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September 2003

What is CIDP?

By Robyn Gobert.

Chronic Inflammatory Demylonating Ployneuropathy (CIDP) is closely related to Guillain-Barré syndrome (GBS), both of which are due to inflammation and demyolination (stripping) of the covering of the peripheral nerves. It is a very rare condition. The symptoms people experience with both of these and other related conditions vary considerably and often mimic similar symptoms of other unrelated conditions.

Paul and Robyn.

Our story

Paul was a pocket dynamo. He was a dedicated worker and very seldom complained. So when he began coming home from work and was falling asleep on the lounge I knew something must be seriously wrong. He said his legs also felt stiff and bloated.

We visited the local doctor and his advice was panadol, a good sleep and to come back if it got worse. That didn't take long to eventuate - by the following morning one side of his face was immobile. He had contracted Bells Palsy but no one could tell us why.

The next ten days were a continuing worry. Paul was off work and he wasn't getting any better. He spent his time sleeping on a bed we'd put in the lounge room, so he'd be warmer by the fire. He didn't complain, he just slept.

The household chores were increasingly falling to me. I was worn out. Finally, frustrated and tired, I said, "Look, I can't do everything around here you know? Couldn't you at least go up to the shed and bring down a bucket of wood for me?"

He was more than willing. Watching him walk back down to the house I noticed how strangely he was walking - sort of peg-legged. To my horror Paul toppled over and tried repeatedly to get back up but only succeeded in rolling him over a few times.

I dialled 000 and asked for an ambulance. Then I called Paul's workplace and asked, "Quickly!! Who's the best doctor on the Tablelands?!" The lab technician who'd answered the phone screamed, "Who's the best doctor on the Tablelands?" and five voices in the background all called the same name. I dialled the number and said to the receptionist, "I need to speak to the doctor, NOW!"

The calm, assured voice we came to love and trust answered immediately. Goodness knows what he made of me as I blurted, "You don't know us. Paul's collapsed and is on his way to the hospital. He already has Bells Palsy and says his legs feel like they're wrapped in tight plastic film. We want you to see him."

By the time night was falling Paul could not get up off a chair without help. In a few days he could not move his legs very well. He said his hands and legs were tingling, like a mild pins and needles effect and everything about him felt tight. I was frightened.

Home again - Paul was now out of hospital but off work and the bills were rolling in. I was coping alone, loading him into the car so we could go for twice weekly doctor and specialist appointments.

The medical profession did not seem to know what was wrong with Paul. Our GP suggested it might be Guillian Barré Syndrome (GBS). However, the "specialists" were foxed because of the unusually slow onset of the symptoms.

It was frightening how quickly Paul lost all condition in his legs when he was no longer using them. At first he had a walking frame, then a wheelchair. His muscle tone was completely gone from the places that had become paralysed. Another extenuating circumstance was that Paul's feet were not effected (they became floppy but he did not have a loss of sensation in his feet and this seemed to keep the medico's exasperated).

One night I'd been reading a magazine article about a man whose symptoms sounded remarkably similar to Paul's. The next morning we went to the specialist for our visit and I asked him straight out what he thought it was. He was twiddling and doodling and couldn't look at us. I blurted, "Just don't tell us it's MND!" He went pale and mumbled, "That's exactly what we think it might be. Motor Neurone Disease."

We were given the option of driving to Townsville, five hours away, to see a Neurologist. At a loss as to what else to do, we took this option, even though it was difficult to transport Paul by this time.

Although he was not in pain from the condition (as many people with GBS recount), his lack of muscle control meant that any jolting or being moved about made him very tired and his internal organs felt sort of bruised.

We arrived in Townsville to find there'd been some sort of a communication breakdown at the doctor's. We were not expected but we were told, grudgingly, we would be "fitted in". Paul's examination was rushed and we were advised, bluntly, to go home and get things in order. Paul was supposed to have a rare, rapidly progressive form of MND and he'd have three months to two years to live.

As we left the doctor's rooms this doctor said coldly to me, "You realise it 'll be more like three months than two years, don't you?" First I cried. Then I got mad. Then I set my jaw and was galvanised into action.

Paul's family was planning to drive up to see us, but Nan, Paul's grandmother, was elderly and unable to make the trip. It was decided it'd be better if we flew down as then the extended family and childhood friends could say "Goodbye". Our doctor said he'd authorise it if we could go immediately.

It was 1990. To comply with flight regulations in those days Paul had to wear a catheter, even though he didn't need it! It was so he didn't have to use the little toilets on board, as he might fall.

We were unceremoniously loaded onto the plane to Sydney via forklift.

It was blowing a gale and it was a rough flight. When we landed at Pelican the family said, "Cripes! The way you came in at an angle we thought we were going to be fishing you out of Lake Macquarie". Paul and I had seen the lake fast approaching and we'd thought it was curtains too, but neither of us cared. We were dying anyway.

Over the next eight days at Paul's parents home we thought the end was coming much sooner than expected. The rough handling had caused Paul to suffer a twisted bowel and he was in serious amounts of pain. We both thought the end was very near.

The day before we were to fly back home I received a phone call from our doctor. "Do you think Paul is able to be transported to the Prince Henry Hospital in Sydney? I studied under a Professor of neurology there. He's one of the top men in Australia and I'm willing to ask him for a favour at short notice. I know he'd be interested to take a look at Paul. Will you go down if I can arrange a bed?"

We were of two minds as there didn't seem to be any hope for Paul and more travelling seemed like an unnecessary burden. Eventually, feeling a little better, Paul decided he'd like to do this in case it eventually helped someone else in the same position.

The professor and his entourage of doctors, all students of neurology, would make their daily rounds. After the second examination the Professor announced, "It might not be anything better, but it's not motor neurone disease. We just don't know what it is at this stage. Puzzling, puzzling ..."

By day Paul would undergo a seemingly endless list of tests. He wasn't feeling sorry for himself. He was quietly angry, just getting through things the best way he knew how. He said his feeling was "one of unfairness", that he'd "completed his apprenticeship of life and was not going to be allowed to live and enjoy it".

One night I became overwhelmed with the atmosphere of the place - the constant stress and grieving around us. I was a country girl and the consistent threat to one's personal security, with signs up everywhere warning not to walk alone at night and the constant presence of police and security guards, was nerve-racking for me.

There was a jail opposite the hospital and helicopters would circle overhead with their bright searchlights scouring the beach for escaped prisoners. Just outside the lift near Paul's bed were signs like "remove guns and weapons before entering"! Inmates coming to the hospital for treatment would grab something as a weapon, a scuffle and chase would ensue. It was an unreal scene in an unreal life.

My nerves were failing, and so was my health. It was the time when my arthritis was first starting to act up and I found it terribly hard to walk the 700 metres to Paul's ward and back several times a day. Our days were filled with what had to be done.

The hospital was short-staffed and I had made it clear that I'd nurse Paul, seeing to all of his needs that did not require medical intervention. We wanted to be together every moment that we could manage.

Late one night I just couldn't take it any more. It was hard to sleep because of worry, and this was exacerbated by the constant ringing of the phone in the hallway just outside my hostel door. This internal phone usually delivered bad news - so you would lie in bed hoping the call was not for you. I waited until the security guard came around and asked them to take me to the ward, so I could be with Paul. When they so kindly agreed I broke down with relief. They were just terrific.

My own inability to cope gave Paul added strength. "Get me into a wheelchair?" he asked. "Come on, we're going down to the lounge and you're going to stay with me for the night."

There was a nurse sleeping on the couch. "Aren't you supposed to be working?" Paul enquired. It was so unlike him that she shot upright and made a beeline for the ward. We stayed there and talked until the sun came up. It was the first time I'd let go! Paul was also going insane sitting in bed all day and night.

Paul with a possum.

As Paul is a nature photographer, we missed going bush and we missed our interactions with the birds and animals within our outdoor lifestyle. We decided that even if there was only weeks left for us, we were going to find ways to live it to the fullest.

The medical tests continued. Paul was becoming more and more paralysed as time went on.

Sometimes, with Paul sick of hospital food, we'd go down to the staff cafeteria and have a multi course meal together. We ate whatever we liked, in what ever order we fancied - pudding first or three helpings of pudding - we'd decided those normal little rules of life no longer applied to us.

At this stage Paul was just "in hospital". As no one could tell us what he was suffering from there wasn't any real treatment. His paralysis, having started in his ankles, was progressing up his body and was now up to the base of his neck. He could no longer hold a cup or transfer to a wheelchair, even with assistance. We were told that the next step would be an inability to breathe unaided, and then he'd be put on life support machines in intensive care.

In a last ditch effort to save his life, it was decided to take him in to conferencing. This entailed all of the doctors from the surrounding area studying the case and asking questions, poking and prodding.

A young American doctor came bursting in, waving a piece of paper, yelling, "I think I've got it! I believe I might know what Paul's condition is!" He went on to explain that he'd remembered reading something like this in an American medical journal. He'd phoned his Mum who'd dug around in the boxes of old magazines in the attic and she'd faxed him this copy. It seems extraordinary, but it's true.

The condition had not been widely diagnosed, but it was called Chronic Inflammatory Demylonating Polyneuropathy (CIDP) and the initial treatment was cortisone.

One would think we'd be ecstatic, right? However, at the time it seemed like it could all end up being a cruel trick. We did not dare believe it. If it wasn't true, to come to terms with his prognosis again would have taken more than either of us had to give, as we had now become resigned for the worst.

The professor put Paul on a large dose of cortisone and we waited to see what would happen. The cortisone sped his body up resulting in difficulty breathing and a feeling of panic. The cortisone dose was immediately lowered, Paul was more comfortable and the waiting game started again.

Once the immediate danger seemed to be over I'd gone back to my dorm to sleep ... and slept for 18 hours straight. Upon waking, I phoned Paul and he told me to go back to bed and sleep some more - all was OK. He just needed to rest.

When I finally appeared beside his bed he excitedly asked for a wheelchair to go down to the bathroom. He urged me to wheel him right into the shower cubicle. Leaning forward he slowly raised his arms and gripped the bars. I was astounded. "Are you feeling that much better?" I asked excitedly. Smiling, Paul shifted forward in the chair and pulled himself upright until he was standing.

He began to wobble slightly so I stepped forward to support him. My elbow knocked the lever tap and we were both sprayed with cold water. We were crying and laughing all at the same time. Two nurses ran down to see if we were all right. When they saw Paul standing and me struggling to hold him up, they came in to help and we all ended up wet, laughing and crying.

There was great excitement. The Professor arrived and Paul began plasma phaeresis (PP) treatments to speed up the progress of his recovery. No one could tell us how much of a recovery he would make or if it would last.

Plasma phaeresis transfusion.

What's PP?

Basically Paul's blood was slowly drained from his body and spun in a centrifuge. The white cells were removed, healthy white cells were remixed and his blood was slowly being replaced via his other arm.

It was almost Christmas Eve. After five weeks in hospital the various doctors were humming and harring over whether or not we could be released. Finally we were sent home and we arrived back to the loving arms of my Mum and daughter late on the night before Christmas. Paul's employers had also been marvellous, sending their workmen to put in wheelchair access at the back of the house before we came home. That Christmas, dinner ended up being something like sausages and mash!

Slowly Paul recovered his strength. He was able to walk again and eventually returned to work on light duties - a big day!

Occasionally there have been recurring symptoms and we were afraid of a relapse. On top of everything we had experienced I found this very stressful. My anxiety increased and I was admitted to hospital for three weeks complete bed rest. Apparently I was suffering classic symptoms of post traumatic stress syndrome.

In 1996, after writing of our brush with death for That's Life! magazine, we were phoned, written to and even called in on by a host of people from across Australia and New Zealand. They urged us: "People are dying from these misdiagnoses - you've got to do something!"

So in 1996, I founded the GBS & CIDP Support Network of Queensland Inc. It's still being run by a merry band of recoverees with much of the credit going to Ro Harre. I've still got a finger in the pie. There's also an active group in the Bundaberg region and Ro is a member of the national GBS Foundation and keeps us informed.

Paul feels he is now clear of CIDP. He is certainly well and active again but we wait and see whenever he gets the flu or has a stressful time.

Paul sitting on a lantana vine in the Queesnland rainsforest.

Just while writing this tonight I found myself wondering why, when I was in such need at the Prince Henry, I didn't try to source some kind of support group or assistance - that's obviously what I'd do now.

CIDP support groups

Australian contacts:

International contacts:

Google links

End of Google links

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