Thursday, April 26, 2018

Our Hero

Each spring, public and private schools in New Orleans and its surrounding Louisiana parishes nominate one student from their school to receive an inspirational hero award given by a private sponsor.  The heroes are children who have made “overwhelming achievements despite physical, emotional, psychological, or social challenges faced on a daily basis.”  

This year principals and teachers from our local school nominated Henry.

He sure has been our hero.  Henry often makes surmounting obstacles look easy when we know theycan be very hard.   His resilience truly has been inspiring.

Henry goes to the Lusher Charter School.  We are very grateful for the kind understanding and support we've found there. 

Friday, January 19, 2018

Midface Distraction Osteogenesis (2nd Phase)

We returned to the hospital on Tuesday, January 2, for the 2nd surgery of the Midface Distraction Osteogenesis.  This allowed for an earlier removal of the rigid external distraction (RED) device that  had been attached to Henry's skull way back in the fall, just before Thanksgiving in November.  It also embedded metal plates and more grafted bone to hold in place the outward extension of his upper jaw that had been gained by daily adjusting the screws on the distraction device.  Embedding the extra plates and bone was an unusual measure that his surgeons felt was worth a try, since the alternative was to leave the device on for 60 more days.  The choice was Henry's to make.  He choose this extra surgery.

Henry had worn the device, which we called a halo, for 45 long days including a period of about 20 days in which, twice daily, we adjusted it slightly to pull the mid-portion of his face outward, after the 1st surgery had cut bone to free his upper jaw. Almost every phase of the Midface Distraction Osteogenesis, both surgeries and in between, was pretty excruciating and all of us including Henry's surgeons were anxious to get this all behind us.

"He sure is a tough kid," friends have commonly offered in kind observation.  He is indeed, in almost never complaining through the most difficult or painful challenges. But by nature he is far more innocent than tough. It's his own unique strength of incredibly sweet character that seems to carry him through.  He's just a really good kid.

Adjusting the Device
Adjusting the device required one full turn of two bolts on the front of the device once each morning and again each night.  Henry said he did not feel any pain each time we adjusted the device outward, even in the later stages when the turns noticeably had more resistance and when it was visibility obvious that his upper jaw had been pulled significantly forward.  This was perhaps the only part of the process that initially sounded worse than it actually was.

General Discomfort
The halo portion of the device was attached to Henry's skull with four bolts on either side and it was a substantial piece of equipment, made from titanium.  It was heavier than expected.  It was quite secure, however, and Henry got used to it relatively quickly.  The front-post distraction portion of the device had thick wires attached to a metal bar fitted to part of his upper jaw inside his mouth.  This appeared much more difficult to put up with than the halo itself.  His upper lip, which had been stretched and bruised during the 1st surgery, needed to rest against this bar.  Applying Aquaphor ointment on his lip to keep it moist helped a little. The area around the bolts on his head also needed daily care, with Bacitracin and dressing changes.  He was able to take baths and showers with a little extra caution.  Our doctors decided that a liquid diet needed to be continues pretty much throughout, although soft foods like yogurt or extra-boiled ramen noodles could be phased in within a few weeks after each surgery.  Henry just happened to undergo an adolescent growth sprout at the time, so he didn't so much lose weight as suddenly grow very thin and long-limbed.  (Overall, he did lose a few pounds.)

The 2nd Surgery
The difficulty of the 2nd surgery was eclipsed beforehand by the incentive that it would allow for removal of the halo device.  It turned out to be another lengthy and complex set of procedures, especially in embedding the two additional metal plates and more grafted bone material into the newly opened spaces between the vertical bone incisions that were made during that previous surgery on either side of his upper jaw.  Post surgery the result was a lot of very painful-looking swelling behind his cheeks and beneath his eyes, probably the most extensive swelling we've seen. This 2nd surgery required two full 2 days in the hospital that could easily have been extended to three, but Henry was very anxious to return home and our medical team has become quite trusting of us.  His hip is still very sore from where the bone was extracted for grafting, but its incision is healing well.

Three weeks after the 2nd surgery, most of the swelling had subsided, and Henry was able to return to school.  The Midface Distraction Osteogenesis definitely affected his appearance by setting his upper jaw more correctly in line with his lower one.  Less visibly he now has solid structure behind his upper lip, which will allow his medical team to next begin to plan for upper teeth.

The downside is that the distraction process severely stretched the roof of his mouth, which was homemade to begin with.  It is now very difficult for him to speak and his voice has a new nasal tone, from too much air from his nasal cavity mixing with air from below that would normally be cut off by his soft palate.  He struggles to pronounce many basic sounds.  His doctors say this could clear up in time as things stretch on their own or, more likely, the problem will need to be addressed through another surgery (p-flap).  At present the problem is creating some challenges in readjusting to school, but he is handling them well.

Tuesday, November 28, 2017

Midface Distraction Osteogenesis (1st Week After Surgery)

One week after his surgery Henry is home from the hospital and settling in.  The surgery itself was intense and for most of the week it wholly overshadowed the attachment of a rigid external distraction (RED) device to his skull. It was a variation on a procedure often used for moving the upper jaw and mid-face area forward to treat serious upper jaw misalignment with the lower jaw.

Called a Le Fort I osteotomy, this surgery basically cuts the bone to separate the upper jaw along a common horizontal fracture line where the bone is weakest just below the nose. Vertical cuts on either side of the upper jaw complete the separation. In Henry's case, a relatively large curved plate was installed in an additional step to span the open gap above his upper gum line where previous procedures had inserted grafted bone.  This will add stability to the comparatively soft bone-grafted bridge behind his upper lip.

Three surgeons, each taking turns, worked from within Henry's mouth and behind his upper lip to avoid leaving external scars. The procedure took about six hours since with so much hardware already installed in his mouth there was very little room in which to work and extreme care was needed to avoid cutting into several important facial nerves located in the same vicinity, nerves that were often exposed during the operation.

The expected three-day stay was challenging and we needed to push very hard to be released. Generally they let hospital patients go once they can be disconnected from IV liquids after handling a similar liquid intake on their own.  Similarly, patients also need to be able to expel liquids on their own and be able to handle non-IV medications.

During this surgery it was apparently very difficult to keep an enormous amount of blood from draining from Henry's mouth into his stomach, and more than could be adequately suctioned out as things were wrapping up in the operating room.  Beforehand, this was a child who had vomited maybe two or three times ever.  After the surgery he would almost immediately vomit up fairly large amounts of foamy blood and mucus each time we gave him small amounts of clear liquid and this continued well into the second day, which really set back his recovery time.  He safely turned a corner and was taking liquids by that second evening, but it was difficult to convince the resident doctor on call that things were going satisfactorily on day three, despite the advice of our attending nurses who agreed with us.

Henry really wanted to go home.  Eventually, we were able to record that he was drinking the equivalent of about a cup of liquid per hour, even still attached to IV fluids, and we were finally released on the evening of day three, returning home just in time for Thanksgiving the next day.

Photos we had seen beforehand of patients wearing the rigid external distraction (RED) device tended to otherwise show relatively mild effects.  These photos must have been taken weeks after the initial osteotomy surgery, which produces a lot of painful-looking swelling and continual drainage of a mixture of blood and mucus from the nostrils, both lasting more than a week.  This drainage is just part of the healing process.  The patient's lips may also be very tender and sore from the stretching that takes place during the surgery. 

Liquid Diet
Post-surgery we were asked to keep Henry on a liquid diet for at least a week before gradually also adding in very soft foods.   This was a challenge for him given that his stomach had already been made a little sensitive and that the large number of medications (pain, antibiotics, etc.) we needed to give him were also all in liquid form.

The RED Device
The rigid external distraction (RED) device attached to Henry's skull turned out to be a different model than the one we had seen as a example, but it might be an improvement.  It attaches to his head with four bolts on each side instead of three and along more of a curved line.  The result seems very stable, which had been a concern.  It is obviously unconformable but so far he is handling it very well.  Home care requires daily cleaning and changing the dressings around these bolts.

We returned to the hospital five days after the initial surgery for the first of the daily turns of two front screws on the device, which will slowly extend the middle portion of his face outward.  Our medical team adjusted the time frame for this process after the surgery.  Basically, they would like us to make the turns twice each day for a little more than two weeks and then they'll review the progress.  Four days along Henry doesn't feel anything from the turns yet, and doesn't seem to mind the routine.  We've been told that he will very likely begin to feel some discomfort around the second week or so.  One can see that his upper jaw has already visibly begun to move forward.

Once this mid-face extension process is complete, it seems the options for what to do next have now been reversed.  As of today Plan A would keep the device attached for about 60 additional days, hoping for supporting bone to grow.  Plan B would remove the device sometime just before Christmas in another surgery that would embed plates and more grafted bone to secure the outward extension.  We'll see.

Other than general discomfort, the primary challenges posed by the device seem to be achieving a good head position for sleeping, which we've addressed by tilting the front portion of his mattress upward instead of using pillows, and that the device makes it extremely difficult to drink from a cup, which we've addressed by collecting a battery of syringes in a variety of sizes that Henry largely uses himself.

Henry has handled all of this by absorbing each challenge and then assembling the will to overcome it, and at this he is very practiced. But it’s clearly been very hard. He’s a remarkable kid.