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