One Family’s Fight for Their Daughter’s Eyesight

Incontinentia pigmenti (IP) is a type of ectodermal dysplasia that causes complex issues for the people it affects. Skin rashes are the hallmark symptom of IP. But, the eyes can also be greatly affected.

It is imperative that babies diagnosed with IP have a complete medical eye examination as soon as possible after diagnosis. This important step can help assure your child’s vision and eye health.

New Resource

An infant may have abnormal retinal blood vessel formation and retinal bleeding in infancy. If there is bleeding, this can cause the eyes to be small. These are just a few of the many possible eye challenges. You can read more about how IP can affect the eyes and treatment protocols.

Lily has curly blonde hair. She's wearing red glasss and an off-white sweater.
Lily Light is a nine-year-old girl affected by incontinentia pigmenti.

We have published a brand new resource in our library called, “Tips for Incontinentia Pigmenti Eyes.” Lacy Light, the mother of a child affected by IP wrote the article to help other families better understand common terminology. She also outlines suggested best practices and provides information about what kinds of exams your child with IP should have and when.

Dr. Richard A. Lewis, an ophthalmologist from Baylor College of Medicine and member of our Scientific Advisory Council, reviewed and contributed to the article.

Download Tips for IP Eyes

Eye Exams Your Child Will Need

The authors provide tips on what to take to your eye appointments and what examinations the physician should perform.

DO NOT take ‘No’ for an answer unless your ophthalmologist assures you and all exams have been performed showing that the retinal blood vessel development is normal and completely mature. Your child’s eyesight could depend on it.

– Lacy Light

Also, you can learn the signs to look for if the retina has a tear or detachments in adolescents and adults such as flashes of light or blurred vision. If you or your child have IP, you will want to download and print this helpful resource to have with you.

Lily’s Story

Lacy’s knowledge about IP comes from her first-hand experience as a mom. Read Lacy’s story about her family’s incredible journey to fight for their daughter’s vision.

By Lacy Light

My daughter, Lily, was born in 2012 with all the typical IP blisters and symptoms. At first, doctors thought she had a newborn rash. Then, they believed she was allergic to the soap her clothes were being washed in. When steroids and antibiotics weren’t working, they sent us to dermatology. She was two weeks old.

When she was being diagnosed by the dermatologist and she was looking her over, she said we needed to go to ophthalmology straight away. She believed our daughter could be going blind. She believed this because our daughter had one blue eye and one brown eye.

We were originally told this was because her blue eye hadn’t gotten the pigmentation that her brown eye had and that the blue eye would get darker as she got a little older since she was a little early coming into this world.

A Tough Day

I waited for what seemed like forever in the ophthalmology office to be seen as an emergency walk-in with her. I’m sure it wasn’t nearly as long as it felt, since I was waiting to see if my child was indeed going blind. During her exam, her eyes were dilated and she was strapped to a papoose board so they could do an in office eye exam.

Before this, I was told she would need an EUA (exam under anesthesia) for a better look of her eyes. After the exam, she was scheduled for an EUA with surgery. They told me that her right eye had a bleed and they needed to get it stopped as soon as possible to save her vision.

We were sent for blood work, more testing and scheduled for other appointments she needed. We were at a major medical facility. So, it was a bit easier since all the doctors she needed were in the facility. My mind was going a hundred miles a minute trying to absorb it all. I hadn’t been able to tell my husband or anyone else what was going on yet.

I was extremely overwhelmed when our day was over. What I thought was just going to be another day at the doctor trying to heal our daughter’s skin turned our lives upside down. We were crushed!

Exams and More Exams

My husband and I arrived for her surgery bright and early. They told us they were going to do a more in-depth exam on her eyes and laser the area where the bleed was. She would be back in two hours.

Two hours came and went. When her surgery was done, they discovered the vessels in her right eye were not fully formed and the bleed was worse than they had expected. Her eye had hemorrhaged and they had to do a lot of lasering to correct this.

But, we wouldn’t know for a while how much damage had already been caused by the hemorrhaging. On the bright side, we were told her left eye was completely healthy.

This is a scan of her eye as a baby before she completely lost the vision in her right. You can see the damage in the back top picture.

She went back in a couple of weeks for another EUA to see how the surgery was healing. They came to see us in the waiting room while she was under. We learned she was having contracting on her retina from the scar tissue and they were going to need preform more surgery. They trimmed back her scar tissue and lasered the side of her retina that was being pulled at. We were scheduled to come back in a few weeks for another EUA.

Traveling for Treatment

My husband was leaving to go to the field for training for a few weeks so I was at this surgery alone. During surgery, they found that she had another bleed in her right eye and more contracting. They had to place a gas bubble in her eye to hold her retina in place and did more lasering.

She was admitted to the hospital. Her retina had torn and things were not looking good.

During all of this, my husband was away for military training and would be red crossed home so that she could be discharged in few days. She would need to stay face down at all times for a couple of weeks and it wasn’t something I could do alone. She got a medical car bed in replacement of a car seat to keep her face down. This is not a pleasant contraption and she would scream all the time.

When we went back for her next appointment, we were scheduled for yet another surgery to put an oil bubble in. That didn’t require her to be face down, so she could fly to another state for surgery with a doctor who was familiar with IP and had been contacted for help. Her doctor did all the fighting to get her sent there with our insurance and we were on a plane just a few weeks later.

We Thought Everything Was Going to Be Ok

Cameron is sitting on a couch holding baby Lily in his arms. She's wearing a pink onesie with a bear on it. She has an eye patch and is wearing arm splints.
Lily’s brother, Cameron, holds her after her first surgery in Michigan. Her eye is patched and she wears braces on her arms to prevent scratching and pulling at the patch which is protecting her eye.

When we arrived, we met a man who I still think did the absolute best he could and knew of to save out daughter’s eye, which we believe was already too far gone to be saved. He had seen many IP patients from around the world and thought he could help our daughter. He had all her surgical notes and would perform an EUA and correct anything he saw. (I do not remember if she had an angiogram at the time. She was only three months old and I was still processing so much.)

At that time, he would also take a look at her left eye which we were always told was perfect. He agreed that her left eye looked good and just had a few abnormal blood vessels that wouldn’t cause her any issues in the future.

Lily with her eye patched after a surgery.

We stayed there a month and went home patching her good eye to strengthen the bad eye. We thought everything was going to be okay. Everyone seemed so confident that this time, things were looking up. Her eye seemed to be doing okay.

A few months later, we were flying back out to see him again to try and save her eye. Her retina detached again and the scar tissue was continuing to pull on the retina causing it to contract and tear. We were there for months during this trip. She had a vitrectomy, lensectomy, shots in her eyes and many other procedures and surgeries.

Losing the Fight

Unfortunately, after months, the doctor told us that there was nothing else that could be done. She was about 10 months old and we had lost the fight to save our daughter’s right eye and went back home to Washington.

Lily has a baby onesie on with yellow ducks.
Here’s Lily with her patch off after we were told there was nothing left that could be done. Her eye is very small at this point because her eye has started to “die”.

Her eye doctors were heartbroken for us and wished there was more they could do. We have a special place in our hearts for all of her eye specialists she has seen over the years. They all did the best they knew with the information they had at the time. We were told she would still need yearly visits until she was three for her right eye, but everything looked great.

As time went on, her eye shrunk and she would now need a prosthetic shell in her right eye to keep her skull growing normally and to give her a more natural looking eye. Her eye was very small, cloudy, and the pupil was popped open.

These photos show the process of Lily getting her first prosthetic eye.

She was 12 or 13 months old when we went to Kirkland, Washington for her first prosthetic shell. It was a very interesting process. After a week, she had a beautiful, new, hand-painted eye. It is so natural looking and no one can tell that she has an artificial eye.

Following My Instincts

I found a support group online for others with IP syndrome and their families during her surgeries. I posted in the group and was told by another momma that I should get in contact with an eye doctor in South Florida that has an extensive background in IP.  She suggested that we try to see her or speak with her if we can.

Lily is looking into the camera. She's wearing a gray shirt and has blonde curls with a pink glasses on her head.
Lily got her second prosthetic eye in the summer of 2019. We flew back to Washington State for this eye.

Florida was across the country and I had no idea how we would make this happen. A few months later, my husband was told he was being sent to school for military training for an assignment and we could pick five choices for places we would like to be PSC’d (moved) to after he was through. We chose Florida, Georgia and anywhere that we could to get us closer to that doctor because something in my momma heart told me WE HAD to see her.

We were lucky and got stationed four hours from the hospital that would be our saving grace and would lead us down another journey with our daughter’s eyes. Our insurance sent us to a doctor an hour away and wouldn’t approve my choice to see the doctor in South Florida without reason. I was crushed! I wanted nothing but to have our daughter seen by that doctor.

Closer to My Dream Doc

At this time, my daughter was now two. We made the hour drive to see an ophthalmologist who referred us over to a retina specialist close by that my insurance would approve. I was once again beside myself because I wanted to see the doctor in South Florida and I had this feeling in my gut that we needed her and soon.

We went to our appointment with the retina specialist. It was the start of my prayers being answered in getting her where I whole heartily believed and felt she needed to be. During our first appointment, the retina specialist said he wanted to do an EUA with her ophthalmologist.

But, he wanted to refer us over to a colleague that he studied under who specializes in IP syndrome that he thought she would be the perfect fit for our daughter. He wanted to know if we were willing to travel to South Florida to see her to have a more extensive exam.

I broke down in tears in his office and told him that’s all I had wanted for a long time now and thanked him so much. She had her EUA with him and her ophthalmologist where they saw no concern in her left eye as well. But, he still wanted us to go to Miami to the other specialist and have a fluorescein angiogram.

Heading To Miami

When her appointment with our dream doctor came up, I had so many emotions. Our initial visit included talking about her eyes, IP, making sure we were seeing all the right specialists and an office exam. She knew so much about IP. This made us all the more comfortable since we had not seen anyone that knew as much as she did about our daughter’s rare condition besides the dermatologist.

We were scheduled for an EUA with angiogram at that appointment and she explained why this was being done. This exam was necessary in order to get a thorough look at the back of her eyes and to see any bleeding that the naked eye is unable to see. We had her EUA and angiogram later that week.

A short time into her exam, her doctor came out to tell us that our daughter had some vessel leakage that had been going on for some time now in the left eye. We do not know when this leaking started, but it was not a fresh leak and could only be seen with an angiogram. Without one, it would have been missed entirely until it became a heavy bleed.

Saving Her Vision

With IP, it is of the most importance to catch these things as fast possible to prevent blindness. My worst fear had come to light and we were going to need surgery in her left eye. Our emotions were all over the place with the “what ifs” and if we were headed down the same path as before.

The doctor in Miami was able to save her vision and we will forever be grateful for her. Here we are, five years after her last bleed, and our daughter is now nine years old. She has lost some peripheral vision in her left eye but still has good vision in her remaining eye.

We go to Miami once a year now for her angiograms but have been released to her ophthalmologist for appointments in between. She was seen every three months for years. Then, we moved to four months and then to six months. Then, back to three after some leakage that she kept an eye on because at that time, our daughter was having blood clotting issues.

Lily is standing outside and wearing a rainbow formal dress with a rainbow colored bow in her hair.
Here’s Lily on 9th birthday earlier this month!

At her next appointment, the leaking had stopped and had not caused any further damage to her eye. We believe that leak was due to the underlined blood thinning she had at the time. She hasn’t had any issue since but has slowly moved to stretched out appointments over the years.

She had frequent appointments due to everything she had going on with her eyes. She will require yearly appointments for the rest of her life. Lily is doing great and we expect her next appointment to go just as swimmingly as they have the last five years.

We will always be on guard with her left eye and do not know what the future holds but what we do know is that she is strong, resilient and will take whatever the future throws at her and make the best of it.

3 comments on “What You Need To Know To Help Your Child’s Vision If They Have IP”

  1. 1
    Adriana R. on April 7, 2021

    This touches my heart deeply. The same doctor has saved my daughter’s vision as well. I thank God for her, for you, for Lily and for our IP group, because without you my little one probably would be blind by now. God bless you all. And Lily looks so beautiful, look at her!

  2. 2
    usama on April 13, 2021

    Great Work

  3. 3
    usama on April 13, 2021

    Hits Deep in My Heart, best wishes for Lily.

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