Dear All,

I am happy to announce i finally received my first IVIG treatment.

I suffered an acute SCLS attack on May 8th, 2019, after 3 years of the initial acute episode in 2016. 3 years on which mild chronic flares  kept me in constant alert.

I found myself on travels to a high altitude city (2,500 masl), whose pressure could have been the cause of the attack onset. Symptoms were acute diarrhea, extreme vomiting, painful cramps, myalgia, +4kg edema, severe dizziness, lack of breath, exhausting stomachache, long-lasting nausea, rhinitis and fainting sensation. Once at ER they found hypoalbuminemia, hyponatremia, high hematocrit and an important hypotension.

I was admitted for internal medicine care in which slow-paced fluids with sodium along with albumine and 250mg of methylprednisolone were instituted. I was discharged upon 3 days and flew back to Barcelona 2 days later. 3 days after landing in my city, the symptoms commenced turning stronger and stronger taking me back to the hospital where I still remain inpatient. I have had 3 attacks during these 10 inpatient days and finally, today Monday May 27th I finally started receiving IVIG infusions. They are presently instituting 25grs per day for 5 days resulting in 125gr for my 50kg of weight.

Moreover, I continue with my oral daily treatment with terbutaline, bilastine and spironolactone, however, throughout these 3 years, the doses have increased up to 40mg Terbutaline, 20mg Bilastine and 900mg Spironolactone (Crazy but imperative due to a high ideopathic hyperaldosteronism). 

It has been 3 years that I still experience anhidrosis (lack of sweat) and amenorrhea (lack of menstruation). My doctors and I have come to the conclusion that the lack of breath I experience happens to appear when in the prodrome phase.

I would appreciate some of your insight in regards to IVIG doses and the side effects you might have experienced. It has been 3 hours I am receiving the infusion with no headache or nausea at all.

I truly hope and wish it works.

Keep fighting and learning to live with our neighbour Clarkson, my friends.

Warmly,

Valeria

Gook luck to you, Valeria! Headache may occur 1 or 2 days after infusion.

Will your insurance cover for the IVIG cost?

Hi Nhan!

Thanks for the good wishes! I’ll receive the second infusion today, and on a daily basis until Friday. 

I’m still inpatient at a private care hospital, today’s my 11th day here. 

My private care insurance is fortunately covering IVIG without constraints.

In the meantime, the infusions prophylaxis details are pending to be settled by my doctors to every 3 or 4 weeks.

Upon documentation completion for my private insurance, I suppose they will authorize the above mentioned. Otherwise, public healthcare has already confirmed full coverage. I have to say that Spain is one of the greatest places to live with extensive complimentary healthcare and highly convenient private insurance plans. 

Have a blessed day! 

Valeria

 

Many thanks for the update and I hope that the IVIG works for you also.  Many of us had to go through a "baptism by fire" before receiving this treatment and stabilizing our health -- and we have the physical and mental scars to prove it, just like soldiers do.  The unfavorable side effects of IVIG are usually mild and temporary; they can often be managed by varying the speed of the infusion (the slower, the less the side effects), and by taking Tylenol before and after as needed.

I too get headaches from my infusions. That seems to be the only side effect. Sometimes I’ve had diarrhea too, but usual a headache. My first infusion was the worst headaches. I just finished my 15th round yesterday and I have a headache right now. Like aporzeca said, taking Tylenol as needed helps. I do take Tylenol and Benadryl as a pre-med before each infusion. I’ve never had an allergic reaction. The Benadryl helps me nap during my infusions though. My dosage is 80 grams of IVIG (Privigen), back to back days, lasting 4 hours each day. I start at 30mL/hr, every 15 minutes doubling up to 240mL/hr. So 30mL, 60mL, 120mL, then 240mL. I believe that’s based on my weight (220lb). Other than the headaches and sometimes diarrhea, I feel normal after each infusion...just a little drowsy from the Benadryl. I hope this is helpful. And I’m happy for you, finally getting your IVIG.

Hi!

Thanks for your kind input and wishes Arturo and DudeSCLS.

Unfortunately, IVIG has not worked for me since I experienced a SCLS attack upon the third initial infusion (day 3).

I received a daily 0.5grs/kg Privigen doses. It was programmed for 5 consecutive days

The side effects were extremely severe and painful. It took me 4 days to recover, however, I still continue having recurrent and breath-difficulting attacks here at the hospital.

Today is my 22nd day here and with much sadness IVIG has been dismissed from my prophylactic treatment. Infliximab was considered at first, however my doctors dismissed it prior institution due to a lack of solid evidence of SCLS patients treated with this therapy.

My doctors confessed to be worried and that they will continue seeking an alternative treatment. They actually tried to reach Prof. Zahir Amoura in Paris,FR, as he is Dr. Druey's European homologue, to no avail.

Arturo: If there may be any possibility that you could contact Prof. Amoura, or extend any other way to finally reach out to him, I would be extremely grateful. It is quite difficult to reach out to him, he does not reply e-mails and the only day that he is at his office is on Mondays; and as he is visiting patients, he does not like to be disturbed. I’m in real need of some assistance with this matter please.

Thank you very much!

Warmly,

Valeria

 

Hola Valeria, lamento tu respuesta a la medicación. Te cuento que en noviembre me comuniqué con el Dr. Amoura sin ningún inconveniente, le escribí a esos dos mail zahir.amoura@psl.aphp.fr,sophie.goulard@psl.aphp.fr, en su respuesta estaban los teléfonos :

Professeur Zahir Amoura

Secrétariat: 33 (0)1 42 17 80 01        

Ligne directe: 33 (0)1 42 17 82 44

Ligne recours lupus : 33(0)1 42 17 80 79

Espero te sirvan de ayuda...por otro lado, por qué estás con 0,5 g/kg día y no estás con una dosis mayor?

Lorena

Hola Lorena!

Muchas Gracias por la información, todos los datos que extiendes coinciden con los que tenemos. Esperemos que responda pronto.

Mi doctor, el Prof. Miquel Vilardell empezó a darme esta dosis porque dijo que es la dosis inicial en general. Es eso correcto? Peso 50kg sin edema.

Consultarte quién es tu médico, tu dosis y marca de Ivig y cómo trabajó el Prof. Amoura con tu médico? 

Muchísimas gracias por responder tan rápido! 

Si deseas, mi WhatsApp es +34666812169

Gracias miles nuevamente,

Valeria.

Valeria,

If you tried to contact Dr. Amoura directly without success, I suggest that, instead, you have your main physician contact him by email requesting an urgent consultation on your case, to include a summary of your medical history and the specific questions that he/she have for him right now.  Some of our physicians are not as comfortable responding to inquiries received from patients or caregivers as they are to inquiries from fellow physicians.  Lorena's experience with Dr. Amoura may not be typical because she is both a physician and a caregiver to an SCLS patient, and she probably identified herself to him as a medical professional.  In addition, if I were you, I would go ahead and schedule a regular appointment to see Dr. Amoura, because Paris is not that far for you and he has received our patients from throughout the European Community in the past.  Alternatively, your physician may approach a knowledgeable colleague of Dr. Amoura in his same department and hospital, such as Dr. Marc Pineton de Chambrun, see https://www.aphp.fr/offre-de-soin/consultation-37726 and email marc.pinetondechambrun@aphp.fr

IVIg Update

Hi!

I’m happy to share that I finally started receiving the monthly recommended 2g/kg IVIg dose.

Since my kidneys are somewhat fragile due to the recent severe attack followed by the several mild flares I suffered, my doctor decided to extend the IVIg on a fortnightly basis in lieu of 2 consecutive days. 

I received the first half on June 19th, 50 grs infused in 7 hours with no other side effects rather than a slight headache. I did sleep for several hours upon the infusion though. Preventive side-effect prophylaxis was administered prior to infusion.

In the other hand, the second half, (the other 50 grams), will be administered on July 4th, followed by the first half of the second infusion on July 18th.

Last but not least, I will fortunately -and finally- be discharged from the hospital on July 19th, after 2 long and intense months of a happy-ending story. :) 

Have a lovely start of the week and never ever lose Hope. 

Valeria

Ps.- Arturo, thanks for your kind reply about contacting Prof. Amoura and/or his team. I spoke to him on the phone in order to extend the importance of his opinion and he asked for my doctor to send him an email to better discuss, however, he did not respond to any of our emails. Luckily, Dr. Druey did reply in a matter of 3 days. A heartfelt Thank You Arturo, for your restless endeavor in helping and guiding everyone at this forum. 

Hi,

I received my IVIG every two weeks. I still have reactions but not near as bad as the every four week method. My kidney numbers vary as I also chronically leak. I am starting to think I have minor attacks every three or four months it I can handle them without a hospital visit. When my blood pressure is low but not a bad attack I receive extra fluids before treatment. My main problem is small intestine damage.

I hope all goes well for you.