Facing Reality

Sadie in her owl costume on Halloween.

The morning after Sadie's diagnosis, I remember awakening and hoping that the previous day was all a dream. But I quickly had to face the reality that it wasn't. Coming to terms with reality can be a difficult task for parents of special needs children. It's been particularly difficult lately as Sadie has been struggling in so many areas.

We've already faced some pretty tough realities - that our little girl may never walk, talk, or develop like a normal child. But for a long period of time, she was still making gains. She smiled, she held a toy for a short time, she swatted at beads on her toy bar, she rolled over for the first time at 18 months! While these might sound like small, insignificant gains to others, they were HUGE for us - and for Sadie.

I always try my hardest to stay positive, but the simple truth is she no longer does many of these things. Things that we once took for granted - like bringing her hands together, holding and grasping a toy, mashing her little hands against her face, swallowing food, sucking on a bottle. We've had to come to terms that these skills might be lost.

Who knows why she has regressed in some areas. Maybe it's the burden of multiple seizures (now over 20 per day) catching up with her. Maybe it's an unfortunate side effect of the two brain surgeries. Maybe it's just the nature of her getting bigger and it being more difficult to move her body around. Whatever it is, it hurts my heart deeply.



This is what a G-Tube looks like.

Recently, we've had to accept the fact that she needs a feeding tube. Every parent has their own hot button - something that is particularly difficult to accept. For some, it's the wheelchair, or the trach, for us...it was the G-Tube. Her recent swallow study confirmed that Sadie's swallow isn't strong enough to get all her calories orally. The news didn't come as a huge surprise to us. After all, she has had the temporary NG tube in place for almost 6 months now.

The G-Tube procedure will be done at the end of this month. It pains me that she will undergo yet another surgery this year. But we know that for her health, it's what she needs. On a positive note, we'll never worry about dehydration or getting her medicines. I also learned that you can vent a g-tube, allowing gas to expel directly out of the stomach...who knew? When people ask about it, I tell them it looks like the plug for an inflatable pool toy. I suppose it's a natural way to feed, after all, we all fed that way in utero, right?

The "wobble switch" recommended for Sadie.

Despite my melancholy about the hiccup with her development, Sadie has been keeping busy with her weekly aqua therapy sessions. If she would only keep her eyes open in the warm therapy pool. (She seems to think it's a bubble bath.) 

She also underwent an augmentative communication clinic at our local Easter Seals branch. They recommended a few low tech communication devices for her as well as a wobble switch. The switch can be used to activate toys, play music, make choices, etc. It is easily activated by any movement of her hand or arm.

So, while there are a few good things happening in Sadie Land...it's been difficult lately to see the forest for the trees.

The Little Things. A Dash of Realism.

Much has happened in Sadie Land since our last blog post...swallow and sleep studies, a glorious period of seizure freedom, and a lot of new activity.

Sadie had her sleep study last week. The premise of a sleep study is to evaluate sleep patterns and determine if any sleep disorders (such as apnea) exist. As you can see by the photo above, multiple wires and sensors are placed on the body to evaluate breathing, leg movement, jaw movement, EEG patterns, etc. It's no wonder that Sadie slept horribly during this study. She was also recovering from a bad cold, so she was already a little congested. She slept about 4 hours that night.

When the nurse called to give the report, she informed me that no apnea was detected, but Sadie needed a little bit of oxygen (big surprise) and never got into REM sleep.

I asked, "Does apnea sometimes show up only in REM sleep?"
Nurse: Sometimes
Me: So do we have to repeat the sleep study once she's better and off oxygen?
Nurse: That probably isn't a bad idea.
Sigh...

I also asked for a copy of the report. It amazes me how much medical jargon is included in these reports. You'd think it was a contest between doctors. Example, the title of the sleep study report - "Nocturnal Polysomnograph Report." Interpreter, please.

Sadie's seizure activity has been all over the place in the past few weeks. She'll have a few good days, then a few really bad ones. Strangely enough, two weeks ago, the seizures stopped entirely. No medications were changed, nothing...we had 6 beautiful days of seizure freedom. Then the seizures came back 7 days later. We had to administer diastat (emergency med) after she had a long seizure that didn't go away after 40 minutes. It was a hugely disheartening.

Because the seizures now are looking a little different than even a few weeks ago, the doctor ordered another video EEG to determine if she is having different seizure types. This information helps the docs determine which medications will work best.

In positive news, Sadie is slowly coming off oxygen. She can go most days without. We do still have to put her nasal cannula in for naps and at night. Sadie has also delighted us the past few weeks by doing some pretty amazing things.

Reaching for and grabbing a toy - The past few weeks, Sadie has gotten pretty good at reaching and grabbing. I know she is interested in a toy when she lifts her right arm immediately when I bring the toy in front of her. She has started to grasp a few toys as well, even pinching some between her thumb and finger. This is a huge accomplishment. Previously, she wouldn't grasp at all and toys would simply fall out of her hand. She's still working on her grasp with the left hand, but I think she'll get there.

Because of this recent interest in toys, we've enrolled Sadie in Lekotek. Lekotek is an organization that helps families with special needs children learn how to play with toys. They have a variety of different toys that are more accessible to kids with special needs. Sadie has a hard time playing with toys due to her low muscle tone and lack of coordination. She can't press buttons on toys like most kids - many of the Lekotek toys are attached to switches which make them easier for Sadie to use.

Rolling over (almost) - Sadie can now get herself up on her left side. It's actually her preferred sleeping position. She'll wind up and flip her right arm and leg over her body - she has yet to fully roll onto her belly, but she has gotten her entire body over except for her head.

Eating - Sadie actually cooperated and we got through swallow study last week. The good news is, she was awake and swallowed her food. The bad news is she is aspirating some of it. The x-ray showed that some of her formula was getting into her lungs. The solution for the time being is to thicken her liquids. We are using a thickener called Simply Thick, which makes the formula the consistency of a thick nectar. The thicker liquid is more difficult to aspirate. We have an appointment to visit a feeding clinic at Easter Seals this week, and we'll work closely with her feeding therapist to tweak her feeds in the coming weeks.

We started to feed her orally the evening after the swallow study. She was a champ with the solid foods and took them great. Tears of joy filled my eyes as she began to suck on her bottle again. For parents of "normal" children, these proud tears are reserved for graduations, little league games, piano recitals, first proms. As parents of a special needs child, it's these small things that keep us going and give us hope.

There many people who say "you'll never know what she'll be able to do." And, while we appreciate the optimism, we have to temper it with a dash of realism, otherwise, we'd be constantly disappointed.

For Sadie, it's the little things that make us so happy - (almost) rolling over, giggling, sucking her bottle, reaching for a toy, grasping an object. We hope for more of these small achievements in the coming weeks.

Trying Times. A Christmas miracle.

We've been delaying our Christmas update, as were waiting to see if there would be good news to report. Unfortunately, that is not the case. Sadie had a pretty rough holiday season. Her seizure activity began to increase a few days before Christmas. We've been slowly tapering one of her seizure meds, Topomax, in the hopes that we could get her off of one of the three medications and increase her alertness. She's been on Topomax since she was first diagnosed with Aicardi Syndrome, and we haven't been entirely convinced that it is working. Additionally Topomax is not an ideal medication in combination with the ketogenic diet as it can cause kidney stones. So, a few days before Christmas, we started to decrease her dosage. A few days later, her seizures increased 7-10 clusters of a day to 10-15 clusters a day.

We didn't want to jump to the conclusion that Topomax was working. Unfortunately in children with seizure disorders, so many other factors can play a role with seizure increases. For Sadie, it could be the change of routine with traveling, the fact that she was running a low-grade fever around Christmas, coming down with a slight cold around New Years, etc. Our New Years Eve was spent on the phone with her neurologist, and we're working on tweaking the Depakote dosage to see if we can get more seizure control while keeping Topomax at the same level for the time being. Needless to say, we're frustrated and continue to hope that someday soon we'll see an improvement in Sadie's seizures.

While experiencing trying days and nights over the past few weeks, our holidays were also interspersed with a few moments of pure Sadie happiness and joy. She had many moments where she would talk, squeal, giggle, smile...were it not for these moments, we'd be having a much harder time. Of course, our little girl was also showered with love and affection from a host of friends and family. It will take three trips to get all of her gifts and clothes back to our house! Her closet continues to expand.

In addition to the seizures, we've also been battling with some bowel movement issues with Sadie. Warning to readers who may get squirmy when hearing about poo, you may want to skip this paragraph. I feel like the topic of poop has come up way too much in our household lately. It's been about 2 months since Sadie has pooped on her own...that is, without the help of a glycerin suppository. We actually had to give her an enema last week to get her to go, after three suppositories didn't work. Talk about stress. We're not sure what to attribute it to - the ketogenic diet, which can be constipating or the fact that she is taking three sedating seizure medicines, or a combination of both. We've tried everything - miralax, milk of magnesia, prunes, senna, baby massage, reflexology. Day four after reducing her Topomax doseage, Sadie had a bm without help of a suppository. Adin called it a "Christmas Miracle."

Because of these issues, we've added yet another doctor into the mix. Sadie had her first appointment with a GI specialist at Children's Memorial. This month will be a busy one for little Sadie. She'll have another MRI to monitor her choriod plexus papilloma tumor. We also have an appointment at Easter Seals to get her fitted for a specialized stroller and potentially a special car seat.

So, we'll stay hopeful that 2011 will bring Sadie better seizure control, more moments of pure joy and happiness, and the knowledge that she is loved and adored by many people.

For Better or Worse. Here Goes...

We're bracing for impact. What that impact is, we're not sure. After a week of various opinions, we've made a decision.

Let me start at the beginning...

Sadie saw a new neurologist last week to get a second opinion. In the meantime, her current neurologist presented Sadie's case to the team of doctors at Children's Memorial. Apparentely, the doctors do this periodically when they have difficult cases. The result was two differing opinions.

Doctor A recommended weaning a few medicines to try to determine what is and isn't working. "A" also suggested she try a few new medications. Doctor B recommended we start aggressively treating her with prenisolone, an oral steriod. Steriod treatment has always made us a little nervous, given the many side effects, including a weakened immune system. We've opted out of the most commonly used steriod for infantile spasms, ACTH, due to the high mortality rate (1 out of every 12 children), and the fact that many Aicardi parents don't think it works very well for girls with the Syndrome.

After giving ourseleves a few days and seeking out numerous opinions, we decided to forgo the steriod treatment for the time being. For better or worse, we're trying another seizure medication, Depakote.

Sadie was also at the hospital most of the day last Thursday for her Electroretinagram (ERG). We received good news and bad news. The good news is that the ERG showed Sadie to have decent vision in her left eye. The bad news was as we suspected, she most likely has no vision in her right eye....sigh. Although we had mentally prepared for this, it doesn't make the news any easier.

We've started working with two great non-profit organizations. The first is an organization called Hope's Friends. They send a home nurse to see Sadie as needed, a service that will be even more important as cold weather approaches. The nurse came out this week to check out Sadie's bowel movement issues. The ketogenic diet really stops her up :( They'll also be starting Music Therapy with Sadie this week. We'll see if we can get any Katy Perry or Beyonce in the mix, you know how Sadie loves them :) Additionally, an aromatherapist is developing aromas for Sadie to see if it will help with her bowel movements and seizure control. It's worth a try, right?

Sadie also visited the local Easter Seals chapter for a feeding evaluation. She will go back in a few weeks for a seating/positioning clinic to help determine what, if any, special equipment is needed as she gets older. Specifically, we're looking at special feeding chairs, strollers, and car seats. Both organizations have been wonderfully helpful for Sadie.

So, we brace for impact as Sadie starts what is now her third seizure medicine this week. We hope and pray that the third time's a charm.