Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-8448b6f56d-42gr6 Total loading time: 0 Render date: 2024-04-24T17:35:10.238Z Has data issue: false hasContentIssue false

12 - Sickle Chest Syndromes in Pregnancy

from Section 3 - Pulmonary Conditions Not Specific to Pregnancy

Published online by Cambridge University Press:  14 April 2020

By
Ann Kinga Malinowski  and
Nadine Shehata
Edited by
Stephen E. Lapinsky  and
Lauren A. Plante
Stephen E. Lapinsky
Affiliation:
Mount Sinai Hospital, Toronto
Lauren A. Plante
Affiliation:
Drexel University Hospital, Philadelphia
Get access

Summary

common haemoglobinopathy, affecting 283 000 infants born annually. Sickle cell carriers are found throughout sub-Saharan Africa, the Mediterranean, the Middle East and the Indian subcontinent. Estimated frequency for sickle cell trait (HbAS) varies by ethnicity: 1:10 for African-Carribeans, 1:4 for West Africans, 1:100 for Cypriots and 1:100 for Pakistani, Indian. Haemoglobin S is created by the substitution of valine for glutamic acid (CAG → GTG), in the β-globin gene at position 6.

Type
Chapter
Information
Respiratory Disease in Pregnancy , pp. 113 - 125
Publisher: Cambridge University Press
Print publication year: 2020

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Williams, TN, Weatherall, DJ. World distribution, population genetics, and health burden of the hemoglobinopathies. Cold Spring Harb Perspect Med. 2012 2:a011692.CrossRefGoogle ScholarPubMed
Oteng-Ntim, E, Chase, AR, Howard, J, Khazaezadeh, N, Anionwu, EN. Sickle cell disease in pregnancy. Obstet, Gynaecol Reproduct Med. 2008 18(10):272–8.Google Scholar
Ballas, SK, Lieff, S, Benjamin, LJ, et al. Definitions of the phenotypic manifestations of sickle cell disease. Am J Hematol. 2010 85:6–13.CrossRefGoogle ScholarPubMed
Platt, OS, Brambilla, DJ, Rosse, WF, et al. Mortality in sickle cell disease: life expectancy and risk factors for early death. N Engl J Med. 1994 330:1639–44.Google Scholar
Vichinsky, EP, Neumayr, LD, Earles, AN, et al. Causes and outcomes of the acute chest syndrome in sickle cell disease: National Acute Chest Syndrome Study Group. N Engl J Med. 2000 342:1855–65.CrossRefGoogle ScholarPubMed
Gladwin, MT, Sachdev, V, Jison, ML, et al. Pulmonary hypertension as a risk factor for death in patients with sickle cell disease. N Engl J Med. 2004 350:886–95.CrossRefGoogle ScholarPubMed
Perronne, V, Roberts-Harewood, M, Bachir, D, et al. Patterns of mortality in sickle cell disease in adults in France and England. Hematol J. 2002 3:56–60.Google Scholar
Gladwin, MT, Vichinsky, E. Pulmonary complications of sickle cell disease. N Engl J Med. 2008 359:2254–65.CrossRefGoogle ScholarPubMed
LoMauro, A, Aliverti, A. Respiratory physiology of pregnancy: physiology masterclass. Breathe (Sheff). 2015 11:297–301.Google Scholar
Hegewald, MJ, Crapo, RO. Respiratory physiology in pregnancy. Clin Chest Med. 2011 32(1):1–13.Google Scholar
Koumbourlis, AC. Lung function in sickle cell disease. Paediatr Respir Rev. 2014 15:33–7.Google ScholarPubMed
Klings, ES, Wyszynski, DF, Nolan, VG, Steinberg, MH. Abnormal pulmonary function in adults with sickle cell anemia. Am J Respir Crit Care Med. 2006 173:1264–9.Google Scholar
Field, JJ, Glassberg, J, Gilmore, A, et al. Longitudinal analysis of pulmonary function in adults with sickle cell disease. Am J Hematol. 2008 83:574–6.CrossRefGoogle ScholarPubMed
Santoli, F, Zerah, F, Vasile, N, et al. Pulmonary function in sickle cell disease with or without acute chest syndrome. European Respiratory Journal. 1998 12:1124–9.CrossRefGoogle ScholarPubMed
Santoli, F, Zerah, F, Vasile, N, et al. Pulmonary function in sickle cell disease with or without acute chest syndrome. Eur Respir J. 1998 12:1124–9.CrossRefGoogle ScholarPubMed
Oteng-Ntim, E, Howard, J. Green-top Guideline No. 61: Management of sickle cell disease in pregnancy. 2011. Available at: www.rcog.org.uk/globalassets/documents/guidelines/gtg_61.pdf (Accessed November 2019).Google Scholar
Paul, RN, Castro, OL, Aggarwal, A, Oneal, PA. Acute chest syndrome: sickle cell disease. Eur J Haematol. 2011 87:191–207.Google Scholar
Morris, C, Vichinsky, E, Styles, L. Clinician assessment for acute chest syndrome in febrile patients with sickle cell disease: is it accurate enough? Ann Emerg Med. 1999 34:64–9.CrossRefGoogle ScholarPubMed
Castro, O, Brambilla, DJ, Thorington, B, et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood. 1994 84:643–9.Google Scholar
Alabdulaali, MK. Sickle cell disease patients in eastern province of Saudi Arabia suffer less severe acute chest syndrome than patients with African haplotypes. Ann Thorac Med. 2007 2:158–62.CrossRefGoogle ScholarPubMed
Resende Cardoso, PS, Lopes Pessoa de Aguiar, RA, Viana, MB. Clinical complications in pregnant women with sickle cell disease: prospective study of factors predicting maternal death or near miss. Rev Bras Hematol Hemoter. 2014 36:256–63.CrossRefGoogle ScholarPubMed
Oteng-Ntim, E, Ayensah, B, Knight, M, Howard, J. Pregnancy outcome in patients with sickle cell disease in the UK – a national cohort study comparing sickle cell anaemia (HbSS) with HbSC disease. Br J Haematol. 2015 169:129–37.Google Scholar
Okusanya, BO, Oladapo, OT. Prophylactic versus selective blood transfusion for sickle cell disease in pregnancy. Cochrane Database Syst Rev. 2013 (12):CD010378.Google Scholar
Asnani, MR, McCaw-Binns, AM, Reid, ME. Excess risk of maternal death from sickle cell disease in Jamaica: 1998–2007. PLoS One. 2011 6:e26281.CrossRefGoogle ScholarPubMed
Cecchini, J, Fartoukh, M. Sickle cell disease in the ICU. Curr Opin Crit Care. 2015 21:569–75.Google Scholar
Nomura, RM, Igai, AM, Tosta, K, et al. Acute chest syndrome in pregnant women with hemoglobin SC disease. Clinics (Sao Paulo). 2009 64:927–8.CrossRefGoogle ScholarPubMed
Ballas, SK, Gupta, K, Adams-Graves, P. Sickle cell pain: a critical reappraisal. Blood. 2012 120:3647–56.CrossRefGoogle Scholar
Howard, J, Hart, N, Roberts-Harewood, M, et al. Guideline on the management of acute chest syndrome in sickle cell disease. Br J Haematol. 2015 169:492–505.Google Scholar
Caboot, JB, Jawad, AF, McDonough, JM, et al. Non-invasive measurements of carboxyhemoglobin and methemoglobin in children with sickle cell disease. Pediatr Pulmonol. 2012 47:808–15.Google Scholar
Cook, JV, Kyriou, J. Radiation from CT and perfusion scanning in pregnancy. Br Med J. 2005 331:350.Google Scholar
Wan, T, Skeith, L, Karovitch, A, Rodger, M, Le Gal, G. Guidance for the diagnosis of pulmonary embolism during pregnancy: consensus and controversies. Thromb Res. 2017 157:23–8.Google Scholar
Bourjeily, G, Paidas, M, Khalil, H, Rosene-Montella, K, Rodger, M. Pulmonary embolism in pregnancy. Lancet. 2010 375:500–12.Google Scholar
Dang, NC, Johnson, C, Eslami-Farsani, M, Haywood, LJ. Bone marrow embolism in sickle cell disease: a review. Am J Hematol. 2005 79:61–7.Google Scholar
Mekontso Dessap, A, Deux, JF, Habibi, A, et al. Lung imaging during acute chest syndrome in sickle cell disease: computed tomography patterns and diagnostic accuracy of bedside chest radiograph. Thorax. 2014 69:144–51.Google Scholar
Lisbona, R, Derbekyan, V, Novales-Diaz, JA. Scintigraphic evidence of pulmonary vascular occlusion in sickle cell disease. J Nucl Med. 1997 38:1151–3.Google ScholarPubMed
Bhalla, M, Abboud, MR, McLoud, TC, et al. Acute chest syndrome in sickle cell disease: CT evidence of microvascular occlusion. Radiology. 1993 187:45–9.CrossRefGoogle ScholarPubMed
Marti-Carvajal, AJ, Conterno, LO, Knight-Madden, JM. Antibiotics for treating acute chest syndrome in people with sickle cell disease. Cochrane Database Syst Rev. 2015 (3):CD006110.Google ScholarPubMed
Gelfand, MJ, Daya, SA, Rucknagel, DL, Kalinyak, KA, Paltiel, HJ. Simultaneous occurrence of rib infarction and pulmonary infiltrates in sickle cell disease patients with acute chest syndrome. J Nucl Med. 1993 34:614–18.Google Scholar
Davis, BA, Allard, S, Qureshi, A, et al. Guidelines on red cell transfusion in sickle cell disease Part II: indications for transfusion. Br J Haematol. 2017 176:192–209.Google Scholar
Bellet, PS, Kalinyak, KA, Shukla, R, Gelfand, MJ, Rucknagel, DL. Incentive spirometry to prevent acute pulmonary complications in sickle cell diseases. N Engl J Med. 1995 333:699–703.Google Scholar
Canadian Haemoglobinopathy Association. Consensus statement on the care of patients with sickle cell disease in Canada. Version 2.0. https://sicklecellanemia.ca/pdf_2016/CANHAEM.pdf (Accessed November 2019).Google Scholar
Yawn, BP, Buchanan, GR, Afenyi-Annan, AN, et al. Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members. J Am Med Assoc. 2014 312:1033–48.Google Scholar
Mehta, N, Chen, K, Hardy, E, Powrie, R. Respiratory disease in pregnancy. Best Pract Res Clin Obstet Gynaecol. 2015 29:598–611.Google Scholar
Dastgiri, S, Dolatkhah, R. Blood transfusions for treating acute chest syndrome in people with sickle cell disease. Cochrane Database Syst Rev. 2016 (8):CD007843.Google Scholar
Malinowski, AK, Shehata, N, D’Souza, R, et al. Prophylactic transfusion for pregnant women with sickle cell disease: a systematic review and meta-analysis. Blood. 2015 126:2424–35.CrossRefGoogle Scholar
Gladwin, MT, Kato, GJ. Cardiopulmonary complications of sickle cell disease: role of nitric oxide and hemolytic anemia. Hematology Am Soc Hematol Educ Program. 2005 :51–7.Google Scholar
Maitre, B, Djibre, M, Katsahian, S, et al. Inhaled nitric oxide for acute chest syndrome in adult sickle cell patients: a randomized controlled study. Intensive Care Med. 2015 41:2121–9.Google Scholar
Oppert, M, Jorres, A, Barckow, D, et al. Inhaled nitric oxide for ARDS due to sickle cell disease. Swiss Med Wkly. 2004 134:165–7.Google Scholar
Atz, AM, Wessel, DL. Inhaled nitric oxide in sickle cell disease with acute chest syndrome. Anesthesiology. 1997 87:988–90.CrossRefGoogle ScholarPubMed
Mehari, A, Klings, ES. Chronic pulmonary complications of sickle cell disease. Chest. 2016 149:1313–24.CrossRefGoogle ScholarPubMed
Klings, ES, Machado, RF, Barst, RJ, et al. An official American Thoracic Society clinical practice guideline: diagnosis, risk stratification, and management of pulmonary hypertension of sickle cell disease. Am J Respir Crit Care Med. 2014 189:727–40.Google Scholar
Mehari, A, Alam, S, Tian, X, et al. Hemodynamic predictors of mortality in adults with sickle cell disease. Am J Respir Crit Care Med. 2013 187:840–7.Google Scholar
Forfia, PR, Vachiery, JL. Echocardiography in pulmonary arterial hypertension. Am J Cardiol. 2012 110:16S-24S.Google Scholar
Fitzgerald, M, Fagan, K, Herbert, DE, et al. Misclassification of pulmonary hypertension in adults with sickle hemoglobinopathies using Doppler echocardiography. South Med J. 2012 105:300–5.Google Scholar
Parent, F, Bachir, D, Inamo, J, et al. A hemodynamic study of pulmonary hypertension in sickle cell disease. N Engl J Med. 2011 365:44–53.Google Scholar
Burlingame, JM, Yamasato, K, Ahn, HJ, Seto, T, Tang, WHW. B-type natriuretic peptide and echocardiography reflect volume changes during pregnancy. J Perinat Med. 2017 45:577–83.Google Scholar
Ker, JA, Soma-Pillay, P. NT-proBNP: When is it useful in obstetric medicine? Obstet Med. 2017 11(1):3-5.Google Scholar
Sahni, S, Palkar, AV, Rochelson, BL, Kepa, W, Talwar, A. Pregnancy and pulmonary arterial hypertension: a clinical conundrum. Pregnancy Hypertens. 2015 5:157–64.CrossRefGoogle ScholarPubMed
Gei, A, Montufar-Rueda, C. Pulmonary hypertension and pregnancy: an overview. Clin Obstet Gynecol. 2014 57:806–26.Google Scholar
Monagle, J, Manikappa, S, Ingram, B, Malkoutzis, V. Pulmonary hypertension and pregnancy: the experience of a tertiary institution over 15 years. Ann Card Anaesth. 2015 18:153–60.Google Scholar
Al-Balas, M, Bozzo, P, Einarson, A. Use of diuretics during pregnancy. Can Fam Physician. 2009 55:44–5.Google Scholar
Venkata, C, Venkateshiah, SB. Sleep-disordered breathing during pregnancy. J Am Board Fam Med. 2009 22:158–68.CrossRefGoogle ScholarPubMed
Bates, SM, Middeldorp, S, Rodger, M, James, AH, Greer, I. Guidance for the treatment and prevention of obstetric-associated venous thromboembolism. J Thromb Thrombolysis. 2016 41:92–128.Google Scholar
Bates, SM, Greer, IA, Middeldorp, S, et al. VTE, thrombophilia, antithrombotic therapy, and pregnancy: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012 141(2 Suppl):e691S-736S.CrossRefGoogle ScholarPubMed
Canadian Haemoglobinopathy Association. Contraception, Pre-Conception Couseling, and Pregnancy. In: Consensus Statement on the Care of Patients with sickle cell disease in Canada. Version 2.0. 2015:88–91. http://sicklecellanemia.ca/pdf_2016/CANHAEM.pdf. (Accessed November 2019).Google Scholar
National Institutes of Health, National Heart, Lung and Blood Institute – Division of Blood Diseases and Resources. The management of sickle cell disease (4th edn). www.nhlbi.nih.gov/files/docs/guidelines/sc_mngt.pdf. (accessed November 2019).Google Scholar
Naik, RP, Streiff, MB, Haywood, C Jr, Nelson, JA, Lanzkron, S. Venous thromboembolism in adults with sickle cell disease: a serious and under-recognized complication. Am J Med. 2013 126:443–9.Google Scholar
Novelli, EM, Huynh, C, Gladwin, MT, Moore, CG, Ragni, MV. Pulmonary embolism in sickle cell disease: a case-control study. J Thromb Haemost. 2012 10:760–6.Google Scholar
Mekontso Dessap, A, Deux, JF, Abidi, N, et al. Pulmonary artery thrombosis during acute chest syndrome in sickle cell disease. Am J Respir Crit Care Med. 2011 184:1022–9.Google Scholar
Seaman, CD, Yabes, J, Li, J, Moore, CG, Ragni, MV. Venous thromboembolism in pregnant women with sickle cell disease: a retrospective database analysis. Thromb Res. 2014 134:1249–52.Google Scholar
Sparkenbaugh, E, Pawlinski, R. Prothrombotic aspects of sickle cell disease. J Thromb Haemost. 2017 15:1307–16.Google Scholar
Nelson-Piercy, C, MacCallum, P, Mackillop, L, Radcliffe, J. Green-top Guideline No. 37a: Reducing the Risk of Thrombosis and Embolism During Pregnancy and the Puerperium. 2015. www.rcog.org.uk/globalassets/documents/guidelines/gtg-37a.pdf (accessed November 2019).Google Scholar
Bourjeily, G, Paidas, M, Khalil, H, Rosene-Montella, K, Rodger, M. Pulmonary embolism in pregnancy. Lancet. 2010 375:500–12.Google Scholar
Chan, WS, Rey, E, Kent, NE, et al. Venous thromboembolism and antithrombotic therapy in pregnancy. J Obstet Gynaecol Can. 2014 36:527–53.Google Scholar
James, A, Committee on Practice Bulletins-Obstetrics. Practice bulletin no. 123: thromboembolism in pregnancy. Obstet Gynecol. 2011 118(3):718–29.Google Scholar
Horlocker, TT, Vandermeuelen, E, Kopp, SL, Gogarten, W, Leffert, LR, Benzon, HT. Regional anesthesia in the patient receiving antithrombotic or thrombolytic therapy: American Society of Regional Anesthesia and Pain Medicine Evidence-Based Guidelines (Fourth Edition). Reg Anesth Pain Med. 2018 43:263–309.CrossRefGoogle ScholarPubMed
Malinowski, AK, Bomba-Opon, D, Parrish, J, Sarzynska, U, Farine, D. Venous thromboembolism in obese pregnant women: approach to diagnosis and management. Ginekol Pol. 2017 88:453–9.Google Scholar
Morris, CR. Asthma management: reinventing the wheel in sickle cell disease. Am J Hematol. 2009 84:234–41.CrossRefGoogle ScholarPubMed
Knight-Madden, J, Greenough, A. Acute pulmonary complications of sickle cell disease. Paediatr Respir Rev. 2014 15:13–16.Google Scholar
Field, JJ, DeBaun, MR. Asthma and sickle cell disease: two distinct diseases or part of the same process? Hematology Am Soc Hematol Educ Program. 2009:45–53.Google Scholar
Cohen, RT, Madadi, A, Blinder, MA, et al. Recurrent, severe wheezing is associated with morbidity and mortality in adults with sickle cell disease. Am J Hematol. 2011 86:756–61.Google Scholar
National Heart Lung and Blood Institute. Guidelines for the diagnosis and management of asthma. 2007. www.nhlbi.nih.gov/files/docs/guidelines/asthsumm.pdf (accessed November 2019).Google Scholar
Akinsheye, I, Alsultan, A, Solovieff, N, et al. Fetal hemoglobin in sickle cell anemia. Blood. 2011 118:19–27.Google Scholar
[No authors listed]. Practice bulletin no. 145: antepartum fetal surveillance. Obstet Gynecol. 2014 124:182–92.Google Scholar
Alfirevic, Z, Stampalija, T, Dowswell, T. Fetal and umbilical Doppler ultrasound in high-risk pregnancies. Cochrane Database Syst Rev. 2017 6:CD007529.Google Scholar
Mari, G, Hanif, F, Kruger, M, et al. Middle cerebral artery peak systolic velocity: a new Doppler parameter in the assessment of growth-restricted fetuses. Ultrasound Obstet Gynecol. 2007 29:310–16.Google Scholar
National Toxicology Program (NTP), Center for Evaluation of Risks to Human Reproduction (CERHR). NTP-CERHR Monograph on the Potential Human Reproductive and Developmental Effects of Hydroxyurea. NIH Publication No. 08 – 5993. 2008. ntp.niehs.nih.gov/ntp/ohat/hydroxyurea/humonograph20090401.pdf (accessed December 2019).Google Scholar

Save book to Kindle

To save this book to your Kindle, first ensure coreplatform@cambridge.org is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×