cystic fibrosis complications - stanford...
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Cystic Fibrosis Complications
ANDRES ZIRLINGER, MD
STANFORD UNIVERSITY MEDICAL CENTER
MARCH 3, 2012
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INTRODUCTION
PNEUMOTHORAX
HEMOPTYSIS
RESPIRATORY FAILURE
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Cystic Fibrosis
Autosomal Recessive
Genetically transmitted disorder affecting 30,000 individuals in US and 60,000 worldwide
US incidence:1 per 1900-3700 Caucasians
Previously a pediatric disease
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1990: 30% patients in US CFF Registry older than 18yrs
2012: More than 45% older than 18yrs.
Assuming improving care and therapies, the projected median survival is approximately 50 years of age for those born after 2000.
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INTRODUCTION
PNEUMOTHORAX
HEMOPTYSIS
RESPIRATORY FAILURE
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What is a pneumothorax (PTx)?
1803: First description in patients with pulmonary tuberculosis Accumulation of air in the pleural space defined as the space between the lungs and chest wall Primary Pneumothorax:
Spontaneous occurrence in patients with normal lungs
Secondary Pneumothorax: Spontaneous occurrence in patients with abnormal lungs i.e Emphysema, Cystic Fibrosis
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www.nlm.nih.gov, 2007
Normal
lungs
Chest
wall
Pleural
space
Pneumothorax
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•
Normal CXR
PTx Collapsed
Lung
tissue
Shift of
heart
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PTx
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What causes a Pneumothorax?
May be related to chronic inflammation of the airways
Destruction and rupture of small lung units allowing air to leave the lung into the pleural space
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Signs and symptoms
Asymptomatic (incidental finding on routine CXR or CT) Various degrees of shortness of breath Chest pain or pressure Normal to decreased oxygen saturation Increased heart rate Low blood pressure Respiratory failure Cardiac arrest
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Pneumothorax
Commonly reported in CF – 1 in 167 patients each year
CFF registry 1990-1999 3.4% of total population experienced at least one pneumothorax
82% with one event
18% with greater than one event
72.4% patients with first PTx are > 18 old
Average age of 1st PTx 21.9 +/- 9.1 yrs
Average age of PTx did not change over the years
Flume et al, Chest 2005
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Risk Factors for PTx
Pseudomonas aeruginosa
Burkholderia cepacia
Aspergillus / ABPA
FEV1 ≤ 30% predicted
Pancreatic insufficiency
Tube feeds
Medicaid
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Treatment of Pneumothorax
Asymptomatic: PTx < 2 cm
Admit, observation, repeat CXR(S)
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Mildly symptomatic: PTx < 2 cm and <50y Needle aspiration
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Symptomatic: PTx > 2 cm, and >50y
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Persistent Pneumothorax
Consultation with thoracic surgeon
Caution in thoracic surgical procedures as they may make lung transplantation surgery complicated
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Prognosis
Mortality after pneumothorax
48.6% at 2 years
75% at 8 years
*** Higher mortality associated with lower FEV1
*** ?? Early referral for Lung Transplantation despite FEV1 ≥ 30% predicted
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INTRODUCTION
PNEUMOTHORAX
HEMOPTYSIS
RESPIRATORY FAILURE
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What is hemoptysis?
Expectoration of blood from the respiratory tract
Massive Hemoptysis:
Coughing greater than 250ml of blood over 24 hours or recurrent bleeding with >100 ml/ day over several days
• Non-Massive Hemoptysis:
– Any quantity of blood less than mentioned above
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Most common cause of Hemoptysis in CF patients
Chronic airway inflammation affects adjacent arterial supply
Tortuous vessels
Hypervascularity
Weakened vessel walls
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Signs and symptoms
Expectoration of blood
Increasing shortness of breath
Increasing heart rate
Massive blood loss leading to low blood pressure
Respiratory failure
Cardiac arrest
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Hemoptysis
4.1% of CF patients experience at least one episode of massive hemoptysis
74% will only have one episode
26% with greater than one episode
Majority (75%) of patients with first episode occurring after age 18
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Risk factors for Massive hemoptysis
FEV1 < 30% predicted Pseudomonas aeruginosa Staphylococcus aureus Burkholderia cepacia Tube feeds Cirrhosis ***lower risk observed in those patients receiving TOBI and pulmozyme
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Treatment of hemoptysis
Bronchoscopy:
Attempts to visualize the region of bleeding
Not very effective as view might be obscured by blood
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Bronchial artery embolization (BAE)
First reported embolization 1973
Recommended procedure of choice in massive hemoptysis
Studies demonstrating efficacy in non-major hemoptysis
Controls hemoptysis in approximately 90%
10-52% may require repeat BAE
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BAE
Catheter inserted into groin, femoral artery
Contrast dye is injected into the arterial system
Polyvinyl alcohol particles are injected into potential bleeding sited to occlude the blood supply
Coils also used to occlude the bronchial arterial supply
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BAE
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Adverse effects
Chest pain
Difficulty swallowing
Bronchial necrosis/damage
Paraplegia: injury to spinal artery
Respiratory failure
Recent evidence suggests that this may relate to low FEV1 and lung irritation from bleeding and not from procedure
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Prognosis after massive hemoptysis
Reported 44% mortality within one year
Mean age of hemoptysis 26.4 +/- 9 years
***majority of these patients with FEV< 30% predicted
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INTRODUCTION
PNEUMOTHORAX
HEMOPTYSIS
RESPIRATORY FAILURE
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Respiratory Failure
• Inadequate gas exchange – Low oxygen levels (hypoxemic resp. failure) – High carbon monoxide (hypercarbic resp. failure)
• Signs and Symptoms – rapid respiratory rate – breathlessness – tachycardia – low oxygen levels – confusion – loss of consciousness – respiratory arrest
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Respiratory Failure
Most common causes:
1) Pulmonary infectious exacerbation
2) Massive hemoptysis
3) Pneumothorax
4) other
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Respiratory Failure
Previously most adult admissions to the ICU with high mortality
Better survival attributed to:
Better antibiotics
Faster response time to critically ill inpatients
Use of non-invasive ventilation
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Non-invasive ventilation (NIV)
May decrease need for mechanical ventilation
Improves survival
Bridge to lung transplantation
For use in mild to moderate respiratory distress
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Treatment
• Oxygen
• Mechanical ventilation (intubation or non invasive)
• Bronchodilators
• Antibiotics
• Treatment of precipitant (Pneumothorax, hemoptysis, etc.)
• Supportive care: nutrition, prevention of complications
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Respiratory failure and ICU mechanical ventilation
Very high mortality
Associated with low FEV1
Discussion regarding end-of-life should be initiated
Eligible patients may have improved survival with lung transplantation
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Summary
Cystic fibrosis with improved survival Better strategies in treating critically ill patients Pneumothorax and Massive hemoptysis with better outcomes if treated early; recurrence is not uncommon Non-invasive ventilation improves survival, particularly if FEV1 > 30%. Respiratory distress requiring mechanical ventilation has a high mortality
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Aknowledgements
• Dr. Paul Mohabir
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Thank you