Two Steps Forward...

Well, we are blogging today from Children's Hospital in Plano as the big cold front unfolds outside our window.  We had a situation come up that sent us on a surprise trip to the ER...two steps forward and one step back. There's a lot to explain, so I'll just jump right into it...

Wednesday, November 20

Hazel goes to see Dr Bowman every other week for regular checkups on the cornea transplant and the health of her eye. The cornea looks great and was very clear, so no worries at the moment about her cornea transplant.  We have been concerned about her eye pressure since it is closely associated with glaucoma.  A safe and normal range for Hazel's eye pressure should be from 10 to 20...at her appointment the pressure was 34.  Pretty deflating news for us. At the appointment Hazel received an eye drop to help alleviate the pressure. 

By the time Hazel got home at 10:30 am she was screaming and crying without ceasing. By now we are familiar with her tired cry, angry cry, scared cry, hungry cry...this was different. Hazel was crying loud and long enough that we started to worry about her breathing. She was taking in small breaths and sometimes struggling to take air in. Things got scary at this point. Aubree and her Mom took Hazel to our pediatrician to get her chest listened to and to get help. They did not find any breathing issues by listening to her breathe and checking her airways. Her temperature was 96, which seemed low...so Dr. Le sent us to the Children's ER. 

The ER was a new and frightening experience for us. It was full on Emergency Room triage...just imagine someone kicking over an ant bed while screaming. Hazel was surrounded by up to 10 doctors, nurses and EMTs who were all performing their designated job. Aubree and I stood next to the warming bed and tried to hold it together while they worked on our baby girl. Her temperature was down to 93....then 91.  Her heart rate was slow, breathing was weak and an oxygen tube was placed in her nose. They put a warm IV in her head, took a blood sample, urine sample, nose swab, chest x-ray and even attempted a spinal tap (which ended up not happening).  This is another scene which is hard to describe from our perspective. If you ask Aubree she will probably describe this as the worst day of her life. It's hard to think about what would of happened if we didn't take her to the ER when we did. We could have lost her.  It's hard to think about it but it's true...we could have lost her.

After an exhaustive process of questions and retracing her medical history, we were able to identify the source of the problem. Despite multiple experts saying it couldn't be her new drop...it was. The new drop has a beta blocker component which is harmless for adults and has been used on kids, but in rare cases can be harmful to infants. At this point we don't gloss over the small print for medicines or procedures.  So when they say, "In rare cases _______ can occur", we soak it in and remember that our baby is a living rare occurrence.  The beta blocker slowed Hazel's heart rate and respiratory system which in turn lowered her temperature.

One bit of good news from our visit is that we were able to see the pediatric ophthalmologist at the hospital. They were able to measure her eye pressure again and it's down to 18!!!  EIGHTEEN!!!!!!  Hopefully this means her eye pressure will stay in the safe range for the foreseeable future. Hazel has a follow-up appointment next week with our glaucoma doctor. 

Thursday and Friday

We have been in the hospital since Wednesday while Hazel is under observation. They are holding us until the blood cultures come back from the lab. A "contaminant" was found in her initial blood draw that most likely came from something on her skin in the ER.  They took blood yesterday to have a second sample analyzed...which took four attempts with an angry Hazel.  Right now the second blood sample has not grown any contaminants and looks good. We feel confident that the first blood sample was lab error and that we can go home today.  If it was up to us we would have gone home last night...kind of feel like hostages at this point. 

We're thankful for our family and visitors who have brought snacks and come to check on us.  We would still appreciate anyone who can keep Hazel on their prayer list. She is definitely a fighter and keeps working hard against all these hospital visits. 

Through this experience Aubree and I learned that having the best doctors is great and medicines are amazing...but we can't take anything as a certainty.  We will do everything we can to give Hazel expert medical attention, but still lean on prayer and God's grace to keep her healthy. 

UPDATE (4:00 pm, Friday)

We will be in the hospital for another two days or so.  The short explanation is that we need to wait for the contaminant in the blood sample to grow until the medical staff can determine what it is.  Pretty frustrating news, this is the second time we thought we were going home...

Hearing Screening Results

Today was Hazel's in-depth hearing screening to get a better assessment of her ears.  Hazel failed both hearing screenings at the hospital and our first visit to the pediatric ENT in Frisco was inconclusive because they didn't have small enough equipment.  Newborn problems, right?  Anyways, the results were great across the board.  Aubree called and let me know that Hazel is able to hear like an infant should and passed the tests today...which is a huge relief. 

So at this point the only things we have to be concerned about are her eyes and the next surgery for her cornea transplant, which is December 13.  Right now there is not much to report about Hazel's day-to-day activities...but no news is good news when it comes to eye surgery.  Our next follow-up with Dr Bowman is not until next Wednesday, so if there is anything to report about her eyes it would happen next week.

Sorry this is a quick post but life is pretty busy right now and I need to get back to work.  Hope everyone is having a great day and enjoying the winter weather. 

As usual, we appreciate ALL the prayers and support for Hazel.  We couldn't do this without all of you!

Hazel's Life After Surgery

It's been three days since Hazel's first cornea replacement surgery at Cooks Children's Hospital in Fort Worth.  Friday and Saturday turned into much longer days than either Aubree or I anticipated as we stayed overnight at the hospital.  Unfortunately this also meant a trip from Fort Worth to our apartment and back for clothes, plus dropping off our dog Gus in Coppell to stay with his grandparents. 

We were discharged from Cooks at 6:30 am and got in our car before the sun came up for the second morning in a row.  Hazel still had her eye patch on and the "R" and "L" written above her right and left eye for the surgery had not faded.  Normally we would head straight home and jump in bed...but we had an 8:30 am appointment with Dr.Bowman's office for a post-op check.  After a stop at Starbucks we arrived in Dallas for our appointment an hour early, just enough time to close our eyes or zone out for a bit.  The doctor on-call at Dr.Bowman's office was able to remove Hazel's eye patch and take the first look at her new cornea.  The difference is incredible.  After three weeks of sitting with Hazel and looking into her eyes I became accustomed to her light blue eyes and cloudy corneas, but seeing her new cornea with a jet black pupil was like seeing her eyes for the first time.  We talked about Hazel's treatments with the doctor and about four nurses who were all fascinated with Hazel...probably since most of their patients are adults or senior citizens. 

The doctor gave us Hazel's eye drops (two different types) and an additional eye cream which must be applied daily.  Four drops from one bottle, two from the other and one application of the cream.  If you think it is difficult to give an infant one eye drop, just try giving them 6 eye drops and an eye cream every day.  They hate it and you end up feeling terrible for making them upset...that's just how it has to be.  The surgery and anesthesia made Hazel very drowsy and she slept for the majority of the first two days following the surgery.  But late Saturday morning Hazel was in her crib when she opened both eyes for the first time since the surgery.  There is not a good way for me to describe how it felt to watch her look around for the first time.  It was incredible.  It was amazing.  It was a big win for a family who really needed one.  Hazel would look around the apartment before the surgery but now it's very different.  You can see her tiny brain trying to work things out and use her right eye to see more of the world than she previous knew existed. 

If you look at the picture below you can even see light reflecting from the 16 sutures that are holding her new cornea in place.

Hazel the day after her first eye surgery

I've come across a few recurring questions about how Hazel is doing in the days following her transplant, pending tests, her new diagnosis of sclerocornea and her next hearing test...so a few follow-up answers:

What does sclerocornea means versus Peter's Anomaly? 
The new diagnosis is a big positive because it is a less difficult ailment than Peter's Anomaly, which is part of a larger syndrome.  This new diagnosis does NOT change any of the factors related to cornea surgeries for infants.  Hazel's high odds of rejecting the transplant remain and she WILL need multiple surgeries throughout her life to improve her vision.  There is not a set timeline for the surgeries such as "every 5 years" or anything like that.  Her early need for cornea replacements is so rare that every infant patient is going to have different results.  If Hazel's eyes can use her new corneas until she is 4 or 5 years old than they will be a huge success...because that would mean she was able to see and process light into her eyes for the first four to five years.  More surgeries will be scary for Hazel as a child, but they will improve her life.

When will we know if the cornea transplant was a success?
There is not a solid timeframe when they will declare the transplant as a complete success.  It is truly a day-by-day situation.  So right now the transplant has been a big success but the tides can change in a hurry.  The new cornea could fail in 4 weeks, 4 months or 4 years.  We really don't know when but the new cornea will start to fail at some point.  We will have regular visits to see Dr. Bowman and check her eye condition.  Unless you hear otherwise, assume that everything is progressing the way it should be.

When will results be in for the detailed genetic testing?
The blood work for the detailed genetic testing was taken at the hospital when she was born and takes 4 to 6 weeks to come back.  Most newborns do not get genetic testing that is this detailed but Hazel's obviously has a unique scenario.  We don't know anything just yet...still have another one to three weeks to go.

When is Hazel's next hearing screening?
The next hearing screening is next week on November 13 at the same office where she had her initial visit.  However, Hazel responds to noises and voices at home even though she doesn't have the neck strength or control to whip her head around and see what's making noise.  So we have been feeling very optimistic about her hearing and are anxious to see more detailed results on how much she can hear.

Other than that Hazel is doing well overall and is sleeping and eating plenty.  Her schedule is a little out of whack but she is dealing with major eye surgery about as well as any infant can.  The next surgery is going to be December 13 at the same location as her first transplant. 

I know how much time and thought has gone into praying for Hazel and we really do feel like it made a world of difference.  We've been reading all the passages where the Lord has healed the blind and we are asking Him to do the same for our child. 

Thank you again to everyone who has been praying for Hazel. 

One Hurdle Down

(Please excuse any typos or grammatical mistakes on this post as I write from my phone and operate on limited sleep)

The first major hurdle is now behind us as we start the long journey that is Hazel's battle to save her vision.  Today was a good day, maybe even a great day. I suppose it makes sense to give a recap of the day chronologically...but I wanted the lead to be that the surgery was a success. 

We arrived at Cooks Children's Hospital around 5:30 am for check-in and all the fun pre-op procedures. We met her check-in nurse, anesthesiologist, chatted with Dr Bowman, a glaucoma specialist and Linda who is a nurse from Dr Bowman's office. And of course that was followed by questionnaires, changing Hazel into her white tee and socks, being weighed, measured and loaded on to a regular-size rolling bed at 7:15 am.  During our visit with Dr Bowman he was able to tell us more about the donated cornea for the surgery.  The donated cornea is 14 years old and had a cell count around 3,200 whereas 2,500 is closer to average.  The more cells the better. 

After the anesthetics took effect the evaluation of Hazel's eyes began. The doctors started by evaluating which eye appeared to be weaker, testing eye pressure and looking for signs of glaucoma.  Several family members and friends came to sit and talk with Aubree and I in the waiting room. It wasn't as tense as you would imagine...at this point we know the situation and feel prepared for any outcome. We know it's out of our control for the next few hours.  At 8:15 am we had a visit from Nurse Linda who delivers our first bit of good news. They did not find any sign of glaucoma and had chosen Hazel's right eye for surgery. The surgery was about to begin and would take about an hour. 

By 9:15 am Dr Bowman had come out and brought Aubree and I into a private room to discuss details.  The surgery went about as well as possible. After removing her cloudy cornea they were able to determine that Hazel does NOT have Peter's Anomaly.  Hazel actually has a less difficult ailment known as sclerocornea.  In any case, this accurate diagnosis is definitely a good thing and rules out the possibility of the larger-scale syndroms associated with Peter's Anomaly. Her treatment plan is still the same and a cornea transplant will happen on the other eye in December. Overall the surgery went as planned. The new cornea is in place with 16 sutures that are holding it in place. They also did NOT have to remove the lens from her eye, which is a HUGE positive. During the operation they also placed a time-release steroid in her eye which will aid in the transition and slowly release steroids into her eye over the next 7 to 8 months. As you would expect she also received antibiotics during the surgery in and around her eye.  The only negative from the surgery are the blood vessels in the corneas which increase the risk of rejection. 

In the hours following the surgery Hazel has been very sleepy and dazed because of the anesthesia.  Protocol is different for a 3 week old than most children on our floor. The only thing Hazel needs to do is sleep and eat if she can stay awake. She is pretty agitated when she is up unless she is warm and has her favorite pacifier.  It's kind of hard to imagine that we will repeat this process in a little over a month.

We are staying overnight at the hospital for observation and have a follow up appointment in the morning with Dr Bowman.  Other than that, only time will tell how well the transition will go for Hazel's new cornea.  

It was difficult wondering into the unknown today...but ultimately we are just grateful that God gave us a positive outcome. We will continue to give her all the care in the world and appreciate all the prayers and support.