This is the story of one amazing little girl, a fighter. Our daughter, Rachel, has had to fight since the day she was born. She has never known what it's like to lead a "normal" life, never known what it is like to "feel good," never known what is is like to not take medications, never known what is it like to not go to the doctor's office on a regular basis, never know what is is like not to be stuck with needles, never known what it is like not to have heart-caths, never known what is it like not to be hospitalized several times, and never known what is it like not to have had multiple heart surgeries. Her life has been surrounded by adults, mainly nurses, doctors and therapists because she has spent her life in and out of doctor's offices, hospitals and therapy.
We have heard it said that Rachel is "tough" and she is a "fighter" and we couldn't agree more. According to the World English Dictionary, the word tough means, "strong or resilient: durable," and the word fighter means, "a person who has determination," Certainly, these words are perfect descriptions of Rachel. This is why she is truly our hero and we are proud that God blessed us to be her parents.
The Fight Begins
We arrived at TMH shortly after midnight (Sept. 13th) and stayed in the labor and delivery room for what seemed like countless hours. The early morning hours dragged on and finally, after hours and hours of labor, Tammy gave birth to our first-born at approximately 1:58 p.m. on Sunday, September 13, 1992. What a beautiful baby; the prettiest baby ever to be born for sure! We cried with excitement and were both overcome with emotion to see our first child being born. It's a feeling that is indescribable to someone who hasn't witnessed it before. We were both smiling ear-to ear at hearing Rachel cry her first cry, of seeing her look around in amazement at her brand new world and looking into her beautiful little eyes. And did she have a tinge of red-hair? Yep, she sure did! At that moment, it seemed that everything was perfect, but, we would soon find out that it wasn't.
Shortly after her birth, while getting her first "bath" in the nursery, Rachel turned "blue." Initially, we were told by a nurse that as a precaution she would be moved to the Neonatal Intensive Care Unit (NICU) and doctors would be examining her to try and find out why she turned blue. Okay, they probably see this all the time, no big deal. It was later that we learned that our newborn baby, only mere hours old, had something seriously wrong with her. We later found out from the pediatric cardiologist in Tallahassee that our baby had "some type" of heart problem and that she would need to be flown by Life-flight to Shands Hospital in Gainesville for further assessment. You have to be wrong. Please tell us you are wrong!
Questions flooded our minds; emotions took over. Plenty of tears shed. The anxiety of not knowing what was going on scared both of us. How could this be?How could our world turn upside down in a matter of hours?How could we go from being on top of the world as we watched a new life come into the world to a feeling of disbelief and uncertainty? This just did not make any sense. This had to be really bad dream: a nightmare that we expected to wake up from at any minute. Please God, why was this happening to our baby?
Rachel was admitted to the NICU-3 at Shands and remained there for the first two weeks of her life. It was there, in a waiting room, that we found out that our baby girl--- so innocent, so precious, so little --had a congenital heart defect known as "Tetralogy of Fallot" one of the most common cyanotic heart defects and the most common cause of "blue baby syndrome." (This explained why she turned blue shortly after her birth). We were absolutely stunned by this news. There had to be some kind of mistake. This is our baby, not someone else's baby. Please tell us you are wrong in your assessment. We learned that Rachel's pulmonary artery as too small to carry blood through to the other parts of her heart. As a matter of fact, we were told that her pulmonary artery was almost non-existent. It was explained to us that in a "normal" heart, the pulmonary artery would be about the size of a pencil, however, Rachel's artery was about the size of a pencil-lead. Therefore, there was hardly any blood passing through her artery and out to the other parts of her heart. Also, we were told that she had multiple holes in her heart, with the most significant being a large hole between the two bottom chambers of her heart known as a Venrtricukar-Septal-Defect (VSD). And, finally we were told that she has variety of other problems within her heart that would eventually need to be addressed but for now the most pressing issue was the narrow pulmonary artery. She would eventually need surgery but doctors wanted to wait until she had grown a little more before she had the surgery.
After about two weeks in the NICU at shands, we finally got to bring Rachel home for the first time. For the next 5 1/2 months, we got to enjoy the everyday challenges that comes along with being first-time parents and adjusting to having a baby in the house. We enjoyed every minute of it, including those early morning hours when she would wake up hungry. Eventually though, we knew the day was coming that she would need surgery.
In March 1993, at only six-months old, Rachel had open-heart surgery at Shands. Dr. Daniel Knauf performed the surgery. He would put in a conduit from the right ventricle to her right pulmonary artery with the purpose being to provide more blood flow into the pulmonary artery. The thinking being that the more flow of blood along with her growing and getting bigger, the more the artery would grow with her. Nothing in our lives was as tough as the morning of that surgery. Handing our daughter over to a man that we had only met the night before and trusting that he would not let her die, that he would take care of her is a feeling we will never forget. It's a feeling that no parent should have to encounter.
The night before her surgery, one of the doctors asked us if we had noticed anything odd or different in Rachel's development. We did mention to him that she had always kept her right are drawn-up to her body and that she kept her right hand in a fist, that she didn't use her right arm/hand at all. So, an MRI was ordered. We learned from the MRI, that it appeared Rachel might have had a stroke while still in the uterus. The MRI revealed that the left-side of her brain "didn't form all the way" and this would affect the right side of her body, could effect her speech and language development. We were told that because of this she could be susceptible to seizures. Great! Another thing to worry about in addition to her heart problem!!
From after surgery in 1993 until 2006, Rachel had numerous heart-cath procedures and visits to the Cardiac clinic at Shands. At one point we were told not to let anyone ever do a cath on her again that our next surgery would be a heart/lung transplant. When she was around five years old, she had surgery to repair her sub-mucus cleft palate as she had also had palatal problems since birth. Rachel eventually began undergoing speech, physical and occupational therapy. Eventually, we learned that Rachel has "Velo-Cardio-Facial Syndrome" (VCFS), a deletion of the 22q11.2 chromosome, also known as "DiGeorge Syndrome." A syndrome is a compilation of abnormalities that affect a person. We later learned that heart defects are one of the more common abnormalities of VCFS. We also discovered that Rachel has a number of these abnormalities including: palatal problems, learning difficulties (she is developmentally delayed), speech/language impairments, hypothyroidism, seizures, scoliosis,and feeding difficulties to name a few.
It seemed that Rachel was constantly in and out of doctor's offices for various reason as she grew from a baby to a teenager. As a baby, she had numerous ear infections which eventually led to her having tubes in her ears. During the winter months, she would always catch a bad cold complete with severe coughing and fever. She began to experience migraine headaches which would, in turn, make her nauseated and cause her to get sick. It seemed like that if anything came along she caught it. For reasons not known, she would sometimes wake up sick during the middle of the night. This would happen off-and-on for several years. Her file at the pediatrician's office was beginning to resemble a New York City telephone book.
In August 1995, Rachel became a big sister to her little brother, Camden. God blessed up with a perfect brother for Rachel. Of course, as sibling they fought like most siblings do and continue even unto this day. But deep down inside Camden has a special place in his heart for his sister and Rachel has that feeling for her brother. Camden has also had to grow up around nurses, doctors and hospitals because of Rachel but not one time has he complained. He has always been there in support of Rachel. So, it has been hard on Camden as well, but in him being around Rachel and seeing her in the hospital so many times it gives him a greater sensitivity for other children who may have difficulties but just want the love and friends like everyone else. He isn't shy about befriending another child with health problems, both seen and unseen. God truly picked a special person in Camden to be Rachel's brother and for that, we are thankful.
In May 2006, Rachel underwent her second open-heart surgery at Shands. The surgery would be preformed by Dr. Mark Bleiweis, chief of pediatric cardiothoracic surgery. The surgery would entail Dr. Bleiweis replacing her pulmonary artery with a gortex-tube in order to allow enough blood to flow through her artery; to replace the conduit that was put in her when she was six-months old with a pulmonary valve and to close off some of the holes in her heart. Once again, we had to hand our daughter over to a man that we barely knew and had to trust him that he would take care of her. The surgery was successful and after a hospitalization of about two weeks, Rachel was discharged from Shands. However, our stay at home was short-lived as she seemed to regress after we got home; she had to be re-admitted to Shands about a week later. It seemed that after being re-admitted, Rachel did not get better. After weeks and weeks of no progress it was decided that she would need to undergo an Electrophysiology study (EP study), a procedure which tests the electrical conduction system of the heart to assess the electrical activity and conduction pathways of the heart. Eventually, it was decided that Rachel would benefit from having a pacemaker. So, in late July, while still hospitalized at Shands, Rachel underwent the procedure to have a pacemaker inserted to help regulate her heart rhythm. After nearly four months of being hospitalized, Rachel was discharged and returned to her home.
In November 2006, the weekend before Thanksgiving, Rachel experienced her first seizure. We had no idea that it was a seizure; we assumed it was cardiac-related. She was eventually airlifted to Shands and admitted to the PICU. The doctors determined that it was not cardiac-related and suggested that it could have been a seizure. After a few days at Shands, Rachel was released and we traveled back home on Thanksgiving Day. However, a few days later, she experienced another seizure. Eventually, she was seen by Dr. Ricardo Ayala at TMH and was put on anti seizure medication. Our minds went back in time to the night before her very first surgery when it was revealed to us what the MRI had shown and we were told that Rachel could experience seizures as she got older. Are you kidding?!? Hasn't she been through enough already?? Now she is experiencing seizures too!!
In April 2011, Rachel underwent her third open-heart surgery at Shands. Once again, Dr. Mark Bleiweis performed the surgery. The surgery consisted of Dr. Bleiweis replacing the pulmonary valve from her previous surgery because it had begun to narrow and leak and he wanted to close off the VSD. The surgery was successful but once again complications arose afterwards. We learned that Rachel's aortic valve was leaking more than the doctors realized. We had known for several years that the aortic valve was leaking but it had been categorized as "mild to moderate" and that if it worsened something would need to be done to correct the leaking. A heart-cath revealed that the aortic valve was leaking more than before and in doing so the excess blood from the leaking valve would cause the left ventricle not to relax during the relaxation phase of a heartbeat. The doctors were now faced with a dilemma: attempt to have Rachel undergo a fourth open-heart surgery to try and replace the aortic valve, which would have to be replaced about every two years, thereby putting Rachel through multiple heart surgeries or list her on the national transplant list for a heart transplant.
On June 7, 2011, Rachel's name was officially listed with the United National Organ Sharing transplant list to receive a new heart. Dr. Bleiweis told us that a new heart would give Rachel something she has never had before: a healthy heart. He also said that after she receives her heart she will finally experience the joy of what feeling good feels like.
So here we are, waiting on a new heart. 18 years of battles and our little hero, refusing to go down but instead fighting in the next round of her fight with life. Our little hero, so strong, so resilient and so durable, determined not to give up. Looking at her, it's as if she is saying to life, "This is the best you can do to me? Well, I'll show you? I'll take your best shot and give it right back to you?"
So the next time you hear the words "tough" or "fighter," please remember one very special girl. A girl who is small in stature but is strong, resilient, durable and most of all, determined. That little girl is our beautiful hero, Rachel.
P.S. - To every parent: If you have a healthy child, take the time to thank God for him or her. Take the time now to thank God that you don't have to experience seeing your child with a long incision down the middle of his or her chest from having had open-heart surgery; or having to watch your child be hooked-up to so many tubes and wires while laying in a hospital bed; or seeing your child with a breathing-tube down his or her throat; or seeing your child get stuck with needles so many times that you wonder if he or she has any veins left; or having to watch your child take so many pills during the course of a day; or taking your child to the doctor's offices over and over; or seeing your child suffer a seizure; or seeing tears stream down the face of your child because he or she is scared of what might happen next. Take the time to wrap your arms around your child today and thank God for them.