I had originally had 1g/kg IVIG. I had trouble with it in oupatient because I had 24 hours only in the infusion center. It was pretty hard on my kidneys. As far as the dosing, do people get the IVIG based on their original weight or the weight with fluid gain? It makes a big difference. We are now doubling the dose and dividing it over 2 days. This still ends up being the same amount daily, and I'm worried about kidney pain. I asked my doc to try every 2 weeks instead of once a month so it didn'y hurt my kidneys so much. The dose is questionable though, as he doesn't seem which weight to base it on. Anyone else know?

 

The standard dose of IVIG for confirmed SCLS patients is 1 gr/kg in Day 1 and another 1 gr/gk in Day 2 every 4 weeks, based on someone's normal weight. 

Some patients cannot tolerate so much IVIG in one (two-day) sitting, so they get 1 gr/kg in one day every 2 weeks.  Some are also experimenting with subcutaneous (as opposed to IntraVenous) IG, which is the way children oftentimes get their IVIG.  One could envision an adult getting 0.5 gr/kg subcutaneously of IG (ImmunoGlobulins) once a week, for example.  This should all be discussed with an immunologist or expert internal-medicine physician.

SCLS patients usually gain weight when given fluids in an ER or ICU hospital setting, but they do not normally gain weight, or keep earlier gains in weight. 

If you are putting on and keeping weight, to the point of wondering which weight to consider in the proper dosage of IVIG, then it makes me wonder whether your diagnosis of SCLS is appropriate. 

Thererfore, I urge you to have you main doctor contact Dr. Kirk Druey at NIH, email kirk.druey@nih.gov  to request a consultation AND make arrangements for you to be seen at NIH, in Bethesda, MD, right outside the District of Columbia.  Dr. Druey is not only the world's expert on SCLS, but he is also a specialist on Mast Cell diseases, which you wrote on your profile page that you suffer from also.  And when/if you are examined at NIH for 2-3 days, Dr. Druey will have you seen by several specialisits in the other diseases which you may be experiencing.  And since they don't charge anything -- our taxpayer dollars fund NIH -- it's a lot cheaper than spending 2-3 days at the Cleveland or Mayo clinics.

My doc is in contact with Dr Druey at NIH. The first dose I was given was at the height of fluid accumulation. The weight used for the dose was based on my swollen weight. I did not tolerate it well. It was 100g of IVIG and given over 24 hours. The hematologist who consulted afterward said it was too much too fast. It was 27g more of IVIG than if it had been based on my pre-swollen weight .  My kidneys didn't take it well. No more IVIG has been tried again since then until a better plan was made and other specialists and NIH consulted as well. Now that I'm swelling again, IVIG is being started again. This time they are basing it on my base weight. This is obviously differnet than what was first done, so I didn't know which was correct. I'm getting 1g/kg on day 1 and 1g/kg on day 2. Hopefully this time it will work and I will tolerate the dose every 4 weeks. I am pretty desperate as my CPK is high.

 

Hi guys, 

     Just curious about chronic leakers who may gain a significant amount of weight during prolonged episodes?         Does the recommended amount of Flebogamma increase during prolonged episodes?  

     Last year 2016 my Dr. raised the amount of Flebogamma I receive from  30g  1x  monthly to  30g  2x  monthly          (a total 60g Flebogamma monthly)  The results were wonderful my body shed 25 pounds in 3 months, my blood results improved and most importantly I managed to avoid visiting the ER every month for an entire year as of March 2017.   YEAH!!!!     

          Although my blood results improved over the last year, the majority still showed multiple episodes of capillary leak, my Dr. suspects that I may be a chronic leaker. 

     Before SCLS my normal weight was 132 pounds after SCLS  I normally weigh around 164 pounds.                               ( I believe that my initial weight gain occurred due to my altered lifestyle.  I used to live a full, active life and then SCLS came along and brought the gifts of lethargy, lack of exercise heat intolerance...)                                             Now whenever I am in capillary leak my weight can get up to 190 pounds!!  

      My Dr.  insists that I rely on BP, weight fluctuations, changes in the color of skin (I appear red or sunburnt during an attack) ... Before he is willing to consider increasing  the amount of Flebogamma I receive, he informed me that each day I must take my BP, weigh myself and photograph the color of my skin, keep a daily record and in 3 months time he will review these results and consider petitioning the insurance company for increase in the amount of Flebogamma I receive each month.  

So back to the original question does anyone know which weight measurement is the correct weight to determine the amount of Flebogamma?   Your normal weight or the weight you often are during an episode?

Thanks Kimberly