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!

First Dentist Appointment with Food Allergies

Food Allergies, Our Latest Adventures

Everyone’s worst nightmare…a trip to the dentist!! At Olivia’s last pediatrician appointment we were told that it was time to take her to the dentist and my heart stopped. Not because they would be checking out her teeth but because they would have to put things in her mouth – things that could potentially contain her allergens! We have to be so meticulous about ingredients in everything that even comes close to her. We know every possible name for dairy, wheat, eggs, peanuts and tree nuts, but I had never had to worry about ingredients in a medicine or at a doctors’ office. Through the years, there have been a few stories where children were given a fluoride or toothpaste that contained their allergen and they had to go into pure survival mode – EPI included. I went home and started doing research. 

Cue Google Search –> Food Allergies and the Dentist

I wrote down all of my questions, all of the names that could possibly mean the same as any of her allergens (ie Casein is another form of Dairy!) and had a plan of attack. 

The morning of the dentist appointment, I told Olivia we were going to the Dentist so the doctor could check out her teeth and she couldn’t have been more excited. She pointed to her teeth all morning and kept yelling “momma, teeth!” It’s so funny how all the fear and anxiety I had been holding on to for this appointment can just melt away with one smile from that little girl. I took a page out of her book and we walked into the Dental office with all the confidence and courage I could muster. 

We filled out our new patient paperwork and waited to be walked in. My stomach was in knots! When we were called back, the dental hygienist told me to avoid the dentist chair and sit Olivia on the bench next to the computer. She said most kids get really freaked out by the big, scary chair and they tend to shut down. She had obviously never met Olivia! She climbed right up onto the big girl dentist chair, with a smile so big it could be seen from the moon. 

“Momma, look, chair” she would say followed by “Momma, teeth!”

While Olivia played in the chair, I pulled the hygienist over and asked her to see an ingredient list of anything they would be using on Olivia for this appointment. I needed to be 100% sure nothing contained her allergens. Unfortunately, the office didn’t have a printed list of the ingredients in their products, which threw me for a loop. My research had shown that they had to have the product ingredients available and the fact that they didn’t have them made me so nervous. Instead, they gave me samples of the Floride and cleaner to have it tested at the allergist to be sure it was safe. I settled for this solution but it did leave me underwhelmed.

Thankfully, the first appointment at this office simply consisted of a short medical history interview, a discussion about what types of foods and drinks are consumed and a quite look into the mouth! Olivia was a rock star and got her teeth checked out an the all clear from the dentist! When they were done checking her teeth, she kept signing more so they would keep going – she is the only kid that LOVES the dentist. I was relieved nothing was put in her mouth today – but to be honest, without knowing the product ingredients – nothing was going in her mouth regardless. 

She picked out a stuffed animal prize and we took a trip to Dee’s One Smart Cookie for an allergy safe cake pop. 

For any allergy parents that might be nervous for their child’s first dentist appointment – there’s nothing to worry about! The first appointment was very straight forward – interview and quick check with no products. Remember, if you can’t see ingredients or don’t have that reassurance that the product is safe, don’t let them use it. Just Keep Advocating!! And tell them why you aren’t able to proceed with your appointment and hopefully they will get you what you need to feel comfortable.