Khai Louies Cranial Vault Reconstruction Surgery

We have been asked by so many friends and family “how they can help” and I think this is a starting point…You don’t realize how an unexpected event, especially one like this can affect your life, and how things add up, quickly. The list is lengthy but almost self explanatory on the finical impact an unforeseen medical emergency like this one can create.


This Tuesday March 24th, he will be undergoing a total Cranial Vault Reconstruction Surgery, NOT for reconstruction, but for his brain to be able to expand properly. Currently Khais Sagittal, and coronal sutures are fused, and his lamdoid suture is almost done fusing. This means that Khais entire skull, over the past two years, has been slowly starting to close. His brain has no where to go as it expands, so puts pressure on things it is not supposed to, leading to ICP and papilldema.

 The surgery will be 6-8 hours long. They will not only cut Khais skull in over 10 places, but he will have his eyes stitched shut, as well as a wire threw his chin area to hold up the breathing tube. His incision will be from ear to ear, over the top of his head.

I have given the hospital 5 pints of my blood for his blood transfusion  as well. That in itself made my heart ache knowing that my two year old would be receiving a blood transfusion, of my blood to replace the blood he will loose in four days....

Recovery time in the hospital is 5-7 days, and then continues at home for 4 weeks.

 As you know, he has been diagnosed with a rare form of Craniocynostosis, called Progressive Postnatal Pansynostosis. He has high ICP (inter-cranial pressure) surrounding his brain, his spinal tap came back at 33, and its supposed to be at 25, so, very high. He has a mild case of Papilldema (swelling of the optic nerve) in his left eye, and small traces of papilledma in his right eye.

 

Im not sure I am ready for this….but going to try my best to handle it.




 Unimaginable to say the least, here is the story on how we got here:

 Wednesday, March 11th , I took Khai to see a Pediatric Ophthalmologist, after going to his 2 year wellness appointment, where his pediatrician said she thought he was farsighted and had an astigmatism in both eyes. Many ask how on earth his doctor knew that he may have needed glasses if his eyes look completely normal…they have some nifty handheld eye camera that takes a picture of the kids eyes, and spits back numbers that may give clue to that that child may need glasses.

During that eye doctor appointment, we left with no glasses, but instead two hours later, she told me that she was seeing fluid behind the eyes,  and that I needed to schedule an MRI of his head, and most likely would need to make an appointment with a Neurologist.

I was mind blown, but I said, okay no biggy, I’ve got this.

I went home, thought about what she had told me and really wondered about the severity of fluid behind the eyes.

I texted my cousin (who’s married to a doctor) and texted my bosses (a lawyer and doctor) to let them know what was going on…and I believe all three of us were on the same page that this particular situation was possible more urgent than the pediatric ophthalmologist may have thought.

I couldn’t sleep much, and wondered if I needed to wake Khai in the middle of the night to take him to the emergency room. At 4AM Thursday March 12th, I got up, showered, packed all of his and my things for a few days, woke him up and took him directly to Children’s National Hospital DC.

We waited for 8 hours before we were seen by the ophthalmologist.  Who then told us that Khai, who hadn’t eaten or drank anything, still couldn’t in case a slot for a sedated MRI and spinal tap opened up.

At 6:30PM, I got word that there was no slot available for Khai until the following day, which meant that after 22 hours, with no food or water, Khai could finally have something. Thank God!

At 10PM, I was told that a CT scan of his head was requested by the neurology department, and that would be enough testing to be discharged from the Emergency Department and admitted to the Neurology Department.

The CT scanned showed no tumor, and I was so relieved. The “worst” case scenario was taken off the table. That still left us with high ICP and papilledma in the left eye. We were all hoping that there was simple protein build up behind his eye, or a Pseudo Tumor scenario, where Khais brain creates the same chemicals that an actual tumor would create, but the tumor doesn’t actually physically exists, but his body reacts symptomatically as if there was one.

 We were then admitted to the Neurology Department and were in a room by 3AM.

 The following day Neuro ordered a sedated MRI and spinal tap… it was the longest 2 hours of my life as I patiently waited for Khai to be in recovery, and begin waking up.

 I honestly figured this was finally his big break, they were going to find nothing, and that we could head home on a medication to help decompress the pressure surrounding his brain. I wanted to go back to him just needing glasses, and just going to his regular therapies. Back to life as we knew it at home with none of this very scary medical neurological terminology, testing and hospital admissions.

 

I was so wrong.



 I am not sure any mom, that originally went to get their kid glasses, could be prepared to hear what I was about to hear.

 The next morning, during rounds, the first doctor to speak to me was the Neuro Surgeon himself.

 He explained that they found things they did not want to find in the MRI of Khais brain, nor the spinal tap. His spinal tap levels were very high, they came back at 33, and that it is normally at 25, which means he has elevated inter cranial pressure (ICP) surrounding his brain, which could be part of the cause of his papilledma.

 But the bigger underlying issue at hand was the sutures in his skull. His sagittal suture is completely fused, coronal is completely fused, lambdoid is almost done fussing and several of the other smaller sutures are beginning to fuse throughout his skull.



 When children grow, their brains grow as well, that brain needs room to expand. When the skull fuses too early, there is no where for the brain to go, and this causes increased pressure around the brain, extra fluid, papilledma, ICP and many other complications that can lead to children going blind, or ultimately, so sick, they could die from the continuous elevated pressure.

 Kids with Craniosynostosis are usually diagnosed at birth as their head shapes are very distinct and abnormally formed. The problem is that  Khai has a very rare type of Craniosynostosis called Progressive Postnatal Pansynostosis (PPP).  There is no way to have detected Khais PPP any earlier as his head looks completely normal.

 There have only been 17 cases of this specific diagnosis and surgery done at Childrens National Hospital, just to give you a perspective as to how rare this form of Craniosynostsis is.



 Because if he rareness of Khai’s Craniosynostosis, many doctors at Childrens are watching Khai’s case. Interested to see what happens after surgery, they have come in to see him, as they don’t see kids that look so very normal on the outside but are experiencing pain on the inside. They don’t see kids who are so happy, who are so active, who are so…Khai!

 Khai will have Total Cranial Vault Reconstruction surgery. It is NOT for reconstruction, but instead for expansion and out of medical necessity, which is also not common. Khai happens to have two reasons, his ICP and papilledma, as well as the fact that parts of his skull have already fused and others are fusing… we are left with no other options.

 It is a major surgery. It is happening.
Tuesday March 24th 2020.

It will not be easy.
 
But with the love and support of our family and friends, I know we can pull together for Khai.

 I love you, Khai Louie.