Hello, my name is Naomi, I'm 49 years old and I live is central Israel.

In 1995 After giving birth to my son I was diagnosed with rectal cancer and had gone through treatment, radiation therapy and surgery.

Between 2000 and 2001 I've began suffering from SCLS but I was not diagnosed with SCLS at the time. My doctors had no Idea what was wrong with me, I was leaking a lot and got a lot of blood donations since they thought that would help but it only got me to leak more and suffer more leakage. I almost doubled in weight and size and couldn’t move from the hospital bed.

after about 2-3 years that I spent most of it in the hospital suffering countless attacks and undergone all kinds of treatment in 2003 I was diagnosed with SCLS. I was treated with theophylline and terbutaline with now success. After a lot of bureaucratic work my doctors put me on thalidomide treatment and my attacks began to vanish.

I was treated with thalidomide for 3 years and then decided to quit the treatment because of its harsh side effects. For 12 years I had no further acute attacks!!

In December 2017, exactly a year ago today I've suffered an acute episode of the disease and couldn’t believe I had to go through this again…I've had excruciating pain in my legs and stomach I had a drop in my blood pressure and was rushed straight to the ICU. My son searched online to see what kind of treatment I should be getting since the doctors had no time for research as they had to keep me alive My son found an article about IVIG, he gave it to the doctors and I was treated with IVIG, Human albumin solution, Hypermagnesemia, ,Magnesium and Potassium.Crystalloid solution

For the past year I've been treated with IVIG 0.4 grams/kg which adds up to 30 grams for my weight. I've had no attacks since. But I have suffered serious allergies a few days after each treatment. Do any of you suffer from allergies after treatment?

I now look for a doctor to look after my case, but most doctors don’t want to deal with something they don’t know. I need to undergo some routine checks…I have a colonoscopy and gastroscopy soon and I was wondering if it is safe to do them?

 I'm scared I might have an attack during or after…

I felt alone dealing with this deadly disease and being the only patient in Israel with this disease. I am really glad I found this community. I don’t feel so alone anymore.

 Thank you for reading. I wish us all health and happiness.

Welcome to our community, Naomi!  I am sorry to read about all that you have been through.

First of all, you are not the first Israeli suspected of having SCLS.  In 2010, six Israeli doctors then affiliated with the Wolfson Medical Center in Holon, Israel -- and most are still there -- published an article about suspected SCLS in a child (then 8 years old) and also in several other members of his family.  Their names were Drs. Racheli Sion-Sarid, Tally Lerman-Sagie, Lubov Blumkin, Dominique Ben-Ami, Ilan Cohen, and Sion Houri, see https://www.ncbi.nlm.nih.gov/pubmed/20123768  While most of them are pediatricians, you have nothing to lose by contacting them, because they could refer you to colleagues of theirs at Wolfson who might be willing to meet with you and take you on.  The Wolfson is a research-centered hospital, so that helps in your situation.

Second, I suggest that you work with the medical doctor who knows you the best and longest to put together your medical history, as complete and with as much detail as possible (e.g., laboratory blood results), to have him/her send it electronically to Dr. Kirk Druey at the U.S. National Institutes of Health, requesting a consultation and his advice on your case.  He is the world's leading expert on SCLS, and his email address is kdruey@nih.gov  He is also an allergist, so he could have some relevant suggestions for you.

In general, SCLS and other patients receiving IVIG do not suffer serious allergic reactions a few days after their treatments, so you should consult with an allergist and/or an immunologist to see how to prevent them.  And in general, episodes of SCLS are not triggered by routine or non-routine medical procedures, especially in patients who have received their infusions of IVIG.

Arturo

 

Hello, first of all welcome to the community. As Arturo says I tried to tell your case to Dr. Druey. Your case has interesting peculiarities. It is not minor that you have been 12 years without attacks, without any type of medication. Do you know the criteria used for a dose of 0.4gr/Kg. As far as I know, the recommended dose is 2gr/kg, although there is a study group with 1gr/kg experimentally. Are you within these programs of study?

It will be difficult for you to find specialist doctors. In my case I found in my country a good general practitioner, curious, and with the support of a major university hospital, which I believe is what one can aspire.

Studies and surgeries do not seem to be a complication for those of us who have Leak.

Finally, as far as possible, try to change the brand (Laboratory) maker of the Ivig, to begin with.

Ariel.

Thank you both for replaying, sorry for the late replay

As far as I know I was the first case of SCLS in Israel, my first leak was in 2000-2001, my doctors told me about someone from northern Israel who died from the disease around that time.Yes for 12 years I did not have any major leaks, from time to time I have experienced a few 'small' leaks. I Believe the treatment I received back in 2003 had a lot to do with that, I was treated with thalidomide for almost three years, because of the severe side effects I decided to stop the treatment it made me pass out and it had effected the nerves in my toes.

when I decided to stop the treatment I did not experience any major leak for 12 years. and the spike in my GAMMA PEP blood test vanished. My recent blood test shows the spike again my PEP results are not thrilling, do any of you who get the IVIG treatment have seen better results at blood tests over time? Or do they stay the same?

I Know the treatment I receive now is not the recommended treatment I need to be getting at least 60 grams but since It's expensive I think they decided to give me less than that as I get 0.4 per gram it adds up to 30 grams which is not enough.  As to my allergic reactions It could be happening from all kinds of different reasons, but I associate it with the treatment because I did not suffer from allergies at all before the IVIG treatment.

Sadly I don’t have any Doctor right now who will translate all my documents. All I can do right now is have my son translating the current documents I own. Is it ok to send it translated by someone privately or do I have to get a doctor to do that?

I would like to say thank you again for answering.

Naomi

Naomi,

Of course, have your son translate and scan the documents you own, but then have the doctor who knows you best send them electronically to NIH with a request for a consultation about your case.

Arturo