We’re in the Emergency Room at Kosairs and they’re going to admit our little one for a viral infection. She’s running a pretty good fever and is very uncomfortable. Right now they don’t think it’s pneumonia but they want to observe her and rule out all possibilities. Say some prayers! We’ll keep you updated through this blog.

Kyle
Saturday evening:  Reese has a viral infection in her lungs.  They are monitoring her–she seems to be getting better.  We are praying that she gets to come home tomorrow (Sunday, July 22nd).  We truly appreciate everyone’s prayers.

Elizabeth

The first time I ever heard the phrase “medically fragile child” was during our second week long visit at the children’s hospital. One of the doctors said it and it stuck with me. We are now parents of a medically fragile child. What’s considered normal for our friends and their children is not normal for us. Their are rules and certain steps for Reese that we must take everyday to avoid another hospital visit. She has a team of doctors and nurses who know us and her by sight when we visit the hospital. She’s part of a program at the hospital for children who visit frequently at long periods of time and face complex medical care. We have a loud oxygen machine in the middle of our home with an oxygen hose long enough to stretch to any room in the house. We travel with oxygen tanks and they must be delivered and picked up every week. We have to take tanks to her babysitter and to the grandparents homes. When their is an air quality alert, we can’t even take her outside. We had a beach vacation planned at the start of the summer and we went without her because the doctors said she couldn’t travel through this summer.

Each morning and every night she gets a breathing treatment, we use a suction machine to take out any mucus in her nose and throat, she gets 3 to 4 medicines twice a day, and then we feed. She doesn’t eat like a normal baby, an 8 ounce bottle can sometimes take her up to an hour to eat. Each night we must put a pulse/oxygen machine on her with the loudest, most dream interrupting, sit straight out of bed, alarm on earth. It lets us know if her oxygen or pulse dips below safe levels established by her doctors. My wife and I have these routines down like a science, each of us has certain responsibilities and we work together like synchronized swimmers. It’s a 2 hour process each night just to get her to sleep and for us it’s the new normal.

A fever for Reese can mean many things and teething is the most normal one but any fever will give us serious pause. Last time she had pneumonia and was hospitalized, she was a happy normal baby all day long. Around 11:30 that night, she got fussy and wouldn’t go to bed or take milk. About 12:30am she felt warm and we took her temp and it was 99.8. At that time nothing to be alarmed over. 20 minutes later she had green stuff coming out of her nose and was moaning like she was in serious pain. She felt hotter and we took her temp again, it was 103.9!!!! We didn’t blink, it was to Kosairs immediately. When we walked in the ER, her pulse/oxygen machine alarm was going nuts, we were carrying her oxygen tank and she was moaning and coughing up nasty mucus all over my shirt. The lady at the desk took one look at us and motioned us to the nurse in triage within seconds. That nurse took less than 10 seconds to listen to her lungs and said “We’re going back, I’m guessing your part of Hearts and Hands?” That is the name for the special care group at the hospital that we’re part of that assist families of medically fragile children who visit often. When we walked to the back we had 2 nurses waiting for us and a doctor walked in right behind us. It was at that moment, I realized our journey as parents will never be the same, this is our life.

It’s so easy to worry about your first child, you worry about germs, you constantly call the pediatrician’s office, and your life revolves around your child’s day. This is normal for all first time parents. I feel like Elizabeth and I are on hyper worry when it comes to Reese’s care and at times we speak another language to each other. We discuss her heart rate, how many ounces, if she took this medicine, what her oxygen level is, what milestones she’s hit today, when her next appointment is, and if we can even take her outside. It wasn’t supposed to be like this. When my wife and I were born, we both walked by nine months! We were just sure when Reese was born she would follow in Mommy’s and Daddy’s footsteps, literally. Instead, we now get excited when her physical therapist tells us she sat up on her own longer than 2 minutes or our paediatrician tells us her lungs are clear and she gained weight this month. This is our life.

It was a Friday evening around 6:30 and I’ll never forget the phone ringing and the look on my wife’s face when she answered it. It was our neurologist and he should not have been calling us for any reason. He had received her genetic tests and they turned up a serious abnormality. She has what is called XQ28 duplication syndrome. This is a rare genetic disorder for children and even more rare for girls. We’ve been told she’s 1 of around 15 known girls who have the disorder. It’s more common in boys and they have it very severe with 100% of them having mental and phyisical disorders. The known girls is a range of issues but all still have mental and physical problems. What everyone seems to experience is low muscle tone, respiratory problems, eating issues, and an overall developmental delay. All of these we have experienced in abundance and will continue too.

One of the genes that is active in XQ28 chromosome is the MECP2 gene. This is the more popular name to call her disorder. In fact, when you google MECP2 syndrome, you get all kinds of information and almost 99% of it is about boys because it’s so rare in girls. The list for boys is very disheartening but I’ll put it below.

Characteristics of MECP2 duplication in affected boys:

*Hypotonia (low muscle tone)

*As a result of hypotonia, motor development including sitting, crawling, and walking is severely delayed or impaired

*Mental retardation (in 100%)

*Recurrent respiratory infections (in 75%)

*Epilepsy (in 50%)

*Constipation and/or reflux

*Limited or absent speech

*Autistic behaviors

*Ataxia

*Progressive spasticity (usually noticed in the legs more than the arms)

*Stereotyped movements of hands
*Teeth grinding

*Developmental regression occurs in some boys

We read all of this the night of the phone call and both of us just broke down. Why God? Why? My wife ate nothing but healthy food during her pregnancy, she walked 3 to 4 miles almost everyday, and in fact, the night her labor started she had just walked 4 miles in 90 degree weather! The birth was easy, she barely needed an epidural and pushed 3 times and Reese was out. We had a miscarriage over a year earlier and had planned on waiting a while longer to try again. God had other plans but this was the big plan? He gives us the most precious gift of all that we prayed over so many times and it’s a child with this?

Mental retardation? Possible Epilepsy? Another article we read said only 50% of boys live past the age of 25! Another study of 4 girls that have it had one dying before the age of 3! This is our future? A child who will never drive a car, fall in love, go to college, and maybe never even run into our arms and tell us she loves us. For months leading up to her birth I joked about buying guns to keep boys away from her, now she’ll never have a man to love her as a wife? I will never walk her down the isle and give her away, I’ll never take her shopping for her college dorm or to buy a prom dress. At no point in my life did I ever consider this as a possibility for my child. This was other people’s problems and I always smiled and said a silent prayer when I saw children with these issues. Now these issues are our life. Where do we go from here?

Continue and read Part 3 here

For months now, I’ve told myself it’s time to start a blog about our journey with our daughter Reese. The problem is, it’s very hard to put into words about how difficult it’s been for everyone involved. I know we have needed to put a blog together to inform our friends and family. So many people don’t understand the situation, her future, and our status. I hope I can clear up any confusion, questions, or concerns everyone may have.

Reese McClain Nichols was born on July 25th, 2011. Her birth was absolutely perfect and is chronicled here in a wonderful blog post by our friend Elizabeth Lauer. On the 2nd night at the hospital they took Reese back into the NICU because she was having trouble breathing. The official diagnosis was a bacterial infection of the lungs. She stayed another week in the NICU unit of the hospital till she was released home. Our first few weeks went fine but with every passing day we were presented with new challenges. She had severe re-flux to the point that she projectile vomited almost every time she ate.  We struggled to get her to grow and even maintain the required ounces of milk she needed each day. We had many tests ran, we switched multiple bottles, formulas, and pulled our hair out every single day as we prayed she would grow.

Finally, we took a beach vacation in October and she got sick on our way there. We had to give her pedialye and did not have a small enough nipple to feed her with. We purchased our fourth brand of bottle and she took the pedilyte very quickly. Elizabeth decided to try it with the milk and she sucked it down quicker than ever. By the time we got home from vacation she was taking more milk a day than ever before. We don’t know if it was the new bottle or maybe she just loved the healing qualities of a beach vacation but she turned a major corner that week and started growing and eating like a champ.

As the months progressed we still knew she wasn’t growing as well as she should but also noticed she wasn’t hitting other milestones. At her 6th month checkup our doctor recommended that she be referred to physical therapy. At that time she wasn’t reaching for things or even moving her eyes to track and responding to us. At physical therapy, they recommended we see a pediatric neurologist along with having her hearing and vision checked. She passed both tests with flying colors but we could not get an appointment with a neurologist until June! A week after this news Reese developed pneumonia and was admitted to Kosair Children’s Hospital. While there we were able to see one of the best neurologists in the country and we immediately felt like God was answering some serious prayers. They ran all kinds of tests and discovered that she had a thinning of what’s called the corpus callosum in her brain. It’s basically the highway that connects your left and right side of your brain. She was also diagnosed with severe hypotonia, which is bascially low muscle tone.

Even today, as I write this she is 11 months of age and still cannot sit up or crawl. While on her first hospital visit, the doctors ran some genetic tests that they stated were just routine to rule anything out. In between our 1st and 2nd visit to the hospital we got the call that would change our lives forever.

Continue and Read Part 2 here