Sadie's first ambulance ride. Prayers, prayers, prayers.

Looking at the title of the last blog post, I hope I didn't jinx poor Sadie. After battling cold symptoms for a few days, Sadie awoke early Tuesday morning with labored breathing and a "barky" cough. We hooked her up to the pulse ox to check her O2 levels. The machine read 83%...we started panicking. Luckily we had the emergency oxygen her home nurse had sent over the week prior. We started her on O2 and she quickly rose to 100%. Adin and I knew that we had to call an ambulance to transport her to the hospital, as we couldn't take her without supplemental oxygen.

The ambulance arrived at 2 am, and delivered Sadie and I to the ER at Lutheran General Hospital. Ambulance protocol is to take the patient to the nearest ER. I was a little nervous being at a different hospital than Childrens, which we know so well. Although I prefer Sadie's doctors at Childrens, we've been quite pleased with the staff and facility at Lutheran General.

We are on day 4 in the pediatric ward. What started as cold symptoms turned out to be metapneumovirus, which is similar to RSV (the virus that hospitalized Sadie in January). Sadie is also showing some cloudiness in her lungs, which could be a mild case of pneumonia. The doctors are trying to figure out why these respiratory viruses are so hard on Sadie. They suspect aspiration, which is where some of the liquid one consumes goes into the lungs instead of the stomach. She will be having a swallow study to explore this further. In the meantime, all liquids are going through an ng tube (through nose, down throat, in tummy). She REALLY did not like that ng tube going in, and actually punched the nurse with her little bandaged IV hand.

Throughout her hospital stay, her seizure activity has been quite low. And, we finally were blessed with her first seizure free day*. I always feel like I need an asterisk after that statement, as it is possible she had a twitch here or there that I didn't see, or a mild episode in her sleep. The picture above is the page from Sadie's journal showing her first day with no seizure activity. We have been keeping these journals since she was 4 weeks old (we're now on journal #3.) Each page is a different day. We track the time and duration of the seizure. I used to count every single spasm per cluster, but once they got up into the hundreds it was just too depressing.

We also track her fluid intake and diapers in the journal (since she has decided to hold her pee for hours and hours, and poo for days. More about that in a future blog post.) We have also been advised to see a pulminologist to oversee Sadie's respiratory issues. Her team of doctors grows.

The analyst in me wants to overanalyze exactly why her seizure activity has been so low. (I see a spreadsheet in our future:) But for the time being, I'll be grateful for this blessing. Maybe all of your prayers are working. Keep them up!

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.

Gratitude.

Last week, friends of ours hosted a benefit fundraiser for Sadie. You can view photos of the event here. We cannot properly express in words our gratitude to those individuals who put this event together - Chip & Lisa, Jeff & Erika, and Josh & Heidi - we are forever indebted to you for offering up so much of your time and energy.

Proceeds from the fundraiser will go to Sadie's Special Needs Trust. The funds will be used to pay for anything that is not covered by insurance - prescription co-pays, specialized equipment, extra therapy sessions. While we are thankful that insurance is picking up a huge tab for Sadie's care, we are learning very quickly that there are a number of things that simply aren't covered.

A portion of the proceeds will also be donated to the Aicardi Syndrome Foundation. The Foundation connects families affected by this disease through a biennial Family Conference. They also assist with funding two major research teams who are doing research on Aicardi Syndrome.

Many, many other people also donated time, items for the auction, money - we will do our best to thank each and every one of you personally. We have been completely overwhelmed by the support we have received, and the people who have supported us...many of whom we have not seen in years (yes, you). It's beyond heartwarming to know that Sadie is loved and so many people want to help out. Please know that your donations will go to help our little girl live the most fulfilling life that she can.

Sadie's godmother always says, "that little girl is going to change the world." We think she already has.

If there's anything we can do (continued)...

As we learn more about Sadie's condition, we also learn more about our needs as parents as we navigate this new reality. So, as a continuation of our "If there's anything..." post, we decided to add a few more things to the list.

6) Advocate. We were disappointed to learn that Illinois ranks dead last in many services to individuals with disabilities. Read more about that here. (For example, Sadie is currently on a wait list of 20,000 people to get any sort of assistance for her disability.) Send a letter or e-mail to your Illinois representative about providing more programs and services for individuals with disabilities.
7) Give to Sadie's Special Needs Trust. We've set up a special trust for Sadie to pay for items that are not covered by insurance. (i.e. therapies, medications, special equipment, etc.) The trust ensures that any funds set aside to care for Sadie will not be counted against her once she turns 18, and will enable her to access public funds such as Medicaid and Social Security Income. It also will provide for Sadie's care if, heaven forbid, anything were to happen to Adin or myself. If you'd like to make a donation to Sadie's Special Needs Trust, please let us know. You can also donate with a credit card through PayPal (simply click the link on the right).
8) Attend a benefit fundraiser for Sadie and the Aicardi Syndrome Foundation. We are blessed to have great friends who are planning a fundraiser next week in South Bend, IN. It will be a fun party with great music :) Click here for more info. We hope to see many of you there.

Home Sweet Home. Sleep Sweet Sleep.

Sadie has been home from the hospital for about a week. She is still struggling with kicking this recent respiratory virus. At her pediatrician's request, she is now the proud owner of a pulse oximeter and a nebulizer machine. We will use the pulse-ox at home to monitor her O2 levels should she get sick again. We've been using the nebulizer machine to give her albuterol breathing treatments three times a day. We'll continue with the treatments until the virus works its way out and her breathing returns to normal.

While in the hospital, we finally weaned Sadie off of one seizure medicine. While Clobazam looked promising at first, it seemed to be having an adverse affect, as Sadie's seizures went up with each increase in dosage. Once we backed off the dosage, her seizure activity went down. We also started Sadie on Dilantin when she was having a rough time with seizures at the hospital. I hesitate to say that her current seizure medication cocktail (dilantin, vigabatrin, topomax and clonazapam) seems to be showing some improvement. The seizures are at their lowest level. She still has episodes daily, but we've gone from 20+ seizures a day down to 3-5. Our next step is to slowly wean a few of these meds that we're not entirely sure are working, something we've been wanting to do for quite some time.

We also received some troubling news a few months ago that I didn't feel comfortable blogging about at the time. The doctor noticed some new brain abnormalities in Sadie's recent MRI. According to the doctor, there were a few areas that showed some "disbursed white matter." She said the changes looked consistent with a mitochondrial or metabolic disorder. "Instead of her Aicardi diagnosis?" I asked. "In addition to her Aicardi diagnosis," she responded. Wonderful.

This time around, I did no googling, no researching...I simply just ignored the fact that our daughter was being tested for an additional disease and kept on living. As a friend so wisely stated, "no point in going down paths that aren't yours."

I've come to the conclusion that we are the big losers when it comes to the battle of the odds. If a medication has a extremely rare side effect, Sadie is bound to get it. As it turns out, that's what the doctors think is happening with these MRI abnormalities. So far, all the mitochondrial and metabolic tests have come back within normal ranges. So that leaves the medicine. Apparently, one of Sadie's seizure medications, Vigabatrin, can cause MRI abnormalities that are similar to those shown in Sadie's MRI. The problem occurs with high doses and when children are very young (check, check). The good news is these abnormalities often revert back to normal once the medication is weaned. So, the plan is to slowly get her off vigabatrin and hope it is a transient finding and the MRI goes back to its original state.

We are overjoyed to be home after our 7-day stay at Children's Memorial. Sadie has been delightful this week. Smiling, chatting, happier than we've ever seen her. She dove headfirst into her therapies this week and did a great job. The picture above was taken during one of her monthly music therapy sessions. She liked the rain stick toy, but she liked her therapist's diamond ring even more. Smart girl.

The only trouble we've had with her is sleep. The girl simply doesn't sleep. And when I say that, I mean she is up about every hour at night. I found that one side effect of dilantin is insomnia. However, I'm not willing to give up seizure control for sleep. Insomnia combined with the coughing make for long nights for the McCann clan. We've been encouraged by her doctors to explore melatonin, which is a mild, naturally-occurring sleep aid for children. With all the medicine that she's on, we've been hesitant to add one more thing...but give us a few more nights and we may be there. For now, we thank God for espresso.

Hospital, the sequel.

We again found ourselves in the ER at Children's Memorial after Sadie was coughing and struggling to breathe. Our ER visit began the same way as our visit last month. She was assessed by a triage nurse who raced her back to the trauma room when she saw her O2 levels at 80%. It was as if we had roles in a poorly-scriped sequel where the plot line is the same, but all the characters are different.

The doctors tried their hardest not to intubate her, but just couldn't get her blood pressure or oxygen levels high enough. So, again she was intubated and put on a ventilator. A scary procedure for us to witness, as they had trouble getting the tube in her throat through a swollen airway. We saw her oxygen levels dip into the 10s, twice.

We are now at day 6 of her hospital stay in the ICU. Sadie seemed to be recovering and in good spirits until yesterday when out of nowhere her seizure activity spiked. She was given three sedatives to get the seizure to stop...then another dose after another cluster started an hour later. One step forward, two steps back.

Our strong little girl continues her feisty behavior - pulling out her nasal oxygen tube, squirming out of her arm immobilizer. She also decided that the x-ray table was the perfect place for a poonami. We hope this bodes well for her release soon.

Perspective Redefined - Part 2

It's been a while since Adin and I attended the Aicardi Family Conference last July. I think we needed some reflection time before being able to properly communicate our experience. The Aicardi Syndrome Foundation hosts this bi-annual conference for families affected by Aicardi Syndrome. As fate would have it, it just so happened to be three weeks after Sadie was diagnosed, and in the western Chicago suburbs.

We received a call from another mother encouraging us to go. We weren't sure if we were ready to face this reality, but decided that it would definitely be helpful for us to learn more about this new world we were about to enter.

I still remember our first day at the conference. Pulling into the hotel parking lot, I had ideas of what I might see - lots of handicapped vehicles, girls in wheelchairs - my heart was beating out of my chest as we entered the registration room. Everywhere we looked, there were girls affected by the Syndrome who seemed very limited in their abilities. Many were unable to walk, speak, hold their heads up independently...it was very emotional for both of us. As another new parent so appropriately put it,"It's like looking into a crystal ball...but you don't like what you're seeing."

We were greeted with open arms by the families. We were so grateful to attend this conference and learn more about Aicardi Syndrome, as well as the many issues that we will be dealing with as Sadie gets older - advocacy, special needs trusts, therapies, specialized equipment, etc. But most of all, it gave us a support group of familes going through the same thing. We met some great people who we will keep in touch with for many years.

When you're raising a disabled child, your perspective of others with disabilites is quickly adjusted. By the end of the three-day conference, I realized that I was no longer seeing these children in terms of what they could NOT do, and instead was seeing what they COULD do.

A New Set of Problems.

Sadie was finally released from the hospital on Saturday, eight days after first arriving - well, nine if you count the day she was there for her MRI.

I'll start where I left off. On Day 5, we were moved from the PICU to the 9th floor. I have to say, while we have always been impressed with the personnel at Children's Memorial Hospital, there is a level of care and attentiveness that Sadie received in the ER and PICU that we didn't see once we were moved to a floor. Our stress level rose knowing that there was no longer a nurse right outside her door all night. I understand that this is to be expected as your child's health improves, but there is something comforting knowing that a trained medical professional is keeping an eye on your child while she sleeps.

It also didn't help that our trip to nine started with Sadie crying out in pain, then the seizure cycle started up again - and for the second time during our hospital stay, she was given Ativan (a seizure suppressant/sedative) to calm her down. The exploration for the acute pain began. The doctors x-rayed her belly, which was puffy with air from being on the ventilator and high-flow oxygen. Sadie also received an ultrasound to check for kidney stones. She again began her pain cry/seizure cycle the next day, and again Ativan was administered.

We wracked our brains looking for an answer. One of the nurses suggested that perhaps her tummy was upset from not having food for so long, so we started her on Zantac for acid reflux. She seemed a bit better and the pain cries went away. Mystery solved, or so we thought...

So, we've been back at home for a few days now. Sadie seems to have kicked RSV, but with one resolution comes a new set of problems. Her seizures are again on the rise - occurring every 30 minutes or so. She's also been quite irritable lately and has been crying out for no reason. We suspect it's her new medicine, which can cause irritability. We see her neurologist this week and hope to have some resolutions.

P.S. Last week, another Aicardi Family, the Verdecchia's, were profiled on The Today Show about their daughter's brain surgery. Here is the link to the story. We were planning to write a post about this, but our friend Neil Pasher beat us to it. His question is one that resonates with us as we make difficult decisions about Sadie's care. Read his eloquent blog post.

Another year, another hospital visit

Just as soon as things got better, they got worse. Sadie was at Children's Memorial Hospital for an MRI last Thursday. Despite receiving a bit of oxygen following the procedure, she was fine. We were home on Thursday night and she went right to sleep.

Friday morning, Sadie arose with horrible breathing, coughing, and irritation in her throat. She had received a tube in her throat during the procedure, so we thought it was irritation from the tube. As her breathing progressively worsened, we decide to take her into the ER.

We again found ourselves at CMH. After a quick assessment by the triage nurse, they rushed her into one of the larger operating rooms. Doctors and nurses quickly started working on Sadie. In the time it took for Adin to park the car, about 20 medical professionals had filed into the room. It was quite scary. They first put her on a mask with a breathing treatment, which seemed to clear her airway. They also gave her a steroid to stop the swelling in her throat.

She seemed fine for a few hours, then the seizures started. One cluster, the two, then three...they progressively got worse until she was almost status epilepticus (a state of nonstop seizure). She was given a sedative to stop the seizure, but it was not terribly effective. The doctor decided to give her a dose of fosphenytoin, a paralytic, to stop the seizing. In order to administer the med, the had to intubate to protect her airway. That procedure was even scarier, at least 5 doctors and 20 other medical professionals raced into the room for the intubation.

So began our hospital stay. They determined that Sadie had a virus called RSV, which most likely was the cause of the raspy breathing. RSV is a cold-like virus that can be very serious in infants. The breathing tube stayed in for a few days, we were very relieved when it was removed without incident a few days later.

We write this from the bedside at Children's Memorial and hope to have Sadie back at home very soon.

Fourth Time's a Charm. Exhaling.

Well, things are getting better. We thought we'd be much more excited, but the way in which things came about put a damper on Sadie's progress.

The past few weeks have been rough. After the new year, Sadie's seizures continued to get worse, to the point that she was having very little awake time when she wasn't having a seizure. After experiencing three clusters of seizures in an hour, I spoke with her doctor about changing up her medicines (again). We had been increasing Depakote to see if we could gain more seizure control, but obviously this strategy was not working. The doctor has decided to abandon Depakote, and add Clobazam (now Sadie's fourth seizure medication).

While many doctors use Clobazam for seizure control, it is not yet FDA approved in the US, which means we have to go through a Canadian pharmacy for the medicine and pay out of pocket. While this is not ideal, we are more than willing to do it if it means that it will help Sadie. Until the medicine comes in from Canada, we are using Clonazapam as a bridge. Sadie's first few days on her new medicine were trying. The good news is, she is having fewer seizures, the bad news is she was so drowsy from the medicine that it was hard for her to even take her bottle. Our hope is that once we start weaning her off these other medicines, she will become more alert and interactive.

Sadie's therapies continue to progress quite well (that is when she is awake for them). Her reach and grasp has improved quite a bit with help from her Occupational Therapist. Last week I picked Sadie up and she promptly reached out and grabbed my hoop earring. I had to lay her down on the floor to get her to let go! I guess sometimes teaching her to reach and grab has it's consequences.

Bowel movements have become much better as well. She receives a "laxative cocktail" once a day which consists of half a Senna, a teaspoon of Miralax, and a teaspoon of milk of magnesia. We will have a poo-nami (as we like to call it) every 5-6 days, but the past few times she's gone without the help of a suppository.

Thanks to Easter Seals, we've also placed orders for Sadie's first pieces of special adaptive equipment. She is soon to be the proud owner of a Kid Kart XPress Pediatric Stroller, a Rifton Bath Chair, and a XPanda Feeding Chair. While these names may not mean much to many of you, the equipment will be a huge help for Sadie so that she can be positioned properly while being transported, bathed, and fed.

We are taking a few deep breaths now that we have better seizure control. As we venture into 2011, we hope and pray that this new medicine will continue to work for Sadie - controlling her seizures while limiting the side effects.