Plasma Exchange treatment description

[Alex]

Hi all, my 10 year old son completed Plasma Exchange/ Plasmapheresis (PEX) one week ago. I wanted to write a description of the process for anyone who is considering it becasue we had a hard time finding out the details to our own satisfaction prior to treatment.

 

My son's course of PANDAS was very similar to many of the descriptions we have read on here. He developed rapid onset OCD about 5 weeks after a sore throat/fever. In the weeks prior to the OCD kicking in, he had lots of emotinal volatility, frequent urination, and separation anxiety/ night fears.

 

Over the course of the last few months he was treated with antibiotics and prednisone and improved under both. After the prednisone he regressed some and was still a long way from symptom free. We decided that we wanted to continue with a further course of treatment and decided to go with the PEX over IVIG. The main reason we went with the PEX was we believed, possibly mistakenly, that it was more likely a one time cure for PANDAS then the IVIG. Our reason for this was that in the one study done on these treatments, PEX was a somewhat more effective treatment than IVIG, although both proved largely effective. Also, our Neurologist, Dr. Latimer, told us that of the patients she has treated, she has had patients have IVIG treatment and then later PEX, but never the other way around. Lastly, the PEX that they do at Georgetown is not considered to expose your child to any donor blood product whearas the IVIG does. There is a chance, however small, that the IVIG could contain an as yet undiscovered pathogen. But again, it was a really hard decision and I'm sure our research/reasoning had some gaps. It basically came down to a coin toss.

 

We arrived at the hospital on Monday, August 17 for a consultation with the Oncologist/ Hematologist who oversees the actual PEX. I told her all the details of my sons PANDAS including the diagnosis of PANDAS from several different doctors. I don't think she would have been comfortable with proceeding if there were any doubt about it being PANDAS. We returned the next day to be admitted and start treatment. The treatment takes place entirely in the Pediatric Intensive Care Unit(PICU). Once arrived, they don't leave the ICU until discharged. The first step is to put in a standard IV which remains throughout the hospital stay. It is used for the administering of anaesthesia when the catheter for the PEX is inserted, for saline drip throughout the stay, and for any other medication that needs to be inserted intravenously. An ICU nurse attempted to put the IV in my son's arm unsuccessfully. She couldn't tap the vein for some reason. So they chose to wait on the IV and have the anaesthesia nurse put it in prior to the insertion of the catheter. When it was time to do the catheter, the anaesthesia nurse put the IV in on top of his hand with no problem. After the IV was in, my son was given Propofol intravenously to put him to sleep for the catheter insertion. I was asked to leave the room at this point. The catheter was put in by one of the PICU attending Physicians, a pediatric intensivist. He gave us the choice of using either the jugular vein in the neck, or the femoral vein in the groin area for the catheter. Apparently there are advantages to both. We chose the femoral because we were told it is slightly safer. Not sure why, farther from the heart I think. It took what seemed like a long time to get the catheter in. Afterward the Doc told me he had a hard time finding the vein again. Ugh.

 

It was mid morning by the time the catheter was in but he did not start the first exchange until late afternoon. My son and I passed the time playing video games, board games, watching movies and reading. They have a great set up there at the hospital.

 

For the first treatment, the nurse arrived with the PEX machine around 3p.m. The machine works by removing the plasma from the blood using a centrifuge. The blood cells are then combined with a synthetic plasma called Plasmanate and then returned to the body. Dr. Latimer prescribes three full rounds of PEX. Each exchange removes 75% of the plasma. So after three treatments, pardon my weak math, well over 90% of the original plasma has been replaced, effectively removing well over 90% of the harmful antibodies. The nurse, who is a Dialysis nurse, who filters peoples blood in various ways day in and day out, was great. It took her about 15 minutes to get the machine up and running and get the lines hooked up to the catheter lines. Along with the plasmanate the machine also adds calcium and saline to the blood mix returning to the body. The whole process took about three hours. There is a Doc from the pediatric oncology/hematology department on the floor during the treatment but it is the nurse who runs it. My son was awake for the procedure and we watched a movie and I read him a book. He had a few minor complications during the first treatment. His blood pressure dropped somewhat and the nurse called for the Doc. It was a different doc then we did the consult with but he was great too. He upped the saline content of the return blood and the blood pressure returned to normal. Later, my son had tingling in his hands and lips, indicating that his calcium level was too low. The doc upped the calcium level and everything was fine.

 

After the treatment was over, my son had to pee desperately because he had been sitting there for three hours getting pumped full of fluids. He did not want to eat or drink anything after the treatment until really the next morning when he got his appetite back.

 

Ten minutes into the second treatment my son started sneezing/sniffling, said his throat hurt and got a few hives on his face. The nurse stopped treatment, called the Doc, and he called for a dose of Benadryl intraveneously. The benadryl caused a pretty strong burning sensation in my sons hand that he really did not like, but the benadryl took care of the alergic reaction and the treatment was resumed. Apparently allergic reactions are pretty rare, but seem to be more common in PANDAS kids, maybe due to hyperreactive immune systems.

 

We had to take a day off before the last treatment to let my sons blood clotting factors rebound(fairly common) and then did the final treatment uneventfully on Fri evening. At about 2a.m. Saturday, a resident on the ICU floor removed the catheter. My son seemed to be in a great deal of pain when they removed the tape holding the catheter in place. I could tell that the resident felt he was overreacting and he had had a great deal of aprehension about the catheter coming out, but, his groin was black and blue, I assume from the difficulty they had finding his femoral in the first place.

 

We left the hospital the next morning. My son limped for a day or two but now seems back up to speed physically, but his groin still looks bruised.

 

Dr. Latimer does not play a big role while your child is in the hospital. I'm not sure what her standard practice is but she did come by the hospital and check on us during the last treatment but I think we sort of guilted her into it. I think we were expecting her to play a bigger role but there really is nothing for her to do. It probably should have been a question I should have asked during our consult with her.

 

Overall, the treatment was a really big deal. As Dr. Latimer said to us though, you chose a big deal treatment to treat a big deal problem.

In the end I would say it was a fairly traumatic week for my son. My wife had a very hard time watching him go through what he went through during his treatment. Was it worth it? If it winds up being a cure than I would say yes. If not than I think I will have some major regret and sorrow that we put him though it. So far one week post treatment I would say he has made some progress but is far from PANDAS free. Tonight in fact he had a ton of OCD/ separation anxiety. I can't deny we are a little discouraged but realize that the healing process can take a long time. I will keep you posted. I'm sure I left out some details that some of you considering this option might be wondering about. Please send me a message and I will try to answer. Alex

[dee45]

Hi Alex,

thanks so much for sharing you sons story you made me understand the entire procedure --we are not near that route at this point just starting steroids in the am but don't know what the future holds. I hope you see big improvments in your son keep us posted. I will say a special prayer for him tonight and for you and your family, hope you have a wonderful weekend.

 

Deanna

[LNN]

Alex,

We were at Georgetown for PEX a week before you, also under Dr Latimer's direction. Our experience was very similar, although my son did not have any allergic reaction and we had 3 uneventful days in the PICU, aside from some minor issues with blood clotting on its way out of the catheter line on its way into the pheresis machine. They had to occasionally stop and use heparin or saline to draw the clots out and resume the process. But at no time did I feel there was any danger. The entire staff was very experienced. I can't say enough about the Hemonc doctors. They were awesome. We met with Dr Latimer prior to our admission, but never saw her during or after our stay.

 

I would say the worst part of the entire process was the removal of the 2" x 2" adhesive bandage that holds the catheter line in place for 3 days. I helped the nurse, but she was way too conservative with the use of adhesive remover and my son screamed as tho he were in labor. I think he scared every kid in the PICU. He was an absolute trooper for 3 days of needles, pain, and being cooped up. So I don't think he was over-reacting with the pain that came from removing the adhesive that was millimeters away from his scrotum. The actual removal of the catheter line was nothing. But the adhesive bandage? Ugh!

 

I do want to give you one word of caution on your expectations. Like you, we had already started to see a decline in my son's symptoms after a year of almost constant re-infection and exacerbation. In May, just after we sent a blood draw to Dr Cunningham (183% above normal Cam Kinase II, near the SC kids when she charted his results), his tics got so bad he actually started "freezing" like a statue for about 3 seconds at a time, and he'd stop speaking mid-sentence for abut 3 seconds. This was the second episode where this had happened. It was terrifying. In July, he had a T&A and was starting to heal on his own. But because of previous "freezing" episodes that reminded me of von Economo's, I couldn't handle the fear that there was still a time bomb inside that would go off again as soon as he was re-exposed to something. I wanted that junk out. We too weighed IVIG. But because his chorea and tics had been so bad in the past, I worried whether one IVIG treatment would be enough. And I figured that it made more sense to take the junk out and then, if need be, put a donor's "good stuff" in instead of the other way around. We too were told by Dr Latimer that she'd never seen a kid who'd had PEX come back for another treatment (I believe Dr Swedo had one case). So off we drove, 9 hours to DC...

 

We got home two weeks ago today. The first week, his tics were same as they'd been before our trip (his OCD was also mild before and after). But there was a subtle but significant improvement in my son's demeanor. He was calmer, when he was told no about something, he let it go and said ok instead of arguing endlessly about getting his way, he could play by himself, he was respectful of his sister, not bossy or dictatorial. There was cooperation. He said he felt better and for the first time in almost a year, he started going to bed without saying the words "my muscles are sore and I have a mixed up feeling". We were cautiously optimistic.

 

Unfortunately, this past week, his cognitive "fog" has returned and his tics are worse - stronger, more frequent. And we're seeing the return of tics we hadn't seen all summer. His muscles are also sore again. It's been a rough week on us emotionally. Very hard to be philosophical or tell yourself it's a healing process. I've been told that Dr K says IVIG kids who seem to have an inner struggle after treatment seem to do the best long term, but no one seems to know if this is also the case with PEX. I do know that another Pandas boy who had PEX the same week we did also saw his tics get worse about a week after he got home. His family's been on vacation, so I don't know how he is right now. MomMD also mentioned a sawtooth pattern to her son's recovery. On a positive note, my son's improvements in temperament/demeanor have not gone away. So I hold on to that and hope that this is just part of the process.

 

I dread the start of school, fear swine flu, and cannot seem to mentally get to a place where this feels that it's behind us. There are times I get angry at the lack of followup on PEX patients (or IVIG ones for that matter) to chart progress and determine what's "normal" during recovery. But for the moment, all we can do is wait and continue to be strong - and patient. If after 3-4 months we don't see the improvement we were hoping for, or if swine flu hits our house with a vengeance, perhaps we'll look to IVIG for an immune boost. That's my plan B that helps me sleep at night.

 

Please keep me posted - with good news or bad. I share everyone's frustration at feeling adrift. I am getting close to the point of taking our life savings, reserving a conference room at the Leavy Center at Georgetown, inviting all the families and doctors there, and locking the doors until we emerge with some answers. But first I guess I need to make sure we won't need our life savings to pay medical bills!

 

I wish your family all the best in recovery!

[Alex]

LLM, thank you so much for your response. Just hearing someone elses story is reassuring. As I mentioned in my post, removing the tape covering the catheter was by far the worst part of the process for my son, just as for yours. I think for now we just need to keep my son healthy, do everything we can to keep him strep free and let the process take it's course. I sent you a PM last week with our phone number. My wife would love to speak with you if you get a chance to call. Thanks, Alex

[LNN]

Alex,

I didn't get your pm from last week. I just pm'd you with my contact info. Would love to speak in person.

[EAMom]

Alex and LLM...Thanks for posting! It's great that we are getting more parents on this forum from Dr. Latimer (who have had PEX). I think this will give others valuable information all available treatments. Plese continue to keep us updated both your sons.

[NancyD]

This is GREAT...I concur...it's very valuable having this insight into PEX. Thank you! I wait to hear updates in the future.

 

Nancy

 

Alex and LLM...Thanks for posting! It's great that we are getting more parents on this forum from Dr. Latimer (who have had PEX). I think this will give others valuable all available treatments. Plese continue to keep us updated both your sons.

[LNN]

Diana has hooked me up with a mom whose daughter had PEX 8 years ago (should someone tell Diana that she's reached "star" status and can be recognized by just her first name, like Oprah or Prince?)

 

I only traded a quick email with the mom and will hopefully talk with her over the phone. But she said it took 6 months for healing. But her daughter just left for college yesterday - far far away - packing Advil as her only medication. So hope has returned in our house (tho it's tempered by the start of school tomorrow) :(

 

Will keep everyone posted...

[dcmom]

LLM- that is great news. This illness is so tricky, but it definately seems pex has worked for most. I was thinking- pex removes the antibodies, but if there was alread inflammation, that may take time to heal. Would it be possible to try a steroid burst to jumpstart that healing?

 

Please let us know what the mom you make contact with says. We are getting closer to pex at Georgetown, so I am desperate for any and all info.

 

Hang in there!!

[LNN]

LLM- that is great news. This illness is so tricky, but it definately seems pex has worked for most. I was thinking- pex removes the antibodies, but if there was alread inflammation, that may take time to heal. Would it be possible to try a steroid burst to jumpstart that healing?

 

Given that we've already thrown away the body's "standing army" of antibodies to all sorts of illness, I'd be very leary of prednisone and suppressing the immune system even further. My son started this nightmare with his first strep infection 51 weeks ago - the second week of school last year. Between that and H1N1, I'd rather just let the body move at its own pace and only do things that will boost the system, not suppress it.

 

On a positive note, today there is noticeably less humming (vocal tic). Will keep you posted, but am turning the PC off for the day to go to the park on our last day of summer vacation. Sometimes it's too easy to spend too much time researching and forget to enjoy the kids.

[mom md]

We did PEX at Georgetown July 6th so we are almost 8 weeks out. We did see drastic improvement, the most astonishing was the moods swings were gone. Almost immediately he was patient, calm, and slept well. His anxiety resolved as well as most of his chorea. He even said he felt better. About 10 days ago we saw some mood swings come back but they were gone again before I could contact Latimer. I still see sporadic chorea (it is subtle and I am sure my husband and I are the only one who see it) but his mood is great. We went out of town this weekend to a church retreat and really pushed his system by staying up late, we had just started school, and he had a cold. I can actually say he looked just like all the other 8 year old boys running around. I am convinced he is SO MUCH better. This morning I got up and he had made his bed, dressed himself, brushed his hair, and was downstairs eating his breakfast he had made. These are all things that NEVER would have happened pre-treatment. I am glad to see what that mom said though about it taking 6 months. I think that explains some of the chorea I still see. I am still struggling though about how not to over-react everytime I see a glimpse of the stuff we saw before. as my pediatrician said, "it is like a parent whose kid had leukemia, everytime they see a bruise they relive the fear." I think I have PTSD! Hopefully, time will help that.

My best to all,

Claire