What is PFV?

Our Latest Adventures, Retina & Cataract Removal, Strabismus Surgery

Persistent Fetal Vasculature or PFV is a developmental disorder that occurs at birth. In utero, the eye is built by the body using different structures – called stalks. In a typical child, the stalk will regress or wither away when it is done building the eye. In people with PFV, the stalk fails to regress causing structural abnormalities that usually lead to impairment in their vision (ASRS). In most cases, PFV will only affect one eye.

When looking at the eye, you will first see the cornea, followed by the Pupil, Iris and lens. But, the structure doctors look at first when dealing with a PFV case is the Retina. The retina is a thin layer of nerve tissue that is very light-sensitive that sits at the back of the eye. When a person sees light, the light enters the eye through the Iris and heads to the back of the eye where the light is processed into images which are focused and reported up to the brain, producing sight. Since the connecting the retina to the front of the eye never retreated in people presenting with PFV, the light is unable to filter properly through the Iris and its trip to the retina is disrupted. This causes poor to no vision in these individuals. This disorder usually does not progress after birth but as the eye continues to grow, the stalk and its components will not grow with it. As the stalk holds back other structures of the eye, it may cause other issues moving forward. The stalk may cause the retina to detach or the person’s eye to develop cataracts.

As you can see from many discovery stories on our blog, diagnosis of PFV is hard to come by. Some are lucky enough to spot it at birth but in others, it may present itself in other ways that lead to misdiagnosis. For example, PFV can present itself as the child being cross eyed (Stabismus), having abnormal eye movements (nystagmus) and as having a lazy eye (amblyopia) (ASRS). In many people with PFV, the affected eye will appear smaller than the other, however this isn’t the case for everyone.  When a doctor believes the person may have PFV, they will look at the back of the eye to see if the stalk is present. Ultrasounds or MRI scans may be required to rule out other disease like Retinoblastoma – a rare tumor that may present itself on the back of the eye. 

When Retinoblastoma is ruled out, the doctor may suggest surgery to remove the lens, remove the stalk and in some cases, reattach the retina.  The surgery may not completely restore the childs’ vision to a state of “normal” but, without the stalk holding back the structures of the eye, the function of the eye can be improved and the affected eye may grow at a typical rate post surgery (ASRS). 

For most children and families, post surgery starts an entirely new journey. A contact lens is usually prescribed to children who had their lens removed during surgery. They may also get glasses to use instead of or in addition to the contact lens. Soon after surgery, your child may begin patching therapy to help with their strabismus or amblyopia. 

Unfortunately, peer reviewed studies of people with PFV are far and few between. When you do a general search of PFV, you will be directed to articles published that are full are very medical terms with a lot of conjecture and not many reliable numbers. The truth is, PFV is rare and doctors simply have not had the opportunity to study this disorder to its fullest potential. Until more research is done, we will continue to share what we have learned from these various medical journals and will continue to hope for more research moving forward!

I’m not a helicopter parent…just a mom

Food Allergies, Our Latest Adventures

With Toy Story 4 out in theaters, we decided to show Olivia Toy Story. She LOVED it and has been dancing with her daddy to “You’ve Got a Friend in Me” for days. So, in the spirit of fun toys that talk and play we decided to throw on “The Toy that Time Forgot”. I had never seen it before but it came free with our “Toy Story” purchase so I thought, why not! I’ve never been more disappointed in Disney than I was today…

Only 2 minutes into the movie, a scene plays out where the dinosaur is dressed up as a pretentious looking mother – pointy glasses and all. The dinosaur yells at the little girl playing the waitress that there is a bug in her sons ice cream and her son is “ALLERGIC TO BUGS”. So the little girl flicks the bug out of the ice cream and says “there you go sweetheart, enjoy”. And then the movie moves on.

In our world, food allergies are constantly seen as a burden and a joke. This scene does nothing but perpetuate this unfortunate view point. When a child watches this movie and sees this scene, they may learn that by simply removing an allergen from someones’ food, it is now safe. They may see food allergies as funny, simple and as a simple inconvenience. This scene in this movie does food allergies an extreme injustice – making a life threatening occurrence seem joke worthy and mundane. The mother who says her son has a bug allergy is shown as a know it all ‘helicopter’ parent and the waitress brushes off the allergy like the mom is simply being a pain.

At restaurants I am hit with constant dirty looks and hear whispers under their breath about how ridiculous I look wiping down the booths and chairs, bringing our full course meals into perfectly nice dinning rooms. I thought I never wanted to be that kind of parent – never wanted to be the ‘neurotic’ mom who keeps her kids in a bubble. That is until I became an allergy mom. My judgements are GONE. Now, I lock eyes with other moms who may be seen as ‘neurotic’ and give them a supportive nod. I want other people to know, that I am not being neurotic. I am taking care of my baby in the best possible way that I know how. Having food allergies in a world that uses them as the butt end of a joke is tough. When characters in movies laugh about something that could kill my daughter is heart breaking. But I know that Olivia is going to teach others about why this movie was wrong and educate others on how to keep her safe.

So, please talk to your kids about why this scene is so wrong. Simply removing an allergen from the food will NOT remove what makes kids like Olivia deathly ill. Removing that bug from her sundae would not get rid of the bug protein. I know – Im talking about bugs and pretend ice cream sundaes – but to kids, these stories are as real as they can be. And give that mom insistently wiping down the tables and chasing their kids around as much grace as you can, because you do not know what they are going through. So no, I’m not a helicopter parent – I’m just keeping my little girl safe.

In the meantime, just keep advocating!