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| Ella waiting in the ER at Children's Memorial Hospital (March 2012) with quite the sweaty head! |
March 2, 2012. The temperature was dropping. Raindrops were changing gracefully into large fluffy snowflakes being blown in the winter's wind. The only person in our family who was sweating was Ella.
She has always sweated but only when sleeping. Prior to her pneumonia the sweating was of little concern since it was so mild. Post-pneumonia brought some new developments for Ella.
Since her discharge from Children's Memorial Hospital (CMH) on Feb. 20, after fighting the RSV and pneumonia, Ella has shown marked signs of apparent regression. We noticed her becoming heavier and seemingly more "limp" when we held her. The amount (and duration) of her sweating increased...from mild sweating while sleeping to a soaking sweat throughout the night and random head sweating during the day associated with very little physical exertion. The most disturbing development was her decrease in energies and abilities...she seemed to be going downhill rapidly.
Within the two weeks she had been home she gained one full pound. For Ella, this is too rapid of a weight gain in such a short period of time. Her muscles cannot afford the energy needed to sustain this rapid gain. She had the 1.5 Pediasure formula and needed the regular formula...in other words she was getting 1.5x the caloric intake.
The adjustment was quickly made by diluting her portions to accomodate the caloric intake she needed. While this slowed the weight gain, the problems of excessive sweating, lack of energy, and apparent regression persisted.
On Friday (March 2) just two weeks after being discharged from CMH we took Ella back to the ER.
Our concerns were simple.
The first being that we felt that her diet of Pediasure was not appropriate for her, being a child with SMA. Pediasure is higher in fat compared to other formulas. More importantly, though, is the type of fat it contains. There are long chain fats and short chain fats. Pediasure has long chain fats. These fats are very difficult for kids with SMA to break down and metabolize...short chain fats are much easier for their bodies to work with. There are other formulas that are lower in fat and are also short chain fats. The one that we were seeking for Ella is a formula called Pediatric Vivanex.
The second of our concerns lay with her Bipap settings (Bipap is her ventilating machine that she uses during naps and overnight sleep). The general idea behind the machine is that it assists with her inhaling and exhaling while she sleeps so she can conserve energy as well as fall into a deep (REM) sleep--while maintaining her O2 and CO2 levels. We would like to be sure that she is getting the optimal support specific for her.
After 5 hours in the ER, CMH admitted us to the PICU (Pediatric ICU) in order to monitor her while sleeping with her Bipap settings.
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| Ella waiting patiently in the ER (it's getting close to midnight) |
They were looking for acceptable O2/CO2 levels. They explained to us that in order to change her settings she would need to be monitored through a sleep study. A sleep study will give us a specific picture of everything that is going on with her body as she sleeps. During a sleep study her Bipap levels can be tweaked to find her optimal support level while in a controlled environment.
Ella has a sleep study scheduled for early May but has been placed 'first' on the cancellation list through CMH.
The focus then turned to the sweating issue. We spoke to several doctors, nurses, "teams", and a nutritionist. We explained our concerns over and over. They were receptive and worked with us to solve this problem...or at least address it to maybe ascertain why the sweating was getting so out of control.
The bottom line was that we did not want to change more than one variable at a time. Lindsay and I felt strongly that her diet was playing an important role in all that we were seeing her go through in the past two weeks. We advocated for her that her diet needed to be adjusted to what we felt was optimal for her based on what we know about Ella, what we have researched, and the experiences of other SMA families. Digesting and metabolizing food can take a lot out of child with SMA...our call is to help her maximize her efficiency.
Ella had been visited by so many different people and she gave every one of them her signature smile. At one point she was hooked up to several wires while getting an EKG and chest x-ray to make sure her heart was working properly (they wanted to rule out heart problems causing the excessive sweating).
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| Ella hooked up for EKG while she sleeps early Saturday morning |
In our 22 hour stay at CMH we ended up switching her to the Pediatric Vivanex formula. We are making the recommended supplementations (to her new diet) based on that fact that she is a child with SMA.
We are also posed and ready to work on optimizing her Bipap settings to give her the best breathing support possible as she sleeps.
Ella is a delight to be around. She is funny, easy-going, smart, and endearing. Her personality draws you in, takes a gentle yet firm hold of you, and places you at ease as the world around you is in chaos.
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| Ella getting ready to leave on Saturday morning |
Ava & Ella missed each other...on Sunday morning they caught up with one another...
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| The girls... |
