First babies

Elsa and Barnaby have become buddies.  We got Barnaby six years ago soon after we moved to Medford.  For a long time he was very leery of the children and easily pestered by them, but now that Elsa is older he's warmed up to her.

I think Barnaby has finally resigned himself to the fact that the children are here to stay so he'd better get used to them.  He now follows Elsa to her bed at naptime and bedtime and often curls up right next to her.  What a sweet kitty.



Snitching again

We have to watch this kid when it comes to food.  Remember when she got into the granola?

We realized that she had gotten unusually quiet after finishing her lunch,

and then realized we had left the tempting flat of berries just a bit to close.



Even fairies have to go to market

Sunning

This lovely silhouette caught my eye one morning.  From the kitchen sink all I could see was the shadow,

 but from the vantage of the dining area window I could see Ms. Tabitha Twitchit enjoying her morning sun.

She is an absolutely charming feline.  She chaperones the children as they play in the yard (and lets them hug and love on her), and is the most effective rat-catcher we've hired.  And so far she has been quite content being compensated for her services in food and daily scratching under her chin. 

Two days, 7 appointments, 5 doctors, 3 scans, 2 hospitals, 1 baby, and 2 exhausted parents.

We were given so much information during our two days in Portland that I am finding it difficult to summarize it briefly and logically, but I will try.  The most brief summary is that we learned that Oliver's condition is more complicated than previously understood, but that he is doing very well in spite of it and we were generally encouraged from what we learned from the three scans and five doctors we saw.

The more detailed summary that follows does not include all the information that we were given, but it highlights the most important things we learned and focuses on the short-term plan for Oliver's care.  Also, we won't have the information from several of the scans and all the bloodwork until the doctors have looked at them all.

The cardiologist again confirmed Oliver's WPW arrhythmia (the extra electrical pathway that causes super fast heart rates) and cardiomyopathy (the thickened and enlarged ventricles) with an EKG and echcardiogram (ultrasound on the heart).  He also saw some previously undetected conditions in Oliver's heart and lungs.  The equipment at the cardiologist's office is more specialized and higher definition than what we have in Medford.  The ultrasound showed myofiber dissaray, a condition where the fibers in the myocardium (the heart muscle) did not develop in a proper parallel pattern.  It also showed that Oliver has mild pulmonary hypertension (high blood pressure in the arteries in the lungs).  The third and most important finding is that Oliver has left ventricular noncompaction (LVNC).  When a baby's heart is just forming the tissue is spongy to allow lots of blood to flow in and around the muscle fibers as they develop.  LVNC happens when, the heart muscle does not compact together to form a solid mass and instead remains spongy.  From what I've read, noncompation cardiomyopathy has only been identified since the mid 1980's so it is a pretty newly identified condition, meaning that there is relatively not a lot of information about it (compared to a condition like high blood pressure).  While we are not happy that Oliver has LVNC, we are glad that they identified it because it is probably the root cause of the other problems with his heart and lungs.  Thankfully, in spite of having all these issues, Oliver's heart is sill functioning well.  The current plan for monitoring his heart is to do regular echocardiograms and Holter studies (like a 24 hour EKG) several times a year, and to pray for his heart to heal.

We also met with a pediatric surgeon about getting a g-tube (gastric-tube, a feeding tube that plugs directly into his stomach) for Oliver to replace his ng-tube (nasal-gastric-tube, his current feeding tube through his nose).  This is an elective surgery, meaning it is low priority and not vital to his health, but would simplify our lives (he couldn't sneeze his feeding tube out at 3am anymore) and be a bit easier on him too (no tape/tube irritating his cheeks, nose, and throat).  If we get clearance from his cardiologist we'll aim to have the surgery done on Aug. 9th, but if the cardiologist has any reservation then we'll wait. 

Our last formal appointment on Friday was with a geneticist.  Overall she was thrilled with Oliver's development, especially at how alert and social he can be - that was very encouraging to us.  She identified three possible families of syndromes that would explain some or all of Oliver's condition and we are testing for all of them.  We appreciate their approach to test more aggressively with babies because the information will be useful to all of his doctors as they decide how to treat him.  The geneticist's expectation follows Occam's razor (the simplest explanation is the most likely) and is that there is one single genetic mutation that is causing Oliver's multiple conditions, and not a different gene mutation for each one.  Following this logic, Oliver most likely has a yet-to-be-formally-identified-and-named syndrome that is being talked about in the scientific literature that presents with both noncompaction cardiomyopathy and displastic kidneys, Oliver's two biggest conditions.  So, there's no way to test for this "new" syndrome, but the results from the other two families of syndromes (the full Noonan panel and cardiomyopathy panel, about 20 genes total) will come in about two months. 

Finally, and possibly the most shocking part of our trip, was our stop at the lab for bloodwork.  By the end of the day Oliver had orders from four doctors for lab work, and needed to have 12 mL of blood drawn.  You may remember that Oliver does not give up his blood easily, and getting 0.4 mL out of him every week takes several hours and is a terrible pain, no pun intented.  The OHSU phlebotomists took about 90 seconds to get 12mL.  NINETY SECONDS!!!!!!!!!!!!  For TWELVE mL!!!!!!!!!!!!!  I knew that they are highly practiced specialists, but I was and still am awestruck.  And now that I know it can be done I will have no reservation in being a stronger advocate for him in the lab room. 

We have an appointment with nephrology (kidney and hypertension) on Aug. 7th and then we'll hear the results of the scans on his kidneys and learn how his blood pressure has been doing.

Sorry, no photos tonight, but check back soon!

How am I doing?

A few of my closest friends have asked me that recently.

That's a complicated question, and it has been a difficult one for me to answer. It depends on the day, the hour, how long we've been sitting at the hospital, how much sleep I've gotten for the last two nights, what I've been eating, how consistent I've been at disciplining the girls, how many times his alarm has gone off, and if I've been able to decide what we're having for dinner tonight. I am an actress, and it is easiest for me to respond with smiles and hope and sarcasm.

There are days, like last Sunday, when I lose it in spite of having a clean kitchen, and I am a complete mess until several hours after Joel tells me to go take a nap.

There are other days when I awake to find that my dear man picked up the house before he left for work, new flowers have bloomed in the garden, unexpected help arrives with dinner, the sisters play happily together, and Oliver wakes up for a few hours and acts like a normal baby.

But then there's another level of "How are you doing?" that inquires deep below the daily ups and downs that are present in everyone's life. Besides the bi-polar, sinusoidal extremes there is another part of the equation that describes the baseline of the heart's condition. But it can be frightening for the actress set aside the wit and sarcasm to confess the tension of what frightens her heart and what strengthens it.

I'm scared that my baby may not ever smile. There, I said it. I even wrote it. It tears me up inside and I scream in my head from the pain. I struggle with jealousy when I see other grinny babies, even though I have two healthy, precocious little girls. I am tempted with anger when, upon seeing Oliver's tubes and wires, the well-meaning dad describes how his baby had acid reflux for a few weeks. I find myself thinking that my education, organization, creativity and desire to do life excellently will get our family through this, and then I am tempted with despair and weariness when I can't keep up with the laundry or the dishes or anything and I've accidentally shattered several of our glasses from clumsy fatigue.  

But, unlike the depression that captured me years ago, these fears and temptations, though very real and present, do not consume me. They creep in and startle me on busy days and try to take advantage of my fatigue, but the peace and strength and power of God are so much bigger and much more real than these poison darts. While I am greatly concerned about our family, I am not overrun with worry. I have bad days and I succumb at times to discouragement and fear, but, praise God for this miracle: those times do not rule my heart and my spirit is at peace.  My days are still filled with wonder and beauty.

So, how am I doing? Life is really hard right now. Really hard. We are optimistic about Oliver but it's still hard.  I am exhausted, emotionally pushed to extremes every day, and learning the deep beautiful rest that comes from trusting in God's providence.  I hunt avidly for the next blessing, the next gift to add to my list.  "The Lord is my strength and shield. I trust him with all my heart, and I am helped." He is the only safe place, and that's where I try to stay. If I left I would be scared out of my mind.

How is Oliver doing?

That's a complicated question too. From what we can tell on the outside, Oliver has been pretty stable; the medicine for his blood pressure and WPW-syndrome continue to do their work, there's some variability in how awake he is from day to day, and some gradual, slow improvement in nursing and how often he sets off his apnea monitor. His developmental assessment two weeks ago was encouraging - for his adjusted age (based on his due date) he is a little behind, but not as bad as the therapists were expecting, and the areas he was most behind on were obvious and expected (like nursing and being awake more often).  We'll continue to watch his development closely (Nana, you need to come visit and get him to smile!). But even though he is doing well and seems pretty stable on the outside, we still have a full schedule of weekly lab draws, weekly pediatrician visits, and frequent scans, therapy visits, or other medical appointments to monitor what we may not be seeing on the outside.

Oliver was a "complicated discharge" from the NICU. In other words, coming home from the hospital did not signify the end of his need for intensive medical care, it just meant that after his blood pressure stabilized there was no medical reason for the the doctor to justify his remaining in the hospital. We are so thankful that he is home, but we still have a long road ahead of us with Oliver's medical needs.

You know those journal pages that doctors sometimes encourage parents to fill out to keep track feedings and diaper changes? Oliver has a three-ring binder. It reminds me of my lab book from grad school. We keep track of his meals, nursing, meds, poops, wake-time, monitor alarms, lab work, reports, appointments, etc, everything except the color of his blanket.

Things are slowly getting easier - getting off caffeine last month meant one less medicine, and we hope that he will no longer need the apnea monitor by the end of the month. Several people have asked me about the monitor, so here it is: it consists of two electrodes stuck to a foam band that velcros around his chest. The wires attach to a box (about the size of a small laptop) that records heart beats and breaths, and that sends off a loud, obnoxious alarm when the heart rate is too high or too low or he stops breathing for too long. I am thankful that we could have it to take care of him, but I can't wait for it to be gone. You know how annoying it is when your cell phone/laptop/whatever is low on battery and you have to continue your conversation or work plugged in somewhere slightly awkward? It feels like that, but all the time. It has a battery so it's mobile, but you can't just pick the kid up quickly without taking the wires and box with you.

One thing he has been doing well is gaining weight.  The pediatric nurses at the hospital that try to draw his blood every week affectionately call him "Michelin Man."  He has four fat creases just on his ankle.  FOUR.

Regarding nursing he is still slowly improving. At this point he nurses up to a half of his meal 1 to 2 times a day, and he rarely chokes anymore, which is the big improvement. But he still is just too sleepy to nurse more often and still gets really fatigued easily as he has to work around his high breathing rate. We'll be meeting with a surgeon next week about putting in a gastric-tube (g-tube) that go directly into his stomach so we can get rid of his nasal tube.  His doctors are also considering giving him a port - a device placed under the skin to give permanent, direct access for blood work.  His weekly standing lab orders only require 0.4mL of blood, but it still takes an average of 4 to 5 attempts (we're usually at the hospital for several hours) and the sample is often clotted badly. 

Next week we actually have two full days of appointments in Portland at OHSU and Legacy Emmanuel Hospital. We are so thankful that we could group them all together and make one big trip up there. We'll be getting doing some specialized scans for his kidneys and meeting with a surgeon, and doctors in urology, cardiology, and genetics. Please continue keep Oliver, our family, and all of his doctors in your prayers.

We love this kid so much.  I could kiss his cheeks all day.  Nora and Elsa continue to help take care of him, and Nora especially is very concerned when he cries and she thinks he needs his "nigh nigh" blanket. 

And THANK YOU, THANK YOU to all our dear friends who keep helping us to watch our children, fold our laundry, and bring us dinner.  What a blessing you all are.  God has used you so many times to help us get through the day!