Clinical Case Database / Category: Case Based Discussion

A 4-year-old boy with sickle cell disease & acute chest syndrome

Publication details

Dr Florian Gahleitner, Dr Ben Cambers, Dr Kim Sykes
Foundation Years Journal, volume 8, issue 6, p.46 (123Doc Education, London, June 2014)

Abstract

A 4 year old boy with sickle cell disease (SCD) presented with abdominal and leg pain to his local district general hospital (DGH). He was treated for a presumed vaso-occlusive crisis (VOC) receiving antibiotics, fluids and analgesia. During the fist 24 hours, he rapidly deteriorated with respiratory distress, increasing oxygen requirement and left lower lobe consolidation on chest X-ray (CXR). He was intubated, ventilated and retrieved by PICU with a working diagnosis of acute chest syndrome (ACS). Multi-organ dysfunction syndrome (MODS) ensued over the subsequent 24 hours. He required high frequency oscillation ventilation (HFOV), inhaled nitric oxide (iNO), inotropes, and broad-spectrum antibiotic cover. Exchange transfusion decreased his HbS fraction from 45% to 15%. Over the next 12 days he required mechanical ventilation, inotropic support, haemofitration, antibiotics and antifungal treatment. Ongoing consolidation of one lung prompted a bronchoscopy revealing plastic bronchitis. Rigid bronchoscopy with instillation of DNAse and saline was required to remove the plastic casts. He was extubated two days later making a swift recovery thereafter.

This article discusses the management of a child with SCD with reference to the current literature. Important aspects include the recognition, rapid progression, clinical management and outcome of ACS. Our discussion highlights the pitfalls of managing an unusual complication of ACS and the complexities of the decision making process.

Access the Clinical Cases Database

A subscription is required to read the full article. Please subscribe using one of the options below.

Authors

Dr Florian Gahleitner (Corresponding author)

ST5 Paediatrics, Paediatric Department, Southampton,
General Hospital, Tremona Road, Southampton, SO16 6YD
florian.gahleitner@doctors.net.uk

Dr Ben Cambers

ST2 Paediatrics, Paediatric Department, Southampton,
General Hospital, Tremona Road, Southampton, SO16 6YD
benc39@hotmail.com

Dr Kim Sykes

Consultant Paediatric Intensivist, Paediatric Department,
Southampton General Hospital, Tremona Road, Southampton, SO16 6YD
kim.sykes@uhs.nhs.uk

References

1.  Castro O, Brambilla DJ, Thorington B, Reindorf CA, Scott RB, Gillette P, et al. The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood. 1994;84(2):643-9.

2.  Vichinsky EP, Styles LA, Colangelo LH, Wright EC, Castro O, Nickerson B. Acute chest syndrome in sickle cell disease: clinical presentation and course. Cooperative Study of Sickle Cell Disease. Blood. 1997;89(5):1787-92.

3.  Vichinsky EP, Neumayr LD, Earles AN, Williams R, Lennette ET, Dean D, 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(25):1855-65.

4.  Needleman JP, Benjamin LJ, Sykes JA, Aldrich TK. Breathing patterns during vaso-occlusive crisis of sickle cell disease. Chest. 2002;122(1):43-6.

5.  Kopecky EA, Jacobson S, Joshi P, Koren G. Systemic exposure to morphine and the risk of acute chest syndrome in sickle cell disease. Clin Pharmacol Ther. 2004;75(3):140-6.

6.  Paul RN, Castro OL, Aggarwal A, Oneal PA. Acute chest syndrome: sickle cell disease. Eur J Haematol. 2011;87(3):191-207.

7.  Steinberg MH. Management of sickle cell disease. N Engl J Med. 1999;340(13):1021-30.

8.  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(11):699-703.

9.  Aldrich TK, Dhuper SK, Patwa NS, Makolo E, Suzuka SM, Najeebi SA, et al. Pulmonary entrapment of sickle cells: the role of regional alveolar hypoxia. J Appl Physiol (1985). 1996;80(2):531-9.

10.  Frenette PS. Sickle cell vaso-occlusion: multistep and multicellular paradigm. Curr Opin Hematol. 2002;9(2):101-6.

11.  Styles LA, Schalkwijk CG, Aarsman AJ, Vichinsky EP, Lubin BH, Kuypers FA. Phospholipase A2 levels in acute chest syndrome of sickle cell disease. Blood. 1996;87(6):2573-8.

12.  Vichinsky E, Williams R, Das M, Earles AN, Lewis N, Adler A, et al. Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia. Blood. 1994;83(11):3107-12.

13.  Mallouh AA, Asha M. Benefiial effect of blood transfusion in children with sickle cell chest syndrome. Am J Dis Child. 1988;142(2):178-82.

14.  Emre U, Miller ST, Gutierez M, Steiner P, Rao SP, Rao M. Effect of transfusion in acute chest syndrome of sickle cell disease. J Pediatr. 1995;127(6):901-4.

15.  Miller ST. How I treat acute chest syndrome in children with sickle cell disease. Blood. 2011;117(20):5297-305.

16.  Raghuram N, Pettignano R, Gal AA, Harsch A, Adamkiewicz TV. Plastic bronchitis: an unusual complication associated with sickle cell disease and the acute chest syndrome. Pediatrics. 1997;100(1):139-42.

17.  Moser C, Nussbaum E, Cooper DM. Plastic bronchitis and the role of bronchoscopy in the acute chest syndrome of sickle cell disease. Chest. 2001;120(2):608-13.

18.  Madsen P, Shah SA, Rubin BK. Plastic bronchitis: new insights and a classifiation scheme. Paediatr Respir Rev. 2005;6(4):292-300.

19.  Manna SS, Shaw J, Tibby SM, Durward A. Treatment of plastic bronchitis in acute chest syndrome of sickle cell disease with intratracheal rhDNase. Arch Dis Child. 2003;88(7):626-7.

20.  Ballas SK, Gupta K, Adams-Graves P. Sickle cell pain: a critical reappraisal. Blood. 2012;120(18):3647-56.

21.  Ruperto N, Ozen S, Pistorio A, Dolezalova P, Brogan P, Cabral DA, et al. EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part I: Overall methodology and clinical characterisation. Ann Rheum Dis. 2010;69(5):790-7.

22.  Almeida A, Roberts I. Bone involvement in sickle cell disease. Br J Haematol. 2005;129(4):482-90.

23.  Anand AJ, Glatt AE. Salmonella osteomyelitis and arthritis in sickle cell disease. Semin Arthritis Rheum. 1994;24(3):211-21.

24.  Hirst C, Owusu-Ofori S. Prophylactic antibiotics for preventing pneumococcal infection in children with sickle cell disease. Cochrane Database Syst Rev. 2012;9:CD003427.

Disclaimers

Conflict Of Interest

The Journal requires that authors disclose any potential conflict of interest that they may have. This is clearly stated in the Journal’s published “Guidelines for Authors”. The Journal follows the Guidelines against Conflict of Interest published in the Uniform Requirements for Manuscripts Submitted to Biomedical Journals (http://www.icmje.org/urm_full.pdf).

Financial Statement

The authors of this article have not been paid. The Journal is financed by subscriptions and advertising. The Journal does not receive money from any other sources. The decision to accept or refuse this article for publication was free from financial considerations and was solely the responsibility of the Editorial Panel and Editor-in-Chief.

Patient Consent statement

All pictures and investigations shown in this article are shown with the patients’ consent. We require Authors to maintain patients’ anonymity and to obtain consent to report investigations and pictures involving human subjects when anonymity may be compromised. The Journal follows the Guidelines of the Uniform Requirements for Manuscripts (http://www.icmje.org/urm_full.pdf). The Journal requires in its Guidelines for Authors a statement from Authors that “the subject gave informed consent”.

Animal & Human Rights

When reporting experiments on human subjects, the Journal requires authors to indicate whether the procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the HelsinkiDeclaration of 1975, as revised in 2008.

About the Clinical Cases Database

T​he Foundation Years Clinical Cases Database is​ a selection of 600 peer-reviewed clinical cases in the field of patient safety and clinical practice, specifically focused on the clinical information needs of junior doctors, based around the Foundation Year Curriculum programme (MMC). The cases have been chosen to align with the Foundation Year Curriculum.

The database is fully searchable, or can be browsed by medical specialty. Abstracts can be read free of charge, however a subscription is required in order to read the complete cases.