Coping with Marfan Syndrome, Heart & Lung Failure, Visual Impairment, Chronic Pain and a Few Other Problems
By Greg
If you find this post useful or interesting you might want to check out two other posts: Marfan and Other Chronic Conditions – Things That Worked for Trenna; and Things That Worked for Trenna -Part 2.
As the name suggests yet another instalment in a series of posts that tell the things Trenna tried in an effort to make her or my life a bit more bearable. As with the others, the tips and tricks won’t work for everyone but hopefully there is something that you find useful, or at the very least, interesting.
And as a reminder of the sorts of medical issues Trenna was coping with
In her last several years she was coping with
- deteriorating eyesight – she was legally blind
- chronic pain and the effects of medication to treat it
- atrial fibrillation
- arthritis
- muscle weakness
- heart failure
- lung failure (COPD)
- skeletal problems, including scoliosis
- esophageal dysmotility
- primary Sjogren syndrome
- dural ectasia
There were many other things, but these were the most debilitating.
The Lemon Meringue Pie and Cream Diet
In Trenna’s later years she had trouble putting on and retaining weight. Trenna’s sister Nancy mentioned she likes Lemon Meringue Pies, but she has to watch her weight.
The Lemon Meringue Pie Diet was created! Each night, 7 days a week I would heat in the oven a frozen lemon meringue pie, and then serve it up with a half a tub, yes, about 100 mls (more than 3 ounces) of “Dollop Cream”.
It absolutely worked to help put weight on. My guess is that the large amount of cream made the pie less tart (if you know what I mean) and also contributed a lot of the calories.
I have written an entire separate post on gaining weight for people with Marfan Syndrome, it is here.
Podcasts at Dinner Time
Trenna an I made a point of not eating in front of the TV. We would almost always sit at a set table to enjoy our evening meal. We had some great conversations, and also kept informed of what the other had been doing and thinking. I recommend it to those who don’t already do that.
However, as heart and lung failure progressed she found it harder to eat AND talk. So, we started to listen to podcasts played on a bluetooth speaker. We both had a shared experience, but it was less arduous for her.
We tended to go for true story series. I know we both very much enjoyed “S Town”, “The Story of the Big Day Out”, “Missing Richard Simmons”, a few seasons of “Serial”, “Tom Brown’s Body”, “The Eleventh”, a few seasons of “Unravel”, and also “Dr Death” (but some of those scenarios were a bit too close for comfort).
Small Knob on a White Cane
I’ve mentioned the long white cane before as being useful for alerting people to stay out of your way. When Trenna was being instructed in the use of the cane she tried a variety of knobs at the end of the cane.
She was convinced that a small one was best for her. I mention it here just so that everyone (who needs one) are aware that there are a variety of sizes of cane tip, and you should experiment to find what suits you.
Clothes Airers Instead of a Clothes Line
Trenna was the main clothes washer for most of our time together. But quite a few years ago she realised just how hard and painful it was for her to reach above her head to peg clothes on a clothes line.
Yes, we had a clothes dryer, but she and I were conscious of the environmental, and monetary cost of using it when it wasn’t necessary. Instead she got a few clothes airers – fold up stands – to hang the clothes on.
Incidentally, for towels she would wash them on Monday, put them on an airer, indoors, and then on Tuesday morning she would give them half an hour in the clothes dryer to finish them off and make them fluffier and soft – it worked well.
Evidence Based Medicine
Everyone has their opinions on alternative medicines and treatments. Although Trenna was open minded she was very much a proponent of evidence base medicine. Treatments that had been scientifically studied and proved to be effective.
Keeping a Water Bottle Visible at Home
I’ve mentioned the importance of staying properly hydrated in an earlier post. Trenna was a huge fan of that and would get a large refillable bottle of water out of the fridge each morning and leave it on the kitchen bench where it served as a reminder to drink.
Weather in Perth tends to be quite warm for a good part of the year so we usually had two bottles on the go. One on the bench, and the other in the fridge waiting to be swapped over.
In the evening, after dinner she would take the bottle into the lounge room so it was on hand whilst she watched TV.
Someone Coming and Preparing Meals
Eventually it got to the point where Trenna couldn’t do preparation for the evening meals. She was always conscious of my time constraints, especially when I was working full-time.
As an alternative to buying meals that had already been prepared, or using the companies that give you all the ingredients and a recipe we did get some people to come into our home and cook meals in our kitchen. She felt she had more control over the process and what went into the meals.
We tried it with both people coming in and using Trenna’s recipes, and also people coming in and doing pre-agreed recipes from their list.
I think it is worth considering if you find you can’t cook, but still put a high value on the process. As it turned out, Trenna was never really satisfied with the interlopers, and once I retired I did almost all the cooking.
An extra tip, if you are on the Australian National Disability Insurance Scheme (NDIS) they may well pay for the cooking service (but not the ingredients).
Veneers on Front Teeth
Trenna’s overcrowded mouth and Marfan high arched palate caused havoc with her teeth as a child and teen. By the time I met her her teeth looked OK but she did have one front tooth that had the corner missing.
Not long after we met Trenna started going to the dentist at the end of the street in which we lived. Rosemary the dentist suggested veneers on the two front teeth. They were fabulous, great for Trenna’s self esteem, and caused no problems at any point.
Electric Soap Dispenser
We never actually got this one, but it was recommended by Ruth the OT. Trenna used sorbelene instead of soap for all but a few body parts when having a shower.
There are battery operated electric soap dispensers that can dole out sorbelene or liquid soap. Trenna and I both thought it was a good idea to get a couple, we just never got around to organising it.
Not Leaving Retirement Too Long
I’m not saying I have regrets, but I probably did leave retirement a little too long for our particular circumstances. There were lots of factors in that decision including Trenna wanting to make sure that we would be financially secure in retirement, and she wasn’t quite sure I wouldn’t drive her mad being around the house the whole time.
As it turned out, those four or so years we were together almost 24 hours a day were very special.
When I first retired I started trying to organise one last European holiday together, but as soon as we started to look at the details it was apparent we had, in fact, left it too long. By then it wasn’t feasible for Trenna to go on an overseas trip.
Are you are trying to decide whether to leave work and look after, and be with the one you love? It’s a no brainer – be with the one you love.
Tenacity and Perseverance
I think some of Trenna’s most impressive qualities were her courage, her tenacity and her perseverance.
If she just did what she could easily do she wouldn’t have done anything for the last 10 or so years of her life. On one occasion at the cardiologist Trenna remarked on how tired she got. The cardiologist said said “well, most people in your position would have given up years ago!”
She really tried to keep going, and I was so grateful that she did. Things like the Rug for Jack and Jade, and the one for Zara took SO MUCH effort, but she could see that it would be worth it.
I don’t think anyone could blame someone who had a chronic illness, and who knew that ultimately there would be only one outcome, for giving up, or for losing hope.
Trenna didn’t take that route, and I know she felt better because of it.
Neck Support/Travel Pillow for a Plane, Car or For Watching TV at Home
Trenna got one of those neck supports people use on long trips. Her particular one was a foam one, rather than the inflatable ones that are also popular.
She did use it when travelling, but she far and away got the most use out of it by using it each night, sitting on the sofa watching TV. She would frequently fall asleep watching TV and the neck pillow stopped her falling in an uncomfortable way.
Hairdryer on Standby for Spills
If you can’t see, it is inevitable that glasses or bottles of water will get knocked over. As Trenna’s eyesight deteriorated this became a more common occurrence and Trenna was getting quite upset about the inconvenience it caused.
As it happended we had a spare hairdryer in the house. I designated that one as the one for helping clear up spills, and I also had a spare, old towel on hand.
After that, because we were prepared it didn’t seem like such a big deal. It didn’t actually diminish the amount of work to right things, it just felt much more manageable – we were in control.
Travel Insurance
I’m putting this one in just in case people with “pre-existing medical conditions” as the insurance industry likes to call them are put off from applying for travel insurance.
We travelled overseas together many times and were always 100% honest when applying for travel insurance. Our experience was that there is additional paperwork and a, quite small, additional premium but then you are covered for most eventualities.
We always felt it was worth the effort. Here is one story where we got a pay out from our travel insurance.
ACROD Permit (and Finding What Added Benefits There Are)
In Australia a disability parking permit is called an ACROD Permit. It is of course invaluable for those who need it. If you are eligible you can organise it through your doctor.
However, the benefits don’t just stretch to being able to park in the blue and white parking bays with a picture of a wheelchair on them. Some municipal councils give you additional time for your parking, or free parking or other benefits.
Some other countries also recognise Australian (and presumably other countries) ACROD permits, so it might be worthwhile taking it with you when you travel.
Do your research. It is already invaluable, but it might have more value than you think!
Companion Card
In Australia, and I’m guessing elsewhere, the carer of a disabled person can often get a Companion Card. This allows the carer to go to concerts, sporting events, cinema and theatre, exhibitions and all manner of attractions for free. Even the Australian Grand Prix!
It is a system that works in the best interests of everyone so is well worth supporting.
Members of Parliament
Some times you just need a bit of help when working with bureacracies. In Australia that could be the NDIA/NDIS or it might be some other government department or agency.
You should try to resolve those issues without using your local member of parliament, but if you try, and you just aren’t getting anywhere sensible, use them.
A good first step is to ring up the office of your local Member. Their staff usually have a pretty good understanding of the systems and how to unclog them if necessary. Often a phone call is all that is needed for your application/grant/account/request to be brought to the top of the pile, or to be looked at in a different light.
If that doesn’t work, you can make an appointment to meet with your local member of parliament, eyeball them and make your case. It helps if their side of politics is in government at the time, but they can often have success even if they are the opposition.
It also doesn’t matter whether or not they are a member of a party you support – they are there to represent you. And they will be hoping to win your future vote.
In due course I will write a seperate post about this issue and telling the tale of one meeting I had with our MP to speed up Trenna’s NDIS plan review. (Spoiler alert: it got sorted out within a week, after months of waiting).
Oximeter, Thermometer, Blood Pressure Machine
We owned all three of these handy diagnostic devices.
Thermometer. We went without one of these for years but eventually we got one and it did help in either reassuring us, or in some cases, reinforcing our worry! One of the problems Trenna was experiencing in later years was food entering her lungs and causing infections. The thermometer gave a clue as to when this problem was occurring.
Blood Pressure Machine. This was handy for reassuring us that Trenna’s blood pressure was low and not putting too much strain on her compromised blood vessels and heart.
I read once that a big difference in blood pressure between the left and the right arms was a possible sign of Aortic Dissection – something all Marfans think about. If you think you might be having a dissection I’d suggest you call an ambulance or get to an Emergency Department rather than bothering taking your blood pressure.
Oximeter. Even before Covid-19 made an oximeter a handy thing for all households to have, we had one and used it regularly. Trenna’s heart and lung failure meant that this small device gave really valuable information. If your heart and lungs are on the way out, get one of these now.
There is of course a fourth machine that is indispensable for those on anticoagulants, the INR Machine. I talk about it here.
Reverse Cycle Air Conditioner and Solar Panels
There are lots of ways to cool and heat a house so use whatever works for your situation. My point is that living with chronic illness is bad enough without also coping with extremes of temperature.
We made the decision some years ago that we would use as much heating and cooling of the house as we needed to be comfortable. In our last renovation 15 or 20 years ago we fitted reverse cycle, ducted airconditioning (even the garage is airconditioned) and it is one of the best things we did.
In more recent years we fitted solar panels to our roof to generate electricity to help reduce the running costs. Another good move.
Quarter of a Clonidine if on Opiate Patches
This was a real revelation when a pain specialist doctor suggested it. Trenna had Norspan (opiate) Patches that lasted a week. She would put a new one on each Saturday morning.
When she was using the patches (which was all the time once she started) she was sure that I was argumentative and quite obnoxious almost every Friday evening right through to Sunday evening. There was quite a bit of yelling and tears.
I was sure I was no different on those days to others, and it was my theory that it was Trenna’s perception of how I was, rather than how I actually was.
One of us raised this with the Pain Specialist, and he said, “that sounds like underdosing of the opiate patch as the old one runs out and before the new one is working properly”. He suggested that Trenna take a quarter of a Clonidine tablet on Friday night, Saturday morning, Saturday night and Sunday morning.
Sure enough, from then on I was much better behaved on the weekends!
Internal Walk
Trenna liked to go for a walk every day. In the last year or two this proved impossible, but before that she was good at getting out for a walk outside.
However, some days it was just too rainy, or by the time she was ready it was already too hot outside – for her, that was anything above about 26 degrees.
On those days she put on her walking shoes, told me to get out of the way if I was home, and walked through most of the rooms in the house until she had completed a predetermined amount of walking. If the air conditioning wasn’t already on that would be used to make the experience a bit better.
It wasn’t as good as a walk through the neighbourhood but it was better than no walk at all.
If you found this post useful or interesting you might want to check out two other posts: Marfan and Other Chronic Conditions – Things That Worked for Trenna; and Things That Worked for Trenna -Part 2.
Part 4 in the series is already half written and will be published within the next couple of weeks.