Optic Glioma

My daughter was diagnosed with an optic glioma on 8/23. She had her first eye exam on 9/1 and her vision was 20/125. (She is two years old). Her next MRI on 11/23 showed now tumor growth but her eye exam (she is more verbal) was 20/400 in her left eye (where the glioma is). We met with an oncologist on 12/23 to discuss chemotherapy. They wanted to do vinchristine and carboplatin 1 x a week for 15 months. We asked for another eye exam since this was based on one exam. On 12/27 she had an eye check and with patching and glasses she was at 20/250. It was enough to wait on chemo for now but they told us that any vision loss between now and her February check will be permanent. Now I watch her constantly and though small it seems like we are losing ground again. I think we are going to ask for another vision test but I think I am scared they might recommend the chemo again. Has anyone been in this situation? I feel like this is a nightmare and I can't wake up. I always feel so anxious and depressed. I barely make it a day without crying. I just feel like this glioma has taken so much from our daughter and from our family.

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Our situation was the same. My daughter was 2 and half when they found the glioma and she couldn't really get an accurate eye test. They decided to start chemo because her glioma is on both nerves and extends right from the back of her eyes all the way to her hypothalamus. She is done chemo now, the glioma are stable according to MRI results but her vision testing has gotten better (probably because of her age). The ophthalmologist says that her optic lenses are pale, so the tumour is definitely causing damage. The only thing we have noticed is some difficulty with depth perception. She wears glasses for a lazy eye.
It is frustrating! At her age, she doesn't know enough to realize that her vision is getting worse. I think the little ones just adapt to their limitations and we never notice the problems. We had no clue about a glioma or NF until she had an MRI to check her spine for something else. By then, the damage had been done.
We share your anxiety and depression! I don't know what to say about starting chemo. My daughter did 70 weeks of Vinblastine and had very few side effects or complications. Maybe you should try to find out about the health of the optic nerve/lens from the ophthalmologist before deciding....(?)

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That is very helpful - the consents for the chemo were nauseating. I have never read about so many side effects. It made me ill.

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I have a 17 year old daughter recently diagnosed with NF2. When she was 5 years old, she failed an eye exam at pre-school. It was recommended that we take her to an opthalmologist to have a full exam and the finding was that she had a rare condition in her right eye called 'epiretinal membrane'. She had very poor vision in the right eye and it was explained to us that surgery to remove the membrane was necessary. We were then told that even after surgery, the membrane could return. Alexa has the surgery and recovered well but at best, her vision was 20/100 in the right eye. We eventually didn't need to keep having her seen for this issue and she exelled in school with A's and B's. Alexa has been a very happy and healthy girl growing up.

12 years later, Alexa is now getting ready to get her driver's license...we moved from Pittsburgh to Texas so I was now faced with finding a good eye doctor. I felt I should take Alexa to have an exam to find out if there are any further problems we needed to know about before she had to take the eye test to get her driver's license. This eye doctor was told about Alexa's eye issues when she was little and about the surgery she had. He examines her and finds swelling and elevation behind the optic nerves in both eyes. He decides to send us to an Opthalmologist to rule out pseudotumor. He doesn't feel Alexa has this problem, but because of the swelling he is seeing, he wants another opinion to rule it out. If pseudotumor is her problem, she will then need a lumbar puncture to drain some spinal fluid to release pressure on the brain....this made me very nervous.

We go see the Opthalmologist and give him Alexa's history. He was able to rule out pseudotumor but was also seeing swelling behind the eyes and not sure what was the cause. We are asked to make another appointment to be seen in 5 months. We are then sent to see his colleague for yet another opinion. At this next appointment, Alexa is examined and given eye drops and told to make an appointment to be seen again. I was annoyed that nobody seemed to have a diagnosis and they kept telling us to come back in so many months. In the meantime, Alexa was doing wonderful in school and she seemed to be fine. Eventually, Alexa began having chronic neck and shoulder pain. She would tell me she would wake up during the night in discomfort and it would eventually be fine as the day went on. I took her to her PCP to address the issue...Alexa was carrying a very heavy book bag at school and she was carrying it on one shoulder which I thought may be putting too much weight on one side and maybe causing a pinched nerve. We were given a prescription for an anti-inflammatory drug and it was suggested that Alexa start using a backpack to at least distribute the weight of her school books evenly. Alexa started taking the medication and said everything was better....until the pills were gone and then the pain returned. I took her back again to address the problem and the doctor didn't like what she was hearing so she ordered an MRI of Alexa's spine. Once we had the MRI done, it was determined immediately that Alexa has NF2. She has several tumors on her spinal cord. A VS on each side in her head, the one on the left is larger than the right side and has caused a significant amount of hearing loss in Alexa's left ear.

When I was told this information, I was in shock. I couldn't understand how she could have tumors and be so healthy and be doing so well in school. I immediately began researching and I was amazed at how complex and complicated NF2 is. I then decided to type in 'epiretinal membrane and Neurofibromatosis Type 2' and amazingly, there were many links leading me to information. I started reading studies that were done on groups of children who had NF2 that also were diagnosed with epiretinal membrane. I was astounded and angry. I couldn't believe that none of the eye doctors Alexa has been seeing didn't catch this! I am hearing and reading of people with NF2 who have eye problems, that the eye problem is 'lazy eye' and this is not the case. In NF2, eye problems are different than in a person that does not have NF2. This seems to be an issue and I am trying to find out how to make the doctors aware of this. If I am finding this information out and seeing that it is published in medical journals, something is very wrong.

It is very important to keep your own accurate medical records at home once you are diagnosed with NF2. I have already started doing this and I am even able to get Alexa's records from 12 years ago. All I had to do was contact the doctors she was seeing and they send me a release form to sign and the records are released to me. I was amazed that they even still had Alexa in their files after all this time.

Alexa is scheduled to have surgery on a C-spine ependymoma on January 25, this tumor is IN Alexa's spinal cord. I too have had my days when all I can do is cry but something inside me just believes that Alexa is going to come through this and be fine. I can't explain it but I just know it. NF2 is a very serious condition but already, Alexa is very positive and continuing to go to school every day. Normalcy is good. Alexa is aware of everything that is going on. We are seeing several specialists; 2 Neurosurgeons, an ENT, a Neuro-Oncologist who runs an NF2 Clinic and a Neuro-Opthalmologist. I believe we are in very good hands with these specialists and they are wonderful with Alexa at every appointment. Alexa doesn't seem like there is anything wrong with her to just look at her....but her MRI scans tell a different story. It is devastating when you first hear the diagnosis 'NF2', and it is overwhelming to take in. But I am learning that crying and falling apart all the time will get me nowhere. I continue to research and keep records. I have folders full of information that I take with me to every appointment and I carry Alexa's MRI CD's with me. I have gotten 2nd opinions and even 3rd opinions are in the works but everything points me back to where we are already getting Alexa care. It is important to keep organized and understand how NF2 works. Each case is different but I have learned so much from this website and people here. I also registered with the NF2Crew and have made good use of the message boards there. People on the NF2Crew are extremely knowledgable and supportive. I do want to stress that although these websites are a wonderful source of information, it is still mandatory and important to seek out NF2 knowledgeable specialists to get the care you need. I offer my life experience for information purposes only and do not in any way suggest the information I post here be used in place of qualified specialists.

~~ Jules ~~

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Hi,
I have a 2 yo girl with a suspect of optic glioma, it is just a thickness right now, we have to repeat MRI in 2 months and check the situation.
Doctors saud if the thikness is worse in 2 months time she will need to start chemotherapy.
My question is: how can you do a proper eye exam in a 2 years old kid?
How did your doctrs do?
I understand your anxiety and I also cry every single day. My little girl has a complicated case of NF1 and knowing there is so little to do to fight this horrible disease is killing me but I guess we must be strong and be there for our kids.
Take care

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Lilmissesmom,
Please read our daughter's story. If you have any questions or want to email me directly please feel free to do so from her site.
www.caringbridge.com/visit/arieljerome
I'm sure you will make the best decision for your child even if you don't think so at first.

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