Introducing a new nighttime breathing device to patients with Duchenne muscular dystrophy (DMD) and mental health or cognitive conditions carries unique challenges, which were illustrated in a case report recently published in Frontiers in Pediatrics.
The authors described their experience with two patients with DMD, one with autism spectrum disorder (ASD) and one with attention deficit hyperactivity disorder (ADHD). Both patients’ disease had progressed to the point where they needed to begin using assistive devices to help them breathe during sleep, called nocturnal non-invasive ventilation (NIV).
“In light of the findings presented in these cases, it is concluded that a combination of careful preparation and psychological treatment holds promise in facilitating the implementation of home NIV in adolescents with DMD and comorbid neurodevelopmental or (internalized) behavioral symptoms,” the authors wrote.
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Case 1
The first patient was a 16-year-old boy with DMD and ASD. A previous neuropsychological evaluation at age 15 reported strong non-verbal concept formation and weak verbal skills. Due to behavioral program issues, his family and physicians thought that successfully starting NIV in a strange setting such as the intensive care unit (ICU) would be unrealistic.
NVI implementation required a multidisciplinary care team including his parents, nurse, child neurologist, child psychologist, special education specialist and speech therapist. The team used pictograms to acquaint the patient with the NIV equipment. His family put effort into developing a detailed schedule focusing on maintaining a normal home routine. Additionally, the patient began taking the antipsychotic drug Risperdal several weeks before NIV was introduced.
The patient successfully adapted to NIV ventilation, and Risperdal was tapered down after some time.
Case 2
The second patient was a 17-year-old boy with ADHD. Previous neurophysiological examinations revealed average intelligence scores. His initial NIV attempts were performed in the ICU. However, this resulted in an unsuccessful and traumatic experience.
To the authors’ judgment, the patient was not involved in the decision-making process and the initial plan was insufficiently detailed. In preparation for a home NIV attempt the parents involved a multidisciplinary team, which implemented the use of stress reduction techniques (meditation and hypnotherapy), as well as cognitive behavioral therapy interventions. Furthermore, the use of the antidepressant fluoxetine improved his mood and decreased obsessive thoughts.
He initiated home NIV with slow and moderate success, but a respiratory infection disrupted the process. Several months later, the patient decided not to continue NIV and expressed that he preferred to focus on quality of life rather than longevity.
About NIV and DMD
Current guidelines recommend patients begin using nocturnal NIV when they meet certain parameters, such as high levels of CO2 in the blood or symptoms related to hypoventilation.
Evidence shows that NIV leads to better sleep quality and architecture, increases life expectancy and improves quality of life.
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