Nolan has been having increasing severe attacks over the last month. The last one on July 26th landed him in the hospital for a week. He just got home yesterday. He narrowly escaped dialysis. He got his first dose of IVIG last Monday and had a reaction to it at first. (Chills, hard time breathing, and drop in blood pressure.) It was quickly reversed when they stopped the dose. He received that rest of the dose the next day and had no problems with a slower rate and premedcation with tylenol, benydral, and predinsone. We are trying to get monthly injections for him. Is not the dose suppose to be 2grams/kg? My insurance said that they would pay for it. We hope that this will help him. Thanks, Wendy

Dear Wendy: On the one hand, it is really too bad that Nolan has been having increasingly severe attacks in recent weeks. On the other hand, it is great that his insurance company has agreed to monthly treatments with IVIg -- and indeed, 2gr/kg is what they do in Europe and parts of Canada. Would you mind sharing with this SCLS community how you and your doctors achieved this feat? I wish you both the best of luck.

désolé de ne pas pouvoir communiquer en Anglais.. Je suis bien contente que votre compagnie d'assurance puisse payer votre traitement. Moi, j'ai un traitement mensuel depuis 1 an et tout va bien depuis. avant mon traitement, j'étais hospitalisé à chaque semaine. Ma dose de IVIG est aussi de 2gr/KG mais sur 2 jours donc, je reçois 1gr/KG la première journée (en 5 heures) soit : 30 ml/ hre pour 30 min. 60 ml/ hre pour 15 min 100 ml/ hre pour 15 min 200ml/ hre pour le reste des bouteilles. et le lendemain, je reçois la même dose. je pèse 67 kg donc je reçois un peu pus de 3 bouteille de 200 ml. aussi, la veille de mon traitement, je dois prendre 150 mg. de predinsone. soit 50 mg. à 6h00 le soir 50 mg à minuit et 50 mg à 6 heure le matin. le mois passé, j'ai oublié de les prendre et j'ai eu une réaction allergique...alors c'est important pour moi de les prendre. Je suis contente de d'autre personnes essaie ce traitement qui fût tellement pour moi tellement bénifique. Bonne chance. et si vous avez des questions plus technique, je suis certaine que mon médecin se fera un plaisir de vous répondre. Bonne Chance. Je ne sais pas si le monsieur qui a traduit mon texte pourra le faire de nouveau. si oui,,, un gros MERCI Josée

This is my attempt at translating what Josée wrote: "I'm so sorry I can't communicate in English! I am happy to hear that your insurance company will pay your [husband's] treatment. For my part, I have been getting a monthly dosis during the past year and all has been well since. Before my treatment [with IVIG], I would have to be hospitalized every week. My dose of IVIG is indeed 2gr/kg but spread over 2 days, such that I receive 1gr/kg the first day over a 5-hour period: at first 30 ml/hr for 30 minutes; 60 ml/hr for 15 minutes; 100 ml/hr for 15 minutes; and then the remainder at the rate of 200 ml/hr. And the next day, I receive the same quantity at the same pace. I weigh 67 kg, and therefore I receive a little more than 3 bottles of 200 ml each. Also, the day before I get IVIG, I must take 150mg of Predinsone: 50mg at 6PM, 50mg at midnight, and 50mg at 6AM. This past month, I forgot to take the Prednisone and, sure enough, I had an alergic reaction [to the IVIG], so it's important for me to take the Prednisone. I am pleased that others are getting to try this treatment plan that has been so beneficial in my case. Good luck to you, and if you have more technical questions, I am certain that my doctor will be pleased to answer them. Good luck!" Josée: Donnez-nous s'il vous plaît le nom, l'adresse, téléphonez et les renseignements de contact de courrier électronique de votre docteur et hôpital?

I concur the dose is 2g/kg but spread over 2 days - the information about the steroids and the rate of infusion is useful. Phil Greipp

Un gros merci à Arthuro pour la Traduction. le nom de mon excellent médecin qui a su diagnostiquer cette maladie en moins de trois jour est le Dr. Sephr Javaheri. il travaille à l'hopital d'Amos au Québec. vous pouvez le rejoindre au numéro suivant. 1-819-732-3341 poste 2444 l'adresse électronique de sa secrétaire est le: monique_brouillette@ssss.gouv.qc.ca Pour la dose, je crois que Claude (qui est inscrit sur ce site) reçois la dose la même journée. mais pour moi, ce n'était pas possible, j'ai vraiment une réaction lors que je reçois une dose trop grosse. j'espère que Nolan pourra recevoir une dose à tous les mois..... c'est MERVEILLLEUX.... Josée

Hi Iam kind of a computer geek and I have found that if I hight Jose'e text that a arrow symbol appears at the bottom of the text. If I click on it a menu appears. One of the choices on the menu is google translate. It does a nice job at translating her posts into english for me. Hope this helps Ritz

Thanks for your help with the dosing information. We really didn't do anything special to get our insurance to approve the IVIG. I guess they were tired of paying $10,000 every time Nolan takes the life flight to Salt Lake City. He will receive his second dose on Wednesday and Thursday this week. Nolan hasn't had any more hypotension since he had his first dose of IVIG. He has had intestinal bleeding. He bled enough last Sunday that he had to have 5 units of blood. They have scoped him up and down and found no source of bleeding. Luckly, he stopped bleeding on his own. He is not on any blood thinners at this time. He will swallow a camera next week to see if they can find a source. Has anyone else had trouble with intestinal bleeding?

Bonjour, Je vais penser à toi Nolan, car j'ai moi aussi mon traitement Mercredi et Jeudi. De plus, le 26, cela fera un an que je n'ai pas eu de crise......l'immunoglobuline, c'est merveilleux !!! Moi, je n'ai jamais eu de saignement intestinal. Bonne Chance à toi. Josée

I have an intravenous Immunoglobulines (IVIG) treatment since April 2005. Between 2003 (first shock) and 2005, I had 6 differents severe capillary leaks. Since 2005, I had no new capillary leak. The IG have well known side effects : headache during the infusion or in the days following the infusion; potential allergy (rare); phlebitis at site of infusion; potential transmission of viral diseases (HIV, hepatitis, etc). The different preparations of IG are probably all effective in preventing the recurrence of a leak and I myself had tested 3 different IG without new episode of leak. The dose proposed by Prof. Amoura (Paris) is 2 g/kg of weight, ideally infused during 2 days. In my case (70 kg) I have a monthly infusion of 140g IG during 12 hours to avoid the occurrence of phlebitis (often after the 20e hour of infusion). The pharmacist conditions IG for me as follows : 2 x 1 infusion bag of 70g (Octagam 5 % = 1400ml) + 600ml glucose 5%). Total of 2 bags = 4000ml. Furthermore I take 20 mg Prednisone 12 hours before infusion (H-12) and the same dose 2 hours before infusion (H-2) ; diclofenac 50mg (H-2 and H+6); anticoagulation using enoxaparine 40mg (Clexane®) subcutaneous (H=0). Since using IVIG I never presented any particular problem concerning cardio-vascular nor renal dysfunction. Claude Pfefferlé

Claude-Do they mix the glucose 5% into the Octagam? What is the reason for the glucose? What is diclofenac and enoxaparine in American?

I dilute the Immunoglobulines (IG) in order to reduce the side effects. Using dilution with glucose 5%, I have less headache and I avoid the phlebitis. Diclofenac belongs to the group of drugs called nonsteroidal anti-inflammatory drugs (NSAIDs). It works by reducing hormone that causes inflammation in the body. Other name of specialty: Voltaren. Cataflam, Flector, Inflamac, Olfen, Grofenac, Diclosifar, Diclo-Basan... I use anticoagulation by ENOXAPARINE (heparine with low molecular weight) because during infusions, I remain in my bed and I want to avoid a recurrence of thrombosis of my lower limbs. I have already done 3 thrombosis of lower members when I took 2004-2005 an experimental treatment against SCLS including thalidomide (300 mg/d), corticoids (dexamethasone 40 mg /day during 12 days/month), and tranexamic acid (cyclokapron 3x500mg/day). Claude

In the USA our NSAID's are acetaminophen, ibuprofen, naprosyn like alleve, tylenol and advil. I infuse lots of Octagam at Johns Hopkins but we are not allowed to dilute it. Another person with SCLS had thrombosis during leaks. How did the thalidomide work? NIH and Mayo are considering revlimid.

Arturo, thank you once again. I have reposted into the discussion forum you have dedicated to Judy. Ruth