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1/16/2012

Increased risk of GI motility issues: z z z 22q- (velocardiofacial syndrome) z Corneldlia de Lange syn drome, z Degenerative neuromuscular disorders (muscular dystrophies, mitochondrial cytopathies) z Trisomy 18 GERD & Constipation zChromosomal translocations Terry Broda, RN, BScN, NP-PHC, CDDN

GastroGastro--esophagealesophageal Reflux GERD

z Reflux of acid from the stomach into the z Reflux : 5-7% in general population In persons w/ DD z Due to defective or z Overall prevalence is 10-15% stretched sphincter z But w/ known risk factors: 30-50% (LES) between the stomach & esophagus, z 70% in those w/ severe/profound DD! a hiatal or motility

Risk factors Symptoms

In persons w/ DD Additional factors z Burning pain in throat z Scoliosis z (90%!) & esophagus z Cerebral palsy z Obesity z z Severe & profound DD z z Thoracic (chest) pain z Non-ambulatory z z Certain Rx z But … z Older age what if the person is non-verbal?

T.Broda, Solution-s 1 1/16/2012

GERD or CB? A word about food refusal..

z Refuses to eat or food avoidance z Breathing vs eating? z Refusal to sleep, lay down(on their back) z Taste & texture z Depressive behaviors (isolates oneself) z & z Irritability after meals & during the night z Food & Rx interaction? z Screaming, aggression & SIB (head-banging, hand-mouthing/biting) z Medical procedures (spoon vs. scope!) z Stripping (pants, belt, bra): too tight? z Dental pain z Excessive water drinking (pica?) z zConstipation, obstruction (pica/)

Signs & Symptoms Signs & Symptoms

z Wt loss, poor growth, FTT in children z Regurgitation and rumination 1-2 hours z Arched back with difficulty sitting after meals (p.c.) or overnight z Recurrent vomiting/aspiration z N & V, , coughing, wheezing, z Recurrent episodes of pneumonia, throat clearing, yawning, hiccups asthma exacerbations z Sinusitis, pharyngitis, otitis z Hoarseness z z Dental caries z Constipation (as a contributing factor) z Aphthous ulcers

When is it GERD? Monitoring

Predisposing risk factors along with z Scatter plot occurrence of any of those behaviors, z Calendar a minimum ofhf 4 times per month: z A-B-C sheet s SHOULD RAISE SUSPICION z Food diary TO z Breau pain scale HIGHEST LEVEL for GERD! MEDINA (2010)

T.Broda, Solution-s 2 1/16/2012

Red Flags (Alarm symptoms) Risk factors: Meds (**notify MD ASAP!)

z Chest pain (cardiac type) z ASA z Calcium channel blockers z Choking (coughing, hoarseness & SOB) z Alpha-blockers z Anti-cholinergics z Narcotics z Dysphagia (?strictures) z z Nicotine z Vomiting () z Benzodiazepines z Nitrates z Pain on swallowing () z Beta-blockers z NSAIDs & COX-2 inhibitors z d/t GI bleeding ( stool) z Bisphosphonates zBloody vomit or coffee grains** z Theophylline zWeight loss >5%

Complications Diagnosis

z Esophageal strictures Mild Severe z Recurrent inflammation z S/S less often (<3/wk) z S/S more frequent =>erosion z Low intensity z Present for > 6 months z Not at night z More intense => Barrett'h(f/)'s esophagitis ( if s/s > 5yrs) z No interference w/ life z Wakes at night, or z Increased risk of esophageal with z pain rated 1- restless nights Barrett’s ( if s/s > 5yrs) 3/10 z Interferes w/ life z No major complications z Pain rated 7/10 + z Complications

Gray, p.721

Dx Tests Other possibilities?

z Blood/urine testing (r/o anemia) z Dyspepsia z Cardiac pain z X-ray (fluoroscopy: barium swallow) to z Pyloric z check for hiatal hernia. z z Esophageal cancer z Hirschsprung’s z disease z Ambulatory 24-hour pH monitoring* z Esophagitis z Upper endoscopy (not conclusive?)* secondary to z Esophageal motility studies* esophageal motility *for refractory esophagitis or structural issue **Diagnosis can be made without endoscopy based on history.

T.Broda, Solution-s 3 1/16/2012

Dyspepsia or GERD Planning

Dyspepsia GERD For at least 3 months z Frequent regurgitation z Review & modify risk factors (if possible) z chronic/recurrent or heartburn z Have MD assess for comorbid dx epigastric pain z Epigastric pain z Have MD assess if problematic Rx can z postprandial fullness z Nausea z Or early satiety z Dysphagia be adjusted or D/C’ed Other s/s: z Odynophagia z Adopt an individualized plan z z Nausea

Therapeutic Goals Lifestyle modifications

z Relieve S/S z Avoid exercising/ z Fill in food diary bending on a full z Avoid trigger foods: z Improve QoL stomach chocolate, tomato- z Promote healing of esophagus z Stop smoking based foods, alcohol, z Limit ETOH ppppeppermint , onions, z Prevent compl(lications (strictures, caffeinated products, z Attain healthy weight bleeds & Barrett’s) citrus fruits and , z Avoid lying down for 3 high fat meals z Prevent recurrences hours p.c. or eating before hs z Avoid tight clothing around waist/chest z Avoid large meals z Elevate head of bed (10 cm, 6-8 inches, or use 2-3 pillows)

Treatment: Rx Meds

Mild Moderate/severe z CYP450 interactions & AEDs z (Aluminum ( & omeprazole) z PPI (proton pump hydroxide, Magnesium inhibitor) (see next z salts, Calcium carbonate Prokinetic (domperidone?) slide for examples) or combldbos: Rolaids, Tums, (heal up to 90% by 12wks) Maalox) z Step-down Tx (PPI z Alginates (Gaviscon) first, then H2RA after z H2-RA (histamine receptor healed antagonist)

(see next slide) z May even require Sx: z (BID dosing of H2RAs fundiplication relieves s/s in 60%, heals tissue in 40%)

T.Broda, Solution-s 4 1/16/2012

Constipation:

a symptom NOT a disease!

How serious a problem? What exactly is Normal?

Frequency: z 50-85% of older people w/ DD suffer z 3 X per week to 3X per day! from constipation (2005, Australia, Management Guidelines: Developmental Disability) Average passage time: z Up to 70% of persons with dx of z 50 ()(men) to 57 h(hrs (women )b), but moderate to profound MR have it can vary from 20-100hrs! (2001, Netherlands, JIDR) Average weight: *Especially for non-ambulatory residents z 100g = 3.5 oz.

Transit times What exactly is Normal?

Usual color: z Mouth: 1 min z Esophagus: 4-8 secs z Brown but can vary from reddish or z Stomach: 2-4 hrs greenish dark brown to lighter z SlliiSmall intestine: mustard brown 3 to 5hrs Consistency: z Colon: 10 hrs to z 70-75% actually H2O! several days (in bacteria & undigested plant cells) z 50-66% bacteria

z 33-50% undigested plant foods (fiber)

T.Broda, Solution-s 5 1/16/2012

Cause & Effect: Risk factors

z Female > Male (2-3X); Age over 65 z IQ < 50 (moderate/severe/profound MR) Constipation: z Diagnoses: CP, (DS), WS, how it can DM, comorbid GERD happen z Inadequate food/caloric & fluid intake z Inadequate z Immobility/non-ambulatory z

Kids & adults? Meds

z Similar prevalence so likely is present z ; or meds w/ antiCH SE z Anti-psychotics, , early in life & does not necessarily Anticonvulsants, benzodiazepines, develop over time Antiparkinsonians, z Intrinsic motility problem rather than z Ca+ channel blockers overstretching (functional retention) z z Antacids (Al hydroxide, Tums, Maalox) z Fe+, Ca carbonate z (narcotic ) & NSAIDs

Rome II criteria: 2 or > for at least 12 weeks in last year: For >25 % of : z Straining z Lumpy or hard stools z Sensation of incomplete evacuation z Sensation of anorectal obstruction z Use of manual maneuvers to facilitate evacuation of stool Less than 3 defecations per week Loose stools are not present Insufficient criteria for IBS Dx

T.Broda, Solution-s 6 1/16/2012

Behavioral Signs: Red Flags (Alarm Symptoms) (**notify MD ASAP!) ¾ SIB/aggression ¾ Irritability z Abdominal pain ¾ Positioning : legs bent at the knee, z N & V with thighs elevated to pressure z Mel ena, rectal blbldieeding, recta l pain on the abdomen (crouching) z Fever (rocking on the toilet seat) z Weight loss

Other concerns? Monitoring

z (4Fs!) z & PUD z Scatter plot z H. Pylori z Calendar z Meds z A-B-C sheet s z G-tube placement z Food diary z Bristol Stool form

Dx tests? Planning

z Measure abdominal girth? z Abdominal X-ray (flat plate) z Review & modify risk factors (if possible) z Distended abdomen & masses LLQ z Have MD assess for comorbid dx z TFTs z Have MD assess if problematic Rx can be adjusted or D/C’ed z Pb screening if pica z Adopt an individualized plan

T.Broda, Solution-s 7 1/16/2012

Therapeutic Goals (symptoms)

z Regular BM within 48-72 hours z Intermittent pain in , during z Appropriate use bowel movements z Avoid complications: z Blood stains in underwear, shorts z From straining: hemorrhoids, hernia, z Various complaints such as burning, GERD, coronary & cerebrovascular itching, swelling dysfunction in elderly z z Long Term: hemorrhoids, incontinence, Protrusion of internal impaction, obstruction, , noted anal fissures,

Hemorrhoids Hemorrhoids

Causes: unknown, but several Interventions: aggravating factors( heredity ): z Try to eliminate constipation z Cream to pain & z Spicy foods inflammation z Alcohol z Observe for any bleeding** z Dehydration z Pregnancy z Chronic constipation

Tx Options: Health Education

z Normal BM: frequency, type z Bulk-forming z Hyper-osmotic agents z Dietary modifications: z Lubricants z Increase calories z Increase fiber, fruit s & veget abl es z Osmotic saline agents z Increase fluids z Stool softeners z z Stimulants Lifestyle modifications z Increase

Please see chart…

T.Broda, Solution-s 8 1/16/2012

Continued monitoring

z Frequency: scatter plot z Bristol Stool form z Improvements in behavior Questions? z Breau pain scale z Food diary

Thank you!

T.Broda, Solution-s 9