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Systemic Capillary Leak Syndrome

What is Systemic Capillary Leak Syndrome?

Systemic Capillary Leak Syndrome (SCLS) is an exceedingly rare, life- and limb-threatening disorder characterized by acute and severe recurrent attacks featuring a rapid fall in blood pressure due to the temporary leak of plasma out of the blood circulatory system.

This virtual community is dedicated to the memory of Judith (Judy) Lynne Davis (1958-2009) (judithdavis3), one of its founding members and a victim of a very severe episode of SCLS that took her life in November 2009.

We also mourn the death of eight other SCLS patient members of this community: Mario Gatto (mariogatto) from Naples, Italy, who passed away in December 2009; Denise Weston (mdweston) from Ohio, USA, who passed on March 2011; Bruno Galien (bruno) from Nord-Pas-de-Calais, France, who passed away in February 2012; Guy Allen Overland (allenoverland) from the Washington DC area, USA, who passed on January 2015; Marilyn Meaux (maire602) from Louisiana, USA, who passed away in March 2017; Julie Eady (jodono) from Perth, Australia, who passed on September 2017; Cara O'Hagan (Cara) from Dublin, Ireland, who passed away in February 2018; and David Colburn (davec) from Gainesville, Florida, who passed on in September 2019.

 

Synonyms

  • Clarkson or Clarkson's Disease

Systemic Capillary Leak Syndrome (SCLS) is an exceedingly rare, life- and limb-threatening disorder characterized by acute and severe recurrent attacks featuring a rapid fall in blood pressure due to the temporary leak of plasma out of the blood circulatory system.

This virtual community is dedicated to the memory of Judith (Judy) Lynne Davis (1958-2009) (judithdavis3), one of its founding members and a victim of a very severe episode of SCLS that took her life in November 2009.

We also mourn the death of eight other SCLS patient members of this community: Mario Gatto (mariogatto) from Naples, Italy, who passed away in December 2009; Denise Weston (mdweston) from Ohio, USA, who passed on March 2011; Bruno Galien (bruno) from Nord-Pas-de-Calais, France, who passed away in February 2012; Guy Allen Overland (allenoverland) from the Washington DC area, USA, who passed on January 2015; Marilyn Meaux (maire602) from Louisiana, USA, who passed away in March 2017; Julie Eady (jodono) from Perth, Australia, who passed on September 2017; Cara O'Hagan (Cara) from Dublin, Ireland, who passed away in February 2018; and David Colburn (davec) from Gainesville, Florida, who passed on in September 2019.

Acknowledgement of Systemic Capillary Leak Syndrome has not been added yet.

Less than one in 1 million people are affected by this disease. The onset of SCLS will usually occur in adults. However, SCLS can affect people of all ages.

Name Abbreviation
Clarkson or Clarkson's Disease Clarkson

Systemic Capillary Leak Syndrome (SCLS) is idiopathic. Thus, at present, there are no known causes. Probably a mid-life gene mutation takes place that renders those affected vulnerable. Possibly immune-deficient in some way to these curiously self-reversing capillary leaks.

Many patients report having a runny nose, flu-like symptoms, gastro-intestinal disorders, a general weakness or pain in their limbs, swelling in the face or hands and feet, or very cold hands and feet, but others get no particular or consistent warning signs.

Name Description
Swelling swelling
Myalgia Myalgia is muscle pain
Rhinorrhea Rhinorrhea is a runny nose
Dizziness Dizziness
Lightheadedness Lightheadedness
Hypotension Hypotension is abnormally low blood pressure
Hemoconcentration Hemoconcentration is the decrease of the fluid content of the blood, with increased concentration of formed elements
Hypoalbuminemia Hypoalbuminemia is low levels of protein in the blood
Nausea Nausea
Excessive thirst Excessive thirst
Generalized edema Generalized edema
Decline in clinical picture Clinical picture declines rapidly within hours
Cold limbs and sweating Cold limbs and sweating
Rapid swelling and compartment syndrome Rapid swelling of all limbs with development of compartment syndrome, especially during IV fluid administration
Decreased urine output Decreased urine output
Vomiting Vomiting
Intestinal cramps Cramps
Diarrhea Diarrhea
Fatigue Fatigue
Headache Headache

The diagnosis of SCLS is made partly by exclusion, namely, by eliminating the possibility of other more common diseases, and is based on measurable, clinical symptoms such as hypotension or low blood pressure, hemoconcentration or decrease in blood volume, hypoalbuminemia or an abnormally low levels of a protein called albumin in urine, and the presence of a protein called Monoclonal Gammopathy of Unknown Significance (MGUS).

Some diagnostic tests may include blood and urine tests to check for abnormalities such as dark urine, concentrated blood, or low serum albumin in the blood.

  • Methylprednisolone 125 mg intravenously one time, then repeated as needed.
  • Judicious use of intravenous fluids (IVF) in boluses and drips to keep central venous pressure (CVP) above zero.
  • Phenylephrine or Norepinephrine for hypotension, early institution.
  • 50 ml of 25% albumin, then repeated as needed.
  • Continuous CVP monitoring, immediate (stat) and continuous (serial) lab work including createnine phosphokinase (CPK) and lactate.
  • Immediate orthopedics consult and compartment pressure measurement early on to prevent limb fasciotomies if compartment pressures or CPK high. 
  • Venous doppler for deep vein thrombosis (DVT) prevention, may need full anticoagulation to prevent blood clot.

Treatment of a fully developed SCLS episode requires recognition that there are two phases. The first phase, which often lasts a couple of days, is called the resuscitation phase because the dual aim of the emergency room (ER) and intensive care unit (ICU) treatment is to stop or control the capillary leak and to raise the patient's blood pressure from near zero. In this initial phase, an albumin and fluid leak from the capillaries into the tissue spaces causes swelling, especially into the extremities rather than the abdomen or organs (such as the lungs). The blood pressure falls and the red cells concentrate. This loss of fluid has similar effects on the circulation as dehydration, slowing both the flow of oxygen carrying blood to tissues and the output of urine.

Glucocorticoids (steroids like methylprednisolone) are recommended to reduce or stop the capillary leak, and albumin and colloids usually help to increase the remaining blood flow to vital organs like the kidneys. Keeping up with the fluid loss is important because sustained low blood pressure can damage vital organs such as the kidneys. Even though blood pressure readings may reach and remain at very low levels, it is important to avoid overly aggressive intravenous fluid administration causing massive swelling of the extremities.

The goal of saline and vasopressors administered should NOT be to restore a "normal" blood pressure (or urine flow), but to maintain it at a minimal level sufficient to avoid permanent damage to vital organs. Measurement of central venous or arterial pressure in an ICU setting is often necessary to achieve this delicate balance. When too much fluid is administered, the result is excessive swelling, and the patient may well require surgical decompression of the limbs. In this procedure, known as a fasciotomy, the skin of the arms and/or legs is incised to release the compressive pressure the retained fluid is having on blood flow to and from the extremities.

The second phase of the treatment is known as the recruitment phase, when fluids and albumin are reabsorbed from the tissues during at least a couple of days. In this phase, the capillary leak has ended and the main threat is fluid overload. If intravenous fluids were given in excess, they usually cause an accumulation of fluid in the lungs and around other vital organs. Most of the patient deaths happen during this recruitment phase so it is important that diuretics be administered to help patients discharge all the fluid previously given -- and to keep them from backing up, especially into the lungs.

As concerns episode prevention, two approaches have been tried: β-agonists like theophylline and terbutaline, and a prophylaxis with intravenous immunoglobulin (IVIG) infusions. In recent years, more and more patients have been migrated from the former to the latter in Canada, Europe, the United States and beyond, because IVIG therapy leads to superior results. No episodes or fewer and lighter episodes than compared to no therapy or the other therapies, and does not have as many adverse side effects as does treatment with β-agonists like theophylline and terbutaline.

The prognosis is uncertain and depends on (a) how well episodes are managed, in terms of preventing permanent damage to vital organs and extremities; and (b) the ability to prevent episodes altogether.

There are two main treatments to prevent episodes of SCLS. The oldest is the Mayo Clinic’s approach of a preventive therapy with theophylline (or aminophylline) and terbutaline tablets taken on a daily basis. However, these medications, meant to reduce endothelial hyperpermeability, have very unpleasant side effects, and often prove ineffective, providing partial and transient improvement.

The newest is the French preventive regimen, which involves monthly infusions of immunoglobulins (IVIG). There is growing evidence that IVIG (usually, 2 gr/kg per month, administered over two consecutive days) has worked for many patients in Europe for over 10 years now, and is proving extremely successful among patients who have tried it in North America and beyond in the past several years, thus having become the standard of care.

Name Description
Medical help

Find yourself a compassionate physician, preferably a specialist in internal medicine or hematology affiliated with a major university hospital, willing to do his/her homework on this rare disorder (namely, read the literature and follow the instructions), and willing to consult with the few SCLS experts available:

In the United States,
Dr. Mark S. Pecker
Professor of Clinical Medicine
Weill Cornell Medical College
New York, NY
tel. 1646-962-2605
email mpecker@med.cornell.edu

In Europe:
Prof. Dr. Zahir Amoura
Département de Médecine Interne
Hôpital de la Pitié-Salpêtrière, 47-83 Bd. de l'Hôpital, 75013 Paris
tél. 0033 1 4217 8001
email zahir.amoura@aphp.fr

Clinical Study Volunteer
Patients who have been diagnosed as having SCLS and who are at least 16 years old are wanted for participation in the only scientific study of the illness taking place anywhere in the world: at the National Institutes of Health in Bethesda, MD, right outside Washington DC. You must have a documented medical history including at least one acute episode of SCLS or else continuous symptoms of periodic hemoconcentration, hypotension and protein leakage. Have your primary doctor contact Ms. Linda Scott at LMScott@niaid.nih.gov, before sending in the requisite letter of referral with your medical history and laboratory studies to the lead clinical investigator, Dr. Kirk Druey, kdruey@niaid.nih.gov, tel. 301-435-8875. Once accepted into the clinical research study, you will be invited to come to NIH and spend about 4 days there for the purpose of being examined, donating blood, and being subjected to various tests (e.g., clinical digital photography of your blood vessels). Depending on circumstances, you probably will have time off to do sightseeing in the capital area during your stay at NIH. Those who wish to be greeted in person during their stay by this community's founder, please contact aporzeca@american.edu

 

Please see the Disorder Resources section.

Muscle pain Created by DavidS
Last updated 25 Nov 2020, 05:49 PM

Posted by DavidS
25 Nov 2020, 05:49 PM

It's been almost 2 years since my husband has had an episode thanks to ivig every 4 weeks.

He had episodes probably for about 5 years before we understood what was happening and went to the emergency room with his last most severe episode. His swelling was significant but didn't require surgery.

In the past few months he's been suffering with very painful muscles in his buttocks and back of thighs. An orthopedic specialist ruled out an underlying nerve involvement. It seems it's just his muscles. 

I understand that his muscles and nerves may have been damaged by the past episodes, but not sure why this pain has come on more recently rather than right after his hospitalization.

Wondering if others with SCLS have experienced this type of pain, and if so what you're doing to treat it.

Thank you! 

Rebekah Sellers

Any Impact on IVIG availability due to Coronaviris Created by WazzaACT
Last updated 24 Nov 2020, 02:18 PM

Posted by DavidS
24 Nov 2020, 02:17 PM

Hi Cristina, my husband is getting Gamunex C and takes benadryl with it, has little side effects. He actually often goes to work for a few hours after 5 hours of infusion, though I wish he would just rest on those days! HVAC contractor, not a desk job by any means.--Rebekah

Posted by WazzaACT
24 Nov 2020, 05:27 AM

Hi All. Thanks for your inputs. We find ourselves in Australia in  a very fortunate position as we head into Christmas. We basically have zero commuunity transmission with only quarantine cases of Covid 19 from people returning/arriving in Australia. These are numbered in the 2's and 3's a day

In advance of an effective and available vaccine it feels as safe as it can with this virus. My thoughts and best wishes to all in our communnity as we head into Christmas and the prospect of a vaccine in the near future.

Posted by saxamonde
23 Nov 2020, 08:10 PM

I agree with gandcburns:  Thank you everyone for responding. It does help and Happy Thanksgiving to those who live in the States. Here we had our local fiesta last week: San Román, a harvest feast, just like Thanksgiving.but without indians..

View Full Thread (14 more posts)
Protocol Development For Chronic SCLS Created by stedrick
Last updated 1 Nov 2020, 03:26 AM

Posted by aporzeca
1 Nov 2020, 03:26 AM

Susan, cases of chronic SCLS such as yours have proven to be the toughest to crack and resolve.  My suggestion is that you ask your physician -- the one who knows you best -- to reach out to Dr. Kirk Druey at NIH and see if he has any treatment suggestions to make derived from his experience with other cases of chronic SCLS.  He is the repository of whatever knowledge there is, because he has met and followed several patients like you, so if anyone has what could become the elements of a treatment protocol for chronic leakers, he is the one.

I wish I could be of any additional help.

Posted by stedrick
29 Oct 2020, 02:23 AM

Dear Cohorts,

I have the chronic form of SCLS, where the swelling and resolving are milder but persistent and frequent. On Sunday, I experienced a serious flare which involved a weight gain of 9 pounds over 2 days. I am home again and resting.

The good news: the emergency physician looked up SCLS and looked at the protocol in this discussion group.

The bad news: she had no access to information regarding the treatment of a chronic flare where the systemic edema may damage tissue and organs, severe dehydration may occur,  fluid management likely varies from acute SCLS crisis, etc.

Applying the protocol for a full blown SCLS crisis to a chronic patient can be not only unhelpful, but dangerous.

Although the term "protocol" has a more precise medical meaning, I use it to stimulate discussion and perhaps research into an algorithm for the management of this serious form of SCLS that can flare as well as trigger a classic SCLS crisis.

Thank you in advance,

Susan

 

Covid Positive Created by gandcburns
Last updated 30 Sep 2020, 09:02 AM

Posted by DavidS
30 Sep 2020, 09:02 AM

Thanks for sharing your great news with us about your recovery! We hope your full energy returns soon, Rebekah Sellers 

Posted by gandcburns
29 Sep 2020, 09:43 PM

Hello Everyone,

 

Thank you for the well wishes. Unfortunately, I was unable to get my blood sent to Dr. Druey, but my doctor was able to fax him my blood work and test results. I will reach out to Dr. Druey to secure a kit to be able to send him blood if this happens again. I am doing much better. The leak was effectively stopped with an extra treatment. My legs took a few days to return to normal size, but are functioning well. The virus lasted 12 days and zapped me of all energy. I am back to work, but taking the exercise portion slowly. I hope everyone remains healthy and COVID free. 

Cristina

Posted by Caroline uk
25 Sep 2020, 07:05 AM

Hello Cristina, thanks very much for sharing your recent experience, that's good to hear about you doing well with IVIG treatment despite testing positive. I hope and pray you continue to recover well and your family also have a good recovery. Best wishes, Caroline 

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Clinical trial on SCLS Created by Barney
Last updated 30 Aug 2020, 01:18 AM

Posted by aporzeca
30 Aug 2020, 12:02 AM

Having corresponded with Dr. Druey at NIH, I can provide context for this news.  First, to my knowledge, nobody besides Dr. Druey and his team at NIH, and some colleagues he has partnered with in various medical schools, are carrying out biomedical research on SCLS -- or what is best described as primary capillary leak syndrome, because SCLS is not the consequence or side effect of any other bodily illness.  It is a stand-alone phenomenon.

However, there is ongoing research on capillary leaks that are precipitated by other illnesses, like sepsis, certain autoimmune diseases, differentiation syndrome, engraftment syndrome, hemophagocytic lymphohistiocytosis, ovarian hyperstimulation syndrome, viral hemorrhagic fevers, and snakebite and ricin poisoning.  Moreover, there is new research on capillary leaks caused by chemotherapy medications gemcitabine and tagraxofusp, as well as certain interleukins and monoclonal antibodies.  In other words, there is research money and effort going to areas related to secondary capillary leak syndrome, because they affect many more people than those of us who experience SCLS -- cancer patients, in particular.  Therefore, I have been very hopeful that some of that research would provide new insights for Druey and team.

And this announcement is the first example.  Here is a Korean biotech company, PharmAbcine, whose bread-and-butter business is developing new cancer antibody drugs, and they came up with something (a novel Tie2-activating antibody) which apparently inhibits blood vessel leakiness in mouse tumor models.  When they heard that Dr. Druey has also been doing research on capillary leakage using not just mice but also endothelial cells from SCLS patients, they entered into a contract with NIH to jointly determine whether PharmAbcine's new creation might also inhibit vascular leakage using human cells grown from patients with primary capillary leaks.

This is all research that will be carried out in laboratory conditions, so no human trials are envisioned at this point.  If anybody experiencing an episode of SCLS in the United States would like to contribute to this and other research efforts, please have your family or other caregivers request that your doctors make arrangements to deliver your episode blood samples -- namely, the first blood draws upon your arrival to the hospital -- to Dr. Druey via overnight delivery under conditions that he will gladly spell out (e.g., chilled but not frozen blood).  Episode blood cannot otherwise be obtained, and since most of us are not having episodes anymore, it is in very short supply for use in experiments of the kind envisioned in this instance.

Posted by Arielbatt
28 Aug 2020, 02:00 AM

Although as they say it is long term, it seems very hopeful.  2020 has not been a year of good news for the community and this hope fills me with joy.  If these things begin, with a laboratory that puts resources, and a team behind like the NIH, it is because they have many indications that they are on the right track.  Dr. Druey is one of the 2 people who knows the most about our syndrome, that he and his team handle this gives us an added possibility.  And if it does not come to fruition, it will surely help them learn something more about our rare disease.

Posted by HansDeWit
27 Aug 2020, 12:07 PM

I read this article and I think this is very interesting and hopeful BUT as far as I can judge it will take still some long time before there are results.  On the other hand let us be hopeful for a efficient treatment of SCLS!!!

View Full Thread (1 more posts)
IG levels in blood to stay well & sheilding Created by Caroline uk
Last updated 28 Jul 2020, 04:41 PM

Posted by krogers
28 Jul 2020, 04:41 PM

Hi,

Stress has certainly been a trigger for me too as well as viral infections. 

T cell immunity is a seprate way the body fights infection to the production of anbtibodies.  Some scientist are specvulating that the reason for fairly low levels of people with antibodies to covid is due to T cells being the mechanism to fight covid 19 infection.  Its all rather speculative at the moment.  Where you test for the virus when you went into hospital by the normal RT-PCR test?

Best of luck,

Kevin

Posted by Caroline uk
28 Jul 2020, 08:21 AM

Thanks very much for your messages Areilbatt and Kevin. Yes I think I will take the approach you mention and try and live separately inside our home in the first instance and reconsider if the infection rates increase but our children still in school. Kevin your situation does sound tricky with older children at home and also your wife in educaton too. I'm glad to hear you are able to continue working from home and you have good space to isolate as much as possible at home. Yes as my children are young, the risk should not be high, that is something I can bare in mind. The school are also staggering start and finish times which should help with drop off/pick up of the younger ones.

In terms of these episode while on the full recommended amount of IVIG and higher I have a few ideas but not sure. I realise it is very strange as it works well for others. One thing, although I haven't had any Covid symptoms it's possible that I came into contact with it at work. Following my first emergancy hospital admission while on the recommended dose in March, I returned to work from home as my workplace had sent employees home to work. I found out then that a colleague who sits a few metres from me had Covid symptoms and later tested positive for the virus. This is therefore a possible reason I've been having problems although I had felt symptomatic the day before my infusion the month before also. When it became possible to have an antibody test in hospital in June I took the test but this was negative. I understand now that a T cell test is more accurate but I don't think they are available. My only other idea is that it has been quite stressfull in lockdown and I know emotional stresses as well as physical stresses seem to trigger my leaks. My doctor has updated Dr Druey though not made contact with Dr Amoura before, though I have now passed on that contact to him. 

Yes perhaps the IG levels varies with different people. I have had two types of IVIG since I started depending on which is available either gamunex or flebogamma, also my dose had reduced by a bottle after the first 6 months as I'd lost some weight. One thing I have notice through this time is that I know very clearly when a leak is starting. As well as the thirst, heavy/achy arms and legs and feeling tired, my resting heart rate increases. I took my blood presure and heart rate daily over a number of months and for me my resting heart rate is always in the 60/70's. The only time when it went into the 80's was when a leak was starting and if it went into the 90's I know I should be in hospital. My heart rate is rising to support my blood pressure which had been dropping - then my blood pressure goes up a little. Also IVIG is very effective to stop the leak when it starts.  When I went into ICU in March apparently I was in the same condition when I went in the very first time when I nearly died, This time they had started the IVIG treatment and I quickly got better and was out in a few days. I do wonder though if I have been in a cycle that the IG levels in my blood are reduced as it has somehow got used up treating a leak and then not lasting as long to act in a preventative way until my next infusion.

Thanks again for your response.

Best wishes,

Caroline

Posted by krogers
27 Jul 2020, 05:09 PM

Hi Caroline,

I am sorry to hear IVIG has not stabilised your condition.  Is there any clues to what is triggering the episodes?

My children are a little older than yours but I have exactly the same dilemma.  I have no real options to live seperately but will try to isolate as much as possible in our house.  Luckily we have quite a large home with 4 floor levels which will makes this to some extent feasible.  Things are not help my myself and my wife being teachers, although I will be teaching from home.

I think it is now well established that children under 10 years old are weak spreaders of the covid disease as thhey have low levels of the ACE2 receptor in the lungs which the virus uses to enter cells.  Hence the risk is much lower in younger primary school aged children. 

These are really difficult decisons but my youngest got a fever  few days ago and it was really worrying but we have all tested negative for covid but this alone was very stressful. 

I have no idea about the IgG levels although these are monitered but probably varies with the different people.

Best wishes,

Kevin

View Full Thread (3 more posts)
Taking care of grandchildren Created by HansDeWit
Last updated 24 Jul 2020, 04:28 PM

Posted by HansDeWit
24 Jul 2020, 04:28 PM

Hello Arturo and Kevin

Thank you for your reactions. First of all note that I realise that since March this year we are not living any more in a world we used to know. The danger of the Covid virus is everywhere and is not yet over. On the contrary, after a period of a little bit of some optimistic news (infections going down) some people thought the virus was gone and acted like the virus did not exist any more. In Belgium (and I think in several countries  in Europe)  the infection rate is going up again and we all hope that people take their responsibilities again.

For the moment (and this since March) me and my wife only meet very few people and only  ) outside in the garden and keep 2 meters of distance (including family. One of the reasons of my question is that since I was diagnosed SCLS there was hardly a normal time. I left the hospital in December last year and in March there was already a lockdown. So I would like to hear from other patients how they coped with the split between the CSLS disease and meeting litlle grandchildern being a litlle bit sick in non Covid times.

So I keep on being very careful and I hope for a solution for this Covid-19 soon, although I realise that a vaccine will not be available very soon. So hoping for better times.... 

Posted by krogers
24 Jul 2020, 05:59 AM

Hans,

We are not in normal times - Covid is still spreading in society and will almost certainly increase as we go into winter and more people return to school & work as well as spend more time indoors.  Belgium and the UK are both faired badly in terms of the spread of this disease and some parts of the US are heading for very high levels right now.  Have a look at Our world in data website for the infection rates in different countries.  Infection rate are still significant.

Schools in the UK plan to open full time and full capacity in September.  I have spoken to friends & colleagues who taught whilst schools partly reaopened in June/July - social diatancing of children simply does not happen deapite continual reminders.  I  am a teacher and was very worried about returning but my school has agreed for me to continue teaching from home.

I cannot beleive you are considering meeting up with grandchildren when schools reopen without social distancing - for me its completely crazy.  When my chlidren go back to school I will be isolating myself from them as strickly as practicaly possible.  Not easy but worth it to ensure I don't get this virus.  Remember as I understand it we are probably oner of the most vunerable groups of people as not only can the covid-19 disease kill us but it can also trigger an attack of our condition.

Needless to say I am very caustious.

Best wishes,

Kevin

 

 

Posted by aporzeca
23 Jul 2020, 09:41 PM

Hans, your question is an important one, especially now given the Covid-19 pandemic.  Before the pandemic, we helped take care of our grandchildren even if they were sick. As a result, we often caught their colds and got sick ourselves, but we weren't afraid of my having an episode of SCLS because my full dose (2 gr/kg/month) of IVIG had always protected me.

In the meantime, my older brother (he is 77 years old) and his wife, who are both retired physicians and they don't have any serious, underlying medical condition -- never mind SCLS -- had a policy that they would not visit or host their grandchildren if they had a cold or other sickness. 

But then in January my wife came down with Influenza A and she gave it to me, though it was not the fault of our grandchildren, and even though we both had been vaccinated against the flu, but this time it triggered my first episode of SCLS in more than 10 years, see https://rareshare.org/topics/1967  Therefore, even if there was no Covid-19 pandemic, now we would probably not see our grandchildren if they were sick.

One of the few good things that have come out of the pandemic is that during these months of strict isolation, our grandchildren have not been sick, because they have not been playing with other children. Therefore, we have spent a lot of time with them and we have also not come down with any sicknesses.  We have enjoyed this togetherness very much.

The dilemma we face is what are we going to do, as a family, if schools reopen in a month's time, even if for two days a week, rotating smaller groups of children so that they can keep their distance from other children?  Will our grandchildren start to pick up colds and other childhood illnesses once again -- never mind Covid-19 -- despite the best efforts of schools and parents?  Should we stop seeing them for a month or two, until we see what happens?  It is a very tough decision.

I don't know about you, but spending time with our grandchildren keeps me motivated to live on.

Arturo

View Full Thread (3 more posts)
Looking for medical specialists SCLS in the Netherlands Created by Sas
Last updated 11 Jul 2020, 02:10 AM

Posted by aporzeca
11 Jul 2020, 02:08 AM

Since there are no experts on SCLS in Holland or most countries, my earnest recommendation is that your pediatrician make an appointment for your daughter to be seen by Europe's leading expert on SCLS:

Prof. Dr. Zahir Amoura, Département de Médecine Interne, Hôpital de la Pitié-Salpêtrière, Bd. de l'Hôpital 83, Paris 13e, tél. 0033 1 4217 8001,
email zahir.amoura@aphp.fr, and for appointment information see https://www.aphp.fr/offre-de-soin/medecin/505439/066/04

He is often overbooked and thus hard to see, but he has a younger colleague that she could otherwise see: Dr. Marc Pineton de Chambrun, tel 0033 1 4217 7832, email marcdechambrun@gmail.com, and for appointment information see https://www.aphp.fr/offre-de-soin/medecin/3280725/066/59

To make the eventual trip to Paris most productive, make sure that your pediatrician prepares a summary in English or French of your daughter's medical history, to be emailed ahead of time to Dr. Amoura or Dr. de Chambrun, and/or that she takes it with her to her appointment.

Arturo

Posted by AndreasGunsser
9 Jul 2020, 02:50 PM

Hi,

I know a doctor in Munich, Germany, He speaks English very well.

If you cannot find someone in the Netherlands or abroad, but closer, please contact me via agunsser@gmx.de.

 

Andreas

Posted by Sas
8 Jul 2020, 02:08 PM

Hi,

The pediatrician of my daughter thinks there is a possibility she might have SCLS and would like to come in contact with specialists of SCLS in the Netherlands (or abroad). Her first episode happened when she was 6 years old, which looked like a anaphylactic shock, she was swollen. Last year she had 2 episodes of presumed septic shocks, with one ICU. She is now 16 years old. 
Please let me know if you have got some information, thank you.

Starting IVIG. What to expect??? Created by Di_dee71
Last updated 1 Jul 2020, 11:56 AM

Posted by Di_dee71
1 Jul 2020, 11:56 AM

Many thanks to you all for your reply's. This site and the lovely people on it are life savers. 

It has been great having this information to prepare. I still feel a little unprepared as I read the reply's a little late. I have no one to blame but myself. And late is better than never :-). I have had a couple of days with good fluid intake and will take some headache relief before I start. I have a good book, salty snacks and earbuds charged :-). 

Tomorrow is the big day. It is so encouraging reading how well this has worked for others. It is also very encouraging to hear that it is worth it. I suffer from intercranial pressure already due to having Arnold Chiari Malformation, so I am quite nervous about headaches and migraines. 

I am hoping to return to work on the 20th of July after having the 5 months not being able to work at all. Here is hoping, I also will be able to stop the diuretics. Which at the moment are the only things that are keeping me from total incapacity and being stuck in bed or hospital?

I will be back on shortly to learn more and update you all on how it has gone.

Posted by kmiclon
26 Jun 2020, 08:05 PM

Hi,

I have been getting IVIG for a year now, every 8 weeks and felt pretty unprepared - this site has been immensely helpful.  I seem to react differently every time, the first was my worst because I did not know how to prepare, and when I asked at the infusion center and my Dr, they said people don't usually have a reaction.  I usually have a heavy, dull headache the afternoons of 2 day treatment, diarrhea starting during the 1st day;  the 2nd day may turn into migraine, vomiting, and have had times where I felt hungover for several days. It can also be fine one day and fall back to the hangover (did I drop off my fluid intake. . . ?)

From this site, I have learned to greatly increase fluid intake 3-4 days before, during and after treatment.  I pre-medicate with benadryl and tylenol 1/2 hour before treatment starts, and then at bed again, just in case. I sometimes will continue taking the tylenol for a few days to attempt to fend off headaches (I tend to wake up with them).  I try to eat as simply and cleanly as possible, and get extra sleep.  I see an Integrative Health Specialist and she has recommended a magnesium (chelate) supplement a week before and during, which does seem to help.  I also have nausea bracelets that I put on before treatment begins. (trying to cover all my bases :-)

With all of this being said, it is worth EVERYTHING - I had a chronic leak for 10 years, ER visit every 12-18 months for dehydration, then a very different episode in February 2019 (just updated in my profile) which put me in ICU for almost a week.  Since I started IVIG, no episodes and no need for a diuretic at all - I feel so much better, not constantly puffed out and bloated.  My feeling is that learning to prep for/cope with/manage these side effects is a good way to take care of me and (maybe) feel a little more in control of my health.

Very best of luck to you -

Kim

Posted by lisamccoleman
21 Jun 2020, 04:42 PM

Hello there... I have been on IVIG for 8 years now and gone from a monthly infusion to bi-weekly to now trying SCIG because of COVID and the side effects I have had with infusions.  With my bi-weekly infusions, I would take 20mg reactine & 500 mg tylenol at treatment. As well, my specialist had prescribed a low dose of steroid to help with the side effects that was given through IV which I was slowly weening off over the years. Drinking water prior to treatment, through treatment, and after treatment was suggested and I found it very helpful. My product was infused slowly at a rate of 200 ml/hour and I also had  D5W infuse with it to try and help with side effects. It seemed to help a bit, I think. For me the side effects were flu-like symptoms for a few days after treatment and then a migraine would set in for 2-3 days.  But for many people on this site, they have very few side effects. I hope you are one of them. I suggest you take a good book, download your favourite music (I actually had a treatment playlist), take salty snacks, water, and have a good set of earbuds that can cancel out the noise around you if needed. 

I hope this helps...Thinking of you...

Lisa

View Full Thread (2 more posts)
Any adverse reactions to anesthetic Created by WazzaACT
Last updated 27 Jun 2020, 04:54 AM

Posted by WazzaACT
27 Jun 2020, 04:54 AM

Hi John. I am fine but of course a little on edge given this coronovirus outbreak. We can count ourselves fortunate in Australia that we have so little community transmission and an excellent medical infrastructure for all. How about you? Warren

 

Posted by Jcarson
23 Jun 2020, 10:09 PM

Hello Wazza,   I hope all is well.  I have had a couple of light aneasthetics for coronary angiograms etc.  really only midazolam, but had to be prepared for GA if things went pear shaped.  Absolutely no trouble.   I hope corona is leaving you and all of the rareshare group alone?    John

Posted by krogers
18 Jun 2020, 12:02 PM

I had a few general anesthetics when my legds needed stitching up  after  Fasciotomies without problems. 

View Full Thread (5 more posts)
Community News Articles
Default article

Hospitals, Doctors, Medical Teams: Navigating Barriers of Rare Diseases (Patient Navigation)

Publication date: 12 Sep 2016

Community: Systemic Capillary Leak Syndrome

Podcast

Arturo Porzecanski, a rare disease patient and advocate, gives us some tips on navigating decisions involved in choosing hospitals, doctors, and medical teams.
Featuring Arturo Porzecanski (American University). (Music credit:www.bensound.com)


Community Resources
Title Description Date Link
A natural mouse model reveals genetic determinants of SCLS

Abstract: SCLS is a disorder of unknown etiology characterized by recurrent episodes of vascular leakage of proteins and fluids into peripheral tissues, resulting in whole-body edema and hypotensive shock. The pathologic mechanisms and genetic basis for SCLS remain elusive. Previous histological studies of skin and muscle of SCLS patients have failed to uncover gross abnormalities within the microvasculature that could account for this phenotype. Here the authors identified an inbred mouse strain, SJL, which recapitulates cardinal features of SCLS, including susceptibility to histamine- and infection-triggered vascular leak. They named this trait “Histamine hypersensitivity” (Hhs/Hhs) and mapped it to Chromosome 6. Hhs is syntenic to the genomic locus most strongly associated with SCLS in humans (3p25.3), revealing that the predisposition to develop vascular hyperpermeability has a strong genetic component conserved between humans and mice and providing a naturally occurring animal model for SCLS. Genetic analysis of Hhs may reveal orthologous candidate genes that contribute not only to SCLS, but also to normal and dysregulated mechanisms underlying vascular barrier function more generally. Future studies including assessment of expression and sequence of top Hhs candidate genes in SCLS patients and mice and their role in endothelial responses to inflammation will be essential to determine their contribution.

11/18/2019
Capillary leak syndrome: etiologies, pathophysiology, and management

Capillary leak syndrome: etiologies, pathophysiology, and management

Abstract: In various human diseases, an increase in capillary permeability to proteins leads to the loss of protein-rich fluid from the intravascular to the interstitial space. Although sepsis is the disease most commonly associated with this phenomenon, many other diseases can lead to a “sepsis-like” syndrome with manifestations of  diffuse pitting edema, exudative serous cavity effusions, noncardiogenic pulmonary edema, hypotension, and, in some cases, hypovolemic shock with multiple-organ failure. The term capillary leak syndrome has been used to describe this constellation of disease manifestations associated with an increased capillary permeability to proteins. Diseases other than sepsis that can result in capillary leak syndrome include the idiopathic systemic capillary leak syndrome or Clarkson’s disease, engraftment syndrome, differentiation syndrome, the ovarian  hyperstimulation syndrome, hemophagocytic lymphohistiocytosis, viral hemorrhagic fevers, autoimmune diseases, snakebite envenomation, and ricin poisoning. Drugs including some interleukins, some monoclonal antibodies, and gemcitabine can also cause capillary leak syndrome. Acute kidney injury is commonly seen in all of these diseases. In addition to hypotension, cytokines are likely to be important in the pathophysiology of acute kidney injury in capillary leak syndrome. Fluid management is a critical part of the treatment of capillary leak syndrome;  hypovolemia and hypotension can cause organ injury, whereas capillary leakage of administered fluid can worsen organ edema leading to progressive organ injury. The purpose of this article is to discuss the diseases other than sepsis that produce capillary leak and review their collective pathophysiology and treatment.

03/24/2017
Chronic SCLS treatment with IVIG: Case & literature

Chronic systemic capillary leak syndrome treatment with intravenous immune globulin: Case report and review of the literature

Abstract: A rarely described chronic form of SCLS (cSCLS) presents as refractory edema, with pleural and/or pericardial effusions and hypoalbuminemia. These entities are differentiated by massive and periodic episodes of capillary leak, which can result in shock in SCLS, and chronic refractory edema in cSCLS. The etiologies of these disorders are poorly understood, but both acute and chronic forms often present with an associated monoclonal gammopathy. Flares of the SCLS have been reduced by treatment with intravenous immune globulin (IVIG). Only six cases of cSCLS have been reported, and previous treatments have included steroids, terbutaline, and theophylline. Based upon the reported responses of SCLS to IVIG, we present the case of a 54-year-old man with cSCLS where ongoing treatment with IVIG resulted in a marked and sustained improvement in the signs and symptoms of the capillary leak syndrome.

03/17/2019
Clinical Presentation, Management, and Prognostic Factors of SCLS

Clinical Presentation, Management, and Prognostic Factors of Idiopathic Systemic Capillary Leak Syndrome: A Systematic Review

Abstract: A total of 133 case reports (161 patients) and 5 case series (102 patients) of idiopathic SCLS were included in a survey of articles published through end-2016. The findings include that patients had hypotension (81.4%), edema (64.6%), and previous flu-like illness (34.2%). They were often misdiagnosed as having hypovolemic shock, septic shock, polycythemia vera, or angioedema. Thirty-seven patients died (23%) mainly because of complications from SCLS (78.4%). There were significant differences in the survival rates between patients who were treated with prophylactic b2 agonists, methylxanthines, and intravenous immunoglobulins and those who were not. The estimated 1-, 5-, and 10-year survival rate of patients treated with intravenous immunoglobulins was 100%, 94%, and 94%, respectively. The results of this review suggest that prophylactic use of intravenous immunoglobulins is the most effective treatment in reducing the mortality rate of SCLS patients.

11/04/2018
Comment on SCLS

Comment on The Systemic Capillary Leak Syndrome.

Abstract: The authors report on 2 additional patients from the United States with SCLS in whom prophylaxis with terbutaline and theophylline failed, but who had no further episodes after the initiation of IVIG therapy. There are additional published reports of successful prophylaxis with IVIG cited, and the authors are also aware of yet another case. Given the present state of knowledge and despite the high cost, the authors strongly believe that IVIG is the optimal prophylaxis and should be the initial choice to prevent attacks in patients with SCLS.

03/23/2017
Genome-Wide SNP Analysis of SCLS

Genome-Wide SNP Analysis of SCLS.

Abstract: Polymorphisms in genes whose functional annotations suggest involvement in cell junctions and signaling, cell adhesion, and cytoskeletal organization, correlate with our previous mechanistic studies of SCLS sera. Such annotations provide a framework for future allelic discrimination strategies to validate top-ranked SNPs discovered here, as well as novel SNPs unique to the SCLS cohort detected by exome capture sequencing. Although the findings must be corroborated in a larger cohort, they provide a springboard for discovery of underlying pathophysiological mechanisms, biomarkers, and avenues for therapy.

03/23/2017
Handling Shock in SCLS: Less Is More

Handling shock in idiopathic systemic capillary leak syndrome (Clarkson’s disease): less is more

Abstract: SCLS presents with recurrent potentially life-threatening episodes of hypovolemic shock associated with severe hemoconcentration and hypoproteinemia. Here the authors summarize 40 years’ experience in treating shock in Italian SCLS patients to derive a therapeutic algorithm. Records from 12 patients were informative for treatment modalities and outcome of 66 episodes of shock. Episodes are divided in 3 phases and treatment recommendations are the following: (1) prodromal symptoms-signs (growing malaise, oligo-anuria, orthostatic
dizziness) last 6-12 hours and patients should maintain rigorous bed rest. (2) The acute shock phase lasts 24-36 hours; patients should be admitted to ICU, placed on restrictive infusion of fluids favoring cautious boluses of high-molecular-weight plasma expanders when SAP < 70 mmHg; and monitored for cerebral/cardiac perfusion, myocardial edema and signs of compartment syndrome. (3) The post-acute (recovery) phase may last from 48 hours to 1 week; monitor for cardiac overload to prevent cardiac failure; in case of persistent renal failure, hemodialysis may be necessary; consider albumin infusion. Complications listed by frequency in our patients were acute renal failure, compartment syndrome and neuropathy, rhabdomyolysis, myocardial edema, pericardial-pleural-abdominal effusion, cerebral involvement, acute pulmonary edema and deep vein thrombosis.

07/09/2019
High-Dose IVIG Therapy for SCLS

High-Dose IVIG Therapy for SCLS.

Abstract: We evaluated IVIG prophylactic therapy in a cohort of 29 patients with Systemic Capillary Leak Syndrome in a longitudinal follow up study. All patients received treatments at the discretion of their primary providers and retrospectively via questionnaire recorded symptoms beginning with their first documented episode of the SCLS until May 31, 2014. Twenty-two out of 29 patients responded to the questionnaire, and 18 out of the 22 respondents received monthly prophylaxis with IVIG during the study period for a median interval of 32 months. The median annual attack frequency was 2.6/patient prior to IVIG therapy and 0/patient following initiation of IVIG prophylaxis (P = 0.001). 15 out of 18 subjects with a history of one or more acute SCLS episodes experienced no further symptoms while on IVIG therapy. In conclusion, IVIG prophylaxis is associated with a dramatic reduction in the occurrence of SCLS attacks in most patients, with minimal side effects.

03/23/2017
Idiopathic SCLS (Clarkson syndrome) in childhood

Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review

Abstract: The authors performed a systematic review of the literature on Clarkson syndrome in subjects less than 18 years of age, and identified 24 reports, published since 1989, providing data on 32 otherwise healthy subjects, who experienced 67 well-documented episodes of SCLS. The condition affected more frequently girls (21, 66%) than boys, presented throughout childhood, and was preceded by a mostly viral illness in 75% of cases. The presence of a monoclonal gammopathy (MGUS) was never reported. Uncompensated circulatory shock, muscle compartment syndrome, acute kidney injury, pulmonary edema, and either pleural or pericardial effusion were, in decreasing order of frequency, the most common complications. Four patients died. In sum, SCLS develops not only in adulthood but also in childhood, but of potential significance is that in this age group the condition is not linked to an MGUS, and thus it could be that it does not play a pivotal pathogenic role.

11/04/2018
Idiopathic systemic capillary leak syndrome (Clarkson disease)

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Abstract: The enigmatic systemic capillary leak syndrome (SCLS) named for Dr Clarkson is characterized by transient and severe but reversible hemoconcentration and hypoalbuminemia caused by leakage of fluids and macromolecules into tissues. Although less than 500 cases of SCLS have been reported in the literature since 1960, the condition is probably underdiagnosed because of a lack of awareness and a high mortality without treatment. Treatment of acute SCLS remains primarily supportive. Prophylaxis with IVIG appears promising, but this therapy is nonspecific and expensive. Mechanistic understanding of SCLS is in its infancy. As a result, clinicians today cannot predict when or how badly SCLS will flare; targeted therapies do not yet exist, and prolonged remission or cure remains elusive. Our working hypothesis invokes exaggerated microvascular endothelial responses to surges of otherwise routinely encountered inflammatory mediators. This emerging disease model lends itself to innovative patient-centered translational research in the ways highlighted above. It is our hope that detailed and personalized investigation of intraendothelial responses among individual patients with SCLS might illuminate novel genetic and molecular control mechanisms. In turn, such advances could deliver the diagnostic, prognostic, and therapeutic tools sorely needed to combat this devastating disease.

03/23/2017
Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated SCLS

Intravenous Immunoglobulins Improve Survival in Monoclonal Gammopathy-Associated SCLS

Abstract: We conducted a cohort analysis of all patients included in the European Clarkson disease registry between January 1997 and March 2016. From diagnosis to last follow-up, studied outcomes (e.g., the frequency and severity of attacks, death, and evolution toward multiple myeloma) and the type of preventive treatments administered were monitored every 6 months. Sixty-nine patients (M/F sex ratio 1:1; mean ± SD age at disease onset 52 ± 12 years) were included in the study. All patients had monoclonal gammopathy of immunoglobulin G type, with kappa light chains in 47 (68%).Twenty-four patients (35%) died after 3.3 (0.9-8) years. Fifty-seven (86%) patients received at least one preventive treatment, including intravenous immunoglobulins (IVIg) n = 48 (73.8%), theophylline n = 22 (33.8%), terbutaline n = 22 (33.8%), and thalidomide n = 5 (7.7%). In the 65 patients with follow-up, 5- and 10-year survival rates were 78% (n = 35) and 69% (n = 17), respectively. Preventive treatment with IVIg and terbutaline were the only factors significantly associated with survival in multivariate analysis. Neither the use of thalidomide nor theophylline was associated with improved survival. Five- and 10-year survival rates in patients treated with IVIg were 91% and 77%, respectively, compared to 47% and 37% in patients not treated with IVIg. Patients treated with IVIg were more likely to be free of recurrence, severe recurrence, and alive at the end of follow-up. Furthermore, all but one patient who did not experience a severe relapse were treated with IVIg. Since preventive treatment with IVIg was the strongest factor associated with survival, the use of IVIg is suggested as the first line in prevention therapy.

07/05/2017
IVIG as Treatment for SCLS

High-Dose Intravenous Immunoglobulins Dramatically Reverse Systemic Capillary Leak Syndrome.

Abstract: The objective of this study was to report the dramatic improvement of patients with systemic capillary leak syndrome obtained with high-dose intravenous immunoglobulins. Systemic capillary leak syndrome is a rare and life-threatening disorder characterized by hypotension that can lead to shock, weight gain, hypoalbuminemia, and elevated hematocrit secondary to unexplained episodic capillary fluid extravasation into the interstitial space. Because its cause is unknown, systemic capillary leak syndrome treatment has remained largely supportive. Intravenous immunoglobulins administration to a patient with refractory systemic capillary leak syndrome yielded dramatic improvement. The patient is still alive 11 yrs after systemic capillary leak syndrome diagnosis and receives intravenous immunoglobulins monthly. Later, based on that result, intravenous immunoglobulins were successfully given to two other patients during the acute phase of systemic capillary leak syndrome. Both are still alive 8 and 1.5 yrs after receiving intravenous immunoglobulins at the onset of each flare. In conclusion, intravenous immunoglobulins were effective against systemic capillary leak syndrome symptoms in three patients, but their exact mechanism remains unknown. Their immunomodulatory effect merits further investigation.

03/23/2017
IVIG as Treatment for SCLS

Immunoglobulins for Treatment of Systemic Capillary Leak Syndrome

Abstract: A 43-year-old white woman in France diagnosed with SCLS was put on the recommended combination of Theophylline plus Terbutaline, but she nevertheless had 10 episodes of severe capillary leak during 2001-mid-2007, necessitating intensive care unit admission for her last 3 episodes. She was then put on IVIG administered every 6 weeks, and this yielded a dramatic improvement such that she has had no more episodes and has returned to her normal lifestyle.

03/23/2017
IVIG in SCLS: A Case Report and Review of Literature

IVIG in SCLS: Report and Review of Literature.

Abstract: In recent years, IVIG has become a common first-line prophylactic therapy in most patients with benefits at the dose of 2 gr/kg once a month. Here the authors report the case of a 49-year-old male patient in Italy -- he is a member of this community -- with SCLS treated successfully with a lower dose of IVIG (1 gr/kg monthly) in the maintenance phase. He presented no acute episodes in a follow-up period of 28 months. The authors describe prophylactic treatments for SCLS in the literature and compare their patient to another 18 who received IVIG in follow-up.

03/23/2017
IVIG: A Promising Approach to SCLS

High-dose intravenous immunoglobulins: A promising therapeutic approach for idiopathic systemic capillary leak syndrome.

Abstract: The article reports the case of a 40-year-old woman with chronic SCLS treated in Berne, Switzerland, with high-dose intravenous immunoglobulins (IVIG) after a prophylactic therapy with theophylline and terbutaline (T&T) was poorly tolerated and failed to decrease the frequency and severity of the attacks. During the 5 years she was on T&T the patient suffered from about 20 similar episodes of mild to moderate shock, often requiring hospital re-admission and supportive therapy. So far, 10 months of prophylactic therapy with IVIG (2gr/kg/month) have resulted in an impressive reduction of intensity and frequency of attacks, confirming the finding of other case studies.

03/23/2017
Laboratory Evidence of SCLS and of the Effectiveness of IVIG

Vascular Endothelial Hyperpermeability Induces The Clinical Symptoms of Clarkson Disease (The Systemic Capillary Leak Syndrome)

Abstract: The authors report clinical and molecular findings on 23 subjects, the largest SCLS case series to date. Application of episodic SCLS sera, but neither the purified immunoglobulin fraction nor sera obtained from subjects during remission, to human microvascular endothelial cells caused vascular endothelial cadherin (VE-cadherin) internalization, disruption of inter-endothelial junctions, actin stress fiber formation, and increased permeability in complementary functional assays without inducing endothelial apoptosis. Intravenous immunoglobulin (IVIG), one promising therapy for SCLS, mitigated the permeability effects of episodic sera directly. Consistent with the presence of endogenous, non-immunoglobulin, circulating permeability factor(s) constrained to SCLS episodes, we found that two such proteins, vascular endothelial growth factor (VEGF) and angiopoietin 2 (Ang2), were elevated in episodic SCLS sera but not in remission sera. Antibody-based inhibition of Ang2 counteracted permeability induced by episodic SCLS sera. Comparable experiments with anti-VEGF antibody (bevacizumab) yielded less interpretable results, likely due to endothelial toxicity of VEGF withdrawal. Our results support a model of SCLS pathogenesis in which non-immunoglobulin humoral factors such as VEGF and Ang2 contribute to transient endothelial contraction, suggesting a molecular mechanism for this highly lethal disorder.

03/23/2017
Lessons from 28 European Patients with SCLS

The Systemic Capillary Leak Syndrome: A Case Series of 28 Patients From a European Registry.

Abstract: The article describes the clinical characteristics, laboratory findings, treatments, and outcomes of patients with SCLS who were not previously reported in the literature. These European patients with SCLS were treated and monitored from the start of 1997 until end-July 2010. Survival rates were 89% at 1 year and 73% at 5 years; instances of death were directly related to SCLS attacks in 6 cases (75% of total). Treatments of various kinds increased the chances of survival: Five years after diagnosis, survival rates were 85% in 23 patients who had received a treatment and just 20% in 5 patients who had not. The authors provide additional evidence that a prophylactic treatment with IVIG tends to reduce the frequency and severity of attacks, and may improve the survival of patients with SCLS.

03/23/2017
Mayo Clinic write-up on SCLS

The Mayo Clinic's summary of the diagnosis and treatment of SCLS.

During an episode of systemic capillary leak syndrome, fluids are administered intravenously to maintain the patient's blood pressure and to prevent damage to vital organs such as the kidneys, heart and brain. The amount of fluid must be carefully controlled. An attempt to normalize blood pressure through aggressive fluid administration can cause destructive swelling of the body's extremities and overload the kidneys and lungs when the body needs to eliminate the excess fluids after the episode passes.

Glucocorticoids (steroids) are often injected during an acute capillary leak syndrome attack to reduce or stop the capillary leak. This is sometimes successful. Fluid pressure in muscles may be monitored. Emergency surgery may be needed to relieve pressure and minimize damage to muscles and nerves in the arms and legs.

Once the capillary walls stop leaking and fluids start to be reabsorbed, patients are usually given diuretics to speed up elimination of the fluids before they accumulate in the lungs and other vital organs, which can be a fatal complication.

Patients who avoid organ and limb damage in a capillary leak syndrome episode tend to recover their health after several days, once the capillary walls return to normal and the accumulated fluid is expelled from the body through urination.

Although no cure has been found for systemic capillary leak syndrome, the frequency and/or severity of episodes is often reduced by having patients take certain asthma medications: theophylline and terbutaline. Patients also may benefit from intravenous treatment with immunoglobulin or by taking thalidomide.

Patients may also be prescribed corticosteroid pills such as prednisone to be taken at the first sign of symptoms of another capillary leak.

03/23/2017
Mechanistic Classification of SCLS

Mechanistic Classification of SCLS.

Abstract: The authors analyzed circulating mediators of vascular permeability and proinflammatory cytokines in acute episodic sera from 14 patients with SCLS, and sera from 37 healthy control subjects. They monitored barrier function of human microvascular endothelial cells (HMVEC) after treatment with SCLS sera using transendothelial electrical resistance assays. Consistent with their previous study, the permeability factor vascular endothelial growth factor (VEGF) was increased in sera from acutely ill subjects with SCLS. An analysis of samples from one SCLS patient who has not responded to any preventive therapies (and who is a member of this Community), suggests that SCLS may have clinically varying forms, and that within the group of patients with SCLS, different cytokines may mediate the capillary leak. Therefore, quantitative molecular and humanized cell-based assays for humoral mediators of permeability should improve diagnostic specificity for SCLS and enable clinicians to screen for effective therapies.

03/23/2017
SCLS in Children

Idiopathic Systemic Capillary Leak Syndrome in Children

Abstract: Adult subjects with systemic capillary leak syndrome (SCLS) present with acute and recurrent episodes of vascular leak manifesting as severe hypotension, hypoalbuminemia, hemoconcentration, and generalized edema. We studied clinical disease characteristics, serum cytokine profiles, and treatment modalities in a cohort of children with documented SCLS. Six children with SCLS were recruited from the United States, Australia, Canada, and Italy. Serum cytokines from SCLS subjects and a group of 10 healthy children were analyzed. Children with SCLS (aged 5-11 years old) presented with at least 1 acute, severe episode of hypotension, hypoalbuminemia, and hemoconcentration in the absence of underlying causes for these abnormalities. In contrast to what is observed in adult SCLS, identifiable infectious triggers precipitated most episodes in these children, and none of them had a monoclonal gammopathy. We found elevated levels of chemokine (C-C motif) ligand 2 (CCL2), interleukin-8, and tumor necrosis factor α in baseline SCLS sera compared with the control group. All patients are alive and well on prophylactic therapy, with 4 patients receiving intravenous or subcutaneous immunoglobulins at regular intervals. The clinical manifestations of pediatric and adult SCLS are similar, with the notable exceptions of frequent association with infections and the lack of monoclonal gammopathy. Prophylactic medication, including high dose immunoglobulins or theophylline plus verapamil, appears to be safe and efficacious therapy for SCLS in children.

03/23/2017
Sharing the Pain [of living with SCLS]

Sharing the Pain [of living with SCLS]

This article from The Washington Post newspaper tells the story of how this SCLS virtual community was created, the story of its founder and, more generally, of this fantastic RareShare site.

 

03/23/2017
Successful Treatment of SCLS with IVIG

Successful Treatment of Systemic Capillary Leak Syndrome with Intravenous Immunoglobulins.

Abstract: The authors report on a 48-year-old woman in Spain who had her 1st episode of SCLS in 1997 and was initially put on a regimen of terbutaline and aminophylline, but went on to endure 20 additional episodes in the subsequent 3 years. She was then treated with melphalan-prednisone for a year and the frequency and intensity of her episodes diminished and even disappeared. In 2005, however, the episodes returned and in 2008 she was finally put on a regimen of IVIG (2 g/kg) every 6 weeks. She has had no more episodes since then.

03/23/2017
Systemic capillary leak syndrome: recognition prevents morbidity and mortality

Systemic capillary leak syndrome: recognition prevents morbidity and mortality.

Abstract: The authors report on a case of SCLS in Australia involving a 61-year-old male who was properly diagnosed after his third episode, to increase awareness of the condition and to highlight the benefits of prophylactic intravenous immunoglobulin (IVIG) in this condition. The diagnosis was made by exclusion and clinically by a classic triad of hypotension, hypoalbuminaemia and haemoconcentration. There have been recent advances in understanding the pathophysiological basis for SCLS and in effective prophylaxis, and the authors and patient benefitted from said advances.

03/23/2017
The Clinical Picture of Severe SCLS Episodes Requiring ICU Admission

The Clinical Picture of Severe SCLS Episodes Requiring ICU Admission

Abstract: SCLS is a very rare cause of recurrent hypovolemic shock. Few data are available on its clinical manifestations, laboratory findings, and outcomes of those patients requiring ICU admission.  This study was undertaken to describe the clinical pictures and ICU management of severe SCLS episodes.  This multicenter retrospective analysis concerned patients entered in the European Clarkson's disease (EurêClark) Registry and admitted to ICUs between May 1992 and February 2016.  Fifty-nine attacks occurring in 37 patients (male-to-female sex ratio, 1.05; mean ± SD age, 51 ± 11.4 yr) were included.  Among 34 patients (91.9%) with monoclonal immunoglobulin G gammopathy, 20 (58.8%) had kappa light chains.  ICU-admission hemoglobin and proteinemia were respectively median (interquartile range) 20.2 g/dL (17.9-22 g/dL) and 50 g/L (36.5-58.5 g/L).  IVIG was infused during 15 episodes (25.4%).  A compartment syndrome developed during 12 episodes (20.3%).  Eleven (18.6%) in-ICU deaths occurred. Bivariable analyses (the 37 patients' last episodes) retained Sequential Organ-Failure Assessment score greater than 10 (odds ratio, 12.9 [95% CI, 1.2-140]; p = 0.04) and cumulated fluid-therapy volume greater than 10.7 L (odds ratio, 16.8 [1.6-180]; p = 0.02) as independent predictors of hospital mortality.  In conclusion, high-volume fluid therapy was independently associated with poorer outcomes.  IVIG use was not associated with improved survival; hence, its use in an ICU setting should be considered prudently and needs further evaluation in future studies.

07/05/2017
The Mayo Clinic Experience with SCLS

Idiopathic Systemic Capillary Leak Syndrome (Clarkson's Disease): The Mayo Clinic Experience

Abstract: Of the 34 patients whose records were reviewed, 25 fulfilled all diagnostic criteria for SCLS. The median age at diagnosis of SCLS was 44 years. Median follow-up of surviving patients was 4.9 years, and median time to diagnosis from symptom onset was 1.1 years (interquartile range, 0.5-4.1 years). Flulike illness or myalgia was reported by 14 patients (56%) at onset of an acute attack of SCLS, and rhabdomyolysis developed in 9 patients (36%). Patients with a greater decrease in albumin level had a higher likelihood of developing rhabdomyolysis (P=.03). Monoclonal gammopathy, predominantly of the IgG-kappa type, was found in 19 patients (76%). The progression rate to multiple myeloma was 0.7% per person-year of follow-up. The overall response rate to the different therapies was 76%, and 24% of patients sustained durable (>2 years) complete remission. The estimated 5-year overall survival rate was 76% (95% confidence interval, 59%-97%). In conclusion, SCLS, a rare disease that occurs in those of middle age, is usually diagnosed after a considerable delay from onset of symptoms. The degree of albumin decrement during an attack correlates with development of rhabdomyolysis. A reduction in the frequency and/or the severity of attacks was seen in nearly three-fourths of patients who were offered empiric therapies. The rate of progression to multiple myeloma appears to be comparable to that of monoclonal gammopathy of undetermined significance.

03/23/2017
The Systemic Capillary Leak Syndrome

Narrative review: the systemic capillary leak syndrome

Abstract: The systemic capillary leak syndrome (SCLS) is a rare disease of reversible plasma extravasation and vascular collapse accompanied by hemoconcentration and hypoalbuminemia. Its cause is unknown, although it is believed to be a manifestation of transient endothelial dysfunction due to endothelial contraction, apoptosis, injury, or a combination of these. Fewer than 150 cases of SCLS have been reported, but the condition is probably underrecognized because of its nonspecific symptoms and signs and high mortality rate. Patients experience shock and massive edema, often after a nonspecific prodrome of weakness, fatigue, and myalgias, and are at risk for ischemia-induced organ failure, rhabdomyolysis and muscle compartment syndromes, and venous thromboembolism. Shock and edema reverse almost as quickly as they begin, at which time patients are at risk for death from flash pulmonary edema during rapid fluid remobilization. Diagnosis is made clinically and by exclusion of other diseases that cause similar symptoms and signs, most notably sepsis, anaphylaxis, and angioedema. Acute episodes are treated with vasopressor therapy and judicious fluid replacement, possibly with colloid solutions for their osmotic effects, to prevent the sequelae of underperfusion. Prognosis is uncertain, but patients who survive an initial severe SCLS episode are estimated to have a 10-year survival rate greater than 70%. Much remains to be learned about SCLS, and clinicians should consider the diagnosis in patients with unexplained edema, increased hematocrit, and hypotension.

03/23/2017
Whole Exome Sequencing of SCLS Patients

Whole Exome Sequencing of Adult and Pediatric Cohorts of the Rare Vascular Disorder Systemic Capillary Leak Syndrome

Abstract: The extent to which genetic abnormalities contribute to SCLS is unknown. The authors identified pediatric and adult cohorts with characteristic clinical courses and sought to identify a possible genetic contribution to SCLS through the application of Whole Exome Sequencing (WES). On the basis of 9 adult and 8 pediatric SCLS patients and available unaffected first-degree relatives, they did not identify a uniform germline exomic genetic etiology for SCLS. However, WES did identify several candidate genes for future research.

11/04/2018

Clinical Trials


Cords registry

CoRDS, or the Coordination of Rare Diseases at Sanford, is based at Sanford Research in Sioux Falls, South Dakota. It provides researchers with a centralized, international patient registry for all rare diseases. This program allows patients and researchers to connect as easily as possible to help advance treatments and cures for rare diseases. The CoRDS team works with patient advocacy groups, individuals and researchers to help in the advancement of research in over 7,000 rare diseases. The registry is free for patients to enroll and researchers to access.

Enrolling is easy.

  1. Complete the screening form.
  2. Review the informed consent.
  3. Answer the permission and data sharing questions.

After these steps, the enrollment process is complete. All other questions are voluntary. However, these questions are important to patients and their families to create awareness as well as to researchers to study rare diseases. This is why we ask our participants to update their information annually or anytime changes to their information occur.

Researchers can contact CoRDS to determine if the registry contains participants with the rare disease they are researching. If the researcher determines there is a sufficient number of participants or data on the rare disease of interest within the registry, the researcher can apply for access. Upon approval from the CoRDS Scientific Advisory Board, CoRDS staff will reach out to participants on behalf of the researcher. It is then up to the participant to determine if they would like to join the study.

Visit sanfordresearch.org/CoRDS to enroll.

Community Leaders

aporzeca

I had my first episode of what turned out to be SCLS in November 2005, and was very lucky to have survived it (though with permanent disabilities in arms and legs, and thus in hands and feet) and to have been diagnosed correctly within days.

I went on to have 2 other life- and limb-threatening episodes in April 2007 and March 2009, requiring 2+ weeks of Intensive Care hospitalization to keep my organs alive and emergency fasciotomies to preserve the muscles and nerves I still have in my extremities.

I also had 7 episodes of lesser severity (Dec. 2007, June 2008, June 2009, July 2009, September 2009, and two in November 2009), because I realized I was having them early on, which allowed me to get a massive dose of steroids (Prednisone pills and/or injections of Solu-Medrol and Albumin) that effectively stopped the capillary leak phase of SCLS.

Given the increased frequency of my episodes of SCLS, despite having taken the recommended doses of the traditional medications (e.g., Theophylline, Terbutaline and Singulair), I was given my first infusion of IVIG in November 2009 and have had monthly infusions since then with no adverse side effects whatsoever. So far, so very good: for over a decade I had no more episodes of SCLS, though I did have one in February 2020, which I hope will prove to be the exception to the rule.

While I was among the first SCLS patients in the United States to benefit from an IVIG therapy, most other patients who had previously been getting this medication in Europe, and virtually all patients around the world who have since received IVIG, have stopped having episodes of SCLS.

Our stories are now told in a number of case studies published in various medical journals, and there is also a scientific article showing the efficacy of IVIG in countering SCLS in laboratory conditions based on our blood samples before and after receiving IVIG, as well as several articles with the results of surveys of SCLS patients who have been on IVIG.  The evidence that IVIG is the best and almost always successful therapy for the prevention of episodes of SCLS is now overwhelming.

My address is aporzeca@american.edu

 

Expert Questions

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Caroline uk Message
1 Mar 2019, 10:27 PM

Hello Arturo, 

I'm 44 and live in the UK and have been diagnosed a few weeks ago with probable SCLS from my hospital doctors at the end of a 5 day hospital admission with hypotension. I had a previous admission to hospital at the end of September last year with severe hypotension though on that occasion I was in ICU for a week requiring life support which at the time was considered a septic shock reaction though no infection was found. Now I have had these two presentations in hospital fitting the diagnostic triad hypotension, haemoconcentration and hypoalbuminaemia it is considered consistent with SCLS. To treat this I have been given Terbutaline and Theophylline which I am taking. I can see from your and others experience that IVIG is a preferred treatment however. Do you know of any doctors in the UK who have experience of SCLS and treating with IVIG as it is not currently the first line of treatment for this condition here as I understand it. Also from your knowledge is SCLS usually treated under haematology or immunology clinics? 

Looking forward to hearing from you. Kind regards, Caroline.

Answer

A diagnosis of SCLS is not the death sentence it used to be when I got the news 13 years ago.  We've come a long way, and our survival rates are now high, on average.  The operative word is "on average," because according to one of the most comprehensive studies available, "5- and 10-year survival rates in patients treated with IVIG were 91% and 77%, respectively, compared to 47% and 37% in patients not treated with IVIG. Patients treated with IVIG were more likely to be free of recurrence, severe recurrence, and alive at the end of follow-up. Furthermore, all but one patient who did not experience a severe relapse were treated with IVIG. Since preventive treatment with IVIG was the strongest factor associated with survival, the use of IVIG is suggested as the first line in prevention therapy." (See https://www.amjmed.com/article/S0002-9343(17)30602-2/fulltext)


There are 2 reasons why some doctors, especially those working for underfunded government health plans, hesitate and delay in putting their SCLS patients on an IVIG therapy.  One is sheer ignorance, so in order to combat that, I will email you 4 key, recent articles for you to give to your doctor(s), so they may be informed.  The other is rationing, because since IVIG is extremely expensive while terbutaline and theophylline (T&T) are extremely inexpensive, some physicians know that they won't get approval for an IVIG therapy before first having demonstrated that T&T have failed.  (We have this problem in the USA with the government program called Medicare, which provides health care for the elderly and disabled -- although we're doing something about it.)  The downside risk, of course, is that you never know when the next episode will be your last -- because you didn't survive it.
To expedite a favorable resolution, you can ask and insist that your doctor(s) should obtain a 2nd opinion in writing -- which can then be added to your file to buttress your case -- from one of the two world authorities on SCLS: Dr. Zahir Amoura in Paris or Dr. Kirk Druey in Washington DC, see their contact information on the page "Community Details," under "Tips or Suggestions," see https://rareshare.org/communities/systemic-capillary-leak-syndrome#community_details Your doctor(s) should send in and give you a copy of your hospitalization records, and if you'd like you can also make an appointment to see Dr. Amoura in person if you're willing to visit Paris, see https://www.aphp.fr/offre-de-soin/consultation-37642


And finally, to your last question, the best physician to have on your side is a veteran GP or Internist with a teaching and/or research appointment in a medical school -- namely, someone who is competent AND compassionate.  And since you live in a city with a medical school nearby and a very fine hospital, you should be able to find someone to coordinate your care, and to call on a hematologist or immunologist when needed.  (The latter are familiar with IVIG, the former are less useful.)

Gwendolyn20 Message
4 Jan 2017, 08:33 PM

My friend was diagnosed with SCLS about 2 years ago and has been on IVIG for almost the 2 years after his first episode. He now decided to stop the IVIG infusions, though he's very well aware of the risk that comes should he get a second episode. I'm just wondering if there's anything I could do to help him in this transition as I don't know what his reaction might be. Or if there should be any red flags I should look for in case he gets an episode, or any signs that he could be getting one, or should get back on the IVIG.

Answer

Until the specific cause(s) of SCLS can be identified, and a more targeted therapy can be developed, the experts at the National Institutes of Health recommend that confirmed SCLS patients should receive monthly IVIG infusions indefinitely.

If I were you, I would encourage your friend and his main physician to reach out and consult with Dr. Kirk Druey at NIH, especially if this was not done previously. (His coordinates appear towards the bottom of the tab "Disorder Details.") I would want to make sure that (1) my friend's diagnosis of SCLS was made correctly -- mistakes are common -- and (2) that an informed dialogue between knowledgeable physicians and my friend had taken place.

The upside of your friend stopping his therapy is that if by chance (a) he was indeed a confirmed case of SCLS and (b) he has no more episodes despite being off his medication, that would certainly provide worthwhile information to the rest of us who endure these expensive and intrusive treatments indefinitely.

The downside of your friend stopping his therapy is that he could have a life- or limb-threatening episode. The episodes are characterized by 3 clinical features measurable in a hospital's Emergency Room setting: hypotension (unusually low blood pressure), hemoconcentration (too many red blood cells per unit of plasma), and hypoalbuminemia (low albumin levels in the blood). (See more under "Disorder Details".)

And if he were to have an episode, tell him to demand a crash infusion of IVIG upon confirmation of the 3 clinical symptoms. (And it would help a great deal if he were to carry with him a letter from his physician stating that he is to be given IVIG if he should be having a confirmed episode.)

While there is limited experience with SCLS patients surviving an episode thanks to getting a last-minute infusion of IVIG -- and I have personally watched two SCLS patients die in the hospital despite getting their first taste of IVIG during their episodes -- at least nobody could blame themselves for not trying, in a life-or-death emergency, what seems to work best when received on a regular, monthly basis.

Rubeanie Message
29 Dec 2016, 04:15 AM

I am not sure if I am a rare breed or very fortunate (so far). In February of this year 2016), I had a "rash" from head to toe. I was misdiagnosed, but then in April I had what my doctors believe was a full-blown episode of SCLS. I have had several small ones since then. I was put on montelukast (Singulair) and have not had an episode since. My question is: will the montelukast always work, or will the SCLS flare up again?

Answer

If you really have SCLS, then you are both a rare breed -- because SCLS is a one-in-a-million, exceedingly rare disease -- and very fortunate so far -- because there is zero evidence that montelukast (Singulair) prevents episodes of SCLS.

Before the good news about IVIG for SCLS became widely known (namely, before 2008), I used to take theophylline, terbutaline and montelukast to prevent further episodes -- and yet I went on to have them again and again until I received my first dose of IVIG in late 2009. If I had not, I would probably have died in 2010.

Therefore, my advice to you is as follows. First, you must have your diagnosed confirmed, and the best way to go about doing that is to have your treating physician send in ASAP a copy of your medical records to Dr. Kirk Druey at NIH (see contact information at the bottom of the tab "Disorder Details") requesting an expert evaluation. If Dr. Druey suspects that the diagnosis may be correct, he will probably invite you to fly in to NIH (located in Bethesda, MD, in the outskirts of Washington, DC) for a full, in-person assessment.

And second, if and when your diagnosis of SCLS is confirmed, then Dr. Druey will work with your physician on a treatment plan which may well include an IVIG-based therapy.

Please do not delay: if you really have SCLS, then it's only a question of when, not if, you will experience your next episode -- and it could be fatal.

cpollet Message
20 Nov 2016, 10:27 PM

I'm a young Italian doctor that is following for about 6 months a male patient, 57 years old, for a suspected diagnosis of SCLS. His history began in 2014...

...

I'd like to know if, in your experience, I could improve his clinical situation by the addition of other drugs (Immunoglobiln iv? Terbutaline?) and if so in what dosage. My experience in SLCS doesn't exist and all that I know results from reading some literature. I ask you for help to improve my experience and my patient's health

Answer

Dear Dr. Pollet:

As you will understand, we are strongly discouraged from making diagnoses and recommending specific treatments on this website.

Therefore, I suggest that you request a consultation from any one of the leading authorities on SCLS identified towards the bottom of our page "Disorder Details": Dr. Kirk Druey at NIH; Dr. Mark Pecker at Weill Cornell/New York Hospital; and/or Dr. Zahir Amoura at the Hôpital de la Pitié-Salpêtrière in Paris.

Their emails are provided, so all you need to do is write to them giving them the details you sent to me plus whatever additional medical history you can deliver, and I am sure they will write back to you with their best advice on how to proceed.

LorraineKH Message
24 Jun 2016, 05:02 PM

A family member (nephew) was recently diagnosed with SCLS, while out of state. He seemingly received excellent care, and now I am trying to help with resource finding and financial assistance for what undoubtedly will be a very expensive trip to the hospital. I would like to be his ombudsman in the healthcare system he is in, do you have any idea how I might go about doing that? He is very intelligent, but I expect him to go through some periods of grieving and adjustment after his elation of finally coming home. Finally, are there any physicians in Colorado with experience treating SCLS? I have a bazillion questions, but will stop here for now! Thank you for being here!

Answer

Welcome to our Community! You will find here just about all the information you and your nephew need, so start by reading the main sections ("Disorder Details" and "Disorder Resources") and then going through the "Discussion Forum," picking among the topics that interest you most. You'll want to print out some of these sections and have your nephew take the printouts whenever he sees a doctor or finds himself in a hospital's ER

If your nephew was diagnosed correctly -- and especially if he wasn't -- the first thing he will need is a top-notch internist to serve as his "anchor physician." Forget about finding an expert on SCLS; you need to find a veteran, compassionate physician who will make the time to manage a rare-disease patient, is willing to read up on the condition, and is pro-active in consulting with the handful of physicians who really know about SCLS, as mentioned in the "Disorder Details" section.

My advice is to look for one who is affiliated with a medical school in Colorado (preferably, the Univ. of Colorado), and has the rank of Professor or at least Associate Professor, as long as they practice within, say, a couple of hours driving distance from where your nephew will live. Check out the list at http://www.ucdenver.edu/academics/colleges/medicalschool/departments/medicine/GIM/Faculty_Staff/Pages/Faculty-Roster.aspx

And please encourage your nephew to educate himself about his diagnosis and to join this Community. Many of our members, myself included, are alive and have a reasonably good quality of life thanks to what we learned here -- and only here. Besides, it's therapeutic to interact with others who provide information and moral support.

davec Message
21 Jan 2016, 07:38 PM

As I recover from my bouts with SCLS, I am curious if exercise in the gym, jogging or tennis is recommended? Second, how long after an episode should one wait before traveling internationally again: 6 months, a year?

Answer

As far as we know, physical exercise and international travel do not cause episodes of SCLS.

We have a few patients in this Community who were very active (e.g., biking or skying) for decades before they had their first episode of SCLS, and then we have a vast majority of patients who exercised moderately or little before they were hit. Likewise, we have a few patients who used to do a lot of domestic and/or international travel before their first episode, and a large proportion of patients who did not travel much.

Of course, we also have many patients who would like to engage in moderate or vigorous exercise and sports but cannot because of the damage they suffered in feet, legs, hands, arms or vital organs in the wake of their first episode of SCLS.

We also have patients who would like to travel abroad but are afraid to do so because they don't want to fall ill far away from home, family and their medical doctors.

In this regard, consider yourself very fortunate to be able to contemplate re-engaging in sports and in international travel.

The question you should ask yourself is: Why am I still having bouts of (presumed) SCLS? Being vulnerable to further episodes is the most risky thing you can do, because if you really suffer from SCLS, and you are not receiving a therapy that is effective in preventing the episodes, then you are vulnerable to having an episode that will leave you crippled or dead.

If you haven't done so already, I recommend that you have your physician send your medical history to Dr. Kirk Druey at NIH, see bottom of the "Disorder Details" section, so that he and his team may confirm your diagnosis and work with your physician to come up with an effective course of therapy for you.

janiegrrrlaz Message
9 Dec 2015, 07:04 PM

Are you aware of any treating physicians in the Phoenix (AZ) area? I was turned away by both the Mayo Clinic here and in Rochester, MN. I was told they do not "have any one qualified" to treat me. I am waiting to hear back from NIH also. Thank you!

Answer

I'm sorry that the Mayo Clinic seems to have dropped the ball in your case, but you really don't need them.

While I don't have the name of any physicians who have treated an SCLS patient in Arizona, I have two common-sense suggestions good for anyone in any place.

First, make absolutely sure that you have been diagnosed correctly by having your medical and hospital records sent by your main physician to Dr. Kirk Druey at NIH, requesting a consultation on your case. It sounds like you already initiated contact with him, so that is good. Having a correct diagnosis is crucial to everything else.

Second, if your current physician is not interested in your case, or seems overwhelmed by it, then find a new "anchor" doctor, preferably a seasoned internist on the faculty of a university hospital, to provide a fresh pair of eyes, accept a diagnosis of SCLS, discuss your case with Dr. Druey, coordinate your care, and decide on therapy options including IVIG.

It doesn't matter that he or she may not have even heard of SCLS before: by now there is plenty of useful literature, see the Disorder Resources section of this website.

In Phoenix, you are lucky to have the University of Arizona Medical College, so I would look there. Ask for an initial appointment with the Chair of Internal Medicine, Dr. David Wisinger, tel. 602-344-5027, davidwisinger@email.arizona.edu If he doesn't take you on himself, he will have recommendations for you.

mweston Message
25 Jun 2015, 03:13 AM

I am currently battling breast cancer, and my oncologist has recommended me for a clinical trial involving T cells. It involves taking my blood, removing the T cells, growing them and coating them with antibodies, and then infusing them back into my body so they may fight cancer. The purpose of contacting you is that one of the drugs to be given to me during this trial, Interleukein-2, supposedly can trigger the Capillary Leak Syndrome! Have you heard of this connection? Should I participate in this clinical trial?

Answer

I know nothing about this, and my suggestion is that you have your oncologist contact Dr. Kirk Druey at NIH to ask him what he knows and thinks about it. He knows more than anybody about SCLS and he has a lot of top-flight colleagues he can consult with. His contact information appears toward the bottom of the "Disorder Details" tab.

Tvaughn Message
24 Jun 2015, 12:20 AM

My daughter-in-law has recently been diagnosed with this disease. I want to find out as much as I can in order to understand what the disease does to her, and what I can do to help her. Are there any treatment options that we can use in or near Dallas, Texas?

Answer

Welcome to our Community, and the best thing you and her doctors can do is read the information appearing in the "Disorder Details" and "Disorder Resources" sections of this website -- because when it comes to exceedingly rare diseases like SCLS, the lack of accurate and timely information can be disastrous for the patient and her caregivers.

And of course there are great care and treatment options available in or near Dallas, TX. I suggest that she find herself a senior internal-medicine physician affiliated with a top hospital like the Baylor Univ. Medical Center or the Methodist Dallas Medical Center.

Blinski Message
31 Mar 2015, 10:50 PM

I am a physician and am quite confident that my father, who has been hospitalized a number of times in the past 2 years, has SCLS. His symptom complex and lab results at times of attack, and difficult recoveries due to aggressive fluid resuscitation, are literally a textbook case. His current physicians are failing him miserably, so have you any suggestions of any others in the South Florida area that could help? Also, how do you find a physician that is willing and able to initiate IVIG infusions?

Answer

So sorry to hear about your father! It is bad enough to be sick, but it is worse still to be sick and without an appropriate diagnosis -- never mind a suitable therapy.

While I don't have the name of any SCLS expert in South Florida, I have two suggestions good for any place. First, get hold of your father's medical and hospital records and send them to Dr. Kirk Druey at NIH requesting a consultation on his case.

Second, find a new "anchor" physician for your father, preferably a seasoned internist on the faculty of a university hospital, to provide a fresh pair of eyes, consider a possible diagnosis of SCLS, discuss his case with Dr. Druey, coordinate his care, and decide on therapy options including IVIG. It doesn't matter that he or she may not have even heard of SCLS before: by now there is plenty of useful literature, see the Disorder Resources section of this website.

While immunologists are obviously very familiar with IVIG therapies, and one of them could be enlisted to support the initiation of a course of therapy for your father, the clinical judgment and follow-up of an internist will be very important. Involving a hematologist has sometimes helped, but most of them have a narrow interest in oncological cases, so they are often not interested or helpful.

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My wife was diagnosed with SCLS Oct. 2014. That was her third attack with severe cerebral involvement (brainstem, hippocampus, thalamus)

 

 

2009 is when she had her first episode after a...
I had a diagnosis two months ago (October 2014) for seems a mild and chronic form of SCLS. By remembering what happened since January 2014, I realized that I was probably ill as soon as February,...
Hi my name is Enny. My daughter is 5 and was loosely diagnosed with SCLS earlier this year when she was still 4. This month will mark one year since my daughter fell sick and ended up in trauma...
32 year old mother of two from Rowlett, TX. Diagnosed in September 2014, a year after onset of symptoms. Looking for solid advice and info on doctors in the Dallas area with any knowledge of this...
I'm currently casting a medical mystery docu-series for the Discovery network.
I was diagnosed with SCLS Jan. 2014. July of 2013 is when I had my first episode. I had flu like symptoms and excruciating pain in my right lower back area. I couldn't straighten out my leg cause...
Previously fit (extremely) GP. I fell ill with my first attack on the day I retired, in July 2014. I am not sure how I survived the first attack with an Hct of 219, sudden drop in renal function,...
I have recently been diagnosed with Clarksons Syndrome also known as Ideopathic Systemic Capilliary Leakage Syndrome.

 

I would like to join your community to find out more about it and add my...
I'm a young doctor who's studying idiopathic systemic capillary leak syndrome, or Clarkson's disease.

 

I work in Milan, at L.Sacco Hospital, with prof. M. Cicardi. We're conducting medical...
Medically retired hospital executive diagnosed with Clarkson's Disease, pituitary disease, secondary adrenal insufficiency and autonomic dysfunction.
I'm one of the social media interns for RareShare. I help raise awareness for rare diseases and rare disease support groups
My friends husband has Systemic Capillary leak Syndrome
Hi- have scleroderma, polycythemia, dermatomyositis, allergic angioedam and hashimotos thyroid. Am still working and enjoy my work. Life is hard and would like to be a member of some groups where...
I am in fourties & survived an attack of SCLS this year in Feb. I suffered compartment syndromes with its complications requiring fasciatomies. I am now going through rehab, getting monthly IVIG &...
49 Year Old Male.

 

Living Sydney Australia

 

Diagnosed with SCLS April 2014
I have Clarkson disease and struggle everyday. I have total body edema along with joint pain. Also suffer from flu like symptoms. I have been treated in Rochester Mn at the Mayo Clinic. I started...
I live near Annapolis, Maryland. I was diagnosed with SCLS in March of 2014. That was my 3rd attack in 15 months, survived the other two on my own pretty well, but didn't know what was going on....
My daughter was first diagnosed with Capillary Leak Syndrome in 2006. However, she had other symptoms that didn't fit the condition, so after nearly 2 weeks in ICU, she was discharged with primary...
hi
I am new to the community. I just received my diagnosis a couple of weeks ago. My head is still spinning and I am trying to read everything I can get my hands on. I have been prescribed waist high...
friend of a person with scls
Pathologist
I am a capillary leak patient and have never had the opportunity to speak with anyone else who deals with the problem. I would like to connect with others willing to share their experiences. I must...

I am the mother of a wonderful 12 year old girl .She has systemic capillary leak syndrome. 3 severe episodes requiring hospitalization. Being treated with sub Q igg therapy. Hoping to connect with...

Our son Connor has had three acute episodes from March 2009 to Nov. 2011 between the ages of 4 and 6 1/2. He hasn't had one since.

 

 

My wife, Jen, is also a member of the site, but I...
For years I have been looking for answers to my medical issues. The closest diagnosis that my drs and I have come up with is SCLS/Clarksons. After looking into hereditary angioedema. I am still...
A caring friend of someone I've known for over 30 years. I'm trying to learn what I can to understand their situation better and hopefully help as I'm deeply shaken by this all.
My 8 year old son has just been diagnosed with Systematic Capillary Leak Syndrome. We live in Sydney Australia, I am scared to death of this disease, but it does help to read how everybody copes...
SCLS diagnosed 2008 Jul
hello,

 

I am a french woman who suffers capillary leak syndrome.

 

Doctors diagnosed two weeks ago .

 

I would like to discuss with you because there is no forum about this ill in...
I live in India ( Rajasthan) with my father and mother. My father is a retired officer , 64 yrs of age.He had always been healthy and with no ailments. Yet last year in April he suffered from...
To Come
my brother in law is suffering frm the ailment hence would like to join the community
Hi I am relative of Mr. Rahul Bali who is a Patient and is presenlty in ICU
I am a close Relative of Mr. Rahul Bali Who is Suffering from This rare disease

     My name is Kimberly and I was diagnosed with Idiopathic Systemic Capillary Leak Syndrome in January of 2012 by Dr. Carl Lauter at Beaumont Hospital in Royal Oak, Michigan....

Parent of a young man with Clarkson's.
My boyfriend and I have a total of three children, his daughter has just been diagnosed with SCLS. Well, maybe not diagnosed, but that is what NIH has decided she probably has after lots of testing...
I research symptoms that my husband suffers and read about possibilities that I can then take back to the doctors. Latest is systematic capillary leak syndrome. He has been told repeatedly that the...
44 y.o. single parent - developed scls 8 months post op status post mesh placement 2/2010 - systemic shock severe issues began 10/2010 - mesh removed 11/2011 - immune system damage...

Just in the centre of the Netherlands I live in Nijkerk. We have 4 boys.

Until my 50th I seemed to be healthy. I had a very busy and inspiring job as general practitioner. I loved all kind of...

My mother died in 1992 from SCLS . I am curious about new developments in treatment of this illness, and if there have been any causal factors that might have been identified.

 

We have a...

 

A first occurrence took place on 25th of October 2005 and it will supposed to be interned in the ICU (intensive care unit) of HOSPITAL UNIVERSITARIO RIO HORTEGA in Valladolid (Spain) where I...

I am 53 living in Paris , France.I discovered in May 12 I had the SCLS and my doctor is Pr Amoura at Hopital Pitié Salpétrière in Paris .

 

Before that my first "crisis" started in summer 2010...
I am a retired high school principal.
I am the mother of scls patient, Marilyn Meaux, or maire602
I am a christian mother of two beautiful little girls. I am very loving and have a gentle and kind heart.
Hi Everyone,

 

After just returning from 4 days in the hospital (my 5th episode) in So. CA while visiting my daughter & her family, I have decided it's time to get serious about finding help...
My mom suffers from SCLS and I'm working hard to help her find the best treatment possible to prevent her from having any further episodes. I appreciate this community and am excited to learn from...
My friend has been diagnosed with capillary leak.
Father of a patient.
My little brother (34 years old) has been gone through two SCLS crisis in last two years (Feb and oct 2012). He is now taking IVIG once a month and its been a while with no other hospitalization....
..
Diagnosed at University Hospital, London, Ontario, Canada by Immunologist (now retired) over 20 years ago, still alive, and have an episode on avg 1/yr. Interested in treatments, experiences, etc
Sister -in- law of SCLS victim who passed on Feb. 19, 2011. We would like to spread more awareness about this disease.
I am the proud mom of two beautiful children. Last year my world felt like it was crumbling around me when my 4 year old son was diagnosed with Capillary Leak Syndrome on his 2nd admission to...

40 year old, mother of four, diagnosed on Dec. 8, 2011 with SCLS. Hospitalized in ICU for 5 days, 4 of which were spent in an induced coma, intubated. Fasciotomies were done on all four extremities...

I am a 44 yr old sahm to identical twin boys. I was diagnosed with Capillary leaking syndrome 9 years ago after many unexplained "attacks" of what the doctors thought was me...

Husband & advocate of Marilyn SCLS patient 1/2008 6wks LCMH, ER, ICU, floor, physical rehab. 11/2008 mild case,12/2008 4wks ST. Lukes Episcopal Houston Texas, 1/2009 Mayou Clinic Rochester,...

50 Years old

 

Male

 

Living near London in the UK

 

Married with 3 children (all boys)

 

I was a research Biochemist for 12 years and am now a teacher at a secondary school

...

My 4 year old nephew was recently diagnosed with SCLS. He has had four episodes in the past 12 months - one in January 2011, one in March 2011, one in June 2011 and one in Dec 2011. Each episode...
Bonjour,

 

J'ai un problème lymphatique depuis la puberté (1950). Hyperperméabilité capillaire de 20% avec oedème généralisé. Le Pr Lagrue (Mondor-Créteil) a diagnostiqué un oedème cyclique...
*scls

 

Have had CLS for 4 Years first time was in I C U for 11 days my Doctors did not know what was wrong .

 

My blood pressure and count dropped. So they said I was in dehydration gave...

My first episode was in 2008. I was misdiagnosed with anaphylaxsis up until fall 2011. I started IVIG treatment in February 2012. Since then, I have had no episodes. I am now on bi-weekly...

My daughter Ela (3 this November 2011) was diagnosed with SCLS, September 2011, after her last serious attack.

 

 

Ela has had 4 episodes in the past year (September 2010 to date). 3...
I was told that I have chronic capillary leak but that I do not fit into the strict category of SCLS because I do not have an episode resulting in an ICU stay or proven hemoconcentration. I am...
One of my best friends is suffering with this disorder, and I wanted to find out more about it.
In early July, 2010 (the hottest weekend of the summer) I arrived at the emergency room with a low pulse, rapid heart rate and profuse sweating. When they checked my blood, they saw what they...
My name is Amy and I have been just informed I have Clarkson's Disesase. I am a mother of 3 and grandmother of 5. I have been battle with this for over 21 years and finally came across a doctor at...
*I was diagnosed on June 24, 2011 by Dr. Griepp. My story: In Feb. 2011 I was having swelling first in my hands and then spread to my feet and legs. Went to my Primary doctor. She ran some test for...

I had my first acute attack of CLS around 10 years ago and again 3 years ago. My most recent attack was around two months ago and it was very severe. This last time I was diagnosed with CLS. The...

I have been diagnosed since April 2011

 

I was a puzzling case 3yrs ago due to showing similar symptoms to MS all test came back negative,

 

I was discharged after two weeks with right...
friend of someone who died from scls
I am a family member of Mary Jane K, hoping to get more information on this disease.

I was diagnosed in 1998 with SCLS. I had been ill for 6 months, and gained 80 lbs. I was given prednisone too prevent an allergic reaction to a test. It worked wonders, and I lost the fluid. I...

i was diagnosed with scls in November 2005 or actually 3 months later in February 2006. After I had emergency open heart surgery to put a window in my heart sack at age 38. In a coma for nearly two...
I am at retirement age and have suffered from an auto-immune disease for the past 15 years.
My sister Shannon suffers from SCLS; diagnosed 5 years ago (2006).

 

 

Shannon has just been admited into Cardiac Care to monitor her current episode. We have been fortunate to have the...
I am a 61 year old female. I started swelling when I was 14 years old - just a week after my father's sudden and unexpected death. After his funeral, we went to visit family that could not attend,...

Previously healthy individual - jogger and active in excercising. First episode 9/24/10. Thought I had the flu. Extremely dizzy, nauseated. Doctor came over and couldn't find pulse. Put on 30...

wife has capillary leak syndrome

Sept 4, 2010 - Persistant Flu-like symptoms for several days led me first to my doctor's office and then to the Emergency Room ICU. I was admitted with extremely low blood pressure. I was put...

I am a retired family physician with MGUS and have had progressive symptoms of recurring edema, low blood pressure, near syncope that may be a chronic variant of SCLS.
I was diagnosed with SCLS in December of 2010 after suffering multiple episodes for over a two year period.

 

 

I was initially put on Theophylline and Prednisone with one minor recurrence...
I am the husband of someone with (?) SCLS and/or angioedema
my 4 yr old son was dagnosed with capillary leak syndrome. he has had 3 episodes in the last 6 months and thie last episode led to a cardiac arrest. he was put on life support and luckily recovered...
Physician brother of a member
I'm 19 years old, living in Oklahoma. I'm in my second year of college, and am double-majoring in Forensic Science and Funeral Service. I enjoy playing the piano , guitar, and singing; going to...
As new member but with diagnose of SCLS in April 1988 after a severe collapse and a first hospitalization in distress and 3 more other until January 1989, I think I will, probably, be at time the “...
My first attack was 3/2000. RAK amputation, nerve damage in both hands & left foot. In hospital for 4 months.

 

Second attack 4/2005. Was in hospital for a week, but thankfully no physical...

I have been diagnosed with systemic capillary leak syndrome which I have had the symptoms of for about 9-10 years. I am seeking information and other people who also have this as I live in New...

Diagnosed by the Mayo Clinic in Rochester , MN , with SCLS in August 2011. This was my second trip to Mayo. The first trip was in August 2010 and resulted in a 2 week stay and a suspected / working...

I have just been diagnosed with SCLS -8/18/2010 at the Mayo Clinic in Rochester, MN by Dr. P. Greipp. My first episode of this disease was June 2007. I played a round of Golf and had eaten dinner...
Wife of Mahaff

I have SCLS and think I am one of the oldest persons with this disease in this group. First attack, May 2007 after severe bronchial infection, 8 days in ICU in an induced coma; 14 days in hospital...

I was diagnosed with Systemic Capillary Leak Syndrome in May of 2010. This was after 3 months of extensive testing of every kind that did not explain the fluid retention that I had. I was first in...
My cousin has been diagnosed with this rare disease.
Atypical Systemic Capillary Leak Syndrome (current working diagnosis) Diagnosis made this year at Mayo Clinic in Minn. I am taking Theophylline and had my first IVIG a month ago. I first recognized...
I am a 56 year old female who has suffered from systemic capillary leak syndrome for 10 years. I leak almost constantly but my episodes are relatively minor with my largest 24 hour weight gain at...
Surprise! I've got a rare disease! I'm still going to be a pain in the butt, though.
Searching for answers that no doctor has been able to find in over 5 years for my wife. In the beginning she was having severe body aches, menstrual problems, severe acid reflux. After having a...
My daughter, Rebecca, was diagnosed with SCLS in 2007, when she was 2 years old. I've joined this SCLS community to learn more about this illness and its symptoms and treatments, and to share our...
High,

 

 

My name is Wout and my mother is diagnost with the clarkson capillary leak syndrom. We just recieved this diagnose 3 days ago two weeks afther she had an attack and had to go to...
Dear sir

 

I have the pleaser to write about my casI`ll try to explain about may case .

 

All start at 17/02/2008 I had a cove and I went to the emergency they give me anti biotic it was...
My medical history briefly. It began in June 1995. After several months of hospitalization, I was diagnosed with SCLS of November 1995, immediately started plasmapheresis with a transfer of plasma...
my extremely good friend was diagnosed 6 years ago and I am agressivley seeking information to assist him. We live in Longview, Texas.
seeking helping in treatment of my 16 year old daughter newly dx of idopathic systemic capillary syndrome

My husband's first episode:

  • Husband and I were ill with upper respiratory infection (confirmed to be Human Metapneumovirus).
  • Husband had to be hospitalized Dec 2009 
  • He received a least...
I have suffered SCLS since 2007 and have had two near fatal attacks. My condition is currently being successfully controlled with Ventolin and Theophylline tablets, and I have had one attack in 18...
I am the mother of a 5 year old with systemic capillary leak syndrome. My daughter was diagnosed with this disease at the age of 17 mo. She receives infusions of IVIG, 25g every four weeks, and is...
Our son Connor (born 4/7/05) was diagnosed with this syndrome on Christmas Eve 2009. This was his second episode in 9 months. His first one (March 2009) was when he had the flu and was misdiagnosed...
I am an infusion nurse who worked very briefly with Judy Davis and I would love to read the article on your website. Thanks.

 

 

I'm Judy Davis' sister
My sister, Judy Davis, died on November 27, 2009, after a five-year battle with SCLS.

 

 

Bonjour,

 

Un urologue d'un service hospitalier m'a diagnostiqué cette maladie rare il y a quelques années.Le traitement proposé à l'époque, me faisait plus souffrir que la maladie...

 

Je...
My father has just passed away from possible systematic capillary leak syndrome. He was not diagnosed with the condition eventhough he suffered from it more then once.

 

I suppose I'm on a life...
I am a gastroenterologist working at the Department of Gastroenterology of the Ospedale V. Cervello, Palermo, Italy.
My sister-in-law, Denise, has SCLS. She is married to my brother, Mike The picture is of their daughters, Kate & Brooke. My nieces! They are wonderful! Denise was diagnosed in 2009. And, with...
I've been diagnosed in 2006 with Idiopathic Capillary Leak. It is a severe case that has been constant since December,2004.I am being treat by a "Kidney-Doctor" and I'm on 20-mg of Lasix2x/day.My...

first attack in 02/2009. the doctors find the exact diagnosis after 24h and in the meanwhile, I had two fasciotomies in the legs. quick recovery and no server damage in nerves and muscles. The...

I am a 61 year old male living in Decatur Al. I had my first major attack in 3/05, with 2 more in 07, and 08, 3 in all. I was diagnosed in 4/07 at Vanderbilt in Nashville,Tn by a research doctor,...
I am the wife of a Systemic Capillary Leak Syndrome patient. I am interested in corresponding with other patients or family of patients.
Parent to a daughter with Systemic Capillary Leak Syndrome
I am french, I understand english but I don't write In french now Je suis malade SCLS depuis avril 2008 pendant 1 an j'ai eu des perfusions d'immunoglobuline et maintenant tous les 2 mois 1g/kg...
My name is Maria, I was diagnosed with SCLS in Feb 2011. I have the chronic form of SCLS with attacks every few days. I had my 1st major attack in January of 2004 at the age of 33, but I started...
Update 2016:

 

 

I have been off IVIG since February. For now I am on Singulair and an ACE inhibitor. I have been having minor attacks but have been able to stay out of the hospital.

...

I am a stay- at- home mom of 7 children, (5 sons and 2 daughters).

 

And yes, they are all ours,

 

no, we have no twins, and

 

no, we are not Catholic.

 

These are always the first...

[Updated on January 2013]

 

My daughter is 7 years old and has been diagnosed SCLS in May 09. She had 4 SCLS attacks, the first one in February 08, the last one in April 09 (she was 3-4 yrs...

My daughter, age 41, has been battling illness for almost 2 years now. It appears that she has systemic capillary leak syndrome, a very rare condition. We are very anxious to learn all we can about...

I noticed swelling in my ankles in spring of 2007, which increased through that summer. I was working full time at a desk job, then on evenings & weekends at our home business helping with...

family physician , patient with scls
My first attact was in Oct 2005. I was disgnosed in June 2006 with Capillary Leak Syndrome. The first time I entered Mass General in Boston Mass the Dr's thought I had a virus due to the fact that...
I was just diagnosed with scls and am looking for any information on it, since my doctor was unable to give me much of anything yesterday. So I am taking it upon myself to do some of the research...
sono la moglie di mario gatto se qualcuno in italia vuole contattarmi per avere informazioni sulla malattia puo' farlo su facebook mi chiamo marcella iannacchero
I live in Christchurch, New Zealand, and if I could find some way of connecting with some supporting people I would be very grateful.

 

 

I have been living with SCLS since October 2005. I...
Name: Walt Breidigan, Bethel Park, Pa. Born 1957

 

 

I have had CLS since February, 2005.

 

 

The first incident gave me two total drop feet and no feeling from calves to the toes...
Heading a research study on Systemic Capillary Leak Syndrome at the National Institutes of Health.

I have been on IVIG therapy for 5 years without any SCLS episodes.

I have had 7 recurring episode of Severe Capillary Leak Syndrome in the past 20 years. My last episode was in January 2013. Each...

Widow of NC SCLS patient
I have had SCLS since 1997, It took 6 years to get it diagnosed. Aftyer it was diagnosed I went into remission for 5 years. After coming down with a systemic viral infection in the fall of 2008 my...
my brother at age 43 suffered a capillary leak attack and was diagnosed 6 months later in 2004 at the mayo clinic in rochester by dr greipp and his associates. i was with him both times. he is now...
White male diagnosed with Capillary Leak Syndrome at the Mayo Clinic in Rochester MN after a severe illness in Nov. 2003.
Im from Guatemala. The MD told me that my mother have capillary leak syndrome, but here is not a specialist. I need help. I prefer if can write me in spanish. Thank you.
My name is Claude Pfefferlé. I live in the french speaking part of Switzerland.

 

First acute episode of systemic capillary leak syndrome on May 2003. Failure of different treatments and 5 new...
j'ai été diagnostiqué avec le Syndrome de fuite capillaire depuis Mars 2006. j'ai pris des médicaments durant deux ans sans aucune rechute. En Janvier 2008, ça recommence... des hospitalisations...
I am from Canada, My friend, 38 years old, mother of two beautiful childrens has this desease since 2006, and presently her situation is not controled. She is desperate to find a way to controle...
Am an Indian national, want to join this community on clarkson's syndrome to know more about it, its treatment, cures if any and what prophylactic measures can taken.
I AM AN INDIAN LIVING IN KANPUR( UTTAR PRADESH) & I HAD 2 SEVERE ATTACKS SINCE 2007 (& HAD TO BE ADMITED TO ICU FOR RECOVERY), ALONG WITH 6 MINOR ATTACKS SINCE 2004 , AS PER MY DOCTOR . I AM DOING...
Physician focused on disorders of blood pressure control and sodium metabolism. Some experience with Idiopathic Systemic Capillary Leak Syndrome. Based at Weill Cornell and The New York Hospital...
First scls attack on valentines day 2005. Many minor attacks and hospitalizations untill beggining IVIG therapy March 2010 I believe. Life changing cessation of symptoms until October leading to a...

My husband Jeff was first diagnosed with SCLS when he was hospitalized in February 2005. We're in Washington, DC. He's been hospitalized numerous times since then.

Friends, family and patients with SCLS

 

 

I am a physician at the Mayo Clinic in Rochester MN. I have been interested in helping patients with SCLS for many years. I am happy to support...
Mark is my name, I am the partner of my lovely girlfriend Cara, who has recently been diagnosed with SCLS.

 

 

She has had a difficult time recently, with admissions to ICU and the upset...
I am 36 years old and was diagnosed with SCLS in January of 2007. The first episode I had of this was quite severe and landed me in the ICU on a ventilator and in a medically induced coma. I was 11...
I have had this syndrome since June 2007. I was diagnosised in Dec. 2007. I was having episodes about every 5 to 6 days. I had a severe episode in November of 2008. After a bad episode I would...
I am married to Richard and we have 2 wonderful sons 17 and 20 years old. I have been a Hopkins pediatric infusion nurse for almost 30 years. I enjoy church, reading, cooking and my family. I...

I had my first episode of what turned out to be SCLS in November 2005, and was very lucky to have survived it (though with permanent disabilities in arms and legs, and thus in hands and feet) and...

My name is David Isserman and I am one of the co-founders of RareShare. If you have any questions or feedback regarding RareShare, please feel free to contact me directly at david@rareshare.org.

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Discussion Forum

Muscle pain

Created by DavidS | Last updated 25 Nov 2020, 05:49 PM

Any Impact on IVIG availability due to Coronaviris

Created by WazzaACT | Last updated 24 Nov 2020, 02:18 PM

Protocol Development For Chronic SCLS

Created by stedrick | Last updated 1 Nov 2020, 03:26 AM

Covid Positive

Created by gandcburns | Last updated 30 Sep 2020, 09:02 AM

Clinical trial on SCLS

Created by Barney | Last updated 30 Aug 2020, 01:18 AM

IG levels in blood to stay well & sheilding

Created by Caroline uk | Last updated 28 Jul 2020, 04:41 PM

Taking care of grandchildren

Created by HansDeWit | Last updated 24 Jul 2020, 04:28 PM

Looking for medical specialists SCLS in the Netherlands

Created by Sas | Last updated 11 Jul 2020, 02:10 AM

Starting IVIG. What to expect???

Created by Di_dee71 | Last updated 1 Jul 2020, 11:56 AM

Any adverse reactions to anesthetic

Created by WazzaACT | Last updated 27 Jun 2020, 04:54 AM


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