Duchenne/Becker musküler distrofi tanılı olgularda aile işlevselliği ve davranış problemleri: Kesitsel bir çalışma

Öz

Amaç: Çocuklarda; Duchenne/Becker Musküler Distrofisi (DBMD) duygusal ve davranışsal problemlerle ve aile fonksiyonlarında ki bozulma ile ilişkilidir. Bu çalışma, aile işlevselliğini ve davranış sorunlarını, DBMD olan olgularda araştırmayı amaçlamaktadır.

Yöntemler: Çalışma; Ocak 2019 ve Mart 2019 tarihleri arasında Dokuz Eylül Üniversitesi Tıbbi Genetik ve Çocuk ve Ergen Psikiyatrisi Polikliniği’ne başvuran 28 DBMD tanılı çocuk ve ergen ile 50 sağlıklı kontrol olgusunu içermektedir. Duygulanım Bozuklukları ve Şizofreni Görüşme Çizelgesi-Şimdi ve Yaşam Boyu Şekli-Türkçe (K-SADS-PL) ile araştırmacılar tarafından değerlendirilen katılımcılara; sosyodemografik ve klinik özellikler ile ilgili sorular içeren veri formu, Wechsler Çocuklar İçin Zeka Ölçeği (WISC-R) (sadece DBMD vakaları), Beck Depresyon Ölçeği (BDI), Durum ve Süreklilik Kaygı Envanteri (STAI), Ebeveyn Tutum Değerlendirme Ölçeği (PARI), Çocuk Davranış Kontrol Listesi (CBCL) ve Aile Değerlendirme Ölçeği uygulanmıştır.

Bulgular: DBMD'li çocukların anneleri, Beck Depresyon Envanteri ve Durum-Sürekli Kaygı Envanteri ölçeklerinde, kontrol grubundakilere göre endişeli ve depresif durumlarla ilişkili daha yüksek ancak istatistiksel olarak anlamlı olmayan puanlar saptanmıştır (sırasıyla P=0.888, P=0.584 ve P=0.646). DBMD vakaları, Çocuk Davranışları Kontrol Listesinin çoğunda (aktiviteler, çekingen / depresif, somatik şikayetler, vb.) anlamlı olarak daha yüksek puanlar göstermiştir; bu, ailelerin işleyişini ve hastanın yaşam kalitesini etkileyen birçok problem alanına sahip oldukları anlamına gelmektedir (P<0.001).

Sonuç: Bu çalışma, DBMD'li çocukları olan ebeveynler için DBMD'nin yaşamları ve aile ilişkileri üzerinde olumsuz bir etkisi olduğunu göstermiştir. Bununla birlikte, daha güçlü sonuçlara ulaşmak için daha büyük örneklem büyüklüğünde daha fazla çalışma yapılması gerekmektedir.

Anahtar Kelimeler

• Kas distrofisi,Çocukluk çağı davranış problemleri,Aile işlevselliği

Family functioning and child behavioral problems with Duchenne/Becker muscular dystrophy: A cross-sectional study

Öz

Aim: Duchenne/Becker muscular dystrophy (DBMD) in children is associated with emotional and behavioral problems and impairment at family functioning. The current study aimed to explicate family functioning and child behavioral problems with DBMD.

Methods: The study involved 28 child and adolescents with DBMD attending Dokuz Eylul University Medical Genetics and Child and Adolescent Psychiatry Outpatient Clinic from January 2019 to March 2019 and comprised 50 healthy control subjects. The participants who were evaluated with Kiddie-Sads-Present and Lifetime (K-SADS-PL) by blinded professionals completed a data form containing questions regarding sociodemographic and clinical features, Wechsler Intelligence Scale for Children-Revised (WISC-R) (for only DBMD cases), the Beck Depression Scale (BDS), State-Trait Anxiety Inventory (STAI), Parenteral Attitude Research Instrument (PARI), the Child Behavior Checklist (CBCL) and Family Assessment Device. 

Results: Mothers of the children with DBMD demonstrated higher scores in Beck Depression Inventory and State-Trait Anxiety Inventory scales, which is associated with anxious and depressive states as compared with those from the control group but not statistically significant (P=0.888, P=0.584 and P=0.646, respectively). DBMD cases demonstrated significantly higher scores in most of the Child Behavior Checklist (activities, withdrawn/depressed, somatic complaints etc.), meaning that they have many problem areas affecting family functioning and the quality of life of the patient (P<0.001).

Conclusion: This study demonstrated that, for parents who have children with DBMD, DBMD had a negative effect on their lives, and their family relationships. However, further studies with larger sample sizes are required to reach stronger conclusions.

Anahtar Kelimeler

• Muscular dystrophy,Child behavioral problems,Family functioning

Kaynakça

1. 1. Wicki J, Seto JS, Chamberlain JS. Duchenne Muscular Dystrophy. In Stanley Maloy Kelly Hughes, editors. Brenner's Encyclopedia of Genetics. WA USA: Elsevier, 2013; p. 421-4.
2. 2. Sarnat HB. Muscular dystrophies. In Kliegman RM, Stanton BF, Schor NF, St. Geme III JW, Behrman RE, editors. Nelson Textbook of Pediatrics. 19th ed. Philadelphia: Sounders; 2011; p. 2119-23.
3. 3. Kenneson A, Bobo JK. The effect of caregiving on women in families with Duchenne/Becker muscular dystrophy. Health Soc Care Community. 2010;18(5):520-8.
4. 4. Nereo NE, Fee RJ, Hinton VJ. Parental stress in mothers of boys with Duchenne muscular dystrophy. J Pediatr Psychol. 2003;28(7):473-84.
5. 5. Abi Daoud MS, Dooley JM, Gordon KE. Depression in parents of children with Duchenne muscular dystrophy. Pediatr Neurol. 2004;31(1):16-9.
6. 6. Fritts SL. The impact of chronic illness on the family, the educators, and the community: an ethnographic research study [dissertation). United States California: California State University, Fresno and University of California, Davis; 2004.
7. 7. Cieurzo CE. Family environment, parental coping and distress, and socioeconomic status as predictors of psychological distress in chronically ill children [dissertation]. United States-New York: Fordham University; 2002.
8. 8. Schaefer ES, Bell RQ. Development of a parental attitude research instrument. Child Dev. 1958;29(3):339-61.

9. 9. Küçük S. The validity of the Turkish form of the PARI subscales II, III, IV [thesis]. Istanbul (Turkish): Bogaziçi University, Faculty of Medicine; 1987.
10. 10. Epstein NB, Baldwin LM, Bishop DS. The McMaster Family Assessment Device. Journal of Marital and Family Therapy. 1983;9(2):171-80.
11. 11. Bulut I. Handbook of Family Assessment Device (FAD); Ankara: Özgüzelis Press; 1990.
12. 12. Beck AT, Steer RA, Ball R, Ranieri W. Comparison of Beck Depression Inventories -IA and -II in psychiatric outpatients. J Pers Assess. 1996;67(3):588-97.
13. 13. Spielberger CD, Gorsuch RL, Lushene R, Vagg PR, Jacobs GA. Manual for the State-Trait Anxiety Inventory. Palo Alto, CA: Consulting Psychologists Press, 1983.
14. 14. Öner N, LeCompte A. Manual for the State-Trait Anxiety Inventory, Second Edition, Boğaziçi University Press, İstanbul, 1985;p.333.
15. 15. Erol N, Şimşek Z. Mental Health of Turkish Children: Behavioral and Emotional Problems Reported by Parents, Teachers and Adolescents. In N. Singh, J P Leung, A N Singh, editors. Elsevier, 2000;223-247.
16. 16. Erol N, Arslan BL, Akçakın M. The adaptation and Standardization of the Child Behavior Checklist among 6-18 Year-Old Turkish Children. In J Sergeant (ed.), Eunethydis: European Approaches to Hyperkinetic Disorder. Zurich: Fotoratar, 1995;97–113.
17. 17. Gerdes AC, Hoza B, Arnold LE, Pelham WE, Swanson JM, Wigal T, et al. Maternal depressive symptomatology and parenting behavior: exploration of possible mediators. J Abnorm Child Psychol. 2007;35(5):705-14.
18. 18. Johnson JG, Cohen P, Kasen S, Brook JS. Maternal psychiatric disorders, parenting, and maternal behavior in the home during the child rearing years. Journal of Child and Family Studies. 2006;15(1):96-113.
19. 19. Sienko S, Buckon C, Fowler E, Bagley A, Staudt L, Sison-Williamson M, et al. Prednisone and Deflazacort in Duchenne Muscular Dystrophy: Do They Play a Different Role in Child Behavior and Perceived Quality of Life? PLoS Curr. 2016;8.
20. 20. Landfeldt E, Lindgren P, Bell CF, Guglieri M, Straub V, Lochmüller H, et al. Quantifying the burden of caregiving in Duchenne muscular dystrophy. J Neurol. 2016;263(5):906-15.
21. 21. Peay HL, Hollin IL, Bridges JF. Prioritizing Parental Worry Associated with Duchenne Muscular Dystrophy Using Best-Worst Scaling. J Genet Couns. 2016;25(2):305-13.
22. 22. Van Oers HA, Haverman L, Limperg PF, van Dijk-Lokkart EM, Maurice-Stam H, Grootenhuis MA. Anxiety and depression in mothers and fathers of a chronically ill child. Matern Child Health J. 2014; 18(8):1993-2002.
23. 23. Pinquart M. Psychische Gesundheit von chronisch körperlich kranken Kindern und ihren Eltern – Ergebnisse von Metaanalysen. Praxis Der Kinderpsychologie Und Kinderpsychiatrie. 2017;66(9):656-71.
24. 24. Colombo P, Nobile M, Tesei A, Tesei A, Civati F, Gandossini S, Mani E, et al. Assessing mental health in boys with Duchenne muscular dystrophy: Emotional, behavioral and neurodevelopmental profile in an Italian clinical sample. Eur J Paediatr Neurol. 2017; 21(4):639-47.
25. 25. Darke J, Bushby K, Le Couteur A, McConachie H. Survey of behaviour problems in children with neuromuscular diseases. Eur J Paediatr Neurol. 2006;10(3):129-34.