The story of Gillian
Sadly, since writing Paul’s story on Limbic encephalitis anti-LGI1 and neuromyotonia (UMN), we are grieving because he passed away in mid-April this year.
I would therefore like to add the following:
This is a plea to all concerned to read the book that is the patient, especially given the absence of follow-through in the dearth of medical literature on the combined effect of two extremely rare diseases – do not look at one disease and its protocols: look at them both, together. At the same time, patients and caregivers should not give up advocating for what they feel is essential!
We consider that the latter deterioration in my husband’s health was especially due to the re-appearance of antibodies associated with his NPLE (Limbic encephalitis anti-LGI1) and renewal of the active stage of disease, as well as the myokymia and tremors related to his neuromyotonia. Increasingly, the Phenytoin levels in his blood dropped and the dose was increased – i.e. I feel it was masking the effects of active disease and making Paul far sleepier than necessary.
The family and I therefore believe that, had Paul received his second treatment of the delayed autumn Rituximab cycle at this stage, it would have improved his level of comfort, as he became very disturbed at night – i.e. it would have been palliative – which is a good reason for treatment, if the patient can tolerate it. It might even have helped retain better muscle control and stability, possibly reducing the number of serious falls Paul experienced – he was still taking short walks unaccompanied, but there were several falls in the home; it was winter weather and he obviously needed to be accompanied out. Until the end of March, we relied on very part-time help in the morning and then in the evening, so that my days were 20 hours, which was an unsustainable situation and put us both at risk. Due to the complex care requirements, I spoke with or interviewed about 20 caregiver candidates over a period of 3 months before finding someone who agreed to help care for Paul.
Paul wanted to be independent but his cognitive and physical deterioration rendered him increasingly dependent on care and supervision. He remained interested in the family and friends, reading, music, nature documentaries, and professional literature as a forensic biologist for another few months. We had been pretty much isolated at home long before COVID-19 lockdown which also impacted on Paul’s mental state, with less interaction than previously and his clarity of speech also deteriorated, due to his condition. He enjoyed communicating with family via video chats where the visual interaction engaged him and he was able to respond better verbally, too.
After a medical consultation at the hospital before lockdown, in March 2020, Paul was being considered for treatment as soon as it would be safe – but tragically it was too late. We were constantly worried by the risk of COVID-19 infection and took every precaution. Paul was losing weight again, became increasingly weak and unstable and had a further fall in the home, made an initial recovery and we remained hopeful. He was being visited by home hospice and para-medical professionals at home and we thought we would survive the pandemic together, somehow, especially once we had a carer who would provide stability of sustained care, especially as he was receiving all his nutritional needs via PEJ.
It was Passover: Paul said how much he enjoyed the festival, although we were in isolation. The family and I created magical, special moments on video chats, especially with our baby grandson, so that whatever happened, we would be able to treasure them. Thoughts like – what will happen next, will Paul regain some strength, were uppermost in our minds, there were better days as well as poorer ones. Sadly, we did not realise how tenuous Paul’s hold on life was: it was an immense shock to us all when he passed away unexpectedly during his morning nap on 16 April 2020. May his memory be blessed.
I dedicate this appendix to Paul’s story in his memory and to help other families advocate for their loved ones.
Â