Michelle Stroebe MS RD Adult Cystic Fibrosis Center Stanford Healthcare Overview What is DIOS Why is DIOS only in CF SignsSymptoms of DIOS Common Triggers Treatment Prevention Impact on Nutrition ID: 561580
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Slide1
DIOS: A Dietitian’s Perspective
Michelle Stroebe, MS RD
Adult Cystic Fibrosis Center
Stanford HealthcareSlide2
Overview
What is DIOS?
Why is DIOS only in CF?
Signs/Symptoms of DIOS
Common Triggers
Treatment
Prevention
Impact on Nutrition
When to Call Your Medical TeamSlide3
What is DIOS?
DIOS =
D
istal
I
ntestinal
O
bstructive
S
yndrome
Specific to Cystic Fibrosis (CF), mostly pancreatic insufficient
Lifetime prevalence of DIOS
: ~16% of CF adults, ~10-20% post-transplant CF adults/children
Type of constipation caused by mucus buildup in intestines
May cause
complete
or
incomplete
blockage of stool
at junction where small intestine ends and large intestine (colon) begins
Onset of symptoms may be sudden and may cause extreme discomfort
Recurrence is commonSlide4
What is Constipation?
Stool impaction in
colon
Occurs in both pancreatic insufficient and sufficient patients
Usually occurs gradually over time
Associated with gas, bloating, mild abdominal pain
Hard, pebbly stools that are hard to passSlide5
Why is DIOS only in CF?
Incidence of
meconium
ileus
at birth may increase risk for DIOS (risk of occurrence 18-44%)
Pancreatic insufficiency requiring enzymes
Missing, skipping,
under dosing of enzymes
Imperfect digestion even with enzyme use
Leads to sticky intestinal mucous (malabsorption)
CFTR gene mutation leading to:
Impaired intestinal motility
Impaired intestinal secretion
dry, sticky gut
Malabsorption + dry, sticky gut harder to pass stoolsSlide6
Signs & Symptoms of DIOS
Decreased number or frequency of stools
i.e. normal stooling = 2-3 medium sized stools/day
Reduced to 1-2 small
stool(s
)/day OR no stool for 1-2 days
Cramping/abdominal pain, usually starting in right lower side of abdomen
Pain continues to worsen and may cause extreme pain
Watery, loose stools that do not relieve abdominal pain
Nausea/vomiting
Inability to tolerate food, decreased appetite, abdominal fullness
Bloating/abdominal distentionSlide7
Common Triggers
Dehydration
Poor hydration at baseline
OR
Inadequate replacement of fluid losses
Discontinuation of bowel regimen
Malabsorption
Inadequate enzyme use or skipped enzymes
Surgery
Immobilization, reduced activitySlide8
Treatment
Easily misdiagnosed by those unfamiliar with the condition…
If diagnosed early enough (incomplete DIOS):
Oral laxatives (i.e.,
Miralax
,
GoLytely
) usually at home
Titrated by your medical team to cleanse intestines of impacted stool
If diagnosed late and
suspected complete DIOS
:
“Clean out” likely requiring hospitalization
Abdominal x-ray
Enemas, laxatives
IV hydration
NG tube for decompression to help with abdominal distention/nausea &/or to administer laxatives into stomach Slide9
Prevention
Strict adherence to enzyme regimen &/or adequate enzyme coverage
Skipping enzymes
malabsorption & sticky stools
Talk to your team about any change in
stooling
Good hydration!
Increase with exercise/summer months
Consistent bowel regimen
Goal for 2-3 stools/day, type #3-4
Dietary changes
Good blood sugar control in diabetic patientsSlide10
Impact on Nutrition
May create fear of food intake after episode
May require several days of NPO (nothing by mouth) until impaction clears
Weight loss, malnutrition, muscle mass loss
Change in diet to help manage symptoms/prevent DIOS
Higher fiber diet OR lower fiber diet
(based on symptoms/individual
work with your dietitian!)
Increase hydration on a daily basis
May require change in enzyme regimen to prevent malabsorption Slide11
Why do I need to talk about my stool every
clinic visit?
Assessing
stooling
behaviors
assess
potential for constipation or DIOS
Assessing adequacy & appropriateness of enzyme regimen
Taking at correct timing?
Taking appropriate dose?
Taking with correct foods?
Assessing adequacy of bowel regimen
What dose works for
you?
Prevention of DIOS!Slide12
When to call your medical team
Increasing abdominal pain, especially in right lower abdomen
Decreased number or frequency of stools
OR
no stool in 24 hours
Decreased appetite, feeling of abdominal “fullness,” nausea/vomiting
Abdominal distentionSlide13Slide14
References
Cystic Fibrosis Foundation.
Use the right gastrointestinal medications.
(2014). https://www.cff.org/Living-with-CF/Treatments-and-Therapies/Nutrition/Use-the-Right-Gastrointestinal-Medications/
Declercq
, D. & Van
Biervliet
, S.
Nutrition and pancreatic enzyme intake in patients with cystic fibrosis with distal intestinal obstructive syndrome.
(2014). Nutrition in Clinical Practice, (30)1, 134-137.Houwen, R. et al.
Defining DIOS and constipation in cystic fibrosis with a multicentre study on the incidence, characteristics, and treatment of DIOS.
(2009), Journal of Pediatric Gastroenterology and Nutrition.
Schindler, T. & Michel, S.
Nutrition Management of Cystic Fibrosis in the 21st Century.
(2015). Nutrition in Clinical Practice.
Van
der
Doef
, H.P,
Kokke
, F.T., Van
der
Ent
, C.K.,
Houwen
, R.H.
Intestinal Obstruction Syndrome and Constipation.
(2011). Current Gastroenterology Reports. (13)3, 265-270.
Yale School of Medicine, Pediatric Respiratory Medicine.
Distal intestinal obstructive syndrome (DIOS): Information sheet.
(2009).