Dear all,

Nice to meet you! My name is Marielle, I am 28 years old and currently doing a PhD on Snacking behaviour at a university in the Netherlands. 

I am new to the forum, but not to SCLS. I have been dealing with SCLS for almost 5 years now, and next to struggling with the devastating effects of SCLS, I have also struggled with many difficulties in hospitals (due to a lack of Health care workers' awareness/knowledge/empathy/willingness to broaden their knowlegdge). My previous specialist gave me homework to explore SCLS in literature, because of my academic position (So I can also contribute scientific literature to this forum!). He however turned sour when I wrote a proposal to advocate the initation of IVIG for my case. He simply did not believe in the efficacy of IVIG without the presence of M-protein, even when literature shows sufficiently strong contradictory evidence to his proposition. He did not want to cooperate in improving my situation. So, I had myself transferred to another hospital November last year, to a specialist who has a heart for his patients and treats people like ... well ... human beings.

However, because all my medical data is scattered over time/hospitals, and oftentimes missing pivotal biomarkers, I am still waiting for confirmation of the diagnosis SCLS. I need to adhere to a set of quite strict criteria (set up February this year) for my next attack so I can be clinically observed in the hopital. However, most attacks I experience are mild-ish.  I have had a few major attacks (mainly in the first few years) which almost knocked me off this earth, but most of the attacks now are mild and very frequently occurring (the time between attacks is a week or so when I am lucky). These mild attacks are bad enough to make you feel very sick, but not (yet) bad enough to be hospitalized... so I am a little in limbo, awaiting something bad to happen before I can hopefully be helped, but also progressively experiencing less quality of life.

Anyhow, over the years I have experienced quite some "complications". Many sudden severe infections (thrombophlebitis mostly) due to blood clots in leg, but recently also arm veins, constant sensible edema in the legs, microvascular angina, no heat tolerance and more. However, there are a few odd things I cannot really ask my doctors about (because they have no idea) and for which I have not really found a lot of evidence in literature yet. So I am wondering whether any of you have experienced the following:

- Leukopenia/neutropenia: my WBC/neutrophil levels are consistently low, even declining. Is this recognizable for any of you? 

- Recently, I have experienced neuropathy (constant pins and needles feeling, burning sensations) in my legs and arms. Does any of you have to deal with this too? 

And if you recognize these matters... do you also have some more (experiental) knowledge on possible underlying mechanisms in relation to SCLS?

Thank you for reading and maybe answering... Finding this forum is on one hand a true gift (so much recognition, sharing our stories, supporting each other) and I am very grateful for the existence and efforts you guys put in .... but I would have also wished for people not having to deal with SCLS... because it can truly be difficult ( and I think you can all relate unfortunately).

By the way, I have contracted Dr Druey and Dr Pineton de Chambrun for support in acquiring the right diagnosis. Dr Druey is retired now, but Dr Pineton de Chambrun stated his willingness to help... so amazing that these doctors are doing so! He will review my case. Have any of you also had experienced with these doctors in assisting you in your case? And if so, what did this assistance entail?

 

I am sorry for my many questions... so once again, thank you for your efforts in reading and maybe responding. Please let me know if I can assist in sharing literature or experiences of some sorts.

 

All the best!!

 

Regards,

 

Marielle

My WBC and Absolute Lymphocytes are consistently below range  

If neuropathy symptoms are present please rule out B12 deficiency which can cause wide ranging neurologic symptoms and is easy to remedy. If treated early enough one can reverse early symptoms of neuropathy and avoid more serious complications of B12 deficiency. I had 3 years of cascading multi-systemic symptoms before finally being diagnosed with B12 deficiency twenty years ago. 

My doctors tell me B12 deficiency is often seen in those with MGUS (I do have M protein) and of course MGUS almost always present in SCLS  It all seems to be related, they just don't know why.

 

Dear Cara Cozine,

 

Thank you very much for your reply!
Are you being treated for the SCLS? How do your doctors deal with your low WBC/Lymphocytes? Do you also experience this during attacks?

Thank you for the tip on B12 deficiency. I have had a deficiency 10 years ago (which they found due to spontaneous small bruises all over the body), long before the start of SCLS, and have ever since been treated with a weekly injection of B12. I am so sorry to hear you have also suffered for so long with B12 deficiency before they diagnosed you correctly.

Interesting correlation, the B12 and the MGUS... It would be nice if more research would be devoted to the possible underlying mechanism of this correlation... 

 

Anyhow, thank you for reaching out!

I was diagnosed with SCLS in Dec  2022 and have been treated with IVIG infusions monthly since Jan 2023. My first SCLS attack was in 2018. I had 4-5 attacks before one triggered by COVID landed me in the hospital for 8 days. That prompted me to go to Mayo Clinic MN where a hematologist diagnosed me nearly immediately.  I had a couple of attacks where blood work was on record and then the hospitalization was well recorded. Most of my leaks resulted in 7-10 lb weight gain ; 30+ pounds in hospital. 

There was only once my WBC was low enough to be concerned. It was retested in two days and had improved. These abnormals are now my new normal. My platelets run low between around 90-100, but not dangerous and normal for me.