7 year old female with CIDP
AnonymousJuly 24, 2008 at 4:31 pm
It’s me again….I just wanted to update that Hailey is on day 3 of her IVIG and the port has been a real pain in the butt so far but I’m sure in the long run it will be very nice to have. The first time they tried to access it, it was a problem. They poked her a total of 8 times til they finally got it accessed and then the next day it wouldn’t flush and she had to be re-accessed so she’s pretty tired of the poking at this point. Anyway, I wanted to post that she is getting Octigam IVIG. Let me know what you think of this brand. Also I did get an Epi-pen and did have the nurse stay the first time but now I am administering her during the day. I’m also doing what you suggested with the benadryl and also tylenol. So far so good though she’s been feeling really good and strong (for the most part) and I have been keeping a closer record this time. Thanks again for all your help. I took your advice Dawn and posted on the main forum instead.
July 24, 2008 at 5:04 pm
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.
Dawn Kevies mom
AnonymousJuly 24, 2008 at 5:59 pm
I’m glad to hear that Hailey is doing well. Hopefully this new treatment schedule will work out well for her.
Emily had some access issues with her port at first also. I actually think it’s pretty common, especially in kids. You should ask the nurse to use a longer huber needle. Emily uses a 1 inch & she was supposed to only need a 3/4 inch.
Octagam is an IVIG product that has to be reconstituted. Since you are doing IVIG at home without a nurse do you have to reconstitute it yourself?
There have been more reports of adverse reactions to IVIG products that have to be reconstituted as opposed to those that already come in liquid form.
It still concerns me that you are doing the IVIG at home without a nurse. I think it is extremely dangerous, even if you have an Epi-pen in the house. You also need to have an IV start kit with a vial of Benadryl in case of emergency.
Make sure that you are taking off the sticker, on the IVIG, that has the batch number on it, in case of an adverse reaction.
Here is a link to info on Octagam:
Keep in touch & let us know how Hailey is doing.
AnonymousJuly 24, 2008 at 6:56 pm
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.
You must be logged in to reply to this topic.
7 year old female with CIDP
AnonymousJuly 12, 2008 at 7:44 pm
My 7 year old daughter is currently in the hospital with her 4th relapse of CIDP in the last 4 months. The first two times she received IVIG for 5 days. The 3rd time she received IVIG 3 days and high dose of steroids and didn’t respond to IVIG so we were sent to Denver from Montana where she received 5 days of Plasma Exchange. Now this time we’re currently back in the hospital in Denver getting Plasma Exchange again and talk of long term high doses of Prednisone. If anyone can help me with experience with different treatments and what has worked and if anyone has been able to get the condition in a long term remission. What treatment got you into remission? Some doctors talked about Cytoxan (chemo therapy) as an option to hopefully trigger a remission. The doctors that we’ve been seeing just have very limited experience with CIDP in children and I heard about a great doctor in Dallas, TX and think that may be my next stop. If anyone can help I would greatly appreciate it. I’m trying to make a decision on what to push for as far as treatment goes by Monday.
Holly from Montana
July 12, 2008 at 9:19 pm
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
AnonymousJuly 12, 2008 at 10:05 pm
Thank you so much for replying so quickly I really appreciate that. She received the first IVIG (12 grams per day for 5 days) and recovered quickly, 4 weeks later the symptoms returned and she received another 5 day course of IVIG, that time she didn’t recover as quickly. 4 weeks later the symptoms returned again and she received 3 doses of IVIG and her weakness continued to progress and at that point she started plasma exchange. Is cell cept and plasma exchange the same? So correct me if I’m wrong but for your son you use IVIG on a monthly basis before any weakness reoccurs and this has kept him healthy for the most part. So does he still have any signs of the CIDP? Also I would be very interested in having you send me the abstracts info. If you could email me at [email]email@example.com[/email] then I would be glad to give you my address. Thank you so much! It’s so nice to just not feel so alone in making these huge decisions about my daughter.
July 13, 2008 at 12:17 am
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
July 13, 2008 at 12:29 am
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.
AnonymousJuly 13, 2008 at 1:15 pm
Thanks Dawn!! When you say a loading dose, do you mean a one day treatment or 5 days of treatment? Oh and yes it must come in 1 gm because for sure Hailey received 12 gms each dose. I looked it up on her medical records to confirm that. As for her symptoms, they have been weakness starting in the feet and working up to paralyzed hands, not being able to walk & etc. but not ever going into her respiratory muscles (thank god). Also she had a nerve conduction during the last relapse and both the myelin and axon have been effected. About the doctor issue, here at the Denver Children’s Hospital, they have been receptive to all of my suggestions. Last time we were here, a specialist from Germany, Dr.Toyka was here and evaluated her and said getting aggressive with treatment now would be our best chance at getting long term remission but it’s just so confusing to me about treatment and what is best. Thats why its so nice that you guys are here to help by sharing your experience. oh yah and she does have a port already. It was suggested last time by the picc nurse to get that put in if she had another relapse so I pushed for that this time and got it. thanks again for all your help, Holly
AnonymousJuly 13, 2008 at 4:16 pm
[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.
AnonymousJuly 14, 2008 at 3:32 am
My daughter Emily was dx’d with CIDP at 4 years old. She’s 6 & has been relapse free for 17 months now! (KNOCK ON WOOD!) Currently she gets 20 grams of IVIG every 3 weeks & is 53 lbs.
Just to let you know I have been through the wringer with Emily & her treatments, so I know exactly what you are feeling right now. Emily is a really unusual case & has received more IVIG than anyone I have ever heard of. She was getting 200 grams a month at one point. That was the start of better things to come though – it really helped her out a lot.
I hope knowing a bit about what we have gone through helps you to not feel so alone. When Emily got sick there weren’t many parents on this board at all. I felt really alone at times – although the adults with CIDP have been super supportive as well. It just helps to talk with other moms, I think.
After reading your posts & speaking with Dawn (she called to tell me to respond to you), I think your daughter needs more IVIG & more often. At this point I would recommend following what we did with Emily. Her loading doses are 20 grams every day for 5 days. This floods the system with anti-bodies much faster. It sounds like Hailey really needs to be hit hard with IVIG to stop the attack on her myelin.
Just for the record, Dawn & I disagree a bit with this protocol. We still agree that a loading dose is 2 grams per kg though. I just think it needs to be per day & not broken up over 4-5 days.
Emily has seen 3 dr’s who all follow this protocol for a loading dose (her neuro, an MDA dr neuro & a pediatric physiotherapist. And the director of the hospital she was at agreed to it as well). And just to be sure I wasn’t crazy I called Emily’s infusion nurse tonight & checked with her on this. She said that most of her patients get the same protocol that Emily does. And then just to verify that info I called my dad (who is the administrator of a nursing home) and had him call around. He got in touch with a dr & his director of nursing & they both agreed with it as well. So I’m not crazy & this protocol is followed on a regular basis here. It’s not often that Dawn & I disagree on anything to do with CIDP or IVIG. I think it’s just different for each patient & each dr.
Following this protocol would mean that Hailey would get the 2g per kg every single day over 4-5 days. I think Dawn said she gets 45 grams over 4 days every month. Doing this would mean that she would get 45 grams every day for 4-5 days for a loading dose. Then you would need to figure out her relapsing pattern & adjust the dosing amount & time frame to that.
Blood tests should be done a regular basis to check her iron, kidneys, etc. Make sure that she gets pre-medicated with Benadryl & either Tylenol or Motrin. And she needs to be really well hydrated, so she’ll need to drink LOTS of water & Gatorade. Popsicles count as a liquid too 😉
I hope all of that made sense. I know how overwhelming this can get.
You said that the dr from Germany suggested that they get aggressive in treating her CIDP & I agree with him. I think aggressive IVIG dosing is the way to start. If that doesn’t work then you start looking into steroids & things like Cellcept. I’m actually quite surprised they are doing PP on a 7 year old.
I’ve read a few studies that say if you can get the attack under control within 2-3 years after onset then you have a better chance of her going into remission. I also read another study which followed 15 (or 16) children with CIDP. 10 years later all but 2 of them were symptom & disease free. The other 2 were disease free but still had a little bit of disability – not much though, it was classified as “slight”. None have received any treatments in the last 10 years. They all went into a remission.
Do you know how long after Hailey’s last treatment she started having symptoms again? If not, you need to keep a detailed record of this. Most of us CIDP mom’s keep a big binder with all of the test results, nurses notes, presciptions, etc in it. It’s also a good idea to keep a calender or journal to record how she is doing day by day. Not only is it good to show the dr’s but it also will help you to assess her on a long term basis.
In the beginning I would do a standard neuro evaluation on Emily every day. That would include walking on her tip toes & heals across the room, jumping up & down 10 times on 1 foot, sitting on the floor with her legs crossed & getting back up as quickly as possible, having her squeeze my pointer finger with each hand and having her follow my finger with her eyes. Now I have her do push ups for me, LOL.
Did Hailey get any immunizations recently or was she sick at all before being diagnosed with CIDP? I’m just wondering.
Let me know if you want to talk on the phone or if there’s anything I can do for you. Remember that we’re here if you need us. We’ve all been where you are now so we’re very understanding of what you are going through. If you need to vent or cry or share good news, we’ll be here.
PS, Google, Google & more Google. You’ll be able to find studies to read & you’ll learn SO much that way. That’s how I learned everything & now I know more about this disease than Emily’s dr does, LOL.
July 14, 2008 at 10:02 am
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
July 14, 2008 at 10:04 am
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.
July 14, 2008 at 11:27 am
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.
AnonymousJuly 14, 2008 at 12:11 pm
Warning…this is LONG.
Like Dawn said – I’m just a mom & this is what worked for my child. I re-read what I wrote (at 3:30am!) and I talked with Dawn this morning & I want to make sure that you look into what I said before doing it. I’d hate to give you bad info or tell you to do something that wasn’t safe. Also note that Emily has a very rare case of CIDP, she has right eye involvement. Something like only 3 % of CIDP’ers have this.
Where I live & the people who treat my daughter do the loading doses like I told you last night (this morning). I’m trying to look for info online about it but I’m not having much luck. Most sites do say do the .4 g a day for 5 days. Obviously that isn’t working for your daughter though. Maybe do 45 grams once a week or once every 2 weeks. I dunno…
The whole point of a loading dose is to flood the system with good anti-bodies then you can find a maintenance schedule that works for you. I guess that’s why we do such a heavy duty loading dose.
All I can say is that dosing Emily the way that we did with IVIG was the only thing that helped her come around. After that 2 month flood of good anti-bodies she went from relapsing constantly to being able to go 12 days without a treatment. It was risky but I would do it again in a heartbeat if I had to, especially seeing where she is now.
I’m going to attempt to get a hold of Emily’s nurse again today to discuss the loading doses – just to make sure that I’m not giving out bad info. I do know that another one of her patients, a child with another auto-immune disease, gets 45 grams a day for 5 days as a loading dose. She’s 10 years old & about 70 lbs.
Just a bit of a background on IVIG…I pulled out my big binder & I’m going to go through what Emily has received.
Emily was originally dx’d with GBS on Dec 28th 2005. She was given 2 consecutive days of 20 grams of IVIG then released. By the end of the 2nd treatment she was back to walking, running, lifting her arms, she could jump & climb once again. Her eye wasn’t back to normal but it was stuck in the middle.
When we saw her neuro for follow up he decided to give her 20 grams of IVIG 1 time a week for 3 weeks because of her eye. We also saw a pediatric eye dr who told us that her eye would get better with time & to put a patch on the good eye, to make the bad eye work harder.
Emily relapsed in March 2006. Her eye had gotten worse & was stuck in the corner of her eye & she started tripping. She was admitted to the hospital, had another spinal tap & was dx’d with CIDP.
This is what her IVIG schedule was like at that point:
In the hospital:
20 grams of IVIG every day for 5 consecutive days.
Through home care: (note all IVIG infusions after this point are done at home)
20 grams of IVIG 3 times a week for 2 weeks
20 grams of IVIG 2 times a week for 2 weeks
20 grams of IVIG 1 time a week for 2 weeks
After her 2nd dose of IVIG during that 3rd week her eye had returned to normal. Then she relapsed after the 1st week of 1 time a week.
We then did 20 grams of IVIG 3 times a week for 1 week & then went back to 20 grams of IVIG 2 times. She got 20 grams of IVIG 2 times a week for 8 weeks then relapsed again. Then I came up with this schedule:
20 grams of IVIG 2 times a week for 4 weeks
20 grams of IVIG 3 times a week for 2 weeks
20 grams of IVIG 2 times a week for 4 weeks
20 grams of IVIG 3 times a week for 2 weeks
We started this in August 2006 but after the 2nd week she had a central line infection (she had a Broviac catheter). She was in the hospital & taken off of all IVIG & given anti-biotics. Her central line was removed & she went 5 days before she relapsed again. On August 31st she had a port put in & started with another loading dose of 20 grams a day for 5 days.
Then we started the above schedule on Sept 11th 2006 & we did this through November. In December 2006 we switched neuro’s & she was taken off of all IVIG, to see what would happen. Emily went 12 days before we noticed the start of a relapse. She didn’t get really bad for 15 days after that. She received 20 grams of IVIG 1 time, on December 23rd & it had no effect what so ever. At this point the dr talked me in to doing oral Prednisone. Emily was put on 5 mg’s a day & there was no improvement.
In early Jan 2007 she had another loading dose of 20 grams per day every other day for 5 doses. This is the protocol that they use at Children’s Hospital in Detroit, MI. I was told that in females they want to do it every other day & in males they can do it every day…not sure why though.
We noticed a dramatic improvement after the 3rd dose – by the 5th she was back to normal. The new dr then decided that she would only get 20 grams of IVIG every 4 weeks, Emily lasted 12 days then relapsed again. Once again she got a loading dose of 20 grams of IVIG every other day for 5 doses & her steroids were increased to 15 mg’s a day.
After 12 days, once again Emily relapsed. She had yet another loading dose of 20 grams of IVIG every other day for 5 doses & the steroids were increased to alternating 17.5 mg’s a day & 15 mg’s a day. After only 9 days on this new protocol she relapsed. I had enough at this point & the neuro was insisting we keep increasing her steroids. I thought this was a bad since being on the highest dose she relapsed 3 days sooner than she had previously.
I switched back to our old neuro & started weaning Emily off of the steroids by 5 mgs a week. Emily got 5 consecutive days of 20 grams of IVIG & then was put on 20 grams of IVIG 1 time a week. She was on this protocol until the summer of 2007.
Then we did 20 grams of IVIG once a week alternating 20 grams of IVIG once every 10 days. By the time school started in September 2007 she was on 20 grams of IVIG every 2 weeks. We did this until just a few months ago, when we alternated 20 grams IVIG every 2 weeks with 20 grams IVIG every 3 weeks. Now she gets 20 grams of IVIG every 3 weeks. She’s scheduled to receive this dose for 4 months. If all goes well, we will do 20 grams IVIG alternating 3 weeks & 4 weeks then move her to every 4 weeks before the winter.
We saw Emily’s neurologist a few weeks ago & he believes that she is healed. He mentioned remission but I’m just not ready to get my hopes up. Emily’s nurse agrees that she’s in remission but we’re going to keep her on IVIG for some time still. Since the full life of IVIG is 42 days, I want to make sure she can at least get to that before we discuss taking her off completely. As you can see, we definetly were on a roller coaster ride for awhile & I just don’t want to go through that again…for myself or for Emily.
I wrote this out so you can see what we did & what worked for us. As I said, Emily is has a rare case. I’m sure your daughter won’t need as much IVIG as Emi did…but she definetly needs more than what she’s getting now.
July 14, 2008 at 11:03 pm
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
AnonymousJuly 14, 2008 at 11:31 pm
Yes Dawn I got the fax!! Thank you Thank you Thank you! You’re the best! Sorry I didn’t call you back I intended too. But you know how things are in the hospital. I did talk to the doctors about the IVIG more often and at a higher dose and they wanted 24 hours to do some research and get back to me in the morning. I read all of the pages you sent and it was very helpful info. Also i wanted to thank Kelly for all the info about her experiences with her daughter. I will call you soon Kelly maybe tomorrow. I’m hoping that we can leave for home tomorrow. It’s a 8 hour drive. Take care! oh yah thanks for the hint about the thread I’ll remember that for next time.
AnonymousJuly 15, 2008 at 3:35 am
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
AnonymousJuly 15, 2008 at 10:26 am
Another thing I wanted to add that I forgot to say on the phone is to make sure you give her a multi-vitamin WITH iron in it. IVIG can lower the iron slightly & can case anemia. I give Emily a Children’s One A Day with Iron in it.
Also, make sure that the dr’s order blood tests to check for blood viscosity and a CBC, if she’s going to receive the frequency that we talked about on the phone. This is VERY important! The blood should be taken before the start of the IVIG infusion on the 1st day by the nurse.
We also tested Emi’s IgG levels once a month when she was on her highest dosage. This was important as we could see what level she needed to be at to make a difference.
I also forgot to mention to try and avoid salty food or foods with a high sodium content while Hailey is still on the steroids. They contribute to the puffiness.
I’ve been thinking about Hailey’s IVIG schedule & I do hope that the dr’s will consider doing what we talked about on the phone. I think every 2 weeks, in the beginning, is a good place to start – as long as her blood tests indicate that everything is ok. Then I think doing a maintenance dose every 3 weeks seems reasonable – since you said you notice the relapse at 30 days. If you do IVIG more frequently like we talked about it is EXTREMELY important to have a nurse there for the whole infusion every time. The more IVIG that is put into her the more chance of a reaction happening.
With Emily we usually go 3-4 months on a new protocol before lengthening the time again. You could try to push to be done with the every other week by the time school starts & then you could do the maintenance dose. That way it’s not as much of an interruption to Hailey’s life.
Feel free to call & talk whenever you need to.
July 16, 2008 at 12:04 am
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.
AnonymousJuly 16, 2008 at 9:12 am
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.
AnonymousJuly 16, 2008 at 9:41 am
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.
AnonymousJuly 25, 2008 at 1:05 am
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
You must be logged in to reply to this topic.