Hypoventilation in Cystic Fibrosis

Jennifer L Taylor-Cousar

doi:10.1055/s-0029-1222442

Seminars in Respiratory and Critical Care Medicine, Inhaltsverzeichnis

Semin Respir Crit Care Med 2009; 30(3): 293-302
DOI: 10.1055/s-0029-1222442

Hypoventilation in Cystic Fibrosis

Jennifer L. Taylor-Cousar¹

¹Divisions of Adult and Pediatric Pulmonary Medicine, Department of Internal Medicine, University of New Mexico School of Medicine, Albuquerque, New Mexico

Abstract

ABSTRACT

Cystic fibrosis (CF) is the most common life-shortening genetic disorder in Caucasians. With the improved treatments that have become available, the median survival for patients with this disorder has increased to 37.4 years of age. Unfortunately, the overwhelming majority of patients still die from respiratory failure. Hypoventilation, arising from a variety of etiologies, may occur as part of the disease process and causes increased morbidity and mortality. Although inspiratory muscles training, oxygen therapy, and noninvasive ventilation are used in the treatment of hypoventilation in CF, more data are needed to guide their optimal use.

KEYWORDS

Cystic fibrosis (CF) - hypoventilation - hypoxemia - noninvasive ventilation (NIV) - sleep disordered breathing (SDB)

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Referenzen

REFERENCES

1 Bobadilla J L, Macek Jr M, Fine J P, Farrell P M. Cystic fibrosis: a worldwide analysis of CFTR mutations: correlation with incidence data and application to screening. Hum Mutat. 2002; 19 575-606
2 Davis P B. Cystic fibrosis since 1938. Am J Respir Crit Care Med. 2006; 173 475-482
3 Cystic Fibrosis Foundation Annual Data Report. Baltimore; Cystic Fibrosis Foundation 2007
4 Knowles M R, Boucher R C. Mucus clearance as a primary innate defense mechanism for mammalian airways. J Clin Invest. 2002; 109 571-577
5 Gibson R L, Burns J L, Ramsey B W. Pathophysiology and management of pulmonary infections in cystic fibrosis. Am J Respir Crit Care Med. 2003; 168 918-951
6 Mason RJMJ, Nadel JA, Broaddus VC Textbook of Respiratory Medicine. 4th ed. Philadelphia; Elsevier Saunders 2005
7 Lagerstrand L, Hjelte L, Jorulf H. Pulmonary gas exchange in cystic fibrosis: basal status and the effect of I.V. antibiotics and inhaled amiloride. Eur Respir J. 1999; 14 686-692
8 Soni R, Dobbin C J, Milross M A, Young I H, Bye P P. Gas exchange in stable patients with moderate-to-severe lung disease from cystic fibrosis. J Cyst Fibros. 2008; 7 285-291
9 Aris R M, Merkel P A, Bachrach L K et al.. Guide to bone health and disease in cystic fibrosis. J Clin Endocrinol Metab. 2005; 90 1888-1896
10 Aris R M, Renner J B, Winders A D et al.. Increased rate of fractures and severe kyphosis: sequelae of living into adulthood with cystic fibrosis. Ann Intern Med. 1998; 128 186-193
11 Henderson R C, Specter B B. Kyphosis and fractures in children and young adults with cystic fibrosis. J Pediatr. 1994; 125 208-212
12 Logvinoff M M, Fon G T, Taussig L M, Pitt M J. Kyphosis and pulmonary function in cystic fibrosis. Clin Pediatr (Phila). 1984; 23 389-392
13 Lands L, Desmond K J, Demizio D, Pavilanis A, Coates A L. The effects of nutritional status and hyperinflation on respiratory muscle strength in children and young adults. Am Rev Respir Dis. 1990; 141 1506-1509
14 Hanning R M, Blimkie C J, Bar-Or O, Lands L C, Moss L A, Wilson W M. Relationships among nutritional status and skeletal and respiratory muscle function in cystic fibrosis: does early dietary supplementation make a difference?. Am J Clin Nutr. 1993; 57 580-587
15 Lands L C, Heigenhauser G J, Jones N L. Respiratory and peripheral muscle function in cystic fibrosis. Am Rev Respir Dis. 1993; 147 865-869
16 Bradley S, Solin P, Wilson J, Johns D, Walters E H, Naughton M T. Hypoxemia and hypercapnia during exercise and sleep in patients with cystic fibrosis. Chest. 1999; 116 647-654
17 Pinet C, Cassart M, Scillia P et al.. Function and bulk of respiratory and limb muscles in patients with cystic fibrosis. Am J Respir Crit Care Med. 2003; 168 989-994
18 Enright S, Chatham K, Ionescu A A, Unnithan V B, Shale D J. The influence of body composition on respiratory muscle, lung function and diaphragm thickness in adults with cystic fibrosis. J Cyst Fibros. 2007; 6 384-390
19 Dunnink M A, Doeleman W R, Trappenburg J C, de Vries W R. Respiratory muscle strength in stable adolescent and adult patients with cystic fibrosis. J Cyst Fibros. 2009; 8 31-36
20 Troosters T, Langer D, Vrijsen B et al.. Skeletal muscle weakness, exercise tolerance and physical activity in adults with cystic fibrosis. Eur Respir J. 2009; 33 99-106
21 Szeinberg A, England S, Mindorff C, Fraser I M, Levison H. Maximal inspiratory and expiratory pressures are reduced in hyperinflated, malnourished, young adult male patients with cystic fibrosis. Am Rev Respir Dis. 1985; 132 766-769
22 Mier A, Redington A, Brophy C, Hodson M, Green M. Respiratory muscle function in cystic fibrosis. Thorax. 1990; 45 750-752
23 Ionescu A A, Chatham K, Davies C A, Nixon L S, Enright S, Shale D J. Inspiratory muscle function and body composition in cystic fibrosis. Am J Respir Crit Care Med. 1998; 158 1271-1276
23a O'Neill S, Leahy F, Pasterkamp H, Tal A. The effects of chronic hyperinflation, nutritional status, and posture on respiratory muscle strength in cystic fibrosis. Am Rev Respir Dis. 1983; 128(6) 1051-1054
24 Marks J, Pasterkamp H, Tal A, Leahy F. Relationship between respiratory muscle strength, nutritional status, and lung volume in cystic fibrosis and asthma. Am Rev Respir Dis. 1986; 133 414-417
25 Shook J E, Watkins W D, Camporesi E M. Differential roles of opioid receptors in respiration, respiratory disease, and opiate-induced respiratory depression. Am Rev Respir Dis. 1990; 142 895-909
26 Koh J L, Harrison D, Palermo T M, Turner H, McGraw T. Assessment of acute and chronic pain symptoms in children with cystic fibrosis. Pediatr Pulmonol. 2005; 40 330-335
27 Festini F, Ballarin S, Codamo T, Doro R, Loganes C. Prevalence of pain in adults with cystic fibrosis. J Cyst Fibros. 2004; 3 51-57
28 Palermo T M, Harrison D, Koh J L. Effect of disease-related pain on the health-related quality of life of children and adolescents with cystic fibrosis. Clin J Pain. 2006; 22 532-537
29 Abbott J, Hart A, Morton A M, Dey P, Conway S P, Webb A K. Can health-related quality of life predict survival in adults with cystic fibrosis?. Am J Respir Crit Care Med. 2009; 179 54-58
30 Lee A, Holdsworth M, Holland A, Button B. The immediate effect of musculoskeletal physiotherapy techniques and massage on pain and ease of breathing in adults with cystic fibrosis. J Cyst Fibros. 2009; 8 79-81
31 Ravilly S, Robinson W, Suresh S, Wohl M E, Berde C B. Chronic pain in cystic fibrosis. Pediatrics. 1996; 98(4 Pt 1) 741-747
32 Robinson W M, Ravilly S, Berde C, Wohl M E. End-of-life care in cystic fibrosis. Pediatrics. 1997; 100(2 Pt 1) 205-209
33 Muller N L, Francis P W, Gurwitz D, Levison H, Bryan A C. Mechanism of hemoglobin desaturation during rapid-eye-movement sleep in normal subjects and in patients with cystic fibrosis. Am Rev Respir Dis. 1980; 121 463-469
34 Milross M A, Piper A J, Dobbin C J, Bye P T, Grunstein R R. Sleep disordered breathing in cystic fibrosis. Sleep Med Rev. 2004; 8 295-308
35 Tepper R S, Skatrud J B, Dempsey J A. Ventilation and oxygenation changes during sleep in cystic fibrosis. Chest. 1983; 84 388-393
36 Francis P W, Muller N L, Gurwitz D, Milligan D W, Levison H, Bryan A C. Hemoglobin desaturation: its occurrence during sleep in patients with cystic fibrosis. Am J Dis Child. 1980; 134 734-740
37 Stokes D C, McBride J T, Wall M A, Erba G, Strieder D J. Sleep hypoxemia in young adults with cystic fibrosis. Am J Dis Child. 1980; 134 741-743
38 Ballard R D, Sutarik J M, Clover C W, Suh B Y. Effects of non-REM sleep on ventilation and respiratory mechanics in adults with cystic fibrosis. Am J Respir Crit Care Med. 1996; 153 266-271
39 Milross M A, Piper A J, Norman M et al.. Predicting sleep-disordered breathing in patients with cystic fibrosis. Chest. 2001; 120 1239-1245
40 Milross M A, Piper A J, Norman M et al.. Low-flow oxygen and bilevel ventilatory support: effects on ventilation during sleep in cystic fibrosis. Am J Respir Crit Care Med. 2001; 163 129-134
41 Dancey D R, Tullis E D, Heslegrave R, Thornley K, Hanly P J. Sleep quality and daytime function in adults with cystic fibrosis and severe lung disease. Eur Respir J. 2002; 19 504-510
42 Amin R, Bean J, Burklow K, Jeffries J. The relationship between sleep disturbance and pulmonary function in stable pediatric cystic fibrosis patients. Chest. 2005; 128 1357-1363
43 Naqvi S K, Sotelo C, Murry L, Simakajornboon N. Sleep architecture in children and adolescents with cystic fibrosis and the association with severity of lung disease. Sleep Breath. 2008; 12 77-83
44 Villa M P, Pagani J, Lucidi V, Palamides S, Ronchetti R. Nocturnal oximetry in infants with cystic fibrosis. Arch Dis Child. 2001; 84 50-54
45 Urquhart D S, Montgomery H, Jaffé A. Assessment of hypoxia in children with cystic fibrosis. Arch Dis Child. 2005; 90 1138-1143
46 Mallory G B, Fullmer J J, Vaughan D J. Oxygen therapy for cystic fibrosis. Cochrane Database Syst Rev. 2005; (4) CD003884
47 Frangolias D D, Wilcox P G. Predictability of oxygen desaturation during sleep in patients with cystic fibrosis : clinical, spirometric, and exercise parameters. Chest. 2001; 119 434-441
48 Coffey M J, FitzGerald M X, McNicholas W T. Comparison of oxygen desaturation during sleep and exercise in patients with cystic fibrosis. Chest. 1991; 100 659-662
49 Versteegh F G, Bogaard J M, Raatgever J W, Stam H, Neijens H J, Kerrebijn K F. Relationship between airway obstruction, desaturation during exercise and nocturnal hypoxaemia in cystic fibrosis patients. Eur Respir J. 1990; 3 68-73
50 Narang I, Pike S, Rosenthal M, Balfour-Lynn I M, Bush A. Three-minute step test to assess exercise capacity in children with cystic fibrosis with mild lung disease. Pediatr Pulmonol. 2003; 35 108-113
51 Uyan Z S, Ozdemir N, Ersu R et al.. Factors that correlate with sleep oxygenation in children with cystic fibrosis. Pediatr Pulmonol. 2007; 42 716-722
52 Dobbin C J, Bartlett D, Melehan K, Grunstein R R, Bye P T. The effect of infective exacerbations on sleep and neurobehavioral function in cystic fibrosis. Am J Respir Crit Care Med. 2005; 172 99-104
53 Waterhouse D, Reilly C M, Gallagher C G. Respiratory and limb muscle weakness during acute pulmonary exacerbations of cystic fibrosis [abstract]. Pediatr Pulmonol. 2007; 42 A881
54 Royce S W. Cor pulmonale in infancy and early childhood; report on 34 patients, with special reference to the occurrence of pulmonary heart disease in cystic fibrosis of the pancreas. Pediatrics. 1951; 8 255-274
55 Goldring R M, Fishman A P, Turino G M, Cohen H I, Denning C R, Andersen D H. Pulmonary hypertension and cor pulmonale in cystic fibrosis of the pancreas. J Pediatr. 1964; 65 501-524
56 Moss A J, Harper W H, Dooley R R, Murray J F, Mack J F. Cor pulmonale in cystic fibrosis of the pancreas. J Pediatr. 1965; 67 797-807
57 Florea V G, Florea N D, Sharma R et al.. Right ventricular dysfunction in adult severe cystic fibrosis. Chest. 2000; 118 1063-1068
58 Fraser K L, Tullis D E, Sasson Z, Hyland R H, Thornley K S, Hanly P J. Pulmonary hypertension and cardiac function in adult cystic fibrosis: role of hypoxemia. Chest. 1999; 115 1321-1328
59 Ziegler B, Rovedder P M, Lukrafka J L, Oliveira C L, Menna-Barreto S S, Dalcin Pde T. Submaximal exercise capacity in adolescent and adult patients with cystic fibrosis. J Bras Pneumol. 2007; 33 263-269
60 Costa M, Potvin S, Berthiaume Y et al.. Diabetes: a major co-morbidity of cystic fibrosis. Diabetes Metab. 2005; 31(3 Pt 1) 221-232
61 Finkelstein S M, Wielinski C L, Elliott G R et al.. Diabetes mellitus associated with cystic fibrosis. J Pediatr. 1988; 112 373-377
62 Bismuth E, Laborde K, Taupin P et al.. Glucose tolerance and insulin secretion, morbidity, and death in patients with cystic fibrosis. J Pediatr. 2008; 152 540-545, 545, e1
63 Amadori A, Antonelli A, Balteri I, Schreiber A, Bugiani M, De Rose V. Recurrent exacerbations affect FEV(1) decline in adult patients with cystic fibrosis. Respir Med. 2009; 103 407-413
64 Koch C, Rainisio M, Madessani U Investigators of the European Epidemiologic Registry of Cystic Fibrosis et al. Presence of cystic fibrosis–related diabetes mellitus is tightly linked to poor lung function in patients with cystic fibrosis: data from the European Epidemiologic Registry of Cystic Fibrosis. Pediatr Pulmonol. 2001; 32 343-350
65 Rosenecker J, Höfler R, Steinkamp G et al.. Diabetes mellitus in patients with cystic fibrosis: the impact of diabetes mellitus on pulmonary function and clinical outcome. Eur J Med Res. 2001; 6 345-350
66 Iiyori N, Alonso L C, Li J et al.. Intermittent hypoxia causes insulin resistance in lean mice independent of autonomic activity. Am J Respir Crit Care Med. 2007; 175 851-857
67 Tasali E, Mokhlesi B, Van Cauter E. Obstructive sleep apnea and type 2 diabetes: interacting epidemics. Chest. 2008; 133 496-506
68 Redline S, Storfer-Isser A, Rosen C L et al.. Association between metabolic syndrome and sleep-disordered breathing in adolescents. Am J Respir Crit Care Med. 2007; 176 401-408
69 Leeper-Woodford S K, Detmer K. Acute hypoxia increases alveolar macrophage tumor necrosis factor activity and alters NF-kappaB expression. Am J Physiol. 1999; 276(6 Pt 1) L909-L916
70 Madjdpour C, Jewell U R, Kneller S et al.. Decreased alveolar oxygen induces lung inflammation. Am J Physiol Lung Cell Mol Physiol. 2003; 284 L360-L367
71 Sharma R, Florea V G, Bolger A P et al.. Wasting as an independent predictor of mortality in patients with cystic fibrosis. Thorax. 2001; 56 746-750
72 Asher M I, Pardy R L, Coates A L, Thomas E, Macklem P T. The effects of inspiratory muscle training in patients with cystic fibrosis. Am Rev Respir Dis. 1982; 126 855-859
73 de Jong W, van Aalderen W M, Kraan J, Koëter G H, van der Schans C P. Inspiratory muscle training in patients with cystic fibrosis. Respir Med. 2001; 95 31-36
74 Sawyer E H, Clanton T L. Improved pulmonary function and exercise tolerance with inspiratory muscle conditioning in children with cystic fibrosis. Chest. 1993; 104 1490-1497
75 Enright S, Chatham K, Ionescu A A, Unnithan V B, Shale D J. Inspiratory muscle training improves lung function and exercise capacity in adults with cystic fibrosis. Chest. 2004; 126 405-411
76 Reid W D, Geddes E L, O'Brien K, Brooks D, Crowe J. Effects of inspiratory muscle training in cystic fibrosis: a systematic review. Clin Rehabil. 2008; 22 1003-1013
77 Houston B W, Mills N, Solis-Moya A. Inspiratory muscle training for cystic fibrosis. Cochrane Database Syst Rev. 2008; (4) CD006112
78 Nocturnal Oxygen Therapy Trial Group . Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Ann Intern Med. 1980; 93 391-398
79 Report of the Medical Research Council Working Party . Long term domiciliary oxygen therapy in chronic hypoxic cor pulmonale complicating chronic bronchitis and emphysema. Lancet. 1981; 1 681-686
80 Stewart B N, Hood C I, Block A J. Long-term results of continuous oxygen therapy at sea level. Chest. 1975; 68 486-492
81 Eaton T, Lewis C, Young P, Kennedy Y, Garrett J E, Kolbe J. Long-term oxygen therapy improves health-related quality of life. Respir Med. 2004; 98 285-293
82 Heaton R K, Grant I, McSweeny A J, Adams K M, Petty T L. Psychologic effects of continuous and nocturnal oxygen therapy in hypoxemic chronic obstructive pulmonary disease. Arch Intern Med. 1983; 143 1941-1947
83 Borak J, Sliwiński P, Tobiasz M, Górecka D, Zieliński J. Psychological status of COPD patients before and after one year of long-term oxygen therapy. Monaldi Arch Chest Dis. 1996; 51 7-11
84 Clini E, Vitacca M, Foglio K, Simoni P, Ambrosino N. Long-term home care programmes may reduce hospital admissions in COPD with chronic hypercapnia. Eur Respir J. 1996; 9 1605-1610
85 Weitzenblum E, Oswald M, Apprill M, Ratomaharo J, Kessler R. Evolution of physiological variables in patients with chronic obstructive pulmonary disease before and during long-term oxygen therapy. Respiration. 1991; 58 126-131
86 Zinman R, Corey M, Coates A L et al.. Nocturnal home oxygen in the treatment of hypoxemic cystic fibrosis patients. J Pediatr. 1989; 114 368-377
87 Spier S, Rivlin J, Hughes D, Levison H. The effect of oxygen on sleep, blood gases, and ventilation in cystic fibrosis. Am Rev Respir Dis. 1984; 129 712-718
88 Gozal D. Nocturnal ventilatory support in patients with cystic fibrosis: comparison with supplemental oxygen. Eur Respir J. 1997; 10 1999-2003
89 Yankaskas J R, Marshall B C, Sufian B, Simon R H, Rodman D. Cystic fibrosis adult care: consensus conference report. Chest. 2004; 125(1, Suppl) 1S-39S
90 Piper A J, Parker S, Torzillo P J, Sullivan C E, Bye P T. Nocturnal nasal IPPV stabilizes patients with cystic fibrosis and hypercapnic respiratory failure. Chest. 1992; 102 846-850
91 Granton J T, Kesten S. The acute effects of nasal positive pressure ventilation in patients with advanced cystic fibrosis. Chest. 1998; 113 1013-1018
92 Madden B P, Kariyawasam H, Siddiqi A J, Machin A, Pryor J A, Hodson M E. Noninvasive ventilation in cystic fibrosis patients with acute or chronic respiratory failure. Eur Respir J. 2002; 19 310-313
93 Serra A, Polese G, Braggion C, Rossi A. Non-invasive proportional assist and pressure support ventilation in patients with cystic fibrosis and chronic respiratory failure. Thorax. 2002; 57 50-54
94 Young A C, Wilson J W, Kotsimbos T C, Naughton M T. Randomised placebo controlled trial of non-invasive ventilation for hypercapnia in cystic fibrosis. Thorax. 2008; 63 72-77
95 Moran F, Bradley J. Non-invasive ventilation for cystic fibrosis. Cochrane Database Syst Rev. 2003; (2) CD002769
96 Fauroux B, Le Roux E, Ravilly S, Bellis G, Clément A. Long-term noninvasive ventilation in patients with cystic fibrosis. Respiration. 2008; 76 168-174

Jennifer L Taylor-CousarM.D.

Divisions of Adult and Pediatric Pulmonary Medicine, Department of Internal Medicine

University of New Mexico School of Medicine, MSC 10-5550, 1 University of New Mexico, Albuquerque, NM 87131-0001

eMail: JTCousar@salud.unm.edu