Hostname: page-component-cd9895bd7-gvvz8 Total loading time: 0 Render date: 2024-12-18T15:27:19.907Z Has data issue: false hasContentIssue false
  • English
  • Français

Mixed method study of quality of life for children with trisomy 18 and 13 after cardiac surgery

Published online by Cambridge University Press:  22 January 2020

Meaghann S. Weaver Open the ORCID record for Meaghann S. Weaver [Opens in a new window] ,
Nicole Birge ,
Howard Hsu ,
Christine Woell ,
Jacob E. Robinson ,
Christopher Wichman  and
James Hammel
Meaghann S. Weaver*
Affiliation:
Department of Pediatrics, Children’s Hospital and Medical Center, Omaha, NE, USA Department of Pediatrics, University of Nebraska Medical Center, Omaha, NE, USA
Nicole Birge
Affiliation:
Department of Pediatrics, Children’s Hospital and Medical Center, Omaha, NE, USA Department of Pediatrics, University of Nebraska Medical Center, Omaha, NE, USA
Howard Hsu
Affiliation:
Department of Pediatrics, Children’s Hospital and Medical Center, Omaha, NE, USA Department of Pediatrics, University of Nebraska Medical Center, Omaha, NE, USA
Christine Woell
Affiliation:
Department of Pediatrics, Children’s Hospital and Medical Center, Omaha, NE, USA Department of Pediatrics, University of Nebraska Medical Center, Omaha, NE, USA
Jacob E. Robinson
Affiliation:
Department of Pediatrics, Children’s Hospital and Medical Center, Omaha, NE, USA
Christopher Wichman
Affiliation:
Department of Pediatrics, University of Nebraska Medical Center, Omaha, NE, USA
James Hammel
Affiliation:
Department of Pediatrics, Children’s Hospital and Medical Center, Omaha, NE, USA Department of Pediatrics, University of Nebraska Medical Center, Omaha, NE, USA
*
Author for correspondence: M. S. Weaver, Division of Pediatric Palliative Care, Children’s Hospital and Medical Center, Omaha, NE68114, USA. Tel: +1 402 955 5432; E-mail: [email protected]
Get access Rights & Permissions [Opens in a new window]

Abstract

Background and Objectives:

Cardiac surgical interventions for children with trisomy 18 and trisomy 13 remain controversial, despite growing evidence that definitive cardiac repair prolongs survival. Understanding quality of life for survivors and their families therefore becomes crucial. Study objective was to generate a descriptive summary of parental perspectives on quality of life, family impact, functional status, and hopes for children with trisomy 18 and trisomy 13 who have undergone heart surgery.

Methods:

A concurrent mixed method approach utilising PedsQL™ 4.0 Generic Core Parent Report for Toddlers or the PedsQL™ Infant Scale, PedsQL™ 2.0 Family Impact Module, Functional Status Scale, quality of life visual analogue scale, and narrative responses for 10 children whose families travelled out of state to access cardiac surgery denied to them in their home state due to genetic diagnoses.

Results:

Parents rated their child’s quality of life as 80/100, and their own quality of life as 78/100 using validated scales. Functional status was rated 11 by parents and 11.6 by providers (correlation 0.89). On quality of life visual analogue scale, all parents rated their child’s quality of life as “high” with mean response 92.7/100. Parental hopes were informed by realistic perspective on prognosis while striving to ensure their children had access to reaching their full potential. Qualitative analysis revealed a profound sense of the child’s relationality and valued life meaning.

Conclusion:

Understanding parental motivations and perceptions on the child’s quality of life has potential to inform care teams in considering cardiac interventions for children with trisomy 18 and trisomy 13.

Keywords

Trisomy 13trisomy 18quality of lifecardiac surgeryfamily impact
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 2 , February 2020 , pp. 231 - 237
Copyright
© Cambridge University Press 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

McGraw, MP, Perlman, JM. Attitudes of neonatologists toward delivery room management of confirmed trisomy 18: potential factors influencing a changing dynamic. Pediatrics 2008; 121: 1106–1110.10.1542/peds.2007-1869CrossRefGoogle ScholarPubMed
Adams, DM, Winslade, WJ. Consensus, clinical decision making, and unsettled cases. J Clin Ethics 2011; 22: 310–327.Google ScholarPubMed
Guon, J, Wilfond, BS, Farlow, B, et al.Our children are not a diagnosis: the experience of parents who continue their pregnancy after a prenatal diagnosis of trisomy 13 or 18. Am J Med Genet A 2014; 164A: 308–318.CrossRefGoogle ScholarPubMed
Janvier, A, Okah, F, Farlow, B, et al.An infant with trisomy 18 and a ventricular septal defect. Pediatrics 2011; 127: 754–759.CrossRefGoogle Scholar
Boss, RD, Holmes, KW, Althaus, J, et al.Trisomy 18 and complex congenital heart disease: seeking the threshold benefit. Pediatrics 2013; 132: 161–165.CrossRefGoogle ScholarPubMed
Cooper, DS, Riggs, KW, Zafar, F, et al.Cardiac surgery in patients with trisomy 13 and 18: an analysis of the society of thoracic surgeons congenital heart surgery database. J Am Heart Assoc 2019; 8: E012349.CrossRefGoogle Scholar
Pallotto, I, Lantos, JD. Treatment decisions for babies with trisomy 13 and 18. HEC Forum 2017; 29: 213–222.10.1007/s10730-017-9319-2CrossRefGoogle ScholarPubMed
Lantos, JD.Trisomy 13 and 18--treatment decisions in a stable gray zone. JAMA 2016; 316: 396–398.CrossRefGoogle Scholar
Graham, EM.Infants with trisomy 18 and complex congenital heart defects should not undergo open heart surgery. J Law Med Ethics 2016; 44: 286–291.10.1177/1073110516654122CrossRefGoogle Scholar
Muneuchi, J, Yamamoto, J, Takahashi, Y, et al.Outcomes of cardiac surgery in trisomy 18 patients. Cardiol Young 2011: 21: 209–215.10.1017/S1047951110001848CrossRefGoogle ScholarPubMed
Costello, JP, Weiderhold, A, Louis, C, et al.A contemporary, single-institutional experience of surgical versus expectant management of congenital heart disease in trisomy 13 and 18 patients. Pediatr Cardiol 2015; 36: 987–992.10.1007/s00246-015-1109-5CrossRefGoogle ScholarPubMed
Kobayashi, J, Kaneko, Y, Yamamoto, Y, et al.Radical surgery for a ventricular septal defect associated with trisomy 18. Gen Thorac Cardiovasc Surg 2010; 58; 223–227.CrossRefGoogle ScholarPubMed
Maeda, J, Yamagishi, H, Furutani, Y, et al.The impact of cardiac surgery in patients with trisomy 18 and trisomy 13 in Japan. Am J Med Genet A 2011; 155A: 2641–2646.10.1002/ajmg.a.34285CrossRefGoogle ScholarPubMed
Peterson, R, Calamur, N, Fiore, A, et al.Factors influencing outcomes after cardiac intervention in infants with trisomy 13 and 18. Pediatr Cardiol 2018; 39: 140–147.10.1007/s00246-017-1738-yCrossRefGoogle ScholarPubMed
Baty, BJ, Blackburn, BL, Carey, JC. Natural history of trisomy 18 and trisomy 13: I. Growth, physical assessment, medical histories, survival, and recurrence risk. Am J Med Genet 1994; 49: 175–188.10.1002/ajmg.1320490204CrossRefGoogle ScholarPubMed
Graham, EM, Bradley, SM, Shirali, GS, et al.Effectiveness of cardiac surgery in trisomies 13 and 18 (from the pediatric cardiac care consortium). Am J Cardiol 2004; 93: 801–803.10.1016/j.amjcard.2003.12.012CrossRefGoogle Scholar
Musewe, NN, Alexander, DJ, Teshima, I, et al.Echocardiographic evaluation of the spectrum of cardiac anomalies associated with trisomy 13 and trisomy 18. J Am College Cardiol 1990; 15: 673–677.CrossRefGoogle ScholarPubMed
Matsuoka, R, Misugi, K, Goto, A, et al.Congenital heart anomalies in the trisomy 18 syndrome, with reference to congenital polyvalvular disease. Am J Med Genet 1983; 14: 657–668.10.1002/ajmg.1320140409CrossRefGoogle ScholarPubMed
Nelson, KE, Rosella, LC, Mahant, S, et al.Survival and surgical interventions for children with trisomy 13 and 18. JAMA 2016; 316: 420–428.CrossRefGoogle ScholarPubMed
Embleton, ND, Wyllie, JP, Wright, MJ, et al.Natural history of trisomy 18. Arch Dis Child Fetal Neonatal Ed 1996; 75: F38–41.10.1136/fn.75.1.F38CrossRefGoogle ScholarPubMed
Meyer, RE, Liu, G, Gilboa, SM, et al.Survival of children with trisomy 13 and trisomy 18: a multi-state population-based study. Am J Med Genet A 2016; 170A: 825–837.CrossRefGoogle ScholarPubMed
Kosiv, KA, Gossett, JM, Bai, S, et al.Congenital heart surgery on in-hospital mortality in trisomy 13 and 18. Pediatrics 2017; 140.10.1542/peds.2017-0772CrossRefGoogle ScholarPubMed
Varni, JW, Limbers, CA, Neighbors, K, et al.The PedsQL™ Infant Scales: feasibility, internal consistency reliability, and validity in healthy and ill infants. Qual Life Res 2011; 20: 45–55.10.1007/s11136-010-9730-5CrossRefGoogle ScholarPubMed
Desai, AD, Zhou, C, Stanford, S, et al.Validity and responsiveness of the pediatric quality of life inventory (PedsQL) 4.0 generic core scales in the pediatric inpatient setting. JAMA Pediatr 2014; 168: 1114–1121.10.1001/jamapediatrics.2014.1600CrossRefGoogle ScholarPubMed
Raj, M, Sudhakar, A, Roy, R, et al.Health-related quality of life in infants and toddlers with congenital heart disease: a cross-sectional survey from south India. Arch Dis Child 2018: 103: 170–175.10.1136/archdischild-2017-313165CrossRefGoogle ScholarPubMed
Tahirovic, E, Begic, H, Tahirovic, H, et al.Quality of life in children after cardiac surgery for congenital heart disease. Coll Antropol 2011; 35: 1285–1290.Google ScholarPubMed
Berkes, A, Pataki, I, Kiss, M, et al.Measuring health-related quality of life in Hungarian children with heart disease: psychometric properties of the Hungarian version of the pediatric quality of life inventory 4.0 generic core scales and the cardiac module. Health Qual Life Outcomes 2010; 8: 14.CrossRefGoogle ScholarPubMed
Uzark, K, Jones, K, Slusher, J, et al.Quality of life in children with heart disease as perceived by children and parents. Pediatrics 2008; 121: e1060–1067.CrossRefGoogle ScholarPubMed
Varni, JW, Sherman, SA, Burwinkle, TM, et al.The PedsQL™ family impact module: preliminary reliability and validity. Health Qual Life Outcomes 2004; 2: 55.CrossRefGoogle Scholar
Pollack, MM, Holubkov, R, Glass, P, et al.Functional status scale: new pediatric outcome measure. Pediatrics 2009; 124: e18–28.CrossRefGoogle ScholarPubMed
Krippendorff, K.Content Analysis: An Introduction to its Methodology. Thousand Oaks: SAGE Publications; 2013.Google Scholar
Bland, JM, Altman, DG. Statistical methods for assessing agreement between two methods of clinical measurement. Lancet 1986; 1: 307–310.CrossRefGoogle ScholarPubMed
Carkeet, A.Exact parametric confidence intervals for bland-altman limits of agreement. Optom Vis Sci 2015; 92: e71–80.10.1097/OPX.0000000000000513CrossRefGoogle ScholarPubMed
Davisson, NA, Clark, JB, Chin, TK, et al.Trisomy 18 and congenital heart disease: single-center review of outcomes and parental perspectives. World J Pediatr Congenit Heart Surg 2018; 9: 550–556.CrossRefGoogle ScholarPubMed
Kusztrich, A, Huseman, D, Garten, L, et al.Survival, medical care and quality of life in children with trisomy 13 and 18. Klin Padiatr 2016; 228: 240–244.Google ScholarPubMed
Champagne, CR, Lewis, M, Gilchrist, DM. Should we mend their broken hearts? The history of cardiac repairs in children with Down syndrome. Pediatrics 2014; 134: 1048–1050.10.1542/peds.2014-1739CrossRefGoogle ScholarPubMed
Haug, S, Goldstein, M, Cummins, D, et al.Using patient-centered care after a prenatal diagnosis of trisomy 18 or trisomy 13: a review. JAMA Pediatr 2017; 171: 382–387.CrossRefGoogle ScholarPubMed
What quality of life? The WHOQOL Group. World health organization quality of life assessment. World Health Forum 1996; 17: 354–356.Google Scholar