Featured Child~ Benjamin

Benjamin Adam Walker was born October 2007 after an uneventful pregnancy, by planned c-section at thirty-eight weeks due to him being breech. Full term he weighed 3 lbs 11 oz and was in respiratory distress. Since it was a planned C-section there was a NICU nurse there who got him quickly to the NICU and on a ventilator.

Within an hour we were told his diagnosis. Left sided Congenital Diaphragmatic Hernia. We had never even heard the term before, and now our son was born with it. His liver was the only organ that was not in his chest. He struggled to breathe, as the Drs struggled to stabilize him, his blood gases were very high, over double what would be considered safe to operate. When he was 36 hours old, we were told that there was no more they could do at this hospital and he needed to be flown to CHoP where they had an ECMO machine, and that would help stabilize him and give him a chance to have the surgery. There was a bed available for him, and they were arranging for the helicopter to come and get him. We live about an hour and a half from Philadelphia, so we began making arrangements to get me checked out of the hospital and get ready to go.

Ben had other plans. They had to take one last blood gas to give the flight team the most current numbers. Those numbers came back almost half of what they had been, and the surgeon felt confident he would be better off staying where he was and having his repair surgery there. So we stayed.

The next day, he had the surgery and we were cautioned that he was not out of the woods, and that he still had a long road ahead. In the NICU he needed 3 blood transfusions, dozens of x-rays, 2 weeks on a vent, 2 weeks on cpap, a week with a nasal canula, IV feeding, NG tube, a chest tube, MRI’s and countless other tests and procedures. We were able to hold him at 12 days old, and we did as often as we could.

He struggled, but made good progress, and was eating out of a bottle by 5 weeks old. He spent 6 weeks in the NICU and went home on only an apnea monitor, and a calendar full of Dr appts. His left lung did not form correctly and he had a difficult time fighting germs, so his first year he had limited contact with other people. He needed to be followed by a pulmonologist, optamologist, his surgeon, a neurologist, a cardiologist and of course his pediatrician.

For the first 10 months he did fairly well, though he remained small and growing slowly. By 10 months he had completely fallen off of his growth curve, and we started seeing a Pedi GI. By 13 months, he was diagnosed Failure to Thrive (a diagnosis that just about crushed me), severe food allergies, Reflux, and Eosinophilic Eoshphagitis. He was placed on an elimination diet in which he was only allowed 4 foods. Chicken, sweet potato, prunes, and barley. Elecare was and is his main source of nutrition. He had a difficult time feeding and vomited upwards of 12 times a day, everyday. By the he was 19 months old he was admitted for placement of an NG tube, and he had that for 5 months. He began to grow, and he even started to get hair! He however, still was vomiting all the time and could not eat anything beyond Elecare.

We saw several GI drs, and they all said the same thing. He has allergies, EE, and Reflux. The vomiting is fine. They prescribed zantac over and over. It didn’t help and I stopped filling the rx. At 2 ½ he was admitted for placement of a G-tube, repair of an umbillical hernia as well as surgery to move his testicles down, as they were too high.

Within a day of the surgery he got very sick. He had a thick tube placed in his nose and down his throat, and that and the G-tube were used to drain green billious from him constantly. After 2 days of him getting sicker, he had a bowel test and it showed a complete intestinal block. It was 10pm by the time we got the results and the surgeon said the surgery had to be done NOW, so he went into Emergency surgery late that night.

We were warned just how serious it was and he may lose part or all of his intestines. The surgeon that performed the surgery was the same that did his orginal CDH repair. The block was from his intestines adhering to his synthetic diaphragm and slowly cutting it off. The surgeon said the biggest indicator we would have had to a problem was he would have had intense constant vomiting.

HE DID. I TOLD EVERYBODY. NOBODY LISTENED.

He suffered for a year and a half, he almost lost his intestines and he almost died because every doctor we saw was quick to say he vomits because he had reflux.

After that surgery he did well for a few days, but then again became very sick. His incision was red and swollen and obviously infected. By this time his surgeon had left the country on a mission trip and there was no covering pediatric surgeon. The only other in the county was at a hospital about 10 miles away, so after 10 days in the first hospital he was transferred by ambulance.

He needed ANOTHER surgery to open the incision, clean it, pack it and get antibiotics in. He was in that hospital for another 3 weeks, and was put under daily for the first week, and every other day after that to clean the wound and repack it. He spent all of that time on round the clock antibiotics to get the infection under control.

The would was left open to heal and we cleaned it at home and kept it covered, and him away from other people again for another 2 months so he could recover at home. It took 3 full months for his body to heal from that ordeal on the outside and much longer for him to get past it, though I think he will always carry part of that trauma with him. To this day I can not take him to the dr without giving him a complete run down of what is going to happen, why we are going, and what we are going to talk about.

Today he is a relatively healthy 4 ½ year old who keeps us laughing, keeps us busy and makes us grateful for everyday we get. He still suffers from food allergies and EE, so there are not many foods he can have, and he will likely always depend on Elecare to thrive, but he is THRIVING. He is now on the growth charts (at the bottom, but on there!) He needs 2 to 4 breathing treatments a day, is on several medications for the EE, reflux and seasonal allergies and is G-tube fed at night to help him get the calories he can’t take by mouth. He drinks the Elecare well but simply can not get enough in by mouth to grow.

His lungs are still fragile, and it takes him weeks to get over a “simple cold”. Some days are great Some days are incredibly hard, and we live with the constant fear that his patch could break, but we pray daily it does not happen.

~Katherine Walker

Featured Child- Elijah

Elijah

Featured Child~ Sydney

Sydney’s Story

I was 34 when my husband and I decided to have another child. We already had a 12 year old son, a 9 year old son and a 9 year old daughter. After about 2 months of trying I found out I was pregnant. We were so excited and the pregnancy was just so perfect. At 19 weeks pregnant my OB sent me to another hospital that had a level 2 ultrasound because of my age. We found out that we were having a baby girl, which would round out our family very nicely. After several minutes the ultrasound tech went to get the doctor. After several minutes the doctor told us that our daughter had Congenital Diaphragmatic Hernia. He quickly explained what it was and in the same breath told us that it was going to be a very long road and since we had three healthy children we should “bail out”. We decided to have an amino then and there. As we were leaving the doctor told us that Children’s Hospital of Philadelphia (CHOP) would be the only hospital in the area that could help her.

I called CHOP as soon as I got home and made an appointment for the following week. During that week I did nothing but surf the internet for anything on CDH. I read many blogs and medical journals. Most of the information that I read was horrifying! After spending six hours at CHOP having a fetal MRI, Echo, and extensive ultrasound we met with an OB and a general Surgeon, DR. Flake. We were told that our baby had a RCDH and it looked as if most of her organs were in the chest. The said that her HLR was .62. I knew from all of my research that anything under 1.0 was not good at all. Dr. Flake told us that she had at best a 10% chance of survival. We asked what her quality of life would be if she survived and what they would do if this was their child. Of course they could not answer, but they did say that we still had a small window of time if we decided to terminate. On the way home, my husband asked what I wanted to do. I just could not make that decision. Gary said “If God wants her, then let him take her”. This was the start of our incredible journey.

Sydney was born on Nov 5, 2008 at 36 weeks via C-section due to my high blood pressure. Six hours after birth she was placed on ECMO.  We were told on a daily basis that they did not know if she was going to make it. At 9 days old they decided that it was time to do the repair.

Sydney was missing the right side of her diaphragm. Her liver, stomach, intestines and gallbladder were in the right chest. Her heart was pushed to the far left wall of her chest. The right lung was about 20% the size and the left was about 75%. Because of the way the liver developed, it would not fit in its proper place and needed to be manipulated and turned to fit on the left side. Her intestines were not able to fit so she had a silo for 12 days. We were told after surgery that they did not know if the bleeding would stop due to all of the manipulation that the liver had endured. We held our breath as they placed the drains in to see if it was clear or blood. Thankfully it was clear.

At 19 days old Sydney had to come off of ECMO due to a grade 2 brain bleed. When they trailed off the day before she did not do well at all. When we walked into her pod, they had placed the homemade keepsake blanket on her warmer and asked if we would like to “hold” her since we were not able to before. Everyone was convinced that she was going to pass away that day. They Dec annulated and she did amazing. At six weeks we were first able to hold her and at 7 weeks (Christmas Eve) they extubated her and put her on 4 liters high flow nasal cannulas. That was such a wonderful day, our three oldest were able to hold her for the first time. Two weeks later she had to be vented again. She was not able to get rid of the carbon dioxide. Again two weeks later she was put back on high flow nasal cannulas with 5 liters oxygen. She had a very hard time with weaning of the meds while on the oxygen. She was miserable; no one wanted to care for her. All she wanted was for her mommy to hold her.

In March, at four months they decided to do a Nissan because they felt that she was micro aspirating. A month later they decided that the only way that Sydney would be able to do anything besides breath, she would need a trach and vented. When they told me this, I thought it was the end. After two weeks of recovery, Sydney was a different child. We never realized how difficult it was for her to breathe until she was vented.

She soon began sitting up, playing, smiling. She was the baby that god intended her to be. Before we could leave CHOP Gary and I had extensive training in changing trachs, troubleshooting the vent, and suctioning.

We left CHOP NICU after 224 days. She had a trach, was vented for 24 hours a day. She had a G-Tube and received all feeds through it. She was on Viagra for pulmonary Hypertension, Diuril, Potassium, and Bethanical (for reflux), and dual hearing aids.  When we came home she had 16 hours of nursing a day, was receiving PT, Speech, and seeing a teacher of the deaf. Three months after coming home (Syd was 9 months) the docs started to sprint her off the vent. In October of 2010 she came off the vent completely. They took out the track in April of 2011; two years from the time they put it in. We were told that she would probably have the trach for at least 3 years.

Sydney is doing amazing. Her lungs are both growing and functioning well. The right lung will most likely never be full size but she is doing very well with the one lung. She is off all meds except for Xopenex when she has a cold. We still have the G-tube, but she eats!! We still get feeds overnight due to her being only 6% on the growth chart. We are considering weaning off the night feeds this month.

We were told that Sydney would have many delays due to the severity of the brain bleed. When she turned 3 she was tested by the early intervention. She scored right on target for her cognitive, above age level for speech (were told she would not talk) on target for fine motor. Sydney has a slight delay in her gross motor. They feel most of it is caused by fear. Oh and her hearing loss is so mild now that she does not need the aids. She started pre-k this November and loves it. Sydney is truly an amazing little girl that has an amazing will. She knows what she wants and will not stop until she gets it. I am eternally grateful for the nurses, doctors and staff at CHOP and for the entire answered prayers and miracles god has given us.

~Kathy Taborelli- Sydney’s Mommy

Featured Child~ Adam

    Adam’s story begins about 6 weeks before he was born. July 2nd, 2002, I was working for a major retailer as a department manager and stocking shelves in my department. I had just emptied a box of wicker baskets and was bringing them to the shelf where they belonged. Someone (we never found out who) left an empty red plastic pallet full of rain water laying on the sales floor that fateful day, it was better than spilling water all over and making more work for themselves. I came around the corner with my huge seven and a half month belly and six wicker baskets further obscuring my view of the floor and saw the pallet there. I went around it and when I thought I was passed it moved just a little to the left side of the aisle towards where the baskets belonged. I caught my foot on the very edge and since it was full of water instead of being empty like they usually were it was weighted down and did not move. I fell on my belly. A co-worker, I don’t remember whom, passed by a second later and saw me sitting on the floor obviously shaken. My mother, who was working at the same store, was called immediately as was security, for the incident report. I spent the next 24 hours at Lakeview Hospital in Mandeville. Now you may be thinking, “Why is this important to my story?” They did 2 ultrasounds while I was there making sure that my precious boy was all right. They didn’t see that he was not. It was the last chance they had to diagnose him before he was born.

    Adam was born on August 14th, 2002 at about 1 pm at North Oaks Hospital in Hammond, La. Everything was going well except that they were having trouble getting a stable heart beat on him so they needed to place internal monitors to keep better tabs on him. They did not know. When he was born he let out one lusty scream and quit breathing. I have only half joked with pregnant women since then, in telling them that when the population in your delivery room triples you know there is a problem. Adam was whisked away to the NICU where they were going to try to stabilize him and find out what was wrong.

   About 2:30 pm a NICU doctor came in and introduced himself to two very scared and worried parents. Dr. Villalta told us that Adam has a condition called Congenital Diaphragmatic Hernia and his was kind of rare since it was on his right side. Since it was on the right side and the liver was in it’s normal place it was not seen on the ultrasounds, my fall did not cause it as the diaphragm is formed at about eight weeks. Worse news was too come. “North Oaks does not have the facilities needed to treat his condition but when I was in med school I interned under a fantastic doctor who specialized in this kind of situation and his hospital has ECMO which he may very well need,” is what he told us. Adam needs to be life flighted as soon as it can be arranged; we are sending him to Oschner hospital in New Orleans, where Dr. Adolph will care for him.

    Dana left right away with his mother to meet them there leaving me with my mother to comfort me. At about 4:30 they came in with him for me to say good-bye they gave me a Polaroid picture of him and told me how the flight was planned to go.

    Adam was in such a critical condition that they were afraid that altitude changes might kill him so they were planning a flight over Lake Pontchartrain just above the treetops. The 30-minute flight seemed like a lifetime to me.

    The nurses left me in the room he was delivered in so that they could keep a close eye on me, encouraged me too start walking as soon as I could because I was going to need to be able too the next day. Exactly 24 hours after he was born they sent my mom and I on our way.

    It took us forever to get there. My oldest needed more clothes, my 2 iguanas needed to be brought some where because we did not know how long it would be before we came home, mom insisted we clean up the house from 2 days before so that I didn’t have to worry about it when we did finally come home. We had to stop here and there for one reason or another. I swore I was going to go nuts because she had a hundred things to do and I needed to be with my son. I just knew I needed to be at Oschner.

    When we finally got there I was exhausted and my husband insisted that I sit in a wheel chair and he would take me to see our baby. I got there and he called the nurses station to let us in. Adam was on what looked like a padded table. He had a tube from his belly button to a machine; he was on a high frequency ventilator that seemed to bounce him all over. The nursing staff gave me a minute to be with him and then brought all the paperwork they needed to have so that they could care for him, they also called Dr. Adolph so he could explain what was going on and a councilor to help with our needs. That was when we were told about Cherubs, and given our first real information as to what we were looking at.

    Adam was stable enough on the third day to do his repair. He was too critical to move to an operating room because the altitude change may have destabilized him, so they closed the NICU and did his surgery there. We stayed out in the waiting room with all the other parents until one came in upset that they wouldn’t allow her into see her baby since they were doing surgery on “someone’s brat.” I walked out of the room and sat in the hall while other parents berated her for her insensitivity. When the surgery was finally done Dr Adolph came out and told us that all went well.  His hernia was larger than they thought and the patch was sewed directly to his ribs on 3 sides, they had to remove his appendix as it was in the wrong place, and he has a mulitcystic dysplastic kidney that he didn’t know if it was functioning. That was something that could wait until he was stronger to find out since his other one is normal.

    That night we stayed at the Ronald McDonald house on the other end of NewOrleans. We received a phone call at 7 am on day 4. Adam did not have a good night. His respirator quit working and they were unable to get his saturations backup on the new machine. Dr. Adolph thought his best chances for survival laid on a path that scared us to death. ECMO. We consented to the operation and they started right away. By the time we got there at 8 he was on the machine and doing better. For those who have never seen ECMO imagine your precious infant paralyzed with a small clear garden hose coming out of his neck, filled with blood. A machine pumping all of the blood out to oxygenate it then putting it back in. This machine destroys blood cells while doing all this so they are constantly checking his blood, and adding blood parts, 23 units in  10 days in our case. He is on blood thinners to keep it from clotting because a clot can spell disaster, anything from mental disabilities to a killing stroke. Adam spent 10 days on ECMO, on day 7 they decided that he needed a chest tube to drain off the fluid in his lungs. They drained enough fluid to fill a 2-liter bottle.

    We stayed at the hospital hotel, my then 4-year-old daughter was brought to us, and mom made sure we had everything we needed. The hospital provided day care for Skylar so that we could visit Adam and she didn’t have to see him in the condition he was in unless she wanted too. They also helped with food vouchers so we could eat in the hospital for free. They needed us to be close by all the time in case something happened. I will be forever grateful of the care we got while Adam was at his most critical.

    On day 14 he came off of ECMO to our great happiness. Adam was doing much better his O2 saturations were up around 97% and he was on a regular ventilator. They were slowly able to wean him off of it in 11 days. Adam still had a long road though. When they got him off the vent they started feeding him all the milk I had been faithfully pumping because he was going to need it. We hit a roadblock at this point. Adam had developed nipple aversion we think because of the respirator; at first it was all we could do to just get him to suck a pacifier. He also had severe reflux and would vomit most if not all of the milk; his diaphragm was not strong enough to hold down food. Adam needed a fundoplication and at the same time they would place a g-tube so that they could get rid of the nasal canula.

    We did eventually find out that the kidney was non-functioning and as good as dead but did not need removal as it was in a sterile environment and would eventually be broken down by his own body. As long as his health was stable they would leave it alone.

    About a week after the last surgery I was changing his diaper and noticed that he was a bit blue around his genitals. The nurse said it was nothing to worry about that they knew about it and were looking into why. About an hour later we were called and told that they had discovered another hernia and that a loop of his intestines was caught in it. They corrected it with no problems, and we have since joked about him being a holey baby.

    To our great joy Adam came home 2 months and 3 days after he was born, but his story is not over. Adam came home with a g-tube, in home nursing care, an apnea monitor, and 5 therapy sessions a week trying to get him to eat and catch up to where an “normal” 2 month old should be. He was 9 months old when I happily announced to the Cherubs forum (then a Yahoo Group) that Adam took an ounce of apple juice. Within a week we were back in the hospital, Adam was having a major asthma attack. We changed his medications and he pulled through like a champ! He came home after a week in the hospital the day before his first Easter. His g-tube was removed when he was 13 months old.

    Adam had another asthma attack in October of 2007 and we decided that he needed a pulmunoligist. He had not been seeing one before because we were not told he would need one. We did a lung function test and found out that Adam has Interstial Lung Disease probably caused by scarring on his lungs. We don’t know what caused it. He was amazed that we had not been seeing a pulmunologist or a cardiologist since Adam was an ECMO survivor. He suggested we start seeing one right away and that he got an Echocardiogram
done ASAP.

    He was diagnosed with a Patent Ductus Arterosis in December of 2007. PDA is a valve in the heart that bypasses the lungs during gestation. At birth it is supposed to close, Adam’s did not. They decided to watch it, as it was not causing significant problems other than leaving him tired and winded after exerting himself. In January 2011 they decided he needed it repaired. Dr. Lucas at Oschner was his surgeon this time and he was able to repair it out patent! Adam came home that night with strict instructions for the next 2 weeks to take it
easy.

    In December 2010 we he was having trouble urinating so we brought him into the urologist where they did an ultrasound on his kidneys to find out what condition they were in. The non- functioning kidney was not found his body has broken it down and it is gone. His good kidney is healthy, slightly enlarged but that is normal considering his other one. The problem he was having was corrected outpatient later that month.

    In May 2006 we welcomed our third child! Katelyn was born completely healthy. We were so scared of having another child because of the experience with Adam. A huge blessing came from her birth, the ability to relax and enjoy being a mom for the first time. When you have your first baby you worry about every little thing. When you have your second you are a little more relaxed as you have been through all the normal stuff once, that is unless your second has huge problems! When my third came along I finally allowed myself to relax a little and settle into just being mom. I have learned that being carefree for short periods and just playing with them is a huge boost to my spirit! Take a minute and watch your kids play together and play with them! They are fun if you allow yourself to join them for a while!

    We are now in June 2011 Adam is my miracle, my blessing, and my joy. He is an active boy who loves to ride his bike, play video games, and be with his friends. I swear he has no fear. He is entering 4th grade (home schooled with his 2 sisters). This is one little boy that doesn’t stop; he is always doing something amazing. I live in wonder of him as he has faced such giants and beaten them at such a young age. I have told people he is like David, small, strong, and courageous. At almost 9 he weighs in at a whopping 43 pounds and is 3’8” tall. He is skinny as a rail but we praise God that his growth is slow and steady. His pediatrician does yearly x-rays to make sure all is well. We praise God that he has not re-hernated, which is possible with the patch they used and the size of his hernia.

    I would not change the fact that we didn’t know. If we had known we might have been a bit more prepared, we would have skipped the life flight incident, and he would have been born at Oschner instead of North Oaks. Nothing else would have changed. I was blessed in my ignorance, as I am a person who worries over everything and stresses out over nothing. In my preparations and “needing to know everything” the statistics would have had me freaked out and I would not have been able to enjoy the pregnancy and the wonder of life. My not knowing allowed me to relax instead of working myself up into needless worry and endangering my health at a critical time for my son. Instead God allowed me to be in ignorance of the blessing that was coming to me, and of the test of faith He was allowing to happen. Today I am closer to God than I ever was. He is my strength, my support, and my best friend.

~ Lisa Thibeau

Featured Child~ Axael Lyxander

Axael Lyxander was born on December 14, 2010 at 9:58 am. It was a long wait but he was finally here. When i first saw him I was surprised he wasn't crying or moving he was a funny blue purplish color. The doctor tried his best to get  him to breathe but only got a few short low yelp from him. He was quickly wisked away by the NICU team and the got to work. He's not crying I kept telling my husband. My husband just kept his hand on my shoulder looking over at the baby. He was xxrayed and that's when the found out he had CDH and more health problems the main being CDH. Congenital Diaphragmatic Hernia, What in the world is that you might ask? I asked my self the same thing. They gave me no explanation so I took to google.Thank Heaven for Google. CDH is when the diaphram fails to properly form in the early months of pregnancy, causing the organs in the lower abdomen to go into the chest preventing the lung or lungs to  properly grow. He was transferred to Texas Children's Hospital. I couldn't go with him. I was forced to stare at the pink walls in the recovery room and wonder what was going on with my baby. I kept going over the list I read on the website about CDH and what are some of the treatments. The last thing I read was ECMO. I didn't know what it was but it sounded dreadful. ECMO is an extracorporeal technique of providing both cardiac and respiratory support oxygen to patients whose heart and lungs are so severely diseased or damaged that they can no longer serve their function  The next day we were able to be by his side. The doctors and nurse were so nice and explained just about everything I wanted to know about Axeal and his condition. Even though his night was ok it was decided he needed to go on ECMO. My heart dropped as I signed the consent forms. After a few hours he was  successfully on ECMO  and were able to see him. 

The hardest thing a mother would ever have to see is her little day old baby on such a complicated machine. On December 16, 2010, His surgeon though is was the best day to repair him. After more consent forms and hours of waiting the surgery was complete he  was stable. The surgeon found that Axeal had no diaphragm on the right side and his right lung was one sixth the size of the left lung. Axael was 5 days on ECMO. The days of the trail of was such and exciting days. Many people piled in the smallest ECMO room they had. My son, the center of all the attention. It  went well so on Monday December 20, 2011 he taken off ECMO. 

He was a true fighter. In the days after that I was able to do the thing I had wanted to do since I found out I was pregnant: HOLD AXAEL! He was kept intubated for a while and was transferred to the pod he would stay in for the rest of his stay. He was on and off CPAP a few times on and off nasal cannula. 

He was having such bad tet spells (blue spells). Crying and crying turning blue and purple right away and no one knew why. His stats would drop to the mid 20's and he would pass out and recover. In the end it was found to be his heart that was causing the problem. On March 21, 2011 Axael had open heart surgery. He had two holes repaired by the Dr. D. Morales. The surgery took hours. He came out great. After a week in the CVICU he was brought back down to the NICU, to his old spot A7. After a week he was like a new baby. No  more blue spells and just as happy as can be. A swallow study was done and he was found to have silent aspiration. Meaning anything he ate would go into his lung. A big no-no in for CDH babies. A G-button was placed on April 7,2011 and Axael was discharged April 14. 2011. Four months to the day he was Admitted. Very grateful to all the nurses we met while there all the purple pod girls and a few guys :). Especially NP Vanessa Nurse Amy and Nurse Mercy, and of course Dr. Guillory (her dedication and determination in getting Axael his heart surgery was unbelievable, FOREVER IN HER DEBT). Axael will be turning 1 this December 14, 2011. Something I can say at times I didn't think possible. He has been off oxygen since June and is only on one medication. He is on the little side but nothing time and food can't fix. He is now able to wear twelve months clothes. Heck even eighteen month and some twenty-four months clothes. He's about fifteen pounds and is twenty-seven and a half inches long. He had RSV in October. He stayed in the hospital a week and another two days in early November with a little cold. He'll always be at high risk of getting sick, but he's a fighter. 

He bounces back fast. He is sitting up, waving bye bye, shaking his head no no when people say he's so cute, wanting to crawl but not quite there yet. He's just about the happiest baby ever. He is always smiling laughing and in such a good mood 24/7. God has blessed me with a wonderful baby and a great life learning opportunity in being the mother of AXAEL. Also can't go without being grateful to my family, my husband and my four year old son Jando. Jando went thru a lot this past year also and has become the best big brother and baby brother could ask for. I love my boys!


~Amanda Morena

Featured Child~ Liam

Liam was born on July 14th 2011 at 10:59 pm. We were expecting a strong, healthy little boy. When Liam was born he wasn’t breathing. After forcing air into him, he let out one small cry. What I thought was the best sound in the world turned out to be the worst. Liam was born with a condition called Congenital Diaphragmatic Hernia, CDH for short. Meaning his diaphragm didn’t develop properly leaving a hole allowing his stomach, intestines and spleen to go up into the chest cavity. Because of this his heart was pushed over squashing his lungs leaving his left lung under developed. Liam’s case was what they consider mild for this type of birth defect. Babies with CDH aren’t supposed to be allowed to breathe and aren’t supposed to be bagged. They are supposed to be paralyzed and antedated until after their repair surgery. Because Liam was undiagnosed they had no idea that anything serious was wrong. Once they realized what Liam had they had to life flight him to UCSF because they were one of the few hospitals that could help him. Thankfully UCSF specializes in cases such as CDH. The staff at UCSF did everything they could to stabilize Liam. When all else failed they put him on ECMO.



ECMO is used as a last chance scenario because the chances of a baby surviving is so slim. Liam was on ECMO for about a week when he finally was well enough to be taken off. A few short days after ECMO, on July 21st Liam under went his repair surgery.



It was the best birthday gift a mother could ever receive. Not only was it my birthday but the news was awesome. Liam was very blessed. He only had part of his stomach, part of his spleen, and some of his intestines in his chest cavity. His left lung wasn’t as under developed as everyone first thought. We were blessed even more when Liam had enough muscle tissue to close up the hernia without the need of a patch. A patch is a synthetic material like rain coat material they use to patch up the hernia is there isn’t enough muscle or tissue to use. The patch does not grow as you grow so around age 5 a patch recipient will most likely have to have another surgery. Liam was very blessed in not needing a patch. He still has a chance of re-herniation but it’s slimmer than with the patch. After getting extubated, Liam was on C-PAP.



They start with the pressure setting on high and slowly wean down. The first time they excavated him, he turned blue within minutes. After being given breathes and searching for the right face mask for him they finally stabilized him. Unfortunately Liam only lasted two days on CPAP before his right lung collapsed and had to be re-antedated. After two days they excavated him again. For Liam the magic number was "six". His right lung, the good lung, would collapse. After the right lung collapsed the second time they found that his right side diaphram was high and they thought it wasn’t allowing his lung to expand properly. They were thinking he would have to have another surgery to tack the right side diaphram lower. They took x-rays and ultrasounds to find that even though the diaphram is high that it was moving properly not interfering with the lung. Just a few days later Liam’s lung was strong enough to breathe without any problems and they were able to remove the CPAP and put him on a high slow nasal canula. After very slowly lowering the nasal canula, Liam was able to be put on a regular nasal canula which he only had for 4 days. Right before his discharge they did a MRI and found that Liam has “white noise” on his brain that could possibly affect his cognitive and fine motor skills. It’s something that they have to keep an eye on. CDH kids have been known to have both progressive hearing and progression vision loss. On top of that ECMO causes both vision and hearing loss. Even though Liam passed both his vision ad hearing test doesn’t mean he won’t develop hearing and vision loss in the future and because he is both a CDH and was on ECMO his chances are even higher. There’s so much we have to watch for with Liam and God only knows the hurdles that are to come. We have to attend CDH clinics and wellness clinics up at UCSF every few months to keep track of his progress as well as see his local pediatrician on a regular basis. Thanks to the doctors and nurses at UCSF my son is a CDH survivor.

Liam had an 8 day stay at UCSF for an NG tube and additional study on his high right side diaphragm from September 29th to October 7th 2011. We were only home for 2 days before he got sick. We took him to the local Emergency room where they didn't do anything but take an xray. The next day, October 11 2011 we took him to Valley Children's Emergency room where we was admitted and currently there.



He has acid reflux and cought a cold. The cold made the acid reflux worse than normal which cause him not to be able to hold down any food. We learned on our current stay that his good lung is actually half the size of his "bad lung" and his stomach is malrotated but his bowels are normal.



Liam will have to have surgery to repair his high right side diaphram but they would like to wait as long as possible before they do. Ultimately they would like him to gain weight. He is failure to thrive and at the age of 3 months still wears newborn clothes.





~ Aubin, Liam's Mommy

http://www.wix.com/cherubliam/cdhfund

Featured Child~ Emery

The Story of Emery Elizabeth (a.k.a. E3)

My husband & I went in for our 18 week sonogram & took our 3 kids with us, so they could find out with us whether they would have a new brother or sister. "I have girl parts" the technician wrote on the screen – my son, and only boy, sobbed in the corner (he REALLY wanted a brother.) As the scan continued, we were told that our newest little girl had a left-sided Congenital Diaphragmatic Hernia or "CDH". Even after a very brief physical explanation of the defect, we had no idea how this diagnosis would so profoundly affect us. It almost seemed unfair that a 10, 8 & 5 year old had to go through this too.

From Womack Army Medical Center, we were referred to UNC Hospital in Chapel Hill where they would take over my care and be able to handle her much needed intensive care after birth. From there we started our journey with CDH.

Emery Elizabeth was born in October 2009 at 7 lbs 12oz and began her fight. Immediately after birth, she made an attempt to cry, but it was only a muffled squeak and was quickly intubated – that would be the last cry we would hear for a very long time. For the first few hours she seemed to be doing well, but all that would change in the overnight hours. During the night the NICU staff was having increased difficulty in controlling her pulmonary hypertension, so at 5 AM it was determined her best chance was to be put on ECMO. Within an hour, she was moved from the NICU to the PICU for the procedure. Fortunately, she was relatively stable at the time, so the transition onto ECMO was manageable. Emery stayed on ECMO for 15 days – she had 1 circuit change and 2 failed trial-off attempts before she was finally able to be successfully taken off ECMO. The following week she had her 1st surgical repair.

The surgery went well, but we were told her defect was very large. 2/3 of her back-left diaphragm wascompletely missing, she showed some indication of potential complications with reflux and her left lung was about 1/3 of normal size. A Gore-Tex patch was placed. She came back from surgery wide-eyed – thatwas the first time we saw her with both of them open – I cannot explain the flood of emotion that brought us!

Then after 37 days, Emery was taken off of the ventilator and we heard her scratchy cry for the first time since she was born. What a beautiful sound – it still brings tears to my eyes when I think about it. Emery was in the PICU for a total of 45 days before transitioning to an intermediate care unit. Unfortunately, it didn’t last and Emery was returned via Rapid Response call to the PICU where it was determined after ultrasound that she had reherniated. She had her 2nd surgical repair on Thanksgiving Day 2009. After recovering from her second repair, she was moved to “the floor” and activities were focused on getting her to eat and gain enough weight to be able to go home. She had another surgical procedure to place a g-tube and 3 days before Christmas, after 76 days in the hospital, we were finally released to go home!

Emery went home on 1 liter of oxygen & continuous pump feeding through her g-tube. She was scheduled for monthly surgical follow-ups, follow-ups with Pulmonology, referred to Child Developmental Services for Speech/Feeding & Physical Therapy evaluations. At our first surgical follow-up, her surgeon was so excited to see her, and said that (clinically) she looked great, but after a close look at her x-ray we learned that Emery had in-fact reherniated again – and had shown none of the obvious signs. This was at the same time as my husband was receiving calls that he was to deploy with his unit to Haiti to help earthquake victims. The following week, while my husband was in-route to Haiti, Emery & I were at the hospital for her 3rd repair. Her hospital stay was only 10 days this time, but because of her previous experiences it was difficult for the medial staff to manage her pain as she had a very high tolerance to the medications she was given. We had a couple minor scares, but once we made it back out of the PICU and up to the floor, we were able to successfully wean her from oxygen. So home we went with one less tube!

She continued to be monitored by her surgeon, now on a 6 month schedule & we continued our monthly visits to the Nutritionist. She “graduated” Pulmonology follow-ups and Feeding Therapy and evaluations for Physical Therapy have shown us that Emery is on-track and age-appropriate! This is truly amazing considering what she went through. This past November, Emery had a cold and ended up having a Febrile Seizure and passed out – her lips were blue and it was incredibly frightening so we went by EMS to the emergency room where they took a chest x-ray looking for lung-collapse or pneumonia. Fortunately she didn’t have any of those issues, but upon review of the x-ray with her pediatrician, we found she had reherniated AGAIN! She was taken back up to UNC, where they confirmed the defect. This 4th repair, was approached by a cross-functional team of Pediatric & Cardio-Thoracic surgeons since her reherniation was in a difficult spot – adjacent to her aorta, esophagus & spinal cord. Fortunately, it went well, even though we spent the majority of the 10 days in the PICU. We’ve had long talks with Emery that this is NOT going to
become a family Thanksgiving tradition!

While everyone hopes that this latest surgery was the last time, we remain guarded and vigilant when it comes to her care. The issue we continue to monitor most is her weight gain and caloric intake, so we can get her weaned from the g-tube. This has been a very difficult and long road, but one we hope will end soon. We recently enrolled her in daycare a couple times a week in the hopes that peer-pressure will get her eating!

CDH has been a long and rocky journey thus far, a tremendous exercise in patience and a test of faith. We cannot explain why Emery got to be here, particularly given the severity of her defect, in fact we were told long after she was “out of the woods” by her surgeon that he many of the staff in the PICU were terrified because they didn’t think she was going to make it. While that is a scary thought, I am somehow comforted in knowing what a tremendous strength she has and that she had so many people fighting the fight & praying for her.

Now, she is a very active toddler (almost 2) and by looking at her you would never know the struggles she had starting out – until she lifts her shirt to show you her “button.” Her numerous scars left over from multiple surgeries, ECMO, chest tubes & central lines serve as a constant reminder, but these battle scars are ones of courage & triumph, not of pain & struggle. For the most part, we haven’t seen CDH slow her down – my bet is this is a sign of things to come. Bring it on – I don’t believe there’s anything this kid cannot do.

-Shannon – Emery’s Mom