All scientific information on this website was compiled by Rowan’s father, Dan. He is an assistant professor of Neurosciences at UCSD. All references and conclusions were independently reviewed by faculty at University of California San Diego and the Salk Institute.

All of the research listed below was readily available to cardiologists prior to Rowan’s death in December 2013. Much of it has been readily available since before Rowan was diagnosed in 2012.

It took our son’s death, and our adamant questioning of our own doctors’ claims, for our community of parents to finally have ready access to the resources on this website.  We are told that the Williams Syndrome Association is now updating the information they provide to doctors and parents.  We are grateful for their efforts now.   However, these professionals (particularly WSA’s cardiology adviser Dr. RT Collins) had the ability to inform our community much earlier, in plenty of time to save Rowan’s life, but withheld the information.   Instead, they continued to widely spread misleading information regarding anesthetic risk. Sadly, their current efforts to correct their mistakes have come along too late for Rowan.

” The WSA administration and advisory group seems to lack organization, at least in terms of collecting relevant medical information and disseminating that to parents and health care providers. I am surprised that their leadership was not aware of any new information in terms of anesthetic risk in recent years.  I do agree that they should focus on the larger issue of the sudden passing of children with WS, but that is not an excuse to delay the collection and distribution of all the currently available data to the WS community. The consequences of this delay to Williams Syndrome families are profound” (Asst. Prof. Neurobiology, Salk Institute).

We thought we were lucky.  Rowan’s geneticist (LM Bird) was an author on a paper regarding anesthesia, advertised on the Williams Syndrome Association website.  Rowan’s cardiologist was Nationally renowned, claimed he was experienced with Williams Syndrome, and answered our many questions at length.  They all reassured us and approved anesthesia.  Even so, we went as far as writing on our consent form that we assumed the anesthesiologist was well-versed in the anesthetic risks associated with Williams Syndrome.   She read it, told us she was well-versed, and said she “was not concerned”.

Out of the five doctors we questioned, all told us they were experienced in Williams Syndrome, and none told us anything about the references below, or followed any of the guidelines included.  If just one of Rowan’s doctors had taken the time to read and tell us of the current research, Rowan would not have been killed by anesthesia.  Sadly, each of Rowan’s doctors is still practicing today, without the slightest apology for his/her role in Rowan’s journey to his death.

The following papers discuss estimates of anesthetic risk associated with Williams Syndrome, estimates of coronary artery stenosis among people with WS, and recommendations for hospitals administering anesthesia.

If you have chosen to skip to this page without learning of Rowan’s life, please know that he was a beautiful, happy, and energetic little boy.  Without Rowan, and his death, this website would not have been written.  With Rowan’s kindness in our hearts, Rowan’s mom and dad

Ready to go to story time, 3 days before Rowan died

Ready to go to story time, 3 days before Rowan was killed

Rowan, in the waiting room, the morning he was killed

Rowan, in the waiting room, just before he was killed

References:
1. Comparison of electrocardiographic QTc duration in patients with supravalvar aortic stenosis with versus without Williams syndrome. McCarty HM, Tang X, Swearingen CJ, Collins RT 2nd. Am J Cardiol. 2012 May 15;111(10):1501-4. McCarty and Collins 2012
2. Evaluation of coronary artery abnormalities in Williams syndrome patients using myocardial perfusion scintigraphy and CT angiography. Ergul Y, Nisli K, Kayserili H, Karaman B, Basaran S, Dursun M, Yilmaz E, Ergul N, Unal SN, Dindar A. Cardiol J. 2012;19(3):301-8. Ergul_2012
3. Clinical significance of prolonged QTc interval in Williams syndrome. Collins RT 2nd. Am J Cardiol. 2011 Aug 1;108(3):471-3. Collins_2011
4. Sudden cardiac death under anesthesia in pediatric patient with Williams syndrome: a case report and review of literature. Gupta P, Tobias JD, Goyal S, Miller MD, Melendez E, Noviski N, De Moor MM, Mehta V. Ann Card Anaesth. 2010 Jan-Apr;13(1):44-8. Gupta_2010
5. Congenital supravalvular aortic stenosis and sudden death associated with anesthesia: what’s the mystery? Burch TM, McGowan FX Jr, Kussman BD, Powell AJ, DiNardo JA. Anesth Analg. 2008 Dec;107(6):1848-54. Burch_2008
6. Coronary artery disease and anesthesia-related death in children with Williams syndrome. Horowitz PE, Akhtar S, Wulff JA, Al Fadley F, Al Halees Z. J Cardiothorac Vasc Anesth. 2002 Dec;16(6):739-41. Horowitz_2002
7. Risk of sudden death in the Williams-Beuren syndrome. Wessel A, Gravenhorst V, Buchhorn R, Gosch A, Partsch CJ, Pankau R. Am J Med Genet A. 2004 Jun 15;127A(3):234-7. Wessel_2004
8. Prevention of cardiac arrest due to anesthesia in Williams syndrome. Wiwanitkit V. Ann Card Anaesth. 2010 Sep-Dec;13(3):269. Comment on Gupta_2010
9. Sudden death in Williams syndrome: report of ten cases. Bird LM, Billman GF, Lacro RV, Spicer RL, Jariwala LK, Hoyme HE, Zamora-Salinas R, Morris C, Viskochil D, Frikke MJ, Jones MC. J Pediatr. 1996 Dec;129(6):926-31. Bird_1996
10. Cardiovascular disease in Williams syndrome. Collins RT 2nd. Circulation. 2013 May 28;127(21):2125-34. Collins_2013


** all quotes regarding Rowan’s care and diagnostics within this website, unless otherwise noted, were obtained from Rowan’s medical records, provided by Rady Children’s hospital.  These quotes were taken from the written medical notes of: Rowan’s cardiologist Dr. Raymond Fripp, Rowan’s geneticist Dr. Lynne Bird, Rowan’s pediatric cardiac anesthesiologist Dr. Kathleen Kaya, and Rady’s Chief of Cardiology Dr. John Moore.  Additional notes taken from personal conversations with ophthalmologist Dr. Shira Robbins and neurologist Dr. Doris Trauner, and letters from Rady’s Dr. Irvin Kaufman.

(To our parental knowledge, this list is current as of May 2014.   This should not be considered a complete list of resources. Please be aware that this website will not be updated.  Please refer to your cardiologist for the most recent information).

Copyright © rowansmile 2014. All Rights Reserved. No part of this website or any of its contents may be reproduced, copied, modified or adapted, without the prior written consent of the author.