MAYO UPDATE-THE RACE CONTINUES

Ever since we have returned from our trip to the Mayo Clinic, I have been nearing a nervous breakdown- for real. I have been trying to keep a grasp on all of this but I really feel like it is all racing toward me at a pace I can not quite keep up with. I feel like I am now in a sprint to the finish line, but can't quite actually visualize what crossing it will look like. I am trying to be brave. I am trying to be optimistic but right now I am very emotional and I am starting to get scared.
Every morning when the alarm goes off, I thank God for giving me another day and everyday I get out of bed because God whispers, "Yes Mary, I do have a purpose for you." and so with that, I move forward but here are some very honest truths about my life right now.
I am sick of this race.
I am sick of dealing with this disease every single day.
I am sick of talking about being sick.
I am sick of writing about being sick.
I am sick of feeling so overwhelmed that I am losing what mind I have left.
WITH THAT SAID-
The most AMAZING thing about being sick is that I get to see firsthand how God works through others. How he takes people and places them in our lives at exactly the right time, in the way we need them most. It makes me feel so secure knowing so many people are supporting us and even better knowing that if He has to, God will carry me over his shoulder the rest of the way so I can finish.
So I say thank you-for all you have done so far, all you have offered to do when we get home and at every point in between. I wanted to let you all know, I am going to write this post and then I  will not update again until I am done with surgery and can report that I am victorious!  I would like the last 2 weeks before this surgery to be able to focus on my family and not this illness that has consumed so much of our lives lately. I would like to NOT talk about it anymore, for awhile anyway.
OUR PLAN:
On November 13 we will travel back to Rochester and on November 15th my neurosurgeon, will again, perform brain surgery through my nose.. This time, he will remove the entire right side of my pituitary gland. (partial hyphosectomy) in hopes that he will get the lesion they can  see on the MRI but also any other bad cells that could potentially regenerate in the future. Sometimes in the case of recurrent tumors, a more extensive removal of the gland is required to provide a better outcome. Recurrent tumors are usually more aggressive than the original.
 YES, I am freaking out about it AND I am very scared-BUT, no matter how scared I am, you could not convince me to back out.
The pituitary is the master gland of the body. without it, you would not be alive. Dissecting this gland is anything but minor. When we left our appointments last week, we were just so happy to have saved my adrenal glands that we did not realize that this option is only marginally better.
Removing half of my pituitary definitely has risks.
I risk destroying the whole gland and leaving my entire endocrine system without regulation.
I risk not being cured and STILL having both of my adrenal glands removed. My first surgery had a success rate of 80-90% for which I failed. This time around I am at a 60% cure rate.
I am also at risk for spinal fluid leaks, diabetes insipidus and meningitis.
I am taking a risk that my left side of my pituitary will never again "wake up" after surgery and I will have a complete loss of function and a lifetime of replacement therapy.
I am at risk for a crisis and even though my risk is lower than if I were to remove my adrenal glands, it is still a very real possibility.
I risk that the surgeon will go in and see that some of this tumor is wrapped around my carotid artery, making it inoperable and then not only would I have to recover from surgery but begin radiation as well.
I have risks even if the surgery is a success.
Even with a successful pituitary surgery I will be on some sort or replacement hormones until my left side begins to function, assuming it will begin to function. If the surgery is successful (and it will be), I will have a post operative "CRASH" within 24-48 hours, which I am told by reliable sources-(Google and my neurosurgeon himself), it is pretty horrible. They say it is like a heroin addict quitting cold turkey- fever, chills, vomiting, shaking and all of this with gauze packed in my nose to keep my brains in place. My body will freak out because it went from making too much cortisol to none at all. After this crash, and please pray that I have one, they will bring me back up synthetically and slowly wean me back down. Not many people get to say they know what it feels like to be a drug addict having never used drugs so I guess I will get to add that to my life experiences list! To be considered a successful surgery and into remission I must have this CRASH. PRAY that I have it, pray my children do not witness it, and pray that my brains stay in place.
But for now, I must stop talking about it, writing about it and  worrying about it and KNOW I have a great team backing me up and I will win this race!
More from mayo in a couple of weeks...

Winning The Race

 

 

Being ill is like running a race. You might have a clear starting point and the end in sight but no clear picture of what the race will be like, who you will be running with or against, who will stop to help you if you fall, who will cheer you along the way. and what obstacles you will encounter on your path.  My path from diagnoses to treatment plan has been long and difficult. Even now, with the end in sight I still struggle. I not only want to win this race but I want to finish in style . The pursuit of health is exhausting. You must be relentless in your pursuit of good health and expect nothing less than to finish the race. I have learned that I will not accept anything less. I have learned the power of preparing for my race, including tireless research and learning self advocacy skills. I have learned that doctors have a lot of answers but not all of the answers and that real life is never the same on paper as it is actually living it.

Cushings disease has taken so much from me. It has taken my immune system, my sleep, my life. It has put ugly names to annoying symptoms-buffalo hump, moon face, central obesity. It has made me weak, tired, sad, anxious and irritable. My life has been so consumed by this illness that sometimes I no longer want to be a part of its team.  I want to curl up it a ball and quit fighting. BUT I have to remember my whole family has my cushings disease so even if I want to quit the race, my relay team would lose as well. Sometimes I do find myself wondering, what if this disease will be my life? What if this is how I will feel forever?

Some days it is so hard to remind myself that even though this disease is a part of my life it is not my entire life. 

We know, without a doubt the BEAST is back but it has been a long process trying to figure out how to get rid of the BEAST. This has been an extremely difficult time for me because unfortunately, no matter how I feel both physically and emotionally life is still moving around me. I still have to be a Mom and a wife.  We have been in a constant phone circle with the Mayo Clinic as well as with my doctors here.  The wonderful thing about the Mayo Clinic is that they are a multidisciplinary facility. They call in the troops for each and every case.  Everyone is involved DIRECTLY with my care. Endocrinologists, neurologist, surgeons, radiologists, and those I call by name- Shirley my endocrinologists' secretary and Joni his appointment secretary- who are now both on my Christmas card list. Not one of these people are standing on the sidelines waving a flag and cheering me on, they are running this race with me. And we have a plan: A FIRM plan but one that could of course change...

The treatment of choice for people with Cushings disease (ACTH secreting pituitary tumor) is transphenoidial surgery, which is basically brain surgery through your nose. I had this surgery in April of 2008.  However, for people with recurrent cushings disease, a bilateral adrenalectomy is an option with a high rate of curing the disease not just putting it into remission. Because I have what they consider a failed pituitary surgery, this is a good treatment option for me, one that we have agreed to proceed with but one that will change my life.  On my MRI they saw a small abnormality. It could be residual tumor, the tumor could be embedded or in the cavernous sinuses. I could have the same neurosurgeon go in and identify the tumor cells visually but for my comfort level he is WAY to close to my optic chasm and carotid arteries to be "looking around" I loved my neurosurgeon, but not that much. SO my treatment of choice is a bilateral adrenalectomy and on November 14th, we will travel to the Mayo clinic to remove those two organs and hopeful get my life back at the same time.

About those two organs and how removing them is truly life changing.

 

 

 

 

There are 2 adrenal glands located on top of the kidneys.  They play a very key role in maintaining harmony. They have an outer portion(cortex) and inner portion(medulla) The cortex produces 3 hormones all corticosteroids.

Cortisol is a glucocortoid, a corticosteroid that maintains blood pressure, suppresses the immune response and is released as a part of the bodies response to stress.

Cortisol production is regulated by the pituitary.

Cortisol is essential for life.

Because of cushings disease my body makes too much, without my adrenal glands it will make none at all.Hormonal balance after an adrenalectomy is a major concern but it means I get to control how much cortisol my body gets instead of it controlling me.

MY # 1 QUESTION is, Am I trading one disease for another (adrenal insufficiency)?  After they remove my adrenal glands I will be on lifetime replacement medicine.

Cortef/Hydrocortisone: Without adrenal glands I must replace cortisol at a normal level- A happy level. Some people never find their happy.  If I am experiencing stress, I have to recognize this and up my dose and I am really not sure how my type A personality will do with this.

I will also need Fludrocortisone to replace aldosterone.

TO MAKE MATTERS MORE COMPLICATED

I am at risk to have what they call an adrenal crisis. This risk is not only possible, but probable especially in the weeks following surgery but possible for the rest of my life.

You need adrenal hormones to live. The system that pumps blood through the body can't work during times of physical stress such as illness, injury if there is a lack of cortisol (or its replacement) Risk factors for adrenal crisis include physical stress (infection), dehydration, trauma or surgery. In a crisis an injection of hydrocortisone must me given to me immediately, if it isn't, it rapidly leads to coma or death. This is a pretty serious thing to have looming over my head for the rest of my life. Lets face it, I still have to go into the quiet room at the dentist can I trust myself to inject my own body?

After surgery I will also need to wear a medical bracelet, not just have one and leave it on the bathroom counter. I will have to carry a "crisis letter from my doctor wherever I go. I will need to keep shots near me and I will have to hope that the EMT'S are trained in adrenal crisis situations because from what I have read, the statistic are not great.  Currently, they are not required to carry Solu-Cortef on board but they are also not allowed to inject my personal supply.  What do you about that little roadblock?

But we will move forward with our plan.  At the end of October, we will travel for pre surgery appointments.  We will process that information and return on the 14th of  November for surgery.  We have had so many offers during those times to help with our children but Kevin and I have made the decision to take them with us on both trips. This was a difficult decision but ultimately because of the struggles Ben is having at school and Gabe's insecurities about my health we decided we would be best together as a family. We will need help upon returning from the Mayo clinic so we hope your offers will still be good.

At this moment I can do nothing but pray.

Pray that I will get my life back.

Pray for relief.

Pray to not only win this race but to win with style.

I know nothing happens by chance: I remember reading this quote recently, "God causes things to happen at exactly the right time. Your job is not to figure out when but to make up your mind that you wont give up until you cross the finish line."

Going through this will no doubt leave scars-physical, emotional, financial and lasting.  I am not naive to think I will instantly get better. I know this is not a sprint to the finish but more of a slow and steady wins the race.

So I will continue to trust that God is in control and let him act in my life, knowing that trusting Him will bring me peace and rest and every night I will continue to ask that He helps me to trust his plan for me. I do feel your prayers and I appreciate you support.

Waiting, Waiting and MORE waiting

Getting a Cushings diagnoses in 2008 and now trying to confirm the recurrence has taught me something.  Cushings is a hurry up an wait disease.
I called the Mayo Clinic today and have confirmed, my MRI CD is there now I will wait for a call from my doctor. I will probably wait, and wait and wait...
I started praying my 7 day novena tonight because I know that God truly listens to me, and answers my prayers directly After my last surgery in 2008 i wrote the following post- click on the link to see The Power of Prayer 
I will continue to wait and know, as I have said before, God has great plans for me.
I can feel your prayers and I appreciate your calls and outpouring of support!

Mary the Medical Mystery

Left to right- 2008 2 months before my surgery when I was at my worst. 2 months after my surgery and 2011 when it started to show again.

I am fighting what seems to be a battle I will never win.
A battle I am tired of fighting.
A battle I am forced to deal with every single day.
 Cushings Disease has been a part of my life since 2007- too long as far as I am concerned. To think I am actually considered one of the lucky ones makes me sad. I am sad knowing there are so many others who are not yet diagnosed. Sad that there are those who are not yet treated.
I have also had several close calls and times when I thought I would be going back to the Mayo Clinic. Times when my blood work and/or symptoms were abnormal but not alarming but today it is not an if but a when and for what.
So almost 5 years after my original diagnoses, I am facing the beast head on- hoping this time I will win! I am one test away from making a decision that will change my life in so many ways.
 But for now, for today I need to get it all on paper.
Why? Because I must find a way to discuss my situation without falling apart. When people ask how I am doing, it is difficult, and I am always so close to losing it. When I write my story down, I write not for people to feel sorry for me or to pity me,  but because I know the more people that know, the more who will pray and knowing people are praying for me brings comfort. I never want to use this disease as an excuse for the way I act,but it does come out in my behavior and reactions to what to some may be minor upsets but to me they are quite large.  I also know that it is all important information and I must keep record of it for my family and my doctors. I also write because it helps ME to gather my thoughts and to process my medical mess.
Cushings Disease has been the hardest thing I have ever had to deal with.  Some doctors will go their entire careers and never see a patient with cushings- it is a rare disease. I have done so much research, possibly more that the CIA does and I still don't understand it.
 When my doctor and my friends have asked me how I knew it was back  I say:

I have once again become THAT person who is always tired, always sick and always with a pain. It is hard to have become that person again who I do not know or recognize. I wish I could be a typical mom- running committees, volunteering, being more active at church, but my reality is, it is just hard to be...On a daily basis I deal with
*Short term memory issues. I can only do one step at a time. Give me 2 and you are in for a laugh.
*word retrieval. I find myself searching for words a lot.
* I have no stamina.
*I have feelings of isolation and because of my sleep problems, I am a roller coaster of emotions and lack any sort of motivation.
* I am usually a motivated person but lack of energy and lack of interest now hinders that.
* This disease has made me unreliable and I hate that.
* I do not know what morning will bring and my body aches every minute of the day.
* My ACTH levels have been on a steady rise for a long time and the migraines are back.
*I have to force my brain and my body to work together just to do the things I used to enjoy.
* Little things, good or bad go to the extreme in my head.
* Depression
*Anxiety
*Stretch marks, a side effect of the disease ( this makes me mad. I birthed 2 children without a single stretch mark)
* Loss of friends. This is the one I struggle with the most because it leaves me hurt. I have friends that have been loyal to me, that forgive or ignore my shortcomings, who know that underneath this illness, I am a good person. but I also have friends that do not understand, that have distanced themselves, and friends that no longer call. Lets face it, I am a high maintenance friend, To some it was OK some it was not.  I know it is OK because the friends that matter the most are by my side now- still some days it stinks!


I am what I once heard described as "CUSHINGS-FULL THROTTLE" All symptoms, all the time, to the extreme.
Kevin and I have always agreed that we would wait it out until the time comes where it is taking over our lives, and that time is now because my symptoms are reaching the point that waiting just doesn't make sense anymore.
So after an especially trying summer of denying we are prepared to deal with it.
I have completed several tests here that have all indicated a recurrence. On Monday, I will have an MRI with dye contrast and I am hoping for a BIG FAT TUMOR. I hope it screams out to the radiologist, "Come and get me." because that would make it a little less complicated.

IF THEY FIND A TUMOR
I can repeat the surgery I had in 2008, which is basically brain surgery through my nose. If the MRI  shows a visible tumor this would be the treatment of choice and it SHOULD be what I hope for BUT this is a hard one for me because this time I know what it will be like  and the reality is, it is because of this knowledge I am more terrified than the last time. I know the risks and I know how I will feel.This will include but not be linted to:
Excess bleeding of the nose- we are talking bath towel, not Kleenex.
The stuff they shove up your nose after surgery to keep your brains in place hurts.
Other risks include meningitis, spinal fluid leaks, spinal headaches and oh boy the congestion. Not the sniff, sniff kind more like blood clots in your nose the size of Texas and this is made worse by strict orders not to sneeze or blow your nose for 2 weeks.
After I returned home, there were days I could not get out of bed.  It is knowing all of this that makes it worse this time.
So what will they do if they do not spot a tumor? I can either undergo a bilateral adrenalectomy or I can remove my entire pituitary neither of which sound appealing. Both will require a lifetime of hormone replacement. I cant really figure out any pros to these options other than they have high cure rates. I do know my recovery would be much longer and because my body would no longer produce cortisol I would require a lifetime of replacement therapy. the other issue I have is that people die in the months or even years following surgery from adrenal crisis. It happens a lot! Yep! pretty scary if you ask me!
So Monday is the day of the MRI- I would hope for immediate results but know it will take time. to send the film, to read them and to get back to me. I will try to be patient. I know God's plans for me are not hindered by my fears and my struggles so I will try to "be still and know"

Wisconsin 2012

  Friday night at the school carnival! Mason, Ben, Tess and Delaney ready to ride

 Ben and Mason on the octopus!

 Kirby (giant boy in the middle of his followers) was so sweet and rode every single carnival ride with Ben- even the ones he was not thrilled about and even when Ben yelled out when the ride was done  "Kirby was so scared!"

 We went to Mason's football game EARLY Saturday morning!

 Tommy and Gabe spent the morning wrestling

 Having a great time!

 Gabe hauled Tommy all over the football fields!

 Tommy did the same for Gabe, well, sort of anyway!

 Of course, Ben was always there as a backup!

 Saturday night we watched Kirby and Jack play football.  If I could watch every single football game from inside a private box, with food, drinks and comfortable chairs, I might just become the biggest fan around!

 At kickoff everyone was attentive, sitting down and watching the game.  By halftime, it looked like a tornado had ripped through that box, raining down pretzels, Doritos, and popcorn. By the third quarter Gabe was asleep on the floor oblivious to food and footballs falling on his head!

 This is Tommy. Cute as a button and LOVES i Phones or any other hand held device. After his repeated requests, I caved and had a halftime photo shoot!

 Kirby # 68 and Jack #79.  How did they get to be so gigantic??

 The drive home- always a bummer. Ben was exhausted after his sleepover with his cousins!

 I hope after they slept in the car so much they will go to bed tonight!!

Water Baseball

 Ready to bat!!!

 Somehow, pounding the bat on the cement helps!

If that fails, rub it on your backside- it might work!

 The koosh ball is amazingly fast!!!!

 Jump and swim to first base!!!

 It is harder than you think!!!

 Gabe smiles like this, unless he thinks you throw poorly, did something on purpose, or call it wrong.

 The swing!!!!

 Off he goes!

 Bruiser is the outfielder! He does a great job, running after the ball.

 Unless of course, it is an over the fence home run!!!

 Outside the fence, back in the trees!

 This Koosh ball is very durable!

 Victory for the boys!!!

The tradition of water baseball goes way, way back! It started at the country club where we were members. We still use the same "bat" which is actually a water skateboard and the same "ball" a Koosh ball. Sometimes there are a lot of kids playing and sometimes, like tonight it was just our family. Kevin is always the pitcher, Bruiser always plays outfield! I am always the referee!

1st Day of School 2012

 This is a reminder that this day is real. We are not up at the crack of dawn for no reason.

Either I am getting older, or I just am not that into anymore but I was racing around this morning looking for water bottles, lunch supplies, etc. I finally got in all together. I sure wasn't that organized!

"Homework is horrible, school is OK"- Ben Kahl 5th grade

"I love school a very much A LOT!"- Gabe Kahl 2nd grade

 Brother love and support! Dad peering out the window!

 Bruiser is making a plan in his head. He is deciding how to torment his mother.

Off they go!

The crew in order according to age.

 Here comes the bus- which was a little earlier than schedule- I hope that is not the case every day!

 I had a moment of sadness as the bus left and I walked into the house. After 5 minutes, I was better!