Many boys with Duchenne muscular dystrophy (DMD) are being treated with steroid medication – either prednisolone or deflazacort. When these medications have been used on a long term basis, boys can develop adrenal insufficiency.
Adrenal insufficiency occurs when steroid medication stops the body’s natural production of steroid hormones called corticotrophin-releasing hormones and adrenocorticotrophin-releasing hormones from the hypothalamus and pituitary glands. This then reduces release of natural steroid hormones from the adrenal gland. This is called adrenal suppression.
Adrenal suppression means that when your child is ill, having an operation or has a serious accident, they may not be able to mount the usual ‘stress’ response in the body by increasing the amount of steroid hormones circulating in the blood. This can result in your child becoming very unwell, very quickly. This effect can continue for up to a year after stopping steroid medication.
If at any stage, you become concerned about your child, please use your fast-track access to your local children’s ward or take your child to the nearest Accident and Emergency Department.
Always state that your child is on long-term steroid medication and take this information sheet with you to show the medical and nursing teams.
What to do if your child becomes unwell
Trivial illness, such as a mild cold
No extra steroid required.
Significant illness, temperature 38°C or above but steroids can still be taken by mouth
Increase your child’s steroid medication to twice daily doses 12 hours apart instead of once daily.
When your child is well again, go back to the once daily dose of steroid medication.
Diarrhoea and vomiting (D&V) illness and is unable to take the dose at any time
Use your fast-track access to your local children’s ward or take your child to the nearest Accident and Emergency Department straight away.
Your child will need a steroid injection.
Planned or emergency operations or serious accidents
Your child will need extra steroid medication to be given.
Always tell doctors that your child is taking long term steroid medication.
Please read this information sheet from GOSH alongside the patient information leaflet (PIL) provided by the manufacturer. If you do not have a copy of the manufacturer’s patient information leaflet please talk to your pharmacist. A few products do not have a marketing authorisation (licence) as a medicine and therefore there is no PIL.
For children in particular, there may be conflicts of information between the manufacturer’s patient information leaflet (PIL) and guidance provided by GOSH and other healthcare providers. For example, some manufacturers may recommend, in the patient information leaflet, that a medicine is not given to children aged under 12 years. In most cases, this is because the manufacturer will recruit adults to clinical trials in the first instance and therefore the initial marketing authorisation (licence) only covers adults and older children.
For new medicines, the manufacturer then has to recruit children and newborns into trials (unless the medicine is not going to be used in children and newborns) and subsequently amend the PIL with the approved information. Older medicines may have been used effectively for many years in children without problems but the manufacturer has not been required to collect data and amend the licence. This does not mean that it is unsafe for children and young people to be prescribed such a medicine ‘off-licence/off-label’. However, if you are concerned about any conflicts of information, please discuss with your doctor, nurse or pharmacist.