Meeting the needs of those with Huntington’s Disease

Editor’s note: The following was submitted as an open letter to Michigan’s congressional delegation from Mid Michigan College biology students.

Dear Representative Moolenaar, Senators Stabenow and Peters,

Greetings! As biology students at Mid Michigan College, most dual-enrolled at Shepherd High School, we have studied a wide range of life science issues and their ethical implications. One of the most concerning maladies is Huntington’s Disease (HD).

HD is a rare (~5 in 100,000) inherited condition which causes progressive degeneration of neurons in the brain. Signs and symptoms usually develop between ages 35 to 44 years and may include uncontrolled movements, loss of intellectual abilities, and various emotional and psychiatric problems. People with HD usually live for about 15 to 20 years after the condition begins. It is caused by mutations in the HTT gene and is inherited in an autosomal dominant manner. There is no cure.

Walter Isaacson writes: “If ever there was a case for editing a human gene, it would be for getting rid of the mutation that produces the cruel and painful killer known as Huntington’s disease. Caused by an abnormal repetition of letters in a DNA sequence, it eventually leads to the death of brain cells. Beginning in middle age, victims start to twitch uncontrollably. They cannot focus. They lose their jobs. Eventually they are unable to walk, then talk, then swallow. Sometimes dementia sets in. It is an agonizing death in very slow motion. And it is devastating for the families — especially the kids, who watch their parent’s gruesome decline, face the pity or ridicule of their schoolmates, and eventually learn that they have at least a 50% chance of suffering the same fate.”

MID student Vidaliah Sykes, 20, of Saginaw writes “My whole life I have been terrified of Huntington’s Disease. My mother has Huntington’s Disease, as does my sister. Her two small children and I have not yet been tested. Growing up and watching a parent deteriorate mentally and physically is the worst thing I have ever experienced. By the time I was ten I was making my own doctors appointments, making my mother’s doctors appointments, forcing my mom to take her medications. I feel like my entire life has been unstable because of this disease. I have watched my mom walk into a river and try to kill herself because she couldn’t mentally handle telling her kids their possible future fate. I have seen her go in and out of mental hospitals because administrators tell her she’s fine and good to go home, when she was clearly not mentally stable. Please, all I am asking is to help us find a way to fix this, to help ease the suffering of families afflicted with Huntington’s Disease.”

We thank Sen Stabenow for co-sponsoring the Huntington’s Disease Disability Insurance Access Act S.868/H.R.2050 and ask Sen Peters and Rep Moolenaar to do the same, expediting payment of SSDI benefits and eligibility for Medicare coverage for those with HD.

While some of us initially thought CRISPR was needed to edit embryos before implantation, we realize the goal of Huntington-free offspring of affected parents could be won by less controversial means of preimplantation genetic diagnosis. We therefore ask each of you to collaborate with each other across both the aisle and chambers, the Huntington’s Disease Society of America, and the Huntington Study Group, to craft a bill which would cover the following:

• Allocate increased funds to translate CRISPR use with mouse models into work being done in humans.

• Free IVF services and/or preimplantation genetic diagnosis through a certified/well established/trusted clinic for any potential parent who wants a child without inheriting a mutated HTT gene.

• No cost in-home or nursing home skilled care in final 2 years of life or when family is unable to provide.

Thank you for listening to and acting on the deep concerns of your constituents in central Michigan! We look forward to hearing from you soon.

Brennan Bass, Shepherd Haley Bendele, Shepherd Jackie Bliven, Shepherd Mason Cross, Shepherd Alan Gamble, Mt Pleasant Killeen Kladder, Gladwin Katie Snow, Harrison Vidaliah Sykes, Saginaw Ariel Wieferich, Shepherd Eli Williams, Shepherd Janelle Yarnell, Shepherd Hadrian Wiltse, Shepherd Ren Vandermey, Mt Pleasant