Amongst the multiple authors of this paper were the four French neurologists (image below) who, in the late 1980s and early 1990s, had discovered and described this disease that was occurring in a French family and many of their relatives . By 1993, the term CADASIL had been coined by Marie-Germaine Bousser.
Source: brainfacts.org
L-R: Elisabeth Tournier-Lasserve; Hugues Chabriat; Marie-Germaine Bousser; Anne Joutel.
In 1996, Elisabeth Tournier-Lasserve discovered that a Notch3 mutation was responsible for causing CADASIL in this French family.
“As a first step to set up a reliable and feasible diagnostic test, we undertook Notch3 mutations analysis in a group of 50 unrelated patients with CADASIL, searching for potential clustering; we also tested 100 control individuals so that we could characterise Notch3 polymorphisms to be differentiated from pathogenetic mutations.” (Source: “Strong clustering and stereotyped nature of Notch3 mutations in CADASIL patients”)
This analysis of 50 patients discovered 10 CADASIL-causing Notch3 mutations (see Table 1: Notch3 mutations in CADASIL patients in the paper at the top).
From that list in Table 1, I calculated that 55.6% of all the Notch3 mutations discovered in that 1997 study occurred on Exon 4. Of note, exons 3 and 4 combined accounted for 71.1% of all known mutations at the time.
Additionally, most of those mutations involved adverse changes to EGFrs 3 or 4. Mutations in EGFrs 1-6 are known to cause more severe CADASIL than EGFrs 7-34. See paper linked below:
In 2008, an Australian paper (above) was released that listed all known Notch3 mutations, at the time, that cause CADASIL (as sourced from the Human Gene Mutation Data Base, Cardiff) – listing a total of 70 mutations.
This dataset showed that exon 4 accounted for 52.9% 0f mutations and exons 3 and 4 together were 61.4%.
And in 2009, this paper (above) looked at 131 people and listed 178 mutations (you will need to scroll to the bottom after opening the link and click on “Supplementary Data” which will allow you to download the mutations table as a zip file). Data sources are listed below the table.
The 2009 data showed that exon 4 accounted for 31.5% of mutations and exons 3 and 4 together were 42.7%.
I created a table with the 1997, 2008, 2009 and 2014 data further below (underneath the photo of the Sydney Opera House and CBD).
And to 2014
The latest (and most current) complete list of Notch3 mutation variants known to cause CADASIL was published in 2014 (yes, many more have been discovered since and published separately in various papers but there is currently no Notch3 database that keeps an up-to-date list of all known variants. More to come on this further below). See:
More specifically, the list is in Supplementary Table 1 as sent to me by one of the authors, Julie Rutten of Leiden University (in addition to the paper above):
By 2014, there were a total of 206 CADASIL-causing Notch3 mutations spread across 21 exons. There a range of nucleotide changes (e.g., c.421C>T), amino acid changes (e.g., p.Arg141Cys), and affected exons (e.g., 4) and EGFrs (e.g., 3) – which just happens to be my mutation – that account for the 206 mutations.
As a breakdown for 2014, Exon 4 accounted for 31.6% of mutations and, again, EGFrs 3 and 4 featured prominently, along with EGFr 5 on this occasion. This time around, exons 2, 3, 4 and 5 combined accounted for 53% of mutations.
Up until 2014, Leiden University in The Netherlands maintained a comprehensive list of Notch3 mutations on the LOVD3 (Leiden Open Variation Database), being curated by leading CADASIL researchers Saskia Lesnik Oberstein and Julie Rutten.
It has not been updated for some years, with Julie telling me:
Dear Patrick,
This database does not contain a comprehensive overview of all CADASIL-causing NOTCH3 mutations. Updates depend on whether physicians upload their mutations into the database, there is no periodical update unfortunately (due to lack of time and personnel)
In 2014, I have made a list of all CADASIL-causing variants known at that time, see attachments. Of course, new variants have been published since then.
We are currently writing a EU grant proposal which includes setting up a registry of all known CADASIL patients in Europe, which may in the end result in a more comprehensive and up- to- date overview. But this will take several years, and it is still uncertain whether this project will be funded.
Best regards,
Julie
So, if your mutation isn’t to be found in the 1997, 2008, 2009 or 2014 lists, there may be a good chance it can be found in one of many case reports published since (or not).
Notch3 Pathogenic CADASIL Mutations by Exon
* Errors in data are all mine.
P.S.
There are several research papers (readily available via the internet) that have published results of Notch3 CADASIL mutations in specific populations. I have seen such papers for those with CADASIL in Italy, The Netherlands, China, Portugal, Japan, Columbia, Taiwan. And the mix of mutations and prevalence of particular mutations can vary somewhat from those reported above. And I have seen CADASIL case reports from Egypt, Turkey, Saudi Arabia, Serbia, Malaysia and more.
I’ve seen a few papers in the last few months that say there are currently around 280 Notch3 mutations that are known to cause CADASIL but I was unable to find out where they obtained the information. Nor did I save any of those papers to my computer.
Not all Notch3 mutations are pathogenic and the LOVD3 database has a list 0f those mutations.
Finally, I found links to several other gene databanks but they are only accessible on a commercial licence basis or to academics and students of tertiary education facilities.
For my non-CADASIL friends (I’ll explain the CADASIL bit soon), I just want to let you know I have been in Royal North Shore Hospital since Monday evening after having a lacunar stroke.
CADASIL is a form of cerebral small vessel disease that can (and does) cause stroke in the deep part of the brain, without having any of the usual risk factors for “normal” strokes that occur as a result of a clot or a bleed. I’ll include a link to my website below for those who want to dig into the more juicy details about CADASIL.
Mobility has been affected on my left side – leg mobile but too weak to support walking; until late this morning, my left arm was completely immobile (managed to lift it slightly a few times in the last 30 mins); left side of face numb but I can eat and drink.
Here for a week before being transferred some time next week to a live-in rehab facility.
Moving from hospital to the rehab centre was a bit scary at first – it all happened quite quickly on Wednesday afternoon/evening. I was leaving my safe cocoon and the wonderful physio who had started my therapy. Mixed day yesterday – broke down and wept loudly on phone call with Josie, followed by visit from speech therapist, occ therapist, physio assessment, lunch, neuro registrar assessed me (she is wonderful and an absolute cack), a physio gym session (nice young guy who works me hard, including walking in a vest suspended from a guide rail on the ceiling), psychologist chat, then dinner. And Jos was here for much of it.
In between times, I’m listening to music, done some brief meditations, sampling a few different binaural and Solfeggio tones, reading – I’m starting to settle in here.
No program schedule made up for me yet but I imagine a couple more physio sessions on the cards today.
Love and peace to all. ♥
18 June 2021
Rehab – Day 2
Quiet start to the day – woke early, had breakfast, did some reading, listened to a binaural tone for a bit, listened to some music, had a shower (on a seat with my left arm in a sling).
Shortly after, I was nabbed for physio – walked (swaying awkwardly), with my physio hovering close by, the 25 metres from my room to the gym where I did a stepping drill and something else that now slips my mind. Walk back to my room, where lunch awaited me.
A bit of quiet time then more physio. This involved being strapped to a medieval tilting torture table. I was strapped in tightly with my left foot resting against a footrest. The table was then tilted at a 35° angle. “We just need you push your own body weight up and get that knee in a straight position. Off you go. 150 reps and here’s a clicker for you to keep count.” Um……yup, no worries.
Polished off in quick time, I then went on a short trip via wheelchair to my new room. From having my own room, I am now sharing with another bloke. Time for me to readjust again.
Psychiatrist dropped by for a 20 minute chat over dinner. Jos and Sara visited for about three hours and endured my grumpiness.
Spent 30 minutes trawling for interesting podcasts – found some promising ones.
Another day almost done – looking forward to a good sleep tonight.
Love and peace to all. ♥
19 June 2021
Rehab – Day 3
It’s Saturday – rehab shuts down for the weekend. I continue to do my own exercises for my leg and I’ve done a few transitions from bed to a chair and vice versa (this involves taking a few steps from one to the other while a nurse lightly grips my wide rehab belt in case I begin to topple).
My roommate, Kevin – who has been in and out of this place for several months and, hence, knows everybody here – managed to secure a wheelchair for me (even though the nurses thought that there wasn’t a spare one available).
Jos then took me for a wander around the two wards, which have floor to ceiling windows offering views over the surrounding suburb(s) – grabbed a few pictures on my camera phone. Under doctor’s orders (mine), she only stayed for a couple of hours this afternoon – needs her rest and sleep too. Kevin and I had less than optimal sleep last night – he has an air mattress and the pump was regularly through the night. To distract myself, I listened to a couple of podcasts about grief – they were both excellent and enlightening. Eventually drifted off into a short but deep sleep, to be woken just after 7.00am when breakfast was delivered.
Cold, wet and windy outside today but I don’t mind that kind of weather. A good excuse to stay in bed for a while whilst watching a bit of Rage (for my overseas friends, this is a music video show). Even better, it began with one of my favourite songs – Shine by Collective Soul.
Looking forward to tonight’s dinner – a beef rendang.
Love and peace to all. ♥
21 June 2021
Rehab – Day 4
A belated post.
Did bugger all yesterday (overseas friends: means doing nothing in particular). Delighted when a high school and uni mate – Danny – dropped by yesterday (Sunday) morning. Chatted amiably for about three hours. He left me a couple of Australian history books with a comical bent – Girt and True Girt. Have already elicited a couple of chuckles. (overseas friends: yes, girt, NOT grit. As far as I know, Advance Australia Fair, is the only national anthem to use the word “girt”.)
So good to see you Danny.
Then a spot of lunch just as Josie and Sara turned up (bringing along a home-made egg and bacon roll) – delish!
As we started to go for a st(roll) down the hall in the wheelchair, an ex-work friend came walking towards us. She joined the convoy as we made our way to the waiting area outside the hydrotherapy pool. Large windows gave us a good view across the road to suburbia, the wind-blown trees, slowly moving heavy clouds and the unheralded arrival of a former high ranking Federal Liberal politician.
Another amiable chat before st(rolling) back to my room where Christene and I continued to natter whilst Josie and Sara made their way home. Thanks for the visit Christene, who left just before dinner arrived. After dinner, watched a couple of episodes of Wheeler Dealers (I prefer Edd China to the new mechanic), read a bit of Girt.
Feeling absolutely exhausted, I drifted off to an early-ish slumber.
Peace and love to all. ❤
21 June 2021
Rehab – Day 5
Woke up completely drained. It’s the way of things at the moment. It was hard to get my eyes open and keep them that way.
Breakfast has become a routine of watery scrambled eggs (scrumptious), gluggy porridge, diced peaches, yoghurt, and a white tea with one sugar. I was feeling a bit flat to begin with and it took a while to get myself into the right frame of mind. This is going to be a gruelling week of rehab in the physiotherapy gym.
With what amounted to less than a dollop of enthusiasm, I was escorted to the gym (sounds like I couldn’t be trusted, right?). In truth, I walked part way there (shadowed by an occupational therapist). I did a session of “sit to stands”, a series of steps (standing on a spot and taking one step forward and then returning the foot to its original spot) – this was quite the challenge when stepping forward with my right foot, placing all my weight on my weak left leg. Then lying on my back, I had to lift my knees to my chest – well, that’s what I was asked to do, so I settled for lifting them TOWARDS my chest as I really didn’t fancy tearing my glutes. Then, as a cool down, the physio asked me to grip her hand with my left one and, although not a grip, four of my fingers curled inwards! A couple of millimetres! Overwhelmed, I burst into tears.
An unremarkable lunch followed by a snooze before the occ therapist had a session with me. With my eyes closed, she placed various objects in my left hand to see if I could describe them and/or identify them. Correctly identified three out of four objects.
Afternoon physio was late and cut short due to a shortage of staff. Spent 10 minutes walking up and down the gym floor, then walked back to my room (distance from gym to room is about 35-40m.
Sat in my chair for quite a while until my bum became sore. Transitioned to bed for a few zzzzzs before dinner arrived. One of the dentists who works with Josie arrived at the same time, bearing a card and a small bag of fresh fruit. She kindly opened my pre-packaged dinner container, Apple juice, crèmè caramel, and jiggled my tea bag (people…seriously, get a hold of yourselves) and commented that she liked my unruly hair. Very sweet. And now I am anticipating an early night, encouraged by the evident progression of my rehab and by the comments from my physios and nurses.
23 June 2021
Rehab – Day 6 (Tuesday, 22 June 2021)
Woke up feeling despondent – insufficient sleep and a lovely red and blue colour combination in my feet have me feeling a little unsettled.
My usual breakfast, although I remembered this time to order some honey to add to my porridge.
About 9:20am, a speech therapist arrives for a session. A small range of vocal and cognitive exercises ensue. These include comparing two words to identify two similarities and two differences between them, e.g., “conversation” and “speech”, and another exercise asking me to describe how I would undertake particular tasks, e.g., brushing my teeth, and making an omelette (no, Hiratio, not at the same time…). Satisfied that I am enunciating well and fully understandable, she bids me adieu but not before I recommend that she should read Norman Doidge’s “The Brain That Changes Itself” – which she just might do, as she wrote down the details.
Five minutes later, Doris arrives to escort me to the physio gym – I walked. Doris is a short, young Asian woman with immaculate makeup and an impressive collection of colourful ink on both arms. I do a set of transfers. Sitting on an exercise bed, Doris surrounds me with three chairs. The first set involves me standing up (unaided), turning 90 degrees with small steps to my right (good side) then sitting in that chair with a controlled descent – no plonking down in an inelegant fashion, thanks! “Do 20, then go in the other direction” (my weak side). Managed these quite well. Other exercises for the remaining 40 mins then an accompanied walk with Doris back to my room. Lunch of a chicken and mayonnaise sandwich (wholegrain – the bread, not the chicken Horatio).
Gym again at 2.00pm. Walk from my room with Doris keeping a close eye on me, with a light grip on my waist belt. This was a 90 minute session – a mixed bag of balance, strengthening, and movement exercises. Bending down to pick up cones (plastic cones, Horatio, not reefers or whatever the young hipsters call them these days) from a stepping block and placing them on a seat next to me. Then back again. Two more sets. My first short session on a treadmill – three minutes. “Times up. But do you want to stay longer and do some shoulder and wrist exercises?” Why not? Some more movement in my left fingers and then encouraging controlled movement in my left wrist followed by arm curls with an empty water bottle clenched (this implies strength and control which isn’t quite there yet).
Good session. Walk back to my room. Doris says I am improving exponentially (her words exactly) and that I will no longer need a wheelchair to get to my sessions.
Rest of day is quiet while I lie in bed with my usual fatigue. Play some word games, catch up with the news, watch some TV, dinner of poached(?) salmon, carrots, peas, and mash.
Go to sleep around 9.30pm.
Prelude to Wed’s post: student nurse helping me to transfer from bed to bathroom. “Fifty-seven?! You don’t look your age at all!” Me: “Are you going to tell me I look 65?” Nurse: “No. You look like you’re in your 40s.” How insulting! People who know me know that I don’t look a day over 38…
Peace and love to all. ❤ �
24 June 2021
Rehab – Day 7 (Wednesday 23 June, 2021)
Another day where breakfast seems to arrive too soon. About 7.10am, I hear the clatter of my hard plastic meal tray upon my bedside table. Not long after, changeover from the night nurses to the day nurses takes place in the hallway outside my room. And the overhead lights come. As in the several days before, it’s not a gentle wake up process.
Having grown tired of the tired looking scrambled eggs, I have opted for an omelette – thinnish, firm on the outside, not quite fluffy on the inside, somewhat dry. Otherwise, breakfast is the same as usual. Sustenance for survival, not gourmet for enjoyment.
An almost wholly unremarkable day. I make some incremental gains during my two physio sessions, doing my first group class to improve my balance – yes, it’s called Balance Class. It’s a series of different exercises done for five minutes each over the hour, with small breaks in between. Afternoon session, more arm exercises and stepping board work. I walk to and from both sessions.
Back to my room. A bit of a chat with my roommate Kevin – he’d been out on day leave, having ventured down to Macquarie Shopping Centre to dine out big time and returning mid-afternoon with a stash of donuts (he buys them for the staff and a bucket of KFC. Not for me Kev!
A bit of a doze and then dinner arrives – felafel with couscous. Microwaved felafel. It was okay but nothing to write (text?) home about – so I didn’t. Orange and almond cake was quite nice. As always, tepid and weak tea. I’m watching TV when at about 8.15pm, a nurse comes in to give me an injection. What is this about? Who ordered this for me? And what is the purpose of the injection? “It’s an anticoagulant” and something about DVTs is mentioned. A doctor hasn’t even examined me today – how could I possibly be diagnosed with a DVT? Telepathic medicine? My frazzled mind tells me to say no but I allow the injection to happen.
When the nurse returns a bit later, I ask that a doctor see me ASAP in the morning – I am not happy that a doctor hadn’t told beforehand about the injection and why I was having it. I lie in bed for a bit and, becoming somewhat anxious, I ring my CADASIL neurologist in Newcastle, NSW. He answers almost immediately, telling me he is on ward rounds. But he lets me briefly tell my story. I am reassured listening to him.
Nevertheless, I am still unsettled and spend a couple of hours playing Candy Crush, until I can’t concentrate anymore and drift off to sleep.
Peace and love to all. ❤ �
25 June 2021
Rehab – Day 8 (Thursday, 24 June, 2021)
What the…?! It can’t be 7 o’clock already?
It was a short (aaah!) but deep sleep. Knackered. I seriously consider giving this morning’s physio a miss. I’m still irked by last night’s shenanigans and I’m keen to confront the doctors about how this was allowed to happen. Takes me 30 minutes to wake up. But to call my current state of consciousness as awake is generous. I tune into ABC Breakfast to discover that COVID has even infiltrated the NSW Parliament, with the Minister for Agriculture testing positive and the Health Minister being considered a high risk for having close contact with a positive (or likely to be positive) case.
How many others while come down with COVID following their self- congratulatory carrying ons after the NSW budget was delivered.
But I digress. Just after 9.00am, I ring my Team Contact Person, Sandra. She doesn’t answer, so I leave a message asking her to attend the meeting I requested to have with the doctor. I spend some time drafting my belated Day 7 update. Doris swings by. “Ready for physio?” she chirps. She is always so cheery (not in an annoying way), so I can’t say no (welllllll, I COULD…..but, I can’t).
My second balance class. “Watch him – he keeps falling over” chuckles Doris. Pleeaase – I’m not that bad. Okay – sometimes I am but only when my left knee gets really fatigued (and that’s often). Towards the end of my session, the head doctor approaches me to talk about what happened the previous night re the injection. He acknowledges that I, indeed, should have been informed before hand and he apologises for the oversight. I told him I nearly refused to have the injection and, needlessly, he itells me I would have been within my rights to do so. Again, I walk inelegantly to and from the gym. Lunch was a salad sandwich on wholegrain (not bad) accompanied, sir, with a fine, delicately bodied apple juice that lingers on the palate with faint notes of truffle and bark of orchard tree. Another tepid white tea and another vanilla custard.
Then off to physio again for another hour – sit-to-stands, stepping block exercises (firstly, stepping up in a forward direction and then doing side steps). For some reason, the side steps are easier. Then a five minute session on the treadmill. Doris then videos my spectacular walking style.
The OT is waiting for me when I get back to my room. She gives me five minutes to recuperate then comes back. The OT treatment will take place in the OT treatment room, which is conveniently located RIGHT NEXT TO THE PHYSIO GYM, from whence I had just journeyed. “Do you want to use the wheelchair or shall we walk?” “Oh (that’s a long, drawn out “oh”)’ I spy the trap you’re laying. I will be walking, thank you very much, even if I have to slide on my back for the last 20 metres.”
An hour spent in the OT room, trying especially to encourage further movement in my left arm (mostly my wrist). My arm is worn out by the end. Glad to walk back to my room where I chat with Kevin for an hour (well, he does most of the talking), snooze for a bit over an hour, and dinner arrives – I’ve opted for the hot salmon and vegetables dish again. Josie and Sara drop by with fresh clothing and some sweet goodies and chit-chat. It’s a short stay tonight as our friends Geraldine and Danny soon arrive to collect them for the drive home. Dismayed to see Danny has completely lopped off his dark, curly locks. He makes up for it by handing over a small container of more sweet edible delectables – my teeth are going to crumble at this rate.
Watch a bit of tele before succumbing to the Circadin (melatonin) tablet I took an hour before. At 3.47am (Friday) I awake with the top half of my chest covered in sweat. I think I had bunched up a couple of blankets around my neck while sleeping on my right side. A nurse takes my temperature – normal. She removes my top, replacing it with one of my favourite t-shirts – Photo of my Ass.
Peace and love to all. ❤ �
26 June 2021
Rehab – Day 9 (Friday, 25 June, 2021)
Another groggy start to the day. As per each of the previous days here in rehab, I’m not even remotely feeling refreshed, in any way at all. Before breakfast arrives, Kevin emits a generous, sustained fart that does not waver in tone or volume. I’m reminded of a Monty Python movie: “I fart in your general direction”, smugly stated in a perfectly executed Inspector Jacques Clouseau accent.
Breakfast is not very appealing today – it’s the same breakfast as yesterday but my appetite has dropped a bit. I ignore the tray for 30 minutes, trying to get some more rest, before I decide I really need to get some fuel onboard before balance class.
I pick up the omelette, take two bites and toss it aside. I can’t even bring myself to open the bowl of porridge. I tackle the vanilla yoghurt and diced peaches instead, washing them down with – no, not tepid white tea this time but a hot(tish) mug of tea, as the nurse went off to get me a fresh mug of hot water and a new tea bag.
The nurse helps me to have a shower. After getting me seated and turning on the water, she leaves the bathroom to get some waterproof covers for her shoes. Unable to find any, she soon returns with white disposable gloves covering the front half of hers shoes. I snicker. With her short height, face mask, and “webbed feet”, she resembles a dark blue duck. A minute or two into the shower, she manages to step on some of the glove fingers projecting from one of her feet, miraculously avoiding a fall.
I spend a languid 40 minutes reposing on my luxurious bed, waiting for a physio to collect me and assist with my walk to the gym. Adam duly arrives just before 10 am to ensure my walk to balance class is a safe one. It was a really good class, went well. Lost my balance three or four times but not disastrously so. There were four of us in class. The other three would be in their late 60s or early 70s. Bruce, who attended yesterday’s class in a shabby hospital gown, arrived today in a pair of new denim jeans and a lovely blue thin jumper in the half-zip style. With a tanned, balding head fringed with silvery-white hair around the sides and back of his head, midway between the top of his dome and his collar, he is a handsome, well groomed and presented man – I instantly award him the “best dressed in class”. As Bruce and Mr Wang are escorted back to their rooms, Prasad and I await out turns. I turn around to chat to Prasad, who is an amiable man of, I suspect, Persian descent. He is a little more advanced in his recovery than I am. He says “You are doing really well. How long has it been? Four or five days?” “Seven”, I say. “What about you.” “Four to five weeks” says Prasad. I’ve been getting good feedback from the physios but to hear this from a fellow patient is heartwarming and appreciated.
Lunch is an assemblage of salad slapped between two slices of wholegrain bread – entirely acceptable. Apple juice, vanilla custard, tea. More physio from 1.00-2.00 pm. Step work. Sonia gives me the 10” step, quite an upgrade from the day before. Up and down, up and down.
Walk back to my room, where I am expecting the OT to visit at some point during the afternoon. She arrives about 3.15pm, carrying a hard plastic case. “I thought we’d try some electrical stimulation on your arm”, says Sarah. Yes please! I have a small TENS device at home, so I am familiar the concept and the sensations that one experiences using these devices. She applies two medium-sized sticky pads to the hairy upper side of my left forearm and sets a low power emission. There is an immediately small but perceptible twinging from my arm muscles, wrist, and fingers. An increase in power elicits a more noticeable response and, towards the end of treatment, more tweaks to the settings, brings on a startling and exciting reaction, with my wrist bending backwards much more than before (no, Horatio, not to the point of snapping).
Wow.
Time for a snooze, then Lee the Dentist arrives for an unexpected visit just after 5.00pm. She is almost giddy with excitement when she learns she has arrived just before dinner is to be delivered – I think she wants to mother me by cutting up my dinner (poached chicken with gravy and vegetables) and feeding me. We chat for a bit and I get a call from one of my aunties. As I chat with Margaret, Lee gets a call and has to go back to work. Idly watch TV for the evening. Fall asleep around 11.00pm, contented with the progress I have made in my first full week of rehab.
Love and peace to all. ❤ �
27 June 2021
Rehab – Day 10 (Saturday, 26 June, 2021)
Bing!
Woke up at 6.30am. First day off after five days of rehab and I couldn’t manage to sleep in.
It’s odd having fatigue all the time. Your body is telling you it is not happy about the lack of energy you have but MY body won’t allow me to get more than about seven hours of sleep – on average – on most nights. And, here in rehab, that is disturbed sleep.
It’s a quiet morning – nothing exciting really happens. Except, of course, Kevin displays his impressive ability at expelling flatus once again. For morning tea I have tea, crackers and cheese. Big deal, you say. Well, on the other few days I’ve had them, it wasn’t. Today, though, my stomach started grumbling half an hour later and soon started having spasms. I press the buzzer for a nurse to help me to the bathroom. The 10 minutes it takes for the nurse to arrive seems only like mere hours – luckily, I was able to draw on my extraordinary, zen-like, powers of emission control.
After the inevitable takes place, (and, even though the bathroom door is closed, there is nowhere to disguise what is happening to me in a shared room with a visitor and roommate just outside, pretending nothing is going on. “C’mon everyone. Nothing to see here. Move along, move along.”) My vanity disappeared long ago, within a day of being admitted to the ward back at Royal North Shore Hospital.
I emerge from the bathroom and comment to Kevin that the nursing station buzzers need an urgency scale: “At Your Leisure”; “Sometime In The Next 10-15 Minutes Would Be Good”; “Code Red: Catastrophe Imminent”. Josie arrives just before 3.00pm, bearing, inter alia, an egg and bacon roll and a hot tea with milk and sugar. I know! I know! But my tummy was feeling okay again. I complete an online order for a new iPhone 12 Mini (“Navy Blue please”) plus a case and screen (on sale at Good Guys at a good price). I’ve discovered that my current Oppo phone is too unwieldy to operate with one hand, with its 6.2” screen and my small hands. There are apparently no small Android phones available in Australia, so I nab the iPhone 12 Mini with it’s 5.4” screen.
We st(roll) to the level 1 foyer and look outside as the sun goes down – very pretty. I then pose for some fetching portraits, as I take on the role of director whilst @skyblu50 snaps away with her trusty Samsung. “If you turn my wheelchair, facing that way, you will be able to get an evocative image of me and my shadow, with the sun angling across this lovely sandstone wall”. An hour later…well, you can guess the rest.
By this time, the whole hospital had become subjected to a one designated visitor only rule. Crap – Sara won’t be happy (and she wasn’t – very upset when we spoke to her a short time later).
Dinner arrived at about 5.30. One of my favourites – roast chicken with vegetables. The nurse arrives with a dose of Gaviscon. Ah! Now, that should do the trick.
An hour later…well, you can guess the rest.
Exhausted (can’t believe it was possible to be more exhausted than I already was), I take to my bed. Far out!
A bit of chit-chat, some TV watching (more entertaining when it is switched on) and Josie leaves at 8.00.
About 20 minutes later, a couple of nurses go around telling everybody that as of RIGHT NOW, no visitors will be permitted – nada, zilch, none. I understand why but I can’t bear to ring Josie and tell her, lest I burst into tears. Ten minutes later, she rings to tell me she is home, so I break the news.
We hang up and I become very quiet. Keep web surfing for a while, looking for some comfortable earphones that I can wear in bed (I mostly sleep on my side). My fingers start to cramp, so I select the Music folder on my iPad and ramp up my 5-star collection (no, Horatio, not your 5-star collection…your taste in music is…well, you can guess the rest). After two hours, I began to feel drowsy.
Nevertheless, the music must continue. It has to continue…
Love and peace to all. � � ❤
28 June 2021
Rehab – Day 11 (Sunday, 27 June, 2021)
Other than writing and posting my Day 10 update for Facebook, nothing much happens this morning. The place is very quiet – very few staff, no visitors.
Routine stuff otherwise – Kevin farts, I have breakfast (no milk for my tea or porridge – I’ve been having some skim or full strength milk but it is not lactose-free, which is what I use at home. We will see if excluding the milk helps with my tummy issue, which is feeling better but is still not happy.), have a shower.
Following my shower, I sit in a chair to write and post my Day 10 update. Having done that I then languidly laze around, doing nothing much at all, apart from breathing – one of my favourite activities.
Josie rings at some point and I listlessly mumble a few words and tell her I don’t feel up to talking much. We hang up. I text later to apologise – she texts back to say she understands completely, and that we will get through this.
All this activity makes me a bit tired (yes, Horatio, I’m being a wee bit sarcastic) and I go back to bed. Sleep evades me for a while. Eventually, I drop off (to sleep, Horatio, not the bed) and slowly wake up just before 4.00pm, which is what the clock that hangs askew on the wall indicates. Some more nothing much happens. Dinner arrives and is devoured, which suggests I attacked it with some gusto. I did not. It went in my mouth, I chewed it, I swallowed.
A bit of tele, some more web surfing, time to call it a night.
At 2.00am, Nurse Irene wakes me take my obs. She says “You’re cold” and throws a blanket over my sheet, and wanders off to the next room. I want some water but she forgot to move my table back to my bedside. I buzz. She arrives. “I’m notorious for doing that”, she says. I have a drink and try to move the table to the side, so it is not projecting over my bed – I’m going to bang my knee on it as I try to roll over on my side. The table is caught on the bed somewhere and I can’t move it far enough out of the way. Irene will need to move it. I buzz. She arrives. “Yes love”. “I’m trying to roll over but my knee keeps hitting the table”. “What would you like me to do for you.” ?????!!!!!! Seriously? “And I’m just changing a patient at the moment, so if you press the buzzer again, I won’t be able to get here as quickly as I just did”.
It takes me a long time to get back to sleep…
Peace and love to all. � ❤ �
29 June 2021
Rehab – Day 12 (Monday, 28 June 2021)
For breakfast this morning, I have ordered the soy milk to go with my porridge (which is always labelled as “rolled oats”). Imagine my dismay when I see that the soy is in a small Tetrapak with one of those small, bendy straws plastered on the side. Just as bad as the small milk bottles I was never able to open.
A nurses aide opens the soy for me and pours it over my “rolled oats”. Are not rolled oats a dry ingredient, that with the addition of water or milk, some heat, and a dose of love, turns into porridge?
Today’s balance class was smaller (three people instead of five) due to the need for social distancing. I was surprised at how well I went, considering the break for two days over the weekend.
Straight after gym, went to a group occupational therapy session where, again, participants were kept well apart (no, Horatio, not because we can’t stand each other. It’s about the COVID thing that’s going on – social distancing?)
OT is a real workout of a different kind, focussing on strengthening and coordinating my left arm and wrist and to get my fingers to increase their movement. Arm extensions, wrist flipping, the mirror exercise, gripping and lifting a deflated mini soccer ball (no, Horatio, the ball wasn’t feeling down in the dumps, it simply didn’t have much air in it).
At 11.50am, I’m back in my room to log in on my phone for a Telehealth consultation with my main neurologist, Prof Chris Levi. He tells me more about whereabouts in the brain my stroke occurred and why it had the effects on me that it did. He asks about my rehab and the progress so far. He tells me that he trained the neurologist who supervised my treatment whilst I was in hospital. Twenty-five minutes passes by as if it were three-and-three- quarters.
After a lunch of one chicken and one salad sandwich, it’s off for an individual OT session. My new OT reviews my left arm abilities and then treats me with a 20 minute electrical stimulation session (no, Horatio, the treatment pads were applied to my left foreman, not my temples, and, no, I was not sitting outside in the middle of a raging storms full of lightning bolts). Natalie uses a red permanent marker to trace where I can place the pads when I get to treat myself while I am still here.
Immediately after, it is an individual physio session comprising walking backwards (a weird sensation, as I can’t see where I am going but there is tape on the floor to mark the endpoint); sideways walking; step work. Snooze for an hour before dinner (roast chicken and veggies).
Later on, I keep searching for the perfect pair of wireless earphones to wear to bed.
Apart from my fabulously enjoyable teleheath consultation with Chris, I later have phone calls throughout the day with Dad and my sister Kim (she also sees Chris but was able to do so in person in Newcastle, NSW) and then with Sara (she was bored at work) and later again with Josie.
Bed.
Love and peace to all. � � ❤
30 June 2021
Rehab – Day 13 (Tuesday, 29 June 2021)
Faaaark!
I very rarely swear (and, as you can see here, even when I do I can’t do it properly). Australians might recognise that this is the Graham Kennedy version of the word when broadcasting on live TV.
Man, I’m really feeling it today after yesterday’s heavy schedule of rehab fun and games. I scan my body for somewhere that doesn’t hurt………aah! Ear lobes don’t seem to be too bad…oh! And my hair doesn’t hurt one bit. Breakfast. I’ve gone off the eggs and one bite of the omelette is more than enough. I eat the “rolled oats”, fruit, honey, and soy milk, gulp down my black tea and sugar.
Shower and brush my teeth – to be clear, Horatio, I shower my body, not my teeth. Make a note to shave tomorrow – all I can see of my face are dry lips and tired eyes.
Spend some time doing my Day 12 update. One-finger typing is cumbersome and adds to my fatigue (believe it or not) but at least I can do it. I plough on because I need, and want, to do this.
Speech pathologist turns up for an unscheduled visit at 9.30am. Well, in her diary, she had scheduled 9.30 but the printed schedule on my “GR5 Journey Board” indicates she isn’t due until until 1.00pm. So, we speech pathologise to our hearts’ content. She can’t understand only one of the words I say – “careers”. Pretty sure it was a hearing issue on her part, Horatio.
Balance class goes really well. Only twice did I totter a bit and I managed to regain my balance without intervention from staff. OT class is excruciating after only 10 minutes but it’s okay…only 50 minutes more to go. Off to lunch, where I plan to sleep while eating it.
As one o’clock approaches, after much research I have decided which wireless headphones to buy: Sony Wireless Noise Cancelling Headphone Silver (WH-1000XM4). I place my online order, at a bargain price. This causes much joy and elation throughout Ward GR5.
Not one to miss an opportunity, Doris comes to nab me for another gym session. “But, but!” I protest. So I submit to a gruelling session, until my left knee wants to keep collapsing.
At 2.00pm, I’m wheeled to the carpark under the rehab centre. Whilst under cover (no, Horatio, it’s not assuming a different identity), the entrance is exposed to the elements and it is f..f..f..f..freezing! I’m wearing my gym shorts and a t-shirt, as I came down directly from gym. I’m having a “How to Get In and Out of a Car” lesson. I nail it first time. It was the front passenger seat.
Back to my room and within 30 minutes I’m asleep.
Dinner then I watch Anh Do’s Brush with Fame and spend the last 10 minutes weeping copiously. Sorry for what you’ve been through Dr Harry. Watch Outback Truckers for some light relief, and take a sleeping tablet to ensure a good night’s sleep.
Peace and love to all. � � ❤
1 July 2021
Rehab – Day 14 (Wednesday, 30 June, 2021)
I slept, I woke up.
First day of breakfast without eggs. Find that porridge with a bit of honey, diced peaches and soy milk, washed down with an uninspiring, lukewarm tea with one sugar is all I need.
After brekkie, time for a shower and some man scraping/grooming (no, Horatio, not down there). My hair is wild and wooly and needs a bit of taming, and the beard needs a trim. The hair cuter doesn’t work, so I do it all with my trimmer.
Have another really good, socially distanced, mask-wearing balance bootcamp. We have been wearing masks in all our rehab sessions. Group OT session immediately follows in the dining room (which is simply a group of tables in an open area where people can eat their meals if they wish). These OT sessions are extremely taxing and sap my energy. I need to strengthen and encourage mobility in my left shoulder (in all directions), my left wrist (especially, if you imagine laying my arm flat on a table, with my palm down) bending it upwards, and curling and straightening my fingers. What was once so easy is now frustratingly difficult.
Lunch of sandwiches, apple juice, diced fruit. A doctor arrives halfway through lunch to examine my right ear. It is nit infected (phew!) but there is a lot of wax right near my eardrum – this is an ongoing issue for me, as I am just a prolific producer of cerumen. Waxsol will be despatched to treat the ear.
At 1.45pm, I press the buzzer for a nurse as I want to use the toilet to open my bowels. Just before 2.00pm, a nurse still hadn’t arrived when my OT comes in to collect me for a session. I tell her why I might have to leave part way through. Eagerly, she says “I’ll take you” and, nonplussed, assists me into the bathroom. As I heading in there, I turn to Kevin and ask if he will be okay, as he had buzzed shortly before me for the same reason. “I’ll be right. I’ll just shove some cement render up my arse”. I laugh and Natalie says she didn’t hear what he said. I said “You don’t want to know”. Then I change my mind and tell. Everyone here knows Kevin and what he is like. A little smile crosses her face and she says “he is a character”.
I drops me daks (overseas friends: trousers or pants) and she watches intently as I lower myself slowly to the toilet seat, where buttocks and cold porcelain will soon meet. Satisfied, she then leaves me to my business. One task ticked off: he can safely lower himself on to a toilet seat without falling in or off.
OT session which includes electrical stimulation therapy (think of a stronger type of TENS machine). After, I am given the machine on loan for self- treatment. Part way through, the main doctor Dude pops his head in to see how I am and tells me I no longer need to keep taking aspirin. Meat pie, mash, peas and corn for dinner.
Short chat to Josie and Sara, then time to swallow a Circadin tablet (melatonin) and try to enter dreamland.
Love and peace to all. � � ❤
Oh! And I learnt how to put my own sling on (leave the straps as they are, loop it over my neck, feed my left arm into the sleeve and make sure the loop goes around my left shoulder).
2 July 2021
Rehab – Day 15 (Thurs, 1 July, 2021)
Woke up feeling nauseated. This, along with a heart beat reading of 45, prompted a call to the night doctor. She arrives, asks some questions, and starts prodding my stomach. “Does it hurt here? Here? Here?” An anti- nausea medication is prescribed. A review is made of my previous nights’ pulse, all of which were in the forties. I show them my iPad with my previous 12 months of morning blood pressure and heart beat readings- my average morning pulse over that time was 51.
After the commotion dies down, my occupational therapist Natalie arrives at 8.00am to watch me take a shower. Am I able to clean, dry and dress myself well enough to gain a pass mark? It seems I did well.
Back in my room, I feel well enough to eat breakfast. After breakfast, I put my shoes on and tie my laces one-handed for the first time: https://youtu.be/ U4n1Tti3eR0 (below).
A social worker, Amy, drops by for a chat. She will bring print-outs tomorrow re NDIS and Disability Support Pension eligibility criteria.
Balance class then OT group (as difficult as yesterday).
Just after lunch, the psychologist, Marta, calls around to see how I’m going. As she leaves, she asks if I need anything – I ask for a pen. She comes back a couple of hours later with one.
I go to bed for a quick nap before physio. I wake up later, having slept through my physio time.
Dinner is felafel and couscous. I then treat myself to an electrical stimulation session on my left arm. Incredibly draining. Josie and I chat on the phone but I barely have the energy to talk.
Soon I’m in bed, unsure of how I got through the day.
Love and peace to all. � � ❤
3 July 2021
Rehab – Day 16 (Friday, 2 July, 2021)
8.00am: I’m off to report for Breakfast Club! I couldn’t be less enthused if I tried…
The venue is the Dining Room, down the hall and around the corner. When I arrive, I see a group of socially-distanced tables. Some are for the attendees to dine at, others are variously adorned with a toaster and bread, cereals, milk, and fruit juices, a jug with mugs, tea bags, instant coffee, sugar. The three men who attend get to awkwardly prepare our own breakfasts with our assembled range of physical limitations. Supervising us is an occupational therapist and two assistants. There are several “good works” and “well dones” and, for me, “don’t forget to use that left hand” – the only highlight was having a slice of raisin toast for the first time in weeks. Still no lactose- free milk in sight (“there’s soy milk!”), so I swallow down yet another bland black tea.
Unable to totter away quickly enough, I am accompanied back to my room.
Quick shower – mostly washing and drying myself unassisted, dressed and in the chair to put my shoes on. Manage to tie the laces on one shoe quite well but get the shits with the next one- it’s too loose.
Good balance bootcamp session again followed by a tedious and testing and frustrating OT group. This is currently my Achilles’ Heel and it’s the biggest challenge I currently face – it’s a must do for me, I know that. After lunch, Natalie collects me so that I can do a load of laundry. I’m guessing it’s a 30m walk to the laundry. I’ve carried the clothes down there and I get to load the front-loading machine (“don’t forget to use that left hand” she says cheerily). Set the machine and back to my room for a rest before physio.
I report for physio at 2.00pm. Making my way to one end of the parallel bars, I expect a physio to tell me what they would like me to do. All are busy with other patients, so I work out my own routine and get underway. Thirty minutes in, “Sarah, is there anything in particular I ought to be doing?” “Oh? Let me check who’s supposed to be looking after you.”
At 3.15, Michelle, an OT assistant, takes me back to the laundry so I can place my cloths in the dryer. Four hours later I go back there with a nurse to collect them. She starts to carry the bag for me, I protest – it’s part of my therapy to carry the bag myself.
About 7.30, the psychiatrist wanders in to see how I am doing. “O….kay…”, I mutter, in a way that betrays that I’m not feeling at my mental health peak. I say some stuff, he says some stuff, and we agree that from Monday, we will increase my dose of anti-depressants. Having no visitors, not having been outside in almost a month, having constant fatigue, working hard in rehab, and being too listless to talk in any meaningful way to Josie and Sara, and experiencing – mostly – Groundhog Day from one weekday to the next…well, gets me down a bit at times.
Love and peace to all. � � ❤
4 July 2021
Rehab – Days 17 & 18 (3 & 4 July, 2021)
It’s the weekend – very quiet, nothing really happening.
Visitor ban still in force.
Pass my time watching television, reading, listening to music, sitting in the chair doing some of my rehab exercises, occasionally looking out the window, napping, doing some research on how to develop my best frame of mind whilst living with a chronic disease, the occasional chat with Kevin, having my obs taken every now and then.
No phone calls today – my ears are blocked and I am going to ask a doctor to syringe them on Monday. My ear canals are also itchy. Not helping with the balance issues I already have, not to mention how it affects my hearing. Oddly, the weekend hasn’t taken as long, up to this point, as I thought it would.
Love and peace to all. � � ❤
6 July 2021
Rehab – Day 19 (Mon, 5 July, 2021)
Woke up this morning with very little in the way of energy reserves. “Careful: hot”. The usual refrain as the kitchen hand delivers my breakfast tray. Trying a pancake and muesli today.
OT assistant arrives to assess my showering capabilities. I decline to have a shower and go back to sleep. Doctors arrive a bit later to check me out. I think I have a perpetual, low-grade migraine. Mersyndol is prescribed (turns out they have none). What about my blocked ears? Waxsol softens my wax but does not get rid of it. Turns out the pulsing machine has a critical piece missing and the place does not have an old-fashioned stainless steel syringe. Apparently, stroke patients don’t get blocked ears. I hear some muttering about borrowing one from Ryde Hospital next door… Left alone, I sleep through physio and OT. Feel a bit better after lunch, so go to afternoon physio. OT visits me after that to try out a shoulder brace on my left shoulder. There are no left-sided braces available, so I’m fitted with a reversed right-sided brace – not ideal…
Electrical stimulation, dinner, early night. Take two melatonin tablets to help me sleep.
Kevin farts.
Love and peace to all. � � ❤
1:55-2.00pm, Tuesday, 6 July, 2021
6 July 2021
Two days short of a month, I spend my first time outside since my stroke. I sit in the Graythwaite Rehabilitation Centre’s second-floor courtyard, absorbing some golden rays of sunshine and feeling the cold breeze flow over me.
Aaaaaahhhh…..
7 July 2021
Rehab – Day 21 (Wednesday, 7 July, 2021)
Time again for Natalie, the occupational therapist, to monitor me while I have my morning shower. Having completely disrobed, I make my way to the showering chair. One of my little toes contacts one of the chair legs and throws me off balance. I stumble towards one of the walls and Natalie frantically grabs me before I tumble to the floor.
Adrenaline pumping, I clean and dry myself with no assistance, don my gym wear and back I go to my room to do the one-handed lace tying trick. Balance class as usual, followed by group OT session – I was the only one there. One on one helped big time – I improved in every activity and finally got some decent movement in my left wrist.
Managed to have some free time after lunch, then back to physio. Towards the end of physio, Josie phones me to say she is at reception in the entry foyer down stairs. She has fresh t-shirts and undies(!), my new (smaller) mobile phone, my new wireless headphones and some other bits and pieces. Doris (my physio) offers to take me down to reception, where Jos and I maintain a distance of three metres whilst wearing our masks. I can tell she was smiling – she wasn’t expecting to see me. Doris collects the bag for me (she also is wearing a mask), then immediately retreats to where I’m standing. Doris says “show her your arm”. I raise my left arm straight into the air above my head whilst Jos takes a photo and she then takes a video of me demonstrating my improved walking technique. All up, the interaction took less than 90 seconds. Under the COVID rules, this would be classified as a compassionate visit (it included bringing in my ear syringe and some spray to help clear my ears).
Doris accompanies back to my room, deeming the bag too heavy for me to carry in my current state (would make walking too difficult).
Before dinner, I thread new shoelaces (part of my delivery) on my shoes – the ones I already had were a bit stiff and hard to tie with one hand. Doing this was a labourious task but one that should make it much easier to tie with one hand. Okay, Horatio, I’ll tell them – I opted for the bright orange laces. After dinner, I setup my new phone� . I hadn’t saved my contacts to my SIM card, so this also proved labourious.
Time for bed.
10 July 2021
Rehab – Day 22 (Thurs, 8 July, 2021)
I made some good progress with occupational therapy again today. Afternoon physio session saw me doing some treadmill work followed by some leg presses.
Most noteworthy event today though was being given a notice to vacate the unit we have lived in for almost 30 years. The only place our 22 year old daughter has ever called home. The owner is moving back in after all these years (after, no doubt, a complete renovation). No, Horatio, the renovation will be of the unit, not the owner.
Kevin says we can live with him in his unit in a retirement village (in which, incidentally, many residents succumbed to COVID last year).
Inconvenient, to say the least but we have three months to find another place. And the property agent is already looking through her books for similarly priced rentals in our area. And we might end up finding a place that is more suited to my needs.
As part of my afternoon physio, Doris takes me for a short walk outside. After a few weeks of rehab walking on a flat floor, this is a (welcome) diversion and challenge. The concrete footpath works it’s way slowly uphill and is, at times, a little uneven but attentive Doris is eagle-eyed. Amazing how the slightest uphill walk at this stage of rehab feels like running up a dune when I was at my healthiest. Probably ambled about 350 metres. As The Mandalorian would say “This Is The Way”.
May The Force be with us.
Love and peace to all. � � ❤
10 July 2021
Rehab – Day 23 (Friday, 9 July, 2021)
Today, I have three breakfasts.
Muesli is delivered to my room on a tray. I thought it had been cancelled because…
Breakfast Club is scheduled in the Grand Dining Hall at 8.00am (no, Horatio, it is not a smaller version of Hogwarts). After downing my muesli, I head down to make raisin toast and a STEAMING HOT cup of Twinings English Breakfast tea (no, Horatio, this is not a paid promotion).
I stay a bit longer to keep Peter, who arrived a bit late, company. He’s been here for two months after having a stroke while cycling down Bradley’s Head Rd near Taronga Zoo (for those not familiar with the area, it’s a steep road). He spent a perilous two months at Royal North Shore Hospital before being transferred here.
Another good balance bootcamp but the soles of my feet are bloody sore, as if the delicate bones are being mashed around. Towards the end, we have what Doris calls a “quoit-off”, whereby two patients have a small quoits contest. I laugh, as I have before, at this phrase and I suddenly remember why I find it amusing – it reminds me of Sheldon’s non-plussed use of the word “coitus” in The Big Bang Theory. A few minutes later, bootcamp finishes and Doris quietly approaches me and says discreetly, “speaking of coitus………. …….has anyone spoken to you yet about sex and intimacy after having a stroke?” Apparently, somebody will at some stage (possibly the team contact person Sandra, who has done research into intimacy and sex concerns following a stroke).
Today’s OT session was cooking my third breakfast of the day. Painfully slow process which I didn’t enjoy as I had to keep standing on my protesting feet for the whole hour. And don’t forget “to keep using that left hand”. And I washed up after finishing the cooking. Yes, it was an achievement but the effort left me more fatigued than usual.
And the fatigue of CADASIL is not an issue that is appreciated here – it was there before my stroke and the stroke has only added to it.
About 2.00pm, I’m still listening to music through my freshly charged and programmed wireless, noise-cancelling headphones, awaiting someone to escort me to physio. I close my eyes for a bit, soaking in the wonderful sounds. I’m suddenly aware of a presence within my force field of personal space and and wake up to be startled by Doris’ grinning face about 30cms in front of mine. “I knocked and then called out your name” she laughs. Okay, noise-cancelling works…very effectively, as it happens.
Afternoon physio included a ten minute walk on the treadmill, some leg presses and bum raises whilst prostrated on a treatment bed – “engage those glutes”, chirps Doris.
Josie drops off a couple of other items for me, getting driven here by a friend just prior to stricter COVID restrictions coming into place. I don’t see her this time as I’m still finishing physio. Afterwards, Doris pops down to reception and picks up the delivery for me.
Not much else happens for the afternoon, other than a quiet collective relief that our program for the week has ended.
Another quiet weekend – a smaller lockdown within the wider Sydney lockdown. But, hey, I’m safe in here and acceptance of the reality of what is is the only way to go.
Sunday is almost a repeat of Saturday, but I speak to a few virtual visitors on the phone.
In between times, I binge on “Home Before Dark” on Apple TV.
Bring on Monday.
Love and peace to all. � � ❤
13 July 2021
Rehab – Day 26, Monday, 12 July 2021
Yay! I’m awake at 4.00am. Don’t know why – just am. I can tell I won’t get back to sleep before brekkie arrives and I don’t. Spend three hours tossing and turning, a bit of breathing meditation, eventually watching the sunrise. Nurse Nicole wanders in just after 7.00am to say g’day and see how I’m going. Of course, I have a whinge. After half an hour, she disappears – found out today, she was re-allocated to another ward. Two student nurses look after Kevin and I until the afternoon shift change.
Just before 10.00am, Amy, one of the social workers, drops in for a chat and an update. Inter alia, she tells that my proposed release date is this Thursday. I’m a bit alarmed – I’m not even walking independently yet and I need to develop more confidence about my balance and walking, as well as doing more strengthening work on my left leg before I leave. I don’t have any gym equipment at home and currently am unable to visit a gym. Amy agrees that Thursday is too early and will be advocating for a later date.
Balance boot camp was again a success. OT group saw a bunch of new rehab exercises tossed my way. Ten reps of 45 seconds each, starting…now! They’re designed to broaden the range of motion in my left wrist and left thumb. When I’m finished, I’m sore and wiped out.
Lunch and 10 minutes before I’m due to go back to physio, I buzz for a nurse so that I can go to the toilet. One of the student nurses turns up 15 minutes later just as Jack arrives to escort me to physio. I tell them I buzzed ages ago and we soon discover that my buzzer wasn’t working – I’d heard the buzzer chime but it didn’t register at the nurses’ station. The student nurse just happened to wander by to check how Kevin and I were doing! Jack shepherds me to the toilet: “a number 1 or 2?” he says. “Well, it was number 1 when I first pressed the buzzer but it’s become a number 2 in the meantime.”
By the time I get to the gym, there is only enough time to spend 12 minutes on the treadmill and 15 minutes to do some leg presses.
Jay, the afternoon nurse, finds a replacement buzzer that works but it is a little sensitive – I brush my arm against it and accidentally set it off. Better than not working but I’m careful not to breathe on it after that…
Rest of the afternoon passes quietly, dinner tray comes and goes, I listen to some music, and then it’s an early night.
14 July 2021
Rehab – Day 27, Tuesday, 13 July, 2021
Thanks to taking two Circadan tablets the night before, I had the best sleep I’ve had in a long time.
Kevin issues his now standard pre-breakfast salutation.
When he wakes up, he complains about a woman in the room next door who kept calling out for a nurse throughout the night. For some reason, she doesn’t use the buzzer and on previous nights I’ve also heard her calling out: “Nurse, a pan please”. Waits a while (as there is no nurse within earshot). “Nuuurrse, a pan please……..oh, pleeeaaase…..” But last night, I slept through it all.
The nurse on morning shift hears Kevin’s story and I say I didn’t hear a thing. She tells me I must have had a deep sleep as apparently during the night, when a nurse came in to take my obs, I flailed my arms wildly around, causing her to abandon her attempt and to leave me alone. Nuh…no recollection.
At Balance Bootcamp at 10.00am, my classmates are Katherine, a cheeky 93 year old and Eileen, a recent admission, who is not much younger and has recently embarked on her stroke recovery journey. Only a few days prior, Katherine had been in ICU in Ryde Hospital after having a pulmonary embolism whilst here in rehab.
Straight after, I’m doing the gruelling new OT exercises designed by Natalie the day before. Just as challenging as the day before.
Lunch seems to pass by in 15 minutes but as I’m about to head back to the gym, Sarah – one of the physios – erases some of the notes under the Physiotherapy section of my GR5 Journey Board and replaces them with an (I). This means my status for walking has been changed from supervised whilst wearing a transfer belt (with grab holds for a physio or nurse to use in case I begin to fall) to independent – no-one need accompany me any longer when I am walking. This is a milestone, exciting and somewhat scary at the same time.
Newly independent, back in the gym I start off on the treadmill. I put in 15 minutes at the staggering pace of 2.7 km/h and, after a short rest, do a stint at the leg press, doing several reps of double-leg presses initially followed by single left leg presses.
At 2.00pm, I gather with a couple of other patients outside the gym. We are to head down to the undercover staff carpark to receive instruction on how to do car transfers (modified way of getting into, and out of, the front passenger seat). Rather than getting in the way I have for decades, I now have to go in bum first. I need to approach the car as normal but once the door is opened, I need to step to turn so my back is facing the door opening. Once the back of my legs can feel the car, I reach to the sides to steady myself on the dashboard and either the grab handle in the ceiling or the door sill on the B-pillar. Now I start my bum descent to the seat. Once seated and stable, I can then swing my legs in. Getting out is essentially the reverse process. I give it a couple of goes, as I wasn’t really happy with my exit the first time.
Rest of the day is my own, spent mostly in my room searching online for a shower chair that isn’t stupidly expensive. I find one at a good price and place my order.
After that, I pass the time doing nothing in particular, until it’s time to hit the sack.
Love and peace to all. � � ❤
15 July 2021
Rehab – Day 28, Wednesday, 14 July, 2021
Another night of rubbish sleep – well, good sleep until I woke up at 4.00am again and couldn’t get back to sleep again.
At changeover, the night nurse tells the morning shift that I slept through the night and wondered how I could in such an environment. The mournful plea from next door for a bed pan commences just after 6.00am…
I walk myself to the gym and do the balance class with the two lovely elderly ladies from yesterday and OT sees me using the high intensity training approach from yesterday – six different exercises focussed on getting more motion in my wrist. Five 45 second reps again for each exercise. High repetition = greater chance of regaining as much functionality as possible. Lunch is a very unappetising chicken and pasta salad – the tomatoes and capsicum are sliced far too thickly (as they were in the two other salads I have had) and the pasta sauce tastes almost like the disgusting relish that was on my beef sandwich a couple of days back.
Back in physio, I learn how to get myself back off the floor after having a fall. I need to manipulate my body into a position that will allow me to get on my hands and knees. Having done that, I can then crawl to chair so that I can haul myself back on to my feet. Having completed three mock self-rescues, I find myself breathing hard – this is the hardest work I’ve yet done in the gym. I then do some stair walking.
At 2.30pm Josie arrives at reception. She’s been given special clearance to be trained in what to look out for when she is walking with me and to ensure I follow safe procedures when I encounter kerbs. After that, Josie comes back to my room so that Natalie, my OT, can show her how to ensure I safely get into, and out of, the shower (as we need to step over a hob).
Then at 3.00pm, we have a family conference with the neurologist registrar, team coordinator, social worker and others to discuss my progress and to negotiate a target discharge date (rather than tomorrow, which was proposed late last week, without input from me). I present my six prepared points arguing why my stay should be extended: I need to practice my independent walking more – in a safe environment – to build my confidence; I need more time to strengthen my left leg (I don’t have the appropriate equipment at home and won’t be able to visit a gym anytime soon); I need time to purchase, and have delivered, assistance aids to help with my new needs – a shower chair, a transfer belt, kitchen aids, and more; before discharge, I need to develop more awareness of where my left foot is when I am near chair and table legs; Josie needs to satisfied I won’t be a danger to myself when I am alone at home for extended periods, e.g., when she and Sara are at work; and it’s only been just over a week since my dose of Zoloft was increased. I have solid support from several of the staff and it is agreed I need to stay longer.
Josie takes some of my belongings in preparation to make impending departure more manageable (as I will be using the patient transport service to get home, not being keen to catch a taxi or Uber car – who knows where in Sydney they have been or the state of health of the drivers or their previous passengers?)
I farewell Josie down in reception, and then head back to my room. As I approach the ward’s front desk, Citra, the neurologist registrar who was in the meeting, was leaving the staff room with handbag on her forearm. She stops and looks me directly in the eyes. A couple of seconds pass and she says “I am so proud of you” in apparent reference to my rehab progress and the work I’ve done so far to get to this point and, possibly, also to my coherent and eloquently presented case to stay her a bit longer.
Back in my room, I do a search for transfer belts, finding exactly the same one I have been using here at rehab. This one has worked well for me here, so it will good enough for me at home.
On Wednesdays, meat pie is on the menu. I have one for dinner along with mash, peas and corn. Very filling.
Love and peace to all. � � ❤
16 July 2021
Rehab – Day 29, Thurs, 15 July, 2021
Same breakfast I’ve been having for the last couple of weeks now – Carmen’s Crispy Deluxe Fruit & Nut Muesli with soy milk, a slice of wholemeal bread with marg and jam, tepid black tea with sugar and a small apple juice. And my tummy rumbles again after I’ve finished.
Nurses arrive to hand out my morning meds and a new starter is among the faces. Karl, a young, tall handsome bloke with short cropped, neatly parted black hair – only the third male nurse I have seen in the four weeks I have been here.
At 7.50am, my OT Natalie arrived to supervise my showering again. She sets up a pretend hob for me to step over (as she had yesterday). The shower head is placed high to emulate the position of the shower at home. I’m in control of everything – disrobing, sitting myself in the chair, turning on the taps, pressing the plunger on the liquid soap bottle, soaping up, washing ALL parts of my body, shampooing my very short hair, rinsing off, drying off with three towels, dressing myself, brushing my teeth. All the while, Natalie and I chat amiably about this and that.
About 8.45am, Natalie accompanies me to the laundry so that I can wash my clothes. As we near the ward front desk, Citra rounds the corner. She stops and looks at me, again directly into my eyes. With a mix of mild incredulity and admiration, she says: “The Australian Brain Alliance?” I say: “Mmm…what have you found?” “Your photo on the internet along with this article about your family’s journey with a rare brain disease.” Says I: “Aah, that…did you find my website as well?” “Uh-huh.” She extends her hand and she says: “I’m proud to know you, man!” Genuinely stunned and humbled, I say “thank you”.
Washing machine off and running, I shuffle back to my room. At 9.00am, Neil, one of the new physios, pops in to see if Kevin is coming to the gym – he can get himself there in a wheelchair but he hasn’t got out of bed yet. He’s had a dummy spit. “No. I’m not going anymore. You can shove it up your arse.” Neil says “Can I ask why?” “No, you can’t.” “I’ll pass on the message”
Balance class and it’s about time I share what usually happens. It’s a series of exercises done for five minutes at a time over about 50 minutes. At the beginning, each was done with feet, shod in non-slip socks, firmly planted on the vinyl floor. And progressively I moved to doing them while standing on foam floor mats (about 6-7cms thick). In no particular order: sit-to-stands (no mats, ever); heel raises; toe taps (lifting one foot at a time gently onto a step and bringing it back down again); standing with one foot in front of another (initially, side by side with one foot further forward than the other and, later, one foot directly in front of the other, then swapping halfway through); quoit throwing; four-step shuffle (stepping forward, one foot after the other, then repeating it to the right, then backwards then to the left. Swap half way through and throw in some diagonal stepping occasionally); bending down to pick up cones from a step board or the floor and turning to place them on a seat, then moving them back down again; facing the wall, squatting down to touch my toes then reaching as high as I can up the wall – 5 reps on the left, same on the right, and then repeat.
OT group same as the day before – repetition is a very crucial element in stroke recovery.
Lunch is a couple of sandwiches, vanilla custard, orange juice and a tepid black tea.
Afternoon physio is more treadmill work and leg presses.
And the formal part of the day is over and done with.
Recovery until dinner arrives. Consume, watch tele, blah, blah, blah…
Love and peace to all. � � ❤
18 July 2021
Rehab – Day 30, Friday, 16 July, 2021.
(Edited to add MAS test)
Breakfast is the usual, which includes, as always, a sachet each of salt and pepper, no matter what I am eating.
Michelle, one of the OT assistants, pops her head in to see if I would like to go to Breakfast Club. This is very last minute and I have just finished my breakfast. I could do with a slice of raisin toast but “oh, we don’t have any today…” Nevertheless, a HOT cup of tea would be welcome, so I trundle down.
Balance Bootcamp feels like an endurance session this morning – the soles of my feet are in great pain and my left leg is feeling lazy.
Following a chat with Josie last night, I ask Citra late morning if it is okay for me to delay my discharge by a day. This will allow Josie to check and remove as many trip hazards as possible (and practical) before I get home. Citra is okay with that – discharge will now be Wednesday, 21 July, 2021.
Whilst I am talking to Citra, she she shows me a a set of slides about CADASIL that she used earlier that morning in a presentation to fellow neuros. Awareness raising, lifting the profile of CADASIL. She’s made reference to my website and my article for the Australian Brain Alliance. “When you leave here, you must be an ambassador”.
After lunch, it’s back to the gym, this time for a 15 minute outing on the treadmill followed by 30 minutes or so of leg presses (using both legs at first and then my left leg only).
Between 2.00 and 3.00pm, my OT Natalie uses a Motor Assessment Scale test to rate the motor function in my upper limbs (yes, Horatio, my arms. Well, because that’s the terminology they use in OT land).
Most of the tasks make sense but a couple are a bit puzzling: combing my hair without moving my head AT ALL (Natalie agrees that this one is a bit odd who doesn’t move their head when combing or brushing their hair?); the second odd one is using a large wooden box with two open compartments and a high divider in the middle (think the board game Battleship). The open compartment on the right has several colourful wooden cubes (~3x3cm). Over 60 seconds, I have to use my right hand to pick up one cube at a time and drop it onto the left compartment without releasing it before crossing the dividing wall, i.e., NO tossing or flinging. So, what’s buzzard about that? Nothing Horatio. “Now, do the same thing with your left hand, staying exactly where you are and moving the cubes again from right to left”. Huh? Dinner is roast chicken and veggies (“But not as we know it Jim!”) Watch a new episode of Midsomer Murders. Slumber awaits.
Love and peace to all. � � ❤
19 July 2021
Rehab – Days 31 & 32, Sat and Sun, 17 & 18 July, 2021
The first weekend since I’ve been here where I have been able to walk around independently – no-one trailing behind waiting for me to stumble, trip or collapse at any moment.
Saturday is weigh-in day and the result shows that I have lost about a kilo since my first day here. This is apparently a result of the physio work, as I have not had a loss of appetite since having my stroke.
I had been hoping to spend some time out on the level 2 courtyard in the fresh air and sunshine but Saturday in Sydney was far too blowy and cold. Instead I spend much of the day alternating between rehab homework, watching tele, going for unescorted short walks around the ward, playing games on my phone and iPad, listening to music, talking crap with Kevin and looking online for a spillproof urine bottle that isn’t overpriced.
Wake up Sunday morning to a more pleasant looking day. After brekkie and a shower (watch out for those bloody shower chair legs!), I decide to head out to the courtyard. There is one other patient out there but I decide to let her stay – there’s room enough for both of us (yes, Horatio… of course I wasn’t going to kick her out).
Leaving her to her metallic bench seat, I head for a chair that is in the open sun. I carefully lower myself and immediately retrieve my phone from a pocket in my shorts. I trawl through various news items whilst enjoying the warmth of the sun (the courtyard is protected from wind – no Horatio, not Kevin’s – by high walls) and building up my levels of vitamin D. Lunch beckons…but I return to my room anyway.
After lunch, I go for another walk around the ward and then out to the courtyard once more, where I indulge in listening to music through my wireless headphones whilst I chillax. And then I feel the need to give my phone camera a workout, challenging myself to find interesting subjects and angles.
Time to walk again and I end up making a video of myself walking. I do a little more exercise (heel raises, sit-to-stands).
Dinner beckons…but I return to my room anyway.
Not much else happens for the evening – chitchat, tele, gaming…
Come 9.30pm, it’s lights out and time to drift off to sleep.
A couple of hours later, I hear a truck reversing down the corridor outside our room. No, no…that can’t be right. Can it Horatio? It’s the emergency signal coming from a room further down the corridor and within moments there is a cacophony of voices as nurses, wards men, doctors descend on the hapless patient. Voices are calm but there is no speaking in hushed tones…So much for the uninterrupted sleep I was hoping for (but not really expecting).
Love and peace to all. � � ❤
20 July 2021
Rehab – Day 33, Monday, 19 July, 2021.
After a restless night (sleep coming in fits and starts, disturbed by the faint scent of urine), I wake up to the beginning of a new rehab week.
Kevin rips off a beauty.
Breakfast, shower, drying, dressing, shave, brushing of teeth. I’m tying my shoelaces (well, attempting to) when Dr Zeman, a rehab specialist, strolls in and asks how I am. He runs the joint.
Time for physio and my favourite activity of the day – Balance Bootcamp with Katherine and Eileen, and this time Jimmy joins us for the first time. As part of this morning’s session, I manage to throw 6 quoits over the peg using my LEFT hand. (Okay, yes Horatio, the peg was a mere 60cm in front of me and my throwing was far from elegant but this task is more about effort than style, wouldn’t you agree?) And it’s a result I couldn’t have achieved only a week ago.
At one point, Elder Eileen was struggling with toe taps, on a very low stepping board. Physio Andy provides encouragement and I tell Eileen she is doing good work. She looks at me and says, with conviction, “I’m going to do what you did” and gets back to completing her toe taps. I’m so pleased to hear this, as only a few days ago she was despondent and feeling hopeless about her prospects of recovery. I had told her last week that I also was unable to walk, let alone stand, when I had first arrived. I think it has helped her to hear another patient’s perspective.
OT group is next up in the schedule. I get straight into my now familiar routine, a series of exercises done in the same sequence, for the same number of reps, in the same time frame. Rinse, repeat is the recipe for progress.
Lunch then back to physio. Ten minutes on the treadmill and the rest of the hour is spent being assessed by Doris to assess my arm and leg movement and strength; the number of sit-to-stands I can complete in a minute; standing on both legs for 60 seconds; standing with one foot in front of another for 30 seconds; doing a butt raise from a lying position (first, using both feet on the treatment bed, then my right only, then my left only); turning 360 degrees on the spot (essentially, a pivot); walking a few metres, rounding a cone and returning to my starting point; seeing how far I can walk in six minutes.
There’s no pass or fail – it’s setting a baseline for future testing and as a comparison against what “normal” people can expect to achieve.
At 2.00pm, I go to sit in the courtyard, expecting a call from a woman at the Royal Rehab, a rehab centre at Ryde that can provide in-house and telehealth physio services for people who need ongoing physio after hospital discharge. With luck, they will be able to help in about two weeks time, for about six weeks. I should find out early next week.
I head back to my room about 2.50 and I overhear Kevin telling someone on the phone that his NDIS package has finally been approved, “all $250000 worth” as he keeps saying. And, on top of that, a respite care place has been found for him, beginning on 31 July. He will stay there until all the NDIS work has been completed at his unit. And, before he leaves here, he will be sure to give selected “f..king c…s a gobful”, as he threatens to do on a regular basis. At 3.00pm, my OT Natalie comes to my room to hand me some ongoing upper limb exercises that I can do at home. She runs through each of them to make sure I have no issues with them. After that, my time is my own.
The usual nothing much happens for a few hours. About 10.00pm, one of our favourite nurses, Mary, reports for duty and checks in on us. She says she will be back very shortly to take my obs before I fall asleep. Twenty minutes latter, she pops her head around my curtain and says “kaboom” so gently and quietly that I feign surprise and say, in a monotone voice, “Oh no Mary, don’t frighten me like that”. She giggles, takes my obs and then cheerily says “good night” to Kevin and I.
Love and peace to all. � � ❤
21 July 2021
Rehab – Day 34, Tuesday, 20 July, 2021
The bin liners get changed each day, usually 10-15 mins before breakfast arrives. It’s never a quiet process, as the bin liners are made out of that crinkly, noisy type of plastic. Some of the cleaners are more considerate than others – the blokes are noisier than the women, banging the bins, slamming the bathroom door.
At 9.00am, I get a phone call from Gabriella, who works for a service called Compacks. They provide volunteers who can help you out at home for six weeks after discharge, providing services such as meal prep, vacuuming, washing up, showering, dressing, grocery shopping, transport to medical appointments.
Then off to my final Balance Bootcamp, where I choose my final playlist for the workout: “70s Essentials” on Apple Music. Previous ones have included “80s Hit Essentials”, Sgt Peppers Lonely Hearts ClubBand in Spatial Audio. Eileen wishes me well for tomorrow.
OT Group isn’t any easier than previous days in the last week. Michelle, one of the OT assistants, tells me I have done amazingly well. I’ve heard this a few times over the last couple of days.
After lunch, it’s back to the gym so that Doris and Sonia can run me through some of the exercises that Doris has prescribed for me for home rehab – several of them are new to me. And, just like that, my formal in-patient rehab program is over.
But then Doris tells me that I am going to be referred to the newly established out-patient rehab clinic in the downstairs gym here at Graythwaite, with the plan being for me to attend on Thursday and Tuesday mornings for the next six weeks. This opportunity is out of the blue and unexpected. And it starts this Thursday!
Back in my room, I think about packing my stuff up. My OT Natalie turns up to ask if anyone has mentioned the possibility of coming back for out-patient rehab – the staff seem more excited than I am about this. “And the taxi vouchers?”
For my last hospital dinner, I opt for the microwaved roast chicken and veggies. And then I manage to pack most of my belongings in readiness for tomorrow’s departure. By the end of that, my left shoulder blade hurts badly and the soles of my feet feel like razor blades are protruding from them.
I collapse into bed.
Love and peace to all. � � ❤
23 July 2021
Rehab – Day 35, Wednesday, 21 July, 2021
Breakfast arrives late. No matter – I am heading home today.
Kevin heralds the new day with his usual flatulation salutation – a double burst!
Breakfast finished, I get on with finishing my packing. Not much left to do and I’m grateful that I had done the bulk of it the night before. I am scheduled to be collected by patient transport at 10.00am but there is no guarantee that they will turn up by them. I was scheduled to be picked up by them at 5.00pm when I was being transferred from Royal North Shore Hospital to Graythwaite Rehab Centre way back when but they didn’t arrive until about 7.15pm.
Just after 9.00am, one of the doctors managing the Day Rehab program arrives to assess me for admission to the program and I pass with flying colours! She goes off to complete the paperwork, as I must be referred to, and accepted into, the day program before I am discharged – talk about in by the skin of my teeth…and they will be issuing cab charges for me to get there and then back home. This is a great relief to me as I will have access to all the resources I had whilst I was an inpatient but on a different floor, and is highly preferable to in-home rehab. I am going to be just the second patient to start in this program and will be doing so the day after being discharged.
Nicole, one of my favourite day nurses, asks me what time I am being picked up. I tell her and she goes off to check for any updates. When she comes back, she tells me that no booking has been made. Aah! She goes off to make a booking for me – so now, it will be 2.00pm.
With nothing better to do, I head off to do OT group, even though I wasn’t scheduled to do so.
Back to my room for lunch (I almost hadn’t ordered any as I was “supposed” to be picked up at 10.00am). I then relax, watching tele until my transport arrives. I approach Kevin “C’mon…what’s your number, then?” He’s clearly chuffed that I’ve asked. He’s a bit worried about who my replacement will be as a roommate. “If I don’t like them, I’m gonna punch ‘em”.
At a bit before 1.30pm, the patient transport crew arrive. Say goodbye to Kevin and Nicole and 10 minutes later, yell out goodbye to the physio team as I pass by the gym and, ten minutes later, I’m strapped into a bed in the back of the van and am on my way home
Post In-house Rehab
Post-Graythwaite Rehab Centre, Day 1, (Day 45 post-stroke), Thursday, 22 July, 2021.
After a cold night at home (no central heating, or much heating at all, in our unit), get up at 7.15am to prepare for my first day at out-patient rehab back at Graythwaite Rehab Centre – this time on level GR4, not GR5 where I was domiciled as a patient.
Josie and I get an Uber at 8.15am and arrive at 8.35am at Graythwaite. A notable drive for a few reasons – normally, at the time of day we went, it would have still have been morning peak hour but today there were relatively few vehicles on the road; the trip would usually take 35-40 minutes (depending on traffic volume and average speed of traffic) but today, only 20; and today was the first day I had to use my new technique of getting into a car (backward approach until I feel the car on the back of my calves, reach out to the B-pillar for support then place bum on seat and swing around).
After signing in at GR4 reception, I am met by the out-patient nurse, Soo Jing, to have my obs done and to check if there are any issues that I might need attending to – yes, there are: gut has not been happy since stroke (no matter what I eat, it grumbles and rumbles, it feels queasy, and, at times, it is painful); stools are a mustard colour (not usual for me); soles of my feet are painful most of the time; I’m concerned about my diet – what modifications do I need to make to help with tummy issues and to minimise weight gain whilst I can’t do very much walking; constant tinnitus and whooshing noises in my head (consequence of decades of migraine with aura and, perhaps, related to white matter disease); pain management (nature of headache changed following my stroke) and there is tightness and pain in my left hand and, to some extent, my right; and, of course, pain from rehab exercises (especially in my left shoulder blade and my right hip).
After that, I spend an hour with the physio Sharon, who I am familiar with from my time as a patient. Assessments and exercises ensue, including one where I had to stand on one leg for three minutes and tap the top of a 15-20cm high plastic cone with my opposing foot (then repeat with the other leg). An hour off (I dine on egg and lettuce sandwiches, a small orange juice, and a tepid black tea with one sugar – sound familiar?).
From 11.00-12.15, I am assessed by the OT Kate – can I do this, that, and the other, and at what level of competence? Try a few upper limb exercises and it becomes apparent why my left shoulder blade gets so sore – my left shoulder is quite a bit lower than my right (partially a consequence of my stroke and partly a result of ongoing poor posture) and some of the shoulder muscles are not engaging properly (or at all).
A taxi arrives to take me home – it’s an older style Kia Carnival with a double- step entry into the rear door. I wasn’t trained for this type of entry but I managed to haul myself in somehow without injuring myself. As we’re driving along, the driver asks me to log in using the QR code and I quickly realise that my new phone doesn’t yet have a QR scanner. I scramble to find one using my one good hand whilst the taxi swerves through roundabouts and rumbles over rough patches of road. When we get home, I ask him to park across the driveway to make getting out easier – instead, he reverses up the driveway and parks on an incline to make my exit more challenging. My left leg almost collapses as I get out of the taxi, due to the effects of the morning physio. I hobble up the rest of the driveway to the foyer door and press the buzzer to our unit and Josie lets in. I’m happy to get inside and slump into a chair.
Looking forward to an early-ish night and sleeping in the next morning, for the first time in over six weeks.
Love and peace to all. � � ❤
7 August 2021
“So, Pat, how you going there, now that you’ve been back at home for a while?”
“Huh? Oh, hey Horatio! Where have you been?”
“Well, you know…here, there, nowhere in particular…”
Yes, it did take a while to become accustomed to being back home, especially as my body is not in the same condition and state of health as it was before going into hospital.
There is much that I miss about the rehab centre – the expertise and encouragement of the rehab staff; the routine of my daily schedule; the skilled and very helpful nursing staff; the shared experience of doing daily balance boot camp with other “strokies”; the infectious Doris; Kevin, for the Minties, toe tickling, yelling at noisy staff and patients to “shut up”, the daily updates of COVID testing numbers next door at Ryde Hospital, his companionship during our lockdown within a broader lockdown, the farts (yes Horatio, perhaps not those).
I need to self-motivate much more at home, to ensure I keep up with my rehab exercises but it is taking me a while to set up a regular routine. But, even if it’s not the full routine, I make sure to do a few exercises every day, including a 20-30 minute walk that includes twice crossing the five-lane road we live next to, stepping up and down kerbs, walking on footpaths, traversing the uneven grass surface (including twigs and stones) that comprise ANZAC Park, unsteady inclines – all challenging my strength, balance and endurance. This week I averaged 1, 863 steps per day. It helps a great deal that I return to Graythwaite two mornings a week for a couple of hours of out-patient rehab, done with therapists with whom I am familiar from my time as an in-patient (alas, Doris is not one of them). The main difference between in-patient and out-patient rehab (apart from the frequency of sessions) is that the out-patient sessions are done one-on-one in disused patient rooms, not in a gym.
And I have a regular taxi driver who ferries me to and from the venue – Adel, The Taxi Man (“yes, Horatio, that’s what is written on his business card”). I commented on how neat and clean his taxi is on the first drive – it’s his own car and only he is allowed to drive it. I now text him the day before each session to arrange a pick-up from him.
My ability to grip and lift a plastic bottle with 120ml of water in it, to my mouth, with my left arm, is much improved. And I am also now doing elbow bends with my left arm whilst gripping a 500g weight. I am showering (in a chair), drying and dressing myself, and using the toilet without assistance, which I had been doing for two weeks prior to discharge (“yes, Horatio, number 1s and number 2s”). I make my own porridge (with honey, nuts, seeds, fruit) and at other times prepare my own Weetbix or toast, and usually make my own tea (“yes, Horatio, with lactose-free skim milk and boiling water”).
I’ve seen Kevin a couple of times (he often passes the time sitting in the downstairs foyer, chatting to the nurse who greets and screens anyone who walks in). Work has commenced to make the appropriate changes to his residential care unit and he hopes to move back in by the end of this month. And I no longer pee in bed during the night (“okay, Horatio, you know what I mean”).
Disclaimer: I am not a medical doctor nor do I have medical training of any kind. What follows is my understanding of the arteriopathy of CADASIL and the arteriopathy caused by hypertension. I did, however, discuss my understanding of this issue with my CADASIL neurologist before posting.
Persistent high blood pressure levels are potentially dangerous, leading to an increased likelihood of developing cardiovascular disease that can lead to heart attacks, heart failure, stroke and other unwanted health issues.
I live in Australia and the Heart Foundation here states that any blood pressure (BP) reading from 140/90mmHg upwards is high.
In the USA, however, their Heart Foundation definition of hypertension was revised in 2017 (as reported here by Harvard Health) as follows: hypertension Stage 1 (130-139/80-89); and hypertension Stage 2 (140+/90+).
So When Does High Blood Pressure Become Hypertension?
A one-off high blood pressure reading does not mean that you have hypertension.
Blood pressure levels fluctuate throughout the day and it is possible to have temporary mildly elevated to highly elevated levels, e.g., during a period of high emotional stress; during intense exercise. This is normal.
What is it about hypertension that heightens the likelihood of developing the cardiovascular issues I mentioned earlier, and why is it so undesirable in those of us who have CADASIL?
One of the most significant and problematic of the many features of CADASIL is arteriopathy (disease of the arteries). The consequence of that arteriopathy, especially in our small arteries and arterioles, is that, over time, the blood vessel walls progressively become thicker and stiffer. This results in a narrower lumen (the bit through which the blood flows) and much less compliant (elastic) blood vessel walls, meaning they will no longer be able to dilate and contract to accommodate changes in BP levels. And in CADASIL, this happens in the absence of hypertension. So, behind the eight ball already. And long term, ongoing hypertension – in the absence of any other disease factors – leads to exactly the same result, although the mechanisms involved are not the same.
In hypertension, the process of thickening and stiffening of arterial walls is called arteriosclerosis. When it happens in arterioles, it is called arteriolosclerosis (note the slight difference in spelling). And the arterial damage that occurs in hypertension is unable to be reversed (as is also the case in CADASIL).
Arteriosclerosis and arteriolosclerosis are not to be confused with atherosclerosis.
Arteriolosclerosis is a structural change in the affected blood vessel walls of arterioles as a consequence of the sustained pressure of hypertension whereas atherosclerosis is a result of plaque build-up inside the blood vessel.
The scale at which arteriolosclerosis happens is minute – unimaginably tiny, especially for the terminal arterioles where blood is dispersed into capillaries.
So how tiny is tiny? One µm (micrometer or micron) = 1/millionth of a metre or 1/thousandth of a millimetre. Hard to imagine that something so narrow could have any meaningful structure let alone be able to transport blood. The image above shows arterioles vary in width from 10µm to 30µm (and check out the width of the arteriolar walls!) Compare these widths to: human hair (17 to 181µm); paper thickness (70 to 180µm); wool fibre (10 to 55µm); cling wrap (10 to 12µm); and, spider’s web silk (3 to 8µm). See Micrometre.
What To Do?
Don’t add fuel to the fire. If the pathology of CADASIL means that the walls of our small arteries and arterioles are going to thicken and stiffen anyway – effectively aging them prematurely – do not give them any further reason to do so.
If you have hypertension or are pre-hypertensive, start NOW to make the changes needed to get your blood pressure levels back to as close to optimal as possible. If you are overweight, especially around the tummy (Oo! Oo! Pick me!), drop a few kilos/pounds; get rid of the salt (read those food labels, and drastically reduce how much you add to food); if you smoke, STOP… RIGHT NOW (well, ASAP). Smoking contributes to arterial damage (it’s not just bad for your lungs). Read here for other cardiovascular risks that arise from smoking and a list of suggestions to help you quit; if you aren’t already, get physically active – yes, my body is in pain all the time too (start gently and, as you feel able, work up to moderate intensity – see Physical Activity Guidelines); talk to your doctor about starting, or modifying, blood pressure medication – there are plenty of options available; reduce exposure to, and manage, stress – plenty of options here as well; get enough sleep – easy to say, I know…I don’t get enough myself, in terms of quantity or quality; cut back on the alcohol – but you don’t need to get rid of it entirely. See High Blood Pressure Treatment.
None of this is easy. We are creatures of habit and changing the habits of a lifetime can be bloody hard! You’ll procrastinate initially; you’ll make false starts; you’ll doubt yourself; you’ll feel physically and emotionally horrible at times; you’ll get frustrated and angry.
Persist. Know that others will accept what you are trying to do and why. Know that others have been down the same path and understand what it feels like.
Be easy on yourself. Forgive yourself. Have faith in yourself.
We all have a notion of what the word “rare” means. “Very few”, “hardly any”, “almost none” and similar phrases come to mind.
To place it in some kind of context, I find it helpful to think about the status of certain species of wildlife – are they abundant? Common? Uncommon? Threatened? Critical? Rare? Or, tragically, extinct?
And yet, as it turns out, when it comes to diseases, asking what is rare happens to be a reasonable question.
So, what is a rare disease?
Well, there is no internationally accepted or agreed definition of the term “rare disease”. Yes, you read that correctly.
Even the terminology itself isn’t a universally agreed one.
And it even gets a little more complicated than that, as some countries determine what a rare disease is based on a prevalence rate (<1 in 2000 people in Australia and the European Union) and others as an absolute number (<200 000 people in the USA).
When my mother was diagnosed here in Australia with CADASIL in late 1998 (and the disease name ‘CADASIL’ only came into being in 1996), our family was told that the disease is rare and affected about 400 families around the world. Using any of the prevalence rates mentioned above, that made CADASIL more than rare – that put it in the category of ultra-rare or super-rare! At the time the United Nations estimated the world population to be 5.9 billion, so 400 families represented a mere speck inside a drop in the ocean.
In 2006, the 400 families figure was still being quoted and even again as recently as 2013 (see second paragraph under “Discussion”).
Estimations of Prevalence
Attempts to estimate prevalence in specific regions, including the west of Scotland and the north-east of England, have been made over the years but getting a better global estimate has been elusive.
The rare diseases online portal Orphanet states that the prevalence rate of CADASIL in Europe is somewhere between 1/50 000 to 1/25 000 people (information checked online Friday 3 August 2018).
The estimate is based on only four different studies, including the two mentioned above in Scotland and England (all of the relevant studies are cited in the 2017 paper), evidence that there have, indeed, been very few attempts to establish the prevalence of CADASIL over the years.
Note also that the 2017 paper states that there are now known to be thousands of families, amongst a diverse range of ethnicities and countries, affected by CADASIL.
Whatever way you look at it, CADASIL is rare but it is certain that it remains underdiagnosed and, therefore, the prevalence rate may be higher than it is currently thought to be.
Today sees the launch of a new CADASIL Facebook group for people living in Australia, New Zealand and the wider Oceania region.
To say the least, CADASIL is a challenging disease to live with, for anybody and no matter where you live. In our part of the world, it is exacerbated by the “tyranny of distance”. I hope this FB group helps to shorten those distances.
As I’ve said in an earlier post, finding out I had CADASIL was no surprise. My Mum had been diagnosed several years earlier; her mother almost certainly had it, having had many strokes in her lifetime, with obvious deterioration in her physical and cognitive health over that time; and I had been having migraine with aura for too long already.
The first few months after my diagnosis, nevertheless, saw me feeling despondent, more depressed than usual, lacking enthusiasm. I felt vulnerable and void of hope, apathetic, listless. The migraines kept on coming.
When I had a meeting to tell my manager about my confirmed diagnosis, he asked “is it genital”? I laughed. Hard. Close to the point of crying. He quickly corrected himself – “I meant congenital”.
Work was as busy as it can ever get at the Australian Bureau of Statistics. We were only a few weeks away from conducting the 2006 Census of Population and Housing. It’s a mammoth undertaking – the ABS had employed over 30 000 temporary workers to deliver, follow up and collect the Census forms. In the days immediately before Census night, calls to our National Information and Referral Service had escalated tenfold, maybe more.
I wasn’t as attentive, or as present, as usual. My mind was elsewhere, I was completely self absorbed. I was struggling to “keep it all together”. I kept volunteering on a weekly basis at my daughter’s school, helping with learning to read and doing maths. Most of the time I didn’t feel like going but was always glad I did. The migraines persisted.
I had been obsessing about CADASIL since not long after Mum was diagnosed and I had become familiar with the symptoms that come with it. My migraines with aura were especially troubling, both in terms of their horrid effect on me and the omen they represented in terms of my possibly having that shitful disease. My anxiety shot skywards and I wondered every day for seven years if I might have it before deciding – finally – to be tested.
To be told for certain I have CADASIL felt like being told to “get my affairs in order”, or something like it anyway. I kept obsessing for some time after being diagnosed.
One day, I realised I hadn’t thought about CADASIL at all during the previous day. It sort of coincided with (I think) a subconscious realisation that my condition hadn’t worsened. I became aware that CADASIL existed on a spectrum, in terms of symptoms, clinical effects, quality of life, severity, life expectancy and more. I’m towards the benign end.
My days of not thinking about CADASIL extended into weeks and, sometimes, months. Over the years, my diagnosis moved from being an anvil slung around my neck to being a source of reassurance. That sounds odd, I know. But I had come to appreciate the certainty it provided. My symptoms were explained – I no longer had to search for an answer. And once it was obvious I have a benign form of the disease, I accepted that it is part of me. I don’t like that I have it but I accept that I have it. (Although, I don’t think I would be so accepting if I had a more aggressive form of it.)
Eventually, I woke up one day and thought to myself…
CADASIL Brochure jointly produced by the Neurogenetic Outpatient Department of the Grosshadern Clinics and the Memory & Aging Program at Butler Hospital
Mid-morning of Friday, 16 June 2006, I was sitting with my wife in the waiting room of the Royal Ambulatory Centre at John Hunter Hospital (JHH) in Newcastle, New South Wales. It is a large waiting room, with seemingly endless consulting rooms running off to the east and west. Patients and their partners, families, friends sat in their allotted areas, some quietly, some speaking in hushed tones, some with kids being kids. A small line of outpatients waited to be processed at the reception desk. Medical specialists drifted in as the time came to see their first outpatient of the day.
Glass formed almost the entire wall on the northern side of the room, overlooking the suburb of Lambton and then taking in sweeping views that spread all the way to Port Stephens, a stunning coastal region and holiday destination about an hour’s drive to the north-east. Cumulus clouds were scattered over the wide sky, drifting gently to the east.
My wife and I looked toward where the voice had come from.
Thirty seconds later we were sitting in a spacious but otherwise unremarkable consulting room. My stroke neurologist – Professor Christopher Levi, Co-Director of the Acute Stroke Unit at JHH and Director of Clinical Research and Translation for Hunter New England Health – was sitting at a corner desk, near the door, on which a computer monitor was displaying MRI brain images – my brain, I suspected.
If I was going to get unwelcome news about my health, I’d want it to come from this bloke – he is engaging, respectful, personable, has a great “bedside” manner and knows his stuff, right on top of his game.
He summarised how we had jointly got to this point. My Mum’s diagnosis seven years earlier, following an otherwise inexplicable stroke; my sister’s mysterious case of catatonia following the birth of her fifth child; the severe migraines that came on after the births of all her children; the family consultation we had had with Chris not long after that.
During that consultation, my decision to get tested was instant – no hesitation; no need to think about what it might mean; no need to see a genetic counsellor (I worked then at an Obstetrics and Gynaecology lab who had genetic counsellors to report increased risks of birth defects to pregnant patients).
For several years, I had been living as if I did have CADASIL anyway. I had obsessed about it – thought about it every day. I was getting no peace of mind. The stress was unrelenting. My concentration was waning, my moods were vascillating, I was more irritable and depressed. And, if it was at all possible, even more self-obsessed and selfish.
Migraine with aura had been plaguing me on a regular basis and, as best as I could tell, I had no triggers that set them off. Beyond that, I had a gut feel that a 50/50 chance of having inherited this disease hadn’t worked in my favour – my other sister and my brother didn’t get migraines, and they were more even-tempered than me, my mother and the elder of my two sisters. The last point is irrelevant to CADASIL but I saw it as an omen.
The exact words elude me after all this time. “Patrick, as we suspected, you do have CADASIL.” “Well, Patrick, the blood test does confirm that you have CADASIL.” “As you know, Patrick, your MRI was strongly suggestive of you having CADASIL and…”
Even though the news was completely expected, there were tears. I knew this result was coming but, truly, expecting such news, and receiving confirmation of it, are worlds apart. My wife grasped my hand and shared my tears.
What occurred throughout the remainder of the consultation is long gone, long forgotten. My wife and I returned to our car and set out on our return trip to Sydney, where our six year-old daughter was struggling to make friends at school and my workplace at the Australian Bureau of Statistics was in final preparations for the largest peace time operation in Australia – conducting the national Census of Population and Housing.
The world didn’t look any different than it had when we had driven up that same freeway a few hours earlier.
As a child aged 10 or 11, I recall having a severe headache that kept me in bed for a couple of days. It may have been a migraine, it may not. Aged 26, I had another severe headache. This time I knew it was a migraine.
I had been home for a quarter of an hour or so, after a 30 minute walk from work. I started getting a very mild tingling at the ends of my right fingertips and it began to move slowly up through all of my fingers, then the rest of my hand and up to my wrist. By the time the tingling had reached my wrist, the lower half of my fingers had recovered their normal feeling.
Within minutes, this wave-like pattern of tingling, accompanied by moderate muscle tightness, had rolled up my entire right arm in a slow pattern of constriction and then release. It was like a narrow band of a weird sensation scrolling up my arm, no more than a centimetre or so in width. Tighten, release. Tighten, release. And all the while crawling with determination towards my shoulder.
On reaching my head, it worked it’s way from the top of my scalp down. When the tingling reached my nose, it felt liked I wanted to sneeze but couldn’t. Aah, aah…aah, aah…
The right side of my lips and tongue felt huge, swollen as if a dentist had needled anaesthetic near my back molar. I was sure that if I looked in the mirror at that point, I would look grotesque.
Instead, I rang my wife at her workplace. I couldn’t speak properly; pronouncing words incorrectly and struggling to find the appropriate words.
The movement through my neck felt strange. Was this what it felt like to be “lightly” strangled, by a one-handed strangler?
The tingling continued with an unerring rhythm – crawl, tighten, release; crawl, tighten, release. Feeling it progress through my chest and stomach, affecting only my right side…was…unnatural.
By the time the tingling could progress no further, and it exited my big toe, 30 minutes had gone by. Not long after, the pain hit. And it was unbearable.
So began my decades-long, intimate relationship with migraine with aura. I soon came to learn that what I had experienced was a one-sided parasthesia and they went on to become a familiar part of my migraine routine. I even had a few occasions where I experienced double parasthesia within a 24 hour period. And parasthesia with no following pain. And “incomplete” parasthesia that aborted half way up my arm, or went no further than my fingers. Almost always, they afflicted only the right of my body.
Over the years, my migraines have lasted from four or five days up to two weeks, from the prodrome to the postdrome. They have varied in pain from 0/10 (the silent migraines) to 100/10 – yes, that’s not a typo. They have been throbbing, searing, booming, pounding, sharply and piercingly focussed, broadly spread and dense, like an impenetrable fog.
Our relationship continues…but we don’t see each other as much as we used to…
As I drove from Sydney up the F3 freeway in late spring on that November day in 1998, my almost five-month pregnant wife sat quietly next to me. It wasn’t entirely quiet though – we were listening to a pop radio station and traffic regularly passed us by in its rush to get who knows where.
I had driven up and down this route many, many times. Today the weather was fine, the amount of traffic was moderate and the driving was not stressful, unlike Friday nights and late Sunday afternoons when, respectively, people flee Sydney at the end of the work week and grudgingly return as late as possible on the Sunday.
It was a straightforward, if somewhat boring drive that day.
I wasn’t sure what to expect when we arrived at John Hunter Hospital in Newcastle, a couple of hours after leaving our home in Cammeray, on the lower north shore of Sydney. I’d received a phone call (was it from my Dad? was it from one of my sisters?) to tell me that Mum had had a stroke.
Not long after being admitted to her small local hospital in Scone, she had been transferred to the John Hunter for more specialised care in a stroke unit.
The Hospital
After an uneventful drive, I parked the car in the back parking lot of the hospital. I felt my body tense up a little. Anxiety? Anticipation? A consequence of being stuck in the same position for a bit over two hours?
You see, Mum and I had a fractious relationship. We were both volatile, quick to anger with short tempers, hopeless at expressing and showing our love for each other and, on social issues, we were miles apart. My wife being pregnant out of wedlock was a source of anxiety for her.
As we walked to the back entrance of the hospital, we passed two or three very pregnant woman standing nearby, slightly apart from each other, smoking. Mum had been a smoker for a long time, as had Dad until the result of an on-the-spot cholesterol test at a school fete a few years before led to him quitting alcohol and smoking, cold turkey. But Mum had never been a heavy smoker; just a few times a week and never more than one or two at a time. As for drinking, barely any.
We entered Mum’s room and found her propped up in bed, surrounded by other members of the family. She was very red in the face, seemed a little confused and disorientated. She could speak and, from memory, had no major deficits from the stroke. All things considered, she didn’t seem too bad – not great but not too bad.
Unexpected and Unwelcome News
Her staff neurologist was unable to explain why Mum had had a stroke. A few days later, he mentioned she might have CADASIL. She might have what?
It took a long time to remember what that acronym stands for, let alone what it means. I soon learnt that it is not a good disease to have, that it is rare , and that me and my three siblings had a 50 per cent chance of inheriting it when we were conceived. It also seemed to mean that, if we also had it, we would have an early death.
CADASIL - My Journey With A Rare Brain Disease 