Joseph Huber

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A few words about our Joseph.  He was born September 15, 2003 after an uneventful pregnancy.  He was small, weighing only 5 lbs (and born at 39 weeks).  The day after he was born, he was transfered to Cardinal Glennon Children's Hopsital in St. Louis and diagnosed with Hypoplastic Left Heart Syndrome.  The doctors informed us that a three staged surgical procedure would palliate his defect and give him a functioning 2-chambered heart.  He had his first surgery, the Norwood, on September 25th, 2003.  They would need to pack his head in ice to cool his body down and stop his heart during the repair while a heart-lung machine took over his vital functions. It was a long, exhausting day and the surgeon told us, "It went as well as could be expected. It's in God's hands now. The first few days are critical."  Seeing him was heartbreaking. Tubes and lines everywhere. He was on medication to keep him paralyzed and sedated.  His chest was left open to accomodate for swelling. I was not even allowed to gently touch him.  My husband and I got the dreaded overnight call, "Joseph is not doing well. You and your wife need to get in here right away."  Joey's blood pressure had plummeted causing the nurses to call a "code blue," and they did chest compressions and gave him some strong medications.  Thankfully, he made it through and (just barely) avoided going on an ecmo (external heart and lung machine) machine for additional life support.  He came home 3 weeks after surgery.  He was able to take a bottle of pumped breastmilk with powdered formula added in to give him extra calories per ounce.  He did not like to eat and we were up every three hours around the clock for over a year trying to get him to eat his required amount so he would gain weight.  


At three months old he had a heart  catheterization to widen his aorta which had narrowed from scar tissue and to check and see if he was ready for his second open heart surgery. At four months old, he had his second surgery, the Bi-directional Glenn. He was home about a week an a half later, but unfortunately he was readmitted two weeks after that with a complication of fatty fluid around his lungs, chylothorax.  He had chest tubes inserted and was placed on a low fat formula and then a no-fat formula.  Then he was taken off of food altogether and just fed intraveniously.  They tried different medications.  Everything was "supposed" to work. Nothing worked.  They tried a heart catheterization to coil some collateral vessels. Didn't work.  Finally, he had a thoracotomy surgery with a thoracic duct ligation as well as some biological glue to help his lungs stick to the chest cavity so fluid couldn't accumulate.  Finally, this WORKED!  We were home after 69 days in the hospital!!  Unfortunately he was readmitted AGAIN with an infection in his central line, but after a week was home to enjoy the rest of his first year.  Eating was a challenge. He had to be on a very low fat diet and believe it or not, we centrifuged breast milk to make it fat free as he gradually incorporated fat back into his diet.  He suffered reflux and threw up A LOT.  Often it would take about an hour to feed him his required amount and he was turn around and throw it all back up...very frustrating. Many tears. As he got older, he would gag on all solid foods except things like fruit loops or cheese puffs which were dissolvable.  I burned out a couple food processors pureeing about everything.  He finally started eating everything at 25 months.  He had the stomach flu. Didn't eat for a week and all of a sudden started eating everything. The next couple years were spent growing and being "normal."  He had two eye surgeries for alignment issues, but those were outpatient procedures. 


His third open heart surgery was three days before his 4th birthday. He had a heart cath the summer before to coil more collateral vessels and make sure he was ready.  The third surgery, the Fontan, went very well.  He was home after a week (we were expecting about a three week stay) .  Then we started living normal lives.  Had some more kids. Started homeschooling. Sports, activies, piano lessons, etc. Progressed to yearly cardiology visits.  Every year the same. Good report. Heart function is great. Doing well. Nothing to worry about in future.  At one point they saw a narrowing in aorta from scar tissue that might require a cath when he was a teenager, but several visits later they decided it was fine.  


Fast forward almost 10 years.  We noticed some swelling (pitting edema) in Joey's legs on the Sunday before Memorial Day 2017.  He was diagnosed with our worst fear, Protein Losing Enteropathy.   It affects about 10% of kids who have had the Fontan surgery. Traditionally, it has a 50% 5 year survival and 20% 10 year survival. Our cardiologist has reassured us that these numbers are outdated and things are constantly improving, but it is hard to find much good news with online searching.  He had a heart cath in July to widen the aorta. They placed a stent after ballooning it open. His heart function and pressures all looked good in the cath.  He is taking 9 different medications.  His protein numbers have been up and down.  It seems to be a long, slow process.  Nonetheless, Joey is showing so much character through this all, and we are so proud of him! He gets very nervous and asks a lot of questions...understandable for a 14 year old, but he is quite a trooper.