I must start by revealing to you (if you didn't already know) that we are all different. That goes from us as people to the disease itself. The range of individuals, their ability to cope with the day to day acceptance of their loss of function and the sometimes enormous differences in the course of their particular form of MD is often confusing and difficult to cope with. However ...... take heart my friends, take each day as a challenge and give yourself (and your carer, bless their hearts) a smile or a grimace of congratulations as you cross each barrier.
My physical data.
I am 74 years old now having been diagnosed at 49 with "Late Onset Limb Girdle Muscular Dystrophy ". I was, and probably still am 6ft 3in tall and around 12-13 stones in weight (although I haven't been weighed for the last 15 years. Prior to my diagnosis I was very active and played sport regularly. I have 5 sons by my first marriage and another son with my now wife for 28 years, Pauline.
My present condition.
My MD is well advanced. I cannot move, save my finger tips, the tips of my toes and my head a little. Amongst other smaller problems, my eyelids don’t close fully (lagophthalmos). I cannot swallow food nor drink and my voice is very weak. My breathing is very shallow and my digestion depends upon gravity alone (with manual evacuation). Basically I cannot perform any individual task without assistance.
On feeding tubes
About 8 years ago I was fitted with a gastrostomy feeding tube. The first was very crude being fitted surgically (from the inside) and consisted of a rubber tube with a perforated ball inside my stomach (very heavy and difficult and painful to change). Now I have a much smaller and light tube from Kimberley-Clark (0100-24). It's about 20cm long and 8mm in diameter. This too has a ball inside the stomach which is deflated for insertion and removal. Pauline can now change this for me at home.
A better option for many is the Button type that lies almost flat to the skin. It has a connector to which is fitted the extension tube for feeding. We chose to have the feeding tube incorporated only because the surgeons fitted my tube right in the main body crease. This makes tending the stoma very difficult. The tube (0100-24) has much more latitude for keeping the stoma healthy as we adjust it to give more distance between the tube surface ring and my stomach wall, though at night we have to stick the tube to my stomach to keep it from moving about.
Over the years we have grown used to our routine. We aim to inject a minimum of 1200 calories per day spread over four feeds (for my level of activity this keeps me very stable). We use a liquid food that gives 1.5 calories per ml of food …. So I take approx 200ml of food four times a day.
Now, the amount of water I take can be a bit variable. I find that it is a fine balance. Too little causes a dryness and tightness in the lungs plus the chance of bladder and other internal infections. Too much causes an excess of mucous and a certainty of mucous entering my lungs …. Not very clever.
We use a mechanical pump to administer the food and water. Each intake of food ends with a 25ml flush. After this I take a variable amount of water, dependent on how I feel (usually between 50 and 80ml) plus another 25ml flush. Additionally if I feel ‘dry’ I start off the feeding cycle with a 25ml water flush.
As you can tell it is all very flexible and this is important because, as I have said before, water is the regulator. So much of ‘feel’ of my digestion will be down to the ‘balance’ of my liquid intake
I also take an acid ‘blocker’. This again is important. So much hinges on regulation of the stomach acid. Acid easily seeps from the stoma causing pain and difficulty keeping the stoma calm and healthy. Acid also easily erupts into and up the oesophagus causing ’heartburn’ and irritation to the trachea and larynx. This can cause over time, significant major problems.
I find that increasingly I have to take my food sitting ‘well up’. As the sphincter valve at the top of the stomach is so weak now that as food goes in, it tends to come up the oesophagus into my throat. Yes, another danger to aspirating stuff into my lungs.
We use a variety of creams to maintain a healthy stoma. A baby cream barrier is a standard dressing supplemented with one of a steroid cream, an anti fungal cream, an anti bacterial cream and an anti histamine cream as appropriate. We have also found it useful to apply the liquid from an antacid medicine to areas of skin when acid seepage has taken place.
On my occupation
My daytime occupation is working on my computer. I construct and manage a few web sites, manipulate digital images and generally "do" things on "my box".
I am writing this missive using an on screen keyboard by "WiViK" which has a word predictor called "Word Q". This allows me to select letters and words etc. using a trackball. My right arm is supported above the trackball and my first and second finger tips move the ball. The right click micro switch button is attached to my head rest and is operated by my left ear. The left click button is operated with another micro switch button under my left thumb tip. This arrangement has gone through some changes but is working well at the present time.
My environment and equipment.
We live in Spain in a single storey dwelling with double patio sliding doors (handy for stretchers and ambulance crew)
I have on loan from my local hospital a food pump and they supply the systems that hold the food and flush water.
The equipment loan company have supplied; a nebulizer, an aspirator and a cough assist machine. They take care of servicing and spares etc.
We have an "Auping Duomat" double bed with separate sides. We use "Tempur " mattresses. My side has motorised leg raising and back raising controls. The other side is similar but manually controlled.
We have an overhead track fitted the full bedroom length running over the centre of my mattress. The hoist a "Whispa by Chiltern Medical" is battery (chargeable) operated. We have a full body sling and a two part strap sling.
We use a light wheelchair/commode for toiletry and showering.
I have a charity supplied wheelchair, a "Y.E.S Puma" with mechanised full tilting and leg raising facilities.
I have tried many cushions, very expensive ones too but finally the one that gives me the longest comfort during my 7-8 hour day is an Air Impulse by "Aquila corporation USA".
Our daily routine.
Our day starts at 7.15am. Pauline clears my sleep filled eyes with a moist ‘wipe’ (that ladies use to remove makeup), clears my throat and mouth with the aspirator and then sits me up by turning me from my side, sitting me forward, placing the two part sling under my thighs and back and lifting me with the hoist. She tidies the bed and lays me back in bed in a fairly upright sitting position (the bed raised and three pillows complete the sitting up positio).
Then it’s time for a pee, make me comfortable and prepare my first food of the day. Whist this is taking place she does a blood test to check my blood/sugar level. Whilst the food goes up the g/tube she injects up the tube my diabetics tablet dissolved (best possible) in about 10mgs of food. This feed takes about an hour or so to complete after which we allow another hour for this to settle. Meanwhile Pauline has breakfast, showers etc.etc. and I do my facial and breathing exercises.
Next I am lifted into the shower wheelchair. First is toilet (manual excavation) then into the bathroom for teeth cleaning, shaving and then showering. That’s all fairly straight forward, ending up with one clean and dry body back in the bedroom. Then it’s the first application of ointment to sore bits, areas prone to fungal attacks and any other skin type problems. Then lift up, back into bed sitting up for phase two of skin attention. Stoma, groins and stomach creases.
Then, dependent on how I’m feeling I might have to clear my lungs with the ‘Cough Assist’ or maybe use the ‘Nebuliser’ if my chest feels tight or dry. Then it is get dressed time and lift into my wheelchair to be up and hopefully ready to face the world by 12 o’clock midday.
Most days I then toodle out into the garden to enjoy the sun, air and our mountain views. At 1 o’clock it’s time for my acid blocker tablet followed my food which I normally take whilst catching up with things on my computer. After food and a time after that to let digestion take place the rest of the afternoon is ours to enjoy. We can go out in the car, work in the garden, entertain guests now and then, well anytime we want until around 6.30 pm when it is time to prepare for the 7 o’ clock feed.
So to bed for the 7 pm feed. Half undress in the chair (I always wear shorts with popper fixings down the outside) lift up in the full sling, swing into position then pile in three extra pillows behind my back and remove sling. My feet slot into a foam pillow that Pauline cut to hold my feet upright and to protect my heels at the same time.(Cut two square holes nearly the full depth, nearly the full width and as long as the feet, plus a bit more on the length to allow extra soft padding below and to the side of the feet)
After the food and the digestion period (and some TV time) Pauline cleans the stoma and applies creams and fastens the tube to my stomach with a strip of sticky plaster with a piece of lint between the glue and the tube. Then I’m pretty well set to start my sleep pattern at 9.30 pm.
I sleep on my side (Pauline is strong and, over the years we have developed a technique that suits us). From laying flat Pauline (kneeling next to me on the bed) folds my legs at the knees and turns them down to the left. Then she puts both hands under my hips and pulls me towards the centre of the bed. Then pulls and turns my shoulders to match the angle of my hips. She repeats these movements until I am nearly to the centre of the bed then takes my knees and pulls them over to point to the right. My body naturally turns over to face the right. The whole manoeuvre is completed be pulling my knees and shoulders to the appropriate place on the right hand side of the bed.
I normally turn over about two hours later by us adopting the same technique in reverse. We awake at 1am for food. Pauline straightens me up by turning my knees upwards and kneeling on the bed next to my hips pulls my hips to the centre of the mattress. The on with the two part sling, lift up, reposition pillows and sheets and drop me back to the sitting position.
Next food and water, an hour for digestion and a listen to the radio then repeat all of the above to take us through to 7.30am for breakfast etc.