August 31, 2008 (Sunday)

A quiet, relatively peaceful day today. As usual we spent the morning in church services and enjoyed meeting with good friends. Most seemed to have already found the blog and spoke favourably about how useful it was for them. I suppose that gives me more motivation to keep posting and I am glad that some have found it helpful. Those that help Gracie in her nursery at church were sad, a) that she has cancer and b) that they will likely not see much of her after next week. The doctors have encouraged us to avoid crowds and especially lots of other children when she is on chemotherapy so we will probably not take her to her nursery class again after next week. Plans may change but that seems to be the plan for now.

It continues to be difficult waiting for the week of treatments. We know that hospitals and doctors have many patients to see but sometimes we wish that they were ready when we were. Again, I re-emphasize, our doctors have been excellent and we are grateful however we would also like to get going with Gracie's treatments.

We remain confident that Gracie is strong and the prognosis is great, however we still go through a variety of emotions every day. I found a good article on that a couple of days ago at http://www.retinoblastoma.ca/psycho.htm (I love the address by the way. It actually refers to psycho-social aspects and the impact of retinoblastoma on the family and not some psycho with retinoblastoma). The article makes going through emotions sound fairly linear but we have found that the feelings of shock, grief, anger, despair, anxiety, peace, etc. seem to flux from day to day, hour to hour, and even minute to minute sometimes. I would like to think that this is normal, however if it is not normal I am positive that it is not infrequent for people in our situation.

Given all of that, we find an overarching hope and happiness in trying to follow our Saviour. We know that it is through Him that real peace and a perfect understanding can come. And so we push forward in faith and are continually buoyed by the love and prayers we receive from all of our family and friends, wherever they are.

August 30, 2008 (Saturday)

Well, there is no additional news on Gracie treatments or developments to report today. It really felt like today was pretty normal, except that it is hard to know what day of the week it really is. There were no tests to do or doctors to see. Days like today should be treasured but today we were anxious to get going and do things, like chemotherapy. That may sound bad but now that we know about the cancer we want to get to work killing it. I know there needs to be preparation and tests before chemotherapy but it is hard to just live life normally when you know what you need to do but have to wait for it.

For those of you who have been following the blog daily, two days ago I added a few pictures and some narratives on those pictures to previous posts. You can use the site navigation buttons on the page to see prior posts if you have not seen the pictures. I have also taken the liberty to correct spelling and grammatical errors in previous posts, of course this only occurred once they were pointed out to me. I will likely continue that practice in the future.

There was a question raised from the Wednesday (3 days ago) post. It seems that some interpreted what I said to mean that Dr. O and Dr. P saw two different things in their independent examinations. This is not what I meant nor what I think occurred. Rather they saw the same problems but had different treatment proposals for what they saw. There is no indication that Dr. O missed something Dr. P saw or vise versa. Both had the same diagnosis of bilateral blastoma, with several tumors in the right eye (one dangerously close to the optic nerve) and 2 in the right eye. I removed one sentence from the wednesday post to clear up the confusion but this does not change the content of the post for that day.

Another question that has been asked in various ways to us is how we feel about Dr. O now that Dr. P appears to have given a much more promising prognosis. I think this is a fair question and one that many people would ask in similar situations. As a follow up to that question is why would the two doctors give such different prognoses given that they saw the same thing. I will try to address those two questions.

First of all, we think that Dr. O is a wonderful doctor and look forward to working with her in the future. She comes highly recommended and has not disappointed. Importantly she was the one that insisted on us getting a second opinion to be sure of our decision and even set up the appointment with Dr. P's office. Remember too that retinoblastoma is rare and not seen that often. Dr. O likely sees a few cases throughout the year where Dr. P sees a new case every 2 weeks or so. Dr. P works in a larger center which has a multidisciplinary medical group that specifically focus on retinoblastoma. This group follows cases and studies the results from different treatments. So current knowledge of treatments is likely higher for Dr. P then for Dr. O. Even given all that Dr. P was not surprised and did not find Dr. O's recommendation (immediate removal of the right eye) unreasonable. She explained that their group's current protocol for treating bilateral retinoblastoma was to first start chemotherapy to reduce the size of tumors. Then if there was an eye where vision was unsalvageable, enucleation would be performed. So in following that protocol and based on her experience she gave that recommendation and gave as an opinion that an eye like Gracie's right eye had a 50% chance of being salvageable.

While talking with Dr. P she mentioned that if the disease was unilateral (only in one eye) and that one eye looked like Gracie's right eye, she would likely recommend immediate enucleation. Unilateral disease is more common for retinoblastoma patients and there are fewer complications when it comes to treatment when compared to bilateral. So it is not at all unreasonable for a doctor to recommend enucleation more often than not when an eye looks as bad as Gracie's right eye. Given all of these factors and our many positive experiences we are very pleased with Dr. O (and Dr. P). They have not brought us the best of news but have helped us out so much and we are very grateful.

Since I have spoken briefly about it, I will just mention some of the reasons why there are different treatments depending on the bilateral or unilateral condition. Firstly, bilateral retinoblastoma means that the child has a genetic mutation in every cell of the body and thus more likely to have cancer in the future than a child with unilateral retinoblastoma. Secondly, bilateral disease means that both eyes must be treated as opposed to one eye in the unilateral condition. So for a child with the unilateral disease, chemotherapy, radiation and other such treatments will not even be necessary if the cancer is surgically removed (this means the eye comes out too). In the bilateral disease, other treatment options must be employed anyway so enucleation is not necessarily the top of the list. Also any treatment to the eye could result in some visual impairment. In unilateral blastoma the non-cancerous eye will not be affected by treatments thus visual impairments are reduced considerably compared to children that require treatments in both eyes. So every effort is made to preserve whatever vision is in either eye in the bilateral case and minimize future visual difficulties.

I hope I was at least somewhat clear with that explanation. My family and I truly admire the medical professionals that we have been working with! They are amazing people and literally work miracles. We feel so blessed to have such good people that we can trust and find solutions for helping our little Gracie. Gracie has undoubtably been protected by a loving God. As I have said before it is miraculous that with so many tumors, the central visual centers remain unscathed. It is a miracle that we were able to find the tumor when we did (thanks to my wife and her keen awareness regarding Gracie's health). And it is a miracle that we have been cared for in such a timely manner over only a matter of days.

Thank you again for your prayers and for your thoughts.

August 29, 2008 (Friday)

So this morning we had our first real appointment with an oncologist. Dr. L met us to discuss chemotherapy options. It was nice to hear more about how chemotherapy would work. Dr. L had not spoken to Dr. M (the oncologist we spoke with briefly on Wednesday) who would work with the ophthalmologist Dr. P on Gracie's treatment, but suggested that the two drugs we would probably use most are: Vincristine and Etoposide. I may be corrected on that but I think these are the two. Vincristine works but stopping the spindle fibers from forming during cell division and Etoposide stops DNA synthesis. Side effects do include nausea and vomiting, but those can be controlled by other drugs. In addition there may be hair loss and potential negative effects to hearing and kidney function. She will be monitored closely of course for all of these things. Even if we travel for the chemotherapy treatments to be near Dr. M, Dr. L will monitor Gracie between treatments.

The plan is to have 3 days of chemotherapy as an out-patient in a hospital or in a cancer clinic every 28 days. She should try to stay away from other sick children and particular for the 5 to 7 days during the cycle that her blood counts will be low. This low blood count period will likely occur 5 to 8 days following the start of chemotherapy. Dr. L spoke positively about us being able to undergo treatments near our home and have Dr. M monitor our progress however working that type of schedule out is proving frustrating. Dr. L was quite easy-going and very friendly. She took lots of time with us and addressed all of our questions. They had a child life worker there with Gracie and Gracie played with the Sammy doll. She inserted a port-a-cath under the dolls skin then administered chemotherapy, just as they will for Gracie. Gracie also loves the colourful decorations in the offices and rooms. We were shown around the treatment rooms and given free ice cream. The child life worker took information to set us up with several agencies that assist cancer patients and their families. It was a real positive appointment.

Right after the appointment I went to work for the first time in a couple weeks (I had been on vacation for a week and then with Gracie's diagnosis had not returned until today). It had felt like months since I had been there.

Throughout the day, my wife and Gracie's cancer care coordinator, was busy making phone calls and trying to set up appointments. By the end of the day it still seemed like Dr. M and Dr. L had not really spoken or at least weren't on the same page yet. We had two separate days in two separate cities for the insertion of the port-a-cath (I guess I should explain that this is a devise inserted just below the breast and under the skin that gives direct access to the subclavian vein, for either administration of chemotherapy or blood work). Base level testing of kidney and hearing functions were scheduled for Sept 9 and chemotherapy was to being with Dr. M on the 10th through the 12th.

So on the one hand we are really excited however this is a week longer then we really wanted to wait. Now all of the doctor's offices are closed until Tuesday (Monday is Labour day) and so our questions are likely to go unanswered until then.

I would still like to know

1) is there anyway to arrange so that chemotherapy can be done closer to home (like with Dr. L)?

2) are the chemotherapy appointment dates very rigid (i.e. do they happen every 28 days regardless of holidays, how Grace feels, how well the last treatments went, etc.)?

3) how much is this all going to cost (no one really seems to know this and while it is secondary to the whole matter I think it prudent to plan as well as one can)?

Hopefully those questions will come and maybe no one really knows the answers right now but hopefully in a few months we will be able to give someone in a similar situation a good idea as to what to expect.

So today was great in that lots of my chemotherapy questions that I had come up with were answered but then a bit discouraging in that it feels like we are being told to sit tight for a 1.5 weeks and wait for everything to start rolling. This is driving my wife crazy as she feels like if we're not killing the cancer it's growing. So let's start killing it now! However, we are really happy with the medical professionals that we have met and that have cared for us. We continue to be amazed and grateful for the love and support of family and friends. Gracie continues to be pain free and a real delight in our home. She has been so good at all of her appointments and accommodating during all of her treatments. As you hopefully know, we love Gracie and are finding out how many others really love her too.

August 28, 2008 (Thursday)

The great news continues today!! We were so worried that after getting our hopes up that Dr. P would find something in the EUA that she had not seen the day before and tell us that our only option was enucleation. However, the EUA happened ahead of schedule and when Dr. P came and spoke with us she told us that she thought there was a 50% chance of saving the right eye with chemotherapy! That is really exciting. I had been terribly sick over the last two days and so it was hard to be too overjoyed but it was a great relief to know her vision may be spared in both eyes. And yes we consider even the possibility of saving the sight in both eyes a miracle.

Our baby had gotten sick with strep throat earlier in the week, I

 had fallen ill early yesterday and today Gracie was becoming sick. The nurses thought that maybe she should not go under anesthesia however Dr. P insisted. Gracie went through everything fine and seemed actually quite chipper after the exam. Once we arrived back home we all went to our family doctor and got medication. Gracie needs to be better so that she can start chemotherapy as soon as possible. We are hoping to start Tuesday next week. Dr. M (our oncologist) should be calling tomorrow to give us more information. We are going to visit another oncologist, Dr. L tomorrow morning. Dr. L will be taking care of Gracie between chemotherapy appointments so that if there are any problems or additional monitoring that needs to occur we do not have to make the 3.5 hour drive to Dr. M.

We have been truly blessed and feel the Lord's hand in our life. We are grateful for our many friends and family members who have prayed for us and rendered such incredible service. We came home today to a sweet smelling, spotless home and beautifully cared for yard after being gone for a couple days. We have people of many faiths praying for us and following Gracie's story. We feel such strength and energy from you all. Thank you so much and please continue as your prayers are working!

Here is Gracie after her EUA today. She was pretty perky and really wanted a hamburger. I did not correct the red eye so that you can all see that the red eye reflex is still very much present in both eyes (its absence is usually what pediatricians look for in children and may indicate retinoblastoma). It's more visible if you click on the picture to see it large.

August 27, 2008 (Wednesday)

At 5AM this morning my wife woke me up and said that she would like to go for a second opinion (Dr. O had recommended we go for a second opinion and even set up the appointment for today, but we were hesitant to have Gracie undergo more treatments then were really necessary). We had an appointment with another ophthamologist Dr. P at 9:30AM and it was 4 hours away. So we rushed to get things ready for an overnight trip (the Exam under anesthesia, EUA, would be performed Thursday). We left the older children with friends and headed out.
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.

Gracie sleeping at our hotel. Such a little cutie. She would not take off her backpack, her crocs or let go of her sunglasses. I think she was afraid of us losing them and then I can hardly blame her for holding onto them. Sometimes I wish that not losing something was as easy as just holding on tight.

August 26, 2008 (Tuesday)

We made lots of phone calls today. We had an appointment for a second opinion on the diagnosis to be performed in another city but have decided that the two specialists and our own observation of the pictures are evidence that things should move more expediently. We are trying to proceed with the enucleation of the right eye and chemotherapy as soon as possible. Currently we have oncology and oculoplastic consultant appointments arranged for Friday. This would result in surgery next week. We hope to move that forward but have not heard back from the doctors. Many friends and family have extended loving support and comfort. For this we are extremely grateful however it is still difficult at times to think about what we are asking Gracie to go through. We are still uncertain of any chemotherapy regimes and wait for a clearer picture of the future.

August 25, 2008 (Monday)

Getting to the hospital in the morning was quite the adventure as our van had a flat tire and required repairs. I sent Gracie and her mom in a taxi. We called ahead to the hospital and they wanted to reschedule but were convinced to keep the appointment. The exam and MRI happened on time. A retinal specialist and our pediatric ophthalmologist performed the exam, taking several pictures of the retina for us to see. They also reviewed the CT results from last Tuesday and the MRI results from today. The diagnosis was worse then we had ever even considered. Instead of a small tumor the right eye was full of tumors. The left eye also had two smallish tumors. Bilateral retinoblastoma was the diagnosis. That meant that the condition was hereditary and that chances of secondary cancers was greatly increased.
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.

August 20-24, 2008

The rest of the week was full of making arrangements to see doctors and talk to specialists. Many people offered support and we were connected to some of the best medical professionals in the area of retinoblastoma care/research. We learned much and realized that the prognosis seemed good. Gracie was older than most for identification of retinoblastoma (particularly bilateral retinoblastoma) and much of the evidence seemed to indicate that the cancer was not well advanced. However the opaque appearance in the eye seemed more frequent and was disconcerting as this seemed to be indicative of what they call visceral seeding. Visceral seeding in retinoblastoma patients is difficult to treat and often means that the eye needs to be removed. This was not exactly the solution we were hoping for less than a week ago but if it prevented the cancer from spreading we were willing to have the eye removed.
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.

August 19, 2008 (Tuesday)

Our friend called back and said the CT scan was definitely needed. We arranged for the scan of the brain and orbit without contrast (per instructions from our own pediatric ophthamologist). It was explained to us that we were looking for calcium in the scans. Calcium in the eye would strongly indicate retinoblastoma where the absence of calcium would mean it was likely something else.
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.

August 18, 2008 (Monday)

A very kind ophthalmologist and family friend, Dr. L, opened up his office early in the morning so that Gracie could be evaluated. We assumed that he would be able to address the opaque eye issue and give us some advise like, "This happens occasionally. Just watch it for a couple more weeks and let your pediatrician know if it does not go away. It is really no big deal." or "It is really nothing, don't worry." Instead he told us that there was certainly something there and we should have it looked at by a pediatric ophthalmologist as he had never seen it before. (He did dilate her pupils but she was not under anesthesia.) He was not sure if it was serious or not. He mentioned that it could be cancer, the result of trauma, or something else that he did not know about. He told us to make an appointment as soon as we could with our pediatric ophthalmologist, Dr. O.
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.

August 9, 2008 (Saturday)

Today while my wife was bathing Gracie, she noticed that Gracie's pupil was opaque (opaque is the word she uses and I often describe it as white instead of black). She thought that it was kind of odd but was not overly concerned. She mentioned it to me and we discussed it on and off over the course of a week. She noticed the opaqueness a few times and then her mother and I noticed it as well. We were away on vacation and as such had no access to her pediatrician however thought it prudent to find a doctor to have it checked out.

What is Retinoblastoma?

According to the Retinoblastoma.ca site:
"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."
http://retinoblastoma.ca/whatis.htm

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.

Why the Blog?

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.