All about Guillain-Barré Syndrome - Lauri's story of Remitting/Relapsing GBS, IgG Kappa Gammopathy of undetermined significance

My name is Lauri Lowen, and I live in Redmond, Washington, USA, a suburb of Seattle. Here is my story, which extends from 1996 to 2002.

I had nausea, a headache and severe diarrhea in May of 1996. My neurological illness began on July 3. The interval was between five and six weeks. After I learned that Jejuni Campylobacter was sometimes associated with Guillain-Barré Syndrome, I asked my internist to test my stool for the presence of the bacterium. The results showed that it was present in the exact amount for that infection to have occurred at the time I had diarrhea in May.
It may be assumed, then, that I got GBS from eating undercooked chicken in a restaurant (since I would never serve it that way).

While driving on July 3, 1996, my limbs started aching. I felt fine, not sick. Then my arms and legs got very heavy. I could lift them, but it was an effort to do so. This went on for several days. I could not fold laundry, cook or take out the garbage. I could still walk (I shuffled) and climb stairs slowly. I could not hold a newspaper up or use a hairdryer.

Throughout this experience, though I have been affected to some extent all of the time, the severity of the symptoms varies from mild to severe. Exercise, sleep, eating or state of mind seem to have no correlation to the severity of the symptoms. This is not typical of Guillain-Barré Syndrome, which made it very difficult to diagnose and prompted some to imply that I was making it up.

When I had a friend drive me to the Emergency Room, I could barely walk and could not lift a glass or can to drink. I was admitted to the hospital, where I was for eight days.

My illness progressed very slowly, which also contributed to it not being diagnosed very quickly.
My arms and legs at first got worse in the hospital. My left leg would wiggle when I was helped to walk. (They decided then to not allow me to walk, so that I “would not train my brain to do the wrong thing.”) My food had to be cut up. I could not lift the water bottle.

Gradually, after I was transferred to the Rehab unit, I became able to walk with the parallel bars, then with a walker, and then with a quad cane. Two MRI’s were done, of brain and neck. A spinal tap was done. Someone told me that the fluid was not perfect, but not bad enough to be considered abnormal. I was told that I did not have a brain tumor, MS, cancer or Guillain-Barré Syndrome. I was totally unfamiliar with what Guillain-Barré Syndrome was. They told me it was paralysis caused by a virus. (Sometime after my hospitalization, my neurologist, who I later learned had never had a GBS patient before me, decided that I did experience Guillain-Barré Syndrome).

My Guillain-Barré Syndrome had an extremely slow progression. In retrospect, and after talking to experts in the Guillain-Barré Syndrome Foundation, I realize that it was too early in my illness for the spinal tap to have shown protein. If normal GBS protocols had been followed, a second spinal tap would have been done.

While hospitalized and beginning to doze, I experienced sudden difficulty breathing, along with vision fading to black at the edges and pressure (not pain) in my chest. My blood pressure, which had normally been about 90/60, was 200/110. Two other times I was awakened by a wave of nausea, and learned that my blood pressure was elevated (170/120). Other times it peaked suddenly without me feeling much.

After I was discharged, I had periods of increased weakness, when I was unable to stand straight or walk without help (I had been using just the cane). I recognized this later as a loss of muscle tone in my trunk and neck, which also incapacitates my arms and legs. Though my arms and legs continued to improve, the symptoms affecting my trunk and neck continued to get worse, and during those periods when I "lost everything," all improvement in my arms and legs would disappear. This intermittent increased severity of symptoms has been something I have heard of in no other person. (My current neurologist is not concerned about it being atypical.)

I remember watching my face “melt” in the mirror when I was getting a haircut. The corners of my mouth drooped down a little, and my eyelids closed halfway. After my hair was shampooed, I was unable to lift my head from the shampoo sink, and the beauty operator had to lift it for me. Improvement in my arms and legs continued. My parents paid to outfit my house for being slightly disabled (grab bars and shower chair, slide-out shelves holding the heavier kitchen items, a chair with arms to support my arms when on the kitchen phone, a telephone headset in my den). I used my cane and ate by sliding my forearm up and down the table edge.

Following my discharge from the hospital, I sometimes lost muscle tone in my trunk and neck. I sometimes had trouble breathing. My body wanted to exhale and just stop there. I found that if I did deep breathing, very deliberately and slowly, I could force my chest to expand. If I did not try very hard to do deep breathing, my breaths were very shallow and infrequent.
It was during one of these periods that I felt my face start to do funny things. My mouth was contorting. This also is not typical of GBS. I called the neurologist and was told to call 911. When the aid car came, they watched my mouth go from contortion, to normal, to another contortion, etc. The fourth of the men there (an aid car person), took one look at me and said, “She’s got Guillain-Barré.” With my mouth contorted and my speech unclear, I said, “No, I had a spinal tap and it showed that I didn’t have that.”
My blood pressure, taken twice, by two different emergency techs in two different arms, was 190/176.

I was taken to the hospital, where my blood pressure dropped to something over 48. The doctor unkindly said that they had done all the tests in my recent hospitalization and didn’t have any left to do. Despite the problem I was having breathing, she sent me away. I asked to be transferred to University Hospital. They arranged it, though my neighbor had to drive me, curled up like the letter C in her car, looking at the floor, with limited ability to move my arms and legs. I felt that University was the appropriate place for a disease that’s difficult to diagnose.
There, a doctor who thought I got sick because I “should be lonely being alone on an important American holiday like the 4th of July,” said it must be stress. (I told him that on the 4th of July I was writing a paper on the health effects of PCBs and making eggplant salad to take up to my friend’s cabin with my potluck group.) That doctor ordered a Psych consult.

The psychiatric social worker, though she made sure that I intended to keep my appointment with the psychiatrist who was a part of the original hospital's Rehab team, found that I had a wonderful support system of friends, did not pine over my divorce in 1985, was close to my family and my ex-husband’s family, had lovely children I’m proud of, and though I am currently interviewing for jobs (I’m between jobs now), have plenty of money in the bank and a paid-for house. After being examined in the ER of University Hospital, I was given an appointment for the Neurology Chief’s Clinic. I then was sent home.

As the trunk/neck/face/breathing problems became more frequent, friends stayed with me. My son called from his business internship in Washington, DC and informed me that he was coming home to care for me. I told him absolutely not to quit his internship. He said he wasn’t quitting but was leaving early, and he had made plane reservations before talking to me, because he knew I would try to talk him out of it. My parents and friends convinced me to hire an agency to provide 24 hour home health aides until my son got home (three days). We didn't know if I would stop breathing altogether, and the hospital had made it clear that I was not welcome.

When I had the trunk/neck/face/breathing problems, the aides took my blood pressure and pulse. When they could hear/feel them (sometimes they couldn’t), the blood pressure went from high down to 80/40. The changes would take place, back and forth, as they listened/felt. I later learned that this is because blood vessels lose tone, also. I asked the Home Health Aide who was there one day to write a note describing what she saw for my internist, with whom I had an appointment.
This is the content of her note: "July 26, 1996. Dr. Failor, I am a home health aide and I work for Providence-Marianwood Home Care. Lauri has asked me to describe to you what I have observed in the last two days while I have been with her. It is difficult to describe what happens but she has episodes that occur with varying duration, intensity and symptoms. She has sudden changes in muscle control, during which at times her breathing slows dramatically, her pulse becomes weak and her B/P fluctuates and becomes very hard to hear. Her skin color changes from flushed to slightly cyanotic. At these times she is unable to hold her head up and without support it will either fall forward or back. Her arms and legs become limp. These are some of the symptoms I personally have observed. I hope this will be of assistance. Linda Covey, CNA, MA."

I was eventually examined at the Neurology Clinic at UW by Dr. Eric Kraus and Dr. Bell. Their best guess was that it was a neurological viral syndrome which would go away eventually and not make me stop breathing. I had my appointment with Dr. Dipple, the psychiatrist who was part of the original hospital's Rehab Team. At the end of our discussion, he said that he dealt with problems, and I didn’t seem to have any other than my illness. He said that he saw no reason to see me again. His contact with me had been limited to a conversation in the hospital, and this one appointment. Months later, by seeing his diagnostic code on the bill, I became aware that what he told me was not his true opinion. The diagnostic code was for conversion disorder. Because I was a psych/education major, I was familiar with that term. My opinion is that my atypical Guillain-Barré Syndrome, which looks bizarre to everybody, including me, led him to believe that it was a physical manifestation of a psychological condition. This note in my file followed me, leading people to not believe me when I had relapses.

My arms and legs improved, and episodes including my trunk and neck were much improved and decreased in number. I could walk without my cane for several moments at a time, without having my knees start to bend more and more after a dozen steps. When I walked, I walked like Frankenstein. The physical therapist said “slapping” is the name for what my feet did. He says it happens when there is not enough strength to appropriately roll from heel to toe. One day I started to feel numb at the back of my tongue and my throat. I had experienced tongue tingling and numbness before, but never beyond the tip (and then middle) of my tongue. I was able to swallow, but it felt weird when I did. I was annoyed because I thought that the new stuff was over. I didn’t think that there was anything left to be affected, and those that had been previously affected were improving. Eventually, the "new" stuff stopped happening and I got better and better, and my neurologist decided that I had GBS after all.

After several months, I was left with some weakness in the lifting muscles of my shoulders and thighs. I stand with a wide stance for stability and cannot hold heavy things in front of me, but I can live without being able to pass a turkey. If I carry things against my body, I'm OK. If I try to walk up more than one flight of stairs at a time, I have to pause to let my thighs stop burning, as they get muscle exhaustion quickly. I can't hold my arms up long enough to change light bulbs. I use a folding cane in my purse to walk on inclines. I can live with these things.

That was the first part of my story.

What happened next was unusual. I had a small relapse at home during the first year after my illness. I did not go to the hospital because I knew that they didn't believe me and would send me home again. My family cared for me, and thankfully it was a brief and mild relapse.
Another relapse in that first year, in 1997, was so bad that I had to go to the hospital. I went to University Hospital, where they kicked me out after three days because they did not believe me after getting the other hospital's records and reading the psychiatrist's report.
I got better slowly at home and was determined not to miss my son's graduation from the University of Michigan. With the help of a wheelchair, I was able to fly to Michigan and sit in the handicapped area of the stadium. After that, my third time for what had finally been positively diagnosed as GBS, I did not have any more significant relapses. I thought that it was behind me. I was wrong.

On October 3, 2001, I entered the hospital for a routine gall bladder removal. I was supposed to be there for one night. During the operation I began bleeding, and the surgeon thought he solved it by cauterizing it. In the recovery room, I awoke unable to move and having trouble breathing. GBS didn't even enter my mind. After my blood count was found to be dangerously low, they realized I was bleeding internally and took me to surgery for the second time in a few hours. They usually depend on the muscle's natural contraction after awakening from anesthesia to help prevent bleeding. They saw that, for some reason, my bleeding muscle had not contracted. I needed two units of blood, in addition to the blood they suctioned out of my abdomen and returned to me with a Cell Saver.

When, in the post op ward, I still could not move, I thought it was a side effect of having two surgeries with complications and losing blood. Not until the surgeon came in the next day and said that my GBS was back did I realize what this familiar sensation was. Neither I, nor the people at the hospital, thought that GBS could relapse from surgery. It accounted for why the muscle didn't contract after surgery; my torso was already paralyzed. I started off so weak that people had to turn me to avoid bedsores, but made steady improvement when they transferred me to the Rehab ward. This time, they believed me, since I had gotten the GBS diagnosis following my earlier hospitalizations. When I would have the periods with breathing difficulties they would help me... a nice change from earlier times. I never needed a vent.

When I was discharged my son stayed with me for a few days, then a home care lady for a month. The last relapse was as bad as the first time, except it came on strong instantly, instead of very slowly. I saw a neurologist at Virginia Mason Hospital, Dr. Ravits, who is an expert in GBS. (He was in "People Magazine" the week I first saw him!). He diagnosed me at once with Relapsing/Remitting GBS. Everyone I had seen before thought that relapsing GBS had to be CIDP, and tests showed I didn't have CIDP. Relapsing/Remitting GBS, unlike regular GBS, can be turned on by surgery. So there I was, five years later, dealing with GBS again. I had PT in a swimming pool this time, and eventually got back to driving.

Here is where the story gets even stranger.
This wonderful doctor did all kinds of tests to learn if there was anything else going on that could cause neuropathy. He found that a protein in my blood was elevated. I had full body X-rays and bone scans looking for evidence of myeloma (cancer of the bone marrow). The hematologist/oncologist I was sent to determined that, because the level of this protein is not elevated enough to be cancer, I have "IgG Kappa Gammopathy of Undetermined Significance." (Really -- that's the name.) I will need to be checked for the rest of my life for the levels of this protein, as 25% of the cases become cancer. I decided not to worry much about it.

Then the hematologist/oncologist found something more serious. We now know that I have myelodysplasia, which is bone marrow failure. My red and white cells are abnormal and continue to reduce in number. At some point my platelets, which control blood clotting, will also start to become fewer and fewer. It is invariably fatal, though it may take six or more years to get to that point. People with this disease die of infection or bleeding. Astronomer Carl Sagan died of this while awaiting his second bone marrow transplant.

I continue to feel healthy, though I get some troublesome infections and am not the strongest person in the world. But since GBS, I wasn’t that strong anyway. If it was not for this relapse and finding this wonderful GBS expert, we would not have discovered my blood abnormalities until the disease had progressed much more, when it was possibly too late for a bone marrow transplant.

I knew there was a silver lining someplace.

Lauri Lowen
GBS 1996 (twice), 1997 and 2001.
Redmond, WA, USA