Nigel’s Story, by his wife Pauline
Nigel, when I first knew him in our late teens, loved country walks, was District Long-jump runner up in the sixth form, tried sailing at his polytechnic; later, he regularly played badminton and croquet and was mad about his mountain-bike; he had boundless energy and scarcely knew a day’s sickness. Having been the driver (and a responsible one) for all social occasions since passing his test at 17, he scarcely drank alcohol. He loved his food, ate a healthy mixed diet, was never overweight and never smoked a cigarette in his life, let alone touching more potent drugs.
It seems doubly cruel and unfair that such a man could be dead at 57 after enduring 15 years of chronic illness, but so it is.
In his early forties, Nigel suffered a series of chest infections, and eventually bronchiectasis was diagnosed. This was ascribed to his having had a severe bout of whooping cough in childhood. No longer able to cope with intense exercise, Nigel exchanged badminton for golf, mountain biking for trail-bike riding, and adapted to life with chronic lung disease. A few years later, he was dogged by gallstones. After having his gall-bladder removed, he had three months of really good health; he was on top of the bronchiectasis and felt much better in himself.
This was not, alas, to last.
That summer – 2006 – Nigel had constant problems with a sore mouth and, eventually, a sore throat, which was diagnosed as persistent oral thrush. He also had a small lesion on his right cheek which refused to heal. Gradually, he gained more lesions on his face, especially round his mouth and nose; his airways became even more congested than usual; his hearing worsened. Over the next couple of years he was sent from specialist to specialist – dermatologists, ENT, immunologists, even an Aids clinic – eventually ending up back at the lung clinic, no further on, and then doing the rounds of all the others again. They did countless ever-more-complex blood tests; they took biopsies; no-one could diagnose what exactly was wrong. Unusual tropical diseases were investigated, despite the fact that Wales was the nearest he’d got to foreign travel since our early twenties! He was treated with a variety of medicines, some of which seemed to ease symptoms temporarily, but none of which worked long-term. He developed encrusted lesions on his limbs. Bone in his face deteriorated; he lost the two upper front teeth and developed a hole in his palate. He also developed nasal septum perforation. His voice became hoarse from damage to his vocal cords. He lost weight and could not put it back on, even with food supplements. Unsurprisingly, he had less and less energy as time went on.
Wegener’s (which we had never heard of before) was first mentioned to us as a possibility in the late summer of 2009. The problem was, Nigel’s symptoms (particularly the nature of the skin lesions), although they ticked many boxes, did not altogether seem like Wegener’s and at some point the tests had included an ANCA test, which had come up negative. The alternative “favourite” was some kind of fungal infection, but they could not identify one. Eventually – with the risks clearly spelled out to us that if he was treated for Wegener’s with immuno-suppressants, then it turned out to be an infection after all, the treatment would allow the infection to become even more rampant, and probably would kill him – we went ahead in November 2009 with Cyclophosphamide pulse therapy which, at first, had really good results; Nigel’s appetite returned, and some of his energy; he was able to enjoy short country walks; some of his skin lesions healed; he put on weight. We hoped that doctors had found the answer, that the Wegener’s could be contained, its worst symptoms eradicated and we could resume something like a normal life.
Unfortunately, though, during 2010, treatment for Wegener’s was severely curtailed by the infections he developed and Nigel’s symptoms worsened again. At this point, he took long-term sick leave from his work as a Computer Network Manager in a school. He was never well enough to go back, and the following year retired early, on the grounds of ill health. By late autumn, his condition had deteriorated still further and he was finding it very hard to swallow or to speak. That December, a PEG tube was inserted and by the end of January, he was having all liquids and nutrition through this, as any attempt to swallow resulted in him ingesting some of the food or drink into his lungs.
2011 was marked by Nigel’s self-referral to a London specialist, Valerie Lund, whose paper about Wegener’s he had read on the internet. She referred us on to Professor Pusey at the Hammersmith hospital. It was Professor Pusey who recommended treatment with Rituximab and the dermatologists at the Hammersmith who finally confirmed that the skin biopsy they took showed results consistent with Wegener’s. However, our Midlands doctors were understandably reluctant to begin treatment which would lay him still further open to infection. As an interim measure, he was put on a low dose of mycophenolate (MMF) and also, regular infusions of immuno-globulins were started, though there was a delay in this whilst funding was sought. When the rheumatologist up here agreed to rituximab treatment, funding was again a delaying issue, and when Nigel finally got rituximab in late summer, sadly proved of little benefit to him.
In November 2011 Nigel was rushed into hospital with pseudomonas infection, and then released early in December, but he seemed very low and poorly throughout Christmas and was readmitted to hospital on January 12th with raging infection in his bloodstream. At this point, we were told his chances were less than 50-50. He did get through the infection and picked up considerably, but on February 14th his condition suddenly underwent massive deterioration; he had suffered a pulmonary haemorrhage and died that afternoon. My son and I made it to his bedside just in time.
All the many doctors we saw agreed Nigel’s case was unusual, maybe even unique, and was complicated by him having more than one serious condition. This undoubtedly was a major factor in the delay in diagnosis. Fortunately most Wegener’s sufferers will enjoy a better outcome than Nigel. However, further research and publicity are both vital to early diagnosis and effective treatment; even more importantly, we hope that research will pinpoint what causes it and how it may be cured and even prevented. All who knew Nigel and the remarkable courage he showed in the face of terrible adversity wish to support the Wegener’s Trust in any way we can. Nigel himself wished that in studying his illness, doctors would learn more and disseminate that information so that his legacy would give hope to other sufferers.