Preparing for surgery

Duchenne muscular dystrophy (DMD) is a muscle disorder affecting around 1 in 5000 boys. Children present with delayed early motor milestones, including sitting and standing, as well as toe walking and enlarged calves. The muscle weakness affects their whole body, including their breathing and eventually their heart. This makes surgery a risky time and careful planning is needed with everyone working together to give children with DMD the best outcomes.

Before the surgery

Ensuring good health before surgery is the best way to ensure a good outcome after surgery. This is a team effort and many different people from different medical specialties will need to be involved.

Before surgery you or your child may have:

  • a blood test to check the liver and kidneys are working well.
  • lung function testing, because if there is an issue with breathing, this can be helped by training patients and parents with devices that help with breathing and coughing.
  • an electrocardiogram or ECG  to make sure you or your child's heart is working well.
  • other tests like chest x-rays, echocardiograms or sleep studies might be needed, you can discuss this possibility with your doctor.

As nutrition is very important to a good outcome after surgery and you may be asked to see a dietician.

Many children over the age of 5 years are on steroids. An extra steroid dose around the day of surgery might be needed. You can discuss this possibility with your doctor.

In surgery

People with DMD have particular requirements when it comes to the anaesthetic agents used in their surgeries, so make sure your anaesthetist knows you or your child has DMD.

After surgery

Getting up and moving as quickly as possible plays an important role in all patients recovering from surgery, but it is particularly important in people with DMD. There should be a plan to make sure good pain relief is available and that movement is begun as quickly as possible. When choosing pain relief, we know people with DMD are extra sensitive to some medication like codeine. Using medication like paracetamol or ibuprofen may be a safer choice. Discuss with your doctor which pain medication is best.

Key points to remember

  • DMD causes problems with all muscles including the heart and the muscles around the lungs.
  • Preparing well before surgery by checking lung function, heart function and sometimes blood levels is key to good recovery after surgery.
  • If breathing problems are identified, training with devices to assist breathing or coughing may be needed.
  • If you or your child are on steroids, an extra dose around the time of surgery may be needed.
  • Adequate pain relief is important to make sure that early movement after surgery is possible.
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References

  1. Bushby, K., et al., Diagnosis and management of Duchenne muscular dystrophy, part 1: diagnosis, and pharmacological and psychosocial management. Lancet Neurol, 2010. 9(1): p. 77-93.
  2. Bushby, K., et al., Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care. Lancet Neurol, 2010. 9(2): p. 177-89.
  3. Birnkrant, D.J., The American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation. Pediatrics, 2009. 123 Suppl 4: p. S242-4.
  4. Hull, J., British Thoracic Society guideline for respiratory management of children with neuromuscular weakness: commentary. Thorax, 2012. 67(7): p. 654-5.
  5. Marchant, W.A. and R. Fox, Postoperative use of a cough-assist device in avoiding prolonged intubation. Br J Anaesth, 2002. 89(4): p. 644-7.
  6. Cripe, L.H. and J.D. Tobias, Cardiac considerations in the operative management of the patient with Duchenne or Becker muscular dystrophy. Paediatr Anaesth, 2013. 23(9): p. 777-84.

 

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