Spinal Musküler Atrofi Tip 2 ve Tip 3 Hastalarında Ağrı

Yıl 2025, Cilt: 47 Sayı: 1, 16 - 21, 17.01.2025

Arife Derda Yücel Şen , Özlem Uğur , Coşkun Yarar , Kursat Bora Carman

https://doi.org/10.20515/otd.1486835

Öz

Spinal musküler atrofi (SMA), alfa motor nöronların dejenerasyonu ile karakterize otozomal resesif geçişli bir nöromusküler hastalıktır. Nöronal olmayan sistemleri ve yaşam kalitesini etkileyen multisistemik bir hastalıktır. Spinal musküler atrofili çocuklarda ağrının prevalansını ve özelliklerini araştırmayı amaçladık. Bu tek merkezli çalışmada, SMA tip 2 ve tip 3 tanısı alan toplam 13 hastaya ebeveynler eşliğinde görsel analog ölçekler kullanılarak kronik ağrının varlığı, ağrı sıklığı, süresi, yeri, şiddeti, ağrı nedenleri ve baş etme yöntemlerinin sorgulandığı bir anket dolduruldu. Çalışma 13 hasta ile yürütüldü ve tüm hastalar kronik ağrı yaşadıklarını bildirdi. Tip 3 hastaları, tip 2 hastalarına göre her ay birkaç kez olmak üzere daha sık ağrı yaşadılar. "Dakika", "hafif" ve "aralıklı" terimleri her iki grupta da ağrının uzunluğunu, yoğunluğunu ve seyrini tanımlamak için kullanıldı. Vizüel Analog Skala'ya göre ortalama ağrı şiddeti tip 2'de 35.526.3 mm, tip 3'te ise 25.110.2 mm idi. Ağrının lokalizasyonu çoğunlukla sırt ve alt ekstremitelerde yoğunlaştı. Ağrının en sık nedenleri fizik tedavi sırasında yapılan esneme egzersizleri ve duruş bozukluklarıydı. Ağrıyla başa çıkmanın en yaygın yöntemleri dikkat dağıtma stratejisi ve masajdı. SMA'lı çocuklarda ağrı sık görülen bir sorundur. Ağrının yönetimi SMA hastalarının yaşam kalitesini artırabilir. Bu çocukların tedavisinde multidisipliner bir yaklaşım göz önünde bulundurulmalıdır.

Anahtar Kelimeler

Spinal musküler atrofi, ağrı, çocuk

Pain in Spinal Muscular Atrophy Type 2 and Type 3 Patients

Öz

Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease characterized by degeneration of alpha motor neurons. It is a multisystemic disease affecting non-neuronal systems and quality of life. We aimed to investigate the prevalence and characteristics of pain in children with spinal muscular atrophy. In this single-center study, by using visual analog scales, 13 patients diagnosed with SMA type 2 and type 3 accompanied by their parents filled out a questionnaire involving questions about the presence of chronic pain, pain frequency, duration, location, and intensity, causes of pain, and coping methods. All patients reported that they experienced chronic pain. Patients with type 3 experienced pain more frequently than those with type 2—multiple times each month. The terms "minutes," "mild," and "intermittent" were most commonly used to describe the length, intensity, and course of the pain in both groups. The mean pain intensity according to Visual Analogue Scale were 35.526.3 mm in type 2 and 25.110.2 mm in type 3. The localization of pain was primarily concentrated in the back and lower extremities. The most common causes of pain were stretching exercises during physical therapy and posture disorder. The most common methods of coping with pain were distraction strategy and massage. Pain is a common problem in children with SMA. Management of the pain might increase the life quality of SMA patients. A multidisciplinary approach must be considered in the treatment of these children.

Anahtar Kelimeler

Spinal muscular atrophy, pain, child

Kaynakça

• 1. Cancès C, Richelme C, Barnerias C, Espil C. Clinical features of spinal muscular atrophy (SMA) type 2. Arch Pediatr. 2020;27:7S18-7S22.
• 2. Bonanno S, Zanin R, Bello L, et al. Quality of life assessment in adult spinal muscular atrophy patients treated with nusinersen. J Neurol. 2022;269:3264-3275.
• 3. Merskey H, Bogduk N, International Association for the Studyof Pain. Classification of chronic pain:descriptions of chronic pain syndromes and definitions of pain terms. Seattle: IASP Press; 1994.
• 4. Heller GZ, Manuguerra M, Chow R. How to analyze the Visual Analogue Scale: Myths, truths and clinical relevance. Scand J Pain. 2016;13:67-75.
• 5. Engel JM, Kartin D, Carter GT, Jensen MP, Jaffe KM. Pain in youths with neuromuscular disease. Am J Hosp Palliat Care. 2009;26:405-412.
• 6. Uchio Y, Kajima K, Suzuki H, Nakamura K, Saito M, Ikai T. Pain in Spinal Muscular Atrophy: A Questionnaire Study. Phys Ther Res. 2022;25:150-155.
• 7. Guy-Coichard C, Nguyen DT, Delorme T, Boureau F. Pain in hereditary neuromuscular disorders and myasthenia gravis: a national survey of frequency, characteristics, and impact. J Pain Symptom Manage. 2008;35:40-50.
• 8. Padua L, Aprile I, Frusciante R, et al. Quality of life and pain in patients with facioscapulohumeral muscular dystrophy. Muscle Nerve. 2009;40:200-205.
• 9. Lager C, Kroksmark AK. Pain in adolescents with spinal muscular atrophy and Duchenne and Becker muscular dystrophy. Eur J Paediatr Neurol. 2015;19:537-546.
• 10. Sluka KA, O'Donnell JM, Danielson J, Rasmussen LA. Regular physical activity prevents development of chronic pain and activation of central neurons. J Appl Physiol (1985). 2013;114:725-733.
• 11. Schillings ML, Kalkman JS, Janssen HM, van Engelen BG, Bleijenberg G, Zwarts MJ. Experienced and physiological fatigue in neuromuscular disorders. Clin Neurophysiol. 2007;118:292-300.
• 12. Miró J, de la Vega R, Gertz KJ, Thong ISK, Jensen MP, Engel JM. Do Commonly Used Measures of Pain Intensity Only Reflect Pain Intensity in Youths With Bothersome Pain and a Physical Disability?. Front Pediatr. 2019;7:229.
• 13. Hoffman AJ, Jensen MP, Abresch RT, Carter GT. Chronic pain in persons with neuromuscular disease. Phys Med Rehabil Clin N Am. 2005;16:1099-xii.
• 14. Hunt A, Carter B, Abbott J, Parker A, Spinty S, deGoede C. Pain experience, expression and coping in boys and young men with Duchenne Muscular Dystrophy - A pilot study using mixed methods. Eur J Paediatr Neurol. 2016;20:630-638.
• 15. Vu-Han TL, Reisener MJ, Putzier M, Pumberger M. Skoliose bei spinaler Muskelatrophie [Scoliosis in spinal muscular atrophy]. Orthopade. 2021;50:657-663.
• 16. Hanna RB, Nahm N, Bent MA, et al. Hip Pain in Nonambulatory Children with Type-I or II Spinal Muscular Atrophy. JB JS Open Access. 2022;7:e22.00011.
• 17. Delgado A, Ok SM, Ho D, Lynd T, Cheon K. Evaluation of children's pain expression and behavior using audio visual distraction. Clin Exp Dent Res. 2021;7:795-802. 18. Flynn DM. Chronic Musculoskeletal Pain: Nonpharmacologic, Noninvasive Treatments. Am Fam Physician. 2020;102:465-477.