Respiratory/ Nervous System
Rett Syndrome Health Checklist Symptoms Aspiration (The accidental inhalation of food, fluid or reflux into the lungs)
What to look for/be aware of - Arching or stiffening of the body during feeding
Investigation & treatment and/or referral pathway Lower respiratory tract infection is the most common cause of death in Rett Syndrome. Prevention of chest infections is key to longevity in patients with Rett.
- Irritability or lack of alertness during feeding - Refusing food or liquid - Failure to accept different textures of food - Long feeding times - Difficulty chewing - Difficulty swallowing
Comprehensive multi-disciplinary feeding assessment to ensure correct posture for eating and drinking will help avoid aspiration. Video-fluroscopy/barium swallow if aspiration or unsafe swallow is suspected.
Further Reading Tarquinio et al. The Changing Face of Survival in Rett Syndrome and MECP2-Related Disorders. Pediatr Neurol. 2015;53(5):402–411. doi:10.1016/j.pediatrneurol.2015.06.003
Reverse Rett Aspiration in Rett Syndrome Fact sheet
Check for reflux symptoms and address actively.
- Difficulty breast feeding in infants - Coughing or gagging during meals - Excessive drooling or food/liquid coming out of mouth or nose
Inform carers of precautionary steps:
- Difficulty co-ordinating breathing with eating
Maintain 45 degree positioning during meals/eating/drinking/tube feeding and for 30 mins after.
and drinking
Minimal distraction during meal times.
- Increased stuffiness during meals - Gurgly, hoarse or breathy voice quality - Frequent spitting up or vomiting - Recurring pneumonia or respiratory infections
Enable patient to control speed of meal. Good oral hygiene, including teeth brushing and clearing mouth including pockets of mouth and leftover food around the mouth and lips.
- Less than normal weight gain or growth
Respiratory/ Nervous System 2