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Case Studies
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1/1/2004

Ventilation of an End-Stage Cystic Fibrosis Patient with the LTV
Connecticut Children’s Medical Center, Hartford, CT - Joline Farris, BS, RRT, Stacey Halgreen, AS, RRT, Lisa LeBon, MAE, RRT, Steven Low, AS, RRT
Pre-Hospital History:

The patient is a 17-year-old female with end stage cystic fibrosis awaiting lung transplantation. At home, she was on 4-6 liters of oxygen during the day, and nocturnal bilevel therapy via the Knightstar 335® with a Breatheasy® nasal CPAP prongs. Her settings on the Knightstar were:

IPAP16
EPAP5
Oxygen4 lpm

Hospital Course:

The patient was admitted to the hospital for intermittent slow ventricular tachycardia. Upon admission, the patient’s vital were as follows:

HR140
RR55
Temp.99.9
SpO296%

Verapamil brought the patient’s ventricular tachycardia under control. Five days post admission the patient’s oxygen requirements began increasing, as did her intervals on the Knightstar. One particular coughing episode resulted in acute respiratory decompensation as evidenced by, higher FiO2, higher RR, and increased respiratory effort.

The patient complained of not being able to exhale, most likely due to the increased flow rate caused by the increase in inspiratory pressure and oxygen flow rate. The patient was on the following setting on the Knightstar:

IPAP22
EPAP5
Oxygen30 lpm

The patient was admitted to the PICU and placed on bilevel ventilation via the Puritan Bennett 840® with a nasal mask. This gave her a more comfortable flow, increases oxygen, and less resistance on exhalation. Her settings on the 840 were:

ModeBilevelSIMV
High Peep10NA
Low Peep or Peep55
Rate1010
I time1.0 sec1.0 sec
Pressure ControlNA15
Pressure SupportNA15

When the patient went to sleep on the 840, she had a large leak from her mouth. The 840 could not compensate for the air leak and alarmed low minute volume and low tidal volume. The patient was switched to SIMV and then to a full-face mask in both SIMV and bilevel. The alarms were decreased to the lowest settings, but the alarms continued. She also complained of increased respiratory distress on the 840.

The patient was then placed on the Servo 300â with the following settings.

ModeNPPV SIMV/CPAP
FIO255%
Pressure Support55%
PEEP5
Rise Time Profile7
Floe Termination25%
Time Termination1.3 sec

Note that the LTV is not PEEP compensated, therefore she was receiving an actual pressure support of 12 cm with the above settings. On the LTV, she was able to exhale against the PEEP, have a comfortable flow rate, and maintain a higher FIO2.

For about a week the patient chose to use the LTV at night and the Knightstar 335 during the day. After a week, she was switched to the LTV during the day and night. She was able to use nasal prongs while awake on the LTV. Some adjustments were made when she had acute coughing episodes or acute anxiety episodes. The rise time profile was decreased to 6, and the pressure support setting was increased to 20-22cm (to obtain an actual pressure support of 15-17cm) to meet her increased respiratory demands.

Outcome:

The patient was called for her lung transplant and was discharged from the hospital in less than 2 weeks. She no longer requires oxygen or bilevel ventilation.

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