LANDEN JOHNSON: I am Landen and I am seven. I am three feet tall.
LANDEN JOHNSON: One in four million.
LANDEN JOHNSON: Hi, my name is Landen and I am seven .
INTERVIEWER: How tall are you?
LANDEN JOHNSON: This tall.
COMM: Weighing only 24 pounds and standing at just three-feet tall, Landen has one of
the rarest types of dwarfism in the world.
AMANDA JOHNSON: He is seven years old and he is almost about the same size as his three-year-old sister.
LUKE JOHNSON: He has primordial dwarfism type DNA Ligase four. Landen, his type of primordial
dwarfism, he is one of 28 that we know of in the world.
AMANDA JOHNSON: He weighed five pounds, six ounces when he was born.
AMANDA JOHNSON: There were no clues that there was anything wrong.
AMANDA JOHNSON: It wasn’t long after he was born that we noticed he wasn’t gaining
weight. He was referred to different doctors to figure out, try to figure out what was going on.
LUKE JOHNSON: When he was six months old, we were referred to a geneticist and that’s
when she, that day she diagnosed him with primordial dwarfism.
LUKE JOHNSON: Primordial dwarfism is the rarest form of dwarfism in the world. Those who are
affected by primordial dwarfism are proportionate in size. They are just very small. I think
you feel as parents some fear and I concern for the future and what it meant for Landen.
AMANDA JOHNSON: Landen’s dwarfism is different compared to the other types in
that his immune system fails.
DR PHILIP ROEHRS: This condition is so rare that we really don’t know the true incidents.
There is only probably less than 100 cases worldwide. We still are learning a lot about
the condition. His genetic condition led to a broken immune system. His own immune system
was attacking his body.
COMM: When they discovered Landen’s particular type of dwarfism, he and his family were told
it was likely he would need a bone-marrow transplant at some point in the future.
LUKE JOHNSON: It was last year, we got the phone call saying it was time for him to have
the bone marrow transplant. They told us that this was going to be the best job for him
to live a long healthy life. That’s why we decided to just go ahead and get through with the transplant.
AMANDA JOHNSON: His brother and sister were not a match and so in the end we went to
the National Bone Marrow registry and they owned a donor in Europe.
LUKE JOHNSON: He had to go through two weeks of conditioning, which they used chemotherapy.
They tear down his current immune system, get it to zero, so that they can do the transplant
with the donor cells and then build a new immune system for him.
DR PHILIP ROEHRS: So today is a routine follow up for Landen. He is about five months after
a bone marrow transplant.
DR PHILIP ROEHRS: Since the transplant, Landen has been in clinic at least three times a
week. He has been re-admitted a couple of times for complications of the bone marrow
transplant. So we see a lot of Landen.
LUKE JOHNSON: I mean he handled it really well. Some of the side effects was you know,
of course nausea and vomiting. But he lost his hair after the last one. Think about chemotherapy,
he is still experiencing some of the side effects of that. I mean it’s just a powerful drug.
LUKE JOHNSON: The transplant was a success and it will help provide him the immune system
that he needs to be able to fight off infection as he gets stronger and he will able to get
back to life as a, as a child, as a normal child and without all the medicine and fluid
and all the things he is having to do now.
COMM: It can take up to a year for bone
marrow transplant patients to make a full recovery and unfortunately, Landen has been
experiencing problems since his procedure.
DR PHILIP ROEHRS: TMA, Thrombotic Microangiopathy,
this is a newer complication of bone marrow transplant and now we have a treatment for
it. And, so far Landen has been doing very, very well with the treatment that we have instituted.
DR PHILIP ROEHRS: His donor’s immune system is still learning that Landen is the
new home and hopefully over the next several months that immune system will really start
working well and control these viral infections.
LANDEN JOHNSON: How you doing?
DR PHILIP ROEHRS: How am I feeling? Well, Mondays.
LUKE JOHNSON: It’s heartbreaking because he is
completely different now than he was before transplant.
LUKE JOHNSON: He tires out quickly.
LUKE JOHNSON: He goes off to sleep. He doesn’t take naps or never took
naps before. Energy levels have been greatly affected.
AMANDA JOHNSON: Biggest thing
he is not able to do since his transplant is to be out in public, which is devastating
for him because he is a people person. So that’s been the biggest challenge.
LANDEN JOHNSON: I got to say, you are beautiful.
JOHNSON: Sam, I got to say you are..
MANDY: Sam? Sam?
LANDEN JOHNSON: I mean Mandy.
MANDY: Yes.
LANDEN JOHNSON: I got to say you are beautiful.
MANDY: Yeah? Thank you, honey.
LANDEN JOHNSON: You are welcome.
MANDY: You are so sweet.
AMANDA JOHNSON: He’s been able to go outside only a handful of times since transplant.
LANDEN JOHNSON: I am going out to play. Yay!
AMANDA JOHNSON: Today was special to see him go out and he ran a little bit in yard. That
was a big step for him.
LUKE JOHNSON: Landen, Landen loves to pretend. He has got a huge imagination. He just loves to play.
LUKE JOHNSON: He is teaching me a lot about faith, I admire his faith and his strength
through everything he’s been through.
LUKE JOHNSON: I would say he loves being with family and having family time.
LUKE JOHNSON: He is going to be smaller and he may have more health challenges that he,
that he, he doesn’t have to limit himself. So I expect to see him productive in his adult
life and especially as determined as he is and as wide open as he is.
He can do anything he wants to do.