UBP 2.6 (Short Form): Pulmonary - Cystic Fibrosis

Question 1

What are your concerns with this patient?

(A 14-year-old girl with a history of cystic fibrosis presents for urgent appendectomy. She reports a history of progressive abdominal pain associated with nausea and vomiting over the last 18 hours. She had an episode of respiratory failure requiring intubation 6 months ago. Her medications include Ciprofloxacin, Pancrelipase, Prednisone, Albuterol, Guaifenesin, and Beclomethasone MDI. Vital Signs: HR = 110, BP = 98/38 mmHg, RR = 24, Temp = 39 ºC)

Answer 1

My primary concern is

• the pulmonary function of this patient with cystic fibrosis and a history of respiratory failure.

Chronic lung disease from cystic fibrosis could place this patient at increased risk for –

hypoxia (ventilation/perfusion mismatching), bronchospasm (bronchial hyperreactivity), pneumothorax (bullae formation), postoperative respiratory failure, and heart failure (pulmonary hypertension and cor pulmonale).

However, I would also be concerned that

• hepatic, pancreatic, and gastrointestinal involvement of her cystic fibrosis could place her at increased risk for –
  
  • coagulopathy (hepatic involvement and malabsorption of vitamin K),
  • pseudocholinesterase deficiency (hepatic involvement),
  • diabetes (pancreatic involvement), and
  • electrolyte abnormalities (malabsorption).

Given that she is experiencing acute appendicitis with nausea, vomiting, and a fever, I would be concerned about the possibility of –

• peritonitis,
• the increased risk for aspiration and/or sepsis, and
• possible hypovolemia (secondary to vomiting and/or peritonitis).

Considering the emergent nature of the surgery, I would be concerned about –

• having an inadequate amount of time to optimize the patient’s medical condition.

Finally, I would be concerned about the various complications associated with laparoscopic surgery.

Question 2

How would you evaluate her pulmonary function?

(A 14-year-old girl with a history of cystic fibrosis presents for urgent appendectomy. She reports a history of progressive abdominal pain associated with nausea and vomiting over the last 18 hours. She had an episode of respiratory failure requiring intubation 6 months ago. Her medications include Ciprofloxacin, Pancrelipase, Prednisone, Albuterol, Guaifenesin, and Beclomethasone MDI. Vital Signs: HR = 110, BP = 98/38 mmHg, RR = 24, Temp = 39 ºC)

Answer 2

I would begin by obtaining a careful history concerning –

• the progression and treatment of her cystic fibrosis,
• her recent episode of respiratory failure and any other hospitalizations,
• any recent and chronic pulmonary infections,
• the quality and quantity of her pulmonary secretions,
• her exercise tolerance,
• the use and effectiveness of bronchodilators, and
• the results of any recent pulmonary function testing.

I would then auscultate her lungs in both the upright and supine positions, and perform a physical exam to identify any signs and symptoms of respiratory distress, such as the use of accessory muscles of respiration, nasal flaring, and increased respiratory rate.

Depending on my findings, I would consider ordering an ABG (significant elevation of the PaCo2 suggests end-stage disease and carries a poor prognosis), chest radiograph, and/or pulmonary function testing to further define her pulmonary status and risk of respiratory failure.

While this appendectomy is an urgent case, I would delay this case as long as was reasonable for patient optimization and additional workup that might influence my choice of anesthetic (regional vs. general) and/or ventilator management.

Question 3

Would you order pulmonary function tests?

(A 14-year-old girl with a history of cystic fibrosis presents for urgent appendectomy. She reports a history of progressive abdominal pain associated with nausea and vomiting over the last 18 hours. She had an episode of respiratory failure requiring intubation 6 months ago. Her medications include Ciprofloxacin, Pancrelipase, Prednisone, Albuterol, Guaifenesin, and Beclomethasone MDI. Vital Signs: HR = 110, BP = 98/38 mmHg, RR = 24, Temp = 39 ºC)

Answer 3

While pulmonary function tests would potentially aid in determining the severity and progression of her lung disease, whether her lung defect was primarily obstructive or restrictive in nature, and how responsive she was to bronchodilator therapy,

I would not delay this urgent surgery for PFTs unless I believed the information would significantly alter my anesthetic plan (i.e. preoperative optimization, ventilation management, regional vs. general anesthesia).

Instead, I would:

1. review her most recent pulmonary function tests;
2. obtain a thorough history (i.e. progression and treatment of her cystic fibrosis, details concerning her recent episode of respiratory failure, history of hospitalizations, history of pulmonary infections, the quality and quantity of her pulmonary secretions, her exercise tolerance, the use and effectiveness of bronchodilators);
3. perform a physical exam with special focus on her cardiac, pulmonary, and hepatic function (i.e. signs of pulmonary hypertension, right heart failure, and coagulopathy);
4. discuss the risk of prolonged intubation and mechanical ventilation with the patient, her family, and the surgeon; and
5. attempt to optimize her pulmonary condition as time allowed (i.e. bronchodilators, perioperative antibiotics, and chest physiotherapy).

The results of any pulmonary function tests would likely show –

• decreased maximal mid-expiratory flow rate, increased residual volume to total lung capacity ratio (RV/TLC), and a decreased forced expiratory volume in 1 second to forced vital capacity ratio (FEV1/FVC).
• The response to bronchodilators tends to be unpredictable and varies with the patient’s underlying pulmonary status.
• With the progression of the lung injury and fibrosis, the restrictive component becomes more prominent, demonstrated by a decreased total lung capacity and vital capacity.

Question 4

What is the pathophysiology of cystic fibrosis (CF)?

(A 14-year-old girl with a history of cystic fibrosis presents for urgent appendectomy. She reports a history of progressive abdominal pain associated with nausea and vomiting over the last 18 hours. She had an episode of respiratory failure requiring intubation 6 months ago. Her medications include Ciprofloxacin, Pancrelipase, Prednisone, Albuterol, Guaifenesin, and Beclomethasone MDI. Vital Signs: HR = 110, BP = 98/38 mmHg, RR = 24, Temp = 39 ºC)

Answer 4

Cystic fibrosis (CF) is a systemic, recessively inherited disease, in which a defective cystic fibrosis transmembrane conductance regulator (CFTR) gene leads to abnormal movement of salt (sodium chloride) in and out of cells and the development of thick, viscous secretions that are associated with luminal obstruction and glandular destruction in the lungs, pancreas, liver, and gastrointestinal tract.

Mucous plugging, inflammation, and chronic infection lead to bronchiectasis, emphysema, hypoxemia, cor pulmonale, cardiomegaly, hepatomegaly, and ultimately respiratory failure.

Enzymatic insufficiency and impaired gastrointestinal mobility secondary to pancreatic and gastrointestinal involvement lead to malnutrition and diabetes.

Hepatic involvement and impaired absorption of vitamin K can lead to decreased plasma cholinesterase and coagulopathy (clotting factors II, VII, IX, and X are vitamin K dependent).

Question 5

How is cystic fibrosis treated medically?

(A 14-year-old girl with a history of cystic fibrosis presents for urgent appendectomy. She reports a history of progressive abdominal pain associated with nausea and vomiting over the last 18 hours. She had an episode of respiratory failure requiring intubation 6 months ago. Her medications include Ciprofloxacin, Pancrelipase, Prednisone, Albuterol, Guaifenesin, and Beclomethasone MDI. Vital Signs: HR = 110, BP = 98/38 mmHg, RR = 24, Temp = 39 ºC)

Answer 5

The current treatment of cystic fibrosis involves a comprehensive, multidiscipline-oriented, intensive care program.

Therapeutic goals are prevention and control of pulmonary infections, promotion of mucus drainage, and provision of adequate nutrition.

Treatment of pulmonary disease requires antibiotic therapy combined with physical and respiratory therapy (bronchodilator therapy in select individuals).

Pancreatic enzyme replacement, adequate nutrition, and vitamin and mineral supplementation are employed to treat exocrine pancreatic deficiency and the associated abnormal digestion of fat and proteins.

Question 6

What type of anesthetic would you provide for this case?

(A 14-year-old girl with a history of cystic fibrosis presents for urgent appendectomy. She reports a history of progressive abdominal pain associated with nausea and vomiting over the last 18 hours. She had an episode of respiratory failure requiring intubation 6 months ago. Her medications include Ciprofloxacin, Pancrelipase, Prednisone, Albuterol, Guaifenesin, and Beclomethasone MDI. Vital Signs: HR = 110, BP = 98/38 mmHg, RR = 24, Temp = 39 ºC)

Answer 6

(Answered well by mock examinee)

Given her potentially full stomach and history of severe cystic fibrosis with an episode of respiratory failure, I would prefer to utilize epidural anesthesia and avoid the increased risk of bronchospasm, aspiration (abdominal pathology, pain, and the emergent nature of the surgery), atelectasis, prolonged mechanical ventilation, and other pulmonary complications associated with providing a general anesthetic to this patient.

However, the high levels of neuraxial blockade (required to achieve adequate muscle relaxation and avoid diaphragmatic irritation from gas insufflation), trendelenburg positioning, and pneumoperitoneum required for laparoscopic surgery, may not be tolerated by this patient with significant pulmonary dysfunction.

Therefore, assuming there were no signs of coagulopathy (increased risk for coagulopathy with cystic fibrosis) and that the patient and her family agreed to regional anesthesia (relatively young patient), I would ask the surgeon to consider performing the appendectomy as an open procedure so that regional anesthesia would be better tolerated.

If regional anesthesia were planned, I would discuss all the risks and benefits with the patient and her family, reassure the patient that I would keep her as comfortable as possible at all times, provide minimal sedation as necessary, place a catheter in the epidural space, and slowly induce neuraxial blockade, with the goals of ensuring adequate analgesia and avoiding unnecessary respiratory compromise from excessively high neuraxial blockade or preoperative sedation.

Question 7

Would you order any additional lab work or studies prior to proceeding to surgery?

(A 14-year-old girl with a history of cystic fibrosis presents for urgent appendectomy. She reports a history of progressive abdominal pain associated with nausea and vomiting over the last 18 hours. She had an episode of respiratory failure requiring intubation 6 months ago. Her medications include Ciprofloxacin, Pancrelipase, Prednisone, Albuterol, Guaifenesin, and Beclomethasone MDI. Vital Signs: HR = 110, BP = 98/38 mmHg, RR = 24, Temp = 39 ºC)

Answer 7

Given this patient’s appendicitis and cystic fibrosis, I would order a –

• complete blood count,
• electrolyte panel (potentially altered by both appendicitis and cystic fibrosis),
• blood sugar (due to possible pancreatic involvement of cystic fibrosis),
• coagulation profile (due to potential hepatic dysfunction secondary to cystic fibrosis), and
• an arterial blood gas.

Moreover, since she is a female of childbearing age, I would order a pregnancy test.

Finally, depending on her history and my physical exam findings, I would also consider a –

• chest radiograph, pulmonary function tests, electrocardiograph, echocardiogram, and liver function tests.

Xtra Question: – Mom says her daughter is not sexually active, so she does not want HCG test, what do you do?

– Mom still refuses, then what?

Question 8

In consultation with the surgeon, it is decided that there is insufficient time for PFTs or an echocardiogram.

How would you optimize this patient for this emergent surgery?

(A 14-year-old girl with a history of cystic fibrosis presents for urgent appendectomy. She reports a history of progressive abdominal pain associated with nausea and vomiting over the last 18 hours. She had an episode of respiratory failure requiring intubation 6 months ago. Her medications include Ciprofloxacin, Pancrelipase, Prednisone, Albuterol, Guaifenesin, and Beclomethasone MDI. Vital Signs: HR = 110, BP = 98/38 mmHg, RR = 24, Temp = 39 ºC)

Answer 8

I would attempt to optimize her pulmonary status, as time allowed, by providing chest physiotherapy, bronchodilators, humidified nebulizers, and perioperative antibiotics.

Considering her appendicitis with nausea and vomiting, I would provide aspiration prophylaxis (i.e. an H2-blocker or nonparticulate antacid) and ensure adequate fluid resuscitation (potential hypovolemia from vomiting and/or peritonitis).

Finally, I would correct any coagulopathy or electrolyte abnormality as time allowed.