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

GOOD NEWS, BAD NEWS.

Having VHL, you expect to get tumors and cysts.  Everywhere. 

Every year you get scanned and you prepare for the worst.  I know that I can handle any issues as they come up.  I know it's coming and each year, as I deal with this and that, I know the odds are thinning.  When is the BIG DEAL gonna show up?  Another year and I Thank God.

My kids get scanned and once they get results, I'm the first call.  MiMi, all clear.  I hold my breath till I get that call...and then a big sigh of relief and Thank You God! 

Then Rolly.  We didn't know, I kept telling myself, No, he doesn't have it...we'd have known by now.  There would be symptoms.  Something.  He is young, he is strong, newly married, expecting a baby boy in January...life looked pretty sweet. 

Liz pushed for a DNA test.  That would be the definitive answer.  Yes or No.  I kept asking, any news?  Any results yet?  The longer it took, the better I felt, no news is good news, right?

We knew he probably had a Pheo (pheochromocytoma).  We didn't know how many or how big.  The other stuff was revealed last Thursday and confirmed by Dr. Michael Yeh yesterday. 

History of von Hippel-Lindau syndrome with the following findings:


1. Bilateral enhancing solid renal masses, concerning for renal cell carcinoma, with additional bilateral cystic lesions. (these masses are Renal Cell Carcinoma and are less than the 3.0cm guideline for metastisis.  VHL patients knowing they will have many kidney cysts and tumors through out their lives will "budget" surgeries using the 3.0cm rule. The kidneys can only tolerate so many surgeries.)


2. Bilateral heterogeneously enhancing adrenal masses, the largest of which measures 7.6 cm on the right and 3.4 cm on the left, concerning for pheochromocytomas. Two additional left suprarenal masses identified, possibly adrenal or extra-adrenal in
origin and may represent either pheochromocytomas or paragangliomas.  (Pheo's are bad, really bad.  They can cause heart attack, high blood pressure and stroke at any time.  If you have a Pheo and go into surgery, that Pheo could fire off and cause a crisis. ie: major uncontrollable blood loss.  They have to be removed immediately).


3. Heterogeneously enhancing 3.7 cm pancreatic head mass, possibly representing a nonfunctional neuroendocrine tumor.  (Nonfunctional tumors do not make extra amounts of hormones. Signs and symptoms are caused by the tumor as it spreads and grows. Most nonfunctional tumors are malignant cancer.).


4. No evidence of metastatic intra-abdominal disease. (this is good news).


5. Recommend dedicated brain and thoracic/lumbar spine MRI to evaluate for hemangioblastomas and/or metastatic disease.








The Pheo's must be removed before any other surgeries.  The Bilateral Adrenalectomy is scheduled for December 16th.  Rolly will be taking Alpha Blockers twice per day until surgery.  This will block the adrenal gland from producing adrenalin.  He will be taking vital signs twice per day and email the Dr every three days to monitor how he is doing.

Rolly can live without adrenal glands but must take synthetic hormones for the rest of his life and wear a Med Bracelet stating he is Cortisol dependent and has not adrenal glands. This is forever.

There is a tiny chance that they may be able to salvage a portion of the one adrenal gland, but they won't know until they get in there.  There is an artery close by and it may survive but then you risk the chance of another Pheo.  You don't necessarily need adrenal glands to get a Pheo.  The Dr will make that decision later.

Having VHL, we are predisposed to RCC, (Renal Cell Carcinoma). I'm thankful that the Kidney Tumors are less then 3.0cm. 3.0cm is the size that Renal Cell Carcinoma is likely to spread.  It's still cancer.  We will be seeing a Kidney Specialist within the next week or so.


Having VHL, we will get Pancreatic Cysts and Tumors.  For the most part, we leave cysts alone until they start producing problems ie: digestive issues and pain.  Tumors are another ball of wax.  Expecially this tumor. P-Net is what they call it.  Non Functioning Nueroendocrin Tumor.  Most of these tumors are malignant.  VHL Tumors on the Pancreas act like cancer but different. The size of this tumor is over the 3.0cm size and is of concern.  If they have to remove it, they will do a Whipple Procedure.

Whipple Procedure - The goal of the Whipple procedure (also known as a pancreaticoduodenectomy) is to remove cancer in the head of the pancreas or bile ducts. In the procedure, your surgical oncologist will remove the cancerous tissue, remove portions of the pancreas, bile duct, small intestine and stomach, and perform immediate reconstruction.

The recovery from this procedure has a whole host of other issues.  My brain can't go there yet.

Good news that there is no other signs of metastisis in the abdomen.  They want to do a brain and spinal scan to be sure.

Roland and friends, at the age of 12, were playing with a BeeBee Gun and Roland got shot in the neck.  I remember him telling me later that night, he was afraid he was going to get into trouble for shooting guns at each other (oh and he would have), so he told me that he thought the little nick in his neck was just a superficial wound.  I believed him.

Here we are about to get an MRI and they ask if you have any metal objects on or in your body.  My son, says; "Well, there might be".  What???  They do an XRay and yes there is a BeeBee near the C4 Cervical Vertabrae.  Now they have to determine whether or not this will cause problems in the MRI.  So far, they think if it's in soft tissue, he should be ok.  If it's closer to the bone...another story.  We wait for a definitive answer.

I CAN BREATHE.

While all of this sounds horrendous and has scared me half out of my whits.  I can breathe knowing the potential of Kidney Cancer is lower.  That's a biggy.  RCC is no joke and is the number one killer of VHL Patients.

THIS IS THE LATEST ON MORTALITY.

Mortality/Morbidity: Due largely to the high incidence of renal carcinoma (kidney cancer), the average life expectancy of individuals with VHL disease is 49 years, although diligent surveillance may increase life expectancy. The mortality rate in carriers of VHL disease is high, with death occurring at a mean age of 41 years. Hopefully, the surveillance strategies will favorably impact this statistic.

The morbidity of VHL varies, depending on the particular organ system involved.

* The most serious sequelae of VHL disease involve malignant degeneration of renal cysts. Renal cysts are seldom clinically significant; however, they have an appreciable rate of malignant transformation, and renal cell carcinoma (RCC) is the leading cause of death in patients with VHL disease (35-75% prevalence in one autopsy series). Average age at which patients with VHL disease develop RCC is 44 years. These facts encourage the use of renal imaging on a regular basis.

* The second most common cause of morbidity and mortality in patients with VHL disease is CNS hemangioblastomas (brain tumors). The mean age at diagnosis is 29 years, and CNS hemangioblastomas typically are located in the cerebellum. While the lesions are rarely malignant, enlargement of the tumors within the confines of the CNS can result in neurologic compromise and death, if they are unresectable. Retinal hemangioblastomas, while not malignant, can result in considerable morbidity through retinal detachment or visual loss, which results directly from an enlarging lesion.

I can handle anything, when it pertains to me and my health.  But when you watch your children in pain, or see the fright on their faces...it's awful.  I've made myself sick this week with worry.  Today, after being able to breath...my body aches.  Every muscle hurts. 

It's my job to be strong for my children.  And I will.  I'll be at every procedure, every surgery, ever consult...  I ask questions, I ask hard questions sometimes, my kids know that I will always tell them the truth, no matter how painful or scary. My kids, while listening to the doctor, never take their eyes off of me.  I know that my reaction is the barometer for them.  If I panic or lose it...

I CAN'T.  I WON'T.

I won't lose it.  My friends have done a great job at lifting me up, starting prayer chains, giving me hope, keeping me strong.  I need that to re energize myself before the next consult, scan or whatever VHL brings.  I'm blessed with these amazing women who many of, have stories of their own.  Some of them are going through life changes, are survivors or have health issues or are just making it day to day and they still supported me.  Thank you.  I can't say how much it all means to me but, Thank YOU!

CONVERSTATIONS WITH MY SON.

Rolly is scared.  He's watched from the sidelines as his grandmother dealt with blindness and his sister has had surgery after surgery on her brain, watching and waiting for results on other parts of her body.  And he watch as Davonte had one of his adrenal glands removed.

He also reads all about VHL online.  Good or bad???  I told him to cut it out...it's just gonna make you crazy.  I told him he has to stop worrying.  It doesn't change anything.  We worry, when we have something to worry about.  Am I gonna make it?  Am I gonna live?  I don't want Cancer! I don't wanna die!  I address each one.  Yes, you're gonna make it, you are in the best hands possible. Yes, you're gonna live but you have to stay on top of VHL, you have to go to the Doctors regularly.  We don't see cancer metastisizing yet and if does, we'll deal with it before it causes problems.  You will die one day, but not any day soon.  Who is going to be in the OR with me? A large team of specialists, you'll be in good hands. Rolly, when your brain takes you to that dark place, I want you to change the channel.  Go to Channel Liz.  Think about this amazing woman who loves you and is the love of your life.  Think about meeting your newborn son. 

Each question and concern will be answered.  So far, he's ok.  He started his meds today (Alpha Blockers)...with questions; Are you sure this is the right medicine?  How is it gonna affect me? It said the side affects are.....  What if I have a reaction?  I answer every question.

 

VHL REARING IT'S UGLY HEAD...

Rolly and Liz day before wedding. 8/9/2014

I always had it in the back of my head...does Rolly have VHL?  No!  He didn't have any of the symptoms...well, maybe the sweating?  But he didn't have any pain, no dizziness, no significant weight loss, no head aches, no nausea, no high blood pressure...  My mom had it, her brother had it, I have it, my brother has it, my daughter and grandson have it, my nephew has it, my cousins have it. 

He found out he had VHL after a DNA test...after the urging of Liz.  They are expecting a baby boy in January and she wanted to know, for sure.

Rolly called me and said Mom...I knew it in my gut, I kept up the chatter, How are you? How is Liz?...he said "MOM".  I have it.

Silence...deep breath, don't cry.  It can't be too bad, we caught it early, didn't we?  Ok, Ok, Ok don't panic, call and make appointments, for Referrals for Labs, Blood Work, Urine tests, MRI's, CT Scans, Ultra Sounds, XRays and Eye Appointments.

First he had a very small Angioma on his Retina.  Zap it!  Gone.  Next!!!   Urine test....blood in the urine.  Arghhhhh.  Can you see it? No...they saw it under a microscope...ok, then maybe we caught it early.  Metanephine levels are high...that and the sweating, I know he has a Pheo.   Ok, Dee had a Pheo...we can do this.  They will give him Alpha Blockers to block the adrenalin for a few weeks before they can do surgery to remove it. 

A Pheo (Pheochromocytoma is a rare tumor usually on the adrenal gland which causes high blood pressure, heart attack and stroke).  You DO NOT want to go into any type of surgery with a pheo.  We lost our mom this way.  Ok, we can do this!!!  Calm down, breath.  Many conversations with my son...we can do this.

We know about VHL, we've watched our loved ones deal with VHL, there was always a possibility that you could have VHL, we can do this and we'll stay on top of it as we deal with each thing at a time.  Don't worry yet...we'll worry when we have something to worry about.

Why didn't I make him go to the doctors sooner.  Why wasn't he checked years ago.  I was diagnosed in 1993.  I didn't have any symptoms.  Our insurance had changed and the doctor that diagnosed my mom was now in our plan...I went to see him.  He found it immediately.  So I think we just need to check the eyes.  Everyone was checked and they found it in my brother.  Roland had had many physicals for sports, he had his eyes checked, no one mentioned high blood pressure or anything unusual.  The things he did have could happen to anyone.  I think back and wish I'd just had his DNA checked (this is the only sure way of knowing).  Shana was diagnosed in 1994, she had bad headaches and we found the tumors.  We've dealt with each one as it came.

Ok...well now I'm F#$king Worried!!!!! 
Thursday, Liz asks me to come and talk to Rolly...he's nervous, he's scared...I can't let him freak out.  He knows about the pheos...but I'm not telling him what we just found out.  We will wait till we see Dr. Yeh (a specialist) so that any questions he has will be answered right then.  This so sucks.

I drive to Santa Clarita to talk to my son.  At that time, all I know about is the Pheos.  We talk about what will happen, he tells me he's scared, he doesn't want it to be cancer, he doesn't want to die. Wait a minute, we don't know what  you have, they haven't said anything yet.  If it were an emergency, you would have heard by now.  We talk about what they are looking for and the good thing is, it looks like we caught things early and we'll stay on top of it all from now on. 

I've told him about the MRI's and the CT Scans and the Laser Surgery and everything has gone exactly as I said it would. 

We get back from our walk and talk and Liz has a look, I ask her what?  She said she sent me an email and it isn't good.  Rolly is back in the room and we get ready to go out to eat.  I'm nervous.

We have a nice dinner, I look at my son's face, I see how excited he is about his new life with Liz and the baby.  He's so young...he's so happy.  He is so scared.

I get home and rush to the computer to find this:

IMPRESSION:

History of von Hippel-Lindau syndrome with the following findings:


1. Bilateral enhancing solid renal masses, concerning for renal cell carcinoma, with additional bilateral cystic lesions.


2. Bilateral heterogeneously enhancing adrenal masses, the largest of which measures 7.6 cm on the right and 3.4 cm on the left, concerning for pheochromocytomas. Two additional left suprarenal masses identified, possibly adrenal or extra-adrenal in
origin and may represent either pheochromocytomas or paragangliomas.




3. Heterogeneously enhancing 3.7 cm pancreatic head mass, possibly representing a nonfunctional neuroendocrine tumor.


4. No evidence of metastatic intra-abdominal disease.


5. Recommend dedicated brain and thoracic/lumbar spine MRI to evaluate for hemangioblastomas and/or metastatic disease.




I'm frightened because they don't mention the size of the Kidney Tumors or how many.  The rule of thumb is they remove them when they reach 3cm before they metastasize.  That scares me so much.  VHL patients are predisposed to Renal Cell Carcinoma (kidney cancer).  We know we will have cysts and tumors throughout our lives and we manage them knowing that the kidneys can only tolerate so many surgeries.

The Pheo's are large and on both Adrenal Glands...we try and save as much of the adrenal gland as possible...synthetic hormones wreak havoc on the body. 

Pancreas...I don't know what they are talking about: Heterogeneously enhancing 3.7 cm pancreatic head mass, possibly representing a nonfunctional neuroendocrine tumor.

I cry.  I scream. I pray. I ask my friends to give me strength and hope.  I stayed in bed for a whole day.

I feel better today...still worried and scared but ready to hear what the doctor says.  Dr. Yeh is head of Endochronology at UCLA.  He's done surgery on several of my family members.  He has Rolly's scans and wants to see him right away.  I'm confident with whatever Dr. Yeh's plan is.

I have to be strong for my son.

Thinking positive thoughts, hoping, praying...God please send a healing to my children...give it all to me.  Please let them live a long healthy, happy lives.  Please.

Roland and Liz

VHL Sucks

LASER SURGERY

So I had Laser Surgery on January 24th to zap seven little "*hemangioblastomas". My doctor found 7 new ones but they were small and all responded well to the laser. 

Hemangioblastomas are little knots of blood vessels.  The laser cauterizes them.  It's called photocoagulation.  After the procedure, I go home and rest.  The Doctor said "no restrictions" so  I was able to run the next day. YAY!  I did notice something new this time, I had little black dots and swirls, kinda like floaters in my right eye.  But they have diminished.

They also found cateracts.  They aren't affecting my vision right now but when/or if they do, we will deal with it at that time.  Probably just old age.


LOSING WEIGHT

I've been losing weight lately...I started a new diet in September and eliminated fast food, soda and sweets from my diet.  Adding Homemade Harveys,(http://homemadeharvey.com/) when I get a sweet craving.  I eat them three times per day, they are yummy, no additives, kosher and one packet gives me 2.5 time the daily requirement of fruit.  I've lost 25 pounds.  Yay!  But since my husband has been back from his latest business trip, I've fallen off the wagon...weird thing is, I'm still losing....hmmm?  It could be all the running I've been doing.  I signed up to run my first Ultra Trail Marathon.  (that's a race more than 26.2 miles).  I wanted to challenge myself and I'm determined.  I ran 65 miles last week.  (Imagine Forrest Gump) 

Now compared to other tries at weight lost, this has been a little too easy.  ...there is a little voice in the back of my head saying I should go see the Endochronologist.  They did find cysts on my pancreas and kidneys.  I'm a bit concerned at the borderline diabetic comment on my latest labs.  We will see.


MOM'S DAY RUN

In the throws of training, and planning the 4th Annual Mom's Day Run.  Hoping for another successful year.  Raising money and awareness for VHL is the promise I made to Mom.  This is "Her Day".  I miss you mom. <3 

MOM'S DAY RUN FACEBOOK PAGE HERE: https://www.facebook.com/MomsDayRun


HATFIELDS AND MCCOY'S MARATHON

Next year we are gathering the troops for The Hatfield and McCoy Marathon.  It starts in Kentucky and ends in West Virginia.  Did you know that the McCoys had VHL? (http://www.vhl.org/wordpress/?s=hatfields+and+mccoys)  Check out the marathon here: (http://p9.hostingprod.com/@hatfieldmccoymarathon.com/index.html)   We will be raising money for The VHL Alliance.  Whoohoo!


*Hemangioblastomas (capilliary hemangioblastomas) are tumors of the central nervous system that originate from the vascular system usually during middle-age. Sometimes these tumors occur in other sites such as the spinal cord and retina. They may be associated with other diseases such as polycythemia (increased blood cell count), pancreatic cysts and Von Hippel-Lindau syndrome (VHL syndrome). Hemangioblastomas are most commonly composed of stromal cells in small blood vessels and usually occur in the cerebellum, brain stem or spinal cord. They are classed as grade one tumors under the World Health Organisation's classification system.

I Can See Clearly Now....but...

After getting Health Insurance...I've been getting scanned, xrayed, probed, poked and all that goes with it.

I have an HMO...so I have to jump through hoops.  I had to ask my primary care doctor to get a referral to an opthamologist and then ask that doctor to refer me to Jules Stein.  It's not as easy as it seems.  The opthamologist, a local doctor, who I'm sure is a very capable doctor, saw me and made his recommendations which is not what my doctors usually say or do.  He said he'd just watch them.  My hair stood up on end.

I guess I could just watch them...but my experience is...you watch them GROW and then it's possible that when you do start treating them, you don't get it all or they leak or hemorrhage or cause another set of problems.   I asked for the referral and he says, I never send anyone over to Jules Stein.  What???

Yesterday we visited  Jules Stein Eye Institute.  I feel safe there, I feel like I'm in good hands.  They know VHL, they know me. 

I get there early and they take me in right away...unusual, it usually takes forever, they are really busy.  I told them that when I visited ER last year, they saw three new tumors in my right eye...and since I didn't have health insurance, I would have to wait...these little hemangioblastomas usually grow slow.  I could tell right away, that the doctor saw them.  I was dialated and then send up to take photos, and then another set with contrast.

I then went back to see Dr. McConnell.  She treats all of my family since Dr. Straatsma retired.  Dr. Straatsma diagnosed my mom in 1962.  She told me that there are 6 new tumors and she wants to laser them immediately...while they are small.  They also noted the beginnings of cataracts.  (no worries, they grow slow and are age related).  She sends me up for more photos of the fundus?  Four hours later...I'm done.

Surgery is scheduled for Jan 24th.  Their first opening was Thursday...but I have a race coming up and want to do that first. ha.

While I get a little anxious having treatments and surgeries, this will be my 7th time.  I know what to expect...it IS a process but it's over pretty quickly.

My family has gone through HELL with VHL...I'm grateful that I'm still here and that I have my vision.  For as long as I can remember, I've been afraid of the dark, one of my biggest fears is losing my sight.

I watched my mother lose her sight at 25 yrs old, she raised three small children...and I can't imaging what she experienced personally.  For me, she was strong and fearless...she never complained...she kept moving forward.

My mother was a WARRIOR!



My mom <3



Uncle Bill and my Mom, both VHL Warriors.