Treatment 1: The Healing Period

We documented Enler's progress until resolution of his bruise post first treatment. Here is our photo log.

Treatment Day

getting cuddles from mommy

Day 2

Day 3

Day 4

Day 5

Day 6

Day 7

Day 8

Day 9

Day 10

Day 11

Day 12

Day 13

Day 14

Day 15

Day 16

Day 17

Day 18

Day 19

Day 20

All healed up and ready to play! Thanks for following along with our journey :)

First Treatment

We got home a little while ago from Enler's first treatment. We are sitting here on the couch next to my mom who is cuddling our beautiful sleeping baby.

The poor thing developed increasing congestion and a cough yesterday. It was a bit worse this morning but we weren't sure if that would affect the treatment or not so headed to Phoenix Children's Hospital (PCH) to find out. We were up at 4:45 am (although I had gotten up at 2:45 for his last feed prior to the procedure). It was raining and lovely outside. We got showered and woke up the wee man and got him dressed. We were out the door by 5:30 and checking in at PCH at 6 am. Mom met us there.

The whole process went very smoothly. After checking in at the front desk we went back to admitting. There I had to fill out some paper work and pay the facility fee ($200). We will still be billed for the physicians. Shortly after we were called back to pre-op. We had lovely nurses who took Enler's vitals and gathered his information. The anesthesiologist came and met with us and said it was OK to proceed given his congestion and cough. We were also visited by the eye doctor, who is lovely, and the dermatology fellow. The dermatologist was running a wee bit late so we wouldn't see him until after. Within 30 minutes we were taken back to the procedure room. Enler slept through most of this - what a good boy! He only fussed when the drops were put in to his eyes to dilate them.

Back in the procedure room there was a lot of commotion. There were approximately 8 people in the room and it was very bright. It made a difference that everyone was so welcoming and warm. We laid Enler down on the procedure bed and the strawberry anesthesia mask was placed on him. Once he was out we each gave him a big kiss and headed to the waiting area. We experienced a bit of anxiety and sadness leaving our little guy. The only way we were able to make that journey out there was knowing that the physicians and other staff were going to take excellent care of our little man.

One by one the docs came out to tell us how it went. First, the eye doctor. No sign of glaucoma! His eyes are looking great. We don't need another visit until 3 months from now. Then, the dermatologist and fellow arrived. They said the procedure went well. For his first treatment they started with the lower power on the laser. The treatment was focused on the darker area towards his mid face and they avoided going past his hair line. I guess there has been some hair thinning in other babies. They don't get to that area until he's older. Next treatment will be in 2 months (although we'll wait until February until I have some more time so I can have the day off with him). In regards to his healing, we should expect that his bruising will improve over the next week. It should be all gone within 10 days. We have an antibiotic cream to apply twice a day until then. If it blisters, which they don't think it should, we have an prescription for steroids. We probably won't see significant improvement for 4 -5 months - and we'll already be into the 2nd treatment by then. What an adventure.

The last doc was the anesthesiologist. He was such a sweet man. He said everything went well although Enler experienced laryngeal spasm and required intubation. This was most likely secondary to his congestion. He was now extubated and doing well getting cuddles by the nurses. We were so thankful.

We were then taken back to the post op area. It was a bit of a shock at first seeing Enler with the big purple bruise on his face. Although we had looked at photos of other kiddos who had been through the treatment, nothing can prepare you for seeing your own son all bruised up. His face had dark purple dots on a dark pink background and it was swollen. All we wanted to do was give him lots of kisses and cuddles.

By 9:30 am we were headed home to heal. A very quick and successful procedure. Here are some photos from the journey. We will continue to update as he heals.

Grandma Christine and Grandpa John's Impression

My mom was in the room with us when Enler was born. She was a great support during the birth and helped document his entry into the world. Meanwhile, my dad patiently awaited his grandsons (which he predicted from the start) arrival with my brother Joe. They were the first family members to meet our new little guy. Here are my mom and dad's thoughts about Enler's birthmark.

There was soooo very much excitement and energy around Enler making his appearance into the world, when I saw and then heard Graham saying something like, "oh goodness, does he have a bruise on his face?" In the businesses of all that was happening the physicians and nurses took a look and said something to the effect that, "it may be a bruise or a birth mark." While the whole process of checking him, weighing him, cleaning him and taking care of his Mom went on, it became clear that it was a birth mark of sorts. I could tell that Graham was worried and Juliana was concerned and I shared the worry of something unknown. I was so caught up in the miracle of it all, and the fact that with the confidence Juliana had in her doctors, the feeling of it would be okay, surely was okay.

Honestly, writing now at 6 months all I can think of was what a perfect baby he was. What a miracle and how lucky we were and what a wonderful delivery team Juliana and Graham were. Seeing all of him and holding him he was that...our perfect miracle.

I was so in awe of the birth and of Enler and happy when we kept getting scores and reports of his perfect health. So the birth mark was something that would be explored and understood and dealt with at a later stage. It would take some time, a few days, before we got all the information to realize that he had a Port Wine Stain. So we had a name of what it was and gradually the whole story of what his journey could have been and what it would be.

When his Grandpa John and I talked about it he said that he knew that the birth mark was of concern when he first looked at his grandson. His thoughts were immediately for Juliana and Graham that they not feel disappointed that he had it and that it took away from him. He also said that he wouldnt want them to worry what people would say about Enler. In fact, all along he has said to leave it (except for the medical reasons) because it was a part of him.

Granny Ann & Granda Danny's impressions

Graham and I asked his folks to send us their thoughts and first impressions of Enler's birthmark. We are so blessed to have such a supportive, loving family.

Here are their beautiful words.

"Dear Graham, Jewel and Enler,

It is with a heavy heart that I write this. When Enler was born, and Graham told us the baby had a Port Wine Stain on one side of his little face, I felt very sad. Then some photos arrived and we saw for ourselves the Port Wine Stain on such a beautiful baby. Dad thought it was the least that could have happened to a new born baby.

I know Jewel & yourself are the best parents any child could have and the procedures Enler has in front of him will break both your hearts but it is the best in the future for the child."

in a later letter...

"Enler's first laser treatment is this month. Poor wee darling. It breaks my heart to think of him going through it. I know how busy you both must be, wish I was there to help you.

Lots of love, Mum & Dad"

These pictures are from Ann & Danny's month visit to Arizona in June to meet their new grandson. The top one is taken in northern Arizona in Williams and the bottom from our professional family photos. These are the first Ann & Danny have ever had at a photo studio!

Insurance approved treatment!!!

We just found out last week that the insurance has approved Enler's first treatment. We are very excited. We are also coordinating his first treatment with his next eye exam (so the eye doc can check his eyes out while he is under anesthesia). Everything is coming together so well!

Next post will probably be post-first treatment. Wish us luck!

Port Wine Stain Resources

During all of our research we have stumbled upon some very helpful and supportive resources in regards to Port Wine Stains. We thought we'd compile them here to help other families going through the same thing, or for anyone else who is interested.

Vascular Birthmarks Foundation: http://birthmark.org/

The leading not-for-profit in the world for children and adults affected by hemangiomas, port wine stains, Sturge-Weber Syndrome, Klippel Trenaunay Syndrome, and other vascular malformations and syndromes.

Things that we found helpful about the website include up to date research, comments from experts, how to navigate health insurance coverage, and access to forums of families going through the same thing as us.

KidsHealth from Nemours: http://kidshealth.org/parent/medical/heart/port_wine_stains.html

KidsHealth is a website that combines health information for parents, kids and teens that is free of medical jargon. As part of The Nemours Foundations's Center for Children's Health Media, KidsHealth also provides families with perspective, advice, and comfort about a wide range of physical, emotional, and behavioral issues that affect children and teens.

From the link above you can go to a page that is designed for kids. It has a lovely story about a boy Evan who was born with a Port Wine Stain. When he was young, he asked his mom why there were no children's books with kids with birthmarks. They couldn't figure out why - so they wrote and published one! Its called "Buddy Booby's Birthmark." We just ordered it and can't wait to read it to Enler!

Medline Plus: http://www.nlm.nih.gov/medlineplus/ency/article/001475.htm

Medline Plus is a service of the U.S. National Library of Medicine and the National Institutes of Health. Here you can find all the facts about Port Wine Stains and lots of helpful links.

You Tube Videos:

There are many beautiful videos compiled by families and kids themselves affected by port wine stains. Here are a few of our favorite:

Port Wine Stain: A Documentary - by Dakota

http://www.youtube.com/user/365Dakota

Calley's story

http://www.youtube.com/watch?v=no9vF8douq0&feature=related

Aiden's story

http://www.youtube.com/watch?v=oty5h12DMCQ&feature=related

This is all we have so far! We'll update as we find more resources. :)

Here's a recent pic of our little guy playing with paint at school.

Pediatric Dermatologist

The next step in our journey to treatment was the pediatric dermatologist. I had called our pediatrician and she recommended someone at Phoenix Children's Hospital (PCH). I called to make an appointment but they were scheduling about two months in the future (when I was already back at work!). They said I could get the pediatrician to call and try and get an earlier appointment and that I could get on the wait list for earlier appointments if they came up. I did both and lucky for us we got an earlier appointment. It meant we had to cut our Twaddell Williams trip back by a day, but it was no problem.

Graham and I both went to see the dermatologist with Enler. It was fun going as a family. PCH is a gorgeous campus and the dermatologist office was lovely. Best of all, there was a very cute kids movie on in the waiting room. We giggled in amusement as the dogs told silly jokes to one another. We didn't get to watch long though as Enler was brought back quickly to see the doctor.

As soon as the doctor entered the room I think we both knew we would like him. He's an older, grey haired jolly looking man and was wearing a turquoise bolo tie (Arizona's state neck wear!). He asked about Enler's name and as soon as Graham got the word Ireland out of his mouth the doc dove into stories about his very recent trip there. He had been to the Giant's Causeway and we spent some time sharing our awe of the natural wonder. He even had a pint in the historic Crown Bar in Belfast, a bar that is next to the bar Graham worked at for many years. If we had been nervous prior to this introduction, we were definitely not now.

The doctor then started to talk about Enler's birthmark and its treatment. He called Enler's port wine stain extensive and said he would definitely recommend treatment. He said he would probably need between 5 - 12 treatments and that we could start as soon as we were ready. We would need to leave at least two months between each treatment. He likes to treat babies before they are four because that is when they develop permanent memory. The treatment is done with a laser and actually only takes about 20 minutes. The thing that takes long is getting him under anesthesia. They do that because the laser treatment can be painful. Enler will have a big bruise on his face for about a week after his treatment. The doctor said he would give us a note to have regarding the treatment so no one thinks we were abusing our baby (apparently that has happened in the past with his other patients!).

Then, the doctor asked us what insurance we had. Up to this point we hadn't even considered the fact that treatments might not be covered under insurance and we may have to pay out of pocket. Financial aspects of treatment had not entered into our previous conversations. We told him we were on United, but switching soon because I was starting work (and my work insurance would be about $300 less a pay check then we were paying now). He said that was too bad because United usually covers the procedure with no problem. Blue Cross Blue Shield (which was basically what we were switching to) gives his office a lot of headaches. However, if he discussed the concern for glaucoma and sturge weber (given the distribution of Enler's birthmark) he thinks it could be covered. Oh dear, what a mess! On top of adjusting to Enler's birthmark, researching the associated diseases, deciding whether or not to treat and following up with all the necessary doctors, we also had to figure out insurance issues!

With the insurance issue in mind, we discussed staying on United. However, we had just got a letter in the mail from United saying they were no longer going to cover our pediatrician. We really liked our pediatrician so didn't want to switch. We decided we'd switch to the Mayo plan (Blue Cross Blue Shield for Pediatrics in Arizona) and take it from there (and that's where we still are). We have scheduled Enler's first treatment for October as I scheduled a week vacation during that time. Graham can take some time off too and we'll be able to hang out at home with him. The doctors office is currently checking with our insurance to see if the procedure is covered. We will find out soon! If not, it will cost $5000 a treatment.

So many things to think about! At least we loved the dermatologist and really trusted him. He's been at PCH for awhile and has treated hundreds of kids with port wine stains. He says his results are better than the research shows. Most kids get marked improvement and that's what he expects for Enler.

As of now, we'll be heading to PCH on October 21st around 4:30 in the morning for Enler's treatment. Its comforting to me that I'm familiar with the facility (as I did my third year rotations there) and I've met most of the anesthesiologists! We'll keep you posted!

Eye Doctor

The first required visit for Enler's birthmark was to the opthamologist. I wanted an appointment before I went to work so had to go to the office fairly far away in Mesa. Graham's folks had just got into town the night before, so we left them sleeping although prepared to be ready for us coming back for lunch. Graham was at work so Enler and I were flying solo that day.

The eye doctors office was a bit chaotic. It was shared with an adult opthamologist so half of the waiting room had kids toys and animated movies playing while the other half was just flat out boring. Enler was asleep when we got to the office. He was still at the stage that when he was awake that usually meant he needed to feed, otherwise he slept.

The medical assistant saw I had a lot to carry when she called me back so she helped carry us in. The eye doctor was a very sweet man who worked quickly but with such compassion we felt listened to and taken care of. He wanted to take a look at Enler's eyes so needed them dilated. After the medical assistant placed the drops in his eyes we were back in the waiting room giving the drops time to work. Twenty minutes later Enler was on my lap as I sat in the exam chair and the opthamologist explored the backs of Enler's eyes with his fancy tools. Enler only cried a bit. My good boy.

The doctor said he looked completely normal. However, because of the extent of his birthmark and the portion above his eye he still had a 10 % chance of developing congenital glaucoma. This type of glaucoma is different than adult onset glaucoma as it develops rapidly and is very obvious. If Enler got it, his eye would get very large. The doctor wanted to see him every 6 weeks for the next year to keep track of his eye.

Because Enler is such a big kiddo, the doctor said he could coordinate future visits with our dermatologist (who he knew and highly respected) to look in his eyes . When Enler was under anesthesia for his treatments, he would pop in and take a look! Sounded like a good deal (and Enler wouldn't be traumatized by all the poking and proding).

I felt good leaving the doc. Relieved Enler's eyes looked perfect so far, empowered I knew what to look for in the future and comforted that the doctor would follow him closely and alongside the rest of our physician team!

Newborn Pediatric Visit

While I was still pregnant I went on a Pediatrician scavenger hunt. It ended up being one of the easiest parts of our preparation for parenthood. I asked a trusted Chandler mommy source for her advice and she recommended Mesa Pediatrics. Turns out their new office was literally across the street from us. Moreover, my brothers and I had gone to the original Mesa Peds office when we were kids. I met with our future pediatrician and absolutely loved her. She trained at PCH, spent a year as a chief and has her MPH from U of A. She is perfect!

We were instructed at the hospital to make an appointment with our pediatrician the day after we got back from the hospital because Enler had a slightly elevated bilirubin (break down product of hemoglobin - the blood component that carries oxygen). We were in a new parent daze but excited to introduce our new little man to our new pediatrician.

It was at this appointment that his diagnosis of port wine stain was confirmed and when we first started talking about treatment and surveillance options. Our pediatrician said we didn't need to worry about setting up any appointments now but could discuss future options at our one month visit. She told us we'd need to schedule an appointment with the opthamologist first. This was necessary because port wine stains can be associated with congenital glaucoma (increased pressure in the eye with accompanying vision changes). The likelihood is increased when the birthmark is above the palpebral fissure (the eye opening). Enler only had a small part of his birthmark in that area so chances were less likely - but enough to necessitate an eye appointment.

We had the opportunity to ask all of our burning questions. Why did Enler get this birthmark? Was it anything we did? Is it genetic? How prevalent are port wine stains? Our pediatrician addressed all of our questions. Port wine stains occur in 3 out of every 1,000 people (which made Graham think "wow, what a special little guy we have"). We were relieved to hear it was not something we did and they don't really know if its genetic. When she said that she wasn't sure if its something that Enler could pass on to his kids, I got very weepy (thanks to the pregnancy hormones). The thought of my new baby having his own kids one day drove me to happy, proud tears.

Walking out of the appointment we felt confident that his medical condition had been addressed and he would be well taken care of by our pediatrician. It was at this point that the dialogue between Graham and I truly began about what to do as far as treatment. What are our options? Do we look into laser treatments? Or do we just leave it? We spent time researching on the internet, in our baby and pregnancy books and I even pulled out some old textbooks from medical school.*

As my research developed I found some journal articles that discussed the advantage of early laser treatment for port wine stain. A few well designed studies found that treating earlier led to better results with fewer number of laser treatments. The results described included complete resolution of the birthmark to significant lightening of the birthmark. With this information, and the other research we had done, Graham and I decided we wanted to proceed with a dermatology consult. Because I would be returning to work soon I called our pediatrician before the one month visit to arrange the consult. She had also been doing more research on port wine stain treatments and agreed with a earlier rather than later consult with dermatology.

* It was helpful having experience in medicine to navigate our web search. It was amazing the things that came up in our Google search, including some scary pictures and some ones that had no relevance to port wine stains at all!

First Impressions

From Daddy

When I first saw Enler's birthmark, I was a little sad and thought of it similar to a disfigurement on my new babies face. The delivering doctor said "Oh, he's got a bruise" and then later said "no, its a birthmark, they are very common in newborns and it will fade away." At some point we were told it was a port wine stain, which was a permanent mark. In the back of my mind I had thought it would not clear up. I could not get the image of a guy in my home town with a red bulbous nose out of my head and thought "oh how sad, my boy is going to be like that." It didn't take long for me to see right through the discolored stain on his face to see how beautiful and cute and gorgeous he was. And then I started to think of it as kinda like a badass thing - like my scars on my leg that I'm very proud of. To me, they are my tattoos.

Right after Enler was born I called my folks in Ireland and told them the good news! I told them about his birthmark and my folks were relieved to hear that he was 100 % healthy and said that a birthmark was nothing to be concerned about. They had known several families that had babies with serious illnesses. They were relieved our family was safe and healthy. My folks are worriers.

I spoke to my folks every day while in the hospital. On the last day I mentioned to my father that I might give Enler the nick name "Patch" because of his birthmark. He severely scolded me and said "they'll be no such talk like that" then abruptly handed the phone over to my mom. I think I upset him. My father's seriousness and tone made me take the situation a lot more seriously from that point on.

The pediatrician at the hospital confirmed that this was a permanent port wine stain, which could be treated (erased or reduced) through laser treatment. This was when Jewel and I were introduced to the possibility of this course of action.

Its crazy to think I had these feelings and emotions about Enler's port wine stain because now I seldom see it and when I do I think of it as a positive piece of him. He's become so much more than his birthmark.

From Mommy

When I first saw my beautiful baby boy it took me awhile to fully comprehend that he was mine. He looked so foreign to me - I don't even know what I was expecting - I guess I just really couldn't picture him when he was in my belly. I was also exhausted from all the pushing. Labor and delivery was such an indescribable experience that I think only other mothers truly understand. All the drama and pain and blood and emotions from the delivery were going through my mind so I didn't even start thinking about his birthmark until later that night when we were on the post partum floor. Then, there were so many other things to think about, such as my swollen 'down there,' inability to pee as of yet, need to breastfeed my baby (even though I had no clue what I was doing), need to change him - on top of being in the hospital overnight as a patient for the very first in my life. I don't even remember when I first really let myself think about his birthmark. I think it may have been the next day, or even after we had got him home.

It was easy to go to my intellectual side and get into my doctor jargon with friends, family and the pediatrician. It was relieving to hear the pediatrician say that the fact his birthmark followed the V2 distribution of the trigeminal nerve was a positive sign. This distribution is less commonly found in sturge-weber (vascular malformations in the brain) and glaucoma. However he would need to be followed by an opthamologist. I had to think back hard to my first two years of medical school to dig out any other relevant factoids regarding birthmarks and port wine stains. For some reason I just kept thinking about Gorbachev. What an image...

When I allowed myself to really feel what it was like to have a baby with a medical condition - something even mildly associated with a disease as serious as sturge-weber- I wept. I just kept looking at my gorgeous baby boy and thinking it would be so much easier if he was just born without the birthmark. I wondered if I could rewind time and erase it from his face in utero. And then I felt terrible about thinking these things and just wanted to be strong and love him and let him know that it didn't matter and he was going to be a gorgeous, strong little man regardless. I only shared these thoughts with few. I wanted everyone to know how much I loved my little guy and it didn't matter to me. And I do - I love him more deeply than I've ever loved anything. I just had to give myself time to mourn his birthmark (the pregnancy hormones didn't help this out at all).

Sometimes I still think about it and cry. It catches me by surprise. For example, when I went to the breastfeeding support group - my first time bringing him out with other babies - his birthmark was so evident around the other babies. I felt I had to explain it. I still see it when I look at him. But it has just become Enler - just like his cute little toes, chubby little legs and heart melting smile. He is my gorgeous wee man.

First Entry

Graham and I had a beautiful baby boy on May 3rd, 2010 at 6:41 pm in Phoenix, Arizona. He weighed 10 lbs 5 oz and was 23 inches long. We named him Enler after a river that runs through Comber, the town where his dad grew up in Northern Ireland. We feel so lucky to have such a beautiful, healthy baby.

Our baby was born completely healthy and with a beautiful birthmark. The name of this birthmark is a port wine stain. Unlike other common birthmarks babies are born with, this one will not fade with time. Enler's port wine stain is on his mid right face covering his cheek and lower eye lid (see picture above).

We read up on his birthmark and found out he would need laser treatments by a dermatologist to eliminate or reduce the mark. We have decided to seek out treatments, which will begin in October. Enler also needs to be followed by an opthamologist because of the risk of glaucoma.

This blog will document our families journey through Enler's port wine stain treatment.