My name is Wendy. I am 33 and I have had CIDP for three years. I work as a free-lance musician, playing and teaching the cello. I had no hospitalisation or illness of any kind prior to being diagnosed with CIDP.
The initial onset was very slow. I noticed tingling in my fingertips in early April 1997. As a musician I was very aware of this extra sensation and I went to my GP. Nothing was obvious at this stage. About mid-April my fingers were becoming clumsy and I was suddenly not able to play the cello as I couldn’t reach the top strings with my bow arm. I believed I had a shoulder or neck problem. Friends started giving their opinions of what they thought I had, including frozen shoulder and RSI. The possibility of not being able to play for weeks or months was very distressing. After seeing a sports medicine doctor, having numerous visits to a physio and giving bowen technique a try, I saw a different GP and was sent to see a neurologist. I didn’t get a direct answer from this initial visit and I believed I was wasting my time bothering with a follow up visit. The possibility of MS was ruled out at this first visit and I hadn’t even considered the notion that there could be anything seriously wrong with me. I continued having physio.
My condition deteriorated over the next few weeks and I had difficulty walking and moving my arms and fingers. I was still very stubborn at this stage and I was continuing my teaching but becoming very frustrated as I became weaker. Mid-May I saw my GP who sent me straight away to see the same neurologist. He was annoyed that I had not had the follow up appointment the previous month. His nerve conduction tests revealed an initial diagnosis of Guillain Barré. I was in Royal North Shore the next morning, and following a lumbar puncture and further nerve conduction tests I was admitted. Over the next eight days I had five plasma exchanges. After the first one I had to have a vas/cat inserted below the collar bone to enable a good blood flow. By May 25th I was able to play my cello again. I thought that was the end of my problems as my strength returned quite quickly.On June 9th I had my first relapse with strong tingling returning in the fingers and arm restriction beginning. I had one plasma exchange as a day patient on June 20th but this had little effect. I was admitted to RNS on 30th June. An attempt to insert a vas/cat under my right collar bone was unsuccessful that evening. No X- ray was carried out following this procedure and I went to sleep feeling very sore and numb from all the local anaesthetic. The following morning I was not feeling well but I showered and returned to bed feeling rather out of breath. Another vas/cat was inserted on the opposite side successfully downstairs in theatre but it was discovered I had received a punctured right lung from the previous attempt the night before. A chest drain was inserted to fix the lung and I began plasma exchanges that afternoon. I was wondering at this stage whether the hospital was a safe place to be. A nerve biopsy was carried out a few days later. This confirmed CIDP rather then GBS as the diagnosis. A week later the lung had healed and four plasma exchanges followed by 24 grams of Intragram saw me finally discharged.
I was back again three days later due to jugular thrombosis caused by being immobile while the vas/cat was in and the lung was healing. Following my discharge after four days, I began twice daily injections of Clexaine to prevent further clotting. These continued for several weeks. As much as I appreciated the care I received from the nursing staff on Level 7 of RNS, I was well and truly sick of hospital.
On July 26 I began relapsing and was admitted several days later for an overnight treatment of 60 grams of Intragram. I started on Prednisone in August. After another relapse in early September I was in overnight for another 60 grams of Intragram. I also started on Immuran as well as Prednisone. I was becoming such a regular on Ward 7B even the tea lady knew me.In early October I received 36 grams of Intragram following another relapse. This was not enough to bring me back to normal. I had not realised until this point that Intragram was in very short supply and that I had been fortunate to receive it on previous occasions. I was unable to work and it was increasing difficult to manage at home on my own when my husband was at work. I was prone to falling over and not able to get up from chairs or the toilet. Plasma exchange was now a problem due to access since the punctured lung on the right side and the thrombosis on the left meant no more shunts in under the collar bone. I had very restricted movement and was admitted again in mid-October. I was by now an expert at presenting myself at emergency at RNS. It was decided to give the drug Ciclosporin a try as plasma exchange wasn’t looking too hopeful. I had two plasma exchanges spaced out over several days to rest the veins. The third was unsuccessful. I was rather depressed at this stage as I knew that had the Intragram been available, I could be back to normal and back to work. I was discharged after five days as more plasma exchanges were not possible and there was no Intragram available. I was quite miserable at this stage.
I did not recover and soon began to become weaker. Towards the end of October a single plasma exchange was attempted but it resulted in little more then very bruised elbows. I began ringing the hospital neurology registrars to check with the blood bank but there was still no Intragram available for me. I was determined to get to my brother’s wedding in Maryborough Queensland in mid-November. I left Sydney being able to walk but my legs began to give way quickly. I fell up the stairs to the plane at Brisbane airport and this relapse was the most aggressive I had encountered. Within four days I became a quadriplegic, with no movement in my arms or legs. I had what I would describe as the worst few days of my life despite the efforts of my husband and family, who ended up having to do everything for me. The high humidity that weekend made my situation worse. With no movement in my arms or legs, the CIDP was starting to affect my swallowing and breathing. It was a toss up whether to be admitted to hospital where I was or fly back to Sydney as planned. I returned to Sydney in a wheelchair. I was loosing my voice, unable to cough, having difficulty swallowing and on admission to RNS I had my hands splinted to relieve cramping in the fingers. I couldn’t lie down due to painful cramps in the calf muscles. The following day I received the long awaited 60 grams of Intragram. Had I been able to have it a week previously I would have been spared considerable agony. After four days some movement gradually returned. I had been promised a further 60 grams of Intragam by the blood bank earlier in the week, but this did not materialise once I was showing signs of improvement. My recovery was slow this time due to deteriorating so badly before treatment. The drug regime I was on seemed to be having little affect.
On December 8th I was admitted to RNS once again, this time for an A V fisstula in my right arm. This was decided to be the best course of action for when Intragram was not available again. I could have a plasma exchange via the fisstula, ensuring a good blood flow. Unfortunately the first and second attempts to construct the fisstula in my right wrist were unsuccessful. The third operation in the elbow two days later cut off the blood supply to my hand. In a forth operation the fisstula was narrowed and worked successfully. I began relapsing during this admission and was given 60 grams of Intragram.
Following another relapse in mid-January 1998 I began having plasma exchanges on a weekly basis using the fisstula. These became fortnightly but I wasn’t recovering completely and by May it was becoming increasingly difficult to play with any ability due to weakness. I began receiving Intragram again in May, July, September and November of 1998, lasting a little longer each time between treatments.
During 1999 I received Intragram every six to eight weeks with the intervals between infusions gradually increasing. I was able to return to a work-load resembling that prior to my illness, but my ability to continue playing cello at an acceptable level was always dependent on receiving Intragram. I couldn’t ever count on being able to get the Intragram when I needed it and that is still the case at present. I had a very good run for eight months while I was pregnant, only requiring one infusion. My daughter Ashleigh was born on the 8th of April this year. A fortnight after this I had quite a sudden relapse, with my arm strength and movement disappearing overnight. I could only just lift up my daughter and I was afraid of dropping her. Intragram was available on this occasion so I recovered within a few days.
Having CIDP has made me appreciate the little things in life. Loosing your movement, strength and independence has a very sobering effect. To all those people who donate blood on a regular basis I am very grateful. Intragram is the difference between a relatively normal life and one that doesn’t bear thinking about.