Please enable JavaScript.
Coggle requires JavaScript to display documents.
Cystic Fibrosis - Coggle Diagram
Cystic Fibrosis
PERT
500-4000 U Lipase per gram dietary fat,
max dose:
10,000 lipase per KG BW/day
Aim for lowest effective dose, use individualised approach, distribute enzymes throughout the day in relation to fat content of fats/fluids (review once a year), monitor wt, growth, bowel signs, pts should discuss PERT before ^ dose
1 10,000 creon per 10g fat as a start or 1 25,000 per 25g fat, should be consumed within 30mins of eating
Aims to correct malabsoprtion, gastric acid in stomach dissolves capsule releasing microspheres that mix w/ food in stomach, food enters small intestine, activation of enzymes occur to release PERT
Steps for PERT review
: calculate total fat intake/day, calculate total no. of enzymes/day, calculate average enzyme to fat ratio, calculate IU lipase/g fat, divide amount of fat in each meal by ratio, educate pt as to dosage, re-evaluate
What is it?/evidence
genetic disease, CFTR mutation
Thick/sticky mucus can block glands, ducts in areas such as respiratory tract, GI tract, pancreas, liver, reproductive organs (PEN)
Bone mineral density exam should be performed from 8-10
Small intestine bacterial overgrowth occurs due to decreased intestinal motility = decreased fat absorption/appetite affecting nutritional status
Patho of energy imbalance/wt loss
increased energy expenditure:
chronic lung inflammation/infections, increased work of coughing/breathing, stimulation of metabolism by broncodilator therapy
Malabsorption, maldigestion, other losses:
exocrine pancreatic insufficiency, reduced bile salts, ^ intestinal mucosa
Inadequate intake
poor appetite worsened by infection related anorexia, dyspnoea, early satiety, dry mouth, taste changes due to oxygen/drug therapy, behavioural factors
Nutrition Support
Infants <2yrs:
OPTIMAL; wt for lngth >50th percentile and weight/length tracking and within 2 percentile bands of each other. ACCEPTABLE: wt for lngth 25-50th percentile and wt/lngth tracking and within 2 percentile bands of each other. SUBOPTIMAL: wt for lngth 10-25th percentile and/or weight/length decreasing >1 percentile band and/or no wt gain. PERSISTENT UNDERNUTRITION: persistent wt for lngth <10th percentile and/or wt falling >2 percentile bands w/ stunting of growth and/or failure of prev nutrition interv to improve nut status. HIGH BMI: not applicable, use growth chart to identify rapid wt gain
Children/Adolescents 2-18:
OPTIMAL: BMI 50-85th percentile using CDC growth chart or BMI 50-91st percentile using NZ WHO growth chart. ACCEPTABLE: BMI 25-50th percentile and wt/ht tracking along previous percentiles and no recent wt loss. SUBOPTIMAL: BMI 10-25th percentile and/or wt loss/plateau over 2-4mnths. PERSISTENT UNDERNUTRITION: BMI <10th percentile and/or wt falling >2 percentile bands w/ stunting of growth, and/or failure of previous nutritional interventions to improve nut status. HIGH BMI: overweight - BMI 85-95th percentile using CDC growth chart or BMI >91-98th percentile using NZ WHO growth chart, Obese - BMI>95th percentile using CDC growth chart or BMI>98th percentile using NZ WHO growth chart, high risk of developing overweight/obesity: unintentional wt gain resulting in increasing of >2 BMI centile bands
Adults:
OPTIMAL - female BMI 22-27kg/m2, male BMI 23-27kg/m2, ACCEPTABLE - female BMI 20-22kg/m2, male BMI 20-23kg/m2 and no unintentional recent wt loss. SUBOPTIMAL - BMI <20kg/m2, and/or >5% unintentional wt loss over 2 months. PERSISTENT UNDERNUTRITION: BMI <18.5kg/m2 and/or >5% unintentional wt loss over 2 months despite interventions regardless of starting BMI. HIGH BMI - BMI >27kg/m2 and/or unintentional wt gain from acceptable BMI >5kg within a year.
Classification of COPD
Mild
- few symptoms, breathlessness on moderate exertion, little/no effect on daily activities, lung function: FEV1 60-80% predicted
Moderate;
increasing dyspnoea, breathlessness walking on level ground, cough/sputum production, infections requiring steroids, FEV1 40-59% predicted
Severe
- dyspnoea on minimal exertion, daily activities severly curtailed, chronic cough, FEV1 <40% predicted
Pancreatic insufficiency
Symptoms of malabsorption:
loose stools, large stools, greasy/floating stools, foul smelling frequent trips to the bathroom, excess gas, bloated stomach, stomach cramps/pains, wt loss
Low bicarb secretion, reduced/nil enzyme secretion, hyperacidity, reduced bile acid secretion (limits emulsification of fats)
Salt replacement therapy
sweat electrolytes are elevated (low serum sodium, chloride, osmolarity), potential for sodium depletion/dehydration in hot weather/exercise
Symptoms of dehydration:
(thirst response not stimulated due to low osmolarity), fatigue, loss of appetite, impaired performance, difficulty concentrating, can lead to difficulty expectorating sputum/blockages in distal intestine.
GI problems
40-50% complain of intestinal issues, viscosity of intestinal mucosa is 1.8x greater than healthy controlled, altered motility of gut, GOR, distal intestinal obstructive syndrome
Vitamin therapy:
biochemical deficiency of vitamins, A, D, E, K reported in CF pts, asymptomatic, CF specific vitamin preparations higher in FSV e.g. vitabdeck/bio-logical, A, D, E, K tablets
diagnostic tests
newborn screening, heel-prick test, checks for CF/other genetic conditions at birth
CFRD:
Average AOO: 18-21 (screening recommended over 10), clinical presentation similar to T2DM, onset insidious/ketoacidiosis rate, loss of lung function/wt are main concern, microvascular complications seen in CFRD but not macrovascular