Life With A Rare Genetic Disorder

 I wrote this about four years ago as a potential submission to an online magazine which is focused on the disability community and it was not published there. I've never done anything with it otherwise and finally decided to post it here since I do think it is worth reading and someone might actually get something positive from it.  I have also added a small addendum at the bottom about one related recent event. 

Life With A Rare Genetic Disorder

 

On a bright, sunshiny day in June 1992, my wife Candice walked down an irregular stone path to the main overlook of New River Gorge National Park on her father’s arm and we were married.  At the time, she was in her early twenties, seventeen years younger than me, and so far as we knew she was perfectly healthy.  She had been running college track just a couple of years before at the University of Wisconsin-Platteville.  She quickly obtained a job as a state social worker in Eastern Kentucky where I had been raised and quickly adapted to life in Appalachia although she had never been in the region before we met. 

Four or five years later, she began having difficulty with walking over irregular terrain and the thing I remember most vividly is that sometimes she would simply lock up in the soft dirt of our garden.  We began a series of encounters with neurologists and tests for every neurological disease known to the medical world.  All the tests results were negative and one doctor even told us Candice’s symptoms were psychosomatic.   This cycle eventually brought us to Dr. Joseph Berger, M. D., at the University of Kentucky Hospital in Lexington where he was the department chair and a professor of both neurology and psychiatry.  Dr. Berger is one of the most intelligent, professional, and dedicated humans I have ever known.  After several visits and a repeat of the tests for ALS, MS, MD, and other diseases, all of which were still negative, Dr. Berger sat down with us one day and said, “I don’t know.” for about a half hour which is still one of the strangest experiences I have ever had.  He suggested that we consider going to the National Institutes of Health in Bethesda, Maryland, which Candice agreed to do. 

At NIH, we met Dr. Elif Arioglu, M. D., an equally brilliant, caring, and dedicated young Turkish Muslim woman who was on a fellowship at NIH.  She became Candice’s regular primary physician during the five years or so we traveled to Bethesda.  Candice’s initial health history at NIH was done by the Director of the National Institute of Diabetes and Digestive and Kidney Diseases Dr. Simeon Taylor.  When he walked into the room and introduced himself along with his job title, I realized even more than I had when Dr. Berger said “I don’t know” that we were in truly deep water. I knew that if the president had been ill with similar problems Dr. Taylor and his associates would have been on the job.  

The Human Genome Project was in full swing during the time we were traveling to NIH and we learned about it and got some hope that when the human genome was fully understood we might have a solution to Candice’s problems.  Candice was enrolled in some research protocols, tested in every way known to modern medical science, and provided maintenance medications and some adaptive equipment as her disease continued to progress.  We met some of the most brilliant medical practitioners and researchers in the world along with a growing group of the most exotic patients and diseases we could imagine.  NIH was a highly educational and sometimes inspiring place to spend four or five days every three to six months.  But five years rolled by with no easy answers.  Dr. Berger and Dr. Arioglu eventually co-authored two journal articles about Candice’s condition and her father who had a similar body type and other mild symptoms was also brought into the NIH programs for a few years. 

But nobody came up with any ready answers and eventually Candice grew tired of the endless poking, prodding, tests, and the endless parade of brilliant medical students who repeatedly asked the same questions for their own education.  After five years, she withdrew from NIH but had continued to work for the state of Kentucky the entire time.  She found it necessary to transfer from the Department for Social Services as an investigative worker after she progressed from a cane to a walker to a wheelchair.  But for ten years, she continued as a food stamp worker, and eventually secretary, at the local food stamp office before she finally retired on disability after having worked in that office for ten years after she had progressed into a manual wheelchair.  During this period of slow steps backward, she also began to drive with hand controls and eventually took her state pension and Social Security.  We remodeled our house with an accessible bathroom and ramps.  We learned to adjust to life with a wheelchair and a ramp van.  But answers were not forthcoming other than knowing that she had a rare inherited genetic condition which was linked to both spinocerebellar degeneration and lipodystrophy.  Finally, a researcher in the Toronto area who was working on diabetes research, was able to use NIH generated data along with the data from her one visit years before to the Cincinnati Children's Hospital and isolate the particular genetic flaw which caused Candice’s problems.  Dr. Berger finally told us a few years into the problems that the flaw had been found in only two other patients in all of North America and that he believed all three had a common ancestor.  But there were no further answers.

No one ever believes they, or someone they love, will become a patient with a disease for the medical journals.  Those things don’t happen to ordinary people.  But it happened to Candice and me.  By the time Candice had decided she could no longer work in an office setting, the medical records we mailed to the Social Security Administration were shipped in a medium sized cardboard container with the first piece of paper in the box being a letter from Dr. Berger stating that in his professional opinion Candice was totally disabled.  We have no idea if any worker at Social Security ever read that entire box of records and we suspect that the collective weight of Dr. Berger’s letter and the weight of the box settled the disability case whether the records were ever read or not. 

Over the past twenty years, Candice and I have adjusted to our lives together as well as any two people could.  We have traveled to both Canada and Mexico.  We attend concerts regularly and love to find new things to see, do, and experience.  But we still wonder how life might have been different if NIH had truly been the magical place we thought it might be the first time we entered those doors.  Dr. Arioglu has taught medicine at the University of Michigan for most of the last twenty years.  Dr. Berger has retired from the University of Kentucky and works with the same dedication as always as the Chief of the Multiple Sclerosis Division at the University of Pennsylvania.  Candice and I still live in the same modified home in Eastern Kentucky and work to accept our lives together without any answers. 

Copyright 2019 by Roger D. Hicks

ADDENDUM

This addendum is being written on New Year's Eve 2023 and about two weeks ago our phone rang for the second time over the last twenty years with Dr. Elif Arioglu (Oral), now married about twenty years with two grown sons, seeking the latest news about Candice and her health and also asking if she might be interested in participating in some of Dr. Arioglu (Oral's) ongoing research at the University of Michigan.  We have always both respected and appreciated this woman who, despite her intense work schedule as a doctor, medical researcher, educator, and director of two research programs at the university, has never forgotten us and has found time to become more a friend than a doctor we once knew.  If you have to be saddled with such a rare medical condition, it is an incredible blessing to also be gifted with knowing such a brilliant and caring doctor.   

A Belated Liberal Political Action Plan

 Author's Note: I wrote this about 3 years ago and never posted it to this blog at that time which I should have done. Needless to say, I stand by it, every word.  So should all of you who read it.  If you like it, feel free to share it anywhere you wish with one simple piece of cooperation: IF YOU SHARE IT PLEASE GIVE ME, ROGER D. HICKS, FULL AUTHOR CREDIT! 

A Liberal Political Action Plan

Introduction:

November 3, 2020, was a mixed day for liberal and Democratic political aficionados.  While we won the White House, the election is obviously under attack from TRAITOR Trump and his allies both in the US and in Russia.  While nearly every legitimate world leader has congratulated and consulted with Joe Biden, Vladimir Putin is still denying the winner any acknowledgement and, like his puppet in Washington is striving to hold onto their ability to damage America.  Democrats managed to retain control of the House of Representatives and had  a net gain in the senate but the two runoff races in Georgia leave the Senate in doubt.  In Kentucky, where most of us live or have roots, we have an excellent governor who is under attack from a slate of elected Republican officials and a solidly Republican state legislature.  They have extensive plans to further damage the governor’s ability to successfully manage the state in a time of steadily increasing crisis and they probably have the votes to override most of his potential vetoes of their damaging legislation.  The Kentucky Attorney General is joining in every Republican law suit he can in order to further damage the governor’s ability to succeed.  The one bright side to the Attorney General’s situation is that he has shot himself in the foot with his highly questionable actions in the Breonna Taylor grand jury and seriously damaged his standing with the African American base from both parties which had previously coalesced to elect him.  Kentucky and the nation are both under unrelenting attack by Right Wing Radical Republicans. 

In this writing, I am setting forth what I consider to be goals for each of us as individuals and all of us as a group for which we should be working on a daily basis just as we all did in the months preceding the November 2020 General Election.  I fully realize that all of you might not agree with all of my goals and recognize your right to do so.  But, we are small, hard working, intelligent, committed group of politically motivated women and men who have proven over the past several months what we are capable of as individuals.  We can prove over the next months and years that we can, as a cohesive group, achieve a great deal more.  I do not visualize this group of people becoming a major national force in politics but I also know that we can help promote rational, progressive values in our area and, to a lesser degree in the nation as a whole since each of us has some level of networks with other likeminded people all across the nation.  In my original communication to you about forming such a group, I mentioned the idea that, at some time in the future, we might consider meeting face to face at least once.  I would love to see that happen.  But, for the foreseeable future, that is not possible or advisable until the Covid Crisis is under control.  But the dangerous issues being debated in the nation require immediate actions and we have all proven that we are capable of engaging in those actions on an individual basis.  I am setting out below several important goals we can work on and I hope you agree with me and decide to take some actions to support those goals. 

Let me also say that I have absolutely no personal political goals other than being a continual force for good.  I do not intend to run for political office and I never will.  But I do want to put your mind at ease about the possibility that my intentions could be less than altruistic.  I am nearly seventy years old and my wife has been in a wheelchair for more than twenty years.  I am also working nearly daily on my career as a published Appalachian writer.  I have more than enough to occupy my mind and my time.  So let’s look at what I consider to be important actions we can take to help what I hope can become a changed and grateful nation.

Immediate Goals:

1)    Support President-elect Joe Biden and his administration to overcome the irrational and anti-American opposition to his election which is rooted in the both the legal smoke screens and the Stochastic Terrorism of TRAITOR Trump.  We can speak out on Joe Biden’s behalf.  We can support whatever actions it becomes necessary for him to take to legally occupy the White House on January 20, 2020, as mandated by the US Constitution.  We can write letters to newspapers and magazines on his behalf.  We can confront the supporters of TRAITOR Trump wherever and whenever we encounter them.  We can make telephone calls to radio and television stations to support him.  We can each make whatever monetary contributions to his transition team which we can afford. 

2)    We can also support the ongoing efforts in Georgia to elect Jon Ossoff and Raphael Warnock to the US Senate in Georgia.  As of this writing, the Republicans hold 50 seats in the US Senate without winning either seat in Georgia.  If Warnock and Ossoff both win, Vice-President Harris will hold the tie-breaking vote in the senate which would make President Biden’s work much easier.  We can each support these two important candidates in all the ways we sought to help Joe Biden, Amy McGrath and other candidates.  We can volunteer to make phone calls for them without ever leaving our homes.  We can write letters.  We can make whatever monetary contributions each of us is comfortable with. 

3)    For those of us who live in Kentucky, we must support Governor Andy Beshear in his work to save this state both medically and economically. In order to do that, we must actively oppose the efforts of the elected state officials to destroy the governor’s work.  We must publicly, in every medium possible, confront their attacks on the governor with telephone calls to their offices; telephone calls to our friends and random strangers; letters to newspapers and television and radio outlets; and continuing everything each of did to help elect the governor and to fight for those candidates who would help his cause. 

Intermediate Goals:

1)    In two years, one third of the US Senate and the entire House of Representatives will be up for reelection.  In 2022, Rand Paul will be up for reelection in Kentucky and we have several potentially good candidates to replace him such as Charles Booker, Andy Beshear, and others.  We must work to defeat Rand Paul just as hard as we tried to replace Mitch McConnell this year and, in this case, we must win. 

2)    We will also have an opportunity to replace many of the Republican state legislators in Kentucky including, especially, Goforth, Wheeler,  and the entire Republican state delegation.  We work to defeat them by assisting viable candidates, inducing candidates to run, and registering and turning out Democratic votes.  We must develop a plan to register a majority new eighteen year old potential voters, to educate those voters, and to keep them in the Democratic fold.  I have at least one good idea on that front which I will discuss later. 

3)    In 2023, we will be facing another statewide election in Kentucky and we must work in all the ways above to elect a statewide ticket of Democratic candidates including Governor Beshear if that is what he believes is his best option.  We should also do all we can to induce as many of his inner circle of cabinet members and key advisors to seek either lesser statewide offices, congressional offices, mayoral posts in our larger cities, or positions as county judge executives. 

In Conclusion:

This has been worked out in my head for several months although this is the first attempt I have made to put it into writing.  It is at best a rough draft, a preliminary plan, which I wanted to get to all of you before the fire in our bellies dies as we move away from the recent election.  But always remember that the next election always begins, at the latest, when the votes have been counted in the most recent one.  We must keep fighting as if our lives, our nation, and our state depends on it, which it definitely does.  Please send me your impressions of this set of idea along with your honest criticisms and your own ideas which may differ from mine.

What Scares Me This Halloween!

 Today is October 31, 2023, and in 7 days Kentucky voters will go to the polls to elect all the statewide officials from governor and lieutenant governor to secretary of state, attorney general, state auditor, all the way down to commissioner of agriculture.  Those officials will be in charge of state government for the next four years along with the already elected state legislature which is arguably one of the worst Right Wing Radical Repugnican state legislatures in the nation which is nearly always as bad or worse than the legislatures of the worst run states in the nation, Texas, Mississippi, and Florida.   We have been truly blessed in Kentucky to have the best governor in America for the last four years and he was our attorney general for the preceding four years.  Governor Andy Beshear has been the one thing that has protected and saved Kentucky from the incredibly destructive actions of the other elected officials both in the legislature and in the lesser statewide constitutional offices.  In this election, Governor Beshear is opposed by the worst attorney general in the state's recent history, Daniel Cameron, who has worked daily for the last four years to destroy constitutional freedoms in Kentucky, to robe the state's public school children of funding in order to give that money unconstitutionally to private religious based schools in clear violation of the Seperation of Church and State.  Cameron is joined on the Right Wing Radical Repugnican ticket by a group of equally dangerous, destructive, and unfit candidates.  Those candidates include the current secretary of state Michael Adams who under a purported effort to "improve voting in Kentucky" has worked daily to make voting more difficult for Democrats, disabled, and minorities.  Actually, none of the current slate of Right Wing Radical Repugnican candidates in Kentucky are any better than Cameron and Adams.  They are all dangerous and unfit.  

Thankfully, in nearly all polling in the governor's race, Andy Beshear has been leading the polling by somewhat comforting numbers.  But polling can be deceiving and a great deal of Right Wing Radical Repugnican polling and the companies which conduct is deceptive, goal directed to paint a false picture of actual political trends and geared to assist whatever candidate, party, or political action committee is paying for it.  It is generally not empirical and is almost always goal directed instead of directed toward achieving an honest assessment of the voters' opinions on statewide issues.  This is a very dangerous election for many reasons: 1) the Repugnnican candidates are totally unfit to hold any position of public trust at any level; 2) voters, or at least a large portion of voters in Kentucky are both politically naive and willing to swallow any lie a candidate tells them which they believe fits into their particular set of likes and dislikes.  We are facing the possibility that an unfit Right Wing Radical Repugnican governor and an equally unfit under card of statewide candidates could be combined with what is already the worst possible state legislature.  If they gain control of all three branches of state government the damage they will do the the state in the next four years will be equal to that already done by their equals in Texas, Florida, and Mississippi.  It is imperative that Democratic candidates must be elected in this election in Kentucky.  We must, for the sake of the young, elderly, ill and infirm, people of color, LGBTQ, and all other minorities, reelect Governor Andy Beshear and give him an under card of equally competent statewide officials if we are to keep Kentucky as a functioning democracy.  Go Vote on election day, Tuesday, November 7, 2023!  VOTE STRAIGHT DEMOCRATIC, Every Election, Every Race!  Help save this state from the most unfit slate of candidates the Right Wing Radical Repugnican party has ever put forth in this state.