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.

Hope

     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!

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.

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.

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.

Hindsight

      When I began this blog I referred to each new week of my pregnancy as small victories.  I said they were like small victories because this pregnancy was fraught with worry.  I didn’t worry with my first pregnancy but everyone told me it was normal, 'pregnancy is a state of worry'.
     Around 30 weeks we figured out the baby was breech, and thus began my final worry.  We were planning a home birth but the midwives' policy was no breeches and none of the local hospitals would perform a breech vaginally.  So for weeks I talked to my baby about getting into position, even made up a song to sing her.  I took homeopathics, performed various stunts of inversion acrobatics including an intense yoga position involving my very pregnant body upside down against the wall.  We kept a board propped against the couch in the living room so 3 times a day I could lay upside down for 20 minutes.  I tried ice on the top of my belly, acupuncture, moxibustion and a trip to the chiropractor.  I researched turning a breech baby and found a statistic saying that 3-4% of breeches are from a congenital malformation.  I hid this information away, hoping to discard it for good.  But as the weeks continued and my baby didn’t turn it crept out more and more.

     Even when Freya was born via cesarean and couldn't nurse in the beginning, I let myself be talked into thinking that she was just a bit earlier than we had thought.  I believed that she wasn't totally in this world yet and needed some time.  I planned a rebirth ceremony and cranial-sacral therapy for her actual due date a few weeks later.
     But those first few weeks were terrifying.  She was such a quiet, still baby and there were times, as I held her skin-to-skin against my chest, that I could almost feel her slipping away.  I would be going about my business (which in the first few weeks of post-cesarean wasn't much) and suddenly Freya would feel distant, almost lighter.  I would, probably too roughly, pull her away and speak loudly to her, pleading with her to wake up, please wake up.  Often times it would be long, intense moments before she would even stir.
     The night of Dia de los Muertos I sat up with Andy for a few hours trying to find a name for her.  I had convinced myself that she needed a name to help keep her in this world.  We could only come up with a middle, Jude.  I fell asleep that night, Baby Jude on my chest, knowing that I needed to see her through this night, that I had to fight for her as I had been fighting for her from the beginning of this pregnancy.  Perhaps it was my hormones, but fear is a powerful emotion.  The next morning I woke to my baby who had stayed with me through the night, her umbilical cord finally fell off and we named her Freya, after the Viking Goddess.  
     I felt safer knowing that Freya had a name, but was still afraid that I would, at some random moment, notice that she was no longer with us.  These were awful, indescribable feelings.  Then one night, about 2 weeks later, my midwife came over and said we needed to talk, that they were concerned.  Through my tears, and the thought that I had known this all along, I asked if she thought that Freya wasn't going to make it.  The look of surprise told me what I need to know.  "No," she said, "if I thought that I wouldn't be going home at night."
     And that was enough.  My baby was not on the verge of leaving me, and that made anything else seem absolutely bearable.  I finally had my baby and could enjoy her.  It was months before we got an actual diagnosis and there was more grief.  However, as I said in my first blog entry, I couldn't believe what people said about these first few months being the hardest.  I saw the future as a nightmare of health issues, shots, behavior problems and doctor visits.  Not too mention the emotional turmoil that goes along with all of those.  And I am only 5 weeks into the 'first few months are the hardest' phase so I won't act as if I still don't question so much of this.  But I have already begun to hope again and can see on the horizon a future of acceptance and understanding.  
       Perhaps it is that these first few months are spent questioning this experience.  The what's, the how's and especially the why's. But on that horizon I can see a time when we get past the questioning and trust in ourselves and this experience.  Each day I try to take another step forward.

Overwhelmed

          We have begun the myriad of doctor's appointments for Freya's PWS.  Three days in San Francisco for a total of 2 hours in doctor appointments; long enough for them to tell us it will be 5 months before she can begin the growth hormone treatment.  I could go into the details of why we have to wait (approval from insurance) and what hurdles need to be jumped (the necessity of a sleep study) in order for this very effective treatment to happen.  But honestly, that is not why I am writing this blog.  I am writing this blog because I am frustrated and confused.  I feel humbled and overwhelmed navigating these twists and turns of the bureaucracy of Western Medicine.  Many parents I am in contact with already have their babies on GH.  They are in physical and occupational therapy. They also travel from far and wide to see a special doctor in Florida.  Should we be doing more?   Should I have thrown a fit when the Regional Center said it would be a month before she could be evaluated for her therapies?  Should I have demanded they get her on the GH faster?  What can I do?
     There is part of me that believes, with our sincere devotion and dedication to our daughter and her needs, we can successfully handle her syndrome.  On these days I feel hopeful.  But the evidence is there.  Growth hormones have a strong, positive affect on people with PW. And the doctors, who agree it is the best treatment, can do no better than 5 months.  So today I feel stuck; and confused, worried that I am missing something or not fighting hard enough.  Today I question my ability in being a successful advocate for Freya.
     But I remind myself Freya is only 4 months old.  Once she begins GH treatment she will be getting a shot every night.  And, we found out, she will most likely have to wear a mask when she sleeps.  For now she is a healthy, happy, albeit small and weak, baby.  But she is improving and growing at her pace.  She has even begun to laugh.  Perhaps it is a blessing that she will not be whisked too quickly into a world of needles and machines.

Pump, Pump, Feed...

     My life these days is centered around pumping and feeding.  If I'm not pumping, or feeding, I'm cleaning pump parts or counting time until Freya's next feeding and homeschooling our 7 year old.  In some ways it may seem pleasant to have a baby that doesn't cry or fuss.  Well, I guess I would say its the consolation prize.  My baby doesn't cry and fuss and cause a huge distraction.  She doesn't wake up every 15 minutes to squirm and cry for milk.  My first one was a bit more like that, and it is hard, very hard.  But there is a lot of pressure on a parent who's baby does not eat of her own free will.  I set the alarm at night so I can wake up and feed her.  The plus side is that I am assured sleep in 4 hours doses.  The down side is that occasionally, when I've forgotten to turn the alarm on or turned it off in my sleep, I wake up frantic because it has been 6 hours since she has eaten.  I jolt awake to find Freya sleeping peacefully by my side.  I rush to get a bottle prepared and warmed as I berate myself for being irresponsible.  But I am human.  And while a 'normal' 4 month old can (if only we were all so lucky) sleep for 6 hour stretches without eating,  it's yet another obstacle to the growth of my already small baby.  Thankfully these moments are few and far between, but racked with guilt none the less.
     There are times when Freya seems to be hungry and enjoying her bottle, when she sucks it down in 20  minutes.  These moments are a blessing.  There are times when she is too sleepy and getting her to eat is   almost impossible.  Sometimes the milk backs up in her throat without notice and she coughs and chokes and can't breathe.  These moments are terrifying.   Sometimes I struggle to get the last of her bottle into her, only to have her spit it back up.  My life is ruled by Freya's feeding schedule, which unfortunately she couldn't care less about.  But it's what parents do.  We alter our lives for those of our children.  We make sacrifices so that they may have the things they need.   Our children's needs may seem unbearable at times; the crying, the whining, the struggle to feed.  But for every moment when we feel as if we can't continue with this path, can't keep up with these struggles, there are countless other moments when our lives are so enriched by these little people we can't, nor care to, remember what life was like with out them.  So, while my time is not my own these days, I look with joy on this being that has entered our lives.  We are trying to prepare ourselves for the struggles we will face with her, but all the while revel in the joy and happiness she has and will continue to bring.

Irony

     Irony, an outcome of events contrary to what was, or might have been, expected.    
     It is a sincere form of irony that most PWS infants are first diagnosed with failure to thrive.  They need interventions like feeding tubes and bottles to help them gain weight.  In many cases it is because of the hypotonia that gives them a poor ability to suck.  Freya is still classified as hypotonic but has always had a strong suck.  Her issue seems to be tiredness and many times a total lack of interest in her food.  It has gotten a bit easier to feed her but still, far too often, she just doesn't seem to want food.  The common phrase heard around my house, when someone is frustrated from her lack of interest, "well, she'll want it soon enough."  Why?  Why is it that this baby can be so uninterested in food now, yet someday that will potentially be her driving force?  We will spend her infancy struggling to keep her weight gain up until, one day, (and I heard that some parents have noted to the day when the extreme hunger kicks in) we will be fighting the opposite battle.  That, my friends, is irony.
     Life is full of ironies.  I could, in proper Alanis Morissette fashion, write a list of ironies in my life and of those around me.  I'll spare you.    But I do often wonder, as I struggle for almost an hour to get 3 oz. of milk into Freya, is there a purpose to this irony, to any of the ironies of life?  Are these ironies mere coincidencies given meaning to by our unending quest to understand our world; or are they some cosmic regulation of what we believe our reality to be, lessons per se that the universe thinks we need to learn?

4 Months

     Freya is 4 months old today.  I haven't come up with a good response for the endless comments of how new my baby must be.  When I tell people how old she is they inevitably ask me how much she weighed at birth.  The 6 lbs. 4 oz. response stumps them every time.  I've begun telling these well-meaning strangers that she has a genetic disorder, which gets about the same reaction as the unscripted reply of "not so good' to the cultural norm and nicety of "how are you?".
     I've given away my copy of "What to Expect in the First Year" and have learned to really appreciate the things she does.  She has been smiling for a few months now.  In the beginning they were, of course, gas smiles.  But I remember the first real smile I saw on her face.  Haven, her older sister, was trying to feed her (one of the benefits of bottle feeding), gave up (the irony is that PW infants can't or don't eat well) and walked away.  I watched Freya crane her little weak neck around to try and find her sister.  I asked Haven to come back and when Freya saw her she gave her a beautiful, open-mouthed, ear to ear smile.  Since then the smiles have been frequent and sometimes she even lets loose with a weak, cooing like giggle.  She has a few toys that rarely fail to elicit a smile. One is a bright red, mini feather duster.  This toy is also great for her tracking as she will follow it wherever we move it to.  Another is Andy's Yurok hat, a black and blue knitted cap with a traditional design.  I think learning the meaning of the design will give us a better understanding as to why she likes it so much, though Andy isn't convinced it's anything other than the colors.

Surrender

     I learned with my first daughter that becoming a mother involves the ability to surrender.  It begins first with surrendering your body to the growing of a new being, surrendering to the earth shattering pains of child birth, surrendering sleep and other basic necessities.  I thought that this time around I would have the surrendering part down.  But Freya has taught me a new kind of surrender.
     My oldest daughter's birth was one of the most empowering of my life.  I birthed her at home, in the water, with my and my partners own hands.  She nursed right away and continued to do so for 2 and a half years.  When she gets growing pains I rub her leg with homemade herb oil.  When she gets a fever I give her a warm bath and herbal tea from the garden.  I was ready to do this all over again.
     When I walked into the birth center for the last resort external version to turn my breech baby my first thought was, "I can't have my baby here".   That was the first surrender.  A few hours later I was laying in a hospital bed, hooked up to an IV of pain medicine and a catheter, holding my newborn that had been brought into the world via the gloved hands of doctors and surgical equipment.

The Beginning

     When I was pregnant, Fridays were the day I tracked my progress, the magical day when I would be transported from one week of new growths and developments to the next.  On these mornings I would read to my older daughter from the different Internet sites that compared my baby’s size to a variety of fruits and introduced her to new words like lanugo and amniotic.  Every week was like a small victory, one more week that the baby had survived, one more week closer to another perfect home birth.  

     It was a Friday night in January when I got the call from our pediatrician.  Now Fridays are the days I mark off from when I got the diagnosis, as if each new week will bring some thing new to my processing.  They say the first few months after diagnosis are the hardest.  I have a hard time believing this.  These first few months she’s a sweet, helpless baby who smiles fairly often and never cries.  These are not the months or years when she may be crying because she’s hungry, or finding a way to manipulate someone into giving her food.  These first few months I am not giving her a shot every night or posting a schedule of our menus and meal times.  These first few months I am not fearfully waiting any sign of extreme hunger.  These first few months it is not foreseeable how low her intelligence may be.   For now I can imagine her life with Prader-Willi as a success story, her as one of the ‘lucky’ ones who has a normal intelligence and, with a routine, can live a fairly normal life.

     This blog is about the journey we have begun, the emotional side of raising a 'special needs' baby, and the joy of her developmental achievements as they come.