My daughter, Madison, was born with a condition called PHPV. My wife and I were writing a blog about her condition, but, like most blogs (including this one), it didn’t get updated as much as it should. As such, we have taken down the blog, but I wanted to document her story so far so that other parents with children with PHPV can read about our experience.
Madison was born in late December 2009. My wife noticed within the first few weeks that her left eye seemed smaller than her right eye. Her left pupil did not seem to dilate with light and seemed constricted for most of the time.
We brought this up with her physician at the time, who did not think there was anything abnormal with the eye. My wife was not convinced, and, so, I asked one of my colleagues who was an ophthalmology resident at the time to take a look at some pictures of her. She suggested that I bring her by the hospital that weekend so she could take a better look.
When she was checked out by my colleague, she made the diagnosis of PHPV. She referred us to a pediatric ophthalmologist, who we saw the next day after she was diagnosed. He gave us information on the condition, and essentially we had two choices: do nothing and let Madison develop normally with the use of one eye, or, the second option, to do surgery to remove the cataract caused by PHPV and then go through 7 years of daily patching with Madison, as well as the possibilities of complications, like glaucoma and retinal detachment. There was also little hope of having useful vision in her affected eye, even if we followed through with the surgery and daily patching.
My wife and I ultimately decided to go with the more aggressive approach. She underwent surgery to remove the cataract. She tolerated the operation well and only had to wear an eye shield overnight.
Over the next month or so my wife noticed that her affected eye seemed to be getting bigger. When we attended our followup visit from the surgery, her ophthalmologist noted that her eye pressure was elevated and made the diagnosis of glaucoma. We were aware that this was a possibility after the first surgery, and that outcomes were typically not good if glaucoma developed. She was started on oral medications to try and lower the eye pressure. We were referred to a pediatric glaucoma specialist, who arranged for an EUA. The pressure continued to be elevated despite the medication, so she was booked to have a tube inserted in her eye to relieve the pressure. She underwent a second operation to do this, and it seemed to be successful.
Since the tube (Ahmed valve) was inserted, Madison has undergone 3 EUAs and everything seems to be developing normally in her affected eye. Her optic nerve remains healthy.
We put in a hard contact lens into her eye everyday. She wears a patch for 6 awake hours. She generally tolerates the patching well, except when it is bright out, as her pupil does not constrict in her affected eye. Her vision seems to be pretty good in her affected eye. She can do everything with the patch on that she can do with the patch off. So in that respect, I think the outcome is better than what we hoped for.
Patching at the beginning was very difficult. We started with doing 1 hour a day, which was challenging. We usually had to split it up into 15 minutes at a time. It progressively became easier as time went on. The biggest issue now is that she can take the patch off herself. We generally go through 2-3 patches a day.
We found that it was difficult to find good patches locally in Edmonton. We end up ordering patches from the USA and from a supplier in Toronto. Our first favourite is the Ortopad Elite. They are available in Canada through InnovaMed. They are a little pricey, but they seem to be the most comfortable for Madison, and they seem to stick on her skin the best. They do not have online ordering — you have to call them and they will ship them to you.
Our other favourite patch is the MYI Occlusion patches. They ship directly from the USA to Canada and they have online ordering available. They are quite a bit cheaper that the Ortopads, seem to stick well, don’t fit as well as the Ortopads. They are available here.
I will continue to update this blog as Madison gets older to keep everyone informed of her progress.
UPDATE- May 6, 2012 – Madison now has a pair of bifocal glasses to help her focus on far away distances. She’s done very well with the glasses. She wears her patch, contact lens, and glasses for most of the day, and then just her glasses and contact lens.