green tick

Dr Viv

Lynne has, for many years, been a well loved but complex patient. She first attended my practice in the role of carer for her aging parents. She found this increasingly onerous, as her mother’s dementia rapidly escalated. Her mother had Huntington’s Disease with late onset, and Lynne was gene positive, as were all her adult offspring. I often wondered, and marvelled, at Lynne’s equanimity in the face of this horrifying prospect, and sometimes suspected a degree of frontal lobe “disinhibition” causing Lynne’s lack of concern. How wrong I was! Once Lynne’s mother died, she focussed on her increasingly difficult and querulous father. Jack became quite ill with a perforated diverticular abscess, and required a colostomy.

Care of the stoma seemed too difficult for the old fellow and Lynne met the challenge bravely. She had a lot of her own health challenges with which to contend, and she was often teary and depressed. Services in a rural area being what they are, she had very little help, and even less respite. She reported some increasingly bizarre behaviour by Jack, but to me he always appeared cogent and plausible.One day he began hallucinating and he was admitted to hospital. Ultimately a diagnosis of Lewy Body dementia was made, and slowly arrangements were made to have him taken into care.

Lynne, without any support from her siblings, made all the arrangements and visited him daily. She also took on full care of the family farm and horses. Then she met Alf. After twenty celibate, lonely years, Lynne fell in love. I thought that finally good fortune was smiling down at her. Admittedly, she was becoming somewhat forgetful, necessitating a Webster Pack, and phoned reminders before each appointment. It was difficult to gauge how much of this emanated from the stress of her declining father, and how much was her Huntington’s.

A month ago Lynne appeared in my waiting room. To my horror she was completely ataxic, unable to walk without veering to one side. She had increased peripheral reflexes, hard cerebellar signs and decreased strength in her upper and lower limbs. She was admitted to hospital, where an MRI showed no space occupying lesion or CVA. She was transferred by air ambulance to the Huntington’s Unit in the city, although she kept insisting that “this is not Huntington’s!” Unfortunately the hospital staff were less than convinced, not having witnessed her lack of neurological pathology previously. She was returned home while I was overseas, and on my return she was stoically bearing up, despite her severe ataxia, with Alf at her side. I arranged an urgent neurological consultation, suspecting Guillain Barre Syndrome. This was confirmed by the visiting specialist. Unfortunately there had been a delay of four weeks in making this diagnosis. Arranging Intragam infusions in a rural hospital took some doing, and a lot of negotiation between Emergency, Day Surgery and the Blood Bank, but it was finally achieved.

Lynne’s lot in life has never been easy. She is still somewhat ataxic, forgetful, and emotionally labile. But she is in love, and I have never met a braver person. Lynne thinks she is the luckiest person alive!

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