Article Figures & Data
Figures
- Figure 1
Stakeholders related to the management of spinal muscular atrophy (SMA) in the context of COVID-19.
- Figure 2
Nusinersen loading and maintenance dosing regimen.
Tables
- Table 1
Consensus Statements for the Management of Spinal Muscular Atrophy in Saudi Arabia.
Number Statement 1. Recommendations for patients with SMA and their supporting families and caregivers R1.1 SMA patients and their families must exercise extreme caution to minimize the risk of infection by implementing all national and international recommendations for COVID-19 prevention. R1.2 SMA patients with no respiratory weakness, bulbar dysfunction, or other risk factors should not be considered at high risk of contracting COVID-19 R1.3 All SMA patients infected with COVID-19 must be closely monitored to avoid a possible rapid decline in their respiratory function. R1.4 SMA patient assessments should continue either in-person, if logistically and medically appropriate, or via telemedicine or phone. R1.5 It is recommended that the Saudi Center for Disease Prevention and Control guidelines be followed closely to limit the spread of COVID-19, especially in vulnerable populations, such as SMA patients. These guidelines include: (1) staying at home as much as possible, (2) washing hands effectively with soap or hand sanitizers with at least 60% alcohol for at least 20 seconds, (3) maintaining a distance of at least 2 meters between individuals, (4) covering mouth and nose when coughing or sneezing, (5) avoiding touching eyes, nose, and mouth with unwashed hands, and (6) routinely cleaning surfaces and tools with household cleaning products. R1.6 General measures to minimize the risk of infection should extend to the intervals before and after providing care to SMA patients, such as feeding, bathing, and other everyday activities. R1.7 SMA patients should have a secure emergency care plan with their neuromuscular specialist and seek immediate medical attention if they or anyone in their family show symptoms of COVID-19. R1.8 Patients should stay home and follow and use telehealth and phone clinics to assess non-urgent situations. R1.9 Caregivers for SMA patients are encouraged to update treating neurologists or neuromuscular specialists when needed. R1.10 In case of emergencies, patients or caregivers should call the corresponding emergency healthcare numbers so hospitals can take the necessary steps to minimize the risk of exposure to infection. R1.11 Patients should be directly admitted to the treatment room as they enter hospital units. R1.12 If patients need to wait upon admission, they should maintain a safe distance from all surrounding personnel. R1.13 It is recommended that the number of supporting caregivers in treatment rooms be minimized, and no visitors or unnecessary family members be allowed inside. R1.14 All precautions related to patient transportations to and from the hospital should be followed. R1.15 After hospital discharge, all SMA patients and caregivers should be educated about avoiding COVID-19 exposure through adhering to hygiene procedures and social distancing. R1.16 It is recommended that all follow-up visits be scheduled virtually via clinic phone calls or video links. R1.17 Treating physicians should provide regular awareness and reassurance messages, along with educational materials. R1.18 Physicians should advise parents and caregivers on how to maintain their mental health and wellbeing. 2. Recommendations for Facility and Hospital Readiness for SMA Management 2 R2.1 SMA medications are fundamental, and their administration is considered a high priority in SMA treatment plans. R2.2 Early and uninterrupted management of SMA is crucial to ensure better clinical outcomes, especially in infantile-onset SMA type 1. R2.3 Healthcare providers should collaborate with SMA patients and their families to maintain medication administration and avoid treatment delays or interruptions. R2.4 Nusinersen is a critical therapy for SMA patients to maintain a steady drug concentration in the cerebrospinal fluid by adhering to a scheduled dosing time. R2.5 Treatment delays must be avoided as much as possible for new and currently eligible patients. R2.6 In case of a missed or delayed loading dose, Nusinersen should be given as soon as possible with an interval of at least 14 days between doses. R2.7 In a case of a missed or delayed maintenance dose, Nusinersen should be given as soon as possible, and the following doses should be continued every 4 months as per the original dosing timeline. R2.8 Management of SMA type 1 patients is more urgent due to its aggressive natural history and known rapid decline. R2.9 Initiating therapy for SMA type 2 and type 3 patients depends on their medical condition and the availability of management resources in the era of COVID-19. R2.10 The decision to start gene therapy (Onasemnogene abeparvovec-xioi) should be individualized through the decision of MDT experts in SMA. R2.11 It is recommended that the initiation of gene therapy be delayed in stable patients due to the risk of immune suppression by corticosteroids. Number Statement R2.12 In the case of gene therapy initiation, corticosteroids should be continued in SMA patients and should not be discontinued or interrupted unless discussed with the treating neurologist. R2.13 Corticosteroid-treated patients and their families should take further strict precautions in practicing social distancing and other recommended precautionary measures. R2.14 During corticosteroid therapy, it is recommended that patients have virtual follow-up meetings along with home blood draws for laboratory monitoring of liver functions, troponin, and platelets. R2.15 All healthcare personnel should abide by the national infection control guidelines developed by the Saudi Ministry of Health and the Saudi Center for Disease Prevention and Control. These include: (1) All consent should be taken verbally and documented in the system. (2) All personnel should keep a reasonable distance from patients during all procedures. (3) Avoid sharing commonly touched objects like pens, telephones, and clipboards. (4) All healthcare workers involved in the injection procedure should use personal protective equipment. R2.16 Surgical masks should be worn by healthcare providers even during non-procedure times in the hospital. R2.17 Routine aseptic techniques should be followed throughout the entire injection procedure. R2.18 Minimizing the number of personnel in the intrathecal procedure room, including healthcare staff and family members, is of the utmost priority. R2.19 Healthcare personnel not involved in the procedure should avoid entering any procedure rooms occupied by patients. R2.20 The alignment and readiness of the radiology team should also be considered in the case of fluoroscopy-guided intrathecal injection. R2.21 Virtual multidisciplinary rounds to align treatment strategies with dietitians, pharmacists, social workers, and care-coordination staff are recommended. 3. Recommendations for MDT Approaches R3.1 Patients and their families are advised to continue physiotherapy support as planned per their appointment schedule via virtual clinics with documents about home exercise programs. R3.2 Physical therapists should use their professional judgment to determine when and how to provide care. R3.3 A motor functional scale assessment should be performed every 4 months, one day prior to intrathecal injection, to avoid frequent and unnecessary exposure to hospital settings and risk of infection. R3.4 SMA patients who use noninvasive positive-pressure ventilation or mechanical airway clearance devices are more vulnerable to secondary infections. SMA - Spinal muscular atrophy
MDT - multidisciplinary team
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