Your stories: Evie’s little eye adventure

· by · in cataracts, patching, PHPV / PFVS. ·

Georgina kindly shares the story of her daughter’s diagnosis and treatment of PHPV with us.  Many, many thanks! – Ann Z

I wanted to share our story and experiences of discovering that our little girl had something wrong with her eye – a condition called PHPV (Persistent Hyperplastic Primary Vitreous, also known as Persistent Fetal Vascular Syndrome or PFVS). I found I searched the internet until I was blue in the face (not that I think it does any good), and it was nice to hear of people that had been through the same or similar.

You can see the cloudiness in Evie's left eye

You can see the cloudiness in Evie’s left eye

It all began at Evie’s 8 week check, where the doctor noticed she did not have a red reflex in her left eye. Ok I thought, she has a perfectly good right eye so we can deal with this, as it was mentioned it may be a cataract. However, within hours I had received a call from the doctor himself, and we were seeing a specialist first thing the next morning. I wasn’t feeling so relaxed about it anymore – things don’t happen that quickly unless it’s serious in my mind. Luckily my husband could get the time off, and we went together, to be told that they thought it was possibly a birth defect which would dramatically affect her vision in that eye, if she had any at all, but that there was a chance it could be retinoblastoma (something you never wish to hear, cancer). My husband looked like he was going to collapse, but I don’t think it sank in immediately with myself. I knew nothing about the condition, so was slightly oblivious to what it could mean, but my husband had already been Googling things, so was far more in the picture than I was (I preferred my way, ignorance is bliss sometimes). It soon became apparent that this was serious as, within a matter of hours of returning home, The Royal London Hospital had been in touch to see how we were doing, and to arrange for Evie to go in and have further investigations in four days time.

So, on the following Monday, we left our little boy and travelled to London. We had an appointment with the specialist, who discussed with us what the following day would entail. At this point it almost seemed assumed that Evie did have retinoblastoma. The staff were fantastic and answered everything wonderfully. We went out for dinner, and actually had a rather lovely evening, including dinner and a walk along the Thames (funny how you deal with things).

I woke Evie to feed as she had to go four hours without (the bit I worried about most as she loved her food), but all went incredibly smoothly as she woke hours later, and only a little while before her general anaesthetic.

I took her down to surgery and held her, desperately trying to hold it together and not cry, but I challenge any one not to. Watching your baby being put to sleep is so heartbreaking. I think it’s the feeling of being utterly helpless even though I knew she was in the best place, and everyone was just wonderful. Whilst waiting for her to come back we had to have eye drops, as it can be hereditary, which took our mind off things, as I couldn’t see a thing and had to be guided to the restaurant and giggled trying to see what button I should press in the lift, or what was on the menu.

Finally I could get her, and we awaited the results of the investigations.  When I was told that it was a cataract (later to discover Evie’s condition was called PHPV, which is similar) I broke down. It must have been the sheer relief that after all of this she just had a dodgy eye.

Evie's pupil after her cataract surgery. She has had pupilloplasty surgery to fix this but may have to have another as they need to see into her eye to check her lense etc. As she can see it's teenie in comparison to the other and does not dilate.

Evie’s pupil after her cataract surgery was teeny and did not dilate. She has since had pupilloplasty surgery to fix this but may still need to have another.

Evie’s pupil after her cataract surgery. She has had pupilloplasty surgery to fix this but may have to have another as they need to see into her eye to check her lense etc. As she can see it’s teenie in comparison to the other and does not dilate.

We were shortly referred to Great Ormond Street Hospital, where Evie has had two surgeries, and is likely to need a third, as her pupil has gone teeny as a result of the initial cataract operation. Luckily these operations were a success, and Evie now has a very swanky new lens in her left eye. The surgery was when she was 12 weeks old, and she was not fazed by it one bit.

Great Ormond Street Hospital is a very special place; it feels so upbeat, and it’s a very magical place to be. It can also make you realise that your little one really isn’t poorly at all in comparison to most of the children there. Although of course it’s still your baby and all you want in the world is for them to be ok.

I felt with Evie’s eye that she may not be able to see out of it, but at least it doesn’t cause her any pain or discomfort, and she doesn’t know any different anyway. Also, she could see the world and all its beauty, so if she was missing a fraction to her left it really wasn’t a big deal.

I wanted to share our story, really to let people know what happened after the surgery, as we were advised that it was unlikely she would regain her vision in that eye. After months of battling to get eye drops in, at times on an hourly basis (swaddling was the answer for us by the way), we finally  got her first pair of specs when Evie was 5 months, and we started patching on a daily basis with the aim to get to four hours a day. At first this seemed impossible, after 15 minutes she was beside herself, and seemed so scared and confused due to the lack of vision.

Evie's first specs at 5 months

Evie’s first specs at 5 months

But here we are four months later from receiving those first specs, and we are comfortably managing four hours a day of patching (we find it works best to pop it on when she is having a nap). Her prescription has gone from +9 to +8, and it seems her little eye is likely to keep improving. It’s amazing seeing the difference – I set her little challenges, and the other day she picked up a 5p piece with her eye patched and NO glasses. I find this incredible.

Evie in her patch and glasses at 8 months

Evie in her patch and glasses at 8 months

Well there you have it, a not so brief version of Evie’s little eye adventure, but I wanted to share to offer any advice regarding patching, drops, etc. and to show people that it is actually ok, and she looks adorable in her little specs. They are part of her.

24 responses to “Your stories: Evie’s little eye adventure”

  1. Georgina, Thank you so much for sharing yours and Evie’s stories. I am so happy that her doctor was so observant and that the medical teams were so diligent in helping her with her vision needs. I will share your story so that others will recognize the importance of having a baby’s eyes checked – early! Please keep us posted on Evie’s progress. She is so adorable!

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    • Thank you Katherine, I will. We go back on the 15th March so fingers and toes crossed that her eye may have improved again.

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  2. Even though I know you all and what was happening at the time, it sounds such a touching story and bought tears to my eyes. I’m sure your experiences will be very comforting to others who are also unfortunate enough to have to endure a similar time in there lives. She is
    the most cutest thing ever. Love you all xxxxx

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    • Hi Georgina

      Thank you for sharing your and Evie’s journey. If it would be okay with you I would love to connect. My son just got diagnosed with PFV and microsomia at 10 weeks and we are currently still waiting for a surgery date.

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  3. Thanks for sharing your story Georgina…she is a cutie! My daughter also had a cataract removed from her right eye when she was 5 months old. I cried so hard when they took her away from me at the hospital (I didn’t get to sit with her why they put her under). However we weren’t able to put the lens in Ruby’s eye & we deal with contact issues and patching (which very rarely happens). I’m always so happy to hear stories of patching success! Keep up the work! It’s a battle for sure!

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  4. Hi, I read ruby’s story I have no idea how you get a contact into a little ones eye. There has been talk of Evie having a contact but she keeps specs on so well I am not keen! Will they try again on the lense front? We were so lucky it was successful. Patching is amazing so worth the hard work. I hope things get easier for you x

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  5. Hi!! My daughter was also born with a cataract. We had hers removed at 5 weeks old and she also had a retina detachment which we had surgery to reattach. So far she’s ok we go back tomorrow for a check. She has a contact in and will also be getting glasses. So we started patching 2 weeks ago and find it so impossible. I would love to hear more about evie and things you have done. It’s been an emotional roller coaster for my husband and I.
    Love it if we could chat

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  6. Just an update
    To quote the doctor “this is the best result we have seen in a baby of her age with this condition. Most surgeons wouldn’t have operated and this is exactly why we think it’s worth a shot” prescription remained the same but apparently this is linked with eye growth rather than vision, didn’t know that, but eye tests where the best yet and were redone to check as she didn’t think it was right it was so good. One happy mummy x x

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  8. Thank you for sharing your story! I was born with PHPV in my left eye, and am now 26 years old. I too had a cataract and it was removed at 6 weeks old. My parents patched my eye daily (althought they say it was torture for them) and they tried using the contact lens for about a year and a half. As any infant would, I continuously woudl rub them out and my mom would find them in my bed, and that did not last. Throughout my life I have had about 6 surgeries and my last one was in 2004, to correct the laziness of the muscles. I have been wearing a contact lens in my good eye since 2004 and glasses in the evening. Currently, my doctors want to try to put a contact lens in my left eye in hopes to exercise the muscles. I am just curious what they tell you is possible today for your daughter? My doctors told me that in order for hopes of restoring some vision in that eye, you have to establish the relationship with the eye to the brain before the age of 12. That is when the brain can still make the connection. I am recently doing my own reserach in hopes to find something to help and simply to have a better understanding. This condition, as I know it, has not effected my quailty of life, simply because it is all I have ever known. I have a regular drivers license and do all things everyone else does, except for catching balls and playing some sports. The depth perception is not as strong as some. I wish you all the best with your beautiful daughter.

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  9. Hi, Thank you for your message. My aim of sharing our story was to help people if I could, in a similar situation, so its nice you have got in touch. Obviously the age difference is a big thing, we were told that the brain may have already stopped using that eye and therefore we may not be able to restore the vision. Some surgeons won’t operate if babies are over 5 weeks apparently as there is research to show this is too late. However our surgeon considered it to be worth a shot and it has paid of for us, she is doing brilliantly. She succesfully had a new lense at three months and is just getting better and better. I have been told that up to the age of around seven you can patch etc but after that the vision is what it is. So apparently we will keep patching and the glasses until her vision stops improving. They referred to it has physio for the eye, which I guess the lense for you may be what your doctor is thinking. Unfortunately I couldn’t comment on whether or not it would help you as Evie is still only tiny. I wish you luck and let us know how you get on.

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  10. Hi Georgina our 5 day old baby boy Max has just been diagnosed with PHPV, the doctor who has just diagnosed this condition has advised us he does not believe surgery would be the best plan of action as firstly there is a 30-50% chance he could develop Gloucoma, secondly Max’s vision may not be improved that much after surgery. Were you advised of these risks before you decided to go ahead with the surgery for your daughter Evie. Any help and comments would be greatly appreicated as we are struggling to make a decision on what to do next?
    Many Thanks in advance

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  11. Hi Robert, do you have an email address? I would be happy to share with you more about our experience and progress so far. Evie is a year now and yes we were advised of all the same risks and told it was unlikely we would restore the vision. If you are happy to share your email address let me know.

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  12. Hi Robert, I have sent you an email I hope it gets to you I have had a few issues recently so let me know if you don’t get it. Thanks Ann for putting us in touch.

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  13. Hi

    My son had/has the exact condition as your little girl, firstly he had a cataract removed from his left eye, which then the doctors discovered it was Persistent Fetal Vascular Syndrome. Mason has undergone 4 operations to date (he is only 3). He is currently wearing a contact lens and glasses. Patching hasnt been very successful but I have been using Atropine drops for the last 6 months. Mason had an appointment with the consultant and his left eye has impeoved slightly, which is great news.

    I totally agree with what you say, even though it is an illness and its heartbreaking as a parent to see your child go into surgery etc, they know no different, I am blessed to have such a perfect son (even though he has very little sight in his left eye) :o) xx

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    • Hi Michelle,
      It’s nice to hear from people with little ones in a similar situation. I am glad Mason has shown some improvement in his eye. I am eagerly awaiting our next visit to see how things are going. Evie does amazingly when patched with her glasses on so we are a world away from when this journey first started.I am a member of this page on facebook and its great People with Persistent Hyperplastic Primary Vitreous (PHPV) Unite!

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  14. Georgina This story was inspiring to know that as scary as a PHPV diagnosis can be there is a light at the end of the tunnel! My daughter is just 3 months and has had surgery to remove her lens already. She sees a specialist in two days to discuss contact lenses and patching. We also had to have her pupil fixed and her eye looks aesthetically amazing. I am hoping that she can have some vision in that eye. I would love to connect with you. My e mail is christieharcus@hotmail.com.
    Thank you so much for sharing!!
    Christie

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  15. Hi Georgina

    I have just read your story about your little one and I am very happy to see her improvement. My daughter Akki who is 10 month old have been diagnosed with PHPV cataract in her right eye yesterday. Her both eyes looked the same way from her birth. there is a very slight difference of the size of her right eye which is a bit smaller than the normal left eye, which became visible recently. Our doctor said that there is no surgery available at this age and I was totally heartbroken as we did not have this condition detected earlier. I am trying to patch her good left eye but my daughter gets very fussy and wants it removed. I am so heartbroken and would like to know how to make things easier for my daughter.

    I also would like to learn about her improvement with her eye. Please let me know. My email address is madhu.viswanadharao@gmail.com, please email me.

    Regards

    Maddy

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    • Hi Maddy, I have sent you an email. Check in your junk file if you can’t see it as I have had some issues with them this week x x

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