Moving Along and Rolling Over!!

We are moving along and getting some dates set. Jack will have his surgery for bilateral cochlear implants on 11/20, pending insurance approval. They are changing a lot of the protocols and they said he might not even have to stay overnight. He is likely to be the first case since they go in age order with the youngest being first. They do this because of the fact that they can't eat prior to the anesthesia and the babies have the hardest time with that. I am happy we are moving along with it. If all goes well he should be hearing before Christmas.

Jack will get his helmet on Wednesday the 23rd. We went for the fitting last Friday. They put a socklike material shaped like a hat on his head. That material had sensors all over it. Then they used a type of gun that read all the sensors. All that data went to a computer and it made a 3-D model of his head. They will use that to make his helmet. I got to pick from a bunch of different patterns but I decided on basic blue.

Jack rolled over!! He finally did it! He went from front to back in therapy but without being touched. He has since done it a few times at home. I am very proud of him and relieved. He is seeing a Developmental Neurologist on Wednesday so I can get a better idea where he stands with everything, but I am thrilled that he finally rolled over.

Jack's hearing aids are not doing anything for him. I have not been putting them on him much because I am concerned that he will choke on them or swallow the battery. Button batteries are a huge big deal if a baby swallows them. If that should happen they usually require surgery to get them out and and caustic if they are left in. If he is not getting any benefit from the hearing aids I prefer not to chance any issues. I do sometimes put them in when he will be right in my field of vision, but never in the car and I am leery about it for daycare.

Jack is a very verbal baby. People always ask me why he coos and yells so much if he is deaf. I am told this will stop if he is not implanted. That is one of the reasons why it is important to get the cochlear implants so early. All the cooing and repetitive noises babies make are the building blocks to language. He needs to do this in order to learn to speak later. If I sit him on my lap and make certain noises he is able to copy them. When this first started happening I was sure they had made a mistake and he was not actually deaf. The teachers from the deaf school and the evaluators all told me that deaf babies do this. He sees how I am moving my mouth and copies that. They said that is one of the reasons they do universal newborn hearing screens now, because deaf babies fool us. They said that, before the screening, they would not know the babies were deaf until they failed to develop language at 3 or 4 years old. That is really late to find out that a baby is deaf. If you want the child to speak you need to find out early and start therapy right away.

Jack has physical therapy twice a week, speech therapy once a week and cranio-sacral therapy once a week. NYU told me they want him to have auditory therapy four times a week and the deaf infant program wants him to attend twice a week for 2 1/2 hours each time. I am having difficulty getting all these things done, getting to all the doctors appointments, getting to work and doing everything for Julianna. Our house is still under construction post hurricane Sandy. It is getting closer to completion. I am hoping it is finished soon. When it is I would like to hire a full time, driving nanny to take Jack to a lot of his appointments as well as get Julianna on and off the kindergarten bus. Right now I am getting by with luck, prayers and the help of great friends.

I went to get my hernia checked again and it is looking like I will need the surgery very soon. I am hoping I can coordinate it with Jack's surgery and we can recuperate at the same time. I hope it works!

We are busy but we are enjoying fall. We have gone to the pumpkin farm and apple picking. I took Julianna to the Halloween events at Sesame Place and she had a ball. She is still in ice skating, dancing, violin and arts and crafts.

I just passed the 4 year anniversary since my cancer surgery. I have only one more year that is considered pretty risky. We are super happy and life is good!!

Jack Needs A Helmet

I took Jack to the neurosurgeon because his physical therapist said she is concerned that his torticollis is not responding to therapy as much as she had hoped. Jack's torticollis was so bad at first and it got much better right away with therapy. It wasn't completely better and he seems to continue to want to hold his head to his right side. Since he does this his head is developing an odd shape.

The neurosurgeon examined Jack. He said he is generally against helmets. He feels that a baby's head shape will get better by itself as the brain grows and they start spending more time on their stomach.
He also said that he felt Jack was an exception to this. He said Jack had as bad a case of torticollis and plagiocephaly (misshaped head) as he has seen. He said that since Jack is not yet rolling over and we don't know when he will, that it might not self correct. There is a small window of time where the skull is soft enough to correct the odd shape and we may miss it if we don't act soon.

He gave me a prescription for the helmet. I need to take him to an orthotist where they will make it for him. I am hoping they will figure out a way that it can work with the hearing aids and then the implants.

I feel so bad that Jack has to endure all these treatments. He is a happy guy and doesn't seem to complain much but it seems so unfair. I am happy that he is getting it all now and I remain hopeful that he will progress to the point that he doesn't need more than the cochlear implants by the time he goes to school.

Jack is ten months old now. He is still not able to roll over. I keep being assured by all the therapists that he does not have cerebral palsy. I remain unconvinced. I don't get any answers when I ask why he is not progressing. He has therapy four times a week and I do the exercises with him as much as possible yet he does not seem to be getting much better. I am looking for different specialists to take him to to see if I can get any new ideas.

Hearing Aids, NYU, Dr. Citron

We finally got the hearing aids. We got them on Wednesday the 11th. He didn't seem to have any notable response to anything with them in. There is a lot to the care and testing of the hearing aids. I had a lesson on everything when we picked them up.

On Thursday and Friday last week we went to NYU. We had three appointments. Two of them were to test Jack's hearing in the booth. They put us in a sound proof booth and put a small ear phone like device in Jack's ears. Then they send sound through them to see if he responds. If they think he does they make a stuffed monkey light up and play the cymbols.

They felt he responded to certain sounds in his left ear at about 75 dB. He only responded at certain frequencies. Personally, I really didn't note him to be responding all that much.If he moved his head or turned at all they were saying he was responding. He is a pretty antsy guy so it is really hard to tell why he is moving. Hearing at 75 dB is still a pretty profound hearing loss and he still qualifies for cochlear implants, but it is a lot different than the results of the auditory brainstem response (ABR) tests that he had at LIJ.

The audiologist told me that his hearing may be a bit better than it was when he was previously tested. If that is the case, his hearing aids are programmed wrong. She said that they could be hurting his ears and they may need to be reprogrammed. So I took them off him. She asked me if I wanted Jack to have another ABR at NYU. I told her that I do. The ABR is objective and the behavioral booth is very subjective. I don't want to damage any residual hearing he may have by using hearing aids that are programmed wrong.

The next day he had the same test for his right ear. He was a little irritable and the test was coming out all over the place. His right ear has auditory neuropathy and that means he doen't process sound even if he hears it. He did not test well with that ear.

Jack was also evaluated by a speech therapist. She was listening to his babbling and counting the different types of sounds he makes. She told me his cry sounds immature and she thinks he may have some vocal cord damage. He will need to be evaluated for that. It seems like we hear bad news at all of these appointments and I find it pretty frustrating.

We go back to NYU this Thursday for two more appointments. I am going to try to take the train this time. It was pretty tough driving in there and I was forced to park in a garage costing $37. I think it might be cheaper and easier to take the train even though it will be rush hour. We will see.

On Sunday we had my Dad so Betty could go to a baby shower. He is getting pretty bad and it was hard dealing with him. Julianna was very helpful and she is pretty understanding about Pop-Pop's brain disease. It was nice to be able to give Betty a bit of a break.

I had an appointment with Dr. Marc Citron my medical oncologist. He took blood and sent me for a chest x-ray. He did an exam and said he thinks everything looks normal. He also told me that I am now in the time where I have the greatest risk of recurrence. Once this year passes my chance of recurrence will start dropping dramatically. It can be pretty stressful to think about so I try not to.

Julianna is enjoying school and her activities. She has violin, ice skating, dance and arts and crafts. I am trying to find a place for swimming lessons that is a little closer to home than our last place. Jack i s happy and is proud of his two new teeth. We are all enjoying fall and having a good time!

Kindergarten, No Hearing Aids Yet, Pictures

Julianna started kindergarten on Tuesday!! It was a big day for both of us. She was excited and I was nervous and sad that she is growing up so quickly. My little 3 pound baby is with all the big kids now. She did say some of the kids said she looked like she was three and that made her sad. I told her to please remember that Uncle Rich was made fun of for being small and now he is the tallest person she knows. Kids can be mean. I hope she learns to let it all roll off.

Jack was supposed to get his hearing aids yesterday. I took the day off for the appointment. About two hours before the appointment they called me to say the hearing aids weren't in and I needed to reschedule the appointment. The lady there (LIJ) was pretty nasty as usual. After speaking to the supervisor I was able to get an evening appointment for next Wednesday. I hope it works out this time.

Here are some pictures:

Julianna ready for the first day of kindergarten.

Julianna getting on the bus. I followed in the car.

Jack at 9 months old. Super happy.

                                    View from our dock in PA. We are sorry to see summer end.

Genetic Testing Results

The results for Jack's genetic testing came back and everything was negative. He did not have any of the connexin mutations. There is also a mitochondrial mutation that can make people more susceptable to gentamicin induced hearing loss. Jack was negative for this as well.

Everyone was pretty sure the drug interaction between the gentamicin and lasix caused Jack's deafness. However, they did say there was the slightest chance he could have had one of these genetic issues. I am glad he does not have any of these genetic issues, but it makes me pretty mad that they did this to him.Anyone who googles these two drugs can see that they should never be given together. I would also understand if there was no other option but to give him these drugs to keep hime alive, but that was not the case. He got the gentamicin (an aminoglycoside antibiotic) for a "rule out". That means he didn't have an infection, but they gave it to him just in case it came back that he did. He did not, and he never did.

A lot of hospitals are moving away from using gentamicin, especially in the NICU population. Here is an interesting article about that subject.  http://exutero2.wordpress.com/2010/12/16/why-we-use-zosyn-instead-of-ampicillin-and-gentimicin-at-tulane-lakeside/
A lot of places say they are still using it. They say it is a good drug because it is cheap and it is broad spectrum. In Jack's case it is certainly not cheap. The cochlear implants and associated therapy for his lifetime will certainly be in the millions. The implants alone will be $250,000. That is just one surgery.

I am currently trying to work out the financial part of his implant surgery. They are saying the insurance will cover only 80% of it because they are out of network. There is not anyone in network who does this surgery so I am hoping they will pick up more of it since 20% of it will be over $50,000. The NICU had also applied him for medicaid. He was eligible for despite my income because he was under 1200 grams. I am now trying to find out if that is still in effect and if not if there is any way I can get him on it. I am hearing conflicting information, but there are certain disabilities where Medicaid/SSI eligibility is automatic (such as being born at under 1200 grams) and some people are telling me deafness is one of those and some say it is not. I am waiting to find out officially.

Jack had a CT scan today to check the anatomy of his inner ear and to check the thickness of his temporal bone. He is having all types of testing done to make sure he will be a candidate for the cochlear implant. He was a good sport for the test despite the fact that he couldn't have a bottle after midnight last night. We were at the hospital a bit after 6 and he had the test about 7:30. They gave him a short acting sedative so he was awake and eating shortly after.

Hearing Aid Molds

Jack had an appointment yesterday for his hearing aid molds. It was at the LIJ Hearing and Speech Center. He was very cooperative. They put a silly putty like substance in his ear and then wait about 5 minutes for it to harden. They did this for each ear.

We also had to pick out the color of his hearing aids. I decided on a metallic blue and black. They had what the called "flesh color", but it was not the color of Jack's flesh, so I decided against it. Even the Crayola crayon company has gotten rid of their "flesh" color crayon since it was not the color of a lot of people's flesh.

The molds will now go to a lab where they will make the part of the hearing aid that goes into his ear. The woman told me that they like to get a very tight fit or else everyone (except Jack) will hear a whistling noise coming from the hearing aid. We will go back in two weeks to pick them up.

The hearing aids are covered by Early Intervention which is great because they cost $1750 each. We were originally told we would get loaner hearing aids since we are hoping Jack will get cochlear implants and then he won't need the hearing aids anymore. I asked about this and the woman said he was already approved so we should just get them. She also said they didn't currently have any loaner hearing aids. I don't have to personally pay for them but it does seem a waste of money. She said I could bring them back when he is finished with them. I think I will find someone who needs them myself and send them to them. So, if you are reading this and need hearing aids for your child contact me and I will get them to you when I can.

I asked her if she thought the hearing aids will help Jack hear. She said Jack's hearing loss was beyond the limitations of their testing equipment. She said it was unlikely they will help him hear but you never know. They have two purposes other than helping him hear. One is to stimulate his auditory nerve and the other is to prove that he can't he helped by hearing aids so that the medical insurance will pay for his cochlear implants.

I hope they help him but I am having visions of them making a high pitch noise, the dog howling and everyone freaking out. Jack is able to ignore all the chaos but no one else is. Should be interesting.

Even though I am very aware that Jack is deaf, (or to be politically correct, I should say he has profound hearing loss) it is still a killer to hear them say, "his hearing loss is beyond the limits of our testing equipment." Julianna has no problem accepting it and Jack has no idea that anything could be different, but it still sometimes gets to me. I am not sure I will ever get to the place where it is no big deal.

Jack has been having some issues with his physical therapy. His therapist said she thinks he needs a helmet for his torticollis. I was really hoping to avoid it. He made great strides with therapy at the beginning. The therapy still seems to be helping him, but it is going a lot slower than the physical therapist would like. She said it will get harder and harder to correct as he gets bigger so we should do everything possible now. The helmet is weighted so it will force him to tilt his head to the left instead of always to the right, which is what he is doing now. He can turn his head to the left now. At first he couldn't go to the middle or the left. It seemed like his head was fused so he was turning all the way to the right. He has made a lot of progress and he can now go to the middle or the left, but he generally prefers to have his head turned to the right.

I asked the therapist if the helmet covers the ears. She said it does. I asked her how that would work with hearing aids or the cochlear implants. She said she didn't know since it had never come up before. She said they want the babies to wear the helmets 23 hours a day. I asked her how it would work if he wore his hearing aids or cochlear implants (when he gets them) in the day time and the helmet at night. She said she wasn't sure about it but she would find out. I really like his physical therapist. She has a former preemie herself, a 24 weeker, so she gets it. Her daughter had a bunch of issues but she is doing better now, although she still has significant visual impairment from having retinopathy of prematurity. She lives in Wantagh School District as well and said they are really great with special needs kids and offer a lot of services. That is good to know since it looks like we will be needing a lot.

Jack is still working on general body movement. He is still not rolling over at almost 9 months old (5 1/2 adjusted). My friend's baby is 2 1/2 months old and rolling over. All the babies in his class are sitting up. I know you aren't supposed to compare but it is tough not to. I can't help but wonder if we will ever get to the point where he is walking. He can bear weight on his legs and that is a good sign, so I hope we get there. It took him forever just to have head control, but it looks like he has that down now. I take some comfort in knowing that Julianna was very behind and eventually caught up. She was only 6 weeks early but she was many months behind in just about everything. No one would ever know that now. I hope Jack follows in her footsteps!

We still have physical therapy twice a week, speech therapy once a week and cranio-sacral therapy once a week. I do exercises with Jack constantly. I am hoping it all works. He is so happy and he laughs through all the exercises. Sometimes it hurts him and he gets mad but most of the time he is smiling and laughing. He just loves Julianna and he is happiest when he sees her. I try to get her to sit on his left side so he is forced to turn that way to see her.

Jack has a sedated CT scan on Monday. The scan is to check his inner ears and to see how thick his temporal bone is. It has to be a certain thickness in order to anchor the cochlear implant. Sometimes babies do not have a thick enough bone and the surgery has to wait until they are older. They are also going to check to make sure all his inner ear structures are present. He has an appointment with his doctor tomorrow to be cleared for the sedation.

On Tuesday night I went to the Jimmy Buffet concert at Jones Beach. While I was there I remembered the last time I saw him, four years ago. On that day I was told by a doctor that the hormone status of my cancer was the worst case scenario ( I later learned that was untrue due to herceptin) and it was an 8 out of 9 on the scale of how aggressive a tumor can be (Bloom-Richardson scale). http://maryellenandjulianna.blogspot.com/2009/08/tumor-hormone-status.html I made me think about how things often turn out a lot better than we think they will. I made it through that. Jack made it through his 106 day NICU stay where I heard over and over how he might not make it. Julianna made it through her early issues.  Maybe I will be able to bring them with me in a few years and they will BOTH be able to sing along. How cool would that be?

Updates - Genetic Testing

I haven't been keeping up on this as much as I would like. It has been a pretty busy summer so far. 

I took Jack for his genetic testing on 7/30. We went to Long Island Jewish. It took me about 3 months to get this appointment. I had to take the day off to go. They made us wait a long time and I always hate that.

When we got in there were two women there. The room was about 5 x 8 and we (Jack and I ) and the stroller were also in there. The woman started telling me what a gene was, what a chromosome was, etc. Pissed me off again. Why do they automatically assume you are an idiot? I explained to her that I was a biology major in college and I had a pretty good genetics background and I really just wanted to get the tests done. (Not a kindergarten level genetics lesson.) I told them we were supposed to have the deafness profile sent to Iowa and then a few more tests due to his cleft palate.

They then informed me that we couldn't do the deafness profile. The deafness profile gets sent to Iowa. The lab there is not a New York State accredited lab. Because they are not NY State accredited there are only so many tests from NY State they can do each year. They told me that the NY State limit had been reached the night before we got there. They told me we can send a few tests locally but we can't get the whole profile done. She said I can come back in January to have that done or I can go to another state to have his blood sent. Great. I waited three months to get in there and they can't send what I wanted sent. I hate LIJ. Immensely.

So we sent a few things and they said they will call me when the results come back. Luckily I do not have to go back there and have them hold my hand while they give me the results. I had to do that when I went for the genetic testing (BRCA) after being diagnosed with breast cancer. It was strange that they can call you up and tell you that you have cancer but they can't tell you the genetic test results unless you come in. They say it is because they wouldn't want to give you upsetting genetic news over the phone. But they can tell you that you have cancer over the phone. Go figure.

We should get the results in a few weeks. Until then we are working hard with physical therapy, enjoying summer and having fun!