Elsevier

The Journal of Pediatrics

Volume 160, Issue 3, March 2012, Pages 447-451.e1
The Journal of Pediatrics

Original Article
Prevalence and Risk Factors for Feeding and Swallowing Difficulties in Spinal Muscular Atrophy Types II and III

https://doi.org/10.1016/j.jpeds.2011.08.016Get rights and content

Objective

To identify the prevalence and risk factors of feeding and swallowing problems in patients with type II and type III spinal muscular atrophy (SMA).

Study design

Cross-sectional data from 108 genetically confirmed patients with SMA (age range, 3-45 years; 60 with type II and 48 with type III) were analyzed. The questionnaire survey included demographic data, current motor function and respiratory status, feeding and swallowing difficulties, and consequences. The risk factors were analyzed via logistic regression.

Results

The 3 most common feeding and swallowing difficulties in patients with type II and III SMA were choking (30.6%), difficulty conveying food to the mouth (20.4%), and difficulty chewing (20.4%). Current motor function status was an independent risk factor for feeding and swallowing difficulties (sitters vs walkers: OR, 7.59; 95% CI, 1.22-47.46). All 4 nonsitters (ie, patients with type II SMA who had lost their sitting ability) had feeding and swallowing difficulties. Patients with feeding and swallowing difficulties had significantly higher rates of underweight and aspiration pneumonia than those without these problems.

Conclusion

Patients with type II and III SMA have a high prevalence of risk factors for feeding and swallowing difficulties, suggesting that an individualized treatment plan should depend on current motor function status.

Section snippets

Methods

Between September 2008 and November 2009, 108 patients with type II or III SMA were enrolled in this study. The inclusion criteria were genetically confirmed SMN1 homozygous deletion, clinical diagnosis of SMA type II or III, and age >3 years. Patients with type I SMA were excluded, because prophylactic interventions are routinely performed for feeding difficulties in this group of patients.9 A questionnaire survey was conducted at the Kaohsiung Medical University Hospital, Kaohsiung, Taiwan.

Results

We analyzed the questionnaires of 108 patients with SMA (50 females) who met the inclusion criteria; this included 60 patients with type II and 48 patients with type III. They ranged in age from 3 to 45 years (mean, 16.3 ± 10.6 years) (Table I).

Discussion

In our study cohort of patients with type II and III SMA, choking was the most common feeding problem, with a prevalence of 30.6%. A previous study reported that difficulty conveying food to the mouth and chewing difficulties were the most common problems in patients with type II SMA.6 Another study reported that choking (45%) was more common than chewing problems (38%) in patients with type I and II SMA.17

We found a correlation between current motor function status and feeding and swallowing

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    Citation Excerpt :

    Weakness of bulbar muscles and of muscles of the gastrointestinal tract have also been described [7–9]. Feeding difficulties, including swallowing problems and aspiration, chewing problems and over- and underweight, have been reported frequently in SMA type 1 [10–14], to a lesser extent in SMA type 2 [7,9,11,14–18], and sporadically in SMA type 3 [11,15–18]. Only a limited number of studies have investigated the prevalence of feeding difficulties in SMA type 2 (Table 1).

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Supported in part by the Kaohsiung Medical University-Spinal Muscular Atrophy Fund, Kaohsiung Medical University. Y-J.J. holds patents for hydroxyurea treatment for spinal muscular atrophy and the method for diagnosing spinal muscular atrophy; he also serves as editor-in-chief of Pediatrics and Neonatology. The other authors declare no conflicts of interest.

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