A sixty-five year acquaintance with ITP – By Dave Bagshaw

It began one March evening in 1955. At about 8.00 p.m. petechiae appeared on my arms and legs, followed by bruises, blood blisters on my tongue, and passing blood in urine. Mum set off on the long walk to the phone box to call for medical help while a neighbour supervised me and my younger sister. Our G.P. visited a few hours later, took one look and pronounced that I had Purpura. It was the first time I heard the term, but it seemed apt as by then purple seemed my dominant colour.

The next morning friends walking to school were surprised to see me entering an ambulance, as was Dad as he turned the corner returning from his night shift in the steelworks. So began over four weeks in the Children’s Hospital being treated with cortisone.

I have vivid memories of the enforced bed rest; of watching the four occasions a syringe needle was forced  into the marrow of my hip and the discomfort as the contents were withdrawn; daily blood tests and the pricking of thumbs and earlobes to absorb blood onto a circular filter paper for testing; and the senior doctors pinching the bruise free areas of my torso so that their juniors and students could watch the new bruising appear. The hospital was not child friendly by today’s standards. I was allowed to sit up to eat but otherwise was expected to lie down in bed which was difficult for an eleven year old who did not feel especially ill. Parents ‘ visiting was 6.00 to 6.30 p.m. and strictly enforced. Doors opened at six and there was a two-minute warning bell for parents to say goodbye before visiting ended.

Weeks later I was at home, overweight, and moonfaced as a result of the cortisone treatment that had been administered, friends needing to look twice before recognising me.

Some doctor had told my parents that I should not participate in sport because of the danger of a recurrence of the problem. Fortunately our family doctor had the view that I should do whatever I chose to do and enjoy my childhood.

In March the following year ITP returned and led to a hospital stay of five days before my blood returned to normal. Two days later I was running in the school cross country race for my age group.

In the following years I ran middle distance track races, marathons, then some ultra marathons (55-57 miles), instructed at skiing, and canoeing, and dabbled at sailing, windsurfing, and waterskiing.

Our next contact was in December 1978. Resting after a mixed doubles badminton match my partner was horrified to see petechiae appear on my thighs and arms. The following morning when I visited my GP there were blood blisters on my lips and tongue, blood in mouth and throat, and bruising on back and shoulders from turning in bed in the night. Off to hospital to be told there was a haematology appointment in two weeks. Warned by my doctor that I would need to be assertive (that I should threaten to yell and scream in reception creating a scene, and mustn’t leave  until on the list) I demanded attendance at the clinic. Blood tests showed all was normal. A mistake in reporting and obviously wrong and soon corrected. The consultant took a sample from the sternum to check platelet production. Platelets were in single figures and even lower two days later. There seemed little likelihood that there would be a quick response to prednisolone and that one of my colleagues would have to lead the ski trip to the Dolomites the following week. A week after the first symptoms and the day before my flight, I had my third consultation with Dr. Brown after blood tests. “Do you believe in miracles?” he asked. I nodded.  He told me the lab reported the platelet count was normal, but given the previous mistake he would take another sample for confirmation. All was fine. “Don’t forget your medication and steroid card. It doesn’t matter too much now if you break your leg. Enjoy the skiing” were his parting words. Less than 24 hours later I was on the slopes.

The Summer of 1982 it was back again. This time, like the last, I was feeling well when symptoms appeared. It took longer for prednisolone to have any effect but by October I was training for the London Marathon. As I trained with friends, I seemed to get weaker, not stronger even though platelets were normal and medication stopped after three months. Something seemed amiss but tests a month before the race couldn’t pin it down. The weakness continued. The night before the race I had what my friends referred to as “Dave’s common sense attack” when I said I wouldn’t be running. I then found out that they had hidden my running kit to prevent me being able to compete.

Free of ITP for a while I carried on as normal with work and family life, and competing in a few two day mountain marathons where competitors had to be self sufficient carrying tent, sleeping bag, spare clothing, stove and food, and had to navigate, visiting checkpoints on a route in the mountains.

Then it came back in 1989. Feeling good after a holiday in Brunei, Sarawak, and Thailand I was driving to college in late September for my first lecture of the academic year when symptoms appeared. My reaction—“Oh (expletive deleted)” I said, and arranged for someone to take my place. I didn’t get back to work until the following July after students had left for the summer vacation. In the intervening period, steroids hadn’t been effective. Dosage of prednisolone of 40mg was raised to 60mg then for one week to 80mg before a staged reduction. I rapidly gained weight, trouser seams we’re close to bursting, and I was jokingly referred to as the Michelin Man. Unable to run because of the impact and potential bleeding I was swimming three times each week. The pronounced fatigue resulting from the steroids led to a progressive reduction from doing twenty lengths to achieving only four with a rest between each. Just swimming caused further bruising. It seemed likely that ITP would return yet again and steroid treatment had been less effective than previously. When platelets improved I had a splenectomy and was left with a wound that took weeks to heal and two more operations followed to deal with internal infections. Hopefully the splenectomy would work. I did take penicillin for two years but no more. After a series of eye infections I now have two weeks supply of amoxicillin to start if I feel it necessary and before seeing my doctor.

For a trip to Bangladesh and Nepal in 1996, I familiarised myself with the advice on travelling after splenectomy which I had read in a BMJ published a few months before, ensuring I had anti-malarials for visiting the coastal areas even though the friends I was visiting wouldn’t be taking them. My limited immunity made me cautious.  I had had Pneumovax prior to splenectomy, and again before this trip, even though the BMJ indicated that in some instances it could trigger ITP.

All was well until 2005. Two weeks after a further dose of Pneumovax, one Saturday morning while taking our son to the climbing centre, I noticed the return of my nemesis. Off to hospital. Platelets in single figures led to immediate admission to P3, the haematology ward. That night my partner discovered the ITP Association, emailed seeking advice, and when visiting next day produced a reply from Drew Provan, and a copy of the haematology guidelines on ITP, enabling me to be better informed than most patients. I was home after a week. Prednisolone had worked.

There followed a few years of regular relapses —three in 2006, in one causing some panic among members of a meeting when they saw me bruising and starting to wipe away the blood issuing from my mouth. Spells on P3 were like an annual holiday, with good views of the city from P floor, and in the familiar company of staff who were like friends. When I wasn’t bed bound I exercised every day, walking laps around the lift landings between the wards and walking down six floors and then back up to my bed for a short rest. Twice a day was maintaining a little muscle tone.

Then in 2009, with severe pain in my right calf I visited The Walk in Centre. Blood tests seemed to show DVT but the medics there were puzzled by the low platelet count, thinking the two were incompatible. Later I found that isn’t so but don’t fully understand how they coexist, and how low platelets can make DVTs more likely. So began a two-month spell back on P3 with bilateral DVT and bilateral pulmonary embolism. Anticoagulants couldn’t be used with virtually no platelets and it was proving difficult to raise the count. It was incredibly painful leaving the bed for the short time needed to change the bedding or visit the loo. I had the bed laid flat and then angled with feet towards the ceiling— not quite like a bat but a way to ease the pain. Two bags of platelets were necessary to enable the insertion of an IVC filter to protect from errant blood clots. It was calculated that my immune system wouldn’t kill them before the procedure was completed. Platelets increased and I returned home. Recovery took a long time. A neighbour had a large plastic barrel at the side of the lane which was my target for my first walks of a few yards. Day by day he moved it further along presenting a new challenge then further still as I got stronger. I had a burning sensation in the soles of my feet, and my legs which for years had always tried to follow my instructions became rebellious, often refusing to do what I asked of them. I grudgingly accepted I am no longer young and perhaps too demanding.

Prednisolone, IVIG, tranexamic acid, cyclosporin, and rituximab, have been tried but still ITP lurked in the background and worse was to come. I had celebrated my sixtieth birthday, only nine years late, by walking up Helvellyn, my favourite Lake District mountain, when platelets dropped. Back to P3, where there was amusement amongst nursing staff when tests came back once showing platelets-zero. Surely some were hiding somewhere. One day trying to write a dedication to a friend in a book as thanks for joining me in the mountains I found that I couldn’t write a word. My partner did the job for me.

I was losing concentration, and left sentences unfinished. By now, after four weeks platelets were near normal but I wasn’t. Obviously there was a problem but no one could find out what was going on. Scans showed no serious abnormality, and other observations and checks provided no clarity. Then six days later I had a fit in the early hours. It took six people to get a canula into this writhing person, one holding the head, one on each leg, one for the arms whilst a nurse sucked blood from a bleeding mouth and a doctor inserted it. I knew nothing about this at the time, but over a few days regained some contact with reality. This episode of viral encephalitis really scrambled my brain. I recall hearing someone mention that I was having hallucinations and felt irritated. Why couldn’t they see the words on the ceiling that collapsed into the corners when I stared at them, the green spots on visitors’ clothes, the tiny cockroaches running in a circle around my partner’s cheek, and the lake with the colony of beavers?

These are still vivid memories years later. Stating name and date of birth is a daily requirement in hospital as medication is dispensed or on performing procedures. It felt odd, and embarrassing, not to be able to do so, feeling I knew but couldn’t tell. And naming the Prime Minister was way beyond me.

After one week I requested the use of an exercise bike I had seen in a corridor.  With a physio’s approval it was placed at the foot of my bed, and several times a day I cycled for one minute, increasing to two minutes on the third day, before returning home to convalesce.

Recovery was slow and months went by before I could concentrate enough to read more than ten or twelve pages per day of a novel. Thankfully I didn’t attempt  “War and Peace”. A year later I was driving around and life had returned to normal. Mycophenolate was keeping ITP at bay. An appendectomy and later hip replacement necessitated extra precautions but caused no problems except operation wounds taking several weeks to heal.

Over the year’s prednisolone, Ivig, cyclophosphamide, tranexamic acid, rituximab, and mycophenolate helped maintain good health. Attempts to avoid indefinite impairment of my immune system by increasing platelet production were unsuccessful. When platelets were low romiplostim initially showed signs of being successful, but not for long. When mycophenolate was reduced (originally 1000 mg bd) to 250 mg twice a day, after six months, platelets suddenly dropped dramatically. Eltrombopag caused unpleasant side effects, loss of balance, general fatigue, muscular weakness and dizziness. So back to mycophenolate at 500mg twice daily, along with acyclovir (an antiviral), apixaban to prevent blood clots, and lamotrigine to protect against any further fits.

Over the years the increasing knowledge and treatment of ITP has helped me lead a happy and fulfilling life. I have learnt so much about myself and my illness, and how individual experiences are so varied. I have been fortunate that I have only been affected periodically whereas for others it is a constant in their lives. I also discovered how one committed, dedicated individual, seeking knowledge of a little known disease can  harness the support of family, friends, and professionals uniting them to form and nurture an organisation that has informed so many, supported research, and developed international connections with groups sharing patient experiences. I feel privileged to be a member.

Dave Bagshaw