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TABLE OF CONTENTS
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| 1 | Spinal Muscular Atrophy: An Overview of Disease and Nutrition
Barbara Godshall, MMSc, RD, CSP, LD, CD, CNSC, Brenda Wong, MD
Nutrition management of spinal muscular atrophy (SMA) is not usually taught in
academic dietetic curriculums. The likelihood of working with a child with SMA is
increasing as children with the most severe form of SMA are living longer due to
improved medical and nutritional management. SMA is an autosomal recessive
neurological disease with an incidence of approximately 1 in 6,000 to 1 in 10,000 live
births. SMA is characterized by degeneration of the spinal motor neurons, resulting
in progressive muscle weakness and atrophy. The disease is divided into different
categories based on degree of weakness, ranging from severe—inability to sit
unsupported—to less severe—difficulty standing or walking. As the muscle atrophies,
lean muscle decreases and body fat increases (1). Medical problems for affected
children include pulmonary, gastrointestinal, metabolic, and nutritional issues. |
| 5 | Nutrition for Orally Fed Children with Spinal Muscular Atrophy
Erin Seffrood, MS, RD, CSP, CD, Mary K. Schroth, MD
Many infants and children with spinal
muscular atrophy (SMA) are able to
safely eat by mouth. Although it is well
known that disease severity runs along
a continuum in SMA, classification or
type of SMA, often defined by age of
onset and motor strength, helps guide
management and is useful in determining
if a child will be able to eat orally
(1,2). Based on clinical experience and
clinical practice guidelines, it has been
noted that the majority of infants and
children who eat orally will be diagnosed
with SMA type II or III, rather than
SMA type I (2). Due to the severe muscle
weakness and risk of aspiration in
children with SMA type I, it is often not
possible or safe for them to eat orally;
and they require enteral nutrition to
meet nutrient needs (3). This article will
explore the nutritional assessment,
dietary needs, and challenges of children
with SMA who eat by mouth. |
| 8 | Nutrition for Enterally Fed Children with Spinal Muscular Atrophy
Rebecca Hurst Davis, MS, RD, CD, Kathryn J. Swoboda, MD, FACMG
Nutritional concerns in spinal muscular
atrophy (SMA) relate to the progressive
deterioration of the motor neurons
of the brainstem and spinal cord resulting
in skeletal muscle atrophy and
weakness (1,2). In many children with
SMA who have significant muscle weakness,
this process can result in bulbar
dysfunction and dysphagia due to
involvement of the cranial nerves affecting
the mouth, tongue, and throat. In
addition, children with SMA often
exhibit gastrointestinal dysmotility
which can manifest variably with gastroesophageal
reflux, constipation,
delayed gastric emptying, and other
digestive issues (2-4). |
| 15 | Overview on the Principles, Implementation, and Management of the SMA Amino Acid Diet and Common Complementary Therapies in SMA Nutrition
Mary Marcus, MS, RD, CSP, CD
The spinal muscular atrophy (SMA)
amino acid diet is a nutritional approach
that many families of children with SMA
type I, and some families of children
with SMA type II, follow because they
believe it has a positive impact on their
children's lives. Although this diet has
not been well studied by the scientific
community, it is important for pediatric
registered dietitians (RDs) to be aware of
this diet. The amino acid diet may not fit
with common nutrition practice or standards
of care, but families feel very
strongly that this diet improves their
child's quality of life. |
Copyright 2011 Pediatric Nutrition Practice Group, ADA.
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FOR THIS ISSUE:EDITORMichelle S. Trumpy, MPH, RD, CSP, LD
Clinical Nutrition Manager, Adolescent Services
The Emily Program
St. Paul, MN
Co-EDITORHolly A. Van Poots,, RD, CSP, LDN
Pediatric Nutritionist
Cone Health; Greensboro, NC Communications Team ChairLiesje Nieman Carney,, RD, CSP, LDN
Clinical Dietitian IV, Publication Specialist
The Children's Hospital of Philadelphia LOOKING AHEADVolume 35, No 2: Undernutrition
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