Thursday, May 12, 2011

Featured Child~ Laycee

Laycee Renae entered this world on April 17, 1993, full term, 8 pounds and, with an undiagnosed left CDH. Because our local hospital was not set up for such a critically ill child, arrangements were made to send her to Cook Children's Hospital in Fort Worth. During the flight, the physician determined she would need ECMO in order to survive, so she was rerouted to Presbyterian Hospital in Dallas where she would spend the next 4 months in NICU. She was on ECMO for 21 days and during that time, her hernia was repaired with a gortex patch. She also suffered a massive stroke to the left side of her brain, literally wiping out all centers for speech and right sided motor movement. I did not know my Laycee at this point...she would enter my life at the age of 4 months at the time of her discharge from the NICU. I am an RN who was providing foster care for medically fragile children. My job was to care for the children, make them as "user friendly" as possible and work closely with the parents to prepare them to care for their special child. In Laycee's case, her mother was all of 15 and simply unable to take on such a huge task. I adopted Laycee and buckled myself for the roller coaster ride of my life.

When she was discharged from the NICU, Laycee's physicians told me to "have fun because she would just stare at the walls." Her brain injury was severe and her chances for a positive neurological outcome were slim. Of course, I could have easily slapped the man, but the NICU door locked before I had a chance to respond. I could tell with my first encounter that this girl was a fighter and she was not going to be held back by a "little" brain issue. Laycee was still very ill when she was discharged from the NICU.

I often wonder if they did not send her home with the thought that she would die. I had other plans. I was determined that this child deserved every chance possible to live. She still had a huge PDA and SEVERE GE reflux. I kept her crib in my room and with the continuous oxygen, monitors, feeding pump, looked like intensive care. I remember one night I marked a piece of paper every time I had to get up and help her breathe because she had vomited and was choking...the next morning I counted the marks and there were 27. TWENTY SEVEN times that this child had had to fight to breathe. Anyway, I had her care transferred to Cook Children's Hospital and she finally had the fundoplication to allow her to be fed without all the drama. I will always remember the surgeon, Tim Black, coming out after the surgery and telling me that the surgery took twice as long because she was literally glued together on the inside. He said her liver looked nothing at all like a liver and it took him 10 minutes to even figure out what it was. But it worked!

We still had the issue of the PDA and she was beginning to decompensate. Her oxygen needs were becoming greater and she had gone into congestive heart failure. At the age of 8 months, she underwent her PDA repair. Because her heart was on the right, they had to go through her sternum to perform the repair. The day after her surgery, she had to be rushed back in for an emergency procedure to drain the fluid from around her heart. She came out with tubes coming from and going into the sac around her heart. She had a Group D strep pericardial infection and, rather than spending the traditional week in PICU, she was there for 27 days. She spent her first Christmas in the hospital and, because I had previously been scheduled to work that day, I did not get to be with her. The nurses were kind enough to call me and tell me how precious she looked in her Christmas outfit.

The following years were great. Laycee literally thrived. She was walking by the age of 3, and despite the lack of speech centers in the brain...she NEVER stopped talking. It was wonderful to see her so happy.

When she was 8 years old, Laycee developed a respiratory infection that simply would not go away. After a few weeks, she began experiencing severe headaches and was unable to even stand up due to the pain. She was diagnosed with viral encephalitis and, once again, we were strapped in for the roller coaster ride. Her behaviors became very unpredictable. She would get up in the middle of the night and leave the house. I had to install locks high on the doors to keep her safe. I was terrified. She also developed seizures...a new issue. Luckily, with time and a lot of patience, she recovered and became the happy, bouncy little girl I had always known.

Of course, she could not let it rest. She was diagnosed at the age of 10 with Tourette's Syndrome (her maternal grandmother had Tourette's). At first, I was determined it would be ANYTHING but Tourette's but, finally had to come to terms with it. I realized she had been showing signs most of her life but, we had always attributed her behaviors to just being "Laycee." Now, I hardly notice it...she can scare the pee out of people who do not know her or her Tourette's though. Luckily, she was not one of the 10% of Tourette's sufferers who curses.

Next crisis...Laycee required a scoliosis repair at the age of 11. It was soooo very difficult to allow her to have the surgery but it was progressing very quickly and her lungs (which only one is "good") were being compromised. She underwent the surgery and, because of her history of CDH, they were not able to perform the anterior approach to her spine. Instead her orthopedic surgeon, Dr. David Brown (God Bless him) used a technique that has rarely been used in the United States. He used a different type of screw that was much longer to prevent her spine from twisting as she grew. That was five years ago and she looks great.

Now we can rest...right? WRONG? Just a few weeks after her 13th birthday, Laycee woke up one morning and complained of a tummy ache. I briefly considered appendicitis but quickly realized that with her CDH history, I really did not know where her appendix was. She was still eating and drinking and pooping so, I thought she probably had a virus. By the third night, her abdomen began to swell. I took her to her pediatrician and she was diagnosed with a bowel obstruction. A direct result of her birth history of the CDH (the ugly monster just never goes away). She was sent to Cook Children's Hospital where she underwent surgery. I was told the surgery went well and that her intestines looked good (no areas of necrosis). She was transferred to the surgery floor to recover and, in her usual fashion, was up and walking and wanting to eat within 24 hours. All of a sudden, she doubled over in pain and I knew something terrible had gone wrong. Her abdomen began to swell again, and, at one point, her pain was so great she did not even recognize me. She was having difficulty breathing and began requiring oxygen. Despite all this, her surgeon (not one we had used before or that I would recommend) kept telling me she was "fine." Finally I asked that her pulmonologist be consulted because of her breathing problems. She is woman who does not hesitate to step on toes if she sees a child in need. Sure enough, she took one look at Laycee, placed her on antibiotics and called the surgeon to tell him she HAD to return to surgery immediately. She was rushed back to surgery, now in septic shock.

The anesthesiologist told me he did not think she would make it through the procedure given the grave nature of her condition. I was devastated, furious and scared to death. She made it through but was now in extremely critical condition and in PICU on a ventilator and all types of supportive drugs. It turned out that she had developed a leak in her intestines and they found 2000cc of feces in her abdominal cavity. That poor baby had suffered with that for almost 48 hours before being taken to surgery. Five days later, I thought things were getting better when she began leaking feces from her abdominal incision. Once again, she was rushed back to surgery (only after I insisted that a new surgeon be brought in because her present one said the leakage was nothing to worry about). This time they cleaned 1800cc of feces from her abdominal cavity. Three days later it happened again. This surgeon suggested that we might just want to "wait and see." I think it was his way of saying, "let nature take it's course." That is fine, except my daughter wanted to live. I insisted she be taken back and, once again, another 1800cc of feces. This time she came out with an ileostomy and the surgeon told me there was nothing further that could be done if she leaked again. I was thrilled she made it through another surgery and terrified that she might leak again. She didn't. Of course, the next 2 months were a nightmare. She developed ARDS because of her severe sepsis and had to be placed on nitric oxide because of pulmonary hypertension. I lost track of the number of times she had to be drained of abcesses in her abdomen.

Finally, after almost 3 months, she was weaned off the ventilator but still required bipap in order to breathe. At that point, I began making plans to bring her home. I knew she still needed a lot of care but, I also knew she would do much better at home and, I would no longer have to fight with medical professionals to keep her alive. It was so difficult to convince them that prior to this illness she had been a "normal" happy little girl. They saw a swollen, scarred, critically ill child with a g-button and assumed she was a "vegetable." Pissed me off!!!

She made it home and we began the long road to recovery. She developed pancreatitis due to TPN (she was on TPN for 8 months) and had to have her gall bladder removed. In addition, her ileostomy was reversed (YIPEEEE) and she had to be rehospitalized for a fungal sepsis (another Christmas in the hospital). It has taken a full 3 years, but Laycee seems to be Laycee again. She does suffer from lung problems because of the ARDS and requires frequent oxygen supplementation. She also was diagnosed with autonomic nervous system dysfunction (probably as a result of the severe sepsis) and has times when her blood pressure and heart rate drop to dangerously low levels. I had a port implanted a year ago so that I can give her IV fluids when this happens and we can avoid a hospital stay. She still has her G-button (placed when she was 3 months old) and it really comes in handy when she is sick and requires extra nutrition. Otherwise, we just use it for medications.

I truly believe Laycee is a miracle. Some people look at her history and wonder "Why?" Why put her through so much? Believe me, I have asked the same questions many times but, it always comes back to one thing...Laycee wants to live and absolutely loves life.

She can make a room shine just by walking into it. She has an uncanny ability to sense when someone (even strangers) are hurting and she can empathize with their pain. She has walked up to people in a parking lot and said, "Are you okay, you look sad." They look at me as if to say, "How did she know? "She will hug them and they walk away smiling. I feel that Laycee will know when her time on this earth is complete and I will be there for her. In the meantime, we will continue to fight and will continue to enjoy each and every moment she breathes the beautiful air God has provided. She is my joy, she is my oxygen.


Wednesday, May 4, 2011

Featured Child~ Claire

We found out Claire had CDH during our 19-week ultrasound.  We had never heard of CDH before, and it was only after we got home and looked it up on the internet that we realized the severity of it.  Claire's CDH was labeled as "moderate" (stomach and intestines were up), and no one really knew how she would do.  At 36 weeks I had to be induced due to pre-eclampsia.  Claire was born weighing 5lbs 8oz and surprised us all by pinking up and crying LOUDLY.  But she soon started having a lot of trouble breathing, so they intubated her and transferred her a few blocks away to the children's hospital.  She was able to remain on a conventional ventilator, and had her repair surgery at 6 days old.  They were able to do a primary repair laparoscopically without using a patch, but had to pull her existing diaphragm really tightly to attach it to the other side.  She came off the ventilator at 10 days old, and was able to go home after 22 days.  She came home on oxygen and drinking milk from a bottle.  She came off the oxygen at 6 weeks old, and was eventually able to breastfeed.

At her 1-year follow-up appointment the surgeon told me Claire might be developing scoliosis, but that it was "mild".  At her 2-year follow-up appointment we saw a different surgeon, who looked at her chest x-ray and also said the scoliosis curve was mild.  I noticed some asymmetry of her ribcage, but was told it was probably due to the CDH.  By her 3-year follow-up appointment last summer I was becoming concerned about noticeable curvature of her spine and the way her bottom left rib poked out.  The surgeon looked at the x-ray and agreed that the scoliosis was worse, but also noticed that her diaphragm had reherniated and part of her colon was coming up through the hole.  We were really surprised since she'd had no symptoms of reherniation.  The CT scan confirmed it, and she had surgery to repair it last October at age 3.  This time they put in a gortex patch, and were able to do everything laparoscopically again.  While in the hospital she acquired a C diff infection following the routine dose of antibiotics they put in her IV after the surgery.  She became severely dehydrated due to colitis and was in the hospital for 7 days.  We returned to the ER a few days later for a bladder infection.

Last summer we were referred to an orthopedic doctor about her scoliosis.  He did a full x-ray of her spine and measured the curve at 38 degrees!  It was difficult for the CDH surgeon to see how bad the scoliosis was from a chest x-ray alone because he was only seeing the top half of her spine.  The orthopedic doctor said the scoliosis was probably due to the tension on her diaphragm from it being pulled so tightly during her first repair surgery.  She began wearing a Boston brace for 16+ hours per day last November to try to keep the curve from getting any worse.  She will need to wear the brace until she is a teenager and stops growing.  But since she is so young and the curve is already pretty severe, chances are she will eventually need spine surgery.  We are trying to prevent this or delay it for as long as possible.

Claire will turn 4 years old next month, and you would never know she has faced so many obstacles.  She is very energetic and is always smiling and dancing and singing.  She has lots of friends and loves going to preschool.

To Read More about Claire