TY - JOUR
T1 - Feeding problems and malnutrition in spinal muscular atrophy type II
AU - Messina, Sonia
AU - Pane, Marika
AU - De Rose, Paola
AU - Vasta, Isabella
AU - Sorleti, Domenica
AU - Aloysius, Annie
AU - Sciarra, Federico
AU - Mangiola, Fortunato
AU - Kinali, Maria
AU - Bertini, Enrico
AU - Mercuri, Eugenio
PY - 2008/5
Y1 - 2008/5
N2 - The aim of the study was to conduct a survey using a dedicated questionnaire to assess feeding difficulties and weight gain in a population of 122 Spinal Muscular Atrophy (SMA) type II patients, aged between 1 and 47 years. All the answers were entered in a database and were analysed subdividing the cohort into age groups (1-5, 6-10, 11-14, 15-19, 20-29, and 30-50 years). Six out of our 122 patients (5%), all younger than 11 years, had weights more than 2SD above the median for age matched controls, whilst 45 (37%) had weights less than 2SD below the median. Chewing difficulties were reported in 34 of the 122 patients (28%) and limitation in the ability to open the mouth in 36 (30%) and both were increasingly more frequent with age. Swallowing difficulties were reported in 30 patients (25%). The results of our survey suggest that a number of patients with SMA type II have limited jaw opening, and chewing and swallowing difficulties. Our findings raise a few issues concerning standards of care that should be implemented in the monitoring and management of feeding difficulties and weight gain.
AB - The aim of the study was to conduct a survey using a dedicated questionnaire to assess feeding difficulties and weight gain in a population of 122 Spinal Muscular Atrophy (SMA) type II patients, aged between 1 and 47 years. All the answers were entered in a database and were analysed subdividing the cohort into age groups (1-5, 6-10, 11-14, 15-19, 20-29, and 30-50 years). Six out of our 122 patients (5%), all younger than 11 years, had weights more than 2SD above the median for age matched controls, whilst 45 (37%) had weights less than 2SD below the median. Chewing difficulties were reported in 34 of the 122 patients (28%) and limitation in the ability to open the mouth in 36 (30%) and both were increasingly more frequent with age. Swallowing difficulties were reported in 30 patients (25%). The results of our survey suggest that a number of patients with SMA type II have limited jaw opening, and chewing and swallowing difficulties. Our findings raise a few issues concerning standards of care that should be implemented in the monitoring and management of feeding difficulties and weight gain.
KW - Dysphagia
KW - Feeding difficulties
KW - Spinal muscular atrophy
KW - Swallowing
UR - http://www.scopus.com/inward/record.url?scp=43449122152&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=43449122152&partnerID=8YFLogxK
U2 - 10.1016/j.nmd.2008.02.008
DO - 10.1016/j.nmd.2008.02.008
M3 - Article
C2 - 18420410
AN - SCOPUS:43449122152
VL - 18
SP - 389
EP - 393
JO - Neuromuscular Disorders
JF - Neuromuscular Disorders
SN - 0960-8966
IS - 5
ER -