No surprise here: February 2013

Down Syndrome - Reading (alphabet) and Math (number recognition)

My son is a strong visual learner, so colorful pictures and videos work well for him.

Reading: as mentioned in a previous post we are teaching my son whole words reading (sight words). However, at the same time we are working on letter recognition to get him ready for phonics and phonetic reading. We are focusing on letter sounds, rather than letter names, as the sounds will be way more useful for reading.

By far, for us, the best DVD for teaching letters was Leap Frog "Letter Factory" which we initially got from our local library, but worked so well that we decided to buy it. Another good one is Signing time "ABC" episode, especially if your kid already knows and loves Signing Time series. BTW, Leap Frog "Let's go to school" is a great video to get your kids ready of their first day of kindergarten.

Math: Teaching numbers from 1 to 20: Sesame Street "The great numbers game". Teaching numbers from 1 to 100: Scholastic Emily's first 100 days of school. We are now working on number recognition from 1 to 100, so we've posted on our fridge this numbers chart. Cheap and it does the job.

Down syndrome apnea - CPAP or no CPAP

At this point my son has mild apnea, with no symptoms. He sleeps well, has no behavioral issues, good developmental progress. Sleep apnea is a very serious condition that needs to be addressed aggressively, I totally believe that. For many CPAP is the best and only solution. However, we were offered CPAP and refused. Again I'm not a doctor (just a parent obsessing over issues), but I'm worried about long term use of CPAP in children (maybe even adults) with DS. The best I can explain it is this: my son has some degree of facial hypotonia. CPAP is a device fitted snugly on the face - basically pushing on the muscle, bones, etc. And, because of poor muscle tone, nothing pushes back. I'm afraid that long term this will have an impact on his teeth, on face bones, on skull.

And just to show you this is not a crazy idea.

Craniofacial changes after 2 years of nasal continuous positive airway pressure use in patients with obstructive sleep apnea.

Journal: Chest
Article published: 2010 Oct;138(4):870-4.

Tsuda H, Almeida FR, Tsuda T, Moritsuchi Y, Lowe AA

Abstract

BACKGROUND: Many patients with obstructive sleep apnea (OSA) use nasal continuous positive airway pressure (nCPAP) as a first-line therapy. Previous studies have reported midfacial hypoplasia in children using nCPAP. The aim of this study is to assess the craniofacial changes in adult subjects with OSA after nCPAP use.

METHODS: Forty-six Japanese subjects who used nCPAP for a minimum of 2 years had both a baseline and a follow-up cephalometric radiograph taken. These two radiographs were analyzed, and changes in craniofacial structures were assessed. The cephalometric measurements evaluated were related to face height, interarch relationship, and tooth position.

RESULTS: Most of the patients with OSA were men (89.1%), and the mean baseline values for age, BMI, and apnea-hypopnea index (AHI) were 56.3 ± 13.4 years, 26.8 ± 5.6 kg/m(2), and 42.0 ± 18.6/h. The average duration of nCPAP use was 35.0 ± 6.7 months. After nCPAP use, cephalometric variables demonstrated a significant retrusion of the anterior maxilla, a decrease in maxillary-mandibular discrepancy, a setback of the supramentale and chin positions, a retroclination of maxillary incisors, and a decrease of convexity. However, significant correlations between the craniofacial changes, demographic variables, or the duration of nCPAP use could not be identified. None of the patients self-reported any permanent change of occlusion or facial profile.

CONCLUSION: The use of an nCPAP machine for > 2 years may change craniofacial form by reducing maxillary and mandibular prominence and/or by altering the relationship between the dental arches.

Down syndrome apnea - sleep study 2

Six months after tonsillectomy and adenoidectomy my son was scheduled for a second sleep study. In children with DS is not uncommon for apnea to persist after T&A.

We had our second sleep study in February 2012.

So, the results of our second sleep study. Apnea-hypopnea index went down from 7 to 2.7 per hour. Good, but this is still apnea. The REM got way up. This is great. At this point we were given the option of waiting and monitoring the apnea, or considering CPAP (continuous positive airway pressure). We politely refused CPAP and are scheduled for the third sleep study soon.

Total Recording Time (TRT): 509.8min.

Total Sleep Time (TST): 404.5min.

Sleep Efficiency Index (TST/TRT): 79.5 %

Sleep Latency: 15.5min.

REM Latency: 198.5min.

Wake after Sleep Onset: 88.5min.

Total Arousals: 101

Arousal Index: 15.0/hour

Sleep Stages

Stage 1 Minutes 69.0 %TST 17.1 Normal %TST 5-10

Stage 2 Minutes 136.0 %TST 33.6 Normal %TST 45-55

Stage 3,4 Minutes 70.5 %TST 17.4 Normal %TST 10-15

Stage REM Minutes 129.0 %TST 31.9 Normal %TST 20-25

Respiratory Events

Obstructive Apnea number 0

Obstructive Hypopnea number 17, average duration (sec) 10.6

Mixed Apnea number 0

Central Apnea number 1

Apnea-Hypopnea Index : 2.7/hour

Respiratory Effort Related Arousals (RERA): 16

RespiratOry Disturbance Index «apneas+hypopneas+RERA)/TST): 5.0/hour

Oxygen saturation awake: 97%

Nadir oxygen saturation during sleep: 84%

End-Tidal C02 awake: 39.6

End-Tidal C02 asleep peak: 48.7

Percentage of time with End-Tidal C02> 52mmHg: 0.0

Periodic Leg Movements:

Periodic Limb Movements with arousals: 0

Periodic Limb Movements : 0

Electrocardiographic Monitoring: Average heartrateasleep: 87

Down syndrome apnea - tonsillectomy and adenoidectomy

Two weeks after our sleep study we were scheduled for a T&A (August 2011). In preparation for the surgery we had a cervical XRay to check the atlanto-dens interval. It was within normal limits. It is really important for the anesthesiologist to know if it's safe to extend the neck during the surgery and to be on alert for possible complications due to the muscle relaxing effects of anesthesia. Seriously, there was an article published about two weeks ago about a 7 years old boy (did not have DS) who had undiagnosticated atlanto-axial instability and who developed quadriplegia after an elective surgery to remove his tonsils and adenoids. We were told by our ENT and the anesthesiologist that even with the normal results, they will still be very careful and will operate with the neck in neutral position.

Our surgery was short and painless. The recovery was everything but. We spend the night in the hospital, which is standard procedure for children with documented apnea - they have too much air all of the sudden and apparently that's not good either. The insurance did not want to approve the stay, but our ENT (which we love) send them a bunch of literature and convinced them.

So, about the recovery. The pain lasts 10 days. It's not like there is a lot of pain in the first days and then is gets better and better. No, the pain lasts 10 days. I highly recommend around the clock medication - we alternated ibuprofen and Tylenol every 3 hours. I know that some surgeons do not recommend ibuprofen for fear of bleeding, likely ours did - Tylenol alone just doesn't cut it. Don't worry if and what your child eats. He needs to drink. Ice cream was our friend. A really nice tip we got from the blog below was to use Tylenol suppositories during the night. It really helps if you do not have to wake the child. Remember, around the clock means around the clock - we set the alarm in the middle of the night.

After the recovery was over - sleep improved a lot. No more waking in the middle of the night. Tongue protrusion which is an ongoing issue for us, got so much better, almost immediately after surgery.

This blog is the best source of mom advice post T&A. Great tips.

Down syndrome apnea - sleep study 1

We are very lucky that my son is very healthy and he doesn't have the problems associated with DS. Except for sleep apnea. It was always an issue. He had apnea of prematurity at birth. For the longest time every time we mentioned to our pediatrician that something is off with my son's sleep our worries were kind of brushed off. Finally, when my son was 3 years old, we had our first sleep study. It is not a pleasant experience - not for the child and not for the parent. It turned out that my son had moderate apnea. In terms of his symptoms - he did not snore, he was not tired during the day, he was sleeping in a weird position with the neck super-extended, and he was waking a lot during the night.

We had our first sleep study in August 2011.

So, the results of our first sleep study. I'm not a doctor, but apnea-hypopnea index 7/hour is not good (most doctors think 1 is too much in children). Also, the sleep pattern is completely off. He spends 4.5% of this sleep time in REM compared to 20-25% normal. This is the stage when he dreams, when his mind is supposed to really relax, when everything he learned gets stored in long term memory (or something like this) - it's really important. Conclusion - tonsillectomy and adenoidectomy, or T&A.

Total Recording Time (TRT): 518.8min.

Total Sleep Time (TST): 422.5min.

Sleep Efficiency Index (TST/TRT): 81.8 %

Sleep Latency: 7.5min.

REM Latency: 212.5min.

Wake after Sleep Onset: 86.5min.

Total Arousals: 88

Arousal Index: 12.5/hour

Sleep Stages

Stage 1 Minutes 54.5 %TST 12.9 Normal %TST 5-10

Stage 2 Minutes 246.0 %TST 58.2 Normal %TST 45-55

Stage 3,4 Minutes 103.0 %TST 24.4 Normal %TST 10-15

Stage REM Minutes 19.0 %TST 4.5 Normal %TST 20-25

Respiratory Events

Obstructive Apnea number 2, average duration (sec) 10.5

Obstructive Hypopnea number 47, average duration (sec) 12.1

Mixed Apnea number 0

Central Apnea number 0

Apnea-Hypopnea Index : 7.0/hour

Respiratory Effort Related Arousals (RERA): 19

RespiratOry Disturbance Index «apneas+hypopneas+RERA)/TST): 9.7/hour

Oxygen saturation awake: 93%

Nadir oxygen saturation during sleep: 83%

End-Tidal C02 awake: 33.0

End-Tidal C02 asleep peak: 50.1