Petty Syndrome

This will be a short update as it is approaching 9pm and it’s been a long day. Nora is asleep but she has not been such a happy girl this evening. She is not a fan of how her little legs feel after they get a shot in each thigh. That was this morning, along with our appointment to check on her weight and to review our UVA trip with her pediatrician here. We felt grateful to see the numbers on the scale going in the right direction again. She weighed 6lb 9oz this morning. We will continue to weigh her at home and don’t have any scheduled appointments now for a month. That feels good!

I’m attaching two things here for anyone interested. One is a collage of pictures of Nora and Kali. Nora is really enjoying looking up at who is holding her and her smiles are particularly sweet for her big sister. The flowers in the middle signify a turning point in our year – time to venture outside more with Nora. Kali had helped us determine when we would know that Nora could come outside and her criteria was when we saw “three yellow flowers.” The daffodils were a welcome site for this family. And we have enjoyed a few more times outside lately – including a short family walk the other evening. We look forward to more of those times in the future.

The other attachment is the article describing the syndrome Nora has been diagnosed with by the geneticist at UVA. I wasn’t sure whether or not to send this on but know that some of you will be interested in more detail. Jason and I have both been surprised at how we have experienced some relief and even hope in having a little more information. Also, the new things Nora is doing all the time can’t help but encourage us as well (she has discovered her toes and knees!).

It is clear from talking with several doctors and persons that work with feeding issues at Kluge Rehab in Charlottesville that it is important that Nora be with me as much as possible for the foreseeable future so that she can breastfeed on demand. So the next steps for us are figuring out how to juggle my work and our family life in a way that is sustainable and works for all involved. I imagine being quite tired in the coming weeks. On the flip side it has been good for me to have more time with Nora to get in a groove and rhythm with her care. I feel like we are understanding each other much better and that is a relief.

We’ll be in touch more later, Janelle

Diagnosis???

I am writing this minutes after our arrival home from another day of appointments at UVA. It sounds most appealing to spend this time slouching in a chair and allowing Kali to gorge herself on pbskids.org while Janelle and I and Nora recover from the six straight hours of physicians compassionately and competently puzzling over our family’s future. Kali spent the morning with our friend and neighbor Kristin Yoder Kaufman and baby Phoebe, and the afternoon with her aunts Sue and Karen, so she’s doing just fine.

The reason I’m not slouching in a chair is that I’m not sure I’d be able to get myself moving again soon (at least not with a good attitude), also we know many interested persons are curious about the decisions made at said appointments, and furthermore it is good for us to make a record of these days while they are still fresh in our minds, and we may as well include you.

I’ll write this like a newspaper article, with the big news first and the details to follow:

Though Dr. Braddock left the pertinent article in his office and couldn’t be convinced to recite the characteristics by memory for fear of contracting foot-in-mouth disease, he thinks that he has found a known genetic category for Nora’s condition (he will e-mail us the details as soon as possible). As was suspected, her presumed category is not at all populous, with the total number of reported cases being in the single digits. We have no idea what the implications of this are, and it’s possible we will be essentially making it up as we go along for many years, but I’ll say that it should not be assumed that because her condition is extremely rare that it will be extremely grave. Dr. Braddock will be trying to contact the authors of the papers he’s read, even though some were quite dated, to see how those kids did as they matured. Incidentally, it seems Nora will be the subject of a presentation by Dr. Braddock this August to some sort of assembly of syndrome-oriented geneticists from all over the world. He feels comfortable with this diagnosis, but has not yet convinced his mentors or a few key peers, which helps me remember that this could actually be still the incorrect diagnosis. Indeed, some of her symptoms, namely the underformed skull bones, are not necessarily characteristic.

Because of those underformed skull bones and her continued low weight, the neurosurgeons are not going to attempt her skull surgery at this time. And though they don’t yet know why this is so, they are surprised by the lack of asymmetrical distortion in her brow line. They are thinking that it will be best to keep seeing her periodically and only operating if and when conditions dictate and/or allow, which one doctor hazarded a guess as being around the age of 18 months.

So we don’t have to get her ready for surgery in June. That is a bit of a relief. Now, if we could just get her to grow!

Here’s the plan as far as that is concerned: Starting tomorrow (for this week at least), Nora will be going to work with Janelle so she can nurse directly from the breast 24/7 instead of needing to undergo the stressful hassle of trying to suck it out of a lukewarm, rubber contraption that doesn’t fool her for a minute. From our keeping pretty careful track of her eating over the past week, it is obvious she eats much better from the breast. Why am I not surprised? We are quite grateful for the flexibility shown to us by CJP, and Janelle’s supervisor Lynn Roth in particular, in allowing us the chance to give this interim solution a try. Also, the early intervention folks at the Community Services Board may help us try to improve the bottle feeding situation.

If neither of those approaches work, we’ll probably have to spend a few days in Charlottesville at the Kluge Child Rehab center working intensively with feeding specialists. Apparently this is something they are really good at.

Failing that approach, the next step would be a feeding tube. Dr. Braddock encouraged us to consider the directly-into-the-stomach kind instead of the nasal or oral route kind because even though their installation is more invasive, they are supposedly not too complicated or risky, and the resulting logistics are, in his experience, much more conducive to healthy development and family life.

Other than that, she’s fine. Really. She’s probably a little behind on her development, according to our regular pediatrician, but that’s supposedly to be expected when she’s got so many fish to fry at once. We anxiously await Dr. Braddock’s report that will hopefully help to prognosticate a little, but in the meantime he encouraged us to know that she seems to be doing well aside from the weight gain and other growth issues, insisted that we hear his compliments on our parenting, and prohibited guilt (even for the Dad who can’t get his baby to take a bottle). He also noticed her first tooth showing up under the gum! However, I noticed on the CT scan we saw on the neurosurgeon’s computers that a section of her front upstairs chomper buds seems to be missing. Dr. Braddock seems to think that means those teeth won’t come in, but it’s unclear to me whether that applies to adult teeth also or only baby teeth.

I would like to be able to neatly sum up this update for you, but I have no idea where to start. We continue to appreciate all of the caring and support that’s being shown to us. It makes a huge difference. I wish I could tell when we’ll be standing firmly on our own eight feet, but all I can say so far is that it’s not quite yet, but we’re doing our best to get there as quickly as we can.

Jason (see p.s below)

I, Janelle, just finished up Traveling Mercies yesterday and wanted to share one more quote before shelving that book until the next time. As Jason said we are very grateful to those who continue to share aspects of this journey with us. I’m also attaching a picture of our first walking iris bloom which we were gifted with yesterday.

“This is the most profound spiritual truth I know: that even when we’re most sure that love can’t conquer all, it seems to anyway. It goes down into the rat hole with us, in the guise of our friends, and there it swells and comforts. It gives us second winds, third winds, hundredth winds. It struck me that I have spent so much time trying to pump my way into feeling the solace I used to feel in my parents’ arms. But pumping always fails you in the end. The truth is that your spirits don’t rise until you get way down. Maybe it’s because this—the mud, the bottom—is where it all rises from. Maybe without it, whatever rises would fly off or evaporate before you could even be with it for a moment. But when someone enters that valley with you, that mud, it somehow saves you again. At the marsh, all that mud and one old friend worked like a tenderizing mallet. Where before there had been tough fibers, hardness and held breath, now there were mud, dirt, water, air, mess—and I felt soft and clean.” Anne Lamott

ps. Below is a portion of the email we just received from Dr. Braddock. Very fresh news…

“Janelle and Jason, It was a pleasure to meet with you and Nora today. I am sure that you all must be exhausted from the long day! I apologize for not having the following information with me during the visit but wanted to share with you what I have found. My leading thought for diagnosis for Nora is the condition described by Elizbeth Petty, Renata Laxova and Hans-Rudolf Wiedemann in the American Journal of Medical Genetics (volume 35, pp 383-87, 1990). Unfortunately I do not have an electronic version of the article, but will copy and send you a hard copy. This condition has the wrinkled skin, poor growth, hypoplastic (underdeveloped) nails and digits, abnormal hair growth, umbilical hernia, small lowset ears, thick eyebrows and a thin calvarium (skull). The two cases are females; ages 5 and 40. Normal intelligence was noted…Stephen R. Braddock, MD”

Weight going the wrong direction!

It’s a quiet, cloudy afternoon here in Keezletown. Quiet because Kali and Jason are at the school they volunteer at Tuesday afternoons and cloudy because it sounds like some thunderstorms are on their way to us later today.

Nora is sitting on my lap watching me type and I imagine this will not last too terribly long. She has been content today, even with a morning nap disrupted by our scheduled doctor’s appointment. Our good friend Kristin came down the hill with her baby daughter Phoebe this morning to play with Kali so that Jason and I could go alone with Nora to the appointment. We both felt the need to focus on our time with Dr. Ashton and to also be able to express how we felt depending on what the scales showed. It was a wise decision.

Neither Jason nor I knew what to expect but we were both pretty sure there had been no monumental growth in the past two weeks. Nora has been consistently eating very little from the bottle on the days that I’m at work and this has been frustrating and discouraging. While we think she attempts to do some catching up on the off days with me, we are pretty sure she is not able to make up the difference.

She weighed 6lb 6oz today, down 1 ounce from her weight about 2.5 weeks ago. While this is not a lot down, it is definitely moving in the wrong direction and worrisome to us and her pediatrician. While it may be there is something going on that is completely out of our control and that no amount of breastmilk will solve, there are a number of things we are to try in the coming days/weeks. Jason and I aren’t yet convinced that it isn’t a matter of Nora failing to consume enough, so we are open to experimenting with the feeding program. We will begin fortifying the breastmilk Jason feeds her in bottles. Jason will also be trying alternative methods of feeding – cup, spoon, syringe. We are also to start weighing her before and after feedings with me to try to assess what she is taking in (this is something not widely done anymore and is probably not best for already super anxious moms, but we’ll see if it gives us any information that is helpful). We go back again in 2 weeks for her next RSV shot and immunization and will see where we stand then. We are also starting her on a very small dose of Zantac to help with reflux.

In the meantime we have an appointment next Tuesday in Charlottesville – a follow-up with the neonatologists and then with the neurosurgeons/plastic surgeon about the possible/probable surgery Nora will need on her skull later this spring or early summer. I hope to connect with the geneticist at UVA yet this week via email to see if we might also be able to connect with him when we are in the area next week. Kali will have a fun day with Kristin and Phoebe in the morning and Aunt Karen and Aunt Sue in the afternoon.

Well, Nora has blown out in her diaper while she sits here so I better take care of that before continuing. That is probably enough of the raw data for you all anyway. Before I end the summary on Nora’s health, I should say that she continues to do new things. She is getting very good at her “tummy time” and is lifting her head and moving it to both sides. She was very happy at the doctor’s today cooing at the butterflies suspended from the ceiling. She is getting easier to do things with (like the fact that I’ve typed a page with TWO hands with her sitting on my lap) and sometimes enjoys carting around in the Snugli baby carrier that we have. Sunday was another warm day here and Kali and I managed to get three loads of laundry hung out and a walk to the mailbox with Nora in tow. Warmer weather is no doubt going to provide some relief for all of us!

The last several weeks have been trying emotionally for Jason and I. Where are we headed? How do we sustain ourselves in the midst of the uncertainty? How can we make this time as positive for Kali as possible, and for ourselves? How do we find beauty in the pain, joy in the sorrow, and hope while experiencing grief and feelings of loss? And how does one get more sleep in order to better do the above?

I do feel like Jason and I have both had times of self-care lately that have been helpful for us. I’m not sure if Jason would frame it that way but he has had several long outdoor work sessions which I know is always rejuvenating for him. I’ve enjoyed a walk, lunch and some phone conversations with friends as well as a massage that was paid for as a gift from another friend. I’ve had chances to air raw feelings with others which is always a start to healing for me and to moving beyond those feelings.

I am still SLOWLY making my way through Traveling Mercies by Anne Lamott. This afternoon I was finishing a chapter in which she reflects on her changing relationship with her mother as she ages, and on the eyes with which her son Sam views his grandmother. While our journeys are very different, I found myself connecting with some of her reflections and helped by seeing someone else sharing the range of their emotions experienced in one’s journeys with family. I’ll end by sharing a few quotes with no commentary and you can get the full story by reading the book and maybe some more of my thoughts connected to them will come in a later update.

For now, Nora is ready for attention and our small group will be joining us for dinner in about an hour. Jason called recently and after a quick stop at the library to look for some more Amelia Bedelia books at Kali’s request they will be on their way home. The wind is picking up and daylight is waning.

Blessings to each of you, Janelle

“Sometimes, holding her soft warm hand, I want to take it and hurl it to the sand beneath the wheels of the oncoming lifeguard’s jeep. But oh, God, the trust with which she keeps holding it out for me to take! Without someone to steady her, she cannot find her balance. And I guess when you take away the resentment and disappointment, it’s that simple. It is what we do in families: we help, because we were helped. Now when I look at the picture taken on the Fourth, my stomach aches with a miserable sort of poignancy because she is so friendly and eager to please. For just this moment I look like I am in love with her and she with me. For this one moment, we are. I am learning very slowly to savor the minutes between us that work, that cut through my life long hunger for a more perfect mother…Who was it that said that forgiveness is giving up all hope of having a different past? The pictures do help sometimes, and I’m gentler with my mother, more understanding. They help by making me sad at how hard we all try and how far short we fall, and the sadness softens me for love. I go around trying to do better by her, trying to be God’s tender hands and eyes…I tell you, families are definitely the training ground for forgiveness. At some point you pardon the people in your family for being stuck together in all their weirdness, and when you can do that, you can learn to pardon anyone. Even yourself, eventually…I keep watching. Sam takes his grandmother’s hand. I feel like sobbing with grief that my mother is old, that she won’t always be around, and with despair at what an erratic daughter I am. But it’s not a big deal to Sam. He just wants to help her find her balance.”
Anne Lamott

Only a 4 ounce gain in 1 month!

Kali's half birthday is today. She is a 4 1/2 year old bundle of energy! Today I got to enjoy some of that energy as it was warm enough to venture outside with Nora in the Snugli. I learned something about myself - I enjoy imaginative play much more outside. We took a walk in the "rain" with her umbrella. Then the weather kept changing and then it was "snowing" and she got on her "hat" and "gloves" and we made a "snowperson." We also had two "picnics" along the way with "strawberries" and "grass cake." Nora slept through most of it. And we also managed to be somewhat productive getting laundry hung up. I "hired" Kali this morning and she earned some coins helping me do a little straightening and cleaning. Besides the one moment when Nora had just fallen asleep and I was sitting down to rest while Kali played on the computer for a few minutes and something went wrong for Kali on the computer and she melted down waking Nora up and ruining my chance at a quick rest, the day was a good one. That is up until the doctor's appointment!

It was about a month ago since Nora was weighed at Harrisonburg Pediatrics so we were really hoping she might have reached the 7 lb milestone (about double her birth weight and reaching Kali’s birth weight). Neither Jason nor I knew if this was a reasonable hope but she had been gaining close to ½ ounce a day and that would have put her just over 7lbs. So you can probably imagine our horror when on the one scales she weighed in at 6lb 5oz. (last month she had weighed in at 6 lbs. 3 oz.). The nurse took us then to where they had last weighed her and she was 6lb 7oz. It didn’t feel a whole lot better to Jason and I. Only four ounces in a whole month!

How do we focus on the positives when gaining weight feels so essential? Dr. Ashton tried to be reassuring by letting us know that really bad would be losing weight, bad would be staying the same, and so-so is gaining but not gaining as quickly as we’d hope. He also said once again that he really has no idea what is “normal” for Nora and that there is no book he can pass to us to help us know what to expect (we are writing it as we go). He did feel like it might be important to consult the metabolic specialists at UVA just to see if they would provide some expertise that would be helpful as we try to figure out how to best care for Nora.

Complicating all of this is the fact that we are likely looking at surgery on Nora’s skull yet this spring/early summer. Yes, CT scan results from UVA did show that the coronal suture on the right side has prematurely fused. We go back to UVA on March 11^th to meet with the two world renowned neurosurgeons that would do the surgery, along with the plastic surgeon who works with them. If they feel surgery is important to open up the suture that will likely take place in May and would be a fairly major surgery. Dr. Ashton feels it is important that she is showing that she can grow and gain weight before she is put through major surgery which will take so much energy to heal from. So we will be working with him in the coming weeks as we have some big decisions to make. He wants to see us again in two weeks to check in on her weight again.

On the positive side of things, she took her two shots (another Synegis to continue to protect her from RSV and her first HIB vaccine) like a trooper today. She seems much more grown up to Jason and I. And while she may not be packing on the ounces at any kind of notable rate, she is doing new things every day and that feels good. She is cooing and smiling many times a day. She interacts with us and her favorite toys consistently. She loves to be carried around holding her little head up and looking all around. And while she continues to have skinny little legs and arms, they must be developing a pretty good layer of muscle from all the kicking she does and batting her arms around (particularly when she has her little sock rattles on).

So we journey on – with the ups and downs and the many questions that remain unanswered. I recently picked up Traveling Mercies: Some Thoughts on Faith by Anne Lamott that I had read some years back. In one chapter she is reflecting on a time when she was waiting for test results about her little boy, Sam. One line in particular stuck out to me: “But patience is when God—or something—makes the now a little roomier.” Many days I feel I could use a bigger dose of it!

There are days and moments where I feel that we will make it through this time in our lives, that there will be brighter and clearer days ahead. Maybe we’ll even look back on this time able to see more of the gifts and beauty in it. But it’s hard a lot of the time to not feel really overwhelmed by it and to feel completely sucked dry. One of the things I’m particularly struggling with right now is feeling unable to really do much of anything well and it leaves me feeling spread thin and exhausted. I’m working 3 long days a week in the office and even on my most productive days I feel like I can hardly scrape the surface of what needs to be done. There are a lot of changes in the works at CJP and that always creates additional meetings and extra work. It would be a lot easier for me to let go of the fact that I can’t get everything done if I didn’t care so much about the people I work with, the work that I’m doing and about the organization. Then I come home wanting nothing more than to sleep but need to attend to the needs of two little ones, our home, making sure everyone is fed at least a semi-balanced dinner and trying to plan and prepare for the next day. I feel like I often am not able to be present to Kali at all because of Nora’s needs and there are many nights I just end the day feeling disappointed with my inability to be who I want to be (and at one time could be) for Kali. I’m grieving the changes in my relationship with her; what feels like a loss of something really precious. I am reminded by others that children are so resilient and I believe somewhere deep down that Kali and Nora are probably the two members of the family doing the best with all we are facing. Nora seems pretty oblivious to the great stir she has made by her entrance into the world!

Speaking of Nora, it seems her nap in daddy’s arms is about over and I’ll probably be called in for some nourishment. We’ll be in touch again before too long – hopefully on a day when the one of us writing this update is feeling a bit more creative and a bit more hopeful. It’s been a good exercise for all of us to say one thing we are thankful for before meals. Kali almost always says “Nora.” Today I was thankful for the sunshine to dry laundry outside. Jason mentioned feeling thankful for times that provide a reason to feel hopeful. While we may not feel so hopeful about things in our immediate family, he was feeling hopeful about the possible outcome of our upcoming presidential election. I think we are both looking forward to the time when we can have more energy to look outward again!! Blessings, Janelle

CT Scan - she did it!

We have just arrived home from another UVA trip…scheduled appointments for a CT scan of the head and then a follow-up with a UVA Neurologist, Dr. Rust.

No news is good news, right? We hope so because that’s essentially what we still have. The CT scan was performed successfully, but that’s as far as our information goes. Neurosurgery (Drs. John Jane or Junior of the same) is supposed to review the scan on Friday, with their interpretation being delivered to us in a phone call Monday. Does that mean the radiologist will have reviewed the scan? If history is instructive, not necessarily. Actually, if history is instructive, neurosurgery will be asking us whether radiology has reviewed it and what they said about it. Sometimes it is very unsettling to feel that all the various specialties are referring Nora to each other but somehow the system seems to be relying on us to put all the pieces together into an integrated whole. I feel, for one, like we could so easily be missing, neglecting, or forgetting something. Also I wonder what this kind of experience is like for those whose upbringing and/or educational background have not prepared them to communicate with medical professionals meaningfully or integrate the material. To be fair, I suppose Nora’s pediatrician is the one responsible for integrating all the medical data, and he doesn’t comment much on the big picture simply because nothing that has emerged thus far from all the consultations, tests, and procedures has been very helpful in bringing the big picture into focus. Yes, that is frustrating, but we have to keep reminding ourselves that probably the best possible scenario from here on out is for this state of affairs to continue indefinitely. If current trends continue, she may establish a track record of equilibrium that makes her of less interest to the medical system as time goes by.

Neurology. It’s good that people who can deliver such scary, sad news can be as kind as Dr. Rust. It was a relatively short appointment (except for the waiting) that delivered the kind of news we’re getting used to: they can’t really see anything wrong (and this seems to be mildly surprising to them). The long and short of it is that he thinks she seems to be doing well, and yet wants to follow up with her in 4 or 5 months. If that had been our only reason for traveling to UVA, the sense of having wasted our time would be keen, though the encouragement received there will probably sustain us more than we are aware. The only thing Dr. Rust noted was the same thing others have…her tendency towards “sundowning” or rolling her eyes downwards. He referred us to neuropthalmology (sp?). An odd moment of the appointment was at the beginning, when my sister Emily’s college friend Aaron Trimble walked in the room like he belonged there! It turns out he was accompanying Dr. Rust to his appointments as a part of his medical school training.

I have thus far failed to mention the anxiety producing experience of trying to get the CT scan accomplished, but it bears mentioning. For a 3-D CT scan, the patient must keep their head perfectly still for around 5 minutes (about 30 seconds of actual scan time). It’s not that Nora never leaves her head still…how in the world can a person predict which particular 30 seconds are going to be the still ones unless the baby is sound asleep and not easily awakened by machines whirring away like a Star Trek contraption in close proximity. If it can’t be done without sedation, sedation is required. This means an overnight at the hospital, since they would need to keep her under observation until the sedative cleared out of her system. We were determined to avoid this, but it felt somewhat out of our control…without sedative there is no way I know of to force a person to sleep (maybe they should try hypnosis!)

We arrived with a sleeping baby and high hopes at a little after nine, expecting, as per verbal telephone reassurances in the prior days, that sleeping babies would receive priority, expedited service. At the front desk they made it seem that way, too. So we were surprised to have to wait in the dressing room for around 45 minutes while they made it through their scheduled prior appointments. Nora awoke about 5 minutes before they came to take us back. We were more than a little hot under our respective collars! When we finally reached one of the sanctum santori of UVA imaging, the tech who ran the thing was surprised to see a baby, and listened with (rapid) sympathy to our sob story. And Janelle did actually sob a little, which I’m sure helped. The tech explained that she had no idea what the situation was and would have surely accommodated us, had she been told. I felt like shouting, “I cannot solve your department’s communication problems!”, but assumed that doing so would not engender support for accomplishing the scan in time for the 1:30 with Neurology, and therefore refrained.

The rest of the story felt long, but it is short. They gave us a room to crash in and we did our daddy and mommy thing until she had drifted into one of her sweet-sleeping naps of unfortunately unpredictable length. With pounding hearts we notified the staff and were quietly whisked into a different sanctum sanctorum; one staffed by persons that seemed really very competent and sensible. With crossed fingers and a few people praying (some who claim not to do that very often) we watched the machine lift her sleeping little body and insert her head into that space-aged, donut shaped radiological device. Nora didn’t move a muscle. Cheers all around! Janelle and I walked out with Nora and her diaper bags in our arms and with Jello in our legs. A minute later, Nora woke up. It was 12:30. A half hour later and we would have had to walk out of there with our hearts in our shoes.

So was it a good day? I have no reasonable frame of reference for answering that question. Maybe that’s the biggest thing this whole experience is teaching me so far. Sweeping generalizations about what is bad and what is good fail to respect the nuances of true daily experiences, especially when they matter. Onward we go…thanks for listening. Jason

A p.s. from Janelle – Some might be interested to note that Nora weighed 6 lb 10 ¾ oz today (but with clothes on and on a different type of scales so who knows compared to the pediatrician’s). And I can’t think about too much else to add after a night of little sleep last night and a baby squirming on my lap. Kali had a fun day today, thanks to the generosity of Bill and Dottie who once again not only opened their home to us last night but also played and cared for Kali today.

Wake Med milk bank and fingers in car doors...

What a day it has been so far and it is only 12:30pm. I’m typing with Nora’s laying half-asleep on my chest with her little bottom propped up by the ledge of the laptop. So far so good and I’ll make quicker progress on this update if I can continue to use two hands – though my one-handed typing is improving drastically. Kali and Jason left home about an hour ago for Shenandoah Valley Community School for their first day back volunteering since Nora was born. Nora and I are here together to recover from the morning.

It started out more or less like many other days. The catch was that we had to get to Nora’s doctor appointment by 9:30am – early for this family these days. We managed to get diapers out of the washer and put on drying racks as well as breakfast in everyone before heading out the door shortly after 9.

Nora didn’t like the ride across town much more than she enjoyed the trip to UVA but thankfully we made the 15 minute trip without any stops! She was worked up when we got there and wanted to eat. We assume it was more about being worked up and chilly than hunger because when the nurse joined us Nora promptly returned most, if not all, of what she had just eaten to her blanket. That, in addition to two blow outs in her diaper this morning, probably contributed to her weight being slightly lower than what we were hoping for. She weighed in at 6lb 3oz and was 20 ½ inches long. She’s moving in the right direction at least. Slowly but surely…which is all the doctor is looking for from her. A huge milestone this week for Jason and I is that we ditched “the notebook.” This being our record book of the last two months; our attempt to document every bit of breast milk Nora has consumed and ever diaper change including its contents. It was getting to feel more and more tedious, so we have been enjoying the freedom of one less thing to keep track of (while putting up with the “is she eating enough” pangs that I, more so than Jason, continue to experience from time to time). It’s one more step moving us towards parenting Nora using our intuition rather than some scientific formula. That feels good.

When Kali heard this morning that Nora had to get multiple shots, it was clear she was having sympathy pangs for her baby sister and she expressed verbally that she wished Nora didn’t need to get shots. Me too! We had requested that we not do more than two today – her second Synegis shot (to protect her from RSV) and the first of many vaccines (Pertussis). It feels important to Jason and I to start slow on her little body and see how she does, while also making sure she is immunized soon for things that are a current health risk to her. We were grateful to the two nurses who came in and offered to give both shots at the same time. What a brilliant idea! Nora was very clear that she felt the shots were a bad idea all together but we are convinced that it was better to just get both over with at the same time. We had to stay for about 20 minutes afterwards to make sure she didn’t react to the Synegis one and then were free to go.

During the time we were inside, snow had fallen and there was a light dusting over most of the world around us. Suddenly the flip flops I had grabbed for my feet as we hurried out the door seemed even less appropriate! Nora sacked out as soon as we got in the car and slept the whole way home. Phew!

Our landing home included excitement for me and tragedy for Jason. He informed me that the milk bank had delivered another cooler, only minutes before slamming his finger in the car door after helping to get Kali out. He will likely be without one finger nail in a week or so from now! I worry now that I didn’t provide enough sympathy for his pain as Kali and I scurried around, in the half hour she and Jason had before needing to leave for school, trying to get the cooler packed and ready to be picked up by FedEx (and we needed to get their lunch packed, Kali’s bladder emptied, and Nora was starting to think it was time to eat again). I imagine for at least some of you some questions may be arising in your minds: Milk bank? Coolers being delivered and returned by FedEx? Why the urgency to use that half hour of time to try to get it packed?

The short of it is that my supply of milk greatly exceeds Nora’s demand. While a wonderful problem to have, we only have one small chest freezer! They encouraged me in the NICU to pump every 3 hours, even when I warned them that from past experience with Kali I knew I was prone to produce a surplus of milk. So we were sent home with bags of containers full of frozen milk. When we arrived home our freezer couldn’t hold all of it and I had little choice but to pour ounces and ounces down the drain. It felt ridiculous after arriving home, especially around 3am, to be pumping and throwing it out. Finally, I researched the option of donating the milk and found WakeMed Mothers’ Milk Bank in Raleigh, NC. Since that time I’ve sent about 800 ounces of milk to them and even have an official donor number! This has made the 3am times of pumping feel a little more worth the disruption of sleep and having this outlet for all surplus milk also means that as of right now we once again have ample freezer space!

Since Jason and Kali left, I’ve spent more time than I would like to admit on the phone with doctor offices and our insurance company. I have a new found sense of horror at what persons go through who are juggling health issues, multiple appointments with multiple providers and trying to figure out insurance questions. Thankfully I’m healthy and can juggle carrying Nora around in one arm, the phone propped on one shoulder and the explanation of benefits I’m calling about in my other hand. And thankfully most people on the other end the line really do want to be helpful – including Janelle (yes, spelled the same way as mine) at BlueCross BlueShield, whose birthday is two days after mine, who daughter’s birthday is three days after Kali’s, and whose sister’s birthday is the same as Nora’s. She was able to fix the claim that was warning us that UVA could charge us just under $100,000 for just SOME of the services provided during her time in the NICU. That was a typo in their system I was eager to correct!

Other calls involve trying to coordinate appointments at UVA to minimize future trips over the mountain. That is proving difficult as it seems that different areas of specialty have different clinic days. So now I’m trying to sort out which appointments are really necessary and really urgent, and which can possibly wait – at least until winter is over and Nora’s weight hits the double digits. That can be hard when she continues to be a baby that her pediatrician will make absolutely no predictions about. He said to us today, “I don’t know how or why she is doing what she is doing but I like what I see” (referring to her weight gain, continued mineralization of the skull, what he felt was a decrease in size of her umbilical hernia--this isn’t the right term but Jason isn’t here to help me out--, etc…

So we continue to journey with our little mystery baby. She has started to brighten our home on many occasions throughout the day with her smiles. You can see one for yourselves on the little video clip at http://www.youtube.com/watch?v=ll3M9sD01B0

She has also started to remind us more of Kali – the fact that most days she just doesn’t seem to sleep near as much as most babies her age would, that she likes to snack often for a short time, her bright inquisitive eyes, some of the little facial expressions and sounds she makes while sleeping, and that she loves being carried and is put to sleep easily by an OUTDOOR walk in the Snuggli.

Yes, I did say outdoor!! This past week the temperature hit the 60’s one day and I could not restrain myself. I went for a walk WITH Nora OUTSIDE. It was wonderful. It made me feel better than normal. I found myself longing like I never have for spring time and warmer days. The feeling that family walks might not be only a thing of the distant past, but something that could return to our routine in the not too distant future felt like a dream coming true. I didn’t stray too far from home because when Nora decides she no longer likes her particular situation for whatever reason, she doesn’t delay in making that known until it is fixed. She has started to be a little patient for feedings, but we know not to push our luck for too long.

This past week I had an afternoon where Kali and Jason went and did errands for 4-5 hours. It was the first time that I had a significant block of time with just Nora. I found myself listening to music, looking out our big picture windows at the mountain, and feeling so many things that I was unable to verbalize. Tears came, but they weren’t exactly sad tears or happy tears. I think they carried some weariness, some hope and some fear in them. And as I walked back and forth in the front room with Nora in arms, I realized that I was starting to also feel something else – love. So many people have encouraged me to give myself time and grace regarding my journey of bonding with Nora. So many looking in on our experiences of the past number of months have affirmed Jason and I as parents. Yet inside I’ve often felt and still feel inadequate and unprepared for the journey ahead.

The song that brought on the tears initially is a song by Over the Rhine called Born. While the song was written regarding the relationship of the husband and wife musicians, the refrain has become very meaningful to me as I journey with Nora and our family in the present and think to the future.

“I was born to laugh, I learned to laugh through my tears.

I was born to love, I’m going to learn to love without fear.”

The laughter in our home has been on the rise – mostly due to the creative, fun and continuously-chattering 4 year old in our home. Her most recent string of consciousness that had us doubling over was overhead by Jason one day when she was reading books to herself. Don’t try to analyze it too much but here it is: “Knowledge is knowledge and cramps are cramps…(brief pause)…Knowledge will have to go and cramps will have to go but knowledge is better.” We aren’t all that certain that she could really give a working definition of knowledge or cramps…

Well, I’m in danger of going onto the fourth page here, even with margin modifications. Nora has needed a snack and a diaper change in the process of this letter but is once again sacked out on my chest held up by the computer and wrapped in an afghan my mom made when Kali was born. I’ll attach here a recent picture of Nora and one of the other two members of my family whom I love dearly, and who make me smile quite a lot when the younger of the two ropes the elder into imaginary play!

Until next time, Janelle

Returning to UVA for appointments

We are home! We are tired!! It seems a little hard to believe that it was just yesterday that we packed up and headed over the mountain to UVA (a scheduled appointment) for the first time since bringing Nora home in late November. A number of times on the trip, I commented to Jason that I was glad we hadn’t tried to travel at Christmas and that I think it will be quite some time before I choose to travel any significant distance as a family. It was complicated. First of all it felt like we had enough stuff with us that we should have been going somewhere for a week – cloth diapering does that to us. Secondly, Nora really is not on much of a schedule except that she does seem to be starting to tank up on milk and clear out her bladder and bowels during the day. That meant that we had to stop two times on what should be an hour long trip to feed her and change her diaper. She also seems to be getting used to having parents that respond to her needs, when expressed, quickly. She wasn’t so impressed that the laws regarding car seats kept us from responding immediately. We were grateful for the unseasonably warm weather which made it possible to stop along the road for “emergency” baby care. We were grateful to arrive safe at Bill and Dottie Scott’s around 5pm. They once again received us warmly and opened their home to all four of us this time. Nora took awhile to settle in the evening but then did one of her first 5-6 hours of sleep without eating. I am quite sure that Jason and I could get used to that!

Today was exhausting and emotionally draining. We didn’t go into the appointments with many expectations and once again there is no grave news to report. There is actually some good news. She has crossed another milestone – 6lbs! And the microarray analysis results are in – normal! The geneticist said that he continues to feel that her package of symptoms has some genetic cause, but he is not able to pinpoint what that is. However, he feels that time is on our side because she is doing well and continues to develop. What he feels most right now is that our diagnosis may be that Nora is Nora.

For me the appointment with neurosurgery was more unsettling. The appointment was primarily to look at her skull bone, or at least what there is of if, in her case. While we feel that some mineralization has occurred since birth, she clearly is still lacking in the brain protection department. That is not the primary concern at this point since she is not mobile yet and not in much danger of head injury. The one concern at this point is that two of the plates on the side may have fused prematurely. If that is the case it can cause problems as the brain grows. Therefore the neurosurgeon really wants a CT scan done in the next month to check that. IF they have fused, he may recommend surgery to separate them. I think it was the word surgery, which we heard a number of times, that really got me. I think I’m just not ready for a re-hospitalization, for watching Nora suffer again, for having other people being her primary care givers, for feeling scared and out of control and unable to make things better for her…

Some days it would be so easy to just think we have a baby, a normal healthy tiny baby. She really has had so few issues. She is not on any medication. She has not needed any additional doctor appointments for anything since we brought her home (other than her weight checks). She is feeding by mouth and getting better at it and wanting to do it more often (like right now as I’m trying to quickly finish this update!!). She lets us know when she needs something. She pees and poops regularly enough to make us wash diapers almost daily now. She is smiling at us and the ceiling fans and her red stripe on the cloth by the changing table. She makes noise other than cries – expressions of excitement or contentment. She’s even growing out of some of her preemie clothes.

Then, on a day like today, it feels like we have anything but a healthy, normal baby. We enter the medical world where Nora is a mystery. We get a list of appointments for the future and see our schedules involuntarily rearranged to accommodate them.

I’ve reflected a lot in the past number of weeks about our lives being composed of many individual moments – little snapshots put together to create the tapestry of our lives. I’ve tried to give myself the freedom to enjoy some of those moments, even while wishing that this stage in our family’s life looked very different from what it is. Sometimes it is hard for me to allow myself to feel happy, for fear I’ll somehow then not be able to handle the hard moments. Today I felt like what I need for myself is to somehow get to the place of accepting that the next few years are going to hold a lot of hard moments and a whole lot of uncertainty for us. If I can come to at least accept that reality and not have every new hard turn throw me off balance once again, maybe I can free up some space to enjoy the beautiful moments that will come and allow myself to be surprised by them when they do come – like just moments ago when I was frantically trying to type this letter before bedtime with Nora in my arms (which slows me down considerably) when I look down and catch a big smile.

Nora isn’t smiling right now, however, so I think my time is about up. Daddy is pacifying her for me, but I’m not sure she would feel like sending you all this update was a good enough excuse to delay responding!

We continue to feel grateful for the support that we have felt from many of you. Till next time (which will likely come after her next appointment with her pediatrician on the 15^th when she gets her next RSV shot and her very first vaccination.) Janelle