The Terrible Sleeper

Usually I'm the one who delicately mentions some issue I've noticed with one of our kids, and waits for my husband to deny it for a while, before we finally get on the same page. But it was my husband who started this one. "Do you think Gili might need her tonsils out?" Not surprising, given that Big Bro and Big Sis both had theirs out; I'm waiting to get mine out; and it seems pretty common for CMV kids.



Bed I want for Gili.
http://www.comfyliftbed.com/

But Gili can't wait any longer. We hold her a good portion of the night to help her sleep better. She officially has "disruptive sleep apnea." And she's refused solid food for the last three days. And for her, "solid food" is mashed banana with baby cereal, applesauce, yogurt, mashed avocado, oatmeal, tehini... We're sending her back to school tomorrow, so maybe they can get her to eat.

She's sleeping on me now and I'm going to attemp to put her down and get some sleep.



Walker Gili is trying out
http://gal-kal.co.il 

No luck. I'm back. Someone in our CMV Facebook group posted this special bed that looks perfect for Gili. Exactly what she needs. Security without being in a cage. But it would cost $350 just to ship it to Israel, not to mention customs, on top of the cost of the bed.

We're also trying to get Gili a specialized walker. We did all the paper work but for some reason the Ministry of Health denied our request completely. So we'll just have to jump through some more hoops, but I don't think we'll have to pay for it on our own.

So, besides the material goods I'm coventing...
Gili has been clappng for a few weeks now. Not very purposefully, but she is getting her hands to meet in the middle. Today I had her in her high chair while I made her a bottle. She was pretty hungry. When I walking by with the bottle she lunged for it! I'm going to call that communication.

Her teachers say she is making progress. She explores the classroom. And her teachers call to say they miss her smile when she's out. I think she really lights up her class like she lights up our life!

Metabolic Disorder?

I'm waiting in the hospital for Gili to see a new specialist who just returned from London. The results of her MRI were inconsistent with congenital CMV and indicative of a degenerative metabolic disorder. I looked into the main one mentioned in the results - adrenoleukodystrophy (ADL). That's what ''Lorenzo'' had in Lorenzo's Oil. All her symptoms and previous tests still feel more consistent with CMV. But hey, things were getting boring around here, right? The private neurologist (Dr. Ashkenazi) called my cell phone when I was in the dollar store with Big Sis buying party favors for Gili's third birthday in school. So Big Sis is thirsty and needs a restroom, and I'm in a corner of the store trying to memorize all the details the doctor was giving me. I didn't think there were a lot of things worse than CMV. I thought they might tell us she was probably having seizures, or a chunk of her brain was missing, but I was not ready for them to start throwing around a word like ''degenerative''. Now it's 2:00 pm and our appointment was at 10:30 am! I hate this department but I've never waited over 2 hours!

Happy birthday Gili! So, that passed; along with the 50% chance she would walk by the time she was three. Thank G-d she is still making progress. She's feeding herself better and standing and cruising better, and I do think she will walk one day. I don't have much hope for her ever talking. I'm not sure if I'm supposed to cry about that. I guess I've always felt that this is just how Gili is and how she was meant to be, and we are lucky she is in our lives. I try not to think about what if I was more careful when I was pregnant, or what would she have been like if an evil virus hadn't eaten away at her brain while she was developing. But I have had some darker thoughts lately.

When I was holding Gili yesterday I starting making up a ''once upon a time'' story like I do for all the kids, with some moral or analogy. This one was a tad morbid. But aren't all fairy tales? I'll post it later.

I guess I should say something about the Israeli ''situation''. The news has been on all day in the hospital, but I don't understand a word. They are showing clips of missiles but I don't know the details. I actually heard an air-raid siren as I was entering the hospital this morning (a very long time ago it seems), but I think it was in another city, because people inside didn't here it. Then everyone was watching the news and the caption said something about Gush Dan, which is technically the greater area including Petach Tikva, where we live. We don't live in a very safe building and I'm confused about what we're suposed to do and what kinds of provisions we should have in the house. Maybe we'll order two pizzas for dinner and freeze one :-p

Oh, some good news!! While I was waiting, I got the dental specialist to check Gili's teeth and they are... Drum roll please... Okay! She survived all that grinding and falling asleep after bottles! Woohoo! Unfortunately, the dentist woke her up after she had finally fallen asleep in her stroller. Amazingly, she seems to be in good spirits.
I kept going back and forth about who would take Gili to this appointment. I couldn't find someone to watch Lil Bro, and Hubby wouldn't let us both go with him. Last night and this morning I started feeling bad about not spending enough time with Gili, so I decided I'd take her. But now I'm just tired and hungry. Not exactly having the great mother-daughter day I had planned.



HERE'S THE REPORT ...Five hours later...
I should have known this, but I couldn't read the MRI results in Hebrew. The results showed demyelination - some regression in the development of the white matter. We waited so long because we wanted to see a specific doctor who speciallizes in metabolic disorders - Dr. Avraham Zeharia. He mostly asked us a lot of questions about her history.
Gili is just barely within the normal hight for her age (she can reach the stove!), but her weight (10.9 kilos) is low. She is boarderline microcephaly. Her spleen is still a little enlarged, but he didn't feel it at first because it was near the back (whatever that means). We have to go back on Tuesday morning to take LOTS of blood. Some of the test will get sent to Jerusalem. They're doing tests an amino acids, organic acids, long chain fatty acids, and so on. They will also test her for the genetic Gaucher's Disease. One test requires her to fast for four hours.

Then we'll go back in two months to discuss the results. Unless they call us before then. But for the next two months, no news is good news.  Dr. Zeharia was appalled (as I am) that Gili was not currently getting physical therapy (the therapist is on maternity leave) and that her school doesn't have communications (speech) therapy this year. My friend, a nursing student, met me in the hospital around 2:00 pm and went in with me. She said that this doctor is very good. But for Gili's sake, I hope we won't be seeing him too often!

Note to self: Schedule BERA (hearing test).1

New Eye Doc

My husband took Gili to a new eye doctor in Kfar Saba - Dr. Noyman., who's name we got from Gili's school last year. It was a very good experience; very different from the overly "efficient" treatment in Schneider. I asked him to write down everything he remembered.

We arrived at Dr. Doron Noyman’s office at 4:15 for our 4:20 appointment.  The secretary keeps our information on a 5x7 index card which goes in his old fashioned file system.  He writes down all the important things we did for his internal records on these cards.  We had to wait around 30 minutes to see the doctor as there were several other appointments around the same time.

Dr. Noyman speaks excellent English and I saw on the wall he spent 3 years in Los Angeles at an eye institute.  He started out by asking about Gili’s medical history and looking over her past appointment summary sheets. 

The first test he did was to see if Gili could follow objects.  He noticed that she sometimes followed but then would lose interest.  She was quite good at grabbing close objects so that was a good sign he said.

The next test he had a toy mouse that was mounted on the wall.  He would activate the mouse to see if Gili looked at it.  She did notice the toy.  When he covered up her right eye she tried to move around to see, but when he covered up her left eye she didn’t have a reaction.  He said that this shows a weakness in the left eye.

He calls cross eyes “squinting” and said that he did notice Gili doing it intermittently but he said that she wasn’t doing it at such an extreme angle that he would recommend surgery to correct it.

Gili then got her eyes dilated with drops and we came back 45 minutes later for a refraction and cataract test.

The refraction test showed that she had a slight astigmatism and had slight weakness in her far field vision.  In an adult this would require lenses, but because Gili doesn’t really use far field vision (i.e. for driving a car) he doesn’t recommend glasses.

The cataract test came back negative, though he said that cataracts can occur in CMV positive children.

He saw the scar in her left eye and described it as being right in the middle of the retina.  This is known as a macular scar and was due to the virus attacking the eye while Gili was developing.  He said that it is a significant scar due to it’s central location and is contributing to reduced vision in her left eye. 

Even though Gili does have multiple vision problems, there are no treatments he can recommend that will be effective.

Dr. Noyman suggested that we only need to come back on an annual basis if we feel that there may be a change in her visual ability.

Dr. Noyman said that macular scarring is very common in kids with CMV, but, unlike in cases of toxoplasmosis, if does not "flare up." By the time they found it at 2.5 months of age it was already innactive. The doctor said it is definately effecting her vision and it is about the third of the size of a scar that would cause her to be completely blind.

Update to Our State of Mind

Gili's first Walker - August 21, 2012

Two years ago today, I made a list of things I wonder about. Now I have some answers.

- Will Gili be able to be in a regular class when she starts grade school?
No

- Will strangers be able to tell she's "special" from her face? (I don't mean her sparkling blue eyes and infectious smile.)
Yes

- Do CMV babies live as long as other people?
No. See this thread from Facebook. Life expectancy of children with cerebral palsy is greatly dependant on whether or not they walk.

- How often will I be taking her to physical therapy and other types of therapy?
Thank G-d, I know longer have the shlep her around. She gets all her therapy during the school day. When she begins school next week she will have therapy, activities, and love until past 5pm most days. She also is provided transportation to and from school. How far we've come from when I started this blog!

- Will teachers or parents worry about her or my other children being contagious? Should they worry?
It doesn't come up much, but I use it as an opportunity to say, all kids of preschool age should be regarded as conatagious. Wash your hands.
-
 Will she get married and have a normal life? Will potential suiters consider her defective?
It is unlikely that Gili will get married and lead a "normal" life.

- Will she "catch up", we'll forget she ever had CMV, and this blog will be abandoned into the cyberspace abyss?
It doesn't look like that will happen, short of a great miracle. We'll take whatever little miracles we can get. I'm sure there are many miracles that I am unaware of that allowed her to even be born, and more that have allowed us to still have her in our family today.

If you'd like to skip to my first blog posts, check out the "In the beginning..." links in the right hand column.

Life Expectancy and Lungs

I hope it's okay that I'm posting this thread from a private Facebook group. I think this is a question we all want to ask, but don’t want to think about. I mean no disrespect by replacing the beautiful names of each child with “my son.”

 

 

 

HK: I hope this question isn't too hard but we are pretty new with our diagnosis still and learning still.

 

What tends to be the cause of death for cCMV kiddos? In the short time I've been here [a member of this forum] there have been a few deaths and I'm curious if there's something I haven't been told by our docs?

 

Thanks!

 

RL: From what I can see, it's all over the board. Feeding difficulties, seizures, illnesses, bodies just plain shutting down. Sorry, I know that doesn't answer anything. Just remember that everyone is different, and our kids very often surprise us.

 

HK: Thanks RL. I appreciate it! If anyone has any other info I'd be interested in hearing it as well :-)

 

AE: Hi HK, Here's my take on it... and mine is 11yo now so I am definitely no expert. And I guess I look at it some from a nursing perspective. I think with most of the problems our kiddos have, the lungs tend to be the major area of concern for me. I’ve seen the lungs go bad in so many different situations... seizures, swallowing issues, etc. I guess the thing that scares me the most is how quickly their little lungs can go bad. I haven't looked at the stats in a long time, but the number one cause of death in children and adults with disabilities used to be aspiration pneumonia. Not sure what it is now. Nevertheless I am very strict about who feeds my son and I don't tolerate anyone that doesn't take safe feeding seriously. I am also vigilant about making sure I keep him up and moving, swimming, bike riding, whatever it takes to make sure his lungs don't get weak.

 

HK: That's helpful AE. Your explanation really helps bring what our doc said to something more meaningful. When we asked about prognosis, etc, he said it would depend on mobility and any other secondary issues. I hadn't connected all the dots in my mind until your explanation, so thank you.

 

We struggle with feeding issues. My son is g-tube dependent. He is now 9 months old and doesn't take any liquids by mouth, not for lack of trying on our part, but because little man can't figure it out. He had a severe bout of RSV back at 6 weeks old as well and has been having repeated upper respiratory problems recently. I've heard that the RSV alone can take a toll on the lungs in a healthy child, so I'm getting nervous about this winter and how he will do.

 

AE: Don't get nervous HK... I tried it for a while and it doesn't help at all. Just think about it like this... The lungs are meant to be upright for the larger part of the day. Even if your son is not very mobile (you won't really know until he does or doesn't meet milestones), there are lots of ways to keep him moving, even if it just means carrying him upright. We were blessed in that our son was able to hold his head up fairly well by the time he was a couple years old so we started putting him in a walker (just a regular baby walker) which kept him upright, forced him to gain trunk control, and also put pressure on his legs which is very important for normal leg length and strength. We also scheduled MBS studies so that I could be the one feeding him and tried many different positions to see which was least risky for swallowing issues or aspiration. For many years, a reclining position worked the best for him and we kept our study results close by because every nurse you will ever meet will insist that children must sit up in order to swallow safely... don't ever, ever let someone that doesn't understand your child's individual needs dictate the care he gets. You know your child best and don't let anyone ever tell you any differently. Safe feeding can only be determined by MBS done with you in the driver’s seat. Another thing that I want to say to you is this (with reference to winter). Cold weather has never ever been the cause of an infection. Infection is caused by bacteria, virus, or other type of bug. Now with that being said... the weather can make a difference in whether or not a bug can survive. Heat and humidity makes for a much better growth medium for bugs. Embrace the cold winter months... Check out the statistics.... people from northern colder states actually live longer than those nuts like me who hang out with mosquitoes and snakes and such. My best advice for you though is to cherish every moment and have fun with your son. I spent several years waiting for the shoe to drop and at some point I realized I was wasting precious time and just made myself start doing "fun stuff" instead. I know I will one day lose him, but I'm determined to make the very most of the time we have, no matter how long that might be. He is my joy and I truly believe that God sent him to me because I was exactly what he needed and he was exactly what I needed. We are a team. Others may not understand us, but we understand each other and that is what matters most.

 

HK: That was beautiful :-)

 

DB: Hi HK, I asked Dr. Stuart Adler, a pediatric CMV expert, this question. He told me that CMV per se does not lower life expectancy; it is the resulting conditions that would affect that. So, a child with cerebral palsy, or respiratory issues, or whatever other health issues, would be subject to the risks associated with those specific conditions. I hope that helps!

 

HK: Thanks DB, it does help!

 

LM: Hi HK. My son passed away in 2010 because of respiratory failure. As he got bigger, he could no longer cope with the demands of his body because of the severe neurological damage. I am also sure that his epilepsy had an impact. Winters were also scary times for us. The trick, and it is one of the lessons we learnt, is don’t think too far ahead, especially in the early days as it can seem so daunting. Take it day by day and things will "normalize" in the sense that you will become more used to dealing with your special man’s needs. As I am sure he is already doing, he will surprise you in so many ways and bring you joy in ways you never thought. Don’t get too scared about what has happened with other kids, each are unique. Always here to chat, Lots of love xxxx

 

HK: Thank you LM! I am sorry to hear about your sons passing. I look at my son when he is not fighting off any of the infections or viruses and he seems so healthy; but when he gets sick, he gets it so much harder than his sisters. He has had a month of back to back illnesses now and woke up wheezing this morning similar to when he had RSV and we almost lost him. It sure is hard to try to lead as normal a life as possible but balance that with trying to keep little man healthy.

 

Appreciate your sharing! Thanks!

 

Autism Ambivalence

Gili's 'diagnosis' of autism hasn't really changed anything, but my husband and I do think about it once in a while. It really hit me when I saw it on the Neurologists's report. It was one of the few things I could read.

My husband said, "I thought Gili couldn't be autistic because she's special needs." That's what I thought, too. I thought you had to be higher functioning to fall within the autism spectrum. I still don't think Gili has autism. The neurologist just said that her behavior, like the repetitive motions she makes slapping the floor and repeating the same sound like, "d'ge, d'ge, d'ge," are consistent with autism specrum disorder.

I've taken this opportunity to join a couple of the many autism groups/forums. I figure some extra advice can't hurt. Though I still feel like I get the best advice from the other CMV moms. I think the main difference is that Gili has no concept of right and wrong and therefore she can't "misbehave."

Yesterday Big Sis asked my husband why he loves Gili the most. I don't think he came close to denying it, he was just somehow surprised that she noticed. I joke that Gili has the best father in the world... even better than her brothers and sister.  He answered that he loves everyone the same. But to me, he answered that it's because she's the best behaved. He's blind the the things that would be horrific if "normal" two year olds did them. It's not likes she's an angel, she just doesn't know better, so he can't get mad at her. It made me think about how we get stressed out about our other kids misbehaving, ignoring us, etc. Really they are no worse than other kids their age. They can be monsters at home, but I am grateful that I can usually trust them to behave for others, teachers, other parents. There are pre-teens and teens who actually come play with them for free. So thank G-d, they are basically sweet kids.

I think that my husband give Gili the most love to compensate for the attention she doesn't get from others. Big Sis is obsessed with Lil Bro and only wants to play with him "because he's so cute." She rarely even touches Gili. And babysitters, even with the best intentions, are a little stumped on what to do with her. "So, what does she like to do?" Even now, I'm home juggling two little ones, it's little Bro who's sleeping (with a fever) on my chest, while Gili is kicking the door behind me in need of attention. As much as I wish my husband would be more attentive to the needs of our infant, it's good that Gili has an advocate. It's a shame we can't just be on the same team, but I naturally put the needs on my helpless nursling first. So it's good that hubby makes sure Gili gets what she needs, even if she's not the "squeekiest wheel" in the house.

Gross Motor Function Classification System for cerebral palsy (GMFCS)

Tracy McGinnis of the Brendan B. McGinnis Congenital CMV Foundation posted this GMFCS PDF link to our CMV Mommies group on Facebook. It describes how they classify the severety of Cerebral Palsy, at least in the US. Check it out!

I think Gili would be classified as lavel 3, but she might reach into the level 2 category.

LEVEL III: Children maintain floor sitting often by "W-sitting" (sitting between flexed and internally rotated hips and knees) and may require adult assistance to assume sitting. Children creep on their stomach or crawl on hands and knees (often without reciprocal leg movements) as their primary methods of self-mobility. Children may pull to stand on a stable surface and cruise short distances.
Children may walk short distances indoors using a hand-held mobility device (walker) and adult assistance for steering and turning.