The good news: Joe had a brain tumor when he was 5 years old that was successfully removed at Children’s Hospital of Philadelphia (CHOP).

The not-so-good news: Joe lost his pituitary gland along with the tumor, creating a condition called panhypopituitarism.

More good news: Specialized care at the Neuroendocrine Center at CHOP has helped Joe manage the myriad hormonal irregularities that come along with panhypopituitarism so that he’s a typical teenager and has a bright future.

A preschooler with headaches

When Joe was 4 years old, he started having headaches. By March, they were nonstop and getting more intense, to the point he had to lay down frequently. The family came to CHOP for an evaluation, where doctors discovered he had a benign brain tumor called craniopharyngioma. While that type of tumor is noncancerous, it was growing and causing increasing pressure on his brain.    

It was completely removed by Phillip “Jay” Storm, MD, Chief of the Division of Neurosurgery and Co-Director of the Neuroscience Center.  

“Joe spent his fifth birthday in the intensive care unit (ICU) recovering,” says Joe’s mother, Kourtney. “Dr. Storm is our superhero. He and our ENT surgeon, David Lee, were able to pull the tumor out through Joe’s nose. It was fully out.”

Tiny gland, big consequences

Because the tumor had enveloped Joe’s pituitary gland, it needed to be removed as well. While the pituitary gland is tiny — about the size of a pea — it has an enormous impact on the body. It monitors and regulates many bodily functions through the hormones it produces. These include growth and sexual/reproductive development and function, other glands, such as the thyroid and adrenal glands, and  various other organs, such as the kidneys.

Removing his pituitary gland caused a cascade of other hormone-related conditions: pituitary failure, adrenal insufficiency, growth hormone deficiency, hypothyroidism and diabetes insipidus (when the fluids in the body are out of balance causing excess urination and thirst).

Managing panhypopituitarism requires Joe to take a combination of synthetic hormones to replace the ones it would have produced. Getting the dosages right is a balancing act made more complicated when Joe has an illness or other stresses. 

A close call

Three months after his brain surgery, Joe developed a fever. His parents gave him a stress dose of hydrocortisone. In normal circumstances, his pituitary gland would tell his adrenal gland to release cortisol to combat the illness. But without a pituitary gland, the adrenal gland couldn’t do its job. When his father, Tim, called Dr. Jean Belasco, a member of CHOP’s Neuro-Oncology Program, to reschedule a follow-up appointment and told her about Joe’s condition, she said to bring Joe to CHOP immediately.  

“If we hadn’t, I think Joe would have died,” Tim says.  

Joe spent 10 days in a coma and weeks in the ICU as doctors combated sepsis and worked to even out his hormone levels.     

“That’s when CHOP saved Joe’s life a second time,” Kourtney says. “We feel incredibly lucky that Dr. Belasco was our go to for Neuro-Oncology. She is brilliant.” 

Staying on track

The Neuroendocrine Center brings together the specialists that patients like Joe need to manage their complex situations. Endocrinologists, neurosurgeons, neuro-oncologists, geneticists, neuro ophthalmologists, nutritionists and others build a care plan tailored to each child and tweak it as necessary to account for growth and other factors.  

For Joe, now 13, that means several pills (hydrocortisone, levothyroxine and desmopressin) and a couple of shots (human growth hormone and testosterone) a day to replace missing hormones. He has blood tests regularly to make sure dosages are in line with his needs.  

“I basically live normally,” Joe says. He swims recreationally, builds Lego contraptions and draws on his iPad using a graphics program. He and his friends come into Center City Philadelphia on the train to hang out. As a rising eighth grader, his favorite subjects are social studies and geography.  

Ups and downs, but mostly ups

It hasn’t always been easy. There have been “a ton of doctor appointments” and several Emergency Department visits, especially when he comes down with the flu or another illness. Joe has been hospitalized a few more times over the years, but only for a day or two.   

“I feel kinda of sad about it because I didn’t have a normal young childhood,” Joe says, “but I know it’s getting better as I’ve grown. I have appointments every six months now.”  

Managing panhypopituitarism, with its ups and downs, has imbued Joe with a level of sensitivity other teens may lack. “He sees when people are hurting and wants to help,” Kourtney says. Joe concurs: “I’m more in tune with things like that, because of what I’ve been through.”   

Endocrinologist Christopher Gibson, MD, leads Joe’s team, and he puts everyone at ease. “He tells a lot of dad jokes, and that helps me relax,” Joe says.   

“We just love him” Kourtney says. “Having someone like him in your corner makes me feel safe.”

’Science is magic’

Joe and his family said they’re grateful to live so close to CHOP and for the treatment advancements for panhypopituitarism.   

“If Joe had been born 50 years earlier, he wouldn’t be here,” Kourtney says. “They know so much now about hormones and hormone replacement now. Science is magic.”  

“I’m so glad we had a place like CHOP nearby,” Joe says.