Duchenne Muscular Dystrophy: Gastrointestinal - CARE-NMD

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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
• These experts should
– Guide the patient to maintain good nutritional status and wellbalanced 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 gastrooesophageal reflux with medication and non-medication
therapies
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)
• 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
• 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)
• 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)
• 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)
• 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)
• 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)
• 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
• 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
• 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
• 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
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