Nutritionist Resource Pages

  • Patient and Parent Perspectives on the Use of Nusinersen for Spinal Muscular Atrophy

    At the end of 2016, the U.S. Food and Drug Administration (FDA) approved nusinersen for the treatment of spinal muscular atrophy (SMA).1 Because of the high threshold for FDA approval, an abundance of data on nusinersen and its physiological effects on SMA patients has been collected in recent years. However, given […]

  • Sleep Disorders in Spinal Muscular Atrophy

    Those with spinal muscular atrophy (SMA) often suffer from sleep disorders, but the specific reasons for sleep disturbances vary. For instance, some studies on SMA report sleep disturbances resulting from breathing disorders like sleep apneas, whereas others report problems with the architecture of sleep, which can be […]

  • Assistive Devices and Equipment in Spinal Muscular Atrophy: Feeding

    Feeding is often a challenge in people with spinal muscular atrophy (SMA), particularly in those with more severe forms, and feeding difficulties can lead to malnutrition and suboptimal body composition.1,2 Loss of ability to sit is associated with the need for feeding support, which occurs earlier in SMA type 1 than […]

  • Spinal Muscular Atrophy: Awareness and Attitudes

    There is a paucity of information on awareness of and attitudes towards spinal muscular atrophy (SMA) and medical approaches to the disease.1,2 However, research into what is known about SMA has revealed that the public is largely unfamiliar with SMA, and even physicians and other healthcare providers have a limited […]

  • Spinal Muscular Atrophy: Effects on Home, School, and Leisure

    Those with spinal muscular atrophy (SMA) face psychological and social challenges that often hinder quality of life.1 Even those who are able to live in their homes and participate in school, employment, and leisure activities are burdened with challenges related to their illness.2 One of the most frequently cited […]

  • Abnormal Fatty Acid Oxidation in Spinal Muscular Atrophy

    Fatty acids are an important source of cellular energy, and deficiencies in fatty acids and their oxidation can lead to a variety of health issues, including liver dysfunction, cardiomyopathy, skeletal myopathy and hepatic disease.1 Cancer studies have shown that excessive fatty acid oxidation can induce muscle […]

  • Nephrolithiasis (Kidney Stones) in Spinal Muscular Atrophy

    Nephrolithiasis is not a classic or well-documented symptom of spinal muscular atrophy (SMA). Nonetheless, anecdotal reports from physicians, patients, and caregivers suggest that patients with SMA may suffer from kidney stones more frequently than an otherwise healthy population of children/young adults.  Kidney […]

  • Spinal Muscular Atrophy: Chewing and Swallowing Problems

    Patients with Spinal Muscular Atrophy (SMA) types 2 and 3 frequently report weakness of the muscles of mastication and bulbar impairment leading to chewing and swallowing issues.1 Patients with SMA type 1 have such significant bulbar weakness that precludes safe feeding and therefore have early prophylactic […]

  • Spinal Muscular Atrophy: Fluids and Hydration

    Patients with Spinal Muscular Atrophy (SMA) have nutritional needs that vary depending on their ages, the severity of their weakness (non-sitter versus sitter), and the acuity of their situation.1  Chronic Hydration Needs SMA is associated with dysphagia and gastric dysmotility, and the most severe forms of SMA will […]

  • Spinal Muscular Atrophy: Nutrition and Supplementation

    The nutritional management of patients with progressive neuromuscular diseases like Spinal Muscular Atrophy (SMA) is complex and is achieved optimally with an individualized approach from a multidisciplinary care team.1,2 Patients with SMA need nutritional monitoring at least annually with assessment of caloric intake […]