Pretty Pink Cheeks

Don't you just love her little pilot cap? We bought upon the suggestion of our audiologist who thought it would be hamper Marley's ability to pull off her hearing aids and plop them into her mouth. Marley has mastered the skill of pulling out her hearing aids so well- even if they are attached to her clips. We either have to have a very watchful eye or throw the pilot cap on. Fortunately, she doesn't seem to mind. So what do we do when she figures out how to take off the cap?

The saga of the sleep apnea and snoring has been temporarily resolved. A 2 week course of antibiotics has cleared up her congestion greatly though it hasn't been eliminated. But no more supplemental oxygen, no pulse ox, and most importantly- no surgery. Her next procedure will be Friday, November 13th (yup, you read that right) for her sedated hearing test, MRI and CT scan. I'm sure that the CT scan will reveal that her adenoids are enlarged and I'm sure that at some point in the next year, she'll have her tonsils and adenoids removed- but for now it can be avoided.

So yes, I chose for my child to have anesthesia on Friday the 13th because I figured it would be a light day in the OR at Children's Hospital as many parents are way more superstitious than I am. Hopefully, things will go more smoothly than they have in the past.

On a bright note, Marley has been accepted into a University of Washington study on eye communication in toddlers with developmental delays. She has been randomized into the treatment group and will get 16 weeks of treatment. And it's all free!

Sadly, Marley has not grown in the last 4 months. While she is height/weight proportional her head is not growing which means her brain is not growing. I feel like I feed her so much and just can't understand why she doesn't grow. Ah, it's probably that darn unknown genetic syndrome. But it is infinitely frustrating! Our pediatrician has mentioned Marley getting a G-tube which means she would get feedings at night through a hole in her stomach. I am not thrilled and have not agreed to that plan. I have so many misgivings about G-tubes- most importantly, it's just one more thing that means that my kid is not normal.

I know Marley is not normal, typical, average- she never will be. But why the fuck do I have to subject her to a surgery and a way of life that completely sets her apart from her peers? I wish I knew just 1 other mom who understood what I was going through and the hard choices I have to make. I just wish there was one other mom out there who also had to face everyday with the unknown.

Okay, enough of that. My intention is to at least end each post with something positive. Let's see...Oh, Marley is getting 3 new teeth...which means...she can eat more, she's developing and most importantly- we don't have to see a pediatric dentist to figure why she hasn't gotten in almost all her teeth yet. Yay!!!

Really lame update

So Marley's health saga continues. Marley has always been a congested baby. She drools almost constantly and this whole summer I have had to keep a rag handy to wipe the ever-present stream of snot dripping from her nostrils. And with congestion comes snoring. Big, loud, keep your parents awake snoring.

About 3 weeks ago the snot production stepped up a notch and the snoring became deafening. And then came the apnea. Apnea simply means an absence of breathing. And that's what Marley does. At first in only lasted a few seconds, then the periods of apnea became longer. 5 seconds, 10 seconds- followed by a gasp and often a cough. Long enough for me to lie in my bed listening, counting and praying that she would just take a fucking breath!

I made an appointment with our pediatrician but could only get fit in with one of the residents, Dr. Radesky. Ah residents! Bless their hearts. So young, so eager to please. She was able to get Marley in for a sleep study within a week, when the next available appointment wasn't until January. She called me after hours and even gave me her cell phone number.

On September 30th, Marley and I arrived at the sleep center at 7pm for her sleep study. I was dreading this test, and rightly so. Poor baby was hooked up to so many wires, had a nasal cannula stuffed up her nose, and had her head wrapped in layers of gauze so she wouldn't pull off the electrodes stuck on her head. How could anyone sleep that way?

After 2 attempts to rock her to sleep I finally had to just let her cry it out. She kept rubbing the nasal cannula out of her nose so the sleep tech had to keep coming back to pop it back in which woke her up, made her cry and made for one long, very miserable night.

The next day, Dr. Kifle the sleep specialist called to tell me that Marley failed the study big time. She recommended that we start her on oxygen at night and get into see her ENT, Dr. Sie ASAP. After much prodding and pushing on my part and on the part of our pediatrician, Dr. McPhillips, we can't get in until September 14th. Fortunately, it seems that she will have surgery soon after to remove her tonsils, adenoids and have a broncoscope to evaluate her trachea.

Hopefully, we can also coordinate the MRI and hearing test as well so that she won't have to be sedated again 2 months from now. In the meantime, we've got blow-by oxygen directed at Marley's face while she sleeps, a cool-mist humidifier pumping out some pretty cold air, and a pulse oximeter strapped to her foot to monitor her O2 levels while she sleeps. For the most part, her O2 sats stay in the 90s though they do occasionally slip into the high 70s. A quick look at Marley's room may remind some of a hospital NICU but we are adjusting to all the equipment- even the 75lb O2 tank sitting in our bedroom.

I hate that she has to have another surgery. This will be the 3rd one in a year. Every time I think we are safe- that nothing else can pop up, the rug gets pulled from under me and I feel so defeated. I'm hoping that removing her tonsils and adenoids resolve her snoring and apnea but if it doesn't then what? I'm not ready to explore that possibility.

Marley the pirate

Last week we met with a wonderful opthamologist, Dr. Cadera who recommended that Marley start wearing a patch over her right eye 2 hours a day to strengthen the vision in her left eye. While we're pretty sure Marley has decent vision, but she is clearly right-eye dominant due to the coloboma in her left eye. By patching we can ensure that she retains vision in her weaker, left eye. So far she has been tolerating the patch rather well. If I can keep her distracted she whines much less.

Dr. Cadera is also confident that Marley will need glasses to treat her strabismus. The patch will strengthen her eyesight; glasses while straighten out her eyes. He predicts she will get glasses within the next 6 months. How long she will need them depends on how long it takes for her eyes to straighten.

I find glasses awfully adorable though I can't imagine how we will manage hearing aids and glasses. I have promised Jason that he can pick out the glasses, something in his opinion "not too girly". I think he finds Marley's pink and purple hearing aids a bit too much.

Today I took Marley to a nutritionist. She has unfortunately lost 1/2 lb though she has gotten slightly taller. At 17 months her body is very slowly morphing into that of a toddlers though she still can't crawl or walk. Strangers still guess her age at 6 months. Sigh.

But OT and PT are going well. Marley continues to make progress though at a snail's pace. She loves to stand, supported by our fingers and she is constantly giggling and smiling. She puts out her arms when she wants to be picked up and she whines and fusses when she doesn't get her way. She still babbles constantly but has no distinct words. She is constantly "communicating". She loves music, especially hip hop and pop, and watches my mouth intently when I sing to her. She wiggles constantly, rolls everywhere and loves being thrown into the air.

On Friday she was scheduled to have a sedated hearing test and MRI. It once again got cancelled when a nurse noted in her chart that the cardiology clinic has forbade any elective sedation for at least 4 months after her cardiac cath. The more time I spend at Seattle Children's Hospital, the more I am disappointed. I mean really, how hard is it to schedule an MRI? Are they just complete morons? I am relieved that Marley won't be getting poked a zillion times but geez, what does it take to get them to do something right?

Let's rock...and roll

In an effort to not seem completely morose I think I should mention some of the highlights with Marley since her our horrible experience with her cardiac cath.

1. Marley is rolling. She's a rolling fool. When she first learned, it seemed like it was the only thing she wanted to do. Now that she has accomplished rolling we can move on to her next gross motor skill- moving from a lying to a sitting position.

2. Marley is growing! In the last 4 months she has grown 2 inches and her head circumference has grown too. She is still holding steady at 17 lbs but that's okay. I almost feel like her body is slowly turning into that of a toddler's. She's leaner, longer- all that rolling and twisting is given my chunky, little baby some sweet abs.

3. Marley now has 4 teeth. Sharp, little, gorgeous teeth. She loves to grind them together and flash them when she makes her happy grins- and she makes lots of them!

4. Marley is giggling a lot- and at totally random times. A year ago as we were emerging from 4 months of hellish colic I could never have imagined I would one day have a giggly baby. She has the sweetest chuckle I have ever heard.

So all in all, doing pretty well. We've also had to deal with impetigo, pink eye, constipation, allergies and some unknown skin rash- but things all easy to deal with in comparison to her cardiac issues and seizure disorder. In August, Marley will need a sedated hearing test and MRI and I've promised myself it WILL be the last anesthesia for a long time. We also have a cardio follow up, neuro follow up, nutrition follow up, new neuro consult and ENT consult. 

My little unicorn

Marley's cardiac cath was on Friday.

We checked in at 7:15 am and surgery began at 8:30. It was tough to let her go but I felt confident that she was in safe hands. Surgery was over and she was transferred to the PACU by 11am. The cardiac surgeon came out and let us know that the cath went well, that he was able to clearly identify the stenosis in her pulmonary arteries and the coarctation in her aorta. He ballooned the areas, but being so flexible, they resumed to their original state. While he couldn't "fix" her that day he did tell us that she will probably just need another cath when she's a few years older to place stents to keep her arteries and aorta open.

Not exactly what we wanted but at least she was okay and we found out the problem is fixable- just not for another few years.

We went up to her hospital room to await her arrival. 2 hours passed before she was wheeled into the room. The nurses explained that she had a small blood clot develop in her right leg where the cath was placed. It seemed to have resolved on it's own but the cardiologists would be up momentarily to talk to us about it. Okay...

A couple of surgical residents arrived and took a look at Marley's leg. It was definitely mottled, dusky and cooler than her other leg. The doctors tried to find the pulse in her foot (pedal pulse) but couldn't locate it. I admit, it's a pretty tough endeavor what with Marley being a little chubster, but they weren't having any luck.

They discussed with me starting Heparin, a blood thinner to help resolve whatever clot might possibly be there. I concurred. I did until I found out that they had to draw her blood every 3-4 hours to check her coagulation levels so that they could titrate the Heparin dose to a therapeutic level.

Blood draws every 3-4 hours!!! Have I mentioned before how difficult a stick Marley is? Squeezing water out of a cactus with my bare hands would be an easier feat! I suggested starting another IV. As hard as that would be, I wanted to save her the torture of multiple needle pokes.

Well easier said then done. One of their most experienced IV nurses poked her 2 times. No dice. Then a phlebotomist did 2 heel sticks followed by a finger stick. He got enough blood but it clotted by the time it was brought to the lab. In between the screams and tears (mine and Marley's) a doctor would come by trying to find the pulse in her foot.

I was fed up. REALLY FED UP! I just couldn't take it anymore. I was ready to just pick her up, pull out her existing IV, and take my baby home. The nurses rationalized with me and Jason threw in his two cents which I think calmed me down. I asked the doctors what would happen if we just couldn't get blood out of Marley. Their response? If it takes 27 pokes, we will get blood.

Gee, what a great plan!

I agreed to one final attempt. This time I was promised that Cindy, an IV nurse with hundreds of years of IV experience would attempt the impossible. I bargained for some type of pain medication to help lessen Marley's pain, and the doctors agreed to give her Morphine. It may not completely take away the pain, but it would mellow her out a bit so that perhaps she would scream.

When Cindy arrived I quickly left the room to take a walk. I felt guilty for leaving my baby but I just couldn't see her go through another painful stick. When I returned the IV was in her head (yikes!) but she wasn't crying and I felt so relieved. That was until Cindy told me that she had to shave a small potion of Marley's hair to insert the IV. She had wrapped up the hair in a bandaid and ceremoniously handed it to me exclaiming, "her first haircut!" Yup, that was it. I lost it. I started bawling uncontrollably. I couldn't believe my baby's first haircut was in the hospital. 

But the IV was in and the blood was drawn. The IV actually worked pretty well for at least 2 blood draws until the vein collapsed. And the Heparin worked quickly. Within just a few hours her leg's appearance resumed back to normal and things were starting to look up. And gosh did she look so cute with this funky IV tubing sticking out of her head. She became my "little unicorn" and was nicknamed appropriately.

The next morning I left to go home and shower and when I returned Jason did not have very good news for me. The doctors STILL couldn't locate her pedal pulse and were now threatening to keep Marley for another night if her pulse could not be found. Oh, that was the last straw for me. I had the current cardiologist paged to our room immediately. 2 young doctors arrived. They explained the situation while I tried as best as I could not to bitch-slap them. Fortunately one of them was finally able to find her pulse, of course in a spot on her foot that they had not evaluated earlier. She suggested that they mark the spot to which I responded very sarcastically, "wow, that is the smartest decision you guys have made since we got here!" Not surprisingly, I did get a dirty look.

The mark was made, the heparin was discontinued and we eventually were discharged home with a prescription for baby aspirin that Marley needs to take every day for the next month. But I didn't care. We were home and consequently, I had the best mother's day ever.

But I feel really let down by the doctors at Seattle Children's Hospital that I entrusted my baby with. Why didn't they mark where her pulses were before the surgery? Why didn't they start another IV in the PACU when she was still asleep when they discovered the clot? Why did it have to take so long to get the Heparin started? I'll probably not have any of my questions answered. Maybe it doesn't matter anymore. But I can't shake the anger and frustration I have towards the people that were suppose to be the experts.

And poor Marley. She was such a trooper through all of this. It tore my heart out every time she got poked. I don't know who was in more pain, her or me. 

Since we got home she's been more emotional. She's very happy but it's hard to put her down for naps and bedtime. She has become more clingy, more needy and I can't help worrying that she was scarred by the events at the hospital. It just doesn't ever get easier.

A step forward?

I really can't tell where I'm at.

We met with the neurologist who recommended (though Marley's EEG was normal and she hasn't had a seizure in over a month) that she start on the anti-seizure medication, Keppra (or the generic which is too hard to spell).

I can't say I was surprised and if I was looking for a doctor's appointment where there seemed to be a solution to a situation, this was the one. His rationale was that most likely she will have another seizure in her lifetime- be it febrile or complex, so why not try to nip it in the bud before it becomes the big, ugly monster that a seizure disorder can quickly turn into? Sadly, I had to agree. In my heart I know she will have another seizure. It's only a matter of time. And I can't wait on edge for the next one to hit at anytime. It's like living on the San Andreas fault in a  flimsy little glass shack. I'm insane if I didn't think the "big one" will hit in my lifetime.

Unfortunately, the Keppra is no guarantee that the seizures won't happen. Isn't that completely fucked up? Fortunately, Keppra has few side effects- mostly sleepiness. 

So after much hemming and hawing we started giving it to her a week ago. Instructions were to start at 80 mg daily for 1 week then increase to 80 mg twice a day. I haven't mustered up the courage to move to twice a day. Not that she's having any side effects- hell, I can't really tell. She sure isn't any sleepier but she does seem more agitated, which could just be normal teething behavior. It's constant teething around here nowadays.

I just didn't want to go there. I just didn't want to admit that my baby has to be on medications. I use to say to myself when Marley was an infant, "with everything she has, at least she doesn't have seizures or is on any medications". Strike that. I feel so defeated, like what do I have to look forward to? We're still diagnosis-less, we still don't know what the future holds for her or us. There are no answers, no direction, the road is empty, wide open and there are no signs directing me as to which way to go.

So for those who see me on a daily basis, or maybe weekly or hardly at all- when you ask me how I am or how Marley is, I apologize for my vague answer. Honestly, I'm just okay and so is Marley. I wish I could be more cheerful, more hopeful but I just don't feel that way. So please don't be put off but my meek smile; I really do appreciate the gesture.

Seizures just suck

I wish I had good news to update you with.

So Marley had a febrile seizure the day that she got her Hep A, Varicella and MMR vaccinations. I was so unprepared. One moment she was acting totally normal then she screamed and seized for about 45 seconds. After a visit to the ER and a night in the hospital, we came home...all very exhausted.

Nine days later, after she went to bed I heard her wake up, moaning. She eventually fell back asleep and when I went to check on her I noticed that she was very stiff and barely breathing. I picked her up and she was very lethargic- rather postictal like she had just had a seizure. As I held her and we rocked, the right side of her body seized up. She was crying and her head turned to the right while her eyes deviated towards the right too. She was breathing but completely unresponsive. Once again we went to the ER and she continued to seize for the next 2 hours. She eventually fell asleep, and when she woke up a half hour later she was back to her usual goofy self. A CT scan was performed and came back normal. We were discharged with rectal valium but no anti-seizure medications. The reason being is that kids are not prescribed anti-seizure meds until they have had at least 2 real seizures.

So we went home and waited...on the edge of our seats. Marley slept with us for at least a week before I felt comfortable enough to let her sleep in her own crib. Since that horrible night I check on her constantly. I haven't had more than 3-4 hours of straight sleep since. I am exhausted.

Marley had an EEG on Monday that was normal. Great, right? Well a normal EEG doesn't mean she won't have anymore seizures. I know in my heart she will have another; its just a matter of time. I teeter between complete paranoia and total despair. I try not to think about it too much because the fear is overwhelming. I can't prevent it, I can't know when it will happen...I just know it will.

We meet with a neurologist next week. I have so many questions. I don't even know where to start.