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| Riley, 2009 |
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| Riley and Hayley |
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| Riley (2/2006) |
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| Riley (2/2006) |
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| Riley (2/2006) |
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| Riley in the NICU (2005) |
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| Riley 2-1/2 months |
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| Riley the night of his unifocalization surgery |
We began going for monthly ultrasounds to check the kidneys, and at the 29 week visit, the doctor informed us that he could not locate the baby's pulmonary artery. He sent us to CHOC (Children's Hospital of Orange County) for a fetal echo, but told us not to worry too much because the baby was probably just positioned in such a way that it was difficult to get a clear view of the heart. We went to CHOC for our fetal echo on my birthday, and We learned that day that our baby had Tetralogy of Fallot with Pulmonary Atresia and Major Aorto-Pulmonary Collateral Arteries. We were absolutely devastated. From that point on, we stopped going anywhere near the baby sections of stores and began to talk about life as if our baby would not make it. As if this would actually protect us from the immense pain that comes with losing a child. My prenatal care was switched to the high risk OB at St. Joseph's Hospital, and the baby was to be born there and moved immediately into the NICU at CHOC. We were informed that the baby most likely had some sort of syndrome. The doctor performed an amnio which came back normal, but we knew that there was a good chance that our baby had DiGeorge Syndrome (babies with DiGeorge often have TOF), or some other syndrome. The FISH for DiGeorge would be performed after birth. Knowing that if the baby lived, he would most likely require a unifocalization surgery, we began to search for information on the best hospital and surgeon to take him to. We decided prior to his birth that if he lived, we would take him to Dr. Frank Hanley at Stanford (Lucille Packard Children's Hospital) for his TOF repair/Unifocalization.
I began going to fetal monitoring twice a week to check the AFI and to monitor the baby's heart rate. Two weeks before my scheduled C-section, I woke up and realized that my water had broken. I began crying hysterically, as I was not ready to give up my baby. We rushed to the doctor and he, being one of the elders at our church, led us in a prayer, telling us that our baby's days were already numbered and that whatever happened was in God's plan. That afternoon, Riley came into the world, pink and screaming. It was the most incredible moment of our lives. The doctors gave him to Brad and let me touch him and kiss him before he was whisked away to the NICU for tests and observation. Our little miracle was able to breathe on his own and was doing quite well given the circumstances. After a cardiac catheterization, we were informed that he would not need any heart surgeries until about 6 or 7 months of age. It appeared as if his collaterals were providing him with just enough blood flow to the lungs at this point. This was great news.
During Riley's stay in the NICU, we received two more crushing blows. First, his supposed good right kidney was not working well and he would most likely need to be placed on dialysis, until about the age of 3, when he would need a kidney transplant. Second, he did indeed have DiGeorge Syndrome. At this point, nothing the doctors told me had much meaning as long as my child lived. I had fallen so in love with him that I felt I could deal with any medical problem.
Just as he was getting ready to go home, when we finally began to allow ourselves to buy baby items, Riley's sats began to drop into the 50's and he began to look sicker. The cardiologist performed an echo and informed us that what he thought could not happen, had happened. Riley's main collateral was getting smaller and he would need to have a shunt placed prior to going home. This would normally be a fairly routine surgery, but with a baby as sick as ours, with so many medical problems and such small pulmonary arteries, it would be very risky. The decision was made to transfer Riley up to Dr. Hanley at Stanford via life flight that weekend. He never made it there.
Two days later, as we were preparing to go to Stanford, we received a phone call from the head of the NICU at 3:30 in the morning informing us that Riley was struggling to breathe and was being intubated. We rushed to the hospital and When we got to the bedside, Riley was already intubated and his sats were falling into the low 20's. The doctors were bagging him and giving him dopamine in efforts to keep him alive. Nothing can ever prepare a parent for an event like this. The cardiologist informed us that Riley would need an emergency central shunt placement. We were told that we should spend a few minutes with him, as he may not make it through the surgery, but that if he did not have it immediately, he would definitely not survive. We walked to the operating room with our baby and kissed him goodbye, thinking that this was the last time we would ever see him. After an excruciating 4 hours, Riley's wonderful surgeon, Dr. Mark Bleiweis, came out to inform us that the surgery was successful and he was able to save Riley's life. Riley was taken to the PICU to recover, and this is where I lived for the next three weeks. He came through the surgery and then had another surgery two weeks later to have a dialysis catheter and g-tube placed. We were told that he would need a g-tube because babies who are on peritoneal dialysis often stop eating. He was to start dialysis the day after discharge, but by some small miracle, his creatinine began to drop the day before he came home. He was discharged on 12 meds and has been home for 8 weeks now. He has thankfully not needed dialysis yet, but his catheter will stay in until his TOF repair, as being on the heart/lung machine often decreases kidney function.
Having Riley at home has been such a miracle, and we thank God for every day. He recently started smiling and is the sweetest and bravest boy we know. He deals with terrible reflux and is still very small, but he is now gaining weight fast. His TOF repair/unifocalization has been scheduled with Dr. Hanley in Stanford for January 4, 2006, but we have recently been told that a full repair may not be possible at this time due to the size of his branch pulmonary arteries. Right now, we pray to God during every free moment of the day, that Riley will make it to his next surgery, that he will make it through his next surgery, that he will grow up here with us, that God will help the doctors save his life for a second time.
February, 2006
We took Riley to CHOC at the end of November for his cardiac catheterization and the results were wonderful. His true pulmonary arteries had grown much larger than his cardiologist had expected as a result of the central shunt placement. We took Riley to Stanford on New Year's Eve and he was admitted to Lucille Packard Children's Hospital on January 3. Dr. Hanley was able to perform a complete, one stage repair/unifocalization on Riley and the surgery was a success. I was a complete wreck and was convinced that my baby would not make it, but Dr. Hanley said that he never had any doubts.
Riley did extremely well and was discharged in 2 1/2 weeks. The care that he received at Lucille Packard was absolutely terrific. We would like to thank all of the staff at LPCH for taking such good care of our child, especially Dr. Frank Hanley for the exceptional job he did at repairing our baby's heart, and the head of the CVICU, Dr. Roth, for his wealth of knowledge, caring demeanor, and endless patience with a crazy, worried mother.
Riley has been admitted to the hospital several more times since his surgery due to GI illnesses/stomach flu's, and during the most recent hospitalization, I am happy to say that he was finally able to have his dialysis catheter removed. The heart surgery did not cause any decrease in kidney function. Since he has come home, Riley is like a brand new baby. He smiles and laughs all day and is much more active than he was prior to his heart repair. He is the strongest and bravest boy we know and though he may only be a mere 12 pounds, he is and always will be our hero.
July 2009 Update
I have received several emails over the last few years from people
who have read Riley's story. After getting the latest inquiry about his
progress, I finally decided to take some time to post an update. A lot
has happened since the last update.
In September of 2006, we took Riley back to Stanford for a heart
catheterization and to begin the process of his kidney transplant
evaluation. As a result of the heart cath, Riley was diagnosed with
pulmonary hypertension, and the doctor discovered that his pulmonary
valve was leaking very badly. In fact, it was almost non-functional.
At this time, blood tests revealed that I was a good match for Riley and
could donate my kidney to him, but Dr. Hanley decided that his heart was
not in good enough condition to handle a kidney transplant. He was
placed on sildenafil for his pulmonary hypertension and sent home. In
December of 2006, while Riley was being evaluated for placement on
dialysis, his condition became critical. He quickly became very fluid
overloaded and was having a very hard time breathing. He was admitted
to CHOC and was transferred to UCLA a few days later for emergency
placement on dialysis. His cardiologist a CHOC felt that he was just
too medically complicated and should be transferred to a hospital with
more advanced medical treatments. He started hemodialysis and was
stabilized. Over the next 2 years, his pulmonary pressures returned to
normal as a result of the sildenafil. He returned to Stanford in April
of 2008 for a pulmonary valve replacement with Dr. Hanley. Following
this surgery, he was deemed to be eligible for a kidney transplant.
Unfortunately, new blood tests showed that, as a result of the 15 blood
transfusions that Riley has had over the years, he has developed
antibodies against me. So I can no longer be his kidney donor, as he
would reject my kidney. His dad cannot donate, as he was also born with
renal problems. As it turned out, blood tests also revealed that Riley
had developed antibodies against 100% of the population. At that time,
he was not transplantable. Treatment was started to temporarily
decrease his antibodies. This was successful in decreasing the
antibodies, but not against me. Riley was supposed to be placed on the
transplant list following this treatment, but in September of 2008, he
began a course of 8 blood infections in a row from his dialysis
catheter. He was very sick for a long time. He was on 8 rounds of
antibiotics and had his catheter replaced 3 times, but the infections
kept coming back every time the antibiotics were stopped. The decision
was finally made to switch Riley to peritoneal dialysis at home, in
order to give him some chance of remaining infection free and getting a
transplant. This type of catheter does not sit in the bloodstream.
Everyone was hesitant about doing this type of dialysis, as it had made
Riley very sick in the past. He is currently doing well and was finally
placed on the transplant list a few weeks ago. He was made top priority
because all of his major veins are occluded and this new dialysis
catheter is his only lifeline. We pray that he gets a kidney soon, as
we cannot imagine how we would survive if we lost him. In addition to
his heart and kidney issues, Riley has also suffered from SEVERE cyclic
vomiting for his entire life. This was recently brought under control
with the use of Neurontin. It is our miracle drug.
In December of 2007, Riley was joined by a beautiful baby sister named
Hayley. She is thankfully very healthy, and oh, what a difference it is
to care for a healthy baby!
With regards to Riley's development, he is quite delayed. At almost 4
years old, he still has no speech and communicates through sign language
and pictures. He has been very slow to learn signs, but he is smart.
He can point to anything we ask him to when he has pictures to choose
from, and he is quick to pick up new pictures. He is very vocal, and
understands everything we say, but just has no real words or functional
sounds. In addition, he is still not walking more than a few steps on
his own. His feet turn completely outward when he stands, so that he is
standing mostly on his inner ankles. He has foot braces and a walker,
and gets around quite well as long as he has something to balance
himself on. At a recent visit, the orthopedic doctor said that he will
probably need surgery on his feet and ankles when he gets older.
Despite these issues, he is completely unstoppable and is always on the
go. He also has a submucosal cleft palate, but the craniofacial doctors
do not want to repair it yet, as he has been through so much already.
Riley is in the special needs preschool program and has a teacher, OT,
and PT, who come to our home 5 days a week.
Having a child with so many special needs is very challenging, but also
very rewarding. This child is the strongest, bravest soul that I have
ever been blessed enough to have in my life. He is so special and
loving. He is constantly giving kisses and hugs to everyone he meets,
even doctors. He loves attention and praise, and his smiles and
laughter still bring me to tears. We would greatly appreciate it if
anyone who reads this could add our little boy to your prayers.
October 2009 Update
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| Riley, post-kidney transplant |
Once again, I feel the need to express how amazed I am by my brave little boy. He is the strongest, bravest, most incredible person I know. How he can suffer so much and still come out of it just as happy and loving as he was before is a mystery to me. He came home from this ordeal smiling, laughing, playing, and loving even more than he did before the transplant. We are so blessed to have him in our lives and I hope that sharing his story can inspire others.
