Sean Forney

Joseph Sean Kaj

Sean's Story

Sean is kind of an interesting case...ok, maybe I mean complicated! LOL!!! He was born with a birth defect called CDH (congenital diaphragmatic hernia) which basically means that his diaphragm never completely developed. As a result, most of his abdominal organs grew in his chest, keeping his lungs from growing properly. We were told at 33 weeks gestation that he likely would be stillborn because I had polyhydramnios (too much amniotic fluid) and that even if he did survive his chances were pretty slim. I was put on bedrest for preterm labour, and 3 times they did an amniocentesis removing 1500cc each time. Finally, when he was born via emergency csection at 38 weeks, he was placed immediately on a vent, had surgery at 1 week to do the repair and they found his intestines were badly malrotated. They didn't want to remove any at that time, and because of the way they had grown they unkinked them as best they could... hard to do though! TO tell you how badly kinked up he was, his spleen was wrapped around his spine behind his heart, and is horseshoe shaped AND in the correct location in his abdomen, his appendix ended up behind his stomach and was removed at the time of his 1st surgery! His stomach is shaped like a butterfly folded in half, and when they do gallbladder US they have a very rough time seeing the difference between his bowels and the gallbladder. Anyway, he remained on the vent for 2 more weeks, and eventually was able to come home at 2 months of age, though we had been told to expect a minimum of 6 months.

At 3 1/2 months, he got a flu bug, or at least that is what we thought it was. He had been vomiting most of the night, so we called the peds office. The staff there said to give him pedialyte and let them know how he was doing, but fortunately we also had an appt that day with the surgeon. He took one look at Sean and said I had a choice to go home or go to the hospital.... we live over an hour from the hospital and even though my husband was out of town and I had the other 2 kids with me, I chose the hospital. Thank God we did... 36 hours later he just kept getting worse and worse, vomiting ugly green goo, very tender tummy, had several x-rays done, and for some reason the surgeon felt he needed to come in at midnight to check on him... he said he just felt that Sean needed to be seen ( the nurses were not really keeping in touch with him like they should have). Anyway, when he looked at Sean, he said we are operating immediately... any questions? We have to go NOW! I carried him down to the OR myself, he hurt too much to move him to the crib, and I was terrified it would be my last memory of him. I went back up to his room and prayed and cried myself to sleep, (Brian had flown home to be with the kids at our house.. didn't want to wake them at 1 am!) and several hours later the surgeon came upstairs and said that Sean would have died within a couple of hours had he not gone in. His intestines had kinked off at the ileocecal valve and he had to have 40% of the small as well as the valve itself removed. As a result, he of course had an ileostomy. He was in PICU and on a vent for 4 days. a couple of days later he started deteriorating again, so back to OR he went again... lost several inches of his large bowel that time.

One week later he had surgery 1 more time to place a central line, as there was no way he could survive without TPN nutrition. He had been FTT prior to the flu bug, and losing so much intestine we really had no choice but to do this. He was in the hospital for 5 weeks, came home and went back a couple of days later as he had a staph infection due to a line break that a stupid.... (long story!) nurse had done. Finally came home on my birthday! ( June '97) Over the summer and fall he had a few minor tummy bugs, hospitalised for a day or 2 just to be on the safe side, and in November he had his take down/pull through/ileostomy repair. Sadly, at that point he developed a severe case of reflux as well as an oral aversion, he had been eating 7 oz. of bmilk or formula, and 7 oz of solids but refused everything by mouth from then till now. He had lots of tests done, including travel to Seattle in February to get a 2nd opinion on the placement of a fundoplication... he was hospitalised there for several days for the testing, as well as he got ANOTHER flu bug while we were there. We were supposed to stay with my aunts for the week we were there, so they got a good glimpse of what our lives are like dealing with Sean and his health stuff. The last thing he had done in Seattle was an endoscopy to check for problems... he was OK, so they placed an ND tube so we could experiment with tube feeds to see if he could tolerate them. He refluxed it back out ( all 22 inches of it!) a few days later... I don't think he was quite ready to do that!

In May of this year he underwent the fundoplication, g/j-tube placement, and to reshape his stomach something called an anteroplasty. it basically made the stomach bigger in the narrowed section. He did OK with it, but after a few days started acting like he had LAST May when he got so sick. Dr kept an eye on him, rounded up a room in PICU just in case, then all of a sudden his central line broke ( at 2 am!). Now it had broken 8 times before, but this time it could not be repaired, so he scheduled surgery for the next day to replace is ( finally! we had been begging for months to redo it!). While he was on the phone learning about the c-line, all of a sudden he refluxed and out came the g/j-tube... the balloon had popped! I am so glad I wasn't the nurse on the phone with him when she had to tell him that too,.... he freaked out and yelled at her what on earth is going on in that room!!!!!!!!!!!!!!!!!!!!!! LOL... he called back to apologise a few minutes later... he had just gotten to bed from a loooooong day of surgery all day. they placed a regular IV line, and every 20-40 minutes we had to change the gauze on his tummy as he bled from the g-tube site alot. The next day he got his new c-line, and they put in a g-tube only and when he came upstairs from OR he was ready to play in 10 minutes! Much different from how he was post op the previous week, and made me feel much much better about choosing to the surgery.

When he came home we oh so very slowly increased feeds in the g-tube by a total of 4cc per feed per week. Eventually by Christmas we had him increased all the way up to equal amounts of TPN and Peptamin Jr. Unfortunately, he ended up vomiting a few times, and just after Christmas we found out via upper GI that he had a hiatal hernia, and at least 1/3 of his stomach had moved past his diaphragm and into his chest. We drastically dropped his feeds and within a week he was back in the hospital for yet again more surgery. When the Dr went in, he found that the stitches around the fundoplication had come undone (in his diaphragm but no in the Gore-Tex patch from his cdh surgery) but the fundoplication had help up just fine. He did however have to undo it in order to put the stomach back where it belongs. In addition he made Sean's diaphragm bigger by cutting a slit in it and placing another patch called Marlex in it. Poor Sean's diaphragm looks like a patchwork quilt these days! He was only in the hospital 5 days this time around, about 1/2 the time we expected. Since then we have had to keep peptamin Jr feeds to a very small amount, about 1/5th of his total so that we don't stress out the new surgery by going too fast too soon. I am expecting it will take a much longer time than originally anticipated to totally discontinue the TPN, but as long as he is getting something in his intestines it is better for his life and for his liver.

Sean was also recently diagnosed with Scoliosis. As a result of the CDH and his spleen being wrapped around his spine he has a curvature of the spine. It seems that there are at least 1 or more hemi-vertebrae in his spine causing the curvature. A hemi-vertebrae is a triangular shaped piece of the spinal column that puts things off centre. Some kids have to have this surgically removed or frozen, others can live a totally normal life. It just depends on how Sean grows and how things develop for him. Scoliosis is fairly common in CDH, so I guess Sean is normal, for him anyway!

I am so thankful that we have this surgeon... he is very sensitive to Sean's needs, and has a lot of experience in dealing with short bowel kids. It has been a very long 2 years since we had Sean, but I wouldn't trade a minute of it for anything in this world. God has blessed us with a very special little boy, and his brothers as well as my husband and myself could not imagine what life without this little miracle could be like.