A blog about having a child with PHPV or PFVS

A blog about having a child with PHPV or PFVS

Persistent Hyperplastic Primary Vitreous

also known as

Persistent Fetal Vasculature Syndrome

and micropthalmia (small eye)

Our experiences with 3 surgeries, 2 EUA's, patching, contact lenses, scleral shells, prosthetic eyes, emotions, places to get support, links to other sites and general info on vision impairment. I really hope my blog helps and educates and I would love to hear from you with any questions you have, or even if you just need to talk to someone who has "been there, done that".


Perth, Western Australia

Tuesday, June 28, 2011

EUA for tomorrow has been cancelled

They've double booked the operating theatre at the small hospital we were going to, so we've been moved back to Wednesday. This is actually probably a good thing because Joel was awake last night from 11pm - 3am, crying and grizzling so I think he may be coming down with something. It's just inconvenient because Annalise doesn't have school on Wednesdays so now I need to find a baby sitter.

Monday, June 27, 2011

How to get an appointment

I recently had an email from a lady with a child who is suspected of having PHPV. I say "suspected" because she has been finding it really hard to get answers. I absolutely know how frustrating this is, being told you have to wait 6 months for the next available appointment, needing referrals etc etc. Think about it though, if YOU started going blind in your left eye, would YOU wait 6 months to do something about it? The answer is probably "no", so why are we expected to wait that long for something to be done for our kids? When they are babies, their brains are being wired from learning, what goes in auditorily and visually. If one eye isn't seeing clearly, the brain will learn to ignore that eye and then the damage is done. If your baby is 8 weeks old and you get told you can't get an appointment until they are 6 months old, that four months of processing your baby's brain is missing out on. If they have surgery at 6 months, it's another month until you can start properly patching and getting some light and images into that eye. It's too long to wait.

When we noticed something was wrong with Joel's eye, we went straight to the Emergency department of our state's biggest children's hospital. I really think this was the best move we made (we had no idea at the time that it would be a blessing, it was a Sunday and everywhere else was closed!). Although they couldn't help us on the day, we were immediately referred to the Senior Opthamologist Dr Lam. We were able to get an appointment with him for the Thursday because I called and called, begging to be seen. My calling had nothing to do with being pushy or demanding, it had everything to do with being desperatley worried for my newborn baby. You can read the blog posts from the very start and see just how desperate I was! I was hysterical and didn't cope with "not knowing" very well at all. After we'd seen Dr Lam and he had diagnosed PHPV / PFVS, I wanted to get a second opinion. I googled Pediatric Opthamologists, found the only other two in WA and went to my GP and got referrals for them. I called their offices the next day and was told there were no appointments for 4-6 months. This was obviously too much for my hysterical mother's brain to take so I had an absolute melt down over the phone, bawling and babbling and begging to be seen. I got in within 4 days.

I am not saying to scream like a banshee until they agree to meet you, but you are the biggest advocate for your kids so don't be scared to be a bit more pushy than you naturally would be. There will be plenty of other times in your child's life they need you to stand up for them, PHPV kids just sometimes need that to happen a bit earlier!

EUA this Thursday

It's Tuesday today and Joel's EUA (Examination under anesthesia) is booked for this Thursday. This will be his fourth general anesthetic in the 17 months he's been alive and I can tell you it never seems to get easier. I am glad this time that there's no invasive surgery, just a look at the eye and an impression of his little face and area around the eye taken so they can make the prosthetic. His EUA is at 1pm and he has to fast from 7am. He LOVES his food, so I'm not sure how this will go. It was easier the last few times because he was still a baby and not having solid food, but now he has discovered the delights of things like meat and carbs, he eats us out of house and home. I think I'll give him as much breakfast as I can at around 6:30 then drop Annalise off at school, come home for a couple of hours then drive around and hope he falls asleep in the car. He can have apple juice until 11am, apparently that helps to make them feel full.

As I am having a few complications with this pregnancy, I have been on bed rest since 22 weeks (I am now 29 weeks). I only really seem to leave the house for things like doctors appointments for both kids (it's been ear infection central for the past few weeks), Obstetric appointments for me and Opthamologist appointments for Joel. I am glad we're going to a smaller private hospital this time, instead of PMH as it will mean there's less room for Joel to run around in while we're waiting which means less up and down and chasing him around for me. Bed rest is really hard with the two kids but I try to sit down as much as I can. Rich has been helping to put the kids to bed, cleaning, cooking, as well as working 6-7 days a week. I will be glad once this pregnancy is over, Joel's prosthetic is sorted and life can continue as normal.

In one way, I am actually glad this is all happening to us. Our marriage has had it's trials by fire and I know that we are strong enough to stick together through anything now. It takes a lot to rock our boat now, we've been through the worst of it over the past 5 years. I do hear stories about couples who have sick or special needs kids and the arguments they have, the financial and emotional stress and toll it takes, and how appointments, check ups, surgeries, everything that goes along with it just filters in and take over your life for a while. We were like that, we had arguements about the best way to put Joel's lense in (when he was wearing one), who would look after Annalise while we were at appointments with Joel, the never ending question of "how much will THIS cost us?" If you're new to the journey, please be assured that it doesn't last forever! Your "new normal" will become just that, normal, and life will continue on a different and more blessed path because you have this amazing little person in your life. It certainly has given me more to be grateful for, made me appreciate the health of our children and really given me an insight into life with a child who isn't like the other kids. It's opened my eyes to societies reaction to someone "different", the stares and questions I never thought I would have to answer. Joel's education will be different, his teachers will need to make accomodation for him, there will be other facets of his life that we wouldn't have had the chance to experience if he wasn't blind in that eye and for that I am grateful. As parents, and as human beings, we can learn so much from these kids. We are humbled by them and in awe of them, of their weakness and their many strengths. Hug your kids tight and tell them how special they are.

Friday, June 24, 2011

Proud of my kids

When Annalise was little, I was so proud of her for a lot of things - most of those were things that every parent would be proud of. I was proud because she was so clever, walked, talked a lot and had heaps of words, was sociable, cute with a huge personality.

I am proud of Joel for those same things too, but there are other things that make me more proud. Like when I can see he is struggling slightly with trying to work out how far away something is, a pencil he's trying to grab. I am so proud when he picks it up first try and starts scribbling.

Annalise was asked by another little girl the other day "what's wrong with your brother's eye?". Straight away she said "he has one eye open and one eye closed but today both are open". She was proud of him for having a "good eye day!" Both eyes were open and to her, that's pretty great. The other little girl said "yeah but it's all yucky". Annalise looked at Joel and said "That's his sore eye". The other girl seemed happy with that and although she kept looking at his eye, they continued playing and nothing more was said. This is the first time anyone has ever asked her about his eye and I couldn't have been more proud or happier with the way she responded. To her, it's nothing. To us, he's just Joel. To everyone who knows him, he's perfect.

Financial costs

Even though we reached the Medicare safety net within 3 weeks of the new year, our out of pocket expenses for specialist appointments, prescriptions, private health insurance and the excess for hospital admissions are, most of the time, more than we can afford. Yes, it is our choice to have private insurance and for that I am grateful - we were able to choose our surgeon for Joel and can choose our hospital for his surgeries and exams, but the insurance doesn't cover things like his drops or the gap for our specialist appointments. We don't have a health care card to enable us to access cheaper medications, therefore every bottle of drops costs us around $18. Since the start of this year, we have bought 18 bottles. Seeing Dr Lam costs us around $75, with about $30 refunded through Medicare. His hospital admissions are $250 each time and on top of that we pay about $400 in "professional fees" (I have no idea what they are, the invoices come in with codes so we can put that through our insurance and Medicare.)

This pregnancy, I have been classed as high risk due to a placental abruption, IUGR and subchorionic hemmorage, so we are spending huge amounts (around $190 a week) on scans and consultations with the Maternal Fetal Medicine specialist. I honestly don't know how we have survived financially for as long as we have. But now, it has all come to a head and Richard has had to take on a second job to try to pay some of our medical bills. There are so many things we need and want; a new car, to finish off our front yard for the kids to play outside, new clothes, things for the kids, but it all has had to take a back seat to these medical costs.

I guess it's one of those sides to having a child with something like this that people don't often think about. More so, we are asked how we are doing emotionally, how we are dealing with the pregnancy complications, things with Joel's eye. The money side of things just doesn't enter most people's thoughts. We are lucky that we live in Australia and have access to a lot of free health care and services, it's just seems that if you have private insurance, earn a decent wage and work hard, you are punsihed more than rewarded when it comes to these things. One lady I know gets the same drops for $2.60 because she has a health care card and doesn't work. It's hard not to get bitter about it. My husband works so hard for his family yet we have no spare money after paying so much for the same services that other people receive for free.

It has been suggested to us to look into what benefits we can receive for having a "disabled" child, but to me, Joel isn't disabled. He just has one eye that can't see. I really hope he never thinks of himself as disabled, that he knows he is just as capable fo doing everything else that other little boys can do.

Thursday, June 16, 2011

EUA rebooked

We couldn't do Joel's EUA and take the impression for his eye because he had an ear infection and was just really sick so the doctor suggested maybe putting it off until he could handle the GA a bit better. It's been re booked for the 30th June. Now he has sprained his little foot, I have no idea how. He is constantly tripping and falling, more so than a toddler with perfect vision. He seems to struggle with depth perception a bit; stairs and small steps are a hurdle. He has learnt to do some other things way before his sister though, he can ride a little 3 wheel bike like he's been doing it for years and has absolutely no fear of climbing, getting down is he problem though, He just can't seem to work out how far he has to drop, whether it's 10cm or 100cm. His latest bruise on his forehead is from dropping way too far onto the floor, face first. I was worried about his foot because he was limping and I thought I'd better take him to get it checked out as soon as I could because if I left it and it was fractured and they looked at the bruises on his head I could be in trouble! Poor little guy looks like he's been beaten up! He's such a trooper though, he'll just keep trying things until he works it out and I really try not to do too much for him now because I don't want him to grow up thinking that he can't do all the same things as all the other kids. He is getting cheekier by the day too, makes me laugh all the time. We have finished with the Maxidex drops and his eyeball is yellow and brown, like an old bruise. I have heard that hyphema's can cause staining of the cornea and I am pretty sure that's what's happened this time, I doubt we'll ever see his iris and pupil again.

We have also been to see a geneticist, I have a letter fromt hem about what they think about his PHPV so I will try to scan it in and post it for those who are interested in any genetic link.