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RCP 115 Ch 15
Cystic Fibrosis
| Question | Answer |
|---|---|
| What are some anatomic alterations of the lungs for someone who has CF? | - Excessive production and accumulation of thick, tenacious mucus in the tracheobronchial tree. - Partial bronchial obstruction (mucus plugging). - Hyperinflation of alveoli. - Total bronchial obstruction (mucus plugging). - Atelectasis. |
| What is the most common fatal inherited disorder in childhood? | CF |
| CF is an autosomal recessive gene disorder caused by? | - Mutation in a pair of genes located on chromosome 7 |
| There are over 1700 different mutations in the gene that encodes for what? | Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). |
| What is the abnormal expression of the CFTR results? | Abnormal transport of sodium and chloride ions across many types of epithelial surfaces. |
| Another abnormal expression of the CFTR results in? | - Thick viscous mucus accumulation in the lungs. - Mucus blocks the passageways of the pancreas, preventing enzymes from the pancreas from reaching the intstines. |
| Six classes of CFTR Mutations | - Class I: Stops. - Class II: protein Defect. - Class III: Gating Defect. - Class IV: Conductance Defect. - Class V: Limited Quantities. - Class VI: Accelerated Turnover. |
| Six classes of CFTR Mutations; Divided into three broad catagories: | 1) Class I and II; little or no functional CFTR. 2) Class III and IV; the function of CFTR at the cell surface is affected. 3) Class V and VI; reduced QUANTITY of FUNCTIONAL CFTR protein. |
| What is the GATING DEFECT (e.g., Class III) of CFTR? | Where the channel does not open. |
| What is the CONDUCTING DEFECT (e.g., Class IV) of CFTR? | Where the channel is open but chloride does not move efficiently. |
| How the CF Gene is inherited; What type of gene disorder is CF? | Recessive |
| How the CF Gene is inherited; How many copies of the defective CF Gene does a child must inherit to have the disease? | - Two copies: One from each parent ( CF carriers). |
| How the CF Gene is inherited; If both parents carry the CF Gene, the possibility of their children having CF follows what standard? | The Mendelian Pattern |
| How the CF Gene is inherited; How many Americans are unknowing, symptomless carriers of a mutant CF Gene? | More than 10 million (estimated) |
| What are screening and diagnosis based on? | - Clinical manifestation associated with CF. - Family history of CF. - Laboratory findings. |
| What two categories must be met to diagnose CF? | 1) Clinical symptoms consistent with CF in at least one organ system. 2) Clinical evidence of cystic fibrosis CFTR dysfunction. |
| What is the Clinical Evidence of Cystic Fibrosis CFTR dysfunction? | - Elevated sweat chloride greater than 60 mEq/L (on two occasions). - Molecular diagnosis (genetic testing). - Abnormal nasal potential difference. |
| Molecular diagnosis (genetic testing) has the presence of what? | Two disease-causing mutations in CFTR. |
| Most infants with CF have elevated blood levels of what? | Immunoreactive trypsin (IRT). |
| How is the immunoreative trypsin measured? | From the blood dots collected on all newborn infants on the Guthrie cards. |
| What should all families of infants identified to have CF through newborn screening programs should receive? | Genetic counseling. |
| What is the Gold Standard Diagnostic Test for CF? | Sweat Test |
| The Sweat Test is a reliable test for the identification of what percent of patients with CF? | About 98% |
| The sweat test measures what? | Amount of Na and Cl in patients sweat. |
| What is Pilocarpine? | colorless, odorless, sweat producing chemicals. |
| How many times is the sweat test done? | Twice |
| What is the sweat test sometimes called? | Sweat Chloride Test |
| Patients with CF, sweat glands secrete up to about how much sodium and chloride? | Up to 4 times the normal amount. |
| All patients with the following characteristics should undergo a sweat test to help confirm the diagnosis of CF | - Infants with positive CF newborn screening results. - Infants with symptoms suggestive of CF. - Older siblings with symptoms suggestive of CF. - Members of the patient's family with confirmed CF. |
| When should newborn screening sweat test for CF be performed? | After 2 weeks of age and greater than 2 kg if asymptomatic. |
| Sweat Test Interpretations; Infants 6 months or younger: NORMAL (CF very unlikely) = | < or = to 29 mmol/L |
| Sweat Test Interpretations; Infants 6 months or younger: Intermediate (Possible CF) = | 30- 59 mmol/L |
| Sweat Test Interpretations; Infants 6 months or younger: Abnormal ( Diagnosis of CF) = | > or = to 60 mmol/L |
| Sweat Test Interpretations; Infants older than 6 months, children, and adults: Normal (CF unlikely) = | < or = 39 mmol/L |
| Sweat Test Interpretations; Infants older than 6 months, children, and adults: Intermediate (Possible CF) = | 40- 59 mmol/L |
| Sweat Test Interpretations; Infants older than 6 months, children, and adults: Abnormal (Diagnosis of CF) = | > or = 60 mmol/ L |
| With a sample of the patient's blood or cheek cells, a genetic test can be preformed to do what? | Analyze DNA for the presence of CFTR gene mutations. |
| Intermediate results of sweat chloride testing should be further investigated with what? | DNA analysis |
| What are some other test that are done to determine if someone has CF? | - Genetic testing. - Nasal potential difference. - Prenatal testing. - Stool fecal fat testing. |
| The impaired transport of Na+ and Cl- ions move across the epithelial cell membrane, lining the airways of CF patients generate what is called? | Electrical potencial difference |
| In the nasal passages the electrical potencial difference is called? | Nasal potencial difference (NPD). |
| Prenatal Testing: True or False; If the mother of the fetus tests positive for CF mutation, the farther of the fetus can be tested | True |
| If both parents test positive for CF mutation, what chance does the fetus have for having CF? | 1 in 4 chance. |
| True or False: Genetic Counseling is very important? | True |
| What does the Stool Fecal Fat Test measure? | The amount of fat in the infant's stool and the percentage of dietary fat that is not absorbed by the body. |
| Stool Fecal Fat Test is used to evaluate what? | How the liver, gallbladder, pancreas, and intestines are functioning. |
| Infants with CF and pancreatic insufficiency will have a fecal elastance of? | < 50ug/ g of stool. |
| What is a normal fecal elastance ? | >300ug/ g of stool |
| The Cardiopulmonary Clinical Manifestations; What would you see in someone with CF? | - Atelectasis. - Bronchospasms. - Excessive bronchial secretions. |
| Physical Examination for CF; Vital signs: | Increased: - RR (Tachypnea) - HR (Pulse) - B/P |
| Physical Examination for CF; Use of accessory muscles: | - Either Inspiration or expiration. - Pursed lip breathing. - Increased anteroposterior chest diameter (barrel chest). - Cyanosis. - Digital clubbing. - Cough, sputum production, and hemoptysis. |
| Physical Examination for CF; Chest assessment finding: | - Decreased tactile and vocal fremitus. - Hyperresonant percussion note. - Diminished breath sounds. - Bronchial breath sounds (over atelectasis). - Crackles. - Wheezing. |
| What is the Physical Examination for CF; | - Spontaneous Pneumothorax. - Chest Assessment. - Vital Signs. - Use of accessory muscles of inspiration or expiration. - Pursed lip beathing. - Increased anteroposterior chest diameter (barrel chest). - Cyanosis. - Digital clubbing. - Cough, |
| PFT findings moderate to severe CF; (Obstructive Lung Patho.) Forced Expiratory Volume and Flowrate Findings: | FVC, FEVt, FEV1 / FVC ratio, FEF 25%- 75 ↓ ↓ ↓ ↓ FEF 50%, FEF 200- 1200, PEFR, MVV ↓ ↓ ↓ ↓ |
| PFT findings moderate to severe CF; (Obstructive Lung Patho.) Lung Volume and Capacity Findings: | Vt, IRV, ERV, RV - N or ↑, N or ↓, N or ↓, ↑ Vc IC FRC TLC RV/TLC ratio - ↓, N or ↓, ↑, N or ↑, N or ↑ |
| Arterial Blood Gases: CF | pH. PaCO2. HCO3. PaO2. SaO2/SpO2 ↑. ↓ ↓. ↓. ↓ (but normal) |
| ABG: CF Severe Stage: Chronic Ventilatory Failure with Hypoxemia (Compensated Respiratory Acidosis) | pH. PaCO2 HCO3 PaO2. SaO2/SpO2 N ↑. (Significantly) ↓. ↓ |
| ABG; Acute ventilatory changes superimposed on chronic ventilatory failure: | Because acute ventilatory changes are frequently seen in patients with chronic ventilatory failure. |
| The respiratory therapist MUST be familiar with- and ALERT for the 2 dangerous ABG findings: | 1. ACUTE Alveolar HYPERventilation superimposed on Chronic Ventilatory Failure (possible impending acute ventilatory failure). 2. Acute Ventilatory Failure (Acute HYPOventilation) superimposed on CHRONIC ventilatory failure. |
| Abnormal Lab. Tests and Procedures: Hematology; | - Increased hematocrit and hemoglobin. - Increased WBC. |
| Abnormal Lab. Tests and Procedures: Electrolytes; | - Hypochloremia; (CHRONIC Ventilatory Failure). - Increased serum Bicarbonate (CHRONIC Ventilatory Failure). |
| Abnormal Lab. Tests and Procedures: Sputum examination= Gram- Positive Bacteria: | - Staphylococcus. - Haemophilus influenzae. |
| Abnormal Lab. Tests and Procedures: Sp[utum examination= Gram-Negative Bacteria: | - Pseudomonas aeruginosa. - Stenotrophomonas maltophilia - Burkholderia Cepacia Complex. |
| Radiologic Findings: Chest Radiograph= | - Translucent (dark) lung fields. - Depressed or flattened Diaphragm. - Right ventricular enlargement. - Areas of Atelectasis and fibrosis. - Tram-tracks. - Bronchiectasis (often a secondary complication). - Pneumothorax (spontaneous). - Absce |
| Common Non respiratory Clinical Manifestations: | - Distal intestinal obstruction syndrome (DIOS). - Malnutrition and poor body development. |
| What are some manifestations with malnutrition and poor body development? | - Deficiencies of vitamins A, D, E, and K. - Nasal polyps and sinusitis. - Infertility (Males). (pancreas not working properly). |
| General Management of CF; What is the primary goals for CF? | - Prevent pulmonary infections. - Reduce the amount of thick bronchial secretions. - Improve air flow. - Provide adequate nutrition. |
| General Management of CF; Who should be instructed regarding the CF disease and the way it affects bodily functions? | The patient and the patients family. |
| What are the Respiratory Care Treatment Protocols? | - Oxygen Therapy. - Airway clearance therapy protocol (vest). - Lung expansion therapy. - Aerosolized medications. - Mechanical ventilation protocol. - Other medications and procedures by the physician. |
| What are the other medications and special procedures by the physician for the Respiratory Care Treatment Protocols? | - Correctors. - Potentiators. - Antibiotics. - Ibuprofen. - Inhaled corticosteroids and systemic glucocorticoids. - Lung or Heart- Lung transplantation. |
| Respiratory Care Treatment Protocols: What are two aerosolized medications? | - Bronchodilators. - Mucolytic agents. |
| What are some Mucolytic agents? | - Inhaled Dnase (Dornase alpha) (Pulmozyme). - Inhaled hypertonic saline. - Inhaled N- acetylcysteine (Mucomyst). |
| Respiratory Care Treatment Protocols: Correctors (medication) | Drugs which help mutated CFTR reach the epithelial cell surface where CFTR protein normally functions as a transmembrane regulator of chloride movement out of the cell and sodium transport into the cell. |
| Respiratory Care Treatment Protocols: Potentiators (medication) | Drugs which help mutated CFTR function more effectively at the epithelial cell surface transporting the movement of sodium into the cell. |
Created by:
tracyb34
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