Plasma Exchange in children- Need Advice!!
AnonymousJune 29, 2009 at 4:10 am
Hello there, I’m Jen and I’m writing on behave of my daughter Abbi who will be 8 this July. She was diagnosised back last Sept with CIDP. We started steriods and IVIG, after the first dose of IVIG she did great! She did not tolerate the high doses of predinsone. In December we had a PICC line placed due to poor venous access and started doing IVIG every two weeks and High dose IV steriods at the same time, all seem to be doing ok until she delveloped a Pseudomonisis bactermia in Feb, she was very sick! They never found the actual source of this infection stating she was immunocomprimised from the meds. It wasn’t her urine, or lungs. and the PICC was clean but had to be pulled. Weeks later a second one was placed that one lasted 10 days and had to be pulled due to puss coming from insertion site. Needless to say Feb was a tough month. We decided not to put another PICC in and went back to monthly IVIG and to weekly doses of predinsone. Abbi seemed to be doing ok. Well June 9th we returned to CHOP (children’s in Phila) to have another EMG, it shows no improvement. Clinically she doesn’t seem any worse but she’s never had the great results from IVIG expect for that 1st dose. So now I’m being told that we need to try Plasmaphoresis, this requires a perm cath another central line. Abbi’s tract record with foregin bodies in her is not so great, and on top of all this they’ll like to start her on Imuran. I’ve hit my level of frustration! I don’t want to do anymore steriods because of all the wicked side affects she suffers. I don’t want another central line. and lastly Imuran scares me. I’m a critical care RN and I see this drug with transplant patients and chemo patients. They don’t seem to have alot of data on use in kids, they take an adult dose and tweak it based on clinical observations and it requires frequent lab draws and has its own side affects. I had my neurologist speak to another doctor up in Boston as kinda a second opinion and that doctor seemed to think this was the best course of action. Is there any other parents out there that have some some experience with the PE?? They can’t tell me if it will be a one time thing or monthy or every 2-3 months so I don’t know what to do about the central line thing. Now that its summer placing another line will prevent Abbi from swimming one of the few activities she can do without issues. She was soo “immunosupressed from steriods” what’s Imuran going to do to my little girl. I understand that she’s not going to dye from CIDP, but jeez the treatments could kill her, I know I’m getting dramatic but I just need some other perspectives. I appreciate all that take the time to read this. God bless.
June 29, 2009 at 8:48 pm
My son is 12 now, 10 when he got cidp. Anyway, you mention that your daughter has difficulty with foreign bodies. While some people really cannot tolerate foreign bodies, it could be as simple as the broviac just being too much to keep clean with an active 8 y/o. Also, some DO NOT do well with steroids, in fact some with cidp depending on the variant do worse with steroids. Was she getting a loading dose? (2g/kg) Children CAN tolerate higher doses, as high as 4g/kg. PP might be difficult to continue for a child over a long period of time. One possibility that I have always kept in the back of my mind if we ever needed a master plan would be to do 1 round of pp to cleanse the body of as many autoantibodies as possible and the immeadiately follow with ivig so the antibodies have the best chance to flood the system. (just mom thinking) If you were to do pp, any meds would be washed out(immuron) Ivig as well, but it would be working differently than the immuron and put back in. Have you tried a loading dose bi-weekly? then you would be getting 4g/kg monthly?
Regarding your comment about her ncv/emg not being any worse. That IS good news. You have maintained the process, you just need to slow it more. It does take a while to show improvement. Kevin was dx in Oct. 06 and this past month was the first time he had a normal ncv/emg. The others were never worse, but pretty flatline regarding improvement. It does take time. She also may have lost ground during the infection time and it may have taken some time to build up the ivig.
Check you r private messages, I sent you one.
Dawn Kevies mom
AnonymousJuly 1, 2009 at 6:36 pm
Hi there. My daughter is now 7, almost 8, and has had CIDP since she was 4.
Emily had a Broviac catheter when she was 4 that got infected after a nurse cared for her that carried some germ. She too has poor venous access. So I totally feel your pain.
PICC lines are notorious for becoming infected. They don’t really like to do them on kids. I was told most PICC lines don’t last more than 10-14 days. I’m assuming your PICC line was not a Broviac or Hickman, but a venous access in the arm, correct?
Another option might be a port. I can understand your fear of that since everything you’ve been through. You might want to talk with an infectious disease dr & a pediatric surgeon about the risks with one but I think it would be worth the consideration. The port was one of the best decisions we ever made for our daughter. And they are more difficult to get infected since they are under the skin.
I don’t know too much about plasmapheresis. I do know that they don’t want to do it to children under 5 years of age, it’s a tricky procedure & it should be done only under constant supervision. It should be done only by experienced dr’s too.
Another mother posted many months ago about her 8 or 9 year old daughter getting plasmapheresis. She’s in Montana, I believe, and doesn’t post on here anymore. I’m sure you could search around on the site for her old posts to see if she gives out any info on the procedure.
Why did you go from bi-weekly IVIG infusions to monthly & increase the frequency of steroids? It would seem to me, at least, that you would wean the steroids before weaning the IVIG as steroids have more serious side effects & are more likely to cause a weakened immune system. Also IVIG stays in the system longer than steroids do, so that could eventually mean less IV accesses – if you are worried about her veins.
I think if it were my child I would explore other options before doing Imuran.
Emily isn’t your typical CIDP case & required massive amounts of IVIG to improve. Steroids made her worse & methotrexate was mentioned for her. I refused & we kept on with the IVIG. She’s improved & is currently getting 20 grams of IVIG every 2-3 weeks.
It took us over a year to get Emily stable & to see any improvement in her EMG’s. It’s all about finding the right treatment, at the right dose & at the right frequency. We’ve said a millions times on this site that treating CIDP is an art form & I really truly do believe that. Each CIDP’er is so different.
Almost 4 years since Emily was diagnosed she looks like a perfectly normal kid. No one can tell that she has CIDP. The only physical difference is she is not that fast of a runner – she does everything else that a “healthy” kid can do. So please don’t get discouraged, it just takes some time & remember that 70% if children with CIDP will go into remission usually within 5 years of starting treatment. (don’t stress out on that timeline though…I certainly am not)
How many grams of IVIG & steroids does your daughter receive? Do you have home care or do you go to the hospital for her infusions? What brand of IVIG does she receive?
Hope that helps. And if you want to speak on the phone you can PM me & we’ll exchange phone numbers. It really helps to connect with other moms.
PS, you also might want to post on the main forum. I know there have quite a few adults who have gone through plasmapheresis & I’m sure they can offer you some insight.
AnonymousJuly 1, 2009 at 10:25 pm
I have a few small suggestions and comments. First, I want to say that CHOP is a world-class children’s hospital. It is a good place to go if you have a child with a rare illness. This does not mean that it is always the best place possible nor that you do not have to advocate, but there is a lot of potential and experience there including doing pheresis in children.
Second, that it might help just a tiny bit to clarify that wise Dawn means immunoglobulin when she speaks of loading doses. The dose and frequency of immunoglobulin matters. Some doctors are very by-the-book about giving immunoglobulin and give 2 g/kg (loading) dose the first time and then 1 g/kg after that. Each person has a different level that they need for response. If your child needs the equivalent of 1.5 g/kg to respond, she would get a lot better on 2 g/kg and maybe only stay stable or slowly worsen on 1 g/kg per month. The amount of immunoglobulin also may vary with disease activity, circumstance, other infections, etc. If she were given 1 g/kg every two weeks, this actually works like more than 2 g/kg a month. Since you are a critical care nurse, think of this like vancomycin. You want a trough level that is effective. Each person is somewhat different in dose needed to get that trough level and to maintain a trough you can give a larger dose less frequently or a smaller dose more frequently. There are a lot of doctors that do plasmapheresis followed by immunoglobulin as Dawn says–to reduce the antibody load and then fill the system up with non-autoimmune immunoglobulin for protection and to suppress production of bad immunoglobulin. This may be a way to get more impact and to try again with immunoglobulin as the major treatment.
Third, there are a lot of catheter options out there. You may want to discuss your concerns with catheters with a pediatric surgeon or pediatric oncologist or immunologist or infectious disease doctor. I work in a pediatric oncology and immunodeficiency clinic and we see lots of catheters. I would not be discouraged by two infections. There are double lumen ports through which people can do plasmapheresis. They are typically rather large for an 8 year old, but you could ask if this was an option. You could do a temporary pheresis line for the first round of pheresis to see if it works well before committing to a permanent line capable of doing pheresis since these lines are bigger in order to manage the rate of blood flow for plasmapheresis.
Fourth, I have seen imuran used very sucessfully in immune-mediated conditions and with considerably less side-effects than in a bone marrow or solid organ transplant situation (in which the dose is often pushed high to prevent rejection/GVHD). The biggest positive about imuran is that the goal of its use is to reset the immune system–to cure–whereas, in reality immunoglobulin (and predominantly steroids) is addressing the manifestations of the illness not its cause. It is just that people have to be really patient with imuran because it requires slow and steady persistence and may take 18 months or longer to show it is working. Lots of people get impatient and keep increasing imuran and this can lead to infections or other complications of immunosuppression. You have to have patience for imuran to work and often have to have an additional way to limit damage until the underlying disease starts to get better.
WithHope for a cure of these diseases
July 2, 2009 at 12:03 am
I re-read your post and noticed you were worried about Abbi not being able to swim w/a picc line. Kevie has a port and can do everything, we have a pool and he always swims. From an emotional standpoint, ir is not obvious to other children and he does not have to worry about fitting in. My only suggestion would be to do your homework as to size of the port being placed and where it will be placed. Another friend of mine just had her daughters port re-placed because the other one was too small, as well, now that she is a young lady she was concerned with its visibility while wearing bathing suits etc. Kevin has a problem with the port being too deep into the tissue. In the long run it is better because Kevin plays like a typical boy, football, trampoline etc. The negative side to this is his access does not always go well. Last month it took 7 tries, 4 by the nurse and three by the surgeon. We now hope to have a handle on how hard it must be held and isolated. Hopefully all will go well this month as we are moving to every 2 weeks instead of once a month. Be sure to discuss all options with the surgeon should this be an option for you. we were in a rushed situation and I did not know any better. Good luck! By the way, after re-reading your post, I also noticed you are a critical care nurse, now I feel really silly giving you advice that I am sure you already knew!!!! But I still could give you moral mom support from all that we have been through and learned!!!
Dawn Kevies mom
AnonymousSeptember 4, 2010 at 2:15 pm
Hopefully by now you’ve made a decision that was the correct one.
Sorry I’ve only been on the Forum for less than a year, it is time consuming, time I don’t have.
But while looking up perma caths I decided to check the Forum to find out how long others have had them. My daughter is an adult (at least chronologically) so different issues. She’s had a perma cath, double lumen, for 2 years. The hard part is finding someone who can ‘flush’ it every week, at least every week we do not go for PE. She gets PE once every 3 weeks.
Did you get the perma cath? Maybe you have the skill to flush it every week, that’s one thing I am staying away from. We were fortunate to find a home health care nurse who learned how to do it. The other issue I have is everything I do with my adult daughter is an emotional struggle, so that is very important and better left to someone else. We (I) are now having difficulty with insurance paying for it. It is a major struggle to get her to the hospital for everything and a relief not to have to do it with this. It is the ONLY caretaking that I have help with.
Would like to hear how the perma cath is going for you if you decided for it. Yes, the swimming is a no go and bathing is nearly impossible!
Hope you see this and can respond.
AnonymousSeptember 5, 2010 at 12:57 am
[COLOR=black]It is possible to at least shower with a perma-cath. I have had one for nearly five years and have figured out a way that works for me. My wife helps me with the preparation, but it is possible for me to do it by myself. [/COLOR]
[COLOR=black]I take a washcloth, about 9″ square, and put a 2″ wide strip of a waterproof tape on it. I tape the cloth to my chest, above the dressing, so that the catheter is roughly in the middle of the cloth. I then put on a cape like the one you would find at a hair stylist. Thus armed, I then shower my head, below my waist, and forearms, taking care not to let water flow underneath the upper portion of the cape. Once finished, I take off the cape and wash my back, lower torso, and upper arms, taking care to keep the shower from hitting the cloth. I then sponge-bathe my chest.[/COLOR]
[COLOR=black]Once done with the shower, I dry my head, arms, and upper torso. Only then do I remove the cloth. [Since the waterproof tape really isn’t, sometime the cloth is loose, sometimes not, so when to remove the cloth is not a hard-and-fast rule.] I then sponge-bathe the area under the cloth. To ensure that I did not get the dressing wet, I take a tissue and press it tight against the dressing. If the tissue is damp, but away from the catheter, I do what I can to dry the area, usually by pressing more tissue against it. If it were to come out wet, we would redo the dressing.[/COLOR]
[COLOR=black]This sounds like a lot of extra work, but if you are careful, it only adds about five minutes to the shower. The purpose of the cloth is to keep water that condenses underneath the cape from wetting the catheter area and to provide extra protection.[/COLOR]
[COLOR=black]I hope this helps and Godspeed.[/COLOR]
AnonymousSeptember 6, 2010 at 3:13 pm
Dear MarK, thank you. You are a dear for responding. But, the problem is that my daughter still cannot stand, let alone walk, after 2 years. Her fine motor skills do not seem to have returned to her hands, although she has strength so can pull up if given something to grab. For a while she could stand with the AFO’s and a walker, but fell out of the power wheelchair & broke her ankle in May, and is only now standing briefly with AFO’s and walker. Nearly fell again yesterday. It is impossible to get her in the shower in my house since the doors are too narrow for a wheelchair to that bathroom. The larger bathroom has a tub and hand held shower and we can make that work but getting her out is precarious, so I put the extended bath chair over the edge and we can kind of make that work. It is exhausting for me!
You did give me a couple good ideas though. We’ve been taping a baggie over the lumens and I just carefully work around the tegaderm that holds it in place. Everything around here is salted with emotion, too, and so much physical struggle for both of us.
I have been trying to figure out how much life the perma cath has. Hers is 2 years now and got gunked up pretty bad over the last few months. I got the County Hospital to recognize the problem (actually they no longer had access) and they put altapese (sp) in it for one hour which seems to have opened it up. I looked up ATP, it’s primary use seems to be for blood clots. There was a lot of consternation at the hospital about who would administer the med, at first I thought we’d have to wait for vascular access, but then someone decided that the doc, a resident, in the blood bank could do it. It evidently is fairly risky but the risk was outweighed by the need to have access. Have you had anything like that?
I search the Forum for info, not to complain, and exchanges like this are very helpful to me. Would like to hear about your experiences with the perma cath, there is so little info, and there’s nothing like personal experience.
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