Veer Basal, age 14, first started showing signs of Friedreich’s ataxia when he was around ten years old. The first sign that something was wrong was when Veer began to have difficulties with his gait. His movements became unsteady and he had trouble holding objects in his hands because of trembling. Though he was once an active boy who enjoyed biking, soccer, and cricket, this rare disease transformed Veer’s life forever.
About Friedreich’s Ataxia
Friedreich’s ataxia (FRDA) is a neuromuscular genetic disorder which is characterized by abnormal gait due to the degeneration of nerve tissue in the spinal cord. The disorder is linked to a genetic mutation of the FXN gene. The degeneration of the nerves causes the spinal cord to become thinner and neurons begin to lose some of their myelin sheath, a fatty insulating layer that protects these cells and is necessary for normal function. Symptoms of Friedreich’s ataxia include muscle weakness affecting the limbs, heart disorders, poor coordination, diabetes, high foot arches, vision and hearing problems, scoliosis, and slurred speech. Symptoms begin to appear at 7-10 years and the disorder progresses slowly overall. Management strategies for the disorder may include ACE inhibitors, surgery, speech and rehabilitation therapy, and orthoses. Life expectancy is around 50 years. Heart problems are the leading cause of death. To learn more about Friedreich’s ataxia, click here.
Living with The Disease
In talking with Veer’s father, Vikas Basal, on what was most challenging as a parent of a child with FA, he said
“Seeing him lose ability bit by bit is a very tortuous thing to deal with. That’s the worst thing about FA. As the years have passed, he can’t walk without extra support.”
As a father, Vikas wanted to allow his children to make their own choices and valued seeing them become more independent, but Veer’s condition has forced him to adjust. The name “Veer” is of Hindi origin and means “the brave one.” As a young boy, Vikas said that his son was mild-mannered, but as Veer came to accept his diagnosis, his true courage emerged:
“Even a cold or mild virus can leave him exhausted because of FA. There was a time when Veer was sick for a week and the next Monday, Veer told me that he was ready to go back to school. I offered to drop him off but he said he wanted to take the bus like he used to. By the time I got downstairs he was heading to the bus with his heavy bookbag over his shoulder, even though he struggled to walk.”
Vikas is very proud of how his son had learned to live with this rare disease. In the first couple of years after the diagnosis Veer struggled with his motivation, but he was comforted by his family and the companionship of their new labradoodle Buddy. Vikas uses the term “guru” to describe his son Veer because seeing him adapt and continue life with his diagnosis has taught him a lot about persisting and living life in the present moment.
“He keeps teaching me how to live life and how to manage yourself. Veer had reminded me about what is truly important.”
There are currently no approved treatments for the disease, but that could be changing soon. A drug called omaveloxolone has demonstrated great promise in FA patients age 16 or older; it was able to slow disease progression and produce substantial improvement in symptoms and functionality. This was all without causing serious adverse effects. However, the FDA is calling for an additional trial, which under the current circumstances would likely take at least a couple more years.
The Friedreich’s Ataxia Research Alliance (FARA) has delivered a letter to the FDA and Reata Pharmaceuticals, the developer of the drug, calling for an urgent New Drug Application (NDA) that would allow patients to access the treatment more quickly.
If you would like to read the letter and add your signature, click here.
