"Don’t wait for the fire", warns DMD Care UK cardiologist
The new DMD Care UK cardiac recommendations – funded by Duchenne UK and our charity partners – took centre stage at the Action Duchenne Conference.
Cardiologist John Bourke presented the three key recommendations of the cardiac working group for boys with Duchenne muscular dystrophy (DMD). They are:
- Baseline testing should be carried out by a cardiologist at age six
- Boys should have yearly hearts scans from the age of six
- Heart medication (ACE Inhibitors or ARBs) should be prescribed by age 10
The recommendations also recommend that female carriers should have regular heart checks, as they can also be affected by heart muscle weakness.
Dr Bourke showed a photo of a house damaged by fire, illustrating that there is nothing you can do if you start too late. Medication must be taken by the age of ten, even if there are no symptoms of heart problems yet, he stressed.
DMD Care UK is an initiative to establish standards of care for DMD across the UK – based on the International recommendations published in the Lancet in 2018.
The cardiac care recommendations were published in the prestigious BMJ Open Heart, and have been endorsed by the British Cardiovascular Society. A version of the guidance for patients and families is available here.
Dr Bourke hopes that DMD parents will now have the confidence to present these guidelines, and the paper in BMJ Open Heart, to their clinicians to make sure they are aware of the evidence.
Duchenne mum and blogger Jo Eames was in the audience. She described how she did exactly that in her blog – My boy, Duchenne and me
“Today as we walked into his Cardiology appointment, his paediatric cardiology consultant, who we are lucky enough to have as William’s specialist, welcomed us with a big smile. He started to assess William with his echo cardiogram and once he was happy it was all healthy another doctor who was learning took over scanning William.
The consultant started to tell me how I should be proud. I was a little confused so he went on….18 months ago I took research literature (I was kind given by a Duchenne friend) to him about ACE inhibitors for Duchenne boys and how they can benefit them to start them earlier than what the standards of care state. He agreed after reading the research and put William on a daily dose of ACE inhibitors.
Since those discussions, unknown to me, the consultant formed a working group across Addenbrookes and Cambridgeshire to look at the care of all Duchenne boys under him and his colleagues. They have since sought further research and have updated their standards of care for ALL boys. Even better, this was across neurology too. So we have all the relevant teams now working together to ensure all boys are getting appropriate and consistent care. He told me I should be proud as it was me that drove him to it! He also asked me to keep in touch as I spoke about Duchenne UK’s DMD Care UK initiative.”
DMD Care UK patient guidelines for adrenal insufficiency and puberty and testosterone treatment can also be downloaded on the DMD Care UK website.