Nick’s NMO Story

By Rosanna D’Elia

In early November 2000, my son Nick started becoming ill. He was just done eating dinner when he vomited across the kitchen – I was shocked. Up to this point, he was a healthy twenty-three-month-old who was active, talking and above average in his skills and was never sick a day in his life. After vomiting, he started having a fever of 103.5 degrees Fahrenheit that would not go away even with an antibiotic. He was checked for infections by the pediatrician, but they found none – we were told it was just a virus.

The day after Thanksgiving, he was very lethargic, with labored breathing and a twenty-four hour bout of persistent hiccups.  I was very concerned and called St. Peter’s Hospital in New Brunswick, New Jersey to speak to the on-call nurses, and they recommended bringing him in. We brought him in, and they admitted him to monitor his lethargy and dehydration. He was mistakenly given too much IV fluids and had a thirty minute seizure as a result of his sodium level dropping from 132 to 118. After they stopped his seizure, the PICU kept him comatose and intubated for three days. After being extubated, he was awake and functional until three days later when neurologically, he declined. He lost his speech, ability to move, and was barely responsive. He still had a fever and started having a rash that spread all over his body. They had tested Nick for all kinds of illnesses and viruses, and all the results came back negative. They performed an MRI, and it showed a lesion in the Medulla – the lower part of his brainstem. They treated him with IV steroids, then three rounds of IVIG. His condition was not improving and only declining each day.

On Nick’s 2nd birthday, December 3, 2000, we decided to have him transferred by ambulance to the intensive care unit of Children’s Hospital of Philadelphia in Pennsylvania (CHOP). Upon arrival, the entire neurology team saw him, and we were told that IF he survived he would remain as he was – unresponsive. He was contracting, was not awake and only screamed day and night. CHOP gave him more IV steroids upon arrival, but he showed no improvement. A few days later, his heart rate lowered to 37 beats per minute, then the next day it was near 200 beats per minute. We were expecting him to crash at this point. They performed another MRI at this time, and the inflammation in his brainstem was increasing into the Pons section. CHOP did not know what he had, but they were assuming that it was either central pontine myelinolysis (from the rapid sodium fluid shift) or ADEM. There was no definitive test at that time for NMO. Nick started to slightly improve with time, crying less, becoming a little more awake, starting to have voluntary movements, and his fever had subsided. They transferred him to their rehabilitation hospital where he received intense physical therapy and care.

Nick started to improve a little each day until New Year’s Day 2001. He woke up that morning with an increased temperature, and his face was all distorted. Half of his face was not moving, he had difficulty swallowing, his eyes were looking in different directions, and the muscles that held portions of his eyes and lids were not working. His cranial nerves were affected badly as was evident on his face. Again he was transferred to the ICU in CHOP, and he was given more steroids and had another MRI. The lesion had now spread into the Midbrain section of his brainstem and was extending into his thalamus. He remained there for another week and was released back to rehab when they saw that he was not getting any worse.

Since we lived two hours from CHOP, we decided to have him transferred to a local rehabilitation hospital in New Jersey on January 21, 2001. He remained there as an inpatient for one week, then was released to start outpatient therapy daily for nine months. He also started vision therapy including patching because his eyes were affected so badly this time.

We were told that what Nick had was a one-time occurrence and would never happen again – we were elated! We saw him recover most of his abilities, and the only residual effects were facial weakness and decreased vision in his left eye which was 20 X 400 – his right eye recovered back to normal.

Nick had remained stable with just a few bouts of optic neuritis (several blurry eye episodes all in hindsight now) for nine years. He wanted to play football, so we allowed him to start summer practice, but he quickly started not feeling well. Once they started practicing with helmets on in ninety degree weather, Nick started feeling sick, unable to sleep, having headaches and then he couldn’t see out of his good eye. We rushed him to the pediatrician’s office who told us to take him to a doctor who specialized in demyelinating diseases. Nick was admitted to St. Christopher’s Hospital in Philadelphia where he was diagnosed with optic neuritis after an MRI was done, and he was given five days of IV Solumedrol. Nick was only able to count fingers in front of his face at that time. He slowly recovered some vision and was released home to recuperate more. Two months later I received a letter from the Mayo Clinic stating Nick was NMO positive, and thus our journey was now on a known course. We took him to the MS clinic at CHOP and went under the care of a neurologist there. At that time we decided not to put Nick on any immunosuppressants unless we absolutely had to.

He was stable until last March 2012. Nick was battling Strep Throat from January through February when suddenly his symptoms started to change. He complained of a horrible headache over his eyes one evening, then woke up the next day slightly forgetful. On March 12, 2012, he went to school, but I was called to get him when the teachers noticed Nick was not communicating verbally and seemed disoriented. I rushed him to CHOP, and by that time he was having difficulty moving his right side.  They admitted him and gave him IV steroids. Within two days, Nick could say nothing more than yes or no and was completely paralyzed on his right side. He had an MRI, which showed a very large lesion on the left side of his brain, so they decided to do 7 rounds of plasma exchange (PLEX) to try to help him. He slowly started improving, but we all decided then that Nick HAD to be on meds. He had two Rituxan infusions, then three rounds of Cytoxan, while starting CellCept for daily use. Nick also has Sjogrens (positive blood test while admitted and parotid gland involvement) so they started him on Plaquenil as well. He improved quickly and was transferred to their rehabilitation hospital. He stayed there until April 5, 2012 when he was released for outpatient therapy at a location closer to our home in New Jersey.

It has been a very long battle for us, more so for Nick, but we try to remain hopeful and stay one step ahead of his NMO. He does have frequent fatigue and extreme heat sensitivity affecting his vision and right-sided movement, but he copes very well. Nick is truly a miracle and an amazing young man! Thank you TMA for your support and research to help those like Nick!

Read about Nick’s experience at camp here.