The bump (oops, I mean hole) gets bigger

As of Friday, my Dr. told me to keep a close eye on my vision (nice pun, eh?) and update him if anything changed. Unfortunately, over the weekend, I was noticing increased bluriness, "bugs/floaters", and wavy walls. There was also increased pressure in the eye. I saw the Dr. Tuesday afternoon. After examining me, he was shocked to see the drastic changes in my eye over such a short period of time. What had happened is that apparently the pressure I felt in my eye was the complete detachment of the macular gel from my retina, which unfortunately has caused my macular hole to double in size. That is what caused the significant change in vision. The only treatment for this is to do a vitrectomy. Below is a more detailed description of the surgery...

Vitrectomy for Macular Hole
Detaching the vitreous gel from the retinal surface is an important part of macular hole surgery. In addition, there are frequently thin membranes on the retinal surface surrounding the hole, which are peeled in order to release traction on the retina and allow the hole to close. Perhaps the most important part of the surgery, however, is filling of the vitreous cavity with a bubble of gas. This gas bubble must press against the macular hole in order for the hole to close. Since the macula is located at the back of the eye, the eye should be looking downward in order for the bubble to float against it and exert the maximal amount of force. In order for this to occur, the patient must remain in a facedown position after the surgery. For most patients, 2 weeks of facedown positioning is recommended. The macular hole can be closed successfully in the vast majority of patients. This is usually accompanied by a significant improvement in vision and reduction of distortion. Most patients, however, will not recover all the vision that was lost, and will recognize some limitation.

As with any surgery, vitrectomy has risks. Cataract, retinal detachment, high intraocular pressure, bleeding in the eye, and infection are among the possible complications. Cataract is the most frequent complication of vitrectomy surgery. Many patients develop a significant cataract within the first few years after vitrectomy.

The general population has a success rate of 90% with this procedure, but with VHL their experience shows this may be closer to 50%. It's unclear why - some speculate that it is because there is so much more going on in the eye, as well as scar tissue from previous procedures.

As of now, I have ordered my post-surgical equipment (a massage-type chair and a face support to help me hold the recommended position). I will be seeing my internist tomorrow for hopefully some good drugs to take after this, as well as EKG, bloodwork, etc... Surgery is scheduled for Tuesday morning. I hope to be able to spend one night at the hospital, but unsure whether insurance is going to clear that. Now I need to get my house in order so that over the next few months when I can't see, I will able to find things (or direct others as to where to find things) for the kids. I am overwhelmed by the thought of this and am trying to figure out how I am going to spend the next few weeks of my life facing the floor with no vision (and two kids). Steve is taking off one week of work & we will play it by ear after that. For now I am planning to listen to books on tape, listen to the tv, and hopefully chat on the phone (will have to figure out how to make that work). Some friends have talked about organizing meals and visits. It's hard to know right now exactly what we will need, but am sure it will be very much appreciated. I will let you know what I need after I get home.

Please check back on the blog late Tuesday or Wednesday to see how things went... If anyone has any uplifting audio books they would like to recommend, I'd love it. Keep me in your prayers!

P.S. I just received a package from my college friend, DiTy! It was a iPod nano and iTune gift card. This will come in very handy. Any song recommendations or help setting it up would be appreciated.

Another bump in the road...

After returning from MD Anderson, I spoke with my local eye Dr. about the hopeful options they presented to me there. Unfortunately, he feels that all of the options they presented to me there (other than the Sutent chemo drug) have significant risks of further vision loss. Because I only have one eye, he feels it best to try the Sutent drug and not pursue any of the other options at this time. I also spoke with a woman who is the head of the VHL Alliance. She is extremely knowledgeable on VHL and became a spokesperson after losing her husband 18 years ago. She also has a son living with VHL who is blind, after having tried all of the options they presented at MD Anderson. He also tried Sutent with no positive results. I plan to speak with him personally later this weekend. Needless to say, I am so disappointed. I was so excited after returning from MD Anderson & it's all been shot down. The constant ups & downs of raising my expectations and creating hope and then being let down is exhausting.

On top of that... beginning Monday evening, I noticed my vision drastically getting worse, as well as having a large black floater in my eye. It's like a big black fly is covering the center of my vision. As my eye moves, it moves too. It's driving me crazy & giving me headaches. I left several messages for my eye Dr. and FINALLY spoke with him yesterday afternoon. I told him I felt like I needed to be seen before the weekend because of the significant change in my vision. I knew that there was something wrong. Today I saw the Dr. I no longer have central vision & can't even see the eye chart unless I move around. The best I could get with movement was 20/200. The Dr. immediately noticed a MAJOR change that, believe it or not, it is unrelated to any of my existing tumors. There is no swelling, bleeding or leakage of fluid. What I have is a problem with the vitreous gel that lines the back of your eye. It is tugging so hard in the central portion that the layer is pulling itself away from my retina and distorting the central vision. The macular hole is like a men's button-down shirt that is two sized too small, forming a hole between the buttons. In addition to that hole, the men's shirt is also being tugged outward. This tugging is the gel trying to pull itself away. So now, what shall I do? He will touch base with me this weekend and I will page him if anything changes in my vision. If there are any changes, I will see him before Tuesday. If this does not resolve spontaneously, then it will require surgery to have the vitreous gel manually removed. Otherwise, the macular hole will increase in size. Stay tuned...

Also since returning from Houston, I was lucky enough to get the flu! Fortunately I recovered and am hoping for a sunny and warm weekend. We will be attending Tyler's 8th Grade Play - Bye, Bye Birdie - where he is part of the backstage crew.

I received a wonderful surprise this week! A group of my sorority sisters went together and surprised me with one of Oprah's "favorite things". I am now the proud owner of a new Kindle! The first book I downloaded was Kindle for Dummies! It will be very useful for me this week because it has an audio option and I can listen to books out loud. The other perk is that the font can be enlarged, even larger than large-print books. I am so grateful for their thoughtfulness. When I can see again, I will send along some personal thank-yous.

Two Nuts in Houston

This trip has been very therapeutic and has given me much hope!

Here are the highlights...
Surprise! First class to Houston for Kim. I got to not only experience what it's like to sit on the other side of the curtain, but also enjoy a full lunch service with free drinks to boot! The reason for this is that I flew on a buddy pass with my United flight attendant friend, Jeryl.

My Aunt Jan and Uncle Jim picked us up at the airport and surprised me with a mini family reunion at my cousin Amanda's house in Montgomery (about an hour north of Houston). We sat poolside and caught up on the last 5-10 years of each other's lives. I even got to feed carrots to her two horses. I met Amanda's daughter, Kiley (3) and my cousin Jennifer's daughter Lauren (16) and son Kayden (2 1/2). Even my cousin Sean (36) from Austin was there. We enjoyed the afternoon and topped it off with a wonderful steak dinner cooked by Amanda's husband, Jason. We finally arrived at our hotel around 10 p.m.

Our day began with a 6 a.m. wake-up call for a 6:30 appointment at MD Anderson. The hospital is an incredible cancer research facility. It consists of 15-20 buildings, connected by indoor walkways. You shuttle on golf carts from building to building for various appointments -- very cool!
The first doctor I saw was Dr. Dan Gombos, Associate Professor of Opthamology at MD Anderson. He was very knowledgeable on VHL and left me with several options of hope. 1) Direct or proton beam radiation (which, even if it works, will cause more vision loss), 2) Avastin (a drug given by IV injection into your eyeball - ouch! - which will increase the eye pressure & possibly the macular hole size), and the most promising-sounding is PDT (photodynamic therapy - direct laser following IV injections that that will cause the laser to only affect the tumors and not cause damage to the rest of the eye). Lastly is the Sutent chemo drug, which will not cause my eye to get worse, but may or may not help it get better. Some of the treatments he recommended were "shot down" by the doctors at NIH. Yet he felt they may be worth a try - who knows? Just because it hasn't worked on other VHL patients doesn't mean I would have the same outcome. I left a message for Dr. Meyerle at NIH to discuss her opinions and any research she had regarding treatment options. Luckily, most of my testing reports and discs were sufficient enough for the doctor. I only needed some pictures taken of the eye, an ultrasound to measure the lesions, and he recommended another scan to confirm the macular hole (which it did). This Dr. appointment was encouraging as he felt I would be a good candidate for the Sutent (chemo) trial.

Now it was 1 p.m. and we were off to see Dr. Eric Jonasch - he is the renal oncologist (kidney/VHL dr.). He also has a special interest in VHL - he considers it his baby. He wrote the Sutent protocol and found me to be a perfect candidate. To date, he has had 15 patients with VHL on the drug. Two had stopped taking it due to severe side effects. Two had increased growth of tumors and therefore went off chemo. The other eleven patients have had promising results. Three of them have had eye lesions - if they haven't shrunk, they have remained stable. He has even seen shrinkage of not only kidney tumors, but also pancreatic and kidney cysts. No one has ever bled on this drug & many have had brain or spinal lesions similar to mine. What a relief! I feel this drug holds a lot of promise for people with VHL. If not for the eye tumor getting smaller, it may have a positive effect on my other tumors/cysts. I am now going to research the options I have been given, as well as pros & cons, etc... Lots of homework!!! The soonest he could start me on the Sutent is mid-June, unless I cancel our cruise, because he would need to see me during that time. This is probably best anyway, because I don't want to be out of the country and on a cruise ship feeling sick.

All day we never had a break for breakfast or lunch, and only snacked on Cheese-its and candy. It's a good thing I travel with a bag of gum, skittles, sprees, etc. at all times! We became so slap-happy, that we were caught taking a picture with a blown-up hospital glove by an entourage of doctors. After returning from MD Anderson around 5 p.m., we changed into swimwear and went to sit at the Jacuzzi to discuss the day. What a different & positive feeling it was after having been rejected from the Sutent trial at NIH & knowing that these people have much more experience with the drug. Jeryl and I wanted to go out and celebrate & SURPRISE!, her cell phone rang. It was her sister-in-law, Lydia, who lives in downtown Houston. She wanted to take us out to celebrate Jeryl's birthday and my good news. A few hours later, we were dining at a French brasserie, Max & Julie's. It was a very delicious and upscale urban restaurant. Sorry, Nancy! You will have to join me next time.

We got up Thursday morning after sleeping in until 8 a.m. After sitting in the shade by the pool for about an hour and starting to write the blog, we were picked up at noon by our new cabdriver friend, Mike. He was so sweet. He heard that we ate a lot of snacks the day before and brought us a bag of mini Snickers for our ride home. We have to save his business card for future trips. We nicknamed him "Mike Candy" instead of Mike Cabbie.

Once again, it was first class on our return. Much thanks to all our friends and family that helped with our children while we were out of town and made this all happen.

What's next? First, I will do my research and check out my options. I will continue to see if there are any major institutions studying my type of eye problems. Perhaps there will be one in Hawaii or San Diego or some other tropical location. I could really get used to traveling first class on the buddy pass system. Thank you again, Jeryl, and Happy Birthday!

My Story

I am presently a ripe young age of 47. This is my story “in a nutshell.”

Life was normal as a child until my Dad died. I was almost 7 years old. He died October 24, 1968, at the age of 30. My Dad died from what we now know as VHL. Von Hippel-Lindau (VHL) is a genetic condition involving the abnormal growth of blood vessels in some parts of the body which are particularly rich in blood vessels (please click on the sidebar link "What is VHL" to learn more). On autopsy, my Dad had multiple brain/brainstem tumors, spinal and kidney tumors, as well as being blind in one eye. The brain/brainstem tumor is what eventually caused his death following surgery.

I was diagnosed at age 13 with retinal lesions. I had many laser surgeries and many major surgeries on my left eye, with ultimate blindness in 1990. After many years of pain in the dead eye, it was eventually removed in 1992. Surprise for those of you who didn’t know! I now battle with retinal lesions on my good/seeing eye and also have a large optic nerve tumor. This is untreatable presently, as all methods have failed or caused worse vision, and the nerve is connected to your brainstem. Recently (2009), I was diagnosed with a macular hole, affecting the area of the eye that controls central vision. Unfortunately, I only see about one half of faces, have difficulty reading, telling time, etc…

My “terrible twos” came in my 20’s, at the age of 22, after dizziness, headaches and many falls. I had my first brain surgery. In and out for 48 days, I was eventually released from the hospital only to find there was another tumor directly behind it. The following summer, the “zipper” incision was reopened and this time I felt much better. What a way to spend my college summers! I still remember going out to the bars and my hairpiece falling in to a puddle or shifting on my head with a gust of wind. It was actually quite humorous.

My head was reopened two more times to remove a tumor from my left inner ear that had caused deafness and dizziness.

Over the years, I have had three partial nephrectomies (removal of the kidney) for renal cell cancer. Lastly, I tried a new technology where they burned the tumor. I have a total of one kidney left.

One lesson I’ve learned is you much be your own advocate. Another is not to always think that “no news is good news.” When my daughter, Alyssa, was six weeks old I had spinal cord surgery. Unfortunately, my neurosurgeon and internist failed to check my MRI scan and reports (each thinking the other had done so). Once they finally reviewed them weeks later, they wanted to do emergency surgery. What a scary, messy nightmare. I still have a lot of upper back pain, numbness and shouldn’t lift over 10 pounds – like that was easy with a new baby!

Lastly, I had brain stem radiation in October 2006 to treat a tumor connected to a 2-inch cyst in my cervical spine area. So far, so good – recent scans show that the tumors show no growth and are stable. I love that word “stable.” The down side is because the tumor hasn’t gotten smaller, I still deal with the symptoms of severe nausea, headaches and lightheadedness. My adrenal glands have stopped functioning because of the long-term use of steroids and now I take Prednisone to replace the cortisone your body naturally produces. Prior to discovering this problem, I was a “couch potato” for months and had difficultly focusing. Even a “venti” from Starbucks couldn’t get me moving!

So that about covers my life in a “large nutshell.”

Along my journey, I lost my mother in May 1999. She was the most wonderful, positive person. I don’t know what it must have been like to watch all four of your children suffer and go through many life-threatening surgeries. The only positive note of her death was she got to die before she had to watch her children die first. Ironically, she died from sporadic kidney cancer, found by trying to donate a kidney to my brother. This is probably why our kidney cancer is so prominent.

The medical bills were insurmountable and eventually our story went a bit public. Some of you may vaguely remember my family’s (Kolinsky/Willman) story being covered by Carol Marin on NBC Channel 5 news. She did a series of special interest stories and followed our family for many years during the late ‘90’s.

My sister, Lisa, passed away on September 9, 2001, from a complication related to her brain stem tumor and steroid use. My dear sister had just turned 37 years old as she lay in a coma. I’ll never forget how surreal it was writing her eulogy on 9/11/01. She had many friends/family at the service, but those out-of-state were unable to fly.

I have two brothers that are living with VHL. Steve is 48 and resides near Schaumburg. Jeff, 42, lives near Denver, CO. Both have lost their kidneys to VHL and have had kidney transplants over the last few years. Presently, they are otherwise stable. I also have a half-sister, Robin, 38 in PA. She is unaffected by VHL as she is from my mother’s 2nd marriage, but has cervical cancer to deal with instead. We are all there for each other, especially during a crisis, but otherwise we try not to make health issues our focus.

My goal for now is to seek out possible treatment for both my optic nerve tumor and macular hole. I’m always open to alternative medicine and am trying to focus on a healthier lifestyle.

I am blessed to have so many wonderful, giving friends. You’re all like angels on earth for me. I am also blessed with a great immediate and extended family. Most importantly, I have a wonderful, supportive husband, Steve. In addition to the normal stresses of life, he has the added stress of dealing with my situation. I am doubly blessed with two beautiful children – Tyler (13) and Alyssa (6).

I have no plans of checking out soon and have no time to pity myself. I have grown to appreciate life differently and try to enjoy each day as if it’s my last. I am a SURVIVOR.

It's all about us...

Since I last touched base, we have all come down with the dreaded viral/flu/ear infection/sinus infection/pneumonia/bladder infection. You name it, we've had it at the Gillespie infirmary!

So now what's new with ME...
I am en route to M.D. Anderson in Houston on Tuesday, April 14th for the week to see their eye specialist and oncology team that follows VHL. Wish me luck. They have some new ideas and possibly a new drug trial coming up soon.

This past week, I had a new prosthetic eye made. It was well overdue, as it has been 10 years and you are supposed to get a new one made every 4-6 years. I will be picking it up on Tuesday, after it gets painted and fired. This week I also saw an ocular specialist who deals with visually impaired patients. She gave me some tips on how to deal with my central vision loss, including improving the lighting in my home and software for my computer (this will allow me to blow things up HUGE and even make my computer talk).

Spring Break has been very uneventful... Seems we were one of the few families who stayed in town. We were glad however, since we were all sick! My son, Tyler, is looking forward to taking a late "Spring Break" as he is traveling to Dallas for an international soccer tournament this next week. He is so excited to play the teams from Europe and exchange jerseys. They even have an opening and closing ceremony, just like the Olympics!

Hoping that you all had a lovely spring break & wishing you a blessed Easter. Hopefully, some of you will bring the sunshine back with you from your warm-weather travels!

Love, Kim