Kristin Martin – Cystic Fibrosis

In March 1996, Kristin Martin was 14 months old when she came down with a cold that just wouldn't go away. After several days with a stuffy, runny nose and cough, Kristin developed a fever and gradually became short of breath.

Kristin’s mother, Louise, was concerned when Kristin started wheezing, and she took her to the local emergency department. "Kristin wasn't responding to the cold medicines I gave her, and the wheezing kept getting worse," says Louise. Kristin was referred to Children’s Hospital of Pittsburgh of UPMC. Louise spoke with a physician in the Paul C. Gaffney Diagnostic Service, who recommended Kristin be brought to Children’s immediately for tests.

When Kristin was seen in the Emergency Department, her oxygen level was only 85 percent (normal is 100 percent). She was given oxygen through a mask to help her breathe and was admitted to an inpatient unit.

Kristin’s condition became worse, and she developed respiratory distress (difficulty breathing on her own). Kristin was transferred to the Pediatric Intensive Care Unit (PICU), where she was stabilized, put in an oxygen tent and given continuous albuterol treatments to keep her airways open. David Orenstein, MD, director, Antonio J. and Janet Palumbo Cystic Fibrosis Center, was consulted regarding Kristin’s care.

After 24 hours in the PICU, Kristin was able to return to the inpatient unit, but she returned to the PICU two days later when she developed respiratory distress. She was again given continuous albuterol treatments, which stabilized her enough so she could return a regular patient room.

Kristin then developed subcutaneous emphysema, which is a leak in the lungs that causes air to build up under the skin. "Kristin’s body swelled up from her head to her thighs with the air that was trapped inside, and when I picked her up, her skin snapped, crackled and popped like Rice Krispies," says Louise.

Dr. Orenstein and the entire team of residents observed Kristin as they waited for Kristin’s lung to heal itself and for the swelling to subside. Gradually, Kristin’s swelling began to decrease, but, at the same time, she developed a high fever. Following a chest x-ray, Kristin was diagnosed with pneumonia and had to have a chest tube inserted to help clear her lungs of fluid.

While she was being treated for pneumonia, Kristin developed respiratory distress again. She was rushed back to the PICU, and, this time, she was put on a ventilator to help her breathe. "Kristin had tubes everywhere," says Gary, Kristin’s father. "Tubes to help her breathe; tubes to help her eat; and tubes to clear her lungs."

Kristin stayed in the PICU for four weeks. During that time, a DNA test showed that Kristin had cystic fibrosis, which is a life-shortening, genetic disease that affects the way cells process sodium, chloride and water, causing a thick mucous to build up in the lungs resulting in blockages, infection and inflammation.

To Kristin’s parents, the diagnosis of cystic fibrosis was a mixed blessing. "Gary and I were devastated with the news," says Louise. "But, at the same time, we were relieved to know what was going on because now the doctors really knew what they were dealing with and could treat her."

Unfortunately, Kristin did not respond to treatment. Her lungs began to fail, and the carbon dioxide levels in her blood became dangerously high. At one point, Kristin’s carbon dioxide levels were so high that the doctors determined Kristin only had two hours left to live. Louise and Gary called their families, and a priest gave Kristin her last rites. "I was inconsolable," says Louise. "Kristin had been so tough that we never thought we would lose her."

It was then that Kristin’s courage really took over. "Miraculously, Kristin’s carbon dioxide levels started to drop," says Gary. When Kristin was able to breathe on her own, she was moved back to the inpatient unit. Two weeks later and two and a half months from the time she was admitted, Kristin went home.

Today, following a strict feeding schedule and months of occupational and speech therapy, Kristin is a happy, healthy 5-year-old who loves to swim, play with her dolls, ride her bicycle and play on the swings.

To manage the cystic fibrosis, Kristin receives six breathing treatments and three chest physiotherapy treatments every day. She also takes two medications on a regular basis and antibiotics as needed when she gets an infection. Kristin and her parents also return to Children’s Hospital to visit Dr. Orenstein in the Pulmonology Department once a month.

As Kristin smiles and laughs her way through each day, her parents know that every minute of every day that Kristin is with them is a blessing. "Everyone calls Kristin the miracle baby," says Louise. "It’s a miracle that she’s still here with us. We thank God for sending Kristin the strength and spirit she needs to fight this disease and for bringing the Paul C. Gaffney Diagnostic Service, Dr. Orenstein and the rest of the Pulmonology Department into our lives. Kristin wouldn't be here without them."

Greg suffered his first asthma attack at just 10 years old. Read more about his successful treatment and experience at Children’s Hospital of Pittsburgh of UPMC.
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