Monday, February 29, 2016

Rare Diseases Day

Kris and I Two Days Before Transplant Surgery
Happy Leap Day! Today is also Rare Diseases Day, a day when we who have a rare disease, can feel proud and special. You may hear us say things like "Mine is rarer than yers" or "Maybe, but mine is easier to pronounce".
In the USA, a disease or disorder that affects fewer than 200,000 Americans at any given time is defined as rare.
I'm 'blessed' with a rare disease with many names. Wegener’s Granulomatosis A.K.A. Granulomatosis with Polyangitis (GPA)/Midline granulomatosis/Necrotizing Respiratory Granulomatosis7Pathergic Granulomatosis etc. etc. The prevalence of GPA in the United States is estimated to be 3 cases per 100,000 people. So statistically there should be 3 or 4 of us in Sioux Falls, SD where I live, which would make a rather small support group.
GPA was first described by a German pathologist Friedrich Wegener (pictured below),. In 1989 the American College of Chest Physicians (ACCP) gave Wegener a “master clinician” prize. That prize was rescinded in 2000 when a Mayo Clinic doctor discovered his Nazi past and connections to experiments on Polish Ghetto prisoners. This also triggered a movement to change the name. Currently GPA appears to be the lead horse in this race.
I'm so proud. wink emoticon
GPA vasculitis destroyed my kidneys in March of 1974. My sister Kris, (organ donors and the families of deceased donors are the best people in the world) gave me one of hers in Oct. 1975. In 2013 it became apparent that I was having a GPA relapse, this time as a fibrosis in my lungs (interstitial lung disease). So far the kidney is fine, but my lung function is significantly diminished. I'm currently on a treatment regimen that should induce a remission. We'll know more in a few weeks. In the meantime I'm still 'livin on bonus time'.
Dr. Friedrich Wegener


http://www.rarediseaseday.org/
http://emedicine.medscape.com/article/332622-overview

Tuesday, February 2, 2016

Tuesday February 16th, 2016 (Ground Hog Day)

The followed story is true, mostly.
The South Dakota ground hogs (the Western Marmot aka woodchuck) saw no shadow today, not due to the snow storm; but because they, as 'true hibernators', are asleep in a deep burrow and emerge only in early spring. February 2nd in South Dakota is definitely not spring by any stretch of the imagination.
For much of the time we have lived in this old house, we have shared our space with one or more marmots. One who lived under our deck was rather special. We named him Woodrow R. Charles and called him Woody. He was quite fond of apples and peanut butter.
Since he lived on our property, ate our apples, and was considered part of the family we decided he should be listed as a dependent on our income tax return. It is necessary to provide a social security number for each dependent declared. The United States Social Security Administration had no particular objection to marmots having a SSN, but they stubbornly insist the each have a birth certificate. We soon discovered that if you fill out the blank on the birth certificate application asking "where born" with "in a burrow in the ground" there is little chance of getting a birth certificate.
One day, when a door was left open, Woody wandered into the garage and then when the door was closed became trapped there overnight. In the morning he was in full panic mode, trying to jump to a high window and causing a lot of racket. Worried that he would injure himself, with the help of Animal Control and apples and peanut butter, we succeeded in a getting him in a live trap. He was then escorted on a one way trip to the woods. I imagine he found other Marmots with whom to interact and form relationships. But he is probably wondering whatever happened to his apples and peanut butter.

Tuesday, September 8, 2015

An Equestrian Adventure

 Back in  1974 a type of vasculitis called Wegener's Granulomatosis caused my kidneys to fail. Forty years later, my transplanted kidney is still working splendidly, but there has been a relapse of the vasculitis. This disease is now affecting my lungs (fibrosis). The first line of treatment is high doses of prednisone which are, over several weeks, tapered down to a maintenance level. In my case the maintenance level is about triple the I have been on for nearly 40 years to prevent rejection of the kidney. So now I am re-living all the side effects. Incidentally, the good Dr. Wegener, who first described this type of vasculitis, it turns out was not so god after all. He was a Nazi during the 30's and 40's and was suspected of being involved with 'medical' experiments at Ausschwitz. The medical community now refers to that disease as Granulomatosis with polyangiitis, or GPA.


I wrote a poem about being on higher dosages of prednisone.

The Ride on the Prednisone Horse

“I think I have just what you need”
“It’s like an equestrian ride on a steed”
Answered the doctor when I asked please
Can you help me to stop this disease

So I will Ride on the Prednisone Horse
The name of my new treatment course
A metaphor befitting the verse
Called the Ride On The Prednisone Horse

Now I’m riding the prednisone horse
Feeling so good I could burst
Ravenous appetite, feeling the best
I'm lovin this prednisone hoss

As I’m riding the prednisone horse
A bit of a wild ride on this beast
No saddle or reins to coerce
Or tame this incredible force

While the prednisone horse does the healing
I’m having some uneasy feelings
Moon face and puffiness swelling
While my unstable feelings go reeling

Rages and tantrums are par for the course
When riding the prednisone horse
If the man in the moon is the look you choose
Then come ride on the prednisone horse

“Beware of this horse it's the devil’s
Those little white pills
Come with all kinds of ills”
They warned me to no avail

With weight gain and blood sugar perverse
Thinning hair, bones and skin are the curse
Blurry vision, fractures and worse
It’s barely better than riding the hearse

I hate you damned prednisone horse
Your double edged sword is the worst
I want to be rid of this treatment course
You’ve turned my good life into farce

But I do love you dear prednisone horse
You’ve knocked my disease off its course
I need you my prednisone horse
Or the disease will just keep getting worse

So I’ll stay on the prednisone horse
But with just a little remorse
It is more of a cure than a curse
And for now there’s no better recourse




Sunday, June 21, 2015

The Origin of the Term B.S.
A reminiscence on Father’s Day 2015
My Dad, Kermit passed away in 1974 from liver failure. He would have been a few months short of 101 on this Father’s Day.
Mom and Dad on 25th Anniversary

From Top: Dad (Kermit), Millard, Elna, Carter

My Dad as Baby ca. 1915

With many faults and strengths; he was not the greatest father that ever lived, but still not the worst.
Dad was a big strong man, six foot, 250 pounds, with enormous hands. Mom told me that once, before my time, he and my mother were driving in a Model A Ford and had a flat tire. Finding they had a spare tire but no jack, my dad lifted the car at the rear while my mother removed the wheel and replaced it with the spare. Then there was a legendary bar fight in Worthing South Dakota when two men trying to restrain him, one on each arm, were flung backwards as he pursued someone who had angered him. That provocateur fled out the back door not to be seen at that bar for some time.

As far as I know, my father never physically or verbally abused anyone in our family. There was sometimes a slap or a spanking when a child misbehaved, but that task was always performed by my mother. I did however observe one time where my dad was abusive.

In south Lincoln County South Dakota in the 1950’s nearly every farm had one or more milk cows. In order for the milk cows to produce milk, the cow had to periodically get pregnant and give birth to a calf. In those days, before artificial insemination was prevalent, this process would require the presence of a bull. Not every farm kept a bull, since it used farm resources, pasture, hay, etc. but contributed only one thing and that only happened once in a while. If you did not have a bull when needed, you could usually borrow one from your neighbor. Then you may end up pasturing that bull until another farmer needed it.
A Bull

On this occasion, my dad’s bull had been residing at a neighbor’s farm with ten or twenty cows, but was needed at home to do his bullish duties. My dad had an old Model A Ford that he used around the farm much like farmers today use gators and four-wheelers. There was a canvas tarp across the seat to protect our posteriors from springs protruding through the upholstery. A hole in the floorboard allowed passengers to watch the road pass by.
1930 Model A Ford (similar to my dad's)
He grabbed me and we drove in the old Ford a couple miles to and into the pasture to get the bull. My job was to open the gates through which we needed to herd the bull and block the open gates and driveways until we got home. When we approached each opening, Dad would slow the Ford and I would get out and run ahead.

As we got farther from the pasture and his cow harem, the bull became less and less willing to continue. Dad honked the Model A’s oogah horn and nudged the bull with the bumper all the while yelling curses in Norwegian at the bull. The bull then decided to double back through the ditch. Dad threw the Model A in reverse and sped backwards past the bull then into the ditch with me holding on for dear life. As the nudges became more and more forceful, the bull stopped in the middle of the road, turned around, snorted and scratched at the gravel with his a hoof. The Model A honked “oogah, oogah”, Dad shouted Norwegian curses and “wham” the radiator grill connected with the bull’s head. That was when the bullsh*t started.
There is a biological phenomenon called the fight or flight response. Evacuation of the bowels and bladder among other related things often happen when an animal feels threatened. As the Model A continued to nudge the bull, the bull continued to defecate splattering the front fenders and windshield with runny B.S. When we reached the home yard the bull manure was dripping from the front of the Model A along with profuse radiator leakage.
There are a lot of etymological explanations for the term bullsh*t. To me, the word simply describes the behavior of an actual bull trying his best to appear fierce and belligerent while his profuse excrement exposes his fear.
Nowadays every nudge of that Model A’s bumper would probably be considered animal abuse eligible for a hefty fine or if the bull was injured it might even be a felony. Back then, at least in south Lincoln County, it was just bull herding.

I know that as much as I wished to be, I probably was/am not the greatest Dad in the world; I'll never know. But, I can say with 100% certainty, that I have never abused a bull.

Tuesday, December 25, 2012

Before There Was Light Part Two

The Christmas Tree 

This is a continuation of a story that hasn't been posted yet (Before There Was Light). Think of it as kind of like the prequels to Star Wars, minus the Jedi Knights, all the weird hair and costumes.

Christmas Before Electricity 

The farm where I grew up had no outside source of electricity until I was about four or five years old. The Delco light plant (a 32 volt home electric generator system) had been submerged when the basement flooded and could not be repaired or replaced during the war (WWII). After the war, they knew the REA (a government subsidized rural electrification program) was coming, but not exactly when the electric lines would reach our farm. So the Delco plant was abandoned with the expectation of light poles appearing up and down the road. Many of my earliest memories are of that year or two when there was no electricity in that farmhouse in Lincoln County South Dakota.

The Christmas Tree

That Christmas in 1945 or 46; my sisters wanted more than anything to have a tree to decorate. I wanted a tree too; I had seen them in town all covered with electric lights, colored balls and tinsel. Looking at the tree with all those lights was was a very pleasant experience for me, enchanting almost to the point of hypnotic.

So my mother decided we should have a tree and that we would have to light it with candles.
Getting a tree was no problem, there were evergreens in the grove, but this time we bought one from a store in town. I remember walking with my mother and two older sisters, Suzy (Anne) and Nancy; my younger sister Christy (Kris) was usually left with Aunt Ann who lived in Beresford. We walked all over downtown Beresford looking for candle holders for a Christmas tree. First to the dime store, then to the K and K dry goods store and finally to Gambles hardware store. I think the clerks probably said something like, "Sure we used to have those, but we haven't sold any for a while." I think we finally went back to the dime store and bought just the candles. As I remember, the candles were in different colors with spiral twists much like these birthday cake candles only larger:

When we got home, my mother and sisters got the tree set up. Momma went upstairs to the store room and  after a while came back with a couple strings of Christmas tree lights in a box. She took the 32 volt bulbs out and inserted a candle where each bulb had been. They strung the lights around the outer boughs and then added the colored glass balls and tinsel icicles. We couldn't light the candles until Christmas eve because they could only be lit once and then it would be over. We had our Christmas present openings on Christmas eve. --Jesus was born that night you know, so that was when Gods gift to mankind took place and was therefore the only appropriate time for opening gifts-- After the dinner (often oyster stew), and the table was cleared and all the dishes were washed, we could sit down in the front room (a.k.a. living room) and the Christmas presents could be opened.
On that evening, I imagine the tree looked something like these pictures:


It must have been incredibly beautiful, but to me it just wasn't as pretty as the trees with electric lights like the people in town had. By the next year, the REA had brought electricity to our farm and new electric Christmas tree lights were bought at a store in Beresford or Canton. For the rest of my young life I was mesmerized by lighted Christmas trees. Sixty-five years later and can still remember that feeling. But that enchanting experience faded as I grew older, replaced by other pretty things (like Bonnie) and other adventures. But that's another story for another time.

FYI, here are two links with interesting information about the history of Christmas lighting http://en.wikipedia.org/wiki/Christmas_lights
http://inventors.about.com/od/cstartinventions/a/Xmas_Lights.htm

Monday, December 17, 2012

It's What Grandparents Do

A grandparent's take on the Newtown tragedy.

So it's Friday December 14th and Old Whatshername is glued to the TV. She hates it when I call her that, but I'm old and it's hard to remember everyone's name all the time. She is switching between CNN, MSNBC, CBS and Fox News to get the latest updates (yes Fox News, I was shocked too). I was about to suggest that maybe she was getting a little too vicariously involved when she said to me: "Those children are Jamie's age. What if something like that happened to Jamie?"
Jamie in Wrestling Head Gear
at Schweinfurt Germany
Well, she had me there. Nothing more to say. The grandchild card trumps everything. I went back to reading the paper. But of course I couldn't stop thinking about the "what if something like that happened to Jamie".

Jamie Practicing Fielding
at Schweinfurt Germany

As the day wore on, it was revealed that twenty children had been killed. I was thinking about the parents and then realized that, most probably, each child had grandparents too. When I was that age I had four grandparents who, I believed, thought I was the greatest grandchild ever. Even my grandpa August, who was getting more than a little forgetful by the time I was six. So on that Friday, something like forty grandparents had their hearts ripped out.

The Power Plant

When I was a little boy my Grandpa August and Grandma Bertha lived in Beresford. One day, when I was something like 4 or 5 Grandpa August took my hand and we walked a couple of blocks to the Beresford municipal power plant. At that time, before the hydroelectric systems on the big Missouri dams, some towns like Beresford, and Sioux Falls, had their own power plants. The Beresford power plant was in a huge building, at least to a little boy, along highway 46. As I remember, there were two huge things (dynamos) that made a lot of noise. The men there greeted my grandpa by name, and asked him about me. When we got back to the house, everyone seemed a little excited and were really questioning Grandpa August. I didn't understand what all the commotion was about; I just felt very special that my grandpa had taken me to the big place with the huge noisy things.
My Grandparents Erik August and Bertha, ca. 1950, the barn
where the pipes and fittings were stored in left background

The Well Driller 

Grandpa August had been a farmer near Westerville, a general store owner in Westerville, a dry goods store owner (K and K) in Beresford, and a well driller. He never stayed at one occupation for very long, but identified most with well drilling. My mother always said well drilling was a dead end because once every farm had a well, you were out of a job. Behind their house in Beresford  was a big barn-like building. Stored in the upstairs of the barn were stacks of pipes and pipe fittings. Wonderful stuff to play with but we weren't allowed in there. One time we came to visit and there was a big well drilling rig sitting in the driveway. My aunt and grandmother were fussing about how he was too old to do that any more and that man was just going to have to come and take it back.

The Grandparents

I had other grandparents, Grandma Bertha, Grandma Rachel, Grandpa Adolph; but those are stories for another time. Right now, I can't stop thinking about those forty or so grandparents who lost their sweet little grandkids in Newtown. I hope there are people reaching out to them with some words of comfort, but I know if it were me I know I would be inconsolable. In the next months and years there will be a lot of words about gun control, changing the culture of violence, better care for the mentally ill, warning signs, presidential commissions and congressional hearings; but little of that will matter to the grandparents who only know there is this black hole where their heart used to be.

Monday, June 25, 2012

Death Panels

A Story of Government Intervention in Health Care

Whenever I heard the phrase "Death Panels" in discussions about health care issues, I would think of my friend Sue from St. Paul. I met Sue at the transplant clinic where her husband had just received a kidney transplant.
The story begins in April of 1974. I was 31 years old and recently admitted to a hospital in Minneapolis with kidney failure. I was receiving dialysis at Hennepin County General Hospital (later renamed Hennepin County Medical Center); without dialysis I would survive two or three weeks. A transplant was possible, but due to high levels of a kidney damaging antibody in my blood, I would need to wait twelve to eighteen months for a new kidney. I had good medical insurance, but there was a lifetime dollar limit. Dialysis and transplantation were very costly then as now. So it might be a year; it might be eighteen months, but I was certain that, without some miracle, I was going to hit that lifetime limit. There was also uncertainty about my ability to work at all or keep my job and whether I would be able to keep my health insurance. My wife Bonnie was working, but her job had no benefits like health insurance.

HR1

While I was in the hospital in Minneapolis, some friends, coworkers and benefactors back in Sioux Falls started a fund drive. As I was talking to a social worker at the hospital, I mentioned the fund drive. The social worker said, "Well you won't need that." She then explained that the miracle I thought I needed had been signed into law by President Nixon in 1972. That miracle was Public Law 92-603, Social Security Amendments of 1972, but commonly known as HR1. On signing the law, President Nixon's statement added "...and it extends Medicare coverage for kidney transplants and renal dialysis--the cost of which is beyond most individuals--to workers under social security, and their dependents."(1)

The God Committee

 How HR1 came to be leads back to my friend Sue. Several years before, Sue had been a member of a health resource allocation committee aka "God Committee", a committee to allocate the scarce resources for kidney failure patients. When there was an opening for a new patient at a dialysis center, the committee would decide which of the waiting patients would be chosen to fill that opening. The committee decisions, at that time, were literally life or death. One could call the committees, as Sarah Palin so succinctly described: a "Death Panel".
To understand the "God committees", we have to go back to 1962 and Dr. Belding Scribner of the University of Washington. Dr. Scribner developed the Scribner shunta medical breakthrough making it possible for patients to receive repeated dialysis treatments. Prior to this device a patient in need of acute dialysis could survive for perhaps six weeks before they had exhausted all the possible sites for accessing their blood stream. With the Scribner shunt, dialysis could be a chronic procedure that could last many years. At the time, an estimated 10,000 Americans were dying from renal failure each year.
In January 1962, the Artificial Kidney Center at Swedish Hospital in Seattle opened as the only dialysis center in the country. Three treatment slots were available, an insufficient number for the 60 or so patients in the surrounding area who needed them. Scribner reasoned that choosing among medically eligible candidates was a social concern, not a clinical decision; as such, the burden of choosing should be shared by the public.


In 1962, Seattle's Swedish Hospital established the Admissions and Policy Committee of the Seattle Artificial Kidney Center. It was charged with deciding which terminal patients would get access to the artificial kidneys. The seven committee members, a lawyer, a minister, a housewife, a state government official, a banker, a labor leader, and a surgeon, were unpaid, and anonymous. The criteria they considered were the prospective patient's net worth, nature of occupation, education, marital status, church attendance, potential ability to work, and how many dependents the patient had. 


In November 1962 Life magazine ran a story written by Shana Alexander about the committee. The article, "They Decide Who Lives, Who Dies: Medical miracle puts moral burden on small committee,” drew considerable national attention to the controversy unfolding in Seattle. The exposé sparked a national debate on the allocation of scarce dialysis machine resources. Alexander gave a speech thirty years later titled "Covering the God Committee"; the name stuck from that point on


As the dialysis technology was spreading across the country, the need for selection committees continued. The new committees tended to be less explicit about judging human worth than the first Seattle effort. Doctors and hospital administrators had learned from Seattle experience; avoid the perils that come with high visibility in choosing who would receive treatment. The decisions were now usually made by estimating which patients could fulfill the demands of dialysis treatment, including a rigorous diet, careful hygiene, and faithfully meeting dialysis schedules several times a week, in addition to the likelihood of returning to a productive role in society.(2)


At the same time many hospitals struggled with the prospect of gearing up for dialysis or adding dialysis capacity to meet the demand of end stage renal disease (ESRD) patients. Many of these patients would survive long past their ability to pay: while once accepted for treatment, they could not ethically be removed for lack of funds. This scenario was hardly an incentive for increasing the supply of dialysis units.


Between 1962 and 1972, lobbying by physician groups and other advocates were effective in persuading Congress to establish Medicare based universal funding for dialysis. Virtually overnight the supply of dialysis machines and facilities increased. At the same time the agonizing ethical predicaments of allocation disappeared, along with the "God committees" used to remedy them." (2)

What Does Any of This Have to do with Obamacare

Arguments against the Affordable Care Act include that it is a government takeover of health care, it would result in rationing and "Death Panels", that the mandates are unfair and unconstitutional, and that it will be too expensive and bankrupt the country. My experience with the government intervention in end stage renal disease makes me believe that these arguments are fallacious. 
  • Government Takeover, in the case of ESRD, no dialysis centers were taken over by government. The majority are now owned by private enterprise, the rest are owned by non-profits. 
  • Death Panels, in the case of ESRD, were not created as the result of government intervention; in fact the "God committees" disappeared.
  • Rationing, in the case of ESRD, was not intensified; it was alleviated with government intervention.
  • Mandates, in the case of ESRD, were effective, constitutional, and universally accepted; in this case the mandate is the payment of Social Security and Medicare contributions in the form of payroll deductions. This week the Supreme Court will announce their decision on constitutionality; fairness should not be in question when everyone is treated the same. 
  • Expense, while ESRD programs are expensive (nearly 40 billion dollars per year) America spends more than 2 trillion dollars each year on health care.

The Rest of the Story

As for me I am thankful every day for Medicare and government intervention in health care. After nearly 37 years with a transplanted kidney I should be. Could I have survived the eighteen months before the transplant without the Medicare coverage? I can't say for sure. I do know that HR1 enabled the opening and expansion of dialysis centers across the U.S. including Sioux Falls.
I am also thankful to my sister Kris, who gave me a kidney; to my wife Bonnie, who rescued me from that Sioux Falls hospital and stuck with me through it all; and to the many doctors and nurses.

References

1 Nixon, Richard, 389 - Statement on Signing the Social Security Amendments of 1972. October 30, 1972
 the American Presidency Project, http://www.presidency.ucsb.edu/ws/index.php?pid=3673#ixzz1y5rsJOgp
2. Satel, Sally, M.D., "The God Committee: Should criminals have equal access to scarce medical treatments?" Slate.com 2008, http://www.slate.com/articles/health_and_science/medical_examiner/2008/06/the_god_committee.2.html

Sidebars


In 2008
$2.3 Trillion Spent on Health Care
End-stage Renal Disease (ESRD) Prevalence (2008): 547,982 U.S. residents were under treatment 
382,343 U.S. residents with ESRD received dialysis
Number of kidney transplants performed: 2008: 17,413
Cost for the ESRD program (2008): $39.46 billion in public and private spending
Incidence (2008): 112,476 U.S. residents were new beneficiaries of treatment
Mortality (2008): Among U.S. residents with ESRD, there were 151.7 deaths per 1,000 patient years. There were 88,620 deaths in all patients undergoing ESRD treatment.