January 30, 2011
"I was born with a congenital diaphragmatic hernia- basically a hole in the diaphragm. It’s a life-threatening condition for infants. Part of the condition is that most of the organs are basically squished up into the ribcage, which prevents development of the heart and lungs. On my left side there was a partially formed lung, and on the right side was only lung tissue. When I was about 5 days old I had surgery to correct all of this- clearly a risky operation. They successfully patched up my diaphragm and moved everything to where it was supposed to be (although my stomach is now facing backwards) and even took out my appendix while they were in there.
My doctors predicted that although my lungs would probably develop normally, I would be severely inhibited by shortness of breath and asthma for my entire life. I would never be able to run, participate in sports, or anything like that. They said I probably wouldn’t even be able to walk without getting out of breath.
These days, I do suffer from asthma, but I am also a singer. My diaphragm does not expand correctly, so all of my muscles in my chest have adapted so that I can breathe a bit deeper and sing, as well as playing multiple wind instruments. I was in marching band for 3 years of high school, playing snare drum (hauling around 20 pounds of dead weight). I racewalked on my high school’s track team. I do get out of breath somewhat quickly, but it does not stop me, and I have far exceeded all the expectations of my surgeon and doctors. I’m not the best, and sometimes it IS hard to remember, but if I’ve pushed through this my entire life, the rest is just small potatoes."
April 25, 2009
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, etc...it 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.
March 18, 2009
I found out in November of 2003 the morning that I went in for emergency surgery that I was pregnant. Being 35 years old and an insulin dependent diabetic I was considered high risk. I was sent to Fort Worth to a high risk ob named Dr. Tabor. After month number 2 they told me that the baby was going to have problems. First they said it was a heart problem and with each visit it was one scare after another. Around my 6th month they told me that he either had Hydrocephalus or Holoprocencephaly. I cried and prayed every day that God would help my little boy Christopher. He decided on June 18th, 2004 that he was on his way a month early. I went to my local ob for the usual blood pressure and heart rate check and my favorite nurse Deborah was watching me closely and asked if I could still drive when I said yes she told me to get to the hospital. I walked into the hospital it was sunny and all seemed well. Within a hour it was storming out. They had discussed care flighting me to Fort Worth but when they called Dr. Tabor they had no beds available at Harris Methodist so they told me that I would be going to Baylor by ambulance. They tried to stop my labor but Chris was on the way. I knew I would have to have a vertical c-section. A very old and dear friend drove to Dallas that night to be there with me for the delivery. I had never been so terrified in my life. Not only was I alone I was going to find out if my little boy would live more than 2 years or not. Roxann arrived sometime in the middle of the night and I finally broke down and told them to put me in the next open slot because after 36 hours in labor I knew that there was no stopping my son. We thought his lungs were only 50% developed but as soon as he was delivered and given an oxygen blow by we knew he had lungs. Within a few minutes they also came back and told me that he had hydrocephalus and they could fix the problem. I cried and thanked God with every breath that he would be ok.
He was taken 24 hours later to Children's Medical Center and taken immediately to ICU. I had to wait till the next morning before I could go. I had 1 day at Baylor and 1 day at Children's to bond with Chris before he had the first of 13 surgeries. At 3 days old he had a third ventriculostomy. That lasted around 3 or 4 days before the surgery failed and he had to go in for shunt placement. The first 2 surgeries were the worst on me I think because when they call and tell you they are going to be in recovery and you can come back in a little while. The first surgery I waited 1 1/2 hours and they still had not called me to come back. I spent the whole time begging God to let me keep his son for a little while longer.
Chris has undergone an ETV, shunt placement, shunt revision, 2 eye surgeries, 2 oral surgeries, hernia repair, 2 cranial reconstructions and 3 minor day surgeries. Chris had cranial reconstruction at Medical City Children's in february of 2006. I was told 2 days in ICU and at least another week in pediatrics. That surgery was done on tuesday morning and we were at home that friday morning. Before this last surgery he could not hold up his head or sit up. Dr.s Sacco and Fearon removed 300cc of fluid from his ventricle and cut down the back of his skull to fit the smaller size. He is now the head butt king and if he is not sitting up then he is asleep.
God has blessed me with a beautiful, happy little boy. His achievements are mind boggling. I know without God we would not have made it through all of the hard times thrown at us. I know that when parents hear there is a problem with their babies it is the hardest thing to survive but I am walking proof that if you give it to God you will be amazed by the miracles you witness. I came to see the surgeries not as a set back but as an opportunity to witness another miracle.
Right after I had Chris I went to a mall somewhere around the hospital and had gone to the bathroom. I had a mother walk up to me with her daughter and ask if she could pray for my son. I was shocked and said yes. She asked God to heal Christopher's head and after she was done and had left the restroom I realized I had not told her I had a son in the hospital or what was wrong with him. I walked out and tried to find her but they had disappeared into thin air. My mom was walking towards me and I asked if she had seen the mom & daughter and she said no - no one had come out of the restroom. That was the morning that I knew that Christopher would be fine and that his angels would be with us. God bless all the children and especially heavens very special children.
To learn more about Christopher you can visit www.littlechristophersjourney.com
March 15, 2009
Our O-baby was born on 8/08, her name is Mia is she is doing wonderfully.
We were diagnosed with an O (and a 2 vessel cord) at our 19 week ultrasound and it was a long road from there. We were given the option to terminate but for us that wasn't an option. So we were initially sent right to the cardiologist at a local childrens hospital where we were told that in addition to the O (they said it was her intestines) and the 2 vessel cord that our daughters heart was slanted and that these things together most likely mean a genetic disorder. The Cardiologist said she felt there was a very good chance that it was down syndrome and we should read up and prepare. We had done the amnio the day before so it would take some time for the results to come back. The next week we got the FISH results back and everything was normal - but we still had to wait on the final results.
We decided to go to Children's hospital of Philadelphia. (CHOP) for a second opinion. After all days tests - we were told that the O was considered a Giant one because it was the liver (not the intestines as we were first told) and that her heart was not slanted. We had been told by the other doctors that because of the 2 vessel cord she would most likely be born early and very small. But CHOP was not concerned with this. They felt this was an isolated O and it was only about 3 cm of her liver and that the final amnio results would be fine. They were right.
We continued our care at CHOP and would deliver there. They opened the first high risk birth center in the world (in a childrens hopsital) in June and Mia was due in August. So we were very lucky with the timing. She did come 2 weeks early (my water broke at work :)) and weighed 5.8 lbs. She was breathing on her own from the very beginning and never once needed help.
Her O was small and would be repaired with-in her first week of life - it wasn't repaired immediately because they were waiting for pressure around her heart to decrease (this is normal with smaller babies). Her O was repaired when she was 6 days old.
She did have a VSD (heart murmur/hole in her heart) but nothing that was too concerning and these things can sometimes close on there own. Once she was diagnosed with a VSD - they had to do further genetic testing to be sure everything together was not related to a genetic disorder - so we again had to wait and pray and luckily for us everything again came back normal. She was released from the hospital at 21 days old with a feeding tube but with-in a week she was eating on her own.
She is now 7 months old and at her 6 month appointment with the cardiologist we were told that her VSD is closing on its own and he took her off the meds that she has been on since birth. So we are excited to finally be med-free! Mia is small - only about 14 lbs (her height and weight are in the 4-5th percentile) but other than that she is absolutely perfect. The doctors are not concerned with her growth since she has continuously gained weight. Her O scar was made to look like a belly button - and its hard to tell its not one! CHOP did an amazing job!
I know i am luckier than most and our story ended a lot quicker than some but i felt i should share. I feel extremely lucky to have gone through everything we did and be were we are today. It was a SCARY and what seemed like an endless road but before long - its over and its just a distant memory. We were given alot of worst case scenerio's and i thought for sure some of them would be true but we are truely a story of hope and for us, everything turned out better than we could have imagined.
Patti Sam ~ Mommy to Mia
In August of 2009 we went for her one year old follow up with the surgeon at CHOP and they said she looked great and that we did not have to come back - the surgeon said it was basically like she never had the O. At her one year follow up with the cardiologist - they said her hole was mostly closed on its own and that we would only have to come back for annual check ups to be sure nothing had changed. Then at her 2 yr old check up we were told her VSD completely closed and we were all done with the cardiologist! :) So for us - that closed they chapter on the O...hopefully for good! That was very exciting.
Mia is a big sister now - her brother Brayden was born in December 2009 and she will be a big sister yet again in early May 2011 - this time to a little sister. She is an awesome big sister. She is very caring and helpful. She loves playing mommy to her baby dolls and gets soo excited when we see little babies around - so i'm pretty sure she is going to be a big help when her little sister gets here. She loves helping mommy, being daddy's little girl, doing puzzles and is my little "smarty pants" ;)
Mia is still small compared to most - only about 23-24 lbs at 2.5 year old. But still the doctors are not concerned as she has always been on her own growing curve and as the surgeon from CHOP put it - there are worse things in the world than being petite! :) She loves her fruits and veggies and for the most part is a great eater!
Patti Sam ~ Mommy to Mia
March 14, 2009
(Erica's story is told by Sarah Avery a writer for News & Observer.)
The article is entitled:
A Rare Challenge, A Rare Chance
1 baby in 10,000 has this defect. Erica's was the worst doctors had seen.
DURHAM -- Everyone knew from the start that Erica Nance would present challenges.
As a fetus, her abdomen failed to fuse shut in the early weeks of development, leaving all her organs outside her body instead of safely encased behind the rib cage, under muscle and sealed with skin. The anomaly, called a giant omphalocele, occurs in about one in 10,000 births. But when Erica was born in August 2006, every challenge her family and doctors anticipated grew more complicated, forcing ever-higher demands of human and technological know-how.
"The look on the surgeon's face terrified me," said Erica's mother, Lisa Nance, recalling the moment her baby was born by cesarean section at Duke University Medical Center. The tiny girl was normal, except that her stomach, liver, spleen, intestines and colon dangled in a mass outside her body.
Most babies with omphalocele (om-FAL-oh-seal) have some portion of their organs in place, making it possible, using time and gravity, to route the organs safely back inside. Not Erica. Her malformation was far larger than anything the Duke doctors had ever seen. And with everything exposed, her body had never grown an abdominal cavity.
"It went from skin to spinal column," said Dr. Jeffrey Marcus, Erica's plastic surgeon at Duke. "There was nowhere for the organs to go."
Erica was in dire circumstances, and the effort to save her life would last more than two years.
Nobody knows why.....Doctors don't know what causes omphaloceles, except that something interrupts the normal process of fetal development when cells fuse along the midline of the abdomen. A related disorder called gastroschisis, which involves just the intestines, has been on the rise in the United States. Researchers at UNC Hospitals conducted a study earlier this decade and documented the rise in gastroschisis but found no corresponding uptick in omphalocele cases, said Dr. Matthew Laughon, who led the study.
"There is no understanding as to why this happens," Laughon said.
For babies with omphaloceles, the diagnosis can be doubly troubling. About 30 percent of children with the condition also have a chromosomal abnormality that can be fatal or result in physical disabilities and mental retardation.
Nance said her doctor at home in Fayetteville said some parents terminate the pregnancy when omphalocele is detected during that first ultrasound scan.
"I couldn't do that," she said. "I just couldn't live with myself."
Further tests indicated that Erica did not have the chromosomal disorder and was otherwise healthy. So Nance arranged for family members to care for her 4-year-old daughter, Chloe, and scheduled a C-section nearly two hours away at Duke, where doctors could immediately begin work on Erica's organs.
Not until Erica arrived, however, could anyone understand the scope of her problem.
Among the most pressing concerns was the baby's ability to breathe. Without a liver and other organs tucked tightly under the diaphragm, Erica's lungs couldn't inflate properly. She had to go on a ventilator and a feeding tube.
Still, doctors hoped they could coax Erica's organs into whatever space existed in her abdomen. Using a cone-shaped structure that relies on gravity to gently feed the organs into the body, they hoped that Erica, like most babies with omphalocele, would be able to go home in a few days.
"Days turned into weeks, and weeks turned into months," Nance said.
Making a way......Doctors needed to create space in Erica's abdomen, so they decided to surgically insert a small, saline-filled balloon into the small cavity of her pelvis. Using a port open through the skin, they gradually filled the balloon with more liquid, causing it to expand the cavity.
When Erica was about 4 months old, surgeons tried once again to encase her organs internally. This time, everything fit. But a few days later, for reasons no one quite understands, Erica's blood quit clotting, and she began to bleed internally. Doctors had to remove all her organs again. They were back where they started.
"After all that, we decided we weren't going to do any more," Nance said. "I needed her home."
The doctors agreed. They decided to buy time and figure out a new strategy. To shield Erica's mass of organs from infection, they covered it with skin grafts, using tissue harvested from the baby's thighs.
Erica went home to Fayetteville after nearly nine months -- a ventilator in tow, full-time nursing care on tap and treatment needs that at one point cost $44,000 a month. Nance, who tended bar before Erica was born, had not been able to work since her baby was born, so costs fell to Medicaid insurance.
Creative solution......Marcus, who is surgical director of Duke Children's Hospital, said Erica's situation presented a unique problem, because her body simply had nowhere to hold her organs. So he devised a novel way of making room.
In addition to implanting the balloon extender in her pelvis, he implanted extenders on each side of her trunk. Gradually filled with more liquid, these would grow enough skin to cover the distended organ mass like two trapdoors.
The youngster was fitted with a girdle of sorts that another doctor at Duke tailored from a bathing suit and fabric. It supported the organs and increasingly heavy bladders of saline.
"She made tons of progress, and we got to the point where we were ready to do something," Marcus said. "It looked like we had a reasonable chance."
Surgery was scheduled for November. The first step was to take the extenders out of her pelvis and try to fit in as much bowel as possible. Some tucked in, but not all. And the liver remained exposed.
Then doctors removed the balloon extenders on Erica's flanks, thinking they could cover the rest with those flaps of skin.
"It was not remotely enough," Marcus said, noting that only half the organs were submerged or covered.
There was no going back, since they had peeled off the skin grafts that had covered Erica's organs for more than a year. Marcus said they decided to cover the gap with a new synthetic material, a dense collagen substance fashioned from pig tissue. The hope was that it would bind with Erica's skin as it began healing.
After initially looking bleak, Erica's prognosis improved. Each day brought more progress, as her distended belly began receding and her organs began settling in the growing cavity.
Two weeks ago, nurses began weaning her from the ventilator and teaching her how to eat solid foods. On Friday, she was discharged from Duke and headed home by ambulance. One day, she will be able to run and play like any other child.
"Not all that long ago," Marcus said, "she would have died in infancy. Where we won was resiliency. We didn't quit. We stuck with the plan, and it was a good plan."
Miracle! Miracle! Miracle Erica!
March 13, 2009
Our daughter was born fighting. She was diagnosed with Congenital Diaphragmatic Hernia when I was 20 weeks pregnant. She was given horrible odds of survival. Our home is in Iowa. We traveled to Children's Hospital of Philadelphia for her birth. (16 hours away from our home.) We never wanted to look back and wish we could have done more.
She was born in 2006. Boy did she come out fighting. Her third day of life was especially difficult. The doctors told us that she was within one bad blood gas of going on ECMO. (This is a heart and lung bi pass machine.)
She stayed strong and didn't need it. Her 14Th day of life she was prepped for her first surgery. They would bring all the organs (stomach, spleen, large and small intestines, and part of her liver) down to their rightful spots and place a gortex patch in place of her diaphragm that had been missing. Her left lung inflated and started working. It was the first HUGE miracle. She then started her recovery and we were discharged after 47 days in the NICU. She thrived when she came home. She became a normal child. She no longer needed her NG tube for feeding. She began nursing! We had a fabulous first and second birthday for her. We celebrated her life and her health.
I wish I could say that is the end of her "Miracle experience" but it was just the start. Then a month after her second birthday she became really sick. She kept throwing up and became lethargic toward the end of the day. That night she was airlifted to a bigger hospital. We were in shock. Ava was rushed to emergency surgery. It took seven and a half hours. They had found that her intestines some how swallowed up her gortex patch and was trying to expel it through her digestive system. Perhaps it might have worked except they got stuck in her small bowel causing a total obstruction. When we finally saw her she looked really bad. She was on the ventilator and was getting a blood transfusion.
The next few weeks only got worse. Ava's incision got infected and it had to be oped back up an inch for them to drain it out. Next she got a severe burn on her arm from a picc line dressing.
Then her body stopped producing urine. Her tiny 19 pound body swelled to twice it's size right before our eyes. They were talking about dialysis but then switched her medicines and she began peeing. Ava was extubated too soon and a few days later she was reintubated. Her lungs were a mess. At one point she only had a tiny portion of her right lung. The rest was collapsed or had fluid in them. Her stomach kept getting bigger and bigger so we demanded that she have more tests run to show what was going on. Finally the pressure got so great inside her that the only option was to open her whole abdomen up and leave it open so that her intestines could heal.
Well if one of those surgeries weren't enough she was again prepped for a second one. This time they would place a wound vac over the open abdomen for even faster healing. Plus they were going to give her a Hickman line. This would be a central line so that we could "feed" her through IV nutrients. During this last surgery they did her second skin graphing for her arm that got burnt from the picc line. Unannounced to us they had to shave her entire head for the graft. We were so distraught to say the least.
Ava still fought so hard. After getting moved out of intensive care I decided it was time to figure out a way to get her home. She was getting depressed and who could blame her? They taught me all about her central line and her wound vac and all her special needs. On day 47 we left the hospital and we were finally going home. Though our journey was no where near over, she could start the process to really heal.
It was wonderful to finally be home and to be with our other two girls Lexis-10 and Emeline-8. Ava did much better at home but she continued to threw up all the time and to make matters worse it was dark green bile. We went back to the doctor and did a swallow study test. It showed that she still had a bowel obstruction. Her doctor told us that we must wait about 2-3 months before going back in to fix it. Her intestines just were not ready to be operated on again. They had become like "cement". So again we went home and just dealt with her illness. Ava did much better at home. She began to perk up and smile again. She had to learn to walk all over again. On November 21, 2008 she shocked us by taking three steps all on her own.
Ava was admitted to the hospital for a little more than a week due to bowel obstruction complications. But once again we were sent home to "deal" with it all.
Finally after 4months I insisted that Ava HAD to have her surgery. Her surgeon was leary but decided to listen to me. On January 28Th Ava had her final closure surgery. A g tube was placed at this time.
Another hospital stay ( April 9- 12) was inevitable for a few days again due to complications with her bowels and swelling. Her central line became infected on April 11th. She ran a very high fever and wasn't doing well again. Again she was taken to surgery to remove it.
Ava's sixth hospital stay was inevitable as well. (April 18- 22nd) She was still obviously obstructed. Since she had no central line I could no longer give her IV fluids at home so we stayed for a few days to give her some nutrician.
Yet again we were back at the hospital from June 4th - June 17th.
June 5th was her last and final major abdominal surgery to fix her bowel obstruction. She was given another (Hickmann) central line for us to feed her at home. We prayed this would be the last. Ava had gone through so much.
Ava got very ill after her surgery and spiked a high fever. Her stomach grew very big once again. I knew something was wrong and insisted again on a CT scan. They listened. There was a problem. She had an abcess as large as her kidney. They took her again to surgery and placed a drain in her abdomen. Finally she started getting better. It felt so good to be going home.
Two months later she spiked a very high fever again. We immeditely questioned the central line. She was admitted and they started running tests. She had been doing well eating by mouth so we decided to take it out on August 27th. Strangely an infection was never found. We figure it was her bodies way of letting it go!
We left the hospital and Ava became a very different child. She grew stronger and started loving life. We still protected her from people and germs but she did more things than ever before. She began eating more. We were using her g tube to feed her while she slept to add more calories to her diet.
We took her to check-up with her surgeon every three months, then every six months. Our last visit we heard an amazing thing... ONE year visit. I was so happy. Ava has a tough time gaining weight but they were impressed at how well she looked.
Ava got a CT scan in June '10 to check her insides. All looked great. I needed to know things were good in there.
I changed her g tube on June 31, 2010 and couldn't get the new one back in the hole. I called the doctor and they wanted her to come so they could try to get it in. They worked an hour and a half with no luck at all. We again assumed it was her bosies way of telling us it was done with the tube.
Today (July 17, 2010) Ava is doing well. She weighs 30 pounds and does it all. She loves to run. She peddles her bike and loves eating ice cream. She adores her sisters, Lexis and Emeline. We can't imagine our lives without her.
We love you Ava!
What I have learned through Ava.
~ To be her voice. I had to speak for her. I was her advocate. Which saved her life MANY times.
~ Lots and lots of medical things. I had to become a full time nurse.
~ That there are so many wonderful people out there. Ava supporters helped us.
~ To NEVER take life for granted.
~ Tell family and friends you love them!
~ To keep HOPE alive!
Thanks for taking the time to read our story. For more information and current updates about Ava you can visit http://avaslifewithcdh.blogspot.com or http://cdhawarenessvideos.blogspot.com or www.carepage.com (avahelmick)
~Terri L. Helmick