COPD

COPD is a pulmonary disease that causes chronic obstruction of airflow from the lungs.
The cause of the limited airflow is due to chronic inflammation and narrowing of the bronchioles which
becomes deformed over time and produces excessive mucous (mainly in chronic bronchitis) and the loss
of elasticity of the alveoli sacs (mainly found in emphysema).
These problems lead to limited gas exchange such as respiratory acidosis due to extreme retention of
carbon dioxide (CO2) and low availability of oxygen.
COPD is a "catch all" term used to describe diseases that limit airflow which now includes diseases such
as chronic bronchitis and emphysema.
Patients with chronic bronchitis are sometimes referred to as "blue bloaters". This is because of the
cyanosis and edema (hence the bloating) experienced with this disease.
Patients with emphysema are sometimes referred to as "pink puffers". These patients do not experience
cyanosis, although they have low oxygen levels but maintain a "pink" appearance due to the
compensatory state of hyperventilation keeping the oxygen level sufficient enough.
The hyperventilation leads to the increased usage of the accessory muscles which leads to a barrel chest
look (increased anteroposterior diameter noted on inspection).
Signs and symptoms of COPD include:

 chronic dry or productive cough,

 lack of energy,
 extreme dyspnea,
 abnormal blood gases,
 abnormal lung sounds (coarse crackles or wheezing),
 barrel chest,
 tripod position for breathing,
 poor nutrition,
 recurrent lung infection etc.
Complications of COPD include:

 heart disease such as right-sided heart failure,

 pulmonary hypertension,
 pneumothorax,
 pneumonia,
 increase risk of lung cancer.
Chronic obstructive pulmonary disease is diagnosed with spirometry.
Nursing interventions for COPD include:

 monitoring lung sounds and sputum production,

 suctioning as needed,
 monitoring oxygen saturation,
  maintaining SpO2 88-93% (remember patients with COPD are stimulated to breath due to LOW
oxygen levels rather than high carbon dioxide levels),
 administering breathing treatments as ordered,
 educating about pursed-lips and diaphragmatic breathing,
 nutrition needs,
 vaccinations up-to-date,
 how to take medications, and
 side effects of medications.
Medications for treatment of COPD depends on the severity of the condition.
However, they tend to include as ordered by the physician:

 Corticosteroids,
 Phosphodiesterase-4 inhibitors,
 Methylxanthines,
 Short-acting and long-acting bronchodilators.

How these two conditions are the same?

 Both terms are categorized under the term COPD (chronic obstructive pulmonary disease)
 Limit airflow
 Patients have the inability to fully exhale.
 Irreversible….no cure…managed with medications/lifestyle changes.
 Cases vary from mild to severe and some patients can develop both chronic bronchitis and
emphysema.
 Both experience respiratory acidosis
 Main cause is due to inhalation of some type of irritant: main cause SMOKING.
 Medications Treatment: bronchodilators, corticosteroids, theophylline, Pphosphodiestrace-
4 inhibitors: “Roflumilast”
 Spirometry is ordered to diagnose.
How these two conditions are different? Mainly Pathophysiology

What is happening in chronic bronchitis?

The inflamed and deformed bronchioles produce EXCESSIVE MUCOUS due to hyperplasia of the
goblet cells. The cilia are damaged so they can’t move mucus out of the airway, and this limits the
patient’s ability to have proper gas exchange and breathe with ease.

Major Signs & Symptoms:

 Chronic productive cough (lasts more than three months)

  Shortness of Breath
 Cyanosis
 V/Q Mismatch
Pathophysiology of Chronic Bronchitis:

Main issue is with INFLAMMATION OF BRONCHIOLES due to the damage from inhalation of some
type of irritant.

This leads to cyanosis “blue” color and increased lung volume “bloating” and edema (late effects on the
heart for the development of cor pulmonale). Patients with chronic bronchitis are also known as “blue
bloaters”.

The capillary bed (where gas exchange takes place) is NOT damaged so this affects how the body will
compensate. The bronchioles are surrounded by alveolar tissue which start to degenerate (lose form and
disappear). When this happens the bronchiole lose support and become don’t open properly to allow air to
flow into the sacs for gas exchange.

Mainly, the problem is with exhalation…because during inspiration the airways expand due to negative
pressure. HOWEVER, on exhalation (because the lungs are full of air) the positive pressure prevents the
airways from staying open. So, exhaled air does not all the way leave the lungs….hence the patient
doesn’t have the ability to fully exhale. They experience hyperinflation of the lungs overtime.

There is also decreased oxygen and high carbon dioxide in the blood (respiratory acidosis).
Remember the capillary bed works (so perfusion is good) BUT ventilation is very poor. So, there is a V/Q
MISMATCH. Hence, ventilation (V) doesn’t match perfusion (Q).

So, the body tries to compensate by increasing red blood cell production and shifting blood which puts
a lot of pressure on the pulmonary artery. This causes complications and leads to pulmonary
hypertension and eventually right-sided heart failure “cor pulmonale”.

What is happening in emphysema?

Main issue is with damage to the alveolar sac (loses elasticity…becomes floppy and doesn’t inflate and
deflate properly) leading to “air-trapping”.

This condition also leads to hyperventilation (puffing to breathe…compensatory mechanism) and pink
complexion (they maintain a relatively normal oxygen level due to rapid breathing) rather than cyanosis
as in chronic bronchitis. Patients with emphysema are sometimes called “pink puffers”.

Major Signs & Symptoms:

 Shortness of Breath
 Hyperventilation (tachypnea)
 Weight Loss (from breathing)
 Barrel Chest
  V/Q Match Defect
Pathophysiology of Emphysema:

In emphysema, the alveoli sacs lose their ability to inflate and deflate due to an inflammatory response
in the body. Due to the damage to the alveoli sac, there is damage to the capillary bed so there will
a matched V/Q defect (ventilation and perfusion both poor…hence they match). So, areas of low
ventilation (V) have poor perfusion (Q).

Because the sac cannot deflate or inflate, inhaled air starts to get trapped in the sacs and this
causes major hyperinflation of the lungs because the patient is retaining so much volume.

Hyperinflation causes the diaphragm to flatten. The diaphragm plays a huge role in helping the patient
breathe effortlessly in and out. Therefore, in order to fully exhale, the patient starts to hyperventilate and
use accessory muscles to get the air out now. This leads to the barrel chest look and during inspection it
may be noted there is an INCREASED ANTEROPOSTERIOR DIAMETER.

The damage in the sacs cause the body to keep high carbon dioxide levels and low blood oxygen levels
(respiratory acidosis). Inhaled oxygen will not be able to enter into the sacs for gas exchange and carbon
dioxide won’t leave the cells to be exhaled.

The body tries to compensate by causing hyperventilation (increasing the respiratory rate…hence
puffing) and the patient will have less hypoxemia “pink complexion” than chronic bronchitis (who have
the cyanosis) because pink puffers keep their oxygen level just where it needs to be from
hyperventilation.

ARDS
What is acute respiratory distress syndrome (ARDS)? It’s a type of respiratory failure that occurs when
the capillary membrane that surrounds the alveoli sac becomes damaged, which causes fluid to leak into
the alveoli sac.
ARDS has a fast onset and tends to occur in people who are already sick (hospitalized) and usually
develops as a complication to a systemic inflammation process occurring in the body.
What can cause the capillary membrane to become more permeable and leak fluid?
This is usually triggered by an event that leads to major systemic inflammation, which can be indirectly
damage the capillary membrane or directly damage the capillary membrane.
Indirect (source isn’t the lungs): capillary membrane is INDIRECTLY damaged due to a systemic
inflammatory response system (SIRS) by the immune system:
- *Sepsis (most common and very poor prognosis for patients who have gram-negative bacteria)
- Burns
- Blood transfusion (multiple)
- Inflammation of the pancreas
- Drug overdose
- Direct (source is the lungs) ….capillary membrane is DIRECTLY damaged
- Pneumonia
 - Aspiration
- Inhaling a toxic substance
- Significant drowning event
- Embolism

This pathophysiology of ARDS is discussed in-depth in this video and features the 3 phases of ARDS
(Exudate, Proliferative, Fibrotic Phases)

Signs and Symptoms of ARDS (acute respiratory distress syndrome):

- Tachypnea,
- refractory hypoxemia (hallmark sign and symptoms),
- cyanosis, mental status changes,
- abnormal lungs sound like crackles in the late stages,
- tachycardia,
- respiratory alkalosis that can progress to respiratory acidosis, etc.
Nursing interventions for ARDS include:
- maintaining respiratory function with mechanical ventilation with PEEP (monitoring for
complications of this therapy),
- pulmonary artery wedge pressure reading (Helps to determine if this is pulmonary edema due to
ARDS or a cardiac issue),
- prone positioning,
- administering medications (inotropics, fluids, corticosteroids, antibiotics, GI drugs to prevent
stress ulcers etc.

CYSTIC FIBROSIS
Cystic fibrosis is a genetic disorder that causes the exocrine glands to work incorrectly.
This can lead to major complications that affect the respiratory, digestive (pancreas, liver, intestines),
integumentary, and reproductive systems along with the sinuses.
What are exocrine glands? These are glands that produce and transfer it’s secretions (ex: mucous, tears,
sweat, digestive enzymes) via DUCTS to it’s intended area of the body rather than directly into the
bloodstream like the endocrine glands.
In cystic fibrosis, there is a gene that is mutated that prevents the exocrine gland from working properly. It
is called the CFTR gene!
The CFTR gene, which stands for cystic fibrosis transmembrane regulator, is a protein that controls the
channels of sodium and chloride. Hence the sodium and water transport in and out of the cell, and these
channels are within the membrane of the cells that makes our sweat, mucous, tears, and digestive
enzymes.
However, in CF these channels don’t work properly. Therefore, normally these channels would properly
regulated sodium and water transport in and out of the cells, which helps control the flow of water within
these structures among other things. But this is not what happens in a person with cystic fibrosis.
The mucous is thick and sticky rather than thin and slippery. This mucous will block the airways in the
lower respiratory system and block the pancreatic duct from delivering pancreatic enzymes. In addition,
the sweat glands produce excessive amounts of salt.
CF tends to affect Caucasians and males more. The current median life expectancy of a person with cystic
fibrosis is 37 years of age (source: NIH.gov). It is currently diagnosed with a sweat test where pilocaprine
and electric current is delivered to the skin to make it sweat. The amount of salt in the sweat is measured.
Results 60 mmol/L or more is considered positive for CF.
Nursing interventions to cystic fibrosis include:
- chest physiotherapy (chest PT),
- monitor for complications,
- educating about PEP devices,
- preventing infection,
- administering medications and more

ASTHMA
What is asthma? Asthma is a chronic lung disease (no cure) that causes narrowing and inflammation of
the airways (bronchi and bronchioles) that leads to difficulty breathing.
How does it happen? Normally, when you breathe in air, it travels down through your upper airway to
your lower airway, which includes the trachea, bronchi, bronchioles, and alveoli (where gas exchanges
happen). The oxygen you breathe in crosses over into your blood stream, and the carbon dioxide in your
blood crosses over into the airway to be exhaled.
In patients with asthma, the bronchi and bronchioles are chronically inflamed and can become so
inflamed that it leads to an asthma attack (wheezing, chest tightness, shortness of breath, coughing),
which is usually due to a trigger of some type.
Asthma triggers include:
Environment:
 smoke,
 pollen,
 pollution,
 perfumes,
 dander,
 dust mites,
 pests (cockroaches),
 cold and dry air, mold
Body Issue:

 respiratory infection,
  GERD,
 hormonal shifts,
 exercise-induced
Intake of Certain Substances:

 drugs (beta adrenergic blockers that are nonselective),

 NSAIDS,
 aspirin,
 preservatives (sulfites)
Key Players of an Asthma Attack:

Bronchi and Bronchioles

 What surrounds these structures? Surrounding the bronchi and bronchioles are smooth
muscles that wrap around the airway. This muscle helps with dilating and constricting the
airway.

 During an asthma attack, these smooth muscles constrict. This causes

chest tightness and difficulty breathing.

 What is inside these structures? Inside these structures is a mucosa lining which contains
special cells called goblet cells. Goblet cells produce mucous, which helps trap the irritants
and bacteria we breathe in and prevent these substances from entering further into our
respiratory system.

 During an asthma attack, the mucosa becomes very inflamed (this

narrows the airway…decreasing air flow and air becomes trapped in
the alveoli). The goblet cells (due to the inflammatory response)
produce excessive amounts of mucous. Hence, leading to further
decrease in air flow: coughing, wheezing (as air tries to flow through
the narrow airway and around the mucous it makes a musical whistling
sound).

 During this, air is becoming trapped in the alveoli. Therefore, gas exchange
is not taking place and low amounts of oxygen are entering the blood (the
patient will have decreased oxygen saturation) and carbon dioxide is
staying in the blood (patient will have the buildup of CO2…..respiratory
acidosis). The patient will feel like they can’t exhale all the way.
Now asthma attacks vary in severity among patients. It is important for the patient to recognize
the triggers and early signs and symptoms of a pending asthma attack (discussed below). These early
signs and symptoms are different for every patient, but as the nurse you will need to teach the patient how
to recognize them. They will usually have these signs and symptoms 1 to 2 days before an attack. In
addition, the patient will need to follow an asthma action plan created by the MD and the patient.

Signs and Symptoms of an asthma attack include:

 chest tightness,
  wheezing (auscultate…expiratory wheezing and can progress to inspiratory wheezes in severe
cases),
 coughing,
 difficulty breathing (***especially exhaling),
 increased respiratory rate.
How is it diagnosed? pulmonary function test (PFT)

Another thing is to educate the patient about early warning signs:

 starting to become short of breath easily.

 easily fatigued with physical activity
 frequent coughing (mainly at night) and trouble sleeping at night
 symptoms similar to a cold: sneezing, scratchy throat, tired, headache
 irritable
 wheezing with activity
 reduced peak flow meter reading.

Nursing Interventions for Asthma

Presenting with an asthma attack:

 Baseline vital signs

 Keep patient calm and comfort the patient.
 Position in high Fowler’s to help with ease of breathing.
 Administer bronchodilators as ordered by MD.
 Administer oxygen (oxygen saturation 95-99%)
 Assess peak flow meter reading (watch for numbers less than 50% of the patient’s personal
best reading)
 Auscultate lung sounds throughout: (decrease in wheezing? patient breathing easier?
respiratory rate normal?)
 Monitor skin color and for any retractions of the chest, can they talk to you with ease now
or is it still difficult for them to talk?)
 Current peak flow meter reading numbers (if the patient uses this device…ask patient (if
they know) their personal best reading and current readings, and medications they’ve
taken.
What is a Peak Flow Meter: it shows how controlled a patient’s asthma is and if it is getting worse (lets a
patient know if an attack is pending…several hours to days before one)
How does the patient use a peak flow meter? As represented in the picture above, the patient will
exhale as hard as they can onto the device. The device will then measure how much air was exhaled out
of the lungs.

 Many times, a peak flow meter is used with an asthma action plan created with MD. As
stated above, the device measures the airflow out of the lungs (large airways) not small (so
the patient needs to know early warning signs too). It helps the patient know when they
need to take a short-acting bronchodilators and when they should go to the hospital for
treatment.
 When a patient starts using a peak flow meter, they need to FIRST figure out
their personal best peak flow meter reading. This will be the highest number reached over
a period of time. It will be the number used to compare against other reading numbers,
which will allow the patient to know if their asthma is under good control.

 How is the personal best peak flow meter reading figured out? The patient
will use the peak flow meter to figure out their best peak flow reading when
their asthma is under good control, and measure it once in the morning
and once at night for 3 weeks usually and record the numbers BEFORE
TAKING MEDICATION. The highest number they obtain over this
period of time will be their personal best reading.

 Then they will need to continue to use the peak flow meter at the same time every day,
either in the morning or at night BEFORE TAKING MEDICATION and compare it with
the personal best reading. If the reading is 80% or less than their personal best, they need
to follow the action plan created with their doctor.
You will be providing them with education on how to follow their prescribed asthma action plan (quiz the
patient to ensure they understand how to follow the plan).

Help the patient identify triggers (educate them on the triggers), how to avoid (except exercise-induced)
and those early warning signs.

What if a trigger is exercised-induced? The patient doesn’t need to quit exercising (important for overall
health). To help decrease the chances of an attack they can:

 warm up before exercising for 10-15 minutes.

 administer short acting beta agonists before exercising.
 breathe through nose instead of mouth during cold windy days to warm the air before it
enters the lungs (cold air can make the smooth muscles around the airway constrict)
 avoid exercising when sick with a respiratory illness.

Medications used to treat Asthma.

Bronchodilators: opens the airways to increase air flow…. different types

Bronchodilator types used for Asthma: Beta-agonists* and Anticholingerics*, Theophylline

*commonly given as inhaled routes for asthma…theophylline is oral
Short-acting beta agonist (Albuterol):

 inhaler or nebulizer: used as the fast-acting relief during an asthma attack or prior to
exercise for asthma that is exercise-induced NOT for daily treatment
 ***(if patient is using their inhaler more than 2 times a week, then the patient’s asthma
plan needs to be readjusted because their asthma is not under good control).
Long-acting beta agonists (Salmeterol, Symbicort…this drug is a combination of a long-acting beta
agonist AND corticosteroid):

 NOT for an acute asthma attack

****Side effects of these medications: tachycardia, feeling nervous/jittery, monitor heart rhythm for
dysrhythmia

Anticholinergics:

 Ipratropium: a bronchodilator that also is short-acting and relaxes airway….used when a

patient can’t tolerate short-acting beta agonist.
 Tiotropium: a bronchodilator that is long-acting
 These drugs can cause dry mouth….. “sugarless candy helps with this”
Theophylline: given PO

 not as common because of possible toxicity and maintaining blood levels of 10-20
mcg/mL
 AVOID consuming products with caffeine while taking this medication…WHY? Caffeine
has the same properties as Theophylline, which can increase the toxic effects of the
medication.
*****Always administer the bronchodilator FIRST and then 5 minutes later the corticosteroid.

If not responding to treatment, may need intubation and mechanical ventilation.

Anti-inflammatories: decreases swelling and mucus production…used as long-term treatment to control

asthma not an acute attack.

Anti-inflammatories used to treat asthma include: “Corticosteroids, Leukotriene Modifiers,

Immunomodulators, Cromolyn”

Inhaled corticosteroids: “Fluticasone”, “Budesonide”, “Beclomethasone”

 watch for thrush (use spacer with inhaler and rinsing mouth after administration)
 risk for osteoporosis and cataracts (cloudy lens on the eyes)
 May be given IV or PO for severe asthma attack.
Leukotriene Modifiers (oral): “Montelukast”
  blocks the function of leukotriene which causes the smooth muscle on the airways to
constrict and plays a role in mucus production. When this function is blocked it leads to
the relaxation of the smooth muscle and decreased mucous production…NOT for an
acute attack
Immunomodulator (subq): “Omalizumab”

 blocks the role of the immunoglobulin IgE, which will decrease the allergic response…
hence asthma attacks
 given subcutaneously
 used when patient’s asthma is poorly controlled and other treatments are not working
 NOT used as a quick relief
 NO LIVE vaccines while receiving
Nonsteroidal Anti-Allergy: “Cromolyn” (inhaled)

 long term use….not for quick relief in emergency situations

 stops mast cells from secreting histamine. This decreases inflammation.
 While receive the patient may temporarily experience sneezing, burning in nose,
itchy/watery eyes, bad taste in mouth.
Pneumothorax NCLEX Review

Definition: the collapsing of a lung due to air accumulating in the pleural space (the space between the
visceral and parietal pleura which is also called the intrapleural space). Learn more about lung anatomy
and physiology.

Key Points to Remember about Pneumothorax:

 It can be a partial or total collapse of the lung (mainly affects one lung).
 Causes include but not limited to: spontaneous (without warning), trauma to the chest
(blunt or penetrating), lung disease, medical procedures (central line placement,
mechanical ventilation).
 It is diagnosed with a chest x-ray, ultrasound, or CT scan.
 A small pneumothorax usually resolves on its own.
 A large pneumothorax usually requires treatment like a chest tube to remove air from
intrapleural space or needle aspiration (as with a tension pneumothorax).
Pathophysiology of Pneumothorax

The visceral pleura (attaches to the lungs) and parietal pleura (attaches to the chest wall) are separated
by a small amount of serous fluid and this space is called the intrapleural space. In a pneumothorax, this
is where the air collects that causes the lung to collapse.
The intrapleural space allows the visercal and parietal pleura to glide over one another during inhalation
and exhalation which creates a negative pressure. The negative pressure acts like suction to keep the
lungs inflated.

Therefore, if air enters the intrapleural space it causes a buildup of pressure (remember under normal
condition the lungs like negative pressure) which decreases the ability of the lungs to recoil and pushes
the lung away from the chest which leads to collapse.
Types of Pneumothorax to remember for the NCLEX exam

Open Pneumothorax: an opening in the chest wall (from a gun shot, stabbing etc.) that causes a passage
between outside air and the intrapleural space. This allows air to pass back and forth during inspiration
and expiration. Therefore, the body will shunt air through the chest wall opening instead of the trachea .

 Remember, normally you have air passing through trachea (not the chest wall) to the lungs
while breathing. Because of this you may hear a “sucking sound” This type of
pneumothorax is also known as a sucking chest wound.
 Nursing intervention for an open chest wound: place a sterile occlusive dressing over the
opening and tape it on 3 sides (leaving the 4th side free from tape). This prevents the
wound from being occluded. This type of dressing will allow exhaled air to leave the
opening but seal over the opening when inhaling (hence preventing a tension
pneumothorax).
Closed Pneumothorax: when air leaks into the intrapleural space without any outside wound (hence the
chest wall and pleural stay intact). Example of what can cause this: a rib fracture where the sharp, bony
part of the bone punctures the lung causing air to be released into the intrapleural space. Another common
cause of closed pneumothorax is called spontaneous pneumothorax:

 Spontaneous Pneumothorax: a defect in the alveolar wall and visceral pleura where air
enters into the intrapleural space. An example: pulmonary bleb (sac-like blister that
develops on the visceral pleura that ruptures and leaks air into the pleural space). This
causes pressure to build up in the intrapleural space and causes the lung to collapse. It
known as a “spontaneous” pneumothorax because the pneumothorax was NOT caused by
an injury.
 Patients can have multiple pulmonary blebs and they don’t have to rupture immediately.
Exact cause of rupture is not totally understood but things that can increase the rupture of a
bleb include: change in air pressure, taking a sudden, deep breath, or smoking
Two classifications of Spontaneous Pneumothorax:

 Primary spontaneous pneumothorax: occurs in people without lung disease and they
tend to be young <30 years of age and tall and thin.
 Secondary spontaneous pneumothorax: occurs in people with lung disease (copd,
asthma, cytic fibrosis).
Tension pneumothorax: a complication of a pneumothorax (can happen with open or closed
pneumothorax). This is a medical emergency. It happens when the opening to the intrapleural
space creates a one-way valve…where air collects into the space but never leaves. This causes major
compression on the lungs and heart. The patient will have a mediastinum shift, increased intrathoracic
pressure and decreased venous return.
  Patho of tension pneumothorax: AIR CANNOT ESCAPE the intrapleural space -> there
is shift of the mediastinum as pressure builds in the space -> patient tries to compensate by
increasing breathing (tachypnea) to maintain oxygen level but this doesn’t work, patient
will have hypoxia -> there will be compression on the vena cava (remember the vena cava
normally drains blood to the heart but it can’t now) ->suppose to draining blood back to
the heart…the heart then has nothing to pump…hence decreased cardiac output
 Anatomy changes with a tension pneumothorax: mediastinal shift causes heart, trachea,
esophagus, and vessels to shift to the UNAFFECTED side and this will compress the
unaffected lung and venous vessels.
 Major Signs and Symptoms of Tension Pneumothorax:

 Major: Tachycardia, Tachypnea, Hypotension and Hypoxia

 As well as: Respiratory distress, jugular venous distention, tracheal

deviation (LATE SIGN)

 If a patient is on mechanical ventilation w/ PEEP (positive end-expiratory pressure) they

are at risk for a tension pneumothorax due to barotrauma which causes buildup of
pressure in the intrapleural space from rupture of the visceral pleura.
 Treatment for tension pneumothorax: needle decompression: …a needle is inserted into
the intrapleural space to remove air…..performed by physician.
Signs & Symptoms of Pneumothorax:

Remember the mnemonic: COLLAPSED

Chest pain (sharp and sudden and worst on inspiration), Cyanosis

Overt tachycardia and tachypnea

Low blood pressure

Low SpO2

Absent lung sounds on affected side.

Pushing of trachea to unaffected side (tension pneumo.)

Subcutaneous emphysema (escaping carbon dioxide collecting in the skin…crunchy bulges on the
skin), Sucking sound with open pneumothorax.

Expansion of chest rise and fall unequal.

Dyspnea

Nursing Interventions for Pneumothorax:

  Monitor breath sounds (equal sounds on both sides), equal rise and fall of the chest, vital
signs (HR, blood pressure, oxygen saturation), and patient effort of breathing, subq
emphysema (can be found on the face, abdomen, armpits, neck (affects breathing),
administering oxygen as ordered.
 Maintain chest tube drainage system if placed by physician:

 Assessing for air leaks in the system, keep it secure.

 Troubleshooting if drain comes out or system breaks.

 Water seal chamber: may have intermittent bubbling as air

is drained from the pleural space. The water seal chamber
fluctuates as the patient breathes in and out. If it stops
fluctuating, there may be a kink somewhere or the lung has
re-expanded. NOT normal to have excessive bubbling in
the water seal chamber (air leak somewhere).

 Keep HOB of the bed elevated…Fowler’s position

CYSTIC FIBROSIS

What is cystic fibrosis? It’s a genetic disorder that causes the EXOCRINE glands to work incorrectly.
This leads to some major complications that can affect the respiratory (both upper/lower), digestive
(pancreas, liver, intestines), integumentary, and reproductive systems.

What are exocrine glands? These are glands that produce and transfer it’s secretions (ex: mucous, tears,
sweat, digestive enzymes) via DUCTS to its intended area of the body rather than directly into the
bloodstream like endocrine glands

In cystic fibrosis, a gene that makes up the exocrine glands is mutated and this prevents the exocrine
glands from working properly. The gene that is mutated is called the CFTR gene!

The CFTR gene, which stands for cystic fibrosis transmembrane regulator, is a protein that controls
the channels of sodium and chloride. In other words, it controls the sodium and water transport in and out
of the cell.

Therefore, these channels that are within the membrane of exocrine cells that makes our sweat, mucous,
tears, and digestive enzymes don’t work properly. So, the substances produced by the exocrine glands
don’t look and work like they are supposed to.

For example: How is mucous supposed to be? Thin and slippery which helps lubricate the structures.
However, this is the problem in patients with cystic fibrosis

In patients with CF the mucous is THICK and STICKY (rather than thin and slippery) and this
affects the following structures:

 Lungs (upper and lower):

  upper sinuses and formation of polyps (leading to snoring, nasal
stuffiness)

 blockage of the airways: overtime leads to obstructive pulmonary

diseases like emphysema …..air trapping can occur which causes
hyperinflation

 Or bleds can develop and rupture leading to a pneumothorax

 Pulmonary hypertension which can lead to heart failure in

the long term

 Clubbing of the nails due to the lack of oxygen in the blood

 Hemoptysis: coughing up blood either due to infection or

severe damage to lung tissue (mainly in older patients rather
than children)
Very susceptible to lung infections that can create permanent damage to the lungs

 Thick, sticky mucous creates perfect conditions for bacteria to thrive and survive! These
patients are at risk for pseudomonas aeruginosa, staphylococcus aureus etc.

 There is a risk of the development of antibiotic resistance or allergies to

antibiotics, which can make treating the infection very hard.

 Gastrointestinal (blockage of passage ways within the body)

 Pancreas (this structure makes pancreatic enzymes):

 PAL: protease, amylase, lipase: These substances are

secreted via the pancreatic duct into the duodenum and this
helps digest proteins, fats, and vitamins.

 However, these enzymes are not accessible for proper usage because of the mucous
blocking the pancreatic duct. Hence, they will stay in the pancreas and this inflames the
structure and can cause fibrosis of the pancreas. In addition, the patient can develop cystic
fibrosis related diabetes. Therefore, when a patient is not receiving pancreatic enzymes
they will experience:

 weight loss, malabsorption, nutrition major issue, delayed

puberty, GREASY, ODOROUS STOOL, BLOATNG, ABODOMINAL
PAIN

 Many patients will need a feeding tube to help with

nutrition, PANCREATIC ENZYMES SUPPLEMENTS, fat-soluble
vitamins like D E A K

 Liver: mucous blocks biliary ducts which causes the bile to become thick and gallstones
can develop along with gallbladder inflammation.
  Distal Intestinal Obstruction Syndrome (DIOS): blockage in the intestine….intestines
can also make thick mucus like the lungs and pancreas and when thick mucus and stool get
together it can lead to a blockage in the gut.

 Meconium ileus (infants): sticky meconium becomes stuck in the ileum of

the intestine

 Infertility in males and females: most people who have cystic fibrosis are unable to have
children because of the thickness of mucous. For example, women have troubling
conceiving because the cervical mucous is not friendly towards sperm, and males tend to
be sterile because there are issues with the vas deferens duct that helps sperm go for the
testicle to the urethra (thick mucus blocking it or the duct is not formed correctly).
 Integumentary: sweat glands produce too much salt. Therefore, the sweat/tears are very salty.
The patient is at risk for electrolyte complications and dehydration. However, the salty sweat is
helpful with diagnosing CF (sweat chloride test).
Quick Facts about Cystic Fibrosis:

 Most common is Caucasians and males, but can affect any race or gender.
 NIH (National Institute of Health) statistics: life expectancy in 1960 was age 10. Today the
median life expectancy is age 37 (“NIH Fact Sheets – Cystic Fibrosis”, n.d.).
 This is most commonly detected during the 1st year of life…child will have GI and respiratory
issues (ex: infant may have a meconium ileus).
 Severity depends (not all patients are 100% alike in their severity)
 It’s an autosomal recessive disorder….which means the child had to receive one mutated gene
from both parents. In most cases, the parents are carriers of the disease but don’t have
symptoms of CF.

 Diagnosed: Sweat Test: painless test that measures the amount of salt in the sweat

 Pilocarpine and electric current is applied to the skin to help it sweat

(usually on the arm). A gauze is used to collect the sweat which is sent to a
lab to measure the salt in the sweat

 Results:

 39 mmol/L or less negative for cystic fibrosis

 40 to 59 mmol/L needs further testing, not

conclusive

 60 mmol/L or more POSITIVE for cystic

fibrosis

 Diagnosed: Sweat Test: painless test that measures the amount of salt in the sweat
  Pilocarpine and electric current is applied to the skin to help it sweat
(usually on the arm). A gauze is used to collect the sweat which is sent to a
lab to measure the salt in the sweat

 Results:

 39 mmol/L or less negative for cystic fibrosis

 40 to 59 mmol/L needs further testing, not

conclusive

 60 mmol/L or more POSITIVE for cystic

fibrosis
Nursing Interventions and Treatments for Cystic Fibrosis

Nursing Focus: Mucous, Nutrition, Treatment for infection (most patients will die due to a lung infection
or issue of some type), preventing blockages in GI system

Patients who have CF are on a wide variety of medications like the following:

 stool softeners
 pancreatic enzymes
 nasal sprays
 vitamins
 antibiotics
 anti-inflammatories (inhaled or oral)
 mucolytics (oral or nebulizer)
 bronchodilators (oral or inhaled)
Mucous: Huge part of the everyday routine treatment and this includes: Chest PT, postural drainage,
using PEP devices and nebulizers along with huff coughing

 Chest physiotherapy (Chest PT): helps drain the airways of that thick sticky mucous

 It is done with postural drainage along with percussion and vibration to

facilitate the draining of mucous from the lung lobes followed by huff
coughing (THIS IS A HUGE PART OF treatment for CF patients!!!!)
Chest PT: percussion (cupping hands and percussing of the over the lung fields and take special care
when percussing over spine, stomach, breast bone, organs to avoid injury), some patients use vibration
with a special vest and then huff coughing is performed

**Patient usually performs this 2-4 times per day or more depending on if they are fighting a lung
infection. Sessions vary: 20 minutes to an hour.
TEST!!! When should you perform chest PT on a patient? Perform when the stomach is empty to prevent
reflux of food and vomiting……1-2 hours after meals (hence in between meals). NOT after (reason:
regurgitation) or before (reason: risk of decreasing the appetite because coughing up thick sticky mucous
can taste bad which can decrease a person’s appetite).

Airway Clearance devices: PEP (positive expiratory pressure): Helps with clearing the airway

 PEP devices: assists with moving mucous from the lungs…..person breathes in and out of
device (resistance when breathing out and this helps even more to move mucous)….creates
a vibration (performs several times) and then huff cough to expel mucous. Device names:
Flutter valve or Acapella
Prevent infection: wearing a mask due flu times or when sick, hand hygiene, avoid sick people, and stay
up-to-date with vaccinations, lung transplant possible if lung problems severe

Exercise: regular aerobic exercise to help keep lungs healthy and clear secretions. Educate to monitor
sweating because of the risk of losing too much salt (may need salt supplements and increase caloric
needs when exercising)

GI: pancreatic enzymes, fat-soluble vitamins, high calories, high protein and high fat diet

Pancreatic enzymes:

TEST!!! Swallow them! Don’t chew or crush them! If person can’t swallow the capsule, you can open it
and put it in an ACIDIC FOOD like apple sauce. Do not mix in an alkaline-based food like yogurt,
pudding or ice cream because this will inactive the enzyme.

How do you know the patient is receiving an adequate amount of enzymes? The patient’s stool is NOT
greasy or odorous and they are free from abdominal bloating or pain.

TEST!!! When would you administer these enzymes? Administer before all meals and snacks. Example
of these enzymes are: Pancrelipase, Pancreatin

Some patients have feeding tubes because their caloric intake is very high, especially during illness or
during the late stages of the disease. High protein and high calorie and fat diet with vitamin supplements
like the fat-soluble: DEKA

Obstruction and Constipation Prevention

 Drink a lot of water to keep mucous in gut thin, use Miralax Polyethylene Glycol
(common) daily as prescribed by MD to prevent constipation
 Monitor for bowel obstruction: signs: no bowel movement, pain, can’t pass gas etc.
Cystic Fibrosis Diabetes Mellitus: pancreas is damaged….monitor blood sugar (can drop or be high)…
may need insulin