Friday, October 18, 2019

Mosaic trisomy 9

It's true: Not all heroes wear capes. Some are just 3 months old and are already in the fight of their lifetimes. That's the case for Randy James Ahlers - "RJ" - and his parents, Angel and KC Ahlers of Toledo.

By all accounts, Angel's pregnancy was fairly routine and there were no signs that the Ahlers' baby would be anything but healthy. So, it was a shock when RJ was born and doctors discovered he had not one, but two very rare conditions.

The Ahlers' son has Agenesis of the Corpus Callosum or AgCC, and Mosaic trisomy 9. With AgCC, the center of the brain that facilitates communication of the two hemispheres is underdeveloped. About 1 in 4,000 people are born with this. Mosaic trisomy 9 is a rare chromosomal anomaly syndrome. This happens to 1 in 4 million babies.

"Twenty minutes after he was born, the doctors came in and said they wanted an emergency neurological team to look at our son," KC Ahlers said. "They took him from us, and my wife and I were terrified. About an hour and a half later they brought RJ back to us and said the doctor would be in soon to talk with us.

"We were informed that this could mean RJ could have issues seeing, seizures, and mild to severe mental disabilities, and the potential for intellectual disabilities, facial dysmorphism, congenital heart defects, urogenital defects, skeletal defects, and central nervous system defects," KC Ahlers said.

The Ahlers were faced with some very hard to hear facts. Only 50% of children born with the conditions RJ has reach the age of 2. But, the Ahlerses are undaunted - they are exploring every possible option to help their rare son beat the odds. This comes at a steep cost, however. They already have more than $3,000 in accumulative medical bills for his condition from six different specialists, none of whom has ever worked with a Trisomy 9 Mosaic patient. 

"He has medical tests he needs that we have not done yet because we cannot afford them. A DNA/RNA genetic testing of all major organs could locate the areas he has the mutated Trisomy 9. This will let us know what to expect," KC Ahlers said. "This test alone is $10,000 and insurance claims it's not medically necessary. Plus, we have the cost of any future tests and therapy he will need, which is a lot.

"Every day we wake up terrified that this is the day our child will have a hole present itself in one of his organs and bleed out and die. Every cry he makes, we have to worry, 'Is this that moment?' It's like walking in a minefield."

Angel Ahlers says they are willing to do anything for their son, even as medical bills are bankrupting them. They do have medical insurance, and she is a nursing assistant at St. Anne's, but many of RJ's medical tests are being said to be not medically necessary.  

"Just because he is different does not detract our love for him. He has a condition that is rare, and add the AgCC and now he is the rarest of the rare," she said. "We love him so much as the precious, one-of-a-kind treasure that he is, like a 25-carat diamond. How many of those are out there? Same as our son."

The Ahlers family absolutely loves comic books and Halloween - KC and Angel were married on Halloween - and they are combining the two in an effort to raise money to hopefully afford the tests that could extend RJ's odds.

This Saturday, the family and friends will don superhero costumes in honor of their tiny hero, and are asking the community to help fill the buckets for RJ's tests and treatments. They'll be unmistakable in their Marvel and DC Universe costumes at the intersection of Laskey, Tremainsville and Douglas roads in Toledo from 9 a.m. to 5 p.m. If you would like to help, but won't be out and about Saturday, a Go Fund Me has also been established and can be accessed here. 

"He's our fighter," Angel said. "He's our miracle and our gift from God."

https://www.wtol.com/article/news/local/toledo-baby-battles-2-rare-diseases/512-db46e6a8-c476-4cdf-bcd2-f04bc134a2fe

Owen Watson has Mosaic Trisomy 9, a rare chromosomal disorder. As a baby, he was failure to thrive. Now, Owen is walking, using sign language and gracing the world with his unbelievably sweet spirit.

Owen Watson is scooting around in a red and yellow car, his sweet face peeking through the open front window.

He climbs out and walks toward the miniature house. He runs through the front door. 

And then Owen starts signing. It’s a hot day outside in the play area of Riley Hospital for Children at IU Health. He wiggles his fist – the sign for a milkshake, which sounds good to Owen right about now. 

Sierra Watson is sitting on a bench, watching her 3-year-old son. She is in awe of all he’s doing. She’s grateful. She’s blessed.

And, yes, she is sad, too.

As Sierra tells the story of Owen’s journey, it is a tale mixed with smiles and tears.

Owen, who turns 4 this month, has Mosaic Trisomy 9 – an extremely rare chromosomal disorder in which the ninth chromosome appears three times rather than twice in some cells of the body.

The condition is so rare that it’s estimated that just 200 to 1,000 people in the world have the syndrome. Owen is believed to be the first known child diagnosed at Riley. Another patient, Ivy Case, also is treated at Riley.

In many cases, children with Mosaic Trisomy 9 have extreme developmental delays. Some never walk or talk. Many have severe intellectual disabilities. The prognosis for Owen is unknown.

And yet Owen, who was labeled failure to thrive as a baby, is at Riley telling strangers “thank you” by touching his fingers to his chin. He can point out colors.

Sierra often finds it hard to believe that Owen is doing so well. He’s had more than a dozen surgeries, plenty of scares and is still nonverbal. Yet, by all measures, Owen is thriving. 

That can be credited to a solid philosophy in the Watson household. Sierra and Owen’s dad, Daniel, do not let his diagnosis define him or get in the way, Owen still has the same expectations as his 6-year-old brother, Cole. 

“We don’t put limits on anything,” Sierra says. “We really push Owen and I think that is part of why he is doing so well.”

Minutes later, Owen smiles. And then he signs: “I want a cheeseburger.”

Her pregnancy with Owen, Sierra says, was textbook. She had plenty of ultrasounds – Sierra is a labor and delivery nurse – and nothing showed up.

Because she’d had a C-section with Cole, Sierra was scheduled for a C-section with Owen. When Owen was born and taken over to the warmer to be checked out by the medical team?

“I could tell something is wrong,” Sierra says, “but they didn’t say anything.”

Eventually, Sierra was told that Owen would need to go to the neonatal intensive care unit for breathing problems. Being a nurse in the field, Sierra didn’t think that was too unusual. 

But several hours later, she learned something different. Owen had a cleft palate. He had a very small jaw, which made it hard for him to breathe.

The original diagnosis: Pierre Robin syndrome – a condition where babies are born with a smaller than normal lower jaw, a tongue that falls back in the throat and difficulty breathing.

Owen was taken by LifeLine ambulance to Riley. The next few weeks were filled with meetings with specialists. 

 And then, when Owen was one month old, “we get this earth shattering diagnosis,” Sierra says. Mosaic Trisomy 9.

Sierra went to Google. The worst-case scenarios popped up. She was devastated. But soon, she found other families from all over the world who had children with Mosaic Trisomy 9.

And their hope gave her hope for Owen.

“He’s the sweetest kid,” Sierra says. “He’s doing really well.” 

Owen’s biggest issues now are feeding and breathing. He uses a feeding tube, but also eats by mouth.

In addition to those cheeseburgers and milkshakes, Owen loves waffles, peanut butter and jelly, all things carbs.

And he works hard to keep getting stronger – in all areas of his life. Owen does physical therapy, feeding therapy and occupational therapy. He practices sign language at home. And Owen goes to preschool.

As Sierra looks back on Owen’s journey, she says it’s so important to remember: “This is your child. It may seem bad at the time, but it’s going to be OK.”

Just look, she says, Owen is living proof.

Advice for parents of children with special needs: “You have to find time to take care of yourself. Even if that means taking a nap or having coffee with friends or going away for the night, your mental health is important,” Sierra says. “Also, you can’t forget about your marriage. Bringing a healthy child into a marriage brings added stress, but throw in health problems and it can be detrimental. You have to find time to be a couple. And when it comes to your child, don’t be afraid to fight for them and to ask questions about anything being done.”


Ivy Case has Trisomy 9 Mosaic, a rare genetic condition with a devastating prognosis. And, yet, Ivy is beating the odds.

Ivy Case is blowing bubbles. Sometimes, if she’s in the mood, she will curl in her lower lip and crack the tiniest of smiles. She has a sweet babble and will squeal if she recognizes a voice.

She also has a G-tube, oxygen, severe vision impairment and a look that, at times, will cause strangers to stare.

Ivy is 10 months old. She was born with a rare genetic condition called Trisomy 9 Mosaic. Google it and the results are devastating and daunting – an unending list of abnormalities and malformations.

Yet, Renee Case saw sweet Ivy on a special needs adoption posting in the summer of 2017.

“And I could not get her out of my mind,” she says. “I just knew there was something there. Now, we tell her that we found each other.”

Ivy is one of just 200 documented cases of Trisomy 9 Mosaic in the world. She is being treated at Riley Hospital for Children at IU Health.

“She’s literally one in 100 million,” Renee Case says.

Ivy’s condition – sometimes called T9M -- is a chromosomal disorder in which the entire ninth chromosome appears three times, rather than twice, in some cells of the body. Mosaic indicates that some cells contain the extra chromosome 9, while others have the typical two chromosomes.

To Renee Case and husband, Joshua, Ivy is perfect. The couple, who live north of Warsaw, Ind., brought her into their home in August.

They had already fallen in love with another child with special needs – their adopted son Jacoby.  And that love prompted them to seek out Ivy.

“It just felt right,” says Joshua Case. It felt right to choose the path of caring for children with special needs. “There is a reason we were supposed to be both of their parents.”

The reason? Well, it’s wonderful and beautiful. Both Jacoby and Ivy are thriving, beating the odds of their prognoses.

“When your kid is blowing bubbles like this, that’s huge for her,” says Renee Case. “It’s about finding the

Take sippy cups. Joshua and Renee went through more sippy cups than they can count to find one that Jacoby, now three years old, would use. His genetic condition causes sensory issues.

They didn’t know they were adopting a child with special needs, not until after Jacoby was born. They met Jacoby when he was two days old in the hospital. He spent 21 days in the neonatal intensive care unit before going home with them.

When he was 9 months old, the Cases decided to do some testing. They knew Jacoby had been born with microcephaly, a condition in which the brain does not develop properly, resulting in a smaller than typical head.

He also had a two-vessel umbilical cord, rather than three vessels, among other issues.

Jacoby’s genetic disorder is called Chromosome 16 p13.11 microdeletion syndrome. It is characterized by developmental delay, microcephaly, epilepsy, short stature, facial dysmorphism and behavioral problems.

And yet, all those things that were supposed to happen with Jacoby either didn’t or are mild, Renee Case says.

He wears braces on his ankles. Jacoby got his first set right before his first birthday and started walking weeks later. He has had some speech issues and, emotionally, he is also delayed.

Yet, Jacoby is doing so many great things. On this day, he is preparing to be line leader at his preschool the next day.

He knows – and talks about – every kind of dinosaur and whale there is.

“He is so incredibly smart,” Renee Case says. “He knows so much for his age.”

It’s evident Joshua and Renee Case are so very proud of their children. They are masters at putting life in perspective.

Besides all those bubbles and smiles, Ivy is starting to roll over a bit from back to side. At 10 months old, she is developmentally at about four months old.

“Which is really good for her diagnosis,” Renee Case says. “She’s just doing so good.”

The Cases didn’t know how Ivy would do when they took her into their home last summer, adopting her from a family in Texas. But they fought to find out.

For the first two months, Ivy had anywhere from two to five appointments every week – doctors, specialists, therapists and evaluations.

What they ultimately discovered was better than expected. Many of the issues, including heart problems, common to Trisomy 9 Mosaic aren’t present with Ivy.

And yet, no matter what might come along for Ivy – or Jacoby --  in the future, Renee and Joshua Case will be there supporting them all the way.

“We were meant to be their parents,” Renee Case says. “We were put into each others’ lives for a reason.”

No comments:

Post a Comment