I would like to know what others experience as far as side effects from IVIG. My daughter is on Hizentra ( scig ) . Her side effects are so much less severe than when she was on once a month IVIG, but she still has many inconvenient and uncomfortable side effects. They are definitely mild and tolerable! However , her being only 8 yrs old, they do affect her everyday life . I'm not complaining , as we are thankful for this treatment . Just wondering what others experience . Thank you :)

Your child may well be the only child -- in fact, she may be the only, or one of two or three, patients diagnosed or suspected of having SCLS -- in this virtual community who is receiving subcutaneous immunoglobulin, which is a rather new product on the market. Hizentra delivers immunoglobulins at twice the usual concentration, namely 20% rather than the usual 10% featured in intravenous immunoglobulin (IVIG). The advantage is that one can use a small needle and infusion pump and administer it subcutaneously and not into a vein, such that it can be injected at one's convenience by a trained adult at home or elsewhere. The disadvantage, I gather, is that one's body may react much more severely to the concentrated product than the rest of us do to IVIG. According to the manufacturer, CSL Behring -- the same company that makes Privigen, which is what I get intravenously -- "in the U.S. clinical trial for Hizentra, the most common adverse reactions were redness, swelling, itching, heat, or pain at the infusion site, 93% of which were mild while 6% were moderate. Other common side effects observed were headache, vomiting, pain, and fatigue." Talk it over with your doctors, but it seems to me that the practical choices you have are (1) to slow down the infusion rate; (2) to give her a half-dose of the medication every week instead of a full dose every other week; or (3) to switch back to IVIG on an every-other-week basis. We have several adult patients who get IVIG this way -- a minority -- either because the side effects of a once-monthly regimen were deemed to be intolerable, or else because receiving half a dose every 2 weeks seems to prevent what may have been mild episodes of SCLS towards the end of the 4-week period.

Leila could not tolerate monthly infusions, she became violently ill after every infusion. So originally when we switched to scig it was 3x a week at home infusion . Now it's 2 times a week . It's very unpredictable when she will have side effects.... She almost always has fatigue , but the headaches are very random . The things that seem to help lessen side effects , are rest , Benadryl and the most important is hydration. So basically during the hours leading up to infusions and after infusions are spent drinking water and resting , no vigorous playing and sweating because she will for sure suffer a headache if she's not well hydrated. Sometimes headaches are two days after infusion. So what I gather is there really aren't any people here to compare scig side effects with because Ivig side effects only happen the days following large infusion? Well if anyone else in this community has switched to scig , Leila and I would appreciate any input.

Also , thank you for your responses . And thank you Aporzeca for always having helpful input :)

My local physicians are wary of starting me on IVIG due to the fear of anaphylaxis. They want me to wait for my appointment at Mayo Jacksonville. I am experiencing frequent episodes of moderate swelling and significant dehydration very frequently. Any advice?

Susan, My best advice is to have your main physician make a referral for you to visit Dr. Kirk Druey at NIH. Have him/her email or call Dr. Druey ASAP. Your medical history will have to be sent in for review. If you have had symptoms consistent with SCLS, you are likely to be accepted and invited in to Bethesda, MD, for a thorough examination of your case, which takes place during about 3 days. All kinds of NIH specialists will study you and, in the end, give you their best, highly educated guess as to your diagnosis. And once you have a confirmed diagnosis, the treatment options can be discussed and decided upon rationally. A small factor to take into consideration is that, once you arrive at NIH, everything that they do there for you is free of charge. (You'll really feel like you're finally getting something for your taxes!) Trust me, it's a lot better and cheaper than going to the Mayo Clinic, where they don't know much about SCLS beyond what has been published about it -- especially in their Jacksonville satellite! Arturo

Group, I just recieved my latest dose of IVIG last Monday and Tuesday. It seems that the infusion center here switched from the 5% Octagam to the 10% Octagam. I was pleased to see this as the infusion bag was much smaller and the time to infuse was much quicker. This was more similar to the Privigen doses I had started with. The Privigen and now this 'new' Octagam only took around 4 hours vs the 7 or 8 hours the more dilute Octagam I used to get. The issue I had both days, was a rash that occurred primarily in blotches on my arms...one arm one day and the other arm the next. Plus had some in my arm crease and groin area and up to around my waistline. These rashes were raised a little and itchy but not severe in either case. They went away in a few hours after the treatment both days with another dose of Benadryl and also used some topical Benadryl creme. The pharmacist didn't seem to think, slowing down the infusion rate would help. Anyone else run into this? For me I see it as a very minor inconvenience as it goes away quickly and the thought of a SCLS episode compared to a short term rash is not even close....but the nurses and Nurse Practitioner sure seemed to get excited and concerned. Any thoughts/advice appreciated. Marc

Your body reacted to the more concentrated IVIG, that's probably all -- and quite understandable. I suggest you take a Benadryl before leaving home for the infusion. That way it'll be in your system longer and have a chance to prevent an allergic reaction. Talk it over with your doctor and inform your infusion nurse if you go through with it, in case they do/not want to give you another Benadryl.

Thanks Arturo. I will discuss with them. It seems their concern was that maybe the reaction gets more severe over time? At least that is what it seemed to be, as I was not that worried. In fact I didn't even mention it the first day as I did not want them to stop the infusion. IVIG has been a very very successful treatment for me and I do not want it jeopardized. The reaction seemed to come on near the end of the treatment, so I was wondering if giving another dose of Benadryl at the end might be even better. Maybe the earlier dose was wearing off. If I read it right, Benadryl daily limits are 300 mg. I get 50 mg of Benadryl (along with Solumedrol and Tylenol) as premeds. If 300 is the daily limit, it seems you could take 50 mg 6 times throughout the day without going over labelled limits. That is every 4 hours. It was getting to be close to 4 hours after I got the dose of Benadryl, that these red skin bumps/irritations began. They offered me more Benadryl at the end of the treatment. They said I could refuse and take at home if I wanted to, so I did that and within a few hours.....all clear. Thanks again Arturo - it is great to have this opportunity to share and then get feedback from you and others on the site. The idea of having others that 'really understand', is a god send. Marc

My doctor prescribed the steroid Prednesone the day before the infusion, the day of the infusion and the day after the infusion. Ten MG twice a day (take with food). I have never had a reaction. I also get the Benedryl. My infusion rate (Previgen is all I use) is 180 ML/hour. I get one g per kg of weight (850ml). I am on my fourth year of monthly IVIG treatments with no episodes or problems. Ask your doctors about the steroid.

I take Benadryl for two days after the infusion and, get Prednesone in a IV before the I V I G .The Only reaction I get is from the Prednesone is getting about 4 Hrs. sleep,after the I V I G .Before starting the Benadryl I would get the rash and the raised red itchy bumps.

I was dehydrated during my most recent 3-day infusion and experienced headaches, fatigue, swelling, and spikes in blood pressure. My next round begins on Tuesday. I plan to carry a satchel with plenty of fluids and to post-medicate with Benadryl and sumatriptan[Imitrex], as necessary. Over time, I hope to learn to take the side effects in stride, but they were quite impressive this month for about ten day. Nonetheless, thank goodness for this treatment. Thank you, Custom and Barney, for the idea of Benadryl post-infusion.

Hi Susan.. I find that of all the oral medications, Telfast 180mg (fexofenadine) is the most important. I take it every day without fail with my Singulair, and theophylline.

Thank you, John. This is most helpful. I shall be able to purchase fexofenadine over the counter as Allegra in the States. When you mention taking it every day, do you mean each day of the infusion or each day of the month? Thanks in advance, Susan

Every day of the month. To my thinking, it is approaching the same question of histamine release as Singulair (montelukast), but from a different angle. It has absolutely no sedating effect on me, and I just feel better on it... And it is OTC!

Is this for the side effects of IVIG, John or for the SCLS? I tried Singulair during it's medical trial period many years back and it had no effect. Do you think it may be the combination of the two?

I get ivig 2 weekly, and I feel better during and after by using telfast. I found IVIG did not last a month, and have been able to measure the levels of gamma globulin just prior to infusion, when they should be at their lowest point. I just "feel" that the Telfast helps not only the IVIG ADRs, but also the disease process SCLS, itself. I may be wrong in this. I really am not convinced whether singulair works, but am not game to stop it. Please let us know how you go? John

I totally understand how you would not wish to stop the Singulaire. I would be the same! Interestingly I have had severe inflammation of my liver in he last few months, due to a reaction to erythromycin which I was prescribed ( 3 courses of) due to an infection in my leg from an accident on the road. My leak went. I lost a large amount of weight as I was nauseous and could not eat. I am starting to feel better and my high liver enzyme count is reducing and guess what- my leak is returning! Coincidence? Maybe, but interesting..'. C'mon John, you are a lateral thinking medical man. Any ideas?:) IVIG also induced liver inflammation which is why I had to stop it.

Whooah Windows! I am a fan of lateral thinkng, BUT! There are several issues which you raise, and I fully appreciate your ordeal, and sense of dealing with the unknown. First, peripheral oedema makes you more prone to infections in those limbs. Second, erythromycin makes you prone to liver damage, and also nausea associated with GI dysmotility. Third, there may be a few mechanisms for your temporary recovery. It may be coincidence as you mentioned. It may be due to enforced rest with illness. It may be that you have been producing more IgG due to infection. It may be that your body, ( and mine) relies on IgG to protect us. IgG can be catabolised by a healthy liver (inter alia) and if the liver is diseased, the availablity of protective IgG may be raised. Windows, there are so many variabilities, that Dr Druey's work is paramount as he is able to centralise the data. Just from my own perspective the kinetics of my own infused IVIG are the basis of understanding what is going on. It is important for me to measure IVIG at maximum level after infusion, and at minimum, trough levels before the next. Part of the mechanism of action may purely come from its hyperosmolar nature. Is it possible to find another hyperosmolar solution to use during an attack? Maybe? Check with your Dr. Hang in there, buster! John

Well there you go! You have raised a number of very interesting points all of which I will have to digest. Yes my IgG levels were raised a lot and I think they are down now although I have not had a blood test this week. And yes, less activity although still had to move my horses and pick up their manure every day regardless, which normally makes me swell with the exertion and heat ( it has been a very hot summer here). I am not too sure as to what hyperosmolar means although I did google it I will let Dr Druey know as it may be helpful to someone somewhere... Thanks so much for your thoughtful reply:) Am still hanging in!! Sarah

Hi Sarah, Interesting that the 'bending over and picking up ' is raised yet again... I find that 3 or 4 times is enough for me...I automatically hold my breath while bending over, then straining slightly standing up.. Known as Valsalva Manouver, and is just not good for me. Perhaps you need to consider a stand up type pooper scooper? Lol. More than that..if the horses soothe your soul, they are the best medicine! Then you can send the proceeds to me for my veggies! Another point which comes up time and again is the question of temperature, and also exertion. IgG is bound in the CVS and prevented from degradation by FcRn receptors. This process is influenced by temp, and low pH. I am not sure that there is sufficient variability in vivo for this to influence the SCLS. There is sufficient reason theoretically to get tested for cryoglobulins. Heavens, we are complicated people! Like your new pic! John

Goodness this is so complex! I have had a good physiology lesson as have googled all the medical terms you mentioned:) Yes, temperature and exertion is mentioned in nearly everyone's bio on this board so is a common thread for sure. Maybe I should get a stand up type pooper scooper indeed. At the moment it is bending over and thick rubber gloves.Ugh. My face always swells like a balloon Coming your way- a load of poop for you vege garden! Nothing like it. And you are right. The horses give me my calm time out.Nothing like that either for the soul.. Thanks so much for your comments. Much appreciated Sarah

My nurses recommended using reactine(cetrizine hydrochloride) when I got home from treatment and then the next day. As well, they recommended using gravol with advil/tylenol to help treat the migraine. This has helped me tremendously.

Thank you for the migraine tips. I also use Imitrex. Mine seem to start two days post-infusion and last for four days. They are almost intractable.

I had the center slow down my infusion rate, it seems to have helped greatly with the headaches.

I am currently infused five hours per day over three days. Is this average?

Hi Susan and Ritz, My nurses are very cautious about slowly increasing the rate of infusion so as to avoid headache, because that MAY be a sign of bad adverse effects. So my first 200ml bottle of 20% takes about 2 hours... But the whole 5 bottle infusion takes 5.5hrs. I get 5 bottles alternating fortnightly with 4 bottles. (90kg @ 2g/kg/month). Ie 11hrs per month approx. It is not too onerous. Indeed I am eternally grateful for it. I also take low dose aspirin for a couple of days leading up to the infusion to prevent any clotting tendency.

Very helpful, John. My headaches began 48 hours post infusion and recurred daily for six days. They did, and do, wake me up and are accompanied by nausea. They are responsive to Imitrex if I can keep it down. Thanks to your Allegra-Benadryl tips, they are slightly less severe and diminishing more quickly this month, in only four days. I am also being more proactive and aggressive with the halt of peristalsis, with limited success. Lots of distension and severe cramping until day 8 post-infusion. I hope this helps others, Susan

It sounds as though you are winning Susan? Hopefully It sounds as though you are having a tough time. Where do you get the cramps? In your stomach or muscles? John C, a question for you. Is the IgG kicked up during injury and inflammation the same as the IVIG or am I barking up the wrong tree? Sarah

Easy Sarah, No. It is the infusion which raises IgG. Other noxious influences can also slightly raise IgG, but this also depends on the personal response. Some people cannot respond to harmful stimuli. Some over-respond. Abdominal cramps, gastro- reflux, - they all happen, and we don't know why they happen with SCLS. When all is said and done, IVIG is the single factor in helping. It works 85%, (not 100). And the future for us depends on pooling these comments through Dr Druey. Mt grand-daughter said, today, that I should get a fitbit, because that would fix me! Lol. We all have our own road to travel! John

Woof,lol. Ok so theoretically having my IgG level raised by being ill and having inflammation of the liver could have simulated the same conditions as having the IVIG infusion? I feel I felt better on the IVIG... after the initial sorting out of the rates of infusion. Sorry if I appear to be stupid (I'm not!) but not having one single leak during the 6 months of being ill is tantalising! I also did not have muscle cramps during that time either and am back in full force now with the slightest physical exertion. Your grand daughter sounds gorgeous:)

John I have a fitbit, it has fixed me but it does remind me to be active. I feel better if I don't lay around feeling bad. Getting up and move even seems to cut the headaches a little.

I love technology! It can be soo motivating! However my wife had banned any heart relTed exercise because I am inclined to overdo it! Instead she suggested a jawbone! Lol