need help to advise my mom(84) on CIDP treatment

sorry no info

I was diagnosed with CIDP March 2008. I had my first loading dose in April. I made some improvement. I had fallen several times and could not get up, my husband had to help me dress if it went over my head since I could not lift my arms past shoulder heighth. I could walk but needed help to rise from a chair. Again, I improved some but not to the degree I had hoped. I was then given maintenance dose for the next two months (23 days apart). I made no improvement on the maintenance dose. I did not, however, lose any of the progress I had made from the initial loading dose. I was then put back on a loading dose. After two more months of IVIG and time off from work to sleep and rest (I took 2-4 hr. naps each day). I started improving. I improved until I went back to work and gave up the naps. The neurolgist was amazed at how much strength I had regained. I am now very functional. My quads are still not working so I can not get up from the floor without help. The dr. was so pleased that he talked about extending my treatments to every 30 days but to continue to receive the loading dose. However, I seemed to go downhill 2-3 days prior to the 23rd day. So, we adjusted to every 21 days. I have infusions next week and at that time I am scheduled for another EMG. I have not had one since last July. I am looking forward to seeing how much I have improved measured by those tests. I have not felt like there has been any improvement for several months. However, I went to my granddaughter’s soccer game last Saturday and felt much more confident walking across the uneven field than I had felt when I went to her games last fall. I think once you get to a certain point the improvement is much harder to detect. I made such great strides earlier. I do want to mention there is a lovely lady in her 90s where I receive my infusions. She lives on her own but gave up her car last summer. She has been receiving IVIG for several years. I have not seen her walk. She usually starts her infusion prior to my schedule and I finish prior to her. I hope this helps.

My first IVIG was considered a loading dose over several days. The next two months the dosage, which was a maintenance dose, was half of the loading dose. After the first IVIG dosage which consisted of several days throughout a week, I improved. For the first few days afterwards I just felt, not really bad just kinda listless. About the third day I had more energy. Then I slowly had more strength, not a lot of strength, just more than I had. I still had to have help dressing, but I could roll over when sitting on the sofa and push myself up. This was disappointing because I thought with the one dosage of IVIG I would be back to normal. But, I was somewhat stronger in both my arms and legs. My next IVIG was half the orginal dosage. It too was over several days, but it took about half as long each of the days. That was May and June 2007. I did not improve on the reduced dosage, but I did not lose the strength I originally gained after my first IVIG. Again with each of the two maintenance infusions (May and June), for about three days I felt listless. Then my energy would improve but no increase in strength. In July, I went back to the increased load dosage. With the increased dosage along with taking long naps everyday I started gaining strength. After each IVIG during this period of time, it seemed to take 7-10 days before my energy level started improving. We moved the interval from 23 days to 21 days, when I noticed my energy level decreased and my legs had the wierd feelings running up and down of what I call “wired”. It is like the feeling you get when you hit your “crazy” bone in your elbow. That is as close as I can describe it. We did not change the interval until the December to January interval. I had notice in previous months that my energy had fallen off and my legs felt “wired” about three days before it was time for my infusion. But since I had gained strength (late October, November time frame) I had done something each month that the neurolgist thought had caused the problem. One time I walked down four flight of stairs. (You can see I have improved)!! He told me to make sure I kept consistent activity throughout the month to see if we need to move up the interval. So, I made sure during December I did not do anything out of the ordinary to cause the problem. And, I still had problems (lack of energy and “wired” legs) three days prior to the infusion. So for the interval between January and February we moved it up to 21 days and that worked. I had my last infusions on February 2 and 3. I will have my next infusions next week, February 23 and 24.

Again, I have really improved. But, I am far from normal. I told you I walked down four flights of stairs. I can only walk up one flight and it is not a pretty sight. I feel like my legs look very wobbly (they certainly feel that way). But, I feel very functional and if I am not put in a situation where I have to bend too far down that requires quad muscles or walk up more than a few steps, I do not think anyone could tell anything is wrong. I can dress myself, dry my hair (hold the hair dryer over my head –yeah!!), put a dish on the second shelf in the cabinet. I can walk all the way through the grocery store — not just one aisle like before. I could not do any of those things before. Prior to CIDP, I had always been very strong. So, this has been quite a trip!!

I hope you can make sense of my time frames above. I just thought of one more thing. Part of my diagnosis of CIDP was my responding to IVIG. Maybe your mother’s doctors want to see her response.
Again, I hope this helps.

As I understand it, plasma pheresis filters the plasma and replaces it with fresh frozen plasma or with a synthetic plasma. My son had PP initially and then was followed up with IVIG. Our doctors explained that PP should not follow IVIG as it would filter the medication from the blood, possibly reversing the benefit from the IVIG.

My son (GBS Jan 2008 at age 18) was treated at the Regional Rehab at Pitt Memorial in Greenville, NC for his inpatient Rehab and my father (GBS 1993 at age 59/ MS dx in 2003) has been to the Outpatient Clinic at Pitt for PT. The OT and PT services are exceptional there if that is an option for your mother.

It really is a small world!:)
My son was treated initially at Duke for five and a half weeks and then transferred to Pitt for Rehab for ten and a half weeks- I guess his age helped his recovery and his progress helped justify his length of stay for the insurance. When he was admitted to Pitt, he was still had a trach and Gtube and couldn’t move anything below his shoulders. He really had a tough road but worked hard; he was able to walk out at the end of his stay using only the cane. Now he is back to normal- what a change!
My Dad did not have the degree of paralysis my son had- he was affected waist down and didn’t need inpatient Rehab. He was treated at Pitt Memorial for the acute phase of the GBS. His biggest obstacles have come from the MS. His mobility now is pretty limited-uses a walker for short distances and a wheelchair for going out.
They both see a Neurologist at Duke- Joel Morgenlander who is wonderful. My dad did see Dr. Hardy in Greenville initially but decided to make a change when Dr. Hardy did not seem to listen to the problems he was having. His doctors at Duke diagnosed the MS.
I guess the bright spot to this ordeal with GBS is that knowing what we did from my dad being sick really helped us to be the best advocates we could for our son. And since his case was more severe than his Grandfather’s, he really needed that. At the beginning when things seemed so bleak, he drew alot of comfort from his Grandfather’s visits.:)
I hope your mother is able to get stronger and get back to being more independent. Please send her my best wishes for her recovery.

Hello Joan,

I am sorry that your mother has developed CIDP.

It is difficult to say what the administration schedule of IVIg should be, as it depends completely on the individual’s response. Still, when first fighting this disease, people usually get 2 gram of IVIg per kilogram of body weight over five days, then 1 g/kg at least monthly for several months. By that light, your mother is overdue. However, did she have significant improvement in at least one area (strength in her legs or hands, or a reduction in sensory issues)? If she did, has she declined since then? If yes to both of those, I would suggest that the usual dosing and schedule be followed. It might be worthwhile to try a more aggressive approach, though, if no to either question.

The Peripheral Nerve Society has published guidelines on dealing with CIDP ([URL=”http://www.pnsociety.com/Guidelines_CIDP.pdf”]http://www.pnsociety.com/Guidelines_CIDP.pdf[/URL]). Note, though, that these are only guidelines and that you and your mother should push the neurologist if what s/he is doing does not seem to work. However, you will need patience, as effects can a few months to see.

As to plasmapheresis, that is part of my treatment plan. It works, but it carries some risk as well. If your mother does not have good venous access, she would need a catheter. The care and maintenance of one is a chore, and you need to carefully evaluate if her support team will do the necessary work. The actual process of plasmapheresis carries risk for fainting during and for a couple of hours after the procedure. Before starting plasmapheresis, I would discuss it with each of her specialists.

Another issue with plasmapheresis is that it often does not work without other therapies as well. In my case, I take prednisone (large dose once per week) and Imuran (typical dose). Others do well with just plasmapheresis and a low dose of prednisone. You would need to evaluate her likely response to the other drugs she would most likely need to take.

Godspeed in finding an appropriate treatment,
MarkEns