Cystic Fibrosis 08/2015 Flashcards
1
Q
How is CF caused?
A
- Must inherit 2 copies of defective gene (found on chromosome 7), 1 from each parent., to show symptoms.
- It’s a gene mutation and malfunction; defective form or protein called CFTR produced in the cells that line the lungs, pancreas, sweat glands and other systems
- 25% chance
2
Q
How does CF affect organ function?
A
- LUNGS:
- mucus is thick, sticky and hard to move
- traps germs that cause infections
- mucus is thick, sticky and hard to move
- PANCREAS:
- thick, sticky mucus blocks the channels that carry digesting enzymes to intestines
- prevents processing and proper absorption of nutrients
- thick, sticky mucus blocks the channels that carry digesting enzymes to intestines
3
Q
CFTR malfunction leads to…?
A
- impaired water secretion
- salty sweat
- abnormally thick exocrine secretion
Normally the CF proteins allow salt (chloride) to leave the lung cells and enter air space, and the water follows by osmosis which dilutes the mucus layer. With the mutation, neither enters the air and mucus layer is concentrated and sticky–>
- CLOGGING THE LUNGS AND LEADING TO RESPIRATORY INFECTIONS AND INFLAMMATION
- OBSTRUCTS THE PANCREAS DUCTS, PREVENTING DIGESTIVE ENZYMES FROM GOING TO INTESTINES
4
Q
What is current median age survival?
A
Age 37
5
Q
Signs & Symptoms of CF
A
- very salty-tasting skin
- persistent coughing, frequently with phlegm
- frequent lung infections (pneumonia & bronchitis)
- wheezing or SOB
- poor growth (d/t malabsorption)
- weight loss in spite of good appetite
- frequent greasy, bulky stools OR difficulty with bowel movements
6
Q
When is CF diganosed?
A
1st year of life
Most commonly thru routine newborn screening:
Sweat Chloride Test (SCT) is the “Gold Standard”
- colorless, odorless chemical applied to arm or leg, and electrode applied to area stimulates sweating; which is collected and lab tested
- Greater than 60 mmol/L = CF diagnosis
7
Q
What changes occur in the Upper Respiratory Tract from CF?
A
- Nasal polyps are soft, painless, noncancerous growths on the lining of your nasal passages or sinuses, result from chronic inflammation
- block drainage from nose and sinuses
- sinusitis from trapped bacteria causing infection
8
Q
Symptoms of Pulmonary Exacerbation
A
- Pulmonary exacerbations is a change in respiratory s/s from baseline
- Tx with ABX and augmented airway clearance
Symptoms:
- ↑ cough and/or sputum production
- Change in quality of sputum
- ↓ exercise tolerance
- Loss of wt or appetite
- ↓ forced vital capacity (FVC) or forced expiratory volume (FEV) of >10%
- onset of new or increased crackles
Other findings:
- ↑ RR, ↓ O2 Sat, use of accessory muscles for respiration
- Fever, leukocytosis (↑WBC)
- Auscultatory changes
- chest-imaging changes
9
Q
When should pt be admitted to hospital?
A
- A loss of 10% FEV or greater
- Require rapid, aggressive tx (which CFF findings show help maintain lung fx longer)
- GOAL: prevent airway blockage and control infection
- **the PO antibiotics commonly used for suppression therapy are not effective during exacerbations d/t bacterial resistance
10
Q
Sputum C&S
A
- must be obtained with each exacerbation
- must be transported to lab w/in 3hrs
- Most common pathogens:
- pseudomonas aeruginosa
- Staph. Aureus
- MRSA
- B. Cepacia
11
Q
CF Program Team
A
- 2 physicians
- 2 RN
- 1 RT
- 1 Dietician
- 1 Admin Asst
12
Q
CF Program: Respiratory Mgmt
A
- Quarterly evaluation of pulmonary status
- LT use of maintenance therapies (antibiotics/antifungals)
- Adherence to consistent infection conrol procedures
- Tx regimen:
- Oral medications: 30 min daily
-
Inhaled medications: 3.75 hr daily
- Mucolytics are a form of inhaled medications that help thin the mucus in the lungs so people can cough it out more easily. CF causes damage to the lungs even when there are no symptoms. Getting this mucus out of the lungs helps slow the damage caused by chronic lung infections.
- Tobi 45 min BID
- Pulmozyme 45 min daily (aka Dornase alfa)
- comes in sterile, single-use ampules or containers. It contains no preservatives, so once it’s opened the entire contents must be used or thrown away.
- Hypertonic saline 45 min BID
- Mucolytics are a form of inhaled medications that help thin the mucus in the lungs so people can cough it out more easily. CF causes damage to the lungs even when there are no symptoms. Getting this mucus out of the lungs helps slow the damage caused by chronic lung infections.
- Airway clearance therapy: 1 hr minimum daily
- CFRD mgmt, if needed
- Total Tx > 5hrs minimum when pt is well
- w/ CFRD mgmt > 6hrs
-
w/ IV therapy > 8 hrs daily
- doses higer d/t altered pharmacokinetics by CF
- Two-drug combo to reduce risk of inducing resistance
- clinical response not seen for 4-7 days after tx begins
- Tx necessary for 2-4 wks
13
Q
Common Parenteral Antibiotics
A
-
Tobramycin
-
watch for s/s of ototoxicity
- tinnitus, vertigo, nausea, hearing changes
- pt may report “my ear feels full”
-
watch for s/s of ototoxicity
- Ceftzidime - has been used via inhalation (off label)
-
Cefazolin (1g q12h)
- tx sensitive staph
- Vancomysin, Zosyn, Meropenu, Aztreonam
14
Q
Long-term antibiotics/antifungals
A
- Azithromycin 500mg PO every MWF
- LT ABX helps decrease incidence of resistance
- Inhaled Tobi
- only give if pt grows pseudomonas
- Bactrim DS
- only give if pt grows staphylococcus
- Sporanox
15
Q
Airway Clearance
A
4 tx/day while INPATIENT
- Chest physiotherapy
- IPV
- Exercise (encourage pt to get OOB and walk!!)
- Flutter or acapella valve
- “The Vest”
**If pt refuses 2 consecutive respiratory tx, the RT will call CF RN coordinator who will then educate the pt. If refusal continues, pt is d/c from hospital.
