Getting Through A Connective Tissue Disorder Day

My embolistic hit brain needs things laid out clearly or panic, vertigo and unending uneasiness set in.  I sit down then, put my head in my hands or lay down under the window and become immobile.  At least I want to.

My Connective Tissue Disorder Life Day-Bloom

Many times the lack of clarity leads me to wander around the room or house through a confusing mental fog.  I need structure and order during my day.  I need an easy to understand roadmap that helps me keep moving forward, something to held avoid the confusion stroke related dyslexia and ADHD brings.

So when I lay awake at night staring at the stars out the window, I see my day and my week and the year in the form of a big circle, like a clock, probably due to looking at clocks now for fifty seven years.

Even fresh ground coffee in the French press does little to clear out the fog.

But pictures so help.

Since I love flowers I thought I would take the 'clock' concept of a day and turn it into a blossom, a bloom, a 'day-bloom'.

I have printed my day-bloom out and attached to the kitchen refrigerator.  The kitchen refrigerator is an important place to me because it is where I usually end up after wandering around wondering.

When I open the stainless door a flood of bright light rolls out of the cold ice box.  I usually stand still for several minutes mesmerized by the potential snacks and cold air enveloping my feet.  Then the thought of sardines in the pantry and the humming sound of the refrigerator compressor kicking in shakes me out of my zombie like state and I shut the door.

Now my day-bloom will be there, right in front of my face to remind me of what I think I need to be doing.

Yesterday while riding in the vehicle with a friend I kept falling forward and couldn't keep my eyes open, probably because events of the last couple months have given me an excuse for not focusing on my day-bloom schedule.  Remembering the doctor has told me that my number one job was to 'stay alive', I am going to try and stick to consistent physical therapy, exercise and rest.

Using pretty pictures is more fun than a bulleted list.

I can't even seem to keep my eyes open to write this post and it is only 10:20 am.  Struggling with the side effects of the meds controlling a barely beating but loudly beating heart when it is behaving is tough.

Now, life seems like  it may contain only a few really important things; like family, friends and Love.

My day-bloom reminds me of this.

Even when I do not have my reading glasses with me.

Because I can see the hearts and the smiling sun.  And one more smile for me.

Magna Cum Laude and Off to College, Daughter With Marfan Syndrome Overcomes Challenges

Jincy received the Magna Cum Laude award last night during her Mantanzas High School awards ceremony.  It brought a tear to my eye.

Daughter with Marfan Syndrome Graduating Magna Cum Laude

Although she is just seventeen, turning eighteen this summer, she gives me and so many others inspiration each and every day.

After dual enrolling in college while a junior and senior in high school, she faced academic challenges, but despite being several years younger than other students Jincy went on to achieve almost a 4.5 GPA where 'A' = 4.0.

This was notwithstanding the fact the fact that she was right in the middle of her dad's long, hard post dissection recovery period with multiple heart surgeries.  She subsequently learned that her and her brother's aortas were not only beginning to dilate but that she was also faced with other quite difficult Marfan Syndrome health challenges.

Jin volunteered with the Humane Society, working with homeless dogs and cats throughout her late teens, worked with orphans in Honduras and volunteered with Marfan Foundation benefits like the Tommy Tant Surfing Classic held every year in Flagler Beach.  She wrote poetry, taught herself guitar and ukelele and cleaned her elderly grandparents house.  As of late she has been a 'mother'-big sister to her teen nephew and niece whose mother recently suffered a brain aneurysm and has always tried to advocate for disadvantaged groups of people, no matter what their issues were.

Sure, she showed her spirit in ways I did not approve of, like the time when she climbed out her window, leaving a bunch of pillows under her bedspread and a pumpkin pie under the sheet for a strange looking head and face.  She climbed up to the top of our two story house when two years old, seriously frightening her mother and would take off as a toddler, crawling straight out into the ocean waves.

I envy her youthful and unquenchable zest for her adventures and life ahead, around the corner.

She wants to major in Communications while attending University of South Florida (USF).  Her studies will continue to be a challenge for her and she will need to work hard to support herself economically as well as academically.  I am thankful USF is providing as much help as they are.

But she can do it and the desire to overcome her challenges is what fuels her drive and successes.

I am so proud of her.  For now she has showed those Marfan challenges just who is boss.

A Marfan Easter In The Children's Memorial Garden

Marfan Syndrome Family Easter in the Children's Memorial Garden

Easter morning was wet, cool and drizzly.  We tried the sunrise service at Flagler Beach but the sight of the bright, easter-egg colored panties under the dresses of the ladies singing church songs and shaking tambourines, being blown up over their heads by the thirty mile and hour ocean winds scooting across the stage in the park, was not what I was looking for.  Give me a hot, black cup of coffee instead.

Easter Sunrise Service, Downtown Flagler Beach

So we went and had breakfast on the pier across the street, overlooking the Atlantic Ocean.  I love the big pond, a metaphor of eternity to me, much like the sky.

Flagler Beach and the Big Salty Pond

Sometimes I wonder if eternity flies by as fast as time here as we know it flies by.  Seems like I was just starting college last month, but it has been forty years.  I still haven't really figured out any of the answers.

The last year and a half has been a challenge in many ways for our family.  I wake every day to the same and new, different challenges with Marfan Syndrome problems, but bottom-line grateful I awoke.  Because I have finally realized life is so precious and tenuous I offer up a prayer of thanks and beseeching every night before I fall asleep.  If the night is to be my last I want to go out in a thankful mood.

Judy has been fighting her auto-immune inflammation and hopefully may have found some relief with an older acupuncturist here in Palm Coast.  Jincy and Ruairi forge ahead with school and social activities, and with all the competition in school I worry about their dilated aortas.  On an aside note, I do not understand why the State of Florida and Governor Rick Scott have upped the SAT and ACT score requirements form high to nearly impossible for Bright Futures Scholarships, without an exception for children also battling a disability challenge.  Jincy has over a 4.5 GPA and Ruairi a 4.7 GPA (A=4.0) because of they study so hard and do so much extra credit work.  Teens that work that hard, with grades that good all the while battling disabilities should have access to the state's lottery money supposedly dedicated to education.

Jincy and Ruairi have their own Marfan Syndrome Challenges, but they forge ahead, unstoppable-like

Jincy has a really good deal at the University of South Florida in Tampa which she has accepted, and I am grateful for her hard work.

Seems like the two teens time in high school just started yesterday, too.  And the grandchildren teens (son and daughter of Judy's second oldest daughter who recently had a serious brain aneurysm) Dylan and Dharma have been here six months in May.  Yes, time flies.

A year and a half has also passed since baby Heidi (Judy's daughter Leslie's baby girl) died.  Leslie and her husband still struggle with so much grief.  Shortly thereafter I hung a set of wind chimes on a beautiful scrub live oak in the Children's Memorial Garden overlooking the intracoastal waterway.  The memorial garden is full of wind chimes placed there by others in a child's memory.

Children's Memorial Garden Overlooking Florida's Intracoastal Waterway, Live Oak Nestled

The wind along the intracoastal is not as strong and brisk as the ocean front wind but still steady enough to keep all of the beautiful sounding wind chimes singing most of the time.  If I was a spirit child, I would go to that salty riverside place just to listen to the jingles and bells and wind softly blow.  As an old man, I also like to go there to talk to angels woven throughout the misty salt air.

But not only are there beautiful melodies sounding of a child's choir in the air, the garden is full of beautiful colors and textures, art and nature combined together in love.

Heidi's New Wind Chime is tied next to her Frog Chime in the Memorial Garden

Heidi's wind chime was showing signs of wear from the breezes and I wanted to get the four teens out of the house and into nature for their daily vitamin D dose.  I'd found a really cool dragonfly chine with just one dangle and figured it was just the chime to attach next to Heidi's original chime.

The four teens and I talked about thankfulness, remembrance, love, kindness, memories and a bunch of other esoteric topics I can't remember now, on the way to the intracoastal.  We also talked about baby Heidi and her parents and her brother.  I reached up and held the two silver hearts hanging on my medical ID necklace, hearts from my Mom.

The four teens, Dylan (Grandson 16), Marfan teens Ruairi & Ruairi (16 & almost 18) and Dharma  (Granddaughter 14)

One thing I don't want flying time to do is allow us to forget.

The teens and I enjoyed out time in the children's memorial garden.  We listened to the chimes.  They stopped and read all of the little memorial stones, pavers and bricks placed throughout the special nature walk.  They even reached up to dab a corner of their eyes when they though no one was looking.

Heidi has two wind chimes now.  Her frog wind chime is still there.  And someone else has placed a big green tree frog on an adjacent limb to look after Heidi's smaller frog chime.

New Big Florida Green Tree Watching Over Heidi's Smaller Green Frog Chime, Down By The Riverside 

And there is a beautiful red star in the next tree over.

Stars in the Scrub Live Oaks

As we left I looked down to my right.  Lovely easter eggs for the children here.

I think, despite my challenged cognitive state and memory, I think I will come back here more often.  There is much to learn from a special place full of native plants, overlooking the edge of an eternal ocean and full of children's twinkling and chiming voices, especially for an old man like me.

Easter Eggs for the Children in The Memorial Garden down by the Intracoastal Waters

Amazing Power of Marfan Teenagers and Their 'Sibs'

I need to give the two marfan teens and their nephew and niece more credit.  At 14, 16, 16 and almost 18 years old I sometimes think they are still little kids and treat them that way.

Raising four teenagers is a challenge, but they can almost raise themselves now.

Unfortunately my desire to remain 'in control' prevents me from enjoying a clean kitchen, spotless house and a chore-less life.  Yeah, I know I still have to be the boss but the past couple days I've made an important discovery - teenagers can actually be an amazing group of humans!

Judy is off to Ocala to help Melissa with the birth of Jack, our 6th grandchild I think. Yikes!  My memory is so iffy.

When I met Judy she was in proprietorship of Sesha, Kyndra, Melissa, Leslie, Laura and Adam.  We then had Jincy and Ruairi together.

Kyndra's two children, Dylan and Dharma are 16 and 14.  Their mum is now challenged with the after effects of a serious cerebral bleeding episode and while she is doing physical therapy her two are living with Judy and I.

They think they'd rather play video games but enjoy the beach if I make them go...

Time out - I have to go holler through the bathroom door to tell one of them to not allow the water in the sink to continually run full blast while they brush their teeth.  Ok, they are "somewhat" pretty amazing.

The kitchen has been another issue, keeping the sink free of piles of dishes has in the past been difficult when 'Nana' off and about.  In the past when Judy was gone I would try and keep order via demands, orders and ultimatums.  'Don't do this' and 'don't do that' were my kitchen mantras.  But the sink would still pile up with dishes.  It was so frustrating!  Last thing I need with a dissected aorta is stress, right?

After repeated failures at being a successful kitchen dictator I thought I'd try something new this time.  Maybe I'd treat them like almost adults instead of little kids.

So I said as Judy left something like, "OK now you are the grownups in the house.   You are in charge of the kitchen and bathroom.  You figure out what to cook, when and clean up afterwards.  I don't care anymore."  The last sentence was a spiteful unnecessary bard said out of needless anxiety.

Three days now and I am wondering why I didn't ever think of this before.  The kitchen has stayed spotless, they have had fun cooking what they want and laughed and sang to stupid teenager songs while cleaning up after themselves.

All they needed was a little freedom and a chance to act better than many grown ups I know.

Jincy and Ruairi are challenged with many trials.  They both fight slightly enlarged aortas with lifestyle modification.  They both push themselves in school (Jincy is graduating with her AA degree because she dual enrolled in college while a junior and senior in high school) and they have to deal with me.  Jincy is my true secret weapon though.  Turn her loose as commander in chief of chores, laundry, trash and food decisions and the other three say "yes, mam!'  No one, especially younger teens, messes with a tall, imposing and almost 18 year old female.

Dyland and Dharma are worried about their mum and miss being at home.  They both, too, are doing very well in school.  I suspect as a child I would have crashed mentally without my parents.   And Jincy is moving away next month so their support system found in an older 'sib' will be gone.  Yes, they are amazing.  But they really need their mom.  Even though grandparents can be nurturing, life without a parent is so different.  Incredibly though, they are not only a pleasure to have living here, they also contribute so much quality themselves to the unique texture of our patch-work family.

So all the worry about scrubbing piles of dishes was a self-fulfilling prophecy in the before.  When I worried about it and paced the kitchen floor asking to see identification if anyone entered, the whole kitchen thing was an ordeal.

Now, well I told Judy over the phone that the kitchen is actually cleaner than I could keep it in my floppy heart condition.  And the kids are storing leftovers and budgeting food, sharing equally between them.  And they are laughing instead of sulking past my kitchen patrol duty.

Wow.  Just when I think I know it all.  Dylan and Ruairi are headed out the door now to catch the school bus.  I'll never know just how they make it down to the end of the street in two minutes but they always do.  I'd take me an hour to hobble that far.

Dylan just turned and said, "bye, Papa K.  I love you."

At 57, I still get a tear in the corner of my eye sometimes.

For Marfan Challenged a Clean Razor Can Save Your Life!

Marfan health tip of the day - use clean razors!

For Marfan Syndrome challenged, an infection can be deadly - always use clean razors!

As someone who knows first hand use how bad a case of endocarditis can be, I am always looking for ways to prevent any instance of infection.  If you've not read my posts about how horrible a heart coated in green fungal slime can be, read here.

An infection, anywhere on the body, can enter the blood stream and impact the heart, causing dangerous inflammation.  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.

There are many web resources pointing out the importance of using clean razors.  Like dental work, shaving impacts parts of the body close to the heart such as the face, head, arm pits or chest.  MRSA, methicillin resistant Staphylococcus aureus and other infectious diseases can be spread through the use of dirty razors.  The CDC Center for Disease Control and Prevention recommends across their web site resource pages that razors be not shared but be clean and stored dry before and between uses.

SImple little good health and hygiene practices can spare one from a very painful, even deadly episode of heart infection.

Marfan Eye Haiku

Walking through Princess Place Preserve the other day I kept rubbing my eyes, seeing the usual bright purple flashes but with a much higher frequency.  Stopping for a moment I quickly noticed the flashes were really violets, new spring violets.  And so here is another haiku.

bright purple flashes

no retina detaching but

marfan violet's show

---

viola palmata, early blue violets

princess place preserve

Living with a Dissected Aorta and Marfan Syndrome, March 2014 Update

Wow, time flies.  It is March 2014 already.  Here are a few updates on my dissection challenge:

One question for my PCP will be - why does my right foot continue to grow while my left foot is shrinking?

• I have all my Medicare paperwork now and the coverage will kick in on May 1, 2014 - just a little over 7 weeks away.  First time I will have had any insurance since my dissection hospitalization!!!  The two year Medicare waiting period is almost over!  I survived (so far)!
• With Medicare in hand I will be scheduling a visit to the cardiologist for my annual CT scan and echocardiograms to see if my aorta is either continuing to aneuryze or is staying the same diameter.  Of course, I am very anxious about these updates and will post the news as soon as I find out.
• I have been having my INR checked once every month, paying for it out of pocket at our local lab.  I am taking 5mg warfarin daily and last time I checked the INR was 2.2.  Fresh garden greens make up a large part of my diet so I can lower or increase INR with the amount of Vitamin K rich greens I consume.  This is easier than adjusting tablet dosage - break one in half, skip a day, yada yada.  My PCP comes up with such complicated formulas.  I've yet to tell her that I am just doing 5mg daily.  This is enough of a challenge for me as it is with my additional memory struggles.
• I do have a list of questions/comments for my PCP, who wants me to come in soon for my annual checkup, and they are:
• My right ankle/foot feels like it is perpetually about to collapse.  It is hard to walk without my forearm crutch.  It hurts.  I am sure there is not a lot to be done about this and I think it is just part of living with a Connective Tissue Disorder (CTD), but I will tell her about it.
• My right wrist is the same way.
• My sternum is still unstable, even after two years since the surgery. I clicks open and closed when I move, much like cabinet doors opening and closing.  Quite painful.
• My right foot continues to grow and my left foot shrinks. I would think this has something to do with a blood flow imbalance.  My right illiac artery is dissected (thoracic aorta is dissected down into my right leg), so it seems to me that the blood flow to the right leg would be impeded and the right foot would shrink, but ???.  
• I will ask her what to do about the chronic pain.  I hurt daily, usually a 7 to 8 pain level while sometimes reaching a 10 for long stretches.  Do Tens units work?  How do I deal with daily chronic pain?
• My left eye has ongoing bouts with a dark, sideways closing curtain.  Is my retina detaching?
• AFIB and VTACH come and go.  Sometimes my pulse will jump, out of the blue - but especially when weather fronts come through - from 50 to 150.  That throws me to the floor, literally and scares me to death.
• I will mention the LOUD clicking my aortic valve makes but I am totally cool with living with a valve that makes noise, so long as it keeps me alive.
• I have chronic fatigue and find myself blacking out or falling asleep right in the middle of the day sometimes.  My diet is wholesome - I do not eat processed foods usually -just fresh meat, fish, garden veggies, some fruits, no grains, no sugar, low salt (sometimes - I love salt).  I exercise - ride bike and walk and do yoga for an hour each morning.  But I think with a 50ish pulse and a heart output capacity of 20-25% that fatigue is to be expected.
• And other questions as they pop into my mind.
• Our teens have genetic testing and their annual echocardiograms in April.  I constantly worry about the teens, especially when their chests hurt or they feel dizzy.
• My Florida drivers license is medically revoked so I have had to learn to get around on my bike or through rides from family and friends.  I try to keep asking for a ride to a minimum because I do not want to be a burden.  Loosing my license after driving for years was a mental challenge.
• Depression is still a looming specter and I deal with it through prayer and spending lots of time outdoors, especially at the ocean and on my bicycle.
• I self-adjusted my Losartan dosage - doubling it from 50mg to 100mg daily because I could not keep my blood pressure down on 50mg.  After a month or two at 150+systolic I upped the daily dose to one 50mg tab in the am and another just before bed.  This has brought my systolic back down to 115.  I could not afford to go to the cardiologist to discuss, and will discuss with him in May - though we have discussed before.  Please do not do this yourself.  

And that's about it for my life with Marfan and other CTD issues.

Stay tuned for more as it happens!