[left:fbe96db12c]http://www.cherubs-cdh.org/Album/new/forney-sean.jpg[/left:fbe96db12c]Sean was born on Jan. 22, 1997, induction, which turned to an emergency C-section due to his heart slowing up during contractions. Following his birth he was whisked away with daddy close behind. We were told that he was put on the oscillatory ventilator- expected to remain on it for some time. His repair was performed on his 1 week birthday and the surgeon found it was worse than he expected. Sean's spleen was tucked up behind his heart and twisted around his spine (they did manage to save it though!). His stomach, spleen, liver, and large and small intestine were all in his chest, while the diaphragm was very small. A gortex patch was used to replace the diaphragm

After the surgery we were told to expect at least 5-6 months in the hospital, though later the surgeon said he didn't expect Sean to ever leave the hospital. Through many tears and prayers, the doctor was proved very wrong! Sean was totally off of the ventilator just 2 weeks after surgery, and came home a breastfeeding baby 1 day short of his 2 month birthday! Granted, he was and still is on oxygen, but is expected to wean off of that in a few months.

We were at home nearly 7 weeks, when Sean got a stomach virus. Back we went to the hospital for what we thought would be a few days of hydration. Instead, his small intestines became blocked, and he had emergency surgery at 1:30am and ended up with 40% less of his small intestines (it had turned gangrene). A week later he had a few inches of his large intestine removed as it was also looking bad. One week after that he again had to have surgery, this time to place a broviac catheter so he could have home IV nutrition (expected to be in use for a few months to a few years). As he slowly regained his strength he finally was able to have pedialyte 3 weeks after he first got sick. After 5 weeks in the hospital, Sean came home complete with not only his new central line, but also with a lovely new ileostomy which is expected to be repaired in 4-5 months.

The doctor told us that Sean's bowels were severely malrotated due to the CDH, so this was not unexpected. Now we are just praying that we are done with surgery for a while, and hoping that eventually we can get him off of the formula and back to breastfeeding.

A year ago we had no idea what our new baby would bring. Little did we know what love, joy, and God's peace would descend on us through all of this. Sean is a perfect miracle, and both the NICU and Pediatric staff's agree that he is a survivor.

Update 1998:

I wanted to tell you about my sweet Sean and what has happened since my last letter to you. I believe I wrote you just after he had bout of the flu and lost a large portion of his intestines due to his severe intestinal malrotation. He was placed on TPN ( IV nutrition) to supplement what I was able to give him at first through an NG tube, and then eventually I was able to have him breastfeed. He did end up with a staph infection a few days after we got him home from the Pediatric unit, but we caught it very quickly and even though it meant another hospital stay for 10 days, it gave me a better chance to learn the tricks of doing his TPN with supervision. And, the best news of all was that he came home from that stay on my birthday! What a gift the Lord gave me/us that day!

Over the next few months Sean had a few stays at the hospital for flu bugs, we all felt it was better to be safe than sorry given how close we had come to losing him in May (97). All continued well, though he gradually began to become a more difficult nurser, it ended up that I could only nurse him when he was asleep which meant that I had to get up every couple hours all night long since he did sleep through the night. I believe this was the start of his Oral aversion that he has to this day. I did a lot of research including a series of email with LLL, but sadly by October he had completely quit nursing. He did take a bottle of expressed milk for a time, ( I had a lot in the freezer!) though by early November I had run out and had switched him over to enfamil.

In early November Sean had surgery to repair his Ileostomy (poop pouch) which made our lives completely different once again! I had not had to change a diaper in nearly 6 months, and having done a great deal of research learned that his stools would likely be quite acidic making a sore mess of his little "virgin" tushie. We had a variety of diaper ointments available to try on him, most of which we found via the internet list servs I had joined dealing with these special gut babies. (none of which have CDH that I am aware of). Since then he has had some really awful diaper rashes, with very few days that he hasn't been sore. Sadly, one other thing that changed was that Sean developed a bad case of reflux, and his Oral aversion strengthened as a result of that and he would take nothing orally at all, despite having had 7 oz of formula and 7 oz of solids per day just prior to this.

For the next few months we tried a variety of meds to see if we could control the reflux, did several tests to see what was going on (upper gi's with small bowel follow through, regular upper gi's) and even went to see another doctor in Seattle to see if he any suggestions other than a fundoplication/g-tube placement. There Sean had a pH probe (he refluxed 15% of the time on it), a gall bladder ultrasound ( he had sludge due to the TPN indication he needed to begin to get off of it), a gastric emptying study ( he had normal emptying but they used too small a volume to test properly) and finally an endoscopy to be sure that his esophagus, stomach and duodenum were ok and mot bacteria ridden from the reflux. Thankfully that was fine other than severe inflammation, and while down there they placed an ND tube so we could attempt tube feeds to see how well Sean handled them. Unfortunately, the ND tube really irritated his bowels a lot, and he refluxed it back out again in a matter of days after having been very uncomfortable.

Finally, we made the decision after lots of prayer and research to go ahead with the fundoplication. So, in May of '98, he had a fundoplication, g/j-tube placement, and something to reshape his too narrow stomach called an anteroplasty. He seemed quite uncomfortable for several days following the surgery, though we did manage to wean him off of the morphine. A day or 2 later, he got very uncomfortable, reminding me of when he had to have the 2nd gut surgery to remove more intestines in the previous May. That night he broke his central line for what would be the 9th and final time ( that was 9 times in 1 year.... and it broke on the 1 year anniversary of its initial placement!). A few minutes later the balloon on the g/j-tube popped and it came right out of his stomach and lay there on the bed. He bled a lot, so all night and the next day we were changing gauze pads every 20-40 minutes until the surgery to replace both the central line and the tube. When he came back to his room he had a g-tube only, and within 10 minutes of his return he was up smiling, playing and standing, all things he had been too uncomfotable to do that week after the first surgery. We have slowly started doing tube feeds, starting first with pedialyte, then moving on to 3/4 strength peptamin jr. Currently Sean is on full strength peptamin jr, and is getting 5 bolus feeds a day. We have been able to increase the feeds so much now that he in only on 3/4 of his original amount of TPN, his liver enzymes are starting to look much better now that he is able to digest some real food, and he has gained an enormous amount of weight... 3 pounds in 2 months over the summer! He is finally at the 50%, which is what his birth weight indicated so many months (20) and surgeries ( ago. We are looking forward to watching him grow even more, and in the next year he likely will be able to be weaned off of the TPN completely. Now, we are doing what we can to stimulate him to put food near if not in his mouth... who knows if he ever will eat on his own! We are just so thankful for the way this has all gone... so many times we could have lost him, and so many times God has come to take care of our little guy ( not so little at 26+ pounds!).

Thanks Dawn for everything, in particular starting Cherubs, and for continuing to add more support to it as you were able to do with the listserv and monthly chats. I look forward to sharing with the other members here, and it is great to know that so many of us are praying for each other when we need it, and that we share such a strong bond with each other through our cherubs.

Updated 2000:

Gosh, it has been a long time since I updated Sean's story. I guess when I last wrote he had been doing quite well on the Peptamin Jr., well that didn't last long. That December he became very retchy and started vomiting again, so we had to do an upper GI. Well, we found that he had a hiatal hernia where over 1/3 of his stomach had moved past a tear in his diaphragm and into the chest. ACK! He had surgery less than a week later to repair it, which meant that the fundoplication (which was still intact) had to be undone then REdone again, this time with the addition of teflon to all the stitches in order to be sure that they remained stable and wouldnt tear again. Also, the surgeon found that Sean's diaphragm was extremely tight, so he cut a hole in the diaphragm and added a patch to release the tightness so it wouldnt tear. I guess you could call that a pre-emptive CDH repair...

A couple days post op, we and the nursing staff at the hospital noticed a lump on Seans back. When we got home we scheduled a visit with the pediatrician, then an orthopedist who found that Sean had severe scoliosis. We all hoped that it would go away in time, but we kept a close eye on it, and sadly it progressed. As all of this happene, his GI tract had more and more trouble with the Peptamin jr, first he could not tolerate large amounts of formula, then he could not tolerate full strength formula. Within just a few months of his Hiatal hernia surgery, he went from 50% of his calories via his GT to 10%. We were so frustrated, we finally persuaded the physician to let us try a different formula, he suggested Nutramigen. That went well, for a few months, then again he started dumping sugar and not able to tolerate full strength.

Mid summer of 1999 Sean had another spinal xray, and we found that we could not wait any longer. His curve had progressed to 64 degrees. Sean was scheduled for an MRI and a 3-D CT scan so we could determine if there were any spinal cord anomolies, such as tethered cord, spina bifida, etc. Thankfully it was normal, though it had taken over a month to get the testing done. He was scheduled for surgery in November to work on his curve, the plan was to place grow-rods on his spine to straigten the curve, and to allow him to still grow for a bit since he was fairly short for his age. When it came to that date, the doc changed his mind, and only the hooks on the vertebrae (to attach the rods) were placed in order to be sure that the grafting had a chance to grow and strengthen. It was supposed to be overnight surgery, however Sean as always startled everyone and as he recovered from the pain, he stopped breathing on several occasions and had to be placed in PICU for 5 days! He kept having spells several times a day, so had to have a 3 day EEG in order to be sure his neural function was ok. While we were in the PICU, the Intensivist took a good look at all of Seans chest x-rays to see if he could determine when Seans spine started to curve. It turned out that you could actually see the curve when Sean was only a few months old, but it seemed as if the radiologists thought it was just an infant moving around a bit for the xray rather than the start of scoliosis. Had we known then that 30-40% of CDH babies someday end up with scoliosis, we would have seen an orthopedist much much sooner.

Anyway, he came home after 12 days in the hospital, and never had a blue pass out spell at home. However, within a month or so his bloodwork started showing some severe problems, and he started complaining of pain in side, so had to have a gall bladder ultrasound. Turned out that his common bile duct was partially occluded, so within a few days he was again in surgery, this time to remove his gall bladder and to place a T-tube for drainage. Thus began the next several months of difficulty. Sean ended up with Pseudomonas and Enteroccocus, so was on 2 forms of IV anitbiotics for 6 weeks. When the T-tube was removed, he somehow ended up with yeast in his system, and had a serious bout of Sepsis and had to be in PICU for 12 days. We came very close to losing him, the surgeon said he was as critical as when he was born with the CDH. Eventually he got better, though he had to have one on one nursing, vitals every few minutes, and blood products several times. He lost 10% of his body weight, and looked so skinny and tired. He came home after 3 1/2 weeks in the hospital, and a month later had to go back for more back surgery! (it had been postponed due to the sepsis).

This time the Doctor did put the grow rods in, and was able to straighten the curve from 80 degrees to 39 degrees, AND Sean grew 3 1/2 inches taller in the 1 1/2 hr surgery. Amazing to see such growth! Unfortunately during the previous several months Seans GI tract had more and more trouble with formula, we changed several times and never were able to get past the 5-10 % of caloric intake. Evenutally he was only able to handle 10-15 calories a day, using 1/6 to 1/8 strength. In June we were able to once again change formula's, this time to a more "real" like formula as it was made from beef, apple juice, peaches, beans and peas. We also added liquid fruit pectin and an additive called Glutamine (which is supposed to help the small intestine to grow and absorb nutrients better). Amazingly enough, within 3 months of changing formula Sean went from 12 calories a day to 500 via his G-tube! We were thrilled, thinking perhaps we could go on to getting him off of TPN by the end of the year or early 2001 perhaps.

He did have to have more back surgery in September, as the curve was progressing quite rapidly. Unfortunately they were not able to get the correction hoped for, additionally they found an infection along the rods so he went on 2 months of antibiotics, so his gut once again is having trouble absorbing nutrients. We are hoping that once the antibiotics wear off he will again do better, though he is due to have more surgery in December so we shall have to play it by ear. Because the curve appears to be stiffer than first hoped for, it appears it will have to be fused in the next year or so in order to prevent it from worsening. This means he wont be able to grow to what his height might have been, but to halt the progression of the curve is very important. Sean is a normally developing child otherwise, he is developmentally as a normal 3+ yr old (perhaps a bit advanced so I have been told) and most of the time as happy as can be. We thank the Lord that he has recovered from so much, and still has his spunky fighting spirit. He is a joy and a pleasure! If anything can be learned from Sean and his experience, I pray that other CDH families will keep a close eye on their Cherubs spines and try to catch possible scoliosis as early as possible.

Written by Seanís mom, Heidi Forney (Idaho)
1998, Updated 1998, 2000