Wednesday, October 19, 2011

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

Tuesday, October 4, 2011

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