Cystic Fibrosis 2[1]

CYSTIC FIBROSIS (MUCOVISCIDOSIS) SATURDAY GROUP Canlas, Florison Capigon, Karen Furio, Larriza Gabriel, Daizele Villanueva, Glady

Cystic Fibrosis •

Multi-system disorder of the exocrine glands, leading to increased production of thick mucus in bronchioles, small intestines and pancreatic and bile ducts & even causes fertility problems • An autosomal recessive disorder • Obstructs small passageways of these organs  Lungs (bronchioles): atelectasis (lung collapse) & Emphysema (Overinflation of the alveoli)  Pancreatic ducts become clogged, impairing digestion and absorption  Small intestine: absence of pancreatic

INCIDENCE • • • •

1: 30 EUROPEANS 1: 3,000 WHITES 1 : 15,000 BLACKS 1 : 90,000 ASIANS

Assessment • Recurrent Upper Respiratory Infection (URI) • Respiratory impairment: Wheezing, dry non productive cough, dyspnea & digital clubbing • Thick Sticky Mucus • Decreased digestive enzymes • Steatorrhea – fatty, foul smelling stools • Meconium ileus • Decreased absorption of Vitamins

CFTR Gene • Cystic Fibrosis transmembrane Conductance Regulator • Transports chloride ions across the membranes of the cells in the lungs, liver, pancreas, digestive tract, skin and reproductive tract. • Mutation of CFTR gene causes sodium and chloride imbalance which can lead to thick-sticky mucus.

Lungs

Crackles / ronchi Chest congestion

Ineffective airway clearance Mucocillary activity Ineffective coughing

Narrowing of the airways Dilation of alvoeli Air trapping

Bacterial growth Chronic bronchitis / Pneumonia Trigger inflammatory response Dilation of hyperplasia bronchioles Sputum fibrosis production consolidatio hypertrophy n

dyspnea fatigu e Activity intolerance hypoxia

Tissue necrosis

cyanosis

Hyperinflation of lung Lung elasticity emphysema Lung stiffening Alveolar collapse atelectasis

Lung abcess

Arterial erosion

Perforation of bronchial wall

hemorrhage

hemoptysis

RESPIRATORY DISTRESS == > RESPIRATORY FAILURE

Respirator y acidosis

Metabolic alkalosis Uncompensat ed respiratory acidosis

Digestive tract pancreas

Pancreatic duct obstruction Decreased / absent circulating digestive enzymes

liver

Inability to digest protein

Vit A, D, E, K malabsorp tion

constipation

AUTODIGESTION

Biliary cirrhosis

malabsorption

diarrhea

Diabetes mellitus

Rectal prolapse

steatorrhe a

MALNUTRITION fibrosis

Meconium illeus

Bile duct obstruction

Pancreatitis Inability to digest fat

Small intestine

Inability to thrive

DEATH

Reproductive System male

female

Obsturction of vas deferens

Thick sticky cervical mucus

Blocks sperm flow

Blocks sperm entry

azoospermia

INFERTILITY

Diagnostic Procedure SWEAT CHLORIDE TEST • Pilocarpine iontophoreses – Used to stimulate sweat production • Analyzes sodium and chloride content in sweat • Done 3 – 4 weeks after birth - A chloride concentration greater than 60meq/L is a diagnostic of cystic fibrosis - Parents often report that infants taste salty when kissed

Diagnostic Procedure 72-hour stool collection (Keep food diary) • Analyzes fat & enzyme content Chest x-ray • To reveal atelectasis & obstructive emphysema

Priority Nursing Diagnosis • • • • • • • • • •

Ineffective airway clearance Impaired gas exchanged Risk for infection Altered Nutrition Altered Bowel Movement Activity intolerance Alteration in Activities of Daily Living Fear/Anxiety Knowledge deficit Risk for ineffective family coping

Nursing Management • • • • • • •

Avoid exposure to respiratory infection CPT Bronchodilator Antibiotics / Mucolytics Monitor hemoptysis HIGH PROTEIN, CALORIE diet Supplement w/ VITS ADEK (in H20 Soluble form) • Monitor weight • Supplement the child’s diet with fluids & salt during extremely hot weather

Pancreatic Enzyme • Pancrealipase (Pancrease, Coazym,Ultrase) • Used in to aid in digestion & absorption of nutrients; • Administered on or before meals and snacks to ensure that digestive enzymes are mixed with food in the duodenum • Enteric coated pancreatic enzymes should not be crushed or chewed • Dose is adjusted to suit normal growth & decrease in the number of stools per

CURRENT TRENDS AND RESEARCHES IN CYSTIC FIBROSIS

Comparison of Hospital and Home Intravenous Antibiotic Therapy in Adults with Cystic Fibrosis

Clinical Manifestations of Cystic Fibrosis Among Patients With Diagnosis in Adulthood

Metabolic Alkalosis Contributes to Acute Hypercapnic Respiratory Failure in Adults Cystic Fibrosis

Inhaled Tobramycin Improves Lung Function and Bacterial Density Associated with Pseudomonas aeruginosa infections in patients with Cystic Fibrosis

The Physical, Psychological, and Social Implications of Caring for the Pregnant Patient and Newborn With Cystic Fibrosis