April 19, 2018 "Optic Nerve MRI #2"

I AM BEYOND AMAZED with this precious boy!! I have been telling him all week about how they were going to lay him on a table and take pictures of his head in the tunnel. He was a little scared when I took him into the MRI room. He was shaking all over and about to cry. He told the technician, Brian, to take off his gloves. I told Brian how Rayden thinks you are going to hurt him if you are wearing gloves. He immediately threw those gloves on the floor. Rayden started laughing. He allowed Rayden to put in his own ear plugs and the two pillows beside his ears. He even played with the helmet a little before putting it on Rayden. I just knew when they moved the bed into the tunnel he was going to cry, but I was wrong. He just laid there and said "that's a loud camera." I believe my baby is growing up. He is officially the bravest three year old I've ever met!

Dr. Elton was floored that Rayden remembered he wore glasses last week but not this week! He said he is very observant, repeating it several times. He just couldn't get over it. He said there cannot be anything wrong with this child's brain if he has no memory loss!

He just doesn't see any evidence of brain pressure. His suspicion is that Rayden does not have brain pressure. He can't explain the optic nerves swelling. He doesn't want to do anything to put his shunt at risk if it isn't necessary. He just feels inclined to watch him. He says the plan is to take new optic nerve photos on May 3...
     1-If they are better, then we are good.
     2-If they are worse, he will operate on the shunt.
     3-If they are the same, then we watch his eyes on a closer basis.
     4-If his vision starts changing then he will operate.
He says he may be inclined to say this is what Rayden's optic nerves are going to look like. His brain, skull, ventricles, feet, hips, ankles, spine, kidneys, EVERYTHING, just doesn't look like everyone else's. So why are we surprised when his optic nerves look different??? Hmm, maybe he's right. Dr. Elton has told me on many occasions that Rayden doesn't follow the text books, he writes his own book. As long as Rayden is happy, and healthy, I am fine. This just reminds me that a Spina Bifida patient is never OUT of the woods. We just learn to survive IN the woods.

April 12, 2018 "SB Clinic and Neuro exam"

Most of Rayden's appointments went great today. There were really only two concerns (below). Dr. Alexander says Rayden seems to be right on target with his developmental milestones. He weighs 33lb. without his cables (60th percentile) and he is 3'3" high (79th percentile). His BMI is in the 25th percentile, which is exactly where Dr. Alexander wants him to be. 

Neurosurgeon

Dr. Elton was troubled by the optic nerve swelling. He says Rayden just doesn’t look/act like a child with shunt malfunction. He says the only way you can get brain pressure if you're not draining fluid but he doesn't see any evidence of this on his MRI. He's thinking there may be debri in his shunt but maybe not enough that is causing it to totally shut down. He could tap the shunt but he doesn't want to expose him to any unnecessary infection. He decided to adjust the shunt valve anyway to drain more CSF off his brain hoping to alleviate any pressure. He scheduled a second MRI for next week. He says the previous one from January showed no tumor or brain bleed. We will have more nerve photos taken May 3. If they are still swollen then he says he will be obligated to operate on the shunt. He will take it apart in the OR to see if it is clogging or whatever. If the optic nerves remain this chronically swollen long it may cause permanent vision problems. Rayden already has residual scarring on his optic nerves but that is probably from his previous hydrocephalus issues. He doesn't need any more damage to his optic nerves. 

Renal Ultrasound/Urologist
We have realized that Rayden has associated gloves with pain. He asked the technician to take off her gloves to do the ultrasound. When she did, amazingly Rayden just laid there and allowed her to take fabulous pictures of his kidneys and bladder. Dr. Ross says his bladder and blood work looks good. She mentioned his kidneys have not grown much since his last ultrasound. She is alarmed but not worried yet.

After six, long appointments today, Rayden is worn out! He is such a trooper to get pulled every direction and he takes it so well.

April 5, 2018 "Optic nerve ultrasound"

I really never thought Rayden would cooperate during the eye ultrasound. What three year old sits still while someone rubs jelly over their eyelids with a probe? Boy, was I wrong! He was amazing.

A few hours after we left, Dr. Grace contacted me with the results. The ultrasound revealed both optic nerves remain swollen but there is no sign of drusen. I was really hoping drusen would be the culprit because the alternative can be really scary. Dr. Grace seemed to be as unsettled as me because she stated she was going to page Dr. Elton, Neurosurgeon, tomorrow. She sent him the optic nerve photos from last week but he was out of town. The fact that she is paging him has me alarmed. She also asked the radiologist to take a second look at the MRI that Rayden had in January. He originally said his ventricular system was mildly increased from last year but nothing out of the ordinary. His re-read was the same. But my mind wonders if he was only looking for intracranial pressure or ventricle sizes NOT tumors or abscesses. Something HAS to be causing his optic nerves to swell.

(Some causes of increased pressure from CSF and papilledema are brain tumors and brain infections, such as brain abscess, meningitis or encephalitis. One condition can cause increased pressure in the CSF without associated swelling of the brain or ventricles. This condition, called pseudotumor cerebri or benign intracranial hypertension, is caused when the body makes too much spinal fluid. This is a common cause of papilledema when the brain scan is normal.) Pseudotumor cerebri really doesn't make sense to me because Rayden already has a shunt that is suppose to drain excess CSF. I'm certainly NOT a brain surgeon but I just have a bad feeling about this.
The life of a person with Spina Bifida...It can be ANYTHING at ANYTIME all your life!!

The next day Dr. Elton's nurse, Melody, called to say he wanted to see Rayden next week. She didn't say what he wanted to do or what he was thinking, just that he wanted to 'see' him.

April 1, 2018 "Easter"

Easter 2018 Celebrations

  

March 29, 2018 "Optic nerve edema #3"

Rayden's vision remains the same and his optic nerves are still swollen! Dr. Grace is sending the new photos to Dr. Elton, Neurosurgeon, because she is not 100% sure his shunt is not malfunctioning. She stated that since his optic nerves have been swollen since January (that we KNOW of) she is calling it "chronic papilladema". The only other thing she thinks it could be is drusen.  (Optic nerve drusen are abnormal globular collections of protein and calcium salts which accumulate in the optic nerve.  Drusen usually become visible after the first decade of life, but can be seen before age ten in some children.) We will be going back to UNC next week to have an ultrasound of his eyes to rule out drusen. She thinks the likelihood of it being drusen is very low but how many times has Rayden NOT followed the text books? She also thinks a repeat MRI may be a good idea.

Left: March 29         Right: February 22

Causes of papilledema in the pediatric population may include, but are not limited to, Guillain-Barré syndrome, spina bifida, hydrocephalus, intracranial mass, trauma/subdural hemorrhage, meningitis, subdural venous thrombosis, arteriovenous malformation and idiopathic increase in intracranial pressure.