Featured cherub of the week, is 21 year old, Sean Forney. Sean is an amazing young man and we have enjoyed watching him grow up. He is so intelligent, a wonderful photographer and knows so many facts. If there is something you have a question on, ask Sean, for we are sure he will know the answer or would be happy to find the answer. Sean’s mom, Heidi Forney, has been a volunteer at CHERUBS various times over those 21 years and helps so much with raising CDH Awareness. This is her account of their CDH journey over the years, published on the forums at www.cdhboards.org.
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.
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 uncomfortable 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!).
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 wouldn’t 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 wouldn’t 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 Sean’s 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 happened, 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 x-ray, 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 anomalies, 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 straighten 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 Sean’s chest x-rays to see if he could determine when Sean’s 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 x-ray 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 antibiotics 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 Sean’s 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. Eventually 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 formulas, 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 won’t 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.
It has been 12 years now since I updated, and a LOT has happened! Sean is 15 1/2 years old, and still just as wonderful as he has always been. Briefly, his scoliosis issues ended up with a fusion at age 5, from T2- T12. They removed one of his ribs so they could do an anterior and posterior fusion, unfortunately it did not completely “take” and his curve started progressing in a kyphotic direction so at age 7 1/2 he had to have the hardware removed and was placed in halo traction with 25 pounds (half of his weight) hanging off his head and spine for 2 months in order to straighten it as much as possible. This was done in Seattle, and his spine was re-fused from T1-L2 a month before his 8th birthday. This time it worked, and instead of the 140 degrees that it could have been without the previous surgeries and rod expansions, it was only 70, and has remained at 70. He has a very large rib hump, and was on long term narcotic pain medication for 7-8 years through all of this, but he walks, runs, and is no longer wheelchair bound though he does tire easily. He also developed osteoporosis, which we are slowly working on reducing now that he can be more weight bearing.
Sean is still TPN dependent for about 50% of his caloric needs. Thankfully we can do this just a few days a week, so he gets a few days off with “just” tube feeds during the day every day. He is still on Compleat Pediatric, which has been a wonderful formula for him, though we no longer give glutamine or pectin. He just can’t seem to absorb enough calories through his gut to grow without the TPN, if we go above a certain # of calories, he ends up with severe diarrhea, also called dumping, and loses weight instead. Not so good! He is a real light weight, at 75 pounds give or take, and just reached 4’9″. He would be closer to 5’4″ if were not for his spinal fusions, as those had to kill his growth plates in the vertebrae in order to stop the curve.
At age 5 we discovered that Sean had severe pulmonary issues. His lungs just were not doing well at keeping him oxygenated, as after one of his many surgeries he had to go back on oxygen and couldn’t get off of it. We took him to Seattle to see pulmonology, and discovered that he had restrictive lung disease, partly from a partially paralyzed diaphragm (thank you patches!) and from the scoliosis and hypoplastic lung. He also has blebs all over his lungs, kind of like someone with emphysema. As a result he has been Bipap dependent at night as he can’t remove CO2 easily himself, and has both kinds of sleep apnea. We found out that had we not pursued pulmonary when we did he would have been trached and vented within months. He came close to that again recently, though we changed machines and that seems to have helped, for now. Lungs don’t grow if the spine can’t grow you know… He also on O2 at night and has been since he was 5.
As if that weren’t enough (I love my complicated sweet boy!) he also developed something called SVC syndrome. That is where he has a tendency to clot his blood vessels off. That is partly due to the central lines constantly going through them, but also, just part of being Sean. He has had 15 cardiac caths (essentially an angioplasty) to place stents to hold open the vessels and/or to balloon open the vessels. We came close a few years ago to where we thought he might need a graft (transplant) to replace a part of his SVC, but thankfully a cardiologist in Boston was able to place a few more stents instead and he has been fine ever since, along with blood thinners for the rest of his life of course. Sean is a traveling machine… loves to travel, thankfully, since we have had several different hospitals need to chime in on his care! Seattle, Boston, Denver, San Antonio, TX… Plus of course when we are traveling as a homeschooling family and “stuff” happens!
Sean was recently diagnosed with Aspergers as well as secondary mitochondrial disease. Nothing horrible, we just make sure he gets certain supplements and it seems to help with his energy levels. He is a happy young man, loves American History and animals, especially his dog and tarantula, loves to learn about hunting and wolf re-introduction, and has volunteered at our local aquarium helping them to write the signs for their new fish! If we had the $$ to travel half the year, we would as he can never get enough travel experience, seeing new places, finding out about the history of our country, and checking out every zoo and aquarium he can find.
I hope this helps to give families hope for the future, though he has been through a LOT (47 surgeries), he is a fighter, and loves life. He doesn’t know the meaning of the word NO…. God has blessed our family with him, and with his brothers (and the newest members, his new sister in law, niece and nephew!). What a LIFE!!!!
Now that Sean is 20, I guess it is time for yet another update! It is hard to believe he is now 2 decades old. There are days that it seems like it was just yesterday that we were told about the CDH, that we were living in the NICU, the PICU, driving back and forth to the hospital or to doctors’ offices on a weekly basis (or more frequently!).
Since my last update Sean has had a few more surgeries including one more cardiac cath, line replacements, and one very scary hospital stay (ok, more than one!). It all started when we finally had a GI move into town that we all felt might be able to help him get off of TPN. Part of that step included doing a trial on medication to stop bacterial overgrowth, which *I* did not believe he had, as he had no symptoms other than dumping (lots of diarrhea). The GI didn’t agree and wanted to try him on some antibiotics for it. Well, after trying a few, he ended up with a sever duodenal ulcer only Sean didn’t tell me that he was stooling old blood (black) until it had been going on for a few days. Not so good… when I took him in to the ER and they did lab work he had already lost nearly half of his blood! SO… we had an admit with several units of blood, a scope, and the doctors decided he needed to have a reduction in his blood thinners. Against my better judgment, I agreed, though I was VERY concerned, having seen what happened when his blood thinners were reduced in the past, and the GI and cardiologist involved had not. 8-9 months later, he had a clot on the tip of his 1 1/2 year old central line, and it was not able to be removed despite every trick our surgeon tried (and if ANYONE can get rid of a clot, it is him! He is a magician when it comes to this kind of thing!
That’s when our horrific month started. Sean was admitted to remove the line, and replace it with a new one. During the line replacement, the tip of the new line hit some scar tissue on his heart and made a tiny hole in his heart. Because he had been on chronic blood thinners for so long, he bled into the pericardium, and over the course of a few days it became enlarged. IN the meantime, the IR department put their heads together with the surgeon and managed to place a trans-hepatic line (in the liver… not the greatest place for a line but it was a last ditch effort because he still needed TPN). The IR doc refused to put it in the correct way per the surgeon, but at least we had some access. (It was a very unpleasant line to deal with, painful to do dressing changes and way too close to his GT for my comfort level for infection control). We went home, but after a day and a half his breathing was really bad, so we went back for an echo. During that time the pericardium had gotten HUGE with the blood draining into it, and he had to be admitted to the PICU. He had several docs hovering over him, interventional cardiologists and our surgeon trying to figure out how to tap his pericardium to drain the blood. Neither of the cardiologists was able to hit the right place, but our hero surgeon (who has performed miracles for CDH kids over and over, especially our boy). Hit the right spot first try. They ended up draining almost 600 ml of blood… keeping the drain in for a couple of days to make sure that no more blood was oozing out. Essentially Sean had suffered from a cardiac tamponade. He was in the PICU a total of a month between the two stays… and back 2 months later to replace that central line because it started to fall out, as expected. This time the surgeon was able to do it HIS way.
That line lasted until April of last year, when Sean was 19 years old. At that time Sean had finally weaned completely off of TPN, was completely tube fed and we were able to remove the line, we hope permanently.
4 months after the cardiac crisis Sean started having difficulty breathing. He was used to riding his bike around the park near our house once or twice without any problems, but for some reason his sats started dropping dramatically after only a short distance. He couldn’t even walk around our house without being out of breath. We put him on O2, and took him in to the cardiologist. She did repeat echo’s over the next few months, including exercise echo’s (an echo, treadmill, followed by an immediate echo). Unfortunately it was determined that probably as a result of the cardiac insult and his maturing age his pulmonary hypertension had returned. For him it is considered to be exercise induced pulmonary hypertension, and we are able to manage it at this time with fairly simple (but expensive!) medication combined with his bipap/avaps machine (this machine is essentially a non-invasive ventilator as it not only gives pressure for breathing at night but also volume for the breaths). He uses it and oxygen at night as well as during the day when he isn’t feeling well.
He is proud of his surgery count… 54 to date. He loves to swing, to write books, to take photos, to travel as much as possible, (going to Alaska this year, and all going well to Africa in the future!). He is an amazing young man! We are grateful to God for allowing us to have this gift of Sean in our lives, and to CHERUBS for allowing us to share this gift so that others might have hope as well. SO many times over the years the doctors would just shake their heads, not sure what his future would be… but he is still here with as much character, love of life, and hope for HIS future as any young man could possibly have. AND…..as he likes to say… He got to VOTE in the presidential election too! Life is good!!!!!!!
I guess I should do an update since Sean had surgery a month or so ago! He is up to a lovely 55 surgeries, and is almost 22 years old… amazing! He needed to have his wisdom teeth pulled, which for typical people is no big deal, but for our hero it required consults from cardiology, pulmonology, and we were SO thankful to have one of our special people involved as well, the director of Child Life, because she KNOWS him well. She was able to get the best pediatric anesthesiologist to help us out with the plans for the day. Because of his pulmonary hypertension we needed to be sure that all of our bases were covered, not only to be sure the anesthesia was done well but also for pain control, because if he is hurting, he isn’t breathing well. Everything went off without a hitch thankfully, and we even managed to get one of our favorite nurses from pediatrics to care for him afterwards in recovery. The only problem was.. pain control. The meds his internist wanted him on caused him severe nausea. Not so good for a kid who has a Nissen, and hasn’t HAD any nausea in nearly 20 years! We ended up going with zofran and tylenol… he had more pain than he needed to because the doc was unwilling to let him have what we KNOW works due to the intensifying concerns out in the world about overdosing on pain meds. Frustrating that experience no longer matters and patients have to suffer.
Sean is working hard on independent living skills, has started doing training to do volunteer work with our local zoo, is a great photographer and is learning some macro photography skills in addition to his regular skills at taking some amazing pictures of animals on our adventures around the country! We finally made it to Alaska a few months ago (we had to postpone the trip in 2017 due to a family crisis) and are working on plans for more adventures in the future. Life is good, and he is truly one of the most incredible young men I know. His knowledge of the world and compassion for others, both man and beast, brings smiles to my face every day.