cystic fibrosis

what organ systems does cystic fibrosis affect?

respiratory, GI, and reproductive. it is a multi-system disease

what does CF most notably cause?

chronic lung disease and pancreatic insufficiency

who is most likely to be born with CF?

caucasians that are male

CF is a disorder of the ________ glands

exocrine

how does someone get CF?

it is genetic, an inherited autosomal recessive disorder

what does CF primarily produce?

thick mucous

what does the excess mucous do to the pancreas?

blocks the pancreatic ducts

what does CF do to the respiratory system?

causes increased mucous production in airways causing obstruction and stasis of fluid and a rich habitat for bacterial growth

when is CF diagnosed?

at brith or within the first year of life

what is seen in sweat due to CF?

excessive salt since reabsorption is impaired

respiratory disease-

reduced rate of mucous clearance, accumulation of thick, purulent sputum, recurrent respiratory infections, progressive loss of pulmonary function

what resistant bacterial bugs do many CF patients have?

MRSA, and burkholderia cepacia

CF respiratory symptoms

crackles, wheezing, diminished breath sounds, productive cough, tachypnea, and hypoxia

what does CF do to the digestive system?

causes the pancreatic ducts to be blocked by mucous, and creates an insufficiency in digestive enzymes to digest foods (fats and protiens)

What must you monitor in children with CF?

their weight to ensure adequate growth because of malabsorption of nutrients, failure to thrive, and overall malnutrition/poor growth.

what is meconium ileus?

the first stool of an infant and it is thick and sticky-creates a bowel obstruction

what does steatorrhea mean?

fatty, sticky stool

why will some CF patients become insulin dependent?

because of pancreatic involvement and long term damage, some patients will have CF related diabetes

diagnostics for CF

sweat chloride test, identify CF gene, newborn screen

what sweat chloride test range is suspicious?

40-60 meq/L- 60 meq/L above diagnostic

what is the goal of CF treatment?

ensure repiratory function, enhance nutrition and promote growth and development

what nebulized meds are used for CF?

bronchodilators

when is a chest CPT (percussion and postural drainage) done?

before meals to prevent vomiting

what is Pulmozyme (Dornase alpha) used for?

it is used to thin mucous- it is a mucolytic enzyme given via inhalation to thin mucous

what are PO digestive enzymes used for?

to facilitate absorption of fats and nutreints

how often are PO digestive enzymes given to a patient with CF?

with every meal and snack

what is megestrol acetate used for?

to increase appetite

-progestin used as appetite stimulant for children with malnutrition

what do Cystic Fibrosis Transmembrane Conductance Regulators (CFTR's) do?

enhance lung function by targeting faulty proteins to assist with sodium and chloride transportation in and out of cells

adverse effects of CFTR's

increased liver enzymes, hepatoxicity, increased bilrubin, cataracts, nasal congestion

contraindications for CFTR's

hepatic impairment, pregnancy

what should you administer with CFTR's?

a fat containing meal or snack

when can a CF patient begin to take CFTR's?

when they are two years old or older

example of a CFTR

elexacaftor, tezacaftor , and ivacaftor (Trikafta)- an oral triple combination medication

what vitamins should be involved in CF treatment?

fat soluble (A,D,K,E)

what is very crucial for a patient with CF?

infection control to decrease illness exposure

what kind of diet does a patient with CF need?

a well-balances, high protien, and high caloric diet

what will most CF patients require (nutritionally)?

supplemental nutrition via G-tube feedings

what must CF patients have for high dose IV antibiotics?

a central line, most have an implanted central line port

recurrent hospitalizations for management of CF involves

scheduled tune ups and for acute illnesses

social implications for CF-

family disruptions, school, social development, body image, hospital routines becoming a major part of life, community resources