According to a recent study, Young children with spinal muscular atrophy type 2 and 3 are at a high risk of bone fragility and me even cause fractures which goes unnoticed. The researchers said that the breakthrough advances in the SMA treatment require filling the gap between the effect of the disease on the health of various organs, parts like bones, and the possible efficacy of therapies on resultant comorbidities.
The main reason for the weakness of the bones is less mobility. It causes bone mineral, density and strength deficiencies. Hence, children with low body mass, less mobility and SMA are at a high risk of developing fractures in the bones.
Low level of Survival motor neuron protein is the underlying cause of SMA. The preclinical data also suggests that this low level of survival motor neuron protein is involved in bone mineralization issues. However, there have been very limited studies and data which can help us to relate between bone mineralization problems and motor abilities in SMA.
Researchers of the neurological institute in Italia and the United Kingdom carried out research to investigate the evolution of bone metabolism, bone mineral density, and fractures along with their relationship with age and motor functions. They carried out the research on 32 children affected by SMA type 2 and 3. Of these, 27 had type 2 and 5 had type 3. Out of these five children, 4 good walks independently.
Initial research of these children previously showed that they had low vitamin D level and high parathyroid hormone, to messenger molecule fundamental to regulating calcium in bones. Increased bone fracture and undiagnosed vertebral fractures were also noticed. Later, all the children went through a lot of tests including X-RAY of spine and bones. Additionally, follow-up evaluation of 12 children was done after 36 months.
Moreover, none of the children had received spinraza or other treatment for SMA. All the children were given a form of vitamin D and dietary calcium at the beginning of the study.
The data from the studies revealed that vertebral fractures were more prominent when there were low levels of vitamin D at the beginning of the study. Hence, the conclusion could be drawn that increasing the level of vitamin D could help in decreasing the rate of bone deterioration.
For patients had and history of lymph and bone fracture at the start of the study. 5 others underwent the same path. This verb mostly because of low trauma and a consequence of bone fragility. The readings were noted down by the researchers.
Bone concentration level and density deteriorated over time. It consistently decreased compared to the start time. The number of patients with low spine mineral density increased rapidly. As much as 75% of the sample space had low spine mineral density in just 36 months. No relation was found between motor abilities and bone density.
Hence, it is quite evident that those children who can locomote are at less risk of developing diseases like osteopenia and osteoporosis. It shows a deep relation between the muscle movement in the development of bones at the early stage of life.
Bottom Line
The researchers concluded that children with SMA are at high risk of bone. The 36-month long research showcased that children suffering from SMA type 2 especially are at a high risk of developing bone fractures. Moreover, the future of the patients currently being treated with spinraza will provide evidence of what is the state of the bone in the long run. Researchers are constantly working on the patients to find out the relation between the disease and bone fractures. They have found out that vitamin D will decrease the rate of bone deterioration in children.
