Sunday, August 31, 2008
Saturday, August 30, 2008
Friday, August 29, 2008
Wednesday, August 27, 2008
We were fully expecting confirmation of what Dr. O had told us. We asked Dr. P to perform a dilated exam today without anethesia so that she could tell us if it was obvious that we should perform the enucleation as soon as possible then work on the left eye. After the initial exam Dr. P informed us that she could see no tumors in the fovea region of the eye. Thus the central vision area for both eyes was potentially salvagable. She explained that chemotherapy would likely shrink the tumor closest to the optic nerve and then the cancerous cells could be killed. The viseral seeding (tumors floating around in the eye) were difficult to treat with chemotherapy but she thought that in Gracie's particular case there was a 50% chance the visceral seeding could be controlled and the eye would not need to be enuclueated.
Now this was her assessment based on a limited examination, information from Dr. O and the pictures of the retina we had brought. She wanted to perform the EUA tomorrow to make a final determination.
Dr. P also introduced us to an oncologist, Dr. M. Both Dr. P and Dr. M are the specialists for retinoblastoma at the hospital. Dr. P sees, on average, a new retinoblastoma patient every two weeks. Dr. M is the oncologist that works with them. Both of them explained that enucleation is not the first option for them. Treatment with chemotherapy before enculeation in fact was thought to reduce the liklihood of the cancer spreading during enucleation.
Dr. M also explained that the chemotherapy would likely be performed as an out patient service for three days straight every 28 days. The first cycle would likely be 6 months. It was continually emphasized to us that there was no gaurantee that we could save the right eye but there was a possibility. In addition neither doctor felt that there was any increased risk to Gracie should be commence chemotherapy in the week. Depending on how the tumors responded to the treatments, the decision to enucleate may be necessary.
Suffice it to say, we had a very positive experience and the prospect of saving both of her eyes is incredible. In fact Dr. P said that it was a miracle that the fovea in the right eye remains intact with all of the growths.
We are really excited about the treatment proposition from Dr. P as it means that Gracie may have a chance to save both of her eyes.
We know that tomorrow may bring a different story after a thorough exam however it is nice to feel optomistic about saving her right eye. It is often difficult to undergo the roller coaster of emotions from one day to the next however we pray continually for improvement and for understanding. We appreciate the prayers on our behalf and are confident that the Lord is mindful of our Gracie. He has truly blessed her and blessed us.
The right eye appeared to be unsalvagable and would have to be enucleated (surgically removed) in order to stop the cancer from spreading. In fact one of the tumors appears quite close to the optic nerve and this is a primary mode of transport for the cancer to the rest of the body. The left eye was treatable and the growths were relatively far from the fovea (place in the retina where most of the light hits and hence how we see). So chemotherapy would be recommended to reduce the size of those tumors before laser treatment. The laser treatment would then kill the cancerous cells and leave only minor scaring.
While these do not appear to be good options they are much better than allowing the cancer to take my beautiful girl.
Gracie was returned from her MRI after our meetings with the specialists and she soon was herself again. Happy and bubbly, her eyes beamed and her smile radiated love. Now the opaqueness in the eye is more frequent and offers a continual reminder of what is ailing her. She seems to have had no physical pain in her eye through all of this and her vision, until very recently, has been fine. Our pediatric ophthalmologist thinks it is likely that the cancer in the right eye has only recently been broken off of the retina and the tumor is not necessarily growing rapidly but peeling off the retina (those are my words not hers) so that it is larger and more visible.
I have spent hours looking through old photos for and evidence of leukocoria (cat's eye or white reflex) as this is often the first sign of a problem. There is only one picture from a couple of months ago that is suspect. Many of the others show a definite red reflex in each eye. Our pediatric ophthamologist says that this is not unusual and that there is no way we would have known there was a problem except with an exam under anesthesia like she did today.
We have many unanswered questions today but know that since it is heritable all other children must be tested and evaluated frequently. It was a real struggle to tell family and loved ones the news that we had so desperately hoped and prayed we would never have to receive. However we feel blessed with the prognosis and the early identification of a problem in the left eye. Gracie is strong and well and we are confident that she will overcome all of the difficulties that come her way. As one can probably imagine there has been a roller coaster of emotions today. We do not really want to believe what is being said but we also want to do everything to avoid further problems.
We spoke to Gracie's siblings today about what must happen and they are nervous for her and often do not understand why it has to be done. However it was nice for them to know and for us to talk to them about how much we love them and are concerned for them. We will do everything we can to keep them safe. They had many questions and I am sure will continue to ask. It is disconcerting for them that Gracie's new eye will not let her see from it but they are happy to know it will look almost exactly like her old one.
No dad wants to experience a day like this one and I think little prepares one for such experiences. However, I am happy to have Gracie and will love her no matter what.
This is the left eye. The top white spot with all the blood vessels coming out of it is the optical nerve. The other two white blurry spots are tumors that must by killed before they grow too large.
This is the right eye with the focus towards the optic nerve (right side of picture, small white dot), The large white mass covering a large part of the eye is a tumor that is not entirely attached to the retina anymore. The biggest concern however is the tumor that is adjacent to the optical nerve (just above in the picture). The optic nerve is the primary means that eye cancer moves out of the eye to the rest of the body, so this tumor must be stopped prior to it growing to the optic nerve.
This is another picture of the right eye (similarly aligned) but more focused on what they call visceral seeds. These are cancerous growths that are floating within the fluid in the eye. You can see quite a few in the bottom left corner of the picture. This is concerning because they are hard to kill as they are not connected to the blood supply.
Tuesday, August 26, 2008
Remarkably an eye examination under anesthesia and an MRI were able to be scheduled back to back on the Monday (August 25, 2008) following our return from vacation. We later learned that scheduling these two events usually takes at least 3 weeks advance notice however we had made the final arrangements less than 5 days prior.
Normally CT scans take longer than a few hours to arrange however the CT scan was performed in the afternoon. Gracie was good enough to be still for the scan. The hospital staff had given her medication to sedate her but she complied before the medicine had a chance to work.
Following the scan we waited anxiously on the results. A couple hours later, word from the radiologist was that there was a small bit of calcium in the eye. This was not what we wanted to hear but it seemed promising that only a tiny amount was found.
Later that night Dr. L called me and explained that he had put a call into the pediatric ophthalmologist that he knew and was waiting for a response. He said that the reason he called was to say that he had done some research and Gracie's symptoms were consistent with retinoblastoma (eye cancer). He did not want to be alarmist but wanted to make every effort to rule it out. He said that the next step was to get a CT scan as soon as possible. He would call us back with more details once the pediatric ophthalmologist got back to him.
"Retinoblastoma (RB), is a rare form of eye cancer that affects the retina of infants and young children. It occurs in approximately 1 in every 20,000 births. About 60% of the children with retinoblastoma will have tumors in only one eye. This is referred to as unilateral retinoblastoma. The remaining 40% will have multiple tumors found in both eyes. This is referred to as bilateral retinoblastoma. RB can range dramatically in severity. No two cases are identical nor are they necessarily managed in the same way. a number of different treatments are available to children with RB. The severity and location of the tumors will determine which treatments will be suggested. With early detection and modern medical care, the survival rate for children with RB is 96%. Ongoing research and new treatment developments are allowing an increasing number of affected eyes to be saved and are maximizing the visual outcome and quality of life of each affected child."
I thought that this definition was pretty good. Other retinoblastoma sites and wikipedia have similar definitions. The breakdown of Unilateral/Bilateral varies from 60%/40% up to 75%/25% but the rest of the definition is pretty consistent with other definitions out there.
Gracie is my daughter. She is almost three years old and has the most beautiful smile and big shiny eyes you can imagine. So imagine my surprise when on August 25, 2008 I was told that Gracie had bilateral retinoblastoma and would need to have her right eye removed. Gracie has many friends and family members who love her and are interested in knowing more about what is happening to her right now. Therefore I have created this blog and I will try to update it on a regular basis and help everyone understand how Gracie is progressing from the initial diagnosis of retinoblastoma throughout the healing process. Any comments or questions that you may have are welcome. I am no expert in retinoblastoma but I am learning lots more each day so may be of some help in addressing any questions.