Osteogenesis Imperfecta and Wheelchairs

In the modern world with advanced technology, well-educated professionals can provide treatments for various illnesses. Treatments help prevent illnesses from reappearing in a patient’s body, which makes patients feel happy. Some illnesses are chronic, such as Osteogenesis Imperfecta. Osteogenesis Imperfecta (OI) is also known as brittle bone disease. It is a genetic bone disease that causes the bones in a patient’s body to become extremely fragile and easily fractured with a simple touch. This genetic disease comes in various types with different signs and symptoms for each type. It is most often first diagnosed after a child is born. There are few treatments for children that can help them with survival. One common treatment is wheelchairs. Wheelchairs are a type of assistive device that is beneficial for people with disabilities, especially serving types of osteogenesis Imperfecta. Wheelchairs are the most effective treatment for children as young as three to seventeen suffering from Osteogenesis Imperfecta because it is used for mobility, independence, and maintaining quality of life. 

Wheelchairs are provided to children who cannot walk or stand on their own. In the academic article, “Long-Term Follow-Up Outcomes of nineteen patients with Osteogenesis Imperfecta Type XI” Nurten Akarsu and her colleagues gather results of the investigation that they encountered in patients with Osteogenesis Imperfecta type XI and Bruck syndrome type I after taking dietary supplements. They developed the results through photos and virtual data charts from all nineteen patients. Nurten Akarsu states, “patients were either never able to walk or lost their walking ability in the first years of age” (634). Since OI causes bones within children to easily break, they are unable to proceed in doing movements on their own. Wheelchairs are available to help them navigate around their environment facilely. Wheels help vehicles, machines, and chairs in this case to have smooth, easy, and faster transportation. This becomes beneficial for OI patients because they have advanced technology that can help them migrate easily and safely. 

Thirteen-year-old Sparsh Shah, a wheelchair user, was able to attend TEDx Talk studio to give his speech to his audience on the challenges he faces due to brittle bone disease. He proudly changed the word “Impossible” to “I’m possible” and encouraged others that it is possible to accomplish anything in life. Shah has the ability with his wheelchair to attend his TEDx Talk session on time, easily and safely. Shah already had one hundred thirty fractures so far only at the age of thirteen. Who knows how many more he might have in the future. If he does experience other fractures, he will still have his wheelchair to help him travel from one environment to the next. Children with Osteogenesis Imperfecta have difficulty with constantly balancing the natural function of their bones on its own due to having “soft” bones that can easily break. After a surgery they are unable to move on their own and so wheelchairs help them leave the hospital to their homes easily and safely. Wheelchairs reduce the risk of children between the age of three to seventeen from falling outdoors, which prevents them from having fractures. Not only does Shah’s wheelchair help him with mobility, it also builds independence. Sparsh Shah for an example, stumbled upon a song called “Am not Afraid” by Eminem that was an inspiration to him. He decided that he was not going to hold himself back from going out into the world and make a cover of it himself. (00:04:34-00:04:39) It eventually went viral on the internet, and he received a lot of praise for his work and dedication from celebrities and loved ones. He made his own decision and created his path by doing what he loved dearly.

Children with Osteogenesis Imperfecta are wheelchair users can get to their destination with or without assistance. They can explore and experience the world on their own. Wheelchairs create a path of independence and success for children with brittle bone disease. For example, a child that is a wheelchair user at home can be able to place or return things around the house without having fractures. Osteogenesis Imperfecta should not have to be seen as holding a child back from living their lives. Children with severe OI who must use a wheelchair should not have to feel as if they are being held back either from doing what they want or love to do. 

According to Sparsh Shah, “I decided that I would not hold myself back and instead decided to go out and display my musical talent to the world, I was able to achieve these among many other achievements” (00:06:05-00:06:17). This illustrates how Shah did not let his disability hold him back from doing what he wanted to do. He is independent because he made the decision on his own to remix Eminem, a famous rapper song by himself. He showed the world his talent which he enjoys doing. His wheelchair gave him the “freedom” to make his own choices. He did not let others’ thoughts and actions become a blockade to him. Additionally, Shah controlled the projector screen on his own while he was speaking without assistance from the producers. Wheelchairs help children as young as three to seventeen navigate the world around them on their own which allows them to develop independence; however, others may say that wheelchairs are not the most effective treatment for children with brittle bone disease.

Treatments such as rodding and therapies are yet much more invasive than wheelchairs. According to Johns Hopkins website, “metal rods are inserted to help hold in place (stabilize) and prevent deformities of long bones” (“Osteogenesis”). This procedure is effective to children with Osteogenesis Imperfecta because it holds much strength to children that break their bones, especially in their legs. When children have rods in their legs, for example, the rods help support the bone until it heals. The rods are held together with screws around the area of the fracture. This helps to keep the bone in a suitable position while it begins the process of healing. On the other hand, wheelchairs are much more beneficial. Having a metal rod in a child’s leg can be painful and cause the child’s legs to become stiff. A three-year-old child should not have to experience that at such an early age. Whereas being in a wheelchair the child has less chance of experiencing pain. 

Another treatment is physical therapy, which can help children strengthen their bones and teach them skills to help exercise their bones. When a child gets surgery from a fracture, they need therapy to help cope with the injury in the best way possible.  However, when children have physical therapy, they are more likely to miss out on their learning time in school. The child is constantly being reminded of their disability and the area where the fracture has occurred. This affects the child’s mental health negatively because they become triggered by the experience which can lead to depression. Bisphosphonate medicines are another treatment provided for children with OI. According to the Johns Hopkins website, “these are medicines that help to strengthen bones and prevent fractures. They may be given by mouth or by IV (intravenous line) into a vein” (“Osteogenesis”). Children at a young age must not take solid tablets. They will have difficulty digesting them. Intravenous line can cause infection that can eventually travel through the child’s bloodstream. Additionally, since IV’s target the veins it can damage a child’s veins. This can leave them to have infiltration which can lead to damaging the child’s tissue. Intravenous can also cause blood clots which can affect a child’s vien by blocking the blood flow in the child’s veins. Hence, wheelchairs are much more effective than other treatments for children with Osteogenesis Imperfecta.

Osteogenesis Imperfecta can be prevented from children at the age of three to seventeen. The article, “Osteoporosis: Prevent where there is no Cure” discusses the effects women had from Osteogenesis Imperfecta following procedures to stop the development of the disease. Women at the age of thirty are more at risk than men. It mostly affects women that are diabetic, have menstrual cycle problems, smoke, and have low calcium. Taylor and Francis stated that “smoking, excessive consumption of alcohol, diets low in calcium and vitamin D increase everyone’s risk of contracting the disease” (12). It is best to avoid smoking, reduce the amount of alcohol drinking, and eat food with a lot of calcium and vitamin D which can decrease the chance of having Osteoporosis Imperfecta. Patients must eat food that has calcium such as vegetables, sardines, and dietary products. 

Additionally, women can do excessive exercise such as running, aerobic walking and cycling. Women must also take supplements for calcium. Taylor and Francis also suggested not eating heavy red meat, especially kidneys, because it destroys the minerals in one’s body. This leads to us becoming healthy which prevents Osteogenesis Imperfecta. On the other hand, Taylor and Francis created a subheading titled, “Prevention is better than cure” (12). On the contrary, Osteogenesis Imperfecta cannot be prevented. 

Osteoporosis Imperfecta cannot be prevented because it is “an inherited (genetic) bone disorder that is present at birth” according to Johns Hopkins website. This means that it is a lifelong condition that cannot be cured. Smoking, excessive, drinking of alcohol, and low calcium and vitamin D does not help to strengthen the bones in children or when they get older, but it does not prevent Osteogenesis Imperfecta from occurring. “It should be a weight bearing activity e.g., running, aerobic walking or cycling done for 30-40 minutes at least three times per week” (Taylor and Francis 12). This does not help children with brittle bone disease because it increases the number of fractures for children. Childrens’ bones can easily break as soon as they attempt such aggressive exercises because of how fragile their bones are. Wheelchairs prevent children from bending, falling, and lifting things so for them to execute these exercises, they will need to be done in wheelchairs, especially for those that have severe types of OI. Therefore, wheelchairs are proven to be the most effective treatment and brittle bone disease cannot be prevented. 

The purpose of this research was to investigate treatments for children at the age of three to seventeen with Osteogenesis Imperfecta and the effectiveness of them. Based on the information collected, it is concluded that wheelchairs are the most effective treatment for young children because it has beneficial elements that go along with it. The Johns Hopkins website, Sparsh Shah, and Nurten Akarsu with her team complimented each other in carrying out the positive aspects of wheelchairs for children with brittle bone disease. But Taylor and Francis proceed to go beyond the opposite of that. Other treatments, such as rods are harmful to children as young as three and should not be used. Though future exploration into upgrading the parts of wheelchairs will continue to improve the lives of children with Osteogenesis Imperfecta and encourage them that they are possible.

Work Cited

Akarsu, Nurten, et al. “Long‑Term Follow‑Up Outcomes of 19 Patients with OI Type XI and 

Bruck Syndrome Type I Caused by FKBP10 Variants.” Calcified Tissue International, vol. 109, no. 6, Dec. 2021, pp. 633-644.,

http://doi.org/10.1007/s00223-021-00879-4.

“How a 13-year-old changed ‘Impossible’ to I am Possible| Sparsh Shah| TEDx Gateway

YouTube, uploaded by TEDx Talks, 23 May 2017, https://www.youtube.com/watch?v=bC0hlK7WGcM   

Taylor, and Francis. “Healthwatch: Osteoporosis: Prevent Where There is No Cure,” Agenda: 

Empowering Women for Gender Equity, no. 15, 1992, pp. 12-13., https://doi.org/10.2307/4065578

“Osteogenesis Imperfecta.” Johns Hopkins Medicine, 8 August 2021, 

https://www.hopkinsmedicine.org/health/conditions-and-diseases/osteogenesis-Imperfecta.

 

THOMAS’S RESEARCH PAPER (REVISION) (1)