Hi Friends,

As you know, Rees had his lumbar shunt removed almost a month ago now and since then he has had problems with headaches and vomiting episodes.  It's been very difficult.  It's been confusing.  There have been so many things happen so I'm just going to get this all out so that I can keep everything straight, but also to provide an update.  I will just start from the beginning and go in order as best I can.

Rees had multiple surgeries last year, as some of our faithful readers know, and one of them was to place the lumbar shunt to facilitate proper healing after numerous complications and a possible CSF leak.  Whether or not it really did what was intended, it worked.  Rees FINALLY healed, though very slowly, and we began to make forward progress.  Then we had the "dud" surgery in July and found that Rees' skull was going through bone resorption but by the time he had the next surgery in October, things were improving and he finally had a surgery that seemed to go really well, without all sorts of complications.  Over time, Rees periodically had small bouts of lethargy and vomiting.  After this had happened a few times, we realized that the issues were likely linked to the lumbar shunt, ... something that is not uncommon.  Knowing that Rees was coming up to his next surgery which was scheduled for January, we felt that it was time for a CT of his skull.  We could feel pockets and divots on his head and gathered that there were portions of his bone that was not filling in or growing as was expected.  We had actually asked about this during the summer but the surgeon did not want to "see it" just yet.  Foreward to December and sure enough, the CT revealed that Rees' forehead is collapsing and he has tumor growing just below the forehead.  The original plan to remove existing hardware and do a bone graft was going to have to be altered slightly as now the craniofacial surgeon felt that Frontal Distraction would be necessary to correct the collapsing forehead.  The consensus was (as far as we can accurately tell) that if Distraction was going to be part of the plan then the Lumbar Shunt should be removed. [ Distraction osteogenesis (DO), also called callusdistraction, callotasis, and osteodistraction is a process used in orthopedic surgery and oral and maxillofacial surgery to repair skeletal deformities and in reconstructive surgery. ]

Soooo ... about a month ago, Rees had the surgery to have his lumbar shunt removed.  Easy Procedure.  Quick.  In. Out.  No. Big. Deal ... Right??  WRONG!!  Well, he was so sick and we were in the hospital with him for four nights.  Since then, he has been quite ill on and off with headache and vomiting, dizziness and lethargy.  It has been so discouraging and even frustrating because we didn't have any clear indication as to why it was happening.  We were told it could just be that he was adjusting to the change because for nine months his body was used to having the fluid around his brain regulated.  We were told that he MAY have a leak somewhere and that it might be necessary to do a Blood Patch.  We were told that maybe he had an underlying GI issue that we just weren't aware of. [ Lumbar–peritoneal shunts are used in neurological disorders, in cases of chronic increased intracranial pressure to drain excess cerebrospinal fluid (CSF) from the Subarachnoid cavity ... ]

Because he was so down and out with these inconclusive, misunderstood symptoms, the craniofacial surgery that was scheduled for end of January was cancelled.  It was rescheduled for today.  However, in the last week, Rees has been ill with the vomiting and then ... THEN CAME THE FEVER.  Oh BOTHER!

On Sunday, After a few good days, the vomiting started again and this time accompanied by a fever.  Nothing outrageous ... considered low-grade, but nonetheless, a fever that caused us to wonder if his surgery would yet again be affected.  We had an appointment scheduled for Monday because Jonah needed follow-up from his surgery and Rees needed pre-Op review.  Well, I called to cancel thinking that they wouldn't want to see him with a fever and that the surgery would be cancelled ....

... BUT they still wanted to see him.  However, once they did see him, they were VERY CONCERNED (Monday was a bad day with the vomiting).  They sent us to the Anesthesia department for evaluation and then to the lab to have some blood drawn.  Rees' (WBC) white blood cell count was nearly double the high end of the normal range, which indicates infection.  Uh-Oh!  This raised all kinds of red flags, understandably ... but frustratingly, as well, as it would likely mean more delays with Rees' craniofacial corrections.  [All this time, Brian and I felt that the real problem was hidden "in plain sight" within Rees' collapsing forehead.]  We were told to monitor the fever and keep track of any additional symptoms.  We were also told to still come in on the day of surgery but to know that there would likely be changes to the procedures.  We were told that the Blood Patch would most likely take place no matter what in the hopes that it would relieve the vomiting/headache issue.  [ An epidural blood patch is a surgical procedure that uses autologous blood in order to close one or many holes in the dura mater of the spinal cord, usually as a result of a previous lumbar puncture. The procedure can be used to relieve post dural puncture headaches caused by lumbar puncture (spinal tap). ]

So, we arrived early in the morning today and went to Pre-Op, as usual.  We were trying very hard not to talk about what was going on and what might happen because we did not want to raise Rees' anxiety level.  But he is a smart cookie and we know that he was contemplating every move.  He knows the rooms, the routines, the questioning, the vitals ... he knows what it all leads to but we told him only that we knew he needed to have additional labs done because that was what we knew FOR CERTAIN before anything else would take place.  The labs were done and we waited.  And.waited. And.waited.  After a while, the neurosurgeon came and spoke with us to let us know that he was recommending that WE NOT MOVE FORWARD with the craniofacial portion of the surgery, however, he wanted Rees to have the Blood Patch, but AFTER having a Spinal Tap to check for infection. [ A lumbar puncture (also called a spinal tap) is a procedure to collect and look at the fluid (cerebrospinal fluid, or CSF) surrounding the brain and spinal cord.  During a lumbar puncture, a needle is carefully inserted into the spinal canal low in the back (lumbar area). Samples of CSF are collected. The samples are studied for color, blood cell counts, protein, glucose, and other substances. Some of the sample may be put into a container with a growth substance. This is called a culture. If any bacteria or fungi grow in the culture, an infection may be present. The pressure of the CSF also is measured during the procedure.]  What's important to know here is that Rees no longer had a fever and his labs came back ALL NORMAL, including the WBC.  

So, I was very hesitant to agree to this because one of the most common side effects of a spinal tap is a spinal headache (which is WAY WORSE than the headaches that Rees has been having; I know because I've had one).  I felt like we were just giving permission to take two steps back, which I was certain would be the case.  I wasn't certain that it was actually necessary because the labs had come back normal so, in my mind, there was no reason to check for meningitis or some other serious infection.  However, we did end up giving after I had my mini-fit in the middle of the Interventional Radiology department.  The Radiologist and Anesthesiologist carefully, calmly, and collectively discussed the importance of the procedure.  There tried really hard to bring me down to a level of rational response ... which they did very well, I might add ... so in the end, we consented because we did feel that it was important to get the information regarding the pressure of Rees' CSF (cerebral spinal fluid), something which had NOT been mentioned until now but was something that we had wanted all along; there just didn't seem to be a connection.  

So, we went to the OR waiting room and chatted and just hung out somewhat deflated because of the detour we were taking.  When it was time for a parent to be able to go to Recovery, Brian went ... it was his turn.  Yes, we take turns.  And when it was finally time for me to get to go back, I was all ready to see my little guy upset and groggy and fussing but he was not.  He looked wide awake, alert, and content.  He was playing on his little device.  I thought, that's odd ... but was then told that he had been crying a lot and that helped to calm him down as well as the versed drug they gave him.  Ooo ... okay, good.  (I wish I would have taken a photo of him like this)  But then in a matter of seconds, a flood of information came in that took us for a sharp turn on this roller coaster ride.  WE WERE MOVING FORWARD with the frontal distraction!!  What?  Really?  What's the deal?  
Apparently, Rees' CSF pressure was measured first during the spinal tap.  Now, I don't completely understand all of this but I am beginning to get a much clearer picture.  Normal "CSF pressure, as measured by lumbar puncture (LP), is 10-18 cmH₂O (8-15 mmHg or 1.1-2 kPa) with the patient lying on the side."  Rees' pressure was 50 at resting state (yep, FIFTY!)  That is not good and is a flashing.red.warning.light!  This is the reason Rees has had so much trouble since the lumbar shunt was removed.  Rees' brain is growing but his skull, due to all the problems he's had, is not growing at the same rate and, in fact, has reduced in size due to bone resorption and collapse and has created space issues.  Uhm, ... didn't we ask about this?  Why, YES we did!  Hmmm ... like three times in the last six weeks!

I wasn't upset, though ... I was ecstatic, really!!  I was just so relieved that the situation was going to be taken care of, that Rees was going to be relieved of all that he had been struggling with since the shunt was removed.  It made perfect sense to us and we don't know why it was overlooked ... too obvious perhaps ... but the shunt was helping to regulate Rees' CSF pressure but when the shunt was removed, the regulation was gone and due to the crowding that was taking place, the pressure was dangerously elevated and needed to be addressed right away.  Rees was taken back into surgery shortly after that and the neurosurgeon removed a part of the front of his skull to immediately relieve some of the pressure and the craniofacial surgeon affixed new hardware and also distraction devices which will be adjusted daily to further tilt the forehead to allow pressure relief and new bone growth.  That is the idea anyway and we are praying that his body cooperates with this plan.  He lost a considerable amount of blood again which was, of course, replenished and will continue to be given as needed while in the ICU tonight.  

So, while we still did not move forward with resectioning the hamartoma (the new tumor growth) and the nasal bone graft seems to get further and further away from us, we are very happy that the issue of his ICP (intracranial pressure) has been addressed and we should hopefully see some improvements after a few days of recovering from the surgeries.  Please pray for that with us!  Please pray for this little trooper-of-a-kid!  He's been through so much and we just want him to stay STRONG in Spirit and Heart.  

There's so much more I could say but this is already a long post so that's it for now ... will update again later.  You can count on it but I'm sure you didn't have any doubts, anyway!  (wink, wink)