麻豆果冻传媒

11-year-old Lizzy was able to receive a life-saving transplant thanks to improved technology and Stanford expertise, along with her own determination and an extraordinary community of support.

By Erin Digitale

When Lizzy Highstreet could finally speak to her parents after an important surgery last July, she had a pressing question for her dad: 鈥淲hy don鈥檛 I have oxygen?鈥�

鈥淲hat do you mean?鈥� Aaron Highstreet said. 鈥淵ou got lungs!鈥�

Lizzy, 11, had temporarily forgotten why she鈥檇 had surgery: Following more than a month in the hospital with severe complications of cystic fibrosis, she had received a聽lung transplant July 18 at Lucile Packard Children鈥檚 Hospital Stanford.

鈥淚t was pretty amazing to walk in there and see her just easily breathing, not struggling,鈥� said Tina Highstreet, Lizzy鈥檚 mom.

Lizzy followed an unusual and difficult path to transplant. Before the surgery, she was kept alive for 37 days by extracorporeal membrane oxygenation, essentially the 鈥渓ung鈥� function of a heart-lung machine. Her doctors believe it is among the longest periods that ECMO has been used as a bridge to lung transplant for a child in the United States.

It was the culmination of a tremendous team effort 鈥� boosted by the expertise of Packard Children鈥檚 caregivers, the power of recent technical advances, the strength of Lizzy鈥檚 family and community, and the extraordinary grit and determination of the patient herself.

鈥淭he remarkable thing about Lizzy is that even though her lungs were so bad, she had very unique mental fortitude to be calm in difficult scenarios,鈥� said critical care physician Bereketeab Haileselassie, MD, who looked after Lizzy in the Packard Children鈥檚 pediatric intensive care unit.

Fighting complications

In the years before her transplant, Lizzy experienced a series of complications of cystic fibrosis. The genetic disease impairs the body鈥檚 secretory glands, making lung mucus stickier than normal and leaving patients vulnerable to respiratory infections.

To manage a rare, chronic lung infection, Lizzy saw a team at聽Valley Children鈥檚 Hospital in Madera, Calif. The hospital partners with 麻豆果冻传媒 Children鈥檚 Health specialists, including pediatric pulmonologist Carlos Milla, MD, who cared for Lizzy in Madera and during her inpatient stays at Packard Children鈥檚 Hospital. Although her health fluctuated, Lizzy could usually attend school and church in her hometown of Visalia, Calif.; go on beach trips with her three siblings and their cousins; and play her favorite sports, volleyball and soccer.

But, after hospitalizations in late 2017 and early 2018, it was clear she would need a lung transplant. By early June she was back in Packard Children鈥檚 again, being placed on a ventilator.

鈥淚t was really tough, the night they intubated her, to see her struggle to breathe so much,鈥� Tina said. She and Aaron relied on their Christian faith at this moment and many others throughout the long hospitalization. 鈥淲hen there were things that felt out of our control, we were praying for God to provide opportunities or answers for her,鈥� Tina said.

After a week on the ventilator, Lizzy鈥檚 physicians switched her to ECMO, using a neck catheter. Instead of blowing air into her lungs, the ECMO equipment carried Lizzy鈥檚 blood out of her body via plastic tubing. The equipment removed carbon dioxide, added oxygen and returned Lizzy鈥檚 blood to her body.

In the past, ECMO wouldn鈥檛 have been considered for a patient awaiting lungs. ECMO patients were traditionally sedated, losing muscle strength they would need to recover from a transplant. And the plastic tubing inside the machines increased the risk of blood clotting and immune reactions, making it hard to match a patient to a donor organ.

But in recent years, ECMO equipment has improved. The tubing circuits are smaller and coated in anticoagulant, reducing the risk of clotting. Critical-care specialists have also begun using ECMO without sedation. In the last year, three Packard Children鈥檚 patients with lung disease who did not need transplants each spent several weeks on ECMO, allowing their lungs to rest and recover. Those successes gave the team the confidence to move forward with Lizzy鈥檚 more difficult case.

鈥淎s long as the patient is moving around, they can be a good candidate for lung transplant,鈥� said cardiothoracic surgeon Katsuhide Maeda, MD, who performed Lizzy鈥檚 transplant.

It wasn鈥檛 easy: Moving Lizzy around with the neck cannula required 12 to 15 people.

鈥淏ut Lizzy took it in good stride and worked with us to keep her muscles strong,鈥� said Haileselassie. 鈥淚t was amazing how much she could do with a full ECMO circuit attached to her neck.鈥�

Still, the wait for donor lungs for a child can be several months. No one was sure if a match could be found soon enough.

Courage for a difficult wait

During Lizzy鈥檚 hospitalization, the Highstreets鈥� Visalia church organized a prayer drive. Participants posted Instagram photos of themselves with raised arms and the hashtag #LizzyBrave, 鈥渉olding us up as we were fighting the battle,鈥� Tina Highstreet said. Lizzy鈥檚 team of Packard Children鈥檚 caregivers also took a group photo of themselves holding up their hands and posted it in her hospital room.

As the campaign spread, Lizzy loved mapping its progress. 鈥淲e got all 50 states and eight countries,鈥� she said. 鈥淲e put a heart in every one as we found out somebody was praying for me there.鈥�

In early July, when Lizzy needed a blood transfusion to help reduce the number of minor antigens that her body was exposed to, family and friends used the #LizzyBrave hashtag to ask for blood donations. 鈥淎bout 60 friends and family came to Stanford to donate blood,鈥� Tina said.

The Highstreets also drew strength from the close ties they formed with Lizzy鈥檚 caregivers.

鈥淗er family is as remarkable as she is,鈥� said Haileselassie. 鈥淭hey were obviously frightened, but they came at this with a very positive mental construct. Every time a new provider came on, they said, 鈥榃elcome to Lizzy鈥檚 team.鈥� They coped with the stresses by rolling up their sleeves.鈥�

A dangerous hemorrhage

On July 13, leaving Aaron at Packard Children鈥檚 with Lizzy, Tina went to Visalia to catch up on things at home. The next morning, on the drive back, Tina鈥檚 phone rang. A blood vessel had broken in Lizzy鈥檚 lungs, likely due to worsening necrosis. She was losing a lot of blood.

Tina arrived in Lizzy鈥檚 hospital room just in time to 鈥渢ell her I loved her, to be brave, to fight, before they sedated her,鈥� she said. 鈥淲e didn鈥檛 know if they could stop the bleeding.鈥�

The medical team intubated Lizzy again and controlled her bleeding, but the incident heightened everyone鈥檚 worry about whether Lizzy would survive to receive donor lungs. On the evening of July 16, Lizzy鈥檚 ECMO connection was switched to a chest cannula to give her better mobility for long-term use. Early the next morning, Maeda told Lizzy鈥檚 parents that she was very weak and would need rehabilitation to regain her strength. Aaron and Tina felt devastated. Would Lizzy live?

Just a few hours later, Maeda came barreling down the hall. 鈥淲e need to talk,鈥� the surgeon told the parents, herding them into a conference room.

鈥淢y stomach fell out,鈥� Tina said. What now? she thought.

But Maeda had good news: Lizzy had matched to donor lungs.

鈥淚 remember feeling ecstatic and terrified and relieved and saddened for the family that was giving up their loved one,鈥� said Tina. 鈥淚t was like being on a rollercoaster of emotions for sure.鈥�

About 40 extended family members, including Lizzy鈥檚 grandparents, aunts, uncles and cousins, drove to Palo Alto and waited up until 1:15 a.m. on July 18 for the call confirming that the transplant would go ahead. At 3:30 a.m., Aaron and Tina heard that surgery had begun and Lizzy was receiving her new lungs. After the transplant was complete, Lizzy spent a few days on a ventilator, partly sedated. Her first completely independent breaths were about a week after the surgery; that鈥檚 when she could finally ask her dad why she didn鈥檛 have oxygen.

Lizzy鈥檚 recovery went well. She was discharged to the聽Ronald McDonald House near the hospital on August 11. She went home to Visalia on October 25, excited to see her friends, her dog, and her very own house.

Before leaving Palo Alto, the Highstreets took a day trip to the beach, a favorite family pastime. Thanks to her new lungs, Lizzy no longer needed a piggyback ride from her dad.

鈥淲atching her walking across the beach with the wind in her hair, able to do that, brought tears to our eyes,鈥� said Tina. 鈥淚t was a very, very good moment.鈥�