Week 356 ” Another sad time for my Family”

Time : Tuesday 9th September 2014

I was asleep in my caravan at Fraser Lodge Caravan park , Hervey bay Queensland when I received the message from Keith that my sister had passed away at 3 am . My first thought I would pack and head home – but 5 minutes later it was obvious I had to fly home. Chris arranged my flight and drove me home from Melbourne to Wangaratta arriving about 9pm.

ROOKS (nee Keenan), Patricia May

ROOKS (nee Keenan). Patricia May Passed away unexpectedly at her Oxley Flats (Wangaratta) home on Sept. 9, 2014 Aged 75 years Much loved wife of Keith for 52 years. Loving mother and mother-in- law of Kym and James Shields (Coomera, Qld).Adoring grandmother of Erin, Euan, and Hamish. The one we loved and will never forget

 

ROOKS Patricia (nee Keenan) 27.09.1938 – 09.09.2014

Suddenly taken from us at her home in Oxley Flats (Wangaratta) with Keith by her side. Sister of Mac & Keiran, Sister-in-law to Jill (dec.) & Louise Aunt to Michael & Amanda, Allison & Chris, Chris & Nicole, Maree & John, Jennie & Ben Great Aunt to Riley & Lucas.                                                                                The favourite Sister, Aunty and Great Aunty of us all.                                                                                                                                     You are now with Mum, Dad & Jill watching over us.

 

ROOKS (nee Keenan), Patricia May

ROOKS. A Funeral Service for Mrs Patricia May Rooks will be held in Holy Trinity Anglican Cathedral, cnr Ovens and Docker Sts, Wangaratta on WEDNESDAY (Sept. 17, 2014) commencing at 1.30 p. m. Private Cremation will take place at a later time.In lieu of flowers, a donation made to Camp Quality would be appreciated. Donation envelopes will be available at the Service.

 

Pat's Camp Quality Family 2011

Pat’s Camp Quality Family 2011

Chris , Lucas & Pat at Eildon 2014

Chris , Lucas & Pat at Eildon 2014

Pat Rooks 50 Years Teaching

Pat Rooks 50 Years Teaching

Pat - Fifty Years of teaching

Pat – Fifty Years of teaching

 

 

 

 

 

 

 

 

 

 

I said I would like to read the Eulogy – I have written it several times and others have read it and between us I am ready. I am not certain whether I can keep my emotions intact.

This was the last thing on my mind as I was sitting on the beach at Hervey Bay. I knew that each night Pat & I would exchange text messages about the day’s events, she was always keen to know what Riley and Lucas were doing. She even bought them each a backpack full of dinosaurs to keep them amused on their trip north.

Pat was born on the family Farm “ The Pines” Majorca to the greatest parents Bill & Gwen in 1938. Our parents had struggled through the depression and the decision was made to sell the farm and take a different direction. With the financial help of Gwen’s parents they purchased the general store in Carisbrook.

Those early days are only what Pat remembered and she often told me about the time she took a baby pig into her grandmother’s bed. Knowing our grand mother in later life I am sure that did not go down to well

Pat was 5 when I was born and I think she thought I was another doll and she could play mother. That’s probably why at the age of 3, I destroyed all her dolls.

With the advent of social media – Pat embraced it and when Lost Carisbrook came she was alive with memories

How she turned the electric fence on when dad went to climb over

How she wouldn’t eat but liked fresh milk straight from the cows teat

The rides in the horse and dray out to the rabbit drives with an old uncle who lived with us

How Dad & Unc took the old horse out into the bush to put him down – they came back several times as they could not do it .

Going to the local rodeo and dad riding a steer through the fence and breaking his arm

She even remembered an air raid shelter being built in the school grounds it’s location and playing in it

Pat went to the Carisbrook Primary school, then to Maryborough High school when our parents sold the shop and moved to Maryborough, Disaster struck and our family home was burnt down and we lived in a sleep out at our Grandma McDonnell’s.

Dad applied for a job managing a store in Dunkeld Pat went to Hamilton High. Pat always-made friends and she kept them for life.

Some memories from Dunkeld include a raw sausage fight in the kitchen, which we were forced to clean up

Pat and her friend Joy entering an entertaining skit in “Star town” a promotional competition organized by our father Bill.

Pat and her friends were all members of a young farmers club

The next was a surprise to Pat and I as suddenly we had a brother. Keiran arrived and Pat at 15 became the devoted sister and mother allover again.

Pat completed her secondary schooling and then the new and never ending part of her life began TEACHING.

She did her training in Geelong Teachers College. Again making lifelong friends.

The family moved to Wangaratta where our father managed D & W Chandlers, which was later to become Keenan’s Hardware Corner.

Pat’s first teaching position was at Walwa on the Murray River. The first time dad drove her there the Ovens was in flood and out to North Wang was almost impassable. We were towed behind a tractor and the car certainly floated most of the way.

Pat went on to teach 3 generations of children during almost 60 years, at many of the local schools including Moyhu, Chisholm St and of course for many years at Yarrunga her second home, then finally Glenrowan.

Pat and Keith met and amongst many things they shared an interest in farming.

They married here in 1960 and 9 years later Kym arrived.

Any holiday trips always included a friend for Kym (usually Wendy or Zoe). They went to Point Lonsdale & Hervey Bay for a number of years to join our family and mum and dad for a fabulous time.

When Kym was 9 she joined Pony Club. Pat and Keith became deeply involved with the organization .Pat became a life member, serving as district commissioner for at least 5 years, , Kym learnt to back the horse float very early because Pat couldn’t do it.

Pat’s sausage rolls & pavlovas are now a part of pony club folklore

The only time she rode a horse was on a trip up north.

Pat & Keith built their first home at 10 Turner Street shortly after their marriage. Their interest in farming led to purchasing, a block of land at Oxley Flats. Located on the bank of the Ovens River, which they called Picaninnie The block was not only challenged by floods, but snakes, blackberries and a large area of lagoons. Rescuing stranded stock from floods, feeding calves every day hell or high water, bonfires to burn the flood debris were highlights there.

They upgraded to the present farm a few years later, which included an old derelict house and outbuildings. Keiran and Louise renovated and lived in the house for 18 months, and when they left Keith’s parents moved in and stayed many years.

The old house was demolished, the present house built, Turner Street sold and Kunawarra was born.

Pat and Keith embraced the Oxley Flats community participating in everything and the CFA became a big part of their lives

Kym and James wedding was another triumph to Pat’s organizing skills with them married in this very church, transported to the family farm at Oxley Flats in a horse drawn buggy. Even the weather obeyed – with a sunny day and the full lagoon beside the marquee looked a picture.

Pat was devoted to her beautiful grand children– Erin, Euan & Hamish. and was always deeply involved in their lives.

When asked their favourite thing about their Grandma,

Erin’s was ‘I liked that she understood what it was like having 2 brothers’.

Euan’s was making cupcakes with grandma.

Hamish loved the collectible stickers she sent to him and always waited impatiently for the post to arrive.

Pat and Keith celebrated their 50th in 2010 and many of you here today will remember that day

Over the years, our lives have taken different courses but we remained a close family celebrating birthdays, Christmases and family events. Pat always was the instigator of keeping these traditions going. We even celebrated the 100th birthday of our parents several years after they had passed

During the past 10 years Pat & I have become closer if that was possible. When Jill was diagnosed with MSA Pat stood beside us through thick and thin and we began having Saturday lunch together. Pat would tell Jill all the things that were happening at Yarrunga where they had both shared so much time. When Jill passed away Pat insisted we continue having lunch each Saturday

Keith, Kym & James will keep Pat’s Facebook page open as a Memorial to a wonderful wife, mother, mother in-law, grandmother, sister, aunty, grand aunty and a wonderful friend and teacher.

Pat may have been small in stature but hugely determined (in other words bloody stubborn), fiercely protective of the children she taught and devoted to her family and friends.   Pat believed that her grandchildren were her future and will live on for her

Losing Pat has shocked and saddened us all

We will always remember our loving, hardworking, generous and thoughtful PAT

 

 

 

 

 

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Week 355 ” North to Hervey Bay”

The planing has gone very well the craven is packed – Jeep all fuel up and tomorrow we head off – alone but nit alone – I always wish I had Jill by my side to keep giving me directions so I don’t get lost.

Pat & Keith arrived with a couple of extra things to pack – Pat as always thinking of the boys Riley & Lucas had visited Aldi on the way and picked out a present fro each of them. She had a plastic backpack full of dinosaurs  which the boys will love. Keith made me a new wrench which will fit my electric drill – to make winding the caravan stabiliser jacks much easier and quicker. Pat texted me just before we left – like mum drive safely.

Chris , Nicole , Riley and Lucas were on their way and were camping just out side Wangaratta at a friends farm – no room for two vans in my driveway. A phone call to coordinate our meeting just north of Wangaratta and we are off.

We headed north along the Hume Freeway to Albury and the Wagga Wagga a couple of stops along the way so that the boys had a play and our first overnight stop was at Forbes . This was the first of several free camps we intended to try. The location was beside a lake and there were already 10 vans parked. We found a great site and were able to be side by side. The boys had slept during the drive and when we were set up ready for a play – early to bed as another long day ahead.

Second day was similar to the first with our destination Narrabri – we had planed to stay at show grounds but changed our mind and opted  for  the caravan park – but a bit sorry very crowded and no room for our boys to play, We decided to move but the manager there offered an area used for tents which was completely empty and since we didn’t need water or power it was ideal – the boys loved the area which gave them room to stretch their legs and play with Grandpa. That evening we walked to the RSL club for a meal . Chris & Nicole had been there before I is called “The Outback Shack” decorated in theme with the name – a nice meal and back to the vans for another early night.

Third we were on our way with our scheduled stop at Lake Broadwater east of Dalby in Queensland. We crossed the border at Goondiwindi and his was our lunch break . The boys had amazed me – playing in the car, sleeping and ready to play with Grandpa when we stopped. We turned off the maid road for the 10 k trip into Lake Broadwater – having no idea what was there other than some excellent reports on WikiCamps App. We pass only one caravan coming towards us and our comment over the radio was why would they be leaving this late in the afternoon. However we continued on and eventually we could see the lake and the main camp area – about 5 van and a huge area to select from . Always difficult when you have such choices. There were two large amenities  blocks  and a play ground which was the first thing the boys saw as well as barbecues along the waters edge. We set up and had a look around and several more vans arrive and disappeared into various areas of the park. We decided to have  a barbecue and as they were wood fire dthe boys were given the task of collecting wood which they enjoyed and I got to use a new toy – a gas fired fire lighter – it worked  a treat. While waiting the boys were surprised with arrival of a small kangaroo and Lucas learnt a new word “ROO” and for the next hour all he wanted to see was another “ROO” Again an early night.

Forth day was meant to be a bit easier and to reach Fraser Lodge Caravan Park  in Hervey Bay mid afternoon. The roads were a bit narrower and there were road trains and a fair bit of other traffic but we still made good time – stopped for lunch and crossed over a scary bridge – single lane, no sides and about a 50  ft drop to he Mary River below. Chris went across first and Nicole radio me the traffic as the disappeared from sight – No traffic and I was across.We arrived in Hervey Bay mid afternoon and were quick set up as Riley had seen the Jumping Pillow as we came through the gate.

The next nine  days saw us at the jumping pillow , making sand castles , feeding ducks, going down to the beech, fishing , going for walks – I don’t think the boys wanted to see the inside of the car for a while and all Lucas wanted to do was walk – not to interested in the pusher. I had a little turn and a scare finished up at the doctor and some medication and I was back to normal. We had great time and finally it was time for the family to head home.packed we waved and they were gone and I felt lonely. Chris text me and rang as they proceeded south to Doreen. I was very pleased when I got the call they were home.

During the next  week I revisited all the places Jill and I had purchased a coffee. I sat at the tables and remembered all those good times and enjoyed the coffee and of course a cake – not good for my sugar levels or my waist line. Each evening I received text messages from  Nicole or Chris, Allison and off course my sister Pat who always wanted to know what I had done that day. Her texts were always about family and usually sent later in the evening.

My next decision was when to come home and I went and booked a day by day situation as my site was actually free until the 19th of September. I made friends with a couple of other caravaners and the one opposite – their family also lived in Doreen quite close to Chris. I had spoken to Lorna & Peter – my great neighbours who check on my house ,mail and of course feed Jessie ( 20+ year old cat) I should not have said just feed – spoil is a better description – hand feed fed with prawns.

I would love to be here with Jill and my memories but that’s not the way. Jill’s sister is again  at Agnes waters and I would have liked to head there but I was too late and there are no sites available so home I must head

Forbes Free camp site

Forbes Free camp site

PANDA

PANDA

Lucas playing near van

Lucas playing near van

Out Back Shack at Narabri

Out Back Shack at Narabri

parked for Lunch at Goondiwindi

parked for Lunch at Goondiwindi

Caravans at Lake Broadwater

Caravans at Lake Broadwater

Dinner is ready - Lake Broad water

Dinner is ready – Lake Broad water

ROO ROO

Lucas sees the kangaroo

Lake Broad water

Lake Broad water

Going out for breakfast

Going out for breakfast

Lucas and Chris at the beach Hervey Bay

Lucas and Chris at the beach Hervey Bay

Nic and Riley building castles

Nic and Riley building castles

Riley & Dad on jumping pillow

Riley & Dad on jumping pillow

Lucas and his ice-cream

Lucas and his ice-cream

Playing in the train

Playing in the train

Riley's first Fish

Riley’s first Fish

 I caught this one

I caught this one

Lucas on jumping pillow

Lucas on jumping pillow

IMG_4925

Feeding the ducks

Feeding the ducks

Coffee with Jill

Coffee with Jill

See you soon on the net

 

 

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Week 353 “DINOSAUR ADVENTURES & MY BOYS”

It’s now a couple of months since I updated the blog – as I said earlier it’s lack of motive. I have had several trips to baby sit Riley & Lucas and am always amazed at the speed they are growing up. Even though we face time each night to see them in the flesh is different. For a while Lucas would look behind the iPad looking for Grandpa. Every visit to the boys seems to end in me bringing home the flu and taking a couple of weeks to get rid of it or a visit to the doctor. As I am writing this – yes I have a cold but have to fight through it as I need to get the caravan ready for a trip to Hervey Bay.

Back in mid July we took the boys to a dinosaur exhibition at Caulfield Racecourse advertised as  “They’re alive – DINOSAUR ADVENTURES , Feel the DINO-ROAR” The boys had a great time as did their parents and Grandpa. The following day Chris and I took Riley to his first football match – We watched Essendon play the Bulldogs at Etihad Stadium with the roof closed it wasn’t too cold. Riley was excited to see so many people and shouted when ever they became excited. At the end of the game the crowd were singing the Bombers song and of course Riley joined in much to the delite of some spectators in the row behind and they asked him how old he was. He answered I’m 4.

Riley & Dad

Riley & Dad

Riley & Dad on train to Football

Riley & Day on train to Football

Riley

Riley

 

 

 

 

 

Riley and a dino

Riley and a dino

Dino's Roar

Dino’s Roar

Lucas looking at dino's

Lucas looking at dino’s

 

 

 

 

 

In the late 80’s a local musician, my neighbour ,Peter Reynolds, decided that Wangaratta was in need of a “Community Radio Station”. In conjunction with Wangaratta’s 150th celebrations of 1988, ‘The Chronicle’ newspaper launched a competition for a poem/verse to commemorate the occasion. Everton resident Mrs. Margaret Glendenning penned the winning entry called ‘The Song of Wangaratta’. Peter Reynolds provided the tune and a cassette of the complete song was produced at Witchetty Grub Studios.?The cassette was forwarded to a commercial radio station and it was hoped that the song would be played during the special week of celebrations. Unfortunately, it was decided that the song did not fit into the station’s music format and was played only once, in a very early timeslot. Understandably disappointed by the outcome, Peter had the brainwave of establishing a “Community Radio Station” where incidents such as this would not occur. A public or “Community Radio Station” would accommodate this type of music, that of the community. The radio is now known as OAK FM Community Radio Wangaratta 101.3. Peter and I were discussing it and with some help we were able to put a video of the song on Lost Wangaratta – Face book page . The video below is not the one Peter used but the file was to large to place here so this is the song with a series of photos included.

https://www.youtube.com/watch?v=3xkhbB0siNI

August saw another trip to look after Lucas and the current cold. On the 9th August Pat , Keith and I went to Wirramina Environment Centre at Burrumbuttock where Keiran helped organise a Farm Art Exhibition . we had great day and saw some magnificent exhibitions. With the success I am sure Keiran will organise one for next year. Last weekend we went to Wodonga caravan show – just looking. Went to Melbourne to baby sit the boys on Saturday night then home to prepare for our trip away

Battle Horse

Battle Horse

Bee Motel 1st prize

Bee Motel 1st prize

Fart Art

Fart Art

 

 

 

 

 

Chris, Nic, Riley & Lucas's new Van

Chris, Nic, Riley & Lucas’s new Van

Outback Expanda

Outback Expanda

I am about to finish cooking and packing the van as I am heading to North with the boys in their new van for a few weeks.

 

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Week 446 “MSA and the Neapolitan Ice Cream Analogy by Pam Bower”

 MSA and the Neapolitan Ice Cream Analogy
by Pam Bower

Think of MSA as the 3 flavoured ice cream called Neapolitan which has
vanilla, chocolate and strawberry all mixed together. Imagine if you
took one scoop of that ice cream and put it in a dish, then took
another scoop and put it in another dish and compared the two dishes.
You would notice that there is not the exact same amount of vanilla,
chocolate and strawberry in both of the dishes.

Multiple System Atrophy is the same as the Neapolitan ice cream.
There are three flavours included in MSA:

1. Shy-Drager Syndrome (SDS) – Think of it as the strawberry ice cream
2. Olivopontocerebellar atrophy (OPCA) – Think of it as the vanilla
ice cream
3. Striatonigral Degeneration (SND) – Think of it as the chocolate
ice cream

Whether someone is told they have SDS or OPCA or SND they all have
one scoop of Neapolitan ice cream in their dish. They all have
Multiple System Atrophy.

If their symptoms are mainly orthostatic hypotension or urinary
incontinence they have mostly strawberry ice cream in their dish
(SDS). If their symptoms are mainly cerebellar ataxia they have
mostly vanilla ice cream in their dish (OPCA). If their symptoms are
mainly tremors they have mostly chocolate ice cream in their dish
(SND). If they have all of the above symptoms then they might have
nearly equal amounts of chocolate, strawberry and vanilla in their
dish.

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Week 445 “Our past few weeks”

Jill and her flowers

Jill and her flowers 

I am getting slower at putting up blogs – I haven’t got Jill to give me inspiration. I went to the cemetery to place some flowers yesterday and as usual I took a photo – the rose bush above had been pruned and looked bare but strong and it reminded me of Jill  – often emotionally bare but for ever brave and strong.

The past month I have not ventured out – been suffering the inevitable winter flu – I blamed Riley and Lucas but I guess the difference is over the past few years I never visited anyone that was sick for fear of passing it on to Jill . I guess my immunity to the various things is pretty low consequently  it has taken a while to shake it off. I missed a chance to get the caravan out and share some time with my neighbours at Gentle Annie Caravan Park – it also rained that weekend – we are planing to try it in couple of weeks but the weather may be the problem – rain expected and Gentle Annie is on the King River which is currently very high.

Riley's Batman Birthday Cake

Riley’s Batman Birthday Cake

Riley's Birthday Party at Kid's Corner

Riley’s Birthday Party at Kid’s Corner

Prior to the flu I had been looking after Riley & Lucas when ever they were not well . We had Riley’s 4th Birthday and with all his friends and relatives, we celebrated  in style at a place called “Kids Corner” in Doreen.  Can you imagine 15-20 4 year olds – the noise , activity and finally the cake.

I have been cleaning up Jill’s Gym – got to get back and do some exercise. I had a glucose tolerance  test back in December and was diagnosed with Class 2 Diabetes. Must exercise and watch my diet. I think over the past year cooking for 1 , I have not been doing it well. Jill always worried about diet and she made sure what we ate was correct. I enjoyed cooking, even though I had some one checking every ingredient that went in. So now I am checking my blood sugar levels twice a day , but can never understand the variations I get. I go back to the Diabetic Clinic in a couple of weeks so guess it will be all explained.

The look is priceless

The look is priceless

This month the arrival of Samuel – Jill’s sisters grandchild added some more excitement to the family – Jill always loved these occasions. Riley and Lucas when to visit Sam and the look on Lucas’s face tells it all – “I am no longer the smallest”

Jessie and me

Jessie and me

My constant companion is Jessie ( 20+ year old Cat) who has been mentioned through out this blog. She  is also showing her age and arthritis is starting to become a problem and is now on a daily dose of anti inflammatory medication . She has put on weight and can now jump onto my lap again. I had been lifting her for sometime. Her eyesight is suffering and she gets lost in the dark. Then her call for help is loud and mournful especial at 4 am in the morning. I think she saying ” Where the hell are you – come and open the door”

Well as I said I must exercise so off to Jillsgym

See you soon on the Net

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Week 444 “2nd UK MSA Research Symposium Feb 27 2014″

MSA Coalition Board Member Pam Bower travelled to London back in February to attend an important MSA research meeting. She shares her notes below:

Notes from the 2nd UK MSA Research Symposium
Queens Square, London UK – Feb 27, 2014
By Pam Bower

Over a dozen MSA researchers spoke throughout the day in this jam packed event.

A few highlights –

It is still a mystery of how the misfolded protein alpha-synuclein gets into the brain cells in MSA. Alpha-synuclein which is a protein, misfolds in MSA and aggregates in clumps inside a type of glial cells which are the helper cells to neurons – the oligodendrocytes. Alpha-synuclein itself originates in neurons. Understanding how it migrates to the helper cells will help unlock the mystery of how the disease originates. Doing major work on understanding the origin (pathogenesis) of the disease is the team in Innsbruck, Austria led by Prof Gregor Wenning.

Prof Wenning summarized all the available animal models of MSA (there are now 4 models) and how work with them is helping to unravel the mystery of the origin of the disease. Each type of mouse model displays particular symptoms and/or brain pathology that mimics human MSA but there is no one perfect model to mimic all aspects of MSA. Prof Wenning discussed the 9 or 10 steps that we now know about that lead to the death of glial cells and neurons in MSA. Much more experimental work is needed to clear this up further. Only by uncovering the underlying cause can we hope to find an effective treatment in future. One exciting recent finding is that when MSA mice are innoculated with alpha-synuclein brain homogenates, the actual disease seems to spread in a prion-like fashion. This is a very important clue to be worked out.

Prof Wenning also went over some recent clinical trial results. Although the drugs Minocycline, Rasagiline and Rifampicin all showed negative results in arresting the disease in humans, they did show some interesting results in mice, this all helps in understanding how the disease progresses. Minocycline in particular was interesting in that it was shown to suppress microglial activation in mouse models of MSA… this means that inflammation is involved in MSA and the drug was shown to improve this. Rasagiline also showed neuronal rescue in mice but at high dose, the dose used in the human trial was very low due to concerns of the drug company about safety. Could a higher dose have been tolerated in humans or a low dose have helped if used much sooner in the disease progression?

Things to watch… MPO inhibitors such as Minocycline, Anti-aggregation compound ANLE 138b now in Phase 1 for Parkinson’s Disease, Alpha-synuclein immunization (drug company: Affiris)
Still in question… whether Mesenchymal stem cell transplants actually work for MSA.. waiting for results of the Mayo clinic stem cell trial currently in progress.

Another exciting line of research happening in the Innsbruck lab is work with grafts in mouse models that help the cells make better use of dopamine. In MSA there is still dopamine produced but the cells lose ability to use it effectively.

~~~

Dr Nadia Magdalinou spoke about Cerebrospinal fluid studies in MSA. The oligomeric version of alpha-synuclein may be the cause of the disease. Measuring total alpha syn in the CSF may be the most promising biomarker to detect the disease much earlier. A combination of imaging and biomarkers is used now to better to diagnose Alzheimer’s Disease so a similar combination may be required to diagnose MSA.

~~~

Prof Henry Houlden reviewed what we know about the genetics of MSA. Candidate genes have been reviewed such as the tau gene, the ApoE gene for Alzheimers and the C9orf72 gene for a common form of ALS. None of these have been found in MSA patients. Genome Wide Association Studies are a powerful tool to identify genetic risk factors for sporadic diseases. These have been done now for many other diseases. Prof John Hardy also discussed this in his talk and mentioned that there is a critical number of cases that must be studied before any useful genetic data can be found. He thinks 2000 cases is the minimum number that will lead to any concrete findings. In Alzheimer’s it took 2000 cases to find 3 genes, 6000 cases to find 6 genes and 30,000 cases to find 25 genes. These 25 genes map to 3 pathways – the ameloid peptide process, cholesterol metabolism, and innate immunity. Parkinson’s disease is similar – 16,000 cases were studied to find 25 genes. These genes control mitophagy and autophagy – internal metabolism of the cell. For MSA only 1000 cases have been studied and there is “no secure finding”. Prof Hardy actually doubts the Japanese gene finding in CoQ2. Prof Houlden also mentioned that the UK team could not replicate the Japanese finding using European MSA patient dna. What they did find though was coQ10 levels in the brains of MSA cases were lower than in controls.

~~~

Prof David Burn spoke about MSA registries, databases and clinical networks. The MSA Trust is providing funding to ensure that the UK clinical centers can form a network to work together more closely. This will be a platform for trials and biomarkers and allow for longitudinal studies. Databases will allow capture of critical information on dna, rna, serum protein and csf measurements. There will also be quality of life questionnaires and active requests for brain tissue donation. Patients will be actively recruited with all stages of the disease and a special focus will be placed on early stage disease. They would like to collaborate with US groups as well. (NOTE: This would be a very good reason to host a US MSA Research symposium in 2015!) Prof Burn spoke of 2014-15 being a time of change for MSA with exciting collaborative opportunities for MSA research.

~~~

Prof Huw Morris spoke about Improving Management of MSA. Currently many people lack a definite diagnosis in the early stages, they may eventually be told PD and given a PD drug to try. It would be better to tell people atypical parkinsonism as a working diagnosis. should provide as much info as needed with early follow up. Give info on the MSA trust and the trust nurses. Provide information on creating an advanced care plan and deciding on whther a peg, trach or ventilator will be part of this plan. Key notes on improvement to management of MSA: 1. people should all have their lying and standing bp measured 2. They should have an MRI 3. they should be given a definitive interim diagnosis.. if there is uncertainty of which type of parkinson plus disorder they have they should at least be told “atypical parkinsonism” and not be left without any sort of diagnosis 4. The diagnostic criteria of MSA of possible or probable should be explained 6. There should be documented assessments from OT, PT, ST etc 7. should be offered the chance to participate in research trials 8. should be offered info on palliative care 9. they should have a written care plan. In the UK 7 centers will join up for longitudinal study and standards of care should come out of this.

~~~

Prof John Hardy discussed MSA Resarch – The way ahead. Where are we and where do we need to go? “For all things collaboration will be essential” For genetics, for stem cell work, for understanding the spread of pathology and for clinical trials. Stem cells offer some prospect of treatment. They can now turn skin cells into neurons or glia in the dish. Can now model the disease much more effectively in the dish. Cells from MSA patients should be made more freely available. The Coriel cell depository is open access. MSA-P and MSA-C skin cells must be deposited there and shared. “MSA will be dragged along in Alzheimer and Parkinsons research”. All are protein deposition disorders. All involve mutations is deposited protein, many have sporadic variants, these diseases all show spread or “Braak hypothesis”. There is evidence of cell to cell transmission, the diseases show different strains ie MSA-P, MSA-C. In mouse models antibody treatment is effective. The disease jumps from neuron to neuron or from neuron to glia. Antibody treatment might work for AD or PD, if so MSA will be tested very quickly thereafter. Five years ago we did not know what we know now – we know more about the spread of the disease, the underlying biochemistry and the genetics. We are now seeing strong parallels with AD and PD and this offers real hope we will eventually move to treatments. How can we be sure MSA does get dragged along? PD is MSA’s big brother, MSA needs good relations with PD organizations. MSA might actually be a better system to use to test PD. MSA needs separate identity and separate charity but needs crossover with PD organizations and PD patients who may actually turn out to have MSA.

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Dr Helen Ling also brought up the long duration variant of MSA in her talk. Dr Tony Lang from Toronto had called to inform them of a patient under his care who had been confirmed on autopsy to have MSA, he had lived 18 years with symptoms. the UK brain bank was checked looking for similar cases and a few were discovered. These were all MSA-P patients who were misdiagnosed as PD in the first 10 years. They had a modest response to levadopa. A concern with these patients is that they may be offered Deep Brain Stimulation which can help PD but has a very poor prognosis in MSA patients and can lead to other complications.

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