Lower Respiratory Tract Disorders Flashcards

Lower Respiratory Tract Disorders

Disorders that occur in the lower portion of the trachea, bronchi, bronchioles, and alveoli.

Acute Bronchitis

• Inflammation of the bronchial tree: right and left bronchi, secondary bronchi, and bronchioles.

• Mucous membranes lining the bronchial tree become irritated and inflamed, causing excess mucous production, leading to congested airways.

• Usually an isolated episode caused by a virus.

• Chronic bronchitis is diagnosed if it occurs more than 3 months out of the year for 2 consecutive years.

Bronchiectasis

• Areas of dilation of the bronchial airways become flabby and scarred.

• Can remain localized or spread throughout the lungs.

• Secretions pool in these areas where bacteria can grow, leading to infection. These pooled secretions are hard to cough up.

Causes

• Usually occurs secondary to another chronic respiratory disorder:

  • Cystic Fibrosis

  • Asthma

  • TB

  • Chronic Bronchitis

• Infection and inflammation in these underlying disorders weaken the bronchial walls and reduce ciliary function.

• Airway obstruction from excessive secretions dilates the airways, which then become flabby and scarred, resulting in bronchiectasis.

Signs and Symptoms

• Dyspnea occurs if the condition is widespread throughout the lungs.

• Chronic cough (very loud and forceful).

• Copious and purulent sputum:

  • May be bloody due to extreme airway inflammation

  • During a coughing episode, a patient can produce as much as 200 mL of thick, foul-smelling sputum.

• Recurrent Lower Respiratory Infections

• Clubbing of the fingertips.

• Cor Pulmonale (right-sided heart failure) can develop with chronic disease due to increased pressure in the lungs from inflammation.

• Crackles and Wheezes.

• Fever with active infection.

Diagnostic Tests

• Chest X-Ray (may not show early disease).

• CT of Chest (provides a better view of the dilated airways).

• Sputum culture to determine infecting organism and guide antibiotic therapy.

• Bronchoscopy.

Treatment

• Aimed at keeping the airways clear of secretions, controlling infection, and correcting the underlying problem.

• Antibiotics:

  • Used intermittently or for prolonged periods

  • Azithromycin (Zithromax) may reduce exacerbations.

• Mucolytics and Expectorants:

  • Loosen and mobilize secretions so they can be coughed up.

• Bronchodilators improve airway obstruction.

• Chest physiotherapy to mobilize secretions.

• Oxygen if hypoxemia is present.

• Surgical Resection:

  • If the affected area of the lung is localized and symptoms are severe, surgery may be considered to remove the diseased area.

• Lung transplant may be considered in severe cases.

Pneumonia

• Persons at greatest risk:

  • The very young

  • Adults over age 65

  • People who are immunocompromised (AIDS, Alcoholism, any other illness that causes immunosuppression)

  • Smokers

  • Those with chronic diseases

Categorization

• Categorized according to where it is acquired:

  • HAP = hospital acquired pneumonia:

    • Pneumonia that develops at least 48 hours after a hospital admission (i.e., ventilator-associated pneumonia).

  • HCAP = healthcare associated pneumonia:

    • Pneumonia that develops in outpatient settings like nursing homes, assisted-living facilities, dialysis clinics, etc.

  • CAP = community acquired pneumonia:

    • Pneumonia that develops in the community and is usually less serious than the other forms.

Pathophysiology

• Acute inflammation and/or infection occurs when an infectious agent enters and multiplies in the lungs of a susceptible person.

• Microorganisms release toxins that induce inflammation in the lung tissue, causing damage to mucous and alveolar membranes.

• Leads to the development of edema and exudate, which fills the alveoli and reduces the surface area available for exchange of carbon dioxide and oxygen, ultimately affecting gas exchange.

Causes

• Bacterial:

  • CAP usually caused by Streptococcus pneumonia (pneumococcal pneumonia).

  • HAP often antibiotic-resistant; tend to be more serious than CAP; caused by E. coli, H. influenzae, K. pneumoniae.

• Viral:

  • Influenza viruses are the most common cause.

  • Patients are less ill with viral pneumonia than bacterial.

• Fungus:

  • Candida, Aspergillus, Pneumocystis jiroveci (seen in AIDS).

• Aspiration.

• Ventilator-Associated Pneumonia (VAP):

  • A type of aspiration pneumonia that develops in patients who are intubated and mechanically ventilated.

  • Secretions are easily aspirated into the lungs because the endotracheal tube keeps the glottis open.

• Chemical:

  • Inhalation of toxic chemicals can cause inflammation and tissue damage, leading to chemical pneumonia.

Prevention

• Flu, COVID, and pneumonia vaccinations are essential to preventing pneumonia, especially for people with chronic illnesses such as kidney disease, heart or lung diseases, and immunosuppression.

• Regular coughing, deep breathing, and position changes for patients on bedrest or after surgery.

• Prevention of aspiration for patients at risk.

• Mouth care and good hand hygiene by patients and HCP.

• Frequent suctioning for patients on ventilators and maintaining the HOB at 30-45 degrees at all times.

Signs and Symptoms

• Fever

• Chills

• Chest pain

• Dyspnea

• Productive cough:

  • Purulent, rust-colored, or blood-tinged

• Crackles and wheezes

• Atypical symptoms:

  • Fatigue

  • Sore throat

  • Dry cough

  • Nausea/Vomiting

• New-onset confusion, lethargy, fever, or dyspnea in elderly indicates reduced oxygenation.

Complications

• Pleurisy and Pleural Effusion:

  • Two of the most common complications

  • Generally resolve within 1-2 weeks

• Atelectasis (collapsed alveoli):

  • Occurs as a result of trapped secretions

• Spread of infection to other parts of the body:

  • Septicemia, Meningitis, Septic Arthritis, Pericarditis, Endocarditis

• Treatment for each of these is antibiotics.

• Most complications from pneumonia occur most commonly in patients with other underlying chronic diseases.

Diagnostic Tests

• Chest X-ray:

  • White areas in the lungs indicate pulmonary infiltrate, which is fluid leakage into the alveoli from inflammation.

• Sputum & Blood Cultures:

  • Done to identify the causative organism and determine which antibiotic will be most effective in treatment.

Treatment

• For bacterial pneumonia, broad-spectrum antibiotics are started first until the culture and sensitivity reports are back; then antibiotics may be changed to more narrow-spectrum agents.

• For viral pneumonia, rest and fluids are recommended.

  • Occasionally antiviral meds are used to shorten the severity and duration of virus.

• Expectorants, bronchodilators, and analgesics for comfort and symptom relief.

• Supplemental oxygen via nasal cannula or mask as needed.

Nursing Diagnoses

• Impaired Gas Exchange

• Ineffective Airway Clearance

• Activity Intolerance

Tuberculosis (TB)

Pathophysiology and Cause

• Caused by the bacteria Mycobacterium tuberculosis, an acid-fast bacillus (AFB).

• Primarily affects the lungs.

• Spread by inhalation of the bacilli from respiratory droplets of an infected person.

• Once bacilli enter the lungs, they multiply and begin to spread to the lymph nodes and then to other parts of the body.

• Patient is then infected but may or may not go on to develop active disease.

• TB infection without disease is called latent TB infection.

• During this time, the body develops immunity, which keeps the infection under control (the bacteria become dormant).

• If the patient’s immune system becomes compromised (as in the elderly), some of the dormant bacteria can become active, which in turn causes active disease.

• Only 5-10% of infected people in the US actually develop TB, largely due to treatment after a positive TB skin test.

Risk Factors

• Inmates and employees of prisons

• Nursing home residents

• People living in homeless shelters

• People in drug treatment facilities

• People in health-care facilities

• Older people

• People with AIDS

• Chronic drug or alcohol users

• People who are taking certain medications (i.e., chemotherapy and some medications for rheumatoid arthritis, Crohn disease, or psoriasis) as these can compromise immune function and increase risk of activation.

Signs and Symptoms of Active TB

• Chronic productive cough of blood-tinged sputum longer than 2-3 weeks in duration

• Drenching night sweats

• Pain in the chest

• Fatigue

• Swollen lymph nodes

• Poor appetite

• Weight loss

• Low-grade fever

• If effective treatment is not started, the patient quickly declines, and pulmonary fibrosis (scarring), hemoptysis, and progressive weight loss occurs.

Diagnostic Tests

• Routine screening is usually done with a PPD (purified protein derivative) skin test:

  • Test is considered positive if a raised area of induration occurs within 48 to 72 hours; this induration is measured in mm.

  • A positive result simply means that a person has been exposed to TB; it DOES NOT mean that they have active TB (symptoms must be present to have active TB).

  • Red area without induration is a negative result.

• QuantiFERON-TB Gold and T-SPOT tests:

  • Newer blood tests that detects the cell-mediated immune response to TB bacteria in blood.

• Chest X-ray:

  • Used as a screening tool in someone with a known positive test.

• Sputum Culture for AFB:

  • Definitive diagnosis of active TB is made based on culture results.

Treatment

• For both active and latent TB:

  • Combination of medications is given for 6-9 months or up to 2 years

    • Isoniazid or INH

    • Rifampin

    • Ethambutol

    • Pyrazinamide (newer med)

    • Vitamin B6

• INH and Rifampin are generally first-line tx for someone who has had a positive TB skin test (whether they are in just latent TB or active TB).

  • Because these medications can cause neuropathy, most patients will also be prescribed Vitamin B6 as well.

  • Patient must have monthly liver enzymes checked, because elevation is common with these medications.

• Additional treatment is just supportive care:

  • Rest

  • Good nutrition

  • Respiratory isolation is continued until their sputum no longer contains TB bacteria.

  • Typically, after about 2 weeks on antibiotic therapy, the patient is no longer contagious, but a sputum culture must be done to confirm this.

Prevention of TB Spread

• Respiratory isolation of patients who have suspected or active TB.

• Negative-pressure isolation rooms that are ventilated to the outside.

• High-efficiency filtration masks (N-95 mask).

• If patient must travel through the hallways for tests or other activities, they must wear a N-95 mask.

• Gowns, gloves, goggles if contact with sputum is likely.

• Prevention will come from adequate treatment.

• A current concern is the development of antibiotic-resistant strains of the TB bacillus, which can develop when patients are noncompliant with drug therapy.

Restrictive Respiratory Disorders

• Lung conditions that limit the ability of the patient to expand his or her lungs and inhale air properly.

• Examples of Restrictive Disorders:

  • Pleurisy

  • Pleural Effusion

  • Empyema

  • Pulmonary Fibrosis

  • Atelectasis

Pleurisy/Pleuritis

• Inflammation of visceral and parietal pleurae, causing friction on inspiration.

• Causes sharp pain on inspiration.

• Usually caused by pneumonia, TB, lung cancer, and pulmonary embolisms.

Signs and Symptoms

• Sharp pain in the chest on inspiration.

• Shallow, rapid breathing because deep breathing increases pain.

• Fever, chills, elevated WBC if the cause is infectious.

• Pleural friction rub heard on auscultation.

Diagnostic Tests

• Chest X-ray or CT scan

• CBC

• Additional tests to determine the underlying cause.

• Diagnosis is usually based on signs and symptoms, including auscultation of a pleural friction rub.

Treatment

• Aimed at correcting the underlying cause (i.e., pneumonia, TB, lung cancer, pulmonary embolism).

• NSAIDS or opioids given to control pain and facilitate deep breathing and coughing.

• If treatment is not started, these complications can occur:

  • Pleural effusion (as pleural membranes become more inflamed, serous fluid production increases, causing the effusion).

  • Atelectasis (because of pain associated with inhalation, patients tend to hypoventilate, which can lead to collapse of lung).

  • Empyema (collection of pus in the pleural space caused by an untreated bacterial infection).

Pleural Effusion

• Condition occurs when excess fluid collects in the pleural space that cannot be adequately reabsorbed by the lymphatic system.

• Normal amount of pleural fluid around each lung = 1 to 15 mL

• 25 mL is considered abnormal.

• In pleural effusion, as much as several liters of fluid can collect at one time.

Causes

• Transudative fluid (watery fluid from capillaries):

  • Heart Failure

  • Liver or Kidney Disease

  • Clear fluid

• Exudative fluid (fluid containing WBCs and protein from inflammation or infection):

  • Pneumonia

  • TB

  • Lung cancer

  • Cloudy fluid

Signs & Symptoms

• Symptoms depend on the amount of fluid in the pleural space.

• May or may not experience pleuritic pain.

• Increasing shortness of breath because of decreasing space for lung expansion.

• Cough

• Tachypnea

• Dull sound heard when the affected area is percussed.

• Lung sounds decreased or absent over effusion.

• May or may not hear pleural friction rub upon auscultation.

Diagnostic Tests

• Chest X-ray or CT scan:

  • Done to determine whether pleural effusion is present.

• Thoracentesis:

  • Done to drain the pleural effusion.

  • Fluid samples are sent to the lab for culture and sensitivity and cytological examination.

Treatment

• Underlying cause should be treated:

  • Diuretics for heart, liver, and kidney failure patients

  • Antibiotics for bacterial pneumonia

• Therapeutic thoracentesis to remove excess fluid and relieve dyspnea.

• If fluid accumulation is large or recurring, a chest tube might be placed to continuously drain pleural space.

Pulmonary Fibrosis

• Lung condition characterized by scarring and fibrosis (hardening) of lung tissue.

• May evolve from injury to the alveoli, causing a chronic inflammatory response.

  • Chronically inflamed lung tissues are then gradually replaced by fibrous connective tissue.

• Alveoli become thick and scarred.

• Gas exchange becomes difficult.

Causes

• Heredity

• Exposure to certain viral illnesses

• Wood and metal dust exposure

• Medications

• Radiation therapy

• Smoking

• Associated with certain autoimmune disorders:

  • Lupus, Rheumatoid Arthritis (these autoimmune disorders have a common characteristic of widespread inflammation throughout the entire body).

• Often called “Idiopathic pulmonary fibrosis” because no specific cause can be found.

Signs and Symptoms

• Progressive shortness of breath

• Inspiratory crackles

• Chronic cough

• Fatigue

• Clubbing of fingers (late sign)

• Average person has a gradual decline in lung function with eventual respiratory failure and death in approximately four years.

Diagnostic Tests

• Chest X-ray may show lung infiltrates.

• CT scan of the chest.

• ABGs may show decreased PaO2 levels.

• Bronchoscopy with lung tissue biopsy can help rule out other causes of symptoms and can show inflammation and fibrosis.

• ANA (antinuclear antibodies) titer may show whether an autoimmune process is involved, such as with lupus and RA.

Treatment

• Two new antifibrotic meds, pirfenidone (Esbriet) and nintedanib (Ofev), can reduce disease progression and preserve lung function (steroid and immune suppressant).

• Smoking Cessation

• Oxygen to maintain oxygenation

• Annual flu/pneumonia vaccines

• Pulmonary rehabilitation to maintain optimum activity tolerance

• Lung transplant for younger patients

Atelectasis

• Atelectasis is the collapse of alveoli.

Causes

• Hypoventilation (most commonly occurring in postsurgical patients who do not cough and deep breathe effectively):

  • Areas of the lung become plugged with mucus, which prevents inflation of alveoli, and as a result, the alveoli collapse.

• Compression from pleural effusion or tumor can also cause atelectasis.

Signs and Symptoms

• Fine Crackles

• Diminished Breath Sounds

• Dyspnea

Treatment

• The focus of nursing care is on preventing atelectasis from happening in the first place by:

  • Doing cough and deep breathing exercises

  • Using an incentive spirometer properly

  • Turning q2hrs

  • Ambulating as ordered

Obstructive Respiratory Disorders

• Characterized by air being trapped in the alveoli, which makes getting the air out of the lungs very difficult.

• Examples of Obstructive Disorders:

  • COPD

    • Emphysema

    • Chronic Bronchitis

    • Asthma

  • Cystic Fibrosis

COPD

• Group of pulmonary disorders characterized by difficulty exhaling because of airways that are narrowed or blocked by inflammation and mucous.

• More effort is required for the weakened alveoli to push air out.

• Air becomes trapped.

• Often times a patient with COPD may have some degree of BOTH emphysema and chronic bronchitis.

• Asthma may also be present, but it differs somewhat because the airway limitation is usually reversible.

• With chronic bronchitis and emphysema, it is NOT reversible.

• COPD develops slowly and may be present for many years before symptoms become evident, and it may be advanced by the time the patient seeks treatment.

• COPD is characterized by periods of remission and exacerbations, which are periods of acute worsening of symptoms.

• These exacerbations are often triggered by respiratory infections.

Chronic Bronchitis

• Chronic inflammation of the airways from inhaled irritants.

• Chronic low-grade infection.

• Hypertrophied mucous glands in bronchi produces excessive thick mucous, which obstructs airways and traps air.

• Impaired ciliary function
  decreases ability to remove inhaled irritants, creating Ineffective Airway Clearance.

• Diagnosed after a patient has had bronchitis 3 months of the year for 2 consecutive years with multiple exacerbations, each lasting 2 weeks or more.

Emphysema

• Caused by destruction of alveolar walls, loss of elastic recoil of the lungs, and damage to pulmonary capillaries.

• Causes air trapping and reduced surface area for gas exchange.

• Creates impaired gas exchange.

Causes of Chronic Bronchitis and Emphysema

• Smoking (single most important risk factor).

• Passive or second-hand smoke exposure.

• Indoor and outdoor air pollution.

• Exposure to industrial chemicals, dust & fumes.

• Some individuals have an inherited deficiency of the enzyme alpha-antitypsin (\alpha1AT), which causes a predisposition to the development of emphysema.

  • Patients with this inherited tendency who also smoke have a very high risk of developing the disease.

Signs and Symptoms of Chronic Bronchitis and Emphysema

• Chronic cough with sputum production (chronic bronchitis):

  • Wheezing and crackles heard on auscultation that may improve after coughing

  • Increased susceptibility to infection

  • Mucous plug formation that may lead to respiratory failure quickly if not resolved

• Chronic cough without sputum production (emphysema):

  • Diminished breath sounds heard on auscultation

• Progressive dyspnea on exertion

• Prolonged expiration because of obstructed air passages and reduced elastic recoil

• Barrel chest due to hyperinflation caused by air trapping

• Activity Intolerance

• ABGs reveal Increased PaCO2 and Decreased PaO2 levels

• Polycythemia (increased production of RBCs in response to chronic hypoxemia):

  • Polycythemia results in a ruddy (red) skin color in the patient (the tip of the nose and ear lobes get very red in appearance from polycythemia).

• Cyanosis may be present in late stages

• Patients may lose weight and become malnourished in late stages:

  • Difficulty eating because of severe dyspnea and increased work of breathing expends more calories

• Patients use accessory muscles to breathe.

• Tripod or Orthopneic position is classic to help with breathing.

Complications of COPD

• Cor Pulmonale:

  • Right-sided heart failure that develops because the heart has to work harder to pump blood to the diseased lungs.

• Bullae (large air spaces) can develop within the lung tissue.

• Blebs (blisters) can develop on the outside of the lung; these can rupture and may cause the lung to collapse.

• Death usually results from respiratory infection or respiratory failure

Diagnostic Tests for COPD

• Chest X-Ray

• CT Scan of the Chest

• ABGs

• CBC

• Pulmonary Function Tests:

  • Forced expiratory volume is < normal, because the patient is unable to forcefully exhale as much air as would normally be expected.

• Sputum culture

Goals of COPD Treatment

• Reduce symptoms and reduce risk of exacerbations

• Improve exercise tolerance

• Improve health status

• Prevent disease progression

• Treat exacerbations

• Reduce mortality

Treatment for COPD

• Stop smoking!!:

  • Stopping smoking can slow disease progression and prolong life

• Oxygen therapy:

  • Used to keep O2 sats between 88% and 92%

  • Ordered at a flow rate of no more than 2L/min

    • Higher flow rates may suppress the hypoxic drive in patients who are chronic CO2 retainers

  • Patients with chronic O2 sats of 88% or < should use oxygen at home

• Medications:

  • Metered-dose inhalers of bronchodilators

  • Nebulized mist treatments of bronchodilators

  • Corticosteroid inhalers to control inflammation

  • Antibiotics during times of exacerbation

  • Antitussive agents should be avoided in patients with COPD, because they need to be able to cough up secretions

  • Oral or IV corticosteroids are used for acute exacerbations (Prednisone or Solumedrol)

  • Low-dose morphine or other opioids are effective in reducing acute dyspnea & anxiety in end-stage COPD

Supportive Care for COPD

• Pneumococcal vaccine and yearly flu/COVID shots are recommended to reduce the risk of respiratory infection

• Avoid crowds and exposure to people with respiratory infections

• Good hydration and a cool mist humidifier help keep secretions loose

• Chest physiotherapy may be used to help the patient remove excessive secretions

• Dietary consult for the patient who has difficulty maintaining weight:

  • Typically, a high protein, high fat, low carb diet is ordered

• Breathing exercises help improve oxygenation and reduce anxiety:

  • Diaphragmatic and Pursed-Lip breathing

• Pulmonary rehabilitation:

  • Helps patients increase exercise tolerance and maintain a sense of well-being

• Surgery:

  • To remove a diseased part of the lung to make space for available good lung tissue to expand

  • This is a high-risk procedure and one that is rarely done

• Mechanical Ventilation:

  • If ABGs worsen despite treatment, intubation and mechanical ventilation may be necessary, depending on the patient’s advance directive

  • Weaning patients off the vent may be difficult or impossible once it is initiated

  • Use of a noninvasive positive-pressure ventilation mask like CPAP or BiPAP may be a good alternative if the patient is still able to breath on their own

• End-Of-Life Planning

  • COPD is a progressive disease, and patients can increase the quality of life and death by making decisions in advance about end-of-life care

  • Patients with end-stage COPD who go into respiratory failure and ultimately cardiac arrest are rarely saved by CPR

Asthma

• Characterized by chronic inflammation and edema of the mucosal lining of the airways and hyper- responsiveness of the bronchial smooth muscles (bronchospasm).

  • This causes narrowed airways and air trapping.

  • Inflammation occurs because allergens trigger the release of inflammatory substances like histamine and leukotrienes, which cause the asthma symptoms.

• Symptoms are intermittent and usually reversible.

• Some people develop permanent changes in their airways, called remodeling, which leads to progressive loss of lung function.

Causes

• Heredity

• Airborne Allergies:

  • Pollen

  • Mold

• Air Pollution

• Smoking

• House-dust Mites

• Cockroaches

• Cat and dog dander

Triggers for Acute Asthma Attack

• Exposure to allergens

• Viral respiratory infections

• Emotional upset

• Exercise

• Stress

• Certain meds like aspirin and beta blockers

Asthma Attack Signs and Symptoms

• Chest tightness

• Dyspnea

• Wheezing (sometimes audible)

• Cough with thick, clear sputum

• Use of accessory muscles indicates severe attack

• Symptoms may be worse at night

• Tachypnea

• Prolonged expirations because air is trapped

Complications of Asthma

• Status Asthmaticus:

  • Severe, Sustained Bronchospasm with worsening Hypoxemia

  • Respiratory Alkalosis Progresses to Respiratory Acidosis

    • Initially, the patient will increase their respiratory rate to compensate for the narrowed airways, therefore, they are blowing off a lot of CO_2 leading to respiratory alkalosis

    • The longer the attack lasts, the patient begins to tire and is no longer able to compensate; this will cause the PaCO_2 levels to rise, resulting in respiratory acidosis

  • Respiratory acidosis can lead to respiratory failure and death if untreated

Diagnostic Tests for Asthma

• History and Physical Examination

• Pulmonary Function Test Results.:

  • Asthma can be differentiated from chronic bronchitis and emphysema during testing by administering a bronchodilator, like an albuterol inhaler, and then retesting; asthma symptoms can generally be reversed with the medication, but in chronic bronchitis and emphysema, symptoms are unchanged

• ABGs evaluated during an acute attack

• Allergy skin testing and increased serum immunoglobulin E (IgE) and eosinophil levels indicate allergic involvement

Treatment of Asthma

• Monitor With Peak Flow Meter

• Avoid Triggers

• Avoid Smoking

• Use of short and long-acting inhaled bronchodilators and corticosteroids inhalers

• Avoid using aspirin, NSAIDS, and beta blockers for hypertension, as these meds can cause bronchoconstriction

Cystic Fibrosis

• Exocrine gland disorder that primarily affects the lungs, GI tract, and sweat glands.

• Thick, tenacious secretions are responsible for many of the characteristic symptoms

  • These sticky respiratory secretions are difficult to remove and cause airway obstruction, resulting in air trapping and frequent respiratory infections

• Pancreatic ducts become blocked, causing retention of digestive enzymes that digest and destroy the pancreas

• Absence of digestive enzymes in the intestine causes:

  • Malabsorption of essential nutrients

  • Frequent foul-smelling, fatty stools

  • Excess flatus

• Patients secrete sweat that is high in sodium and chloride because these electrolytes are not reabsorbed as they pass through the sweat ducts

Cause

• Genetic disorder

  • Both parents must be carriers of the defective gene for CF to be present in their child

  • Patients with CF who marry are counseled on the risk of offspring having the disease

Signs and Symptoms

• Thick, tenacious sputum

• Frequent respiratory infections

• Finger clubbing (which is a sign of chronic hypoxemia)

• Malabsorption of fat-soluble vitamins, resulting in malnutrition

• Fatty, foul-smelling stools (steatorrhea)

• Poor appetite

• Delayed sexual maturation in both males and females with infertility problems

• Death from antibiotic-resistant infection

Diagnostic Tests

• Genetic testing is done first

• Years ago, before genetic testing was available, there was the “Kiss Your Baby” Campaign

  • Parents of children with frequent respiratory infections were encouraged to kiss their babies' foreheads and report any salty taste to their HCPs

• Sweat Chloride Test

  • Determines whether sweat is high in sodium and chloride

Treatment

• Because there is no cure for CF, treatment is aimed at controlling infection and relieving symptoms:

  • Hydration to thin sputum

  • Inhaled mucolytic medication by nebulizer

  • Bronchodilators

  • Chest Physiotherapy

  • Lung transplant

  • Pulmonary rehabilitation programs help patients maintain activity tolerance

• Antibiotics started as soon as signs of infection starts (sometimes prophylactic antibiotics used)

• Annual flu/COVID vaccinations

• Regular exercise to mobilize secretions

• Prevention of infection is vital to slowing the progression of lung damage, so avoid others with infection

• Pancreatic enzyme replacement (pancrelipase [Pancrease, Viokase]) helps reduce symptoms related to malabsorption and improve nutritional status

Pulmonary Embolism (PE)

• Blood clot that has traveled into a pulmonary artery and lodged there

  • Resulting obstruction of blood flow causes a ventilation- perfusion mismatch, which means that an area of the lung is well ventilated with air but has no blood flow, or perfusion

  • This seriously impairs gas exchange

• Remember, in the lungs, the roles of the arteries and veins are the opposite of in the rest of the body

• The pulmonary artery brings de-oxygenated blood into the lungs, and this is usually where a blood clot coming from outside the lungs lodges

Causes

• Most pulmonary emboli originate in the deep veins of the lower extremities (DVT)

  • Every effort should be made to avoid risk factors for DVT

• Less common causes:

  • Fat emboli from compound fracture of a large bone like the femur

  • Amniotic fluid emboli during labor and delivery

  • Air embolism from entry of air into the bloodstream with IV therapy

Prevention

• Early and frequent ambulation after surgery

• Prevention of blood clots in the deep veins of the legs and then prompt treatment of DVT if one occurs

• In patients at risk for DVT or PE, such as post-op patients or during times of immobility

  • Warfarin (Coumadin)

  • Low-dose Enoxaparin (Lovenox) subq

  • Low-dose Heparin subq

• SCDs

Signs and Symptoms

• Sudden onset of dyspnea for no apparent reason (most common symptom):

  • Gasping for breath; VERY ANXIOUS

• Tachycardia

• Tachypnea

• Cough

• Pleuritic chest pain

• Crackles or friction rub heard upon auscultation

• Hemoptysis if lung infarction has occurred:

  • Death of lung tissue due to no perfusion

Diagnostic Tests

• D-Dimer (blood test that if positive = possible PE)

• Spiral CT scan with contrast dye

  • Noninvasive and can diagnose PE quickly

• Lung Scan or VQ Scan

  • Done to assess the degree of ventilation of lung tissue and the areas of blood perfusion

  • If area is well ventilated but poorly perfused, PE is suspected.

• Pulmonary Angiogram

  • Invasive test that can outline the pulmonary vessels with a radioactive dye injected via a cardiac catheter

  • Can show where blood flow is diminished or absent, suggesting a PE

Treatment

• Thrombolytic agents such as alteplase (Activase) or reteplase (Retavase) used to dissolve the clot:

  • Must be administered within 4-6 hours of clot’s occurrence and are associated with hemorrhage

• In patients who cannot tolerate a thrombolytic agent, the clot may be removed with a cardiac catheter, or a surgical embolectomy

• Heparin used to prevent new clot formation

• Warfarin (Coumadin) or rivaroxaban (Xarelto) used for 3-6 months after PE to prevent recurrence

  • Either may be used for long-term treatment for