Wednesday, March 20, 2013

Justus' Story

From Papa Aaron;

My little Justus was born with with a severe right side CDH in 2005. It was not diagnosed until birth, in an inadequate hospital in Alaska. With a zero percent chance of survival, and after the bad weather cleared .... 18 hours after birth.... He was life flighted to Emanuel.

My wife, stuck in Alaska with PIH was unable to fly to Portland and unsure if she would ever hold her baby alive.

He was pre ECMO and was scheduled to have the procedure on a Wednesday night, as I lay in my bed in the ladybug room at the NICU... That night, God touched my little man and stabilized him. No ECMO was needed.

My wife arrived at day 7, he underwent surgery on day 9 with Dr. Davis Bliss.

Three months later he went home to Alaska. Six or so surgeries later, many hospital visits and a few failure to thrives...

In May 2010, we moved to the Portland area where I continued my career. The move was to get him closer to medical resources. 

He just turned 8 on March 13, 2013.

Monday, March 4, 2013

Grace's Story

Our sweet Grace was born two days after her due day on November 15, 2001.  After much ado with her heart rate dropping due to the umbilical cord being wrapped around her neck, she arrived with a loud cry!  I’ll never forget as the nurse handed me my sweet baby girl and reassuringly said, “Look at her she’s pink and healthy”.  Little did we know our Grace would show signs of distress at her two week check-up with labored breathing, a heart murmur, and a coloboma in her left eye.  We were immediately sent to Emanuel hospital in Portland, Oregon where we were rushed into ICU as Grace’s coloring looked much more blue than pink.  Later that day, it was determined after running many tests that Grace had a Congenital Diaphragmatic Hernia.

 Looking at the x-ray with the surgeon, my husband and I felt somewhat relieved that it wasn’t more serious.  After all people get hernias everyday and have surgery and then are just fine- or so we mistakenly thought.  You see, we had no idea how serious CDH is and further, had no idea the problems that seem to follow these little ones long after their initial hernia repair.

Grace was one of the lucky ones. She sailed through the surgery and was out of ICU and off oxygen just days after her operation.  The surgery had gone very well and we were home in less than one week.  What a relief to have it behind us and I, her mother, felt she would thrive at home.  The difficulty began with feeding her.  She would fall asleep within the first five minutes of nursing, had difficulty gaining weight, and it started to appear as if she was uncomfortable as she would squirm and arch her back.

We were referred by our pediatrician’s office to a developmental pediatrician.  She observed Grace nursing and suggested a NG tube because she felt she wasn’t strong enough after surgery to do it herself.  She wouldn’t take a bottle so there was no other way of increasing her calories.  We were very hesitant to pursue this, but it was becoming apparent that she needed some type of supplementation and we felt that if she put on some weight she might be strong enough to nurse on her own.  Grace had the NG tube for about six weeks.  We tried to remove it once but she had difficulty gaining weight.  She started to arch more during her feeding and spit or vomit frequently.  To our surprise, the developmental pediatrician seemed to have no explanation for this and instead referred us to a gastroenterologist for a gastrostomy tube (G-tube).

We were stunned.  What did she mean she didn’t know?  It was second opinion time.  In the meantime, it was evident Grace needed the G-tube although we had no idea what the diagnosis for needing it was. It was apparent she couldn’t take in enough calories on her own and it was explained to us that prolonged use of an NG tube could cause oral aversion. We decided to schedule the G-tube procedure while awaiting a second overall opinion with a gasteroenterologist connected with a feeding team that consisted of a dietician, speech therapist, and nurse.  It was there that we finally got the answers we were so desperately searching for.

“Reflux is common in CDH kids”, the GI doctor explained.  A barium swallow test confirmed the reflux and medication was prescribed.  A malrotated bowel was also observed and was perhaps contributing to Grace’s rather bilious vomit.   The GI doctor also felt the malrotation might be why the medications for Grace’s reflux seemed to be insufficient and referred us back to her surgeon.

While malrotation isn’t always corrected in CDH kids, the Surgeon believed  it might make a difference in Grace’s case. He went on to say that we wouldn’t know how much malrotation was contributing until it was corrected and rather than correct the malrotation and do a Nissen/fundoplication to correct reflux it seemed more logical to take it one step at a time.  

We agreed.  Grace was completely miserable in the month leading up to surgery.  She was now crying at every feeding.  Nighttime was worse.  She would cry and vomit for hours until an exhausted Grace would finally fall asleep only to be awakened when the feeding pump turned on for her nighttime drip.  We thought the surgery would help her; and it did for a time.  Unfortunately, reflux was the root of the problem.  We struggled over the course of a year counting calories and ounces and doing almost daily weight checks.  We wondered how many times one should try to put the same feeding Grace had just thrown up back through her tube.  She was no longer vomiting an ounce here and there, it was three or four ounces sometimes four to five times a day and even in the middle of the night.  Weight gain was an ongoing issue. All the while we kept in touch with our GI doctor and feeding team trying every possible suggestion.  Ultimately, Grace was maxed out on three different types of medications to control the reflux that were giving her little, if any relief.

Working with a GI doctor, dietician, and speech therapist, we had explored every conceivable medication and feeding approach for Grace.  Nothing seemed to make a difference.  Grace still threw up.  It was becoming a consensus that reflux was not something Grace would outgrow.  She was a severe case.We stuck to a strict feeding schedule but Grace would still lose weight.   If we went out to dinner and Grace was sitting upright she would likely vomit due to pressure on her abdomen.  We had to be very careful to not expose her to public places because she would invariably catch a cold which would make her reflux even worse.  Our little girl who I was so sure would thrive was now considered “failure to thrive”.

Reflux was making it impossible for her to lead any kind of a normal life and hindering her development.  As a baby Grace never crawled.  She never had the upper body strength because if we put her on her stomach she would throw-up.  Instead she pursued walking to get upright as she gradually gained the strength to pull up to stand.  She finally walked at fifteen months.  Walking was a huge milestone for Grace but a dangerous one as she wouldn’t bend at the waist to sit down.  Instead she would fall flat either forward or backward and falling always meant throwing up and of course possible injury.  It took her almost two years to get into a sitting position from lying down and it was almost another year to get from sitting to standing.  Looking back now we realize she simply lacked the strength.  It broke our hearts to see her not be able to participate like most toddlers in daily living.  Her older brother who was only three at the time was also in tune with her.  We would be trying to give her a bolus through the pump and I would get up to answer the phone and he would say, “Mom, there she goes again.” And Grace would be throwing up.  Our GI doctor referred us back to her surgeon to discuss a fundoplication,(the surgical correction for reflux). We researched the Nissen/fundoplication surgery and educated ourselves on the procedure as well as the post-op risks and long-term outcome.  We wanted to do whatever possible to avoid surgery.  However, we knew our little girl was completely miserable and the surgery, although with a definite down side, would eliminate the reflux. 

Even though the surgery might create new complications, we were ready to face them and work on solutions to what we hoped would lead to a better quality of life for our daughter. We already knew the surgeon would be opposed to the surgery, but since she hadn’t gained any weight in almost a year he reconsidered.  We proceeded with the surgery after careful consideration.  The fundoplication was done laproscopically and thereby shortened Grace’s recovery time.  We were out of the hospital in just a few days.
Once at home, Grace began to experience one of the most common and dreaded side effects of fundoplication – retching.  Grace seemed to do okay with her feedings at first, but had begun to wretch within days of being home.  While it was wonderful to see her keep her feedings down, they still seemed to cause her great pain.  The retching was almost worse than the vomiting because she would strain until her face turned purple oftentimes breaking blood vessels on her face.  We tried medications suggested by her GI doctor which provided little, if any, relief.  We also experimented with different formulas, but she still retched constantly until it was just a part of her feeding routine. We were at the end of our rope and felt there had to be someone, somewhere, who knew of a solution to the constant retching. 

We decided to take an aggressive approach to the problem.  The question to Specialists became “If not you, than who?”  To be more specific:  If one professional didn’t have a solution, who else could they recommend?  We asked every medical professional we came in contact with and some others we barely knew.  We also utilized the Cherub’s (Congenital Diaphramatic hernia Association) database.  We emailed all parents whose kids had the Nissen/fundoplication surgery asking two simple questions:  Did your child retch, and if so, what did you do about it?  It was in one of those emails we finally found  some answers.  Several moms spoke of a Speech Language Pathologist named Polly Tarbell who they credited with helping their kids to become oral feeders.  Polly was a part of a feeding program at Kluge Children’s hospital and Rehabilitation center in CharlottesvilleVirginia called the Encouragement Feeding Program which boasts a 75% rate of getting kids off their g-tubes.  We set up a time to talk over the phone. 

Polly made two suggestions that made an immediate impact on Grace’s retching.  Polly felt Grace was suffering from intolerance to formula and suggested a blended diet for Grace that consisted of things she would normally be eating at this stage of her development.  She also recommended a drug called periactin that helped with mucous secretions and stimulated a feeling of hunger.  Both suggestions worked immediately and Grace was like a completely different child.  We worked with Polly for four months over the phone and email getting Grace ready for the program. Getting ready for the program required getting Grace’s feeding schedule arranged so that she would have more normal hunger/full stomach cycles.  No more nighttime bolus as that is not what is normal for an oral feeder.  It all made sense.  Grace had to learn how to regulate her own hunger cycle,  which meant we had to allow her to get hungry and resist the natural urge to feed our child.  Letting go and letting Grace do it herself after such a long struggle with counting calories has been the most difficult part of the process,  but we believed in the program at Kluge and we had grown to trust Polly.  It was all up to Grace now.  When we began the two week program Grace ate nothing by mouth.  By the second day she was eating 30% of her calories by herself.  Like learning a new skill, eating had become her new favorite thing to do!  By the end of the first week Grace was taking in 80% of her calories by mouth!

Grace is now a healthy, happy 11 year old!

Tuesday, August 7, 2012

Owen's Story

"My husband Danny and I found out that I was pregnant with our little “Peanut” on July 17, 2006. I had some unusual bleeding at the beginning of my pregnancy, but numerous ultrasounds confirmed that our little one was growing and healthy.

As with my first pregnancy, I was diagnosed with gestational diabetes. Fortunately, I was able to manage it very easily and it didn’t impact me too much except for my belly growing larger than normal. My pregnancy continued and was rather uneventful for the next couple of months, in November we found out that we were having a boy and we named him Owen.

Our story took an “exciting” turn in January. I went in for my 32-week ultrasound to measure the baby to make sure that he wasn’t growing too big due to my gestational diabetes. His measurements were fairly normal in the ultrasound; I on the other hand was measuring big. At 32 weeks my belly was measuring at 40 weeks or full-term. The ultrasound technician discovered that this was due to polyhydramnios, or excess amniotic fluid. My OB checked with our local high-risk OB and he suggested that I come back in 10 days for another ultrasound. By the grace of God we had this additional ultrasound. It was at this ultrasound that Owen was diagnosed with a right-sided Congenital Diaphragmatic Hernia (CDH). Our local hospital is not equipped to care for this type of birth defect. If it had not been detected prior to birth and I had delivered at our local hospital, Owen would have been in respiratory distress and been either life-flighted or taken by ambulance to Portland while I remained in our hospital recovering from a c-section.

My OB immediately sent us to a specialist and they confirmed the diagnosis and said they wanted us to go to Portland to deliver. I did a bit of research but not much and we had consultations with doctors in Portland to prepare us for Owen’s birth. It all went so fast and so slow at the same time. I had those few weeks to mourn the birth that I had hoped for.

Owen’s hernia was missed at multiple ultrasounds. I believe this was because his hernia was not as severe as most. It was not as easy to see because he did not have much of his abdominal contents in his chest cavity. Owen had bowel in his chest cavity and part of his liver, which also acted as a plug in the hole so that more could not pass through. His heart wasn’t impacted nearly as much as it would have been if the hernia were on the left side. We would not know what his lungs looked like until he was born and had his repair surgery.

On Tuesday, March 6, I went in for my C-section. There was a chaplain that came and prayed with Danny and me and for Owen and then I went into operating room. I didn't think I would get to see Owen after he was born but the doctor made sure that I did. He didn't cry, he just gurgled and he was very blue and then they took him away to the resuscitation room next-door. Danny was able to go with him and go back and forth between the two of us. The team of doctors and nurses immediately intubated Owen and sucked out any fluid in his stomach and put a line in his umbilical cord.

I vividly remember the day of his surgery, which was Friday, March 9. Again, there was a chaplain there and she brought my family in to pray over Owen before his surgery. She had me place my hand on his forehead and then Danny's hand on mine, and my sister's on his, my mom's, the nurses, his anesthesiologist and she gave the most beautiful blessing that I can't remember a word of. We waited in my hospital room and there was someone in the operating room that called and told us how things were going in the surgery. Then, when we thought we couldn't wait anymore, the doctor walked in and he had the pictures from the surgery and told us how it all went and when we could go see Owen. Owen did amazing and they were able to repair his hernia (a 5 centimeter hole) laproscipcally. He only had four small incisions, 3 that were 3 mm and one that was 5 mm. We found that Owen’s left lung had developed normally and that his right lung was 2/3 normal size.

On Sunday, March 11, less than 2 days, later he was off of the ventilator. That same day, they slowly started giving him food for the first time through a feeding tube down his throat. After Owen was removed from the ventilator he was put on a machine called c-pap, which provides positive airway pressure to help him breathe.

On Tuesday, March 13, one week after Owen was born I was finally able to hold him for the first time, once his chest tube had been removed. On March 17, his c-pap machine was removed.

We had been warned that the feeding would be the longest, most frustrating part of this ordeal and this was true. In the beginning you survive on adrenalin. Each day is literally a fight for life. Because he was intubated for almost a week and then tube fed for another week, Owen basically forgot how to eat on his own. He had to “remember” how to suck, swallow and breathe all together. It takes a lot of coordination and effort. For a baby that has been through major surgery and that has been on different medications like morphine it is a struggle, especially when there is also a lung deficiency. The whole process was exhausting.

Several of Owen’s nurses told us that we just had to be patient and wait for Owen to “get it”. One day, nearly two weeks later, it just clicked and Owen finished every bottle one day. His doctor told us that if he kept it up for another 24 hours we would get to go home on Saturday, March 31.

In the first few months at home we dealt with a variety of issues; reflux, chronic constipation, a gross-motor skill delay (likely due to spending a month in the NICU), and asthma whenever he got sick.

Now at 5 years-old, Owen is simply amazing. He still has asthma and he takes his asthma medicine through a nebulizer twice a day. With each cold he ends up struggling with his asthma. He is so much better than he was a year ago, but we still have to be very careful about trying to keep him healthy as each cold seems to be very challenging for him. Owen is very tiny, but getting a handle on his asthma medicine has helped him to gain some more weight and grow a bit taller as he isn’t burning so many calories just to breathe.

Owen is in a pre-kindergarten class this year and will start kindergarten in the fall of 2012. Owen is a bright and happy little boy with a kind spirit and an infectious smile. He has blessed our lives in so many ways and has made our family complete."