Friday, January 25, 2008

Update on Emily's ACTH Treatment

As we were starting the final wean of Emily's medication last week, her spasms starting breaking through again. She had even gone 36 hours with no spasms and one day with only one single spasm. I really thought they were under control.

Unfortunately, they came back quickly and frequently. We have increased her ACTH dose twice now and added Topamax as well, and she is having an average of 100 spasms a day over the last five days! [The date on this post is actually correct! :)] She is having less intense spasms, we are even calling them mini-spasms, because she doesn't cry after every one and physically it is just a small jerk. It's very frustrating and we are concerned. But there are other medications we can try and we can continue the ACTH at the high dose and hopefully see an improvement again over time. But each day the spasms continue, it's hard to be patient enough to find what will work for good.


Emily is so tough and only barely cries when she gets her injection each morning. We haven't seen a smile for a few days now, but she is continuing to make eye contact and is starting to follow objects and people with her eyes. What a good girl!!

Emily's smile


A baby's smile sure melts your heart, but when your baby only rarely smiles, it's even more precious.
I can't believe I captured this moment on camera. Emily was sleeping in the glider and I was snapping some shots of Brenna and Carter next to her. When I turned her way with the camera, I caught this smile on the first shot!
Thank you Emily!! You made our day!!

Brenna's Feet




Brenna just recently found her feet, and they are keeping her very entertained!!!! She grabs onto them and smiles. Sometimes she tips over too, and she has also rolled over twice now - from back to front. Go Brenna!!

Feeding Multiples



This is how I sometimes feed two babies at once. We are not on a super strict feeding schedule, so they usually eat about a half hour apart. But sometimes, they just can't wait for someone else to finish.
That's when I pull out these bottle proppers. These were handmade by a woman I found online while searching for bottle proppers. They are a u-shaped pillow with basically a "scrunchie" sewn on top to hold the bottle. The "arms" of the pillow go on either side of the face and the curved end rests on their chest. In these photos they are resting on the high chair for a better angle while sitting up.
They definitely help, but aren't perfect. They tip over, and sometimes the babies lose their latch on the bottle while eating. And they still need to be burped one at a time! A little help is enough though!

Eating cereal

"Maybe if I suck on my fist too . . ."
"What was THAT?"

These faces just crack me up! Nobody was thrilled with their first taste of cereal. Brenna didn't want to open her mouth and Carter screamed. Emily was the only one who smacked her lips to taste it.

We have since added pears and peaches to the menu. Pears seem to be the favorite!

Thursday, January 17, 2008

Sign the guestbook

I added a guestbook at the bottom of the page. Please sign in!!

I figured it out, you can too!!!

Monday, January 14, 2008

Children's Hospital of Philadelphia


Well, Emily and I spent a week at CHOP, from 12/27/07 to 1/3/07. Here she is in her hospital crib with the adorable polar bear she was given by a Child Life worker. They provide toys and activities for the children staying there.
After about two weeks of Emily having what I would call gagging episodes 5-6 times per day, she was admitted to the hospital for treatment. I had taken her to another ER a week prior and she was diagnosed with reflux. Throughout the week, there was no relief from the prescribed reflux meds, and her episodes (which contained 20-30 "gags" each) became more intense. I took her to the pediatrician three times and called them almost every night. They adjusted her meds and we made an appointment with the GI specialist. By the time we had our appt, Emily was having trouble with constipation as well, started a low grade fever on Christmas, and was not eating well. We even tried switching her formula to the hypo-allergenic type to help with the reflux - but no change.
At our appt with the GI doc, he thought her constipation, fever, and lack of appetite could have been symptoms of botulism! And after seeing a video we had taken of her having an "episode", he thought these "gags" looked more like seizures.
As I left the office I just couldn't bear to bring her home to have more episodes with no relief. And I was a bit freaked out about the possibility of her having seizures and botulism!!! So I took her immediately to the Emergency Room at CHOP. They took us in right away and we were admitted that night.
While we were still in the ER, the doctors - who all looked at the video - seemed sure that Emily had been having Infantile Spasms, a type of seizure under the category of Epilepsy. The specific movement her body made during the spasms was the first indicator. Each time she had a spasm, her head would fling forward, her eyes would open wide, her arms would fling out and her body would bend in the middle, like when doing a stomach crunch.
They performed an EEG that night and discovered hypsarhythmia - an abnormal, disorganized or chaotic pattern of her brain waves. She saw a neurologist as well who checked her for visual tracking and motor skills. She presented some delays in these areas, such as no neck/head control, limited tracking with the eyes, and low tone in her arms and trunk. [We were already aware of these delays and she is currently seeing a physical therapist through Early Intervention.]
After meeting with several neurologists throughout our stay, we found out that Emily's spasms are the symptomatic kind, meaning she was showing signs of developmental delays before the onset of the spasms. From what I've read, the outlook for children with this kind of IS is discouraging. Most children with this kind of IS have some degree of mental retardation and continue to have developmental delays. Of course, Emily may surprise us.
It is the goal of the doctors to normalize the EEG and in turn eliminate the spasms. Normalizing the EEG will allow her brain to learn and grow properly. This is our hope. An early EEG before we left the hospital and only 3 days into treatment already showed a slight improvement! Also, she started the shots on a Saturday and on Sunday, smiled several times and laughed for the first time ever. That was great and very encouraging!!
Now 17 days into treatment, Emily's spasms have dramatically decreased to less than 10 spasms per day (before treatment she was sometimes having 100+ spasms/day). So far today she has had none!! They also seem to be decreasing in intensity as well. We will continue the ACTH injections daily until the 22nd and have a follow up appt with the neurologist at the end of the month.
We are , of course, worried about what may lie ahead for Emily, but we'll be there to support her and provide her whatever she may need along the way. We are hopeful that this treatment will stop her spasms for good!
We love you, Em!!!!!

Merry Christmas 2007

Emily seems to be enjoying her stocking stuffers. :)
Carter and Brenna are trying out their new toys.
Brenna
Carter
Daddy and Emily (who had a little fever)
Drew shows off his Spiderman spin brush.
We enjoyed Christmas with family this year at our house. There were many extra hands to hold the babies and play with Drew and his new toys.
It was so nice to have our first Christmas as a complete family! We never thought we would be a family of SIX!!! But it's just one more reason to celebrate this year. Hooray!