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 in SMA type 2.3
The specific challenges that SMA patients face with respect to feeding include difficulty getting food to the mouth and difficulty chewing, both of which were reported in 20.4 percent of SMA type 2 and SMA type 3 patients in one study4 and reported at slightly higher rates when only SMA type 2 patients were considered.5 However, choking may be the most frequently reported challenge, with research showing 30.6 percent of SMA type 2 and SMA type 3 patients struggling with choking when feeding.4Â
Swallowing Challenges in SMA
The same study found that 100 percent of those with SMA type 2 who had lost their ability to sit struggled to eat and swallow.4 The nonoral feeding that SMA patients undergo can contribute to swallowing problems, as the relevant muscles weaken.6 Specifically, jaw movement restriction, stiffness of the neck, and weakness in the oropharyngeal and laryngeal muscles appear to contribute significantly to the loss of swallowing function.Â
These specific difficulties increase risk for aspiration pneumonia and for being underweight.5,7,8 Each child with SMA has a unique set of feeding and nutritional challenges and therefore needs personalized management to ensure that they receive the nutrition they need for normal growth and development.2Â
One study into the details of the malnutrition that commonly occurs in SMA found that energy, protein, and vitamin D intake was inadequate in the majority of the SMA patients studied.8 The majority of caretakers of SMA type 1 patients who were surveyed said that they provide patients with elemental or semi-elemental formula to try to ensure better nutrition but many also reported formula intolerance.12Â
According to experts in this field, more research is urgently needed to inform this type of personalized care and to optimize outcomes for SMA patients. Researchers have therefore called for comprehensive assessments of nutrition to facilitate the development of standards and guide individualized nutritional care for SMA patients.Â
Equipment to Aid Swallowing
Regardless of their specific nutritional needs, SMA patients often need assistance with the feeding process and particularly to help overcome their difficulties with swallowing. While exercises are sometimes recommended to help with swallowing functions,6 orthotic devices may be a more effective way to improve SMA patients’ abilities to feed themselves and to swallow more safely and efficiently.9Â
Gastronomy tubes are other devices that can be used to aid in feeding, but there is no consensus on when SMA patients should have these tubes placed, as the appropriateness of this type of intervention depends critically on a number of factors that are specific to each patient.10 These tubes do appear, though, to be the best option for feeding when a patient has reached unsafe levels of caloric intake.Â
When gastronomy tubes are placed, there are options for how to technically achieve the placement. The tube can be inserted percutaneously with the guidance of an endoscope or can be placed with open or laparoscopic surgical approaches.10 The laparoscopic strategy is associated with the best chances for quick extubation following surgery.11 While percutaneous methods can sometimes be performed with local anesthesia, gastronomy tube placement usually involves general anesthesia.10 As the feeding difficulties and nutritional needs of SMA patients are further clarified, the devices and equipment to help them are likely to evolve and new interventions may be developed.
References
1. Martinez EE, Quinn N, Arouchon K, et al. Comprehensive nutritional and metabolic assessment in patients with spinal muscular atrophy: Opportunity for an individualized approach. Neuromuscul Disord. 2018;28(6):512-519. doi:10.1016/j.nmd.2018.03.009
2. Moore GE, Lindenmayer AW, McConchie GA, Ryan MM, Davidson ZE. Describing nutrition in spinal muscular atrophy: A systematic review. Neuromuscul Disord. 2016;26(7):395-404. doi:10.1016/j.nmd.2016.05.005
3. Finkel RS, McDermott MP, Kaufmann P, et al. Observational study of spinal muscular atrophy type I and implications for clinical trials. Neurology. 2014;83(9):810-817. doi:10.1212/WNL.0000000000000741
4. Chen Y-S, Shih H-H, Chen T-H, Kuo C-H, Jong Y-J. Prevalence and risk factors for feeding and swallowing difficulties in spinal muscular atrophy types II and III. J Pediatr. 2012;160(3):447-451.e1. doi:10.1016/j.jpeds.2011.08.016
5. Messina S, Pane M, De Rose P, et al. Feeding problems and malnutrition in spinal muscular atrophy type II. Neuromuscul Disord. 2008;18(5):389-393. doi:10.1016/j.nmd.2008.02.008
6. Cha T-H, Oh D-W, Shim J-H. Noninvasive treatment strategy for swallowing problems related to prolonged nonoral feeding in spinal muscular atrophy type II. Dysphagia. 2010;25(3):261-264. doi:10.1007/s00455-009-9269-1
7. Sproule DM, Montes J, Dunaway S, et al. Adiposity is increased among high-functioning, non-ambulatory patients with spinal muscular atrophy. Neuromuscul Disord. 2010;20(7):448-452. doi:10.1016/j.nmd.2010.05.013
8. Mehta NM, Newman H, Tarrant S, Graham RJ. Nutritional status and nutrient intake challenges in children with spinal muscular atrophy. Pediatr Neurol. 2016;57:80-83. doi:10.1016/j.pediatrneurol.2015.12.015
9. Willig TN, Paulus J, Lacau Saint Guily J, Beon C, Navarro J. Swallowing problems in neuromuscular disorders. Arch Phys Med Rehabil. 1994;75(11):1175-1181.
10. Wang CH, Finkel RS, Bertini ES, et al. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol. 2007;22(8):1027-1049. doi:10.1177/0883073807305788
11. Yuan N, Wang CH, Trela A, Albanese CT. Laparoscopic Nissen fundoplication during gastrostomy tube placement and noninvasive ventilation may improve survival in type I and severe type II spinal muscular atrophy. J Child Neurol. 2007;22(6):727-731. doi:10.1177/0883073807304009
12. Davis RH, Godshall BJ, Seffrood E, et al. Nutritional practices at a glance: spinal muscular atrophy type I nutrition survey findings. J Child Neurol. 2014;29(11):1467-1472. doi:10.1177/0883073813503988