By Nathalie Bonney @nathaliebonney
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Videographer / director: Adam Gray
Producer: Nathalie Bonney, Ruby Coote
Editor: Thom Johnson
A LOVEABLE 8-year-old boy has a form of dwarfism so rare he is believed to be one of only 28 cases in the world.
Landen Johnson, from Lincolnton, North Carolina, has primordial dwarfism, one of the rarest forms of dwarfism in the world, with experts estimating there are less than 100 cases worldwide.
But Landen’s specific form of primordial dwarfism, DNA Ligase four deficiency or Ligase 4 deficiency, which also affects the immune system, makes him even rarer: his family say they know of only 28 other cases worldwide.
At only three feet tall, Landen Johnson might only grow another foot in his lifetime but whatever his physical height, Landen walks tall thanks to his courage and humour.
Five months after having a bone marrow transplant Landen still has to go to hospital three times a week, can barely go outside and has to take multiple medications. He may not have as much energy as he used to but Landen’s spirit remains unbreakable.
Landen’s father Luke, who is a pastor in Lincolnton, told Barcroft TV: “I admire his strength through everything he has been through. He is teaching me a lot about faith and he has taught me a lot about what it means to follow the lord and be strong, even when you feel so weak sometimes.”
Landen is 3 foot tall and wears clothes for toddlers; weighing 24 llbs (or 10.8kg) - he is lighter than his three-year-old sister and experts believe he won’t grow beyond 3.5-4ft tall.
Amanda, Landen’s mother, said: “He wears a size 3T clothes, which are meant for boys aged three and he has to have [an] adjustable waist.
“He is eight years old and he is almost about the same size as his three-year-old sister, she weighs more but they are about the same height.
When he was born, Landen’s weight was below average but not so light to set off any alarm bells. As Amanda recalled, baby Landen looked, “like the average baby, just a lot smaller.”
She said: “He weighed five pounds, six ounces when he was born, which was nothing alarming at that time. There were no clues that there was anything wrong.
“It wasn’t long after he was born that we noticed he wasn’t gaining weight. We started, probably a couple of weeks after he was born going to the doctor for weight checks every week and then from there, because he wasn’t gaining weight, he was referred to different doctors to figure out what was going on.
“They tried formula with extra calories to see if that would help but it didn’t. Other than that, he ate regularly.”
Instead of putting on weight, Landen initially dropped to 4lbs, 11oz and only then started to gain rate at a slower than average rate. His lack of growth puzzled doctors and Luke and Amanda saw at least four specialists to try and find a diagnosis for their son.
Luke, 34, said: “When he was six months old we were referred to a geneticist at UNC Chapel Hill. We went to that appointment like we did the others not expecting to find anything out, but we went in and saw her, and she started doing a number of detailed measurements on Landen and that is when she diagnosed him with primordial dwarfism.”
After so many hospital appointments without any conclusive diagnosis Amanda and Luke both admit they were floored by their son’s diagnosis.
Amanda, 32, told Barcroft TV: “The best way I know to describe how it felt getting the diagnosis that day was like hitting a brick wall. We were moving on with life, not really expecting anything and all of a sudden, we get this answer out of the blue and we hit a brick wall and then we have to pick ourselves up and say, ‘What now?’
In spite of his small size, Landen was a confident and happy child, who was as comfortable talking to strangers as friends and family. He loved being active, whether that was playing catch or shooting hoops (big bro Brayden, 10, would lift up Landen so he could achieve a slam dunk).
Then, when Landen was three, the Johnsons were dealt another blow: Landen was diagnosed with Ligase 4 syndrome, a rare autosomal recessive disorder, which prevented his immune system from working properly, even causing it to, in effect, attack itself.
Luke said: “2013 is when they found the DNA Ligase 4. They noticed that his platelets were low and some other numbers were low.”
Landen was hospitalised several times, his body’s immune system unable to fight off everyday viruses. Doctors told the family a bone marrow transplant, in the future, was the best way to ensure he would have a long and healthy future and in October last year, they got the call.
Luke said: “They told us that this was going to be the best shot for him to live a long healthy life and that’s why we decided to just go ahead and get through with the transplant.
A sibling is usually the best match for a bone marrow transplant but neither Landen’s sister or brother, Hannah Grace and Brayden, were matches. Eventually a donor match was found, through the national bone marrow registry, in Europe. Before the transfusion could happen though Landen had to undergo chemotherapy.
Luke said: “He had to go through two weeks of conditioning, where they use chemotherapy. The conditioning process tears down his current immune system so that they can do the transplant with the donor cells and then build a new immune system for him.
“He handled it really well. Some of the side effects was of course, nausea and vomiting, he lost his hair after the last one.”
Being treated at the Levine Children’s Hospital in Charlotte, North Carolina, Dr Philip Roehrs M. D, who specialises in paediatric bone marrow transplants, says Landen is still several months – or longer – away from full recovery.
He explained: “His donor’s immune system is still learning that Landen is the new home and, hopefully over several months, we will be able to back down on those immune suppressants and then that immune system will really start working well and control these viral infections.”
Even though Landen has a long way to go, his parents are confident their son has what it takes to make a full recovery.
Luke said: “Our prayer is that Landen lives a long, full and healthy life. We’re going to challenge him and motivate him to do anything he feels God leads him to do even though he is going to be smaller and he may have more health challenges, he doesn’t have to limit himself.
“I expect to see him productive in his adult life, especially as determined as he is and as wide open as he is.
"He can do anything he wants to do.”