What is the treatment for spinal muscular atrophy in children?

If spinal muscular atrophy occurs in children, it will be more troublesome. On the one hand, the disease is quite harmful. On the other hand, there is currently no very effective treatment method, and supportive therapy is mainly adopted in medicine.

1. There is no specific treatment for this disease, and the main treatment is symptomatic supportive therapy. Taking B vitamins and psychological treatment are particularly important. In addition to protecting joint mobility and preventing contractures, moderate exercise can also increase the function of remaining motor units. Physical therapy can also relieve the pain of joint contracture in some children. Care should be strengthened for children in the late stage. Treatment measures are mainly aimed at preventing or treating various complications of SMA, preventing lung infections and pressure sores, malnutrition, bone deformities, movement disorders and psychosocial problems. If accompanied by respiratory insufficiency, a ventilator is required to ensure airway openness and improve respiratory function.

2. Patients often suffer from malnutrition and growth disorders due to weak sucking, obstructed airway or easy fatigue. Negative nitrogen balance may lead to increased muscle weakness and fatigue in children, especially infants. The mechanism of this phenomenon has not yet been elucidated. Some patients suffer from chronic malnutrition, which manifests as fatigue and decreased calorie reserves. Some patients develop organic aciduria due to insufficient nutritional intake, so they need professional nutritionists to provide feeding guidance, adjust feeding plans, feeding postures, and food structure to maximize calorie intake.

3. Scoliosis is the most serious skeletal deformity of SMA. Patients who cannot walk will develop spinal deformities earlier, and most of the deformities are located in the thoracolumbar region. Spinal correction usually cannot prevent or slow scoliosis, but it can help people sit up. Timing is critical for spinal surgery, and surgery can only be performed when lung function is relatively normal. After spinal fusion surgery, the degree of scoliosis will be significantly improved, and lung capacity, sitting, balance and comfort will also be significantly improved.

4. Clubfoot is a manifestation of infantile SMA, but it is relatively rare and generally does not require surgical correction. Other more common deformities include flexion contractures due to movement disorders, which rapidly affect the hips, knees, and ankles and can be prevented with appropriate exercise. It requires long-term persistence and daily exercise, which many families and children often cannot do. Likewise, splints and braces cannot prevent deformities from occurring.

5. In addition, some scholars have used thyrotropin-releasing hormone (TRH) treatment, which is administered through a percutaneous intravenous catheter. The results showed that the peroneal nerve conduction velocity of the children was significantly faster than that of the control group, and the parents of the children also reported that some functions of the children were significantly improved. Therefore, TRH is a useful treatment, but further research is needed to confirm it.