Finally Leaving the Hospital

Well, we still don't quite have a solution to the extended hospital stay Myra had this month (you can read about it here). After a double ear infection, Rotavirus and dehydration, Myra's electrolytes would not stablize. We went home on oral potassium hoping to regulate it there, but both days we took blood after leaving the hospital the potassium came back High! My poor baby has never been ill up to this point (8 months) and I think the ACTH she is on for her Infantile Spasms is irritating things. We go back to the hospital this morning for yet another blood draw after lowering the dosage significantly both days, the potassium came back high. I'm thinking her body is just stabilizing on its own finally and she probably doesn't need the oral supplements.

In the meantime, poor girl can't sleep well at all and nurses constantly. And I'm not exaggerating at all! She was latched on for a whole 2 hours one day this past week! If she's not nursing, she is either fussing or sleeping. She has very little happy, or even content awake times. She won't sleep swaddled on the couch anymore either. She either sleeps in the sling or on me on the couch. I think waking her every 2 hours in the hospital has destroyed her lovely sleep pattern. She was one of only two of my kids that slept through the night (6-8hrs) before 10 months! Poor baby girl....

Hospital Stay, Day 16

Background: May 3-5 Myra was at Riley Children's Hospital trying to get her seizures under control (see story HERE). We found out then she has Infantile Spasms and were to start her treatment the next week. The following Wednesday we were admitted into a local hospital to start the ACTH injections and stayed there for 3 days for preliminary tests and learning how to give the injections (May 12-14). We came home Friday afternoon and I attended a children's clothing resale and Homeschool Resource Expo that Friday and much of Saturday.

Saturday night, after the kids had gone to bed, Myra had an odd episode of gagging, strider breathing and looking like she was choking. She hasn't eaten for over an hour. We weren't sure if it was a seizure or if she was really having breathing issues, so I told Mr. Prince to call 911. The police arrived in, literally, one minute! It was amazing. And in less than 4 minutes we had 3 police officers and 2 EMTs standing in our disastrous messy living room. She had recovered from the episode, so none of them witnessed it. They checked her O2 levels and heart rate, suggested we go to the hospital to get checked out (which they have to do), but we ended up signing a releasing saying we didn't want to go. I had been in the hospital for 6 days of the last week and a half and wasn't interested in going to hear:

"Oooookaaaayy, what was she doing again?"
"Did you put something in her mouth?"
"So, she was choking or gagging?"

I knew unless they witnessed it, going there was not going to give me any more answers, and would just make me feel stupid and self-conscious. So, they told us to not hesitate in calling them back if we changed our minds or she did it again. End of story.

So we thought.

10 minutes later she did it again and it lasted longer. I delayed in calling 911 again because I thought she would just recover again. With the two seconds to decide, I thought we should go to the hospital even if she did recover and figure out what was going on. I grabbed the diaper bag, the EMTs ran in the house and said, "Let's go!" and we were off.

I've never been in an ambulance. Hope I never have to again, but it wasn't as scary as I thought. They put her in a car seat much too big for her (one that I'm not sure would even protect her in a crash) and monitored her all the way there. No sirens, no lights. It actually took them 5 minutes longer to get to the hospital than it usually takes me.....odd. But, at least I could watch her and not have her in the back of a car wondering if she was breathing or not.

They slowly made their way into the ER from the vehicle and we waited for staff to get off their rumps and get us a room. It was all very opposite of what you'd imagine. It was past 11pm at this point and Myra was ready for bed and falling asleep. Despite her wishes to sleep, many nurses came in and asked those irritating questions with a lack of belief in their voices, listened to her now normal breathing and normal heart rate, they drew blood, not once but twice because they forgot to get a level on her Phenobarbital, and basically made it their goal to not let her sleep I think. No one else saw an episode except the person who drew her blood. Go figure.

The blood work came back with elevated white blood cells, a sign of infection, and they started asking if she or anyone had been sick. No, no, no. She was admitted for observation for breathing issues and elevated white blood cells. They gave her a shot of Rocephin.

She had a breathing/gagging/choking episode five times that evening until she went to sleep for the night. Sunday she did it many more times and the nurses eventually saw it. Her heart rate always skyrocketed afterward and she always recovered within a minute or two. Very odd. Her pediatrician found a double ear infection and wondered if swelling or drainage was making her gag reflex kick in. We thought the Rocephin had started working because Myra pooped out of three or four outfits that day. Her fever never got above 99. We were still mostly baffled.

Monday her diarrhea continued and we ended up fashioning a "cloth" diaper over her paper diaper to save the few outfits I had left with me. She had a video EEG from 7am to 10:30pm to rule out seizures. In the meantime, her fever went up to 102.9, her breathing/choking episodes had stopped but she was almost in a constant gag. She wouldn't take her paci or nurse. We had to use her g-tube for feedings for the first time since the NICU.

Can I just say.... I.hate.pumping.

And I hate the gut-wrenching feeling that something so precious to me is being taken away or may have to end prematurely, which is what I've been told over and over. Myra shouldn't be able to nurse with her history. But, she is the daughter of a gracious, powerful God and therefore can.

Medical world, deal with it!

Off my soapbox...
Anyway, she continue to get worse all Monday. I showed the nurses her gagging, her sunken eyes, her lethargy, her blue hands, her tremors, her eyes rolling into the back of her head. Nothing. They seemed confused, but not concerned. I was, though. I asked to weigh her that night after seeing how tight I had to pull her diaper and how thin her thighs looked.

1 full pound less. 11lbs down to 10lbs...in one day!

The girl is already a peanut, she cannot stand to loose anything! I voiced my concerns all night with no return.

In the morning after blood work came back, two pediatricians from her primary's practice rushed in saying her sodium was super high and some other things came back abnormal from the blood work. They looked her over and pinched her tummy skin. It stayed, didn't go back to normal. They said she was.....drum roll, please...

Dehydrated!

Really? Hmm...I noticed that almost TWENTY-FOUR hours ago!! SO irritating!

So, she was brought immediately down to the Pediatric ICU (PICU) because they had such trouble getting an IV in on Sunday for the antibiotics they wanted to give her (tried 7 sticks before they quit). Pack up, move rooms. Once down there, the PICU doctors tried twice in her NECK (ugh!) with no success. They did sub-cutaneous IV fluid into a pocket in her back. After an hour of running the fluid and it being absorbed, they were hoping her veins would pop back up and they could set a normal IV site. 2 stick later, success!

Poor girl was so weak, slept through tube-feedings, still had severe diarrhea, and had been stuck over 10 times at this point. But, throughout the day her jowls starting coming back, her hands started to pink up, and she started to act hungry and suck on the wet sponge I had been wetting her mouth with for a day and a half. I told them she was probably ready to nurse just in time for some new results...

Myra has Rotavirus.

So, they didn't want me to feed her. They said her GI needed a break to slow down the diarrhea. Ummm, when she starts eating, won't the diarrhea just start back up again?? Dude, they have this diarrhea for weeks sometimes! Sometimes I think really good doctors lack common sense.

So, I continued to fight and a few hours later, when the doctor saw just how upset Myra was, he OKed me to nurse. By then she was so upset and I was so full, she couldn't latch on properly. Another tube-feeding. But, two hour later she tried again and kept crying after latching; I think her ear infection was still bothering her. I kept offering and by morning she nursed a full feeding....Praise God!

I had an awesome male nurse in the PICU, Kent. He has a special needs son and kept talking about special needs kids and their ability to withstand greater amounts of pain than the average child. He was very sweet, called Myra his little pumpkin and always stuck up for me and what I wanted. When the doctor originally came in to see Myra when I was requesting to nurse, she had fallen asleep from exhaustion and didn't look very hungry. He came in and assured the doctor that him and at least three other nurses would attest to the fact that Myra had been "angrily hungry" for over an hour now and that, Murhpy's Law, is only asleep from exhaustion. Love those nurses that will listen to you and understand you know your child better than anyone, no matter what medical degree you have!

The blood work Wednesday morning looked much better and the PICU sent us back to PEDS. They kept the IV fluids going through Wednesday night. I had some concerns Myra's heart rate was staying in the 60s and her temperature was 96.7 over and over. No one else seemed concerned, though. The pediatrician on-call did rounds on Thursday morning and said the IV could be discontinued and as long as her input outweighed her output, she could go home that day. Yeah! Angelica Pickles’ birthday was on Friday and I didn’t want to be gone… And I should mention, at this point Sid the Science Kid, Angelica Pickles and Boo Boo were now sick at home, throwing up with diarrhea. They visited me and Myra Sunday before we knew she was sick…. Lovely, huh?

Thursday afternoon rounds came and I voiced my concerns again about Myra’s low heart rate and temperature. They still didn’t seem to be concerned, but were concerned that she was sleeping more than the day before, nursing constantly and still seemed so lethargic. They said she should be getting better, not getting worse again. Doctor decided they wanted another round of blood work before she let me go home. Long story short, God intervened and allowed a mistake to be made in the lab so as to catch the fact that Myra’s potassium was dangerously low. They weren’t even going to run potassium tests! Myra has her own little angels, doesn’t she? So, potassium makes you sleepy and also affects your heart rhythms….

Really?

Again, another things they showed concerns about 24 hours after Mama!

We moved back to the PICU at midnight.

More sticks, blood work, IVs, up every hour for assessments and blood work. They were giving her potassium through her IV and by Friday morning potassium levels were normal, but a few other things were still off!

Good grief! It was never-ending!

Friday afternoon blood work showed a drop in potassium again, and we had another sleepless night of IV potassium and heel sticks. Saturday afternoon, potassium was almost normal….3.3. (Normal = 3.5-5.3)

At this point I was fed up with the ups and downs and seemingly no real steps toward going home or figuring out what was really going on. I kept mentioning the ACTH medication she just started and how it was supposed to mess with your electrolytes, but no one had any knowledge of the drug.

Saturday morning I had to leave Myra to take Princess Pea and Angelica Pickles to their dance recital, so a good friend came to take care of Myra since Mr. Prince was home with sick Boo Boo. While I was gone, they reset her IV in a new location and stuck her another 6+ times, finally, with success. Another God-incidence (instead of coincidence), my friend overheard the nurse talk about flushing Myra's IV with Heprin. Mandy somehow knew that Heprin was a blood thinner and also knew Myra had been bleeding so well earlier in the week that nurses asked if she was on blood thinners. So, she voiced her concerns over them giving that to Myra and they ended up switching back to the salin flush. I absolute believe God used Mandy in that moment to protect Myra from more issues. He is awesome!

Then, the doctor also ordered IV fluids and no more nursing or Pedialite. I was not going to allow my child to starve for no good reason after this torturous week!

I got on the phone with the doctor and we talked about why he would restrict her food (to allow the intestines to build back up the mucus lining to allow nutrients and such to absorb better because the diarrhea had stripped the mucus lining). I still didn’t think that was the answer to our problems and told him I thought he needed to be talking to my neurologist and researching ACTH to see how it is affecting her. He agreed to do so.

When I came back in the afternoon, I talked to the doctor after he did his research. He agreed that the ACTH could be complicating things. He said he was taking her off the IV, resuming her breastfeeding, giving her oral potassium, and had goals to try to regulate her potassium with oral supplementation at home if possible. He said if the ACTH was going to try to keep her potassium down over the next 3 months, we can counter that with daily potassium supplements. I have a feeling after the diarrhea is completely gone, her body will eventually regulate, but I don’t care to be in the hospital for another 1-2 weeks until the diarrhea stops!

Poor baby girl has been stuck over 30x in the last 7 days! I have cut off their attempts at getting blood from her arms. They are only allowed to do heel sticks at this point. I know I’m not the favorite Mama on the floor, but I thankfully have had some nurses who are parents themselves and really understand my side of it. I’m not one to hang my head and do exactly what they tell me to. And, I think that has only benefited my daughter so far. The resounding thing I keep hearing about parents of special needs children are that they have to constantly be an advocate for their kid.

So, call me Queen Advocate! I’m there!

Saturday evening her potassium had gone back down and they were doing oral potassium and IV potassium. They started running the IV late, which meant the third night in a row of IV meds, heel sticks, and assessments every hour.

I was done.

At 4:00am, after the second round of IV meds, I woke to them getting Myra’s blood. I didn’t stir too much because I know she can’t be calmed in the midst of the heel stick, but she seemed to be crying a little harder than normal. When I got up I saw the lady fishing in her arm again for blood! Boy, was I mad! I said very sternly that getting blood work from her arm was OFF LIMITS and that this mistake WILL NOT happen again. After the heel stick was completed, because (surprise!), they had no luck getting anything from her arm and just put yet another hole in her little being, I told the nurse that I didn’t care what the potassium levels were when they got them back. We were NOT to be messed with until she woke up herself in the morning. I knew her potassium could be low, but from the 6am blood work to the 6pm blood work we have no idea when her potassium drops, so I knew she would be fine for a few hours so that we could both get much needed sleep. The girl will never get better if she can’t sleep!

Ya know what, no one came in my room until they noticed Myra out of her bed at 8am! And they didn’t even touch her until 9, so she was able to nurse back to sleep for a while longer. Ahhhh, rest…

And I also had the good nurse back, Kent. He gave her her meds at 9am, and said he wouldn’t bug us again until the doctor came by around 11 and they had to get more blood. Those numbers came back good, potassium at 3.9, so we were transferred back to PEDS around 3pm. Blood work would be repeated Monday morning and if those numbers looked good, we could go home!

We had now been at the hospital for a full week, and 10 days if you add on the 3 day stay to start the ACTH. I have stayed in a hospital with Myra for 16 of the last 22 days. In this hospital stay alone I had stayed in the ER one night, 2 different PICU rooms and 3 different PEDS rooms, moving my stuff 5 different times. And, my poor klan at home! Boo Boo was still having diarrhea, but Angelica Pickles and Sid the Science Kid were feeling better. Princess Pea mysteriously has never gotten sick. We tested Boo Boo for Rotavirus to make sure we weren’t going to spread his virus to Myra or vice versa, because lots of Mr. Prince’s family has stomach bugs (and supposedly adults don’t get Rotavirus). Is it wrong to PRAY for Rotavirus? Only so that we don’t have to deal with diarrhea dehydration issues from yet another virus for Myra or Boo Boo…

I talked to the doctor extensively about the potassium. The IV potassium had caused some swelled hands and legs, I guess because it’s toxic to the veins.

Lovely.

Probably the reason why Myra whaled while it ran into her arm the 7 or 8 times they gave it to her…

The oral potassium was supposedly rough on the esophagus too, so they said to only use the g-tube for it, every 6 hours around the clock. No concerns she’ll get too much even when her body starts to regulate because her kidneys should flush out the extra. They will monitor her electrolytes every few days for a week or two and then probably weekly until the ACTH is out of her system and all is well. I also talked to the doctor about if we needed to monitor her heart while sleeping since it got low (54-65bmp) with the low potassium. Maybe that it would be a first indicator we need to recheck her potassium levels….?? That, and her low temperature… He didn’t seem to think it was necessary, but I think I’ll ask my primary this week anyway. In the meantime, nurse Kent saw that I seemed anxious that I couldn’t regulate her heart rate on my own. He told me to take the stethoscope from the PICU room and told me to just count a whole minute or two every day if I wanted and regulate it myself. How awesome is that! Kent is seriously the coolest nurse on the planet!

I’m also supposed to start “topping off” her nursing with an ounce or two of fortified breastmilk (adding formula to it for extra calories) to stretch her stomach so she can begin to intake more food daily. They said when her brain was damaged, it could have damaged the parts that tell her to eat more to grow and develop because she’s never taken more than 3oz at a time and always seems full. They hope (and I hope!) this will be temporary and just help her to realize she can eat more. I need to offer more baby food, too.

So, her daily medication schedule will look like this:

9am – ACTH IM injection
9:30am – Phenobarbital, Keppra (I might discontinue), Vit B6, Potassium
3:30pm – Phenobarbital, Prevacid, Potassium
9:30pm – Phenobarbital, Keppra, Vit B6, Potassium
3:30am – Potassium

Ugh, no more sleeping through the night! I think Mr. Prince and I will take turns and hopefully we can hook up her tubing before she goes to sleep so we can give it to her and not un-swaddle her and wake her up every night!

Myra’s ACTH is scheduled to be done August 12^th or so. Seems like a long way off… And I really hope this week was the worst of it! I was just realizing (with all this extra quiet time in the hospital by myself!) that once she is off this ACTH and her spasms are GONE (yes, they WILL be gone!!) that she won’t be able to start developing again until September some time. She will start to loose the steroid weights and start to get her personality back (so I’ve read) and then be able to start to develop neurologically. Unfortunately, at one year of age, developmentally Myra will be at 2-3 months. I knew she would have challenges and I knew she would likely be a little behind, but I didn’t expect to have a new baby for a whole year. It’s just a little baffling even typing it. I remember thinking when Boo Boo turned one, that he was my first baby that didn’t walk at his first birthday party… I didn’t care, it was just an observation.

Well, Myra has given us way more “firsts” than we could have every hoped or asked for. And, I just have to sort out all the good parts of that. I guess it makes the things your other kids can do a little more special. Made me think a few times this week (only in my head, of course) how cute my just-turned-four-year-old is when she is arguing with me. She has a wonderful little brain up there that has the capacity to make up some random concoction to convince me to give her what she wants. Irritating, but fascinating at the same time… And watching my just-turned-two-year-old say new words almost daily and give me an ornery grin when he’s eaten something off the counter that he shouldn’t have. I’ve briefly seen things from a completely different perspective. And though I know I’ll have to go back to “normal” thinking with my other kids in order to discipline them appropriately, I have a new love for my kids’ personalities and abilities. I don’t think that will change any time soon. And when Myra starts to do all these same things, how much more rejoicing there will be! And praise to the gracious God who continues to heal her!

Monday morning lab lady comes and I hold my tongue as she fails to truly warm Myra's heel for the heel stick. She had to squeeze the life out of her foot to get a small drop. Grr!! So, I was not surprised when Myra's potassium had since come back HIGH! I understand that, in general, heel sticks are not accurate for reading potassium because the clotting factor messes with the results. But, if you get a good flow and don't have to force the blood out... so far Myra's heel sticks have been accurate. That's great considering they aren't having luck getting blood from her arms anymore. So, I really wish I would have been the "butthead" this morning and told the lady to warm her foot longer. They had to recheck the levels with a arm blood draw and, shocking, they came out fine! 3.5!

So, my immediate thought was, I can go home!

Wrong....

The on-call pediatrician said he was changing her oral potassium to 3x a day instead of 4x a day so we don't have to give her a dose at night. Great! But, why exactly does that constitute me staying in the hospital longer? He said he'd like to do another draw in the morning. Good grief, people! I told him I have 4 kids at home who need me. I reminded him that the hospital was doing nothing for her other than blood draws and if I could monitor her heart at home, there should be no reason I couldn't leave and come back for a draw tomorrow.

We agreed that if a draw this afternoon comes out good, I can go home and come back in the morning. But, he said a heart monitor wasn't necessary. I seriously disagree, but I will just use my stethoscope (thanks, Kent!) and check her myself. I still think these doctors don't know anything about ACTH and underestimate its ability to really screw up an 11lb baby. I think she needs to be monitored a little closer. I think she needs her blood drawn every other day for probably a week or two until we are sure, and then every other day again as we change her dosages of ACTH. Maybe I'm just paranoid, but if something happens to Myra because I neglected to have her monitored properly, I would never forgive myself. And, why don't nurses and doctors get the age-old saying, "Mama knows best!"

Oh, and Boo Boo has Rotavirus. At least we know we don't have two viruses in the house! Now we just need to keep Boo Boo hydrated... Darn hospitals! I completely blame them on giving Myra this, and we spread it to the klan Sunday night before we even knew Myra was sick. Plus, no one we know or have been around has Rotavirus....so, I'm 99.9% sure it came from a hospital. Makes me worry about what else she could be picking up this past week! AHHH!! Breathe, MommyK, breathe...

So, for now I sit in my little hospital room, eating my horrid fabulous hospital food, awaiting the next assessment and blood draw.

Thinking of my littles at home….

Thankful for how God is providing for our family…

Proud to be Myra’s Mama…

I'll update soon.

New Treatment

aInfantile Spasms are a form of epilepsy that does not respond to typical anti-seizure medications. Myra had already tried three medications before we realized these were spasms. There are currently only two proven treatments to stop IS; ACTH and Vigabatrin. Due to the side effects of vision loss with Vigabatrin and Myra's cortical visual impairment, we didn't want to potentially damage her vision further.

So, Wednesday Myra was admitted into the hospital so that I could learn how to give the ACTH injections and go through a handful of preliminary tests. She had a EKG done on her heart (all normal), an 4hr video EEG (still showing only modified hypsarrithmia), basic blood work, and a swallow study. Dr. Anxiety earned his name this week after Myra passed the swallow study with flying colors. He kept telling us that children with Myra's history (or children with cerebral palsy) often do not eat orally and he expected her to have problems with aspiration and solid food intake. I did not have any concerns and put off the testing even though he wanted it done within a month of seeing him. He was wrong and Mama was right. Mamas are almost always right, aren't we? =) So, nursing can continue with no concerns of pneumonia while Myra is on ACTH, yah!

Well, Myra has received 4 injections so far and she is already responding to treatment! After the first dose her spasms went from 100-150 in a cluster to under 100. After 2 doses they were under 50. After 3 they were under 30. And today I haven't seen her do them more than 15x in a cluster! We are so thankful things are changing so quickly and praise God that He is using this medication to begin to heal Myra of this seizure disorder. We are continuing to pray these spasms stop completely and the hypsarrythmia is discontinued for good after this 3 month treatment.

In the mean time, we have some side effects we are praying are minimal during these next weeks. She has been slightly more irritable here and there, but nothing unbearable. But, I have been told to look for high blood pressure, developing diabetes, electrolyte imbalances, extreme irritability, high risk for infection due to immune suppression, and massive weight gain that is only temporary. There is a 4% mortality rate when using this drug. But, God is capable of protecting her from these side effects and I feel a peace that He is going to.

I had a really rough day on Tuesday when I was originally supposed to go into the hospital, questioning whether to start this treatment or not. I went back and forth all day long, almost hourly, trying to decided between two worse-case scenarios: blindness (Vigabatrin) and death (ACTH). It sounds extreme, but it is an awful decision to have to choose between two medications that are both going to possibly be harmful to your baby in some way. You would never choose to hurt your child, and to be put into a situation to have to choose is agonizing. But, I decided that God knew this would be a decision we would have to make and He would help direct us. The more I thought about His ability to protect her, the more I didn't feel the weight of the decision. The more I didn't think about it as blindness vs. death. The more I started to feel peace that this medication would be one more step toward the healing we feel God is doing in Myra's life, and not life-threatening to her. So, I do feel peace about starting to give her ACTH. I still get this ill feeling in the pit of my stomach about 10 minutes after giving her an injection and tears still fill my eyes, but I don't fear for her life. I will do my part in the steps toward healing, but I will leave the end result up to God.

I have been away for 6 days of the last two weeks, so my house and kids are starting to show it. It's been another rough few weeks for us, but we've had a lot of support and help from friends and our church. We are very blessed with the friends we have. And we are thankful for all those lifting our family up in prayer during their own quiet times with God. He is faithful to hear us and respond. And He is responding. Your prayers are effective, so thank you so very much!

New Diagnosis: Infantile Spasms

After a month of contacting my neuro here in Fort Wayne, seeing a neuro at Riley Children's Hospital, and still not getting Myra's 'possible' seizures under control, I was getting quite frustrated. After a bad reaction to a new drug this past weekend, I called both neuros Monday morning. Dr. Calm at Riley has moved to another hospital, so I needed to find a new neuro at Riley. As part of God's intricate plan, I got in touch with the "epilepsy specialist" at Riley, Dr. Z. After hearing a summary of Myra's history and current condition, she felt I needed to go to Riley's ER as soon as possible, be admitted into the hospital, have a repeat EEG and whatever other tests to figure out what is going on with her. It seemed a little extreme at first, but Mr. Prince and I just are wanting answers and figured this was the best way to get them at this point. So, I head down to Riley Monday evening (5/3/10). By God's grace Myra did not cry on the way, even though she had been crying almost the entire day.

I was in the ER from 11:30pm to 4:30am (which was filled with around 6 doctors/nurses/residents waking Myra every 30 min or so and her subsequently crying herself back to sleep after every one) and it was 5:45am by the time we were admitted and settled into our room, which we shared with another baby who was up and crying. Myra's white noise machine was our life saver while we were there and we fell asleep quickly. At 7:15 we were awaken by the nurses saying we had an EEG immediately. 1.5 hours of sleep was not adequate for either of us and the EEG did not go well. I'm guessing the results were inconclusive because we started a 24hr video EEG around noon that same day.

Myra after IV, not happy.

Sleeping in between doctors bugging her.

They glued a bunch of electrodes to Myra's head and I hit a button each time Myra had an 'episode' and they recorded her on camera, too. I could see the EEG running right next to another little girl's. It didn't look normal, but that was all my expertise could tell. My mom came to help for the night. It was a long night, but we got a few hours of sleep. In the morning they transferred us back to the infant unit in the hospital while we waited for results for 6 hours.

Video EEG

Dr. Z came in and said they have diagnosed her with what were called "Infantile Spasms." I had heard of these before and researched them myself and knew it wasn't a good diagnosis. Four doctors, including two pediatric neurologists, had seen these 'episodes' and did not think they were Infantile Spasms (IS). She has atypical EEG findings and atypical physical symptoms of IS.

Infantile Spasms are a catastrophic form of epilepsy that usually starts around 4-6 months lasting until around 2. They are abnormal brain waves which cause clusters of 'spasms' or specific jerky movements that each only last a few seconds or less. Children can lose developmental milestones at the onset of spasms and prognosis is better the quicker doctors get the spasms to stop. The intellectual prognosis for children with IS is generally poor because many babies with IS have neurological impairment prior to the onset of spasms. Spasms usually resolve by mid-childhood, but more than half of the children with IS will develop other types of seizures.

Myra's spasms have increased since they started on March 21st. They started with just one or two clusters a day of 1-3 spasms. They increased on week 3 to 4-5 clusters a day of 20-25 spasms. Week 4 she had 5-7 clusters a day of 50-60 spasms. She was given Keppra on week 4, and they decreased the spasms to 5-7 clusters of 20-25 spasms a day.

During the video EEG she was recorded to have over 10-15 clusters of 20-60 spasms (5/4/10).

Today (5/6/10) from 7am to 2:30pm she has already had 6 clusters totaling over 700 spasms.

Choices for treatment are few and all have the risks of some horrible side effects.

Choice A is Topamax.
This is an anti-seizure medication that has a small likelihood of helping control IS. It would be a month before determination could be made whether it was helping control the spasms. Side effects of Topamax are kidney stones, UTIs, decreased sweating, suppression of cognitive abilities, decrease in appetite.

Continuing spasms for another month would carry it's own side effects.

Choice B is Vigabatrin.
This is not FDA approved and only available in Canada. Side effects are changes in white matter brain tissue after MRI scan when treatment is stopped (inconclusive whether damage is done because most children already have brain damage and it's unknown what limitations they might already have), and significant peripheral vision loss in 1 in 3 children.

With cortical visual impairment, Myra may only have her peripheral vision in tact, so we are not willing to subject her to more damage.

Choice C is ACTH (adrenocorticotrophic hormone).
ACTH is supposedly the most effective drug for IS (60% will stop having spasms after treatment). But many children have a recurrence of spasms after 2-12 months. ACTH is given through injections twice daily. Side effects are serious and potentially life-threatening. Side effects are increased appetite and excessive weight gain in the face and trunk, high blood pressure, electrolyte imbalances, high blood sugar, changes in mental status because of high blood pressure, and bleeding from the gastrointestinal system. Weekly blood pressure checks and blood draws help monitor effects. Mortality rates are around 4%.

So, choices are bad, worse and horrible! I feel like we just got kicked in the gut while we were already down...

We have contact Dr. Z to start ACTH as soon as possible, given Myra's exponential increase in spasms in the last day. It will probably take a week to start treatment.

Please, please, please pray for Myra. Pray these spasms stop before we even start ACTH! I know God has used Myra's miracles in the past to grow many of His children and I'm praying He uses this opportunity as well.