Cystic Fibrosis

• -progressive, incurable d/o, and resp failure is the most common death; organ transplantation to increase survival rates.
• -chronic multisystem d/o (autosomal recessive trait d/o)
• -exocrine gland dysfunction
• ~the mucus produced by the exocrine gland is abn thick, tenacious and copious, causing obstruction of the small passageway of the affected organs (resp, GIT and reproductive -common sx are associated with pancreatic enzyme deficiency, and pancreatic fibrosis caused by duct blockage, progressive chronic lung disease as a result of infection, and sweat gland dysfunction resulting in increased sodium and chloride sweat concentrations.

• RESPIRATORY SYSTEM:
• -(+) stagnation of the mucus in the airway: bacterial colonization and destruction of lung tissue.
• -airway obstruction: (+)emphysema and atelectasis.
• -chronic hypoxemia: (+) contraction and hypertrophy of muscle fibers in pulmonary arteries and arterioles leading to pulmonary hypertension and cor pulmonale
• -progression: pneumothorax frm ruptured bullae and hemoptysis frm erosion of the bronchial wall
• -wheezing and cough
• -dyspnea
• -cyanosis
• -clubbing of the fingers and toes
• -barrel chest
• -repeated episodes of bronchitis and pneumonia

• GIT:
• -NB: meconium ileus (is a bowel obstruction that occurs when the meconium in your child's intestine is even thicker and stickier than normal meconium, creating a blockage in a part of the small intestine called the ileum.)⇒EARLIEST MANIFESTATION 
• -intestinal obstruction (distal intestinal obstructive syndrome) caused by thick intestinal secretions can occur; s/sx: pain, abdominal distention, n&v 
• -stools: frothy and foul-smelling
• -deficiency in vitamins ADEK, which can result in easy bruising, bleeding and anemia.
• -malnutrition and failure to thrive
• -(+)hypoalbuminemia (diminished protein absorption⇒generalized edema).
• (+)rectal prolapse (this result from large, bulky stools and increased intraabdominal pressure).
• -(+)pancreatic fibrosis (is a progressive inflammatory disease of the pancreas)⇒@ risk for DM 

• INTEGUMENTARY SYSTEM:
• -high Na and chloride in sweat
• -infants taste "salty: when kissed
• -dehydration and electrolyte imb occur, esp during hyperthermic conditions.

• REPRODUCTIVE SYSTEM:
• -delay puberty in girls
• -fertility can be inhibited by the highly viscous cervical secretions, which act as a plug and block sperm entry.
• -males⇒sterile, but not impotent, caused by a block in the vas deferens by abnormal secretions or by failure of normal development of duct structures.

• QUANTITATIVE SWEAT CHLORIDE TEST-(+)
• ↳ production of sweat is stimulated, sweat is collected (>75mg is needed), and sweat electrolytes are measured.
• ↳ normal: <40mEq/L 
• ↳ (+) >60mEq/L
• ↳ infants: >40mEq/L (dx in infants younger than 3 mos of age)
• ↳ Chloride concentration of 40-60mEq/L are highly suggestive of CF and require a repeated test.

NB SCREENING: immunoreactive trypsinogen analysis and direct DNA analysis for mutant genes.

CXRAY: (+)atelectasis (partial or complete collapse of the lung) and obstructive emphysema (the air sacs of the lungs are damaged and enlarged).

PULMONARY FXN TEST: (+)abn small airway fxn.

STOOL, FAT, ENZYME ANALYSIS: 72-hr stool sample is collected to check fat or enzyme (trypsin) content, or both (food intake is recorded).

• RESP. SYSTEM:
• -GOAL: px and tx pulmonary infection 
• -improve aeration, remove secretions and giving antibiotic meds.
• -monitor: lung sounds and (+) cough
• -chest physiotherapy: on awakening and in the evening; frequent during pulmonary infection; done everyday to maintain pulmonary hygiene; DO NOT PERFORM BEFORE AND AFTER A MEAL.
• -FLUTTER MUCUS CLEARANCE DEVICE: removal of mucus; store away from small children; steel ball poses a choking hazard.
• -POSITIVE EXPIRATORY PRESSURE MASK: forces secretions to the upper airway for expectoration.
• -teach child huffing (forced expiratory technique) to mobilize secretions.
• -BRONCHODILATOR MEDS BY AEROSOL: opens the bronchi for easier expectoration; give BEFORE CP when the child has reactive airway dse or is wheezing).
• -MEDS: decrease viscosity of the mucus.
• -O2: acute episodes; WOF: O2 necrosis (n&v, malaise, fatigue, numbness, tingling of extremities, substernal distress) bec a child may have carbon dioxide retention.
• -lung transplantation: occassionaly performed.

• GIT:
• -high calorie, high protein and well-balanced diet to meet energy and growth needs. 
• -multivitamins and vitamins ADEK
• -(+)severe lung dse: energy requirement must be as high as 20-50% or more.
• -monitor: weight and failure to thrive; stool patterns (intestinal obstruction)
• -(+)pancreatic insufficiency: replace pancreatic enzymes (given 30mins of eating and given with all meals and snacks); DO NOT GIVE IF CHILD IS NPO.
• -consuming high-fat foods: additional enzyme is given
• -ENTERIC-COATED PANCREATIC ENZYME should NOT be crushed or chewed; capsules can be taken apart and can be sprinkled on a small amt of food,.
• -WOF: constipation, intestinal obstruction and rectal prolapse.
• -WOF: signs of GERD; (+)GERD: place infant in an upright position and sit upright after eating.

• -monitor: glucose levels and signs of DM
• -adequate salt and fluid intake (esp on hot weather or if a child has a fever).
• -monitor bone growth 
• -monitor for signs of retinopathy or nephropathy.

• -child receives immunization at the right time.
• -annual influenza vaccine is recommended.