JUMBLED MESS

This is how I keep my Sanity

HOPE

I wish I were a better writer, I wish I could convey my thoughts through the typed words on this screen.  I lay awake at night and the words, the message just flows...then when I try to type, it's just a jumbled mess.

Here we are...the New Year and everything that it brings.  Like pushing the Refresh Button on this computer, starting a new goal and saying it out loud.  Losing weight, running more, a marathon...

I want to say that, I HOPE.  I want to be able to continue to HOPE.  I want to believe that there is HOPE.  I get stuck in that in between space sometimes...and HOPE seems so far away.  But it is the HOPE that keeps me going.  I HOPE, I PRAY that one day there is a cure.

BILATERAL ADRENALECTOMY
Both of Rolly's adrenal glands had to be removed.  This means a lifetime of steroids, a medical alert bracelet and being very in tune with your body....watching for changes, for colds and flues, for sick kids....being really careful.

December 16th was Rolly's surgery.  We were all at UCLA at 5am.  Rolly and Liz, me and Jeff, Shana, Tiana, Davonte and Neysi all in the waiting room called Maddie's Room (who is Maddie, I wonder what is her story).  As I sit here looking at all the faces around me, I realize that we are not the only ones waiting in the waiting room, waiting for word about our loved one, nervous, worried, anxious...

There is a man at the desk, who asks our patients name, so they can keep us updated.  And there is a board with numbers on it...we are assigned a number and it changes color when the patient is admitted, when they move to the pre surgery suite, then go to surgery, recovery and so on....we stare at that white board with the numbers on it....

Liz and Rolly are in the pre surgery suite.  Liz comes out and we all go in to see him.  He is nervous, I tell him they will give him something to help relax in a minute.  I love you, I love you, too...Liz goes back in until they whisk him away to the surgery suite...and we wait.

We go to the cafeteria, the gift shop, walk the halls...anything to pass the time...but we always make our way back to the white board with numbers on it...hours will pass before Dr. Yeh (head of endocronology) comes out to speak with us.  He says Rolly is doing well, they are half way done.  Good news, we use this as a gage for when surgery should be done.

It was sometime after 2pm when the person at the desk (it's a woman now) comes over and lets us know that surgery is done.  We wait awhile and Dr Yeh comes to tell us that Rolly did well and he is in recovery, we should be able to see him in an hour or so.  He also draws an image of the surgery he'd just done on his surgical pants.  He was able to salvage a small (really small) portion of adrenal gland, we're hoping for good blood flow...then maybe we'll only need to supplement the cortisol. Cortisol is the hormone that the adrenal gland makes to help the body manage stress.

We wait to go to recovery...we keep asking but, they say he is asleep and they don't want to wake him.  It's 6pm before we can see him.  He's kinda loopy, he shivered and was cold, then he'd turn red in the face and be hot, and he was in pain, but not as much as he expected.  I'm just happy to see him.  He told me that he was so happy that he woke up.  He was terrified that he wouldn't...the first thing he said to the doctors was, did you save any of my gland?

Because they were able to remove this peach size tumor and the others (they found one more) via laparascopy, he will heal faster.  He had five incisions in his abdomen.  He also had an IV in his neck.  That was a little scary, he was having an alergic reaction to the plastic tape.  There were blisters under and around the tape on his neck and on his arms.  Since the IV in his neck is a direct line to his heart, they don't want to break the blisters and must keep the area really clean.  The blisters would last a week.

Rolly  was up walking the next day and he measured his healing by the laps around the nurses station.  He was in good spirits and discharged 5 days later.

We had our Christmas on Dec 26th, Rolly looked good and said he felt tired but he was doing well.
Since he has a sit down type of job, he was able to go back to work pretty quick....and then....

BLESSINGS
Baby Michael Joseph was born on New Years Eve.  He was due in mid-January but decided to come a little early.  What a blessing and what a beautiful gift to Rolly and Liz...they needed a blessing right then, they needed this tiny bundle of joy to take their minds off of the last couple of months.  So much pressure and stress on these young newlyweds...He is perfect!!!

Michael Joseph Tolentino, 12/31/14  6.5lbs  19.5in

I think Blessings come when we need them most.  My son and daughter in law haven't been able to enjoy being newlyweds or being pregnant.  They were tossed into this big JUMBLED MESS called VHL.  I can't imagine the pressure or the stress that Liz is enduring (all while being pregnant)...but I have to say that I'm so glad my son has her...she is so strong, she loves him so much.  She is a GODSEND.

As we welcome this new addition...we are given new HOPE and a new reason to FIGHT and we prepare for the next round of surgery.

The Whipple.
The goal of the Whipple procedure (pancreatoduodenectomy) is to remove the head of the pancreas, where most tumors occur. Because the pancreas is so integrated with other organs, the surgeon must also remove the first part of small intestine (duodenum), the gallbladder, the end of the common bile duct and sometimes a portion of the stomach. In the reconstruction phase of the operation, the intestine, bile duct and remaining portion of the pancreas are reconnected.
The Whipple procedure is a difficult and demanding operation for both the person undergoing surgery and the surgeon.

They will do a modified version of this surgery, leaving the duodenum.

Recovery is tough with this surgery.  3-6 weeks.  The incision will be from his left nipple to his navel.  He will be in the hospital from 7-10 days.

The next surgery is to remove the tumors in his kidneys.  They are cancer but, VHL Patients need to manage multiple surgeries, so they wait till they are 3cm before removing them.  This because, as a VHL Patient we know we will have multiple tumors and cysts on our kidneys and the kidneys can only tolerate so many surgeries.  3cm is the threshold for removing these tumors,  because the risk for metastisis is much greater after 3cm.  One of Rolly's tumors is 3cm...it has to be removed.  If it metastisizes it will go to the brain, lungs...and the odds of survival are very low.  The number one killer of VHL Patients is Renal Cell Carcinoma (kidney cancer).

We will HOPE and continue to PRAY for a CURE and pray that Rolly heals well and can go on to enjoy his beautiful family.

DAVONTE
Today, my daughter called me with results from Davonte's latest tests.  He has another Pheochromocytoma.  Davonte had a partial adrenalectomy, seven years ago.  He had cut his knee at school, was taken to the ER and they said his blood pressure was really high.  150/100.  A thirteen year old's blood pressure should be 90/60.  Pheo's can cause high blood pressure, stroke and heart attack.

The ER doctor told my daughter that Davonte had high blood pressure and to take him to his primary care physician.  No one said it's an emergency, so three weeks later he got to see his doctor...Davonte's blood pressure was 187/110.  He was rushed to CHOC (Children's Hospital in Orange County)...I'm  yelling Pheo to anyone who would listen.  They dismissed me...I'm telling them we have VHL, it's a Pheo.  6 hours later, they tell us he has a Pheo and they want to schedule surgery right away.  NOOOOOOO!  You CAN'T OPERATE on a patient with a Pheo!!!  I'm telling them that they must block him first.  I call UCLA and beg Dr Yeh to talk to these doctors.  He did and they now were going to give Davonte alpha blockers for about 2 weeks.

Alpha Blockers, block the adrenal glands from firing off during surgery.  The tumor on the adrenal gland is called a Pheochromocytoma (Pheo), it sucks adrenalin from the gland and if it were to fire off during surgery, there would be an immediate crisis on the table.  This is how my mother died...during surgery to remove a brain tumor, her pheo fired off and she crashed. There was nothing they could do.

Davonte did well during surgery and post op.  His scans up until now have all been clear.  But over the last few weeks, he's been feeling off.  Tired, sleeping alot...sweating.  We all new what that meant.  He took the 48 hour urine test.  Measuring the catacholamine levels.  His are very high.  Now, we must find an endocronologist.

We'd love to take him to UCLA.  They have a VHL Clinic there.  A team of doctors all familiar with VHL and all of the parts of the body affected, Neurosurgeons, Endocronologists, Retinal Specialists etc. ...but, UCLA does not take Davonte's insurance.

Finding a surgeon with VHL knowledge and experienced in Adrenalectomys with a Pheo is tough. Finding one who takes my grandson's insurance....(frightening)  I'm praying Dr Yeh can find us someone who meets all of the above criteria...otherwise, we are taking a huge risk.  PRAYING, HOPING that we find the right person.

SHANA
My daughter has a new brain tumor.  We have to wait for her latest test results to determine if she has a Pheo before taking care of the brain tumor.  We WAIT, we HOPE, we PRAY.... My daughter has been put through the ringer.  Her first brain surgery was when she was 19 years old...she has had 7 brain surgeries to date.  She manages her own health, and her son's.  Sometimes it can be overwhelming, especially when you have to navigate through the insurance authorizations and doctors misdiagnosis.  Sometimes she just throws up her hands...it can be so frustrating.  Shana through her own fears, is the first person to show up when someone is down.  She is so like my mom.

I feel totally helpless sometimes, most of the time.  I gave this dreadful disease to my children, my grand children.  Watching them endure the tests, the anxiety, the fear is so difficult...and I try to keep a strong front to carry them through the maze of doctors, insurance, authorizations...sometimes, I have to fight with the insurance companies to get the approvals for some of these tests, procedures and surgeries.  Some one, in an office somewhere gets to decide what doctors we get to see.  We beg to see VHL Specialists.  Then they refer us to doctors who've never heard of VHL.

Recently, my labs came back with suspicious activity.  We followed up with a MRI and found tumors. I asked my primary care  physician to see Dr Yeh at UCLA.  He said, no, lets do another test.  The tumor disapeared?  Is it there? is it not?  Now, I'm really concerned.  Can I go see Dr Yeh?  No, let's do a CT Scan.  No, it's not there.   How can the radiologist see a tumor and give it's dimensions and it disappear?  I fight...knowing that this type of tumor is slow growing and I have time to fight...but imagine the patients who say "ok doc" and go about their business only to find that they are now Stage IV and they can't do anything about it.  We FIGHT, we HOPE, we PRAY!

I've made it my mission in life to FIGHT, to HOPE, to PRAY for a CURE!   I know that some of my friends, and some family too, get tired of my Facebook Posts about a race I'm coordinating to raise money and promote awareness about VHL.  They get tired of my asking for money while I fundraise to find a cure.

I know they do not understand what it's like to go to the doctors and trying to educate them about how to manage your own health. They don't know what it's like waiting for the scan results every few months, what it is like to be so scared when you get the crumby results...and arguing with my insurance company or the doctor...

I want to LIVE, so I will FIGHT.   i want my  kids to live, so I will FIGHT.  This disease affects less than 10,000 in the United States.  It's rare.  No one has heard of it...many doctors do not know about VHL.

I know that I will one day die from VHL.  I know that this is a fact.  I can live with that knowing that I did my best to raise awareness and raise money to fund cancer research so that MAYBE, my grand kids will live VHL free.  That is MY MISSION.  I will run, I will coordinate races and do whatever I can do to raise awareness.  Because I promised my mom...and I plan on keeping that promise till the day I die.

So, if you're reading this blog post today...think about donating $5 or $10 or whatever you can to find a cure for those affected by VHL.

 www.tinyurl.com/vhlwarriorsuellen

Thank  you so much for your love and support.  Thank you for reading my ramblings.

xoxo
Suellen