According to a story from the East Bay Times, it was 2003 when Isabel Bueso and her family traveled to the US from Guatemala. She was just eight years old, and her family was able to enter the country because she was to receive a life-saving therapy for Maroteaux-Lamy syndrome, a rare disease. US immigration law allows immigrant families to reside in the US if they are receiving medical treatment that they can’t get in their home country.
About Maroteaux-Lamy Syndrome
Maroteaux-Lamy syndrome, which is also referred to as mucopolysaccharidosis VI (MPS VI), is a rare lysosomal storage disease. The effects usually appear at birth. The disease is linked to a deficiency of an enzyme called ASRB. This enzyme normally breaks down chondroitin sulfate and dermatan sulfate. The deficiency is caused be a genetic mutation that is inherited in an autosomal recessive pattern. The disease can cause a range of serious symptoms which can vary between patients. They include nerve pain, cloudy corneas, deafness, delayed walking, growth inhibition, hernias, heart disease, limited movement, skeletal deformities, macrocephaly, and enlarged liver and spleen. Unlike some related illnesses, intelligence is often unaffected. Treatment may include enzyme replacement therapy, certain medications for heart problems or infections, and various surgical procedures. Disease progression and severity range widely as does life expectancy; airway obstructions or heart problems are the most common causes of death for patients with Maroteaux-Lamy syndrome. To learn more about Maroteaux-Lamy syndrome, click here.
Living Under Threat
The family’s life in the US was thrown into chaos in 2019, when President Trump cancelled the deferred action provision that was preventing the family from facing deportation. The Buesos were sent a letter saying that they had 33 days to leave the country or otherwise they would be deported. Losing access to critical treatment would have effectively been a death sentence to Isabel, and thanks to public outcry, the administration reconsidered deferred action and allowed the Buesos to stay for two more years.
In that time, the Bueso family and others like them had advocates in the halls of Congress. Rep. Mark DeSaulnier spearheaded the effort to develop legislation that protects families like the Buesos. After passing in the House last June, the bill was ultimately signed into law by President Biden on December 30, 2022.
“Congressman DeSaulnier’s support and dedication to our struggle, finding a way forward and making it happen in spite of obstacles, is a testament to his commitment to making life better for his constituents and others. I hope that more people can get the relief like I did and that this will inspire others to know that their members of Congress can help.” – Isabel Bueso