It Gets Better?

When Sadie was a newborn, she and I would take daily walks around our neighborhood. There was a local park we'd circle every day with a playground, pool, and softball fields. After Sadie's diagnosis, there were some days when making that loop was just too painful. Every child jumping off monkey bars, running by the pool, pitching a ball - was a vivid reminder of my own child's limitations.

As Sadie is our first baby, Adin and I have no previous experience in typical child development. We only have the children of friends and family with which to compare. Now that summer is into full swing, we find ourselves at friends' barbecues, pool parties, and family gatherings. As I mentioned in a previous blog post, it's quite difficult NOT to compare your own child to others.

Friends of ours, parents of another Aicardi girl, compare it to "the bends." A feeling you get when resurfacing from a deep sea dive. You are thrown into an alternate reality, then just as quickly, you're catapulted back into your own reality. Psychologists call it "chronic sorrow," a pervasive grief experienced by parents of children with special needs. There is no "It Gets Better" campaign for parents of children with special needs. You're simply handed a copy of a poem about Holland and sent on your way.

Don't get me wrong, I LOVE seeing our nieces, nephews and friends' children, and am fascinated with how quickly they grow and develop. But will there always be a twinge of pain when seeing a child do something that Sadie might never do? Does this mean our friends and family should feel guilty for having healthy children? No - absolutely not. We simply hope they cherish every healthy, typically-developing moment.

And while I hate hanging out in the land of "I feel sorry for myself", perhaps I've been thinking more about this lately because Sadie has been experiencing one of the most rocky seizure periods she's ever had. Her Dilantin levels have been all out of sorts - high one week, low the next. We also started a new medicine, Ospolot, a few weeks ago. Since then, she's been having periods of time where all she does is twitch. Sometimes it's mild - just the foot and hand twitching in a synchronized pattern, other times, her whole body twitches. This can go on for over an hour. We tend to err on the conservative side when it comes to administering Diastat (her emergency med), otherwise, we'd be giving it every day. We have given it three times in the past two weeks.

Just when we thought things were starting to calm down, the seizures spiked again for no apparent reason. We also noticed that Sadie's heart rate was extremely high and she was running a temperature. Come to find out, Sadie had a urinary tract infection (UTI). We started her on antibiotics, but the fevers continued to spike. Back to the pediatrician a few days later and they gave her an antibiotic shot - to which she had an allergic reaction. Hives all over her arms and legs.

And, while we thought we had gotten rid of the nasal cannula, she's needed to be on oxygen for the past week. According to her pulminologist, this can happen when kiddos get sick. Grrrrr...the girl simply can't catch a break.

So, I'm patiently waiting for things to get better. Patiently.

The only thing constant

Recently, I've felt like the only thing constant is change. We're aching for some regularity in our lives. Seizures are changing, medications are changing, feeding is changing.

Some changes are for the better....

Like Sadie is now taking all her nutrition by mouth. NO MORE NG TUBE! She is excited about the bottle, excited about solid foods...NOT so excited by her medicine syringes. So we've been experimenting with different methods of getting the medicines in her belly. What complicates the issue is the thickening of the liquid. As mentioned in the previous blog post, Sadie's swallow study showed that she aspirates thin liquids and all of her medicines are thin liquids (compounded liquid or crushed tablets mixed with water.)

Another positive change is Sadie's EEG. She had another video EEG study done last week to look at her brain waves. Sadie's neurologist reported that her recent EEG showed "more organized brain activity."

We are also happy that Sadie is slowly shedding her cords. She has been doing great off oxygen during the day. She even gets through her daily naps off O2. She is, however, still needing a trickle of oxygen at night. But we hope to get rid of the nasal cannula soon! I can't say when we'll be comfortable taking her off the pulse oximeter. There is a certain level of comfort in knowing her heart rate and oxygen levels at all times...but I know that in order for us to live a more "normal" life, we'll have to get rid of it soon.

Some changes are for the worse...

Sadie's seizures are still a little out of control. We've had to use diastat five times in the past six weeks. The most recent episode happened while her aunt and I were out shopping. Sadie began to have a seizure that lasted upwards of 20 minutes. We quickly headed to the car and administered the Diastat in the back of the minivan. As we headed home, her O2 levels dropped quickly and she became unresponsive. We gave her some emergency oxygen, but the O2 levels only rose into the mid-90s. So, we again found ourselves in the ER at Lutheran General. By the time we arrived, Sadie's O2 levels were back up, but she was a little woozy from the Diastat.

Luckily, this time we were released within a few hours. The docs found that Sadie's Dilantin level was high (33.6). Therapeutic range is from 10-20. If the level gets too high, it can cause increased seizures. So, we're working with the doc to reduce her levels. We also started a new medicine this week, Ospolot. Ospolot is not yet FDA approved in the US, but is used to treat seizures in Europe. Clinical trials look promising for Sadie's seizure types. One of Sadie's Aicardi friends, Josie, has used the medicine for years and it has worked well. We are hopeful that this new medicine will also work for Sadie.

We had Sadie out and about in the past few weeks. The photo above shows us at the finish line of the Epilepsy 5K Walk. Sadie also enjoyed her very first tea party for her friend Carmen's fourth birthday! The photo above shows Sadie in her fancy party dress with her Mimi. (Can you tell she likes her hands?)

The changes remind us not to get too comfortable. So, Sadie's journey continues as we change course...and change course again.

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.

Sadie Time

We always joke that Sadie works on her own schedule - "Sadie Time." I have to laugh when people ask me questions like "When does she typically nap?" "What's her schedule?" The girl does what she wants, when she wants. We do our best to have her awake and alert during the day, and keep her sleeping at night - but most of the time, even this proves to be a challenge.

Case in point, we've now had to reschedule her swallow study twice. The first time around, she came down with a cold, so we cancelled and rescheduled for a week later. The second attempt proved beyond a doubt that Sadie is in charge. Despite sleeping relatively well the night before and getting in a good morning nap, she simply refused to wake up for the 1 pm swallow study. We tried taking off her shirt, putting a wet washcloth on her face, turning the lights up high, playing music, etc. The more we tried, the more she slept.

So frustrating when there are three technicians, a speech therapist and doctor in the room all asking why she's not waking up? Isn't she hungry? Did she not get a good nap? I wanted to scream at them and tell them that she does things on her schedule - and four seizure medications doesn't help either. Arrrghhh...

We attempt the swallow study again on Tuesday. I'm fearful because she hasn't taken anything by mouth for the past month, that she's lost some of her swallowing ability. I guess we'll know for sure on Tuesday (if Sadie cooperates.)

Sadie has been having a hard time keeping her oxygen levels up on her own. This week will mark one month on oxygen and the NG feeding tube. We had been slowly weaning her off the O2 when she came down with a slight cold and we had to boost her up again. After speaking with her pulminologist last week, she suspects that Sadie's low O2 levels may be the result of her having a difficult time coughing stuff up. Apparently this is a problem for many kids with low muscle tone. I have an appointment with a respiratory therapist next week to learn how to do chest PT, which will help with this. My nursing repertoire expands.

The pulminologist also suspects that Sadie may have sleep apnea. So, they will conduct a sleep study in the next few weeks where they will observe her overnight. Perhaps this will finally answer our question about her horrible sleep patterns.

If that wasn't enough, this past month her seizure activity has been up and down. She'll go for a few days having very low seizure activity (2-3 is a good day for Sadie.) Then, out of nowhere, she'll have a day where she'll be seizing every 3 minutes. We've had to give diastat (her emergency medication) twice in the past month. The seizures have been looking a little different lately as well. Instead of her typical infantile spasm "crunches," she's been having seizures that look more like your standard seizure (some shaking/trembling, eyes rolling). These look a little more frightening than her old seizures.

I was a little (OK, a lot) disappointed that Sadie had all of her cords attached for her first birthday party. But as you can see in the photo above, she looked adorable and had a wonderful time with friends and family.

We forge ahead, hopeful that we can get her off of oxygen and the feeding tube very very soon. Which, Sadie will do when SHE is good and ready.

P.S. Sadie's Journey now has an option to "Follow by E-mail." To receive e-mail updates with new blog posts, enter your e-mail address in the field to the right.

Social Sadie. Warning, sickness ahead.

The past few weeks, Sadie has been quite verbal and is often taken to having long conversations in her crib in the middle of the night. She's not cranky or upset, she just wants to chat. She'll do this for hours on end, but simply prefers to do it between the hours of 12 and 4 am. We hope this bodes well for her future verbal ability.

She has also been quite active, and has gotten close to rolling over. She keeps herself entertained talking and moving, moving and talking for hours at night in her crib. Adin and I will alternate getting up to check on her. The conversation usually goes something like this.

Cathy: Honey, can you get up and check on her?
Adin: Arrgh...
Cathy: I think she just said "daddy." She obviously wants you.
Adin: Arrgh... (rolls onto his side and climbs out of bed)

Seeing as how she likes to communicate with us, we've started to teach her sign language. We're currently teaching her two signs - "milk" and "more." We've done better with the "milk" sign, and swear that she is trying to do it on her own!

She also does a funny thing when she's dosing. Her eyes are little slits and we ask her if she's "fake sleeping." She typically responds with a big smile although her eyes are still mostly closed.

Speaking of sleep, we broke down and tried the melatonin one night. She slept for a record four hours (four hours, people!). Unfortunately, the next day was a crazy high seizure day for her. Its hard to discern whether it was the melatonin or another factor. So for the time being, we're holding off on the melatonin until we're at a more stable place with her medications.

Speaking of medications, Sadie is still on her four seizure meds (Topomax, Vigabatrin, Clonazapam, and Dilantin). The latter two are the ones that seem to be showing some positive improvement. We're still weaning Topomax, albeit very slowly. Vigabatrin will be weaned next. It will likely take us until June or July to get her off both, as long as we see no adverse effects.

Speaking of adverse effects, we've heard from many people that seizure activity often spikes right before a child gets sick. We think that is what happened with Sadie earlier this week. We spoke with the on-call neurologist after she had a really rough night and morning - seizures happening every 5 minutes. The neuro advised us to give her Diastat (emergency medication). This is a huge thing for us, as it was the first time we've administered Diastat at home. It was a little nerve-wracking, but it stopped the seizures. She, of course, was way out of it for the rest of the day. Not suprising, as Diastat is a form of Valium.

Because Diastat can suppress breathing, we hooked her up to her new pulse oximeter to monitor her O2 level. Pre-diastat, her O2 was about 95%, post-diastat she hovered around 85%. I was again on the phone with doctors, and was trying to avoid the dreaded "take her to the ER" response. (Sidebar - love love love the folks in the ER at Children's, but we feel like the past few times we've been there, Sadie's been hit by a wrecking ball as they try to get her O2 levels back up.)

So, we took her to the pediatrician. Luckily by that point, her O2 had gone back up into the 90s....whew - crisis averted. In the meantime, our lovely home nurse (who sees Sadie once every two weeks) had an oxygen compressor and tanks delivered to our home. So - we've added to our beautiful collection of medical equipment. Lo and behold, two days later, Sadie comes down with a horrible cold. The seizure increase served as our warning sign.

We forge ahead as Sadie approaches her first birthday at the end of this month. So hard to believe that our little girl will be one soon. Even through all the hospital visits, doctor appointments, therapy sessions, etc., she has been our joy and the light of our lives.

From bad to not so bad. Yay for ketones!

The last week of July things went from bad to worse. Sadie had been on vigabatrin, her new seizure medicine, for about two weeks. The medicine seemed to have an adverse effect, as her seizures started INCREASING. Each time the doctor would increase her meds, her seizures would go up. The increased medication was also making her drowsy. By the last weekend in July, her only awake time was when she was having spasms.

We were on the phone with the neurologists every day talking about scary remedies, from administering diastat (an emergency medication given rectally to stop seizures), to admitting her to the hospital to begin ACTH, an injectable steriod treatment. We had already decided that ACTH had too many scary side effects for us to try, one of which is possible death. So we were stuck with a baby who is constantly having seizures.

On top of the seizures, Sadie had a conformer put in her eye last week, and the conformer ended up scratching her cornea. Back to the ocularist to get it taken out, and then to the opthomologist to get medicated drops. Ugh. If only I could get paid for coordinating doctors appointments.

Needless to say all of this was super stressful. We were at the end of our rope when the hospital called to tell us they were able to get her in to start the ketogenic diet right away. Sadie was admitted on Monday. She received her first bottle of KetoCal (a special ketogenic formula) on Monday night. It smells like a vanilla milkshake. Sadie loved it...but of course she's never turned away food. We anxiously awaited the arrival of ketones in her urine, which indicates that the diet is working. I don't think I've ever looked forward to diaper changes as much.

Monday and Tuesday went by without ketones. Sadie's seizure activity held steady. She had a few scary episodes at the hospital, one of which lasted 50 minutes. FINALLY late Tuesday night she had a trace of ketones in her urine. Yay! The doctors came in saying they wanted to pop the champagne.

Speaking of the doctors, I can't say enough good things about the staff at Childrens Memorial Hospital, many of whom are aware of sadie's condition and pulled strings to get her in earlier to start the diet. Sadie was released from the hospital on Thursday night and seems to be getting better, slowly...

We are cautiously optimistic.