Cochlear Implants as a Journey to Hearing: Gracie’s Story

Since Kathleen had experienced a healthy pregnancy, she and her husband Mike were expecting a healthy baby girl.

“Out she popped, and everything was perfect,” Kathleen recalls. “When she failed her newborn screening test, we weren’t that concerned. We knew that babies could fail that test for a number of reasons, even if they have no hearing problems.”

baby-gracie
Baby Gracie

Two weeks later during a visit with her pediatrician, Gracie failed another hearing test. Her family was referred to Providence Speech and Hearing Center.

More testing provided a diagnosis: Gracie had severe to profound bilateral hearing loss. What hearing ability she did have would not be enough to allow her to speak and learn language.

“When I realized my daughter couldn’t even hear me tell her that I love her, I was a mess,” Kathleen recalls. “It felt like the world was falling apart in that moment.”

Gracie tried hearing aids, and although she was finally able to hear her parents say her name, the hearing aids were not enough to compensate for her hearing loss.

Since Kathleen is a special education teacher, she was already familiar with cochlear implants—surgically implanted devices that offer the hope of gaining the ability to sense sound.

“Deciding to pursue cochlear implants was a pretty easy decision,” says Mike. “We didn’t really give it a second thought. As a parent, you just want to give your kid every opportunity possible—especially the chance to hear.”

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Gracie was thrilled to get cochlear implants.

The cochlear implant artificially stimulates the inner ear area with electrical signals, which sends those signals to the auditory nerve, letting a person hear. The surgery is safest in most children around one year of age.

Meanwhile, Kathleen and Mike had been teaching sign language to Gracie and her older sister.

A few months after Gracie’s first birthday, she underwent bilateral cochlear implantation surgery at CHOC Children’s Hospital with pediatric otolaryngologist (ENT) Dr. Nguyen Pham.

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Gracie was excited and happy the day of her cochlear implant surgery.

“When I first met Dr. Pham, I felt like I had known him forever,” Kathleen says. “He answered every single question we had and was honest with us throughout the process.”

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Gracie’s parents were thrilled that her bilateral cochlear implant surgery went perfectly.

Kathleen knew the benefits for Gracie outweighed the potential complications that come with surgery, but as a parent, she was naturally worried about her child’s well-being.

“Every time I saw Dr. Pham I was frantic, and he was always so calming. It was nice to have that reassurance,” Kathleen says. “Dr. Pham is a miracle worker.”

Surgery went perfectly, and Gracie spent just one night in the hospital. A few weeks later it was time to officially activate her cochlear implants.

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Gracie was all smiles on activation day for her cochlear implants.

Even though Kathleen had faith the cochlear implants would work, but she was still anxious for activation day.

“Activation day was amazing and terrifying at the same time,” she recalls. “Everyone said not to worry, but I thought it might be too good to be true. I just kept thinking, ‘How could this little device be the thing that allows my baby to hear?’”

As soon as the cochlear implants were activated, Gracie’s face lit up and she pointed to her ears. Her parents let out a sigh of relief. When they took her home, they found the most joy in the small, mundane sounds around their house. Gracie could finally hear the front door open, the dog bark, and most importantly—her parents say I love you.

Before a child with cochlear implants will begin talking, they need time to get comfortable wearing their implants and undergo speech therapy. For Gracie, that time was just a few weeks. She still undergoes speech therapy twice per week, once at Providence Speech and Hearing Center and once at home.

To say Gracie is thriving is an understatement, according to Kathleen. Not only does Gracie talk and sing non-stop with her big sister Tess, but she mastered her ABC’s just shy of her second birthday. Her language skills now exceed most normal hearing children. Gracie loves going to Disneyland, and her family appreciates any opportunity to spread education about cochlear implants to inquisitive people they meet.

gracie-with-cochlear-implants-pumpkin-patch
At Disneyland and other places, Gracie’s family appreciates any opportunity to spread education about cochlear implants to inquisitive people they meet.

Gracie’s family has much to celebrate this year, but there’s something a few years down the road that Kathleen is most excited for.

“I am excited for her to go to kindergarten at a typical school. You have all these visions of your kids growing up, and for me to just drop my daughters off at one school is a big thing.”

Meet the cochlear implant team

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Big Sister Gives Back to Honor CHOC Children’s

Lily’s big sister, Makenna loved her before she was even born. She was actually the one who chose Lily’s name. Their bond only grew stronger when Lily was born with a rare chromosomal abnormality requiring specialized treatment and extra help at home.

Lily and Makenna
Lily and Makenna

“Even though Lily is different than Makenna and her brother, Ethan, they just see her as their sister,” says mom, Danielle. “They’ve helped Lily learn sign language and practice pronouncing words.”

Lily’s entire family has been involved in her care from day one. Makenna, age 11, would regularly tag along to Lily’s physical and occupational therapy sessions. She was keen to learn how she could help take care of her sister. She watched closely as physical therapist Cathy Lopez taught Lily how to move her body, and occupational therapist Lauren Newhouse taught her how to eat solid food (her favorite is avocado).

Seeing the way CHOC’s rehabilitation services team cared for Lily and the progress she made in therapy inspired Makenna to give back.

Makenna’s fundraiser

When Makenna’s school hosted a fair for students to showcase causes important to them, she chose to honor CHOC. She and a few friends watched YouTube videos to learn origami and created dozens of animal and emoji-themed bookmarks. She quickly sold out of them and donated the proceeds to the rehabilitation department at CHOC.

Lily backpack

“Raising money for CHOC is the least I can do to thank them for taking such good care of my sister,” Makenna says. “Whenever we are there for an appointment, all the doctors, nurses, therapists and security guards are so nice to us.”

Organizing this fundraiser has inspired Makenna to continue her philanthropic streak.

“It was feels good to do it and it was so easy. It makes me want to do more to raise more money for CHOC,” she says.

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Lily and Makenna present a Makenna’s fundraiser proceeds to physical therapist Cathy Lopez.

Lily’s diagnosis and early days

When Danielle and her husband, David, found out they were expecting their third child, they were thrilled. A few months into Danielle’s pregnancy, a blood test showed that Lily had Turner Syndrome, a genetic condition affecting a female’s growth and fertility. That turned out to be a false positive, but a follow-up amniocentesis correctly diagnosed Lily with Mosaic Trisomy 12, a rare chromosomal abnormality about which not much is known.

Lily was born full-term via C-section at a local hospital. Immediately after birth she had trouble breathing and turned blue, and she was quickly whisked away to that hospital’s neonatal intensive care unit (NICU).

Lily needed a ventilator to help her breathe. She also required use of a CPAP, also known as continuous positive airway pressure, which helped keep her airway open. Danielle pumped her breastmilk so Lily’s care team could nourish her through a feeding tube.

Just outside the hospital, CHOC geneticist Dr. Neda Zadeh was starting to make her way home for the day when she heard about Lily’s condition.

“Dr. Zadeh was on her way out, but she turned around and came back to the hospital to run more tests and get a better idea of what was affecting her,” Danielle recalls. “Dr. Zadeh is amazing.”

In the first few days of Lily’s life, her parents weren’t sure if she would make it or not. When she was 3 days old, she was transferred to CHOC’s NICU for a higher level of care.

Lily spent the next 24 days in the NICU. When she learned how to breathe and eat on her own, she was ready to go home for the first time.

While the family spent much of their daughter’s first month in the hospital, NICU nurses made sure the family didn’t miss out on their first holiday together — Father’s Day. With her nurses’ help, Lily created a special Father’s Day craft for David using her footprints.

These days, Lily sees a mighty brigade of pediatric specialists at CHOC on a regular basis. Although not much is known about her Mosaic Trisomy 12, her care team remains fully committed to ensuring she is safe and healthy. In addition to Dr. Zadeh, Lily is under the care of neurosurgeon Dr. William Loudon, endocrinologist Dr. Timothy Flannery, and otolaryngologist Dr. Nguyen Pham. She also sees a neuro-ophthalmologist at UC Irvine.

Danielle is proud of the progress Lily has made during therapy at CHOC and seeing how that progress sparked Makenna’s giving spirit makes her proud as well.

family

“Makenna probably doesn’t realize this, but Cathy and Lauren at CHOC really cared for our entire family, not just Lily,” Danielle says. “The entire rehabilitation team at CHOC was incredibly supportive to our entire family. They were the weekly and sometimes daily reminders that we could do it. They impacted our entire family in a way that words simply cannot express. When we look back on Lily’s first years, they are in our memories and always will be!”

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5-year-old Rayaan fights through battle with brain, spinal cord inflammation

By Hina, mother of CHOC Children’s patient Rayaan

When Rayaan was 16 months old, he caught a simple cold. He had a typical low-grade fever and slept more than normal. I figured he needed the rest and would feel better the next day. The next morning, he was having a difficult time waking up so I thought I should take him to his pediatrician. She recognized something was very wrong and described him as being “unresponsive.” He was immediately transported to CHOC Children’s via ambulance. CHOC’s emergency department team was waiting for our ambulance and ready to care for Rayaan as soon as we arrived. They quickly assessed him and then placed him on a ventilator as he was brought up to the pediatric intensive care unit (PICU).

His care team ordered an MRI of his brain and diagnosed him with Acute Disseminated Encephalomyelitis, also known as ADEM. This means there was widespread inflammation in his brain and spinal cord that damages the myelin, which is a protective covering for nerve fibers. ADEM had affected over two thirds of his brain. We didn’t know it yet, but we would be at CHOC for the long haul.

Over the course of the next six weeks, Rayaan received multiple types of treatment for ADEM. His care team was vast, and included many different specialties: Dr. Nguyen Pham, a pediatric otolaryngologist (ear, nose and throat specialist or ENT), Dr. Gregory Wong, a pediatric gastroenterologist, Dr. Sharief Taraman, a pediatric neurologist, in addition to infectious disease specialists, in-patient physical and occupational therapists, a respiratory therapist, and the feeding team. He was in a coma and on life support for three weeks. During this time, his doctors kept a very close eye not only on him, but also on our entire family. Dr. Nick Anas, CHOC’s physician-in-chief; Dr. Jason Knight, medical director of emergency transport services; Dr. Paul Lubinsky, associate PICU director; and critical care specialists Dr. Juliette Hunt, Dr. Anthony Cherin and all of our nurses became family to us. I remember when Dr. Anas came to check up on Rayaan and he asked me when was the last time I layed next to Rayaan, I replied it’s been a while, and he ordered the PICU staff to transfer Rayaan from a crib to a full-size bed immediately, so I could lay next to my son. Rayaan was connected to every machine and monitor you can imagine, but his doctor was keeping my feelings in mind.

During this extremely difficult time when we were waiting for him to wake up, we were fortunate to have a wonderful support system. The Ronald McDonald Family Room let us escape for a few minutes, occaisionaly breakfast and lunch was provided by generous donors, and my daughter who was only three years old at the time was taken care of by child life specialists, while a social worker and case manager were assigned to us to provide us with counseling. This period was the hardest thing we had ever dealt with in our lives.

After three weeks of being in a coma, Rayaan began showing signs of waking up. He began by slightly moving his hands and arms. A few days later, we noticed his eye partially open. It would take him almost two weeks to be fully awake.  Although he had woken up from his coma, his journey was just beginning. The inflammation in his brain caused severe brain trauma and he lost his speech, and his ability to walk, eat, swallow and drink. While he was still in the hospital, a feeding tube was placed, as his oral muscles were to weak to swallow and chew. After his discharge, the rehabilitation team came in to ensure he would relearn the basics. Nicole Well, a speech language pathologist at CHOC, taught my son how to talk again. A feeding therapist named Polly provided electrical stimulation feeding therapy to make his muscles strong enough again to be able to eat, drink and swallow on his own.

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Four years after his health scare, Rayaan is in Transitional Kindergarten and still undergoes multiple therapies in the effort to make a full recovery from his illness.

Rayaan endured several surgeries and procedures during his hospitalization, and even more after his discharge and as well as multiple visits to the emergency department at CHOC. The CHOC specialists always worked so hard on Rayaan as if he were their own child. I am very grateful to CHOC for saving his life and I know that the comprehensive care we received at CHOC we wouldn’t have been able to get anywhere else.

Today, four years later, Rayaan is in Transitional Kindergarten and still undergoes multiple therapies in the effort to make a full recovery from his illness. He remains under the care of CHOC specialists. I am inspired every day by his strength and his courage to overcome so much at such a young age. Above all, our family is grateful for CHOC, who has provided him with the comprehensive medical care throughout his journey.

Learn more about the pediatric intensive care unit at CHOC

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