As every individual with SMA will experience symptoms differently, there are many aspects to providing care for individuals with SMA
Effective SMA management requires the participation of multiple disciplines and the coordination of many different aspects of care. Comprehensive care generally refers to procedures that address both acute problems and the day-to-day management of the individual’s medical needs.
Although there is no cure, treatment to help maintain physical abilities is now available and is improving the outlook for people with SMA.
Be sure to contact your healthcare provider if you have any questions about appropriate care options.
Respiratory care is a central focus in SMA. Muscle weakness in the chest can interfere with the ability to breathe or cough effectively and increases the risk of an infection.
Noninvasive SMA respiratory care involves techniques that avoid or delay the use of invasive intervention. Special equipment such as a ventilator or bilevel positive airway pressure (BiPAP) machine can deliver a continuous flow of air to the lungs through a mask that covers the mouth and/or nose. A cough assist device may also be used at home to help individuals cough and clear secretions.
Muscle weakness may cause some individuals with SMA to lose their ability to chew and swallow effectively. This puts them at risk of inhaling food and liquid into the lungs and developing respiratory infections. A healthcare provider may recommend a temporary or long-term feeding tube be put into place to provide the necessary nutrition and hydration.
In general, there are 2 types of feeding tubes: nasal and abdominal.
Nasogastric (NG) tubes enter the nose and feed directly into the stomach. These can be easily replaced and are typically used for individuals who need feeding tubes for only a short period of time.
Percutaneous endoscopic gastrostomy (PEG) tubes are placed surgically through the abdomen and into the stomach. They are relatively easy to maintain, which makes them a preferable option for individuals who require longer-term feeding support.
Scoliosis (curvature of the spine) is a common problem found in individuals with SMA due to muscle weakness. An orthopaedic surgeon and/or specialist may recommend postural support (bracing) or surgery for scoliosis.
Individuals with SMA may be too weak to move their joints throughout their full range of motion. This can put them at risk of developing contractures, or tightness of the muscles, which can become permanent and prevent movement. Physiotherapy may consist of exercises to help improve flexibility and overall function. When appropriate, a stander may improve bone strength and can be used at home to help children maintain flexibility at the hips, knees, and ankles.
Palliative care focuses on prevention and relief of suffering to support the best possible quality of life. This may include:
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.