I don’t know anything about “Cowden’s Syndrome…”

After last night’s late night strep diagnosis, there was no way I could send her to Bible School this morning.

But, I had an appointment with the breast surgeon – a routine follow-up that I knew would take 5 minutes.

(That is why I had scheduled it July 16th when I was ALREADY IN the city- but last-minute doctor vacations are just one of the many inconveniences of life these days.)

I knew it would take 5 minutes – after I drove through an hour and a half of traffic, parked the car, walked a half mile, and waited to be called.

traffic

Truth be told it was lest than 5 minutes.  A three-minute groping of my silicone implants and surrounding lymph nodes.  The proclamation was made that everything looks “great” and I should return in 6 months.  I actually was probably dressed and on my way before 5 minutes were up.

fake boobs

But, I HAD to go.  It would have been too easy to cancel.  It would have been too easy to blow it off.  And what if?  What if that one renegade cell…  Nope, I HAD to go.

And, Meghan had to come with me.  She trekked like a trooper to the main hospital to get another copy of the CD of the MRI of her hand for the orthopedist appointment at 2:30. Then, we traveled on the journey to the Clinical Cancer Center.  I had to push her in the push chair today.  The strep was knocking the wind out of her this morning and the hips and knees were bothering her.

push chair

It was also bothering her that people were staring at her.  So it was a great opportunity to give her LOTS of really LOUD pep talks.  I hope a few people overheard.  Some people are really dumb.  Others mean well – but for goodness sakes, don’t just stare at the child.  Say “hello,” “good morning,”  ANYTHING… UGH!  But anyway…

And after the 5 minute appointment there was another hour in travel time back home.

time-warp

Just in time to let the dogs out and run to pick up some chicken breast cold cuts for her to eat before physical therapy.

As she inhaled the chips and chicken I spoke with the therapist.  I am always just so impressed by how smart she is, and how much she actually cares about Meghan.  She took the time to READ about Cowden’s and to try to understand WHY and HOW the small fatty masses on her palm are affecting her.  If only there were more…

Right after therapy it was off to the orthopedist looking for a few answers about the hand and the wrist.

That’s where things unraveled.

Ok.  I understand it’s a rare disease.  I do.

I get that with an occurrence rate of 1 in 200,000 you may not have touched on it in medical school.

rare-disease-day-feature

But, you insisted on the paperwork completed online a full 10 days before the appointment.  You could have read it, or had someone flag it.

And, I made the appointment with the doctor who had been prepped already.

Bait and switch?

The orthopedist today was amazingly young.  I guess the big 4-0 is approaching fast, because I could scarcely believe he was out of medical school.  Everyone seems to be looking younger and younger.

No need to remind me of what that implies.  I get it.

We have seen LOTS and LOTS and LOTS of doctors.  MOST would rather make something up than admit they didn’t know something, which is a problem in and of itself.  Not this guy.

He examined her hand.  Validated the pain.  Looked at the MRI report.  Declared there to be “nothing orthopedic” about her problem.  And then he said,

I don’t know ANYTHING about Cowden’s Syndrome, so you’ll have to tell me what it is and what it does.”

Cowden's Syndrome

I almost asked him to repeat himself, but that would have just been to buy me time for my response.  So, as I was gathering my bags and looking to exit as fast as I possibly could, I gave him a brief lesson on Cowden’s Syndrome.

This doctor was far younger than me.

This is the technology generation.

Step out of the room and hit google.com

The first link is this one http://ghr.nlm.nih.gov/condition/cowden-syndrome

What is Cowden syndrome?

Cowden syndrome is a disorder characterized by multiple noncancerous, tumor-like growths called hamartomas and an increased risk of developing certain cancers.

Almost everyone with Cowden syndrome develops hamartomas. These growths are most commonly found on the skin and mucous membranes (such as the lining of the mouth and nose), but they can also occur in the intestine and other parts of the body. The growth of hamartomas on the skin and mucous membranes typically becomes apparent by a person’s late twenties.

Cowden syndrome is associated with an increased risk of developing several types of cancer, particularly cancers of the breast, a gland in the lower neck called the thyroid, and the lining of the uterus (the endometrium). Other cancers that have been identified in people with Cowden syndrome include colorectal cancer, kidney cancer, and a form of skin cancer called melanoma. Compared with the general population, people with Cowden syndrome develop these cancers at younger ages, often beginning in their thirties or forties. Other diseases of the breast, thyroid, and endometrium are also common in Cowden syndrome. Additional signs and symptoms can include an enlarged head (macrocephaly) and a rare, noncancerous brain tumor called Lhermitte-Duclos disease. A small percentage of affected individuals have delayed development or intellectual disability.

The features of Cowden syndrome overlap with those of another disorder called Bannayan-Riley-Ruvalcaba syndrome. People with Bannayan-Riley-Ruvalcaba syndrome also develop hamartomas and other noncancerous tumors. Both conditions can be caused by mutations in the PTEN gene. Some people with Cowden syndrome have had relatives diagnosed with Bannayan-Riley-Ruvalcaba syndrome, and other individuals have had the characteristic features of both conditions. Based on these similarities, researchers have proposed that Cowden syndrome and Bannayan-Riley-Ruvalcaba syndrome represent a spectrum of overlapping features known as PTEN hamartoma tumor syndrome instead of two distinct conditions.

Some people have some of the characteristic features of Cowden syndrome, particularly the cancers associated with this condition, but do not meet the strict criteria for a diagnosis of Cowden syndrome. These individuals are often described as having Cowden-like syndrome.

Read more about Bannayan-Riley-Ruvalcaba syndrome.

How common is Cowden syndrome?

Although the exact prevalence of Cowden syndrome is unknown, researchers estimate that it affects about 1 in 200,000 people.

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Was that so hard?  Meet me half way people.

It’s still hard for me to believe that cost me a co-pay.

Let’s see if the hand surgeon on Thursday can offer us something better.

Or maybe the rheumatologist will actually call me back.

Taking bets?

Every day is a great adventure!

Exhausted

One step at a time

Tonight, we celebrate the small victories because we are fully aware how important the little things are.

I get to keep my spleen for 6 more months. (And maybe even longer!)

celebrate

The surgeon said that the hamartomas are there.  They are large, but they are stable.  Stable is a nice word.  So, because they are stable it implies they are benign.  This is another nice word.  The game becomes seeing if they remain stable.  So, in 6 months I will have another MRI.  If they have changed – it comes out.  If they haven’t we can continue to talk about keeping it.

6_months

Makes me wonder when keeping our organs became cause for celebration.

That is definitely in the “Post Cowden’s Syndrome” world.

You know I have wondered on and off how you actually “beat” Cowden’s.  Is it by coming through with the most organs still intact and cancer free?  This is such a strange, relentless disease.  It’s research, while still in its infancy is coming.  But,  I have to wonder how much more they will know a year, or 10 years from now.  And, whether I will like any of it.

We are waiting.  And we know that we are not alone.  We are waiting for Meghan’s results, and its nail biting, agonizing waiting.  But, Felix and I talked tonight and wondered what news would make us happy.  There was no easy answer.

please wait

See, last year – January actually – when we transferred the slides from her November 2011 biopsy to Sloan Kettering, the endocrinologist whose team reviewed the slides told us the cells were precancerous.  They had scored a 3 out of 5 on some scale they use.  He told us they would turn.  We just couldn’t predict when.

So, in June when he called and said he wasn’t thrilled with this nodule (one of many) on the left side we were anxious.  But he said, having reviewed her sonogram she could wait 6 more months to be scanned again.

So, here we are 6 months later.  Tomorrow will mark an agonizing 2 weeks since we went for this sonogram.  Waiting.  Worrying.  Wondering.

what if

When they tell you its “when,” not “if,” it changes things.  No matter what they tell us there will be an anxious, uneasy feeling attached.

This is the game with Cowden’s Syndrome.  It’s almost like a time warp.  A terrible cycle of wait, test, worry, results… Wait 6 months and repeat.

time-warp

Six months seems to be all you really get.  Well, now what I have lost a few organs, I get a year on those follow ups.  But everything else is 6 months.  For both of us.

I tried to sync them up.  So that maybe the worry wouldn’t seem continuous.  But it hasn’t worked yet.

I try not to think too far ahead.  You know what Mom says about planning anyway.

I-plan-God-laughs And to think about this in constant 6 month cycles, well… forever.  It’s a little too much to manage sometimes.

So, we take it one day at a time.  Sometimes one hour.  Or, on this never ending road we call Cowden’s Syndrome – one step at a time.

neverending road

Respect the Spleen

I should be sleeping.  It is 1:30 AM.  This is my second post for the night, because I can’t stop and sort out the overflow in my head any other way.

Maybe you have been following the saga of my poor spleen… or not.  Either way, my spleen has issues.  Or, rather, my doctors have issues with my spleen.

respectthespleen

The first time the hamartomas were detected on my spleen was probably when I had an abdominal ultrasound as a screening after my diagnosis.  When I saw my oncologist in August, she wanted an MRI done of the spleen so she could have a basis for future comparison.  When she called me with the results in August she was ready to send me for a surgical consult, based on the significant size of the 4 hamartomas (courtesy of Cowden’s Syndrome – aka PTEN Hamartoma Tumor Syndrome) on my spleen.  Since the spleen itself is only about 11 cm long, the hamartomas across the top equaled or exceeded the length of the spleen.  At the time, I was coming off of a double mastectomy in March, and a hysterectomy in May.  She was forcing my hand to schedule a colonoscopy (which I did,) but I pleaded with her for 6 more months with my spleen.  Reluctantly, she agreed.

spleen

So, when I had the car accident and I was sent for an abdominal ultrasound, that started the whole process over again.  The oncologist looked at the report and ordered an MRI.  I went for the MRI and tried to set up the surgical consult.  The surgeon, who is a surgical oncologist, thinks the spleen needs to come out, but he wants me to see a different surgeon to see if it could possibly done through laproscopy first.  But, he wants to talk to my oncologist before he will set up an appointment to even evaluate my case, and my oncologist spent last week in California at a conference.

So, instead of February… it is now December, and I am waiting.  Waiting to have the fate of my spleen determined.

Well meaning people say to me, “Don’t worry – you can live without your spleen.”

Sometimes well meaning people should smile and nod more, and speak less.

Yes, I KNOW I can LIVE without my spleen.  You can also LIVE without your boobs, your uterus, your cervix and your ovaries.  You can LIVE without your gall bladder, and your appendix, and your thyroid, and one kidney, and part of your liver or lung too.  But, just because you CAN do something, doesn’t mean you should.

I am thinking of asking for a fake fish for Christmas.  One that will remind me I have been gutted like a fish this year.  One that will also remind me that, no matter how many body parts they take, you have to KEEP SWIMMING!

keep swimming

Monday or Tuesday I will talk to a doctor about my spleen.  I would love to keep it.  I just think we have gotten along nicely for the last 39 years.  And, its mine.  But, I will listen to the doctors (after I ask them EVERY hard question I have,) and I will do what is best, and safest.

Heck, I didn’t go through all of this past year to be beaten by my spleen….

Maybe it wasn’t a “Total Loss”

The Insurance Agent called Friday night.  He told me my car was a “Total Loss.”  I think I knew that after I saw this picture the first time, but it was still a little hard to hear.

I really did love my Hyundai
I really did love my Hyundai

Even though I understand the term “Total Loss” has specific connotations in the insurance world, the terminology wasn’t sitting quite right with me.  To me, a “Total Loss” means I didn’t learn anything.  It was a waste.  I took nothing from it.  That couldn’t be farther from the truth.

I have taken something away from every experience I have had in my life, especially the very trying ones that seem to be pelting me like hail on a blustery day.  Sometimes what I take away is positive, and sometimes – not so much.  But I always, always learn something.

1. No matter how long you stop, and no matter how hard you look, and no matter how sure you are that it is safe to go – a speeding car may hit you anyway.

2. If there are no witnesses to an accident – there is no way to “prove” excessive speed. This is the case no mater how many times your car spun around.

3. When you ride in an ambulance its less scary when you take someone you know.   And, there are people kind enough to ride in the ambulance with you even though they hardly know you at all.  There are real live angels among us!

4. When you are in an Emergency Room of a local hospital – burn your socks after walking on the floor, and don’t look too closely at the walls.  Don’t expect the doctors to have any idea – or to really care what Cowden’s Syndrome is, and how it affects your body.

5. There are some really really nice insurance people, and some really obnoxious ones.

6. Many doctors do not accept “no fault” insurance, so finding one to check you out may be a challenge.

7. The pain is worse before it gets better.

8. The pain of being told you are more liable than the guy speeding through the school zone simply because th stop sign is on your side of the intersection may not be physical, but it hurts your pride.  Especially when you know you handled it right.  It  is hard to get over hurt pride, but you can find peace with a clean conscience.  So glad I have one.

9. It doesn’t matter much to anyone that the guy who hit you didn’t even try to stop, swerve, honk, or perform any evasive maneuver before plowing through you.  It’s all about the stop sign.

10. Whiplash, and muscle spasms are real.  Muscle relaxants are useless because they can’t be taken during the day when you have to be a full-time teacher and mom, but they help you sleep a bit at night.

11. When you stop and consider your accident scene, and you realize all the things that could have gone so much worse, you are reassured that the angels really do watch over us.  (Thanks Angel Meghan… and all the others)

12. When you have Cowden’s Syndrome, and hamartomas on your spleen, they will send you for an abdominal sonogram right away, and then – like everyone else around here- be totally unsure what to do with the results.

13. Fax any important test results to a doctor you trust.  I am grateful the spleen didn’t rupture, but for those of you on my team, cheering for it to stay – cheer louder please.  The hamartomas are growing.  I will talk to my doctor at NYU this week.

14. When you are really at your lowest point, hurt, aggravated, and discouraged – make a decision to DO something positive. After realizing a child could have been easily injured in this mess,  I have established a petition for our local councilman to reevaluate the speed limit on the street where the accident occurred, and to label it a school zone, as well as to consider multiple two-way stops and speed bumps.  I have reached out to the local “Improvement Society” who already reached out to DOT on my behalf.  I have parents in my school fully supporting me and working to gain signatures on a petition.  Their children’s lives are in danger every day.  I want some things to change to make the children safer.

15. It is more fun shopping for a new car when you are ready to buy one, but my husband is helping make our current search more pleasant.  Always marry someone with a sense of humor.

16. Wear your seat belt!  Darn it if Cowden’s Syndrome isn’t going to kill me – a car accident won’t either.  So glad I was buckled up.

17. Those silicone implants can take a good hit.  Thankfully – nothing popped!

18.  I am not going on the teacups at Disney World ever again.  I have had enough spinning for a life time!

There… not a “Total Loss” at all…

to-be-continued

“If I get to keep my spleen…”

As I sat in the dentist’s chair a few weeks ago getting another bridge organized, I enjoyed pleasant conversation with my dentist.  I know, that may sound strange, but really she is quite pleasant, and very talented at what she does.  She is also the Mom to twin friends of Meghan‘s from her class, so we have known each other over 4 years now.   She is a mom, wife, dentist, photographer, fellow blogger, volunteer, and a generally nice person to be around.  I am grateful to call her a friend.

As I was getting ready to go she asked me when she could schedule me for scaling and root planing.  After I had her explain the depth of the cleaning that was involved, and even after she explained WHY it was a good idea, my instinctive answer was , “NEVER!”

English: 29px Sharp top of a periodontal scale...
English: 29px Sharp top of a periodontal scaler Deutsch: 27px Scharfe Arbeitsspitze eines Scalers (Photo credit: Wikipedia)

Over the last 6 months alone I have had breast cancer, a double mastectomy, with reconstruction,  and a hysterectomy.  I have been scanned, had countless MRIs, and just recently completed a colonoscopy/endoscopy.  To say I am DONE being poked and prodded would be the understatement of the year.

So, as I listened to her careful list of reasons why this scaling and root planing procedure is a good idea, I just wasn’t sold on the concept of ANY more pain.

I told her, “When they tell me I can keep my spleen, then I will make the appointment.”  She laughed out loud.  But this, this is what life with Cowden’s Syndrome has become.  I am willing to celebrate being allowed to keep one of my organs, with a dental procedure that is probably quite necessary anway.

But, I feel like life in this body is about triage.  I have to take care of things one step at a time.

Traube's space
Traube’s space (Photo credit: Wikipedia)

So, the oncologist called today about my spleen.  They were comparing the ultrasound pictures from April on 2012, and November of 2011 with an MRI in August 2012.  She sent it to a lot of people to look at.  The answer..maybe.

Really, are you surprised?

You see the ultrasounds showed identical, medium size “hamartoma.” (PTEN  Hamartoma Tumor Syndrome is the umbrella term for Cowden’s and several other related Syndromes)  But, the MRI showed 2 distinct, and one fairly large “hamartoma.”  The good news about these is they are benign.  The problem is – if they follow the body’s tendency to grow and grow things, they won’t be able to stay there too long.

I know, you can live without a spleen.  You can live without a uterus, ovaries, a cervix, breasts, and a whole host of other things.  But, just because you CAN do something, doesn’t mean you SHOULD.

So, I will go back in December and repeat the MRI.  While they are there they can make sure the tiny cyst on my kidney stayed tiny too.

As soon as they are done.  Once they tell me it is all stable, and I can keep my spleen.  I promise, I will be a big girl and get my scaling and root planing done.

At least I have a kind, gentle and pleasant dentist – and she is looking to keep things IN, not take them out!

You can read more from my friend Gracelyn at : http://blog.silive.com/gracelyns_chronicles/2012/09/gracelyns_50_random_thoughts_o.html?fb_action_ids=3988683720816&fb_action_types=og.recommends&fb_source=timeline_og&action_object_map={%223988683720816%22%3A431582393573191}&action_type_map={%223988683720816%22%3A%22og.recommends%22}&action_ref_map=[]

AND: http://gracelynsantoschronicles.blogspot.com/