Development and application of a multidisciplinary nursing program for Prader-Willi syndrome with spinal deformity in perioperative period_West China Medical Journal

Authors：

LIU Ying , TONG Bingdu , TIAN Xue ,  CHEN Yaping

Department of Orthopaedics, Peking Union Medical College Hospital, Beijing 100730, P. R. China;

Corresponding author：

CHEN Yaping, Email: 18612672357@139.com

Keywords：

Prader-Willi syndrome; Spinal deformity; Perioperative nursing; Multidisciplinary team

DOI：

10.7507/1002-0179.202108219

Video：

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Objective To develop a multidisciplinary nursing program for Prader-Willi syndrome with spinal deformity and evaluate its effectiveness in clinical practice. Methods In July 2016, a multidisciplinary collaborative team was established before the treatment of children with Prader-Willi syndrome complicated with spinal deformity. For the nursing difficulties in the perioperative period, relevant literature was consulted, and a multidisciplinary collaborative nursing plan was formulated, which included nutrition management and blood glucose control, management of obstructive sleep apnea hypopnea syndrome, behavioral intervention related to mental change, and early identification and management of complications. The developed multidisciplinary collaborative nursing program was applied to three children with Prader-Willi syndrome complicated with spinal deformity in Peking Union Medical College Hospital from July 2016 to October 2018, and their postoperative recovery was evaluated. Results One child had inguinal skin ulceration when admitted to hospital, which was significantly improved after active treatment. The perioperative blood glucose level control of the three children was satisfactory, and there was no postoperative gastrointestinal dysfunction, deep wound infection, respiratory complications, internal fixation failure or other complications, and no asphyxia, fall, loss, or other adverse events. The follow-up compliance after discharge was 100%. Conclusions Multidisciplinary collaboration programs can escort patient’ safety and promote their recovery, improve the professional level of nursing staff, and reflect the nursing value. As a working mode, it can be further popularized and used for reference in the nursing of other difficult diseases.

Citation： LIU Ying, TONG Bingdu, TIAN Xue, CHEN Yaping. Development and application of a multidisciplinary nursing program for Prader-Willi syndrome with spinal deformity in perioperative period. West China Medical Journal, 2021, 36(10): 1382-1386. doi: 10.7507/1002-0179.202108219 Copy

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2.	Emerick JE, Vogt KS. Endocrine manifestations and management of Prader-Willi syndrome. Int J Pediatr Endocrinol, 2013, 2013(1): 14.
3.	Angulo MA, Butler MG, Cataletto ME. Prader-Willi syndrome: a review of clinical, genetic, and endocrine findings. J Endocrinol Invest, 2015, 38(12): 1249-1263.
4.	Nakamura Y, Murakami N, Iida T, et al. The characteristics of scoliosis in Prader-Willi syndrome (PWS): analysis of 58 scoliosis patients with PWS. J Orthop Sci, 2015, 20(1): 17-22.
5.	Nagai T, Obata K, Ogata T, et al. Growth hormone therapy and scoliosis in patients with Prader-Willi syndrome. Am J Med Genet A, 2006, 140(15): 1623-1627.
6.	Alves C, Franco RR. Prader-Willi syndrome: endocrine manifestations and management. Arch Endocrinol Metab, 2020, 64(3): 223-234.
7.	Odent T, Accadbled F, Koureas G, et al. Scoliosis in patients with Prader-Willi Syndrome. Pediatrics, 2008, 122(2): e499-e503.
8.	Oore J, Connell B, Yaszay B, et al. Growth friendly surgery and serial cast correction in the treatment of early-onset scoliosis for patients with Prader-Willi syndrome. J Pediatr Orthop, 2019, 39(8): e597-e601.
9.	Accadbled F, Odent T, Moine A, et al. Complications of scoliosis surgery in Prader-Willi syndrome. Spine (Phila Pa 1976), 2008, 33(4): 394-401.
10.	Nakamura Y, Nagai T, Iida T, et al. Growth hormone supplement treatment reduces the surgical risk for Prader-Willi syndrome patients. Eur Spine J, 2012, 21(Suppl 4): S483-S491.
11.	杜悠, 仉建国. Prader-Willi综合征合并脊柱侧凸的病因、特点及治疗. 中华骨与关节外科杂志, 2020, 13(9): 772-777.
12.	Muscogiuri G, Formoso G, Pugliese G, et al. Prader- Willi syndrome: an uptodate on endocrine and metabolic complications. Rev Endocr Metab Disord, 2019, 20(2): 239-250.
13.	Eiholzer U, Whitman BY. A comprehensive team approach to the management of patients with Prader-Willi syndrome. J Pediatr Endocrinol Metab, 2004, 17(9): 1153-1175.
14.	Khan MJ, Gerasimidis K, Edwards CA, et al. Mechanisms of obesity in Prader-Willi syndrome. Pediatr Obes, 2018, 13(1): 3-13.
15.	Abdilla Y, Andria BM, Calleja-Agius J. Prader-Willi syndrome: background and management. Neonatal Netw, 2017, 36(3): 134-141.
16.	Crino A, Fintini D, Bocchini S, et al. Obesity management in Prader-Willi syndrome: current perspectives. Diabetes Metab Syndr Obes, 2018, 11: 579-593.
17.	Chung MS, Langouet M, Chamberlain SJ, et al. Prader-Willi syndrome: reflections on seminal studies and future therapies. Open Biol, 2020, 10(9): 200195.
18.	中华医学会医学遗传学分会遗传病临床实践指南撰写组, 李川, 谢波波, 等. Prader-Willi综合征的临床实践指南. 中华医学遗传学杂志, 2020, 37(3): 318-323.
19.	Miller JL, Tan M. Dietary management for adolescents with Prader-Willi syndrome. Adolesc Health Med Ther, 2020, 11: 113-118.
20.	Kopel J. Diagnostic and prognostic problems with the Prader-Willi syndrome. Proc (Bayl Univ Med Cent), 2019, 32(1): 167-168.
21.	Anglin K. Prader-Willi syndrome 101: an overview for pediatric nurses. J Pediatr Nurs, 2017, 36: 263-264.

1. Cassidy SB, Schwartz S, Miller JL, et al. Prader-Willi syndrome. Genet Med, 2012, 14(1): 10-26.
2. Emerick JE, Vogt KS. Endocrine manifestations and management of Prader-Willi syndrome. Int J Pediatr Endocrinol, 2013, 2013(1): 14.
3. Angulo MA, Butler MG, Cataletto ME. Prader-Willi syndrome: a review of clinical, genetic, and endocrine findings. J Endocrinol Invest, 2015, 38(12): 1249-1263.
4. Nakamura Y, Murakami N, Iida T, et al. The characteristics of scoliosis in Prader-Willi syndrome (PWS): analysis of 58 scoliosis patients with PWS. J Orthop Sci, 2015, 20(1): 17-22.
5. Nagai T, Obata K, Ogata T, et al. Growth hormone therapy and scoliosis in patients with Prader-Willi syndrome. Am J Med Genet A, 2006, 140(15): 1623-1627.
6. Alves C, Franco RR. Prader-Willi syndrome: endocrine manifestations and management. Arch Endocrinol Metab, 2020, 64(3): 223-234.
7. Odent T, Accadbled F, Koureas G, et al. Scoliosis in patients with Prader-Willi Syndrome. Pediatrics, 2008, 122(2): e499-e503.
8. Oore J, Connell B, Yaszay B, et al. Growth friendly surgery and serial cast correction in the treatment of early-onset scoliosis for patients with Prader-Willi syndrome. J Pediatr Orthop, 2019, 39(8): e597-e601.
9. Accadbled F, Odent T, Moine A, et al. Complications of scoliosis surgery in Prader-Willi syndrome. Spine (Phila Pa 1976), 2008, 33(4): 394-401.
10. Nakamura Y, Nagai T, Iida T, et al. Growth hormone supplement treatment reduces the surgical risk for Prader-Willi syndrome patients. Eur Spine J, 2012, 21(Suppl 4): S483-S491.
11. 杜悠, 仉建国. Prader-Willi综合征合并脊柱侧凸的病因、特点及治疗. 中华骨与关节外科杂志, 2020, 13(9): 772-777.
12. Muscogiuri G, Formoso G, Pugliese G, et al. Prader- Willi syndrome: an uptodate on endocrine and metabolic complications. Rev Endocr Metab Disord, 2019, 20(2): 239-250.
13. Eiholzer U, Whitman BY. A comprehensive team approach to the management of patients with Prader-Willi syndrome. J Pediatr Endocrinol Metab, 2004, 17(9): 1153-1175.
14. Khan MJ, Gerasimidis K, Edwards CA, et al. Mechanisms of obesity in Prader-Willi syndrome. Pediatr Obes, 2018, 13(1): 3-13.
15. Abdilla Y, Andria BM, Calleja-Agius J. Prader-Willi syndrome: background and management. Neonatal Netw, 2017, 36(3): 134-141.
16. Crino A, Fintini D, Bocchini S, et al. Obesity management in Prader-Willi syndrome: current perspectives. Diabetes Metab Syndr Obes, 2018, 11: 579-593.
17. Chung MS, Langouet M, Chamberlain SJ, et al. Prader-Willi syndrome: reflections on seminal studies and future therapies. Open Biol, 2020, 10(9): 200195.
18. 中华医学会医学遗传学分会遗传病临床实践指南撰写组, 李川, 谢波波, 等. Prader-Willi综合征的临床实践指南. 中华医学遗传学杂志, 2020, 37(3): 318-323.
19. Miller JL, Tan M. Dietary management for adolescents with Prader-Willi syndrome. Adolesc Health Med Ther, 2020, 11: 113-118.
20. Kopel J. Diagnostic and prognostic problems with the Prader-Willi syndrome. Proc (Bayl Univ Med Cent), 2019, 32(1): 167-168.
21. Anglin K. Prader-Willi syndrome 101: an overview for pediatric nurses. J Pediatr Nurs, 2017, 36: 263-264.

West China Medical Journal

Development and application of a multidisciplinary nursing program for Prader-Willi syndrome with spinal deformity in perioperative period

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