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Rhett Powell, of Sedley, recently celebrated one year of life, and his parents hope to help him celebrate many, many more as they care for him amid a rare condition he is suffering from called Alagille Syndrome.

“There isn’t a thing I wouldn’t do for my son,” his mother, Jenna Powell, said.

Johns Hopkins Medicine (JHM) reports on its website that this syndrome “is an inherited condition in which bile builds up in the liver because there are too few bile ducts to drain the bile. This results in liver damage.”

JHM noted that the syndrome is related to the JAG1 gene mutation, and the syndrome appears in one out of 70,000 babies and occurs in both sexes. About half the time, the mutation is new and not from a parent, and this is the case with Rhett.

Because of the severe impact this condition has had on his body, Rhett is in need of a willing individual to donate part of their liver to him within the next 10 months. The part of the donor’s liver that they donated grows back within four to six weeks.

Rhett needs a living donor that has B or O as their blood type; positive or negative does not matter. The individual needs to be in the 18-60 age range, in good health with no preexisting medical conditions like high blood pressure or diabetes, no major abdominal surgeries, no illicit drug use, a nonsmoker and possessing a body mass index of around 30.

Those who think they may be a match or who know a potential match can contact Jenna Powell at 757-556-5182.

RHETT’S MEDICAL CONDITION

Rhett is a Sedley native who was born June 13, 2024, to his loving parents, James and Jenna Powell.
“When he was 2 months old, he got admitted for his lab work, his liver numbers being abnormal,” Jenna said. 

Prior to that, Jenna, a first-time mother, was catching all the blame for Rhett not gaining weight correctly.

“First it was my breast milk, then it was me using the wrong size bottle nipple and then it was me mixing the formula wrong,” she said. “And we brought up to the doctor several times that we thought he looked jaundiced, and we got blown off and told that sometimes babies just look jaundiced longer than normal. 

“Well, babies do not look jaundiced longer than normal, but I’m a first-time mom, so I am being told that everything’s fine when it wasn’t fine, and I should have went with my actual gut instincts,” she added.

She noted that knowing what she knows now would not have changed Rhett’s overall condition, but it would have gotten him on itch medications sooner to help with itching, one of the key symptoms of Alagille Syndrome.

Jenna described the situation that Rhett and others with this condition face.

“(Their) liver has fewer than normal bile ducts, so it can’t remove toxins and stuff like it usually would,” she said. “So the bile salts build up in their bloodstream, and they itch until they draw blood. And they just keep scratching. A lot of the kids end up with infections that they have to get treated prior to transplant.”

Echoing and expanding on Jenna’s description, Johns Hopkins Medicine states the following on its website with regard to Alagille Syndrome: “Your liver makes bile to help remove waste from your body. It also helps digest fats and the fat-soluble vitamins A, D, E and K. The body can use these vitamins only if they are combined with fat from the foods you eat. Bile ducts are the channels that move bile out of your liver. If you do not have enough of these ducts, bile builds up in your liver. It damages your liver tissues, and ultimately can cause your liver to fail.”

Jenna noted that Alagille Syndrome is the reason Rhett has liver disease, and Rhett has other medical issues that relate and do not directly relate to the syndrome.

“He has something called pulmonary stenosis, so the arteries coming off the heart going to the lungs are too narrow,” Jenna said.

Rhett’s eyes possess what JHM describes as a classic sign of Alagille Syndrome.
“It’s something called posterior embryotoxon,” Jenna said. “It’s a ring that’s around the cornea that puts him at a higher risk for developing glaucoma.”

Jenna noted that Rhett has vitamin deficiencies in most of his fat-soluble vitamins — K, D and E.

“He has a fat malabsorption problem, so he’s on specialized baby formula that it took me six months to figure out how to get,” she said, noting that she called 50 different speciality pharmacies, tried to obtain it through her insurance provider and persistently called the manufacturer.

Rhett is currently in the zero percentile for his height and weight, as Jenna noted that at 1 year old, he weighs 15 pounds, 6 ounces and is 28 inches long.

“He’s very, very small, which isn’t uncommon for Alagille Syndrome, and it’s definitely not uncommon for somebody whose liver doesn’t work,” she said.

Rhett also has renal tubular acidosis. 

“It makes his blood really acidic, so he takes sodium citrate twice a day to level out his blood so the CO2 and stuff isn’t high, because that can also contribute to poor weight gain,” she said.

She noted that Rhett also has his hearing monitored every six months because there can be abnormal structures in the inner-earbone that can actually cause hearing loss. 

“That’s more of a preventative thing, because they haven’t seen anything wrong with his ears yet,” she said, “but I pushed for that, because this is a rare disease, so there’s no real studies on anything other than the liver, because that’s the most deadly component. Although there are certain things wrong with your heart that can cause you to have open-heart surgery and all kinds of other issues like that.”

But at least when it comes to his heart, Rhett’s condition is not as serious.

“He would just need a balloon surgery one day to open up the arteries,” Jenna said.

WHAT RHETT IS EXPERIENCING

In the face of the aforementioned medical issues, Jenna pulled back the curtain on what Rhett is experiencing.

“He vomits way too much,” she said. “And he never has a solid bowel movement. It’s always like water because his liver doesn’t work. He has to take safflower oil because he has an essential fatty acid deficiency.”

She said his body simply cannot absorb food the same way most people’s bodies can, “and it makes it difficult for him to try to enjoy things other than his specialized formula, because it’s basically like he’s eating air at that point.”

She said, “He has a really hard time sleeping because of the itching, which if you don’t sleep, you don’t grow. So there’s another thing going against him there.

“And it’s stunted his fine and gross motor skills,” she continued. “He doesn’t clap or wave, he can’t sit up on his own, he can’t even pull himself up into a seated position. He can’t crawl. He can roll over, but that took him a lot longer than it should have.”

Jenna said Rhett essentially takes a sedative to help him sleep, and even that does not work sometimes.

“He’s never had a set sleep schedule just because it’s hard to make somebody who doesn’t feel good go to sleep,” she said. “Say he needs to throw up — he might not go to sleep till he has to throw up. Or if his medicine schedule gets even shifted by 30 minutes, that could mess up his bedtime. He basically has set his own bedtime. Usually he gets so exhausted by 11 p.m. from trying to fight sleep and itching that he just crashes.”

HOW RHETT HANDLES HIS EXPERIENCE

Photos of Rhett smiling testify to his remarkable resilience in the face of an incredibly challenging life thus far.

“He is the happiest baby despite everything,” Jenna said. “If you saw him in public, you wouldn’t know that he had all this going on with him unless he was having a bad moment. It comes in spurts. Sometimes he can just have a bad day all day long, but usually even if he does, somebody can still find his favorite thing to get him to smile or laugh.”

HOW HIS FAMILY CARES FOR HIM

To best care for Rhett, Jenna is working and has the benefit of government health insurance. Her husband James is at home with Rhett, providing the unique care Rhett needs that would be too much for a daycare provider to handle.

“Calories for Rhett are the most important thing in the world, and that would have been hard to push at a daycare, because he would have needed a skilled nurse,” Jenna said.

Rhett also has to be held a lot due to the itching.

“(James) is providing the care himself, and I have just recently figured out how to complete a Medicaid waiver so that I can get my husband paid to take care of him every week,” Jenna said.

She estimated she has talked to probably a hundred people simply to make sure Rhett has access to the things he needs.

HOW A LIVE LIVER TRANSPLANT WORKS

As Rhett needs a live liver transplant, Jenna described how that process will work for the donor.

“In (Rhett’s) case, he’s so small, they might not even need the whole right lobe, for example,” she said. “But they would take a portion of someone’s liver and put it in him, and in four to six weeks, the other person’s liver would grow back, and it wouldn’t grow back in the exact shape that it was before, but it would be very similar, almost as if they never even gave a portion of their liver away.”

THE IMPACT OF A LIVER TRANSLANT

Jenna noted that for Rhett, a liver transplant “would solve everything to do with the fat malabsorption, the vitamin deficiency, the jaundice, the itchiness, all of that it would fix. Now it wouldn’t fix the acidosis or his heart problem or his eyes, but it would fix everything else.”

THE TIME FRAME FOR A TRANSPLANT

In a Monday, June 16, interview, Jenna said, “When Rhett was 10 months old, (medical personnel) said that they would like to do the transplant within the year, and it’s already been two months, so we’re down to 10 months, and I hope that we can get that accomplished before then, because there’s certain predictors. They’ve had to do enough liver transplants in kids with Alagille Syndrome that they know certain numbers like their albumin and their bilirubin and their (International Normalized Ratio) INR — they know off of that, based on when a kid is 6 to 12 months old, if they’ll need a liver transplant. And Rhett’s never really calmed down, and the simple fact that he’s already had to have a vitamin K shot to help his blood clot because his INR was so high is a pretty good indicator that his (transplant) will definitely need to be done within that year time frame.”