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Alison’s Puzzle

A collaborative team of doctors treat a rare condition in a unique little girl

2/25/2011 

Albert and Sabrina Garibay delivered their firstborn on July 22, 1999, and named her Alison. “She was a nice chunky, bright pink Alisonlittle girl,” said Alison’s mother. The Garibays’ daughter weighed 7 pounds, 6 ounces and gave no indication of the mystery she would become.

“They call me a puzzle. That’s what I am, a puzzle,” said 11-year-old Alison with a grin. A multidisciplinary collaboration of doctors at Children's Hospital Central California agrees with that diagnosis. They also concur on another matter concerning Alison. Dr. Kenneth Rouillard, a cardiologist at Children’s, put it best. “She’s a superstar,” he said.

puz•zle (pŭz ׳əl)
noun

  1. Something, such as a game, toy, or problem that requires ingenuity and often persistence in solving or assembling.
  2. Something that baffles or confuses.
  3. The condition of being perplexed; bewilderment.
  4. Alison Garibay.

Thermometers can skyrocket in the Garibays’ hometown of Chowchilla each summer, but the days following Alison’s birth were not unusually hot. The new parents wondered why the Valley’s heat seemed to affect their baby girl so severely, and they grew concerned over Alison’s profuse sweating. The Garibays anxiously drove to the emergency department at Children’s Hospital just four days after she was born.

Alison“She was admitted to intensive care and we were told to call family,” said Sabrina. The initial examination revealed cyanosis. Alison’s skin was blue, especially under her fingernails. “They said Alison had a heart defect.” But the unwelcome reports regarding the condition of their fragile infant did not stop there. “It seemed like we’d find something new…,” said Alison’s mother, leaving her sentence unfinished as 11 years vanished in the intensity of her memory. When asked how she felt that day, Sabrina said, “One of the first emotions was just confusion. You always think that will never happen to me.”

Sabrina recounted an unforgettable day before Alison was born. She and her husband were driving to Fresno from Chowchilla on Avenue 9 and saw the construction of what would become the new site of Children's Hospital Central California. The facility looked amazing and Albert said, “I want my kids to go there.” Sabrina told him, “No you don’t,” meaning that she never wanted their future family to have a reason to go to a children’s hospital. “It’s funny how we ended up there,” she said.

The Garibays traveled to Children’s Hospital the first year we opened the Madera campus. They learned Alison’s heart had just one chamber and other malformations. Dr. Edwin Petrossian, medical director of cardiothoracic surgery at Children’s, and Dr. Frank Hanley, pediatric cardiothoracic surgeon, wanted to wait until Alison weighed at least 12 pounds before performing heart surgery.

Alison’s heart would need repair, but Dr. Petrossian was confident she could wait a few months for surgery. "Children born with complex heart disease almost always require heart surgery as a newborn," said Dr. Rouillard. "It's rare for these infants to have the right balance of blood flows to their body and lungs like Alison had."

AlisonSabrina explained the reason her daughter’s surgery could be delayed. “She had one large chamber but there was also transposition, so the blood mixing in the heart was flowing through the body,” she said. “They told us for every malformation in Alison’s heart there was something reciprocal to offset it.” If Alison is a puzzle, then the pieces were crafted by a Creator with a good plan. “It was God’s will. Ultimately it was all God’s will,” said Sabrina. “Each anomaly complements the other.”

Oddly enough, the syndrome that affected the development of Alison’s heart played a role in helping it oxygenate the blood without a proper chamber to do the pumping. Our doctors discovered Alison has heterotaxy syndrome. Her organs are flipped midline and she does not have a spleen. For every 1 million babies born, only four will have this rare condition.  The reason internal organs sometimes rotate during fetal development remains a mystery.

Alison with Dr. AllshouseThe Garibays took little Ali home and did not plan on returning until she was big enough for heart surgery. But Alison began having digestive difficulties when she was 3 weeks old. Her incessant projectile vomiting concerned the young family and they returned to Children’s for answers. Hexterotaxy syndrome had affected the development of Alison’s intestines as well. She required immediate surgery to correct the malrotation of her small bowel to keep it from further twisting and kinking.

Through all these difficulties, the new parents were blessed with a wonderful network of friends and family. “We have an amazing support group,” said Sabrina.

“I’m very proud of my family,” said Nanette Huse, Alison’s grandmother, of how her daughter and son-in-law drew close and strengthened one another. The family also gratefully acknowledges the tremendous difference Children's Hospital Central California has made in their lives. “I love the Hospital,” said Nanette. “I think the Hospital is awesome.” Sabrina agrees wholeheartedly with her mother. “The whole cardiac team has been amazing. They’ve been wonderful,” she said.

Dr. Rouillard with Alison and familyThe amazing cardiac team performed the first of three heart surgeries on little Ali when she was 3 months old. The second surgery occurred after she became a 3-year-old, and the final procedure repairing Alison’s heart – the Fontan procedure – took place on June 9, 2003, one month shy of her fourth birthday. At an age when preschoolers learn to recognize their letters and count to 10, Alison could hold up four fingers not only to show how old she was, but also to indicate the number of times she’d had surgery.

For nearly four years Sabrina avoided dressing her daughter in blues and purples because it accentuated Alison’s cyanosis. “You get used to it,” she said of her daughter’s skin tone. “You don’t really notice it.” But after the Fontan procedure, that changed. Sabrina well remembers the results of Alison’s final heart surgery, and described the scene at the conclusion of the family’s nine-hour wait. The nurses had invited them into the hallway to watch as Alison was transferred from the site of the Fontan to her recovery room.

“We were all standing there – uncles, grandmas, cousins,” said Sabrina. “The moment she was wheeled out where we could see her, she was pink. I can see it clear as day. Her being purple wasn’t a part of her life anymore!”

Alison with her nurses on HalloweenThe family did not say a word, but they all felt the energy. Alison had pulled through and looked truly healthy for the first time since they learned of her heart defect. Other than medications for her heart and antibiotics to compensate for her missing spleen, Alison could lead a normal life.

But this amazing story does not end here. When Alison reached second grade, she began experiencing severe back and abdominal pain. The Garibays turned once again to Children’s Hospital for answers. “I called Dr. Rouillard in tears,” said Sabrina.

Why would a cardiologist know what to do about abdominal pain? “I’d had a recent patient, a boy, with the same symptoms. So I thought it might be Alison’s gallbladder,” said Dr. Rouillard. He ordered an ultrasound for Alison and discovered her gallbladder, which was flipped due to heterotaxy syndrome, did in fact have gallstones and required removal. Alison would undergo her fifth surgery as a third- grader. Is it any wonder Dr. Rouillard calls her a superstar? “I’ve never seen her afraid,” he said. “She’s always bubbly.”

By the time Alison reached age 9, she was experiencing trouble breathing. Her pediatrician thought it was asthma and prescribed an inhaler. “We took it everywhere and used it regularly, but it didn’t seem to help,” said Sabrina. Alison struggled with low energy levels – completely uncharacteristic of her personality – and had begun coughing up what would later be identified as “castings.”

“At first I thought I was coughing up my lung,” said Alison. Castings are made of thick, rubbery-like mucus and inflammatory cells. When expectorated, the casts resemble the bronchial tree.

Alison with Disney charactersAlison was granted a wish by the Make-a-Wish Foundation and elected to take her family, which now included a younger sister and brother, to Disney World. “Initially she wanted to go to a Jonas Brother’s concert, but she changed her wish to a family trip,” said Dr. Rouillard. “That says a lot about who she is – that she would choose something for her family.”

The family flew to Florida for Alison’s tenth birthday. She became terribly ill and had difficulty breathing. Alison became so weak from the humidity and from her need to cough up castings that she required a wheelchair. Shortly after returning home, Alison was admitted to the Hospital with pneumonia, and she brought along another puzzle. What was causing the castings and what could be done about them?
The Garibays and Alison’s doctors pursued every possible resource to solve the mystery.

puz•zle (pŭz ׳əl)
verb
verb, transitive

  1. To baffle or confuse mentally by presenting or being a difficult problem or matter.
  2. To clarify or solve (something confusing) by reasoning or study.
    verb, intransitive.
  3. To be perplexed.
  4. To ponder over a problem in an effort to solve or understand it.
  5. To diagnose and treat Alison Garibay.

Dr. Carl Owada, medical director of the cardiac catheterization laboratory, performed a cath procedure, and said Alison’s pulmonary problems might be a rare side effect of the Fontan procedure known as plastic bronchitis. Dr. Owada measured the pressure in her heart to see if it had changed. If the Fontan wasn’t maintaining proper pressure, they would consider conducting a second procedure in hopes it would alleviate the plastic bronchitis. But the pressures in Alison’s heart were good. Surgery was ruled out as a potential treatment.

Alison with family at SF Giants game“It’s a very unusual complication and sometimes it’s a clue that there’s something else going on with the heart. Fortunately with Alison that was not the case,” said Dr. Rouillard. He and Dr. Owada consulted with Dr. Hanley, and Dr. Mary Anne Tablizo, pulmonologist at Children’s, and concluded Alison’s diagnosis was in fact plastic bronchitis.

“I performed flexible bronchoscopy and saw thick, white rubbery-like mucus obstructing the left mainstem bronchus,” said Dr. Tablizo. “When I got this out, it was shaped just like the L bronchial branches. Post-bronchoscopy, I told Alison’s mom about my findings and informed her that her mucus is consistent with plastic bronchitis. She remembered Dr. Owada mentioning this condition as a possible complication of the Fontan procedure.”

Fundraiser for AlisonThe cardiothoracic surgery team at Children's Hospital is part of the Northern California regional congenital heart program affiliated with Stanford University. Each year, surgeons in the program perform over 1,000 cardiothoracic operations. Between 1997 and 2010, our surgeons conducted 126 Fontans at Children’s, and yet our team has never encountered a case of plastic bronchitis – until Alison. The pulmonology team also faced a new challenge with Alison. “Plastic bronchitis is rare and poorly understood at this time,” said Dr. Tablizo.

“Dr Rouillard said what needed to be done was a mystery because it’s so unusual,” said Sabrina. Encouraged by the environment of family-centered care at Children’s, and desperate to find answers to help her little girl, Sabrina sent e-mails to children’s hospitals in Cincinnati, Boston and Philadelphia, and asked for information regarding plastic bronchitis as a side effect of the Fontan procedure. The cardiology team at Children’s Hospital of Philadelphia responded and asked to collaborate with Dr. Rouillard in studying this rare complication. Sabrina’s hope was restored. “They responded at a critical time,” she said.

Dr. Rouillard readily agreed to share information regarding Alison’s case with Philadelphia. “We don’t have a lot of experience handling cases like this. No one has enough. So there’s collaboration that goes on as we share a collection of case reports,” said Dr. Rouillard. “We sent them all of the studies that had been done – echos, caths – making sure we were doing everything possible.”

The collaboration continued at Children's, where a multidisciplinary team of doctors determined a treatment plan for Alison. “Each one of us has our own specialty, our knowledge and skills, we often have to come together with patients like Alison,” said Dr. Rouillard. “That’s one of the things unique about working here. Everyone works collaboratively. As the sports saying goes, there’s no I in team.”

Alison rollerskating“The treatment for plastic bronchitis is difficult as there has been no proven single therapeutic intervention,” said Dr. Tablizo. “Most of the medications given in the published literature are anecdotal case reports.”

Our doctors prescribed an effective treatment regimen designed to decrease the thickness of Alison’s tracheobronchial secretions and help her to expectorate easily. Treatment incorporates a vest that inflates and deflates rapidly, oscillating the chest wall with gentle pressure. Twice a day Alison wears the vest and takes a combination of medications, some of which are inhaled through a nebulizer. The process takes about an hour each time.

“She doesn’t see herself as being sick or having an illness,” said Stacy Anderson, her home hospital teacher. Anderson has homeschooled Alison since the fifth grade. “She’s ahead of the ball game in science with as many medical terms as she’s been around. Learning about the human body is a fifth-grade standard and she aced that test!” Anderson described her student as an eager learner. “She’s definitely a leader. She’s competitive. She likes to play games and like she likes to win!” Anderson laughed and said, “That’s why we’ve clicked because I’m competitive, too.”

Alison inherited that winning drive from her mom. “I love challenges, so we never shut our minds down and said, ‘Why, why?’ We always had hope,” said Sabrina. “We just pull together and be strong and do what we have to do as parents. It’s hard and difficult at times, but we’re OK,” she said. “Right now we’re kind of on cruise control. It’s working and we’re thankful for that.”

AlisonDr. Tablizo has also noted the efficacy of Alison’s treatment regimen. “We will continue her current treatment as this seems to be working well for her,” she said.

Dr. Rouillard gives his patient much of the credit for her improved health. “It’s not surprising with Alison. She’s a fighter,” he said.

Alison has not been hospitalized since beginning the twice daily, aggressive treatment developed by the team of doctors at Children's Hospital Central California.

“Amazing people, incredible care – most think it’s just a slogan,” said Sabrina softly. “But amazing people, incredible care really, really, really is true.” She spoke of when 3-year-old Alison filled the role as the little heart ambassador for the Heart Walk sponsored by the American Heart Association. “We ended up having Alison’s surgery during the Heart Walk,” she said. Sabrina was sitting with her preschooler in the pediatric intensive care unit when a television commercial for the event began to play. Ali’s mom was tickled to see her daughter on television saying “Hi, I’m Alison” as she lay recovering from heart surgery at Children’s. Sabrina called it a “magic moment” and said, “If we won the lottery, we’d give lots of money to the Hospital. They’re a big part of our heart.”

Alison’s grandmother said, “Most families emotionally have a hard time and parents don’t always make it. But this family has pulled together.”

No matter how impossible a puzzle may seem, its scattered pieces always fit together perfectly. The Garibays and the doctors at Children's Hospital Central California have worked tirelessly to solve the “Alison puzzle.” With patience, perseverance and prayer a beautiful picture has emerged – a breathtaking picture of courage and hope.

 

 

 

 

 

 

 

 

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