duchenne muscular dystrophy gastrointestinal management n.
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Duchenne Muscular Dystrophy: Gastrointestinal Management. Introduction. Patients at risk of both undernutrition and being overweight Range of experts may be needed as condition progresses Dietician or nutritionist Swallowing/Speech and language therapist (SLT) Gastroenterologist

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Presentation Transcript
introduction
Introduction
  • Patients at risk of both undernutrition and being overweight
  • Range of experts may be needed as condition progresses
    • Dietician or nutritionist
    • Swallowing/Speech and language therapist (SLT)
    • Gastroenterologist
  • These experts should
    • Guide the patient to maintain good nutritional status and well-balanced diet (with tube-feeding if necessary)
    • Monitor/treat swallowing problems (dysphagia) to prevent aspiration and weight loss, and assess/treat delayed speech/language problems
    • Treat common problems of constipation and gastro-oesophageal reflux with medication and non-medication therapies
nutritional management 1
Nutritional management (1)
  • Forward planning needed from diagnosis onwards to maintain good nutritional status
    • Poor nutrition can adversely affect almost every organ system
    • Patients require a well-balanced diet with a full range of food types
  • Weight or BMI for age should be maintained between 10th and 85th percentile on national charts
nutritional management 2
Nutritional management (2)
  • Regular monitoring is required for
    • Weight
    • Linear height (ambulatory patients) every 6 months
    • Arm span/segmental length (non-ambulatory patients)
  • Refer to expert dietician at diagnosis and steroid initiation
  • Further referral triggers include
    • If patient is underweight (<10th age percentile), at risk of becoming overweight (85th-95th age percentile), or overweight (95th age percentile
    • Unintentional weight loss/gain, or poor weight gain
    • If major surgery is planned
    • If patient is chronically constipated, or if dysphagia is present
upon referral
Upon referral
  • Diet should be assessed for energy, protein, fluid, calcium, vitamin D and other nutrients
  • Daily multivitamin recommended (including vitamin D and minerals)
  • If this is not general practice, computer nutrient analysis of diet can provide evidence for possible need for specific foods or supplements
  • If suspicion of undernutrition/malnutrition and poor intake, serum vitamin concentrations can be obtained and supplements recommended
swallowing management 1
Swallowing management (1)
  • In later stages, pharyngeal weakness can lead to dysphagia
    • Can further accentuate nutritional issues/loss of respiratory strength
    • Can occur gradually and be difficult to spot
  • Clinical swallowing examination indicated if
    • Unintentional weight loss of ≥ 10%
    • Decline in expected age-related weight gain
  • Referral necessary if any clinical indicators of dysphagia
    • Prolonged mealtimes (>30 minutes), or mealtimes accompanied by fatigue, excessive spilling, drooling, pocketing
    • Persistent coughing, choking, gagging, or wet vocal quality during eating/drinking
  • Swallowing problems necessitating assessment may also be indicated by
    • Aspiration pneumonia
    • Unexpected decline in pulmonary function
    • Fever of an unknown origin
swallowing management 2
Swallowing management (2)
  • Videofluroscopic study of swallowing (modified barium swallow) necessary for patients with clinical indicators of possible aspiration and pharyngeal dysmotility
  • For patients with dysphagia, a Speech Language Therapist (SLT) with training/expertise in treatment of oral-pharyngeal dysphagia should be involved
  • They can deliver an individualised treatment plan of swallowing interventions/compensatory strategies with aim of preserving good swallowing function.
swallowing management 3
Swallowing management (3)
  • As disease progresses, most patients begin to experience increasing difficulty with chewing, and subsequently exhibit pharyngeal-phase swallowing deficits in young adulthood
  • Gastric tube placement should be offered when efforts to maintain weight and hydration by oral means are insufficient
    • Potential risks/benefits should be carefully discussed with family.
    • A gastrostomy may be placed by endoscopic or open surgery, taking into account anaesthetic considerations and family/personal preferences
gastrointestinal management 1
Gastrointestinal management (1)
  • Most common conditions in DMD are constipation and gastro-oesophageal reflux
  • Constipation: typically at older age/after surgery
    • Laxatives, stool softeners and stimulants necessary for acute constipation or fecal impaction; daily laxatives necessary if symptoms persist
    • Use of enemas might be needed occasionally
    • Adequacy of free-fluid intake should be determined and addressed
    • Increased fibre may worsen symptoms, especially if fluid intake not increased
gastrointestinal management 2
Gastrointestinal management (2)
  • Gastro-oesophageal reflux (causing heartburn)
    • Typically treated with proton-pump inhibitors or H2 receptor antagonists
    • Prokinetics, sucralfate, and neutralising antacids are adjunctive therapies
    • Acid blockers commonly prescribed to children on steroid therapy or oral bisphosphonates to avoid complications
  • With increasing survival, other complications are being reported, including
    • Intestinal swelling related to air swallowing due to ventilator use
    • More rarely, delayed gastric emptying and ileus
speech and language management
Speech and language Management
  • Delayed acquisition of early milestones common in DMD
    • Differences in language acquisition and language skill deficits persisting through childhood
  • Referral to SLT for assessment/treatment necessary on suspicion of difficulties with speech acquisition, or continuing deficits in language comprehension or oral expression
  • Oral motor exercises and articulation therapy necessary for young boys with hypotonia and older patients with deteriorating oral muscle strengths and/or impaired speech intelligibility
  • Compensatory strategies, voice exercises, and speech amplification appropriate in older boys if intelligibility deteriorates
  • Voice Output Communication Aid assessment appropriate at all ages if speech output is limited
oral care
Oral care
  • Not yet part of published international consensus. Scandinavian consensus/ TREAT-NMD expert recommendations
  • Patients should see dentist with extended experience and detailed knowledge of DMD
    • Preferably at a centralised/specialised clinic
    • Aware of specific differences in dental/skeletal development in DMD
    • Will collaborate with well-informed/experienced orthodontist
  • Should strive for high-quality treatment, oral health and wellbeing, and function as resource for family and boy’s community dentist
  • Oral/dental care should be based on prophylactic measures to maintain good oral/dental hygiene
  • As progressive loss of arm function systematically erodes ability for independent tooth brushing, this needs to be specifically addressed as an area for attention to uphold oral hygiene
  • Specific alerts necessary if on bisphosphonate treatment
references resources
References & Resources
  • The Diagnosis and Management of Duchenne Muscular Dystrophy, Bushby K et al, Lancet Neurology 2010 9 (1) 77-93 & Lancet Neurology 2010 9 (2) 177-189
    • Particularly references, p186-188
  • The Diagnosis and Management of Duchenne Muscular Dystrophy: A Guide for Families
  • TREAT-NMD website: www.treat-nmd.eu
  • CARE-NMD website: www.care-nmd.eu