Hi Holly,
Glad to hear she is getting treatments!!! Regarding the port, is it new? Kevin had difficulty the first 3 months. Mainly, because of swelling from the surgery. One other thing we learned, the standard protocol 3/4 inch needle does NOT cut it for Kevie. We learned that the reason he was having to be re-stuck is because every time they pushed the huber needle in, the port pushed down too. Once they did puncture through the rubber part of the port, the needle still was not in the port itself deep enough. We now use an inch and a half needle. When they do the first poke, it goes all the way in and once the wings are bent and it hooks on , it lifts the port back up. Once it is all said and done, the needle sticks way up and we have to build it up with sterile gauze before we put the tegaderm patch on. It looks ridiculous, the needle is obviously too long, but each time we try even an inch, it does not allow us access.

About the octigam, honestly, the literature I have read never says one brand is better than another, it only says most importantly, try to stay with the same brand all of the time. It seems as though the main difference is the detergents that are used to cleanse the ivig, for instance gammaguard and I think gammunex use glycine. The ivig itslef is ivig, antibodies. The only reason people try different ones is because they may have reactions to one more than another which is due to the diffferent detergents. S/D or powder form, also is different and sometimes harder on a person, it sure was for Kevin. Also, if Hailey has diabetes issues, make sure she is not getting a sucrose based product.

Good start on the epi-pen, but they usually send a whole kit, a saline drip and a little vial of medicine. Maybe it is the equivalent of an epi-pen, shame on me, I never looked to see what the vial says. I am going to look as soon as I get off line.

Good luck!
Dawn Kevies mom

Hi Holly, Just wanted to give you my opinion. I agree with Dawn on the ivig brands, and after a long discusiion with my neuro and my liasion, I got worse on Octogam, had been getting Gamunex. I am just one of many that can’t tolerate switching to different brands all the time. I now get gammagard and am doing well so far, with the premeds. Have sent in the GammaAssist form to Baxter, and have been reassured by the home infusion co that because they do more home ivig than hospitals, and they are a national co., that they have first priority in getting the ivig. My ER kit at home includes 250cc bag of Normal Saline, and amps. of epi and benadryl for any reactions. Sorry about your port issues, but they should get better in a few months. I still have peripheral lines, but likely in the next year I will need a port. I’ve been at it for 8 years, and so far, so good. I do have to say the pre meds made all the difference for me.
Good luck to you. Take care.

Emma

Holly

Dawn- Kevies mom and Kelly -Emilys mom have lots of ideas, love and support.

Watch for further posts. I am sure they will be able to give you some info.

Wish I could help more 😉

Rhonda from Canada

I have an 11 y/o boy dx at 10 w/cidp. Did she have any response to the ivig initially, how far apart were they? Children CAN tolerate a higher than 2g/kg dose. Did she get the repeat ivig 2-3-4 weeks apart. Currently Kevin gets loading doses monthly, but there is a girl named Emily, Emily’s mom, who got more than a loading dose weekly for a while and is now 3 weeks apart at a loading dose. PM her mom she knows the details better than I. Personally, I would try as much ivig before anything else. Obviously if there is no other alternative, you have to do the cell cept, steroid, ivig combo. give the high ivig a chance. Talk to your doc, I have some abstracts that I soon have to locate for another member and mail them to her for a doc appt. I would be happy to do the same for you. They talk about basic protocol, peds tolerating higher ivig doses and the benefits of ivig over long term side affects of cell cept etc. I have these because our doc was contemplating cell cept and I found solid evidence backing up my thoughts. Obviously, cell cept, steroids may be your only alternative, but it is worth exploring. I will check back later, going to dinner.
Dawn Kevies mom

Hi,
If she is around 70lbs, it works out to 12.8 g. I could be wrong, but I don’t think it comes in 1g doses. I thought it was only available 5,10,20g denominations. They usually round up to say 15. She may have needed closer than 30 days apart. The fact that she reacted instantly the first time is promising. The initial attack may not have been halted and that is why she relapsed. If it were my child, I would at least ask the doc to try again, with a loading dose and in two weeks another loading dose. Do that for a few months and then try every three weeks, it there is a relapse, back to two weeks. You asked about my son, yes, he is relapse free since oct 07 since we started doing the loading doses. Initially in Oct 06 he was dx w/gbs, given ivig and did well from a nearly paralyzed state, unable to walk, dress, hold a pencil or cup, bowels, bladder and breathing affected in picu for 10 days. He relapsed after about 5 months, cidp dx, more ivig, totally recovered in 3 load doses which is not typical, so gbs again, relapse 5 months on Oct 07 and here we are. Once you find the right amount of ivig needed to stop the auto antibodies from overtaking antibodies, you allow the body to heal. It heals a mm a day if no further demylienation (attack ) is taking place. I did the math a long time ago, it is something like 14.37 inches a year (check my math) But as soon as another relapse happens, back to that mm mark you start!! I do not know all of her history or symptoms and I do not want to give you false hope, but I really think it is important for your sanity to know there is a medicine, it is treatable, kids seem to respond and heal better than the adults and once a plan is in place cidp is managable and some people even go into a permanent remission. Some of the abstracts I will send support that. When this first happened, I wanted us all to die, I felt so low and helpless as well as hopeless. Confusion, lack of knowledge and being alone until I found this site. Now, that seems so long ago, and our treatments seem like no big deal. (Well almost, compared to the beginning) So don’t give up and come here when you feel sad and we can help. You can send a private message to someone on the site, go to their name and you will see the prompt. If ivig becomes a regular thing, you might want to consider a port, I can tell you more about it if you want. About the cell cept. If you read on line I am sure you see the side affects, but if you need it, you do. It is an immunosuppressant, it wipe out the immune system. not easy to deal with for a school age child, not to mention the side affects. She may not even be able to go to school during flu season. It is not to be taken lightly. Steroids too obviously come with huge side affects, especially with children and growing. If you would like, pm me and I could give you my phone # Be strong, we are here for you and have been where you are, we understand.
Dawn Kevies mom

Did not answer all of your ques. Cell Cept is not like pp. PP filters the plasma, cell cept is a immunosuppressant drug. Yes, you get the treatments before a relapse, the idea is to stay stable, no attacks, so that healing can start. The reason the reaction is instant, because at this point, the damage is probably just an inflamation on top of the myelin sheath. If left untreated, it goes throungh the myelin (protective coating on top of the nerves) and penetrates to the nerves causing axonal damage) You are on top of this and she is being treated, so that is not going to happen. Is your doc responsive to your suggestions/ questions? That is the most important thing. Don’t waste time if you don’t like him and go to the next doc. It took us 4 docs and a few mis dx. till we got to this doc.
Dawn

[QUOTE=Hailey Marie’s Mom]oh I forgot that also after the first plasma exchange, we did another 5 days of IVIG at home same dose 12 grams which I think is .4/kg of weight. Correct me if I’m wrong but she weighs around 70lbs.[/QUOTE]

Correct me if I am wrong anybody but I thought plasma exchanged would wash away the original IVIG treatments. I thought if you went the IVIG route then changed your mind and went the plasma route it is ok but it would wash out the original IVIG. That you couldn’t interchange back and forth. So if after her last round of IVIG she goes back to plasma exchange then it would wash away the last IVIG treatments she had.

I hope your daughter stabilizes and improves very soon.

Sue

Some brands of IVIg come in 6 g increments. That might explain why Hailey got 12 g.

MarkEns

Hi Holly,
I just read my good friend Kelly’s post. I have to start this out by saying I am just a mom with limited hands on experience and no formal training, so, as I have said before, you have to take what I or anyone else on the site says with a grain of salt and incorporate it into what the docs say. That being said. Mathematically, I figured out what Kelly proposes, it works out to 7grams a month as a loading dose. As I mentioned the one abstract I read said children can go as high as four, but I have never heard of 7g. The kidney’s and heart are affected by ivig, I cannot imagine a child being infused 45g a day over 5 days. That may have been what worked for Emily, but I would seriously consider it before I did it for Kevie or anyone else. I agree FULLY that 12g daily over 5 days once a month is not enough. I would not want to do cell cept or steroids (as I too fought that battle) So I would ask the doc to increase the frequency of ivig. I am not sure if I would ask for it every 2 weeks to start or every one week. I would have to see all of the facts in front of me, such as, how soon after ivig do you see improvement, how soon after do you notice downward change. I guess you would have to start with basic observations, record on a scale 1-5 her ability to walk on heels, the grip test, walk on toes, the ability to resist when her elbows are pushed down on while in a wing flapping position, resistance to the feet being pulled toward you, and the ability to get up from the floor while sitting pretzel style. 1 being the worst,5 the best. I might do this 1 day after completion of the 5 day load, then maybe 5 days later, if things are better, go to 10 daysthen 14. Since you do not want to see signs of a relapse, because by then it has already started, maybe for the first testing month you could push it to 18, then 21 days, but by 21, I think being that you know she cannot make it a month, I would start the ivig. Probably, I would stop observing at day 10-12 and just plan on another loading dose on day 14. Maybe you will find you need it by day 7, but 14 might be a good place to start. This is trial and error and takes patience in figuring out a plan. Being her mom, you can be the accurate record keeper and the eyes for the doc. If you keep careful charts and documentation, the doc would be sure to listen.

If you do have infusions every 2 weeks , 12 g each day over five days, that works out to the 4g/kg as a load for the months, that is the amount on the abstract I read. I will locate that today and get it to you, fax at the hosp w/ the number you provided or mail it if it is not legible. If you were to do it every week, it would be 7.5 grams. You have alot to think about.

FYI, if you do the actual math, her weight, 70lbs/2.2 it equals 31.8, so 32 grams. When X by the 2 g, it works out to 64. When divided over 5 days it is 12.8. Usually they round up, lets say 13. Mar Ens pointed out some brands are in 6g bottles (thanks mark, that is good to know) Others are in 5,10, 20, 25, maybe they could give her a 10 and a 5 of gammaguard to be closer to the actual amount, being that she needs the ivig. Usually they round up. Just a thought. I hope Kelly will not be mad at me, but Ultimateley you are her mom and you and her doc have to make the decisions. I am just the peanut gallery. Good luck.
Dawn Kevies mom

I am going to pm a member who works with children and has experience with ivig, maybe she can give us some input from a professional stance.
Dawn

Hi Holly,
Kelly and I are going to devote our morning to you. We are both looking through ALL of our paper work for treatments we have received as well as saved abstracts. We ARE finding some contridicting info among the general consensus. I will fax the info to the hosp in the next hour, maybe between that and your docs you guys can figure out a middleground.
Dawn

Holly,
Did you recieve the fax I sent to the hospital # you gave me? There were 36 pages plus 3 cover sheets. Also, tyr to post any other threads you start on the main page, not all people check the cidp/child section. I very seldom do, but I could not find your thread so I checked here. hope things went well at the hospital today.
Dawn Kevies mom

Hi, I wanted to add a few thoughts to the excellent advice you have gotten. As you realize, there are many nurses and health care workers on this site since these diseases can affect anyone. I have the fortune to help children with a suppressed immune system in a clinic that gives a lot of IV IgG for immunodeficiency diseases as well as autoimmune diseases, so I have seen it given quite a lot in my many years working there and have seen a lot of discussion about its use and how it works. I think the most helpful thing to say is that there are two goals in autoimmune disease–to flood the system with immunoglobulin so that binding of the bad antibody is prevented and to suppress production of the bad antibody. Each person has a different amount of bad antibody (“titer”) and each antibody has a different stickiness (“affinity”), so the amount of competing (good) antibody needed is different in each person, but one has to get above this amount at the peak in order for the immunoglobulin to stop the damage to the myelin. Each person also has a different drive to produce “bad” antibody (which also can change over time as the body tries erroneously to protect itself–not realizing that it is attacking its own nervous system). At the low point (trough), one wants to have enough immunoglobulin to still “convince” the immune system that it does not need more. These two processes are different and it seems to me that this is why different people need different amounts of immunoglobulin AND different timiing to give it. Again, you need enough to flood the system so that peak allows for release/competition of good antibody for bad antibody and so that the trough amount still is enough to stop the production of more bad antibody. Immunoglobulin given to a person has risks and changes the body. It is a huge protein load and will affect total body metabolism and the viscosity (“thickness”) of the blood. Adults could not take as much immunoglobulin as Emily–there would be a risk of stroke or kidney/metabolism concerns. This is a risk in children as well and most people (any age) could not tolerate that much without serious side effects. Emily is lucky that she tolerates it well and that it works for her. I think most doctors might think of alternative treatments rather than that intensity.

As Sue pointed out, plasmapheresis removes bad antibody. It sounds like Hailey Marie’s body is determined to keep trying to remake the “bad” stuff in the confusion that happens with autoimmune diseases. IV IgG or steroids or immunosuppression helps to prevent the remaking of the bad stuff as well as repleting the body with antibodies to keep it safe. I hope that the two parts of this are clear–that you have to prevent the antibody from further damaging the body by diluting it out enough (IV IgG) or removing it (plasmapheresis) AND you also have to find a way for it not to build back up.

A third important concept I have learned about autoimmune diseases is that you want no “down time”–you want to treat aggressively enough so that you do not get symptom return because symptoms are only the tip of the iceberg and when symptoms happen, there is a lot of damage going on and you are already falling behind. Damage will accumulate and the longer it goes on the harder it is to stop and reset the system.

The fourth major point is that the immune system will reset itself, but it takes a lot of patience to do so, so you have to find what works and then keep it working for a few months and then VERY SLOWLY back down. We often tell parents that children have a very enthusiastic immune system–that tries hard to do its job. Fortunately, not many children get autoimmune diseases, but when they happen, it seems like resetting the system back to normal takes an aggressive approach, but it can probably happen better than adults–who have a stubborn immune system!!!!

All of this being said, it seems that for most people, how Kevie has been treated is the more typical way with 2 g/kg “loading” doses of immunoglobulin and the frequency of administration to prevent
“down time” or symptoms of the illness. As Dawn states, maybe start with every other week. Hailey Marie obviously needs a higher trough than she was getting. Doctors often think of peaks and troughs with certain kinds of antibiotics. If the trough is too low, you can adjust this with a higher peak dose or with a shorter interval. It is probably a bit safer to adjust interval (like done with Kevie) than peak dose, but some kids will have to have really aggressive treatment like Emily.

As to alternative treatments, if immunoglobulin is not “enough” to prevent “down time”, advocate toward pulse steroids–steroids given once a week, or once every two weeks, or other intermittent interval. this is done in a number of other autoimmune diseases, is advocated in adults for CIDP, and has a LOT less side effects than daily high doses steroids. A special problem in young children with daily moderate to high dose steroids is that they will not grow in height in addition to all the other risks of steroids. Cytoxan (cyclophosfamide) is a profound temporary immunosuppression agent and it is often used in autoimmune diseases. It is powerful and quick, but it seems that a lot of people expect it by itself to be enough, and the experience at my hospital is that it is not enough–children’s immune systems are too enthusiastic. So if cytoxan is used to stop the damage, something else to allow for resetting of the immune system should also be used. As others have pointed out, plasmapheresis can be done, but it is harder in little children like Hailey Marie and you have to have good IV access and often go far away to a specialized Children’s Hospital. Imuran and CellCept suppress production of all antibodies resulting in susceptibility to infection (immunosuppression). They are slow to act particularly in reversing the damage–so often people use a “faster acting” method in combination.

It is late and I am rambling some, but I want to go back to the four biggest points. 1. You have to stop damage with something strong enough to do that. 2. You have to prevent the formation of additional bad antibody. 3. the goal should be not “down time” so that additional damage does not occur. 4. People need to make changes very slow so that the immune system can reset itself and get back to normal.

WithHope for a cure of these diseases and all children

Well,
I think you did a great job in showing your courage to the docs. You have set the bar in letting them know you are not a push over. Good Job!!
About the treatment. They are being fairly aggressive, however, I personally in my mom wisdom think it would have been prudent to start the ivig now, seeing as that the blood has been cleansed from as many autoantibodies as possibel. It would be the best scenario for a fresh start to flood the system with the good ivig antibodies. There would have been minimal time for production of the auto antibodies. I do think their plan to start in a week is ok, but not the best, plausible. I am concearned that follow up with maintanence doses may be an issue. We already know that she uses up a full loading dose 2g/kg before 3 weeks, so a 1g/kg probably won’t do the job. Some of the info I faxed you supports this plan, believing that the frequency is more important than the amount of ivig. As well, some of the other abstracts support the flooding, especially in children.

The way I see it, they have met you a little more than half way and Hailley got pp, so she may be at an advantage. I guess you will have to be vigilant in charting backward slides and do your own neuro assesments to chart the progress. If you go inwith a tangible evidence based chart should there be a decline, they will listen to you I am sure. They seem to be responsive. I also feel strongly about something else, you HAVE to have a nurse present during the infusion when you continue the treatments at home. You have been lucky. The fact that your co. did not even send the anaphalaxis (spelling) kit is alarming to me, not to mention your state of mind when seeing your child in distress would probably not allow you to set up the drip and push the benadryl. I know I could not do it!! A parent should always be removed from a life or death situation if possible, emotions do not allow one to think clearly. Kelly mentioned you have bcbs for ins. we do too. They have signed a contract exclusiveley with Coram/Apria because they are the biggest suppliers of infussions, this is who we used, they stay the whole time and insurance is paed no problem. Kevies nurse is awesome (a guy), they play guitar hero on xbox and shoot airsoft guns out side, watch movies, they are like pals, he is like a family member now! There is also Angels At Home, they specialize in pediatrics. So try to pursue that!!!! I can get you a number for Coram if you like, or you could ask the social worker at the hosp. to set you up with an agency that will come to your house. Also, make sure to stay with the same ivig product, it offers the best results if you are consistant.
Dawn

Dawn, Kelly, WithHope, If acknowledgements were given for the most helpful persons, you three would be given top honors.

Dawn and Kelly, you both have suffered with your children in their sufferings. You have done the research, kept journals and charts and been aggressive with drs.

WithHope, you work taking care of serious ill children and have seen some of the most heart breaking illness they and their parents have had to go through.
It was a privilege to have met you recently in Dayton, and hope to again in the future.

I just want to say I admire all of you women and thank you for helping the children and giving support to desperate parents searching for help and answers.

Thank You
Shirley

Holly,

I am so sorry Hailey has relasped so much and has to go through this horrible illness. You have great support and advice from Dawn, Kelly and WithHope and I will be praying that the Drs.,give her a treatment plan to keep Hailey from relasping.

Prayers for Hailey and her family.
Shirley

Hello Holly,
I haven’t posted in a while. My 11 year old daughter Christina has had CIDP since she was 3 years old. She has had every treatment available individually and in various combinations. She was in and out of the hospital often and always needed a combination of Chemo drugs like Cellcept as well as 2g/kilo of IVIG every 3 to 4 weeks. When she was 7 she had a modified bone marrow transplant (really no name for the treatment as it was experimental) Since then she has beed able to stay strong with IVIG treatments at home every 4 to 5 weeks. She no longer needs other drugs in combination with IVIG. She was in a wheelchair for 3 years when this started but she is now walking and strong and able to have a pretty good life. The key was not allowing her to get weak in between treatments. Whatever that took we did it. If you are able to stop the weakness in between treatments the nerves repair themselves somewhat and over time strength can return.
Being able to have the treatments at home with a visiting nurse has helped very much. Christina hated the hospital and was always upset. She still is very fearful of the treatment and fights against the needle access and deaccess but she is able to have a pretty normal life. We haven’t given up hope for a cure but we can accept how things are now. Christina’s doctor felt that the most important thing was to do whatever it took to not see signs of relapsing and weakness inbetween treatments. We wish you the best and know that is so very difficult to watch your child suffer.
All the Best
Steven