The International Standard of Care Committee for SMA was formed in 2005 with the goal of establishing guidelines for clinical care.
In 2007, they published the Consensus Statement for Standard of Care in Spinal Muscular Atrophy, addressing common medical problems, diagnostic strategy, recommendations for assessment and monitoring, and therapeutic intervention in each care area. An updated standard of care document reflecting advances in the treatment of spinal muscular atrophy is currently in development.2
The current guidelines recommend that a wide-ranging team of medical experts be assembled as soon as possible to meet with the family. A care plan should include referrals to the medical experts who will direct care for the child. Often the first referral will be to a paediatric neurologist.2
The level of care may range from proactive to palliative
PROACTIVE CARERefers to procedures that address both acute problems and the day-to-day management of a child’s medical needs, including but not limited to:2
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PALLIATIVE CAREFocuses on prevention and relief of suffering to support the best possible quality of life. This may include:3,4
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The purpose of palliative care is to improve the quality of life for individuals with life-threatening illnesses, though this does not mean that they are near death. According to the World Health Organization (WHO), palliative care in children is best accomplished by a multidisciplinary team that includes the family.4
SMA centres
Some neuromuscular disease centres specialising in SMA may provide families with proactive and coordinated care, including:5
Centralised appointments that enable families to see all necessary specialists in a single visit
Assistance for families that need to travel significant distances (parking, meals, lodging, etc.)
Collaboration and sharing of information among physicians that allows more strategic, coordinated care planning
Locations of treatment centres can vary. While some families may be fortunate to live near one, those who don’t may choose to receive regular care at a medical centre that is closer to home. Some families may also choose to build their own care team with specialists in their community.
REFERENCES
1. Cure SMA. Caring Choices. Available at: http://www.curesma.org/documents/support--care-documents/caring-choices.pdf. Accessed May 1, 2017. 2. Wang CH, Finkel RS, Bertini ES, et al; and Participants of the International Conference on SMA Standard of Care. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol. 2007;22(8):1027-1049. 3. Blinderman CD, Billings JA. Comfort care for patients dying in the hospital. N Engl J Med. 2015;373(26):2549-2561. 4. World Health Organization. WHO definition of palliative care. Available at: http://www.who.int/cancer/palliative/definition/en/. Accessed April 28, 2016. 5. European Network for the Cure of ALS. NEuroMuscular Omnicentre (NEMO). Available at: http://www.encals.eu/center/neuromuscular-omnicentre-nemo/. Accessed April 28, 2016.