Tuesday, April 11, 2017


We now have a door on the kitchen.  My daughter is 5 and a half.  I thought it would be later, when she was older, but the time is here.
I am thankful that the door has worked as a barrier not only to food on the counter or just barely empty cinnamon bottles, but also to a seemingly uncontrollable sneaking of food.
I've read the brochures, the articles on the National PWS site, posts on the PWS FB page, listened to doctors...all of it.  But still you cling onto the hope that your child will be different.  And I still have hope that she will be.  Each PWS phase she reaches I think, "Well, ok, she won't go through the next one, not so bad anyway.  She's on the less affected end of the spectrum."
And really she is.  I am grateful for that.  Freya never had a feeding tube, and while she didn't walk until after reaching 2 years old, she can now run and jump, not like her peers mind you, but she can, and does, and delights in all of her movements.  Her articulation is not the clearest and it is often a struggle to understand her, but she speaks in sentences and can usually make herself understood.  If not, she has a little sister who comes to her rescue.  She knows letters, numbers, shapes...all of the basics a kid her age should know.  She loves stories, dance parties and seeing friends.
But here is the thing, as grateful as I am that she can do all of these things, it does not mask the other characteristics, not to me.

The day we decided it was time for the kitchen door began with a red faced Freya having found an "empty" cinnamon bottle (Cosco size no less) in the recycling, getting a spoon and scraping out what she could.  All while we were blissfully asleep.  A little later, after having left the kitchen for just a few minutes, I came back to find that she had taken a pair of scissors to the bag of steel cut oats.  The slumped shoulders of shame accentuated the look of almost disbelief on her face, as if she had just not been able to stop herself, though she knew she should.  This seemed different, and far more alarming, than the few mornings before when she had eaten 4 bananas before we woke up.  I thought putting the fruit up high would be enough.

She cries for food sometimes, heartbreakingly so, even when I know she should not be hungry.  I come to pick her up from her class and, even if she is in the middle of an art project or a pirouette, the first thing she asks is if I got her a snack.  And won't stop asking until I tell her when she will eat again.
There are times now when she just seems sad, without explanation.  At these times all I can do is hold her and rub her back, telling her how much I love her, while tears stream down her face.  My daughter is different, and I think she knows this.

When we are in public and see someone in a wheel chair or walking with a cane, or someone with oxygen strapped to their nose...anything that makes them stand out to a 5 year old that always asks "what's that" and "why?", I take that opportunity to explain how everyone's body is different and some need extra help.  I now mention to her that her belly and brain will tell her she is hungry when she is not, and that she should not listen.

How do you explain to your child to not listen to something as instinctual as hunger?

Even as I write this I am remembering something I read years ago when we were first learning about this disorder.  The disadvantage of people on the less affected end of the spectrum is that they 'know' they have this disorder.  Depression is a common, and probably logical, reaction.

When I began this blog in the weeks after her diagnosis, it was to be a place to process the emotions of having a special needs child.  Also, it was to be a chance for my friends to read about my child and her diagnosis so they could know what was going on with us without me having to discuss the not so uplifting details at BBQ's and family dinners.
I mentioned to some friends recently the posts I had read on FB, the numerous laments of people having lost most of their friends as their child grew, the challenges being too much.  Now, I have amazing friends, friends who have always been supportive and loving, and of course they assured me that it would never happen to us. And I believe them.  That is how I am made, to have hope.  But a small part of me wonders in trepidation about our future; my family's, Freya's.

See, I've done the research and listened to countless PWS stories.  I know what we are up against.

Saturday, February 4, 2017

Small Release and Big Love

     Just when I think I have adjusted to the emotions of having a child with special needs, when the therapies and daily injections have become such a part of our life that I no longer lament over their need; something, some nugget of compassion from a stranger, watching a video on the advances of PWS medical research, or a sudden visceral realization of a side of PWS that until then was just a checkpoint in an informational brochure, will crash into me.  Then the tears come.
     They are no longer sobs.  No, I am past that.  But the grief is still a part of my life and I realize at these times that it always will be.

It comes when watching her try to interact with peers and not succeeding as well as her little sister.  Drying her tears because, while she may not understand what is happening, she feels the sadness of being left out, of not being able to communicate well enough for the attention of a young child, because her attempt at inclusion is at times taking the toy they are playing with, or simply making a loud, repetitive whining sound not appropriate for a 5 year old.  My attention is easily diverted when we are in a room of children because I am listening, waiting to intervene because my daughter does not always play well with others, though I know she desperately wants to.

It comes when she makes known her overwhelming sense of injustice if she thinks her younger sister is getting more food than her.

It came when, because of bureaucracy and the inability of a staff worker to get the right paperwork to the right people, she was without her growth hormone shot for two weeks.  Five days into what I can only assume was a hormonal roller coaster, I held her while she sobbed like no 5 year old ever should.

It came when, in a state of feeling overwhelmed and looking forward to when free time does not require a babysitter or organizing it with my husband, I had the shocking realization that this freedom may never exist for me. That we will likely not be able to leave her alone.

It comes when she tries to talk to a stranger, or they her; the pregnant pause of them trying to understand what she is saying.  My heart breaks when her Dad and I can't understand and we either look to her little sister for translation, or she simply gives up under our repeated attempts to figure it out.
It comes as I evaluate her body composition, wondering if her belly is a little more bloated, if her thighs are a little bigger, if now is the time we will have to exert total dominion over every bite she takes.

It comes if I think too deeply on the fact that she can sit for hours coloring meticulously with a black pen, the sometimes too methodical way in which she reads a stack of books, the instant irritation if  someone ruffles the mat under her plate of food.

These all pass through my thoughts and yet, life goes on.  I have learned to tuck these thoughts away. I let them pass through without speaking them or giving them much notice.  But at times they must come out.  At times I must acknowledge that they exist, that this life with Freya has challenges.

Where there are challenges, there is also the deepest love and the sweetest successes.

Sometimes we are able to laugh at our misunderstanding of her words, turning it into a silly game of random sentences and laughs.

I watched in delight as she charmed every Guatemalan grandmother on the streets of San Pedro with an 'hola' and a wave.

I am uplifted by the excitement and joy with which she approaches life.  I laugh joyfully at her dance moves and the faces of hilarity she makes to give others laughter.

I indulge in the moments when she has a story to tell, one sometimes difficult sentence after another.

I revel in the absolute love and ease it is to be around her, to sit and color with her at the table or walk quietly amongst the trees.

My heart grows every time I hear her say, whether to me, her Dad or her sisters, "I love you".

Those words I always understand.      

Monday, September 8, 2014

The Naming

Here is something I wrote recently to share with people at our fundraiser for The Foundation for Prader-Willi Research.  Yes, we're all in now, fundraising and all.  I will write more about that later.  But for now:

Freya’s Beginning

I didn’t have time to be devastated.  My intended home birth turned into a ‘non-emergency’ cesarean in about 2 hours.  My fluid was very low and the baby was barely responding.   So we took some deep breaths and down to the OR we went.  
            She didn’t come out crying and, through my morphine haze, I could tell there was some concern.  But then my baby, wrapped snug in her hospital commissioned blanket, was lying on my arm next to me.  I spoke quietly to her and she opened her eyes for the first time. 

I held on to that. 

She may not have come fluidly into the world from between my legs to rest against my naked, intact belly.  I may not have used my hands to help her out of my body and into the world. I didn’t even get to feel my womb tighten around her little body as she resided safely inside.  But I was the first person she laid eyes on. 

And I held onto that.

I spent most of the next few days in the hospital just trying to feed my baby.  My first had nursed so well, but this one wouldn’t, or couldn’t nurse, and she just kept falling asleep.  I tried nipple shields, special spoons, finger feeding and syringes.  My milk came in and I thought she had finally caught on.  She gained some ounces and they let us go home.


My baby was so small…so quiet…so weak…so tired.  My oldest daughter would rub her feet while she attempted nursing to try and keep her awake.  My partner and I tried to understand the reasons; because of the cesarean she wasn’t in her body yet, perhaps my due date was wrong and she was younger than we had thought, maybe she just wasn’t getting enough food to begin with.  Every day she lost more weight. 

I held her, naked, to my chest, for most of the first few weeks.  I slept that way.  I was trying to feed her soul with my touch, to awaken her to this world.  She slept most of this time, as most newborns do.  But there were times that she slept so deeply it took moments of jostling to wake her.  I would wake in terror in the middle of the night; afraid she had passed with out my knowing.  The times when I was awake I could feel a subtle shift in her being, as if she was slowly just letting go.  Those were the scariest moments. 
The last moment I remember; I was home alone.  Baby was sleeping against my chest as I wandered around the house.  Then I could feel it; that slow retreat; the silent, backwards stepping out of a room so as not to disturb those in it.  I could feel her spirit trying to quietly remove itself from that body.

But I held on.

I sang to her.  I called to her.  I jostled and cajoled her.  I sobbed and pleaded and frantically paced the hall as each passing moment found her still asleep.  Finally, her little body stirred against mine and she fluttered open her eyes.  The day was November 1, Dia de los Muertos, Day of the Dead.  I decided then and there that my baby needed a name.  She needed a name to let her know that she was in this world now.  That she was wanted.  A name that I could call to her to bring her back if she tried again to leave.
I would not sleep until she had a name.  I could not sleep lest her spirit decide to take flight from her nameless body.  We spent hours trying on names but could not find one to fit.  We knew her middle name would be Jude, in honor of ‘Hey Jude’ having been played in the OR during her birth.  I fell asleep somewhat consoled, with Baby Jude on my chest. 
The next morning I woke feeling hopeful.  Baby Jude was still with us and had even lost her umbilical cord in the night; to my anxiety ridden consciousness it was a testament to her commitment in remaining with us.  That day she got her name. 

Freya.  The Norse Goddess of love, beauty, fertility, death and war who reigns over her own field in the afterlife, accepting those who do not go to Valhalla. 

Freya Jude…a Viking Goddess and a song of comfort.

Friday, January 3, 2014

A Deep Breath

     I just came through what I imagine to be a normal part of the 'process' of parenting a special needs child.  To say it was a minor period of depression would be a bit dramatic, although fairly accurate.  I can pinpoint various factors that could have contributed to this (ie post-pardum hormones, the 13th moon) but the result is what is important.  I've begun to see Freya in a new light.
     Over the past year I have, for the most part, felt very positive about Freya and her development.  I held what I thought were attainable expectations of her reaching her milestones.  I even thought that I coped quite well as each notch on my time line came and went without apparent success.  A few months ago I had begun to look through old pictures of when Freya was a baby, before diagnosis.  Without going into too much detail I'll just say that now it is obvious to me that there was something different (I cringe thinking of using the word 'wrong').  At the time I was able to convince myself of various things, other than there being something different, and no one corrected me.  While I truly understand why they wouldn't, it has not given me faith that when people say they think she is doing great they really mean it.  As my doubts about my own perception of my daughter were beginning to build something very meaningful to this whole process happened.  I met another PWS baby, 4 months younger, already doing what I had been working so hard for with Freya.  This baby was walking.
     In all fairness, 'they' say that the average age for walking with this disorder is 2 years and from the many families I've met on the internet that seems to be on the young side.  But here is where my mind went into guilt mode.  I wasn't doing enough therapy at home.  I should've gotten her braces for her ankles.  I should've started her on this supplement sooner.  Blah blah blah.  Many well intentioned people, myself included, pointed out that even in 'typically developing' children the age differences for reaching milestones can be drastic.  But all of the little 'defeats' of the past year seemed to catch up with me.          
     Now I could see the concern in the therapists eyes when she asked if Freya ever seemed interested in other kids.  Or the sound of shock in another therapists voice when I told him 2 specialists had said she seemed to be on the less affected end of the PWS spectrum.  I began to notice that perhaps her attention and desire for playing with a basket and a pile of multi-colored string was more involved than just her inability to walk and do something more interesting.  And maybe the fact that she use to say baby but couldn't seem to anymore was more serious than I wanted to believe.  All of this began to build over a matter of weeks.
     I wish that, in order for a more interesting and orderly story, there was some momentous occasion to turn the tides.  But in reality it was in the middle of cooking dinner and silently watching my happy non-toddling toddler play with her basket of string that I realized, I mean, really realized, that Freya is not 'typically developing' and probably never will be.  All of this time, through all of these therapies with their damn questionnaires about what milestones they're reaching at what age had me in the wrong mind set.  I had been thinking of Freya as a child who is delayed.  And if we work hard enough, and do the right therapies and give her the right supplements then some day she'll catch up to her peers and they can all go skipping down the road of 'typical development' together.  In that moment in my kitchen, with the oil snapping in the hot pan and Billie Holiday playing in the background and Freya diligently taking one string out of her basket at a time to add to her neat pile of more string, I realized that Freya is a non-typically developing special needs child.  And I breathed in deeply from the weight that was lifted off of my shoulders.
     Perhaps this sounds like defeat, or giving-up.  On the contrary, it is acceptance and unconditional love.  And it is full of hope.
     We aren't doing any thing differently.  She still goes to therapy and gets her supplements.  I still envision her being able to have a full life when she is older, one where she can fully function in normal society and be happy.  I honestly don't believe that cognitively she is unaware of anything.  Freya is incredibly social and loving.  She is freaking hilarious and is saying new words often.  She has started taking a few wobbly steps on her own.  Amongst all of this I can also see some behaviors (and even some medical issues) that are apparently typical for PWS which is a whole other part of the 'process'.
     But I'm not going to get upset the next time a therapist pulls out one of the obligatory timeline questionnaires.   I'm not going to stress whether she can put the red ball in the red cup or how good she is at pointing.  I'm going to do all of these therapies with her and more.  But I'm not going to think of her as delayed.  I'm going to follow the lead of her Dad and believe in her path and her timing and her ability.
     I was talking about this with a friend on Christmas.  Her words brought such joy to my heart that I have been thinking of them often.  She expressed how loving Freya is and how she has this unique ability to make people feel so special.  Because of this, she pointed out, Freya will always have people on her side routing for her.
     I love how she phrased that, "the ability to make people feel so special'.  We may not be able to check all of the boxes on her development evaluations, but I think this quality will help get her farther in life...at least where it counts.

Friday, May 31, 2013

Life After Diagnosis


     Over a year ago we received the devastating news of Freya's diagnosis.  I remember the call on Friday evening from her pediatrician, then the sobbing on my 7 year olds shoulder because she was the first to come in the room after the phone call.  I feel as if I should say, 'if I had known then what I know now then I wouldn't have been as upset".  But that is impossible to say because back then I had no experience dealing with consistent doctor appt., therapies, daily injections.  It all sounded so daunting and terrifying.  But now, rather than look ahead and fear it, I live it.  And the living, at least so far, isn't nearly as terrifying as the fearing it.  We have a much better understanding of this disorder and some great people in our corner.  We've experienced the fear and have come out of it thinking, "ehh, that wasn't so bad."  Of course there are potentially many difficult things to come but we've learned to live the cliche of 'one-day at a time' when it comes to Freya.  There's no guarantee she will be one way or the other.  Freya has the sweetest personality of anyone I have ever met.  She is very aware, social, fun, and, I think, fairly intelligent.  Her biggest obstacle is her low-muscle tone.  She is 19 months and not yet crawling on her hands and knees consistently, though she does a great combat crawl.  When I help her position her feet properly she can now pull herself to standing.  She has many signs and makes lots of different sounds.  She loves music, animals and her sisters (the newest is 2 1/2 months).  Freya is a delight to be around and has brought us so much laughter.
     If someone had told me a year ago that I would be able to say all of these things, I might not have believed them.  But now I know.  There are of course frustrations, disappointments and a low-grade fear that I believe any parent of a special-needs child carries; but overall we have learned to adapt.  There was a phrase kicking around in my head when I began this post:  Hope is what we're riding on.  We'll deal with reality when it comes.  

Wednesday, October 10, 2012

Life on Growth Hormone

   It's been about 2 1/2 weeks since we started Freya on her growth hormone.  We've had some struggles, as one can imagine an inexperienced person having to give their first shot ever to their infant.  My hands were shaking as I pinched a section of fat on Freya's thigh and pushed the needle into her soft baby flesh.  True to her sweet-spirited nature she barely flinched and didn't seem to notice.  She hasn't even held it against me the times that she has moved her leg and I've bruised her; or like the other night, made a half an inch long scratch on her leg from the needle.  It's times like these that I can't believe I'm allowed to do this; that parents all over are doing this.  But usually the shots are uneventful and I can finally relax when I put the pen device back in the fridge for the night.  
  Freya was already doing quite well, for a baby with her disorder, before she began these shots.  But I have seen a few things in the last week that has inspired me to believe more in their effectiveness.  Freya just feels sturdier, more solid.  She can sit for 20 minutes at a time now without falling over.  She is even putting weight on her feet and beginning to learn how to stand.  We've had some issues with increased heart rate that I have learned not to be too alarmed about.  Her therapists are all impressed with her and the big victory was today, seeing her mark on the growth chart and not below it.  
   For most of my adult life I have believed whole heartedly in more traditional, natural ways of healing involving diet, herbal medicines, acupuncture and things like meditation and visualization.  Of course none of my recent experiences have changed how I feel about those methods of healing.  But I've often discounted or argued against Western Medicine.  To be honest, I still have many of the same feelings about that also.  But I have learned to have a new found respect for some aspects of Western Medicine. In all fairness, Freya may not have even survived her birth without it.  And though we are potentially looking at a life time of struggles with her disorder, I cannot imagine never having met this wonderful being or looking forward to watching her grow and become the person she is.  So, in a rare moment of gratitude for an institution I think needs a lot of revising, thank you Western Medicine, for my daughters life and your ability to help her thrive.

Monday, August 27, 2012


     Well, it has been awhile and not much has happened, medically speaking, with Freya.  But those days are coming to a close.  In a few days we travel south to San Fran to finally get her BiPap mask so that we can, finally, start her on the growth hormone.  I'll write more about that once we get situated.
     These last few months have been great.  Freya has grown and is developing a sweet, fun personality.  She smiles and laughs often and is very pleasant to be around.  I love watching her facial expressions as she visually explores the world around her.  She is sitting up (though needs someone nearby because when she goes, she goes) and shows a lot of interest in her toys.  All of these things bring us great joy.  And on top of all of this we've had great news from the doctors.
     A few months ago we met with our SF Geneticist who has been specializing in PWS for the last 30 years.  After spending some time with Freya she told us that Freya was in the 'above average' end of the spectrum.  Of course this was great news for us.  But the best news came from a specialist in Florida.
     While visiting family we took Freya to a Pediatric Endocrinologist who specializes in PWS.  She has people coming from around the world to see her.  When I suggested us seeing her to our Geneticist, she emphatically approved.  So we took Freya to see this doctor and got the best news we've gotten since this whole thing began.  To begin with, she was very impressed with Freya and her muscle tone, saying that she seemed like a baby already on growth hormone.  Then she spent the rest of the hour telling us of all the hopeful things they are learning about this disorder.  As she put it, 'If you're going to have a baby with PWS, now is the time'.   There are medicines in use that are proving to be effective in controlling the hyperphagia (the excessive eating) and 20 more in research.  She said she could not only offer us hope, but lots of hope, that Freya will have a normal life.  We couldn't ask for more!

Friday, June 8, 2012

Baby Steps

When I titled this post 'Baby Steps', I wasn't thinking of the the seemingly minor successes of Freya into 'normal' babyhood.  Instead, I'm referring to my baby steps into the world of raising a special needs baby, specifically one with PWS.
I'm noticing a pattern; read through the pamphlet, medical alert brochure or the posts from other parents in the online support group, feel the heaviness of grief descend, shed a few tears, try to push past it and resolve to not read or research for a bit.  Perhaps this could be considered a form of denial.  I like to think of it as self-regulating.  I can only handle so much information at once. I can only learn about one or two potential symptoms at a time or read that in families with a PW kid the rates of divorces and siblings with emotional issues are high before I want to throw out all of the information.  Throw it all out so we can return to living in our world where, with all of our love, some special attention to certain things, and our great support network, we can all live a fairly normal life.
Have I told you Freya is going to be the poster child for PWS?  She's going to be the newest success story of the syndrome, you'll hardly know she has it.  She may even be the first PW person to circumnavigate the globe on a sail boat.  This is what we are telling ourselves.  It feels good and healthy to be positive about her future.  But every time I read more into the disorder, the probable reality becomes clearer, and scarier.  At times like this I remind myself of what one of our local doctors so easily handed to us when we asked if he could predict her future cognition.  "Cognition isn't everything.  There are people with 130 IQ living under bridges.  What's more important is that she's happy."  And for now, she is a happy baby.  Baby steps.

Thursday, April 19, 2012

This Too Shall Pass

     I've spent the past few weeks in a 'head in the sand' kind of mode.  We got the news that Freya has moderate to severe sleep apnea.  This spiraled me into a bit of a depression; the knowledge that she has to wear a mask to sleep now. This felt like a turning point of some kind.  Up until then she was doing very well on the spectrum of symptoms; cognitively she's very aware and social, laughing, smiling, holding her head up and her weight is gaining steadily without the use of a feeding tube.  So I had hopes that we could breeze through this sleep study without any negative results.  But alas, her results were like every other infant with PWS that the pulmonologist has seen.
     One of my concerns was for our way of life.  We plan to spend the summer by the river in a yurt, with no power.  We like to travel and camp where there is no electricity.  Now, not only was I going to have to strap a mask to my baby's sweet little face when she's sleeping, but also be attached to an outlet indefinitely.  This was the first time I cried in front of my partner since the diagnosis.  
     Thankfully I am more hopeful now than I was before.  I've learned from friends who have worked with disabled adults that, if they start the mask at a young age, it just becomes part of their routine.  There are also battery packs for the masks.  And it's fairly simple to hook up a small solar power set-up (simple for my partner, unfathomable to me).  So there are options.  
     On a journey such as this, it is important to remember there will be light.  So much of the news seems unbearable at first; a heavy, dark cloud blotting out any ray of sunshine.   The unknowable is scary, but perhaps what's worse are the images conjured up by reading about every possible symptom for this disorder, every obstacle we could potentially meet along the way.  Three months ago a diagnosis of PWS seemed the worst possible outcome (of the disorders they were testing her for).  Today, it is bearable.
     I think the old saying, 'this too shall pass' is a wise sentiment to remind myself of.  And of course I don't mean the symptoms.  But the feeling of being overwhelmed with a new one, the feeling of 'how will we get through this?'.  Because, let's face it, we get through it.  What are the other options?  Life goes on and eventually, if we are open to it, we find contentment, acceptance, and most importantly, laughter.

Saturday, March 31, 2012

To Pump or Not To Pump

     As with a lot of things in life, there are ups and downs.  These first few months have been full of them. Some days I feel very hopeful and positive, as if we are bigger then this diagnosis.  Other days it's hard to pull my feet out of the muck and take a step forward, the diagnosis is too big for me to carry.  Lately my feet have felt fairly stuck.
     My pumping days are numbered.  My theory is that after nursing my first daughter for 2 1/2 years my body is not fooled by the pump.  It's just not the same sensation.  But each day I wake up, take my herbs and pump 4-5 times.  At this point Freya is getting about half breast milk and half formula, and that's a good day.  I know from the core of my being that breast milk is the best for my baby.  But I can't help but wonder, as I look at Freya laying next to me while I strain to pump out an ounce and a half, at what point is it not worth it.  Quite often, as the pumps are rhythmically pumping away, Freya and I are left just looking at each other and I'm thinking of all the other things we could be doing; snuggling, playing, going for a walk or some physical therapy.  The actual time I spend pumping is almost 2 hours a day, and that's not including cleaning the pump parts or mixing the right amount of formula with the breast milk.  I just can't help but wonder sometimes if the time would be better spent in a different, more stimulating activity.  I didn't get a drop of breast milk and I've turned out ok.  Other times I snuggle her little 10 1/2 lb. body and wonder how I could leave her immune system defenseless or cut off the last physical connection between us.  So what if it takes me a total of a half an hour to get 1 1/2 oz. of milk, every little bit counts right?
     I'm trying a different herb blend and am being more careful about what goes into my body.  Sometimes I try to put Freya on my breast, but there's only so many times you can watch your baby make a disgusted face and pull away from you.  For now, I'm hanging in there with the pumping.  For now, the benefits out weigh the cost, or so I think.

We got the results from her sleep study.  That's a whole other issue, and another post.