MDF Magazine Newsletter Issue 57 December 2018

MD
affected boy pushes his abdomen forward (called lordosis)
and his shoulders backward. Rising from the floor unassisted
also becomes progressively difficult, which is also due to
weakness of the muscles around the hips. At the same time
as weakness of the hip muscles becomes evident, there is also
weakness of the shoulder muscles so that the affected boy has
increasing difficulty raising his arms.
The confinement to a wheelchair increases the likelihood of
contractures developing as a result of immobility. The sole
of the foot often turns inward. The prolonged sitting in one
position may result in the gradual curvature of the spine to
one side and so compresses the lung on that side. This is referred
to as scoliosis and can result in serious problems with
breathing and chest infection.
The heart is also affected in DMD and this may aggravate
any respiratory problems.
and thighs, while others that are less weak are often enlarged.
The muscles of facial expression, speech and swallowing and
the involuntary muscles (for example those of the bowel and
bladder) are not affected in BMD.
Things to remember when visiting an
emergency room
The DMD emergency card of the Muscular Dystrophy
Foundation of South Africa (2017b) provides the following
information:
Duchenne muscular dystrophy
Leg fracture
• If ambulatory: Ask if internal fixation/surgery rather than
casting may be possible. Surgery may help preserve
walking.
• If your child has had a fall or a leg injury and has rapid
onset shortness of breath/difficulty breathing and changes
in alertness (confusion, agitation, disorientation), this is an
emergency; go immediately to the ER and alert staff that
symptoms could be due to fat embolism syndrome (FES).
Respiratory care
• Risk: Respiratory failure. Please only give oxygen with
close monitoring of CO2 levels; breathing may need to be
supported (with BiPAP, for example).
• If oxygen levels are low, assisted coughing (with cough
assist machine or Ambu bag) may help.
• Take your equipment (cough assist, BiPAP, etc) with you to
the hospital/emergency room (ER); alert your neuromuscular
team that you are going to ER/hospital.
What are the symptoms of BMD and which
muscles are affected?
The BMD fact sheet of the Muscular Dystrophy Foundation
of South Africa (2000a) provides the following information:
BMD has a mild onset during childhood. Muscle cramps
during exercise are often the only problem at first but a few
affected individuals may also be late in learning to walk.
From early childhood affected boys are unable to run
very fast. During the teens or twenties, muscle weakness
becomes evident causing difficulty in rapid walking, running
and climbing stairs. Later it may be difficult to lift heavy
objects above waist level. Individuals with typical BMD may
become unable to walk in their 40s or 50s, or even later, but
there are also more rapidly progressive variants of BMD in
which the loss of mobility may happen in the 20s or 30s.
Over a period of years some muscles become weak and wasted,
especially certain muscles of the shoulders, upper arms
General recommendations and precautions
• Keep immunisations up to date and get influenza vaccine
annually.
• People taking daily, long-term steroids should avoid live
vaccines when possible.
• Always wear seat belts – in the car AND on the wheelchair/
scooter.
Anaesthetic precautions
• Avoid inhaled anaesthesia.
• IV anaesthesia is considered to be safe (with close
monitoring).
• People with Duchenne should NOT receive
succinylcholine.
• Local anaesthetics and nitrous oxide are safe for minor
dental procedures.
If vomiting and/or unable to take daily corticosteroids for 24
hours
• Go to a hospital emergency room.
• Request substitute IV corticosteroid until oral medications
are tolerated (6 mg of deflazacort equals 5 mg of prednisone).
• Remind clinicals that high liver enzymes (AST/ALT) are
normal for people with Duchenne MD.
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