JB Progress

There is a promising treatment for genetic mutations like mine, called an Antisense Oligo Nucleotide (ASO) . It's a piece of artificial DNA that suppresses the adverse action of a mutated gene.  Two firms in the USA (Ionis and Biogen) have produced an ASO that is designed to tackle the C9orf72 mutation that is the cause of my MND. There are early clinical trials running all over the world, with three centres in the UK: Sheffield, King's College and UCL. With support from the MND Unit at the John Radcliffe, I have been angling to get on one of these trials. I was referred to Kings College, but heard nothing, then recently to the National Hospital for Neurology, part of UCL. Yesterday I received a phone call from the lead investigator for the trial at the National Hospital for Neurology. He would like me to join Cohort 4 in their Phase 1 trial, subject to being assessed against their entry criteria. They are running 6 cohorts with 8 people in eac...

My MND has progressed in subtle ways, causing changes to lifestyle. I found I could no longer get into bed on my own, since as fast as I got one leg on board, the other fell out. More recently, I’ve not been able to get out of bed on my own, either. So Sandie is now helping me at each end of the day, getting in and out of bed and also getting dressed and undressed, as it takes ages to do those things myself, most awkward being to get trousers on. I can still get into and out of my car by myself and drive. 

The pending arrival of my brother Fred and his Canadian wife Sarah from Grand Cayman, where they live, triggered plans for a family weekend to welcome them. We decided it would need to be at my and Sandie's houses, so as to accommodate my needs. Sandie nobly rose to the challenge of doing the catering for about a dozen people. We sourced much of the food from local businesses, such as Green's in Pangbourne and West Berks brewery. Things started with the arrival of my sister Maggie on Thursday 8th August to help with preparations. The plan was breakfasts for all in my house under her supervision, with lunches and dinners at Sandie's. On the Saturday afternoon Jim and Sarah's plane landed at Gatwick from Spain, where they'd spent a week. Most of my five siblings and some of their partners arrived on Saturday and enjoyed dinner at Sandie's. Final arrivals on Sunday completed the assembly. We enjoyed a splendid buffet lunch in Sandie's garden. Here'...

Back home after a fine time at the annual Abbey Dore singing week. See: https://www.englishchoralexperience.co.uk/ Sandie and I stayed at a converted bungalow in nearby Grosmont, which had the necessary equipment. We have attended the annual singing week for about 10 years. Last year I was walking with the support of a rollator. This year by wheelchair. The main issue was getting into the abbey in a wheelchair, as it involved descents by ramps, the first of which was particularly long and steep.I was far from sure that it was feasible, but the conductor, Paul Spicer, took me down backwards with skill. By the end of the week I had 4 able pilots plus willing assistants. Six passages of the ramps were needed each day, 3 up and 3 down. My singing voice has been failing me, mainly in the upper registers, so I was an observer rather than a singer. I attended all the rehearsals and enjoyed them nonetheless. One hears more out in front of the choir as compared to being in the body of...

In order to enable the new shower chair to navigate my small landing and narrow doorways, I've had to remove its push rings and foot plates, making it both narrower and shorter. The consequence is that the only way of moving it about is to proceed backwards, dragging my legs. This is slow and cumbersome. So I contacted Remap, a charity that specialises in making items, or modifying existing items, that can't be bought as standard products.  Remap Berkshire assigned John Morgan, a retired engineer, to design and make a compact foot rest. He first came up with an ingenious idea of using a piece of plastic drainpipe as a removable foot rest. However my floppy legs tended to slip off it. Mark 2 was a wooden equivalent with a small platform on top.  This sparked an idea for a Mark 3, which is photographed below. With this I can proceed forward with feet clear of the ground and quickly manoeuvre over the loo or under the shower, a much more satisfact...

On Thursday 23rd May we did a trip to Lambeth Palace and back, as part of the small St Bartholomew's choir in Lower Basildon. The occasion was the 50th anniversary of the Churches Conservation Trust, who look after and conserve 350 'at risk' churches  https://www.visitchurches.org.uk/ Members of the Trust had visited St Bartholomew's last year when we sang in an evensong there. Apparently we are the only functioning choir in all their churches, which is why we were invited to the 50th anniversary bash. Lambeth Palace is the seat of the Archbishop of Canterbury. He however was not in evidence!   https://www.archbishopofcanterbury.org/lambeth-palace/visit-lambeth-palace The Benbow sisters were the organisers and booked a special coach with a built in wheelchair lift, so that I could come. The coach turned out to be brand new and the lift most efficient. Once in, I was clamped to tracking in the floor. Here is a video of me descending from the coach on...

The arrival of a shower chair has precipitated a change of approach in how I manage transfers. The shower chair is a Freeway T70 Auto, which has 3 functions: to take a shower in, to use the loo, as it slides over it, to use it as a free standing commode. In working out how best to use it, I decided to take a shower. Having stripped off, I attempted to transfer directly to it from my wheelchair. While standing between the two chairs, I dropped my undies, which became entangled in my feet. While trying to untangle them, I lost my balance and ended up on the floor. The mistake was attempting the untangling while standing. I should have sat down first. The last fall was in January, and like then, I was unable to get myself up. My specialist nurse says I shouldn't hesitate to call 999 in such a position. I managed to inch myself along the floor on my back to get to my phone. A call to my neighbours elicited help. She and her teenage daughter were able to haul me on...