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Eric, now 13 years old! |
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Eric backpacking with the Scouts |
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All went well until Eric was about three months old. Over the course of a week at that point, he stopped eating, and frequently arched his back in pain. After several days of fiddling with his medication, we asked for an echo (they weren't as common back then and had to be done in the hospital on an outpatient basis) and discovered that Eric was in heart failure due to a coarctation in his aorta. Our cardiologist was able to balloon the obstruction and after several days, all was well again.
Shortly before Eric's 6-month birthday, we returned to Philadelphia for his stage-II operation (the hemi-Fontan). At that point, Eric weighed 13 lbs. The operation went well—Eric was off the vent and out of ICU after 24 hours, there was no sign of the coarctation, and we were home 8 days post-op (a small pleural effusion held us up for several days). The best part: no more meds.
The next six months were as trouble-free as we could have possibly hoped for, and once Eric recovered from the operation, his growth and development really took off. Immediately after the operation, his saturations were in the upper-70s, but they had risen to 91% when the time arrived for the completion Fontan. At that point, he was 12 months old and weighted nearly 22 lbs.
So once again, we returned to CHOP. Eric had his pre-op catheterization on June 2, 1992, and the completion Fontan operation the following day. Amazingly enough, Dr. Norwood finished the operation in just over 1-1/2 hours (with just 36 minutes of that in circulatory arrest; the hemi-Fontan was 47 minutes). He was extubated and moved from of ICU to the floor the following morning.
This time, Eric developed significant pleural effusions along with croup, presumably from the breathing tube. He wouldn't eat, and would constantly wake up coughing, and then struggle to his hands and knees and cry. To make things more complicated, the doctors put a mist tent over his bed to soothe his throat (oxygen tends to be really drying), so we would take turns climbing inside with him. But we still couldn't convince him to eat. Finally, after 3 days, the order came to drain the effusion and he was taken away to the treatment room. This worked wonders—when Eric returned about 20 minutes later, he was a different kid. He hungrily ate jar after jar of baby food. The daily chest x-ray the next morning showed that the effusions were back and just as extensive as before, but they no longer seemed to interfere with his appetite. Despite all this, Eric was discharged a mere 7 days after surgery, again on no medication.
After we returned home, Eric quickly learned to walk (he was working on it before the operation), and to do all the things a one-year old does. At 18 months, he started going to a "mothers- day-out" day care from 8 am until 2:30 pm, and took to it well. When Eric reached the age of 2, we once again returned to CHOP for his one-year post-Fontan catheterization. It showed that Eric's heart was working well, but there was a potential problem: Eric has the "Fontan with hepatic vein exclusion" that Dr. Norwood was doing during this period and in some of the kids, more blood than is desirable passes through this shunt, lowering saturations (the upside was faster recovery from the Fontan). We returned for a Fontan revision when Eric was three, but it was deemed unnecessary, since Eric's sats were then (and still are) in the mid-80s.
Shortly before Eric's 5th birthday, he had a viral stomach infection that triggered an episode of protein-losing enteropathy (PLE), which sometimes occurs in Fontan patients. Fortunately, in Eric's case, we were able to control it with a low daily dose of Lasix. After a year, we stopped the Lasix and the PLE thankfully didn't return.
Eric started Kindergarten just about that time (an aside: his teacher was concerned about him going to the bathroom several times each morning, and knew it could indicate diabetes until we explained about the Lasix). Eric did fine in kindergarten and made it through the whole year without missing a single day of school—he's very proud of that trophy. Except for a bout with the real flu (despite a flu shot), first grade was also uneventful, and Eric had perfect attendance once again in second grade.
As of this writing, Eric is in the fourth grade. He leaves for school on the bus at 7AM, is in school until 2:45, and then walks two blocks to an after-school program that he just loves. He does his homework and plays there until we pick him up at about 5pm. It's a long day, but Eric enjoys it—of course, he enjoys weekends even more.
August, 2001 Update
In spring 2001, Eric annual 24-hour holter showed that he was spending more time this year than last year under 50 beats-per-minute while sleeping. We had previously talked about Eric eventually needing a pacemaker, and Dr. Johnson (Eric's local cardiologist) said "it's time". After doing some research, we decided to go with a transvenous pacemaker, which is placed in the shoulder and the leads follow a vein into the heart. Most cardiac centers use epicardial pacemakers in Fontan patients, where the leads go on the surface of the heart. However, that surgery would be harder on Eric because his sternum would have to be reopened, and also the leads and the pacemaker don't last as long because higher voltages are needed. The primary concern with the transvenous approach is that the wires enter the Fontan conduit, so there is a small risk of blood clots forming. However, Dr. Murphy (Eric's "hospital" cardiologist since birth, who works closely with Dr. Norwood) has done many transvenous implantations in HLHS kids and none have problems with blood clots.
So in July, we traveled to the Nemours Cardiac Center at the duPont Hospital for Children and Dr. Murphy put in the pacemaker. The procedure took 6 hours because it's quite delicate (the leads have to be very precisely placed within his heart), but it was a complete success. We were on our way home two days later. Eric had a really good result, and the leads were placed in a way that allows the pacer to do its job at a really low voltage. This means Eric's pacemaker will last many years before replacement is needed.
Dr. Murphy also measured Eric's central venous pressure and found significant improvement since Eric's last cath (three years earlier), when his pressures were elevated. The pressures are now in the middle of the desirable range for Fontan patients, which is at least partially due to the Vasotec that Eric started taking after his last cath. Needless to say, this was great news.
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Congenital Heart Defect Awareness Day 2003 |
The best part is that the pacemaker has markedly increased Eric's activity level. His activity level was always fine before, and with the exception of playing soccer, Eric has always done what he wanted to do. However, now he reports that he can run laps at school without stopping to walk and his energy level is through the roof! The pacemaker is rate-responsive, and we believe that this newfound energy is because the pacemaker is making Eric's heart beat a bit faster than it would otherwise do by itself.
Eric just started the 5th grade a few weeks ago, and is doing well (today he proudly told me that he got 100 on the first social studies test). He enjoys school, playing video games, using the computer, and is also a Webelo Scout (something I highly recommend for heart kids—Eric loves it and it's not as taxing as other social activities like organized sports). Eric is also very social and has a lot of friends, which is another reason Scouts are a good match for him.
June 2004 Update
Health-wise, Eric continues to do well. In July, 2003 we returned to duPont to address Eric's saturations. As I wrote above, Eric was going to have a Fontan revision at age 3 to include all of his hepatic veins in the Fontan circulation (he still has one excluded hepatic vein). Eric's saturations have been drifting lower over the last several years, and were frequently running 78-82%.
Since there are hundreds of kids that have excluded hepatic veins,
Dr. Murphy developed a procedure to seal off the vein without open heart
surgery. He performs a cath to check the pressures, then inserts a
needle through the chest wall directly into the right atrium and deploys
an Amplatzer Septal Occluder to close the vein. We've all been
thinking this over for a year, letting Dr. Murphy gain experience
with this procedure.
So in July, 2003 Eric had his hepatic vein sealed off by cath. It took much less time than the pacemaker implantation and Eric was in the cath lab for only about 90 minutes. When Eric came back to the step-down unit, he was pink! Although he was groggy from the anesthesia, he couldn't stop looking at his fingernail beds which were no longer blue. Today, his sats run 92%-95% which is pretty much normal for a Fontan patient. We were home two days later.
Since then, Eric's only issue has been occasional PVCs, which he feels and do worry him, but his doctors insist are harmless. Eric's right ventricular function is somewhat diminished (since age 7 or so) and for this, he continues to take digoxin, enalapril, and aspirin.
In school, Eric had a most excellent year. He finished the 7th grade with mostly A's and just a couple of B's and didn't miss any class time at all because of illness. Last year he was promoted to his middle school's Symphonic Band (the Honors Band), where he plays trumpet. That band just won a Sweepstakes award in competition (highest possible scores in all areas). Then at the end of this school year, Eric just learned that he was promoted again—this time to the Wind Ensemble, which is the top band at his school. Both of these bands are wonderful and Eric really enjoys playing his trumpet. This is a handy skill—when his pet Sparky the Hamster passed away after a long illness, Eric played "Taps" for him as we held a little service in our back yard.
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Eric is also very active in scouting. He recently returned from the week-long Boy Scout summer camp (third year in a row) and this year earned an "Honor Camper" award. He also was tapped out for the Order of the Arrow, which is a scouting honor society. This is a really big honor that he has earned and we are really proud of him. At the age of 13, Eric is now wrapping up the final requirements to be a Life Scout and has his eyes set on Eagle.
After scout camp, Eric went to heart camp for the fifth year (Camp Moss, sponsored by Children's Medical Center of Dallas and Cook Children's Hospital). He really looks forward to this camp every year — it's much different from Scout camp where they sleep in canvas tents in the rain, fending off spiders and other creepy-crawlies. At heart camp, the campers are spoiled with on-demand Wendy's Frosties (they have a machine) and air-conditioned cabins. Eric got an award for "always smiling". Needless to say, he had a fantastic time.
Please feel free to write with any questions or comments. Eric is a special little boy and we're truly blessed that he is here with us.
