Lillian Grace's story: The case for removing half of a baby's brain

When Sandra and Neal Salerno welcomed their second child, a girl named Lillian Grace in December of 2011 – the blue-eyed, blonde-haired little cherub seemed the picture of perfection. But less than two years later, the couple were faced with an impossible decision when doctors suggested removing half of their daughter’s brain to save her life.

“By the time she was 6-months-old, she wasn’t reaching milestones,” Sandra Salerno of Houston, Texas told FoxNews.com.

One of those milestones was the size of Lillian’s head, which was measuring small for her age – a condition called microcephaly. But because Lillian was born four weeks early, the Salernos had largely written off her abnormal head size until she began to undergo early intervention physical and occupational therapies, and her limitations became more apparent.

So at 8 months old, the Salernos took Lillian to a pediatric neurologist for MRI scans of her head. The worried parents’ fears were confirmed when they saw the results.

“We just knew looking right away that something wasn’t right because the right side of her brain was way smaller than the left side,” Sandra recalled. “And that’s when [the doctor] told us that Lillian had a condition called polymicrogyria, and that it encompassed 90 percent of the right side of the brain.”

Polymicrogyria is a developmental malformation of the brain marked by an excessive number of small folds in the tissue. It can cause neurological problems, epileptic seizures, developmental delays, vision loss and muscle weakness or paralysis.

Depending on the severity of the condition, it can be treated with medication or surgery.

Finding the cause

For the most part, Sandra’s pregnancy with Lillian Grace seemed normal until she experienced a placental abruption at 36 weeks, and the baby was delivered via emergency C-section.

“While we were home with what we thought was a healthy child, this virus was just attacking her hearing and her brain.”

— - Sandra Salerno, mother

By the time she was 10 months old, Lillian Grace had already received the diagnosis of polymicrogyria, but doctors at the time told the Salernos that the condition could be something genetic. After tests failed to find any genetic link, Sandra noticed something else seemed amiss with her infant daughter, so she started doing some research.

“She wasn’t looking over or responding to me when I was talking to her on her left side,” said Sandra. “I started Googling hearing loss and polymicrogyria, and what popped up was CMV — I had never heard of it before.”

CMV refers to cytomegalovirus, which is a common viral infection  that can affect almost anyone without symptoms, according to the Mayo Clinic. It can spread from person-to-person through contact with bodily fluids, and can be dangerous as an active infection in pregnant women and those with compromised immune systems.

“CMV is the most common virus most people have never heard of,” Dr. Gail Demmler, an infectious disease specialist and CMV researcher at Texas Children’s Hospital told FoxNews.com. “Most of the time, when their babies are diagnosed with CMV, mothers haven’t heard of it – but it’s also a virus that every woman should know about.”

Sandra recalled a time at the beginning of her second trimester when she got sick, was not gaining proper weight and was feeling generally fatigued. Doctors diagnosed her with bronchitis and she eventually went on three different courses of antibiotics to try and rid her body of the infection. But after doing her research, she wondered whether she had been misdiagnosed.

“CMV can affect the liver the spleen the bone marrow, lungs, heart, but the most devastating thing it can do is damage the brain,” said Demmler. “It causes brain damage, vision impairment, hearing loss … but it can affect any organ system.”

Armed with this knowledge, Sandra took Lillian Grace to her pediatrician for a hearing test which confirmed she was suffering from profound hearing loss. At Sandra’s insistence, she was also tested for CMV infection which yielded a positive result.

“For six months, we lived under the cloud of not knowing and thinking she was fine,” Sandra told FoxNews.com. “While we were home with what we thought was a healthy child, this virus was just attacking her hearing and her brain.”

Sandra made an appointment for Lillian Grace to see Dr. Demmler to try and determine whether her daughter had acquired the infection after birth, or if Lillian Grace was the one in 150 children who are born each year in the United States with congenital CMV.

“Sandra is quite a remarkable mom,” said Demmler. “She found me because she was trying to put together why her daughter had polymicrogyria and profound hearing loss, and she found that CMV is a possible cause of both of those.”

Demmler helped Sandra track down the blood spot taken for tests after Lillian Grace was born. In the state of Texas, the blood spot is saved for one year after birth, so the two women appealed for the sample to be sent to the Centers for Disease Control and Prevention (CDC) for testing.

“All babies, when they’re born at two-days of age, they get a newborn screen for common conditions,” said Demmler. “The tests are sent to state lab and tested for many different diseases, not including CMV.”

The results from the CDC testing proved devastating for Sandra. Lillian Grace’s CMV infection was congenital – meaning Sandra had become infected while she was pregnant and passed the virus onto her daughter.

“I don’t blame it on the doctors that were treating me, it’s kind of going back to the curriculum in medical school – maybe it’s not talked about enough,” said Sandra. “What really kind of upset me is there are in utero treatments that can be done on the pregnant mother – they’re not FDA approved or anything for that use – but they are used off label and have been known to help.”

Demmler started Lillian Grace on antiviral medication, which Sandra credits with halting the progression of her hearing loss. Since then, Sandra and Demmler have been working to have CMV testing added to the newborn screening after birth in the state of Texas.

“I also personally recommend that every woman know her CMV status and ask her OB-GYN for a test,” said Demmler. “If she is antibody negative at beginning of pregnancy, she will know that she is very vulnerable.”

CMV is common among toddlers and school-aged children, so Demmler suggests all pregnant mothers take some precautions.

“Basically if you have an ounce of CMV awareness you don’t share food or drink with young children, don’t kiss them on the face, and wash your hands,” she said. “If you still want to love on your toddler give them a big hug and kiss them on the top of the head.”

Brain, interrupted

For the Salernos, getting a firm diagnosis of Lillian Grace’s condition and learning about how it happened brought some semblance of closure – but unfortunately, it was short-lived.

“We knew she was having seizures but we had no idea about the frequency,” Sandra told FoxNews.com. “By the time she was 18 months old, she was maxing out on three medications and still having up to 300 seizures a day – and a lot of the time it was in her sleep.”

In January 2013, the Salernos took her to see Dr. Michael Quach, a pediatric neurologist at Texas Children’s Hospital, where she underwent a 48-hour electroencephalography (EEG)

“Actually when they came and saw us, she was already having about 25 seizures per hour, so it was kind of shocking on our end,” Quach told FoxNews.com. “At that point, we tried to manage her with seizure medication as best we could, although I did try to bring up to [the family] early on that surgery was a real possibility.”

Fearing Lillian Grace’s condition would continue to deteriorate, Quach and a team of pediatric neurologists took Lillian’s case to an epilepsy specialist conference. The response, he said, was a fairly unanimous vote that Lillian was a candidate for hemispherectomy surgery – or the radical removal of the right side of her brain – and she needed it as soon as possible.

“Counterintuitive as it may seem, you can actually function fairly well with just one half of the brain,” said Quach. “So in her case in particular, we felt very comfortable disconnecting the right side of the brain from the nervous system.”

Lillian Grace had weakness on the left side of her body. At 19 months old, she had not started walking or speaking because of the polymicrogyria and subsequent seizures.

“In the vast majority of people who are right-handed, almost all have the language on the left side of the brain,” said Quach. “In very, very young kids … they don’t tend to pick a side [of the brain] for their language yet.”

Therefore, Quach explained, because young children’s brains have a great deal of plasticity, if you disconnect the side of the brain associated with language skills, the remaining functional hemisphere will pick up the slack. This is not the case with older adults who have already established their language skills, which is one of the reasons doctors push radical hemispherectomies for children like Lillian Grace.

Left untreated, doctors told the Salernos, Lillian Grace would enter a vegetative state.

“Eventually, they said she would die of the seizures if we did nothing,” said Sandra. “So the decision was pretty much made for us, but still heartbreaking for a parent … but without the surgery we wouldn’t have her.”

In August 2013, Lillian Grace underwent the 8-hour procedure at the hands of Dr. Daniel Curry, a pediatric neurosurgeon, and his team at Texas Children’s Hospital.

Surgeons removed part of Lillian Grace’s temporal lobe, down near her ear, to gain access to the fluid-filled ventricles in the center of the brain. From inside the ventricles, they were able to expose the “cables” which connect one side of the brain to the other, and disconnect them.

“The majority of children going through hemispherectomy have a profound improvement in their function: They move better, they think better, frequently, children finally begin speaking,” said Curry. “[Lillian Grace] was a great candidate for this type of procedure because all of her seizures were coming from just one side of her brain … and she was young … so she had all sorts of development that she could reroute and reschedule.”

Lillian Grace recovered in the neurosurgery and inpatient rehab wings for several weeks following surgery, and each day, seemed to show signs of improvement.

[The doctors] told me it could take up to 6 months until she was back to her baseline,” said Sandra. “But she crawled across the hospital room 10 days after surgery, so then we knew she was going to be ok. The staring spells, the twitching – all that was gone.”

The road to recovery

Aside from some peripheral vision loss and weakness on the left side of her body, Lillian Grace’s family have seen only improvements since undergoing the radical procedure.

“Post-surgery, she’s a different child than she was before,” said Sandra. “She’s very active, loves playing with dolls, likes to try to do everything that her big sister does. She’s walking now and tries to do her own little version of running – she never was able to walk before we did brain surgery, so her personality is really started to come out.”

When she’s not running Lillian Grace around for various therapies, Sandra spends time working with organizations like MaddiesMisson.org and StopCMV.org to educate women on the importance of CMV screening.

“To go through what we’ve been through is heartbreaking, but imagine losing your child,” she said. “We’re the lucky ones.”