The Road to St. Louis

Submitted by Nicole on 10/12/12

 As we drove past the Bush Baseball Stadium in St. Louis, Missouri, Sunday afternoon I recalled the time I attended a game on one Sunday in early September—Wrenn’s worst day—the day she had oxygen saturations in the 30’s and 40’s—the day she was put on the paralytic (two weeks prior to receiving her new lungs).  It was a day where the sibling playroom at Children’s was closed because of a holiday weekend and Tanner had to stay with us in the NICU—a task he hated and made sure everybody around the vicinity knew it. How did we deal with a sibling who was miserable and another one who was near death?  We made the difficult decision to walk away from the hospital for a few hours and let the people we had entrusted with Wrenn to do their jobs.

I looked at Dr. Hoft with tears in my eyes and said, “I don’t know what to do—please tell me what I should do.”  Tanner was screaming and throwing a fit and we were literally all sitting on the floor in the hallway of the NICU because Wrenn couldn’t stand any noise or people in her room.  Moments prior to speaking with Dr. Hoft I had entered Wrenn’s room and let her know that mommy was there and held her hand; her saturations dove from 60 to 30 FAST.  I wasn’t helping and I couldn’t bear to be the cause of her decline.  Holding Wrenn’s hand and being with her wasn’t even an option at that point.  I was completely helpless and it was the worst feeling in the world.

Dr. Hoft looked at me and replied, “If you can’t stand to watch her take however long to come back up to normal saturations, don’t stay.  You can call us as much as you want to check on her.  It’s okay to go—nobody’s going to judge you.  Whatever keeps you calm and sane right now is what you need to do.” 

Feeling empty and sad I nodded and decided to I had to do what was best at the moment.  So I did.  We did.  We left.  Nothing at that moment felt right or wrong—the sensation—I was numb.

The Ronald McDonald house had given us advance tickets to a Cardinals baseball game and we had planned on going that afternoon as long as Wrenn was doing okay.  I didn’t realize I’d be going as a zombie.  I don’t recall the team they played, or who won or lost.  My body was there but my mind was with Wrenn.  It was almost as if I were in two places at the same time.  It was the best I could do at the moment—praying, hoping and believing that it would all be okay.  We didn’t get to the game on time and we didn’t stay to the end.  It was about having something to keep my mind off of watching a monitor for those unknown hours—where we went really didn’t matter.

A couple of hours later we walked back into the NICU and Wrenn was sating at 100 percent. Perfect.  She wasn’t able to move now because of the paralytic but she was stable, breathing steady and pink again—no longer a Smurf! Several doctors and nurses had painfully watched that monitor over the course of those four hours—praying, hoping, believing she’d come back up and finally she did—VERY SLOWLY and I was told the emotion in the room was very somber, very silent and very tense— I felt I had made the right decision leaving.

The next day they performed a brain scan to make sure Wrenn didn’t have any bleed outs from losing so much oxygen to the brain.  Things were so close to going so bad and every day I felt my chest getting tighter and tighter as we waited for the perfect lungs. I don’t know how she passed that test but she was still perfect, I thought, I hoped, I prayed.  Only time would tell and it would be many months later when we’d know for sure if there was damage from those crashes in oxygen to her brain.

A few days ago, on Sunday afternoon, we drove by our old Loft’s in the city and I thought of all the trips in and out of the parking garage we took to the hospital—mostly good visits.  I like to think now when we return to St. Louis we’re just visiting our extended family of specialists—the medical team who helped all of us hold it together and who gave my daughter a fighting chance.

Reflecting is something I like to do to remind myself that merely one day of celebration isn’t really what it’s all about.  I prefer to eat a little piece of cake 364 days a year.  Everyday there’s a wonderful reason to celebrate Wrenn’s life. A reminder that we’re all given a gift each day we breathe in air.

This check-up was Wrenn’s TWO year post-transplant visit.  Two years ago, on that one horrible day, I never knew I’d be smiling on the way home from such a great visit with the transplant team.  As a positive person I had hoped to see only good things for Wrenn’s future but we were prepped early on to realize that it’s all a gamble.  I’m glad Lady Luck’s on our side and I truly hope she never leaves.

Monday morning in St. Louis was a busy day for Wrenn.  The lab tech took blood from three veins and filled more tubes than I’d ever seen in history—even the lab tech said Wrenn was getting a lot of tests done at one time. I knew that Wrenn looked great, had been acting great, sounded great lung wise and appeared to be super in every aspect of her being, but still, I worry the rug might be pulled from underneath of us at any given moment.

We had our first appointment with GI.  They were very happy that Wrenn had gained three pounds since April.  They asked me to submit a diary of her food and drink intake to make sure she’s getting enough calories to thrive and/or maintain her weight.  They were fine that we quit the g tube feeds and said as long as Wrenn holds her own, we can continue on this natural path with her meals. 

The CDC doctor asked a lot of questions about her shots and our schedule for the next six months with immunizations.  She will analyze test results and will work along the same lines as our immunologist in balancing Wrenn’s suppression and immune system.  She clarified a few things for us.  She said that they feel pretty confident that this year’s flu vaccination is good and should cover many strains of the flu.  She said that the ONLY thing that keeps Wrenn protected from getting the flu is a mask—but in other words, when it’s not flu season, (like in the summer months) a mask is pretty useless—germs in general are passed via touch and so good hand washing and sanitizer is our best friend!  We have a few additional vaccinations to get in the next three months and they will then test her antibodies to make sure she’s building up immunity to them.

The Immunologist said that Wrenn’s levels were 404—which is GREAT!  Wrenn is making her own anti-bodies from the shots (vaccinations) she receives and since she’s not been sick since before transplant (knock on wood)—she doesn’t need IVIG (which is a monthly infusion, takes about 2-4 hours to administer and is just a royal pain in the butt).  We will check her levels at our next visit to St. Louis in April.

The pulmonary team said Wrenn looked and sounded great!  They said all her cancer tests and levels looked wonderful.  Her VQ tests were spot on and so Wrenn didn’t need a bronc for this visit.  They all noticed she was very busy, talkative, bossy, and a climber—everybody cringed when she jumped off the chairs.

Wrenn’s Eco and CT scan looked great and so no further exams/tests were needed.

This was the BEST visit a transplant kid could ask for.  Leaving after a one day visit!!!  AMEN!

One of the things they are going to do is follow up with a few more stool tests for fat content and parasites—mostly to cover everything that could be causing Wrenn’s multiple diapers.  They said that because she eats so healthy the food could be the cause and as she gets older her body will adjust to control that better—this would be the best outcome, meaning no problems just mother nature figuring it all out.  I don’t feel the doctors think we will find an underlying problem/issue but they are checking all the tests from their list to make sure they cross everything off their list.

We were able to visit the NICU and nurse Beth—she had taken care of Wrenn a lot while we waited on lungs.  Dr. Barb from the NICU popped in to clinic for a while and we also visited with Dr. Hamvas, our genetic scientist who continues to research lungs and stem cell growth, which is one of his biggest projects. 

Tanner had a great time at the Sibling Playroom with his buddy Mr. Pat—who looked worn out from the day when I picked Tanner up at 6:15PM. We love seeing our Playroom family because they were such a big part of Tanner’s world while we were in St. Louis—and the fact that Tanner enjoys himself when he visits—proves that they did an amazing job.  Love you Pat and Rita!

Therapy for Wrenn was a little different this visit.  Rebecca, Wrenn’s ongoing therapist since the NICU, had Wrenn walk in a circle and monitored the oxygen saturations.  Wrenn did a great job and walked for six minutes, which the therapist said was amazing—most kids get bored walking in a circle but not my kid—she’s the little energizer bunny! She proudly wore her mask, held her purse in her hand and showed off her new Minnie Mouse boots while showing off those perfect oxygen saturations.

Rebecca had Wrenn climb up on the ropes, jump on the trampoline, slide down slides, show off her words and colors, toss the ball around, eat and drink while walking and laughing and skip to the next station as she sipped from a tea cup.   Developmentally, Wrenn is ahead and thriving.  Something I never ever thought would be possible on that one dark day when I attended a baseball game two years ago.  Oh, what joy it brings me to witness a daily miracle.  I always say we’re both cursed and blessed.  As long as we have both—I can’t really complain!

I love hearing that the continued prayers are still out there for our family and especially Tanner and Wrenn.  There’s a reason she’s doing so well and I have to accredit everybody for their faith—you have no idea what it means to our family and I’m so glad I’m able to share her progress with you all!

Xoxo nicole

So Long September

Submitted by Nicole on 9/28/2012

Fragile

Labor Day weekend I was quickly reminded how fragile our lives are with Wrenn’s condition.  The reality: In the blink of an eye, things can go from perfect to tragic at any given moment.

It all started at our GI appointment in June. The doctor looked at her growth chart and decided that Wrenn (at 19 pounds and 26 months old) needed more calories to gain weight. I admit, she’s not a great eater and won’t drink a ton at one sitting but I don’t know many toddlers who eat that great in general.  Nutrition is important for Wrenn so she can grow with those large new lungs she received two years ago. (September 23^nd)  So I do take her GI appointments very seriously.

However, sometimes after trying what the GI doctor says, life becomes more miserable for everybody involved.  He wanted Wrenn to be fed for eight hours straight while she slept (continuous feeds) and then three additional feeds during the day.  What I found wasn’t surprising; she stopped eating solid foods during the day and at night, and woke up every hour because she was miserable.    

At our next follow-up appointment in July we were told that Wrenn had gained one pound from all of this, which I felt was great, but the GI doctor got out her growth chart again and compared her to everybody else.  He told me she needed higher amounts at night and increased her volume of formula during the day as well. Wrenn didn’t agree with either plan and so neither did Mommy. After trying this for two weeks, I cancelled our next follow-up appointment and regrouped.

Three weeks ago, I pulled the plug.  I decided to stop all G tube feeds during the day and see how much she’d eat and drink on her own.  I did keep the eight hours of feeds going at night to make up for any lost calories during the day.  She ate and drank a little more during the day but it wasn’t the result I wanted.  So two weeks later I quit the night feeds as well.  Wrenn’s therapist had said that night feeds can greatly affect their appetite during the day and so I went all the way with my new plan.  I decided Wrenn was capable in chewing and drinking—the two things she’d never experienced on her own was getting hungry, and satisfying that feeling to becoming full.  I felt it was time she learned this natural course of events.

The second problem we were having was that her labs in June showed Wrenn had very low magnesium levels and iron.  The GI doctor said she was anemic.  What I realized right away was that her new formula (not soy anymore since she’s lactose intolerant) contained a ton of iron, where her new formula (Neocate Jr.)—(the stuff I just quit) barely contained any iron.  The GI doctor tested Wrenn’s stools and one out of three came back positive for containing blood. (Sometimes you can’t see this and a test is the only way to know for sure). This wasn’t a good thing and so he introduced iron supplementation into her diet once daily.     

Of course, we don’t do anything without first contacting St. Louis to make sure the new plan works well with her rejection medications.  After a few weeks, everybody was on the same page and we went with the new program for the iron supplementation.

At first I was a bit freaked when Wrenn barely ate or drank without those tube feeds but within three days she showed more interest and enjoyed a few midnight snacks.  I counted the number of wet diapers and noticed her stools had changed form but still all over the map as far as frequency and consistency. 

Feeding time has always been the most challenging part with a kid who had to learn to eat and drink at ten months old.  It’s a long, frustrating process and it’s very important that the patient, the doctor and parent be on the exact same page.  This isn’t an area that St. Louis gets too involved in—they leave it up to us and our local GI doctor to figure it out.

I decided in the long run that it was worth Wrenn losing a pound or two for that first month or two to stick with the new program and see if she starts to eat bigger meals and more often.  So far she’s not lost any weight and seems to be much happier and less gassy without all the formula.  She’s sleeping through the night too, which hasn’t happened since we lived in St. Louis.

When Wrenn was having issues with her prolapsed rectum we watched her diapers very closely.  It’s not normal for toddlers to have 10 to 12 diapers a day and that was Wrenn’s norm for a long time.  Once she was on the Flagyl, we went down to three diapers daily and it all seemed better.  The GI doctor told me that when she starts having gas pain and several diapers to call him so we can have her come in or do the Flagyl again. 

Here’s the problem—long term use of Flagyl causes neurological issues. So it’s very important that I know for sure that she’s in need of this drug.  Last Thursday and Friday she was up to 12 diapers a day and there was no rhyme or reason but she was on the 7^th day of eating at her own pace with my new agenda.  I was getting worried and on Saturday morning called the GI doctor on call and asked for Flagyl to be called in to the pharmacy.  This doctor said something to me that I had thought in the back of my mind but had kept in the back of my mind for obvious reasons.  He said, if Wrenn’s in need of Flagyl every couple of weeks, which isn’t the norm, she probably has an underlying issue that is masked by the drug we’re giving her.  The scary word came up and it made me realize how fragile our lives are with Wrenn’s condition. 

“I have seen this many times, parents think their kid is lactose intolerant and has a GI bacterial build up and what they actually have is an intestinal cancer—which is often caused by the two drugs she takes for rejection called Cellcept and Prograf.”

My heart stopped beating.  The doctor then asked me if the transplant team tested for cancers post transplant and I said yes but wasn’t sure if they tested for the one to which he was referring.  You can imagine my anxiety that this question was hovering over me on a holiday weekend. It was going to be a long three days until Tuesday morning when I’d be able to get any answers from the transplant team.

My family and close friends were livid that the doctor on call (a doctor in the practice who had never seen Wrenn as his own patient) suggested this to me on a Saturday morning but I wasn’t mad at him at all.  When we signed on for Wrenn’s transplant we went over a thick booklet with our transplant coordinator stating every single thing that can go wrong with Wrenn post transplant.  We have heard it all and it just depends what path Wrenn is going to take.  Cancer was something I knew could happen but generally it happens with LONG term usage.  The odds are this wasn’t Wrenn’s GI issue but again, every kid is different and at that very moment in time, I didn’t have the answers to this doctor's questions. 

I’m almost certain my daughter was on his mind for much of the weekend. It’s not every day you have a double lung transplant patient at her tender age and it’s a call you don’t forget.  He was very eager to call the transplant team and did so first thing Tuesday morning.  

Unable to think about anything else that morning I got in touch with a few transplant mommies and they weren’t sure either.  This is the moment that you recall what your coordinator tells you: Worry about what is, NOT what might be.  I chanted that all day to myself.  Feeling the need to do something I sat down and wrote a very detailed email to my transplant coordinator, Laura.  I knew it was a holiday but prayed she’d see the panic in my words and reply, giving me something positive thoughts to get through the weekend.  After sending that email I hugged Tanner and Wrenn and realized how fast it could all go downhill. I spent the rest of the day not leaving their sides.  This was all part of the rollercoaster ride I agreed to and I was doing it without a seat belt. 

Even though my mind raced with good and bad thoughts I was on edge and the day for all of us was somber. Wrenn looked so good and although I couldn’t imagine this being a possibility, I couldn’t be certain.  It was an exhausting day mentally and I was tired so I turned in early and when I woke up on Sunday morning I had an email from the transplant coordinator in my mailbox.  I read the email to Jason and I again started to breathe without telling myself to do so.  The test the GI doctor on call suggested had always been negative for Wrenn and Laura said that Wrenn, in their eyes, has been great post transplant.  It was reassurance I needed and on Monday she confirmed this with the GI doctor.  All cancer tests so far have come back negative! J

It amazes me how level I remain when faced with things that are so out of my control.   I was so thankful my transplant coordinator wrote me back so soon and was so grateful for her compassion concerning my fears.  It is out of character for the team to write during a weekend and well, it just shows we have good people around us who care about our family and especially baby Wrenn.

It’s such a life lesson sometimes to experience the reality check like I did.  It’s been such a wonderful summer and after a while you forget what you’ve been through and then something reminds you that life can turn upside down in the blink of an eye.  Life really is a gift and I feel God reminds me often and I am thankful for that. 

We head to St. Louis for Wrenn’s two year checkup on Monday (today). This will be the first appointment where a bronc is NOT required but is scheduled just in case.  Our day on Monday is packed—labs, immunology doctor, Infectious disease doctor (this is new to all transplant recipients), pulmonary doctor, transplant gastro doctor, then after lunch she will be put under and have her PFT’s done (infant pulmonary tests to make sure both lungs are doing well), cardio station, x-rays and CT scans.   Our day goes from 7AM to 5:30PM. 

It’s scary knowing we don’t have a bronc, because we leave wondering if her lungs are rejecting but unless they take a biopsy of the specific spot that is rejecting, the data is skewed anyway.  It’s nice knowing she might not have to have one and put her through the risks of infections or additional complications.

On a great note, we saw the pulmonary doctor at VCU in early August and he was very pleased with her and so we won’t see him again until January. 

June, July and August are the best months for our family and we feel the most normal as we blend in with the world—going out in public and doing fun things like Busch Gardens, going to dinner, playing in the pools at the water park, Water Country USA, shopping with Mommy and playing outside in general.  We rarely mask Wrenn in the summer because it’s not a high risk time for her but we are always aware of good hand washing and use bottles and bottles of hand sanitizer.  We change out Wrenn and Tanner’s clothes if they’ve been playing in a germy area like a park or with other kids (which isn’t very often) and they take showers right away.

As soon as September 1^st arrives we are on high alert.  We’ll still go out to eat but will eat outside when possible and at odd hours of the week like 4PM, not 6PM.  We won’t visit too many public places and play dates will be less often.  Wrenn and Tanner received their flu shots in mid-September and we will get our shots in early October. Wrenn gets a booster in early January.

This summer Tanner took a hip hop class and I explained to all the parents our situation with Wrenn and asked as I handed out my phone number, to let me know if their child went home sick, or had been sick as a courtesy for keeping Tanner and Wrenn safe in our crazy world of germs.  Most people, I should say, ALL people we explain our situation to are compassionate with what we have to go through and have been wonderful in communicating. Obviously, summer isn’t high risk but as we move forward this will be a common speech as Tanner explores his own world with other children. Luckily, he won’t start school until next fall.  That’s a whole new blog J

Wrenn is a smart little cookie. She loves to finish off Tanner’s food. She loves fried eggs with buttered toast. She loves all fruit and salad and still prefers water to anything else for liquid. She loves shoes, hats and purses and is very into her baby dolls right now. She is a climber and wants to be around me at all times of the day, helping me cook, clean, eat, watch TV: she is my little clone. 

Tanner and Wrenn are so close and play well together.  When she takes a nap he wakes her up so they can keep playing (Grrrrr!).  They wrestle, play the Wii together, dance, sing, chase one another around the house, jump on the bed, giggle in the backseat of the car and fight over TV shows.  They melt my heart every day and I am soooooo lucky that I get to watch them as they continue to grow close. 

There was one day in June I didn’t have a sitter available and I had to take Tanner with me to get Wrenn’s labs drawn.  He was sitting in the seat next to Wrenn and the two nurses tried to cover Wrenn so he wouldn’t see but he heard her cry and knew something wasn’t much fun for his little sister.  It was the time they had a hard time drawing blood and so that made it even worse for all of us.  When she was all done he came over to Wrenn and said, “Are you okay Wrenn?”  And he rubbed her head. Clearly, he was in tune to her pain and it put tears in my eyes.  Now when I mention I have to take Wrenn in for monthly labs he says, ‘That hurts Wrenn.”  SIGH!!!

Although, we’ve been lucky.  Wrenn usually needs labs drawn once a month and they are so good at this lab we go to that it’s just one prick and she’s done.  She doesn’t even cry anymore.  She spends so much time in the doctor offices that her favorite toy to pass along the time is playing with the latex gloves.  So when we finish drawing blood she asked for a glove as her reward.  The lab said they could only give her a stuffed toy, which she threw on the floor because she preferred the glove! LOL

Wrenn’s two year re-birth (when she received her new lungs from that wonderful donor) was September 23^rd.  It is a bitter sweet day for our family and I’m sure still is for all those who were along for our journey as we waited daily for that one phone call.  We don’t throw a party on the anniversary of her surgery. In many ways I feel this is a day to reflect, thank God for her good health, and remember and thank our donor even if it’s through prayers.  I also feel in many ways that celebrating will jinx her good fortune and since I’m very superstitious, I prefer to celebrate quietly.  

I greatly appreciate the emails we receive from our cyber family. You guys are the heart and soul of Wrenn’s success and give our family the strength to get through the rough days.  I am so happy to say that things are going well for Wrenn and as we gear up for her major check up Monday in St. Louis, I hope to give you great news.

Xoxo Nicole

Seriously, God, I Have My Limits

Sent in by Nicole 5/17/2012

“SERIOUSLY, God, I have my limits,” I mumbled under my breath.   I looked up at Jason in the Emergency Room as Wrenn screamed her head off while being tortured by the ER team.  They struggled to find a vein for an IV.  “I need those guardian angels back because I can’t watch this!”  The second team came in (veterans, they called themselves) and I hid my head as they tried a fifth and sixth attempt on her little ankles.  I looked up—NOTHING!!! I was so angry and just tried to hold my composure as I again looked away and caressed Wrenn’s sweaty little head.  Her veins were dry and the only liquid draining from anywhere in the room was from my eyes—I finally ended the torture.  

AS I write my blog entry I inhale deeply while thinking about the last six months.  A sigh of relief, finally, but I still feel the tension in my shoulders from all the events that took place.  When I signed up for Wrenn’s double lung transplant, I knew we’d be exchanging one set of problems for new ones but I’m the type of person where I do well if I know what I’m dealing with—then I can battle forward and ultimately conquer any obstacle in my way.  For the last two years, on more than one occasion, Wrenn has been deemed a ‘mystery’ child with a couple of curves that were thrown her way. I’ve had doctors in meetings throw up their arms and say, “I’m just damn scared of the girl.  I don’t know what to do.”   I’ve seen gray hairs appear before my eyes on some of medicine's finest.   I pray that we’re done with the mysteries for a while.

WHEN Wrenn was born she breathed REALLY fast.  She was on 8 liters of oxygen and she was fed a thick mixture of breast milk and milk proteins to help plump her up, so that the transplant team in St. Louis would accept her into the transplant program.  What I can tell you is that her system (Gastro) was a MESS back then.  Her digestive system was working just as hard as her lungs and everything in the body had a cause and effect, especially when organs are working really hard or not in sync.  Her body back then was on Fight or Flight and it reacted to keep her alive.   

ONCE Wrenn arrived in St. Louis in July 2010, her heart and lungs weren’t working quite as hard thanks to sedation and intubation but her digestive system struggled.  Wrenn had loose stools, daily vomiting, minimal weight gain— the list goes on. All signs of a very sick child in desperate need of a double lung transplant. 

EVEN though Wrenn received her transplant in September, her GI issues continued; Bloating, loose watery stools, cramping, discomfort with gas and frequent bowl movements confused doctors and baffled nurses.  To most, it was an easy diagnosis; every medication she took had terrible side effects; cancer, rash, loose stools, frequent stools, cramping, nausea, itching, sun sensitivity, stunted growth—the list went on and on to the point of us NOT wanting to finish reading the risks for fear of what COULD happen with long term use of the rejection drugs. 

IN December of 2010, Wrenn appeared stable when she came home but feeds were always a struggle with the G-Tube.  It was a guessing game for us to know how much was enough, how often, how to introduce food and when?  What would irritate her, would she choke while trying food or worse, aspirate into her new lungs?  It was a process where walking on egg shells became a daily routine. 

GI wise, Wrenn had good and bad days but the loose stools were always a part of our daily life.  We chalked them up to the Milk of Magnesia she took 3 times a day to replace the magnesium the rejection medications depleted in her body.  She had some solid stools, but frequent ones for sure and her weight was at a plateau, which wasn’t good for her new lungs, which needed good nutrition to keep them healthy and strong.  To give you perspective on how rejection drugs affect a baby/toddler—Wrenn is the size of a 14 month old baby, even though she is 25 months old.  She is STILL wearing size 3 pampers (she came home from the hospital wearing the same size over a year ago) and as of 10 days ago, only weighs roughly 18 pounds. 

ONCE we moved back to Virginia things were stable and OKAY for Wrenn standards.  She hadn’t gotten sick, wasn’t constipated (that’s for sure), and we were in what we thought was a GOOD place. HAHA!  Never blink when it’s Wrenn.

ALMOST acutely, back in early February, Wrenn started having painful bowl movements, gas and diarrhea (yep, worse than before) and so I thought she had an intestinal bug.  I waited eight days and she didn’t seem to have fewer diaper changes.  I waited two more days and called the transplant team.  They ordered a ton of stool tests to be done to see what type of infection she was fighting.  Two weeks later her urine and stool tests came back negative.  She was perfect according to the tests, but still not doing well.  I tried for the next three months to take away foods that might have been the problem like milk, cheese, wheat, fruit, etc.  But nothing worked.  Then I thought that maybe she was getting too much liquid and her body was rejecting it (the colon wasn’t able to hold in the nutrition), but that wasn’t helping either and only made her lose weight for the lack of feeds during the day).

AFTER two more months of this nightmare routine of trying new things and taking things away, Wrenn was worse with nightly crying episodes of pain and struggling to let out gas.  The lack of sleep made both of us very cranky and I was very frustrated.  The ONLY conclusion I could come up with was that her antibiotic was bothering her.  She takes this broad spectrum antibiotic for life, Monday, Wednesday and Friday to ward off common bacterial issues like ear infections etc.  It’s a preventative measure and a MUST for her survival.  I knew that antibiotics were hard on the digestive system (they were on Tanner) and it didn’t help that she HATED yogurt.  In fact, dairy products only made her diapers worse.    

I wasn’t sure how but I knew her gut was done as far as good bacteria.  What I didn’t realize at the time was that I needed new eyes to help solve her issue.  I accept that sometimes it’s best to hit rock bottom so you can float back up to the top.  I feel like this is learning the hard way but sometimes it’s the ONLY way.  As long as we get to a better place, it’s all worth it.

HOW we finally reached rock bottom:  In early March Wrenn had two terrible nights of screaming episodes and we did everything possible to make her better but nothing worked.  Finally, Jason gave up sleeping and brought her downstairs.  He fed her some soy milk, hoping she might be happier with a little food in her belly and to help push out some trapped gas since her pain seemed to be in the lower intestines.  Well, it worked!  After that huge bowl movement, he came up stairs with Wrenn in his arms, turned on the light and said, “I think we need to take Wrenn to the pediatrician.”  I looked at him with tired eyes and assumed her diaper rash had gotten worse but what I saw was even more horrible than some minor irritation.  I glanced down at Wenn’s bare bottom and saw that a big red, blooming flower had erupted from her tushie.  “No,” I said, catching my breath and jumping out of bed like a crazy person, “get her dressed; we’re going to the ER!” 

MY first instinct was that she had pushed out her intestines, which I’d never seen in real life outside of a body.  Needless to say, I was freaking out! I didn’t even know where to take her since we’d not been in Virginia long enough to prepare for an emergency type situation.

IT was a crazy 20 minutes.  My cell phone was dead, Jason’s phone was missing at the moment, Tanner was asleep, and neither of our parents would answer their cell phones so we could tell them what was going on.  I finally found a cord, plugged in my phone and called the pediatrician, who told us to go straight to MCV/VCU Children’s ER.  They told us she’d be more comfortable in an ambulance and to keep the area damp.  (The ambulance was 5 minutes away but took 30 minutes to get to our house—still trying to figure that one out!)

MY imagination went to the worst places—when you live in a Children’s Hospital for a year you fear a LOT more.   I kept thinking surgery, possible infections; long term issues that would make her need more surgeries and constant pain without a cure.  I couldn’t stop worrying as I followed the ambulance to the hospital.   Once we arrived they took us back to a private room. 

WE described what had happened to the ER doctor and he kept asking if Wrenn had ever been tested for CF (Cystic Fibrosis).  I immediately assumed that CF was among the first test they had done when she was born with all her respiratory issues but they told me that a blood test isn’t as good and ONLY a sweat test is 100% accurate when testing for CF (a sweat test can’t be done in the NICU, at least it couldn’t be done for Wrenn in the shape she was in).  I started texting my friends at Winnie Palmer and told them what was going on; their support for our family continues to amaze me daily… hugs to them for being there for us through all of Wrenn’s ups and downs.

FINALLY, an hour later, the doctor was ready for the diaper to come off—apparently, the prolapsed rectum had gone back inside.  This was both good and bad.  Bad, because once it happens, there’s a better chance of it happening again and again.  Good, because she was free to go home; but what do we do if it happens again?  UGH!  The doctor told us if it doesn’t go back in within an hour to come back to the ER. Well, this sucks!  Hospitals aren’t a great place for Wrenn with her immune suppression.  She’s going to end up catching something and then to drop our lives every time this happens and rush to the ER isn’t an option.   So far we had more questions than answers.  They referred us to a very reputable GI specialist from MCV/VCU named Dr. Ted Williams. The appointment was set for that following Monday (3 days later).

WE consulted with Dr. Williams who ordered a ton of blood work to rule out allergens, Silica’s disease, immune tests, stool tests and anything else that hadn’t already been done.  He changed her intake on formula and stated his concern for her weight, which has always been a struggle.  I told him I didn’t think it was allergy related although I do see a difference with milk products. 

WRENN did okay for four weeks past that appointment but then, Sunday prior to leaving for St. Louis (mid-April) she had another incident, but luckily the prolapsed rectum went back in—no ER visit necessary.  Then the following Thursday it happened again twice and we couldn’t get it back in (April 19^th).  We again had to take her to the ER and this time, she was so irritated that the ER team couldn’t get it back in either.  So, we had to have her wait for GI surgery to take a look.  Finally they decided to admit her to Peds and it wasn’t until 12 hours later that they got the prolapsed rectum back in. They had given her an IV and morphine to help; otherwise we’d still be there waiting.  The surgeons gave us a lesson on how to put it back in, but let’s face it, this isn’t the entire issue and how in the world was I ever going to have any one else watch her if this kept happening?  This is our child and our responsibility, if it means putting in rectums, then that’s what we’re going to do, but how dedicated is the rest of our family?  L  

MCV/VCU held Wrenn overnight for observation and taped her tushie shut to help with gravity.  They didn’t continue her feeds until the next morning (Saturday).  The doctors at MCV again said we needed to have Wrenn tested for CF, just to rule out Cystic Fibrosis.  I tried to wrap my head around how my child could possibly be cursed with not only the 4^th diagnosed case in the world of a rare lung disease with a double mutation for ABACA3 but then, how could she possibly have CF on top of that?  WHAT A NIGHTMARE I was living at this moment!    

WE left the hospital around 2PM Saturday— unpacked from the hospital stay and then repacked our bags so we could head to St. Louis for Wrenn's 6 month check-up.   The 16 hour drive gave me plenty of time to rethink the last 3 days and I was still in a sheer state of panic.  What if this doesn’t stop happening? What if this is her life of being in constant pain and hating food and losing more weight?  The lungs seem perfect but the rest of her body is taking a beating.  I was so upset and all I could do is worry. 

DESPITE my anxiety the kids were great on the long trip.  The only incident we had was Wrenn’s G tube broke inside of her in Kentucky (luckily it wasn’t in West Virginia, where it happened before and added an extra 3.5 hours to our trip when we visited an ER).  I had a spare G-Tube, which wasn’t the right size but was perfect for a temporary fix.    

AROUND 6pm in St. Louis we had dinner and checked in to the hotel.   Wrenn was on limited food and water for the next two days, which wasn’t going to help her weight loss but we didn’t have a choice.  On Monday morning she had labs.  Sadly she was so dry they couldn’t get a vein or blood out of her at all.  It was a nightmare.  A true sign of all she’d been through and what we’d witnessed in the ER.  I wasn’t looking forward to the multiple sticks and two IV’s that were necessary for her procedures both days. 

NEXT we then headed to Immunology to see Dr. Ellis, who told us great news.  They had checked her levels and said that Wrenn’s body had more than doubled in building up her own immunity since last October.  We had stopped all the IVIG infusions throughout the winter, which meant less risk for infections and less doctor office visits. (Yeah)  This was great news and meant that Wrenn’s body was making its own antibodies and that she was thriving.  It certainly helped that she hadn’t gotten sick during flu season (knock on wood).  Dr. Ellis talked about Tanner going to school but it’s a conversation that will take place a hundred more times before he actually goes, meaning,  I just can’t say where—when—for sure.  We have to take our lives day by day and see how things are going to make such a big decision that affects our entire family. 

TRANSPLANT clinic started about 10:30am.  Both the doctor and coordinator said Wrenn looked amazing.  They were happy with the IGG numbers (From Dr. Ellis) and minus the prolapsed rectum incidents, said that Wrenn looked like a normal little girl.  Since they are a lung transplant program, (work above the waist) they wanted the GI specialist in Virginia to continue working with us on her GI issues.  I was sad that we weren’t going to get answers in St. Louis but would know all the results pulmonary-wise by Wednesday afternoon.

WRENN was put under for all her infant lung tests around 11:00am.  She came out not needing any oxygen and the results on her tests were great!  Both lungs were doing equal work and sounded great, looked great and worked great—all great news. J

AROUND 5PM that evening we met Wrenn’s old therapists from St. Louis (Kayla and Julie) for dinner at our favorite (well, Wrenn’s favorite Italian Restaurant) Zia’s.   It was so great seeing them and they loved visiting with Wrenn, who loved showing off and made them both smile.  

THE next morning we were up bright and early for Wrenn’s bronc.  Around 9AM they took her back to the OR and within an hour she was in recovery, snuggling with her puppy and blanket.  She again did great and didn’t need any oxygen post procedure.  We just had to wait for the biopsies to come back (24 hours) before heading back to Virginia. 

THAT evening we had dinner with one of Wrenn’s nurses from the NICU (Aunt Beth) and Jason’s former boss, Billy.  It was nice catching up, exchanging photos and celebrating great news with people we had shared so much with over the year and a half we had lived there.  Both Beth and Billy had seen Wrenn at her worst.  It’s great to show how she’s beating the odds so far and that God is keeping her healthy and well. 

IN between all the great news Wrenn had another prolapsed rectum.  The doctor on call told us to head to the ER and so we did.  When we arrived we checked her diaper and saw that it had gone back in. We returned to the hotel room and sat on pins and needles, worried about the long drive ahead as her GI issues continued.

WEDNESDAY morning we went to Wrenn’s last appointment at Children’s which was with her long time therapist from the NICU (Rebecca).  Rebecca evaluates Wrenn every six months to see if she needs more or less sessions (right now Wrenn has speech once a week in Virginia) and this is a nice way to make sure Wrenn’s on track developmentally.  Rebecca was amazed at how active Wrenn was.  She watched her climb up and down slides, walk over uneven surfaces, hop up onto the trampoline and jump, step down and chase and throw a ball across the room, pick out heavy toys and carry them to her own spot, giggle, smile, interact, sign, and act like a totally normal child despite all she’s been through.  Needless to say, Wrenn got straight A’s on this visit and our family couldn’t be more thankful. 

THE very next morning we got the thumbs up from the transplant team that Wrenn’s biopsies had all come back negative, NO REJECTION J  With this news we were free to head home.   

OVER the next 15 hours (in the car and the hotel we stopped at in West Virginia) Wrenn had two more prolapsed rectums that followed painful screaming episodes.  The very hour we arrived home in Virginia, we again headed to MCV/VCU ER.  (It was a week ago exactly that the exact same ER team had helped us before).  They were sad to see us back!

WHEN we arrived at the ER at MCV they wanted to put an IV in Wrenn so they could give medications and be admitted to Peds for possible surgery to fix her habitual problem.  The problem was Wrenn was so dry from the days in St. Louis from having missed feeds that the ER couldn’t find a vein again.  Jason and I held Wrenn down while they tried in each arm, wrist and then each leg.  It was painful to watch and I had tears coming from my eyes.  Finally I said, no more.  I told them to give her morphine through her g tube and end this madness!  Within minutes they had her relaxed and the surgical doctor came in and pushed the rectum back in. 

(I want to note that almost 99% of all transplant patients, especially kids, have GI issues.  These are very common issues for the most part because of the side effects from the drugs and the child’s overall health.  It’s easy to misdiagnose a real problem because of the medications and diet).

THE surgical team fed her Pedialyte during the night and chose to do the most non-invasive surgery possible that would temporarily keep the rectum from being pushed out, which would hopefully buy us some time to find out why her GUT had so much pressure.  

THE next day Wrenn was taken to the OR.  The procedure: the surgeon would inject sugar water into the rectum in three different places, causing the rectum to swell and scar so that it doesn’t easily pop back out.  The surgical procedure can last a day or a month or a year, it’s just up to how much better or worse she gets with the GI issues.  They could have done another procedure that staples the rectum inside but it had much more risk of infection and side effects. They don’t like to do that procedure on babies or kids because generally a prolapsed rectum is a temporary issue and corrects itself once the child is older.   

THE surgery took no time at all, in-fact putting her under and finding an IV spot took the most time.  Her arms were so bruised from the last ER visit and then the labs and IV’s in St. Louis; she looked so abused.  

THAT night after the surgery they started her on a drug called Flagyl.  This is a drug the GI doctor thought might help Wrenn if it was an overgrowth of bad bacteria in her intestines.  He said if the drug doesn’t fix her bowls, they’ll have to do a biopsy and test her large and small intestines for other issues. (I didn’t even want to go there but at least we had a plan to cross things off the list).  After dealing with this issue of mysterious pain, gas and crying episodes all night, I just needed answers.

WRENN came out of the surgery in a lot of discomfort but after I had demanded she receive some morphine, slept soundly for two more hours and then woke up thirsty and in a much better mood.  The surgeons ordered no food or water for 24 more hours to protect their work.

THE next day (Saturday) around 3pm she was given a huge lunch and boy did she eat.   Macaroni and cheese, chocolate milk (her new formula called Neocate), peaches, bread, mashed potatoes and pudding for dessert.  Now it was all about watching her for the next twenty-four hours to see how her system would take the food and see if the Flagyl would make a difference.  It was nerve wracking to watch and especially stressful each time she pooped but she did great!

AFTER twenty-four hours we were released from the hospital around 4PM Sunday afternoon.  It appears as though the Flagyl did wonders for Wrenn and so an overgrowth of antibiotics was indeed her problem.  When I think about all the times she’s had antibiotics in her short life it’s no wonder.  Her body was on overload with the stuff and needed something to wipe it out.  It seems easy now, but it’s hard to diagnose a child who is special and I think I have new eyes to thank for this cure. 

WRENN was on the Flagyl for 14 days and I’m so excited to say that she’s a NEW child.  She used to have 20 diapers in a 24 hour period, now she has 6.  She loves food now, sleeps through the night and it just seems like she feels so much better.  I just can’t imagine what she would have said if she could have talked about how much pain she was in.  I dare say she was probably miserable for over a year with these issues and they just went unnoticed due to the rejection drugs and history of GI issues.

SOME kids stay on Flagyl, but we are going to see how it goes as a one-time dosage, now that I know what to look for (I believe I saw her progress to what was the WORST case scenario) and so we can literally nip this in the butt next time. J

WHAT a whirlwind we’ve experienced for the last 6 months.   You can’t blink with a child like Wrenn.  This is why I can’t work outside the home.  It’s impossible to know a child like Wrenn without understanding her daily and nightly routine.  Giving the meds at the precise time is life or death for her.  The amounts she is given are life and death and seeing any type of change with her physiologically is so important and a must to her survival and overall health. 

AT Wrenn’s GI appointment today in Richmond she weighed 19 pounds, 9 ounces, which is a GREAT weight gain in two weeks. (Better than a pound)  We are hoping that she’ll stay on pace and her body will start catching up. She is still at an 8 month old weight and a 14 month old body.  But I’m sure she is going to thrive now that she is holding in nutrients.

 

WE had such great news from St. Louis and they were so happy to hear that this wonder drug made Wrenn ‘perfect’ again.  We are in so many ways cursed and blessed.  I continue to ask God to put the right people in my path to make things better for Wrenn and for our family.  I have to say, I’ve learned more than my share about the human body and medical devices.  I have to say, putting a rectum back in place wasn’t anything on my wish list.  In the ER I felt the need to tell God I have limits—Obviously, I’m not the one pulling the strings.  So the best I can pray for is a DAMN good team to get me through the rough times.

I want to thank my Winnie team for answering all our behind the scenes questions while we were trying to figure out Wrenn’s issues.  You guys are the best!! I’d be so lost without you all. Perri, you’re Wrenn’s little guardian angel and mine, too!

UPDATE on Tanner:

TANNER was really looking forward to seeing his buddies in St. Louis and played every single hour he could in those three days at the Children’s Sibling Playroom.  He was exhausted at night and had the best time with his ole’ pals—Mr. Pat and Miss Rita.  The Sibling Playroom staff is such a wonderful part of our family and we really do look forward to seeing them every six months.  

I also want to thank all our friends and family who prayed for Wrenn when we needed answers and for coming to the hospital to see Wrenn (Mandy, Laura, her MCV doctors) and our parents for helping out with Tanner so we could take care of Wrenn.  Our lives take a team effort and it’s impossible to do it all alone, even though the sole responsibility falls upon our shoulders.  This is the type of support that gets us through the difficult/trying times and we couldn’t do it without you all.

On a personal note:  I’m thinking that someone should seriously present me with an honorary nursing degree because I’m pretty sure I’ve earned one at this point! J (Editor's Note: I agree!)

Xoxo Nicole