CCHS - IEP meeting round 2

I’m so over this. People just amaze me and not in the good sense.

Yesterday we had another IEP meeting and after 7 months of battling the school district on my own, this time I brought an IEP Advocate. We addressed every issue, from the lack of timely response from the district, to the lack of follow through on everything previously discussed in the last IEP meeting back in October, to the ongoing district’s denial of the ASL issue. What it boils down to is this. Wyatt has yet to start pre-K and the school could not explain their lack of implementation. The school also could not explain why the district’s Physical Therapy and Occupational Therapy evaluations have not been performed yet. And when it comes to the ASL issue, the school is simply ignoring it altogether. They are continuously trying to bypass the issue and don’t want to make a decision on an appropriate educational placement. At the same time however, they also are not willing to write a letter of refusal which is what they legally have to do if they disagree with my placement request for Wyatt to attend a Total Communication Program (ASL and English are used simultaneously). The thing that neither I nor my IEP Advocate understand is why they are ignoring all the medical documentation in Wyatt’s file that supports such placement. They are scared of liability because of his cyanotic breath holding episodes yet they are refusing to accept that ASL has been the only effective communication mode that has reduced the number of daily episodes. He has medical clearance to attend school. It is also documented in writing that he used to have 15-20 episodes before using ASL which has been reduced to 1-5 daily episodes with the use of ASL. I even showed them a video to show them what exactly a cyanotic breath holding episode looks like when the communication barrier becomes a problem. Every medical documentation on file, including a private physical therapy evaluation, clearly state that ASL is his primary mode of communication and it needs to be supported to prevent these episodes as much as possible. It is also documented in the school district’s speech and language evaluation back from September that Wyatt is non-verbal and uses ASL. The same is documented on his private speech evaluation with detailed ASL therapy goals yet the school is not willing to even write down any ASL goals because they say they don’t know how many words he understands and can signs. That is a straight out lie as the district has had that detailed list of vocabulary words in their records since their original evaluation back in September. Additionally, they have a list provided by me detailing 4 pages worth of vocabulary in the form of the curriculum we use at home proving that he is far past basic vocabulary, plus I showed them videos of him signing. Educational goals in IEPs, such a recognizing 5 out 10 letters, are established without ever first testing what the child knows. Therefore their argument about needing a different approach to set ASL goals is false. This is not a personal preference, this is a medical necessity to prevent these life-threatening episodes and the school is not even willing to acknowledge that fact. After 3.5hours of this dance we made it very clear to them that a decision had to be made that day. The school only had two options. They could either write a letter of refusal and then we will file due process, which is a fancy term for taking the matter above their heads, or they can get that illusive district person who according to them has authority to decide this matter on the phone and settle this. Side note, legally the IEP team has the authority to make placement decisions even when it means the most appropriate setting for a child is in a different school. Worried we will file due process, the school asked for another meeting scheduled for next week that will include that illusive district person who according to them has authority to decide this matter, plus members of the staff from the Total Communication school to better assess the ASL issue. Our objections were clearly noted that bringing someone new to the table who has no knowledge of what has been going on for the past 7 months is inappropriate as it will start this process all over again. Given the currents team indecisiveness however, we agreed to play along knowing that the next meeting will most likely also be inconclusive and we will file a complaint with the state regardless of that outcome. The only two positive things that came out of that (in the end 4 hour long) meeting was that the district has nursing all lined up and is ready to go on that front, and we have a new district speech therapist involved now that has worked in the Total Communication Program before, is fluent in ASL and is supportive of any and all methods of communication. Therefore, we have asked for another district language evaluation, different from the previous one, that will specifically evaluate Wyatt’s comprehension and use of ASL.

All this just makes me angry. Why would a school district even hire someone to be an IEP compliance person when on a personality level they are indecisive? That in itself is contradictive to their job duties. But what really fuels the fire for me is that this ASL issue has been on table since the beginning. We didn’t suddenly overrun them with the placement request yesterday. ASL being my son’s primary mode of communication is in all of his records even as far back as when he was in the Early Steps program before age 3. The school has all the documentation in front of them and the Total Communication placement request has been provided to them well ahead of time in writing. At the bare minimum, they should have acknowledged that request and already had the appropriate decision making people attend yesterday’s meeting. Instead, it was very apparent that this topic was not supposed to be discusses, much less supported. It was very apparent that this continues to be a pass the bucket game in the hopes that the longer this gets pushed out the more likely I will eventually either walk away completely or give up and go along with whatever the school is comfortable with. It didn’t seem to matter to them that even the IEP Advocate made it clear that the handling of my son’s case has been inappropriate since the very beginning and that we have every reason to turn this into a legal matter. It makes me angry when I get the condescending “but you don’t really want him placed with the deaf and hard of hearing kids, do you?” AS if that is a jail sentence or something. Yes, I do want him placed there! His hearing might be fine but he is non-verbal, uses ASL as his primary mode of communication and therefore the appropriate and least inhibiting placement for him is that of the deaf and hard of hearing program. In his case, it is not a decision based on personal preference, it is medical necessity. Any attempt to place my son in a setting where his mode of communication is not supported is considered negligence as it is clearly documented that such placement would cause severe increased medical risk.

I have a favor to ask. My youngest son has a rare life-threatening condition called CCHS which makes him ventilator dependent during drowsiness, sleep and illness, among other medical obstacles. To try to raise awareness and hopefully increase research funding, one family of a child with CCHS has produced a movie ( "Our Curse") detailing our daily struggles and this movie has now been Oscar-nominated. PLEASE SHARE TO SPREAD AWARENESS! The more people that watch this and share it, the higher the chance of an Oscar win and therefore, the higher the chance of research money for this very rare condition!
http://mobile.nytimes.com/2015/02/02/opinion/our-curse.html?smid=nytcore-iphone-share&smprod=nytcore-iphone&_r=2&referrer

Maybe the CCHS lifestye really can get easier

I think I may have just gotten a glimpse of that mysterious light at the end of the tunnel. 3 ½ years of living with CCHS and fighting to get my son the help he needs and I think we might finally be making progress finding outside help. My frustration with the school district IEP team have led me to take an even more aggressive approach, as if that were even humanly possible. After many tears and sleepless nights, more so than usual when you sleep to the sound of a ventilator every night always ready to be in full emergency mode at any given time, I decided if the school IEP team is not willing to step out of their comfort zone and admit Wyatt needs more services than what they offered so far, then I will bombard them with private evaluations from every specialist and therapy service available until they realize, getting rid of this crazy mom is impossible. So two weeks ago I put myself and Wyatt through yet another depressing speech evaluation but this time particularly focused on his need for ASL instruction and his problems with fine finger movements to improve the accuracy of that mode of communication. The result, nonverbal with severe to profound expressive and receptive speech and language delay and a recommendation for Occupational Therapy along with Speech Therapy to improve and expand his ASL communication. A week ago, Wyatt also had a Physical Therapy evaluation done to address his balance and coordination issues to specifically point out the safety concern of constant falls. During that evaluation, the Physical Therapist also got a glimpse of Wyatt’s cyanotic breath holding abilities and in bold letters wrote in her report the need for behavioral support services. (Bonus points.) Legally the school district now has to also take into consideration these private evaluations when reevaluating Wyatt’s IEP services and goals. Since I am out of patience for this pass the bucket school district game though I went one step further and hired an IEP advocate the write up all the paperwork with the appropriate legal terms and to help represent Wyatt’s case at the next IEP meeting. Hopefully then the school will understand that I’m not willing to waste any more time with politics, it’s been 7 months now of trying to get him to start school, and that I’m serious about my request for an “appropriate” placement. Simply sticking the kid in a self-contained Pre-K ESE classroom with a one on one nurse by his side and a mediocre speech therapist who isn’t willing to expand her own horizons beyond picture communication techniques simply isn’t good enough. The reality is, Wyatt may never talk and even if he does, it may be years before he can form an understandable complete sentence. And even then, with his medical diagnosis, there will always be times when he is hooked up to the ventilator, his trach is inflated and he physically can’t speak because no air can pass by his vocal cords with an inflated trach cuff. Therefore, ASL is and always will be his failsafe primary mode of communication. And it is for that reason that the appropriate placement for my son is in a total communication program, where ASL and English are used simultaneously, where he cannot just be understood by an interpreter but also communicate back and forth with all staff and students around him and expand his ASL skills. Only then will he have the best opportunity to thrive, to learn uninhibited, and to gain an understanding of social norms… But that’s a speech for the next IEP meeting next Friday. Rant over… What made me see the light at the end of the tunnel was actually thanks to Wyatt’s Physical Therapist. She went above and beyond her job description and asked a Psychologist at the hospital to join us during Wyatt’s first PT session so the Psychologist could talk to me directly about the behavioral issues regarding the cyanotic breath holding episodes and to see what she can do to get us help in that area. That seemingly simply 30minute Physical Therapy session turned into a pre-evaluation session with a Clinical Pediatric Psychologist who then managed to get Wyatt scheduled for a complete Neuropsych evaluation. It usually takes months to break into that specialty and with minimal staff available in that department and an overload of patients waiting for services, I had a hard time holding back tears when that Psychologist walked in the door during Wyatt’s PT session. Finally here was someone who was willing to listen, who understood that this was not just a medical or not just a behavioral issue but rather that these two factors were connected and could not be completely separated. She could help us start to manage these episodes better from a behavioral standpoint to maximize Wyatt’s coping skills while minimizing the medical impact, instead of simply reaching for medication to numb this kid’s consciousness. This upcoming Neuropsych evaluation will hopefully not only give us as the parents a new reference to work with to help Wyatt reach his full potential but it can also open doors with the school district. I am forever grateful for that Physical Therapist and that Psychologist for reaching out and going beyond what is expected of them and looking at the needs of the whole child (and his parents).

Thank you!

I recently received a thank you message from a parent who’s child has just been diagnosed with CCHS. It’s humbling to read such a message when in my mind I originally started this blog simply as my own private therapy session. Getting thrown into this CCHS lifestyle, as I can now jokingly refer to it, can certainly mess with anyone’s sanity and none of it comes easy or naturally. It’s a huge learning curve that no one would voluntarily choose to go through and each year the battle simply continues. Receiving that thank you message couldn’t have come at a better time either. We’re in the midst of yet another big hurdle and hearing that my honest, straight forward, raw emotions approach of detailing our journey has helped another family going through similar struggles, well that just makes this whole “lifestyle” a little less scary, a little less lonely and makes my writing about it all a little more rewarding.

So I want to thank this parent for reaching out to me, unknowingly giving me a little more strength to continue this battle, and for encouraging me to keep writing about it all, not just for my own sanity but also to help guide other parents along the way. Thank you for reminding me that writing and sharing experiences is important!

Cardiac Issues in Congenital Central Hypoventilation Syndrome (CCHS)

Cardiac Issues in Congenital Central Hypoventilation Syndrome (CCHS)

(Summary as it relates to Wyatt, 20/33 PHOX2B genotype/mutation)

·         Abstracts from 2008 study on 20/25, 20/26 and 20/27 PHOX2B genotype/mutation http://www.cchsnetwork.org/images/stories/PDF/literature/CCHS_Cardiac_Issues.pdf

 

“Minimum heart rate varied by genotype (w2 2df ¼ 6:89, P¼0.03), with lowest values obtained for the subjects with the 20/27 genotype (genotype 20/25 vs. 20/27 comparison P¼0.02, 20/25 vs. 20/26: P¼0.07, 20/26 vs. 20/27: P¼0.2).”

 

“The longest r-r interval was found to be independent of the baseline heart rate as well as the time of day.”

 

“Though longest QTc interval did not vary by genotype, all children with CCHS had at least one Holter with a QTc interval above 450 msec, and the percent of QTc above 450 msec was substantial considering the overall rarity of QTc greater than 440 msec in normal children.”

 

“Though some children may demonstrate staring spells or syncope at the time of the transient asystole, most subjects are asymptomatic. This underlying propensity for prolonged r-r interval may increase the vulnerability of the child with CCHS when exposed to pharmacologic agents or anesthesia.”

 

“The reticence to implant a cardiac pacemaker may be the result of uncertainty as to the clinical significance of prolonged r-r intervals in the seemingly asymptomatic subject, particularly if subsequent r-r intervals are shorter than 3 sec. Because the impact of prolonged sinus pauses on long term neurodevelopment is unknown, and because childhood is such a critical period for neurocognitive development coupled with the overriding risk for sudden death, recommendation for implantation of a cardiac pacemaker in the child with CCHS and r-r intervals of 3 sec or longer seems to be the most conservative recommendation.”

 

·         Abstracts from the 2010 American Thoracic Society, Clinical Policy Statement: Congenital Central Hypoventilation Syndrome http://www.cchsnetwork.org/images/stories/PDF/literature/CCHS-ATS.pdf

 

“Cardiac rhythm abnormalities, including decreased beat-to-beat heart rate variability, reduced respiratory sinus arrhythmia, and transient abrupt asystoles, have been described (9, 101, 102). Seventy-two–hour Holter monitoring performed annually may determine aberrant cardiac rhythms, sinus pauses that will necessitate bipolar cardiac pacemaker implantation (103), and the frequency of shorter pauses (i.e., less than 3 s) that may have physiologic and neurocognitive impact.”

 

·         Statement on Cardiac Pacing in CCHS written by Dr. Tom Keens

http://www.cchsnetwork.org/images/stories/PDF/literature/cchs%20cardiac%20pacing%20keens%203_13.pdf

Pre-K battles

OPINIONS PLEASE:

Our IEP meeting was Friday. It was less of a battle than anticipated but also did not go exactly I had hoped. Wyatt is approved for the full pre-K program from 8-3 at our local Elementary School (where I used to work before he was born) in a self contained ESE classroom with Speech Therapy 2x30min weekly. While the Speech Therapist does not know ASL and didn’t generally seem very supportive of it, she would teach picture communication as an alternative which is also the main communication method used in this pre-K classroom. On a positive note though, the pre-K teacher does know basic ASL and offered to brush up on her skills if I give her a list of vocabulary words we use at home. I think personality wise the pre-K teacher would be a good match for Wyatt and I walked out of the meeting thinking that this could actually work. Basically, they focus on academics and social skills from 8-12, then lunch and nap before going home at 3, all in the same classroom. The issue comes in with the nursing. The district nurse attended the meeting to ask questions and take notes about his condition and to then forward that information to the person in charge of hiring a one on one nurse for Wyatt. What bugged me was that she dodged some of my questions and just tried to reassure me that the nurse they assign to us would be trach and vent trained and we'd meet him/her first. In the CCHS world, with the nurses we’ve been through with a private duty nursing company, just those basic qualifications don’t mean a whole lot. When I asked specifically how we would train a nurse on a condition that no one knows much about she tried to downplay it as he's essentially just a kid on a vent with a trach. I made it clear that I don’t trust nurses because there had been too many who couldn’t handle Wyatt’s blue spells and I made sure everyone in the room understood that the nursing issue is the deciding factor, that we'd be the ones making the decision whether the nurse they pick is a good fit. That part of the meeting didn’t lead to any solutions because the district nurse is not the person who actually hires the other nurses. So I'll have resume that conversation with the person in charge of hiring and training. I did agree with all the IEP goals set and methods used (and we will continue to pursue ASL at home), a one on one nurse and health care plan clearly stated on the IEP. So as of right now, I signed the IEP and submitted registration papers with the school. But nothing can happen until nursing is actually in place. As far as dealing with his daily blue spells, the school is giving us the wiggle room that they would not have to call 911 every time if we have specific doctor ordered perimeters in place that specify when to simply call me vs when to call 911. So that part is definitely good news.

Long story short, here’s my dilemma I’d like some input on. After the meeting I felt like there is a glimpse of hope at normalcy, that this school group setting might help him progress faster and I’d have a few hours of the day to simply focus on my other kids. (Granted I will complain about school traffic and how it will make scheduling appointments harder.) But now that I had time to think about everything over and over again, the fear of health risk vs developmental benefit is creeping in. There’ll be 8 other 3 year olds running around with varying developmental delays, mostly speech but who know what other behavior issue are thrown in the mix. 3 years olds are horrible at basic hygiene and you know there is at least one kid running around with a snot nose every day. Wyatt has always handled colds well but I’m scared out of my mind that it’ll lead to a trach infection. He’s only ever had 2 trach infections in his life but when he had them he was in the ICU completely lifeless. My husband says he could get sick anywhere so it’s a daily risk no matter what we do, he could pick something up at the library or playground just as easily but that reasoning doesn’t make this decision any easier. Then there is the issue of the nursing. In my ideal world, we’d pick the nurse. Practical skills can be taught but it has to be a personality match for this whole scenario to work, for Wyatt to respond well and for us to trust a nurse again. That nurse can’t be uptight about protocols and paperwork and he/she also can’t overreact when Wyatt throws a blue spell. Assuming we can find someone with that personality, how do we train them? I doubt the school district will let me decide how the training should be done. So is it possible to have it be part of the doctor’s order that any nurse who is to take care of Wyatt has to first train with us for one week at home? What perimeters should there be when to “ignore” a blue spell, when to call us vs when to call 911? How often should the nurse do SpO2 and etCO2 spot checks while in school, at least until she learns to read Wyatt’s behavior better? (We don’t do any spot checks at home unless he’s acting strange.) Should he even be on etCO2 monitoring while napping in school? We don’t hook him up to it at home during naps, only at night when warranted but we also can judge his behavior far better to know when things are slightly off… This is so much to wrap my head around. I do want to give it at least a 3 month trial run to see if this set up can work not just for Wyatt but for our whole family but fear is starting to get the best of me. Any practical suggestions?

IEP evaluation frustration

Venting, that’s the reason I originally started this blog. To set the thousands of thoughts running through my head free and hopefully end up with some sort of clarification or relief. So here we go.

Today was Wyatt’s IEP evaluation. In Osceola County it’s called the PEEP program which is just a fancy name for the school district’s early intervention program that the child can transition into once they turn 3 years old. Wyatt had a few speech therapy sessions here and there through the Early Steps program, which is for ages 0-3, but they were never able to find him a speech therapist who was fluent in ASL and at the time, Wyatt also wasn’t very receptive to therapy sessions. He simply passed out when he didn’t want to participate. So here we are 3 years on this journey and we have to put him through yet another developmental evaluation. Just for the record, I hate those evaluation! Yes, HATE them with a passion! The questions are very generalized and don’t leave room for more elaborate answers. It’s mostly a score of 0-4 on how well he performs a task and the parent has to answer a lot of yes and no questions regarding his abilities compared to what a “normal” 3 year old should be able to do. I have several issues with this type of testing. For starters, the questions do not differentiate between communication vs. speaking. That’s a big deal for a child who is non-verbal. Just because Wyatt does not speak that does not mean that he is not communicating. Therefore, a simple yes or no option for an answer is not sufficient. That’s a big issue and can falsely alter his score. Second, halfway through the evaluation Wyatt lost his patience so cooperation at that point was a problem. There were definitely some tasks that I know he can do but he simply didn’t feel like doing them at that time. The darn evaluation did take 2 hours to complete and what 3 year old has patience for that. And then there were questions that I didn’t even know how to answer. I was asked to specify the different sounds he makes. I don’t freaking know, they are just sounds. There are no actual words and the sounds are not specific vowels that fit nicely into the printed test booklet criteria. But there is a difference in pitch depending on the situation. Those type of things can’t be explained to an evaluator who has never met Wyatt and will probably never see him again. So the whole time I felt like we’re portraying him as this mentally retarded kid when in reality the things he will and will not do primarily just depend on his mood and comfort level with the person who is asking him to do these things on command.  I did request an ASL interpreter to be present during this evaluation to see for myself how well he can be understood by someone who is fluent in ASL since that is his primary mode of  communication. While this was very valuable information for me, it was also very depressing. The interpreter was only able to understand 3 signs Wyatt used. His signs are still too modified and would not be understood out of context. So if he was asked to look at a picture and point to the ball and would then sign the word ball, the interpreter could understand. But if he did it out of context it would have been misunderstood because his fingers are not pointing the right way and his wrists touched. So that information was very important for me to know but it was also very frustrating at the same time. It actually makes me very angry because I have been practically begging for help with ASL instruction and have been ignored for the last 3 years. Everything he knows at this point, though it may only be understood by immediate family, was because of the effort we put in. We’ve have been working really hard to get him to where he is today, to allow him a form of communication that cuts down on his frustration level and therefore his tendency to pass out. But based on this evaluation, outside of immediate family, Wyatt basically has the communication skills of an infant. He taps a person’s leg to get their attention, he point at things he wants and he uses facial expressions to show emotions. As far as getting services approved for him I could look at this result as favorable. Having the worst case scenario on paper sure is helpful. But as a parent, it’s devastating to look at such a comparison. All the hard work we’ve put in over the last 3 years seems like nothing on paper when compared to a “normal” 3 year old. Wyatt has come such a long way and we have made huge progress in our eyes but that progress is so small in the context of a developmental evaluation. I almost wish I wouldn’t have put him through this comparison again. I have such a hard time coping with this information. It took me a very long time to accept who he is and to stop comparing him to the “normal” 3 year old. On a daily basis, I can now happily look at who he is as a person and enjoy every little accomplishment. But when we have to jump through the necessary hoops to get him outside help it’s like a slap in the face. That’s why I HATE this entire process. I don’t ever want him to feel like he’s not good enough. I know he will do all those things eventually in his own time. I hate being pushed into the mindset of having to fit some sort of norm. But as a parent of a medically challenged child those are the uncomfortable steps in the process you have to put up with if you have any hope of getting outside help. Simply put, IT SUCKS!  So, this evaluator will now type up his report and then it gets sent off to a bunch of other people up the ladder to decide what services he qualifies for. That can be anything from nothing at all, to just speech therapy at the local elementary school, to a full school day with all therapy services included. But for that decision to be made could take another 2 months as the school psychologist who administered the assessment warned me. When that meeting is schedule the real battle will begin as to what the school district will offer vs. what we want. It’s very frustrating to fight so hard to simply get outside support. It’s been 3 year and so far this fight has gotten us absolutely nowhere. I’d rather just know once and for all that we’re on our own, as we have been so far, than continuously jumping through these hoops without any results.