IMPAIRED DIGESTION AND ABSORPTION

MALABSORPTION SYNDROMES CELIAC/GLUTEN ENTEROPATHY NONTROPICAL SPRUE CYSTIC 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. Fats 2. Proteins and CHO
3.

Vit D and calcium

4.
5.

Vitamin K Iron, folic acid, vit B12

LEADS TO: 1. Steatorrhea 2. 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 stools Vomiting Wasted appearance Dependent edema Smooth 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, Pizza Prepared 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 MDs 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 1950s 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 diagnosis Sweat test: stimulate the production of sweat, collecting & measuring the sweat electrolytes NL SWEAT CHLORIDE: 5-35 mEq/L CHLORIDE greater than 60 mEq/L up to 200 mEq/:: means diagnosis of CF Test is done on two separate occasions

DIAGNOSIS

Chest xray reveals atelectasis and obstructive emphysema PFTs 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