A teen with a rare condition

Fourteen years ago, Terrell and Craig Cleare added a beautiful baby boy they named Amarion to complete their family; they already had their daughter Angel. The family was happy. But it was around Amarion’s fifth birthday, that the parents began to realize all was not right with their son. They noticed he was not growing like they expected him to, and his head was bigger than his body. They also noticed him exhibiting weakness which warranted medical attention.

Amarion was tested and found to have low glucose. He was further referred for treatment to Miami Children’s Hospital where he was diagnosed with a rare condition – congenital panhypopituitarism – which means the pituitary gland did not develop normally before birth.

The pituitary gland, also called the “master gland of the body”, is a pea-sized organ located in the center of the brain. It produces and regulates the release of many different chemicals, or hormones, that control growth, sexual development and function, metabolism, and the body’s response to stress.

Amarion is a teenager but, looking at him, you would think he is about seven years old; and, when he speaks to you, his voice sounds like that of a toddler. The teenager, who stands four feet, two inches in height and, at his last weigh-in, weighed approximately 62 pounds, is in need of growth hormone injections.

According to his mom, doctors have told her that if he does not begin receiving the growth hormone injections by age 16, he won’t grow anymore. The parents have been quoted an estimate of $70,000 per year for his growth treatment.

According to cincinnatichildrens.org, children may need to take replacement medication for the rest of their lives because they are unable to make some or all of the hormones.

At diagnosis, the Cleares were able to afford Amarion’s treatment. Terrell was employed and had insurance. She was taught to administer his shot. He was able to receive the growth hormone for about two years before the parents could no longer afford the treatment.

Terrell said she and her husband would like to have their child resume receiving his growth injections again but, without insurance, they are finding it difficult.

“I noticed a little difference – a half inch here, an inch there, when he was taking the treatments. If he had been able to continue with the injections, he would have been taller than he is now.”

For most children and adolescents with panhypopituitarism, treatment with hormone replacement therapy (HRT) is effective in restoring normal hormone levels, according to the Children’s Hospital of Philadelphia. And that even if the underlying cause of panhypopituitarism cannot be found or addressed, HRT works to return the body to a healthy rate of development and to normal metabolic function.

The hospital says treatment of panhypopituitarism depends on its cause. And the goal is to restore normal levels of hormones in the body. Drug therapy, they say, may be used to replace hormones that are being underproduced.

Follow-up care

Regular office visits with specialists are necessary to make sure medications are working, and children are growing and developing as expected. When medication is needed to supplement hormone production, periodic follow-up tests are needed to ensure that the treatment continues to work effectively.

While Terrell loves her “mini me”, she said she hurts for her son because she wants him to be like “normal kids”.

“When I look at my family members’ kids – he has to look up to them and they’re younger than he is. He talks like a baby. His voice sounds like that of a two…three-year-old. I just wish sometimes he could be normal,” said Terrell.

Causes

Pituitary hormone production is regulated by the hypothalamus, the area just above the pituitary gland, that helps regulate hormone release from the gland. Reduced hormone output could be due to a problem with wither the hypothalamus or the pituitary gland. Those problems, in turn, may be caused by a tumor or cyst on or near the pituitary gland or hypothalamus; underdeveloped or poorly formed pituitary gland; birth trauma or other injury; infection; autoimmune reaction; pressure from hydrocephalus; surgery; radiation treatment; or sometimes, no exact cause of panhypopituitarism can be determined.

Terrell recalls her blood pressure being high when Amarion was born, and that she had been hospitalized for a month. She gave birth at 35 weeks; Amarion weighed five pounds, three ounces. While in hospital, she said, Amarion had two seizures, but that when they were released from hospital, that they went on as a normal family.

“When he was like three…four years old, he was really short and I was told he would probably be short,” she said after bringing it to his doctor’s attention. One day, Amarion got really weak and she took him to another doctor who brought it to her attention that something wasn’t right with him and questioned his size and weakness. Amarion’s glucose was tested and found to be low. The doctor referred the family to Miami Children’s Hospital where they saw an endocrinologist. And he was diagnosed with panhypopituitarism.

A mother’s love

While he may be small in size and speaks at just above a whisper, Amarion is definitely not fragile but his mom does not see it that way, and she coddles her son.

“He’s so tiny and fragile…I don’t want anything to happen to him,” she said.

Terrell said her daughter, Angel, 20, comes down hard on her for treating him like a “baby”. But Terrell said she is only protecting him.

“Amarion can move around on his own but, me, as a mother, I just tend to do these things for him because he is so fragile. He’s capable of doing things but I don’t want to let him loose.”

Before she leaves the house, Terrell said she can’t function unless she knows he has something to eat at his fingertips as Amarion does not venture into the kitchen, at all.

She admits that she has coddled him to the point where he expects everything to be brought to him, even though she said there’s no reason why he can’t do things for himself. She even has fears about allowing him to get involved in sports out of fear he may be hit.

“If he wasn’t in the position he’s in, it would have been different. Sometimes, if he’s lying in bed, I would say ‘Amarion, you OK, you feeling weak?’ and he would say, ‘yes mommy, I’m fine’, and he gets angry because I’m asking him.”

Worrying about her son, she said, mentally affects her because she knows she is mentally affecting him.

“It’s just that I’m always so paranoid because I always wonder if his sugar has dropped,” she said.

Amarion has also developed into a homebody, who likes to spend his time indoors, away from people.

“Sometimes I say Marion let’s go outside, because he doesn’t really come outside like that and, if he sees other people when he does go outside, he immediately goes back into the house. I wouldn’t say he’s not normal but he’s afraid of people. He doesn’t like to come out. Even before the pandemic, he was really shy, and he’s not outspoken at all.”

Traits that are reflective of his mom who, herself, admits to being a shy person.

“It hurts because I want him to be like normal kids,” said Cleare, who said his shyness has been exacerbated by how he has been treated at school as his peers teased him because he would not grow over the summer months, when they all had.

If there is one thing Amarion likes, she said, it’s the water.

“We take him on the beach and his dad would take him into the water and he would just go, and I would say look at my boy swimming.”

While he needs his HRT, Amarion’s mom said he also needs testosterone, and is currently taking thyroid medication and sugar tablets.

One of Terrell’s proudest moments was seeing her 14-year-old graduate sixth grade last year, even though he should have been in eighth grade.