The tube, the team and the family that give life

Parenteral-Nutrition-Naema-BostonAs 2-year-old Naema Alshehhi turns the pages of her favorite book, her eyes glimmer with curiosity. Sitting with her father, Àbdulla and big sister, Dana, in their temporary Boston apartment, the inquisitive toddler points to a number of shapes and is fascinated by the rainbow of color.

During this quiet moment, you see the gentle outline of Naema’s central line through her tiny shirt. The lifesaving tube — surgically inserted into her chest — provides the nourishment needed to manage the rare intestinal disorder she was born with, called microvillus inclusion disease (MVID). The condition, which causes severe diarrhea and an inability to absorb nutrients, requires specialized parenteral nutrition (PN) and intravenous hydration support at home.

For the Alshehhi family, however, “home” is over 6,600 miles from Boston.

Naema was born in April 2015 in Abu Dhabi, the capital of the United Arab Emirates. Soon after her homecoming and weighing a mere 5.5 pounds, she started having difficulty feeding and became noticeably quiet. Within 24 hours her condition took a drastic turn for the worse. “Naema was very lethargic and we noticed her color was turning blue,” Àbdulla recalls.

Àbdulla and Mariam, Naema’s mother, rushed their daughter back to their local hospital and Naema was immediately admitted to the Intensive Care Unit. As the days turned to weeks, upset and fear mounted. Naema was losing weight at an alarming rate and a diagnosis could not be confirmed. The Alshehhis knew it was time get another opinion.

Getting a second opinion

Parenteral-Nutrition-NaemaAt a fragile 3 lbs., Naema was transferred to a second hospital in Abu Dhabi. A central line was needed to provide the parenteral nutrition — sugar, protein, fat, electrolytes, vitamins and minerals — she needed so desperately.

Following a thorough evaluation, doctors diagnosed her with microvillus inclusion disease and shared some upsetting news. Because Naema’s intestines were not absorbing nutrients and fluids properly, she would need a five organ multivisceral transplant —right colon, stomach, intestine, pancreas and liver, and the Abu Dhabi hospital was unable to perform the procedure.

“We were at the best hospital in the city…but the doctors said that the treatment for this disease was unavailable [in Abu Dhabi],” Àbdulla recalls. “We were given the names of two hospitals in the U.S. and told to transfer her for the organ transplant.” One of the two recommended hospitals was Boston Children’s Hospital.

Traveling the globe for care

Àbdulla, Mariam and Naema, along with her two siblings, Sagr and Dana, traveled by plane 14 hours to a well-known U.S. pediatric hospital. Once they arrived, the family received more upsetting news: The hospital was also unable to perform the lifesaving transplant Naema needed.

“At that point, we lost all hope,” Àbdulla recalls with great emotion.

Heartbroken and struggling with the prognosis, the family packed their bags again and traveled to Boston Children’s. Here they met with Dr. Alexandra Carey, a pediatric gastroenterologist specializing in nutrition, Dr. Bram Raphael, director of the Home Parenteral Nutrition Program and Dr. Rima Fawaz, medical director of the Intestine and Multivisceral Transplant team.

“When Naema transferred to Boston Children’s for an evaluation at 4 months of age, she was malnourished and had a lot of trouble growing,” recalls Carey. “Her electrolytes were very difficult to manage due to large fluid shifts from excessive stool output.”

In order to receive the lifesaving transplant and the life-sustaining parenteral nutrition Naema needs, the Alshehhis moved to Boston in August 2015. They say they knew they were finally in the right place. “We felt hopeful because moving to Boston was the last option for us,” says Àbdulla.

Improving Naema’s health with parenteral nutrition 

While Naema awaits the multi-organ transplant, she receives parenteral nutrition given by her parents at home. These prescribed fluids are tailored to Naema’s specific health needs and delivered by a pre-programmed pump usually at least 12 hours at a time. PN ensures she stays well-nourished and hydrated, and achieves appropriate height and weight milestones and activity levels.

“They did a great job showing us how to give Naema parenteral nutrition at home,” Àbdulla says. “In the beginning, it was very hard for us to learn but after two to three days of doing it at home, we have become very familiar.”

Every three weeks, Naema visits with Dr. Raphael and the Home Parenteral Nutrition nursing team for check-ins. “We really like Dr. Raphael and his team,” Àbdulla says. ”They really care about Naema and she really likes them too.”

Since her arrival at Boston Children’s, Naema’s growth has been “excellent” and she has been without any central line infections.

“Her weight is around the 10th percentile, her Body Mass Index (BMI) is around the 20th percentile and she is growing at a good rate,” Carey and Raphael note. Regular dietitian visits also keep Naema’s growth on track.

Parenteral-Nutrition-Naeme_family

As she continues to grow and get stronger, Naema is an inquisitive and playful toddler — one who lights up a room the moment she arrives.

“Naema’s prognosis is very good,” says Carey. “From the start she was such a sweet child and the nurses and doctors enjoyed spending as much time as possible in her room because she was such a happy baby.”

Learn about the Home Parenteral Nutrition Program at Boston Children’s Hospital.