Jason was born full term and had no complications at birth. He was born with a cleft soft palate and was diagnosed with Stickler’s Syndrome in the first few weeks of life. His two older sisters also have Stickler’s Syndrome which affects their vision. At this time, effects of Stickler’s Syndrome appear to impact only Jason’s hearing. He was diagnosed with a moderate to severe unilateral hearing loss in January, 2008 through and ABR at Primary Children’s Medical Center. Jason hit his developmental milestones at appropriate times, sitting by 8 months, waving bye and “giving 5” by 8 months, crawling by 10 months and walking at 13 months. In July, 2008 he was diagnosed with mild nearsightedness that will require him to have glasses when he is older. In June, 2008, Jason had surgery to repair his soft palate. At that time the surgical team noticed that in addition to the cleft soft palate, Jason was also tongue tied and also had a flap of tissue connecting his upper lip to his gum that was longer than normal. The doctors clipped the tongue to give his tongue freedom to move and clipped the tissue between the lip and the gums. That provided explanation as to why he had limited speech sounds at that time and was not babbling/jargoning with as much variety of speech sounds. In September, 2009, Jason began to display behaviors that were quite different than had been observed in the past. He became more introverted, lost eye contact with others, stopped playing interactive games, and stopped using the few signs and words that he had been using up to that point. Concern was expressed that he was developing behaviors characteristic to Pervasive Developmental Spectrum Disorder (PDD). These concerns were confirmed by Dr. Herrman Peine in September, 2009. At that time, marked changes in the early intervention services he received were made to include increased services from Jason’s service coordinator from EI, Jeanne Fowles, and to involve the agencies specialist on PDD, Jean Delangelo. Jason also was involved in the sensory integration group provided through Davis Early Intervention.
Jason has an interesting auditory history. Jason began Parent Infant Program (PIP) services in February, 2008, at five months of age. He had been tested through an ABR at Primary Children’s Medical Center which revealed a possible moderate to severe unilateral hearing loss in his right ear. At that time he had been diagnosed with chronic otitis media and had just had tubes placed in his ears. His expressive and receptive language scores showed a one month delay in both areas of communication/language development at the start of services, which were consistent with his hearing loss. His family was consistent in alerting him to sounds and attaching meaning to those sounds, and before long he began to respond to and discriminate a variety of environmental sounds and he began using more speech play, especially after his soft palate was repaired and his tongue was free to move easily.
Jason’s hearing evaluations up through March, 2010, show a mild to moderate unilateral hearing loss in the right ear. He wore a hearing aid in his right ear for a year. It took a while for him to build up the wear time, but within a few months he enjoyed the aid and would request it. If the hearing aid battery died, he would take the hearing aid off and give it to his mom. So it apparently helped him or he would not have noticed that the battery was dead and would not have taken the aid off. Because the aid he was using was a loaner, he was able to keep it for a year. The Lutz’s decided not to buy him a hearing aid, primarily because it was not long after the aid was returned that the PDD behaviors started to surface and the focus of his development shifted quite a bit. He has had fluctuating otitis media that has caused a periodic conductive hearing loss to compound the sensory-neural hearing loss, but hearing loss in the right ear was consistent until March, 2010. We saw a flip in his hearing with tests in March and June, 2010, showing now a mild to moderate loss in his left ear and normal hearing in his right. We have no explanations for why this occurred or for why we are seeing this change in his hearing at this time. It does not appear to be related to otitis media. He responds to a variety of environmental sounds and simple commands and statements such as, “It’s time for your bath”, “Do you want a popsicle?”, and he will run to the television when he hears the music to the Blue’s Clues TV show. He’ll also run to the bathroom from another room when he hears the bath water running.
Jason’s receptive language score on the Language Development Scale is at Unit 11 with an age equivalent of 20-22 months of age. He will occasionally follow one-step directions, but typically he does not respond to directions or questions unless he is in the mood to do so. He will respond to questions like, “Are you hungry?” by going to the kitchen, “Hold my hand”, and “Go to the van” inconsistenly, but he can and will respond appropriately sporadically.
Jason uses signs and voice to communicate. Initially the family wanted only to use spoken language with Jason but as time continued Jason became more and more frustrated because he couldn’t easily communicate what he wanted. The Lutz’s began signing with Jason in December, 2008 because he was showing frustration at not being able to communicate verbally.
Jason’s expressive language score on the Language Development Scale was at Unit 9 with an age equivalent of 16-18 months of age prior to the changes in behavior that we saw in September, 2009 with the diagnosis of PDD. Since that time, he has tested at the 10-12 month range in expressive language. Prior to the diagnosis of PDD, Jason would say and sign mama, dada, shoes, more, please, milk, bye-bye, night-night, all done, thank you and popsicle. He would say Duncan, nee-no (for no-no), uh oh, puppy, hi, and he would say mail to request the Blue’s Clues television show. He used to sing along with his siblings when they would sing to him.
Since he began exhibiting behaviors consistent with the PDD spectrum we have seen a loss of many of the words and signs that he used to express, however, he will still use some of the words and/or signs listed above when prompted and will occasionally use them spontaneously. The most common words he uses are popsicle and eat and he will also say bye-bye, love you, and all done. He jargons vocally with a wide variety of consonants and vowels (such as yaya, pa, nana, gaga, lala, etc.) and he can also growl and smack his lips and enjoys mouth play. The list of words that Jason used prior to the diagnosis of PDD has been provided in this report with the hope that they could be targeted in interactions with Jason with the hope that some of this vocabulary might return to his expressive vocabulary.
A simple picture system was put in place a month or so ago to help Jason begin to communicate his wants and needs through selection of pictures and manipulation of the pictures that will supplement the signs and the spoken language currently used to communicate with Jason. He has been doing well with that system.
Jason’s family is very involved with him and concerned about his progress. He has an older brother and two older sisters, and they are all so interested in helping Jason succeed and make progress. They have made renovations to their home to better address Jason’s needs and they work very diligently with him to encourage his communication and interaction skills with them and others. His grandparents and extended family members are also very concerned about meeting Jason’s needs and they are also actively involved with him.
There are a few things that will consistently engage Jason, music, drawing, books, and high energy gross motor play, such as swinging, wrestling, hanging him upside down, and climbing. He is amazingly nimble and his balance is amazing. He does circus act types of activities and he rarely if ever falls. He has a very high pain tolerance level as well, so if he does hurt himself, it rarely affects him much. These types of activities are sure to engage him and encourage learning. Taking advantage of these strengths would be beneficial avenues to take advantage of to help him learn new things.
Since the use of signs, spoken language and pictures have been used successfully with Jason, it is recommended that these three modalities continue to be implemented in the classroom setting. The introduction of a picture system is relatively new to Jason, so this is a system that will need more thought and development. Consistent use of these three modalities is highly recommended.
Jason can become frustrated and irritated with changes in routine, so use of transition methods from one activity to another is highly recommended. Transition strategies such as telling him he has three seconds to put away his things before he leaves or has to move on to a different project, or telling him where you are going five minutes or so before you actually leave so he can mentally and physically prepare for the change are highly recommended.
The Lutz’s identified three primary goals that they would like to see Jason work toward accomplishing in this next year. They are:
- Jason will communicate effectively in some way, using any modality available to him.
- Jason will follow more simple directions and commands.
- Jason will develop social relationships.
Jason is a charming boy and fun to interact with, especially when you can get him engaged in an activity that he really enjoys, such as music or drawing. He can attend for several minutes when he is doing something he loves. Jason loves to learn new things and he has a curiosity about his environment and all that is in that environment. He will bring energy and life to his classroom.