Year 2006 – in the months leading up to Jacob’s birth, things were a whirlwind for us. When we went in for the 20 week ultrasound, the tech knew something looked different. They sent us to the high risk doctors where we had weekly ultrasounds and many different opinions from many different doctors. They essentially were giving us worse case scenarios, but none of them really knew what was going on. The Lord was clearly at work, though, because as they were about perform a fetal surgery on Courtney/Jacob that could have significantly harmed Jacob, our sister-in-law asked for another Ultrasound. Things looked differently this time and they did not move forward with it. After that, Courtney asked to go back to her regular OB and wanted him to deliver this baby.
December – Jacob was born on the 3rd, via C-Section, in Chattanooga, TN at TC Thompson/Erlanger. Six weeks early and weighed 4 lbs, 8 oz. Diagnosed with Cloacal Exstrophy, a severe and rare birth defect affecting only 1 in about 250-400,000 live births. The surgeon on duty at delivery, in conversation with us, determined that Johns Hopkins was a hospital best suited to treat Jacob. By God’s grace, we had family there, too (Courtney’s brother and our aforementioned sister-in-law).
Within 24 hours, he was on a medical helicopter, heading to Johns Hopkins. Mommy and Daddy stayed behind for two days so Mommy could recover enough from c-section to leave. Huge help from our church - $15K had to be paid up front for medical transport. Family in Baltimore checked in with Jacob in the days prior to our arrival.
Three week stay in the NICU at Johns Hopkins. Had omphalocele repair, colostomy bag added and PDA surgeries. Released on 12/23/06, two days before Christmas.
Had bladder exposed (outside of body) for first 18 months of life. We kept it covered with Saline and Saran Wrap.
Year 2007 – this was the year that we really learned a lot about Jacob’s condition. He would have his second major surgery and spend a fair bit of time in the hospital.
January – got RSV and had to spend several days in the hospital (Chattanooga).
February – doctor put Jacob on special formula to try and “fatten him up”. It worked well.
February/March/April – casting procedure on club foot, wore special shoes with bar between them. (ponsetti?), tendon cut, weekly shots with this procedure.
June – while visiting in Baltimore, attending exstrophy picnic, noticed bulge in his lower back. Was referred to and sent to see neurosurgeon, Dr. Jallo, had MRI and determined that he had tethered spinal cord (form of spina bifida) and needed surgery fairly soon.
August – surgery scheduled for tethered cord, at the end of the month, but Jacob got sick just before and surgery was delayed one month.
September – returned to Hopkins for surgery. In the hospital for about a week, but recovery was long due to “packing” the incision points on his back and trying to keep “pee” from spilling from his bladder and around to his lower back. Required that we use a combination of male guards, diapers and women’s pads. Had to bring in a Home Health nurse to assist with packing the incision sites. Recovery took several weeks.
Year 2008 – Jacob recovered nicely from his surgery in 2008 and began to walk a little. We continued to maintain his bladder, still outside of his body, as we had previously (using saran wrap, saline, and cover with diaper). This would be a year remember, though, as he would have two of the most involved surgeries that accompany cloacal exstrophy patients and we would spend approximately over one fourth of the year in the hospital.
June – went to Baltimore for picnic and annual doctor visits. Dr. Sponseller and Dr. Gearhart agreed that it was time for the “Big One” - surgery that is. Our 100 day stay in Baltimore would begin at the end of the followingmonth.
July – arrived at Hopkins for first of two major surgeries. First surgery involved cutting his pelvic bones, inserting a titanium bar with screws, and putting Jacob in a traction style cast with pins inserted in his pelvis. He wore this for approximately 6 weeks.
August – Bladder closure surgery. He remained in the hospital throughout this entire timeframe, which included respiratory illnesses, high fevers that really could not be explained and a lot of separation time for our family.
September – pins removed, but some complications with bladder surgery.
October – Spiked high fever, possibly UTI. Bladder scopes done to see why bladder not draining well. Attempted to cath and resulted in false entrance/opening in his skin. Titanium plate in his pelvis had eroded surgically repaired urethra. Multiple trips to Johns Hopkins ER.
November – headed home to Chattanooga after 100+ days in the hospital. Unable to resolve issue with bladder, so sent Jacob home with SP tube.
Year 2009 – this was a very difficult year for us because the suprapubic (SP) tube had to be changed out monthly and was his only way of peeing. The tube was basically a modified catheter with a “bubble” on the end that was inflated to help keep it in place. It then attached to a urine bag on his leg, that he wore 24/7. Having this in resulted in regular bladder spasms, which were painful for Jacob and required that he take medicine daily. He also took a daily antibiotic to help reduce the chances of urinary tract infection. In addition, his ostomy (for poop) was very tight, resulting in us having the dilate it (basically stab/insert) it daily. This was one of the most difficult things that Mommy and Daddy had to do, because we know it was very painful. Even with medicine, Jacob would awake multiple times each night, as result of spasms, gas/colostomy issues or both. Meds were Sulfatrim and Ditropan.
January – doctor visit with Dr. G and Dr. Lau. SP tube to stay in and must be changed monthly in Chattanooga by Ped Urologist here. Very painful for Jacob.
February – stoma revision attempt scheduled, but decided that it was better to wait. Must continue to dilate with metal rod. Continued to do this and get SP tube changed out monthly throughout 2009 and into early 2010.
July – after playing outside on a slip and slide,
August – James has two seizures a week apart. Starts meds shortly after that. Kepra. Two ER visits.
Year 2010 – after a difficult year, the Lord gave us some rest in 2010. Jacob would have his fourth major surgery at Hopkins.
January/February – Re-do of urethra and titanium bar surgery, this time successful (Thank you, Lord). Also a stoma revision. Spent about 7 weeks in the hospital/at Johns Hopkins.
March – Stoma prolapsed, to the point where nearly 12 inches of bowel were “hanging out” of Jacob, in his colostomy bag. Bag changes required daily, sometimes more often, and very difficult to change, cumbersome for Jacob.
James was able to see eye/seizure specialist at Hopkins and thankfully advised that he was 95% sure that James would grow out of these before he turned 12. Thankfully, he hasn’t had a seizure since April 2010.
June – appointment with doctors, another stoma revision scheduled. Daddy BA shared Jacob’s story and God’s grace at the annual exstrophy picnic.
July – stoma revision surgery. One week in the hospital. Surgery did not take, so stoma again prolapsed.
Years 2011/2012-these two years were fairly low key for us, comparatively speaking. Jacob continued to grow, albeit slowly at times, and we continued to deal with the challenges of the prolapsed stoma. He also started school (kindergarten). Doctor visits were mostly limited to office visits, although Jacob did have to go to the ER several times due to dehydration. Because of his short gut/bowel, he isn’t able to absorb water and nutrients as well and is therefore more susceptible to dehydration, especially from colds and viruses.
Year 2013 – during the summer between kindergarten and first grade, Jacob began to mention how he would like to be dry, like the other kids his age. Up to this point, he wore a pull-up all of the time because his pee would just constantly drain out of the urethra that was surgically created for him in 2010. We knew that the next major surgery for Jacob would most likely be a “bladder augmentation” surgery where Jacob would have a continence stoma added and then be able to use a catheter to pee. While visiting the doctors at Hopkins this summer, we told Jacob that HE needed to tell Dr. Gearhart that he wanted the surgery, not just have us tell him. So God gave Jacob the words and he told Dr. G – “I’m not leaving Baltimore until I have this surgery”. While it couldn’t be scheduled right then and there, it was scheduled for November.
November 2013-January 2014 – continence stoma/bladder augmentation surgery. Spent about one week in the hospital, but 60 days total in Baltimore for healing, removal of kidney stints, SP tube and training Jacob how to cath. He also had a successful stoma revision surgery and no more pro-lapsed stoma. **Lots of great detail and pics on our blog from this surgery.
Years 2014/2015 – once Jacob recovered from his continence surgery, he was almost like a new boy. No more diapers/pull-ups. Jacob learned to cath by himself and several times asked why we didn’t schedule this surgery sooner. But we know God’s timing is perfect and that Jacob likely would have struggled if it had been any sooner.
Jacob truly had a great year in 2014. He was able to do so much more without the restrictions of wearing pull-ups and having 10 inches of bowel hanging out in his colostomy bag.
We knew, however, that there was still one aspect of his condition that we had put on hold. That was his club foot. The doctor recommended that we think about addressing it in 2014/2015, but we knew how much Jacob was enjoying his new freedom, so we held off.
During the summer of 2015, Jacob started getting semi-regular urinary tract infections. We were thinking that they might have been the result of swimming because they almost always seemed to follow right after he was in a pool.
Year 2016
As time went on, however, and as we began 2016, he started to lose mobility whenever he would have an infection. For approximately three weeks in March of this year, he could barely walk and complained of dizziness.
We thought for sure that his tethered spinal cord was “coming back” (it is always there, but once his mobility goes down, it can reach a point where surgery is needed again).
In March, we had an MRI, followed by an X-Ray. The neurosurgeon at Hopkins reviewed it and advised that his spine looked stable, when compared to his last MRI in 2013.
Our focus then shifted back to the urinary tract infections. An ultrasound was performed and reviewed by Dr. Mullins, an adult urologist here in Chattanooga who actually was a resident and fellow, under Dr. Gearhart, at Hopkins. Oh how the Lord works in amazing ways.
With the recurring infections, the thought was that Jacob might have a bladder stone. An ultrasound was done and initially, and while they didn’t see a stone, they did see a lot of mucus and other “stuff”.
In April of this year, Dr. Mullins performed a procedure to clean out Jacob’s bladder. In the process, though, he did find a small stone. We will be having another ultrasound in June and then plan from there. Dr. Gearhart, at Hopkins, advised that we should wait on it for now.
During this same timeframe, we also began making plans to address Jacob’s club foot. A doctor here in Chattanooga, who works in pediatric orthopedics, advised that we should see a specialist at Sinai Medical in Baltimore, next time we are there. He said if he was treating him, he would do a weekly cast for two months, then perform a transfer surgery. Our plan for now is to see Dr. Sponseller, Jacob’s ortho doc at Hopkins, when we go in June. While minor compared to some of Jacob’s other surgeries, it does appear that correcting the issues with his club foot and left leg may be more complicated than we originally thought.
But through it all, the LORD has it all.
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