It's a big deal, a very big deal when it comes to babies. I think in Ellie's first year of life, I must have heard the word tone mentioned a billion times.
You see, Ellie as well as all other individuals with PWS have low muscle tone (hypotonia). Being a first time mother, and not having been around a ton of babies, I had no clue what the doctors meant when they said she had low muscle tone. She was a newborn, they weren't supposed to hold their head up, right?!
As we spent more time in the NICU I asked more questions, and could finally grasp the tone issue.
PWS has two main stages that are talked about most frequently:
Stage 1 - hypotonic, "floppy babies" - failure to thrive
Stage 2 - insatiable appetite, lack hunger and satiety cues
can overeat to the point of danger/death if not closely monitored
And...seven different nutritional phases as defined by Miller et al [2011]
| Phase | Median Ages | Clinical Characteristics |
|---|---|---|
| 0 | Prenatal - birth | Decreased fetal movements & lower birth weight than sibs |
| 1a | 0-9 months | Hypotonia with difficulty feeding & decreased appetite |
| 1b | 9-25 months | Improved feeding & appetite; growing appropriately |
| 2a | 2.1-4.5 years | Weight increasing without appetite increase or excess calories |
| 2b | 4.5-8 years | Increased appetite & calories, but can feel full |
| 3 | 8 years - adulthood | Hyperphagic, rarely feels full |
| 4 | Adulthood | Appetite is no longer insatiable for some |
While in the NICU, David and I also learned a great deal about feeding infants. In order to nurse, babies have to have a strong suck, stamina, energy, tone, and coordinate all this. It truly is hard work! Ellie wanted nothing to do with nursing or bottle drinking. Once she was admitted to the NICU, they placed an NG tube in her nose that went to her belly, and she was "tube" fed every 2-3 hours. I was so, so nervous about everything in the NICU...the cords, monitors, sounds, and tubes. David and I would stare at Ellie's monitors and watch the numbers constantly...jumping when one alarmed and frantically looking for a nurse. Gradually, we became more at ease with everything. We began working with absolutely wonderful speech therapist and lactation consultant. We are indebted to them as they fought for our girl and cheered us on as we learned all the tips and tricks to feed a baby with low tone. David and I had a system down, and I can honestly say that I fell in love with him so much more as we took care of Ellie in the NICU. He provided so much drive and encouragement to me as we learned to feed her. He lovingly pushed me when I didn't want to pump, and was so involved with learning the techniques to feed Ellie...spin the bottle, squeeze her cheeks, support her chin, tap the bottle, crank Ellie's arm..it worked, don't ask us why or how ;)
Many babies with PWS leave the hospital with an NG tube, or some have surgery to place a gastrostomy tube (G-tube) inserted through the abdomen that delivers nutrition directly to the stomach. This way doctors can ensure the children get the nutrition they need. In the case of children with PWS, this tube allows them to get the fluid and calories they need, but practice eating by mouth and eventually get the hang of everything as their energy, stamina, and tone improves.
Each day I can tell Ellie gets stronger, and everything she does in some way can all be traced back to tone. Crying, smiling, laughing, sitting, crawling, standing, pulling, pushing, turning, talking, breathing, sleeping, running, jumping, climbing, kicking, throwing, writing....
I hate, hate, hate that she has to work harder to do these things and hate that they don't typically come easy to her because of lower muscle tone...but I see her drive, determination and strength and I know that it is her way of saying, "Mom, don't worry...I got this tone thing covered, and it doesn't have me!"
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