The Cleft Repair Surgery Story and Other Things

Isabella was an ounce shy of 12 pounds on the day of her surgery and measures 29 inches.

She’s in good health and all ready to brave the cleft-combat. The procedures scheduled that day included checking ear tubes for both ears and possible ear tube replacement, possible draining of ear fluid and cleaning out of ear wax, this will be followed by repair. The doctors said that the whole procedure should only last about 2 hours, however, we had to wait for 4 hours before they let us see her again.

Day 1:


600 am we arrived at the hospital

730 we were let in and prepared for surgery

800am doctors gave us the ok to give her daily dose of Keppra

915 we kissed her goodbye and she went with the anesthesiologist to the OR

130 She was wheeled to her room.

While the doctors reassured us that everything went well and that she was perfect and that there was no drama in the OR, seeing her with a swollen bloody mouth and with her face quite bloated from the general anesthesia was difficult for me, the mom. But I had no other option but to trust the doctors that everything went well.
The doctors told us to make sure that she does not put her hands in her mouth, which is really going to make her angry because “hands-in-mouth” is a recent soothing strategy she has recently self-discovered. Issues with acid reflux also raises concern so medication to help with throw-ups (at least for the next 2 weeks)are necessary, with the side effects being that she will be drowsy and sleeping a lot. Doctor also advised us to stick with tube feeding and skip the semi solids for the next 10 days. All these sounds manageable except that my husband and I both feel that since she is uncomfortable she might not be sleeping well and she’d be fussy and stressed and tired which might trigger a seizure.
Instead of giving her a regular room, the medical team has decided to place her in the pediatric ICU for one night just to be on the safe side. The doctors and nurses said that we do not need to worry about having her in the PICU since she’d only be in for a night and and out the next day. We were going to tell the doctors to give us an extra night of stay, even if she was placed in a regular room, as long as we get to stay in the hospital for one more day. The doctors kept trying to convince us that this won’t be necessary. So, though unsure, we tried to be appeased with what the doctors were telling us.
She was not able to eat anything the first day. She had an IV, and did a small bolus of pedialyte via g tube.

8pm she had her daily dose of Kepra on top of the pain meds and antibiotics

Day 2:

1200 am Isabella had a slight fever.

1230 am she started having a cluster of epileptic seizures

245am seizures were finally controlled

The PICU team controlled the seizure by giving her Ativan and eventually some Diazepam. This is on top of her daily dose of Keppra that, obviously was not enough to control her seizures, which are most likely the result of all the stress that came with the surgery. In the midst of her seizures, she stopped breathing, and intubation was necessary.
Isabella was heavily sedated and given extra supervision. She had 2 nurses attending to her at bedside. While it is good that she gets extra care, it is also worrisome for us because it signals the delicate nature of our daughter’s condition. She was hooked on a breathing machine as she was not breathing on her own.
11am according to the PICU team they will start weaning her off the breathing machine’s support. The settings were changed to low, but machine kicks in to provide the oxygen her body needs. Her breathing was erratic.
1pm while still heavily sedated still, she coughed out her breathing tube and decided unconsciously to extubate her self. Doctors and nurses rushed to her bedside. However, for reasons unexplainable, including how she managed to cough out her tube when it is far down her trachea, she started breathing ON HER OWN. Re-intubation was not necessary
2pm she had her intravenous dose of Kepra on top of the other medicines such as antibiotics and those that control pain, calm her and sedate her. Neuro team decided to double the dose of the anti seizure meds.

Throughout the rest of the afternoon Isabella will have brief moments where she would open her eyes and move. She did not smile nor looked at us, but I know she knows we are just there. Still no real food for her, and all she got for nourishment was dextrose.A check of her blood oxygen level was done and it yielded posiive results.


Day 3:

130 am Isabella had another seizure that lasted 5 minutes and was controlled by Ativan. This time we did not receive a phone call. We were notified when we got to the hospital at…

630am Isabella looks much better. The swelling of her lips have gone down, but the bruises on the sides of her mouth was still visible. We understand that bruises take awhile before they become less visible. She is also not heavily sedated anymore. She didn’t cry, but raised her arms when she woke up so she can get held. She started looking around the room and would sometimes like to be propped sitting so she can take a better look around. She also didn’t need 2 nurses to take care of her.

11am We met the doctors at rounds and we were told that we would be staying another night in the PICU to monitor seizure activity for the next 24 hour period since her last seizure. They still need to discuss with the team if the antiseizure meds dose need to be readjusted. CFT docs suggested we do not feed her by mouth for risk of aspiration, at least for the next 10 days. There is a chance we will be transferred to a regular room tomorrow and stay until Monday, if seizure activity is controlled.

Throughout the day we can see the Isabella is slowly but steadily getting back to her regular self. She has not smiled but would start looking at us and wanting to be held, touched or talked to. She also started making the Mmmmm sounds she usually does and would stare at her hand and stick out her three fingers. Although docs said she cannot put anything in her mouth, we let her lick the back of her hand (technically it was not “in” her mouth anyways… bad mommy!).

There was also talk about doing another MRI, and GI doctor recommended that we giver her acid reflux meds to help take care of the surgery site if she throws up. She also started her round the clock feeds, that were adjusted to 25 mls per hour to give her a gradual start.

We hoped she does well over night. Things have started looking up.

Day 4:

We woke up early then went to Sunday mass then right after, we went straight to the hospital. The very first question we asked the PICU nurse was if she had a seizure that night… she didn’t.

We started asking questions regarding discharge. They said that she might still need to stay a night but will most likely get transferred to a regular floor room if a room becomes available. Otherwise, if all goes well, she stays one more night here in the PICU then we can go home on Monday.

From the Neuro standpoint, we were told that her Kepra dose is now 2mls to be given twice a day. That seemed to have controlled the seizures, even if they have reason to believe that she had a high likelihood to have had a seizure because of the surgery. We had a discussion with the doctors regarding her blinking/jerking movements. We discussed the difference of neurological tics and myoclonic jerks. They did add, and stressed also, that as long as it does not hinder development, cause her to regress or to stop progressing at the rate she is already going, it should not be a cause of concern. Most likely her blinking/jerking is a myoclonic jerk and tics would come out, if she is going to have any, around the age of 1 and is most likely triggered by anxiety such as that of feeling awkward in a social situation, among other things that may cause a person to feel anxious.

We were also able to clarify with the neuro docs regarding mentioning the possible need for another MRI, they said that they have discussed it as a team and find no need for it right now, but they may need to request for an EEG. We told them that she has one scheduled in January. But they are going to put an order in to see if it can be done tomorrow morning since we will be here anyways. When they did the EEG on Day2 of our hospital stay, it showed irregular spikes in her brain activity which is most likely a result of her being administered ativan, and since she has not been sedated for more than 24 hours and has had no seizure activity, an EEG will most likely record better brain activity.

The CFT Doctor said we can try giving her semi solid foods via mouth to see how she does. They said to definitely not feed her via spoon… at least not yet. Her first semisolid food since Wednesday is a very yummy 25 ml serving of strained cream soup. She did not like it. She didn’t finish it. She only took about 5mls. Maybe because it is more like gravy and it would be better is it came with chicken. So I gave her some vanilla pudding instead and she welcomed that with an open mouth and an arm swinging, leg kicking dance. However, 20mls later, she threw up… all of the 25 mls and then some.

She did get nourishment via the kangaroo pump and she showed us that she did by passing out her very fist poo. Yey!
She has not been crying like how she used to. It is not so much how her cry sounds, but it seems that she is trying to suppress sounds coming from out of her mouth. She has been awake a lot today, would do occasional “Hmmms”, but she does not sound as frequently as she used to. Perhaps she is still trying to figure out how to move things around in her mouth.
She smiled twice to nanay today and she also gave a little one sided lip smile to her nurse. She also took a bath. Her BNrother and some froends also paid her a visit. Eloi got to hold her and play with her for a little bit.

I know that my son is trying to act all grown up and okay about this whole mommy-daddy-sister-all-in-the-hospital deal, and though a big part me wants to be with me want to be with him at home and leave Isabella with the care of the nurses, or have him be absent from school and bring him with us here, I want him to still have that sense of "normalcy" in his life. I also do not want him to know or learn about "worry", at least not yet... as long as I can keep him from that cause there'd be plenty of time for worry when he grows up. He told us, right before he left the hospital, that he is sure that we aqre going home tomorrow. When we asked him how he knows this, he says it's because it's been 5 days since we've been here. I guess he had enough of all this "separation".
When we kissed her goodbye for that day, we sent a short little quiet prayer that all goes well throughout the night and that tomorrow we will all be going home.

Day 5

We were greeted at the hospital with a talk about possible discharge. The concerns that might prevent her from being discharged is that of her feeding. Their hope is that throughout the day, she will be able to taken in the volume of semi solids and milk the way she regularly does it at home. Although we know that there are concerns regarding throw up we told them that “throw-ups” are a regular part of our life. So, throw ups, though it may not be the general “norm”, it is OUR NORM.
The day went by and the doctors gave us their updates. CFT had nothing more for her except that we need to see them back for a post-op appointment 2 weeks post surgery which is technically only 11 days from today. Neuro says there is no need for an EEG and that we stick to our already set EEG and neuro appointment 2 months from now. Renal also sends their ok and her creatinin and potassium levels are all good. GI also says everything is going well, except for the not feeding by mouth, which for them is not a red flag concern at this point, afterall she just had surgery in the mouth.

The PICU team asked if we feel comfortable about bringing her home despite the semi-solid feeding issue… in unison, my husband and I replied with a yes. They did add that if we do not feel okay with it, we can stay one more night, and for a brief second we did hesitate, but we responded with a resounding “Nahhhh.”

We picked up her set of meds, signed some docs, the beeping machines were wheeled away, the beeping machine attachments were taken out, the IVs were also taken out, and after a couple of pee pee diapers, a poopy diaper, a cat-nap, a bolus feed, 3 hesitant smiles, and a dose of pain meds later… we were sent home.

…and that my friends, is our 5 day surgery story and other things.

What Is Special?

I feel that as my son grows up, he will have his own journey in coping with having a sister with special needs. What I am not certain of is if he will go through the stages of grief like any parent coping with the reality of raising a special child. Will he go through Denial? Anger? Bargaining? Depression? Then, eventually Acceptance? Is he currently going through one of these stages? Or, with the pure nature of his child-soul and child-love, he will jump straight to acceptance? While it may be pointless to think of these things right now, they do cross my mind, but my biggest hope is that he copes with the reality of it much better than I did (I am?).


As I have written before in a previous post/s, we have exposed Eloi to children with special needs ever since he was small, and we have had conversations with him telling him about children being “special” and his sister being “special” I know that in that little mind of his, he is still trying to understand why his sister is “different” and what the word special really means.


A big part of me wants him to not see that his sister is “different”. My wish is to be able to raise my children without the conscious thought that “this is my special child” and “this is my normal child”… they are my children, period. I don’t want him to try and compare her with other children nor do I want to run a household parenting each of my children differently, with the other one getting “special treatment” because of her “special needs”. My wish for Eloi is to look at Isabella as his sister and not be defined as his “special sister”, with the reference of the word special being “different”, “irregular”, “abnormal”, “non standard”, “uncharacteristic”, “strange”, “deviant”, “weird”… if ever that it is unavoidable for her to be defined “special”, I’d want the context of the word “special” be that of being “unique”… just like each and everyone of us.


But what is special? And how do you really talk about it with a 6 year old, in a way that does not degrade nor show division? How do you talk about it with a child without crossing that line? How do I go about discussing what is special and ave him understand the way I wish for him to understand?


Then, one morning on our way to school, we had this conversation.


Eloi: Nanay I have a classmate and it is his birthday tomorrow. It is also his brother’s, who is in a different 1st grade class at our school, birthday. Nanay, how come his brother and him are the same age and they have the same birthday?


Nanay: Because they are twins.


Eloi: But how come they don’t look the same?


Nanay: Not all twins are identical or looking the same. Anytime a mommy has 2 babies in her belly, those babies are twins. Sometimes there’s even a boy and a girl twin.


Eloi: But how come the one in my class does not talk too much, and the other one talks a lot and he does not really listen to us well when we talk to him. He just likes to talk over and over about cellphones.


Nanay: Because that is what he wants to talk about.


Eloi: But he talks about it all the time. It's the same thing all the time. It's always about cellphones.


Nanay: (I can sense some annoyance in his tone)Why don't you try talking to him about something else?


Eloi: I do, but he only wants to talk about cellphones.


Nanay: Maybe it's just because he likes cellphones so much and people like to talk about the things they like. Much like the time you liked talking about wrestling, or Starwars, or Legos. He likes cellphones so he likes to talk about cellphones.


Eloi: He is weird.


Nanay: ( in my head > OMG, the words he learns from his peers!) No. Don't say that. He's not weird. He is special (The boy has Apsergers), and that is okay. You know, much like your sister is special. Do you think she's weird too?



Eloi: No! But Isay does not talk a lot.


Nanay: They are both special but in a different way.


Eloi: Oh, so you mean something is wrong with him?


Nanay: Why do you say that? Nothing is wrong with him. Cause when you say that, it's almost like saying that when we say your sister is special, something is wrong with her. Do you think something is wrong with your sister?


Eloi: No! (very defensively) She’s okay.


Nanay: Yes. She is doing ok. She is just going to take longer than other children her age to learn things because God made her that way. Like your classmate’s brother talks a lot because God made him that way. Do you think anything is wrong with that?


Eloi: Noooo! Nothing is wrong with that. God made them that way.




Quiet Pause…




Eloi: Am I special Nanay?


Nanay: Why do you ask that?


Eloi: Because I talk a lot.


Nanay: Yes you are special. You talk a lot because you have a lot of ideas in your head and that is okay because God made you that way. All of us are special.


Eloi: So, I’m special cause I talk a lot, and Isay is special because she is small and cries a lot, and you’re special ‘cause you work a lot, and Tatay is special cause he eats a lot.


Nanay: (Chuckle) Yes.


Eloi: We are all special, ‘cause God made us this way.


Nanay: Yes, ALL of us are special.

Rub-A-Dub-Dub

We noticed that Isabella has been more "aware" since the seizure and since she was put on meds. It seems that the only skill she lost (which can happen because of the seizure) is the most recent one she learned, that is sitting with some support from an adult. It looks like her body is still somewhat physically weak from all that brain-wave-bouncing. I believe that there is no reason for her to not regain that skill. It will just take a little bit of time.



A new thing she recently learned is that when she is sleepy she can make herself not fall asleep. While most babies will cry and be fussy-grumpy, my Isabella would smile... even laugh.


Watch this short clip of me rubbing her head to help her fall asleep.

Seizure Shenanigans

It was a regular Sunday morning at the Catiis residence. The sun was out and the breeze is nice and cool. Everyone was abuzz and busy with their daily morning routine and ready to begin with what started off as a relatively relaxed day, until…


8am Isabella coughed and had a little throw up and then she had this frozen expression on her face right after. Her limbs were limp and not moving. It was a seizure… wait it out, wait it out…


I then put her on her side so she does not choke on her saliva or possible succeeding throw up. Her body did some minor jerking movements. She is still seizing, wait it out, wait it out…

Then her eyes rolled up and her body stiffened. Still seizing wait it out, wait it out…


I held her and kept talking to her until the seizure episode, well, ceases. My son was asking me to make him breakfast, but I cannot. He noticed the look on his sister’s face. I had to tell him and explain to him that his sister is having a seizure. I asked him to wait it out with me and then I can make him breakfast.


Minutes passed (that seemed like hours) but she was still seizing. I believe she will stop for a couple of seconds and another one comes along, looking different than the last one. I called my husband (who left for the store right before everything happened) and instructed him to come home right at that instant. My son was already starving… but I cannot leave Isabella. I told my son to hold his sister’s hand and that we pray together to make the seizures stop. We prayed and prayed over and over… she would not stop seizing. My son even said, “Nanay, I don’t think the prayers are working.” I told him it will. They’d better work.


Eventually my husband got home and I instructed him to take care of Eloi before attending to Isabella. When he became available, he took her from me and that’s when I started breaking down. He kept talking to her and was even playing with her, probably thinking that he can distract her from having the seizure, but they just kept on coming.


I told him that now would be a good time to call 911 and so we did. While I was on the line with the 911 dispatcher, she started shaking… the seizures became grand mal and she started turning purple. That’s when I lost it. I prayed loudly and started giving her away to God. I was telling Isabella that if God is calling her to come home, she has a permission to go with him. I assured her she can go and that we will all be ok.


The paramedics arrived, checked her vitals, then took her to the nearest hospital. My husband rode with them.


I was not in the best state to drive. Good thing that the neighbors were on their way to church and saw the ambulance. They drove me to the hospital. I called friends to let them know what was going on and everyone stepped in and took in roles on who will take care of our son and the house and other things.


When I got to the hospital she was still seizing. The doctors and nurses had a lot of questions. We had to let them in on her litany of complications. We had to explain her syndrome. They do not know her here. We told them that she was under the care of many doctors at Stanford, and then they started making phone calls.

They gave her 2 separate doses of Ativan but she was still seizing. She had on oxygen and almost needed to be intubated, but she, like the trooper that she is, somehow managed to breathe not needing the support of a machine. The attending doctor got a hold of our neurologist and was instructed to administer Diastat. After the longest 2.5 hours of our life, the seizures stopped.


The Stanford team was on their way to pick her up. I went home to pack a hospital bag and made arrangements for work the next day and for Eloi’s school the next day. I wanted to give him a sense of normalcy amidst all the chaos. I didn’t want him to learn about “worry”… no, not yet.


The Stanford team picked her up and ambulanced her all the way to Palo Alto. We were instructed to follow and reassured us that she is in good hands and that we do not need to hurry. My husband and I drove ourselves up there, stopping at a Mcdonald’s to get a quick breakfast and to recollect ourselves.


On the drive up to Palo Alto, I would burst into tears… this time Edgar joined me.

When we got to the hospital, we found her in the Pediatric Intensive Care Unit. A Neurologist was already there at bedside attending to her. Isabella was knocked out and she had a fever. We spoke to the doctors recollecting the events of that morning. We were trying to figure out what might have caused the seizure. We told them that the day before she was baptized and we had a party and she skipped her afternoon naps because she was interested with everyone. She didn’t get to really fall asleep until 11 that night, and from 1 am onwards would cry and fuss every hour. The seizure trigger could have been that she was over stimulated from the day before, tired from the day before or she could have caught something and have an infection that may have caused the seizure, hence the fever, or she could just have a fever right after the seizure and the trigger is random. A blood and urine culture was ordered to check for infections.

The result of the culture is negative.

So what triggered the seizures? We had to ask the neuro doctors, and their response was that since seizures is a likelihood with Isabella’s syndrome, they think that it was just the time for her to have the seizures.

Will this be a regular part of our lives? 911 calls, her losing oxygen, ambulances, and emergency rooms? Not necessarily.She will be getting medication to control the seizures and she will be starting with a low dose of 0.5mls, and if she has a seizure for more than 10 minutes we can give her Diastat.

Will the medications control her staring seizures and the occasional brief blinking-jerking thing she does? No, it will not control the baseline, and reminded up that these small seizures were benign.


We left the hospital the following day, just in time for our renal appointment for that day at the clinic across the street from the hospital.


We went home with the good news that meds controlled her seizures within 24 hours and that her renal function is close to normal.


We pray that there will be no more seizure shenanigans.

The 11th Month

Current Stats:

Isabella measures 24 inches long and currently weighs 12 lbs. Our little guppy is getting closer and closer to becoming a beluga. Using the typical growth chart, Isabella is creating her own curve at the bottom. But when her weight and height were plotted on the WHS growth chart, she shows as an average WHS baby.

Feeding

The GI doctor and nutritionist still have concerns regarding her weight. They instructed us to gradually up her nightly pump feedings from 35ml to 40ml per hour. Throw ups are not completely resolved but less in frequency and volume. She throws up about 30 mls of feeds 1-2 times a day. Since she appears to be volume sensitive, we were also instructed to change her daily calorie intake per feed from 24kcals to 28kcals.

Seizure Scare

We had the biggest scare of our life 2 days before her 11 month bday. She had her first big seizure. It all started with a staring/freezing episode then a small throw up, then staring and some jerking and some eye rolling. We know she has seizures but since the neurologist said that they were benign we were just waiting-out the seizure episode. However, 30 minutes later of staying frozen with occasional jerking and her eyes just rolled up and being unresponsive, we figured it was time to contact 911. While waiting for the paramedics to arrive she started to do tonic clonic movements and some stiffness. She also turned purple. She was rushed to the emergency room, intubated, and was given three separate doses of Ativan, but it was not enough to make the seizures stop. Eventually they got a hold of our neuro and the ER team was instructed to administer Diastat. Then eventually the seizing stopped. Her whole seizure episode lasted for about an hour and a half. The Stanford team came to get her and she stayed in the PICU overnight. She spiked a fever after the seizure and doctors needed to know if the reason she had a seizure was because she has an infection which also explains the fever. So they ordered a blood and urine culture but the results were a negative. So doctors said the fever was caused by the seizure and the cause of the seizure is unknown. Neurologist thinks it was just the time for her to have the epileptic seizures that is not uncommon to children with WHS. The doctor ordered daily doses of Keppra to control seizures. We have yet to see how the medicine affects her, but so far, Isabella seems to be back to her usual self, except that she seems to have lost the muscle strength to sit with minimal support from an adult. What is really important for us right now is to have the meds control the seizures and for us to NOT have to go through this big seizure scare ever again.

Renal Report

Kidney doctor says that her kidneys are close to normal functioning. We can try to give her a little bit of food rich in potassium such as potatoes, squash, avocados, and bananas. Another VCUG to check if her kidney reflux has resolved itself will be done around May of next year. Creatinine and potassium levels in her blood read normal.

Skills Update

She can turn with ease from side to side when put on her back and can bring herself to roll easily from her back to her belly. She can push her arms to hold her chest and head up when on her belly. She likes being on her belly and will happily kick her legs which causes her to pivot on the mat/blanket. We think she sometimes does it on purpose so her body will turn to face where she wants to go, such as to face wherever we are sitting in the room so she can see us. She also pulls her knees in so her butt is sticking up. Could it be that she is at the throes of discovering crawling?

She puts her thumb in her mouth and would lick the back of her hand or the inside of her arm to soothe herself. Every now and then she would scratch her head to make herself fall asleep.

She likes to be where everyone is. She cries when she is left in the crib and she cannot see anyone. She smiles when we put her on the highchair and we roll her to the dining room so she can be there while we eat. Every now and then she will make a sound as if she is calling us to look at her and when we do she flashes us a big gummy smile. She still makes “Mmmmm” sounds, but occasionally we hear her do an “Aaahhh”.


She cries to be held and when we hold her she does not want to be held facing mommy’s body. She would keep moving and turning because she prefers to face front so she can see everything and everyone. She enjoys watching people. She still does not play with toys nor does she show interest in them just yet…. She is more interested in people.


We’ve seen her do a social giggle, but she still does not do it consistently. Social smiling is definitely established.

Upcoming Medical Stuff

Isabella is scheduled to have her cleft palate repair surgery next week. The CFteam seem very confident about doing the procedure to our daughter, who they claim to be their youngest patient yet. They are saying that since Isabella only has a cleft on the soft palate, it should be easy for them to get in there and close it and that the whole procedure would only take about 2 hours and she would only need an overnight stay at the hospital.

 

To read about Isabella's previous developmental update, click here