impaired digestion and absorption malabsorption syndromes celiac/gluten enteropathy nontropical...

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