Changing the Pace...

Rush, rush. Hurry, hurry. Must keep up with the pace of life...

For all my life I have been on that kind of pace. Sure there have been periods when life slowed down a bit and there was little to do, but before long the pace went back to a fast clip, demands and obligations creeping back in to intrude on the down time. And as much as I sometimes bemoaned the many things I was juggling, I really felt like I was at my best when I was that juggler with all the plates spinning and balancing them with rarely one dropping.

Something changed in the last 5 years, something I did not expect to see until many years down the road, I am slowing down. Some of it I know is depression over the death of my youngest grandchild and estrangement of one of the most important relationships in my life. Some of it are other causes I will not go into here. But most of it is my body which is betraying me and I for one don't like it.

I've had minor physical issues for as long as I can remember. Foot deformities in infancy resulted in corrective procedures. Always a klutz, I had my fair share of issues because of that. Minor long term upper respiratory issues meant I could count on getting a bug pretty regularly. Nothing that really slowed me down for long. Back on my clarinet a week after having all 4 wisdom teeth extracted because state competition was coming up a week later and I had to practice. I was into my 3rd week of undiagnosed walking pneumonia while in college before I finally broke down and went to the doctor.

The slowing down I'm going through now is the slowing most folks don't start to deal with until their 60's, 70's or even 80's. I am not yet 50. I know I'm not alone as hear a lot of our older Marf's talking about how they are feeling the effects of their condition at an age younger than non-Marf's, but how much is truly Marfan related? That's a good question.

I look at my Marfan husband. He started this type of slow down years ago after his aortic valve & artery replacement surgery. That makes sense. I see that regularly in the Marfan groups I am in. Some folks actually do better after  surgery, but a big number are also just not able to get back to anywhere where they were before hand. I have not had heart surgery so I can't really look at that as a factor.

What happens when Marfan tissue ages? Since the average lifespan for many years was little more than half of that for the general population, there were no serious studies on the effects of aging in Marfan so no one can say for sure. The good news is the researchers are finally looking at this aspect so we hope to have answers very soon...

My Hubby & I are lucky, slowing down gradually, having some time to adjust. Just this weekend a young athlete at the peak of his prime, a newly diagnosed Marf, has suddenly had to face what it took us years to adjust to... to change gears and suddenly STOP doing the very things he planned to do at this time of his life... He is suddenly facing a very different future, but it does not have to be a bad one.
Our Double Marfan family have been keeping him in our thoughts ever since we first heard. There will a lot of changes and adjustments, that is normal. He is heartbroken, it showed in his reaction. Understandable.

Isaiah Austin's dream to play in the NBA may be gone, but he is finding other ways to dream again...  :-)

If there was one piece of advice we could give, it would be: Stay close to your family and faith, listen to your doctors & you will do well! God Bless you Isaiah Austin!

Check out how Isaiah Austin is reaching out to help The Marfan Foundation, and through them, ALL affected by Marfan Syndrome!

Isaiah Austin says he is "Letting God lead the way. Become a part of my story and support the Marfan Foundation. #DreamAgain" - Isaiah Austin


Please check out Isaiah Austin's "Dream Again" Limited Edition T-Shirt in support of the Marfan Foundation!

http://www.isaiah-austin.com/

#Marfan #MarfanFoundation #NBA #Isaiah_Austin

Ambition, Marfan & Pain...

Sunday, January 19, 2014

Again the best laid plans of mice & men...

I had planned to publish this blog in August of 2013, but if your are one of those that know me and how busy my life is now you can understand why it got put on the back burner. I'm finally going to try and get it done. 

There have been many discussions in the Marfan groups we are in as well as on the newly updated Marfan Foundation (formerly the NMF) pages regarding Marfan syndrome and pain issues so it is a very hot topic. As with many chronic debilitating illnesses pain issue are a major concern. I'm sorry to say our family has not escaped it's grasp.

For those who were diagnosed with Marfan in their youth and had to undergo early intensive treatments and surgeries, they have learned from an early age what their limits are and how to stay within them. For those who were diagnosed in their adult years dealing with how pain affects their daily life can be quite a different experience. 

The majority of our Marfan population with later diagnosis have had active lives for many years so increasing pain is a challenge that can change the course of activities enjoyed, how they approach their job, and how to learn to live within their limits. 

Although I can get a bit lazy from time to time I generally have been one to keep going & push myself through my duties no matter what. Minus 20 degrees below zero & the tire was flat, I was out there changing it (it took an hour & a half due to cold but I got it done). Car broke down 10 miles out of town when I pregnant on a less traveled road? I was hiking back to the main road to get help in the blowing drifting snow. Double shifts (16 hours days) working in direct patient care in a busy nursing home? Yup, 2-3 times a week sometimes. Thirty out of 31 day straight through working graveyard at a convenience store with 1 day off work? Yup, did that too. 

Ahh... those were the days of my youth. Yes, I was in pain, but not knowing I was undiagnosed (and that goes for the Hubby too as he was also undiagnosed & working those double shifts right there with me in the nursing home) we just figured, we're young, we can handle it, just push through it...

We continued to push through even into more recent years even as this condition has really started to truly take it's toll. The Hubby was taken down first after his open heart surgery, although his has been less of pain issues and more of fatigue, but even in the last couple of years he has really started to feel the pain too. It's been hard to watch as he has had the ambition to get out and do more, but the reality of how much this condition can limit everyday activities, well it's hard to see.

Get getting back to the title, "Ambition, Marfan & Pain". I was having a conversation the other day at work about where I would like to be professionally in the next 2-5 years, and I couldn't answer that question. 

I am well into mid-life, at the point where many of the Marf's have already had heart surgery or are getting close, and luckily my heart is still in pretty good shape. I don't see any reason why I can't keep pushing upwards in the business world if my heart is any indication, but the pain from the joint issues, well that's a whole different matter. There are some days just getting up and getting moving can seem like a major production, and once I do make it up & out some of those days just keeping the pain at bay just to get through the day is it's own challenge. 

Then there are those days when getting moving is not so bad but sometime in the day the pain hits full throttle. And when they come is anyone's guess. And therein comes the reason the I couldn't answer the question. With increase position comes increased responsibility. With increased responsibility comes the reality that more & more people will depend on you and when you are unable to fulfill those responsibilities it has a not good trickle down effect. Do I really want to put others in the position of not being able to do their job because I can't do mine?

My drive, my ambition, it's definitely still there, but tempered now that I have to balance what I want to do with what I can still do. Still, I am so grateful I can still work and still have opportunities that I can pursue if I so desire, and my heart go out to those in the Marfan community who are not so lucky. I look up to you my fellow Marf's, for as much as I get down sometimes, your strength to continue fighting through some REALLY bad pain, well I don't know how you do it. My hats off to all of you! <3

(P.S) Please keep the family of Kathleen M. in your prayers. She passed away during surgery this week to repair her dissected descending aorta. Our prayers go out to her family... :'(

Time. Is it a friend or foe for those of us living with Marfan Syndrome?

OK, before I tackle that question, I will take care of some business... As for time & this blog, it is WAY past time for this blog to be updated! I will spare you all the reasons why it has not been kept up. Some of the reasons are personal, some are shear procrastination. Nonetheless  it is getting done now, and if all goes well, (fingers crossed!) it will stay updated. We shall see...

So back to the original thought. When Daniel was diagnosed with Marfan in 1994 at age 31, time seemed so much against us. Many Marf's at the time were not living past their 40's & 50's, so it was pretty much accepted that he likely would not see his "Golden Years" unless he was one of the "lucky" few. Although we hoped that he would make it there, we lived with the realism that the odds were against him. 

Something happened along the way to his turning 50 a few weeks ago. Time has also become somewhat of a friend in that we (as a community) have not only grown in knowledge & resources to the point where we know many ways to prevent and minimize many of the effects of Marfan. Now we are seeing members of our Marf family reaching 60, 70, and even 1 former local NMF chapter member was still with us at 81 a couple of years ago. (I haven't heard an update since, but have also seen no obituary). We are now having open discussions on the need to do studies on the effect of aging on those with Marfan. With all the advances being made and every year we get further down the road, hope increases, making Marfan less fearful. Maybe, just maybe, time is on our side... How COOL is that? :-)

Don't get me wrong. We are are all TOO cognizant that their are some still dying too young, including a local young man named Andrew who was in his 20's lost this last fall. The shadow of fear of a sudden aortic dissection still lurks around the corner. We felt that fear again in January as Daniel's routine heart studies were suspicious for a possible small dissection that turned out to be scar tissue. The fear will never totally be gone, but for now, it is not dominant.

This week is again another routine cardiac follow up for Daniel (he is checked every 6 months), and my annual ECHO is a few weeks away, but I don't completely fear them. It is just our reality now. A "new normal" as many of us are fond of saying. And "normal", in whatever way where you chose to define it, is a very good place to be!

On an very upbeat note: Daniel & I just celebrated our 20th wedding anniversary! On the realist side, we have always been cautious and looked at 5 year increments when looking forward and we still do, but with the years ticking off so quickly our silver anniversary (25 years) seems VERY realistic...do we dare to hope for 50? 

In celebration of hitting the 20 year mark, I made a video to honor our accomplishment, and celebrate the gift of time together we have been given. It hasn't been easy, but we've gotten through it TOGETHER!

To my dear Hubby, Daniel Moore, I dedicate this to you & us! 

Happy 20th Anniversary dear, 

and looking forward to many, many more!!! 

♥ xoxoxo ♥

We met in 1991 at work, started dating in 1992, and were married in 1993. Through the trials of the loss of the kids' father in 1994, the diagnosis of Marfan Syndrome for both of us including open heart surgery for Daniel in 2000, helping to raise my kids from my first marriage, blended family stresses, job changes, 3 grandchildren and the death of our youngest grandson Jayden in 2010 to Congenital Diaphragmatic Hernia (CDH), it hasn't been easy, but we vowed forever, and forever we will!

Update on Daniel since the last post. He finally shook the bug, but it did take going to the antibiotics to do it. We really didn't want to go there since he at risk for MRSA, and every antibiotic used now could make it that much harder to fight a bug down the road, but for this bug, it was necessary.

This post comes with a mix of joy & sadness. On June 20th a beautiful little boy named Danny in the UK, who suffers from one of the most severe forms of Marfan syndrome, “Neonatal Marfan Syndrome” (NMS) had heart valve surgery. For those not familiar with it, it is a severe and rapidly progressive form of Marfan Syndrome. Cardiovascular issues can be difficult to treat, causing early mortality in some cases. Danny was in need of heart surgery but not expected to survive according to almost every expert, yet not only did he breeze through surgery but was ready to step down out of the PICU less than 3 days after surgery & is now home & doing great!! So much for the doubters!

The sad side of this came on June 22 when an online Marfan friend named Justine, also in the UK, passed away at age 42 in her sleep. There was no warning, she was just gone. I had only recently "met" her, but she seemed like a bright & witty woman who was trying to live life to the fullest, despite her condition. Our deepest sympathies go to her family... It is also a sobering reminder of Marfan's.

Sunday Daniel turned 49. Not a big milestone for most (other than he is soon to enter his 50's) but 17 years ago when he was diagnosed with Marfan Syndrome we were being told that although life expectancy were beginning to climb, that many were still not making it past their 40's & 50's. Thankfully proper treatment, great support & the blessings of God have come together to get him to today. We know so many examples of friends making 60, 70 & a few beyond, but we also know NO ONE is guaranteed tomorrow, Marf or not. Another non-Marf's friend's daughter & grandchild were in a car accident today, they are OK, yet another CDH family just lost their little baby this morning... Why someone survives to see another day when it could have gone the other way, sometimes it's luck & circumstances, but sometimes we just don't know. That's why it's important to make the best of the time we have as much as possible, and as best we can with what Marfan's gives, live it to the fullest!

Never a Dull Moment...

I pretty much figured no updates here for a few weeks, maybe a month or more, but, no, never a dull moment. Tuesday morning Daniel woke up saying he felt like he was fighting off a bug. It's been quite a while since any of us had a cold or flu & to get one so late in the season is a bit unusual. Also he is usually one of the last to catch a bug as I or one of my kids usually catch it first, but this time he was the first victim.

By Tuesday night the cough had set in & made his already poor sleep due to "Restless Movement Syndrome" tons worse, keeping him from a sound sleep all night. To add insult to injury his knee locked up for the first time. Although I have been fighting lockups for over a year now, Daniel has managed to escape them... until now. A joint locking up may seem minor to other Marf's (many of them fight them for years even before diagnosis) but this could be major issue for Daniel. If it was an isolated lockup then no problem. If his knee joint is weakening to the point where it will lockup regularly, his daily walks may have to be reduced or stopped. This could have detrimental effects as he also has coronary artery disease & hypertension on top of the Marfan's, and the cardiologists believe part of why he is 11 years post surgery with no new CAD blockages is due in part by him continuing to take almost walks. If he has to give them up...<shutter>...

Anyway, by Thursday the bug had really gone a hold of him & the cough was heading straight for his chest. Because he has already had issues with prior acute bronchitis we headed into the doc, and sure enough, no pneumonia (it didn't really sound that bad anyway) but bronchitis appeared to be settling in. Inhaler & cough syrup with codeine (because of another issue we are trying really hard to avoid antibiotics). Praying it does the trick!

The other fun for the week is that Daniel's eyes are VERY overdue for an exam are finally getting looked at this month. We have been noticing for months he has been having a harder time seeing, so we will be going to the ophthalmologist to get a look. I have a hunch he will be joining the 4 eyes crowd.

I was a bad girl tonight. The garden bug bit me tonight & that yard looked like a wreck, so out I went to play in the garden, much to the dismay of my blown CMC (carpometacarpal) joint... OUCH!!! It does looks better though! :-/

Another Bullet Dodged!

May 22, 2012

Well we dodged another bullet for a while. Sometimes it feels like we are playing a game of Russian Roulette, never knowing when we will find the bullet, but for now, we have dodged the bullet once again. 

Both ECHO's were stable, but due to issues unrelated to Marfan's we are making minor medication changes on both of them. 

For my daughter, it will likely have no effect (unless we run into an allergic reaction), but for my husband it COULD have an adverse effect so we will do some extra monitoring to make sure we don't have any issues. He also has heart disease & high blood pressure on top of the Marfan's (a wicked combination) so we have limited options what we can do for medication changes, so this will be a bit of a tight rope act. 

So for the most part, another routine check up, and for the hubby, back in 6 months, for my daughter, back in a year... YEA!!!!

May 21, 2012

This week will be ECHO's & check-ins with the cardiologist for my husband & youngest daughter. It will be either a routine week or it will be one of those weeks that turns our lives upside down, the question is which?

For those new to Marfan syndrome, a little background. Marfan syndrome is a genetic disorder of connective tissue, the tissue that strengthens the body's structures. Connective tissue holds all parts of the body together and helps control how the body moves & grows. Disorders of connective tissue affect the skeletal system, cardiovascular system, eyes, and skin. Because the condition affects many parts of the body, it can cause many complications. Sometimes the complications are life threatening.

Marfan syndrome traits vary from person to person, even in the same family. Some people who have the condition have many traits, while others have few. The most serious complications of Marfan syndrome involve the heart and blood vessels. Marfan syndrome can affect the aorta, the main blood vessel that supplies oxygen-rich blood to the body. In Marfan syndrome, the aorta can stretch and grow weak. This condition is called aortic dilation or aortic aneurysm. If the aorta stretches and grows weak, it may tear or burst and leak blood. This condition is called aortic dissection. It's very serious and can lead to severe heart problems or even death.

Luckily we caught ours before it got that bad, and with good monitoring & in my husband's case, aortic valve replacement surgery in 2000, we have avoided the worst case scenario. It's always in the back of our mind, but we are always aware the danger can arise at any time.

As part of taking care to avoid the worst, my husband has exams done ever 6 months due to him having a family history of heart problems in addition to the Marfan's, and the rest of us are on every year. Now comes 2 of those visits over the next 2 days. Wish us luck that the news is "No change, see ya next time!"

Our Marfan Story Begins...Again!

In 1994 my 31 year old husband Daniel was diagnosed with Marfan Syndrome, one year after we were married. He had his aortic valve replaced in 2000 with a St. Jude valve. Because of other medical issues unrelated to Marfan's he could not work any more and has been on disability ever since. His repair has remained stable and he is doing well at age 48.

After many twists and turns of odd symptoms and a misdiagnosis of a related condition, I and several children from an earlier marriage (unrelated to Daniel) were also diagnosed with Marfan's in the fall of 2011.

The odds of having Marfan's is 1 in 5,000. The odds of TWO undiagnosed Marf's meeting and marrying is 1 in who knows how many... Yet, we found each other!!

As we adjust to the diagnosis, our lives are now changed and will be taking a new direction. Follow us along as we see where this uncharted path takes us...