Featured Child~ Andy

 

I found out we were expecting our fourth child in January 2011 at about two weeks. Due to my history, the doctor wanted to see me ASAP. I went in at six weeks along. Everything looked great. Then around eight weeks I became very sick I was in and out of hospital for IV fluids and medicines.

At twelve weeks along, on a Wednesday I had a home nurse come out to show me how to use the Zofran pump. (For those that don't know this is a slow steady flow of medicine running all the time to help with the sickness.) I had lost 20lbs. A small needle was put through my tummy that delivered the medicine.

The next day I was at work. I worked with preschool kids at my church. I thought I had peed myself as it tends to happen in pregnant women. But little did I know what was about to happen. I had started bleeding everywhere. I was rushed via ambulance to the nearest hospital. I thought I was losing the baby. The doctor wasn't sure if the baby would make it let alone myself. I was diagnosed with Subchorinic Hematoma (SCH).

I was followed very closely until my thirty week point by my OB and a specialist. I had two blood clots in my uterus along side of the baby. They cleared up at twenty-eight weeks. I was told all was good the baby was large and growing fine I came off bed rest around thirty-two weeks when I had the finally all clear and my baby boy was doing good. So our fear of premature birth was gone. No nicu stay was going to happen.

We were all good right there right up until inducement day. My husband and I had this really bad feeling. My thoughts were... I don't want to have this baby. Something is wrong. We got to the hospital at 7:00 and he was here at 10:28am weighing 10lbs 4 ounces and 22 inches long. He was blue and not breathing.

No one would tell me what was wrong. They all turned from me and were crying. I had been given the chance to hold him but said no cause I figured I'd get to hold him after he was cleaned up like I did with my others. We didn't know he was sick.

They took him out in the incubator. They tubed him and came in and told me he had aspirated. I was ok with that. I knew what that was and it would just be a couple days in nicu. Then came the bad news, the doctor said "I have some upsetting news, Your baby has congenital diaphragmatic hernia." I said, Do what!!" And then he explained that he had a hole in his diaphragm and his organs weren't in the right spot.

His hospital keep getting changed due to them not having the right equipment like the ECMO. We finally had a hospital for him to go to. The place I delivered didn't even have a nursery for babies. He was transported by the angel team that evening by the time they got there had him all hooked up and sedated. They told me to say my good byes because they didn't know if he would make the transfer or thru the night. I couldn't go because I had just delivered a baby. I told my doctor I wanted out first thing in morning and I was. With a cracked tailbone I made my way down to Children's hospital, a an 2 hours drive away.

Andy was on a vent that was breathing for him and on a lot of pain medicines.

Things are hard to recall everything as I was in shock, mad, upset and in a lot of pain both mentally and physically. I didn't understand why I was told everything was finally ok and it wasn't. From what all I can remember he only had his intestine in his chest he had a 30% hole they said he has stitches mess and the Gortex patch. He had pulmonary hypertension.  He was born on a Monday had his repair on Friday. It took couple hours at least 5 hours cause I had to pump twice why waiting. I was so worried about going to pump in case the call came in and something happened.

All went well he had it done laparoscopically. He has about a three inches scar on his back and a couple small holes under his arm by his shoulder, where he had a chest tube. He had fluid in his lungs it was a nasty color when it drained out. We were told he would come home until Christmas then it was Thanksgiving it just keep going back and forth.

We still had three other boys to take care of and tell them what was going on it was very hard they didn't get to see their baby brother until he was almost a week old after surgery.

We were scared that he wasn't going to make it. He develop some kind if infection so they gave him high dose of antibiotics.

It was a roller coast. His vent would be turned down and he would be doing good. We would get home and call to check on him and he wasn't doing good and had to turn the ventilator back up. I finally got to hold him at nine days old. The next day he came off the ventilator.

Then came the feeds, he wouldn't eat for the nurses but would for me I wasn't allowed to stay with him at night so during the daytime he would take a bottle and at night would be feeding tube.

He was still having trouble with his breathing and heart rate going up when he would eat. After two weeks of feeds and finally gaining weight, they allowed him to breastfeed and he had a hard time with it. He couldn't breath so it was back to the bottle which at that time I didn't care I just wanted him to eat and come home.

Two weeks old they moved him to another room where I could stay with him. He came home right at a month old. He needed no oxygen! He did need reflux medicine and had to sleep upright cause of reflux and sometimes he would stop breathing if laying flat. He develop another hernia which they are watching now closely. He's one and is into everything. We still have problems with him not wanting to eat or choking on his bottles. He has immature swallowing food allergies and they keep finding more things wrong as he grows. He's a happy sweet loving little boy.

~Amber Parker (Andy's Mom)

Featured CDHer~ James

My CDH warrior,James was diagnosed with a LCDH at an 18 wk ultrasound when the technician noticed that his heart was pushed over a little to the right side. In 1992 there was no internet to turn to to find out information. Our only resource was the local library. Needless to say there was not much information and what there was was very technical and scared us to death. We were living in a small town in Texas at the time and my OB said that we wouldn't be able to deliver our son in the local hospital that we would have to go to Dallas to deliver him. We were given I referral to a high risk ob in Dallas. That first ob suggested that the best option would be to terminate the pregnancy. We told him that that was NOT an option for us. We found another ob who was wonderful and never once mentioned terminating our son. He arrived in the world at 4:15pm on June 10,1992. 

They immediately intubated him in the delivery room and rushed him to the NICU. I got a quick glimpse of his beautiful face as they rushed him by me. Not long after, they brought me consent forms to sign for them to do whatever was necessary to save his life. At approximately 8pm that night his organs began to fail and if it weren't for ECMO he would have died that night. The surgeon did his repair the next day. He came off of ECMO after only three days (the shortest amount of time up to that point for a CDHer). The neonatologist showed me an x-ray of his lungs and told me miraculously his left lung just opened up and he was able to be removed from ECMO. He remained on a ventilator for 17 days, then cpap, then just the nose canula.

He was breathing room air before long and after eight weeks was discharged and able to come home with us.

He did not need any oxygen support or monitors or even a feeding tube for when he came home. All of this was new and terrifying for us because we had 2 healthy girls at home.

James had feeding issues and massive reflux and needed a Nissen fundoplication when he was three. 

He had respiratory issues and was hospitalized a few times with pneumonia and RSV. He needed another Nissen when he was ten due to going through a growth spurt and his first Nissen coming undone.

He has a slight learning disability but graduated high school with a regular diploma.

He has fought and worked very hard and I am so proud to be his mom!


~Elizabeth Randazza

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