Unusual Lifespan of Edwards' Syndrome in COVID-19 Era

Filla Reviyani Suryaningrat, Devatri Hudayari, Natasha Amalda Ediwan, Wan Rita Mardhiya, Fiva Aprilia Kadi, Aris Primadi, Tetty Yuniati

Abstract


Edwards' syndrome is caused by an extra chromosome on the 18th chromosome. Patients with this diagnosis have median survival of only 3-14.5 days, with the majority of infants with full trisomy 18 type die before or shortly after they are born. There is only supportive treatment available for Edwards’ syndrome. During the COVID-19 pandemic, providing health services was challenging, leading to less than optimum treatments added by patients’ reluctance to visit healthcare facilities due to the fear of COVID-19 transmission. This case study presented an infant with Edwards’ syndrome with major malformation. The patient had several organ problems, was admitted to the NICU, and continued with home care for monitoring for 6 months. Patient then died at 9 months of age. Maintaining the best quality of life for this patient was very challenging for parents and healthcare providers. Since the management for this type of sydnome is more palliative, good education, information, and psychosocial supports for the parents are needed to prepare them with the worst conditions, which was especially more challenging during the COVID-19 pandemic.

Keywords


Covid 19, full trisomy, survival, trisomy 18

Full Text:

PDF

References


  1. Katasi C. Edwards’ syndrome: A case study. Indian J Cont Nsg Edn. 2019;20:18–21.
  2. Cereda A, Carey JC. The trisomy 18 syndrome. Orphanet J Rare Dis. 2012;7:81. doi:10.1186/1750-1172-7-81
  3. Bruns D. Caring for an infant with trisomy 18: A case study and guidelines. Clinical Nursing Studies. 2013;2(1):30.
  4. Imataka G, Suzumura H, Arisaka O. Clinical features and survival in individuals with trisomy 18: A retrospective one-center study of 44 patients who received intensive care treatments. Mol Med Rep. 2016;13(3):2457–66. doi:10.3892/mmr.2016.4806
  5. Kaulfus ME, Gardiner H, Hashmi SS, Mendez-Figueroa H, Miller VJ, Stevens B, et al. Attitudes of clinicians toward cardiac surgery and trisomy 18. J Genet Couns. 2019;28(3):654–63. doi:10.1002/jgc4.1089
  6. Kementerian Kesehatan RI Direktorat Jenderal Kesehatan Masyarakat Direktorat Kesehatan Keluarga. Pedoman pelayanan antenatal, persalinan, nifas dan bayi baru lahir di era adaptasi kebiasaan baru. Jakarta: Kementerian Kesehatan RI; 2020. p. 2–3.
  7. Arakeri S, Fatima U, Ramkumar KR. A case report of edward syndrome and review of 152 similar cases published in various journals. Sch J App Med Sci. 2014;22(c):749–751. doi: 10.36347/sjams.2014.v02i02.057
  8. Duque JAP, Ferreira CF, Zachia SA, Sanseverino MTV, Gus R, Magalhães JAA. The natural history of pregnancies with prenatal diagnosis of Trisomy 18 or Trisomy 13: Retrospective cases of a 23-year experience in a Brazilian public hospital. Genet Mol Biol. 2019;42(1 suppl 1):286–96. doi:10.1590/1678-4685-GMB-2018-0099
  9. Pallotto I, Lantos JD. Treatment Decisions for Babies with Trisomy 13 and 18. HEC Forum. 2017;29(3):213–22.
  10. López-Ríos V, Grajales-Marín E, Gómez-Zambrano V, Barrios-Arroyave FA. Prolonged survival in Edwards syndrome with congenital heart disease: a case report and literature review. Medwave. 2020;20(8):e8015.
  11. Bruns D, Campbell E. Twenty-two survivors over the age of 1 year with full trisomy 18: presenting and current medical conditions. Am J Med Genet A. 2014;164a(3):610–9.
  12. Balasundaram P, Avulakunta ID. Edwards Syndrome. [Updated 2023 Mar 20]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023. Available from: https://www.ncbi.nlm.nih.gov/books/NBK570597/
  13. López-Ríos V, Grajales-Marín E, Gómez-Zambrano V, Barrios-Arroyave FA. Prolonged survival in Edwards syndrome with congenital heart disease: a case report and literature review. Medwave. 2020;20(8):e8015. doi:10.5867/medwave.2020.08.8015
  14. FBruns D, Springer S. Feeding changes in children with trisomy 18. Topics in Clinical Nutrition. 2013;28:324–34.
  15. Begum R. A Case report on edward syndrome. Clinical Case Reports. 2019;9:1-2.
  16. Felicia FV, Suarca IK. Pelayanan imunisasi dasar pada bayi di bawah usia 12 bulan dan faktor yang memengaruhi di RSUD Wangaya Kota Denpasar selama masa pandemi COVID-19. Sari Pediatri. 2020;22(3):139–45.
  17. Santoli J, Lindley M, DeSilva M, Kharbanda E, Daley M, Galloway L, et al. Effects of the COVID-19 Pandemic on Routine Pediatric Vaccine Ordering and Administration - United States, 2020. MMWR Morb and Mortal Wkly Rep. 2020;15;69(19):591–3. doi:10.15585/mmwr.mm6919e2




DOI: https://doi.org/10.15395/mkb.v56.3230

Article Metrics

Abstract view : 274 times
PDF - 130 times

Creative Commons License
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.


 


Creative Commons License
MKB is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License

 


View My Stats