Not Quite Five Months

 Naomi has biweekly sleepovers at Grandma and Poppa's that she just loves.  She's been going on her sleepovers since she was 19 months old.  Every time I ask her with her cards if she likes sleeping over at Grandma and Poppa's she says yes and she always says she wants to sleep over more.  She has her own room there and her own bed and gets lots of individual attention, which is just what she likes.  The other grandchildren even refer to it as 'Naomi's room' and 'Naomi's bed.'   I call after I think she should be asleep to ask how her evening went, and I call in the morning to see what time she woke up and what her mood is like.  I usually get good reports, which means she is having fun and her grandparents are having fun with her, which I like.  The sleepovers are not only good for Naomi, but they are good for us and for Lydia too.  We get a break from the stress and strain of caring for Naomi, we get to focus on Lydia and Lydia gets to be the center of attention for a while.  It also means we can do things that can be difficult with Naomi, especially with Naomi and her baby sister together, like going out to eat.  We can keep Naomi somewhat happy in a restaurant if she isn't overly tired, has already been fed, has her portable show player for distraction and entertainment, and isn't assaulted by loud or sudden noises.  It can be hard to choose a restaurant.  It has to be loud enough that Naomi's noise won't ruin the ambiance for the other diners, but not so loud that she will be frightened.  With Lydia, we have another variable to consider that we can't control.  If Lydia starts crying, Naomi will most likely start her shrieking, which means we will need to remove her from the restaurant ASAP.  Her shrieking is a lot like an air raid siren.  To describe is as ear piercing makes it sound a lot more pleasant than it really is.  I think we have attempted taking the entire family out to dinner once since Lydia was born and it was a major failure.  So after we dropped Naomi off for her most recent sleepover, we headed over to a restaurant for a dinner out.  Lydia was great the last time we did this, but not so great this time.  As she is getting older she is less and less tolerant of being strapped into her car seat.  She wants to be out and moving around.  So there was some unhappy squealing and we had to take turns eating while holding her and helping her play with her toys.  It was great, really.  It was so just exactly how it should be with a not quite 5 month old baby.  We were eating, playing with her, enjoying her, enjoying each other, laughing at her antics.  I felt quite happy.  Then I realized I felt happy and immediately felt guilty.  I felt guilty because Naomi was not there with us and therefore not a part of our happiness.  I felt guilty because if Naomi had been there we probably would've already had to leave because Lydia was being noisy and then we would have been disappointed and unhappy instead.  And I felt guilty because even if she were there and dealing with the noise well, having to manage a second needy child would have distracted us from enjoying each other.  Right now, in public, we can handle Naomi or Lydia but not Naomi and Lydia.  The next emotion I experienced was profound regret and sadness.  I thought back to what Naomi was like at not quite 5 months old and what our lives were like when she was not quite five months old and I wished that it had been like it is with Lydia how rather than how it was.  I wished that Naomi did not have batten's disease.  Naomi at not quite five months old was still mostly like an infant, though she had started doing some new things.  And we were very, very sad.  I was still in the weeping all day long, day in, day out phase.  So far, Lydia seems to be doing all of the things that she should be doing.  At this age, Naomi had started putting her hand on the breast when she nursed, she was bringing her hands together at midline, she was pulling up her legs and grabbing her knees, and she had rolled from belly to back one time.  She occasionally smiled to my voice.  She had not yet laughed, she was still pretty much visually unresponsive to anything but light, she showed no interest in toys.  She didn't reach for us.  She never has, in fact.  She wasn't cooing or squealing or using her voice to get our attention.  We had also by this time immersed ourselves in dealing with Naomi's diagnosis and lack of development.  The biggest thing we did was to enroll her in Early Intervention Services.  I was very anxious to get started with therapy in the hopes that I could maximize Naomi's potential.  On that point I was powerfully determined.  Determination is one of my better qualities.  And I was determined that  my baby would be the best that she could be.   So of course I was anxious to get Naomi started with professional therapists. I can't say enough good things about the Early Intervention program, or the providers and therapists that worked with us.  The entire program is designed to serve the needs of the child and the family in the most family centered way possible.  All of the therapy occurs at times and places where the child would normally be and when they are normally alert and awake.  Never at an outside site, never during a nap time, etc.  So Naomi's EI evaluation happened in our family room where Naomi and I spent most of our time.  It was a sunny late February mid morning.  I wanted Naomi to make a good impression (I don't even know what that means except that maybe I wanted the evaluators to think she was cute) so I dressed her in my favorite outfit of hers at the time.  Peachy pink fleece overalls with a little white with pink circle dots top and mutli-colored crocheted booties that she was given as part of her infant set with a hat in the hospital when she was born.  It was an outfit I had bought for her before she was born and I thought she looked deliciously cute it in.  I wore it on Lydia too, but it didn't quite flatter her the way it did Naomi.  Different baby.  I remember the evaluators putting Naomi through her paces and my heart just bursting with love for her...how sweet she looked, her darling facial expressions in response to various manipulations, the way I could see her trying to respond to the various activities.  I knew, from my endless research, that Naomi was still pretty much at the level of a newborn, but being told that her developmental level was 0 months old/100% delayed in all areas was still a painful shock.  No matter how realistic you are about your child's disabilities, or how much you have internalized your child's dismal prognosis, no matter how difficult day to day life with them can be, it never gets easier confronting the truth.  It a painful reality I have to come to terms with again and again, every morning when I wake up, and through out every day.  I am constantly thinking,  'Did this really happen?  Is this really true? Is this really real?  I can't believe this happened.  Why did this have to happen?  I wish this hadn't happened.'  And on and on.  Four and a half years later and it isn't any easier.  True, I no longer weep every day, but tears are all to frequent still.  And my mind still constantly searches for an 'out', for a way around this ugly truth and the ugly events I know the future holds.  Therapy started in earnest in March.  To say that Naomi's therapists were a huge part of our lives from that point until she aged out of the program at 3 would be a vast understatement.  They were some of the only people with whom I felt comfortable.  I didn't have to explain Naomi to them, children like Naomi are not strange or scary to them, they never tried to offer me comfort with unrealistically cheerful expectations, and they never sent me into the depths of despair with insensitive off hand comments.  They accepted Naomi as she was and tried their hardest to help her advance to her next milestone.  And at the same time, though I was bawling my eyes out every day, they were upbeat and cheerful in a way that was natural and soothing...not by pretending everything was fine, or assuming that Naomi was fixable or her problems curable, but by just being there and working with her in week in and week out in ways that were caring and compassionate.  As a stay at home mother in self imposed seclusion with a profoundly disabled child, in some ways they were my only friends and were most weeks my only social outlet.  And we were lucky to have the same group of therapists for Naomi's entire EI career...Marla Wyland for PT, SueEllen Foster for OT, Karen Anderko for developmental, Bev Moss-Oswalt for Social Work, Marilyn Neal for vision and, later, Becky Burns for speech.  They all were, and are, wonderful people and gifted therapists.   Naomi's favorite part of EI was Infant Massage.  She loved it, except for arms and hands, which is so interesting to me since Naomi became so extremely hand defensive as she got older.  So we started EI.  We also took Naomi on a trip to a Catholic shrine in Quebec City, Quebec, St. Anne de Beaupre.  Many people, including my mother, report miraculous cures after visiting this shrine.  (See newspaper my mother below.  You can click on the picture to enlarge it so you can read it.)  It is why my name is Anne.  So when we were given the awful diagnosis the first thing we thought, and what everyone in my family thought, was "We need to go to Canada."  It was a great trip and one we have fond memories of.  We drove.  12 hours each way.  Quebec City is on the St. Lawrence River and in March it is still in the deep of winter.  The amount of snow was incredible and the way the city dealt with the snow was fascinating.  They don't plow the snow.  They can't.  There is so much that there is nowhere to plow it too.  The non-plowed snow pack on the ground was as tall as I am...over 5 feet.  People and businesses erect tents over their driveways and walkways to reduce the amount of shoveling they have to do.  There were canopies 40 feet long from the front door of a house to the sidewalk.  And the city has snow removal vehicles that are like a combination of an excavator and a dump truck.  The snow is not shoveled from the roads, but excavated into the back of the truck and them dumped in the St. Lawrence, which was still frozen over.  Like I said, it was fascinating.  Being in Quebec, the city was French speaking and though there were people who spoke English there and in the province as we drove through it on the Trans Canada highway system, we usually had to ask for them.  We made no concrete plans for the trip either, other than that we would drive up over two days, spend two days in the city and drive back over two days.  We stopped in a hotel for the night when we got tired.  We used the car GPS to find a hotel in the city,The Fairmont Le Chateau Frontenac.  It's a beautiful old hotel, famous, and rather ritzy but because it was the off season it was inexpensive too.  We enjoyed exploring the city.  The food was unbelievably good.  Naomi was actually very easy at that time.  We could take her anywhere.  In restaurants we laid her on the other end of the table and she would sleep.  The trip was exactly what we needed at that time...the spontaneity, the freedom, the escape.  The morning of the second day in the city we took Naomi to the shrine.  It was an emotional experience, considering my family history and where we were in our lives with Naomi at that point.  My Mother had instructed me to ask for complete Earthly healing for Naomi but all I could pray was 'Please don't let Batten's disease take her away from me.  Just let me have her as she is.'  We both felt good after the visit.  When her diagnosis was rescinded 10 months later we wondered if it was the answer to our prayer.  When it was reinstated 6 months after that we were devastated.  But, Naomi is still today pretty much as she was then and I have to wonder if, in fact, my prayers were answered.  I wonder if I was granted the miracle I asked for.  If so, I am deeply thankful.  Naomi is very difficult to care for but we love her as she is and would love to get to keep her as she is.

Lydia out for dinner with Mommy and Daddy

Naomi in the outfit she wore for her Early Intervention evaluation.

Naomi in an outfit I made for her before she was born.  She was already enjoying doing her Big Kicks, just like she does today.

Naomi and I were sitting in front of a big double window in our family room that she loved.  

Naomi in a sweater knitted for her by my friend Annie.

Naomi sticking her tongue out, another thing she still likes to do today.

Naomi enjoying being dressed in only her diaper.    I hate the thought of a tube marring that little belly I've loved so much.

Playing with Daddy.

Naomi with a blanket rolled up to keep her from turning her head.  She repeatedly would  get her head stuck, keeping it only turned to one side until I intervened, and then keeping it turned only to the other.  So I kept a blanket on which every side she was preferring.  I also would lay her so the light from the windows was away from the preferred side to encourage her to turn her head on her own, and would go in when she was asleep and gently turn her head the other way.  This went on for months.  I worked really diligently at it because I didn't want to add torticollis to the list of issues Naomi was facing.  

Naomi draped on the arm of the chair.  This was my modification for belly time to strength her neck and practice head control.  Flat on the floor was difficult for her, and can be scary for the visually impaired, especially for a baby like Naomi who tended to keep her eyes downcast. 

More practicing.  I've always loved this picture.  She looks so sweet.

Naomi and Daddy on the couch, probably watching hockey.

Naomi and Daddy in a hotel in upstate New York on  our way to Quebec.

My pretty little baby girl.

The roofline of the hotel.

Mommy and Naomi in the hotel in Quebec City.

On our way in to the church.

Inside the shrine.  Flash  photography was not allowed.

Walking aides left behind by those cured of their ailments.

Daddy and Naomi outside St. Anne's afterwards.

The St. Lawrence River.

Quebec City.

Downtown.

Shopping District

The Fairmont le Chateau Frontenac

Naomi pulling up her knees!  This was new just on the trip.

More Big Kicks in Quebec City.

Love this picture.  There's the big window she loved.

I took a LOT of pictures.

My sleeping beauty.

Trying to strength her trunk and neck.

Naomi and Daddy relaxing on the porch on the first warm day of spring.

..

Easter Sunday and Other Happenings

We had the surgery consult for Naomi's feeding tube placement last Thursday.  We were both second guessing the decision to have the tube placed leading up to the appointment.  The surgeon, Dr. Scholz, was very thorough and patient.  He answered all of our questions and showed us the device that will be placed, explained how it works and how they are changed.  We felt much more comfortable after the appointment and very positive about our decision to proceed.  In the time since then we have seesawed back and forth about it all.  It's a very hard decision to make, for a lot of reasons, especially when you are dealing with an illness like Naomi's.  Any intervention, like a feeding tube, has the potential to extend life.  Extended life is not necessarily always a good thing.  Each individual case is unique and the beliefs and feelings of the patient and the caregivers must be considered.  I categorically judge no one for the decisions that they have made or will make that are different from those we are making and will make for Naomi.  We decided, when Naomi was diagnosed, that we would pursue minimal interventions and only those that would extend her life while she still has the capacity to enjoy her life.  We do not think keeping her body alive when her brain is gone is what is best for her or, just as importantly, for us.  At the same time, we do not want to deny Naomi interventions that could make her more comfortable while she is still cognizant.  So a feeding tube treads that line.  At this point, we struggle to feed Naomi and the struggle causes us incredible stress, and as such, the stress extends to Naomi as well.  We now also have Lydia to consider.  It is very important to think about and manage the experiences and needs of all family members, not just the sick one.  We believe getting Naomi a feeding tube will improve the quality of life of all members of our family.  Most importantly, we feel we will be able to provide Naomi better nutrition with the tube than we can without it.  I know I feel like garbage when I don't eat what I call real food...not power bars, or protein shakes or other things that have to be made in a lab, but food grown and prepared as, for lack of a better phrase, nature intended.  Half of Naomi's calories come from what is basically liquid powerbar drinks.  We often wonder if her mood would be better, and her other issues, like chronic constipation, better if she ate a better diet.  My plan is to feed  Naomi what is called a blenderized diet through her tube, gradually replacing most if not all of the 'power bar' calories with good, real, food.  The surgeon said as long as it goes through the tube, we can feed it to her.  The ability to improve the quality of her diet was the ultimate deciding factor in favor of the feeding tube.  I am, actually, excited about the blenderized diet and so for the past week I have been doing one of the things I do best: research.  I've been studying blenderized diets, reading, calculating and planning.  It feels good to have something active to do to help Naomi.  So often I feel powerless and useless in the face of the gravity of her diagnosis and  disabilities.
The decision to have the feeding tube placed was and is difficult for other reasons as well.  First of all, it's one more step away from normal.  No, it is not normal to spoon and bottle feed your four and half year old child. But it is very normal to feed your nine month old child that way, so it doesn't feel that, well, unnatural.  Naomi never progressed beyond that so it is just sort of how it has always been.  Feeding your child through a tube placed in her stomach is very, very, very far from normal.  On the other hand, who are we kidding?   We don't live a normal life in ANY way.  So at some point, well, it's all water under the bridge.  It is also hard because it will changer her physical appearance.  (I love her cute little belly.) This is mostly only an issue for me.  Rob isn't bothered by it.  But last summer Naomi fell and chipped one of her front teeth and I cried for days over it because I hated that her teeth were no longer the way they were "supposed" to be.  It's also hard because it is the first tangible step down the inevitable road of her regression to a vegetative state and her death.  That feels very bad.  Very, very bad.
In other news, Naomi has been sleeping much better and eating much better.  She ate a Stage 3 baby food dinner today for the first time in over two years.  As a result, she's been in a much better mood, has been mostly her old self personality wise, and has been having great days at school again, which we really love to hear.  It is ironic that she started eating better pretty much as soon as we decided to get the feeding tube.  The improvement in her mood with better sleep and food, however, reinforces the decision to move forward with the tube.   Easter went mostly well, for a holiday with Naomi.  She was up early, which was unfortunate but because she's less sleep deprived in general she handled it a lot better.  She wore her pretty dress I made for her and Lydia wore the dress I made for Naomi for her first Easter.  They also both got little rabbits I made from a fun pattern by Alicia Paulson of Posie Rosy Little Things.  It would have been more gratifying for me if either of them had been able to play with their rabbits or been excited about their pretty dresses, but maybe next year.  Or the year after that.  As it was,  Naomi and Lydia looked beautiful to me. and it was fun putting the rabbits in their baskets and posing them with them on their laps.  Naomi and Daddy participated in the Easter egg hunt at Grandma and Poppa's church and she really enjoyed it!  It was fun.  Afterwards we had Easter dinner at Grandma and Poppa's with Auntie Sarah,  Uncle Brent, Grace, Margot, Uncle Joel and Aunt Jane.  Then Naomi fell asleep on the couch watching a show.  That almost NEVER happens.  (She was up early.)  We usually use shows to keep her awake.  She was a bit of a grouch when she woke up so we had to take her home but she was pleasant and sweet the rest of the evening.  Daddy had off Easter Monday too, after being off Thursday for her appointment and Good Friday and the weekend, so that extra time was nice.

Lydia's Easter basket was in the jumper! With candy for the adults.  

Naomi's Easter basket was in her swing!   With more candy for the adults and some special juice for Naomi.   

All ready for church on Easter morning.

With their rabbits.

Naomi and Daddy hunting eggs.  Naomi carried her own basket over her arm!  So cute.

There's one!

Get it, Daddy!

All of us.

Asleep watching a show.

Tough Choices

Last Monday we had an appointment at the Neurodegenerative Rare Disease clinic at Children's Hospital of Pittsburgh.  It was Naomi's second appointment there.  The clinic follows kids with all sorts of degenerative diseases longitudinally.  Dr. Escolar is the doctor in charge and I guess for some diseases she and her husband have done bone marrow transplant with some success in halting the disease.  Not so for Batten's, but it is a good resource for us to ask questions and have OT/PT/Speech/Hearing eval with folks who are used to seeing kids like Naomi.

 Naomi has been doing worse.  Over the last two months she seems to have regressed some.  Her sleep has been very difficult.  She is getting up early and not napping well despite us trying all the tricks we have tried in the past (early bedtime, late bedtime, restricting napping etc.)  As a result she has been very prone to tantrums and has had poor ability to tolerate anything for any amount of time. She is shreiking again when her sister makes any noise.  She is impossible after napping.  It is very upsetting to see her like that for us - we want her short life to be a  happy one.  She has not been eating well either.  We are down to oatmeal and strawberry soy yogurt (yuk) and it is 30-45 minutes of struggling and we are noticing things like lots of spitting out and hard swallowing.

We explained all of these things to the folks at the clinic.  They felt like all were signs of the beginning of regression.  Especially the difficulty eating and swallowing.  They recommended a feeding tube.

Living day to day it is easy to put Naomi's future out of your mind.  Sure she is different, but to us her different is normal.  We are not around normal 4 year olds all the time and in a sense don't fully realize how limited Naomi is.  She has always been kinda difficult to feed and very picky, and sure she has gotten more difficult and more picky, but we always felt like that was just her.  It's hard for someone to tell you that it isn't just her, but her degenerative disease.  Before this we could try to forget, or pretend that somehow Naomi was going to be okay (well, not okay but not getting any worse).  This feeding tube suggestion has shaken us out of our delusions.

We are going to meet with the surgeon next week.  We are going to get the feeding tube.  We know that it not going to prevent any further regression or change Naomi's course, but it will truly make her life better.  We won't have to torture her to eat or drink.  She will still  be able to do all the things she likes to do like swimming.  It still sucks.  Our reality sucks.

Naomi decided she needed a nap just as we got to the clinic...at 9am.

Naomi had a good time showing off all of her moves to the Physical Therapist, especially her Big Kicks.

Lydia got bored and cranky had to be mostly stripped down for some play time.   She loves to be unclothed.  Just wait until summer, Lydia!

Naomi, Lydia and Clementine having a morning snuggle.

Naomi and Clementine watching a show.

Naomi and Lydia right before Naomi got on the van to go to school.  Naomi has been picking her outfits and her hairstyles.  She prefers two 'Dakota' (the pony she rides on Saturday mornings) ponies.

Bear on a belly!

All dressed up for school.