Soap and Floss Can Save An Aorta or Your Life

Things we take for granted, like personal hygiene, can literally save your heart and aorta from inflammation, dissection and help you avoid an Infective Endocarditis (IE) incident such as the one I ended up with.

Personal Hygiene and Marfan Syndrome - very important for heart health

Inflammation can cause vascular damage in anyone, but especially in those of us with connective tissue disorders like Marfan Syndrome.

Skin infections can quickly reach the heart through a cut or scrape.  Once the cardiovascular system becomes infected and inflamed, this condition can produce deadly results.

Infective endocarditis is especially dangerous for those with prosthetic heart devices such as the mechanical aortic valve in my heart and the Dacron graft across my ascending aorta.

One of the first things I remember after waking up from my first open heart surgery was throwing up all over my freshly sutured and sealed chest incisions.  Barfing never felt so good I thought as I lay in the hospital cardiac ICU bed.  Volumes of fluid erupted out of my mouth and across my chest and I did not care.  It felt so good to empty my stomach of all the slosh.

I vaguely remember Judy shouting to the ICU nurses who practically ignored her calls for help.  She ended up cleaning the vomit herself for the most part.  My vision whirled for a second then I passed back into the land of post-operative deep sleep.

Ten days or so later I made it home (to our now Palm Coast home) with my new bionic heart parts and met our home health care nurse, Ted Rhodes, RN who always says RN stands for 'writes notes'.

Within a matter of days Ted worriedly sent Judy and I back to doctor's office in Jacksonville.  My chest was turning a bright red above the heart incision.  The nurse practitioner pressed hard down on my chest sore now swelling in size, prescribed an oral antibiotic and sent me home with instructions to come back if the boo boo worsened.

Have you ever had a Physician's Assistant take a scalpel to your chest without painkiller?  It hurts!  But the PA felt like he had to get the infection out immediately.  So he pushed me back on the table and cut.  Judy was gasping in the corner of the room at the site of her husband (me) being manhandled on the exam table, cut and sliced without anesthesia.  Puss and blood of course was everywhere.

To make a long story short, the entire episode of madness lasted for over four months from a reopening of my sternum, pressure washing the green goop from around my heart and Dacron graft, installation of a picc line for daily IV treatment with some of the nastiest antifungal and antibacterial drugs I've ever had.  There were wound vacs taped to my chest and heparin hypos everywhere. I still worry each time I run a slight temperature.

Bless Judy for the many times she was sprayed with the stuff trying to purge a line or add a bag of solution at one am.  I believe she still suffers collateral damage even today from exposure to the IV liquids.

An array of doctors and lab techs poked, pulled, prodded and took so many samples but they never figured out what the green slime around my heart was.  Everything was negative with respect to bacteria.  Infectious disease doctors finally blamed an unknown fungus. 

I know what it was.  It was an infection and it was trying to eat me.

So I am here to say, pay attention to personal hygiene, use lots of soap and floss.

Even the American Heart Association and other health groups have been emphasizing personal skin and mouth care lately.  As the use of antibiotics has been backed away from as a favorite for many procedures, to the contrary cleanliness has remained at the top of every list for preventing IE.

In fact some medical literature says personal hygiene is very, very important.

Legs, ankles and feet are more difficult to reach and scrub during a shower.  But pay attention to all the appendages.  Clean between toes and under nails.  Use a back scrubber and reach behind those ears.

Our bodies usually have staph and strepto populations growing across our skin.  But a cut or scrape can allow those germs to enter our bloodstream and reach our heart.  The bad guys are especially attracted to prosthetic implanted devices like mechanical valves or Dacron grafts.

Let me tell you from experience, you do not want infective endocarditis.  Besides the high mortality rates associated with IE, recovery will take a year or so out of your life.

An aorta free from inflammation caused by infection may also be less prone to dissect or develop an aneurysm.

So grab the soap and scrub and scrub and scrub.

No more going to bed at the end of a long, hard day without a shower and scrub. Never again.  Brush and floss too.

Your heart and your partner will thank you.

Marfan Pain. One Simple Cost Effective Solution.

Connective tissue disorders (CTDs) such as Marfan Syndrome, Ehlers Danos Syndrome and others all have one thing in common.  Pain.

Rice Sock Heating Therapy works as well if not better than any heating pad.

Many times the pain is chronic and intense.  Other times the pain may be moderate. But in most cases, torn and ripped connective tissue hurts bad.

Pain killers are certainly one answer, but in my search for sustainable health I would prefer pain relief with less of an impact on my kidneys and liver than pharmaceuticals.

So that is why I am so enthralled with Judy's rice sock, a cost-effective simple pain relief therapy.

Rice Socks are simple and cost effective - all you need is rice and a sock

Over the past week I've experienced really terrible hurt in my ankles, wrists and right shoulder, not to mention a trip to the emergency room with a huge kidney stone(s).  Tonight the cumulative pain had me running for the bathtub and hot water.

Heat helps me deal with the pain.  Heat also moderates inflammation, a very important benefit since inflammation of any type in my body is not good for my compromised cardiovascular system.

A helpful hint here is that if something ever turns into doubling over, unbearable pain and even narcotics don't begin to touch the agony (like my experience tonight), then find the hot!  A scalding bath is first choice for me followed by a heating pad or the rice sock.

Judy's rice sock is so handy because you don't need a tub and a rice sock is less expensive than a heating pad.  Moreover, sometimes when pain strikes in the middle of the night and stores are closed, most people have both rice and a sock (that is all you need).

Rice socks can be wrapped over any painful muscle or joint.

The key to making a great rice sock is finding a thick, long white cotton sock.  I prefer an unused sock but an old sock can work if it has been washed in wonderful scented detergent such as Tide Ocean Mist (beats the smell of WalMart new).

Fill said sock with rice leaving enough room to tie a knot in the sock top.  Any rice will work.  Jasmine or a long grain rice will produce an especially comfortable rice sock.  Once the sock is tied you have one of the most awesome pain relief therapies ever invented.

Though my wife Judy introduced me to the rice sock there are numerous DIY sites across the web.  Google 'Rice Sock' and check out all the amazing testimonies.  I've read of people using the rice sock for pregnancy pain or even to place over the face at night as a warm eye pillow.

With rice sock complete, head to the microwave.  Some people prefer to warm their rice sock in the oven but I like the microwave because it is so quick.  Just don't try and heat the rice sock in a pan of boiling water or you may end up with a huge mushy mess.

I heat our rice sock for two minutes on medium heat.  The rice sock will be HOT when the two minutes are up so be careful to pick up the improvised heating pad by the knot end.  Place the hot sock carefully where your pain is located.  Heat lasts for about twenty minutes or so.

Relief for me is almost immediate.  They are totally reusable too.  Just keep the rice sock dry.

Funny how it is not until my mid-fifties before I find out all these neat and handy life help hints.  Anyway I am so glad Judy made us one.  Hope you find the rice sock helpful for your pain and be sure to pass along the suggestion!

Whoops.  There goes the microwave bell.  Mine is hot.  I think I'll make another so we can have two.  Or maybe three.

Coumadin Lifestyle, Bruising and Bleeding with Marfan Syndrome

Coumadin, is just a dreaded anti-clotting drug that just needs to be understood (yeah, right!).

Bruising and Bleeding with Coumadin

Even though I've been taking Coumadin for twenty one months now there are times when life's normal activities appear to be doable, but wishful illusion quickly fades away with bruises, bleeding and orange skin.

The other day Ruairi and I replaced spark plugs and wires in the car.  Leaning over the motor I braced myself with my right arm, something I'd done many times before during my pre-Coumadin life era.

Three days later I still have a large and quite painful arm bruise, many other small bruises and lots of nicks and cuts scabbing over.  So predictably, I have to periodically remind myself, 'therapeutic Coumadin life has its own set of requirements that must be adhered to or there will be painful consequences'.

I think I can do something I used to be able to do.  Nope.  Not anymore.

So score another for the great drug that keeps me alive, keeps blood clots from forming around my St. Jude mechanical heart valve but also is responsible for all the nasty little
side effects.

And it helps me to go back and re-read this post about Coumadin from time to time because I quickly forget what the drug actually does to keep me alive and of her side affects.  So here is my Coumadin post...

----------

It happened again.  Out of nowhere comes a slow steady dripping, bright red blotches Jackson Pollock style across the open book page, table or as it was with yesterday's event, all over the shiny white bumper of the Lincoln.  The unmistakeable metallic scent of fresh blood fills my nose but as usual there is no pain, no twinge of a prick or sting of a slicing cut.  The ever increasing amount of afternoon sun brilliant, lipstick red blood splatter now has my explicit attention.

Warfarin and St. Jude Aortic Valve #Marfan

After being on warfarin, also known as Coumadin, Jantoven, Marevan, Lawarin and other brand names, for going on a couple years I now know and am familiar with the whole drippy routine.

First task is to find the source of the flowing blood.  Usually the best places to look are the toes, feet, fingers or head, depending upon where one is in the house or yard at the time the red splots start appearing.  Chances are if I am in the kitchen then the blood will be forehead presenting and a sharp cornered cabinet door probably open.  Yet kitchens too are notoriously awful places for the feet if the teens are in a hurry sweeping up glass shards of an accidentally dropped ceramic mug.

However if I am in the yard, I can expect the red alert will be the result of a garden utensil hiding in the grassy paths. 

Blood in the bathroom is primarily indicative of a head wound from the shower door frame and so forth and so on.

The key to this entire blood thinner/anti-coagulant lifestyle is to find the cut or poke quickly, before you loose a quart of life's thirst quencher.  So yesterday when the red blotches started covering the rear bumper of the Lincoln I headed for the bathroom sink, mirror, hydrogen peroxide and band aids. 

A big mirror will usually tell you right away about the blood's origin without an unnerving shriek the teens or my spouse usually emit as I walk by covered in red.

Laughingly I can honestly say, life on anti-coagulants is not really as bad as it sounds.  When I first started taking Coumadin I heard all kind of negative or bad comments like 'OMG!" and 'I can't believe you have to be on that horrible drug the rest of your life!'.  Other words of encouragement included statements like 'my mother's skin turned bright orange!' or 'my Uncle bled out before' and (really heard this one) 'Shit!  That stuff is rat poison!'.  Even a TEDx talk I watched about aortic aneurysms decried the blood anti-coagulant I was taking.

Understandably, Coumadin and I had a not too happy introduction.

But perception can be quickly changed once one acquires hands on experience.

Yes, it is true Coumadin, the anti-coagulant I was and still am taking, is used as rat poison.  However the more I researched this plant-based miracle drug, the less uncomfortable I became.

Don't get me wrong.  I  wish I never had to take any of the many medications I have to take.  But in reality the Coumadin scare was mostly hype.

Warfarin allows my St Jude device to function without clotting

Again, please don't get me wrong.  I am sure some people have bled and coded out from the drug's use, but a quick meal of fresh garden collard greens or broccoli should stop any bleeding as vitamin K is the antidote for Coumadin overdoses.  I am also sure that there are some Coumadin taking bright orange people walking around out there too.

But what was really interesting was finding out why Coumadin is used as a rat poison and the history behind the drug's discovery!

Back at the beginning of the twentieth century there were incidences of cattle dying after being castrated or dehorned, bleeding to death, their bodies unable to develop and implement the normal clotting and healing process.

Researchers at the University of Wisconsin, including Karl Link and clinics suspected that there was something in the cattle's diet was responsible for their inability to clot when wounded and bleeding.

Studies showed that there was a compound in the fermented clover the bulls were ingesting which interfered with prothrombin, the substance responsible for clotting action.  Further tests isolated a coumarin compound called dicoumarol.  Interestingly, though the clover produced the basic coumarins it was the fungi responsible for the moldy clover that actually converted the coumarins into the dicoumarol.

Without a medical use apparent, scientists and industry looked at and began using the dicoumarols as rodent poison.  When the rats ate the drug they bled out.  The substance interferes with vitamin K metabolism and as we know, vitamin K is essential for our blood clotting process.

One of the first human applications was Dwight Eisenhower when he was provided the drug after a heart attack in 1955.

Coumadin's other name, Warfarin, is actually derived from a combination of the words found in the phrase 'Wisconsin Alumni Research Facility' and the word 'coumadin'.

Coumadin or Warfarin is important to me and many others because the anti-clotting effects allow for foreign objects in our body to function with reduced risk for the potential of blood clot formation.  For me this means warfarin allows my St. Jude aortic valve to open and close for many years without sticking due to clot formation (that could be fatal).  Disconcertingly though, data shows that Warfarin use has its risks and mortality due to hemorrhaging does occur.

Yet to date my bleeding has been manageable.

I do wear a bike helmet when cycling and always take a cautious approach when participating in activities with potential for serious trauma, carrying a variety of tape, band aids and antibiotic ointments in my backpack.  Backpack and band aids go where I go, be it for a short walk or trip to the store or even over to a relative's house.

Checking my PT/INR is easy.  INR stands for 'international normal ratio' and PT is 'prothrombin time' and both are used to calculate the body's clotting time efficiency.  Usually I will ask my daughter or wife to take me to the neighborhood medical laboratory, about a mile away for my once a month PT/INR test.  The lab sends the results to my primary care doctor who reviews the results and then either adjusts my dosage or says 'all looks good' and I wait until next month for another test.

I take on average about 5 mg Coumadin each day.

And I've learned to type on the IPad with a band aid or two on my fingers, though this feat is not an easy one to become accustomed to.

Rest assured, I am not super excited about being on anti-coagulants for the rest of my life.  Yet Coumadin is not as bad as I first imagined.

I am super happy the obnoxiously loud St. Jude aortic valve in my chest is still working as designed, and appreciative that Coumadin helps keep the device from clogging up with fibrous clot material.

And the frequent red splots don't surprise me now as much as they once did.  Thank goodness for fermented cover.

Anonther Dissection or Kidney Stones? Marfan Syndrome Life

Surviving a large kidney stone's exit from the body can be particularly painful, very similar to a dissection but in a different location. 

Ouch!  Its a kidney stone and not another dissection!

My first experience with a kidney stone happened yesterday while we were visiting my parents in Tallahassee, about a three and one half hour drive from our home in Palm Coast.  Tallahassee is lovely anytime of the year and Granny and Grandaddy's house brings back so many memories to the two teens.

We left Palm Coast early Sunday morning, drove through Jacksonville and arrived in Tallahassee mid-day, making the requisite bathroom stops along the way.   Ironically as kids we'd need to stop all along the way during summer vacation trips.  As our bladders grew to adult sizes those wee-wee pullovers seemed to vanish.  However to our dismay they'd return with a fury once we hit the 'big Five O'.   We began to think of interstate rest stops as oasis on the asphalt ribbon's horizon, shimmering mirage vapors calling to us to pull over.  Thoughts of the previously unimaginable began to appear, frightening hallucinations of adult wee-wee pullovers morphing into adult wee-wee pullups.

Delightfully though, our hotel suite was comfortable and they proprietors even served shelled walnuts and raisins as part of each morning's breakfast.

Fatigue had been my constant nemesis the past several weeks and so the evening of our arrival I gave the blame for my exhaustion and desire to curl up in a fetal position to my dissected descending aorta and weak heart.  I quickly remembered my latest bout with chronic fatigue in South Florida where my headed started spinning and I laid down on the concrete, thinking a new chapter in the whole scheme of things was about to occur.

Every thing seemed OK for a moment as I laid on the bed and I pulled the additional, extra small hotel pillow into my abdomen area.  A good night's rest and the fatigue will go away, I told myself.

Monday morning arrived and the fatigue remained and now accompanied by sharp, constant pain in my lower left gut.  Metamucil was back at home and the characteristically delicious travel food like, pizza, chips and snackaroos had been my weakened resolve's choice along the way to Tallahassee.

Judy always packs water, nuts, dried fruit and those really good for you foods before we leave on a trip.  Funny though how scrumptious colorfully packaged food looks stacked neatly on the gasoline station shelves.

The pain seemed to resolve with the morning's activities and the family spent a wonderful day on the grounds of Walkulla Springs, a Florida State Park.   The once crystal clear waters now stained brown with fingerprints of adjacent housing developments and shopping centers are still a great place for an afternoon swim.

Our two teens, Jincy and Ruairi, love to swim.  Their already dilated aortas probably aren't up to jumping off the high-dive platform into the mysterious waters below but what can you tell a teen, (especially a 'Marf Teen')?  In spite of their limitations, their passion for life is as unquenchable as the river basin's thirst for cool cold spring waters. 

Ruairi, 15 after a jump off the high dive into the deep spring

Jincy, 17 and her cousin Suzie going off the top platform into alligator infested waters below

Hotdogs and jalapeno covered cheesey corn chips beckoned from behind the lodge's quaint little diner and Grandaddy even talked me into a double chocolate mint ice cream cone.

Later, back in the hotel room, we were all beat, dog-tired and ready for a shower and bed.  Judy reached over to kiss me goodnight.  Uncharacteristically I pulled the sheet up over my head and told her to leave me alone.  The chronic fatigue beast had returned.  Lethargy was swallowing me up and all I wanted to do was tighten up into the fetal curl, hidden from the world under hotel sheets.

Sharp pain cut through my lower stomach as morning light streamed between the heavy dark curtains. 

'Absolutely no more junk food for me on this trip', I told myself as I had a zillion times before, 'especially those yummy jalapenos'.

'Ohhhh!' I grabbed by stomach and practically ran out of the room, down the hall to the bathroom downstairs by the hotel's fitness center.

Hotel bathrooms are usually always too small.  They are especially too small when they don't have a vent fan.  I always scope out a nearby hallway bathroom when staying at a motel.  The ones near the lobby always have an exhaust vent fan and are usually much more spacious and with insulated walls where those in the adjacent rooms won't raise their eyebrows in surprise.

Ten minutes later and back on the hotel bed the searing pain I was back in the fetal curl.  Pain was not going away.  This was not a throbbing pain but a deep, continued hurt.  I wondered if my left iliac artery was now dissecting.

Herein lies the horrible curse of living with a connective tissue disorder.  You never know what is happening deep in your body.  The sharp pains can be a strained muscle or they may be your aorta unraveling, death moments away.  Flip a coin.

The constant fear of imminent death gives way to a level of acceptance for some, but the adrenalin is always flowing despite beta-blockers and other medications flowing in the bloodstream.  Comparing notes in a support group is one of the only reliably available tickets to sanity.

I headed back to the bathroom.

Five minutes later and with a toilet full of bright red blood and cramping pain I knew it was time to go head out to the emergency room.  For a brief instant I thought of calling 911 but the hospital was only three miles away and if this was a big dissection or aneurysm I really wanted to be in control of my last few moments.

Judy didn't hesitate when I told her I wanted to go to the ER.  The teens would finish packing for the return trip to Palm Coast and we'd have Grandaddy pick them up.

When Ruairi asked me on the way out if I was OK, time for me froze.  If I told him I was scared that another major dissection was underway, his worry and stress levels would skyrocket.  But neither could I lie and tell him everything was OK.  

After considering all possible answers I blurted out, 'Going as a preventive measure, son'.  Children with Marfan parents have it just as bad as parents with Marfan children when it comes to worry about the other.

Fortunately there were few in the emergency room waiting room and after I explained about my descending dissection, aortic valve and Dacron graft, the nurse quickly hooked me up to the ECG unit and found me a room.

Despite the best accommodations, going to the ER while on vacation is not my first choice of activities.

But I was totally impressed with the professionalism of Tallahassee Memorial Hospital staff, their courtesy and manner with which they conducted their emergency room operations.  One of the first tests doctors want to run on me when I have an incident is a CT Scan with contrast dye.  They usually don't even ask me about the potential for contrast dye damage to my kidneys.  I was amazed when Dr. Draper asked me about my ability to withstand contrast dye.

Renal failure had hit me during previous surgeries, so I asked if he could administer a prophylactic medicine before the dye.  Without skipping a beat he told me he understood and would try the CT scan without the IV contrast dye at first.  The dye would be only used if the radiologist could not read the first non-dye scan.  Instead of IV dye I drank several large cups of a bland tasting watery substance.

First he rolled me over and took a stool sample to rule out blood in my colon.  You have graduated with flying colors with a degree in humility after having to raise your butt high in the air wearing nothing but a floppy hospital gown so the medical professionals can inspect goods along the various body ports of entry.  Pride is a word no longer relevant.  Anyway, when you are in that much pain you don't care what is being done to you as long as the hurt goes away.

As usual, when terrible pain hits, the words 'aneurysm' and 'dissection' roll around and around my mind.  I can't tame this fear.  Despair experience of the unknown hurt from my previous dissection always stays fresh on my heart. 

I don't run to the ER every time a new hurt appears.  If that were the case I'd be in the hospital every day.

But when a deep chronic pain sets in, one obviously not going away and one associated with lots of blood, I know it is OK to be proactive, cautious and seek out medical help.  So what if someone else labels me as hypochondriac.  Staying alive can be a tricky job sometimes.

Arriving in the CT scan room my bladder felt like an over filled water balloon.  After the CT scan I ran to the toilet, my hospital gown flying high in the air behind me.

Relief can be an unappreciated word until one is in the position of finally finding an available loo when when carrying around more sprayable liquid than a fire truck.

But this time it was a mixture of blood clots and bright red blood.  'Oh oh', I thought.  Could my renal arteries, already dissected be pumping blood directly into my kidneys?

I knew the aspirin and Coumadin in my body didn't help the situation any, and in fact had grabbed a handful of fresh spinach leaves before leaving the motel room fro the ER, hoping they'd help with clotting.

'Doc, I am bleeding badly', I said, pitifully upon return to the ER

'That may be a good sign' he said.  'Blood in the urine and the CT scan tells me your problem is not aortic in nature.  You have a really big kidney stone!

'Beam me up, Scotty' was all I thought.  No longer on the edge of death, I was instantly transported back into the realm of the living.  Such is the life of someone with a connective tissue disorder like Marfan Syndrome.

I am so thankful to the staff at Tallahassee Memorial Hospital, the doctors and my family. 

Not only is living a breath away hard on me, it is just as terrifying for my family, especially the teens and my wife and parents.

The stone must have cut its way out during my CT scan wee-wee episode, landing in the comode with all the blood clots.

Discharged and feeling so much more alive, I walked out of the hospital to the car.  Judy drove me to my parent's house where the family who had been expecting an extended stay in the hospital were so surprised when we walked in.

Now I know what the symptoms of a kidney stone include.  Next time I have lots of blood, excruciating pain, fatigue and lethargy I will think kidney stone.  But I will also still probably think aneurysm, or another dissection, or ..........

Such is life with Marfan Syndrome.

The Chronic Fatigue Beast and Marfan Syndrome

Chronic fatigue (CF) is a cruel beast I face off with each day.  Some days I win.  Most days the fatigue succeeds in siphoning away any energy I woke with and I want to crash.

Chronic Fatigue and Marfan Syndrome

To those of you who have experienced chronic fatigue this note doesn't shed any new light on the relief of symptoms.  I have tried everything the doctors and my friends have recommended and ended up with no significant relief.

And I can't find any Vitameatavegamin in Palm Coast stores.  (If you have never watched this I Love Lucy episode you are missing out on so may laughs.)

Maybe you are not saddled with chronic fatigue.  If so, rejoice and let us all hope your good fortune continues.

Not sure you're experiencing CF?  Don't worry.  Once you've experienced CF you will know you've been there.  No doubt.   Chronic fatigue is not the normal ups and downs associated with everyday life nor does CF go away with rest.

CF manifests itself in my life with many of the same apparent symptoms as depression.  Some days others assume I am depressed or 'down in the dumps' because of delayed responses to questions or lethargic appearance.

Coffee sometimes helps me for about three or four minutes.  Other times the java won't even begin to help with the apathy.

Last week I rode to Sarasota with two others, the first lengthy long road trip since my dissection surgery.  We left early and the van was quite comfortable.  However once we reached our destination around mid-day I was so exhausted that I sat down on the concrete pad we were gathered on, leaned back against a post and closed my eyes, ready to pass out.  One of my traveling companions who knew about my health issues walked over and took me back to the van.  I had been overwhelmed with a desire to lie down and let the exhaustion envelop me like fog on a wet, humid morning. I wanted to give in to the CF beast and let it devour me.  I did not care about anything but closing my eyes and sinking into the mental and physical fog. On the trip back my memory issues manifested themselves as they do occasionally and I could not remember names.  Two or three days follow up rest were needed to recover from a simple road trip.

Doctors say exercise can improve circulation and stamina and help prevent CF.

And for sure I try to exercise.  Walking, swimming and stretching keep my heart in 'crawl forward' mode and out of park or reverse.

According to Mayo Clinic, chronic fatigue has eight official symptoms.  I am sure there are others.  The eight CF symptoms are:

• Fatigue
• Loss of Memory or concentration
• (Persistent) sore throat
• Swollen lymph nodes in the neck or armpits
• Unexplained muscle pain
• Pain in joints without swelling or redness
• Strange headaches
• Unrefreshed sleep
• Extreme exhaustion lasting more than 24 hours after mental or physical exercise

Mayo Clinic also suggests three possible causes for CF, including;

• viral infections
• immune system problems
• hormonal imbalances

I think the mixture of beta-blockers (Metoprolol), blood pressure medicine (Losartan), Coumadin and other medications may be partially responsible for my chronic fatigue.

Because my descending aorta is dissected from the ascending Dacron graft down into my legs, the doctors keep my pulse below fifty beats per minute.  A slower heart beat means less oxygen available to muscles, organs and my brain.

Additionally, my heart output function has been calculated to be around twenty or twenty five percent according to my cardiologist.  Trauma from multiple surgeries, new valves and synthetic aorta material make my heart's job exponentially more difficult.

Interestingly, though existing literature on the topic is insubstantial, I have found that as my PT-INR rises significantly higher than normal, my fatigue also increases exponentially.

Though the medical profession has not definitely determined specific causes responsible for CF, chronic fatigue is real and debilitating.

Many of those challenged by Marfan Syndrome and other connective tissue disorders must not only adapt to weakened muscles and joints, scoliosis, leaky heart valves and aneuryzed cardiovascular tissue but also cope with continued depletion and exhaustion.

CF has opened my eyes to better understand what a zombie's life is really like.

Though I believe chronic fatigue is not the same as depression, I do consider CF to be a primary cause of situational depression.

As mentioned above, there is no doubt when one is faced with chronic fatigue.  Like I tell people when they ask me how I knew my aorta was dissecting, "there is no doubt', the same holds true for CF.   I'd add the following symptoms to the above list:

• regular inability to breath deeply enough to fill your lungs
• driven need for multiple short naps during the day
• unusual weight gain or loss
• lack of motivation to participate in activities one normally is interested in
• when placing a phone, sending a letter or writing an email call to a family member or friend becomes seemingly overwhelming from an energy perspective
• and others.

I fight the CF beast every day.

Though it would be so easy to give in, close the blinds, turn down the thermostat a couple degrees, crawl under a quilt and go to sleep, I am not willing to surrender yet.  I will not willingly become a zombie.

When my son asks  if I will go to the skateboard park with him I go.  When my daughter says, 'Dad, let's go to the grocery store to buy a gallon of milk' I ride with her.  When my wife asks if I will go to the beach with her, I go, even with the CF beast hanging on my back, beating on my head and biting my neck.

My battle plan is simple.  I'm first going to learn what circumstances invite a CF attack.  Journalling about daily activities is the best way to establish CF patterns and trends.

I know I must continue to exercise.  However when I stand to walk outside and my ankle feels as though it is falling apart, the pain invites fatigue and fatigue then invites lethargy.   Even so I make myself walk or swim, even if it is only for a very short duration.

Healthy foods are important too and this is an area where I am diligently journalling and researching in an attempt to find food patterns and their respective relationships with chronic fatigue and connective tissue issues.  Potassium, phosphorous, saturated fats and certainly sugars all have shown themselves to be somehow related to my CF incidents.

Understanding the CF beast will be a journey across time and one without a clearly defined destination.

Unfortunately there are no easy answers.

Rest is not the answer.  Though daily rests provide respite for my mind, most naps I take produce additional physical fatigue.  I usually wake up so much more tired than when I was before I went to sleep.

Cutting back on medications is not an answer either.  Beta-blockers and blood pressure medications keep me alive.  I have changed medications before and this route has not provided real CF relief.

Exercise and diet may hold promise.  As I learn from food patterns I will update the post here.

In the meantime, I am not giving in to the CF beast.  I am not going to become a zombie.  I am going to beat the beastie fiend as soon as I find the energy to do so, or locate some vitameatavegamin.







 

Disability and Driving Privileges, Killed with Beauracracy

One thirty am and no sleep in sight.

Overhead the ceiling fan slowly whirls and I get up to pull the speed chain.  Spinning blades whistle a little louder and the rocking back and forth of the fan's base against the sheet rock is an annoyance I came blame sleeplessness on.

The State of Florida is killing me softly, with their unending, unrelenting songs of desire.  They want more and more and more.  And with the leisurely bright blue digital numbers staring unconcerned from behind the alarm clock's plastic faceplate, Florida's government is accomplishing what my torn aorta could not.

I never expected being disabled to be easy, but maybe I hoped life would become manageable.

Being a person with disabilities has a full slate of new and additional challenges and challengers, not the least for me being the State of Florida.  You'd think the state would want to help make getting through the daily challenges more basic, effortless.  But that is not the way it works, at least in my present experience with the driver license bureaucrats.

I've been a good driver over the past many years.  I am even a better driver today because of my surgeries.  Today the second letter arrived from the Department of Motor Vehicles (DMV).  Today's DMV letter is the reason I am staring at the ceiling fan instead of dreaming.

The first letter arrived about two months ago (visible via Google Drive here), signed by a Hearing Officer with the Bureau of Administrative Reviews requesting my presence in their Daytona Beach office to discuss 'your ability to safely operate a motor vehicle'.  I suspect the title, 'Hearing Officer', is an attempt to satisfy due process requirements.

Life for all the disabled is hard enough already. 

For me there are quite a few medications to buy, organize, record, take and then coordinate refills with the many different prescribing physicians.  There are frequent PT-INR and other blood tests, iterated blood pressure and pulse readings, prescribed exercise and therapy now and also the task of trying to self diagnose the strange and somewhat disconcerting new pains that occur daily.  Going to the doctor or ER for each unexplained serious symptom is simply too difficult logistically.   Coordinating with insurance and the various doctor's office billing companies is a full time job in itself.

Though taken individually some of this may sound easy, cumulatively the load can be overwhelming, especially for someone who has a hard time somedays walking out to the mailbox.

My primary care physician says 'your job is to stay alive'.

And so we've simplified, trying to keep stress levels to a minimum.  Yes, the beta-blockers like Metoprolol help, but only to an extent.

And I really stressed out thinking the State of Florida would take my driver's license after the first letter arrived.  The day of the scheduled appointment arrived and I sat down with the Hearing Officer who asked me (and recorded) a list of questions about my condition and my medications.  I provided her with the completed medication chart as requested by the State of Florida.  She also spoke with the other driver who accompanied me about their perception of my ability to drive safely.

Ironically, there are some people out there right now who can not clearly see three feet in front of them with driver's licenses.  They are the ones the State of Florida should be after.

The Hearing Officer had me sign a medical release form and collected contact information for my cardiologist.  She informed me the DMV would contact my doctor and once the medical review committee met to consider the information a decision about my license could be rendered.

My cardiologist called me to confirm receipt of the form and tell he would submit the required information to the state.

I made the mistake of thinking the whole exercise was done, complete and history.  Wrong.

Today another letter arrived in the mailbox from the State of Florida (check it out here). This letter contained a much more detailed medical inquiry form.

My cardiologist had told me point blank he supported my driving and made clear his opinions in the form the State of Florida previously required.

The new form was to be completed by a 'personal physician', one I am assuming to be my primary care doctor.  The letter was also accompanied by an informational brochure explaining why someone might be 'red-flagged' as an unsafe driver because of medical conditions.

But as the ceiling fan whirled overhead between one and two am this morning, the new form questions reverberated back and forth through my head.  The State of Florida is killing me softly as I lay here with adrenalin hitting my heart despite Metoprolol.

Why does Florida want my personal physician to state my education level?  Why does Florida want my personal doctor to tell them if I am have been declared disabled?  In my opinion the form is extremely confusing and serves only to duplicate the essential information already submitted on my behalf.

Life for the disabled is hard enough already.

This new form going to be time consuming to complete - I'll have to try to schedule a checkup appointment, explain the circumstances behind the forms, make sure my primary physician office coordinates all records with my neurologist, ophthalmologist, cardiologist, nephrologist and others, complete the forms and submit back to the state within the 45 day limit.

Most other aspects of my life will go on hold now for a couple months, after which the real waiting starts.  I am sure phase one will lead to phase two, for as here it always does.

And time is not the only issue with being caught in the bureaucratic system of forms and computerized phone answering systems.  I can ill afford to spend the significant amount of money these unplanned doctor's visits will end up costing.

Anyone can report another for suspected unsafe driving circumstances here in Florida.

However, I am not sure how I ended up being red-flagged.  My license says 'Safe Driver' and although my short term memory sometimes lacks, the reason I can't remember my last ticket is that my last ticket was well over fifteen years ago.  Since speculation leads to stress, I am not going to worry how I ended up being red-flagged in the system as a potentially unsafe driver who needs to be medically examined over and over to keep his license.

As I've said here before, my heart functions about twenty to twenty five percent of capacity and I deal daily with the implications of a connective tissue disorder and an aorta that is still torn from the top of my heart, down through my abdomen into my leg.  

Life for the disabled is hard enough already.   I think anyone who has struggled with long-term, chronic disabilities may agree.  Life is hard enough without government making life more complicated for the disabled.

In the meantime, the State of Florida is still killing me softly, regardless of how hypnotically the ceiling fan overhead whirls.

Depression and Marfan Syndrome

Writing this post about depression has shaken most every core belief in positive thinking I've held and drastically changed my expectant outlook on life.

Rudbeckia hirtia, Black Eyed Susans setting seed

First let me unequivocally make clear I am not a medical professional and have no qualifications to write about depression and Marfan Syndrome other than from the perspective of someone with Marfan Syndrome who has experienced significant depression lately.

From the time I began thinking about writing a note on feeling down because of chronic pain associated with Marfan Syndrome and connective tissue disorders (CTDs), a disturbing darkness kept me away from the computer keyboard each and every time I was compelled to write.

 I even closed my Facebook account for a week, wanting to hide from all my family, friends, the world and the God who I wondered about, daily asking myself "why, if God was so good, would God not take away pain, depression and suffering?"

My sullenness and dark withdrawal were perplexing symptoms for me to comprehend as I am someone who usually exhibits an upbeat, "it will all be OK", outlook on life.

How many times have we heard "our lives are products of those thoughts we harbor"?

Our words, especially with respect to health and wealth, are supposed to reflect our actual circumstances. Yet in my Marfan Syndrome framed life vocally claiming health and wealth principles never really proved to have a real payoff.  Some said my faith was not strong enough.  Some said my God must not be the God they knew, my unfulfilled expectations a reflection of personal failure.

But I tried to put on a happy face and keep a can-do attitude.

In reality my upbeat attitude for years was mostly buoyed through wildflower photography, hiking (when I felt up to a walk) and music. I so love music, all kinds of music! Being near the ocean helped too. Working with Judy work in her garden made me happy too, before the big dissection.

In the aftermath of my multiple open heart surgeries and the onset of a host of other connective tissue disorders, my upbeat attitude started rapidly changing to more of a downbeat persona.

For a time I tried to stay positive, focusing on my wildflower photography and hiking along the seashore. But the petals, stems and stamen are not a healing God and neither was the ocean. As spring turned to summer the beauty of our Black Eyed Susans and most other wildflowers shriveled to a dried up brown. Seasonal tropical storms washed up endless stretches of trash across the beach.

My nature based emotional crutches were temporal.  Nature's beauty, music, prayer and exercise were all great tools for keeping my spirit lifted but they were only temporary in nature.  I needed a permanent fix to my deep melancholy.

Pretending only goes so far and chronic degenerative diseases are not imaginary. Realization my answers to gloom did not lie in photography, walks, distractions, expectations, faith or hope was disheartening.

I did not look sick and this made things worse. People would look at me when I parked in a disabled spot in a way I shamefully perceived as someone taking advantage of crippled elderly folk by stealing their handy parking spot.

I wanted to shout out that my heart functions at twenty five percent capacity on a good day and when I try to do something with my teenage son like handball, I walk across the court relying on strategy rather than athleticism (and still get beat).

Parking close is important if I have to carry any weight out of the store. The weight of a watermelon could fully aneuryse my existing dissected descending aorta.

I am not going to get better without additional major arterial surgery. Unfortunately and discouragingly, the doctors have talked along the lines of the required surgery being 'too risky'.

Then there was the lady who tried to be encouraging by saying something like 'getting a job would be good for you because I know you really want to support your family'. I wanted to tell her that I fall asleep for hours during the day when my heart has reached its energy limits or retort under my breath 'walking a mile in shoes'. And I do support my family through all the taxes I've paid in over my lifetime. I sincerely appreciate my disability but it is not a gift, I paid for it over thirty years.

The way people look at you sometimes when one is on disability is enough to give self esteem a black eye. 

Moreover all those daily pills and medications that kept me alive also went a long way towards killing my spirit. Jumping for joy and crying out in ecstasy was difficult when my heart was beating only once every one and one half seconds, slowed by beta-blockers and angiotensin II receptor antagonist drugs.

Pain is real for those with CTDs like Marfan Syndrome. CTD pain is sometimes debilitating beyond imagination. Fear of not knowing what the answer to the body's woes is also immobilizing. Happiness is hard to find when CTD hurt is strong. Smiles fade to frowns and laughter to moans when pain and fear induced depression sets in.

Every time a new hurt would shoot through my leg, back or abdomen I'd instantly travel back to the moment my ascending aorta dissected and aneuryzed. Panic would override the beta-blocker medication effect and my heart would race out of control, ventricular tachycardia setting pulse's pace.  New pains brought new fears and more darkness.

I'd wonder (and still do) if my still dissected descending aorta was completely rupturing and how many seconds were left before I fell to the floor, bleeding out internally, way too quick to be driven to the ER. After so many of the same episodes and so many reruns of my life flashing before my eyes I instead began to smirk and whisper "que sera, sera" but inside I felt like crumbling. 

On top of the very real pain issues lie the confusion of forgetfulness my neurologist calls mild cognitive damage, a result of collateral embolistic events occurring during emergency open heart surgeries.  Fear of unknown maladies was magnified as I sometimes could not accurately describe to others what was happening or even remember if I'd experienced the pain before.

I have a hard time with word recall and especially with short term memory. As a writer passionate about blogging, not being able to find the right word is a crushing disability. Many times I can see the thing but cannot remember the word. As a lawyer by education, not being able to open your mouth and express what you want to say is a career killer. More good reasons to slip into the depths of a fathomless dark void of despair.

Getting used to drooling, stuttering and speech difficulties was not an encouraging exercise in joyful life anticipation either. I tried to hide my sadness. Hope prevailed for a while, until my spirit could not find the energy to animate another upbeat moment.   Hope then became just another unfulfilled expectation.

The time had come where I was finally tired of pretending I was not depressed. So I thought I'd write a post article on the topic and possibly I find help there.

Maybe I'd gain insight. Perhaps someone else out there would have an answer.

First action I took was to message some of my friends and acquaintances who I knew were confronting chronic debilitating and even potentially terminal illnesses, asking them how they dealt with depression.

The three questions I posed were:

Question One: Why do you think connective tissue disorders (CTDs) are so prone to having depression associated with them? Is this a physiological, chemical or emotional issue?

Question Two: How do you think depression and fear play off each other in people with CTDs?

and Question Three: Any other thoughts on Marfan, CTDs and depression/fear?

Expecting many thoughtful answers to my question, as I had received many times before on other inquiries, I ended up receiving only a couple of replies.

I felt shunned and even more depressed. Probably those who I asked to share how they dealt with depression had a similar response as I had and wanted to distance themselves from a depression discussion.  Seriously, who wants to talk or read about depression?  I hid mine for quite some time by appearing to be so upbeat. 

But the silence was disheartening. My perceived expectations were once again shattered and I sunk even lower in spirit, feeling like I didn't want to write again.  Totally out of character with my past, I even put my beloved camera away on the computer desk.

Then, over the next month a couple things happened that began to help my understanding of my blue disposition.

First, I started to receive feedback from those I'd solicited about fear and depression. Their replies alone provided me with a spark of enthusiasm.

The initial reply came from Pamela L. Evans-Schink MA, LMFT, CCDVC, a Marriage and Family Therapist turned homeschooling mother of two boys. Pamela practiced psychotherapy in Connecticut and New York for ten years before moving to California with her family. Both Pamela and her two sons are affected by Marfan Syndrome.

Mrs. Evans-Schink had the following responses to my queries about depression and Marfan Syndrome;

Question One. Why do you think connective tissue disorders are so prone to having depression associated with them? Is this a physiological, chemical or emotional issue?

Answer:  I think People with Marfan Syndrome or another Connective Tissue Disorder are frequently in a great deal of pain. Living with constant pain is both physically and emotionally exhausting. Commonplace activities become insurmountable. Favorite activities are given up. There is a great deal of loss and mourning of the individual's previous life.  Often times, people in the affected person's circle don't understand the pain and exhaustion because it is not visible and they have not experienced it themselves. Sadly, this can sometimes include doctors and psychotherapists. I think it is especially unfortunate when a doctor or psychotherapist thinks that treating the depression will eliminate the pain. If the depression is a result of chronic, physical pain, then the providers are essentially chasing a ghost if they do not understand both the psychology and physiology of someone with chronic pain and illness. This lack of support can lead to a feelings of isolation and failure in patients, which would contribute to depression.

Question Two. How do you think depression and fear play off each other in people with CTDs?

Answer:  With Marfan Syndrome, the depression might also be accompanied by fear. Because of the serious cardiac risks involved with this syndrome, people might fear for their lives. People might wonder how long they have to live or if they will survive Cardiac Surgery. Surgeries can be terrifying. And for some, there seems to be no end to surgeries as various organ systems become affected and require repair.

Question Three. Any other thoughts on Marfan, CTDs and depression/fear?

Answer:  Another contributing factor for parents who have Marfan Syndrome or another CTD who have an affected child or children could be guilt. Patients with Marfan might become overwhelmed with the guilt of passing on the gene mutation to children. It is especially hard to watch one's children suffer. Having your child or children be affected by a complex, incurable condition is a terrifying prospect no parent should have to face.

The feelings of guilt and fear for one's children could add an extra dimension to the depression of affected individuals.

Depression can also be a side affect of many medications. Many Marfan patients are on a whole host of medications, including heart and narcotic pain medications. I might add that certainly some individuals might suffer from depression for other reasons as well.

Depression is a common condition that affects the general population and should not be taken lightly, whatever the reason.

Mrs. Evans-Schink's comments concerning fear hit home with my dread of the unknown symptoms, a worry that my pains could either be a cramped muscle or the deadly aneurysm in my descending dissected aorta. Others responded and I became somewhat encouraged.

Soon I even retrieved the camera from the closet, venturing outside to photograph Judy's backyard wildflowers. Other respondents agreed depression and chronic illnesses such as connective tissue disorders and Marfan Syndrome run hand in hand. Coping mechanisms included meditation,  exercises, healthy and cleansing dietary practices, fasting and prayer for depression.

The second important event part my recent bout with depression occurred as I watched a patch of Black Eyed Susans in back yard turning brown and starting to look ragged and withered. At first I sneered and lashed out at God and the universe for creating complex, beautiful life only to allow way too soon dying and withering to then take away forever.

Like the flower, my life had so much promise even in the recent past. But after my dissection nineteen months ago and unfulfilled expectations manifesting themselves with the chronic pain of connective tissue issues, my thoughts became more despair based.

Sometime after I tried to start writing again, through a variety of conversations and interactions with college friends, family, those friends in social support groups and others, a thread of answers about my depression questions began to coalesce.

My friend from college, Bob suggested that God is actually here and that God's voice can be heard in nature, as in the sounds of a flowing creek.  A couple days later on a hike out through Princess Place Preserve a strong, gentle wind came up from behind me, surrounded me and I really did feel the hand of God rubbing my neck and back, holding me with love.  I wanted immense amounts of love and if God really was love then maybe I'd found a source.

I started doing more with my teens, playing handball with Ruairi despite the pain, going fishing with the kids, continued calling my Mom and Dad every night to tell them I love them and spending time with my father-in-law over coffee.  Judy and I would drive to Malacompra and listen to the Atlantic waves crash over the weathered coquina stone.

Another of my college roommates reached out with kindness. Those wonderful friends in the Marfan support groups across the world too were there any time help was needed.

For the brief while my Facebook account was closed during my depths of blueness, they reached out in love to my wife to express their love and concern for me.

And my wife Judy was always there, allowing me space and offering unconditional love as she always has and always will.

Maybe, through no one's fault but my own, I'd been missing out.  Perhaps all my unfulfilled expectations could be satisfied here and now.  I started thinking that maybe I was looking for something in the future that was already here and all around me.

Two things began to stand out. The first was the idea that finally reaching some destination was not the deciding factor for achieving happiness and avoiding depression. Rather the key was appreciating good things in the journey we take through life here and now.

One of my mantras quickly became "focus on the journey not the destination". This was critical to turning my blues around. I'd been in a "I don't have" and expectation mode most of my life.

Now I've ditched expectations and instead am in the thankfulness mode. When expectations and anticipations failed to materialize, depression overtook me. But when I ditched the expectation mode and started appreciating the here and now my attitude began to change.

I did not have to wait for Heaven or God. The good (sometimes super duper good) was here now too, woven in with the bad (yep, sometimes really bad). Instead of stuck in the bad waiting for the good to come I finally saw that the good is already here.

The dried, withered and brown petals of the dying Black Eyed Susans held hundreds if not thousands of seeds for future life. Before I saw only a loss of youth and beauty in the late summer wildflowers.

Wildflowers bloom, set seed and die.  This is life.

Now I see magnificent complexity and beauty in the brown and dry and in all the seasons of life. Same way with the broken stem flower and the torn wing butterfly searching out for remaining nectar traces. Heaven and happiness is here right now and we don't have to wait, we just need to see the good.

Sure there is the chronic pain and many daily disappointments, but no longer do I need to wait on unfulfilled expectations. I am surrounded with such wonderful friends, family and an amazing world. Learning to love the journey really has been an experience that has freed my soul.

No longer am I chained to a constant disappointment from missed or unfulfilled expectations. Focusing on the beauty of the here and now, even if there is collateral pain and suffering, has freed me. For me Heaven and God are here now. And this is different than the 'claim health and wealth' mentality that I flee from.  For I now recognize that the pain and despair are real and here and not going away just because I invoke a supernatural name or phrase.  But I can handle the bad because unlike the 'claim health and wealth' mentality, I now don't have to wait for the good.  All I need already surrounds me.

The love of friends, family and even strangers is the ultimate fulfillment of any anticipation. A life of happiness was always here. I just did not see it.

Instead I saw dark depression.

Now I seek out love.

But I think I am lucky. I think I may have a severe case of situational depression brought on by my chronic pain and living on death's doorstep each heartbeat, moment, hour and day. Others may have imbalances in their body's biochemistry that require much more serious medical intervention.

Don't get me wrong. I did not solve my depression issues with the grasping of some Aristotelian logic surrounding the concepts of love and being here and now. I still sink into fear and despair when a new throbbing pain surges through my body and I wonder if the time has arrived.

I still find myself down in the dumps when thinking of giving up Scuba and other life activities. Sometimes I lie awake at night for hours listening to my mechanical heart valve tick in time and rhythm to some obnoxious song stuck in my mind.  But great love overcomes all else.

And there are still way too many unanswered questions I have about what is happening to my body. For instance, my right foot has grown two sizes in the last year while my left foot has shrunk a size or so, presumably due to my dissected descending aorta. My cardiologist didn't have a clue and neither do others I've asked.

Yet while the fear of the unknown remains and causes me grief on a daily basis, I don't waste my time looking for hope or fulfillment of my old expectations anymore. I've found Heaven now, today, here on earth manifested in the love of my wife, my family and my friends. This love is Heaven. This love is here and now. This love is the fulfillment of all my expectations.

So like the Black Eyed Susans, the wilting and withering are real and not going away. Neither is the chronic pain those with connective tissue disorders have.

But whereas pretending only went so far before and the answers to my gloom did not lie in distractions, expectations, faith or hope, I now know where to look.

I think the answers to my questions about beating my depression lie in the indescribably magnificent love of those individuals touching my life yesterday, today and forever.