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Long-Term Follow-up of Nocturnal Ventilatory Assistance in Patients with Respiratory Failure Due: RESULTS continue

Following initiation of nocturnal ventilatory assis­tance, five of our eight patients required only one hospitalization for respiratory problems and one pa­tient had no hospitalizations in more than five years. Patient 6 required repeated hospitalizations because of inability to clear secretions and patient 2 had multiple hospitalizations, several related to his severe underlying cardiomyopathy. Three patients died dur­ing the extended follow-up period, one of pneumonia when he declined endotracheal intubation. The other two had severe cardiomyopathy and experienced sud­den death, one while using the iron lung. Autopsies were not performed.

Figure 2 summarizes the use of ventilators during the extended follow-up period. Patient 4 used the Pneumobelt for gradually increasing periods of time until he developed pneumonia at 26 months. Trache­ostomy was performed when he could not tolerate lying in the supine position in a tank ventilator, and he has remained clinically stable subsequently. Among the three patients who began using the Pneumowrap, two switched to the iron lung after three and ten months of nocturnal ventilation, respectively. The iron lung was selected because it provided greater venti­latory assistance and helped in mobilizing secretions. Among the four patients who began using a tank ventilator, one died suddenly, two remained stable while gradually increasing their daily amount of ven­tilator use and one elected tracheostomy rather than spend up to 20 h in the iron lung every day.
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Table 2—Mean (± SE) Values for Respiratory Parameters Measured just after (AM) and just before (PM) Nocturnal Ventilator Use in 4 Patients with Duchenne- Type Muscular Dystrophy

Pimax

PEmax

PaCO,

Pa02

f(min-1)

VE(L/min)

(mm Hg)

(mm Hg)

VC(L)

MW(L/min)

pH

(mm Hg)

(mm Hg)

A.M.

26±3

7.9± 1.5

— 22±3

31 ±7

0.43 ±0.07

17.8±4.0

7.37 ±0.03

53±2

92±3

PM.

28±4

7.4± 1.5

-21 ±4

29±6

0.45 ±0.04

17.6±3.1

7.37 ±0.03

54±3

88±3

Patients 1, 5 and 7 underwent overnight oximetry monitoring while using their negative pressure venti­lators. Mean minimum oxygen saturation during 9 ± 1 h of monitoring was 88 ±2 percent, and oxygen saturation was below 90 percent for less than 1 percent of the time.

Ficuax 2. CliniCal course

FIGURE 2. Clinical course and ventilator use for each patient are depicted over time. Each horizontal line represents one patient and lines are grouped according to ventilator chosen initially.

Table 2 shows respiratory parameters obtained im­mediately after and immediately before nocturnal ventilator use within the same 24-h period. Among the four patients studied in their homes, there were no significant changes in respiratory rate, minute ventilation, maximal inspiratory and expiratory pres­sures, vital capacity, M W or arterial blood gas values during the periods of spontaneous ventilation that averaged 11 h.
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