According to a story from Buzzfeed News, Maria Isabel Bueso was born in Guatemala with mucopolysaccharidosis VI, also known as Maroteaux-Lamy syndrome. At age seven she traveled to the US in order to take part in a critical clinical trial. Maria’s role in the trial was essential as the experimental drug being tested has been successful. She currently receives weekly treatments to help manage her illness. There’s just one problem: Maria isn’t a US citizen, and last month, Maria and her family were told that their time to stay legally in the country was running out.
About Maroteaux-Lamy Syndrome (MPS VI)
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.
Deportation Means Dying
Many immigrants who are deported face danger or even death when they return home, but for Maria it would be a guaranteed death because she would not be able to get her weekly treatments. Missing even a single one would cause her disease to progress. The family has been allowed to stay in the country for the past 16 years thanks to a medical deferral, but no longer.
Recently, the administration has responded to public outcry and has officially stated that it is reviewing its policy regarding the deportation of non-citizens who have life threatening rare diseases that can’t get treated in their country of origin.
Patient advocacy groups, including the EveryLife Foundation for Rare Diseases and The National MPS Society, are appealing to the Department of Homeland Security to halt the deportation of rare disease patients. If some exceptions are made, these rare disease patients can continue to get the treatment they need.