Laughs, giggles, and reflecting on year 1

So the good news is after 11 months, Gesina laughed and giggled! And I got it on tape!

She is a daddy's girl and finds him to be the coolest person she knows. She also smiles at new voices. I'm not taking it personal that when I talk, she cries louder or when I pick her up, she fusses. I know I'm the one giving her meds and trying to get her to eat so I'll take the "hit" for now but I will turn her into a momma's girl eventually!

In an effort to add more fun to life, we went shopping. Dr. Aunt Amy decided to jazz up Gesina's look and she wears it well...

So things for Gesina are going well. She is having more good days than bad lately and you'd think I'd be ecstatic. But as it gets colder, the memories of the NICU are sneaking up on me. Seeing Gesina ready for her g-tube surgery, with all the needles and tubes, I had a moment where the NICU emotions washed over me like a tsunami. Of course, that was quickly put into perspective with the "she's going to die soon" crap. But I anticipate these emotions are going to come and go as we move into the winter months.

As we get closer to Gesina's birthday, I've been reflecting on all that we have survived in the last year. What we've gone through is truly mind boggling. Just when you think it can't get worse, another doctor tells you your baby's going to die. I now look at all the products marketed to new moms and their babies, with their constantly present disclaimers that "if left unattended, your baby could die" messages that used to terrify me, and I get mad. No mother should be made to live with the constant fear that something horrifying is going to happen to their baby. Cut new moms some slack. I was just reading recently that if I put a jacket on Gesina in her car seat, I could INTERNALLY DECAPITATE her. Seriously? That's the messages you bombard new moms with? Let the mom's with medically complex kids worry about their babies dying, because it might happen to us. Leave the mom's with babies born healthy alone to enjoy motherhood. It takes some serious soul searching and gumption to survive being continually told that bad things will happen to your child and that milestones may never be reached. To pull yourself out the depths of despair that you feel as you initially here these things, and come around to finding the silver lining every time is emotionally draining and exhausting. I'm sure at some point I'll smugly announce that this experience taught me "how strong I am" or "how to be a good mom" but I'd rather have not been tested this way. I'd rather not have to live with the knowledge that my beautiful, perfect baby girl may leave me early. No matter how much I love her, protect her, and provide her with the best care available. That this is NOT a just world, where only good things happen to good people; that I can't WILL her to good health. So those mothers out there with perfectly healthy babies, kiss them, enjoy those milestones and do NOT obsess about all those negative messages trying to scare you. As for me, I will continue to reflect on how we survived the last year and be a bit melancholy until December 4th. But come December 5th, we will begin Gesina's next year of life with a fierce focus on not just surviving our lot in life, but THRIVING. More adventure, more experiences, and more giggles. I can't keep waiting for things to "settle down" before we embrace all that life has to offer us, because settling down may never happen. Instead, we are going to thrive. We won't put off for tomorrow the fun we could have today. Gesina and I will run a 5K together, even IF I have to push her along in a jogging stroller (and a blanket over her face because she hates the wind :-)). Year 2 is going to be awesome and I can hardly wait.

Hope Floats

My neurologist, Dr. Shah called and we now have a little hope. Essentially, it all comes down to a judgement call. Clinically, Gesina fits the description of Ohtahara Syndrome (OS). So if you were to describe her seizure experiences, the timeline of her seizures, and the difficulty in controlling them with meds so far, she fits OS. However, if you look at her EEGs, she does not fit. She has not shown the suppression-bursts described with OS when you read her EEGs. Thus, some neurologists would conclude that she has OS, however Dr. Shah is not one of them. To make such a damning (my word) diagnosis, Gesina would have to be textbook OS for Dr. Shah to call it. Why? Because the label does not help her and it carries the weight of a miserable prognosis. It doesn't change her treatment plan of seeking the best meds to control her seizures as stopping the seizures is paramount to protecting her from further brain damage. Thankfully, she does not seem to be having seizures that we can't see clinically, meaning she isn't just seizing all the time.

So, what now? A few weeks ago we tried a new drug with the possible side effect of a "deadly rash"... well my sensitive little monkey started getting a rash so we had to stop that immediately. We have her started on a new drug now that is supposed to help "normalize" the EEG according to what I've read. The Children's hospital added another so she is on 4 seizure meds at the moment... we are weening her off one of them but it looks like we are super serious about getting these seizures in check. At this time, we can't say she doesn't have OS but we are not convinced she does. I'm okay with that because it really does give me some hope... maybe she won't die in a year?

As for the g-tube... get a g-tube they said, it'll make your life easier, they said... after 4 days of constant irritation, crying, vomit, and tylenol I went back the GI doc this morning. They've determined her pain isn't g-tube related (for medically complex kids, I think the "not my problem/domain" response is common). The gj-tube was suggested but thankfully she has finally stopped crying and I'm more slowly introducing food through the tube. I'm physically and emotionally exhausted at this point. But unlike everyone else I know, I haven't been as sad. The reason why? I get to look at this beautiful face everyday and she thinks that today is no different than last week... and that's the way I like it!

 

When the worst-case scenario becomes reality

I'll start this post by saying, we will be seeking a "second" opinion from my usual Neurologist. With that said, we went into Children's of Wisconsin for a g-tube and came home with a few years to live.

We went to get Gesina's g-tube and thankfully, that went well. However, in our hospital room a few hours later Gesina started having weird episodes that she had actually started having the day before. The episodes the day before surgery were odd enough that I had gotten video and was going to keep an eye on it. Knowing Gesina had been slightly under medicated for her seizures (weaning off one med, ramping up another) I planned to keep an eye on it. Well, in her recovery room she had the episodes but more severe and more often. She would startle with her arms, take in a loud quick breath, then cry like she was terrified. This would happen quite a few times over the next few hours. We were quickly transferred to neurology for another video EEG. They came back with the news that it's not infantile spasms, which I was happy about, until they told me what they do think she has... which is worse than infantile spasms.

The neurologist at CHW believes Gesina has Ohtahara Syndrome, which is very rare and characterized by progressively worsening, difficult to control seizures with profound and severe mental and physical disability. It is my understanding that those with this syndrome usually die at about 2 years of age. With luck controlling the seizures and loving care, she may live to approach the double digits in age but she will not develop much more than she has.

I'm in such a state of shock that while I had an initial wave of emotion at the hospital after the doctors left, I'm now just functioning on autopilot. I've accepted that I'm just going to cry whenever the emotions hit me. On a cognitive level, I know that this changes everything. My whole plan or play book on what it will be like to be Gesina's mom is going to be thrown out the window. Tummy time? "F" that. Worrying about whether or not her legs are perfectly straight for when, 30 years from now, they figure out how to "fix" CP issues with gene therapy? Not my concern. Worrying about Gesina depending too much on the g-tube and that she will have it "forever", who cares. If she hates eating, we use the g-tube. We live for today because we are not promised tomorrow. Spending holidays at the hospital because "she won't remember her first Halloween anyway" doesn't cut it anymore. We are living every moment up to the best of our ability. I don't get to be a mom for very long, so I have to fit as much joy and love into these days as I can. Gesina doesn't get to live long, so she will experience love, joy, beauty, and all that our life has to offer her. She will not spend every waking moment "trying" to regain "normal" abilities. That isn't in the cards so we are not going to force it. We will spend every waking moment enjoying what we have. So every one one note, come December 5th, we will be having the best, most ridiculous tiara-wearing birthday party I can manage in my mental state. We will celebrate me being 40 (on December 3rd) and Gesina being 1 and not because we are sad or in mourning, but because I made it to 40 and she made it to 1. This is really no small feat. I promise you I will cry, which is why I need to have as many friends there as I can. I don't ask for help, but I need it now. I need help keeping my spirits up as I focus on the joy of life while I have Gesina. I'll also need help in the years to come and when I lose Gesina. I'm not sure I'll recover from this but because I'm no longer concerned about the future, I'll not worry about that until it happens.

Send positive thoughts our way as I try to go through the steps of mourning as quickly as possible because my little Gesina monster deserves better. I'll let you all know what my regular neurologist says and you never know, maybe this will just be one of those times a doctor freaked out for no reason. But I'm not going to be counting on that. It doesn't hurt to live each day like it's your last anyway.

Labels and services

We now have a Physiatrist that I like who seems like a doctor that will help coordinate Gesina's treatments and therapies instead of me feeling I have to coordinate it all. The good news is she is proactive on getting Gesina going with cerebral palsy treatments. We have added baclofen to the medications list (a muscle relaxer) and next week Gesina gets fitted for AFOs for both feet. An AFO is an ankle-foot orthosis, which is a plastic boot fitted specifically to Gesina's feet. The AFO will encourage her feet to grow in appropriately and not let her muscle tightness cause her feet to turn inward or anything that will further complicate her potential for movement. It was so nice to talk to an expert on CP because she filled in so many missing pieces on why Gesina responds to the world in the way she does. I feel that we finally have the doctors we need to fill the team for a little while. The bad news is Gesina finally got the diagnosis/label I've dreaded for a long time. She was diagnosed with "congenital quadriplegia". It's the worse type of CP linked with the worst outcomes, at least statistically. I know every child is different and the severity of her CP hasn't been written down yet but I can't help but have a reaction to that label. I'll come around but it just hurts the soul a bit.

In other news, Gesina has been accepted into the Medicaid program in Wisconsin! Yay to secondary insurance AND it is back dated to April 1, 2014 meaning her April hospital stay and beyond will be covered. Oh and I can now switch from early intervention PT and OT to private therapy that uses a medical model of therapy. This is great because the medical model will allow for more therapy per week AND won't consist of just telling me to work on things with Gesina. I still find that I don't have the time to do all they want me to do and I can't handle the pressure of it. Now the stress can be relieved a bit as now I have therapists for that and some of the stress is off of me. Whew. I did learn that only kids who would be institutionalized if their parents didn't take care of them get approved for the Katie Beckett waiver for medicaid. This is to encourage the families to care for their special needs children and is cheaper than institutionalizing these kids. This has scared the bajeebies out of me. My child is so severely disabled that she would be institutionalized? What happens if I die? So I'm frantically creating a "taking care of Gesina" document, looking into writing a will, and getting some life insurance ASAP. Either way, we are inching towards her first birthday and she's here. I'll keep getting her the doctors, services, and medical devices she needs but with less stress from her OT/PT stuff, I'm going to just focus on being her mom, loving her, and having more fun with her. On that note, here she is trying on daddy's glasses :-)

10 months and 1 day

Gesina is 10 months and one day old today. She's had a string of good days (with smiles) and I don't want to jinx it, but I think the stomach bug is over! She is a healthy 16 pounds 12 ounces, feels "sturdy" lately with more head control, and is finally TOLERATING TUMMY TIME!!! Yay! It only took 10 months for this and when I say tolerating, I mean some days she'll stay on her tummy for an hour and others 5 minutes. But she is consistently allowing it to happen... lately. She even smiled in reaction to MY voice this morning and not just Adrian's voice or her iPad. The little monster is turning into a daddy's girl but given time, she'll realize her momma's pretty neat too!

The good news is that Gesina does NOT have infantile spasms :-) We are so happy to hear that! The EEG was abnormal showing spikes and at times, rhythmic spikes that are like a precursor to a seizure. But during the whole 72 hours, she did not have a seizure. The neurologist is changing her meds because our goal has always been to prevent seizures, not just treat them when they occur. Her new med is Lamictal and it takes 8 weeks to get her ramped up to the right dose. One side effect we have to watch for is a deadly rash... no big deal, just a deadly rash!!! But if there is one doctor on Team Gesina that I trust implicitly, it's her neurologist. He saved her life when she was born so he's used to these life and death decisions and since he was right last time...

Last week I got a second opinion on the g-tube from a new GI doctor that works out of an office close to my home a few days a week. She also works in the same Milwaukee office as the current GI but comes recommended by Gesina's therapists. She was much better about my concerns and reassured me that Gesina needs the g-tube to ensure her limited energy is spent on developing and not on eating. Honestly, I'm still not convinced she needs it as she has chubby cheeks now and is doing so well with food. BUT, if there is any chance, no matter how small, that getting a g-tube will help her advance her development in this small 3-year window when her brain is its most plastic, then I have to try. She is a slow eater (like her momma, grandma, and great-grandma) so that does take energy. Also, I've read that kids with CP need more calories because they burn so much energy with the constantly tight muscles. Those things added together, I figure I'll get the g-tube and may or may not need it. But at least I can say I've done everything I can to help her develop as best she can. Blah. But that happens, with an overnight hospital stay (and a couch for momma), at the end of the month. Oh, and in a way I get the best of both worlds. The previous GI doc is doing the surgery because he is the first pediatric gastroenterologist in eastern Wisconsin and taught everyone in his office how to do this surgery. So I figure, he's the best. But after the surgery I'm switching to the new GI doctor who works in the same practice but has a better "bedside manner." My relationship with the GI doctor and his/her nutritionist is long term and vital so I have to be able to work with them comfortably. I have a pretty stringent selection system for Team Gesina and seem to be going through doctors relatively quickly ;-)

Next up, we meet the only Physiatrist that works in my town a few days a week. She comes recommended by my therapist (who has 10 kids and a few have CP) but our first appointment is in Milwaukee. I'm hoping this will be it for doctors needed for Team Gesina for a while. I'm looking forward to a break from doctors visits and switching out doctors/therapists! I just hope all these med changes and surgery don't mess up Gesina's streak of good moods and tummy time.  

Adventures in Extreme Parenting

I've decided to "repackage" special needs parenting. When times are tough and juggling the expectations and needs placed on me becomes overwhelming, I find myself feeling a little like a victim. That mentality spreads and leads to a sense of desperation and sadness. Also, I find that in general, society doesn't really value parenting, especially special needs parenting. On an average day, I don't need looks of pity when I'm with my daughter. She's beautiful and perfect. If I'm lucky, she'll grow up to be happy and really that's all anyone should wish for for their children. What society does value is "extreme" home makeovers, "extreme" Mountain Dew, "extreme" sports, or any other extreme thing that gets you a sponsor and TV-time. The label "extreme" means that you are tougher than the regular Joe and that you got this... whatever it is. To boost my spirits when I get overwhelmed, I've decided that as a special needs mom, I'm engaged in extreme parenting... that's me, I got this and I'm a bad ass :-D It really does change your mentality as extreme athletes aren't victims, they snack on danger and dine on death! So when I see friends starting a food train because their healthy and fully functioning child has a planters wort, I don't get mad. I laugh, but then, I remember that not everyone can handle extreme parenting ;-) Sorry to offend those who don't get the life altering experience of extreme parenting, but let's face it, you have plenty of other benefits!

So what could be overwhelming about raising Gesina lately? Glad you asked as we have had a laughably horrific few weeks. Since I last left you, Gesina had a stomach bug... well that little bugger never really went away. After 10 days of her off-and-on refusing bottles, vomiting nearly everything she ate, not producing stinky or sometimes wet diapers, and generally being unhappy, I called her GI nurse for the second time and she had me take Gesina to the ER in the Children's hospital 45 minutes away from home. After 3 hours in the car (I work an hour away from home in the opposite direction of the ER), we arrived to a packed ER at 6pm on a Tuesday. It's about 75 people deep and everyone thinks their kid has that dreaded flu that is going around. I didn't have time to stop at home so there I am at the ER with a hungry baby with no diapers, no food, and no 9pm meds. By about 8:30 I ask the nurse if it's going to be much longer because I have no meds and live 45 minutes away. She is great and orders a dose of the meds for me and gives me some applesauce to feed my little Gesina monster. I give Gesina her meds and she promptly throws them up all over me as the nurse calls our name. For the first time ever, I get mean mugged for having a crying baby... at the children's hospital... BY THE WOMAN NEXT TO ME WHO TALKED ABOUT JESUS FOR THE LAST HOUR!!! I couldn't believe it. The hospital ordered more meds and Gesina got an IV drip for mild dehydration. We got home at 3am and we were both exhausted.

Wednesday we wake up and Gesina cries a horrific cry I never heard before. I'm terrified and find out it is her "pain cry". I give her a suppository and tylenol but that doesn't really help. I call the pediatrician who thinks its cramps from dehydration but if it gets worse or doesn't get better, take her to the ER. At 9pm Gesina wakes up screaming her pain cry (which really is horrible and just makes me cry) so I give her her meds, then off to the ER we go. Day two of the ER is even more entertaining as there was a car accident involving teenagers. At first, I'm saddened by the thought but then I sit in the waiting room and realize that I have chosen my seat in error. The family behind me has a very lively dad that is dropping an f-bomb for every other word. So I'm trying to feed Gesina and though she can't understand his language, I can imagine she can feel his anger as he is yelling "How's she supposed to know the car was stolen? She wasn't driving..." with some f-bombs thrown in for artistic value. I'm stuck because if I move (he's sitting right behind me) then I look like I'm making some statement and he's very aggressive right now. So I decide that it's 10pm on a Wednesday night at the children's hospital ER, I'm low on sleep so this is just my form of entertainment. Gesina gets an x-ray characterized as "impressive" by the ER nurse due to the "extreme" gas and poo in her system but no blockage. A suppository and tylenol later, I leave the ER at 6am with a screaming baby and a key for a hotel room because Gesina has an appointment that afternoon for a swallow study at the same hospital.

I'm exhausted but we get through it all. The swallow study shows that yup, now that we've lowered her oatmeal intake per ounce of formula (as demanded by the nutritionist), she may be aspirating but we can't tell conclusively. So days in the hospital and a swallow study later, I've learned that there is nothing they can do that I haven't already been doing. That's over $500 to affirm what I already new. Oh, and Gesina is still struggling off-and-on with the stomach bug and we are going on a month of it now.

Last week we had the all clear from her cardiology appointment (yay), the NICU developmental clinic (where they agreed that a g-tube for Gesina is in her best interests), and a neurology appointment. Gesina has had a few odd instances where she startles and then her eyes roll in a circle. These had happened in clusters of about 3 to 5 at a time. Her neurologist and I hope these are just benign reflexes but decided to do a 72-hour video EEG to rule out infantile spasms (do not Google if you can't handle it, these are bad). We are currently on day 2 of the EEG and it's picture day at daycare! Yup, I have the worst luck in the world. So we decided to go with it and make her electrodes just a Halloween costume. When she grows up and see's these pictures, I'm going to tell her I just decided to dress her up as Rob Zombie or George Clinton for Halloween. Fingers crossed that she just thinks her mom has weird taste in music! Here she is in all her scary glory... grrr!

She's rocking that look if I do say so myself. In the meantime, through all the stomach bug, Gesina has gained the expected amount of weight (wtf). I'm going to get the g-tube but I may still fire my GI. He keeps telling me she doesn't have acid reflux because she's on a good dose of Prilosec. I asked about changing brands and he says they are all the same. I asked about the vomiting a bottle everyday and he says that's to be expected. I asked about her slow digestion, he says she's fine. I can't work with him long term so I have to decide if I want to change GI docs before the g-tube insertion or after.

Next up, pediatrician on Friday where I get to explain to her that Gesina's brain atrophy is not a degenerative disease but the result of a one-time brain injury (per neurologist). Hopefully she will help me navigate the GI doctor issue instead of serving a big slice of the truth pie... as she sees it. Then, the g-tube which is an overnight stay. Not gonna lie, putting a whole in my babies stomach when she's gaining weight, getting taller, and seemingly healthy is not going to be easy. BUT, I recognize that she takes in only 3 ounces a day at daycare so they can't feed her and I barely can so I'll do it... hating every minute of it... because that's extreme parenting for ya ;-)

39 weeks and 1 day old

My little Gesina monster is 9 months old :-) In fact, she is 39 weeks and 1 day old and having been born at 39 weeks, she has officially been on the "outside" 1 day longer than the time she spent on the "inside" :-D Here she is in all her glory with a smile just for being placed in her activity gym...

The last week has been challenging though. We saw the GI doctor on Thursday and even though Gesina had gained the amount of weight they wanted (and went from 12 percentile to 15th percentile) all the doctor would talk about is the G-tube. He is not interested in her acid reflux issues or managing her meds for that, he is just all for the G-tube. We are going to schedule a swallow study as the thinner liquid seems to make her sound congested after a bottle and we need to find out if she is aspirating. After that, we plan to re-evaluate what we need to do next. Needless to say, I left the appointment frustrated and determined to get a new GI doctor. And then... the stomach virus hit :-(

Grandma and grandpa came to visit and got to enjoy the start of the stomach virus. Starting Saturday Gesina started throwing up 5 or more times a day. I was soaked in it and my floors have never been cleaner due to the constant wiping up of vomit. In fact, I've gone through so many t-shirts that today I'm wearing my last clean t-shirt, my free Chicago Cubs shirt... and was accused of being a Cubs fan. Never! I don't support loveable losers as a general rule. I brought her into the pediatrician's office on Tuesday and have spent the last 4 days feeding her 10 mL of formula every 10 minutes and I'm so exhausted. Between Thursday and Tuesday she had lost half a pound even with my best efforts to keep feeding her the appropriate amount of formula. By now I've completely thrown out the playbook and her ideal amounts of formula per day are forgotten. I'm lucky if she keeps down 6 ounces of formula a day. This morning she kept yogurt down and 3 ounces of formula. We are aiming for feeding her every 2 hours about 3 ounces and we'll see if our luck holds.

So, how does this episode relate to the G-tube? I've talked to her many therapists and even the daycare director who is a speech pathologist this past week. They all said something that really halted my thinking on the G-tube. They all independently said that based on their experience with Gesina and other babies like her, Gesina's development is being impacted by her nutrition and acid reflux. The G-tube can help get those two issues under control so we can focus on her motor development, stretching, etc. This fact crosses a line or standard for me. I refuse to let my daughter's development lag just because her mother is stubborn. I can't will her to eat but I can allow her to be assisted in that endeavor so we can focus on more important things. As Adrian said about this issue, with my original path, I would end up with a teenager who can eat but can't do much else. So I thought long and hard on what my reservations are about the G-tube. I don't care if Gesina is "different" as I've always liked different :-) so what I've concluded is that a G-tube represents dependency and I value independence so much that I will do anything to avoid dependency. Now that I can identify my "logic" I realize how silly it is. My 9 month old is dependent anyway! So what if she is dependent on a G-tube. And if she has to have it for life, which is highly unlikely but you never know, than she can still be independent in caring for her own G-tube. So getting a G-tube doesn't sound so bad anymore and now it's time to think about what it would do for Gesina.

A G-tube will not directly help her acid reflux or vomiting for that matter. It only directly helps her nutrition as whatever amount of nutrition/formula she does not consume during the day I can make up for with the G-tube. Indirectly, the G-tube can help with acid reflux and vomiting. How? With a G-tube I can let her refuse bottles because of acid reflux or vomiting during the day and make up the difference at night. This means less pain and more ability to focus on growing/learning/developing during the day. One thing I know about Gesina is when one thing goes wrong (seizure, acid reflux, vomiting, etc.) her abilities regress. She can quickly revert to her 3 month old self that needs to be held at all times or will scream until you give in. I need her issues that cause her to regress to be managed. I have a neurologist working on controlling her seizures, and if I let him, a GI doctor to work on her tummy issues. Once I get those two factors under control, then I can add a Physiatrist to manage her therapies and likely cerebral palsy.

And there it is... I've finally "said" it. There is a strong likelihood that Gesina has cerebral palsy. I had hoped we would dodge this diagnosis because she doesn't show signs of weakness on one side like most babies with her background. But her high muscle tone hasn't gone away and she likely has a spastic form of cerebral palsy called dystonic quadriparesis. Once I have a Physiatrist on board, I'll be able to truly diagnose her type and the severity of it. The good news is therapy makes a difference in outcomes so I'm on it. The bad news is this is another label I'll have to wrap my head around and embrace. Will she ever be able to run a 5K with me now? We still don't know but worse case? I'll roll her along with me in a wheelchair if I have to! As usual, we're down, but we're not out. She's alive, she's relatively healthy, and she's 9 months old :-)