Today's version of a daily conversation

Ruby: Mom, I'm really hungry.

Me: I know sweetie. But you just ate lunch. (about 1/2 hour ago.)

Ruby: Guess what!? We made pizza for snack at school! Not like pizza store pizza, but with a circle kind of bread. I put cheese, then sauce, then more cheese and more sauce. I made two layers!

Me: That sounds good. (calm but mentally berating myself for packing big lunches when they eat these high calorie snacks at preschool)

Ruby: And you know, we also went to the "blue room" and ate honey. I got two pretzels with honey. Not everyone got two, but I did.

Me: Hmmm. (acting neutral)

Ruby: Where are we going now?

Me: Just to the grocery store to get some things for dinner.

Ruby: What's for dinner?

Me: I think we'll have salad and bread.

Ruby: I LOVE bread. Can we have bread with thick cheese spread all over?

Me: No, not tonight, just regular bread with our salad.

Ruby: What's in the salad?

Me: Roo! (I lost my patience) Dinner is still 5 hours away! (I take a deep breath.) You don't need to worry about dinner, sweetie. I'm the mom and I can take care of the food.

Ruby: Well, when I'm a mom and I have babies I'm gonna...

Me: Gonna what, sweetie? (I'm calm again)

Ruby: I'm gonna live far away. (but she's not calm. she's hurt) I'm gonna live next to GrandMary and Grandpa (in NJ).

Me: Oh, ok. (i'm striving for unfazed)

Ruby: I'm not going to live near you.

Me: Hmmmm. (I heard somewhere that saying "Hmmm" a lot keeps kids talking)

Ruby: Can you drive me there?

At that point I burst out laughing. And poor Roo was so mad, so ashamed. She hid her face in her sleeve and wouldn't look at me. I told her I was laughing because when Daddy and I drove from NJ to CA it took us five full days. (It did take us 5 days, but of course that's not what was funny) She knew I was lying. Now I felt bad.

Ruby is always talking about food. I don't know if she is really experiencing hunger all the time or if she is just tuned into the PWS food vibe in our house. Did she learn to drink her salad dressing by copying Oscar or is she also programmed to do that?

I didn't think too much of Ruby's voracious appetite until two years ago when her linear growth slowed and her weight increased. She went from 75% height/50% weight to 35% height/95% weight. And she's stayed right around there..despite a lot of very healthy portion-controlled eating and not much access to dessert or sweets.

The hunger plus the slowed growth and metabolism just sound too much like PWS to me (it's not, we checked) and therefore gets me at my weakest place. All those horrible stories that I read in the NICU when Oscar was born about kids with PWS and the lengths they will go to for food come rushing back.

The literature made kids with PWS seem like monsters.

Eating frozen meat, digging through garbage cans for rotten leftovers, prostituting to earn money for food...

I know differently now, but I can't quite shake the images or the fears. So when Ruby perseverates about food more than her brother who actually has PWS, and we don't know why, I feel the uncertainty and fear rush in again.

I was researching her metabolism and appetite last February when one of her top baby teeth became loose. She'd already lost the bottom two. I asked the dentist if she knew of anything that could cause both premature tooth loss and metabolic issues. That's when we learned about hypophosphatasia -- we discovered the HPP while looking for something to explain her hunger and metabolism. The symptoms aren't, as it turns out, related. HPP might help explain the slowed linear growth, and certainly the lost teeth, but most kids with HPP are super picky eaters and parents in the yahoo group share ideas about how to help their kids gain weight.

Ironically, because we have a child with PWS we think we know what to do about Ruby's obsessive hunger....and yet we really don't. Ruby will live independently in the world and make her own choices about food someday. Oscar will never be in control of his food. As well as he is doing, uncontrolled access to food will put him at serious medical risk and only heighten his anxiety and behavioral challenges. But that's not the case for Ruby. At least I hope not.

So how do I raise her in this Prader-Willi household? I'm concerned about the food issues she might develop because I am restricting her now. But at four she is too young to make all the decisions.

And my gut tells me there is an imbalance of some sort causing her hunger but I don't know how to chase it down.

Meanwhile, I am saying "yes" whenever I can. I sometimes give her small treats when Oscar isn't around. I'm trying to be patient. And I'm always talking about how all of our bodies are unique and we all need different things. "Oscar's body doesn't know when he is full so I help him. Your body can tell you when you are full. You don't need to finish your food if you are full. We can save it for later." I congratulate her on good choices.

I'm doing the best I can but I wish there was someone out there that could help me navigate this tricky area.

At bedtime tonight she was cuddly and asking for lots of hugs and kisses and trying to keep me in her room as long as possible. We play the "Guess How Much I Love You" game a lot, each of us trying to outdo the other. Tonight I went with silly and said "I love you all the armpits in the world". (I know, that doesn't make sense, but we giggled hysterically anyway).

And then she said, "I love you all the way to GrandMary and Grandpa's...and back".

In these moments, the concern melts away and, with love as my guide, I trust that she will be okay.

More St. Louis...

We're home now and resettling into daily life. I am still processing the week in St. Louis, but there is a lot to share. We were released from Shriner's on Thursday, a full day early because they managed to get all of the tests, evaluations and meetings completed ahead of schedule. Wednesday and Thursday were especially busy and it took me a whole 24 hours to recover from the pace and activity of the preceding 4 days.

That's ok...we got a lot of information. In short, Oscar and Ruby have mild forms of the disorder. Ruby is the most affected, but they still expect her course to be mild. And, surprisingly, Abe is unaffected. I'm hesitant to believe this without definitive genetic testing (that they are refusing to provide) but I'm working on it. It is good news after all!

For now, we need to monitor Oscar and Ruby's calcium intake as both of their urine calcium numbers came back high. Ruby's especially was way out of range. (And of course I don't know the exact number). So in addition to all we do for Prader-Willi syndrome we also need to watch calcium and VitD. I can do it, but I am admittedly feeling a little annoyed and frustrated that I have more food monitoring on my plate.

The psychologist who consulted with me about Ruby had some excellent insights. I feel like I now have some understanding and direction about Ruby's personality and behavior. Wasn't it just a few days ago that I said I wasn't sure I'd ever trust anyone to give me guidance? Well, I was wrong. I have more clarity, and importantly, peace about her intensity and frustration. I think Ruby senses it too --she has been more articulate and calm these past few days.

Apparently Oscar's scoliosis is at a point that the spine specialist (who I did not meet) recommends that we STOP using his brace. He doesn't need that, or surgery. An interesting outcome that I wasn't expecting and, again, don't fully trust having not met the specialist, seen the xrays or even heard the exact degree of his current curve. I'm waiting for the actual x-ray and analysis to arrive in the mail before I do anything drastic, like pop open a bottle of champagne...or even take off the brace. I am, however, cautiously optimistic. I'd been feeling horribly guilty that I'd missed his 6 month check up by 8 months and counting. I may have dodged a bullet there.

All three kids had OT and PT evaluations, which I found fascinating. (Only a geek like me would actually enjoy this!) Of course I didn't learn too much about Oscar that I didn't already know, but he is a bit stronger and more coordinated than I thought. Ruby is far more advanced in her fine motor skills than even I suspected but has some weakness that we can work on. Abe impressed the OT with his creativity, focus, and diligence but, according to the PT, he desperately needs to stretch! They all have things to work on that we will fit into 10 minute increments twice a day. (add it to the list....)

The two meetings with the research doctors went reasonably well. I asked lots of specific questions and pushed for clarification, some of which I received. I'm just that way -- I'm interested in numbers and facts. I like to see it all, and they were hesitant to share the data. This was the hardest part for me. While I trust their intentions and commitment to helping kids with this disorder, I really need to be more of a partner in my children's care. I need to be able to pass information to future doctors, I need to compare to previous and future tests. I've signed releases so ultimately I will receive all the nitty-gritty details once they do become available.

I did, however, get them to x-ray Abe's arm to make sure it was fully healed. Certainly he is functioning fine but since one number was elevated compared to previous blood draws I wanted them to check. And I also got them to agree to re-draw Ruby's blood. The second draw was far more successful, thankfully. Ruby was calmer and more comfortable and they got a lot more blood. I am grateful, as a couple of my important questions relied on that blood.

So, on to the fun! We raced from the hospital to Busch Stadium in downtown St. Louis where the Cardinals were facing the Mets at 12:40pm. With its pedestrian-friendly downtown location and brick construction, the stadium is reminiscent of AT&T Park in San Francisco, where the Giants play. We were thrilled to be outside watching a great game on a warm sunny day. I taught Oscar how to keep track of the count on the scoreboard, Abe took tons of pictures, and even Ruby watched a little. We went wild went Albert Pujols hit his first, then second, home runs of the day!


On Friday we enjoyed a riverboat ride on the Mississippi and then headed over to the Arch. Abe and Paul shot photos of the elegantly designed steel structure from all sorts of interesting angles and positions. We learned more about the history of St. Louis, and in the bookstore Abe chose a geography book for kids.We then crammed into a tiny tram and rode to the top. Once there, Ruby scampered up the ramped windowsill to peek out the small rectangular windows and exclaimed "I can see the whole world!" She was fascinated. We spent quite a while up there pointing out landmarks, pools on rooftops, tiny moving dots we knew were people, and of course Busch Stadium. I started to get queasy when the Arch swayed. Its engineered to do that of course, but I didn't find that very reassuring at 600+ feet in the air.
We managed to squeeze in some time at the pool as well as some much needed naps in the luxuriously comfortable hotel we reserved at a great deal on priceline. The sightseeing and comfy hotel was the perfect way to end our trip...a way to relax and regain some balance.

We're back in Berkeley now. A full weekend of music performances and baseball games awaited us. St. Louis was a good experience though, and one I hope we won't soon forget. I've already hatched a plan for the kids to "pay it forward", so to speak.

After every blood draw each kid got to choose one item from a well-stocked toy closet. They also received toys and beanie babies for x-rays, pulmonary function tests, pictures, and just for coming. Bingo night was a bonanza with each kid scoring two items. We are going home with 2 Cardinals hats, 1 Cardinal's t-shirt, 2 dolls, 5 beanie babies, 6 plastic safari animals, a puzzle, an etch-a-sketch, a nerf hoop, and a wooden peg game.

While we certainly don't "need" all this loot, it really did help the kids through the hard parts, and made them feel good about their participation in the study. Even Abe is treasuring his treasures. So, we're going to start collecting new and unused toys and books for our local Children's Hospital. We have some things stored away that we can start with, and we'll ask our friends too. We may even solicit our local toy stores for donations. The kids are excited about the idea -- they know how much those small items meant to them.

So, overall, it was a really good trip. And we don't need to go back for another 3 years. The kids can't wait...seriously.

a lovely letter

We are back from St. Louis. I am still processing the visit and am in the middle of a post about the rest of our hospital stay that I'll probably finish long after you all remember we were gone at all. Life just picked right back up the moment we landed at SFO with music performances at school, dinner with friends, a baseball game and practices. It will take me a while to find the space to sort out St. Louis in my head.

Meanwhile, life goes on. Today, while I was busily emailing vitamin and supplement labels to Shriners (so they could see just how much calcium, Vit D and B6 the kids are getting), signing Ruby up for soccer, throwing in loads of laundry, and not hauling the 8 newly arrived boxes of Walking for Prader-Willi Syndrome t-shirts down to the basement ...a letter arrived in the mail.

It's a letter for Oscar, with a return address of Haven House, the family housing for people who have children in St. Louis area hospitals. We stayed there one night before we checked into the hospital. Yes I opened it. I could say that I needed to make sure it was appropriate or that with his lack of manual dexterity he couldn't have opened it anyway. But really I was just curious.

The letter was actually written simply to "O.H." and was from "A.", a 10 year old student at a local elementary school. A. had received Oscar's initials from her teacher and was writing to tell him about herself and to wish him well during his stay in the hospital.

Dear OH
My name is A.
I am 10 1/2 years old.
I Love to sing.
I Love animals.
I Love to pick up trash in the woods and plant trees and flowers.
I Love to ride my bike.
If I could I would come and visit you.
I have one Brother and no sisters.
I have two crabs for pets.
There names are
Shotting Star and Wacher!
Get Well Soon,
A.

Oscar will love this letter. If he were to write a letter to a child in the hospital it would sound a lot like this. We don't have crabs but we do have a tadpole named Elisabeth. He loves animals and singing and gardening.

The sweetness of the gesture and the words caught my attention in the middle of my "get organized" morning and I felt a tear escape. I am still processing. My kids are fine. They are going to be just fine. But they were in the hospital and we do need to watch some things. It is real, and yet it is not so bad. It is gray, like so much of our life. So much typical stuff intertwined with unending medical appointments and tests. I feel like I am always straddling two worlds not truly belonging anywhere.

My first reaction was that this letter should have gone to someone else, to another child or another family who really needed it. And then I realized...it went to the exact right child. He too is still processing his experience...his lifetime of experience.

I downloaded and printed three copies of all of our St. Louis pictures yesterday -- the ones from the hospital and the ones from sightseeing, and raced to the drugstore first thing this morning for little photo books. The kids each took a book to school today and shared their experience with their friends. When I said good-bye to Oscar he was showing his book to 3 classmates who were patiently ignoring his stuttering and listening graciously.

So, I am grateful for this lovely letter, for the caring gesture of a complete stranger. If Oscar isn't up for writing back, I will send a note thanking both her and her teacher for their wonderful thoughtfulness.

Making Lemonade

We're making lemonade here in St. Louis.

We're here to investigate the newly discovered familial hypophosphatasia. (HPP is an exceedingly rare bone disease that causes soft bones and early tooth loss). It's not what we'd choose to do for vacation, or even instead of regular life, but we're making the most of it. It almost feels like a break, but not quite.

The kids are undergoing all kinds of testing -- I don't even know the names of all the tests. I do know they took 7 tubes of blood this morning. (They only got 1.5 from Ruby because she kicked and cried at full volume for over half an hour.) The boys had pulmonary function tests at 9pm last night, all the kids have had a series of x-rays, and we're all doing 24 hour urine catches (even GrandMary and Grandpa). The boys are peeing into jugs, and Mary, Ruby and I are peeing into "hats" decorated with flowers by the imaginative nurses. We take our receptacles with us in conspicuous red bags everywhere we go.

Today we were escorted to see Dr. S., the dentist with the most HPP experience anywhere, in a Shriner's van. His office had big console video games and colorful giant-sized paintings of sports heroes and princesses in every room. Spiderman was hanging from the ceiling and Superman was punching through a wall. Dr. S. called Ruby "princess" and I've never seen her sit so still in the dentist chair. Usually she screams. He told Abe to only worry about brushing the teeth he wants to keep. Abe's gums are in terrible shape and no amount of nagging gets through, but I think he heard Dr. S. (It helps that he is friends with Albert Pujols and that one full wall is devoted to the Cardinals.) I'd almost fly here every three months. He's that good.

The kids' food is all painstakingly prepared by the research kitchen and is weighed before and after they eat. They want to make sure they know exactly how much calcium each is consuming when they analyze their blood and urine specimens. But there are a lot more things they are testing, things that I've never even heard of, despite having pored over all the technical research papers many times.

I don't like not knowing everything, I don't like feeling like the overly persuasive mom, I don't like it when the doctor calls me "Mrs. Hill" and refuses to call me "Mary", even when I ask. She's a wonderful doctor, though. She got Abe to laugh and also earned his respect by knowing before he said it that he wanted to be a baseball player when he grows up. She also predicted, based on the position of his cowlick, that he could be a switch hitter. (She made him promise to give her credit if he makes it to the big leagues on that information.) Ruby flirted and giggled with her, and Oscar was not anxious. I couldn't ask for better. And she did agree to call me "Hey You!", an excellent compromise and a big step up from "Mrs. Hill" in my opinion.

I've asked for tests that they will not do:
I want Oscar's scoliosis x-rays taken both in and out of the brace. Denied.
I want them to try to draw Ruby's blood again. Denied.
I want them to evaluate Oscar for orthotics. Denied.
I want them to x-ray Abe's arm to check if his fracture has completely healed. Denied.
This is a research hospital and we are part of a study. I need to remember that.
I also need to remember that this is all free. Yes, FREE!

But we are getting starting to get some answers. We think Ruby might "only" have the odonto (teeth) form of the disease which will hopefully be very very mild. We think Abraham and possibly Oscar might just be carriers. (So we'll just have to test everyone they date more than once to make sure any potential offspring would only be passed one mutation and not two, which could be lethal).

We learned that Oscar's scoliosis curve is "very small". (What does THAT mean? I can't believe they didn't give me a number yet! I've taken that kid for x-rays every 6 months since he was two and have charted his progress and regressions. I need a number!). But, they are not recommending surgery at this point and apparently that is all I need to know.

The big conference with all of the doctors is tomorrow so we'll hopefully learn more then.

We've also laughed, a lot more than you would expect in a 4-day hospital stay. And the nurses and therapists have been fantastic. Fantastic! They took the kids to the special toy closet after the big blood draw. Abe chose a cardinals hat (he left his at home and really wanted one for the Cardinals game we hope to go to on Thursday or Friday). Ruby chose a doll which she promptly named "Luna" and carried with her all day. And for Oscar they had a set of six plastic animals, the very kinds he collects. The smile was precious but I didn't have the camera at that moment.



And tonight, the evening nurses agreed to watch the kids while Paul, his parents, and I enjoyed a nice dinner out. I could post pictures of us all laughing so hard we had to wipe the tears away with our napkins. We found many things to laugh about and to be grateful for (like getting to sleep in a hospital bed instead of on a fold out chair). We felt like escapees. We were escapees.

The kids are having fun along the way too. Abe is playing guitar and reading a book with Paul. He's also discovered a new Wii game down in RT (recreational therapy). He's not working on his research report but that is okay. Ruby is coloring, and playing, and making friends in RT. Oscar is making friends too, and reading, doing sand art, and painting. They are getting exposed to and befriending kids with all kinds of orthopedic challenges, using all sorts of assistive devices and starting to see beyond the visible disabilities to the children within.

And I'm learning how to not be in charge, at least not all the time.
We are making a lot of lemonade here in St. Louis.

Goodbye Sea Ranch.....

Today was a day of transition, away from the raw beauty of the California coastline, and on home to Berkeley. We loved the relaxed pace of Sea Ranch, the long walks on the bluffs and daily checks on the mama and baby seals. Fires in the hearth, puzzles on the floor, naps on the window seat, and evenings in the hot tub. We will be home for just 6 days before embarking on the next adventure, Shriner's Hospital St. Louis.

I treasured the drive south, giggling with delight and nerves at how high above the ocean we were winding along that narrow stretch of route 1. Sparkling blue expanse on one side and sheer cliff on the right. I loved it, pure and simple. Nothing makes me feel more alive than the ocean.

As the road straightened and we veered away from the shore towards the bucolic farms of Sonoma County, my thoughts returned to real life and the week ahead. A busy one, full of appointments, baseball games, practices, rehearsals, and possibly a talk to Oscar's 2nd grade class about PWS (an annual event that I keep postponing, for some reason, this year) I also need to bang out those darn social stories.

And, yes, somewhere in there we need to get ready for St. Louis. We're taking the kids to Shriner's for a 5 day inpatient stay to help answer our questions about their bone disorder hypophosphatasia. They will sleep in hospital beds -- all 3 in one room -- with Paul and me nearby on those bedside chairs. Blood draws, xrays, 24 hour urine catches...a whole battery of testing that will rival Oscar's NICU days.

I've not written too much about HPP here. A large part of me wants to think it is not a big deal and that it is my own obsessive need for answers that is leading us St. Louis. But we do have two positive genetic tests (Ruby and Paul) and the boys have clinical diagnoses. Ruby has lost 3 teeth, her xrays show mild osteopenia, and she is slightly knock-kneed. Abe's feet hurt if he runs too long. His broken arm spent 9 weeks in the cast and still wasn't completely healed. The orthopedist that treated him was an insensitive jerk and didn't believe that he even had HPP. He refused to seek advice from the HPP expert in St. Louis. We are lucky with Oscar. I know he has HPP, but I am thankful we just found out. So many of the early symptoms overlap with Prader-Willi syndrome that we might have missed the PWS had we noticed the hypophosphatasia. We would have missed the opportunity to set up the early food routines that I truly believe have helped him tremendously. And the growth hormone shots he receives nightly for PWS might even be helping the HPP, but they're not approved for HPP. But...we don't know how to treat the HPP given the PWS complications. Do we supplement aggressively with calcium (as recommended for PWS) or avoid it (as recommended for HPP). What about his scoliosis, and the surgery another orthopedist is recommending?

So, yes, it's good we're going St. Louis. We'll get answers, an emergency protocol, and a definitive diagnosis so I will be armed the next time a doctor doubts me like Abe's orthopedist did.

In my heart, I trust the kids will be fine, and that compared to PWS this is nothing. But still my anxiety levels are creeping up. I feel an undeniable lump in my throat, my thoughts are racing, and there is a panic rising as I lay to sleep. I am nauseous and short-tempered.

I am trying to keep the images of that beautiful green and blue sea lapping, and sometimes crashing, against those dark jagged rocks. I am trying to feel the cool spray on my cheeks and the wind sending my hair into impossibly sticky tangles. I am closing my eyes so I can hear the waves and recall the briny scent that inexplicably centers and calms me.

But the anticipation is still getting to me. We've been home for 3 hours and I've already snapped at Abe and placed Paul's computer down on the floor with a bit more oomph than I intended. My patience is waning as the overwhelm seeps in like, ha!, waves on the shore. Maybe that is the answer. Maybe each time I feel the panic rise I can envision it receding. Close my eyes and watch it wash back out to sea. I will try.

Goodbye Sea Ranch. Hello St. Louis.