Book 2, case 6-Cystic fibrosis Flashcards
Concerns for CF patients: Go by system
Pulm:
Heart:
Pulmonary function: 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 car 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).
How would you evaluate pulmonary function in pts with CF?
What clue can an ABG give you?
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 PaC02 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.
Urgent case in CF pt: will you delay?
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)
Always discuss the risk of what in CF pts? Always include what in CF optimization?
discuss
the risk of prolonged intubation and mechanical ventilation with the patient, her
family, and the surgeon;attempt to optimize her pulmonary condition as time
allowed (i.e. bronchodilators, perioperative antibiotics, and chest physiotherapy).
What do PFTs tell you?
the severity and progression of her lung disease,obstructive or restrictive in nature, and how responsive she was to
bronchodilator therapy,
What would PFTs show in CF patients?
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 (FEVl/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.
Pathophys of CF?
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.
Medical tx of CF:
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 (br~richodilator 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.
Anesthesia in CF patients; Regional vs general-considerations-esp in laparoscopic surgery
epidural anesthesia can
avoid the increased risk of bronchospasm, aspiration, 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.
Labs for CF patients:
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.
So, you can’t optimize pts respiratory status the way you wanted to in CF due to urgency-wyd?
optimize her pulmonary status, as time allowed, by
providing chest physiotherapy, bronchodilators, humidified nebulizers, and
perioperative antibiotics.
Finally, I would correct any coagulopathy or electrolyte abnormality as
time allowed.
Monitors for CF pts:
In addition to the ASA standard monitors, I would place a 5-lead EKG
to monitor for ischemia and signs of right-sided heart failure in this patient with long
standing hypoxia.
what can go wrong with insufflation of the abdomen? And what would you do if this thing happened?
hypotension and bradycardia suggests that
insuf:flation of the pneumoperitoneum resulted in a vasovagal response, possibly
exacerbated by the decrease in cardiac preload that may occur with increased
abdominal pressure in the setting of inadequate intravascular volume.
I would ask the srirgeon to discontinue insufflation, hand ventilate
with I 00% oxygen, auscultate the chest, ensure adequate ventilation, check the EKG
and pulse oximetry, start a fluid bolus, and administer vasopressors and/or inotropes
as necessary. If her condition persisted and I was unable to identify the cause, I would
· check her electrolytes, obtain arterial blood gases, and consider invasive monitoring,
transesophageal echocardiography, and/or a bedside ultrasound (the latter would be
helpful in identifying or ruling out tension pneumothorax). I
Pt has pulse ox of 89% and increased airway pressures
during laparoscopic surgery.
And then, what would you DO?
(1) partial obstruction of the endotracheal tube (may occur secondary to the
thickened secretions associated with cystic fibrosis); (2) right mainstem intubation
( cephalad displacement of the diaphragm and carina with inflation of the
pneumoperitoneum can result in right mainstem intubation); (3) bronchiectasis
(associated with cystic fibrosis); ( 4) bronchospasm (increased risk in patients with
cystic fibrosis); ( 5) pulmonary edema (i.e. pulmonary hypertension, pulmonary
infection, and/or heart failure); (6) pneumothorax secondary to alveolar rupture
(increased risk due to the bullae formation associated with cystic fibrosis, and the
hyperventilation that is often necessary during laparoscopic surgery to maintain
normal PaC02); (7) aspiration (i.e. full stomach, pain, abdominal process, emergency
surgery); (8) capnothorax (pneumoperitoneum formation may lead to movement of
C02 into the thorax through natural or iatrogenic communications between the
peritoneal cavity and the pleural and pericardia! sacs); and (9) atelectasis.
What I would do: would immediately (1) hand ventilate with 100% oxygen; (2)
auscultate the chest; (3) examine the oropharynx for gastric material; ( 4) verify proper
endotracheal tube placement; (5) check her blood pressure, EKG, and pulse oximeter;
( 6) suction the endotracheal tube; (7) ask the surgeon to observe the hemidiaphragm
for abnormal motion (occurs with tension pneumothorax); and (8) check the PETco2
(increases with capnothorax; decreases with tension pneumothorax and C02
embolism, due to decreased cardiac output; and is usually unchanged with right
mainstem intubation). Depending on my findings and her response to these
interventions, I would also consider (9) deepening my anesthetic (i.e. bronchospasm,increased mucous secretions due to sympathetic stimulation), (10) obtaining arterial
blood gasses, (11) ordering a chest radiograph, (12) releasing the pneumoperitoneum
(i.e. C02 embolism or capnothorax), (13) placing invasive monitors (i.e. arterial line,
central line, pulmonary artery catheter, TEE); (14) administering bronchodilators,
inotropes, and/ or vasopressors; and, in the case of significant pneumothorax, ( 15)
having the surgeon place a chest tube.
What would capnothorax (carbon dioxide ptsx) present like? What would you do?
decreased breath sounds over the field, elevated ETCO2.I would ask the surgeon to release
the pneumoperitoneum, hyperventilate the patient, and, following the resolution of
her hypercapnia, have the surgeon recreate the pneumoperitoneum using a lower
insufflation pressure. Because-if there is no pulmonary trauma, a capnothorax will resolve in 30-60 min without thoracentesis
