Intestinal Failure
Intestinal failure is a severe
reduction in the intestinal absorption of essential macronutrients
and/or water and electrolyte supplements that are needed to
maintain health.
Below is a summary of the care pathway
options:
MDT Assessment by Team (within 2 weeks of
referral)
AIM: TO DETERMINE GASTRO-INTESTINAL ANATOMY, LENGTH OF
BOWEL AND PRESENCE OR EXTENT OF DISEASE
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Specialist Nurses inc. Stoma Nurse/other Nurses Assessment
on the ward:
- Nurse history - as per standard documentation
- Usual Medical and Surgical History
- Family Dynamics
- Home assessment for suitability for HPN
- Financial support and Benefits
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Consultant Paediatric Gastroenterologist:
- Formal History: Antenatal, Birth History, Neonatal period,
Infant and Childhood as relevant.
- Diagnosis if known
- Treatment and management given so far
- History of previous surgery undertaken and assessment of
outcome or complications
- Nutritional and Growth Assessment: PN and EN dependence and
proportion
- Presence of PN complications
- Presence and assessment of Liver disease if present
- Review of previous tests and results.
- Plan for various tests as may be appropriate as outlined
below
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Consultant Paediatric Surgeon including Surgeon(s) with
special interest in bowel reconstruction:
- Details History of problems or diagnosis and previous
surgeries.
- History of Vascular Access
- Details of length of bowel resection undertaken i.e. jejunum or
ileum or colon
- Length and type of Bowel left and if ICV is intact or not
- Stomas fashioned and type
- Bowel expansion programme if undertaken
- Recyling of stomas losses if undertaken
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Dietetic assessment
- Nutritional Status and anthropometry, plot growth chart
- Details and length of previous and present feeding
arrangements.
- Total Calorie Requirement: proportion of Parenteral Nutrition
(PN) to Enteral Nutrition (EN).
- Target Future Weight
- Type of feeds previously had and present feeds.
- Route of feeding: oral or NG or NJ or Gastrostomy
- Is child taking solids?
- Any History of Feeding or food aversion.
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Pharmacist review
- Details of present PN prescription.
- Dosing weight,
- PN Fluid intake: ml/kg/day,
- PN Calories: Kcal/kg/day
- Glucose Concentration.
- Type of lipids and gm/kg/day
- Details of Electrolytes i.e. Na, k, PO4, mg
etc.
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Psychosocial Assessment
- Play leader assessment of developmental play needs.
- Play Specialist assessment to assist coping with
treatment.
- Psychosocial assessment of family context (clinical
psychologist)
- Psychosocial factors involved in feeding behaviour and / or
food aversion (Clinical psychologist)
- Neuro-developmental assessment (clinical psychologist)
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Speech and Language Assessment
- Presence of feeding or food aversion
- Assessment of oral feeding skills
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Occupational Therapist/Physiotherapist
- Neuro-development assessment and treatment.
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Radiologist assessment:
- Review of Previous Ultrasound scans and Doppler's of Neck
veins, leg veins and others as appropriate.
- Details of previous lines: dates inserted or removed
- Details of present line and location
- MRV as indicated ( When available)
- Review of previous contrast studies. Discussions regarding
whether new contrast studies are indicated
Review of Ultrasound scan of liver and biliary tree
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Other Investigations:
- FBC, U&E's, LFTs, gamma GT, Split Bilirubin,
Coagulation,
- TPN bloods: magnesium
- Bone profile: Po4, Calcium, vitamin D , PTH. TFT
- Nutrient bloods: Ferritin, Vitamin B12, Red cell folate,
selenium, zinc, ZPP
- Urinary sodium, d xylose,
- Stool analysis for ph, chromatography, electrolytes
- Echo cardiogram if indicated
Medical illustrations if indicated
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Pathway A: Short Bowel Syndrome
Short bowel syndrome: is characterised by state of malabsorption
after extensive loss or resection of the small bowel. It is an
anatomical and functional definition. The length of resection
results in insufficient nutritive supply, requiring artificial
nutrition i.e. PN.
Pathway A1 Short bowel syndrome
(40 to 90 cm)
1.) Moderately short bowel syndrome as defined by length
<90cm of a term baby*
2.) Multi systemic disease caused by lack of bowel
Pathway A2 Extremely
short bowel syndrome (<30cm - 40cm of a term baby*)
- Consideration for Autologous Intestinal Reconstruction with PN
and EN
- Further Adaptation possible: To continue to challenge the gut:
EN, oral feeds and PN as appropriate.
MDT discussion as regards management (for patients that
initial management is considered appropriate)
- The length of the bowel is intended as a guide only and the MDT
will take this into account along with the bowel functionality
- To encourage intestinal adaptation: Gut challenge orally or via
NG or gastrostomy as appropriate
- Home PN and training commence.
3 Regular
monitoring at home, in patient and out patient as appropriate
4 Monitoring for
complications of PN
Failure of Adaptation of bowel: this will said to have occurred
if there is inability to wean off PN as determined by the monthly
MDT assessment, or failure of tolerance or lack of progression of
EN. Contrast study showing dilated and dysmotile loops of
bowel.
MDT discussion as regards management:
5 Consideration
for autologous intestinal reconstruction
6 Continuation
of HPN
7 Continuation
of EN
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Pathway B:
- Congenital Enteropathies e.g. Tufting Enteropathy or
Microvillous Inclusion Disease
- Chronic pseudo-obstruction
3 Severe bowel
motility disorders
4 Hirsch rung's
disease or severe extensive Agangliosis of the bowel
MDT discussion as regards management:
- EN as tolerated with Home PN
- Monitoring and regular review by MDT to assess progress or lack
of it
- Assess complications like liver disease or Metabolic Bone
disease
- Central line assess
Need to refer for consideration for Intestinal transplant
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Pathway C:
Other causes of Intestinal Failure without significant resection
of bowel:
- Severe Crohn's Disease,
- Mesenteric Vascular Thrombosis
- Small bowel Volvulus,
- Radiation enteritis,
- Severe Malabsorption etc
MDT discussion as regards management:
- To encourage intestinal adaptation: Gut challenge orally or via
NG or gastrostomy as appropriate
- Home PN and training commence.
- Regular monitoring at home, in patient and out patient as
appropriate
- Monitoring for complications of PN
Failure of Adaptation of bowel: this will said to have occurred
if there is inability to wean off PN as determined by monthly MDT
assessment, or failure of tolerance or lack of progression of EN.
Contrast study showing dilated and dysmotile loops of bowel.
MDT discussion as regards management:
- Consideration for autologous intestinal reconstruction
- Continuation of HPN
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Pathway D:
- Referral to Birmingham for consideration for intestinal
transplant
Consider referring for the following
indications:
Patient with Intestinal Failure and one or more of the following
PN related life threatening complications.
- End Stage Liver Disease or Persistently raised Bilirubin
>100
- Loss of Central venous Access sites
- Major fluids and electrolytes imbalances
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