Nothing

...but a missing cyst and clandestine plans...

Missing Cysts

Last December, during a routine scan here at home, a cyst was seen in my sweet son's brain, attached to a tumor and snuggled against the brain stem.

In late December Steven started an oral prescription medication for hair loss.

In February the cyst and accompanying hiccups were noted by the brilliant doctors at the NIH. These doctors, who study VHL more intensely than anyone else, suggested immediate surgery. My sweet son, because he was in the middle of the last semester of college and didn't want to ruin his plans for an August start at law school, asked if the surgery to remove this tumor could wait until May.

After a long and convoluted conversation about symptoms, dangers, and outcomes, all agreed that Steven would wait until May, return to the NIH, have this nasty tumor removed, and start law school without any hiccups or VHL worries.

On the way home I began talking to my sweet son about the importance of keeping his local neurosurgeon advised of these plans. So Steven made an appointment with this Dr in mid-March, and I accompanied him.

Dr. V saw the cyst, commented on symptoms, and asked why the drs at the NIH would recommend surgery when the only symptom is hiccups. We explained what the good drs at the NIH told us. To the best of our muddled understanding, the drs at the NIH are finding that brain stem tumors almost always need to be removed, that beginning symptoms are a precursor to horrible side-effects of the tumor, and that they feel now that it's better to remove these tumors before the horrible side effects occur, as swallowing problems, numbness in limbs, and constant nausea don't always cease after surgery.

Dr. V listened intently, nodded his head a few times, and told Steven to re-scan in May and come back to see him.

Which bring us to the most recent scan of my sweet son's battled brain.

The cyst has all but disappeared.

No miracles, folks, as a miracle would mean that the cyst had completely disappeared. A miracle to us would be a brain free of disease or a gene that corrected itself. A miracle would be a drug that prevented further surgeries.

This is just a cyst that has followed a path not seen in VHL and diminished to a size that is barely visible.

After looking at the recent scans, that were sent overnight to the NIH, a resident dr working in neurosurgery at the NIH phoned my sweet son. This dr mentioned that the difference in the cyst doesn't appear to simply be a different cut of the MRI machine. This dr asked Steven if he has had radiation to that tumor, in an attempt to explain this finding. Steven, of course, replied "no", but he did mention this medication for hair loss that he's been taking since late December. The resident told my sweet son that his scans would be reviewed the next day by the lead drs in VHL studies at the NIH. The next day, in a flurry of missed calls and email correspondences, Steven was told that VHL brain cysts don't simply disappear or diminish significantly in size. He was also told that these drs won't answer any questions about what could make this cyst diminish so brilliantly until his next appointment in August, because cysts attached to tumors in the brain of a VHL patient simply don't diminish significantly. These doctors also deferred to Dr. V in regards to surgery plans. Dr. V, of course, doesn't see surgery as an option now. He would prefer to watch this tumor closely and be informed of the next MRI from the NIH. This MRI will happen in August.

August is also when my sweet son starts law school.

My son, to say the least, is frustrated. He's frustrated because his plans for this summer, including a meaningful internship that he had to pass up and exciting travels that couldn't happen, were cancelled, based on a brain surgery that was all but written in the books. He's abundantly frustrated that even the most brilliant drs in this field don't have answers for him. He is most frustrated that he's now expected to start law school with what we consider a very unstable tumor.

And we, by association, are frustrated.

Clandestine Plans

I teased a bit back in late December, amid the tears and disbelief that accompany a bad scan, that this prescribed medication that my sweet son was taking to treat hair loss might be the answer to prayers for thousands of VHL patients.

I was simply teasing.

After seeing the latest scan, however, I began thinking about the timeline of the changes in the cyst and the medication, the fact that this medication inhibits the formation of certain male hormones, the common but unproven belief that VHL tumors respond negatively to hormones, and the response from the NIH doctors that VHL brain tumor related cysts don't simply go away on their own.

And I brilliantly decided that the decrease size in the brain stem cyst was directly correlated to the taking of this medication.

And I suggested to my sweet son that we now work on proving causation between the medication and the cyst in the little lab that we like to call "the upper left shelf of the corner kitchen cabinet".

I hope this is the miracle pill.

Seatbelts

My sweet boy's operative notes make me cry every time I read them.

It's not the mention of general anesthesia, intubation, or suboccipital midline incisions that make me sad.

I can read about prophylactic antibiotics, steroids, and irrigation with relative detachment.

Even the mention of the bur holes and the drill used to open my sweet boys skull and the documentation of sutures and titanium plates used to put him back together don't leave me overly emotional.

But I always have a very difficult time reading, thinking or talking about the seat belts...the seat belts that so very tightly strap him to the table.

Seat belts.

Isn't it odd that I lose my composure over seat belts?

But all of the tools and procedures that are mentioned in the operative report have big, fancy names that sound impressive and curative. The BrainLab navigation system, 4-0 Nurolin sutures, Midas Rex drill, and Kerrison rongeur all sound like instruments of healing.

And the use of seat belts sounds so barbaric.

The reality of Steven's life with von Hippel Lindau disease, however, is purely barbaric.

Because brain surgery, stripped of the fancy names, is brutal. Opening the skull with a drill and maneuvering a blade through brain matter is horrific at the most basic level.

And brain surgery is the only treatment for VHL.

My sweet boy needs a cure.

Glory Revealed - The Ramblings

When Steven was in the hospital this past June, I had a chance to come home on Tuesday and Wednesday to shower and change clothes. Those were long and lonely rides, friends, battling with emotions and fears that were kept in check in the hospital. Fortunately and with perfect timing, a song called "By his Wounds" was played on my favorite radio station during each leg of the drive.

And this song kept me going.

It gave me peace when I feared for the wound that wasn't only on the back of Steven's head but also deep into brain tissue. It saved me from my fears about Steven's chances of living a strong and full life in light of the disease that caused this tumor. This song grounded me in the belief that we are so very Loved that the Hands that were pierced for us will hold us tenderly and carefully through all of the hardships that we face here on this earth.



I didn't know the name of the song nor the artists that performed it. But I did know the first few words, and this weekend I was fortunate enough to find the video on YouTube!



Funny enough, as I watched the video this past weekend, I kept thinking that one of the singers looks like an old Backstreet Boy...and then I saw the names associated with the song...and guess what!?!

(I think I know who took his spot in the band!)

Happy Scissors!

Before:

After:

Today has officially been renamed "Happy Scissors Day."

Steven is so happy to have the itchy sutures out.

We were all happy that the cerebral spinal fluid had stopped pooling under the skin.

Steven was happy that the physicans assistant who removed the sutures looked around the office for the sharp scissors instead of relying on the dull ones.

Steven and I were both happy that the "under the skin" sutures are disolvable, as there were many, many, many of those stitches...like maybe 70?!? The "above the skin" sutures, the ones that needed to be clipped and tugged, only totaled 13.

Steven was happy to hear that physical restrictions only last July 4th - one month post-op.

I was happy to have a follow-up plan, as we had not been told up to this point what to do next! We will have a follow-up MRI and visit with Dr. V on the 11th of September, thus giving the bruised and battered brain a chance to heal before we scan.
September 11th...less than a month into college...but most kids miss a day here or there, right?

We were both happy to learn that the mesh inserted into the back of the skull is very strong...so future soccer playing is fine!
(After July 4th, of course!)

Steven was all too happy to have a haircut after the stitches were removed!

I am so happy that he seems to be embracing the scar this time.

Friday, June 22, 2007
Happy Scissor Day

The Top Two Reasons

To Be a Neurosurgeon

1. Saving lives.

2. $12,730.00

Steven

Two Weeks

Today seems to be headache day.

Yuck!








We were told that this might happen when Steven was done taking steroids. So we followed the already given advice and Steven took another dose of the Decradon. He also took two extra-strength Tylenol, as we were told last week that people with VHL shouldn't take Advil or Motrin when having a headache...hmmm.

Just Another Bump in the Road

And in the back of the head...

Steven didn't have his stitches removed today. It seems that the small bump that has formed under the incision in the back of his head is an indication that the cerebral spinal fluid has found a tiny leak through the meninges and is collecting just under the skin.

Diane, the P.A. that we saw today, told Steven that this is a normal complication with back-of-the-brain surgery.

Isn't it odd what we consider "normal" now days?



The plan now is to save the gauze, antiseptic lotions, scissors and other stitch-removal paraphernalia for next week. We will return to Harbourside Medical Center next Friday and take another peek at this fluid build-up. If it has resolved, Steven will have stitches removed then. If not...well, we didn't ask!













The good news from today is that Steven is free to drive! He is also able to wean back on the steroids with plans of being totally off medication in about four days. Diane assured Steven that the odd symptoms that he has been having, sleeplessness and a foggy feeling in his brain, are medication related. The follow-up head CT that is scheduled for early July can also be cancelled. No need. And the pathology report came back as "Hemangioblastoma". No surprises there. Interestingly enough, we learned that the brain, in the surgery area, will take about three months to heal.

A little bit of bad news, besides the fluid build up and the itchy stitches, is that restrictions are still in place for any strenuous physical activity. No bending and heavy lifting...

which means no repeat trips to Splitsville for another four weeks!

Oops!

A frustrated Steven

(And yes, we enjoyed a lunch out again today ~ appetizers and all!)

One Week

Reflections

One week since brain surgery.

The scar at the back of Steven's head is healing well.

Without a doubt, this is a haunting experience. Without a doubt.

And my heart breaks for this sweet boy.

Healing the broken heart seems to be a process, just as when the body mends. And maybe we never heal to be the same people that we were before.

Maybe we heal stronger. Maybe we heal with more compassion. Maybe we heal with unimaginable courage.

Maybe when our spirit heals, we heal a little better than before.

So we must be patient.

But week by week, day by day and moment by moment we feel the determination and hope returning.

It is a process.


Seven time down, eight times up.

Day Five

Cranky

Today was Steven's time to be difficult and demanding.

I don't know if it's a reaction to stress or all of the many drugs that have been flowing through his body for the past five days. I do know it happened on day five last time also.

Cranky.

(Just keeping it real.)

Home

And Other Funny Stuff

Steven's been home since 11:30 today. I didn't make it home until 3.

The list of errands was THIS long:

Pick up pain pills.
(Vicodin again...60 this time.)
Find a pharmacy that carried 2 mg Decadron.
(Who knew that steroids would be hard to fill.)
Pick up Clorox and other disinfectants.
(Dale and David are great at picking up.)
(But the house had a very odd smell.)
Get chili-cheese dogs from Campbells for lunch.
(He requested.)
Pick up Vanessa.
(Also high on his list of priorities.)
(My Goodness!)
Buy two new pillows.
(Steven's neck is so sore.)
Get gas.
(I was running on empty!)
(Literally and figuratively)
And so on...
and so on...

Isn't odd how difficult it is to really get home?

And other funny stuff.

Steven was very silly those first few hours in the ICU. Whether from the pain medicine, the anesthesia, or just the relief, I'll never know. But his comments are print worthy.

"Nuni, could you move my pillow up?"
"Now down a little bit."
"No, up a little bit."
"Maybe a little more down...never mind, I'm just messing with you."

"Mom, please itch my shoulder."
"Could you scratch my back."
"Now my leg itches."
"David help Mom...scratch my *****."

A scary low arterial line blood pressure of 50/35 caused a quick visit from a staff neurosurgeon. The doctor was giving Steven a quick neuro-type exam, and he asked Steven to rapidly touch his fingers to his nose then the drs fingers and back to his nose. And repeat, many times, quickly. Steven was slow and labored and at one point got his finger in the wrong place.

"Is it OK if I touch my nostril instead?"
"Oops, it's stuck!"

(The low arterial line b.p. was from a kink in the line! The blood pressure reading from the cuff ran fairly low...somewhere in the area of 90/50...for 24 hours, but not scary low.)

Wednesday, June 6th

9:00 a.m.

We're ready to move! Technically, we're not quite ready yet, as the nurses haven't indicated a quick escape, but emotionally and physically we're ready. Steven's in a steady pattern of eating, sitting, watching t.v. or surfing the web, and snacking on Vicadin. While his neck is still very stiff, he's doing much better, 48 hours post-op, than he did last time. And while the ICU is comfy and private with possibly the world's sweetest nurses, we're definitely ready to move on!

Steven has had the post-op MRI and CT scan. The drs have visited on rounds. I believe that most meds have ended, with the exception of pain pills and steroids. So, we are simply holding steady and waiting for a little more healing and a little more mobility before we come home...tomorrow!

Thanks you for the many thoughts and prayers.

1:00 p.m.

Steven is in a regular room now on the adult neuro-floor. I use the term "regular" very loosely here, as he has a huge room with a great view...and only one bed!

So, all is well ~ very, very well ~ in the world of recovery.

And now the pics...but if you are newly pregnant, naturally queasy or eating a snack...scroll down fast!

The bandage is off, to Steven's great relief.


While the incision is ghastly, Steven is beastly!
Coming out of his shower, 48 hours after the removal of a tumor in his cerebellum, Steven is balanced and strong!

Tuesday, June 5th

7 a.m.

We made it through that first grueling night. I've come to the conclusion that there is no benefit of past experience for brain surgery. I don't know any better how to relieve the pain. I can't reduce that residual nausea any more now than I could last time. I still have no idea how to help Steven relax the very tense and sore neck muscles. Learning curves do no good in brain surgery.

But, this morning he is resting.

The many medicines are still running through the lines. The pain has less edge this morning, the nausea is abating, and the neck muscles, hopefully, will relax a bit more with each new day of healing.

Yesterday evening was my emotional low. After a sleepless night, a tense day of waiting for news, the relief that this tumor is gone, and then the labor intense love of being in the ICU, I ran into my valley.

I just can't imagine that we will have to do this again. The hard part is knowing that Steven will face this for the rest of his life. This is so much bigger than a simple benign brain tumor. Rejoicing over the successful surgery for the pesky tumor felt odd when I know that the nasty brain stem tumor is still invading Steven's life.

But, this morning Steven is sleeping peacefully.

And now we'll have to start climbing back to the mountain top!


6:00

Twenty-four hours later, and he's sitting...

and eating!

Monday, June 4th

8:00

Steven's in surgery now.

Waiting was emotional torture...he was teary a few times, and I asked if he was nervous.

"Not scared Mom, just sad."

"Sad because everyone's not here yet...or because you have to do this?"

"Because I have to do this. Just sad."

I'm not feeling nervous or scared either. Not about this tumor with this doctor.

But I am a little sad too...and a lot angry.

A disease with no cure
that makes my son sad
has me a little bit sad
and a whole lot angry.


10:00 a.m.

No updates yet from the O.R...

...and waiting can be a tiring job!


12:00

We just met with Dr. v. and a couple of his compatriots as Steven is being stitched up and put back together. In about 2 hours he should be in an ICU room. There are details that I'm not yet processing (something about a missing piece of bone and newly inserted mesh) but the good news is that the surgery went exactly as planned. The one pesky tumor is gone, and with it the cyst.


1:30

Steven is being adjusted to a bed in ICU now. It seems that he will be in the pediatric ICU overnight, with great possibility of moving on to a regular bed tomorrow. I did get to see my sweet boy in the hallway on his way out of recovery. He was awake and speaking...but obviously drugged!


2:30

I.C.U.

Steven's sleeping.

They saved the hair!?!

Packing

I'm packing a few bags for Monday morning.

I've got a sweet little bag started for my little girl. She will be spending Sunday night with a dear friend, and then visiting other friends on other days. I have a pile of necessities started for Mary...underwear, p.j.s, shorts, shirts and swimsuits. I've also thrown in a few surprises...some Klutz and American Girl books. This will greatly shock Mary, since I'm always saying that those sort of treats are too expensive. But expensive is not a factor this time. Mary has been slightly weepy over the fact that she may not see me for three for four days. She's feeling about unsteady and unsure. So, expensive is not in decisions right now.

Although I'm trying to pack for Steven, he will most likely come and tell me that he want to pack his own bag. I have started gathering underwear, socks and p.j.s for him, as he will probably only pack the DVD player and a few favorite movies. Based on past experience, magazines and books will not be packed for Steven. He was totally unable to focus on a book or even a magazine last time. So, videos it will be.

I've started making piles of gear for myself. I've packed a clean set of comfortable pants and t-shirt for Monday's long night. While I know that little sleep will be had in the vinyl chair, sleepiness always feels better when you're comfortable! I also have the clean undergarments ready, although none of my socks match. For some reason, as uptight as I can be about most things, I never really care if my socks match! HA! Today I bought little travel sizes of toothpaste, mouthwash and deodorant, so those are in a line and ready to go. I will take a few puzzle books and the novel..."A Hope in the Unseen"...because I'm hooked on it!

And slipped in between all of my paraphernalia in my little bag will be two important bible verses...a verse about struggles, perseverance and hope and of course a verse about the great plans for Steven's wonderful life.

Romans 5:3-5 and Jeremiah 29:11

Pre-Op

A Little More than Blood Draws and Urine Samples

We learned a few things today.

We learned that the "visitor" entrance to the parking lot is really an employee entrance, complete with locked gates and secred codes. And if you happen to get lucky enough to get in, the exit is also a locked gate and secret code.

We learned that pre-op nurses don't know what von Hippel Lindau disease is.

We learned that a 7:30 surgery time means a 5:30 a.m. arrival time!

We learned that nurse-practitioners can just "tell" when a teenager is being dishonest about drugs and alcohol.
(Not Steven, of course! Just an interesting conversation!)

We learned that Steven still relies on his "mutant" jokes...but thank goodness the crazy laugh hasn't returned!

We learned that Steven is a bit more unsteady on his feet than I previously thought...at least when his eyes are closed.

We learned that forgetfulness seems to be my outstanding trait. Not only did I forget my wallet and the enclosed insurance card, thus necessitating a return visit home before the other half of the house was even awake, but I also forgot to ask how long the surgery would be. My best guess...four hours.

We learned that Steven's arms are extremely hairy, thanks to the cute comments from a sweet lab technician.

We learned that the length of the hospital stay is still undecided.

We learned that even though Tampa General is huge, sometimes the beds are full.

We learned that this hospital is on the cutting edge of infection prevention, as evidenced by the package and instructions that Steven received.


The 2% chlorhexidine cloths are to be wiped over every part of the body, except face and head, after a shower on Sunday night and Monday morning...with the important part of the procedure being to "air dry" the body after application! Oh my!
The bactroban ointment is to be applied inside the nostrils three times before surgery!

Questions

and Answers

I have been besieged by questions in the past 24 hours. Here are a few answers that I know, and even a few that I don't!

1. The surgery is set for the 4th of June at 7:30 in the morning.
There are a few pre-surgery tests that will need to be done at Tampa General, but I don't have times or dates for those yet.

Although I was still having a difficult time grasping the idea of celebrating on a Sunday and surgery on a Monday, Steven was warming up to the idea of surgery this coming Monday. A final decision was easy, however, as the necessary pre-surgery tests just couldn't be arranged.

So Monday, June 4th it is.

2. Several have asked why the doctors let the cyst get this large, and why we didn't pursue surgery last February. The answer is tangled in this crazy web of VHL.

The best I understand, and believe me when I tell you that it has taken me two years of sleepless nights and lots of discussion with doctors to accept this idea, these surgery decisions are based more on the VHL diagnosis than the presence of the tumors. Because Steven's tumors are caused by faulty genetics, he will be plagued by multiple tumors during the course of his life. That fact, when considered along with the size of the cyst, associated symptoms, and the ability of the brain to handle only a limited number of surgeries, is the major factor in deciding to go ahead with brain surgery.

Deciding on when to remove a hemangioblastoma in a VHL patient is a balance, and we are just learning this balancing act with Steven.

Be patient with my desire to cure him!

(And of course, I think back to the first visit with Dr v, when I calmly but persistently insisted that the brain stem tumor must be "zapped", and I cringe!)

3. I have no answers for why Steven is relatively symptom free. While dizziness is his best friend right now, Steven really has only stumbled three times that I know of.

4. I failed to ask specific questions about the surgery, much to the chagrin of Dale. I assume that things will be pretty much the same as last time, with the surgery lasting around 4 hours and Steven spending a day and night in the ICU, just for observation. I am also assuming that he will be in the hospital for about 4 days.

5. The neurological risks with this surgery are relatively small.

5%

Relative, of course, is the key word, because the
neurological risks involved include bleeding into the brain tissue, a blot clot in the brain, or death.

Still, this is a low risk in the world of brain surgery.

And I have no idea what 5% means. I'm assuming it means that if 100 such surgeries were performed, the horrible results only happen 5 times.

There are just some questions that I'm still not willing to ask in front of my sweet boy.

6. Other risks involved include pneumonia, blood clots in the legs, and urinary tract infections. These issues happen with a bit more frequency. However, Steven's age and excellent health should prevent these problems.

7. Dr v mentioned another risk. He inspected the old scar and told Steven that he would "unzip the zipper." Dr. v then mentioned the risk of complications with healing because of using the same incision. These complications include a longer healing time, risk of infection at the incision, possibility of leakage of cerebral spinal fluid from improper healing, and resulting meningitis.

8. Steven, however, has such great faith in Dr. v, and I think that makes a world of difference. He has a peace and acceptance with this surgery.

A peace and acceptance that really is beyond his eighteen years.

9. Steven wants a tattoo.

A "badge of courage".

(A small one, of course!)

Just the Facts

Because we just got home, and my mom is making dinner for us so I need to hurry, and there was tons of information to process, and we need to make decisions, and sometimes my heart is just breaking so much for that sweet boy and my energies are spent encouraging him that I can't come here and be uplifting...

today it is just the facts.

1. We saw another new resident/fellow today. Another long explanation of Steven's history.

2. I left December's scans in the van, because no one ever asks for the old scans.

3. I used valet parking, so when I left the room to retrieve the December scans, I had about 5 minutes of panic when I couldn't find my keys.

4. I returned to the exam room and told the resident that I would have just a little bit of trouble finding those scans...

5. Dr. v was prompt today!

6. And he hung the scans in the room!

7. And he had his usual bedside manner.

8. Nobody ignored the cyst this time.

9. After a lengthy explanation of how the serum actually leaks from the tumor, Dr. v gave us options.

10. Option one is to watch the cyst.

11. We all agreed that given the recent growth and Steven's symptoms, this is not a good option.

12. Option two is to use the gamma knife radiation to obliterate the tumor.

13. Dr. v pointed out that gamma knife is not really an option once a cyst has formed.

14. Surgery, through the old incision and then snipping through bits of cerebellum, was option three.

15. After another lengthy discussion about how the brain tissue stretches and the flexibility of the tissue in replacing the "blank space" left by the large cyst, Dr. v mentioned this coming Monday as surgery day. He also mentioned several pre-surgery procedures that would need to happen this week.

16. My brain went into hyper-speed, trying to remember all the important dates/activities in this week, graduation week, and next week, FSU orientation week.

17. I almost didn't notice the look on Steven's face. He just wasn't prepared to hear about a surgery looming that quickly.

18. Monday, May 28th is not a possible surgery day becaues Dr v will be scuba diving off the coast of some far-away island.

19. The other possible day is Monday, June 4th.

20. Dr. v warned that three weeks is a bit long to live with a large cyst that has been growing at the rate that Steven's has.

21. But Steven needs time to prepare...and we have a bit of celebrating to do this week.

22. I have to call the Dr v's office tomorrow morning with the final decision.

The Big Picture

Steven' Brain.

December 2006

February 2007

May 2007

All the same view. All the same tumor and associated cyst. Six months time.

I'm not sure what the appointment tomorrow will bring. I've been fooled the past two times, so I'm hesitant to even try and guess. But, if I'm forced to make a prediction, I would guess that Steven will be going to FSU in the fall, and the pesky tumor won't.

While I can now see what is growing in Steven's cerebellum and causing the episodes of extreme dizziness, I can only guess at what grows in his heart.

I do know that whatever is brewing around in there is tough, very tough. I can't believe that the complaints of dizziness have been as infrequent as they have, given the rapid growth of the cyst.

I do know that whatever is stewing around in Steven's heart is determined,very determined. In the midst of all of this "yuck", Steven has not missed one day of school nor one senior activity. There have been several days when I've seen him hold his head in an attempt to steady the room, only to press on and walk out the door to school.

I know that what ever is growing in his brain doesn't stand a fighting chance against the things that grow in his heart.

From the Beginning...

Just a brief history...for all new to Steven's journey.


Steven began complaining of dizziness in December of 2004. I tended to ignore these complaints, assuming he wasn't eating well or sleeping enough. Dale, Steven's dad, decided that more action was needed, and he took Steven to the pediatrician in January, 2005. An EKG and a CT scan were ordered. While both tests indicated abnormalities, the issue with the EKG was quickly resolved with a quick visit to a cardiologist. Steven's heart proved to be as sweet and healthy as ever!






The abnormal CT proved trickier. An MRI was scheduled for the following Monday, and I was directed by the pediatrician to schedule an appointment with a pediatric neurosurgeon ASAP. The CT indicated an abnormal vascular structure in my son's brain.







Our visit at the neurosurgeon's office prompted more questions than answers. The MRI revealed two small and compact lesions, one being in the cerebellum and one in the brainstem. Steven was poked and prodded that first day, and I was questioned about his overall health, his learning abilities, and family history. Neither the MRI results, nor the findings on Steven's exam, nor my answers to the drs questions provided a path for diagnosis or furthers treatment.






After speaking with a radiologist, the neurosugeon decided that we should scan Steven's spine and kidneys. The radiologist was looking for more tumors, indicating a disease called von Hippel Lindau syndrome.







These tests came back negative, except for that pesky little "incidental" cyst on the pancreas. At the follow-up visit the neurosurgeon it was decided that the cerebellum tumor could safely be removed and biopsied, and the surgery was scheduled for February 24th.


After a grueling night in PICU, Steven made a remarkably fast recovery. His only complaint by day two was that he had to stay in the hospital until the cerebral angiogram was performed. This test was done to look at these unusual blood vessels.




Tuesday, March 1, 2005, at noon our questions were answered. The cerebral angiogram was done under anesthesia at Children's Hospital. The radiologist met me in the waiting room with a description of what was seen. The angiogram showed two small "blush stains" in the areas where the tumors were seen on the MRI, indicating hemangioblastomas were growing under those tortuous vessels.





I immediately began researching hemangioblatomas that same evening. The tortuous, serptentine vessels seen during surgery are associated with these tumors. But more devastating, the disease once alluded to, von Hippel Lindau syndrome, was strongly associated with multiple hemangioblastomas.







The next few weeks were spent looking at Steven's eyes (thankfully still very nice and neat retinas), submitting blood and urine samples (all within normal range still), scheduling a second opinion with a different neurosurgeon (who reported three hemangioblastomas on the MRI...but now seeing five), trying to make our way into a study at the Institutes of Health in Maryland (success!), and receiving genetic testing (the definitive genetics results were received on May 11, 2005)...




...and in the past 18 months we have been shattered and healed, strengthened and humbled.

Join us for the story of Steven's life with von Hippel Lindau disease.