Maria Isabel Bueso, MPS VI, and the Sin of 'Moral Albinism'
In the world of medicine, albinism (being an albino) refers to any of a number of rare, inherited genetic conditions in which the amount of melanin pigment (that which causes skin to tan in sunlight) is dangerously low. Albinism is characterized by almost dead-white skin and hair and - somewhat erroneously - pink eyes. Baby boomers will likely remember rock guitarists Johnny and Edgar Winter and are certainly familiar with journalist Anderson Cooper, all of whom suffer from the condition.
Thus, to be an albino - medically speaking - means to be without any color or shading. It is - without question - a genetic condition. Let’s posit for the nonce that albinism can extend beyond the body, and the term used to describe and define other kinds of human mutations and failings. What I have in mind is what we might call “moral albinism” - an ethical code utterly devoid of conscience, coloration or nuance, and caused not by an inherited genetic mutation, but rather by intense psychological abnormality - which may or may not be a familial legacy. To my way of thinking ‘45 and most of what passes for his revolving-door, three-ring circus of an administration, suffer from collective moral albinism. Let’s face it: anyone possessing even a scintilla of “moral melanin” would find it difficult - if not morally repugnant to the max - to lend support to white supremacists, neo-Nazis or racists; to find no problem with separating refugee or asylee children from their refugee or asylee parents; or from having little or no problem deporting children with life-threatening medical conditions to countries which are virtually incapable of treating, let alone saving their lives.
At this point we introduce one and all to Maria Isabel Bueso, potentially America’s most prominent victim of Trumpian Moral Albinism. Maria Isabel (called mostly by her middle name, “Isabel”) was born in Guatemala. At age 7, she was diagnosed with MPS-VI, also called “Maroteaux-Lamy Syndrome” and mucopolysaccharidosis type VI, a rare and fatal genetic disorder. Permit me a sentence or two as a medical ethicist who is not unaware of compassionate use studies involving MPS VI. This rare condition involves the deficiency or absence of an enzyme called arylsulfatase B which leads to the accumulation of complex carbohydrates. It can easily cause life-threatening complications including coarse facial features, corneal clouding, joint abnormalities, skeletal malformations, an abnormally enlarged liver and/or spleen, hearing loss and death, generally by age 20. This is the disorder Isabel was diagnosed as having at age 7. Without treatment (which was all but nonexistent in 2002) there was little hope she could live another decade.
In 2002, Dr. Paul Harmatz, a pediatric gastroenterologist who practices at UCSF Benioff Children’s Hospital Oakland, Calif. learned about Isabel and inquired as to her interest in coming to California in order to partake in a clinical trial of a new drug (Naglazyme®), a first-of-its-kind enzyme-replacement therapy that extends patients’ lives by more than a decade, on average. Isabel and her family’s willingness to relocate to support her - and armed with a V-2 Visa, helped make it possible for the trial to move forward. Two years later, Dr. Harmatz’s trial led to FDA approval of Naglazyme. For the past 16 years, Isabel has been receiving 6-hour weekly infusions. Not only that; during these sixteen years she has stabilized, graduated summa cum laude from California State University, East Bay, and made other contributions, including the establishment of a scholarship for students with disabilities. Meanwhile, her family members have forged new careers and new connections in their church and community here in the United States. For the past 16 years Isabel, her family, and tens of dozens of other children having life-threatening diseases and disorders, have continued receiving medical care under a government program that defers action on deportations in order to seek medical treatment.
Then Isabel - and so many other children and families - ran headlong into Trumpian Moral Albinism: the program which permitted them to remain in the United States was about to be discontinued and they all had one month to leave the country or face deportation. For Isabel and the other children - whose participation in these clinical trials has led to major medical breakthroughs - deportation was tantamount to a death sentence. Last week Isabel testified before the House Oversight Committee - alongside Jonathan Sanchez, a young Honduran suffering from Cystic Fibrosis - telling them that being forced out of the United States was signing their death warrants.
For its part, the Trump administration has wavered back and forth as to what indeed they are going to do. First, the administration, in a statement from the United States Citizenship and Immigration Services (USCIS), announced an abrupt end to the program which permits non-citizens seeking medical treatment in the U.S. Then, after Isabel’s congressional testimony brought this sinful, inhumane situation to overall public attention, USCIS backtracked a bit and said they would reexamine Isabel’s deferred action application. As of today (September 15, 2019) no one knows what the outcome will be. The one thing the administration has done is to transfer the entire issue from USCIS to ICE (Immigration and Customs Enforcement), whose mandate has nothing - I repeat NOTHING - to do with these sort of deferrals.
So far, the administration has been absolutely closed-mouth about what motivated them to deny medical attention to some of the most vulnerable people on earth. Trump’s legion of moral albinos have taken to social media and charged that these deathly ill human beings are “milking American taxpayers out of their hard-earned dollars” and that “we should take care of Americans first.” Of course many of those making these kinds of charges steadfastly favor eliminating Obamacare, cutting funding for Medicaid and mental health services and deporting any and all who “take” so much as a dime in government services. Then too, they have no idea that most compassionate use studies are paid for by pharmaceutical companies, philanthropic organizations, the National Institutes for Health or national groups devoted to raising funds for and awareness of various medical conditions, diseases and disorders.
This sinful act of moral albinism - larded over with good old-fashioned stupidity and abject meanness - is, quite likely, the POTUS’s attempt to keep his political base happy . . . to show them how terribly tough he can be when it comes to and all non-citizens. Although I find this strategy far more than detestable, I nonetheless can understand it . . . as a political strategy; do anything and everything to keep your political base happy. Again, this I understand. But what mystifies and sickens me the most is that this base is made up largely of Evangelical Christians - people who carry a Bible in one hand and the sword of puritanical moral judgement in the other. For reasons which totally elude me, they find no inconsistency in decrying the moral degradation of modern society while supporting the least moral president in history; of urging “In God We Trust” signs and the Ten Commandments (which include the words “Thou shalt not bear false witness against thy neighbor” in every classroom all the while cheering on a man who never goes to church, tells a minimum of a dozen lies a day and doesn’t even know that “Corinthians II” is called “Second Corinthians” rather than “Two Corinthians,” His base contains millions of people who can quote Scripture from here to Tristan Da Cuna but conveniently become deaf, dumb and blind when it comes to verses which implore us to clothe the naked, feed the hungry and care for the sick and the strangers amongst us.
I guess that when it comes to choosing between appointing conservative Supreme Court justices, restricting abortion access and LGBT rights, supporting the right to own and carry automatic weapons and turning a blind eye to the sin of moral albinism, the choice is easy.
Let’s pray that one day, someone will engage in a clinical trial for creating a successful method of moral melanin replacement therapy. Goodness knows we need it.
421 days until the next election.
Copyright©2019 Kurt F. Stone