All about Guillain-Barré Syndrome - Dennis wrote a detailed account of his encounter with CIDP

I am a 61 year old married male with two daughters and live in Upstate New York, USA. During the mid 1970's I started experiencing periods of weakness, especially in legs and arms. They occurred three to four times a year and lasted four to six weeks, each instance. I was not and have never been debilitated to the point of needing mechanical assistance (cane, walker, etc.) to function.

I was in and out of the Doctor's offices trying to determine the reason of this weakness. There were a number of theories and diagnosis including: Type A personality with a prescription for diazapoxide, Hypoglycemia with glucose tolerance tests, and others I don't remember. I was never so bad that my symptoms were severe enough for my family doctor to send me to a specialist.

Late in 1990 I was on a hike in the Finger Lakes region of New York and became so weak and wobbly, that I had to discontinue my walk and have my wife go for the car to pick me up. I scheduled another trip to a new family doctor. I was examined and it was noticed that I showed little or no reflexes. An appointment was made with a Neurologist in Rochester, New York. It was during this same Doctors visit that it was discovered that my blood platelet level was extremely low. Normal platelet levels are between 150,000 and 400,000 platelets per ml. My level was 13,000. An appointment was made with a Hematologist.

The Neurologist performed nerve conduction tests and a spinal tap. It was this Neurologist's diagnosis that I had CIDP, however he had not treated CIDP cases. He sent me to another Neurologist that is connected with the University of Rochester Medical Center and Strong Memorial Hospital for further evaluation. This Neurologist confirmed the CIDP diagnosis. The second Neurologist was involved in a study where they were trying to determine how IVIG therapy works.

In the meantime the Hematologist determined that I have Immune Thrombocytopenic Purpura (ITP) which is a long title for a condition where the immune system attacks blood platelets. I was put on Prednisone for this condition which initially raised my platelet level but after several weeks it came back down to the low-low level. I noticed no positive effect on my neuropathy from 8 weeks treatment of Prednisone (30 mg 2 times per day).

The IVIG study involved going into the hospital for several days and having two IV attached to my arms. IgG was administered in one catheter while samples of blood were removed from the other over a period of 24 hours. I got a copy of the paper that was published as a result of the study but it didn't mean much to me. I did however develop a good relationship with the second Neurologist who has been treating me since 1992.

In that period I would get IVIG treatments for either my CIDP or ITP condition depending on which required attention. Early on I was getting 120 grams of IVIG in a 3 day session. This would last 3 to 4 months. I would start to feel strength and stability improvements about 10 days after a treatment and start to feel a decline after about 6 weeks. My platelet level would peak about four days after an infusion. In those days I received IVIG more for my neuropathy than the platelet problem.

After several years however my untreated platelet level declined to lower levels and it became necessary to have infusions more for ITP than CIDP. Monitoring of platelet level requires only a fairly simple blood test whereas CIDP demyelization and resulting neuropathy is much more subjective. Therefore it has always been an easy call to reach a low platelet level and receive a treatment while the Neurologist might need a bit of convincing and prodding to prescribe a treatment. My Neurologist seems to be more concerned with cost and IgG availability than my Hematologist.

There was a period in the mid 1990's when IVIG was in short supply because of a recall by one of the major manufacturers. The recall was because it was determined that one member of their donor pool had received Human Growth Hormone which apparently posed a threat for CJD (Creutzfeld Jacob Disease).
As a side note, I did receive some of the product lot numbers that were recalled.
During this period I was receiving 30 g. IVIG once every three weeks almost exclusively for ITP. Over a period of time the level where my platelets stabilized after a treatment kept going lower which meant more product. By 1998 I was getting 60 g. IVIG every three weeks. With this much IVIG, so often, I experienced little fluctuation in my symptoms of neuropathy. I always feel a little weak and wobbly but get around o.k.

I always felt slightly sick after a treatment and on several occasions had what I would term mild reactions to IVIG. The reactions included tightness in my chest and shivers and shakes. My Hematologist started giving me 100 mg. hydrocortisone IV as a pretreatment just prior to administering the IVIG. Lately the hydrocortisone has been switched to 4 mg. of decadron. Both seem to work equally well in minimizing reactions but the decadron usually causes the loss of one nights sleep because it hypes me up more than the hydrocortisone.

In May of 1998 I experienced a relapse of my CIDP and had extreme difficulty getting around. I was given several IVIG infusions and after several months was back to normal level.
During this same period my Neurologist wanted to try a different product WINRHO which is a Rhesus antibody. This product is also given IV but is a much smaller dose and only takes a matter of minutes rather than hours to administer.
After the full dose was given I had a severe reaction and ended up in the ER for 6 hours until my blood pressure came back down to somewhat normal. My platelet level did go up to over 300,000 which I had not seen in years.

The standard treatment for ITP for years has been the splenectomy (having the spleen surgically removed.) I have resisted this procedure because IVIG treatments are effective in treating my CIDP and I cannot count on the Neurologist to prescribe IVIG as I might feel the need. My Neurologist is more inclined to use steroidal drugs which are less expensive than IVIG. With my spleen removed and a more normal platelet level I might end up in worse shape neurologically.
My Hematologist has talked to me about trying Rituxan. The RITUXAN (Rituximab) antibody is a genetically engineered chimeric murine/human monoclonal antibody directed against the CD20 antigen found on the surface of B lymphocytes. My Neurologist has said that it has been used in CIDP cases but not enough so that he knows of studies.

Currently I receive 60 g. IVIG once every four weeks. This is preceded by 4 mg. of decadron given IV as a pretreatment to minimize side effects of IVIG. The 60 g. is given in one day. I do not have a port and have never had a vein problem. I have been through 158 treatment cycles over the past 12 years with no real problems. I know of no side effects other than reactions such as described. Some people have difficulty with these side effects. I know of no long term side effects. My liver function tests are good. An infusion of 60 g. usually takes about 4 hours. The IVIG product that I get is a 10% solution. Therefore the 60 g. is really 600 g. of liquid. The rate is started at 30 g. liquid per hour and increased by 30 g. per hour every 15 minutes. The maximum rate that I receive is 240 g. per hour and I believe this is based on weight.

My current CIDP symptoms include: Some instability in walking; some numbness in feet, finger tips, etc.; tremors in hands; mini-spasms in legs; false hot/cold feelings mostly in feet; slight problem with eye focus. Other symptoms that may or may not be related are dry skin problems, dry eyes and tinnitus (constant ringing in ears.) These symptoms seemed to come about the same time as everything else.

After 12 plus years I don't expect these diseases to go away. I am not seriously debilitated. I get around pretty well and work full time as a mechanical engineer involved in underwater acoustics. There are things that I had planned for this part of my life that I have had to rethink and make substitutions for but I do pretty well. My wife and family are great supporters. I also believe that this was a wake up call from the Lord to evaluate my life and see what my priorities really are. I now live with much more inner peace.

I hope this helps someone else who suffers from CIDP and is using IVIG as therapy. May God Bless You All and help you deal with this disease.

Dennis, CIDP '90
01.2004