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IMPAIRED DIGESTION AND ABSORPTION
MALABSORPTION SYNDROMESCELIAC/GLUTEN ENTEROPATHY
NONTROPICAL SPRUECYSTIC FIBROSIS 2009
CELIAC DISEASE
Intolerance to gluten Gluten is a protein found in wheat,
barley, rye, and oats Less incidence because of delayed
feeding of solids and increased breast feeding
EARLY STAGES OF CELIAC
Fat absorption effected Result: large quantities of
undigested fat in the stool (steatorrhea)
STOOL: frothy, foul odor, excessive quantity
LATER STAGES OF CELIAC
Absorption of the following becomes impaired:
Protein Calcium Iron Folic acid Vitamin D, K, B12
RESULTS OF MALABSORPTION
MALABSORPTION OF
1. Fats2. Proteins and CHO
3. Vit D and calcium
4. Vitamin K5. Iron, folic acid, vit
B12
LEADS TO:1. Steatorrhea2. Peripheral
edema/malnutrition
3. Osteomalacia and osteoporosis
4. Inadequate blood coagulation
5. anemia
PROGRESSION OF THE DISEASE
If disease process is not stopped GROWTH FAILURE RESULTS
Begins slowly when child starts to ingest grains within 3-6 month of introduction
FIRST EVIDENCE OF DISEASE
Failure to gain weight Poor appetite Bout of diarrhea Steatorrhea Constipation vomiting Abdominal pain Irritability
AS DISEASE PROGRESSES
See signs of general wasting Some children do not manifest
symptoms until 5 years of age
CELIAC CRISIS
Acute episodes in which the child has:Profuse, pale, bulky, rancid, poorly
formed stoolsVomitingWasted appearanceDependent edemaSmooth tongue
CELIAC CRISIS PRECIPITATED BY
GI infections Prolonged fasting Eating gluten
DIAGNOSIS Stool analysis for fat Serum albumin (for hypoproteinemia) CBC, Hct, Hgb, for anemia PT for hypoprothrombinenemia Serum iron Folic acid levels Vit B12 levels Immunoglobulin levels
DIAGNOSIS CONTINUED
Xrays for bone age Bowel studies for dilated flaccid
bowel loops Pancreatic function studies Sweat test to rule out CF Small bowel bx
TREATMENT
Remove foods from diet having gluten (wheat, rye, barley, oats)
Substitute with corn, rice, potato, hominy
Provide supplements for malnutrition (vitamins, Fe)
High calorie diet Peripheral hyperal may be required
TREATMENT OF CRISIS Considered life threatening event Correct dehydration Correct metabolic acidosis NGT to decrease abdominal distention IV fluids with K, Ca, mg Albumin infusions to treat
hypoproteinemia IV steroids to decrease inflammation of
bowel
NURSING CARE: teach about diet
AVOID Cereals and
baked goods Check food labels
for hydrolyzed vegetable protein
Check for grains used in processed foods as fillers
CANNOT HAVE:
Bread, Cake, Cookies, chocolate candy, malts
Crackers, Doughnuts, Pies, some ice cream
Spaghetti, PizzaPrepared soups, hot dogs
Luncheon meats, meat gravy
Some prepared hamburgers
FOODS THEY CAN HAVE
Tacos and Mexican dishes
GENERAL GUIDELINES
Very difficult for adolescents Must do forever If diet not followed increased
incidence of lymphoma or GI cancer
Anticholinergic drugs precipitate crisis: inform MD’s and dentists
CYSTIC FIBROSIS
DEFINED
Most common serious pulmonary genetic disease in children
Multisymptom disorder affecting the exocrine glands (mucous producing glands) of white children
SURVIVAL
Changed from a short life expectancy in the 1950’s to 50% of patients survive to adulthood, living an average of 33 years
ETIOLOGY
Inherited autosomal recessive trait (inheriting the defective genes from both parents is a 1:4 chance)
Gene responsible is located on chromosome #7
INCIDENCE
1:1600 births get the disease 1:20 are carriers; can pass on to
children Equal sex distribution Seen mostly with whites, rare
among African Americans or Asians
PATHOPHYSIOLOGY
Increased viscosity of mucous gland secretions which causes mechanical obstruction in small passages in organs
Elevation of sweat electrolytes: Na and Chloride content of sweat is 2-5 times greater than that of normal children
DIAGNOSIS Suspected when the child is identified as
FTT or suffers frequent repeated URI Positive family history aids in diagnosisSweat test: stimulate the production of
sweat, collecting & measuring the sweat electrolytes
NL SWEAT CHLORIDE: 5-35 mEq/LCHLORIDE greater than 60 mEq/L up to 200
mEq/:: means diagnosis of CFTest is done on two separate occasions
DIAGNOSIS
Chest xray reveals atelectasis and obstructive emphysema
PFT’s indicate abnormally small airway function in CF
Stool analysis for fat DNA studies are helpful in the 70%
of CF carriers; prenatal testing not yet available
RESPIRATORY TRACT CHANGES
Increased viscosity of bronchial mucous leads to slower flow rate of mucous, incomplete expectoration and leads to bronchial obstruction
Retained mucous provides medium for bacterial growth
Reduced O2/CO2 exchange leads to hypoxia, hypercapnia (increased CO2 in blood) acidosis
RESPIRATORY TRACT CHANGES
Atelectasis and emphysema Repeated infections (s. aureus, h.
influenzae, **pseudomonis aeruginosa) Become resistant to multiple drugs
making the bacteria difficult to eradicate Fibrotic areas develop Pneumothorax and hemoptysis can
occur
GASTROINTESTINAL TRACT CHANGES
Thick secretions in the pancreas block the ducts leading to degeneration and fibrosis
Fibrosis prevents pancreatic enzymes from reaching the duodenum (lipase, trypsin, amylase) which impairs digestion and absorption of fats, proteins and to a lesser degree carbohydrates
Result: excessive stool fat (steatorrhea) and protein (azotorrhea)
GASTROINTESTINAL TRACT CHANGES
Diabetes mellitus develops frequently (may result from the diminished blood supply to the pancreas)
In the liver: biliary obstruction and fibrosis are common leading to biliary cirrhosis leading to portal hypertension
Salivary glands are blocked so this leads to dry mouth and susceptibility to infection
SIGNS AND SYMPTOMS
Vary widely Could be diagnosed at birth or not
until years later Intensity of involvement varies
RESPIRATORY TRACT SYMPTOMS
Chest congestion, Cough, crackles Limited exercise tolerance Sputum production, with hemoptysis Use of accessory muscles Decreased pulmonary function Repeated bronchitis and
bronchopneumonia
LABORATORY TESTS RELATED TO RESPIRATORY FUNCTION
Arterial blood gas (ABG): acidosis Decreased PaO2
Increased PaCO2
Increased bicarbonate levels Low pH
DECREASED PULSE OX
Progressive disease see over-inflated, barrel shaped chest and cyanosis, clubbing of fingers and toes
GASTROINTESTINAL TRACT SYMPTOMS
IN INFANTS: may be diagnosed as having meconium ileus
SEE signs of intestinal obstruction: abdominal distention, vomiting, failure to pass stools, dehydration
GASTROINTESTINAL TRACT SYMPTOMS
Increased bulk of stools: 2-3 times nl amount, frothy, foul smelling flatus
FTT due to malabsorption Have good appetite Abdomen distended from flatus, but
extremities are thin Deficiency fat-soluble vit (A,D,E,K) Easy bruising (vit K); anemia
REPRODUCTIVE SYSTEM
Females: fertility inhibited by highly viscous cervical secretions
Males: most are sterile
INTEGRUMENTARY SYSTEM:
Parents when kiss infant taste salt Risk during hyperthermic
conditions
TUNE UP
Child hospitalized about every 6 months
Intensive chest PT with vibrating vest Inhaled antibiotics, IV antibiotics PURPOSE: prophylactic prevention of
serious infections Done extensively at Dupont Hospital
for Children
MANAGEMENT OF PULMONARY PROBLEMS
Prevention of pulmonary infection Removing secretions Administering antimicrobials Administer bronchodilators Administer antiinflammatory
agents Administer mucolytics
METHODS OF MANAGING PULMONARY PROBLEMS
Daily chest PT twice a day on rising and in evening
Incorporate play (hanging by knees from a bar, somersaults, playing wheelbarrow)
Bronchodilators given before chest PT Forced expiration (moves secretions)
ANTIBIOTICS Prophylactic antibiotics Antibiotics for actual infection C & S helps guide choice of antibiotics pseudomonis aeruginosa; this is serious Inhaled antibiotics helpful: tobramycin IV antibiotics best:AMINOGLYCOSIDES (class): tobramycin (Nebcin),CARBAPENEMS (class): meropenem (Merrem) CF metabolize antibiotics rapidly
PULMONARY MANAGEMENT CONTINUED
Oxygen used cautiously because of chronic CO2 retention
Pneumothorax common, may resolve on own or require chest tubes
Hemoptysis with pneumothorax of greater than 300 cc/24 hr is considered to be life threatening
MANAGEMENT OF GASTROINTESTINAL PROBLEMS
Replace pancreatic enzymes with meals and snacks so that when the food reaches the duodenum will have the appropriate enzymes
Use 1-5 with each meal Comes in capsules, can sprinkle on
food
PSYCHOLOGICAL SUPPORT
Dealing with child and family facing a chronic fatal illness
Refer to Cystic Fibrosis Foundation Child and family may resent the
restrictions the disease places on their lives
Constant fear of death
DIET
High in calories (150% of recommended daily allowance)
Fat restriction not necessary Multivitamins Vit K Hyperalimentation for FTT (short
term) Salt supplements in hot weather
SURGICAL MANAGEMENT
Lung transplant/pancreatic transplant
Not a cure Reduces symptoms Gradual progression of disease Extends life 10-20 years