making muscle wasting history

If you find yourselves needing to go to the hospital in an emergency situation, there are a range of factors that should be taken into account.

• The diagnosis of DMD, current medication, presence of any respiratory and cardiac complications and the people who are your key medical input should be made clear to the admitting unit.
• As many health professionals are not aware of the potential management strategies available for DMD, the current life expectancy and expected good quality of life should also be explained.

IMPORTANT FACTS TO REMEMBER:

• You are very likely to know more about DMD than the doctors in Accident and Emergency.
• Advise the doctor or healthcare staff if your son is taking steroids.
• If your son has a broken bone, insist that they speak with your doctor or physiotherapist.
• If you can, bring copies of your son’s most recent test results, such as FVC and LVEF.
• If your son’s oxygen level drops, the doctor must be very careful about giving him oxygen or sedating medication.

Steroids
Chronic steroid use needs to be made clear. Tell the staff how long your son has been using steroids and if he has missed a dose. It is also important to let the doctors know if your son used steroids in the past.

• Steroids can dampen the stress response so extra steroids may be needed if someone on chronic steroids is unwell.
• Steroids can increase the risk of stomach ulceration.
• Rarely other complications can present acutely.

Broken bones
Boys with DMD are at risk of broken bones and breaking a leg bone can mean that it is difficult to walk again if walking is already very difficult. Let your physiotherapist and the rest of the care team know if there is a fracture so they can talk to the surgeons if necessary.

• Surgery is often a better option than a cast for a broken leg if someone is still walking.
• Input from a physiotherapist is crucial to make sure that the boy gets back on his feet as soon as possible.
• If the broken bone is one of the vertebrae (backbones) with a lot of pain in the back, input from a bone doctor or endocrinologist is needed to provide the right treatment.

Breathing problems
Try and keep a note of, or remember what the latest tests of breathing (e.g. forced vital capacity, FVC) were. This information can be useful for the doctors assessing your son if he does become ill acutely.
The main risks with breathing problems come when FVC and coughing strength have reduced:

• Help with clearing the chest may be needed;
• It may be important to help with coughing;
• Antibiotics may be needed;
• Sometimes it may be necessary to give support with a ventilator;
• Risk of the breathing muscles needing extra support during an infection can be high in those with borderline respiratory function. Care in the use of opiates and other sedating medication is essential, as is care in the use of oxygen without ventilation due to the risk of rising carbon dioxide in people with compromised breathing muscle strength;
• If nocturnal ventilation is already used, then access to the ventilator is essential during any acute event or intervention. For those who are already ventilated, the team involved with the respiratory care should be involved as soon as possible. If you have a ventilator (or similar equipment) it is a good idea to bring it with you to the hospital.

Heart function

• Try and keep a note of what the latest test results of heart function (e.g. left ventricular ejection fraction, LVEF) were, and what, if any, heart medication your son is on and which cardiologist sees him. This will help the emergency doctors decide if it is likely that the problems they are seeing are due to a problem with the heart.
• Awareness of the risk of heart rhythm problems and cardiomyopathy is important. Anaesthetic risks need to be taken into account at all times if surgery or sedation is needed.