Hello, first I wish you all good health for this 2023.

I will try to detail my las hospitalization as best as possible, if anyone has any questions, I will gladly answer them.

On Sunday December 18 I meet with some friends, during the meeting 2 of them (Family) start vomiting.

The night of Tuesday the 20th I started with a lot of vomiting (a week was left for the gamma) Only vomiting, no fever, no pain.

On Thursday I start with pain in my legs, on Friday they become more intense and I start to get fatigued, and noticing a little edema, I start taking 100mg. of prednisone every 4 hours, at that point I had no doubts about the leak.

On Saturday the 24th, I spent most of the day in bed, waiting to see if it would reverse with the prednisone like other times; 3 days left for the gamma. The edema was greater and at that point every muscle I used hurt, for example when eating.

On Sunday I felt the same way, I considered that it was time to be hospitalized, I talked to my doctor and I went to the hospital for an emergency application. They gave me 90g. of  gamma and I proposed to rest 3 hours before the other 90gr. All of this during the early hours of Monday the 26th (my scheduled infusion was Tuesday the 27th). Then I remained under observation until Wednesday the 28th. I continued my rest at home, I improved very slowly and only yesterday (Friday January 6th) I felt no pain or fatigue, just over 2 weeks after the first pains began.

Laboratory values:

Upon arrival at the emergency room: Hemoglobin 16.1 Hematocrit 48.2

After the first application of 90gr. Hemoglobin 13.6 Hematocrit 40.2

After the second application of 90gr. Hemoglobin 13.1 Hematocrit 39

 

Some considerations:

- My emergency plan worked perfectly, of course this worked in my hospital with a clinical history that scares any doctor, and a doctor wife, I hope I don't have to try it in another country as it already happened to me when I still had no diagnosis.

- Unlike my 3 previous hospitalizations, without diagnosis and without gamma, I remained conscious at all times, which made everything easier.

- I had already presented leaks in 2 of the 4 vaccines but they reversed on their own or with the help of prednisone. I think this time it caught me a few days before the infusion and that changed the situation.

- One comment… the hospital pharmacy had small bottles of 50ml at 10%. I used the 100ml bottle in 28 minutes at home, I considered, remembering the Claude case, that I should double the speed and see if I tolerated it well, that gave me that each 50ml bottle should be taken in 7 minutes and that requires an exclusive nurse with us and all the jars at hand. This is not always easy and they must manage it and it is Hard to convince the medical and nursing staff that it is of the utmost importance.

Thank you for your attention and I encourage everyone to always tell about their situations with the disease and medication, that is what helps us all and those who will come in the future.

Ariel, I'm sorry about your recent scary experience, and I'm very glad that you came out intact and all right!

It sounds like your friends had a viral infection that impacted their digestive system, and that they infected you with it and in your case the virus triggered in you an episode of SCLS just when your "tank" was running very low on IVIG.

Therefore, your experience is consistent with that in most of the cases described in the recent article "Management of Acute Episodes of Clarkson Disease (Monoclonal Gammopathy-Associated Systemic Capillary Leak Syndrome) With Intravenous Immunoglobulins," see https://rareshare.org/topics/2192

To summarize: (1) all viral infections (including influenza and Covid) have the potential to trigger episodes of SCLS; (2) patients who are running low on IVIG are especially vulnerable; and (3) IVIG given close to the onset of SCLS-related symptoms is associated with a favorable outcome.

In my experience, taking prednisone at home and/or on the way to the hospital is helpful in terms of preventing a major drop in blood pressure (and thus in preventing the loss of consciousness), but that should still be followed by the urgent administration of IVIG -- especially when one was scheduled to get it soon, anyway.

Bonjour à tous,
je suis totalement d’accord avec Arturo. Je pense que le débit de perfusion d’immunoglobulines lors d’une fuite peut être accéléré par rapport au débit de perfusion pendant le traitement préventif mensuel. En effet, lors d’une fuite, il ne s’agit plus de prévenir un mécanisme physiophatologique encore mal compris, mais plutôt de traiter un événement potentiellement mortel. Cordialement. Claude

 

Translation into English of Claude's comment: "Hello everyone, I totally agree with Arturo. [Moreover,] I think the immunoglobulin infusion rate during a leak can be accelerated compared to the infusion rate during monthly preventive therapy. Indeed, during a leak, it is no longer a question of preventing a still poorly understood physiopathological mechanism, but rather of treating a potentially fatal event. Cordially, Claude"