The Management of Congenital Muscular Dystrophy ... - Cure CMD

4 Gastrointestinal ManagementNutrition, Feeding, and Oral CareFeeding and nutrition problems are frequentlyencountered with children who have CMD.Other frequently encountered problems mayinclude gastroesophageal reflux (GER),aspiration, constipation, speech difficulties, poorbone health, and difficulties with oral and dentalhygiene. Management of these issues is asignificant priority for optimizing your child’scare and is best addressed by a multidisciplinaryteam, including specialists experienced infeeding and swallowing evaluation, a dietitianor nutritionist, and a gastroenterologist.Feeding andNutritional SymptomsA common problem in people with CMD isdifficulty with gaining weight, or failure tothrive. For other people with CMD, weight gain,often related to the loss of walking, may becomean issue.Other symptoms of feeding concerns with yourchild may include:• frequent pulmonary infections• chest/upper abdominal pain, vomiting• difficulties with chewing, choking, orcoughing• poor oral coordination and excessivedrooling• constipation or diarrhea• difficulties with eating independently pastan appropriate age• length of mealtimes; meals lasting longerthan 30 minutes are considered to be prolonged; this may be a sign of feedingdifficulty• increased family stress or decreasedenjoyment of mealtimes for the childand caregivers.AssessmentGrowth assessments for your child need to occurat every regular visit by measuring weight andheight. Ulnar length may be used for heightmeasurement if your child is older than age 5years and is unable to stand.Children with CMD often have a growthcurve below what is expected for age. This isacceptable if your child is in good health,without signs of fatigue, recurrent infections,or cardiac and respiratory problems. It isimportant to get an accurate weight when yourchild is evaluated and to keep frequent track ofyour child’s weight curve to ensure continuedweight gain along his or her own course.If your child’s growth or health is not adequate,a feeding assessment may be recommended.This should include an oral-facial examination,observation and evaluation of his or her feedingand swallowing skills, and assessment of his orher seating and positioning.Videofluoroscopy or fiber-endoscopic evaluationcan be helpful in diagnosing difficulties yourchild may be having with swallowing that canincrease the risk of aspiration.Other associatedfactors that needto be consideredin the feedingand swallowingassessmentinclude neckweakness,jaw and neckcontractures, weak or high arched palate, poortongue lateralization, dental crowding, scoliosis,weak or ineffective cough, respiratory fatigue,insufficient night-time breathing function, poorappetite, gastroesophageal reflux (GER), anddysmotility.15