Living with Limb Girdle Muscular Dystrophy
An MDA Client perspective – Peter G.
I am writing this article in the hope that others may see the way I cope with LGMD and that my approach may be of help to others.
My situation: I am a 71 year old male who has late onset LGMD or to be specific
‘I have symptoms consistent with a group of symptoms that are referred to as Limb Girdle Muscular Dystrophy’! Despite having participated in various research programs that have included DNA comparisons of both mine and my siblings blood samples, the actual combination of genes that specifically identify those responsible for my disability, remain in one of the ‘still to be identified gene pools.
LGMD affects a girdle of muscles between the knee and hip and also from the elbow to the shoulder.
I was first diagnosed at age 47 although I had symptoms/limitations that I can now identify that reach back to my school days. I am one of eight siblings and I am the only family member with the disease.
I have had a successful life and career as an electronics/ computer engineer.
Status of my Mobility;
The physical effects of my LGMD is primarily the severe weakness in my thigh muscles, this weakness manifests itself in my inability to get up from most chairs, lounge chairs , unable to negotiate up/down stairs, etc. I cannot get in/out of a normal motor car, I have to use an SUV with an appropriate ‘seat height’ for easy access and exit. If I fall it is impossible for me to get up without aides or appropriate help. Equally ones thigh muscles play a major role in maintaining ones balance… Mine is not great.
The upper body weakness is less of a lifestyle inhibitor but it means carrying anything, particularly in front of me is impossible. Pushing up is impossible but lifting and pulling, although weak, is still manageable for some functions..
E.g. I can lift my walker into the back of my car.
My Mobility aides:
When discussing the aides that I use you should realize that these are the ones that I have found best for my situation. There are many alternatives available and only after much trial and error have I settled on the following:
- Mobility: My primary aide to getting around currently is to use a walker. The walker I have is a Peak Care Brado medium with 200mm wheel size which I need primarily for outside activity. The seat height is 625mm which helps. Inside the house I cope with a walking stick combined with furniture walking although this is becoming more precarious. With the slow progress of the disease, I am nearing the stage where a wheelchair/motorized scooter will be necessary and the ability to get out of the seat will be a major selection determinant.
I can still drive a car as my LGMD does not impact the movement of my lower limbs and yes, I get tested each 12 months like all others with a mobility issue.
- Getting up from Low chairs: I have 2 aides.
First aide…I use a brief case size seat cushion that looks like a brief case and is full of hard foam rubber that I use to place on chairs in restaurants and at the breakfast table to provide extra height. It fits nicely on the seat of the walker and is therefore convenient to carry around. This cushion only works for chairs with a hard flat seat and without arms. To source such a case. Go to eBay. See ‘child booster cushions for barber shops’ they come in different sizes and you would need to change the density of foam rubber inside (i.e. at Clark Rubber). My first seat cushion was a carry case for my notebook computer stuffed with appropriate hard foam.
Second aide… Getting up from plane seats: Seats on planes are designed to fit the contour of your back so the briefcase cushion doesn’t work. I use the Mangar Raiser Cushion (from the UK) which involves a very low profile cushion, when deflated, and this is placed on the seat. It comes with a portable pump, which is used to inflate the cushion raising the seat height by approximately 20cm.
I use the following device when travelling on aero planes, in motels when the bed height and lounge chair heights etc, are too low. I also use it for general use around the house.
I find it a better solution than the brief case cushion when I can set it up and leave it in the one position e.g. on my study room chair. I also use it in preference to the electric lift recliner chairs. It will fit any chair including existing lounge recliner chairs and it provides elevation rather than the ‘tip-out’ style features of the others.
- Emergency lift from a fall: In case of a fall, I use the Mangar Elk System…which is the big brother to the raiser cushion and operates in a similar manner. When deflated and placed on the floor it is flat. I maneuver my body into a sitting position on the cushion and then using the pump and controller, it inflates each of 4 layered cushions in turn (not 2 as shown with the raiser above) and lifts me up to a height equivalent to a tall chair which then allows me to stand with assistance.
- Getting off the toilet: The Lifter crutches. One of the most limiting factors I have found with LGMD when trying to maintain a normal lifestyle is the difficulty with getting off the toilet. There are several alternate aides to help but none that I found user friendly and which are also conducive to both a normal home existence and also provides the ability to travel, visit friends, etc.
Frustrated with the options, I designed, built and now use a set of crutches (see below) built using a pair of linear actuators and powered from small in-built batteries. In the ‘retracted’ position the pair of crutches sits under my arms when I am seated. By actuating the switch on each crutch they extend and lift me into the standing position.
This pair of crutches weighs a combined 8 kg in total. Each crutch disassembles into 3 pieces which can then be carried in my luggage for travel.
The lifter crutches have been an absolute blessing in my ability to retain a normal existence.
When I was diagnosed with late onset LGMD I was advised I would be in a wheelchair at age 65. I realize there is no exact means of forecasting such outcomes however I have followed a regime of maintaining fitness levels to the best extent possible. On the subject of exercise, there appears to be two streams of thought
- Too much exercise/exertion can be detrimental to the disease and may exacerbate the problem or increase the progression of muscle loss.
Or 2. Some exercise is beneficial in maintaining the strength of the useful muscle that remains, but not too much.
So it’s about what’s the best way to maintain this balance.
My approach: I am not a muscle builder, but I follow the principle that you can either ‘use it or lose it’ but also given the realities of living with LGMD, ‘moderation is key’ and accept that when it is not possible to do something because of muscle weakness then accept it /don’t force it. My regime includes
. Using my walker I go for a 40 minute daily walk around the nearby park. My speed is not great but I do what I can do. It’s a good time to catch up on the morning news, reset my thoughts, etc.
. Two to three times a week I go to the local gym. I am the only one there who gets around with a walker and I have to get help on some of the machines to set them up properly, but everyone is helpful. I cannot do leg exercises, or others where I have muscle weakness or where I couldn’t get off the low seats should I choose to sit on them, but I spend an hour using various machines mainly exercising the upper body, riding the recliner exercise bike etc. I always leave at least 2 days between visits to allow my muscles to recover.
Does it work? For me it does, I sure notice the difference in my wellbeing if I miss a week or two for whatever reason. But I do accept that my disease is progressing and I can’t do much more than working within my limits and to maintain a positive outlook. After all the alternative is not great.