Great News!

Annie had another sonogram this week to check on the blood clot in her arm. Last month's sonogram had showed no change, but this week the sonogram showed that the clot is "resolved or nearly resolved" so she no longer needs her twice daily shots of Lovenox! We are rather surprised to have it resolve so quickly, but feel so relieved! That arm has been cleared for vascular access now, which is a huge blessing!

Because Annie's platelet levels haven't been stable over the past several months, they will continue to monitor that. She'll have more blood work done just before Christmas, so we'll see what her level is and go from there. If her platelets are too high, she will be more prone to additional clots, so they are going to keep a close eye on her lab values over the next while.

With no more injections, her thighs will also be able to heal from the abscess and hematomas that she's developed from the injections. We are so grateful for good news this week! Now if she can only get over her cold...

Brain MRI Results

Annie had her MRI of her brain last week. She had to be sedated for it and receive contrast through an IV. They had to try 4 different places in order to finally get an IV that worked. Her left arm is off limits for IVs since she has her blood clot, and she had had lab work done the day before which required 3 tries in her right arm, so vascular access was rather limited.

Annie's last MRI of her brain was probably done when she was just 2 years old. Or maybe she had another a couple years later, but either way, it's been a long time and the hospital here had no previous MRI to compare to since her others had been done in Texas. Although the system showed that she had a CT scan of her head done here in 2014 which we don't remember. She probably had that done when she had her terrible infection in her spine.

This new MRI report is a bit more detailed and specific than her MRI when she was little. I remember learning all of the medical terms of the findings 17 years ago when Annie had her very first brain MRI before she was even born. Terms like polymicrogyria, heterotopias, agenesis of the corpus callosum, arachnoid cysts, choroid plexus papillomas, etc. (My Aicardi friends will understand all that, you others can ask me later or go google the terms.) ;)  Reading all those terms again takes me back to the day we heard them all for the very first time, feeling stunned, overwhelmed and grieving the loss of our child. Now I can't imagine a life without Aicardi Syndrome!

So the bottom line is that nothing has really changed and there's nothing to worry about. It's still fascinating to read about all the malformations Annie has in there. It's easy to forget really how many different issues are wrong in her brain! Next time we're at the clinic, we'll ask to see the actual images, which is always very interesting since there are so many malformations.

The one interesting point from this latest MRI report is that it says she has left cerebellar atrophy versus dysplasia. So that means that a part of her brain has weakened over time as opposed to that area having just developed more weakly originally. And they can also tell from the MRI that her seizures are coming from the areas of polymicrogyria--which are the areas where she has extra folds in her brain, but those folds are really shallow and not as deep as they should be. So that's interesting to see and it makes sense that those areas would be a source of seizures.

It's fascinating to think about what Annie really comprehends and experiences with her brain so jumbled. It reminds me how much of a miracle it is that she understands as much as she does, that her seizures are as well controlled as they are, and that she is as happy as she is. After her first MRI before she was born, no one expected her to live after seeing the condition of her brain. But here she is, a happy 17 year old! I'm so grateful for her!

Much Better Numbers!

I don't know what difference a week made, but Annie's lab values yesterday were much improved! Her WBC came down from 15k to 8k! And even her platelets dropped from over 900k to 588k! Annie still has the abscess in her leg with some lingering swelling, but it's no longer sore for her. We'll just continue to watch it.  Next on her agenda is her brain MRI next Wednesday.

Back to the ER

After a great first week back at school, we ended up having to take Annie in to the ER last night. As we were changing her, we discovered that where we give injections on her leg was red, swollen, and hard.  We suspected she must have an abscess that would likely need to be drained or at least require some antibiotics. 

They got sonogram imaging of it at the ER and determined that she does have a fluid-filled sac about 0.5x1.5cm in size. The affected part of her legs is more like 4"x2" in size, but that appears to just be inflamed tissue in response to the abscess.

The ER doctor consulted with hematology, given Annie's circumstances, and they decided it would be best to just watch it over the weekend.  Since she is on lovenox, draining it could potentially pose some risks. And since they don't know if it's bacterial, or what bacteria are there, we want to avoid broad spectrum antibiotics, given Annie's history. There is a possibility that it may resolve on it's own in a few days. So we are just watching it and will talk with hematology on Monday.

Hematology Update

I took Annie to her hematology appointment today. She had her sonogram done yesterday of the blood clot in her arm. It hasn't changed much, but is maybe a tiny bit smaller than last month, but not by much. Her left axillary vein is still occluded and her basilic vein is still nearly occluded.

The twice daily lovenox shots we give her are to help prevent more blood clots from forming because the blood becomes turbulent as it passes over this clot and it can cause additional clotting. They don't anticipate her blood clot to dissolve or go away. We are simply waiting for one of two things to happen with it... 1- wait for the veins to scar over from the clot (other veins would then compensate for the loss of those veins) or 2- wait for the blood clot to get more stably attached to the vein wall. As more time passes, the chance of the clot dislodging or moving decreases. There would still be a narrowing of her vein at that point, but the risks associated with the clot would be lower.

We did lab work yesterday and got those results today as well. Annie's white blood count is back up to just over 15k. And her platelets are over 900. Not good. She is not having symptoms of any kind of illness, so they tested her urine and so far the initial results look great--negative for a UTI--but they will send it for culture as well. The hematologist is concerned that her platelets are so high because that puts her at an even more increased risk of developing clots. He also noticed some vascular patchiness on her left hand that he said is concerning, but told us just to watch it and contact him if it gets worse.

Due to these changes, he wants us to repeat the blood work in a week to get another CBC and check her lovenox level. He may have to make some changes to her treatment, but we'll wait to see what the results are next week. Otherwise, we'll return in 6 weeks for another sonogram and consultation.

Back to School!

Annie made it back to school today and stayed the entire school day! We're so grateful she's finally doing better and is getting back to her normal self! I think she was glad to get back, and it sounds like her friends were happy to finally see her again!

It was a strange day for me. After caring for her full-time for the last 5 months, it was bittersweet to send her back to school. I kept feeling like I needed to go into her room to check on her, to change her, or do some other part of her daily routine. But I'm so glad she's back at school where she can have a lot more fun than hanging out with me at home! I just hope she stays away from any sick kids at school now!

A Pretty Good Weekend

Annie has had a pretty good weekend. We're trying to get her up at a more regular time each day, so she's taking some cat naps during the day, but otherwise doing pretty well. Even her drooling has slowed down. So we are planning to send her to school tomorrow! School transportation picks her up at a dark and early 6:50am! So we'll see how she does.

Hopefully she'll make it through a full school day tomorrow, but Tuesday and Wednesday will be shorter days for her at school since she has appointments at the hospital those days. She has her ultrasound of her blood clot on Tuesday and we meet with the hematologist on Wednesday. So I think we're getting a little bit closer to being back to our more normal routines!

One Sleepy Girl

After suddenly stopping the phenytoin on Monday, Annie did have one day of increased seizures, and long seizures, but she now seems to be back to her baseline as far as the seizures go. She is back to having her regular spasms and startle seizures. If only Molly wouldn't sneeze so often and abruptly!

The big change the last couple days has been Annie's sleepiness. The last two mornings, Annie has slept in until 11:30am, which is not like her! I haven't dared wake her up because I don't want to trigger seizures, and I figure her body must need the sleep. Once she wakes up, she's still a little tired it seems, and she's not quite as active with her toys or as vocal as normal. We wonder if this is still a side effect of coming off the phenytoin so suddenly.

Annie is still drooling like crazy and has a lot of thick phlegm that is requiring more suction than normal. We are hoping its just from the tail end of her cold. We had her IEP meeting at the school on Wednesday, and are looking forward to transitioning her back to school, but I'm not sure how they will keep her dry! Her seizures seem stable enough for her to return to school, but I also don't know how she will tolerate getting up at 6:00am when she's been sleeping in until 11:30 each morning. We are going to try to get her back on a more normal sleep schedule the next few days with the hopes she can return to school on Monday, even if she goes late. She won't have full days of school next Tuesday or Wednesday anyway due to more appointments at the hospital. So hopefully we can do a nice easy transition to school next week.

Justin's Talk About Annie

Justin decided to let me share his talk that he gave on Sunday in church if you are interested in listening. The first half or so is about Annie's diagnosis, abilities, how to interact with Annie, etc., and the second half is a little bit more about how the gospel of Jesus Christ applies to Annie's life and brings us peace and comfort as we face challenges with her.

Slight Change of Plans

Due to the rash that Annie has developed, our neurologist has instructed us to immediately stop the phenytoin. It's usually best to do a slow wean over a couple weeks, but our wean took place over 36 hours! This does put Annie at an increased risk of more seizures, so we'll need to keep an extra eye on her the next few days.

We've had to reschedule Annie's IEP yet again, due to this change. We won't be able to send Annie back to school until we make sure her seizures are stable. We do plan to have her IEP on Wednesday now, whether she returns to school sometime this week or not.

Annie has been feeling a bit better today with less congestion and not as much need for suction. So things in that area seem to be improving.

A New Rash

We noticed this morning that Annie has developed a rash over her whole torso. By this evening, it had spread to her arms. This kind of rash is a somewhat common side effect of the phenytoin, after being on it for a while. Ironically, we had decided yesterday that we would start weaning her off of it today, before we knew that a rash would develop. The prescribed wean would take a full two weeks until we could completely stop the phenytoin, but now with this rash developing, we suspect we might need to do a faster wean. We'll be in touch with Annie's neurologist tomorrow to figure that out.

Even after having only one lower dose of the phenytoin, Annie has had more seizures this evening. We're not sure if they would have happened anyway, or if they are due to the drop in phenytoin. A risk of stopping phenytoin too quickly without weaning off of it slowly, is that Annie could more easily develop seizures. So we may need to find a different seizure medication to take its place until things stabilize.

We had hopes of Annie returning to school on Tuesday, but we're not sure if that will happen. They probably won't let her come to school if she has a rash, and if her seizures are increasing, it's best if I can monitor those with her at home. We still have her school IEP meeting scheduled for Tuesday, so we think we'll keep that in place just in case, and then her IEP will be ready for whenever she does return to school.

In better news, Annie slept through the night last night, for the first time in 2 1/2 months! We also attended church as a family for the first time in that length of time as well. Annie is still far from 100%, but Justin spoke in church today so we all wanted to go hear him. He was asked to talk about Annie to help the congregation understand her Aicardi Syndrome diagnosis, her abilities, how to interact with her, and some of the things we have learned from raising her. I was worried that I would have to sit in the foyer with Annie, so I decided to record the audio from his talk. I'll have to ask Justin's permission, but I may try to share it here on our blog, since he did such a great job.

Plugging along...

Before I get into a little update about Annie, I thought I'd let you know that Justin was asked to speak about Annie at our church service this coming Sunday. He'll be sharing information about her diagnosis, recent illness, her abilities, how to interact with Annie, as well as the blessings she brings to our family. All are welcome to attend. It will be on Sunday, October 13, at 12:00-1:00pm at The Church of Jesus Christ of Latter-day Saints, 12009 S. 84th Street in Papillion.

Annie is still doing about the same. There's not much new to report with her health.  She is still struggling with congestion from her cold and we're dealing with a lot of drool. Her increased secretions may be from her cold, but she also has developed a canker on her tongue which increases her secretions and makes her want to stick her tongue out all the time.

The swelling in her eyelids is persisting, but the swelling in her feet is coming and going, along with her distended tummy. We visited her pediatrician yesterday, and he didn't seem concerned about her swelling. Her blood pressure is good and her kidneys seem to be working well and have recently been scanned multiple times, so he attributes the facial swelling to her cold.

The pediatrician checked Annie's urine yesterday and the UA results were really good, but we'll have to wait a few days for the culture results. She also had a plug in the catheter that is being sent to the lab, since that was unusual and a bit suspicious. She's still having a couple seizures each day, mostly the outbursts of either crying or laughing, which seem to happen mostly in the middle of the night. We won't start weaning off the phenytoin until Annie is over her cold.

Last night Justin and I took Annie with us to the Med Center to attend a class on transitioning to adulthood. They offer a full series of classes to help parents like us plan for our special needs child's future after high school. While the classes are incredibly helpful, they are a bit somber to attend. They are a reminder to every parent in the room just how different of a life our children are living from what we had once hoped and dreamed to experience with them. But that room is also filled with parents who are full of resiliency, have incredible love for their children, and are powerful advocates, dedicated to providing their children with the best future possible. It's easy to look around the room and think, "These are our people--they know our struggles."  It was also fun to see another Aicardi mom tonight that we're friends with. There are very few of us in the Aicardi world, so it's nice to see them when we can.

Last night's meeting was on SSI benefits and managing our adult child's finances and benefits. Other class topics include learning about going to court to get guardianship, DD services, day programs for adults, continuing education, insurance and waiver programs, transitioning to adult medical providers, and so on. There is so much to learn!

On another note, the biggest highlight of my day today was that a sweet member from our church came with her professional cleaning crew to clean my whole house! She owns a cleaning company and called me to say she wanted to come clean our house! I was so humbled and grateful, but I had no idea that she would be bringing two other women to help her and they would spend more than 4 hours cleaning! My house hasn't been this clean since we moved in! We are SO thankful for such meaningful service! With Annie in the hospital and then even at home with her requiring so much care, we often just don't have the time or the energy to spend time cleaning. I was so embarrassed to have them see our dirty, neglected house, but they all just happily worked for half the day! What a huge blessing!

A Terrible Cold

We've suspected for several days that Annie has a cold that is causing some infrequent vomiting. She made it very clear to us yesterday that she does in fact have a cold. Yesterday must have been her peak day. She struggled to deal with the congestion, and I used suction a lot to help her breathe more easily. We're keeping her on Robitussin around the clock, which is helping, but she's still vomiting a couple times each night due to the congestion.

Annie has also developed swollen eyelids. This might be due to her cold. But yesterday her feet also became swollen. I've been keeping her up in her chair all day the last few days to help with congestion, so I wonder if that's why her feet are swelling. That's not typical for her though, even after being in her chair all day, so we do worry about a kidney or heart problem. It's not normal for her to retain fluids like this. So we're not totally sure what's going on.

I cancelled her bladder scan study that was supposed to be yesterday because of the illness--we would not have gotten accurate results. She will still have the study done, but right now we don't have a date.

I've had to reschedule her brain MRI that was supposed to be tomorrow, because of her cold as well. Since Annie has to be under sedation for the MRI, it's not safe for her to have all this congestion when sedated, otherwise she could choke or aspirate on it. Unfortunately, the next available time isn't until November 6th. But the MRI isn't urgent, so we'll just wait until then.

Annie will see her pediatrician on Thursday to recheck her urine and evaluate her edema. We're making tentative plans for her to return to school next Tuesday, but we'll see how she does until then.

I was able to take Annie to the high school today to be in her senior class picture. I really wanted her to be included and be able to be remembered. It's one of those "chronic sorrow" experiences to have her graduating from high school. In no way is she a typical high school senior, and we are missing out on a lot of typical experiences that we won't ever get to have with her. It's strange to think that all the other seniors Annie's age are driving, dating, and making their college plans. It's such an exciting time of life for them and their families, but nothing will be changing for Annie. She is our forever little girl. But I'm so grateful that she is doing as well as she is, that she can be at home with us, and that we can enjoy her smiles and giggles. It's always humbling to think about how Heavenly Father has entrusted her to us.

I'm also grateful for all of Annie's good friends at school. She has so many teachers and friends there, some who she got to see today. It warms my heart to know that she is missed at school. Hopefully she will be feeling better soon and be back to school!

Gelastic Seizures

Annie's vomiting has lessened as we've given her Robitussin--we think she has just had a hard time dealing with congestion. However, Annie is now having more gelastic seizures. It's hard to put a number on how many she's having each day. Gelastic seizures are seizures that present as sudden, unexplainable,  outbursts of emotion.

Annie's gelastic seizures typically present as sudden laughing and giggling, sometimes so hard that she can't catch her breath. However, she has also had some occasional outbursts of crying and sadness.

We're relieved that the vomiting hasn't continued, but we're still using suction regularly. We'll have to do her bladder scan study another time, but she's still scheduled for her brain MRI on Wednesday. And soon we'll start weaning Annie off her phenytoin, so we'll see what her seizures start doing then.

The great news is that our furnace started working again! Justin fiddled with it and it now seems to be working just fine. So that's a huge relief!

Fewer Seizures, More Vomiting

I feel a bit like a broken record with my posts lately. But several people have been asking about Annie, so I thought I better post an update, even though I don't have much new to report.

Annie is only having maybe one seizure a day that we are noticing. We're very happy with her level of seizure control right now. That may change as we wean her off the phenytoin in a week or so.

Annie had a pretty great day yesterday and went a full 24 hours without vomiting, so I thought she was over that. However, overnight last night, Annie started throwing up again. She is definitely congested, so we're trying some Robitussin to help with her secretions and congestion, but so far, I'm not sure it's helping much.

Congestion is really hard for Annie to deal with since she can't blow her nose, so we're using our suction machine regularly to try to keep her comfortable. I'm still not sure if the vomiting is related to the congestion or not. Her vomiting seems less frequent during the day while she's upright in her chair, so maybe her cold really is to blame.

Due to her vomiting, Annie isn't voiding normally, so I had to cancel her bladder scan study that was supposed to be this Monday. There's no reason for us to spend all day doing the study on a day when she's not her normal self. I want the study to be as accurate as possible and be representative of her at her healthiest. It's not an urgent test anyway. We're really just hoping that Annie is well enough by Wednesday to move forward with her brain MRI. Since it's under sedation, she can't be vomiting.

It actually worked out well for me to cancel Annie's all-day appointment on Monday because this morning our furnace broke. We just had it repaired six months ago, so we're surprised to have more problems with it already. A repairman is able to come on Monday, so it works out well for me to actually be home that day instead of being at the hospital all day as previously planned. Fortunately, our temperatures aren't too very cold this weekend. We only turned on our furnace yesterday for the first time this season, mostly just to stay warm overnight. We have some awesome gas fireplaces that put out a ton of heat, and an extra blanket at night should be sufficient.

This weekend is the worldwide General Conference for our church, The Church of Jesus Christ of Latter-day Saints. I'm always excited for conference, but this time I am looking forward to it even more. I think our family really needs this weekend full of uplifting and inspiring messages from the prophet, apostles, and other church leaders. I hope it is a weekend of increased faith, peace, hope, and coziness.

Vomiting is Back

We thought Annie was starting to do a little better. Her seizures have been so mild that we usually don't catch them at all unless we happen to be looking at her hand or holding her hand.  However, last night Annie started vomiting again. She vomited again this morning right after getting her morning meds, so I waited a little while for her tummy to rest, and then re-dosed her seizure meds. I waited a full 2 hours for those to stay down, and then started her on some Gatorade.

While her stomach was empty earlier today, Annie had a big seizure followed by strong retching. If she had had anything in her stomach, I'm sure she would have thrown up. It was almost as if the seizure induced the retching, but I'm not sure.

Annie tolerated the Gatorade all day, so late this afternoon I started her back very slowly on her formula. She did well for a few hours, so I increased the rate, but then about two hours later, she threw up twice. So we went back to Gatorade and will continue that through the night and see if tomorrow is better.

We really don't know what could be causing this vomiting. Today was her last day on the antibiotics, so her UTI should be totally cleared up. Molly has had a cold, so we wonder if Annie has caught that cold from Molly, even though Molly isn't really home much. Colds for Annie can cause vomiting because she can't tolerate any amount of congestion. She can't blow her nose, and ends up gagging on secretions and phlegm which make her vomit. She doesn't sound congested tonight after putting her to bed, but we'll definitely be trying some decongestants tomorrow if the vomiting continues.

So we're back to our goal of just staying hydrated. Dehydration can affect Annie's seizures, but also being off of her normal diet, or anything else that disrupts her routines can also increase her seizures. So hopefully the vomiting doesn't persist. One more week until her brain MRI. Pray that she is well enough for it to move forward. Since she'll require full sedation for the MRI, she has to be healthy and well and not vomiting. She also has to be very hydrated for her bladder scan study coming up on Monday!

A Better Day

Annie had a little bit better day today. Her seizures have decreased dramatically, thankfully. She is still having very mild seizures occasionally where she is clenching and then twitching her fist, but that is a huge improvement over the more dramatic and frequent seizures we've seen this past week.  We aren't sure if her Sabril has finally caught up and is working again, or if it's the Dilantin that has brought about this improvement. We'll find out in a week or two when we start weaning her off the Dilantin.

For the last couple days Annie has been having quite a bit of irritability and agitation. We aren't sure if it is due to pain, disruption of her routine and regular sleep schedule, withdrawal from not having as much Ativan lately, or a side effect of the Dilantin. We are trying to keep her off the Ativan so she can get more restorative sleep and get back into her regular routines which will hopefully help.

Tomorrow is Annie's last day on antibiotics for her UTI, which will be nice. Her antibiotic has it's own negative GI side effects so we're glad to almost be done with that. Overall, I think Annie is starting to improve, but the true test will be when we wean her off the Dilantin. We may have to start experimenting all over with another anticonvulsant to add, but we really pray that her Sabril alone will be sufficient.

Bad Days and Better Days

We've been on a roller-coaster the last few days. On Friday, Annie ended up sleeping the entire day. She woke up early but fell back asleep around 8am. She was awake for only about 15 minutes around noon, but was very spacey and unfocused. She then slept solidly until we finally woke her up around 10pm, wondering if we needed to take her to the ER.

I kept her on an air bed in our family room all day Friday to monitor her and she was rather unresponsive to everything going on around her, although she was still having some mild seizures. Her breathing was obstructing through the day, so I had to keep a close eye on her and keep her positioned just right.

That night, we began to be worried that she still hadn't woken up all day. As a pharmacist, Justin knows that phenytoin, which Annie just started Wednesday, is metabolized very differently in each person and if not metabolized correctly, can cause a coma state, and even be fatal if levels build up too high in the body. Justin calculated what dose of phenytoin Annie should be on based on her weight, and we discovered that her dose is almost twice the recommended amount. We thought we better get her to the ER, but once we got her in her wheelchair, she started waking up. She was very groggy and immediately started having more seizures, meaning she must not have too high a level of phenytoin in her system. She was rather groggy and grouchy, but eventually calmed down and went to bed for the night.

Saturday was much different. We're trying to not give Annie Ativan with the way it might be reacting with the phenytoin, causing her to be so incredibly sedate all day. So Saturday we just tolerated more seizures, although they seem to be much milder. It's possible that Annie had more seizures than we were able to see though, since her jerking was less pronounced, it was harder to catch them all unless we kept our eyes continually on her.

Today, Sunday, Annie seems to be doing maybe a little better, going even a whole hour or two without seizures, unless we are just missing mild ones. But other times, she is still seizing 5-6 times an hour.  She is also rather irritable and cranky at times for reasons we don't understand.We think she might just be wearing herself out from the seizures.

Each day seems to be so different for her, we just don't know what each new day will bring. In a couple weeks, we'll need to take Annie off the phenytoin, so we pray that her Sabril will have caught up by then and kicked in without an increase of seizures as we wean off the phenytoin.

We don't have any appointments or testing this week, that we know of now. But the week after will be a busy one with her bladder scans, brain MRI, and urine check. Then in a couple weeks she has another sonogram of her blood clot to monitor it. We appreciate all the continued prayers for her healing.

Still More Seizures

Annie's loading dose of fosphenytoin lasted about 7 hours last night before some very subtle seizures started back up. With Aicardi Syndrome, we know that we will never get complete seizure control, but we were surprised to see even mild seizure activity after such a huge dose of anticonvulsants in the ER. Annie was incredibly sedated last night and had difficulties breathing, so we kept a close eye on her.

This morning the seizures were back as frequent as ever, occurring every 7-15 minutes. She got her first dose of oral phenytoin, so I waited to see how that might work. She continued seizing through the whole day about every 7-20 minutes, but the seizures today were much more mild than previous days, and a bit more tolerable. They didn't seem to bother Annie much until about 3:00 today when she started crying and fussing. I think she is so tired but can't get any sleep because the seizures keep her awake. So I finally gave her a full dose of Ativan to see if she would calm down enough to be able to sleep. So far it has helped her seizures, but she is still just as agitated and fussy.

We plan to continue the phenytoin to see if she just needs a bit more time on the oral form, but I think we'll have to supplement with Ativan to keep things more under control. We thought that by now Annie would start to build up enough Sabril effect in her brain that things might slow down, but apparently we're going to have to give it more time still. Annie still has a lot going on in her body and is not even close to being in her normal routines yet, especially with her sleep. Unfortunately, all of those disruptions can also contribute to more seizures.

With all of her seizures lately manifesting on the left side of her body, we are concerned that she has some kind of problem in the right side of her brain. That could be anything from a blood clot, damage from an undiagnosed stroke, increasing intercranial pressure, a tumor, or localized brain damage from seizures. We don't really know. And we don't even know if there's a new physical change in her brain causing these new seizures at all. It still might just be the sudden loss of her Sabril from all her vomiting that is to blame. It's just so hard to know for sure. The MRI of her brain is on October 9th, but that seems like such a long ways away. We are anxious to get that done and see what's going on in there, if anything.

Today I took Annie to see her gastroenterologist. He is fantastic and sat and talked with me at length about how Annie has been doing since being discharged. Even though her hemoglobin is still low, we are going to hold off on starting back on her iron supplements because they can be pretty hard on the GI tract. We suspect that Annie just needs more time on her normal diet again to recover. We'll check her blood levels in another month to see where she is, and then possibly consider starting the iron again. Next month we'll also re-check her ESR and CRP, and some other things as well. We also decided that because Annie has a history of c-diff with antibiotic use, we'll start her on probiotics to help keep her gut bacteria well balanced while we're treating another UTI.

We're so eager to get her feeling better again. It's becoming physically and mentally taxing as Annie is requiring continual 24 hour monitoring and care and lots of appointments and testing. Thankfully, Molly and Lexie have been extremely patient and responsible in taking care of themselves and even helping quite a bit around the house. We much prefer having Annie at home than at the hospital. We just keep praying that these seizures will let up and Annie will be able to get some rest soon and get back to her normal self.

A Long Day at the Hospital and ER

Annie had her routine annual CDC appointment today where we meet with several of her specialist and therapists. From there, her neurologist then sent us to the ER. Here's a run-down of what we learned today...

First at CDC, we met with our orthopedist. It was quick and simple. Annie's spinal x-rays look great and he's not worried about her back. He said the chances of her back getting infected again this far out from surgery would be extremely rare.

Next, we met with our urologist, but since we've been in such close contact with him this week about Annie's new UTI, we didn't really discuss anything new. We still plan to have her back in clinic in a couple weeks to do regular bladder scans after every wet diaper over a full day. So far, with Annie hydrated again, she seems to be voiding just fine. But it will be good to see how efficiently she's emptying her bladder so we can then make informed decisions about regularly cathing her.

We then met with the developmental pediatrician who also doubled as our metabolics doctor today too. We got Annie's dexa bone density scan results which are pretty good, so no changes there. We asked about a lot of little random things, like all the injection sites on Annie's thighs that have left drops of blood under her skin. Every injection site is still clearly visible, leaving her thighs looking like a pin cushion from her twice daily shots she needs for the next 3-6 months.

Our dietician did her regular consultation with us to make sure Annie's getting the right amount of nutrients, minerals, and vitamins from her formula and her additional supplements. We always talk about calorie needs and assess her weight, and make sure her diet includes enough fluid for her needs. She's been a little low, so we'll be increasing her fluid intake a bit, but not adding more calories.

Our OT assessed Annie and actually told us about a clinic at UNMC that can help us transition Annie from her pediatric providers to new adult providers once she turns 21. Although that's a few years away still, it's a big deal to us since we love her pediatric providers so much and they have such a long history with her. It will be really hard to transition to new providers who don't know who she is or understand her unique history, or have ever even heard of Aicardi Syndrome. It will be a bit overwhelming, so we're grateful to know of this resource at UNMC.

The hospital pharmacist then visited us to make sure they have everything accurately in their system with all the meds Annie is on. That's always a quick visit.

Next was our neurologist, who we've been in close touch with the last several days with Annie's seizures being out of control. We talked at length, and Annie's last Ativan dose wore off, so our neurologist got to see her seizures first hand and how they recur every 10 minutes or so. She decided to have us go to the ER once we finished at CDC, to get a loading dose of fosphenytoin to stop the seizures since the Ativan isn't keeping them away. The Ativan has been stopping her seizures, but they come right back as soon as the Ativan wears off. Once she's had the loading dose in the ER, we'll start Annie on oral phenytoin (Dilantin) for just a couple weeks until her Sabril can catch back up in her system and start working again. The phenytoin has a lot of really nasty side effects, so we don't want her on it long. We'll wean her off of it in a couple weeks and hope the Sabril is working by then. We also talked about doing smaller doses of Ativan more frequently if we are still seeing seizures on the phenytoin. If Annie's seizures are still not under control once she's off the phenytoin, we'll discuss getting another drug on board, probably Onfi. But we're really praying that the Sabril kicks in here soon and we can get off the phenytoin without having to add anything else back in the mix. We still have an MRI of her brain scheduled in a couple weeks, so that will be interesting to check, since she hasn't had a brain MRI in several years.

So once the neurologist had seen Annie's display of seizures, we gave her more Ativan. Then our PT came in to visit with us. We didn't have any PT concerns, so it was just a simple visit and we mostly just chatted about other stuff. We love our PT and how personable she is. We make good friends with a lot of these doctors and therapists after seeing them regularly for so many years. That's why it's going to be so hard to leave them when Annie turns 21 and has to transition to adult providers.

Next was a good visit with our social worker. She talked to us some more about Annie's transition to adulthood and reminded us of how we'll need to apply for SSI for Annie soon when she turns 18. Between now and the age of 21 is a big transition time. Annie will have to move from her Medicaid waiver being managed by DHHS, to the League of Human Dignity. We learned that the waiver will pay me to be Annie's caregiver when she turns 19, and we can get our attorney fees covered by the hospital's legal aid department when we have to apply for guardianship of Annie. There's still lots more to learn about this transition, particularly whether to stay with waiver services or move to DD services when she turns 21. Both programs are great but offer very different things, so we still have a lot to learn before then. Our social worker is so great to point us to different organizations in the community that are excellent resources for parents like us.

We passed on seeing our pulmonologist today because we really don't have any concerns there. Annie's breathing is suppressed while on these sedating meds, so we've just started using her CPAP when she needs it, even though she hasn't needed it for several years now. We're comfortable with using it but hope its not a long-term thing.

From the CDC clinic we went down to the ER for Annie to get her loading dose of fosphenytoin. They had a difficult time starting the IV because Annie has had so many IVs lately that all her veins are pretty much shot. This fosphenytoin has to be given in the ER because there are risks with getting this large loading dose. So Annie had to be on monitors to watch her heart rate, respirations, oxygen sats, and blood pressure. This medication can cause low blood pressure and arrhythmia. Her blood pressure is pretty low even when healthy. She started at 89/60 and it dropped to 71/48, but once it got back up to 79/55, they let us go home. All her other vitals looked good, but her breathing has become very suppressed and she's easily obstructing.

Between this fosphenytoin and the Ativan from this morning, she quickly zonked out. We stayed in the ER for a few hours to monitor her and then headed home. Wednesday evening now, she's still passed out from these meds, but she hasn't had a seizure since about 12:45!! That's amazing! But I'd like to see her a bit more alert and awake and breathing easier.

While she had her IV in, they drew her labs to get a CBC. The ER doctor came to give us the results and thought we were a little crazy with how thrilled we were that her white blood count is down to 10k!! It hasn't been in normal range for the last 4-5 weeks! So that is some of the best news we've gotten all day! Annie's hemoglobin is 10 with a hematocrit of 33. So while that's still pretty low, it's not terrible. We suspect she just needs some more time to recover in that regard.

So it's been a super long day at the hospital and we are all rather exhausted. We have some mixed feelings about today... We're so relieved that Annie isn't having seizures, but we know there's still a chance they start coming back. And it's rather unsettling to see Annie so sedated with such suppressed breathing and hear her obstructing. We were hoping that she could soon sleep alone in her room without one of us in there throughout the night, but she'll require one of us monitoring her more closely again tonight. We'll see how her regular oral doses of phenytoin affect her, and hopefully her seizures will get controlled, without any breathing suppression, so both Justin and I can start getting more sleep each night. We mostly just want our alert and happy Annie back!

September 23rd: New Culture Results

Annie's urine culture results came back today and surprisingly, she is NOT growing the same bacteria as with her previous UTI she was treated for in the hospital. This time her UTI is caused by enterococcus faecalis. This is a new one for her. It is one of the most common hospital-acquired bacterias. It is also resistant to almost all antibiotics, including the one she's been on the last 3 days as we've been waiting for culture results. So the cefdinir she's been on this past weekend has not been treating it at all. Annie is now on amoxicillin, one of the very few drugs which the bacteria are susceptible to. Hopefully with this change, she'll turn the corner and start to feel better in a few days.

Because of these UTIs, the urologist is concerned that Annie may not be emptying her bladder fully. She has a neurogenic bladder, but her annual urology testing always comes back with pretty good results. We suspect that Annie just hasn't been emptying her bladder well lately because of her dehydration episodes. But we are now scheduled for a bladder scan study in a couple weeks so we can see if there is any (and how much) residual urine in her bladder after she voids. We'll get to hang out at the urology clinic all day just waiting for those wet diapers and then they'll immediately scan her bladder to see how efficiently she's emptying. They'll need to check her after a few different voids, so we'll be there all day for that. If Annie is consistently not emptying, we suspect that they will suggest we catheterize her at least daily to make sure her bladder gets emptied. I'm really hoping that once we get her healthy and well, she'll pass those bladder scans with flying colors!

Annie's seizures are still about the same today. She's having seizures about every 10 minutes once her Ativan dose wears off. We've been giving Ativan about every 10-12 hours and just tolerating seizures for a few hours between doses, until they increase in strength and we give more Ativan. But today our neurology clinic said we can give the Ativan regularly every 6 hours, so we'll try that and see how it works. The seizures seem to start popping up about 5 hours after the Ativan is given.

It's a fine line trying to balance seizure control with sedation. Yesterday Annie fell asleep at 2:30 in the afternoon and didn't wake up again until 4:30 the next morning. She still started having seizures in her sleep late last night several hours after her Ativan wore off, so she got more Ativan. She was so sedated that she kept obstructing in her sleep and we had to put her CPAP on her, which she hasn't needed in years. I was glad she got such good sleep, but it's hard seeing her so groggy and spacey, or seizing, when she does happen to be awake. Now that we know her UTI is still raging with ineffective antibiotics this weekend, we really hope that the seizures will also calm down as the UTI gets treated. But we still think the biggest help will just be time to allow the Sabril to become effective in her brain again.

Continued prayers for her seizures to stabilize and for her new antibiotic to eliminate the UTI, are very much appreciated.

September 22nd: Still Seizing

Annie is continuing to have seizures. We are giving her Ativan twice a day to keep the seizures down, but 5-6 hours after each dose, the seizures start to mildly come back and build up until we give her next dose. The Ativan is very sedating and Annie is almost non-responsive when she has it in her system. It can also suppress her breathing, and since Annie easily obstructs, we watch her really closely around the clock. So Annie is not awake much and when she is, she is groggy from the Ativan or worn out from the seizures.

This frequency of seizures is not normal for her and she hasn't had this level of seizure activity since she was a baby. What we think is going on is that we are finally seeing the effects of her lack of seizure meds about 3 weeks ago. When she was first admitted to the hospital on August 26th, Annie had been throwing up for almost a week, and she continued to throw up for about another week in the hospital. During this time, she often vomited after getting her morning and evening meds, including her regular seizure medication. It's hard to know how much of her medication was thrown up and how much was absorbed, but we believe she threw up probably the majority of her seizure medication over several days. (Her seizure med has no IV formulation, so there was no other way to deliver it.)

Annie's seizure med is called Sabril or vigabatrin and it has a delayed response in the body. Justin could give you much more information regarding how Sabril works with enzymes in the brain to prevent seizures. But basically, when a person starts Sabril, it typically takes about 3 weeks for the medication to reach its full therapeutic level and become effective at reducing seizures. So we believe that Annie's lack of Sabril due to vomiting about 3 weeks ago has finally caught up with her and we're now seeing that effect. So we anticipate, and hope, that in a few days, Annie will come out of this Sabril trough and it will start being more effective for her. We still plan to update her neurologist tomorrow and see if different adjustments need to made, even just temporarily, to get Annie through this high seizure period until her Sabril fully kicks in again.

Annie is doing ok except for these nearly constant seizures and the heavy sedation that the Ativan brings. She's on her oral antibiotic for her UTI and we'll find out tomorrow for sure if the bacteria are susceptible to this particular antibiotic. Annie is having higher temperatures than normal, but not yet in technical fever range. She's continuing to tolerate her feeds well and is staying hydrated. If only we can get these seizures to stop. We miss our perky girl.

This week we meet with nearly all her specialists at her annual CDC appointment, so that will be great to troubleshoot and brainstorm all together. Then the following day we visit her GI doctor too. We are really praying that this week is a week of big improvements. Thank you so much for all of your continued prayers.

September 20th: UTI is Back

Annie had a rough night last night. She was uncomfortable and crying for much of the night and continued to have seizures. We gave her more Diastat in the middle of the night which worked for a few hours before the seizures came right back. We decided that once we got the kids to school, we'd have to take her to the ER.

However, Annie's seizures subsided once we got her up and as we got ready for the day. So instead of going to the ER where we knew they'd just sedate her, we called her neurologist. She let us know how much more Ativan we could still give Annie at home to sedate her here, which is better than doing it in the ER. She said if we did need to go to the ER, we could request only a loading dose of Keppra which isn't sedating. So we'll keep those options in our back pocket if the seizures pick up again.

The neurologist did agree that we should do an MRI of Annie's brain since we've looked so thoroughly at every other organ in her body without finding any problems. But that MRI won't be until October 9th. I don't think we'll find anything wrong with her brain (other than what we already know from Aicardi Syndrome), but it would be nice to just rule out any potential problems there.

We talked to our pediatrician today and found out that Annie's urine culture is starting to grow some bacteria. Final results won't be in until Monday, but we decided it would best to get some antibiotics on board now rather than waiting. So Annie got 2 big injections of rocephin to kick start treatment, and we'll start her oral med tomorrow and adjust if needed, once we have final culture results.

Since this UTI has recurred so quickly, we're led to believe that it's the same infection she had in the hospital and it just didn't get fully treated for some reason. We discovered today that her last urine check before she was discharged was never sent in for culture! So that's frustrating! But hopefully now that Annie has antibiotics on board again she'll start to feel better, be in less pain, and possibly even have fewer seizures.

It's been a difficult 24 hours with almost constant crying and seizing, but the best news to share is that Annie is now urinating! And she even had a BM today! So hopefully things are headed in the right direction again and we'll all be able to get some sleep again soon.

September 19th Update: TMI Ahead!

This post probably has too much information (TMI), but it's our life and what we're dealing with right now. We are so encouraged that Annie is tolerating her feedings. She is getting her regular diet plus some additional fluid, which is great! The problem is that she is not urinating or having BMs. She is no longer vomiting, so we can't figure out why she isn't urinating.

We visited her pediatrician today to get a urine analysis and culture after learning that she has debris in her bladder yesterday. She did have urine in her bladder this morning, so we know her kidneys are working (plus they were just sonogramed yesterday) but her urine was pretty dark, indicating she must still be a bit dehydrated. So we've pushed more fluids today, but she is still not voiding. The UA today looked good, but it will still be sent for culture.

She also hasn't had a BM since last weekend, so that is concerning as well. We gave her some Miralax today and hope that helps soon. That may be contributing to her lack of voiding as well. We just aren't sure.

Annie has been very fussy today and crying quite a bit. We wonder if she is having gas pains or stomach cramps from constipation, or if she does have a UTI and it hurts when she starts to void so she holds it. We just aren't sure. But it's so very hard to watch her feel so rotten.

When Annie hasn't been crying today, she has been having a unique kind of seizure that is not normal for her. I gave her some Ativan earlier today and she was able to sleep for 2 hours, but then once she woke up this afternoon, the fussiness and seizures started again. She needed Diastat just yesterday, so we're hesitant to give her more again today. We'll just have to continue to see how she does with seizures. We're hoping she will relax and sleep tonight. So far this evening she has settled down a little bit, so we're praying her brain continues to calms down on its own. We'll sedate her with the Ativan or Diastat if needed, but we hate to slow down her bowels any more.

It's so hard to know what is causing what. We wonder if Annie might be having some kind of neurological problem, or if the seizures are in response to the rest of her body not being healthy and well. It's very common for Annie to have worse seizures when she's sick or when she is out of her normal routine. We also wonder if she still has an ileus preventing a BM. There are just so many aspects involved right now and each one falls under the care of a different specialist--her GI doctor, her urologist, and her neurologist.

So tonight we are specifically praying for Annie to urinate, to have a BM, and to have her seizures settle down. We fear that we may end up back in the hospital soon even though she is tolerating her feeds like a champ.

September 18th: More Testing, No Answers

We had a second day of testing at the hospital today. Annie had a renal sonogram and full upper abdominal sonogram. She had her routine dexa bone density scan and a routine spinal x-ray just as part of her annual testing.

Her chest x-ray from yesterday was clear. The nodules in her lungs that were reported on the last CT scan ended up being an imaging flaw from all the spinal hardware she has. So her lungs are good!

Annie's blood work from yesterday shows that her white blood count has gone up a little from last Friday when she was discharged. It's now at 18.9k. And still no one knows why. The rest of her lab values don't look too bad. She still has elevated platelets. Her hemoglobin has come up to 11 from 9 on Friday. Although that increase could just be because she's a little more dehydrated now and her blood is more concentrated.

Her sonograms today looked good showing no problems with her kidneys, liver, gall bladder, or pancreas. She does have some debris in her bladder though, so we'll probably have a UA and culture done tomorrow.

We met with Annie's hematologist today. He thinks we need to have our GI scope her again for a possible GI bleed. We've seen just a few flecks of old blood in her last vomit last night, but not much and only that one time. So if we see more, or a scope shows bleeding, we'll have to stop the injection treatments for her blood clot. However, Annie's vomiting has subsided a bit this afternoon, although she is only getting gatorade at a rate of 40cc/hr. The hematologist will continue to monitor Annie's blood clot and will do another sonogram of it in a month.

We definitely prefer to be at home with Annie, but it has been a difficult two days running around getting testing done and trying to contact doctors by phone to figure out what's going on and what still needs to happen. None of the specialists we're working with are on the same page and trying to relay messages through nurses is really hard, so that has been difficult today. We hope to form a better plan tomorrow when we see our pediatrician again and have him coordinate care among her specialists. We also have Annie's regular annual appointment with most of her specialists next week, so if we can keep her hydrated until then, that will hopefully be a chance to get everyone on the same page and make some progress in her treatment.

Today she is doing a bit better with tolerating some clear liquids, but she's having more seizures again, particularly her gelastic seizures. She had myoclonic seizures this morning for about two hours and needed Diastat. We might need to get an additional seizure medication on board when we meet with our neurologist next week. But I'm hoping Annie's seizures return to normal once we get the underlying problem resolved.

September 17th Update

Our weekend at home all together was pretty uneventful as we tried to recover from three weeks of exhaustion. But last night, Annie started vomiting again. She vomited consistently every hour throughout the whole night. We stopped her feeds but she continued to throw up until about 6am.

We already had a follow-up scheduled with her pediatrician today, so that was helpful. Last night I was looking through all the radiology reports from Annie's hospital stay and noticed some findings regarding Annie's pancreas (a pseudocyst or hypodense lesion, and some other pancreatic issues), as well as nodules that were found in her upper left lung. No one mentioned any of these findings to us while in the hospital, so I asked our pediatrician to read through the reports. He agreed that we need to look into these issues further. He believes a pancreatic pseudocyst would definitely cause vomiting, as would mucous from a lung infection.

So Annie and I headed back to the Children's hospital and had a chest x-ray done, as well as blood work to check various things such as her white blood count, hemoglobin, level of her blood thinner, electrolytes, and pancreatic function. We were scheduled for blood work tomorrow with the hematologist, but we were able to get his labs drawn today also, which will save us some time when we meet with him tomorrow.

Annie was already scheduled tomorrow for her annual, routine renal sonogram and dexa bone density scan, so our pediatrician ordered extra imaging of her pancreas as well.


So while it's been wonderful to have Annie home with us, we still have some unanswered questions and issues. We're really praying we can get her the help and treatment she needs without having to stay in the hospital again. We really appreciate continued prayers for her and our family.

September 14th: She's Home!

We were joyfully surprised last night to have Annie finally discharged from the hospital. That was a long and stressful 19 day stay. We didn't expect her to be discharged quite so soon because she had evidence of internal bleeding yesterday morning, but her labs late yesterday afternoon had improved, indicating that the bleeding must have resolved itself. Since we found some clotted blood in her stomach, we suspect she was bleeding from having the J-tube all twisted up in her stomach and causing irritation.

Once the doctors saw that Annie's hemoglobin had recovered from 8.1 yesterday morning to 9.0 late yesterday afternoon, they said there was really no reason to keep her in the hospital. She is not recovered yet from all the issues she's facing, but we no longer need to be inpatient to monitor and treat her.

Annie needs to receive injections twice daily for the next 3-6 months to treat her blood clot. We'll have several appointments over that time for the hematologists to monitor that blood clot with sonogram until we know it is gone.

Annie's lab work is still not normal, with very low hemoglobin and high white blood count, so we will continue to monitor that with regular lab work as well. Annie's high heart rates have been a little lower lately, but still not normal, and her body temperature continues to drop at night lower than it should. So we will be monitoring all these things from home and taking her to several doctor appointments over the next few weeks and months.

So even though Annie is still not back to her normal self, it is a huge blessing to have her back at home with us while we continue to treat her. Thank you so much for all your prayers and well wishes!

September 13th: Another Setback

We awoke this morning planning to take Annie home at some point today. However, her morning lab work is very concerning. Annie's hematocrit and hemoglobin have taken a nosedive in the last 24 hours. This is particularly concerning because she is on a blood thinner to treat her blood clot. So this lab work indicates that she is bleeding internally somewhere. On a better note, her white blood count came back down to 17.3k which is good, although still elevated.

Annie's blood pressure is continuing to trend downwards as well, and she is still having pretty low body temperatures at night. So Annie is not going home today. We'll need to find the source of bleeding and make sure her hemoglobin and hematocrit are stable first.

I drew out stomach contents from Annie's g-button to see if she has bleeding in her stomach and she did have some dark red blood, confirmed by the lab. They suspect that the J-tube coiled up in her stomach the past few days most likely caused irritation to her stomach and has caused some bleeding there, exacerbated by the blood thinner. The blood was dark red and looked like older clotted blood, so they suspect that that bleeding is possibly resolving on its own.

They will check Annie's hematocrit and hemoglobin again this afternoon to see if they are stabilizing or continuing to drop. Her lab work shows that she is working hard to produce more blood, but her body just can't seem to keep up. If she drops to about 7.6 or lower (she's at 8.1 this morning) then they will give her a blood transfusion and be more aggressive in finding the source of bleeding. They may repeat the upper endoscopy to look at her stomach, or do more imaging to find the source. They would also have to stop the blood thinner until the bleeding is controlled.

Fortunately, Molly made it to Minnesota for her band trip early this morning. But my mom is also leaving to go home today too. We're still praying that Annie will be discharged sometime this weekend or early next week.

September 12th: Getting Closer!

Although Annie's CT scan last night failed to get information about the vessels in her chest, we did learn today that the CT scan showed that her J-tube has become dislodged and is all twisted up in her stomach instead of running down into her small intestines. No wonder it's not flushing well! It's all kinked up in her tummy!

They were able to get a new IV started again this morning so we repeated the CT scan with the contrast actually going where it was supposed to go. Thankfully, there are no other clots or embolisms, other than the one in her upper arm.

We were trained on how to give her blood thinner shots, and tomorrow they will check her drug level to see if her dose needs to be adjusted. We'll also have a consultation with Hematology to find out exactly how long she'll need to receive these twice-daily shots, potentially 6 months or more.

They wanted us to resume Annie's regular ulcerative colitis medication that can easily clog her tube, so we went ahead and gave it to her through the G-port of the GJ tube. Just as we expected, it clogged. So this afternoon, we removed the GJ tube, since the J-tube portion was all twisted up in her stomach anyway, and replaced it with her regular G-button. Thank goodness!

So at this point, there is nothing they are doing at the hospital that we can't do at home for Annie. So we suspect that she might be going home tomorrow! She'll still need a lot of monitoring--her white blood count went up today, so we'll have to watch that as well as some other labs that aren't normal yet. Her temperatures are still dropping slightly at night and she's still having higher heart rates. We hope that those are just side effects from the blood clot, and as that resolves over time, everything else will too. Annie will have several appointments in the upcoming weeks as she continues to be monitored, but it will be much easier to do clinic visits than stay in the hospital! She's not out of the woods yet, but it will be such a blessing to have her home with us again! Thank you so much for all your prayers for her!

September 12th: Life Goes On

Last night Annie went for her CT scan to check for pulmonary embolisms and blood clots in her chest and lungs. It took the vascular access team about an hour to find a vein large enough to tolerate the high rate of contrast that has to be pumped in during the CT scan, but they finally found one and carefully placed the new line under sonogram guidance.

However, when we went to get the scan, Annie cried and fussed as they injected the contrast during the scan. Her IV line had failed, so all the contrast had been painfully injected into the surrounding tissues of her arm, causing it to swell up. Because the contrast didn't get into her vascular system, the CT scan didn't show anything. Discouraged, we returned to our room, and they decided they'll try again in the morning. Annie is literally running out of veins that are in good enough condition to tolerate IV lines. The vascular access team is now in again today, looking for larger veins in her upper arm, thigh, and ankle. Once they get a line in place, we'll immediately have the CT scan and cross our fingers that the contrast doesn't blow out the IV again.

Last night they started Annie on a blood thinner, Lovenox. It is given twice a day by injection into her thigh or abdomen. Right now they suspect she will need to stay on it for 6 months or more. But that plan might change depending on the CT results we get today and if they find any other clots or embolisms.

Annie had a pretty uneventful night. She had a fair amount of discomfort, most likely from her swollen arms. Her temperature was a little more stable last night. But this morning her white blood count has gone up. It might be due to the inflammation from the blood clot and the contrast infiltration, but we're not sure. So we'll continue to watch her blood counts daily.

This has been by far the busiest hospital stay we've ever had. It has been exhausting to have so many blood tests run, radiology tests, and procedures. It seems as if we have a steady stream of nurses, residents, hospitalists, phlebotomists, and several other specialists in our room. To say we are exhausted is an understatement.

Previous hospitalizations have seemed much simpler since Molly and Lexie were younger and didn't have so much going on. But life continues on for them with homework, new band classes, field trips, marching band rehearsals, learning show choir music, deadlines for school fees, football games, weekend parties, playdates, early morning seminary, and many other things. Molly even leaves on a weekend band trip tomorrow. It's so difficult to juggle all the things they have going on and be at the hospital to care for Annie, be available to talk to doctors and get various test results to help plan her care. And while all this is going on, Justin still has classes at the university to be teaching, students to help, and meetings he's responsible for.

Molly and Lexie feel the stress too since we aren't at home much to help with homework, fill out permission slips, or get school fees paid, etc. And when one of us is home, we are exhausted, in need of a shower, and still worrying about Annie, and often on the phone with the other one of us at the hospital. And not only are Molly and Lexie worried about Annie, but they still have all their school work and extracurriculars to worry about, all while missing their parents. We are so grateful that my mom is still here to help with housework, laundry, groceries, and making meals. I definitely don't have any energy or time to take care of those things. But she will soon be going home.

I saw a picture online yesterday that has gone viral. It's a picture of a 4 year old boy with his bald head standing over a toilet feeling sick from chemo while his 5 year old sister stands next to him with her hand on his back. The mother posted that picture to show that these tough medical challenges are not just a trial for the child going through it, but it affects the entire family. I could relate to all the feelings she expressed. Our family is definitely feeling the strain of this hospital stay. We're praying that we can get Annie home, even if she's not better, and continue to care for her with our family all under one roof. She'll still need visits to the hospital and clinics to be monitored, but all being at home would be a huge blessing for our whole family. The results of her scan today will help us know how much longer until that wish might be granted.

As I've been typing this, the vascular access team was able to get a large mid-line IV in Annie's upper arm! So it's time to try the CT scan once again. I'll share results later today.

September 11th: A Big Setback

Annie had a fairly uneventful night last night. At 3:00am I woke up and checked what her midnight vitals were. They showed her temperature was down to 96.4, yet no one had taken any measures to warm her up! So I piled blankets on her and turned the heat lamp on for a little while. Her temperature came up to 97.7 and this morning has recovered to 98.2. No one knows why her temperature is taking such low dips at night.

The good news today is that Annie's white blood count continues to drop! It fell by a full 9,000 since yesterday, now down to 17,500! We're so relieved to see that continue to drop.

Annie's heart rate is still elevated. She stays around the 80s at night but it starts increasing throughout the morning and afternoon each day, typically reaching the 130s.

Annie's hemoglobin is lower again today at 9.5, but they drew two blood cultures yesterday, requiring a total of 30cc of blood! So we suspect that just getting her well and not needing so much blood work will help a lot with that.

Annie is on feeds comparable to her at-home amounts, all going through the G-port of her GJ tube, so we hope that we can replace the GJ tube with her regular G-tube soon.

This morning when I got Annie in her chair, I noticed that her left arm, which has her PICC line, is rather swollen! I had the nurse stop the small amount of fluid Annie has been receiving through that line. Because of this issue, they removed the PICC line late this morning and did an ultrasound of her arm and upper chest.

The ultrasound showed that Annie has a blood clot that is blocking two vessels. In just a little while she will head down to radiology to get a CTA scan that will check for clots in her lungs and chest and any pulmonary embolisms which can be life-threatening. Annie will need a new IV of a rather large gauge for the CTA scan. She is running out of veins that haven't been traumatized, so the vascular access team spent quite a while trying to get an IV started.

Annie will need to be on an anticoagulant (blood thinner), possibly for the next 6-12 months. This is definitely not the news we wanted today just as she was starting to make improvements in other areas. However, this new development might be the reason Annie's heart rate has been so high. Blood clots can be pretty serious, so please continue to keep Annie in your prayers, that her clots might get resolved without any problems.

Swollen left arm with the blood clot vs. Normal right arm

September 10th: Bouncing Back!

After such a horrible scare last night, Annie has seemed to bounce back today just fine. She spent the entire afternoon up in her wheelchair watching a movie and playing with her beads on her tray. Her temperature seems to have stabilized and she was able to come out from under the heating blankets and heat lamp around noon today.

Annie's heart rate is still elevated, which it has been for about a week or more. Her hemoglobin is down to 9.7 but they believe it's because she hasn't been getting her normal diet for a while and because they've been drawing so much blood every day for two weeks now.

Between the high heart rate, elevated CRP, and hypothermic episode last night, they suspect she still has an infection somewhere, but no one really knows. We are encouraged that her white blood count came down so much today, but it is still pretty high. They drew blood from two different locations on her today to culture. So we'll have to wait a couple days to get the results from those. If things make a turn for the worse, they will consider imaging her spine to look for infection.

Annie is now on feeds that are equivalent to her diet and fluid intake at home! They are going through her G-tube and she is doing great! The nurse partially clogged her J-tube this morning with meds, so we're going to start giving all meds through the G-tube now. I think Annie can tolerate that now anyway. Hopefully we'll be able to transition from the GJ tube back to the regular G-tube soon and then we won't have to worry about all these clogging issues!

So they are just watching Annie and waiting for blood culture results. If they are negative after just 24 hours, they will consider pulling out her PICC line, which could be a possible source of infection. Annie hasn't been using her PICC line for anything for a day or so, but they want to keep it in a while longer in case blood cultures show that she needs more IV antibiotics. Final culture results won't be in until Friday. We're praying for a calm and uneventful night tonight!

September 10th: A Scary Night

Today is day 16 with Annie in the hospital. It's been a roller-coaster here with her. We don't know what happened in the night, but Justin was woken at 3:00am to our nurse, the charge nurse, the resident, and the hospitalist in the room. Apparently during the night our nurse noticed that Annie had gone very pale and had become non-responsive. Her temperature had dropped to 92 degrees!

They got heating blankets and a heat lamp on her as well as monitors to more closely watch her heart rate, respirations, and oxygen saturations. They also drew blood for labs to see what was going on. Annie's temperature slowly came up to 97 eventually, but she remained non-responsive until about 9:00 this morning.

Surprisingly, all of Annie's labs look pretty good. Her electrolytes are all fairly balanced, and her white blood count has even come down by 6,000 to 26,000! We suspect that the sulfasalzine was to blame for her increasing white count since it made a dramatic drop 24 hours after stopping that medication! So that was great news! But Annie's platelets are still elevated, as well as her CRP which is an inflammation marker in the blood. Annie's sodium was just slightly on the low end of normal, so they are now mixing her formula powder in pedialyte instead of plain water.

We don't know what happened last night to cause such problems, but now this morning she has become more alert and responsive. She is still under the heat lamp and heated blankets, and her heart rate is high, in the 130s, with elevated respirations in the 30s. Morning meds partially clogged her J-tube, but it's not a total blockage like yesterday. We're using more Coke to try to clear it.

Annie is tolerating quarter-strength formula fantastically through her g-tube at a full rate of 60cc/hr.  I think the plan today is to get her to 60% strength formula at a rate of 60cc/hr. That strength and rate is comparable to her at-home diet, so once she reaches that, she will have met her dietary and fluid intake goals!

We are very encouraged by Annie's WBC dropping so much in the last 24 hours. That is a huge relief, but we are not out of the woods yet. We are so grateful for everyone who continues to pray for Annie and our family.

September 9th Update

It was another exciting day at the hospital. A nurse accidentally clogged Annie's J-tube with her morning meds. Since the J-tube is threaded down through Annie's intestines about a foot, we can't just take it out to clear the blockage like we would a G-tube. So we let it sit for a couple hours to see if it might dissolve over some time, but didn't have any luck with that. A nurse suggested trying Coke in her line to break up the clog, so over about two hours we kept her line pressurized with Coke, adding more every 10-15 minutes. Thankfully, after about 2 hours of that, the blockage cleared! Whew! Who knew the power of Coke!

While we were waiting for the Coke to work, we needed to still get Annie's meds in her somehow, so we decided to use the syringe pump to slowly infuse her morning meds through her G-tube. Since she hasn't had anything in her stomach for two weeks, we decided to go really slowly. We thought she was tolerating it great, until I discovered that the nurse connected the J-tube extension set into the G-tube port, which isn't compatible, so the fluid just leaked all over and Annie's tummy was covered in yucky meds running down her waist. So no meds got in her stomach. It was a frustrating morning to say the least. We finally just gave Annie her seizure medication and didn't bother with re-dosing the rest.

Because Annie still needed fluids and we didn't know if we'd ever be able to unclog the J-tube, we decided to try some pedialyte through her G-tube into her stomach.  She was doing well with it, and we want her to ultimately transition to the G-tube anyway, so even after we got the J-tube unclogged, we decided to keep going with the G-tube pedialyte since Annie was tolerating it well. Tonight she has worked up to 60cc/hr of pedialyte through the G-tube! So overnight tonight or maybe tomorrow, we'll move to quarter-strength formula through the G-tube and just use the J-tube for meds right now. So that's great news and gets Annie just that much closer to being able to going home.

The bad news is that Annie's white blood count went up yet again today to 32,000--the highest it's been during this hospitalization. It continues to increase each day by about 4,000-5,000. Her CRP also went up quite a bit, which is an indicator of inflammation. So that is concerning as well. We suspect that Annie might possibly have an infection somewhere that her bactrim has been masking and preventing it from really flaring up but hasn't been able to treat. Annie had her last dose of bactrim today for her UTI, so only time will tell if there is another infection somewhere.

The Hematology/Oncology team believes that her high WBC might be due to a new medication we started Annie on for ulcerative colitis last week since her regular UC med can't go through her J-tube. So we have decided to stop that medication to see if it may be the culprit. So hopefully tomorrow or the next day we'll start seeing her WBC come down a little. If her WBC and CRP continue to trend upwards, they will do more imaging of Annie's spine to see if her spinal rods may be infected.

The next few days will be really telling, hopefully, now that Annie is off the new UC med and the bactrim. So we're praying that Annie's WBC and CRP come down instead of a new infection popping up.

Annie had a happy day today and was alert and awake. She ended up sleeping yesterday for 6 hours straight without stirring, and then had a bad seizure requiring Diastat. It was probably because we tried to wake her up. So it was great to see her happy and alert today after a super sleepy day yesterday that had us concerned. We're hoping for at least some answers this week, and hopefully some good progress too!

September 8th Update

Last night was a little rough. Annie started having some pain but we found that tylenol and a movie helped. The problem with that was that Annie was wide awake the rest of the night. She was generally happy, and just making her talking sounds through the night. She had a bit of a cough as well that kept both of us up, and she got fussy a couple times requiring some position changes in the night as well.

So at 10:45 this morning Annie finally fell asleep and has been sleeping for more than 4 1/2 hours! At 3:15 now, she's still sound asleep! While she's been snoozing, the Hemotology/Oncology doctors came in to visit. They had thoroughly analyzed her lab values as well as her blood samples under the microscope to figure out what might be going on.

They had a ton to say about what each blood component level meant. But they basically agreed with the infectious disease team--that Annie's values don't indicate that there is any infectious disease process going on. They said her increase in white blood cells (which went up again today to 28,000!) is most likely due to a new medication we started Annie on or possibly just the stress she's had to her body over the past two weeks and being on TPN. They said there's also a chance that she's just picked up some kind of virus, although she shows no other symptoms.

Hematology agreed that the best thing would be to get Annie on her regular feeds and get her home and see if her WBC improves. If it continues to increase into the 50, 60 or 70 thousands, they would want to see her and figure out other possible problems, possibly related to her bone marrow. There is also a chance that Annie has something wrong with her spleen, which would affect her blood counts. The CT scan showed her spleen was decreased in size from previous scans, but it's hard to know because her previous scans were done when Annie had terrible infections, meaning her spleen may have just been enlarged at those times and now it's just back to its normal size. So they will continue to monitor her labs.

Annie started on half-strength formula today at 60cc/hr through her J-tube. I've been running the numbers on what they want her to tolerate before going home, (full-strength at 60cc/hr) but that actually puts her over the daily amount that she would normally get at home. If we convert her typical feedings into a true continuous 24 hr feed, it equates only to 3/4-strength formula running at 53cc/hr. So maybe Annie is closer to her home feeds than we thought! She's been tolerating feeds well so far, so we continue to pray that she'll keep doing well and get the green light to go home in a few days.

We'll still have to monitor her closely at home because of her lab values being so concerning, but we should be able to see doctors in their clinics for that, and don't need to be inpatient, unless something bigger is finally found to be the problem. Today is day 11 of the IV bactrim which they think may be a sufficiently long course, but the hospitalist will consult with the ID team to get their opinion before stopping it. Annie's GI tract is still not completely up and running, so that's another thing we're watching.

September 7th Update

Annie's white blood count has risen again, now to 25,000! That's an increase of about 5,000 in just 24 hours. Her last spike was only 3,000 over 48 hours. Her hemoglobin has dropped again but not quite as significantly. They checked her urine again and everything looked great there, so that infection has cleared up nicely. So everyone is still stumped as to what could be causing such a high white blood count. Annie's heart rate has been coming down and is lower than it used to be, but still not back to normal.

With the dramatic increase in white blood cells, and no clues as to the cause, the infectious disease team was brought in today. They said that based on the different types of white blood cells that are all elevated, they don't suspect that she has any kind of infectious disease process going on. And based on past lab results, they don't believe she even had pyelonephritis--which is in line with what the urologist and radiologist said as well.

The ID team believes that Annie needs to get back onto her regular feeds and once her GI tract is working normally again, they suspect her white blood count to return to normal. They weren't sure how to explain her sudden and dramatic drop in hemoglobin, so they are referring us to the Hematology/Oncology team, which will hopefully be in to see us tomorrow and get us some answers.

So for now, we are continuing to wean Annie onto her feedings. She is at the full required rate of 60cc/hr, but is only on quarter-strength formula. Tomorrow we will transition to half-strength formula, and maybe by Monday, try full-strength if all goes well between now and then.  Everything is still running through the J-tube, so then once Annie is up to 60cc/hr of full-strength formula, we can work to transition back to the G-tube depending on her tolerance. She is still on TPN through her PICC line at a decreased rate, but that will be discontinued tonight. We'll keep the PICC line in place though, until Annie is back to her regular feeds, just in case she takes a step backwards and ends up needing it again.

Tonight we all joined Annie in the hospital for a pizza dinner together--the first meal we've had together as a family in two weeks! Lexie played some video games for Annie and we all had a pretty good time together. Annie has been in good spirits with no more bouts of pain--They suspect her pain from the last couple days has been cramping or gas from her GI tract getting up and running again. Annie had a good night last night and has been pretty happy today watching some fun movies.

Evening Update, September 6th

Annie had an abdominal CT scan today. We were sure that they would find something wrong with her appendix or an abscess somewhere or some other explanation for the increase of white blood cells. We even thought she must have some kind of internal bleeding due to her hemoglobin dropping so suddenly. However, yet again, no issues were found. We are stunned and baffled.

We spoke with our GI doctor about the results and decided on a new plan. Our goal now is to focus on getting Annie back onto her regular feedings so she can just come home. She's still receiving TPN nutrition through her PICC line which isn't really great for her, and the PICC line can become a source of infection after a while. Her PICC line is starting to fail again anyway and we don't want to put her through another surgery to replace it. So our goal is to stop the TPN, get the PICC line out, and get Annie home.

In preparation for her CT scan today, Annie had to get 480cc of liquid contrast through her J-tube in two hours! I was super nervous to push that much fluid--a rate of 240cc/hr--when all she's worked up to so far is 25cc/hr of pedialyte! But she did pretty well, only vomiting a small amount twice. So we have high hopes that we can start pushing her feeds and getting her transitioned to her formula by the end of next week. Tonight she is still on quarter-strength formula at a rate of 30cc/hr. Tomorrow we hope to get her to 60cc/hr and possibly even introduce half-strength formula. We'll continue to increase her rate and formula concentration until she is back to her regular feeds through the J-tube and then we will work to transition back to her G-tube.

Once Annie is tolerating her normal feeds, we'll remove the PICC line and get her home. Once she's home, we feel like she will be able to recover a little better. She has experienced a lot these past two weeks with going under general anesthesia twice, having multiple scopes, radiology testing, lab draws, straight caths, etc. That's a lot for her body to experience, so we hope that once we get her home, her body will calm down and her concerning lab values will improve, because we can find no other clinical explanation for those values.

Once we are home, we will follow up with doctors in their clinics to get blood drawn and check lab values, monitor pain, seizures, etc. We really don't know what direction Annie will go once she's home, but there are just no more tests to run at the hospital and no one knows what to do anymore. So we've decided that getting her home might just be the best medicine that she needs.

This has been one of the most exhausting hospitalizations we have ever had, physically and emotionally. Today, Annie was up in her chair so Justin just couldn't help borrowing her bed for a while. We are definitely looking forward to all being under one roof again.

Morning Update, September 6th

It's been an incredibly frustrating morning. Last night was really rough. Annie was screaming and writhing in pain for two hours. She received tylenol but I don't think it did anything. She eventually threw up and fell asleep. She did the same thing the night before. Annie woke up again at 4:30 this morning with agitation. It didn't escalate into quite what is was last night, but she's clearly having some intense pain. We just don't know the cause.

We've suspected all along that Annie has something else going on other than the UTI and pyelonephritis. Even her urologist wrote in his report that her symptoms are atypical for pyelonephritis, and the radiologist told me her imaging shows only a slight possible pyelonephritis. And today Annie's lab values are further indicating that. Her white blood count has gone up yet again, to over 20,000, and her hematocrit and hemoglobin have dropped significantly, indicating that she has a possible bleed somewhere. Her heart rate is also still very elevated, further indicating that there is a problem her body is reacting to. She is on day 9 of IV antibiotics for the pyelonephritis, so she should not be having any symptoms from that anymore.We know the bacteria from the urine culture are susceptible to the antibiotic she is on, so that should be resolved.

We are getting a bit frustrated to say the least, but hopefully today we can get some more testing done and finally get a real diagnosis. This has been the most difficult hospital stay in regards to advocating for our child. Continued prayers are very much appreciated! I hope to be able to finally share a definitive diagnosis with you later today!

On a more positive note, the adjustment to Annie's PICC line yesterday seems to have been successful! We were able to draw labs beautifully this morning! What a relief!

September 5th Update

Today was a pretty good day. Annie's PICC line is failing to draw blood again, so our goal today was to get it working. Annie had a full chest x-ray so the vascular access team could see where the end of the line was in her body. From that, they determined how far they needed to adjust it. They ended up pulling it out 3cm, so the end of it is now near her arm pit inside her body. Once the line was secured in place, we tested it and it drew blood beautifully!

However, in order to prevent the PICC from clotting off again, Annie has to stop her TPN. The TPN she's been on the last week or so has contained all her complete nutrients and calories that she needs, going directly into her blood. However, since her line is clotting from it, they are switching her TPN to a less complete nutritional formulation that has a lower risk of clotting her line.

Because Annie's nutritional needs can no longer be met through IV access, we have had to start trying to feed her formula through her J tube a little sooner than we were planning. So today Annie started getting quarter-strength formula through the J tube directly into her intestines. We started really slowly at 10cc/hr but tonight she is up to 20cc/hr and doing really well with it. We only ever ran the pedialyte at 25cc/hr, so we'll soon be testing new limits as we increase her formula rate. Once we see she can tolerate a larger volume, we'll begin to increase the concentration of her formula until, hopefully, she can get back to her normal feeds of full-strength formula at a rate of 85cc/hr. Even if that's just through the J tube, she can go home on that, and then once home, we can try to transition her back to the G tube.

Because they had to take a chest x-ray to locate the tip of her PICC line, they were also able to look at Annie's lungs. We've been concerned about her aspiration on Monday night and feared that pneumonia might settle in, especially with her breathing troubles that developed afterwards. But thankfully, Annie's lungs look clear with no signs of pneumonia!

Today is day 8 of the IV bactrim for her pyelonephritis (day 11 in the hospital). They haven't decided yet if that will need to be a 10 day course or 14 day course. They will do another urine culture at day 10 to check and possibly extend to 14 days just to be safe.

Annie has been up in her wheelchair for longer periods of time yesterday and today and seems to enjoy it. I think she's really wanting to get back to school and experience her senior year! We're not out of the woods yet, but I'd say Annie has finally turned a corner!

We've had fun watching the construction workers build the outer walls on the new hospital extension outside our window today. But all their progress more clearly marks how long our stay here is becoming! Today Annie enjoyed watching Lexie play more video games and a visit from Grandma and Grandpa. We're so grateful for the extra help!

September 4th Update: A Better Day

Annie has had a much better day today. She is not struggling so much to breathe and has actually been off of oxygen all day! Her heart rate is still really high, around 130, reaching the mid 140s at times.

Annie's PICC line clotted so we didn't get her daily lab results back before the rounding doctors came today. But I've pulled up the results and things look pretty good except for a slight increase in her white blood count. I suspect that indicates that she probably did aspirate the other day and could possibly have pneumonia settling in. She spent most of the day yesterday bringing up a lot of phlegm and seems to be doing better in that regard today. Her temperature went up to 100.2 once today, but now is back down closer to normal. So we're not quite sure if a true pneumonia is going to settle in or not. Only time will tell.

We increased Annie's pedialyte rate to 25cc/hr, with a goal of 40-50cc/hr over the next few days. Annie has vomited just twice in the last 24 hours, which is more than the day before. Both times were after she received her new medication to keep her ulcerative colitis in remission. It is known to cause stomach upset, so we'll have to watch and see if that could be the culprit or if it's just coincidental.

Otherwise it's been a pretty quiet day. We tried out a "shower cap" hair-washing. It worked ok, but was really no match for Annie's thick, curly hair. But now she's looking a bit nicer.