Friday, May 27, 2011

Got her Tracheostomy






Leah got back from the OR where they did her tracheostomy. While they were in there they also did a Superglottoplasty for her laryngomalacia. So, that should help reduce the sound she makes and make her airway wider until she develops it better.
She is looking really good, they have her on some morphine for pain medication but she will only be getting that for about 24 hours every once in a while. So, it's not like she's sedated around the clock. They are also giving her some Precedex which is a sedative for when she gets too agitated and to help her sleep if she can't on her own.
She seems much better now too, now that the tube doesn't seem to be bothering her as much. The doctors also want to start weaning her off the ventilator in a couple of days so that she can just breath through her trach. It will take about a week for her to get weaned off of all the medications they have her on, before they can start on the medication (Carbidopa/Leva-Dopa) they are planning on giving her for the movement disorder. The is
sue right now is also getting
the right dosage and preparing the medicine appropriately for an infant to take.
For now we are happy that she seems more comfortable and that she at least looks happier
Thanks to everyone who is supporting us with their thoughts and prayers
Here are some pictures of the princess today after her surgery, enjoy...
Frani

This is right before she was reintubated, from last Thursday:
Here we are after the naming ceremony on Monday:
This is from today after she got her trach:



Wednesday, May 25, 2011

New findings

Sorry I haven't posted in a while, things have gotten crazy.
Since the last time I posted lots of things have happened.
Last Thursday she had her muscle biopsy. The operation went well, but it seems that they sedated her a bit too much because she remained extremely lethargic for the rest of the day. The nurse bathed her that night and I was able to hold her for the first time in days while she changed the linens.
While I was holding her, we all noticed that her oxygen levels would go from 100 to 75 and then back up to 95 then down again to 80, etc. So we thought that was weird, and it looked like she was taking very shallow breaths, so that was why her oxygen was dropping. A few minutes later, the nurse took her from my arms because her oxygen dropped all the way to 35, and had stopped breathing and she had to start bagging her to give her more oxygen. The respiratory therapist came in and started giving her more oxygen, and the fellow doctor had just happened to be coming in to check on her when all this happened so she was able to act quickly. Another doctor from her team, one of the pulmonologists was also stopping by to see her and was able to assist. Leah was not having any chest rise, so she was not making any effort to breathe. Earlier they had given her some Rubinol to dry up her secretions so they think that that combined with too much sedation at the biopsy was what caused her to stop breathing. They had to intubate her once again, which we were fine with since that was going to help her breathe. Everything happened very quickly and efficiently but it was another scare that came out of nowhere. Over the weekend not much happened, since we were just letting her rest and waiting for the biopsy results. Zev's mom had come in to town Wednesday night so she was here with us until Tuesday morning and was a great support.
On Monday we held a jewish naming ceremony for her, since we had not had time to have one since she was born. There is also a Jewish tradition called "meshanneh shem" where a person who was dangerously sick would change his name in the hope that the Angel of Death, who summons persons by name, would be baffled thereby. The Rabbi suggested it and we really didn't think it would hurt, so we added a name, so that she is now officially: "Leah Raquel Lucia Esquenazi". I chose Lucia because it means "light", which is very appropriate for her now in these dark times; we want her to be filled with light and healing. Lucy was also my maternal grandmother's name, so it also has a deeper meaning, and we chose Lucia, because it was also Italian which is part of my heritage as well. It was a beautiful ceremony, with both of our moms there as well as our "adoptive" parents, Roy and Dolores Starke. One of Leah's godfathers, our dear friend Ben Cohen was there as well. It was great to have them all there supporting us and being a part of her naming ceremony.

Now for some "good" news....

I wrote good in quotations because it's not for sure yet, so we don't want to get our hopes up.
Yesterday the genetics team let us know that they found an anomaly in her genetic code. It is a lower level of a certain muscular neurotransmitter. Normal range is 480-1000 and hers is 250, so Genetics along with Neurology seem to think it could be what is causing all this. This is really good news because if that's what it is, then it is treatable and she can recover. However, they want to make sure they get all the information back from tests like the muscle biopsy and some other blood tests just so that they don't miss anything.
Now one of the issues for treatment is that the smallest dosage of the medication they could potentially give her is still too much for her since she is so little. So that is something they are working on figuring out as well

One of my co-workers, Alex Lehmann, came by the hospital tonight to visit and prayed for her and kept us company for a while. It was great to be able to talk with him about how we're feeling and share experiences. We feel very lucky to have such caring friends and even people who don't know us personally, praying for us and the baby and hoping she comes through all this soon.

We appreciate all your prayers and support.
I will update again soon

frani

Tuesday, May 17, 2011

Mitochondrial Myopathy

So the neuro-muscular doctors came by yesterday and did a muscular test on Leah. Some of the results came back as abnormal, so they are going to do a muscle biopsy to see if it could be a mitochondrial myopathy. At this point, they are not sure, but it's a suspicion that they want to test for. The group of diseases is very broad, so until the biopsy is done, they can't say if it could be a more or less severe case of mitochondrial myopathy. They are doing the other GI study today and we'll hopefully get some results from that in the afternoon.
At this point, we're so on edge from all the different things they keep telling us that it is getting pretty nerve wracking. But we have each other and even though we cry and get upset, we know we have to stay strong for Leah no matter what they find.
Hopefully they are getting closer to finding it out
thanks
Frani

Monday, May 16, 2011

5/16 Update


Sorry we were MIA over the weekend, we were getting a lot of rest, so sort of forgot about posting. But I know lots of people want to know the latest, so I figured I'd post something before I get all the phone calls.
She was successfully extubated on Friday afternoon. They took a look at her airway an
d discovered that she does not have Tracheomalacia, but she has "Laryngomalacia", which looks like this (the one on the right):
It sort of explains what happened the night that she was having issues breathing. They think that because of her vomiting, it inflamed her epiglottis, which was already floppy, and that collapsed her airway even more, which made her work very hard to breathe.
They kept some oxygen with high flow going thru her nose, but she is breathing (still very noisily) fine.
Not too much happened over the weekend, except that she had a very hard time going to sleep. The first night after extubating her, they did not want to give her any sedatives for fear of her not being able to breathe on her own. She had a lot of shakes, sweats and basically did not sleep at all the first night. They finally gave her some Adavan and Benadryl to help calm her down the following morning, which helped temporarily but she continued the be agitated for most of the day. They did the same the following night, but had the same issues, Zev and I took turn trying to get her to sleep, so we were awake most of the night as well. I believe it was Saturday night that they started giving her some Methadone.
Part of the reason why she was having the tremors and sweats more than usual was because she was on some pretty heavy narcotics for the week that she was intubated (Fentanyl, Precedex, and Versed with occasional doses of Propofal and Morphine). So they sort of made her a little mini addict, and then took it away once she was extubated, so she was dealing with withdrawal symptoms. The Methadone helps them wean her off of the drugs a little easier than just stopping cold turkey.
Today she went down to Radiology for a gastric emptying study, tomorrow she'll get an Upper GI study, and once she is feeding again they'll do the PH probe. For now she has not had any food in her stomach (not even with the feeding tube) since they extubated her on Friday. So, she is getting TPN plus fats in her central line IV (going into her jugular vein) which is giving her all the nutrients she needs and calories, but I don't think it really takes away the feeling of having a full tummy, so I'm sure part of her frustration is also feeling hunger. They are trying to do all the GI studies to see how bad her acid reflux is, so they can figure out when to start feeding her again. She also has sort of forgotten how to suck and swallow because she was intubated for a week, so they had an occupational therapist coming to see her to help her get more coordinated.
According to the OT, she is still coordinated but her gag reflex is not really there, so they don't want to feed her and then have her aspirate anything into her lungs. I'm hoping that after her upper GI study tomorrow they can start feeds again. For that study they have her swallow a dye, and then take X Rays to see where stuff goes, and if it refluxes a lot and how long it takes to go through her system.
Neurology is having a Neruo-Muscular Specialist come in today to asses her, since they want to see if there is any way it could be a neuro-muscular problem. They want to make sure he gets any additional tests he thinks she may need before starting the IVIG treatment, which they still think is a good thing to do. They also did a test to see if there is any muscular degeneration and it came back negative, which is good news. So far, everything had been negative from all the tests they've done but they are also still waiting for a lot of tests to come back, especially the Genetics tests.
So again it's back to waiting for all this stuff to happen. We'll update as we get more info.
Thanks
Frani

Thursday, May 12, 2011

Got some good news this morning

So, I sat in on the doctors morning rounds and all the scans from yesterday came back negative for a tumor! They are going to see if Neurology wants to do another MRI, but so far, all the labs and scans have been negative or pending. Which means it eliminates a lot of bad things, like Neuroblastoma. Now, they did tell us that sometimes infants with Neuroblastoma have it and then it regresses before they can find it. So, there might be a chance that that is what happened, and she's dealing with the after effects of it; but at least now there is no tumor, so there won't be a need for chemotherapy.
They are planning on doing a gastric emptying and upper GI series in the next couple of days since we still haven't been able to find out if acid reflux is a part of this whole thing. They have to wait until Monday to do the PH probe, since she is on Protonix and she needs to be off that medication for 3 days prior to the probe.
They are trying to do as many procedures as they can while she's intubated, and they still want ENT (ear, nose throat) to scope her and look at her airways before they extubate her to make sure that she will be ok to breathe on her own. They mentioned planning on extubating her next Monday.
We still have to hear from Neurology what they want to do for treatment now that the scans have been completed and everything was negative. They will probably start the IVIG soon, but they still have to round and come talk to us about when they will do it.
So far, I think it's all good news and we're starting to see a light at the end of the tunnel, so keep praying and hoping and I'll update as soon as I get some more answers.
Love you all!
Frani

Tuesday, May 10, 2011

New updates

Leah went for her CT scan yesterday afternoon and they ended up doing only a scan of her chest and upper abdomen, since the attending radiologist said doing a full body scan was too much radiation and she is too small. From that scan they did not see any sign of a tumor, but again, it was only a portion of her body. So tomorrow she will go in to Nuclear Medicine for her MIBG scan. They gave her a couple drops of the radioactive tracer that will light up any tissue that is neruoblastoma earlier today. They also gave her some iodine so that her thyroid will be protected from the radiation.

She also got another spinal tap today, since they have to do more tests, but luckily we all remake spinal fluid within a few days. They actually froze a whole vile of it in case they needed more later, that way they don't have to poke her again. It was funny because the nurse told us that while she was sedated for the the spinal tap, they had to take off her diaper and clean the area of her back, but that she started pooing and they had to wait for her to finish. The funny part was every time they thought she was done, more kept coming out! :D Apparently the doctor kept telling our nurse: "ew! Elena, clean it!!" haha, she was teasing him about having to get used to it if he was ever going to have kids.

The ophthalmologist came by today to let us know that he showed some videos of Leah's eye movements to his colleague who is a pediatric neuro-ophthalmologist, and he said that it looked like opsoclonus-myoclonus to him and he wanted to come over to see her in person. I think they said they were coming tomorrow. He also mentioned that many people who have opsoclonus don't always develop the tumor and what she is going through may be an autoimmune response of her body that could have been because of her vaccines or sometimes a cold can do this as well. We mentioned that this all started after her vaccines and that when they did cultures from her nose, they found the common cold virus, and the coxackie virus. So, he thinks that it might be able to be treated with IVIG. Again, that's an assumption and we'll have to make sure they rule out the big bad things like cancer, before they can say exactly what it could be.

She continues to respond to our voices immensely to the point that even heavily sedated she manages to open her eyes and melt us. Her breathing issues may NOT be attributed to anything connected to what she's had, other than possibly major acid reflux corrosion that irritated her airway causing her to choke.

The neurology team also came by today to see her, and they don't really have any news, but they did tell us that they are going to reach out to Dr. Pranzatelli, who is supposed to be the best doctor in the world in the specialty of opsoclonus-myoclonus (http://www.omsusa.org/ is his website). So, they are talking to him about Leah to see what treatment options are available for her. Which is really great, since we had done some research and had seen his name pop up and were going to tell the doctors about him, but they are already on it.

We also got a visit from a representative from Chai Life Line (http://www.chailifeline.org/), which is a jewish organization which helps families that have children in the hospital. They were really great and are bringing us lunch and dinner everyday, which is such a relief since buying food at the hospital cafeteria gets expensive and sort of boring (as far as the food is concerned).
They are also working with the social workers here at UCLA to write letters to my work and Zev's work excusing us because of Leah's condition. Not that they wouldn't excuse us, but it's more of a proof thing to have.

We also got a visit from our best friend's parents, Dolores and Ray Starke today who have been incredibly supportive and they took us out to lunch while Leah got her lumbar puncture. It was nice to have them here and be able to talk to them, they are sort of like our Mormon parents, lol. :)

My mom also came by for a little bit, with a mask on, since she is taking care of a little girl who is sick, so she didn't want to pass it on. She was able to look at Leah through the glass door at least.
I don't think I have anymore updates for now, we are just waiting for tomorrow, and taking one day at a time.
Keep Leah in your prayers.

Monday, May 9, 2011

Blood transfusion

Leah received a blood transfusion last night (about 50 ml.) and is doing much better. She had been looking very pale, and her levels were really low, which is why they decided to do the transfusion sooner rather than later.
I went this morning to the UCLA blood center and donated for her just in case she needs more as well.

They are in the process of scheduling the CT scan for later today. They will have to take her off the feeding tube an hour before they do the scan since she will be completely sedated.

The ophthalmologist just came by to check her eyes, he is taking some videos of the eye movements and will be showing them to his colleagues across the street who specialize in pediatric neuro-ophthalmology. He said he would come back later to get some images of her retina as well. His initial reaction is good, since when he shone the light on her eye, she squinted and reacted, even though she is sedated.

More info to come...
thanks
frani

Sunday, May 8, 2011

Taking videos down

We decided that we will have the videos for the doctors on a separate pages, since they can be a bit disturbing for people besides us to watch
We will continue to give updates thru this site though
Thanks
Frani

Leah's health update

So, we have started this blog to let our friends and family know the latest updates on her health issues. We have also created this page to post videos for Leah's doctors to watch and analyze.
We have a lot of them so I will post them in chronhological order, from the most recent to older videos. I'll make sure to add any notes and the dates and times.

Here is a summary of what has been going on:

Leah was admitted to UCLA medical center for abnormal eye and body movements that started about 2 weeks ago, after her first round of vaccines which caused her to have a fever. After that fever she was having a lot of jerking movements and eye movements that they now are calling opsoclonus myoclonus ataxia syndrome, also with pretty extreme back arching.
The first visit the the ER was because of these movements and they did a MRI and a 24 hr EEG which both came back normal. It didn't look like she had any seizures or any obvious neurological issue. They assumed it was bad reflux, possibly something called Sandifer's syndrome, which only affects 1% of babies. They started her on acid reflux medication but it didn't seem to help.
We had to take her to the ER last week because she had a high fever or 102.8, and she was admitted again, this time at UCLA Santa Monica. The fever went down, but the movements were constant, so much so that sometimes she couldn't sleep. Or when she did it was because she was so exhausted from it. They attempted to do a PH probe, a tube that goes down her nose to her stomach to measure the acidity and see if it was indeed acid reflux. She was born with Tracheomalacia, which means her trachea is not rigid, but floppy, so when she breathes it makes a squeaky noise. Because of this, when they did the PH probe, she started to choke and couldn't breathe and started to actually turn blue on us. They pulled it out and she was fine. They tried one more time, but the same thing happened, so they decided not to do it at all. They sent us home on one more acid reflux medication called Reglan, which would help her stomach contents leave her body faster to reduce reflux. They also told us to add rice cereal to her milk to make the liquid thicker and perhaps help it reflux less as well.
The next day we took her to the ER again, because she was vomiting everything she ate, and our doctor did not want her to get dehydrated. She was seen by the same group of neurologists who thought that her symptoms had worsened and changed since the last time they saw her, so the put her on another EEG.
The gastroenterologists came to see her and asked us to take her off the reflux medication because they wanted to try the PH probe again, this time with someone who knows ow to insert them in special cases like hers. They didn't think it was just reflux either, when they saw her, because of the eye movements she had.
So Tuesday they took a lot of blood and urine samples to test for genetic, neurological and infectious diseases. The poor thing looked like a pin chushion, they poked her in about every vein they could use.
The video EEG came back normal again which told us that her movements were not seizures and it eliminated a lot of neurological things it could be, but not everything.
We also got a consult from the Hematology/Oncology department because they think it could be a type of cancer called Neuroblastoma, which is a tumor that could cause developmental delays. Although because she is so young it could be stage 4 neuroblastoma which apparently is treatable and not as scary as when children are older.
On Friday 5/7 she was put under general anesthesia so they could do another MRI, this time with contrast to try and find any genetic or neurological issue. She is also getting a MIBG body scan to see if they can find a tumor, which will be on Tuesday most likely. While she is under they also did another lumbar puncture and get some of her spinal fluid to test for some other diseases as well as for the neuroblastoma.
The results from that MRI came back normal, so her brain looks structurally normal. No lesions, or strokes on any thing that could give an idea as to why this is happening.
They decided to do a CT scan on Monday and the MIBG scan on Tuesday, possibly.

On Saturday 5/8, we think she overfed, which caused her to vomit most of what she had eaten. A few hours later, at around 7 pm, we noticed that she was crying and just being very fussy after I bottle fed her some expressed breast milk. We both tried calming her down, and rocking her because we thought maybe she was just tired. But her breathing got very loud and it started to sounds like she was gasping for air. We called the nurses in and they noticed that she was sweating alot and having issues breathing, so they called the on-call doctor and she came in within a few minutes. He took her shirt off and immediately noticed that she was working way to hard to breathe, since every time she would inhale, her ribs would stick out and she was having a lot of retractions.
They called rapid response and a respiratory therapist came in and tried to help her clear her airway and got an oxygen mask over her. After that things happened very fast, they had close to 20 people in the room, doing what seemed like 100 things at one, trying to help her. They got a PICU doctor to come up and she took control and started getting her on different medications to calm her down and they decided to intubate her so that they could help her breath. We were lucky that the doctor who had come in, knew her very well and was able to let everyone else know what was normal for her and what wasn't. As well as keep us informed of everything that was going on, which was a big relief because we were thinking that we might lose her and he made sure to give us peace of mind by giving us a play by play of all the procedures that were happening. It was all very fast, they had to wait for the ENT to come in because of her tracheomalacia. As soon as he got there he was able to look through a scope as he put her breathing tube in and once it was in, they were keeping her breathing stable with a hand pump, and eventually moved her to the PICU (Pediatric Intensive Care Unit).
As of right now, Leah is still in the PICU and sedated. She is stable now and they have some machines helping her breathe and also have a feeding tube which is helping her get the nutrition she needs.
Because of all the blood they have been taking out, she is now very anemic, and they will most likely have to do a small blood transfusion, so because I am O- blood (universal donor) I will be going first thing in the morning to donate my blood for her.
For now, they don't know if this is related to her initial issue or if it's an isolated event. They are still waiting for dozens of genetic and neurological tests to come back, just to try and find the cause by process of elimination.
I will hopefully have more answers tomorrow after the CT scan
Keep praying for her..
Good night,
Zev and Frani