Hello, I am a french woman who suffers of SCLS. My first attack was in 2009 triggered by fever,the second was triggered by a vaccine against the yellow fever in august 2012,because I share my life between france and africa. My third attack was just before Chritmass day the last december,I was hospitalized during 10 days and doctors dignosed SLCS,unfortunatly...... I am going to go back to the hospital the 11th of february for 3 days to get another exams....doctors said I will have every 3 months an infusion of IVIG . In france there's no forum about this disease it's that wy I am posting here ,to share our misfortune.....

Bonjour starfrance. Bienvenue sur ce site des maladies rares. Je suis désolé pour vous que les médecins aient confirmé le diagnostic de SCLS. Par contre je suis heureux pour vous que vous soyez de la région de Paris. C'est le site de référence européen pour cette maladie rare. Je suis à votre service pour toute question/renseignement. Je suis patient et médecin en Suisse et vous pouvez me contacter par mail : cn.pfefferle@bluewin.ch. Meilleurs messages. Claude Pfefferlé

Bonjour, Bienvenue à ce club très sélect des patients atteints de SLCS.! Gardez espoir car de nombreuses avancées ont été faites depuis que moi-même j'ai été diagnostiquée en 1989 (il y a 24 ans) et que seuls 12 cas étaient connus dans le monde. Il n'y avait alors que très peu d'information à la disposition des médecins, mis à part la communication faite dans les années 60 par le Dr CLARKSON qui a donné son nom à cette pathologie devenue depuis SCLS. Merci de consulter mon profil pour en savoir plus et, tout comme Claude Pfefferlé, je suis à votre disposition par mail à MAG.H@wanadoo.fr, nous sommes les preuves vivantes que l'on peut survivre, d'autant plus que le diagnostic est fait et que des traitements sont maintenant disponibles, ce qui n'était pas le cas pour moi à l'époque.Cordialement, et amitiés à Claude, Maggy

Bonjour Claude, tout d’abord merci de votre réponse, je m’appelle Anne-laure et j’ai 48 ans . Les médecins m’ont annoncé ce syndrome le 11 janvier,c’est donc tout frais...j’ai déjà passé des IRM,scanner, radio du squelette,prises de sang ,urines,myélogramme,et m’ont même prélever une carotte osseuse. Demain j’ai encore une IRM du cœur au centre marie Lann longue car pendant mon attaque de fin décembre mon cœur battait à 125 pulsations /mn avec flutter.La semaine prochaine je dois faire une scintigraphie puis le 11/02 je retourne pour une hospitalisation de 3 jour à l’hôpital de Percy à Paris.Ils m’ont parlé du traitement à l’immunoglobuline en intra veineuse,mais avant de commencer,ils veulent attendre d’avoir je pense tous les résultats. Ce qui m’inquiète, tous les 6 mois je dois faire un myélogramme,car lors de ma prise de sang durant ma crise j’ai eu un pic de créatine.....actuellement, mes résultats sanguins reviennent à la normale,mais l’inquiétude est là. Quel est votre parcours, j’ai lu sur le forum que vous avez fait 5 épisodes, donc depuis le traitement IVIG vous n’avez plus fait aucune attaque? est-ce bien cela? c’est rassurant car il y a deux semaines je me voyais vivre que quelques années !!! mais maintenant que je sais ce que j’ai, dès la prochaine crise je pense que j’aurai très peur de faire un collapsus, quand je pense que j’en ai fais deux ,une en 2009 et une autre en 2012 s’en savoir ce que c’était. Celle de 2012 a été provoquée par le vaccin de la fièvre jaune,car dedans, le virus est vivant et bien sur, mon cher petit syndrome c’est réveillé, mon médecin c’est focalisé sur une mauvaise et rare réaction du au vaccin alors que je lui ai dit :”j’ai les mêmes symptômes qu’en 2009,c’est bizarre ? “mais la prise de sang n’a rien montré d’anormal mise à part les globules blancs en grand nombre.... J’aimerai savoir, si le syndrome se déclenche uniquement lorsque l’on a de la fièvre ,ou peut on en déclencher une même sans fièvre ? dois t-on éviter les personnes infectieuses , grippe ,gastro, rhume etc , ou est ce notre propre système immunitaire qui est capable de provoquer de la fièvre et ainsi se déclencher ?qu’en pensez-vous? Cordialement,Anne-laure.

Anne-laure, Sorry I do not speak French. You had some questions about cases of scls, etc. Look under the disorder details and also the disorder resources on this website ( top left of the page) and you will find many answers. The disorder details which also includes a protocol for treatment is something that many print and carry with them so that the medical personal know how to treat an episode correctly and it also gives contact information for experts in this rare disease.France is a bit ahead of the united states in using ivig for prevention of episodes and treatment during episodes so you are in a good place although I know you may not feel that lucky right now. From what I know the risk for multiple myeloma is slight but I think it is something they check for periodically. I don't remember anyone that posts on this site having that as a subsequent diagnosis. This November my brother will have had scls for 10 years.He was 43 and in excellent health when he had his first episode which nearly cost him his life and left him with a dropped foot that he wears a splint for. He spent a week in a drug induced coma and had 4 fasciotomies due to compartment syndrome from the excessive fluid he was given iv. After he was diagnosed the protocol was followed during subsequent episodes so less fluid was given and he recovered with no complications. He is still in very good health,has a balanced diet, keeps hydrated with fluid intake , takes vitamins and exercises. If he starts to feel sick or run down he increases his fluid intake and just rests. He works full time, travels in he USA and abroad and leads a very full life. Be informed and proactive and inform friends and family of your condition just in case they need to assist you during an episode. I wish good things for you and your family. Linda

Hello Linda, Thank you for your advices but I am lucky because doctors to my hospital knew this disease,in fact they understoood when they saw at first my blood test.... I would like to know if this disease launchs only when we have an infectious with fever or it can be launch without fever? How your attacks arrives? I hope my english is not to bad......

Anne- laure, The triggers are different for different people and sometimes there may not be one. The experts are not even sure if the triggers cause the episode. Minimally I would say they interfere with your bodies immunity or immune response. Scls appears to be a malfunction of the immune system. Some reported possible triggers are flulike virus, upper respiratory infection, stomach flu,'airplane travel,dehydration, stress, many have episodes during the holidays( Christmas, thanksgiving) , exercise, the weather( too hot or too cold) , and other medical procedures. The episodes can be chronic meaning they leak a litlle bit all the time,acute and frequent(severe and every couple months) and acute and infrequent(severe every few years) . Some people think they can tell when an episode is starting but for many that has proved unreliable.. Under disorder details it explains the symptoms of an episode. If you have any doubt you should seek emergency medical care immediately. It is good your doctors are knowledgable. The information under disorder details and disorder resources just explains it in layman's terms and it is a very reliable reference to help educate yourself. If anyone wants to add to what I wrote feel free as I know I haven't included everything. Linda

Linda, My first attack triggered only by fever,the seconde by yellow fever vaccine and the third triggered in last december after I came in France for Christmass day when I left Djibouti city.It began by a upper respiratory infection with fever. Since 2009, I am always leaking a little bit, and more when the weather is hot !!! I live near Paris and you ,where are you from? anne laure.

Chère Anne-Laure, Je vous propose de me donner votre adresse électronique pour que je vous envoie un résumé de mon histoire médicale. Je vous donnerai également toutes les coordonnées du Prof Amoura ou de sa collaboratrice Dresse Gousseff (Salpêtrière). Votre médecin-traitant peut sans autre me contacter pour toute info pratique. Un résumé en français quant à la prise en charge est également disponible sur ce site. Salutations à tous. Bien à vous. Claude Pfefferlé / cn.pfefferle@bluewin.ch

Bonsoir Claude, je vous ai écris ce matin à votre adresse e-mail , mais le message n'est jamais parti... voici mon adresse : viv91@live.fr je vous remercie d'avance pour les coordonnées du Prof Amoura ou de sa collaboratrice.A Percy, ils m'ont dit qu'à la Pitié, ils s'occupent des maladies rares notamment SLCS.Un des médecins qui me suit est en contact avec cet hôpital et je pense qu'ils vont m'y envoyer par la suite. merci ,bonne soirée. Anne laure.

Hello Anne-laure. As others mention, the triggers or signs of an impending episode seem to vary from person to person. My husband has had episodes immediately following some infections (human metapneumovirus, gastroenteritis, staph infection). Yet, he has had a few minor respiratory infections (cold) that did not trigger an episode. He once had an episode immediately after a minor surgery (to have a port installed). Yet he has had minor surgery (to fix a skin graft) and dental procedures (fillings and crowns) that did not trigger an episode. He has had a few episodes within a week of receiving an IVIG treatment (IVIG brand doesn't make a difference). Yet he has several other IVIG treatments with no immune reaction (note: Monthly IVIG has reduced his episodes by 50% in 2012 compared to 2011. However, he suffers from side effects). Regarding symptoms, sometimes before an episode he has some minor swelling in the feet, neck or face, minor weight gain, dizzy spells, fatigue. Yet other times he has none of these symptoms before an episode. The one sign we can always count on is his heart rate. Anytime his heart rate has a sudden rate increase that can't be explained by physical activity, we head to the ER to have his hemoglobin checked. 99% of the time we discover he is having an episode. If you have any further questions about my husband's condition, do not hesitate to e-mail me at liesaevans(AT)live.com. Please put SCLS in the subject line so your e-mail gets through to me. I'm very sorry to hear you have this horrible syndrome, and wish for you the best of health. Liesa "Google Translate":http://translate.google.com/

hi i hope for all my best regardes i have my first dianos at 2010 in franch ( paris) by dr amoura hi is in Europe, Prof. Zahir Amoura, Département de Médecine Interne, Hôpital de la Pitié-Salpêtrière, Bd. de l’Hôpital 83, Paris 13e, tél. 0033 1 42 17 80 81 , email : zahir.amoura@psl.aphp.fr since my 2010 i us IVIG every thig is ok right now i start with 2gr/kg an now iam in 1gr/kg since 2 years so you leve there yuo can call hem and you have to go to him to visit you , i ll will be there at 15/04/2013 my best wishes foro you good bless you and all of us yaser

thanks to elganzory for the e mail adress . I wrote him yesterday and I am waiting for his reply. Josephite for the moment I am luky because my triggers start always by fever and vomiting then my body hurt........I am sorry for your husband who has severals triggers in many way,it doesn't be easy..... This disease is very crazy.....but we havn't choice unfortunatly, it's a big struggle against it. best of health at all !