also have acute attacks as well) and both went many years without a diagnosis and are remarkably disabled from the condition with degrees of daily disability that are on par with some MS patients I've known. They are both housebound for the most part, not working and their quality of life is very poor....... For the sake of both you and your husband, I hope that he gets some much deserved relief from symptoms soon. My email is koac6974@gmail.com, if you or anyone else would like to keep in touch, compare notes, etc

All the best to you and your husband, Anthony

Anthony,

Regarding "I've just received word today that Medicare has rejected my doctor's petition for trial of IVIG and my doctor tells me that there is no ICD code for capillary leak." I'm going to help you on this specific matter.  I will write to you directly, offline.

Arturo

Hi Antony,

Hi Anthony,

I have had a long 40 year history of chronic systemic cyclical leaking and retieving. Chronic Capillary Leak is also my preliminary diagnoses through elimination, altough I do not have access to anyone who has ever treated someone with the condition, except for the team that treated Warren from Canberra. Because he is an accute patient, and I did not fit the same mould, They did not feel able to definativly make that call. It is the only condition that fits perfectly. For me, I used to get an attack monthly, and completely recover between, and as the years went on, it got worse and closer together, untill it was continual. It used to be resricted to my extremities, but is now drasticly effecting my GI tract, lung function and brain function (Migraine), all of which are really just systoms of SCLS. In addition my Cycokine responses are off the charts. Much of what you discuss is familiar, like the abnormal EKGs for example. One scan shows an enlarged heart and the next normal. This can only be changing levels of fluid where it should not be.

I am Australian and so we do not have the ability for IVIG to be covered by an Insurance company. It is only available here for life threatning cases, when the patient is in ICU, but as you know this condition can also be very life-limiting. I am grateful that this condition is at this point, unlikely to kill me, however it never really leaves you, so it is a really just different type of touture.

There is one patient here in Sydney that I am aware of, that is a chronic SCLS patient who has been trialling IVIG through a large public hospital, however she is not responsive to inquiry, and the doctor will not take new patients, so it seems I am still cut out from treatment, amd I am unsure if it has worked for her. 

I have tried many different drugs and different specialists without much assistance, untill recently. So what was been of benefit. For me, I got a years reduction of symptoms with an Italian drug called Aminaphtone, which I imported from Italy. Email me for pharmacists details, if interested. It is an OTC drug, although I do not know what it is derived from, despite extensive research. Sadly after approximately one year, it gradually became less and less effective at over maximum dosage, so I stopped it about 1 year ago. I was very greatful however for that year. Then I had a bad 6 months untill about 3 months ago, my intergrative doctor tried Hydrocortisone. 10 mg at 8 am and 4mg at 12 and 4mg at 4pm. When I feel an episode coming on, I double for 24 hours, which stops it from building. This is currently working well, and I am hopeful that it will not stop working. I will preferance this with the info that I do have low end thyroid and Pituitary function but I did pass the Insulin Tolerance test.  I make cortisol in normal amounts, but only under extreme stress. Day to day however I am very low, in ATCH, cortisol, growth hormone and IGF-1. I did a few years ago try a much higher dose of Prednisone, which did not work. Why the hrocortisone form works, I do not fully undersatnd. I am 58, and the tought of staying on steriods is of course concerning, but in the absence of IVIG, what other options are there for me. I am currently working again, which is wonderful. If I replase again, I will travel to the states to see Dr Durvey. 

Good luck to you, and I hope that this info is a tiny bit helpful. I would love to hear from any chronic leakers who have had success from IVIG, and especially any in Australia, although I think there are only a handfull of patients and mostly of the more acute form. 

Annmarie

Hey, Annmarie,

My apologize for not getting back to you sooner. I had a busy few weeks and many sick days

Wow! 40 years! That's awful. My heart goes out to you. I find it difficult to imagine living with this condition for another year, let alone another 30. I'm happy to hear that you're getting at least some relief from the hydrocortisone.

I think one of the biggest problems with this condition is that it's under-reported in the literature. The avg physician can't even reference any reports of similar cases for comparison and diagnosis. Once they check off common possible causes of generalized edema such as hereditary angioedema, mastocytosis/mast cell activation disorder, allergies, kidney, heart, liver issues, etc, you're on your own more or less, as a patient.

Even in it's "classic" acute form, the SCLS is still a very poorly defined and under-researched condition, which is why they still refer to it as a syndrome rather than a disease. I've read just about every publicly available published paper and still don't see a clearly defined consensus as to what what SCLS actually is. They describe a constellation of symptoms and abnormal labs but offer no definitive diagnostic markers or underlying cause. I've read a few published reports about cancer patients who develop generalized edema and what sounds like a systemic inflammatory response after receiving chemotherapy agents (Interleukin 2 primarily). They refer to this syndrome as "secondary SCLS". Now, is this the same syndrome that's occurring in the idiopathic acute cases? Nobody knows. Also, there are scattered reports of idiopathic cyclical edema, primarily effecting women during their menstrual cycles. These women develop generalized edema but without the hypovolemia and low blood pressure that characterizes SCLS. Are these women suffering from a similar underlying pathology as SCLS patients? Again, nobody seems to know.  It would be helpful if we had a registry of some kind where our physicians could submit reports and possibly share information in order to help others struggling with similar symptoms.

Most (90%) of the published papers on SCLS are simply case studies and reports. There's very little actual research aimed at studying underlying biological mechanisms responsible for the syndrome. The only person I am aware of who has done a fair amount of study in this area is Dr Druey but his research is probably limited by funding.

I'm obviously not a doctor, but I suspect that SCLS is spectrum disease involving varying degrees of abnormal capillary permeability resulting in fluid shifts. The key term here being fluid shifts. It's the loss of vascular fluid along with the edema that distinguishes SCLS from other generalized swelling conditions far as I can tell. That's why SCLS patients are full of edema yet paradoxically dehydrated. There's too much fluid outside the blood vessels and too little fluid inside. Blood vessels even in healthy folks are always permeable to a degree but in the case SCLS the vessels become abnormally permeable which leads to massive fluid loss and edema. On the one end of the spectrum, you've got the classic acute life-threatening cases and on the other end you've got more chronic and difficult to treat cases-- and still other folks who fall somewhere in the middle of the spectrum in terms of their symptoms.

I also think that cytokines and perhaps some other unknown chemical messengers are the main culprits behind the syndrome. If researchers could positively identify these culprits, they could probably get a better handle on managing the condition and possibly develop more definitive diagnostic tests, rather than relying on clinical signs and symptoms. Hemoconcentration, for example, isn't a maker for SCLS but rather a marker of decreased plasma volume (low blood volume). It just means that the blood is concentrated due to fluid loss into tissues. It's not specific for SCLS or any other condition. But that's the best diagnostic test for SCLS that we have at the moment. It would be nice if we had some more definitive and exact blood tests, which would likely lead to more definitive and exact treatments. As it stands now, the best they have to offer in terms of diagnosis are CBC reports, albumin levels and BP monitoring, in addition to clinical signs and symptoms. Patients like myself and perhaps you as well who don't match exactly with the currently proposed diagnostic criteria can easily fly under the radar, IMO. Half the time when I'd show up to ER during an attack, I'd already be urinating off some of the fluid and deflating, so my labs would look normal or near normal.

What they're calling SCLS seems to me like a deranged immune response of some kind, perhaps similar to the biochemical derangement that occur during sepsis and anaphylaxis. For whatever reason, folks who suffer from SCLS seem to develop an inappropriate immune response to normal everyday stimuli that results in abnormal amounts of fluid escaping from the small vessels. If it's severe, it can be lethal; and if it's chronic, it can be very debilitating and possibly lethal as well in the long term. I think this is why it's so difficult to pin down triggers. I'm not sure there are any. I suspect that folks who suffer from SCLS are experiencing an immune reaction to otherwise harmless stimuli, possibly similar to an autoimmune type reaction. The immune system mistakes ordinary stimuli for a life-threatening pathogen and overreacts....Perhaps this is why steroids and the T&T combo work for some patients. Those drugs inhibit the inflammatory response and prevent the vessels from dilating and becoming too permeable...... I have no medical background to back up my theory, though, so my opinion on this matter is basically worthless.

When I first got sick, one of the first symptoms I noticed, in addition to flu-like symptoms and the rapid weight gains and losses, was dehydration and inability to perspire. I'd always sweat quite profusely when active but that stopped after I started swelling. Despite the fluid everywhere else in my body, my mouth was bone dry and the skin on the back of my hands was wrinkled, dry and had the pale complexion of a corpse. Urine output diminished greatly as well and of course blood pressure would drop from my normal 120/80 to 90/50 or thereabouts. My CBC reports showed a total white blood cell count that ranged anywhere from 15k-25k with elevated neutrophils and monocytes as well. This was a consisted abnormality for 8 years but docs at the time never paid attention. They'd always look at the isolated reports and blame it on something transient like an infection, which never made sense to me because I wasn't suffering from any infection that I was aware of. It wasn't until I got fed up with years of misdiagnosis and misdirection that I stapled all the reports together myself and brought them to my GI doc who flipped through the thick stack of abnormal CBC reports lying on her table and immediately referred me to an immunologist. It was only then that I started getting some answers.

I've also had idiopathic pancreatitis and the whole slew of idiopathic inflammatory conditions I mentioned above. My testosterone was very low at one time as well, when I was very sick and swollen, but the levels have returned to low-normal as the swelling has diminished somewhat over the past year. I've had very low early-morning cortisol reading like yourself but they weren't abnormal during ACTH test. These endocrine abnormalities led to misdiagnosis of possible pituitary tumor, which was dismissed after a clean MRI. This was years ago when I was being sent to multiple specialists and getting misdiagnosed repeatedly.

At any rate, thanks for sharing, Annmarie! I believe that the more info we share about this condition the better for both patients and physicians alike.

Best,

Anthony

BTW, here's a published case report of a 64 year old male SCLS patient who presented with multiple endocrine abnormalities like yours which reversed after he was treated with IVIG. His hormone levels returned to normal after his physicians got the SCLS under control. The authors speculate that the hormone abnormalities were a consequence rather than cause of his SCLS. See here : https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4313677/

Additionally, here's another very recently published paper (2019) on the chronic form of capillary leak syndrome. It's the only published paper and review of the literature I am aware of that focuses exclusively on the handful of reported cases of chronic SCLS . It's just an abstract, unfortunately. I wish I could get my hands on the full paper. See here:  https://www.ncbi.nlm.nih.gov/pubmed/30431430?dopt=Abstract

I hadn't seen these two articles, so many thanks for the refererrals! I'm going to include the second one under Disorder Resources.

You're welcome, Arturo!