Hi everybody! My mom has had two very serious scls flares that were nearly fatal. They happened 5 years and 3 years ago. After the second episode she has been given ivig every month. She has had no episodes since then. Sometimes she has a little bit swelling in her calves. No in a few weeks she has had three episodes that caused swelling only to her left thigh and also pretty bad pain in the side of the thigh. She went to hospital in the third time and they examined her. They checked with ultrasound and there were no blockaces in the veins. Her bloodtests were all fine, hemoglobin albumin etc. The swelling and pain seem to go away on its own in a few hours. Has any of you experiensed something similar and could the swelling and pain in one thigh be a minor scls flare eventough her bloodests and blood pressure were fine?

Hi Anni, I am so sorry to hear about your mom. Her story however is very similar to mine. I have had 4 flares which were very intense and scary too, nearing hypovolemic shock and having a myocardiac infarction.  All these flares were triggered by either influenza or influenza vaccination. Other than that, I experience a more chronic pattern in scls, with almost constant minor leakage in my lower extremities. This constant leaking does not show up with great fluctations in either blood work or BP, but indeed presents itself with pain in the area, swelling, some persistent minor weight gain and some malaise/fatigue. Every once in a while I also experience unilateral swelling in for instance my hand, arm, etc. Just recently my right hand and arm started to swell, and blockages or blood clots were not found either. It seems that some capillary bedding in the body might be more fragile and prone to leaking than others. I also experience different areas of my body are leaking to a greater extent in some flares. For instance, one flare I experience a lot of gastro-intenstinal issues and swelling, whereas other flares are more centered around kidneys and leading to pain, pressure and other problems there. I am unsure whether this could truly prevail medically speaking, but it is what I have experienced too. For me, these minor events follow minor triggers, such as just a bit too much heat exposure, overextertion physically or emotionally, etc. Does your mom has any idea of what triggers her flares? I wish you and your mom all the best!!!!

Hello Anni.

Your mother's experience sounds very similar to mine. I was diagnosed with SCLS in 2011. For many years the IVigg managed my issues well with few minor calf swellings between treatments. I had another major leak in 2023. Ever since the 2023  episode, I experience almost a chronic leak. My left leg always swells the most. I used to have a significant catalyst to a leak, but now it seems to happen for no reason, or at lest for reasons that I can't avoid such as heat, exercise, and stress. When I am in a low level leak, I tend to leak wherever I am using my muscles. For example, if I type on a keyboard my hands and forearms will swell. If I walk, my legs will swell. Sometimes my blood pressure will drop and others times it will remain stable. These episodes will last for a month or more, then I will have a few good weeks. Sometimes these episodes end up in a hospital visit where I receive a full month's dose of Ivigg over a course of 4 days.  It is very frustrating. I haven't been able to find a soulution yet. I pray your mother's situation resolves. 

Cristina

Hello!! I was diagnosed in 2010. Around 2012 I became a chronic leaker. After my Doctor spoke with Dr Druey it was decided my once a month treatment would become every other week. So it get 1gr /kg over 2 days every other week. I receive at home treatment and it has made a world of difference. I no longer have chronic leaks. 
 

Shannon

Hello! Than you all for replying!

I am happy to say that my mom is now doing very well and the swelling in thigh is no longer present. It lasted for about two or three weeks. Usually in the night the swelling went away and then it cam back when she got up and started walking. Her doctors believe that it may have been something else than capillary leak and no other symptomps were present. They actually suspected that it may have been due to her fasiciotomy scars and perhaps some kind of hernia.

My mom had her first episode propably about 7 years ago when she had a pretty bad bacterial infetion in her leg. She was not diagnosed at the time and was hospitalised but not in the ICU. Then she had her worst episode in 2021 three days after being vaccinated against Covid. She very nearly died and they had to do fasciotomies in three of her limbs. She was not diagnosed at the time. In 2023 she had an other severe episode that also put her in the ICU when she had the Covid itself. Luckily it wasn't as severe as the one she had from the vaccination. After that episode she was diagnosed with capillaru leak syndrome and has been given IVIG since then, every month 1gr/kg. She has had no episodes since (at least that we know of) and has been quite well. She is now 66 years old. She has been a living normal life, altough trying to avoid getting Covid, Inluenza etc. She has had a normal flu a couple of times and there were no complications.

We live in Finland and the doctors have told my mom that she is the first person in our country ever to be diagnosed with capillary leak syndrome. That is why we (and her doctors) are very grateful that these kind of communities are available and information and support is shared!

Wishing you all well!