Evaluation of Quality of Life and Levels of Participation in Patients with Duchenne Muscular Dystrophy
Abstract
Introduction: The aim of this study was to investigate the quality of life and levels of participation levels in individuals with Duchenne muscular dystrophy (DMD) and assess the impact of preserved upper extremity muscle power and wrist functions on their quality of life and participation levels. The study was performed on 25 volunteers with DMD who were followed at Bakırköy Municipality, Atatürk Spor ve Yaşam Köyü from March to July 2015.
Methods: Quality of life was evaluated using the Short-Form-36 (SF-36), participation levels were assessed using the Craig Handicap Assessment and Rating Technique-Short Form (CHART-SF), upper extremity power was evaluated manual muscle test, and hand and wrist functions were assessed with the Duruöz Hand Index.
Results: SF-36 showed that Physical Health (34.31±5.32) and Mental Health (49.95±10.54) levels were fair to good. CHART-SF revealed that the patients demonstrated moderate to good levels in Physical Independence (61.12±14.22), Cognitive Independence (55.16±29.52), Mobility (64.64±17.87), and Social Integration (81.78±23.11), while the Work dimension was notably low (12.12±19.32). Patients exhibited moderate and low levels of muscle strength in the shoulder, elbow, and hand. Duruöz Hand Index indicated moderate to good hand function. It shows a relationship between shoulder flexion, shoulder extension, and shoulder abduction with the “Physical Health Component” of the SF-36 (p<0.05). It highlights a statistically significant and positive relationship between the “Work” dimension of the CHART-SF and the “Mental Health Component” of the SF-36 (p<0.05).
Conclusion: Patients with DMD revealed fair to good levels of Physical Independence, Cognitive Independence, Mobility, and Social Integration whereas Occupation level was low. It has been determined that impairment in upper extremity use and restriction in social participation affect physical and mental domains of quality of life.
References
- Moser H. Duchenne muscular dystrophy: pathogenetic aspects and genetic prevention. Hum Genet. 66:17–40.
- Villa CR, Czosek RJ, Ahmed H, Khoury PR, Anderson JB, Knilans TK, Jefferies JL, Wong B, Spar DS. Ambulatory Monitoring and Arrhythmic Outcomes in Pediatric and Adolescent Patients With Duchenne Muscular Dystrophy.
- Smith SA, Swaiman KF. Muscular dystrophies. Pediatric Neurology. St Louis, MO: Mosby, 1235-42.
- Eagle KA, Berger PB, Calkins H, Chaitman BR, Ewy GA, Fleischmann KE, Ryan T. ACC/AHA guideline update for perioperative cardiovascular evaluation for noncardiac surgery—executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee to Update the 1996 Guidelines on Perioperative Cardiovascular Evaluation for Noncardiac Surgery. J Am Coll Cardiol. 39:542–553.
- Drousiotou A, Ioannou P, Georgiou T, Mavrikiou E, Christopoulos G, Kyriakides T, Middleton L. Neonatal screening for Duchenne muscular dystrophy: a novel semiquantitative application of the bioluminescence test for creatine kinase in a pilot national program in Cyprus. Genet Test. 2:55–60.
- Davidson ZE, Truby H. A review of nutrition in Duchenne muscular dystrophy. Journal of human nutrition and dietetics. 22:383–393.
- Magliano L, D’angelo MG, Vıta G, Marıka Pane ADELED ’amıco, Balottın U, Angelını C. Psychological and practical difficulties among parents and healthy siblings of children with Duchenne vs. Becker muscular dystrophy: an Italian comparative study. Acta Myologica. 33:136.
- Dubowitz V. Muscle disorders in childhood. Isr J Med Sci. 13:89–92.
- Biggar D. Duchenne Muscular Dystrophy. Pediatr Rev. 27.
- Landfeldt E, Lindgren P, Bell CF, Guglieri M, Straub V, Lochmüller H, Bushby K. Health‐related quality of life in patients with Duchenne muscular dystrophy: a multinational, cross‐sectional study. Dev Med Child Neurol. doi: 10.1111/dmcn.12938
- Hendriksen J, Hendriksen R, Kuijer J, Vroom E. The psychology of Duchenne Muscular Dystrophy. J Hendriksen & Parent Project Production.
- Grootenhuis MA, Boone J, Kooi AJ. Living with muscular dystrophy: health related quality of life consequences for children and adults. Health Qual Life Outcomes Jun. 6:5 31.
- Florence JM, Pandya S, King WM, Robison JD, Baty J, Miller JP, Schierbecker J, Signore LC. Intrarater reliability of manual muscle test (Medical Research Council scale) grades in Duchenne’s muscular dystrophy. Phys Ther [Internet]. 1992 [cited 2023 Nov 3];72:115–126. doi: 10.1093/PTJ/72.2.115 Cited: in: : PMID: 1549632.
- Duruöz MT, Ketenci A. Romatizmal hastalıkların değerlendirilmesinde kullanılan fonksiyonel el göstergeleri. Türkiye Fiziksel Tıp Rehabilitasyon Dergisi. 1.
- Ware JE, Sherbourne CD. The MOS 36-item Short Form Healty Survey, I. Conceptual Framework and item Selection, Med Care,. p. 473–483.
- Richards JS, Bombardier CH, Tate D, Dijkers M, Gordon W, Shewchuk R, DeVivo MJ. Access to the environment and life satisfaction after spinal cord injury. Arch Phys Med Rehabil. 1999;80:1501–1506. doi: 10.1016/S0003-9993(99)90264-2 Cited: in: : PMID: 10569447.
- Hurşit APA, Kayserili E, Hızarcıoğlu M, Gülez P, Tayfun F. Normal Görünümlü Müsküler Distrofili Bir Olgu. Turkiye Klinikleri Journal of Pediatrical Sciences. 1:94–97.
- Alemdarotlu I, Karaduman A, Yilmaz Ö, Topaloʇlu H. Duchenne Musküler Distrofi’de üst ekstremite dinamik egzersizinin solunum fonksiyonu ve yaşam kalitesi üzerine etkisi. Fizyoterapi Rehabilitasyon. 2014;25:78–85. doi: 10.7603/S40680-014-0011-Z
- Jansen M, de Groot IJM, van Alfen N, Geurts ACH. Physical training in boys with Duchenne Muscular Dystrophy: the protocol of the No Use is Disuse study. BMC Pediatr [Internet]. 2010 [cited 2023 Dec 5];10. doi: 10.1186/1471-2431-10-55. Cited: in: PMID: 20691042.
- Mattar FL, Sobreira C. Hand weakness in Duchenne muscular dystrophy and its relation to physical disability. Neuromuscular. :193–198.
- Wei Y. Quality of Life and Health-Related Quality of Life in Children with Duchenne Muscular Dystrophy.
- Bothwell JE, Dooley JM, Gordon KE, MacAuley A, Camfield PR, MacSween J. Duchenne muscular dystrophy--parental perceptions. Clin Pediatr (Phila) [Internet]. 2002 [cited 2023 Jan 19];41:105–109. doi: 10.1177/000992280204100206 Cited: in: : PMID: 11931326.
- Elsenbruch S, Schmid J, Lutz S, Geers B, Schara U. Self-reported quality of life and depressive symptoms in children, adolescents, and adults with Duchenne muscular dystrophy: a cross-sectional survey study. Neuropediatrics. 44:257–264.
- Dijkers M, Yavuzer G, Ergin S. A tale of two countries: environmental on social participitation after spinal cord injury. Spinal Cord. 40:351–362.
- Çakaloz B, Psikiyatri SK-K, 2005 undefined. Duchenne muskuler distrofili çocukların aile işlevlerinin ve annelerinde depresyon ve kaygı düzeylerinin araştırılması. journalagent.com [Internet]. [cited 2023 Jan 19]
Abstract
Giriş: Bu çalışmanın amacı, Duchenne kas distrofisi (DMD) olan bireylerin yaşam kalitesini ve katılım düzeylerini araştırmak ve korunmuş üst ekstremite ve el bileği fonksiyonlarının yaşam kalitesi ve katılım kısıtlamaları üzerindeki etkisini değerlendirmektir. Çalışma, Mart – Temmuz 2015 tarihleri arasında Bakırköy Belediyesi Atatürk Spor ve Yaşam Köyü’nde takip edilen 25 DMD gönüllüsü üzerinde gerçekleştirildi.
Yöntem: Yaşam kalitesi Short-Form-36 (SF-36) kullanılarak değerlendirilirken, katılım düzeyleri Craig Handicap Assessment and Rating Technique-Short Form (CHART-SF) ile değerlendirildi. Üst ekstremite gücü manuel kas testi ile değerlendirildi ve el ve el bileği fonksiyonları Duruöz El İndeksi ile değerlendirildi.
Bulgular: SF-36, Fiziksel Sağlık (34.31±5.32) ve Ruhsal Sağlık (49.95±10.54) seviyelerinin orta düzeyde olduğunu gösterdi. CHART-SF, Hastaların Fiziksel Bağımsızlık (61.12±14.22), Bilişsel Bağımsızlık (55.16±29.52), Hareketlilik (64.64±17.87) ve Sosyal Entegrasyon (81.78±23.11) açısından orta düzeyde olduğunu gösterdi, ancak Çalışma boyutunun belirgin bir şekilde düşük olduğunu (12.12±19.32) ortaya koydu. Hastalar, omuz, dirsek ve elde orta ve düşük düzeylerde kas gücü sergiledi. Duruöz El İndeksi, orta düzeyde el fonksiyonunu gösterdi.
SF-36'nın “Fiziksel Sağlık Bileşeni” ile omuz fleksiyonu, omuz ekstansiyonu ve omuz abduksiyonu arasında ilişki olduğunu göstermektedir (p<0.05). Ayrıca, CHART-SF’nin “Çalışma” boyutu ile SF-36’nın “Ruhsal Sağlık Bileşeni” arasında istatistiksel olarak anlamlı ve pozitif bir ilişki olduğunu vurgulamaktadır (p<0.05).
Sonuç: DMD'li hastalar, Fiziksel Bağımsızlık, Bilişsel Bağımsızlık, Hareketlilik ve Sosyal Entegrasyon konularında orta düzeyden iyiye kadar seviyeler gösterirken, İş seviyesi düşük olarak ortaya çıktı. Üst ekstremite kullanımındaki bozulma ve sosyal katılımdaki kısıtlamaların, yaşam kalitesinin fiziksel ve zihinsel alanlarını etkilediği belirlenmiştir.
References
- Moser H. Duchenne muscular dystrophy: pathogenetic aspects and genetic prevention. Hum Genet. 66:17–40.
- Villa CR, Czosek RJ, Ahmed H, Khoury PR, Anderson JB, Knilans TK, Jefferies JL, Wong B, Spar DS. Ambulatory Monitoring and Arrhythmic Outcomes in Pediatric and Adolescent Patients With Duchenne Muscular Dystrophy.
- Smith SA, Swaiman KF. Muscular dystrophies. Pediatric Neurology. St Louis, MO: Mosby, 1235-42.
- Eagle KA, Berger PB, Calkins H, Chaitman BR, Ewy GA, Fleischmann KE, Ryan T. ACC/AHA guideline update for perioperative cardiovascular evaluation for noncardiac surgery—executive summary: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Committee to Update the 1996 Guidelines on Perioperative Cardiovascular Evaluation for Noncardiac Surgery. J Am Coll Cardiol. 39:542–553.
- Drousiotou A, Ioannou P, Georgiou T, Mavrikiou E, Christopoulos G, Kyriakides T, Middleton L. Neonatal screening for Duchenne muscular dystrophy: a novel semiquantitative application of the bioluminescence test for creatine kinase in a pilot national program in Cyprus. Genet Test. 2:55–60.
- Davidson ZE, Truby H. A review of nutrition in Duchenne muscular dystrophy. Journal of human nutrition and dietetics. 22:383–393.
- Magliano L, D’angelo MG, Vıta G, Marıka Pane ADELED ’amıco, Balottın U, Angelını C. Psychological and practical difficulties among parents and healthy siblings of children with Duchenne vs. Becker muscular dystrophy: an Italian comparative study. Acta Myologica. 33:136.
- Dubowitz V. Muscle disorders in childhood. Isr J Med Sci. 13:89–92.
- Biggar D. Duchenne Muscular Dystrophy. Pediatr Rev. 27.
- Landfeldt E, Lindgren P, Bell CF, Guglieri M, Straub V, Lochmüller H, Bushby K. Health‐related quality of life in patients with Duchenne muscular dystrophy: a multinational, cross‐sectional study. Dev Med Child Neurol. doi: 10.1111/dmcn.12938
- Hendriksen J, Hendriksen R, Kuijer J, Vroom E. The psychology of Duchenne Muscular Dystrophy. J Hendriksen & Parent Project Production.
- Grootenhuis MA, Boone J, Kooi AJ. Living with muscular dystrophy: health related quality of life consequences for children and adults. Health Qual Life Outcomes Jun. 6:5 31.
- Florence JM, Pandya S, King WM, Robison JD, Baty J, Miller JP, Schierbecker J, Signore LC. Intrarater reliability of manual muscle test (Medical Research Council scale) grades in Duchenne’s muscular dystrophy. Phys Ther [Internet]. 1992 [cited 2023 Nov 3];72:115–126. doi: 10.1093/PTJ/72.2.115 Cited: in: : PMID: 1549632.
- Duruöz MT, Ketenci A. Romatizmal hastalıkların değerlendirilmesinde kullanılan fonksiyonel el göstergeleri. Türkiye Fiziksel Tıp Rehabilitasyon Dergisi. 1.
- Ware JE, Sherbourne CD. The MOS 36-item Short Form Healty Survey, I. Conceptual Framework and item Selection, Med Care,. p. 473–483.
- Richards JS, Bombardier CH, Tate D, Dijkers M, Gordon W, Shewchuk R, DeVivo MJ. Access to the environment and life satisfaction after spinal cord injury. Arch Phys Med Rehabil. 1999;80:1501–1506. doi: 10.1016/S0003-9993(99)90264-2 Cited: in: : PMID: 10569447.
- Hurşit APA, Kayserili E, Hızarcıoğlu M, Gülez P, Tayfun F. Normal Görünümlü Müsküler Distrofili Bir Olgu. Turkiye Klinikleri Journal of Pediatrical Sciences. 1:94–97.
- Alemdarotlu I, Karaduman A, Yilmaz Ö, Topaloʇlu H. Duchenne Musküler Distrofi’de üst ekstremite dinamik egzersizinin solunum fonksiyonu ve yaşam kalitesi üzerine etkisi. Fizyoterapi Rehabilitasyon. 2014;25:78–85. doi: 10.7603/S40680-014-0011-Z
- Jansen M, de Groot IJM, van Alfen N, Geurts ACH. Physical training in boys with Duchenne Muscular Dystrophy: the protocol of the No Use is Disuse study. BMC Pediatr [Internet]. 2010 [cited 2023 Dec 5];10. doi: 10.1186/1471-2431-10-55. Cited: in: PMID: 20691042.
- Mattar FL, Sobreira C. Hand weakness in Duchenne muscular dystrophy and its relation to physical disability. Neuromuscular. :193–198.
- Wei Y. Quality of Life and Health-Related Quality of Life in Children with Duchenne Muscular Dystrophy.
- Bothwell JE, Dooley JM, Gordon KE, MacAuley A, Camfield PR, MacSween J. Duchenne muscular dystrophy--parental perceptions. Clin Pediatr (Phila) [Internet]. 2002 [cited 2023 Jan 19];41:105–109. doi: 10.1177/000992280204100206 Cited: in: : PMID: 11931326.
- Elsenbruch S, Schmid J, Lutz S, Geers B, Schara U. Self-reported quality of life and depressive symptoms in children, adolescents, and adults with Duchenne muscular dystrophy: a cross-sectional survey study. Neuropediatrics. 44:257–264.
- Dijkers M, Yavuzer G, Ergin S. A tale of two countries: environmental on social participitation after spinal cord injury. Spinal Cord. 40:351–362.
- Çakaloz B, Psikiyatri SK-K, 2005 undefined. Duchenne muskuler distrofili çocukların aile işlevlerinin ve annelerinde depresyon ve kaygı düzeylerinin araştırılması. journalagent.com [Internet]. [cited 2023 Jan 19]
Details
| Primary Language | Turkish |
|---|---|
| Subjects | Intensive Care |
| Journal Section | Research Articles |
| Authors | |
| Publication Date | December 22, 2023 |
| Submission Date | January 20, 2023 |
| Published in Issue | Year 2023 Volume: 3 Issue: 3 |
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