Segev

From PWS Notes
Revision as of 07:04, 30 June 2019 by MulaLiron (Talk | contribs)

Jump to: navigation, search

Segev


Contents

General #1 (12/2018)

Segev was born at 11/06/2018 preterm, 35+5 weeks. He needed inhaling for 2 weeks, and assisted oxygen for additional 2 weeks Segev was hospitalized at the NICU for 120 days, and was released when he managed to consume about 60 kcal/kg from the bottle. I found about this website when segev was about 2 month, and I wanted to start treating Segev according to the recommendations you can find here, the NICU team wasn't cooperative about supplementing, so we only stated those when he was released from the NICU. Segev started GH at about 2 month, and we didn't see any immediate impact. His formula was changed to Infatrini, which contains MCT, at about 3 month. we saw a change in alertness (moderate one) within 2 days. Segev was released from NICU at percentile 5 (length and weight). Once we went out of NICU we started supplementing with additional MCT, B12 complex, Omega-3, carnitine fumarate, acetyl carnitine, whey, lactoferrin, probiotics. next paragraphs will contain more detailed information. Segev climbed percentiles slowly. By 6 month he was hospitalized twice due to sickness due to the PWS malfunctioning immune system. One of the most interesting things I learned about during this 1st half year, is the Histamine H3R inverse-agonist for PWS hypothesis. I think it is a must read if you're already here.

12/2018 : 1-6 month

summary of the things I remember from the first half year Segev started physiotherapy at ~1 month, at the NICU. First once a week, than every day, than twice a day (we did it, supported by the team). Started feeding therapy at ~2 month, at the beginning he didn't consume a drop, it took about a month to go to 15 ml/feeding and another month to go to 40 ml/feeding (30min x6 feedings/day) We put music in his NICU crib (mostly romantic era) and played it a few hours a day. feeding is really hard, it takes a lot of time and Segev doesn't eat much. The policy in Israel is not to let a baby go home with NG-tube, and we refused gastrostome.

01/2019 : 7 month

Segev is 7 month, 7.5[Kg] and 68[cm]. This is about 20% percentile for both length and weight. Milestone wise: good eye tracking, occasionally flips from facing down to facing up. head-lag is present but mild Nutrition guideline: we aim for MKD (60/25/15 fat/carb/protein) and will gradually shift the macronutrients. Nutrition wise: 5-times 90cc infatrini formula, fortified with creatine-monohydrate (1g/day), carnitine-fumarate (750 mg/day), acetyl-l-carnitine (250 mg/day), chia-powder (~1 g/feeding, for thickening) we had a phase of adding whey powder, which we stopped once we started adding chia-powder, as it has (enough) protein.

02/2019 : 8 month

Segev is 8.5 month, 7.85[Kg] and 68.7[cm]. This is about 15% percentile Following the h3r hypothesis, and the connection between brain-histamine and water intake, we started kutaj, which is an ayurvedic herb, given to infants for many years. Kutaj contains conessine, a molecule which acts as a h3r-inverse-agonist. The ayurvedic medicine uses kutaj to stop diarrhea, which will be useful as well. I started kutaj at 50 mg/day for a few days, and then added to ~300 mg/day. Segev started eating from the bottle a couple of days after starting the 300 mg/day dose. At the 1st couple of days he was eating well only a couple of hours after kutaj dose, and not eating well much later. After another couple of days he was eating from bottle willingly 5 times x 80-100 cc per feeding. I know turning from bottle-refusing (i.e. waving hands to remove the bottle, pushing the bottle with tongue, choking on formula) to bottle-loving (pulling formula from bottle so you can hear the sucking action) in a couple of days duration also happened to other PWS (search in the 'living well with PWS group') and I also will never know what would happen if I didn't administer Kutaj, but I think that the end result is OK.

Still on 02/2019, We ran out of Lactoferrin, and Segev became congested 3 days later. It can be a coincidence.

04/2019 : 10 month

Segev is 10 month, 8[Kg] and 70[cm], still on the 15% curve. We also had an argue with our endo about supplementing and diet type (he was ~60% fat, and she insisted to shift to carb based diet). We reduced some of the fat (now 45%-50%) and now are looking for local nutritionists to support us. It is complicated to balance the different opinions. 3 weeks ago we stopped carnitine and kutaja supplementation, because their taste is really bad and Segev didn't like them. Segev started congestion a few days later, and was sick (some fever) for 2 weeks. we took him to the ICU to get tested but were released after a few hours.

In addition, I think he eats worse, meaning his bottle feeding take longer, and sometimes he don't want to bottle feed. it can be teething and it can be the kutaja, we don't know yet.

at 10 month Segev can flip back to tummy and other side, he can pivot a little, he knows to look towards the light when we ask him.

05/2019 : 11 month

Segev was 'sick' for about 3 weeks. he had a lot of running nose, had hard breathing and a lot of snoring at night. he didn't have fever, but he was weaker and more tired. I think he didn't learn a lot in those weeks, he didn't progress motor-wise.

We re-started carnitine (carnitine in the morning bottle, goes well with vanilla extract drops) and kutaja (mixed with 5[g] of 85%-dark-chocolate-spread and milk) Segev got better after a couple of days, no more running nose, better sleep (we count how many times he is awake at night, using a camera) and he has more strength. Segev started crawling, slowly at beginning, and after 2 weeks he can crawl a couple of meters before tired. Segev also went from belly to knees a couple of times, and maintain there for a few seconds. His feeding also improved (not-so-difficult to feed him, not easy though).

Segev was evaluated at the Israeli PWS clinic, the PWS neuro said she is surprised about his progress given that he is PWS and given he was NICU for 4 month + hospitalized so much. His measurements were 8.3[kg] and 72[cm] which still puts him around 15% percentile.

07/2019 : 12 month

I feel that the past week was good.
eating - improved bottle feeding. it takes ~20 minutes and he finishes most of the volume (we prepare 160cc 4 times/day)
sleeping - I'm not sure, we have a camera above his bed, I think most nights his sleep is fragmented, and he is awake (moving, head up) for 1 minute every 30 minutes. yet there are nights where he doesn't move for 90 minutes.
moving - army crawling improved, pivoting is very good, he can sit for a minute but can't go from lying to sitting. this happened once. he can stand if he's holding to something (chair) or when holding his hands, for a minute or so.
voice - he started babbling a lot a couple of days ago, potentially after introducing ashwagandha
alertness - he is alert most of the day, sleeps twice a day. a few month ago he would go still if not entertained, but now he will keep 'playing' (messing with toys) for a long time when left alone. his base position is on the back with all 4 limbs in the air.
responsiveness - the last week he became much more responsive. it is much easier to make him laugh, by making faces or voices.
supplements/day -
carnitine fumarate 500mg, carnitine acl 250mg, coq10 60mg, b12 cyanocobalamin 1/3 dropper (all for energy metabolism)
omega3 ~1000 dha+epa (brain development)
probiotics - lactobacillus reuteri, bacillus natto (oxytocin)
histamine - kutaja 1/2 tsp (h3 inverse antagonism) bdnf - ashwagandha 1/2 tsp