Acute Respiratory Distress and Failure in the Patient with Cystic Fibrosis by Dr. Emily Barsky.
Today I'll be speaking about respiratory distress and respiratory failure in patients with cystic
fibrosis.
My goals for the video are as follows.
Help residents understand the clinical presentation of respiratory distress in patients with cystic
fibrosis.
Define the important initial information to gather.
Define interventions that should be initiated immediately, and identify red flags that should
trigger ICU evaluations and/or calling the pulmonary fellow.
You are taking care of an 18-year-old male with cystic fibrosis and severe lung disease
who was admitted yesterday for a cystic fibrosis exacerbation in the setting of several days
of increased cough, shortness of breath, and fevers.
He was started on IV antibiotics last night.
It is 5 AM and you have just gone to the call room to lie down when the nurse pages you
that the patient is having increased work of breathing and is now requiring two liters
of supplemental oxygen to maintain his oxygen saturations above 92%.
What is the differential diagnosis for a CF patient with respiratory distress?
Mucus plugging, pneumothorax, pulmonary hemorrhage, pulmonary edema, pulmonary embolism, worsening
viral or bacterial infection, respiratory failure, and sepsis.
What are the initial questions you want to ask when you call the nurse back?
Is the patient on monitors, both cardiorespiratory monitors and a pulse oximeter?
Ask the nurse to obtain repeat vital signs and put the patient on monitors, if that's
not already been done.
What are the patient's current vital signs, including the temperature, heart rate, blood
pressure, respiratory rate and oxygen saturation?
Ask the nurse to increase supplemental oxygen, if needed.
And what is the patient's mental status.
Lastly, tell the nurse that you are on your way to assess the patient.
In the room, the first thing you want to do is to check to make sure the patient is indeed
on monitors, that the oxygen is hooked up, that their IV is working, and that you have
all the necessary personnel in the room to help you.
When you look at the vital signs, what are some red flags?
Any significant change from prior should be concerning.
If the patient is febrile, remember that this itself can cause the heart rate or respiratory
rate to become elevated.
A respiratory rate at either extreme, either low or high, should also be a red flag.
A low respiratory rate could suggest hypoventilation due to sedation or due to fatigue with impending
respiratory failure.
Hypotension is also concerning and could be consistent with a tension pneumothorax or
sepsis.
Next, you perform a targeted physical exam.
What are the areas to focus on?
First, assess the mental status or the patient's level of consciousness.
Is the patient easily aroused or overly sleepy?
Are they oriented and conversant?
Next, assess their work of breathing.
Can the patient speak in full sentences?
Are they tachypneic, do they have retractions or nasal flaring or tripod positioning?
Move on to assess the airway and make sure that the trachea appears midline.
When you perform your respiratory exam, you should assess for air movement, for any focality,
such as decreased or absent breath sounds in one particular place or focal crackles,
wheezing or a prolongation of the expiratory phase, and any change from their prior exam.
For the cardiac exam, focus on evaluating for tachycardia, for a heart murmur, and assess
perfusion with pulses and capillary refill.
Next, what is the important history to obtain from the patient and the family?
Is there an instigating event that triggered the respiratory distress?
Was it of sudden or gradual onset?
Is the patient short of breath?
Do they have chest pain?
Was there any hemoptysis?
If the patient does report hemoptysis, inquire as to the color, amount, and whether the patient
has a prior history of this.
Please view the hemoptysis lectures for further details on this topic.
Next, assess whether or not the patient has a headache, is feeling sleepy, or is confused,
which would all be signs of hypercarbia, or elevated carbon dioxide.
CF patients are also very knowledgeable about their symptoms.
Ask if they have had a similar feeling before, if it feels like a mucus plug, or if they
have any idea what the shortness of breath and respiratory distress represents.
Next, think about what lab studies you would like to order.
You can start with a blood gas to evaluate for inadequate gas exchange.
If this shows a respiratory acidosis, assess whether or not this is acute or chronic.
Or in other words, uncompensated or compensated.
In an acute respiratory acidosis, the pH will be reduced, the CO2 will be elevated, and
the bicarb will be normal.
In contrast, in a chronic respiratory acidosis, there is renal compensation.
And therefore, the pH will be low, but will be closer to normal.
The pCO2 will remain elevated, and the bicarb will now become elevated as well as the kidneys
compensate.
In addition to assessing the current blood gas, look back at prior blood gases and chemistry
panels to assess if the patient is a chronic retainer of carbon dioxide.
If the patient's CO2s have been elevated in the past and their bicarbonate has been elevated,
this could suggest a more chronic CO2 retention.
Consider sending a lactate with your blood gas to evaluate for end organ hypoperfusion,
as would be seen in sepsis.
Point of clarification.
Lactate should be sent from a free flowing specimen or central line.
A tourniqueted specimen that is difficult to draw may provide a lactate that is difficult
to interpret and may be falsely elevated.
Send a blood glucose, as many patients with cystic fibrosis have cystic fibrosis relate
diabetes, and their blood glucose may be elevated in sepsis.
In addition, if their blood glucose is low, this could alter their mental status.
Send a CBC with differential, as well as a C-reactive protein to evaluate for infection,
as well as a change from the prior values.
A chemistry panel will help to assess the bicarbonate and evaluate for electrolyte imbalances,
as well as renal function.
If the patient is having hemoptysis, send a type and screen.
If the patient is febrile, send a blood culture.
If the patient has not already had a respiratory culture recently, send that as well.
Consider viral studies, such as influenza, if this has not already been sent.
A D-dimer is unlikely to be helpful, as it is often high in the setting of a CF exacerbation.
Certainly, do not send this unless you are planning to obtain a chest CT scan if the
D-dimer is elevated.
Next we will discuss imaging.
It is best to start with a chest X-ray to evaluate for a pneumothorax, a focal or new
infiltrate, a pleural effusion, and to assess the degree of hyperinflation.
If the patient is severely tachycardic or you're clinically concerned for a pulmonary
embolism, consider an EKG to evaluate for right heart strain.
Consider a chest CT as well, if concerned for a pulmonary embolism, but discuss this
with the fellow first.
We will discuss further reasons to talk to your fellow in a later section.
Initial interventions to initiate.
Start by providing supplemental oxygen as needed.
However, in patients that chronically retain carbon dioxide, be careful about giving too
much oxygen, as this could suppress the patient's drive to breathe.
Stop all enteral feeds and make the patient NPO.
If the patient is normally on BiPAP at night, initiate this to help with gas exchange and
with the work of breathing.
If the patient has very advanced lung disease and is in severe distress, make sure you know
the patient's code status, so as to guide further management.
Depending on the patient's particular presentation, there are numerous additional interventions
that you may choose to pursue.
For example, if the patient is wheezing, you can trial a bronchodilator.
If you are concerned about mucus plugging, have the nurse do chest physical therapy and
airway clearance, assuming the patient is stable for this.
If you are concerned about a pneumothorax, make sure to hold all chest PT and airway
clearance, as well as non-invasive positive pressure ventilation, such as BiPAP.
If the patient has hemoptysis, stop chest PT and airway clearance and place the bleeding
side down, if known.
Please refer to the hemoptysis video for further information on this.
If concerned for sepsis, consider broadening your antibiotics.
If the patient is febrile or has viral symptoms and its flu season, consider empiric use of
Tamiflu.
Also remember to call your senior, as well as your fellow, and/or the ICU as needed.
Now let's discuss red flags or trigger points to call your fellow and/or the ICU.
Indications to call your fellow include escalating supplemental oxygen requirement, altered mental
status and hypercarbia, a new pneumothorax, concern for sepsis, consideration of need
for broadened antimicrobials, consideration of chest CT to evaluate for pulmonary embolism,
an unclear cause of new distress or persistent distress despite your initial interventions.
Indications to call an ICU consult or an ICU STAT or Code Blue include hypotension and
concern for septic shock, severe hypoxia and an inability to maintain saturations on supplemental
oxygen, altered mental status suggestive of inadequate gas exchange, a blood gas showing
acute hypercarbia, suggestive of impending respiratory failure, a large pneumothorax
or a tension pneumothorax, with a tension pneumothorax either being shown by tension
physiology or by tracheal deviation on exam or chest X-ray.
Also remember that if you call an ICU consult, the ICU has 20 minutes to arrive.
Use your judgment as to whether or not the patient is stable enough to wait.
If not, you need to call a code.
If the patient has increased work of breathing but can maintain oxygen saturations with nasal
cannula, and/or has hypercarbia and needs non-invasive positive pressure ventilation
but respiratory failure is not imminent, you can likely call an ICU consult.
If, however, the patient is in severe distress such as the inability to speak or minimal
air movement on exam, if the patient is minimally responsive due to their hypercarbia or has
a tension pneumothorax, you should call a code.
While these are just a few examples of when to call your fellow or the ICU, remember,
when in doubt, always ask for help.
This concludes our session on respiratory distress in a patient with cystic fibrosis.
Thank you for participating.
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