Hospitalization for Spinal Muscular Atrophy Patients

Hospitalization for Spinal Muscular Atrophy Patients

Both emergency room and inpatient visits are more frequent in those with neuromuscular disorders than in those without these disorders.1 According to one study on children with neuromuscular disorders like spinal muscular atrophy (SMA), respiratory symptoms account for more than half of instances where children are taken to the hospital.2 The next most common symptoms associated with hospital visits for these children relate to gastrointestinal issues. 

The Statistics

When children with SMA arrive at the emergency room, further hospitalization is required in approximately half the cases.2 When hospitalization is warranted, pneumonia is to blame more than 48 percent of the time. Acute gastritis is the next most frequently cited reason for hospitalization in this set of patients, with about one in five of those hospitalized experiencing acute gastritis. 

Scoliosis is another issue that results in hospitalization of children with neuromuscular disorders.3 Compared to those without one of these disorders, those with neuromuscular disorders tend to stay in the hospital longer following scoliosis surgery. Further, this population experiences a higher hospital mortality rate in the case of scoliosis surgery. 

One study showed that the most common examinations prescribed when neuromuscular disorder patients enter the emergency room are complete blood count and C-reactive protein and that the most common treatment is intravenous fluid administration.2 Interestingly, despite respiratory difficulties constituting the majority of emergency room visits in neuromuscular disorder patients, tests related to pulmonary function, including chest x-rays, pulse oximetry, and blood gas analysis are ordered relatively infrequently. 

Preventing Hospitalization

Certain interventions appear to be effective in avoiding hospitalization in the case of SMA and other neuromuscular disorders. For instance, regular follow-ups with neuromuscular specialists are associated with shorter hospitalizations as well as the receipt of care that is more consistent with relevant guidelines.4 Even for those who may not be able to visit a specialist’s office, telemedicine can be a beneficial option. Indeed, the employment of telemedicine has been shown to reduce hospital admissions for neuromuscular disorder patients.11 

One study on SMA patients found that noninvasive respiratory muscle aids like intermittent positive pressure ventilation enabled several patients to stay out of the hospital and remain in their communities, participating in school or jobs.5 There is further evidence that noninvasive management offers a cost-effect way for patients to live at home.6 Oxygen therapy, on the other hand, is associated with higher rates of pneumonia and hospitalization.7

Research shows that both noninvasive ventilation and mechanically assisted coughing allow children under the age of three who have SMA type 1 to avoid acute respiratory failure and hospitalization.8 While both tracheostomy and noninvasive respiratory support can extend the life of SMA type 1 children, only noninvasive respiratory support is associated with a reduced number of hospitalizations in those over the age of five.9 Home mechanical in-exsufflation, which strengthens the ability to cough, has also been shown to reduce the days that neuromuscular disease patients spend in the hospital at both six months and a year following the start of this treatment.10

References

1. Mann JR, Royer JA, Mcdermott S, Hardin JW, Ozturk O, Street N. Hospitalizations and emergency room visits for adolescents and young adults with  muscular dystrophy living in South Carolina. Muscle Nerve. 2015;52(5):714-721. doi:10.1002/mus.24599

2. Kao W-T, Tseng Y-H, Jong Y-J, Chen T-H. Emergency room visits and admission rates of children with neuromuscular disorders: A 10-year experience in a medical center in Taiwan. Pediatr Neonatol. October 2018. doi:10.1016/j.pedneo.2018.09.008

3. Barsdorf AI, Sproule DM, Kaufmann P. Scoliosis surgery in children with neuromuscular disease: findings from the US National Inpatient Sample, 1997 to 2003. Arch Neurol. 2010;67(2):231-235. doi:10.1001/archneurol.2009.296

4. Vry J, Gramsch K, Rodger S, et al. European cross-sectional survey of current care practices for Duchenne muscular dystrophy reveals regional and age-dependent differences. J Neuromuscul Dis. 2016;3(4):517-527. doi:10.3233/JND-160185

5. Bach JR, Wang TG. Noninvasive long-term ventilatory support for individuals with spinal muscular atrophy and functional bulbar musculature. Arch Phys Med Rehabil. 1995;76(3):213-217.

6. Bach JR, Tran J, Durante S. Cost and physician effort analysis of invasive vs. noninvasive respiratory management of Duchenne muscular dystrophy. Am J Phys Med Rehabil. 2015;94(6):474-482. doi:10.1097/PHM.0000000000000228

7. Bach JR, Rajaraman R, Ballanger F, et al. Neuromuscular ventilatory insufficiency: effect of home mechanical ventilator use v oxygen therapy on pneumonia and hospitalization rates. Am J Phys Med Rehabil. 1998;77(1):8-19.

8. Ottonello G, Mastella C, Franceschi A, et al. Spinal muscular atrophy type 1: avoidance of hospitalization by respiratory muscle support. Am J Phys Med Rehabil. 2011;90(11):895-900. doi:10.1097/PHM.0b013e318232883a

9. Bach JR, Baird JS, Plosky D, Navado J, Weaver B. Spinal muscular atrophy type 1: management and outcomes. Pediatr Pulmonol. 2002;34(1):16-22. doi:10.1002/ppul.10110

10. Moran FCE, Spittle A, Delany C, Robertson CF, Massie J. Effect of home mechanical in-exsufflation on hospitalisation and life-style in neuromuscular disease: a pilot study. J Paediatr Child Health. 2013;49(3):233-237. doi:10.1111/jpc.12111

11. Portaro S, Calabro RS, Bramanti P, et al. Telemedicine for Facio-Scapulo-Humeral Muscular Dystrophy: A multidisciplinary approach to improve quality of life and reduce hospitalization rate? Disabil Health J. 2018;11(2):306-309. doi:10.1016/j.dhjo.2017.09.003