Although the parents of children with Duchenne muscular dystrophy (DMD) understand the basics of pulmonary problems, many lack knowledge about advanced respiratory care, found a recent study published in Orphanet Journal of Rare Diseases.
DMD weakens muscles over time, including the muscles needed for breathing. One important tool to help with breathing problems is something called non-invasive ventilation (NIV) — a machine that delivers oxygen through a face mask without inserting a tube into the windpipe. (Bilevel positive airway pressure, or BiPAP, machines are an example.) NIV works by creating positive airway pressure, which means that the pressure outside the lungs is greater than the pressure inside the lungs. This forces air into the lungs (down the pressure gradient) and reduces the effort of breathing.
This study showed that many parents of children with DMD don’t fully understand NIV and feel anxious about using it, which can lead to delays in getting appropriate treatment for their children.
“There is a need for respiratory education programmes for parents and patients, especially as the estimated longer survival time for patients with DMD will make respiratory challenges even more significant,” the researchers say.
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The researchers surveyed 111 parents in Poland whose children have DMD. Through a questionnaire, they asked them about things like routine breathing care, NIV and their understanding of pulmonary rehabilitation (a supervised medical program that helps people who have lung diseases live and breathe better).
The good news is that most parents knew the basics of pulmonary problems, and their children attended regular check-ups with a lung specialist (77.5%) and got regular breathing tests (69.37%). Around half the parents reported that their child did pulmonary rehabilitation, either with a physiotherapist (18.9%) or at home (26.13%).
But when it came to more advanced care like NIV, about 80% of parents admitted they were afraid of using it. Reasons included: not having enough knowledge about NIV, fearing their children’s lungs would become dependent on NIV and cause the condition to worsen, expecting life with NIV to be more difficult and believing their children were too sensitive to use NIV. This fear and confusion meant that even when children needed NIV, few were actually using it.
The study also found that most parents relied heavily on doctors for information, while very few turned to online resources or support groups. The researchers say this is surprising given how much reliable information is available online, though often not in the parents’ language, Polish. They suggest that better education programs — especially ones available in parents’ own language — could make a big difference.
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