Long-Term Follow-up of Nocturnal Ventilatory Assistance in Patients with Respiratory Failure Due: RESULTS
Table 1 shows age at initiation of assisted ventilation, respiratory parameters at initiation of assisted ventilation and at times of early and extended follow-up and clinical outcome in the eight patients comprising our series. Most patients developed respiratory failure at approximately 20 years of age, as is typical for Duchenne muscular dystrophy. Delayed onset in patients 1 and 6 suggests that they may have had a more slowly progressing variety. All patients had severe restriction of their vital capacities and severe hyper- carbia at the time that ventilatory assistance was initiated.
All patients had gradually worsening symptoms of chronic hypercarbia including morning headache and loss of energy for several weeks to months prior to initial presentation, but none had symptoms of an acute respiratory illness. Patient 6 complained of cough productive of clear secretions for three weeks prior to presentation. Patients 4 and 6 complained of weakened voice, and only one, patient 2, had dyspnea at initial presentation. The latter patient was also the only one to develop signs of congestive heart failure, including peripheral edema and transudative pleural effusions, during his subsequent course.
revatio 20 mg
Table 1 —Vital Capacity, PaC02 and Ventilator Use at Initiation and During FoUow-up Periods in Individual Batients
|
|
|
|
|
|
|
At Early Follow-up |
|
|
|
|
|
|
|
|
|
|
|
At Initiation |
|
|
(1-3 months) |
|
|
|
At Extended Follow-up |
|
||
|
Patient |
Age |
FVC |
FVC |
PaCO, |
|
Use FVC |
PkC02 |
|
Use |
FVC |
PaC02 |
|
Clinical |
|
No. |
W |
(ml) |
(% predicted) |
(mm Hg) |
Ventilator |
(h/24°) (ml) ( |
mm Hg) |
Months |
(h/24°) |
(ml) |
(mm Hg) |
Hospitalizations |
Outcome |
|
1 |
27 |
824 |
(15) |
69 |
Iron lung |
8 800 |
51 |
66 |
18 |
300 |
43 |
1 for pneumonia |
Elective |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
tracheostomy at |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
69 months |
|
2 |
20 |
740 |
(15) |
62 |
Iron lung |
8 |
38 |
45 |
23 |
|
32 |
2 for pneumonia; |
Sudden death at |
|
|
|
|
|
|
|
|
|
|
|
|
|
2, congestive |
45 months at |
|
|
|
|
|
|
|
|
|
|
|
|
|
heart failure; 1, |
home |
|
|
|
|
|
|
|
|
|
|
|
|
|
respiratory |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
failure |
|
|
3 |
16 |
880 |
(19) |
54 |
Wrap |
5 930 |
36 |
26 |
|
|
|
1 for pneumonia |
Death 2° |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
pneumonia |
|
4 |
25 |
413 |
(9) |
69 |
Pneumobelt |
12 350 |
51 |
50 |
24 |
|
36 |
1 for gastritis; 1, |
Elective |
|
|
|
|
|
|
|
|
|
|
|
|
|
pneumonia |
tracheostomy at 26 months, at |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
home at 50 |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
months |
|
5 |
24 |
460 |
(9) |
68 |
Iron lung |
8 |
47 |
41 |
16 |
350 |
53 |
1 for pneumonia |
living at home |
|
6 |
27 |
720 |
(15) |
60 |
Wrap |
9 |
51 |
10 |
20 |
620 |
51 |
2 for pneumonia; |
Elective |
|
|
|
|
|
|
Pneumobelt |
|
|
|
|
|
|
1, secretions |
tracheostomy at |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
13 months |
|
7 |
20 |
450 |
(9) |
62 |
Wrap |
8 |
47 |
10 |
12 |
350 |
48 |
1 for pneumonia |
Sudden death at |
|
|
|
|
|
|
|
|
|
|
|
|
|
|
13 months |
|
8 |
19 |
336 |
(7) |
62 |
fortalung |
8 |
39 |
65 |
10 |
230 |
68 |
0 |
living at college |
|
MeanlSEM |
22±1.5 |
603 ±74 |
12±2 |
63±2 |
|
8±1 6931176 |
4513 |
3918 |
1812 |
3701 66 |
4714 |
1.611 |
|
Although six of the eight patients tried positive pressure ventilation administered via a face mask, lip seal or nose mask, all six preferred other forms of assisted ventilation; five of the six chose a negative pressure ventilator and one chose a Pneumobelt. Among the negative pressure ventilators, the tank ventilator and Pneumowrap were selected by four and three patients, respectively. The requirement that the Pneumobelt be used in no less than a 45° semirecum- bent position limits its acceptability for nocturnal ventilation, but the one patient who selected it learned to sleep in the sitting position. Patient 6 used the Pneumobelt in the afternoon in addition to using the Pneumowrap at night.
At the time of discharge from their initial hospitalization, most patients were using their ventilators no more than 3 to 5 h nightly and reversal of daytime hypercarbia was minimal. However, as they gradually extended hours of use and learned to sleep with the ventilator, PaC02 gradually fell to the 40 to 50 range after one to three months of nocturnal ventilator use, as shown in Table 1. Symptoms of morning headache resolved and energy level was improved in all patients and weakened voice and dyspnea were improved in affected patients. The average duration of use at initial follow-up was 8 h. Although only a few measurements of FVC were obtained at early follow-up, no consistent changes were observed.
canadian cialis online
FIGURE 1. Forced vital capacity, daytime spontaneous FaCOs and hours of ventilator use are shown at initiation of assisted ventilation, one to three months after initiation and at extended follow-up. Data are mean± SE. Numbers in parentheses indicate numbers of patients.
At extended follow-up, averaging 39 months, the mean number of hours of ventilator use increased to 18 h/24 h, and FVC fell significantly, an average of 33 percent among the five patients in whom follow-up measurements were available. The increase in hours of use of the ventilator was apparently able to compensate for the reduction in pulmonary (unction because PaC02 remained stable at extended follow-up in comparison with early follow-up. Figure 1 shows these trends in FVC, PaC02 and hours of ventilator use during the follow-up period.
