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Action Duchenne calls for an immediate and thorough review of the use of steroids in treating Duchenne
Action Duchenne has received information from the coroners report concerning the sudden death of a little boy in Wales with Duchenne.I am copying here an updated statement from Action Duchenne based upon recent nformation from the family. Following the coroners judgement we believe that a full and thorough review of the use of steroids for treating Duchenne must be undertaken in the UK. We would expect new guidelines to be published for families. The statement follows:"It is with great sadness that we have to report that a 7 year old boy in Wales with duchenne muscular dystrophy undergoing steroid treatment died suddenly on 27 April 2008. The thoughts of everyone at Action Duchenne goes to his family at this time.
The cause of death has been confirmed by the coroner as a “perforated gastric ulcer in a subject with Duchenne Muscular Dystrophy treated with steroids.” The coroner’s report goes on to say he “was prescribed steroids as part of his treatment; steroids are a recognised cause of gastric ulceration and this would appear to be the most likely reason for the gastric perforation. Histology of the stomach may provide further information."
The family have asked Action Duchenne to bring to parents’ attention the circumstances surrounding his death.
He started his course of steroids at the age of 5 and experienced stomach pains straight away. He was prescribed Ranitidine which was later changed to Omeprazole to help to lessen the stomach pain. The pain decreased but did not totally go away. During his steroid cycle this could be for several times a week for up to half an hour but it was not severe to the extent that he needed pain relief. The family continued to report the pains to his specialist team. His community paediatrician referred him to a gastro enterologist in December 2007 but no action was considered necessary.
He had been completely well until the Friday 25th April 2008 evening when he developed a viral stomach bug, that a number of his classmates had experienced that week. He saw a GP on Saturday who confirmed it was a viral stomach bug and no action was necessary. In the early hours of Sunday morning the pain became more severe and a second GP was called who maintained that it was a simple stomach bug possibly being exacerbated by slight dehydration. He died on the way to hospital 10 minutes after seeing the GP and only 36 hours after first experiencing sickness.
In the light of this tragic incident Action Duchenne is calling for an urgent and immediate review of long term steroid use in treating Duchenne with special reference to this case and the risk of gastric ulcers.
In particular we would expect advice on
• Are steroids a gold standard that we can continue to safely use
• In the light of the this case do the benefits of steroids still outweigh their side effects
• What are the early signs of ulcers in a child with Duchenne
• What is the difference between the symptoms for a general tummy ache, constipation, bad wind and the beginnings of an ulcer
• Procedures for our clinical visits that we believe must require a thorough abdominal examination
• Early signs of distress that require hospitalisation and examination by a gastro enterologist
Until we get this advice we urge parents to consult with their clinical teams before making any decisions about removing their children from long term steroid use. Removal from steroids requires clinical support as it must be a gradual lowering of the dose over time.
It is important to know that steroids can increase the risk of stomach upsets and ulcers. Steroids can also mask symptoms of viral or other illness and so the usual associated high body temperatures for example might not be recorded. In other words a child might be very seriously ill but not showing the normal symptoms.
This would appear to be a rare incident. But as many young people with Duchenne are regularly taking steroids Action Duchenne feels that parents should be reminded of procedures when dealing with a young person with Duchenne who might be taken ill very suddenly.
It is important that families err on the side of caution and act quickly if your child with Duchenne is taken ill.
We strongly advise you to:
• Contact your GP immediately if you are at all concerned about stomach pains or other signs of distress
• Get your child to hospital immediately if the symptoms appear to be persistent, worsening or are severe
• At all times contact your specialist Duchenne clinical team and ask for advice and keep them informed
We send our small words of comfort to the family who have sadly lost such a beautiful little boy."