Something from the "Archives"

Back in 2010, Mommy and Daddy wrote our "Journey with Jacob."  It was intended to give everyone a look at Jacob's life between his birth in 2006 up until his two really big surgeries in 2008 (the point at which we really started posting in this blog).  We thought this had been lost, but the Lord allowed us to find it at just the right time. 

“Journey with Jacob” 2010

Jacob E. Arrington

Born 12/3/06 w/Cloacal Exstrophy (O.E.I.S)

 

The Arrington’s - Bryan, Courtney, James (5) and Jacob (3). 

 

            Our “Journey with Jacob” began in August of 2006.  Courtney was pregnant with our second child.  We went in for a routine visit to find out whether we were having a boy or a girl.  The technician performing the Ultrasound immediately noticed something out of the ordinary with our little one.  She called in Courtney’s primary physician and both agreed it had something to do with the baby’s bladder.  However, they felt that it was serious enough to where we needed to go see high risk doctors at Erlanger.  After weeks of additional office visits, ultrasounds and opinions, the doctors determined that our baby had an omphalocele (that is the “O” in the OEIS designation above.  More on the rest of it later).  This is a condition in which the abdominal wall does not fully form and certain organs are outside of the body at birth.  It is not extremely rare, occurring once in every 5,000-10,000 live births.  During these visits we also determined that we were having a baby boy.  We immediately began preparing for his birth, surgery and the estimated 4-6 week stay in the hospital. 

            Then, about six weeks prior to his due date, the Lord decided that it was time for Jacob to come into this world.  He was delivered at Erlanger via C-Section and sent to the NICU.  He weighed 4lbs, 8 oz.  The surgeon on duty evaluated him and then came back to let us know the scoop.  She advised that Jacob had something a little more complex than what we originally thought.  In addition to the Omphalocele, he also had Exstrophy of the bladder (meaning it was also outside of his body), Imperforate Anus (the plumbing was not where it should be and his pelvic bones were way out of place due to a 30-40% loss of bone mass and lack of development) and Spina Bifida (O.E.I.S).  In addition, he was also born with a clubbed foot (his left).  The surgeon advised that they had never seen this complex condition before and they determined that there were only two facilities in the US that were known for handling it – one was Johns Hopkins and the other was located in Seattle, WA (We later discovered that the facility in Seattle no longer specializes in this condition).   This condition occurs only once in every 250-500,000 live births, making it one of the most rare conditions in the world.  It is also one of the most severe birth defects where human life can still be sustained.  Throughout all of this, we constantly saw the Lord’s hand at work.  We actually have family near Baltimore.  And we must say that having them there has been a great blessing to us throughout this entire journey. 

            With the diagnosis and determination in place, our journey really began to “ramp” up at this point.  We got to spend 24 hours with Jacob and then he was flown, via medical helicopter, to Johns Hopkins.  Once again, the Lord was there.  The airline required payment in full before they could transport Jacob.  The amount was large - $15,000.00.  We only had about $9000.00 available on a credit card, but one of our church elders, who had come to pray with us and visit, had stayed a little longer.  He agreed to cover the remaining $6000.00 with his personal credit card and Jacob was on his way.  We were able to later reimburse him once we coordinated with insurance, but that situation was definitely stressful.  But He is good and He provides, according to His good purposes.

            Now, one of us could have ridden with Jacob in the helicopter, but Courtney could not be released from the hospital, yet, and Bryan decided to stay with her.  So, Jacob traveled to Johns Hopkins without us.  This certainly created some anxiety for us, but we knew he was in good hands all the way around.  About two days later, Courtney was released from the hospital and we were on our way to Johns Hopkins. 

            When we arrived, we immediately went to visit him in the NICU.  He was such a little guy and had all of these tubes, monitors, etc hooked up to him.  Words don’t really do this justice, so we have placed a picture of him on our blog.  We’ll provide that web address a little later.  He had his first major surgery that Friday (12/9).  During this surgery, they closed up the omphaocele (his intestines) and created an ostomy (stoma for his poo), but his bladder remained on the outside of his body.  One week later, he had a second surgery to correct/close a small valve (PDA) near his heart.  This was not a huge surgery, but certainly concerned us because of where it was located.  We did find out that this is not an uncommon procedure, and thankfully, it went very well.  We were also first introduced to Dr. Gearhart, who, along with several other doctors, have, by God’s grace, been a true blessing to us.    

            Jacob remained in the NICU for a total of three weeks.  He was released right before Christmas and we were able to share Christmas with some of our extended family there in the Baltimore area.  The Lord was again gracious and merciful to us in that we did not have to spend Christmas at the hospital.

            Upon returning home, we had quite a routine to follow.  You may recall that his bladder remained outside of his body, even after the first two surgeries.  This required us to keep saline and saran wrap on his bladder at all times, with a diaper over top of it.  His bladder basically “drained” constantly.  With a few weeks of returning home, he came down with RSV (respiratory virus) and had to be hospitalized for about a week.  He was not gaining weight, so the doctor put him on a special formula.  Thankfully, he liked it pretty well.  Since Jacob was born with a shortened colon that required an ostomy, he did not always absorb his food and nutrients very well.  In addition, whenever he gets sick, it seems to linger longer than normal.  He also becomes dehydrated much faster than a “normal” child.  This formula worked, though, and we are again thankful that the Lord led us to find it and also provided the means to be able to get it (it was quite expensive).

            Things gradually improved for us during the next few months.  We certainly had a good bit of help from our family and friends, especially Courtney’s Mom.  We went to see a doctor here in Chattanooga about Jacob’s club foot.   He recommended a procedure that involved placing a cast on his foot and then having him wear a bar for awhile between his feet.  This was a pretty rough time for all of his, especially Jacob, because the bar was very restrictive and had to be worn at night.  Sleeping was definitely optional during this time, for Jacob, Mommy, Daddy and Gran (Courtney’s Mom).

            The summer of that year (2007), we had gone to Baltimore for some office visits at Hopkins and to spend time with family.  While we were there, we noticed a small bulge at the base of Jacob’s spine.  Yet again, the Lord was there (as He always is).  We were able to get an MRI scheduled and determine that Jacob had a tethered spinal cord.  You may recall that one aspect of his condition is Spina Bifida.  Thankfully, his has been on the mild side, which, again by God’s grace, has permitted Jacob to walk.  However, the tethered cord would require surgery.  So, the surgery was scheduled for the end of August 2007.  We again made our preparations and decided that we would go up about a week before the surgery to visit with family and so forth.  However, the night before the surgery, Jacob began coughing.  When we arrived at the hospital the next day, he had developed a cold and could not have the surgery.  Thankfully, we were able to get it rescheduled for the end of September, but it was frustrating to travel up there, only to have to turn around and come back, then return again a month later. 

            Thankfully, the second time was a charm and the surgery was successful.  However, the recovery was quite rough.  Jacob developed an infection in his back at the site where the surgery was performed.  He had to be readmitted to the hospital and ended up being there (at the hospital or in the area) for a total of 27 days.  Even after he returned home, a nurse had to come in and treat these wounds for several weeks.  In the end, though, Jacob was able to walk and crawl fairly well, although his pelvic bone issues certainly restricted him a bit.

            As the year 2008 arrived, we continued to look forward to the “big” surgery.  After speaking with our doctors and some other families who had been dealt with this condition, we pretty well knew it was coming, but weren’t sure when.  The surgery would involve both the Orthopedic and Urology physicians.  There would actually be two surgeries that would need to be performed fairly close to each other.  The first would involve his pelvic bones and the second would be for the purpose of closing up his bladder (which was still outside of his body at that time).  The Orthopedic surgeon generally takes a conservative approach when it comes to these things and while we were anxious for his bladder to be closed, we also understood that the longer he was allowed to grow first, the better the chances for success, especially with the pelvic surgery. 

 

Just prior to the surgeries in July and August of 2008, we began using a blog to chronicle our Journey from that point forward.  For the “rest of the story”, please feel free to visit our blog (we typically update it only when there is a procedure, surgery or visit to Johns Hopkins or if an urgent prayer situation arises).  The website address is www.jacobarrington.blogspot.com.  While the majority of this journey has involved our younger son, Jacob, we have also had an adventure with our older son, James.  Just last year in August of 2009, he had two seizures, exactly a week apart.  He was diagnosed with epilepsy and has been on medication since then.   Thankfully, he has not had any significant seizures since that time.  In addition, we have finally been able to secure a visit with an Epilepsy specialist at Johns Hopkins.  

 

In June of this year (2010), we will be returning to Johns Hopkins for several office visits and a minor (hopefully) procedure for Jacob.  He has developed a concern with his stoma and so we don’t know if he will end up needing a surgery to correct this or not.  It basically has “prolapsed”, meaning that it is really protruding out from his body.  It still seems to be working properly, but is definitely a challenge.  The biggest concern right now is that he is going through a lot of colostomy bags, sometimes 2-3 per day (they used to last 2-3 days or longer between changes).

 

The Lord truly is merciful and gracious, through all of the ups and downs.  Despite his physical shortcomings, Jacob has developed a tremendous vocabulary.  He recently was able to “jump” a little and can even jog a little (in his own special way).

 

We have included some additional photos and even a “centerfold” picture of Jacob that appeared in a Johns Hopkins publication back in 2008. 

 

We thank you all for your continued prayers and this wonderful demonstration of love that you all have shown to us through your offer to assist us with our patio.

 

Through Christ Alone,

 

The Arrington’s

Jacob's Timeline 2006-2016

It is hard to believe, but we are in the 10th year of our Journey with Jacob.  For those who may not know his story (The Lord's story, told through Jacob), or wanted to fill in the gaps, we have prepared the following timeline:

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.