Cystic Fibrosis: Zak’s Story

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Zak’s organizational skills would put an efficiency expert to shame. Two years ago, when he was 7, Zak took on the job of managing his cystic fibrosis by preparing and administering his own medications, therapies and gastrostomy tube feedings. Last year, when he was diagnosed with diabetes, Zak added daily glucose monitoring and insulin shots to his schedule.

Zak with blue hair smiling If that seems a lot for someone so young, Zak wouldn’t have it any other way.

“I really feel strongly about letting other kids know that this all can be scary, but it helps me a lot to learn and know I can do it myself,” says Zak. “That way I am in control.”

An early diagnosis

Zak was merely hours old when his parents, Christina and Jason, knew something was wrong. He vomited after nursing and by his third feeding, his tiny belly had swelled to the point where Christina was afraid her infant son’s skin would split. A scan revealed that Zak’s small intestine was obstructed.

The cause was meconium ileus, a bowel obstruction that occurs when the baby’s first stool, meconium, is thicker and sticker than usual, causing a blockage in a part of the small intestine called the ileum. It is often seen in babies who have cystic fibrosis (CF), an inherited disease that causes mucus in the body to become thick and sticky.

Zak was transferred from the Vineland, NJ, hospital where he was delivered to Children’s Hospital of Philadelphia (CHOP), where doctors performed several procedures to clear the blockage. At 4 days old, he was diagnosed with cystic fibrosis. During Zak’s recovery in the Hospital, the team at CHOP’s Cystic Fibrosis Center trained his parents to manage their son’s disease with chest percussions, enzymes, several medications and nebulizer treatments. At 8 days old, Zak was well enough to go home.

As Zak grew, his weight lagged behind others his age. Children with cystic fibrosis do not absorb nutrients properly. Despite daily medications and respiratory therapy two to four times a day, he was also frequently sick. With each illness, Zak would drop weight from his already thin frame, prolonging his recovery. Christina and Jason tried to keep their son healthy by staying away from others during cold and flu season. They missed a few family gatherings each winter, but the get-togethers weren’t worth the risk to Zak’s health.

A feeding tube

At 5 years old, Zak was admitted to CHOP with a CF-related pulmonary exacerbation. While he was hospitalized, Christina and Jason took the opportunity to talk to Zak’s doctors about another concern. He was still having trouble gaining weight, and they had read that children with CF who have trouble maintaining their weight often do better with a gastrostomy tube (G-tube). A G-tube is placed directly into the stomach through an opening in the belly called a stoma. Liquid food is slowly pumped into the stomach, usually overnight while the child is asleep.

While they worried about adding another complication to their son’s life, his parents knew that in the end, the extra nutrition would keep him healthier. Zak’s clinical team said that he was a good candidate for the procedure. The G-tube was placed before Zak was discharged from the Hospital.

“It was a very difficult decision,” Christina says. “I had discussed it numerous times with his CF doctor and spent many, many nights researching the benefits for CF kids, and the cons. If he could gain weight it would help him fight infections, and that was something that was really important to us.”

As Zak and his family adjusted to the new G-tube routine, Zak began to show a greater interest in his care. He started asking his parents and doctors more questions about every therapy and medication that was part of his daily routine, including how each step worked to keep him feeling healthy and strong. By the time he was 7, Zak had taken charge of managing his own medications, therapies and G-tube feedings.

“It was bittersweet to watch him do everything on his own,” Christina says. “He was determined to do so and that makes me very proud of him. At the same time, it saddens me that at his age he is taking on all these medications and treatments and is willing to do it all himself most of the time. He is a very strong and brave boy. He is our hero.”

Now, four years after having the G-tube put in, Zak weighs 80 pounds — a huge deal for him! A recent hospitalization due to CF exacerbation complicated by chronic sinusitis was the first illness in three years that required Zak be admitted to the Hospital. Before that, it was the longest that Zak had gone without a hospitalization. Despite these recent challenges, Zak has been doing a magnificent job with his feeds, medicines and treatments.

A second diagnosis

Zac smiling standing in front of a christmas tree In the fall of 2016, Zak’s parents noticed some changes in their son. He had been drinking more water than usual, got tired more easily, and often felt dizzy. Zak’s annual testing at the CF Center showed an abnormal glucose level. They referred him to CHOP’s Division of Endocrinology and Diabetes for further testing, which revealed that Zak had developed cystic fibrosis-related diabetes (CFRD).

The mucus buildup associated with CF can damage the pancreas, which makes insulin. This can make it difficult for the pancreas to produce the amount of insulin needed to regulate blood sugar.

Zak dealt with the diagnosis in his typical fashion. He asked the diabetes educators to train him to check his own glucose levels and administer his own insulin shots.

“I was actually really scared about diabetes because of the glucose check pricks and the insulin needles, but it doesn’t really hurt at all,” Zak says. “I was nervous at first to do it myself but I really ended up liking it better than someone else doing it.”   

Zak’s positive attitude has helped his mother deal with her own feelings about her son’s illnesses. When he was diagnosed with CF, Christina says she pushed her fears out of her mind and focused on learning to care for her son in every way possible. Over time, she learned she needed an emotional outlet. Therapy and reaching out to other parents of children with CF helped her process her thoughts and feelings. Of course, it helps to have a son who not only never complains, but who meets the challenges of CF and diabetes head-on.

“I want other kids to know that it is OK to be scared of things,” says Zak. “A positive attitude and deep breaths really help me though.”

Originally published June 2017

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