Signing Time Concert
Dempsey’s speech has been one of the biggest delays that we’ve seen so far. Up until a week or 2 ago, he only verbalized 5-10 words (Mama, Dada, Dempsey, Ball, Hi, Bye) and wasn’t stringing 2 words together. That’s pretty behind for a 2.5 year old. For reference, the Mayo Clinic says...
Between the ages of 2 and 3, most children:
- Speak in two- and three-word phrases or sentences
- Use at least 200 words and as many as 1,000 words
- State their first name
- Refer to themselves with pronouns (I, me, my or mine)
- Can be understood most of the time by family or close friends
Developmental delay (including speech) is definitely one of the more common trends among those with KBG syndrome and on top of that Dempsey has some hearing loss. But we realized that his delay was primarily physical. He seems to understand a lot of what we say to him and sign language has been AMAZING. When we were still in Colorado, Dempsey’s therapist recommended the Signing Time videos. We have a monthly subscription to the app which teaches basic signs in a fun way (mostly songs). We primarily watch Baby Signing Time because a lot of 2 year olds don’t have the fine motor skills to shape their fingers for more advanced signs. We’ve been watching them for a while now and have all learned so much! Dempsey has been able to communicate with us in the last year so much better than he would have been able to without it! I know we definitely have avoided some communication-related tantrums. I recently counted up that he knows over 100 signs!! It’s been so fun to see him progress and grow his signing vocabulary.
Earlier this year, we learned that Rachel Coleman (the creator of Signing Time) was hosting a free concert for kids with special needs in February. (How cool is that?) It was awesome. We learned that she just happened to be coming on vacation to the island and reached out to Dempsey’s Hearing Specialist to see if there were kids/families on the island that would be interested in a free concert. So basically, her family took the time out of their vacation to do this for free. Rachel and her husband have two daughters. The oldest, Leah, is deaf and is a huge part of the signing time videos that Dempsey watches. They also have a younger daughter with special needs. Rachel’s husband ran the electronics at the concert and Leah (who was so little in the videos, but is now in college) participated as well! Nick, Dempsey and I loved the experience. And Nick and I have talked about how cool it was to be in a room full of children (and families of children) with special needs. There were children with Down Syndrome, autism, hearing loss, and other rare syndromes like Dempsey's. Everyone there had been through so much. There was such a sweet spirit in the room. We have both said that it almost made us emotional, just the feeling of the room. It was such a relaxed environment, full of love. Almost immediately, there were kids just running up to Rachel in the middle of a song to give her a big hug. A few songs into the concert, kids were wandering up to the front of the room. For the first time, we didn’t worry about having to chase our kid down. All of the families and parents had this mutual understanding that “hey, it’s okay if we let our kids wander and enjoy this”. Dempsey loved getting to hear the songs that he was already so familiar with, though I don’t think he can fully appreciate how cool it was for him to get to be there. Rachel and Leah took the time after the concert to meet every single person that wanted to meet them.
I honestly think all of the TV that Dempsey’s been watching during this quarantine has strangely helped his speech haha. In the last 2 weeks, he has tried verbalizing SO much more (like Grandma, Grandpa, play, Mickey, Pete, and new ones every day)! He’s even trying to string words together!
I put together a video of Dempsey doing a good chunk of his most-used signs. There are about 80 of them here (excuse my mom voice saying “Can you sign…?” and “Good Job” a million times:
Nick, Dempsey and I with Rachel and Leah
Rachel singing to Dempsey
Dempsey signing along to a song
Dempsey’s Progress
Dempsey has come such a long way since getting his diagnosis in July 2018. He is now a little over 2.5 years old. We are currently down to this list of doctors/therapists:
Genetics- Dempsey will probably always check in with a geneticist regularly for the rest of his life to get updated on new information about KBG syndrome. We continue to learn a bunch about his syndrome from the KBG Facebook group, though we find the comments and questions on that page to be a little overwhelming at times.
Dempsey in his KBG Foundation shirt
Audiology- Continually checking on his mild conductive hearing loss. No changes since his diagnosis last year. He still wears a Cochlear Baha everyday (he has gotten very used to it and even asks for it sometimes). He can generally hear what we are saying when he isn’t wearing it but the clarity/volume is improved with the hearing aid. (I like to best compare his hearing loss to someone who requires mild prescription glasses. They can still see without them, the glasses just bring clarity). And at his age, hearing every aspect of a word is so important to his speech development. If he is able to catch up and his hearing loss doesn't worsen, he may not need the hearing aid forever. There is also a chance that as he grows and his bones/ear structure change, his hearing may improve. If not, he may need an implant or different type of hearing aid as we learn more. Either way, we are so grateful for technology that can help him out.
Right after Dempsey got his hearing aid
ENT- Goes hand-in-hand with some of the hearing loss. His tubes are still intact so we follow-up every few months to check on those as well.
Ophthalmology- He gets seen by the Ophthalmologist pretty often to check on his eyesight, ptosis and strabismus. The two surgeries that he got on his eyes last year seemed to be really successful. He still had some wandering and required patching for a few months. We would patch his left eye to help strengthen his right eye. (Basically, if one eye wanders more than the other, it doesn’t get as good of a picture so the brain will start to ignore the image coming from the weaker eye. So the weak eye can become even weaker until the brain ignores that image completely. To avoid becoming blind in the weak eye, we patch the strong eye to force the brain to use the image from the weak eye). Dempsey wasn't the biggest fan of the patch and it was rare for him to wear it the whole time he was supposed to. Luckily, we don’t have to patch right now but the doctor said she might have us start again anytime in the future if needed.
Right before Dempsey’s ptosis surgery
On the way home from the ptosis surgery, sound asleep (he couldn’t close his eyes for months and still sleeps with his eyes open a lot)
Wide open eyes! As they started healing
More open-eyed sleeping
There were several months where almost every picture I have of him is like this (eyes crossed from the strabismus)
Right before his strabismus surgery, this was the first time that we were able to go back with him for anesthesia
On the way home from the strabismus surgery
Endocrinology- Dempsey started seeing an Endocrinologist when we moved to Hawaii regarding the short stature associated with KBG syndrome. He also has had several x-rays that showed that his long arm and leg bones are still disproportionate (rhizomelic shortening), which is why they guessed he had dwarfism when I was pregnant with him. Those with KBG Syndrome are several inches shorter than the average height so many use growth hormones. Dempsey has always been below the charted percentiles for height and weight. He started growth hormone therapy a few months ago. He gets a shot at home every day and handles it like a champ (he did not like it at first, but after a week it was old news haha). It’s hard to tell if it’s the medicine or a regular growth spurt but he seems to be growing like a weed the past few weeks! Even with growth hormones, he likely won’t reach average male height. We will see! Obviously, short stature is not a life or death symptom but can cause some pain and health issues with the joints and bones if not improved. There are very few and rare side effects to the medication so after hearing our options, we felt very comfortable following the doctor’s recommendation to start it for Dempsey (as long as he tolerated the daily shots, which he barely notices now).
Developmental & Behavioral Specialist- We’ve only met with this doctor once recently (it took a looonnnng time to get in to see him) but it was very helpful! He may be the most helpful and thorough doctor Dempsey's had since we moved to Hawaii. He’s going to look into getting Dempsey some additional direct speech therapy through Kapi'olani Children’s Hospital.
Occupational Therapy- Still working with an OT through Early Interventions on various things (areas that he has fallen behind the benchmarks for his age).
Speech Therapy- As mentioned above, this has been the biggest delay that we’ve seen so far. But we meet with a speech therapist almost every week.
Hearing Specialist- Also through Early Interventions. Gives us great advice regarding his hearing aid, future schooling and keeps us informed about events for kids with hearing loss (like the Signing Time concert)! We've been discussing preschool because when he turns 3, he will transfer from Early Interventions to the department of education. Can you believe Dempsey will start preschool soon? He might qualify for a special classroom with other kids that have hearing loss at a school in the town next to us. We will spend the summer in various meetings with the DEO to figure out the best fit for him, but we're hoping he can get in there.
Neurology- Not a specialist that we have to see regularly but since seizures are very common among KBGers and could start at any age, it’s definitely always on our radar. Fortunately there have been no seizures yet!
Pediatrician- Mostly just the usual stuff :)
That means we’ve been able to ease up a ton on GI and Cardiology! Regardless, Dempsey still keeps me pretty busy with weekly therapies and frequent specialist visits but he is happy and healthy! He's grown used to being able to play outside every day and goes crazy when it's raining. Luckily no cold weather to fight against! We are still learning and adjusting to what life is like with a kid with KBG Syndrome. We are so grateful for the people in our lives that help us. We are still traumatized by his newborn experience so we are pretty anxious about having another newborn in our home this summer. Dempsey can't wait to be a big brother (we think haha). Wish us luck!
