Tuesday, November 4, 2014


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.


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.


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 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!


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.


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