Arturo and Group, I just saw in one post how one patient(a child) is getting Sub Q treatments vs typical IVIG? Is there a track record on this use for SCLS? Seems like something I would like to explore vs. the two full day treatments of Octagam per 4 weeks. The IVIG is absolutely amazing as I have had no issues since on this treatment whatsoever, so I do not want to appear dissatisfied. Just would be very less problematic due to scheduling around my work and challenges in finding a vein. Any thoughts or knowledge on how many patients receive these treatments, how well it works and other pros and cons vs Intravenous? Thanks and hope all are doing well! Barney(Marc)

Marc, Feel free to discuss this option with your doctor. IVIG is given subcutaneously (namely, under the skin but not into any blood vessels) using a small portable pump and small needles. It is usually administered to children, and adults are normally trained on how to do it to themselves at home. My understanding is that it delivers a dose of IVIG in half the time, because it comes in volume of 20% solutions as opposed to the 10% concentration of intravenous IVIG. This is both good and bad. I gather that the advantages are (a) self-administration, including at home at a time of your own choosing, and (b) a good alternative for patients whose veins are too small or have collapsed or are otherwise very hard to find. I hear that there are three downsides to it: (1) because there is only so much you can pump through a thin needle which doesn't go into your veins, you can only administer small doses at a time; (2) since the medication is all injected into one spot, localized allergic reactions and pain are more common; and (3) since IVIG is recommended in high doses for SCLS patients, you would end up having to inject yourself very often and in different spots. Let us know if you try it!

I can give details to how we do scig at home for daughter if you would like.? Just let me know.

Any details would be great. You can put them on a post here on Rareshare or email mhennen7@gmail.com Thanks!

Arturo, thanks for the info as well. Sounds like there may be some challenges. I am a firm believer in...if its not broke, don't fix it. But on the other side, I want to learn as much as I can and explore all options. If I discuss with the doctor I will post info for others. Thanks again, Barney

When we started scig it was every other day at home and they sent a nurse to teach me how to do the infusions . The infusions take a total of around 2 to 2.5 hours for my dAughter . We do a numbing cream first which stays on for an hour . She gets 3 needles and we tape them on , we use her thighs and alternate back and forth. Now she does 2 infusions a week instead of the 3 because with 3 a week she was just feeling terrible all the time. So after we tape the needles and actually start the infusion it takes about an hour because we start very slowly and then turn it on when she's more comfortable . She tolerates the needle sticks very well and has minor pain . Sometimes if we turn the pump on immediey she will feel increased discomfort so that's why we start slowly. Then untaping needles and pulling them out takes another 10 min or so. We always allow for extra time in case something doesn't go just right. The infusion sites are typically red and swollen but do not cause much discomfort , she just says they're sore . The worst thing about the infusions for Leila is the fact that she has very random but almost constant headaches , fatigue , backache , and chest discomfort . Sometimes she has a slight fever as well that we chalk up to infusions. Honestly it's so much better than the big infusions because she became so sick afterwards . Hope this helps. Oh and her brand of scig is hizentra and its 20 ml/4grams twice a week.

Many thanks for the all-important details, leilasmom31. To put it in context, an adult like me gets 90gr/900ml per day of infusion -- two consecutive days every four weeks -- for a total of 180gr/1,800ml every four weeks -- and that's at a 10% concentration. If I were getting Hizentra or some other brand which comes at a 20% concentration, I would need 90gr/450ml per day of infusion -- two consecutive days every four weeks -- for a total of 180gr/900ml every four weeks. Unless I made a mistake in my math, I would need 45 of your daughter's infusions (900ml/20ml = 45) every 4 weeks to get my 180gr of IVIG. Talk about becoming a pin cushion! No wonder we adults routinely get it intravenously! When you think about the subcutaneous alternative, it sure seems to be the only practical way.

To my understanding the scig is kinda like a maintenance . If you were to run labs and check what your igg level is shorty after monthly infusion that would give you a good idea of what it should remain at . So when you do scig three times a week it would be to maintain that appropriate level of igg in the blood . That is my understanding .

Group, thought I would reach out again on this topic. A new product in the Subcutaneous IG market has been approved about a year ago - called HyQvia. I did talk to my doctor about it a while back and she didn't say no, but steered me away due to the fact that the current Octagam 5% is working. The idea of sitting two days, 8 hours each, every 4 weeks vs. one 3 hour treatment at home is interesting. By the way, aside from the minor side effects and the 16 hours every month, the IVIG is a godsend. No issues, no symptoms...... Any more thoughts on SCIG from doctors, patients or family?

My son was getting extremely ill from his IVIG infusion which was every 3 weeks. He is now on the equivalent of 0.5mg/kg of Subcutaneous Immunoglobin (per 3 weeks) which my husband injects at home every 2nd day. This keeps his levels stable and does not make him as sick as the higher dosage, which would peak and then come down. It is much more convenient being able to administer this at home, and only takes approx 1 hour. He was receiving the equivalent of 1mg/kg, but he just couldn't tolerate it. I am told that he is currently under the recommended minimum dosage to treat scls effectively, and that we run the risk that he might get sick, but currently he is well and has been for the last 12 months (apart from occasional minor leaks, that he tolerates better than he did the IVIG).

My daughter is also still on scig , she does have side effects still that make her feel flu like . She had these side effects almost all the time , however they are better than the awful side effects of the large dose Ivig ! As long as the levels stay high in your blood , scig should work the same as Ivig . If you have any more questions that I can answer , I would be happy to help. Best wishes , Kristy and Leila

Kristy and Maccy80, thanks very much for sharing. A few questions and comments: Insurance on regular IV vs. Sub IQ? Similar costs and any concerns from insurance? My doctor seemed concern with Allergic reactions and being away from medical staff or facilities when giving Sub Q IV. Not an issue for you, in fact, your kids are having less side effects? I have a nurse case manager as part of the health insurer I have through work. I can run this by her as well. Advantages I see: Time, of course. Costs of sitting for two days in IV center(Insurance is currently paying but if product is more would service costs be less?) Flexibility(ie....taking two days from work and not being able to make meetings or travel....or my concern on job status overall and issue if I ever try to get a different job). I am now going on my 5th year of going in every 4 weeks and it is working so I keep going back to "don't fix something that isn't broken". The downside here is huge. On the other side, medical advancements are happening and would be terrific to take advantage if we can. Thanks again for sharing! Adults on Sub Q? Or is this treatment focused on children due to dosage or other reasons?

I asked a couple of doctors about this, and they said that given the large amount of IVIG that adults get because of their heavy weight relative to children, the administration of IVIG in the small doses delivered subcutaneously would probably take many days to deliver the dosage of medication that we adults can get intravenously in a session as short as 3 hours. Therefore, you are asking about a choice that, realistically, only makes sense for children.

Each infusion takes an hour. I'm sure you could call an infusion company like Walgreens and get some information from them. Also you could ask your hospital / Doctor where you get your monthly infusions about scig. If it's something you feel could really make your life easier , then by all means , look into it ! My daughter does infusions twice a week now , she's 80 pounds . She has only 3 needles per Infusion but I think they have infusion tubing with more needles which is needed for infusions that are larger . It becomes so routine after doing it for so long , my daughter has been getting her scig for almost 3 years now. It is really convenient , and we do it any time we need to. Sometimes we do morning , sometimes evening . As far as costs - I think the cost is similar, but our insurance covers it and doesn't tell me the cost! We have never had an issue with that. The allergic reactions happen so rapidly from Ivig , but I don't think they do with scig . You can and are supposed to pre medicate with Benadryl before infusions , and also we have an anaphylaxis kit at home that the infusion company gave to us to keep on hand. The scig is absorbed slowly over time , so a sudden severe reaction would be less likely I think . Again , you can always call the an infusion company and/ or look up Walgreens infusion services website . They have a decent website . We use hizentra. Hope this helps ! Best wishes Kristy and Leila

I am newly diagnosed and just met recently with an immunologist. She is hesitant to start any type of therapy because I am doing well with exercise, nutrition and vitamin supplementation. However, I was wondering if it is the clinical standard to receive treatment regardless of my current status to avoid any further episodes. I already have bilateral neuropathy in my feet stemming from compartment syndrome from an episode earlier this year.

Do not let your immunologist steer you away from IVIGG. There are not many doctors who are familiar with SCLS and the treatment goes against what they are comfortable prescribing. Within the small community that is SCLS there is only one person, that I am aware of, that has had one attack and no others without IVIGG treatment. For most of us, it is only a matter of time before another attack occurs. The only way to prevent the attacks is with IVIGG. My two attacks were a year apart. After the first attack, I went on IVIGG but only every 3 months. That is not the recommended dosage, but I thought I was fit enough to stay well. I wasn't and had another attack. After the second attack, I began IVIGG every month and I have been attack free ever since. I urge you not to wait getting on IVIGG. Talk to your immunologist and share this site with him/her. Contact Kirk Druey at the NIH to see if you qualify for the case study. His information can be found under the Disorder details tab and scroll down to the section titled Living With This Disorder. All the best.

Thank you for the valuable input. I would like to go to NIH to be seen.

I agree about the ivig and this being the treatment of choice for adult scls. Do not be lulled into a false sense of security in thinking that diet and exercise alone will prevent further episodes. This is not a disease to be taken lightly. Even treatments with theophylline are things of the past in my opinion let alone just diet and exercise. Please read the thread " Guy Allen Overland" under discussion forum started around 2/2015. This is my brother who tried to control his scls thru theophylline, diet and exercise. His first episode was 11 years ago and he did well for many years with no severe episodes. At that time there was no ivig and the survival rate was poor and theophylline was all they had. He did beat the odds for a while. He hesitated to start ivig because of this and unfortunately 2 weeks prior to starting ivig he passed away from a severe episode after doing so well for all those years. I will never forget Dr. Greipp's words 11 years ago when he was diagnosed: he said " this will happen to u again , we don't don't know when and how bad but it will happen again". Fortunately ivig has made that a statement of the past for most and if does come back it isn't as severe. But as I said thinking diet and exercise will control this is terrifying so please seek out a local doctor who will investigate,possibly a general practitioner and a hemotologist and it would be great if u could see Dr. Druey at NIH . If u cant make the trip then have your doctor call Dr. Druey and I am sure they would be happy to advise. Good luck and be well and please get the treatment u need asap. This is not a wait and see disease Linda