PHPV – A Parent’s Story

PHPV – A Parent’s Story

Emilie-editedMy daughter Emilie is 9yrs old now and lost her eye due to a congenital cataract called PHPV Cataract, the website explains it best http://www.pgcfa.org/kb/entry/159/ when she was a little over a year old.

She was born 11 weeks premature and when they performed a routine eye exam to check for retina detachement from oxygen use the Ophthalmologist discovered the cataract in her right eye. After getting a second opinion from the hospitals head Ophthalmologist it was confirmed. We were told that in the past vision loss was garanteed but they had an operation that could be performed that would give her “some” vision though never as good as a healthy eye. We decided to go ahead with the surgery but unfortunately it was unsuccessful and she had to go thru a few more surgeries due to complications including retina detachment.

Finally the eye was no longer salvagable. We first tried placing a prosthetic on top of her affected eye when she was a few weeks shy of 1yr old. But her eye was so sensitive that it was unbearable for her to wear so nearly two months later in November almost a year to the day of her first surgery they removed her eye. We were terrified of how she would recover from having it removed but she did SUPERB. In January she received her new prosthetic eye. I was terrified of what it would look like especially since her left I had developed a condition called Strabismus and was not set right in the middle, instead if she wanted to look at something further away she would turn her head to the left and the left eye would turn inward.

This made making the eyes look even that much more difficult, but my husband suggested to the ocularist to set the prosthetic slightly off, closer to the bridge of her nose and it worked. Most people thought it was the eye with Strabismus that was artificial. She did have surgery to fix the Strabismus when she was 5yrs old and though we were extremely nervous about it since this was the only eye she saw out of the surgery went well and we’ve not had to do anymore operations since then.

Since Emilie lost her eye at such a young age adapting to the loss was much easier for her. Dept perception has never been difficult for her and although she was uncomfortable when she was younger to “team” sports such as soccer because she does have a “blind spot” she seems to be adapting well now.

She has struggled with infections but since we have it cleaned every 3 months now instead of 6 they rarely happen now. When she gets a cold she does have to clean it more often and of course dry air especially in the winter make it a little uncomfortable so she carries eye drops with her to help with that.

We’re so lucky that so far she’s never been teased about her eye. We live in a small community so everyone who knows her has always known she has a prosthetic. When she meets new children they’ll almost instantly ask her what happened to her eye but only out of curiosity.

Speaking as a parent of a child who lost an eye I wish I would have been able to meet more parents in the same situation and for my daugther to meet children with a prosthetic eye as well. I’ve only spoken to one parent from another province in the past 9 yrs. We’ve always been open and honnest with our daughter about her eye and have always informed friend’s parents and new teachers about her condition.

26 Comments

  1. Avatar for jenn jenn on June 27, 2012 at 10:43 pm

    Hi there, my 5 month old daughter was born with phpv in her right eye, it is also smaller. She wears a contact along with glasses and we patch 4-5 hours daily to god-willing restore her vision. I too desperately want to meet local people with my daughters condition. Are you by chance from Toronto?

    • Avatar for Paul Geelen Paul Geelen on July 3, 2012 at 2:32 pm

      Hi Jenn,
      I believe Daphne Archibald is the ocularist who works with most of the children with Microphthalmia in Toronto. While I understand from your email that your daughter does not require this service it would be a good idea to call her as she would know many children with PHPV and may introduce you to their parents. You can find her details here. I hope this helps,
      Kind regards
      Paul Geelen, Ocularist

    • Avatar for Karla Karla on July 19, 2012 at 9:37 am

      Hi jenn! Our little one has PHPV and is now 21 months old! She wears a contact and patches 5 hours a day. We are also in Toronto. Feel free to email me at ounceofluck@gmail.com and we can chat! Good Luck! Check out my blog too. I don’t keep it up as much as I should but there is a whole group of Eyemoms blogging who are great for support!

  2. Avatar for Michelle Michelle on December 7, 2012 at 1:29 am

    My son, grant, is 2 and he had cataract surgery at 13 weeks. He wears a contact and we try to patch 4 hours a day. He also has microphthalmia in that eye. He started not seeing anything and now when patched he can get around. He an see far put does well up close. He loves using the iPad when patched and does it all by himself.

  3. Avatar for kate kate on January 25, 2013 at 5:13 pm

    My son was diagnosed at 4 months old with PHPV.He has micropthalmia also.
    We were told by consultant peadiatric opthalmologist that it was beyond salvageable and did not reccommend surgeries or patching this is due to potential little success and the trauma would be a waste of time.

    I worry about the future and am learning more as I come across forums etc.
    The only thing reccommended is a cosmetic schleral shell contact lens.
    Has anyone had any success with a contact lens?

    We live in the UK. I had never heard of this condition until now and yet to meet someone with it.I know of no one else and would like to see photos of adults with it and those with copntact lens

  4. Avatar for Ashley Ashley on April 18, 2013 at 4:00 am

    Hi everyone,

    I was born with PHPV in the left eye, I am now 26 years old and have virtually no vision in that eye. My mother patched my eye for 5-6 hours daily and used the contact lenses, but as any infant would do, I liked to rub them out, so that was challenging. As an adult with PHPV, I find that some doctors say theres not much that can be done at this point, that you have to develop the brain and eye connections before the age of 12. I want to know what other doctors suggest for restoring some vision in the one eye? My next appointment, they want to try a contact lens in the left eye (PHPV eye) and see if that helps any. When I was born with this condition not much research was established then. I advise all you with young ones, to seek as many opinions as possible. The one thing that I continue to want is the laziness of the eye corrected. My last surgery was about 9 years ago, and I woould like another one for personal reasons. They say once you cut the muscle so much, not much is left to continue to operate. I wish they would have known more when I was born with this condition, and perhaps they did all they could do. Always be your own advocate! best of luck!

  5. Avatar for Helen Helen on May 22, 2013 at 9:49 pm

    Hi
    Was told when my baby son was 1 day old that he had a cataract ….was giving and emernacy appointment to my local eye infirmary 4 day later …I was told my son had PHPV and the chance of him having sight in he’s left wasn’t that good …he’s now booked in for an operation next week…he’ll only be 4 weeks old ….I feel hurt and bitter that this is happening …I had never heard of PHPV before this ………I’ve spent hours on the Internet goggle information about this condition …but there’s not much on here really so feel like one of my question are being answered ……..would love to get incontat with other parents going through the same thing as me …..or people who have PHPV ……I just need to e assured that y son will live a full life get married one day have kids………everything every parent wants for there kids ……I live in england

    • Avatar for Beth Beth on December 3, 2013 at 8:26 pm

      Hi Helen

      My son Seb has been diagnosed with PHPV in his right eye but we have been advised that surgery is not feasible. He is now 5 months old and we have started patching today. I have been concerned about his vision for a while but had hoped it was due to him being a month prem. I have the same concerns as you regarding his future etc, particularly as he does not have amazing vision in his left eye. I am hoping that by knowing about it at a young age we can support him as much as possible and help focus and improve any vision he.may have. I feel a bit lost as we have no definite answers but taking each day as it comes. We live in South Wales.

    • Avatar for Cathy Cathy on March 12, 2014 at 11:37 pm

      Hi Helen,

      I remember well the feelings you are experiencing now. Please know that your baby will grow up and live a full healthy life! My daughter was born with PHPV and a cataract in her left eye. She had her first surgery at 5 days old, then another at 5 weeks old. We patched her for 6 hours a day for almost 5 years to try and get vision for her in her left eye. Unfortunately, her vision was still very poor, but we wanted her to know that we did everything in our power to try and encourage sight in her PHPV eye. My husband and I showered her with love and didn’t limit her experiences just because she was monocular! When she was a junior in high school, she underwent another surgery to ready her eye for wearing a scleral shell. She wore a shell for a year and a half and then had another surgery to make it more comfortable. Although it has been a challenging road for her, she has excelled in life. She just turned 19 two days ago and is going to university in Venice, Italy! She is full of joy, curiosity and has many dear friends! I live in the US in Colorado. You can contact me if you have any questions. thayes1062@comcast.net

  6. Avatar for Jim Jim on June 29, 2013 at 5:32 am

    my son is 27, and was diagnosed with PHPV when he was a month old – my wife new something was wrong, because when she was breast feeding him on the left side, his right (sighted) eye was pressed against her breast, and he would get very fussy. It was a long time ago now, but some how our pediatrician figured something was wrong, and we ended up seeing several eye doctors in Boston as well as at Baskin Palmer institute in Miami. A few folks wanted to enucleate his eye early on because they could not be sure that there wasn’t a retinoblastoma, which there wasn’t (it is quite small and doesn’t move much), but we hesitated, and when he was in his early teens we got him a scleral shell at Jahrling here in boston (a truly amazing place: http://www.jahrling.com) It took him a long time to really get comfortable with it, but he is happy with the cosmetic result that it gives him.

  7. Avatar for Angelina Cisneros Angelina Cisneros on January 27, 2014 at 1:37 pm

    Hello my daughter too has phpv she is now 8 yrs old I noticed something was wrong the second she was born. Its a mothers intuition, and the fact that her right eye is significantly smaller than her left ,well it took a couple of months for us to get an appt with the specialist. when she was 5 months she was diagnosed with phpv, doctor said no patching nor surgeries were needed their was no sight to try and salvage. My worst nightmare! But because she was born like this she has adapted very well their is nothing she can’t do. Unfortunately she was also diagnosed with another very rare disease that has turned our world upside down.

    I have yet to meet a phpv family 2 very rare diseases for my child what are the odds of that? Does anyone with phpv also have ither health issues?

  8. Avatar for Kourtney Lippert Kourtney Lippert on January 28, 2014 at 11:49 pm

    Hi! I am 24 years old and was born with PHPV in my right eye. I ended up with a cataract and glaucoma. After multiple surgeries, they attempted to patch me and put a contact in. My parents said I hated that contact and when they patched me, they noticed all I did was cry and run into things, so they stopped when I was two. It was determined that I had lost all sight in that eye. I lead a totally normal life. I played fastpitch softball and was a pitcher. My team even went to nationals in Seattle (I am from South Dakota)for nationals. I drive, read, and made the Dean’s List in college. The ONLY thing I cannot do is watch 3D movies. When you are born with no or little sight you know no different. You adapt and develop your own depth perception. If you would like to see pictures of an adult with PHPV, please email me at kourtneylippert@gmail.com.

    • Avatar for Pallavi Pallavi on February 2, 2014 at 4:19 pm

      Hi!!!!!
      Kourtney Lippert,
      I am Pallavi.
      My Son was also born with (PHPV) in his right eye and left eye is normal and he is 5 years now. We got to know when he was 2 months. We have not been advice for any patching or surgeries since there were very less change for him to get any vision in his right eye but looking at him no one will say that he’s got vision with only one eye and only one problem we have is his right eye is smaller than his left eye. He is very fond of garget he only love to play with mobile his computer, watch TV and tab. Hold day he is got something with him.
      I am worried for his future. As you said that u can drive, read and do all normal think which a normal person can do.
      I want to know is do you face any problem in your day to day life or using computer or play games in you mobile and tab or at your work.
      I want to know more about people who are diagnosed with PHPV.I would really like to see pictures of an adult with PHPV. I live in India (Pune).

      • Avatar for foram foram on March 2, 2014 at 5:52 am

        hi pallavi,
        i m foram my two yrs old daughter is also having phpv in right eye also she has micro cornea and posterior catract.she is advised to do nothing wid it.she is doing good so far wid other eye.i want know more about n also wouid like to see d picture of an adult with phpv. i m from chennai in india. also like to c u on fb an too meet more parents

        • Avatar for Pallavi Pallavi on March 16, 2014 at 2:39 pm

          Hi!!!!!
          You can search me on fb Pallavi Khampria
          Thanks,
          Pallavi.

  9. Avatar for Victoria Victoria on March 7, 2014 at 9:58 pm

    Hi. I was born with PHPV in my left eye. I find that my case is much different from many as i have no sight in my left eye at all and its not even half the size of my right eye. My eyelid automatically shuts because there is no eye to keep it open. I’ve had many cases of a calcium deposit growing on my left eye, and have had many painful surgeries in my 16 years.

    Personally, the patching didn’t work at all for me when i tried it, and i now have a prosthetic in, so i don’t even think about it.
    A prosthetic is probably the best route to go if the child is like me, when I’m at the age where i am self conscious. Your child may lie and say they don’t get made fun of at school, but they are different and trust me when i say this, i have been judged way too many times.
    You have to be there with your child and let them know that it’s okay to be different and they have to be the one’s to make the decision on what they want to do with their eye.
    I personally am thinking about getting it removed and getting a thicker prosthetic in as it does nothing but bug me.

  10. Avatar for Eye Surgery Eye Surgery on April 1, 2014 at 4:18 am

    Great post! Been reading a lot about different aspects of PHPV. Thanks for sharing this info!

  11. Avatar for Eva Eva on April 3, 2014 at 12:16 am

    I am reading all this and as familiar as I am with my daughter Isabella’s condition now, I had not read so many stories of children who were born with PHPV on the right eye, it usually is the left eye. I have actually given up on the shell as it has caused traumatic events for my little one, who was diagnosed at 2 weeks old, has had 4 surgeries, 2 cardiac arrests, patching, has lost her sight (right eye), but did not miss it, since she didn’t have good sight in it from birth anyway. She is only 20 months old- a little over a year and a half- and she is a very active little girl. She will not keep her glasses on, will not use the shell either.. I am a bit traumatized with all these changes and events. I find some comfort in this site. Any words of advise or support?

    Eva

  12. Avatar for Gina Gina on April 5, 2014 at 1:31 am

    Hello All,
    My 16 year old son was born with PHPV, left eye smaller than right eye. Surgery as a baby, tried patching (it was heart wrenching) and has worn glasses (mostly for cosmetic purposes) his whole life. He says he can see shadows out of his left eye, but that is it. Yes it is not something that we would wish on anyone, but believe me I am thankful everyday that is/was not something worse. He has many friends and has played sports his whole life: basketball, football and baseball (pitcher!) – He is a smart kid and has a completely normal(moody!) teenage life. He plays snare drum in the marching band, has a job and drives. Yes his friends have inquired if he has a ‘lazy eye’ but people that have known him since he was a little boy say they don’t even notice his eye. Having little to no vision in that eye is all he has ever known, so he just deals with it. I hope this helps any new parents out there…. 🙂

    • Avatar for Pallavi Pallavi on April 12, 2014 at 7:05 pm

      Thanks Gina I got little confidence that my son will also have a normal life ahead.

  13. Avatar for Lisa Lisa on June 4, 2014 at 10:27 am

    Hi, my son was also born with PHPV in his right eye,. He had a total of 13 surgeries for various reasons, lensectomy, glaucoma, shunts, retinal detachment and eventually had it removed. He has never missed a beat, however he will be 16 in a few days and I am scared to death to let him drive. I think this, so far, will be our biggest feat to overcome. He is a lot more confident than I! His only compliant is not being able to see in 3D. I believe that God has big plans for him in life, he has learned that life isn’t always fair and knows how to handle adversity. He lives life to the fullest! Never let anyone tell you your child can’t do things with monocular vision, this is just not true! While I so well know the struggles, I am also able to reap the rewards of being blessed with this amazing child!

  14. Avatar for Ken Ken on June 21, 2014 at 3:04 am

    Hi, all

    I am happy to know that there is such a place sharing the experience of PHPV. My son who is 55 days is just diagnosed PHPV with his left eye two days ago. His case is very serious and belongs to a combination of anterior and posterior PHPV. We are considering if we need to give him a surgery quickly. It is a really brutal reality for such a little guy. Is there any advice? I really want to know if surgery will do some help. I am from China.

    Chen

  15. Avatar for Gail Gail on September 2, 2014 at 10:56 pm

    Hello
    My son is 27 and was born with phpv/cataract. When he was born, in the hospital, he didn’t open his left eye. After a few days at home he opened it. My husband noticed that the pupil was almost too small to see and did not constrict or dilate. We went to the Eye Institute at Children’s Hospital in Milwaukee, WI. He had surgery to enlarge the pupil at 22 days old. We started with a contact by age 3 months. Patched up to 5 hours a day. (God bless the day care center workers that patched a few hours a day for us!) We had to make up games, like “Dr. Patch”
    He has had 2 surgeries to straighten his eye. That worked quite well, although he is bothered that the eye is smaller.
    In high school he decided to quit wearing the contact as he said it didn’t make much difference. Currently his vision in that eye is 20/400.
    So if he should lose vision in his good eye at least he would be able to maneuver around somewhat. That is the benefit of working hard at the patching and the contact.
    God’s blessing to you all. Gail

  16. Avatar for Sophie Sophie on September 15, 2014 at 7:42 pm

    Hi!

    I was born with PHPV in my right eye and had many of the typical operations on my eye from 6 weeks old. My sight couldn’t be saved and I had the lens partially removed in that eye. A little bit was left to create some type of pupil but it was left just looking like a cats eye. I do have a tiny bit of vision but it’s mostly just peripheral and I don’t actually feel like I use it.

    I developed glaucoma at the age of 13 due to trauma to my eye and have now worked my way up to 4 eye drops daily to control it. I am now 20 years old and don’t really feel like PHPV or glaucoma has dramatically effected my life. I was never teased for my eye and have grown up understanding that people will be curious as it doesn’t look normal, so it doesn’t bother me if people ask about it.

    I also have my drivers licence and didn’t really struggle too much getting it. I have only one condition when driving and that’s to always have two wing mirrors on the car (odd, i know). I was also told that depth perception would be any issue, and to be honest i’ve only recently started noticing this. I truly believe that if you don’t make your child feel like they are meant to be struggling when it comes to something like sports, then they will just feel normal and like they want to try harder.

    The biggest problem I’m facing currently is that I want to go travelling. I want to spend a number of years around the world and I’m really not sure how i’m supposed to work in my eye drops and prescriptions throughout those years. My parents are happy to continue sending me eyedrops (which they do currently as i have moved countries) but I feel like that is too risky given that so much is at stake without them! Hopefully I can arrange something, or maybe have another operation to drain the fluid in my eye without the use of eyedrops.

    All in all though, I really don’t think PHPV or glaucoma has effected my life that much. As a 20 year old female i do sometimes wish that my eye could just look normal and i do get self conscious about it occasionally. I just remind myself that I’m unique and that there are people out there going through a whole lot worse. And with all of that in mind, i’ve just graduated as a graphic designer.

    If you want to ask me any questions or talk I would love too! I have never spoken to anyone else with PHPV and think it would be super beneficial! My email is sophie.r.lee@hotmail.com if anyone wants to get in contact 🙂

  17. Avatar for Jen Jen on October 7, 2014 at 7:57 am

    Hi,
    Its so reassuring to know that there is other people to talk to about this. My daughter was born with PHPV cataract in her left eye. She has had 2 check-up’s so far and they have stated that she would have little or no sight in her left eye. Last week she attended her second appointment, where they told us that she was actually born with 2 small eyes, the left 1 is smaller than the right. Now the doctor reckons that she has little or no sight in both eyes, but she does follow lights and objects. She is back next week to undergo tests to see why she has 2 small eyes. Its heartbreaking to think what she has to go through. It’s been so helpful reading all ye’r comments as there isn’t much information online as to what can be done.

  18. Avatar for Laura Laura on November 7, 2014 at 10:04 pm

    Hello everyone, I am currently 22 and was born with a PHPV cataract in my left eye. At 6 weeks old I had eye surgery and after that wore contacts and patched my eye for 7 years. This was a lot of work but as a result when I wear my contact I have the same normal vision as my right eye. I have gained both depth perception, and peripheral vision the only difference is the pupil is a bit smaller. I just want to let you know there is hope if you work for it! I bless you all and hope for the best.

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