Abstract
Purpose of Review
We describe sleep disturbances in patients with cystic fibrosis emphasizing treatment of abnormal breathing during sleep with noninvasive ventilation.
Recent Findings
Advances in treatment have transformed cystic fibrosis from a pediatric disorder to an adult condition. The increase in cystic fibrosis life expectancy renders these patients at high risk for nocturnal and diurnal respiratory failure. Hypoventilation during sleep precedes diurnal hypoventilation in cystic fibrosis. In advanced cases, noninvasive ventilation improves awake ABGs, respiratory muscle strength, dyspnea, subjective sleep quality, and level of daily activities and attenuates sleep hypoventilation.
Summary
Noninvasive ventilation can ameliorate symptoms and improve gas exchange, particularly in cystic fibrosis with advanced lung dysfunction. There is insufficient literature describing the effect of noninvasive ventilation on survival in cystic fibrosis.
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References
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Riordan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z, et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science. 1989;245(4922):1066–73.
Elborn JS. Cystic fibrosis. Lancet. 2016;388(10059):2519–31.
O’Sullivan BP, Freedman SD. Cystic fibrosis. Lancet. 2009;373(9678):1891–904.
Cogen JD, Hall M, Loeffler DR, Gove N, Onchiri F, Sawicki GS, et al. Linkage of the CF Foundation patient registry with the pediatric health information system database. Pediatr Pulmonol. 2019;54(6):721–8.
Dilokthornsakul P, Patidar M, Campbell JD. Forecasting the long-term clinical and economic outcomes of lumacaftor/ivacaftor in cystic fibrosis patients with homozygous phe508del mutation. Value Health. 2017;20(10):1329–35.
Shakkottai A, O’Brien LM, Nasr SZ, Chervin RD. Sleep disturbances and their impact in pediatric cystic fibrosis. Sleep Med Rev. 2018;42:100–10.
Stephenson AL, Bell SC. The Cystic Fibrosis Foundation patient registry. Design and methods of a national observational disease registry. Ann Am Thorac Soc. 2016;13(7):1014–5.
Hodson ME, Madden BP, Steven MH, Tsang VT, Yacoub MH. Non-invasive mechanical ventilation for cystic fibrosis patients--a potential bridge to transplantation. Eur Respir J. 1991;4(5):524–7.
Regnis JA, Piper AJ, Henke KG, Parker S, Bye PT, Sullivan CE. Benefits of nocturnal nasal CPAP in patients with cystic fibrosis. Chest. 1994;106(6):1717–24.
Holland AE, Denehy L, Ntoumenopoulos G, Naughton MT, Wilson JW. Non-invasive ventilation assists chest physiotherapy in adults with acute exacerbations of cystic fibrosis. Thorax. 2003;58(10):880–4.
Milross MA, Piper AJ, Norman M, Becker HF, Willson GN, Grunstein RR, 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(1):129–34.
Young AC, Wilson JW, Kotsimbos TC, Naughton MT. Randomised placebo controlled trial of non-invasive ventilation for hypercapnia in cystic fibrosis. Thorax. 2008;63(1):72–7.
Naqvi SK, 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(1):77–83.
Katz ES. Cystic fibrosis and sleep. Clin Chest Med. 2014;35(3):495–504.
Shwachman H, Kulczycki LL, Mueller HL, Flake CG. Nasal polyposis in patients with cystic fibrosis. Pediatrics. 1962;30:389–401.
Bercovitch RS, Tsai SC. Respiratory medications and sleep. Current Respiratory Care Reports. 2012;1(2):123–30.
Stokes DC, McBride JT, Wall MA, Erba G, Strieder DJ. Sleep hypoxemia in young adults with cystic fibrosis. Am J Dis Child. 1980;134(8):741–3.
Bradley S, Solin P, Wilson J, Johns D, Walters EH, Naughton MT. Hypoxemia and hypercapnia during exercise and sleep in patients with cystic fibrosis. Chest. 1999;116(3):647–54.
Paranjape SM, McGinley BM, Braun AT, Schneider H. Polysomnographic markers in children with cystic fibrosis lung disease. Pediatrics. 2015;136(5):920–6.
Fauroux B, Pepin JL, Boelle PY, Cracowski C, Murris-Espin M, Nove-Josserand R, et al. Sleep quality and nocturnal hypoxaemia and hypercapnia in children and young adults with cystic fibrosis. Arch Dis Child. 2012;97(11):960–6.
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(5):712–8.
Dancey DR, Tullis ED, Heslegrave R, Thornley K, Hanly PJ. Sleep quality and daytime function in adults with cystic fibrosis and severe lung disease. Eur Respir J. 2002;19(3):504–10.
Jankelowitz L, Reid KJ, Wolfe L, Cullina J, Zee PC, Jain M. Cystic fibrosis patients have poor sleep quality despite normal sleep latency and efficiency. Chest. 2005;127(5):1593–9.
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(3):1357–63.
Vandeleur M, Walter LM, Armstrong DS, Robinson P, Nixon GM, Horne RSC. How well do children with cystic fibrosis sleep? An actigraphic and questionnaire-based study. J Pediatr. 2017;182:170–6.
Milross MA, Piper AJ, Dobbin CJ, Bye PT, Grunstein RR. Sleep disordered breathing in cystic fibrosis. Sleep Med Rev. 2004;8(4):295–308.
Douglas NJ, White DP, Pickett CK, Weil JV, Zwillich CW. Respiration during sleep in normal man. Thorax. 1982;37(11):840–4.
Worsnop C, Kay A, Kim Y, Trinder J, Pierce R. Effect of age on sleep onset-related changes in respiratory pump and upper airway muscle function. J Appl Physiol (1985). 2000;88(5):1831–9.
Bristow JD, Honour AJ, Pickering TG, Sleight P. Cardiovascular and respiratory changes during sleep in normal and hypertensive subjects. Cardiovasc Res. 1969;3(4):476–85.
Gothe B, Altose MD, Goldman MD, Cherniack NS. Effect of quiet sleep on resting and CO2-stimulated breathing in humans. J Appl Physiol Respir Environ Exerc Physiol. 1981;50(4):724–30.
Tepper RS, Skatrud JB, Dempsey JA. Ventilation and oxygenation changes during sleep in cystic fibrosis. Chest. 1983;84(4):388–93.
Muller NL, Francis PW, Gurwitz D, Levison H, Bryan AC. Mechanism of hemoglobin desaturation during rapid-eye-movement sleep in normal subjects and in patients with cystic fibrosis. Am Rev Respir Dis. 1980;121(3):463–9.
Lwin N, Giammona ST. Ventilatory responses to inspired CO 2 in patients with cystic fibrosis. Chest. 1972;61(3):206–12.
Paiva R, Krivec U, Aubertin G, Cohen E, Clement A, Fauroux B. Carbon dioxide monitoring during long-term noninvasive respiratory support in children. Intensive Care Med. 2009;35(6):1068–74.
Francis PW, Muller NL, Gurwitz D, Milligan DW, Levison H, Bryan AC. Hemoglobin desaturation: its occurrence during sleep in patients with cystic fibrosis. Am J Dis Child. 1980;134(8):734–40.
Coffey MJ, FitzGerald MX, McNicholas WT. Comparison of oxygen desaturation during sleep and exercise in patients with cystic fibrosis. Chest. 1991;100(3):659–62.
McEvoy RD, Pierce RJ, Hillman D, Esterman A, Ellis EE, Catcheside PG, et al. Nocturnal non-invasive nasal ventilation in stable hypercapnic COPD: a randomised controlled trial. Thorax. 2009;64(7):561–6.
Spicuzza L, Sciuto C, Leonardi S, La Rosa M. Early occurrence of obstructive sleep apnea in infants and children with cystic fibrosis. Arch Pediatr Adolesc Med. 2012;166(12):1165–9.
Chervin RD, Hedger K, Dillon JE, Pituch KJ. Pediatric Sleep Questionnaire (PSQ): validity and reliability of scales for sleep-disordered breathing, snoring, sleepiness, and behavioral problems. Sleep Med. 2000;1(1):21–32.
Nagappa M, Liao P, Wong J, Auckley D, Ramachandran SK, Memtsoudis S, et al. Validation of the STOP-Bang Questionnaire as a screening tool for obstructive sleep apnea among different populations: a systematic review and meta-analysis. PLoS One. 2015;10(12):e0143697.
Medarov BI, Victorson DE, Judson MA. Patient-reported outcome measures for sleep disorders and related problems: clinical and research applications. Chest. 2013;143(6):1809–18.
Clinical indications for noninvasive positive pressure ventilation in chronic respiratory failure due to restrictive lung disease, COPD, and nocturnal hypoventilation--a consensus conference report. Chest. 1999;116(2):521–34.
Mehta S, Hill NS. Noninvasive ventilation. Am J Respir Crit Care Med. 2001;163(2):540–77.
Simonds AK. Home ventilation. Eur Respir J Suppl. 2003;47:38 s–46 s.
Brochard L, Mancebo J, Wysocki M, Lofaso F, Conti G, Rauss A, et al. Noninvasive ventilation for acute exacerbations of chronic obstructive pulmonary disease. N Engl J Med. 1995;333(13):817–22.
Kohnlein T, Windisch W, Kohler D, Drabik A, Geiseler J, Hartl S, et al. Non-invasive positive pressure ventilation for the treatment of severe stable chronic obstructive pulmonary disease: a prospective, multicentre, randomised, controlled clinical trial. Lancet Respir Med. 2014;2(9):698–705.
• Murphy PB, Rehal S, Arbane G, Bourke S, PMA C, Crook AM, et al. Effect of home noninvasive ventilation with oxygen therapy vs oxygen therapy alone on hospital readmission or death after an acute COPD exacerbation: a randomized clinical trial. JAMA. 2017;317(21):2177–86 Suggests that the use of NIV in patients with COPD with diurnal hypoventilation can also be used in patients with CF and diurnal hypoventilation and have the same therapeutic benefits.
Bright-Thomas RJ, Johnson SC. What is the role of noninvasive ventilation in cystic fibrosis? Curr Opin Pulm Med. 2014;20(6):618–22.
Gozal D. Nocturnal ventilatory support in patients with cystic fibrosis: comparison with supplemental oxygen. Eur Respir J. 1997;10(9):1999–2003.
Andrade RG, Madeiro F, Piccin VS, Moriya HT, Schorr F, Sardinha PS, et al. Impact of acute changes in CPAP flow route in sleep apnea treatment. Chest. 2016;150(6):1194–201.
Andrade RGS, Viana FM, Nascimento JA, Drager LF, Moffa A, Brunoni AR, et al. Nasal vs oronasal CPAP for OSA treatment: a meta-analysis. Chest. 2018;153(3):665–74.
Nava S, Navalesi P, Gregoretti C. Interfaces and humidification for noninvasive mechanical ventilation. Respir Care. 2009;54(1):71–84.
Navalesi P, Fanfulla F, Frigerio P, Gregoretti C, Nava S. Physiologic evaluation of noninvasive mechanical ventilation delivered with three types of masks in patients with chronic hypercapnic respiratory failure. Crit Care Med. 2000;28(6):1785–90.
Hart N, Polkey MI, Clement A, Boule M, Moxham J, Lofaso F, et al. Changes in pulmonary mechanics with increasing disease severity in children and young adults with cystic fibrosis. Am J Respir Crit Care Med. 2002;166(1):61–6.
•• Moran F, Bradley JM, Piper AJ. Non-invasive ventilation for cystic fibrosis. Cochrane Database Syst Rev. 2017;2:CD002769 The Cochrane analysis provides a thorough description of how nocturnal NIV used together with overnight oxygen improves gas exchange during sleep to a greater extent than oxygen therapy alone in patients with CF.
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All authors met the four criteria for authorship established by the International Committee of Medical Journal Editors: Pamela Barletta, Alejandro Chediak, Alexandre Abreu, Maria Tupayachi Ortiz, and Matthias Salathe were responsible for the conception, design, and drafting the work, revising the work and reviewing the manuscript. All authors provided final approval of the version to be published and agreed to be accountable for all aspects of the work in ensuring including the accuracy and/or integrity of the work.
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Barletta, P., Ortiz, M.T., Abreu, A.R. et al. Sleep, Obstructive Sleep Apnea, and Chronic Alveolar Hypoventilation in Cystic Fibrosis: Role of Noninvasive Ventilation During Sleep. Curr Pulmonol Rep 9, 110–117 (2020). https://doi.org/10.1007/s13665-020-00252-0
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DOI: https://doi.org/10.1007/s13665-020-00252-0