Rural Trenton resident Lanae Johnson turned three years old on Sunday, celebrating the milestone like any other little girl. While she may be young, she is a pioneer in the medical field after recently undergoing surgery to treat an ailment that puts her in an elite group of patients.
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Rural Trenton resident Lanae Johnson turned three years old on Sunday, celebrating the milestone like any other little girl. While she may be young, she is a pioneer in the medical field after recently undergoing surgery to treat an ailment that puts her in an elite group of patients.
Lanae, the daughter of Tim and Jessica Johnson, was born without a portal vein – the large vein that routes blood from the heart into the liver. At 16 months, she was diagnosed with the condition, known as Abernathey Malformation, and became one of only 25 persons in the world who suffer from the disorder.
Lanae seemed healthy for the first several months of her life. Then her family noticed she seemed blue at times.
?We had been in St. Louis with family and when she woke up from her nap, they said she looked blue. Since I?m with her all the time, I guess I hadn?t noticed because it had happened over time. But they were right,? said Mrs. Johnson.
Over the next month or so, they also noticed that Lanae would be out of breath when she walked or played.
?It got to where she couldn?t even laugh without getting out of breath and coughing,? she said.
Concerned, they took her to the hospital, where doctors discovered she was getting very little oxygen. In fact, she was getting about 28 percent of the oxygen she needed. She was sent via helicopter to Children?s Mercy Hospital and Clinics in Kansas City. Doctors assumed Lanae was suffering from a heart condition, but after conducting several tests, it was discovered that she was missing the portal vein, causing her lungs to not receive enough oxygen. Doctors were planning to transplant the portal vein when they discovered that not only did she not have the vein, her liver had nowhere to connect the vein. In addition, the liver was on the wrong side and upside down. It was decided to replace the liver with a transplanted organ, which would automatically come with a portal vein. Abernathey Malformation is rare, but the remedy they were considering was even more rare. In fact, there was no record anywhere in the world of a liver transplant ever being performed as a treatment to Abernathey Malformation.
So, on April 7, 2003, Lanae went on the liver transplant list. Mrs. Johnson said she was told the wait would probably be six months or so, but the months kept passing.
?They had told me to keep a bag packed, but after a while I unpacked it. It seemed like it was never going to happen,? commented Mrs. Johnson.
For 18 months, Lanae and her family waited, with Lanae kept on oxygen during that time. As she grew, her body received less and less oxygen and her skin toned turn more and more blue. There was no doubt that without a new liver, Lanae would die. Finally, on June 30, 2004, the call came: a liver had been found. The family was in the middle of fixing their television service when the call came and for a minute, confusion ensued.
?My husband was behind the TV and a bunch of stuff had fallen and the phone had fallen back there and then it rang,? she recalled. ?It took us a minute to get to it and realize what they were saying. They said they had a liver and asked if Lanae was feeling okay, because if she had been sick with an infection or something they wouldn?t have been able to do the transplant right then.?
The surgery began at 11:30 p.m. at Children?s Mercy, with Lanae?s parents feeling they had no choice but to go forward.
?The doctors had talked to us and wanted to be sure we wanted to go through with it because of the risks and because we didn?t even know at that time if this was going to be the cure for her condition. But, we really had no choice. Without the transplant she was going to die.?
Lanae was in surgery for 15 hours, with her family unable to see her until 2:30 p.m. on July 1. Following surgery, she spent three and one-half weeks on a ventilator, totally sedated, followed by another week connected to a breathing tube. During that time, she was being given 100 percent oxygen in an effort to help the liver acclimate to her body. On Friday, Aug. 13, the oxygen was removed and exactly a week later, on Aug. 20, she came home just in time to see her brother start kindergarten and to celebrate her birthday.
The entire experience has been eye-opening for the family, according to Mrs. Johnson, who said she had no idea how many sick children there were until she spent weeks at Children?s Mercy. She has also become very aware of the need for organ donors.
?I had always thought I wanted to donate my organs, but this made me realize how important it is. It makes you feel weird because you know someone had to die for her to live,? she said.
Mrs. Johnson doesn?t know much about the donor of Lanae?s liver, but plans to write the family a letter after the six-month waiting period. In a year, if the families wish to meet, they can, and it?s something she would love to do.
For now, Lanae is enjoying being at home with her parents, her big brother, Trevor, and her little sister, Patricia. She is feeling pretty good, although her lungs have sustained damage during her illness. She takes medication to prevent rejection of the new liver and is monitored twice a week for any sign of rejection.
On Monday, just as Mrs. Johnson was explaining the complications that could arise due to rejection, the phone rang: it was the hospital, telling her that, sure enough, Lanae?s latest lab results indicate signs of rejection. What does that mean? Lanae will be returning to the hospital, where a biopsy will be performed on her new liver. Lanae is unaware of anything being wrong and her mother, while somewhat distracted after the call, seems to take it all in stride, giving credit to her faith in God and her friends at Cornerstone Church for helping the family get through the ordeal so far.
?They had already told us that 90 percent of rejections are reversible, so that?s the good news. But you worry about the 10 percent chance. What were the chances of her having something so rare in the first place?? she asks, laughing a little. ?But it?ll be okay. It has to be.?
During the interview, Lanae has shown some interest in the camera and posed for a few pictures. She watches her little sister play and looks at a new book. But as time goes on, it?s obvious she?s getting bored with all the talk. She goes to her mom and tells her she?s ready to go ?bye-bye?. She?s ready for her brother to come home from school. She?s wants to go somewhere else. She?s tired of sitting around with this reporter. Apparently, she?s ready to get on with her life – and thanks to an organ donor and the miracles of modern medicine, it looks like that?s what she?ll be able to do.
By Ronda Lickteig