Answers

I apologize for the lack of updates. Life has finally slowed down after the hustle and bustle of traveling, moving back to Tacoma, and the holidays. Shirley has officially learned to crawl, albeit quite begrudgingly… she still prefers to scoot when she is on the floor. She is now a professional cruiser and has taken as many as NINE unassisted walking steps in a row. We are so proud of her!

The following portion of the post is information dense. So, if you’re just here for the photos, you can scroll to the end.

In my last post I wrote about how we were scheduled to see the Mary Bridge Genetics clinic in September. I gave a short update a couple months ago on Instagram, but I figure I should include the whole story here, as well. At our first appointment, our geneticist agreed that there seemed to be something more going on with Shirley’s development. She suggested a test for Noonan Syndrome (something Joseph and I had already been considering after much research), the test came back negative.

We decided to move forward with a full exome sequencing test. They warned us that the test could come back inconclusive, which wouldn’t necessarily rule out the possibility of a genetic cause for Shirley’s heart anomaly and developmental delays, it just means they couldn’t definitively connect the dots. In order to complete the test, Joseph and I had to get our blood drawn as well. They would compare Shirley’s DNA with ours in order to gather as much information as possible.

We gave our blood samples at the end of November.  On December 28th I received a call from genetics informing us that there was a “finding” that can explain a lot of what is going on. Shirley has a mutation on the PUF60 gene on her 8th Chromosome. It’s called Verheij Syndrome. It is, as of now, extremely rare. Partly because it is a newly discovered/researched syndrome (within the last 5 years) and it takes extensive genetic testing in order to diagnose. The geneticist who called me had to spell it out for me. He wasn’t even quite sure how to pronounce it himself!

We went back in to the clinic for a follow-up appointment last week. We learned that there are two variants of Verheij syndrome. One is a point mutation, essentially a misspelling, on the PUF60 gene (Shirley has the point mutation) and the other is a deletion of the PUF60 gene and sometimes pieces of surrounding genes. The point mutation is significantly less common. Our geneticist said she only found 18 other confirmed cases in her worldwide database. Additionally, Shirley is the first one to be recorded with her specific mutation (misspelling), all the other people who have the point mutation have it on different locations on the PUF60 gene.

The origin of Shirley’s mutation is something they call “De Novo,” which means it occurred spontaneously with her. Neither parent carries it. It is an also autosomal dominant, so if/when Shirley would like to have children, there is a 50/50 chance of her passing it on.

Verheij Syndrome has been linked to Cardiac anomalies (Specifically septal defects – Shirley has both a ventricular septal defect and an atrial septal defect), Joint laxity, Feeding difficulties, growth delays, developmental delays, Skeletal abnormalities, certain facial/physical features, kidney, eye, and hearing issues. Just like any other Genetic disorder, there is a range of severity in all of these areas. In most of the studies, the deletion variant seems to present more severely than the point mutation.

Our Geneticist suggested Shirley get a spinal x-ray, kidney ultrasound, and be seen by both an ophthalmologist and audiologist. Since Shirley has several chest x-rays on file (due to her surgery and pneumonia), and she received a kidney ultrasound while inpatient at Seattle Children’s (due to her UTI), our geneticist said she would have those looked over before ordering another x-ray or ultrasound. Furthermore, Shirley is already seeing both an ophthalmologist and audiologist. So, we haven’t added anything to her plan of care – for now.

I hope I explained everything in a somewhat coherent manner. It’s a lot of information, and we’re still digesting it. One thing we are sure of is this: Shirley is fearfully and wonderfully made. She is God’s beautiful handiwork, and we are so very blessed to be her parents.

Below are some photos from the last couple months.

-Brianna

Questions

People (strangers, mostly) constantly ask me questions when I’m out and about with Shirley, “How old is she? Oh, she’s 18 months old? She’s so little! Was she a preemie?” or “is she walking yet? I bet you’re chasing her all over, huh?” I don’t mind these kinds of questions, really. But I am often unsure how to answer them. Do they want the long answer, or the short answer? I could just say “No, she wasn’t a preemie. And no, she isn’t walking.” Or I could go into detail about her medical condition and how we really have no idea why she’s so small or why she’s not walking yet (or crawling, for that matter). I mean, we know that her muscles are weak, that’s why her gross motor skills are behind. We also know that she struggled with eating her entire first year. But what we don’t know is WHY her muscles are weak, or WHY she has feeding and weight gain issues.

Rewind to the very beginning of the year. I had begun questioning, just to myself, why Shirley’s gross motor development was so slow. I mean I KNEW that delays were common for kids like her… but were other heart babies with her similar histories as weak as she was? Were they as bendy and flexy as she was? Did they have as hard of a time eating as she did? Were they all as TINY as she was? So, I started digging. I asked questions on my heart parent groups on Facebook, consulted therapists, read blogs and articles from medical journals. These are really hard questions to answer because every cardiac kiddo’s story differs from the next, especially ones with Shirley’s condition. One thing I did find was that most children who have the same struggles as Shirley (heart defect, extreme low tone, and feeding issues simultaneously) are also often diagnosed with a genetic disorder. At birth Shirley was tested for the genetic disorders that are most commonly associated with her heart defect, but they came back negative. Are those the only possible disorders she could have, you ask? Absolutely not. But, at the time, her team didn’t see a need for further genetic testing.

At long last, I brought it up with both her GI doctor and cardiologist. They both agreed that her low tone, short stature, and difficulty gaining weight were enough for a referral back to the Seattle Children’s genetics clinic. Especially taking into account that she has only had one heart surgery, and never went through a period where her body lacked oxygen. We were referred in April, but since there are not really any “genetic emergencies” appointments are being scheduled out 9-12 months. So, we’re still waiting. 

Shirley had her 18-month check-up last week. She weighed in at 18lbs 2.5oz, which means she’s gained a pound since her cardiology appointment last month. But, she has only gained 2lbs total since her 12-month check-up (most of which was gained in the last 3 months). Her pediatrician was also puzzled by her slow physical development and began immediately bouncing around different ideas regarding a possible genetic component to her diagnosis. When he heard how long the wait was for us to get into the SCH genetics clinic he decided to put in a referral of his own to the Mary Bridge genetics clinic in hopes that they would be able to get us in sooner. We're scheduled to see them next month.

Let me be clear on this, we are not fearful about the possibility of Shirley having a genetic condition; it is not a source of anxiety for us. We are purely interested in finding out more about what’s going on in her little body so that we can better support her growth and development. In addition, many genetic disorders bring along with them other potential medical issues that we wouldn’t know to look for without a proper diagnosis.

So, we wait. While we wait we’re focusing on the task at hand. We are spending our time teaching Shirley to feed herself, working with her therapists and practicing her gross motor skills. We’re also trying to figure out how to treat her like the 18-month old (non-toddling) toddler that she is. Our girl looks and moves like a baby but she thinks and understands like a toddler. If we aren’t careful about interacting with her at a level that is developmentally appropriate for her age, her cognitive progress may just slow down to match her physical development. In other words, she will live up to our expectations of her. We have our work cut out for us... in the very best way. I have never felt more fulfilled in my life. 

Shirley is a precious gift. We have been thoroughly enjoying every single stage with her. We often ask each other “How did we get so lucky to be her parents?” 

Keep our sweet girl in your prayers while we search for better ways to support her as she grows.

I've included some fun pictures from the last couple weeks. 

- Brianna

The Second Honeymoon.

Today marks the 1 year anniversary of Shirley’s open heart surgery. I recall the doctors and nurses telling us that the days before her surgery were often referred to as “the honeymoon.” A time when everything is beautiful and new and the little babies display no serious symptoms of their CHD. They told us, however, that the honeymoon would not last. The symptoms of her TA would become more pronounced and the need for surgery more apparent.

When they wheeled her away, just after we gave her little kisses, our hearts sank. A visceral sense of grief weighed on us like a ton of bricks and we sobbed.

Would we every hold her again? Would she ever stare back at us with her beautiful blues eyes, so full of life?

The grief did not last though. Long before she was out of surgery we were blessed with an overwhelming sense of peace.

Shirley’s surgery was successful and we have enjoyed an entire year of what feels like a honeymoon.  Although not everyday is easy, the vast majority have been filled with such a great sense of joy and peace. We don’t know how long our honeymoon with Shirley will last but I also cannot recall anyone guaranteeing us that life would be easy. We do not feel entitled to an easy life. We hope for one... but know that it is not certain. Our hope and joy in life is not dependent on our good fortune, it cannot be. If we put our hope in finite things we are certain to lose it when those things pass. Instead we hope in Christ.

People often ask us how we stay so positive. There are many answers to this question but a few things stand out to me.

First, we choose to dwell on the promises of God rather than the expectations of the world. We believe that God loves us, as we are today, redeemed in Christ. The events in life do not occur outside of God's love for us. Shirley's CHD is not God throwing us to the wolves. We believe that God is here with us and is using Shirley's CHD for His glory and our good. We firmly believe that no matter the trials in life, God has meant them for our good. If we believe this to be true, to be a matter of fact, just as we view gravity to be the reason that when we jump we will certainly come back down, then how could we maintain anything but a positive attitude? The world would have us believe that our life is difficult and that we drew the short straw. That, somehow, we have been treated unfairly or that we don't deserve to have such a needy and broken child. There is about as much truth found in that kind of reasoning as there is in a pile of bull's excrement (Brianna made me censor it). We are owed nothing and we deserve far worse.

Second, we simply feel blessed to have Shirley. We believe that children are a blessing, no matter what difficulties might come along with them. Shirley may have more challenges than some children but we are blessed just as much as anyone to have the opportunity to be parents. If Shirley is simply a blessing, then how on earth could we justify being negative? 

Here are some throwback photos of Shirley the day she had surgery. I put a couple recent ones in as well!

-Joseph

One

Shirley is one today! We could write for hours about the past year but we won't. 

We are so blessed to have Shirley in our lives. It has been a wonderful year! 

Please continue to pray for us and our little girl as the years go by. The support we received over the past year has been unbelievable, thank you.

Shirley's heart will never be perfect and she will need other surgeries to keep it beating, but we look forward to the days, months, and hopefully years to enjoy our little girl.

There are some exciting things coming up for us and Shirley. We will be doing some work with the American Heart Association this year as we continue to spread joy, hope, and awareness to those who find themselves in similar shoes.

Please enjoy these pictures we took for Shirley for her first birthday.

-Joseph

No Surgery

Shirley won't be having surgery on Thursday!!!!

Shirley has been without an NG tube since Friday morning and has eaten enough by mouth to keep her hydrated and healthy!!!

We are so so so happy! Shirley's struggles eating over the last 9 months have been the greatest challenge next to her actual heart condition. Brianna has been putting in an amazing amount of effort into everything feeding and 9 months later it has finally paid off. Brianna has been so dedicated and determined to have Shirley feed by mouth. Please pray that Shirley continues to eat as she has been over the last week, indefinitely. There is certainly a chance that she will regress but we want to celebrate such a wonderful achievement

We are continually reminded that there is no exact schedule for Shirley's progress. We can look back on the last 9 months and see how this has helped us grow in patience and brought us closer together. We have not always responded to the stress appropriately but we have been brought closer together by every trial thus far and we look forward to growing together even more in the future. This is certainly not the last trial we will face with Shirley but we are confident that there will continue to be good that comes out of any trial.

Her is a picture of Shirley eating some fruit puree without a tube in her nose and irritating her face!!!!

Thanks everyone for your support and prayers!

-Joseph

Ummm...

Look at that beautiful face!

Changes

Wow! It has been a long time since we have posted an update! Life has been super busy with my new job and Brianna is always running around doing something for Shirley.

It has been a very interesting past few months for Shirley. As many of you know Shirley is a very poor feeder. She would drink an ounce or so from the bottle and on a really great day she would drink two…

NG

As time went on we just settled into life with a NG tube. Shirley wasn’t pulling them out as much and replacing them once a week rather than three or four times a week wasn’t that bad. Shirley’s reflux has calmed down dramatically over the last few months but she still didn’t really like to bottle feed. Sometime back in June, I believe, we began seeing a new doctor concerning Shirley’s feeding. The GI doctor was added on top of the PT, OT, and Nutritionist. We tried a bunch of different things including every bottle known to man. Shirley just didn’t want anything to do with them.

GI

We originally saw the GI doctor to get the ball rolling on the G-tube but she convinced us that it was not the best option for Shirley and that we should exhaust all of our resources before electing for a surgery. She was pretty convincing and we believed that Shirley could wean from the tube, but the last 6 months have been the same thing over and over. We try something new and give it time to start working and it never does. Shirley is just a stubborn little girl. About two months ago now we decided that surgery to have a G-tube placed was the best option. Surgery is now scheduled for this Thursday, December 7th. Brianna’s birthday is December 6th. It’s not really what she wanted for her birthday I don’t think. We met with her surgeon about two weeks ago and discussed the surgery’s risks and rewards. I was against the surgery for the longest time, for a lot of reasons, but mostly because of the risks. I don’t like the 2 or 3% chance of having complications. I don’t want to think about infections or side effects. I really wasn’t thinking much about Brianna and Shirley though. Brianna totes little Shirley around every day, pumps, attempts to bottle feed, attempts to breastfeed, and then resorts to tube feeding. It is non-stop for those two every day. Thankfully Brianna is a trooper but her and Shirley’s day literally revolves around feeding and often the lack there of. It is also not that good for Shirley to have a tube in her nose that runs down the back of her throat all the way to her stomach. One thing that sucks about it is that it holds her esophageal sphincter open making reflux more of an issue. It is also extremely irritating to have in her throat. She is probably used to it by now but the tube pretty much just moves side to side in her throat and stomach all day. It makes her gag reflex more sensitive as well adding to her spitting up those precious ounces of breast milk Brianna works so hard for. Lastly there is some concern that the tube affects her developmentally as fas as speech. It really is time for a change. I might be writing this just to convince myself.

Changes

We have tried two separate times to wean Shirley from the tube and she made it very difficult. The first time we tried the tube was out during the day and Shirley would pretty much go on hunger strikes taking at most 6oz the entire day. The day she took 6oz we were very excited even though it is only a 1/4 of what she needs to be healthy. We would put the tube in before bed and feed her straight water over night to ensure she was hydrated and then the tube would come out again. We only made it 2.5 days before realizing she wasn’t going to be able to wean. Our GI doctor even prescribed some medication to help her stomach empty faster and there was no change. I’m not a big drug guy but I would rather Shirley be on a drug than go under the knife for something. We made one final trip to the GI doctor last week and she wanted to try one last medication before the surgery. I almost laughed at her. We were exactly 10 days out from surgery and she is prescribing something new. We said we would try it though. Shirley pulled her tube out on Friday this last week so we said we would go the entire day and see what she could do. On her first feed she took 4oz from the bottle in under 30 minutes! We were shocked! Then she took 2oz her next feed and by the end of the night she had taken 17oz by mouth. We couldn’t believe it! Not wanting to get our hopes up we said that we would go another day and see what she ate on Saturday. Well much to our surprise she took 18oz by mouth yesterday and today she is on track to beat that. As of right now we are considering calling the doctors tomorrow to have them postpone her G-tube placement. Shirley has been tubeless for three days now!!!

We would appreciate your prayers over the next few days. Brianna and I want to make sure we are making the best decisions for Shirley and her health and we really would love not to have to go through another surgery no matter how minor it may be.

In other news, Shirley got to meet the doctor that broke the news to us that there was something wrong with her poor heart. Dr. McMahon was a huge blessing to us during that very difficult time. From the very beginning she told us that God had plans for Shirley’s life and that she was beautiful and special. We believed those things as well but we didn’t expect it from the random, on-call, doctor that day. She took us under her wing immediately and told Brianna without hesitation that she would now be her primary physician. We got a photo of Shirley and Dr. McMahon the first time they met just about two weeks ago. I’ll put the photo in here. 

Shirley was also on the news again just before Thanksgiving and I’ll attach that here as well. 

Thank you everyone who has been following Shirley and praying for her and our family. We appreciate all the support. Shirley Anne is a beautiful reminder of how blessed we are to have such a wonderful community of people in our lives. I can’t imagine what it would have been like without your support.

-Joseph

Shirley on TV

A while back Q13 interviewed us and got some great shots of Shirley. That interview is supposed to air tomorrow on Q13 at 4pm and then again at 9pm. Brianna and I haven't seen the interview yet so we may look like complete fools but thats ok! Our interviewer, Marni Hughes, said that the shots of Shirley were great.

If you miss the interview on TV it will be available online and I'll put a link in this post when I get it.

I took a ton of photos of Shirley in her highchair the other day and they turned out ok... 

-Joseph

Shirley's Story on Q13 Fox: Part 2

After our original interview Q13 said that they would like to do a follow-up interview and check in on Shirley's progress. Now that Shirley is 6 months old we thought that it would be a great time to do the follow up. We are very thankful to have the opportunity to talk to others about Shirley, CHD, and the joy she has brought to our lives. We don't know exactly when it will air but you can check back here and on Instagram for updates. It will mostly be tomorrow or the following day but we''ll have to wait and see.

Thanks again to everyone who has been praying for Shirley and supporting us.

-Joseph 

Virtues

Life is crazy and life is hard, but is that a bad thing?

 

Virtues

Brianna and I knew that life with Shirley would bring challenges that the average new parents wouldn't have to face. Even though we knew that, there was no way we could have been prepared for or even known about half of what it would actually require from us. Recently I have been thinking a lot about how I think about the challenges that have arose from having such a wonderfully different child. Shirley's care is very demanding and sometimes it simply feels unreasonable. Her care takes a lot of effort. Shirley has at least one appointment per week and often times up to to three. She has a cardiologist, gastroenterologist, pediatrician, nutritionist, physical therapist, and a speech-language pathologist (our feeding therapist).  We also have a medical supply company and pharmacy that we are constantly in touch with to ensure we have Shirley's everyday essentials met. When Brianna isn't busy with one of these appointments or phone calls she is all of these things to Shirley every day. The Dr(s) and therapist don't care for Shirley they really just monitor and give advice. Brianna actually has to implement these things in her everyday care.

Brianna gets up multiple times a night to pump and store milk, heats the stored milk, loads the milk into the pump, starts the pump, an hour later shuts off the pump, flushes Shirley's tube and then goes back to sleep for a few hours before repeating the same thing. That is all done while Shirley is asleep... Around 6am Shirley gets up for the day, and if the excitement of waking up doesn't cause her spit up her entire previous feed she is having a pretty good day. She gets meds at 6am and 9am and a feed somewhere in between. Each feed while Shirley is awake starts with trying the bottle. Heat the bottle for 5 mins just to have Shirley reject it almost entirely, now that you've devoted 20 minutes to feeding orally she has to the be tube fed in order to get the majority of milk. You can't tube feed her without it being a two handed effort so now you are about 45 minutes into feeding her. You can't move her much within an hour of being fed because of her chronic reflux and the worry of her spitting up all over herself and Brianna. By the time she is done eating its nearly time to pump again, or time for an appointment, or time to pick up a prescription, or time to go shopping, or time to get something done around the house, or simply time to take a 10 minute break and catch your breath. 

Brianna will tell you that I can't really stand complaining... and as I write these things I think I am realizing how little I understand how much work it is to care for Shirley every day. I have never had to spend a significant amount of time with Shirley alone and quite frankly I'm not sure how long I would last. I know I would be capable but I think I would be a compete mental wreck after the first day. I don't really have a major role in Shirley's care until I get home from work and as most people can attest the last thing you want to do when yo get home from work is feel like there's more work to be done... But there is, and its not a bad thing. Thankfully Brianna and I can double team Shirley's care on the weekends. 

Now if you think that sounds like a bunch of complaining it wasn't intended to sound that way. We love our life and we love having Shirley here with us! We were just talking about how good we have it in the greater scheme of things. Shirley Requires a lot... but man is it great being her parents. She is so wonderfully unique, so happy, so cuddly, so content, and so darn cute its almost irresistible! Right now as I'm typing this Brianna is tickling the crap out of her and she is just laughing it up, smiling and holding her feet with a face that seems to relate that she doesn't want you to stop!

The whole reason for this post was a thought that has been creeping into our minds recently. That is, that we just wish it were easier, or that we could skip ahead to when Shirley doesn't have a tube and its not as difficult to care for her. When I really start thinking about those thoughts though, as I have been over the last few weeks, I can't help but think that by simply thinking that that we are missing out on a great opportunity. We accomplish nothing by wishing it were easier or hoping the time passes quickly to when she will need less hands on care. Instead we will miss out on the opportunity to grow as people and parents. Isn't patience a VIRTUE? Doesn't hard work build character? In fact what many people consider to be virtues (diligence, patience, charity, humility, temperance, and kindness) are all things Brianna and I have to gain from the apparent difficulties of Shirley's care. By wishing it all away and viewing some tasks as necessary evils we gain nothing... We have everything to gain however by accepting the challenges placed before us and taking advantage of the opportunity we have to grow. Above any of the worldly virtues to be gained from our circumstances we have the opportunity to build our faith as believers. Our faith has certainly been tested and we have both grown and we look forward to the opportunities we will have to grow as life continues on in whichever direction it goes.

So rather than complain about things, we want to see all things as opportunity.  Though sometimes it will be easier said than done, with only a little foresight it can clearly be seen as the best choice.

Medical Update and Major Gains

Shirley weighs in at over 13lbs and looks like a very plump and proportionate baby. She is a tiny little girl according to the stats though. She is in the 6th percentile for weight and the 1st percentile for height among her peers. Her height to weight ratio is very good though, somewhere in the 60th percentile. We were also able to start introducing some solids into her diet. I've always struggles calling mashed up nearly liquid food "solids" but I guess anything that consist of more than milk, water or juice can be called a "solid." 

Her last cardiology appointment went well. She had an echo and everything was looking great. Her function is so good currently that they have begun to decrease her drug schedule. Within a few months if everything goes as planned they would like to have her on only one medication for her heart function. She will still be closely monitored but it feels like a huge step in the right direction. 

Brianna has been doing a ton of research on how to wean Shirley off her tube and we have been having an ongoing discussion with her feeding therapist, GI doc and cardiologist about how to do it well. Its not very easy to make an infant eat orally that has never had to rely on eating orally for nutrition. Shirley knows pretty well that she will be fed by the tube no matter how much she takes by mouth... because of that most people say that kiddos in her shoes can go days without actually having normal hunger cues. It is a little daunting to think of taking Shirley's tube out and more or less forcing her to eat by mouth, but it is really one of the best ways to get her away from being tube dependent and eliminating her chronic reflux. We are pretty confident, though, that Shirley will be able to pick up the skill and within the next few months its our hope to get rid of the tube once and for all.

She does have one new medication being added but its not for her heart. The GI doc wants to try one more med to help Shirley's stomach digest more efficiently before we attempt to wean her by simply taking the tube out. For whatever reason children with heart condition like Shirley's have what is called delayed gastric emptying... pretty much sums it up in the name. Because her stomach isn't doing its job as well as it could Shirley's already insufficient hunger cues are hampered even more by the fact that her stomach is never really that empty. Maybe this treatment will help, maybe it won't, but ether way we think she will be tube free in the coming months!

We have a ton of Shirley pictures and videos to post so... yeah... feel free to marvel at her cuteness.

-Joseph

Reflux Sucks

Cardiology

Shirley's heart is pretty much the same as it has been. Her medication doses were increased after her last appointment because of her growth (12lbs 3oz!). She will have another echo at her next appointment. If all goes well, Dr. Penalver mentioned that they will be toying with the idea of weaning her off some more of her medications. Obviously, it would be ideal for her to be weaned from all of her medications, but we would would be excited about even a slight decrease.

GI and Feeding 

Shirley is still not feeding much better than she was before. She has good days and bad days. Although my milk supply has come back up, she is still not interested in breastfeeding. We've had two visits to SCH GI clinic in Federal Way with Dr. Len. She thinks Shirley looks too good, and feeds too well, to justify placing a G tube. Since Shirley has no oral aversion, and she's a chubby baby, Dr. Len wants to exhaust all options before ordering the procedure. She is quite convinced that Shirley's issue with feeding stems from chronic reflux, and not low energy or a physical inability to feed. So now we're trying to tackle her reflux before trying anything else.

At our first appointment Dr. Len noticed mucous in Shirley's stool, so she suggested we cut cows milk protein out of Shirley's diet (which meant my diet too). Since Shirley has been on fortified breast milk, they switched her to a peptide based formula that's more broken down and easier to digest (still derived from cows milk... which confused us a little). She also prescribed a reflux medication. It's been about 4 weeks since that first appointment and we haven't noticed much of a change in her feeding, but she is spitting up much less. At our most recent GI appointment, Dr. Len was glad to hear that about the decrease in spit up, so she switched Shirley's feeds to plain breast milk, but added about 7oz total per day. This is a huge step! Especially since the new formula tasted horrible and Shirley wanted absolutely nothing to do with it. Although we are excited about Shirley being fed straight breast milk, the increase in volume will give Shirley even more catching up to do in terms of oral feeding... but we'll celebrate our small victories when we can!

Looking Back 

People are always asking me how Shirley is doing. My most common answer is some form of "she's fine, but she still won't eat anything." I say this partly because I'm too lazy to elaborate, and partly because I've been totally obsessing over her feeding issues, so it's really the only thing on my mind. What a bummer of an answer! Although it's essentially true, it's not the whole story. 

Shirley is doing remarkably well. We are all doing well. I have to keep reminding myself of this fact. I am often so frustrated and worried about the slow, almost non-existent, improvement in her feeding that I forget how far we have come. I'm so busy wishing for at least a semblance of normalcy, when I should be relieved that oral feeding is my largest concern. I constantly have these thoughts bouncing around in my head "What if I'm not trying hard enough? If only I had pushed her more when she was younger! Is she too old to learn to breastfeed now? Will she ALWAYS need a tube? What if some other woman were her mother? Would she have been able to get Shirley off the tube and feeding by now?" They repeat themselves over and over, one thought leading to the next. I know they are not healthy, but they are my thoughts all the same. Before writing this, I spent an hour or so reading over our past blog posts when Shirley was in the hospital. Most of the posts brought me to tears... and it helped me to see just how well Shirley is doing these days. She is growing, she is happy, she is deliciously chubby, and she is loved. 

Joseph and I have come a long way as well. Dosing and administering medications has become second nature, prepping her food and feeding her isn't an ordeal that takes an entire hour anymore! We're no longer jumpy and overly anxious whenever Shirley is unhappy, we know that she is a baby... and crying doesn't always mean something terribly scary is happening. We have come so far, and there is still a ways to go. We are learning, we are growing, and I am so thankful for that.

I constantly feel inadequate. I believe I am always going to feel inadequate, because the truth is, I am. Nevertheless, for some strange reason God chose me to be this sweet little girl's mother and I am confident that He will equip me to do the job.

-Brianna

Cute photos below!

These Days

A New Normal

With Joseph being back at work and a lot busier than he was before, I (Brianna) thought I'd take the time to post an update. I know you have all gotten used to Joseph and his writing style, but we're probably going to tag team the updates from here on out. His posts are going to be a tough act to follow... so bear with me. 

Things have settled down and we've begun to feel like we are living a new kind of normal. We have gotten pretty good at packing everything but the kitchen sink when we go out (you'd be surprised by how quickly we can load up and go!). I've even gotten comfortable taking Shirley out alone, on a walk, to coffee or lunch with a friend, things are just getting a bit easier. We've gotten used to strangers asking questions about her NG tube. Although these questions can be repetitive, they have been the start of several surprisingly meaningful conversations with people we've never met before. 

There are moments I find myself forgetting about Shirley's heart condition entirely, which is something I did not think possible just a short time ago.

Struggles 

We are having a difficult time waking up for her middle-of-the-night feeds. Shirley sleeps through them, so we don't have a crying baby to wake us up. We have accidentally slept through them right along with her more times than I'd like to admit. I usually wake up frantically pawing around in the dark for my phone, only to find that it's right next to me and that I must have silenced my alarm several times in my sleep. Then a wave of guilty thoughts rushes into my mind "she's probably starving!" "Do I not care about her enough to wake up for her?!" Although powerful in the moment, these thoughts are fleeting and usually do not last until the morning. 

Shirley has not been improving with oral feeds. For a while she was very interested in breastfeeding, but she has recently decided that she doesn't like it so much anymore. The fact that my milk supply has been rapidly dropping ever since her discharge from the hospital is probably a contributing factor. Bottle feeding is slow going as we work with the feeding therapist to find the right flow rate and nipple shape for Shirley's comfort. We want feeding to be a positive experience for her, so we can only push her so far before she gets upset or overtired. We give her as much as she will tolerate by bottle, and then feed the remaining amount through her NG tube.

Appointments

Shirley doesn't have as many appointments as she had the first several weeks after her discharge. She has in-home physical therapy and feeding therapy appointments that rotate every Monday. The therapists are wonderful! They have been so encouraging to me and I can tell that they truly care about Shirley.

We had a cardiology appointment last week with Dr. Penalver at the Tacoma SCH cardiology clinic. I had seen him once before when I was about 30 weeks pregnant, so this was the first time he officially met Shirley. The appointment went well overall; Shirley was a champ, smiling at everyone! Although she is still quite small, she is healthy and gaining weight (11lbs now!). She had both an EKG and an Echocardiogram done; both had positive results. Her heart hasn't changed much, and any slight changes have been for the better. 

Because of Shirley's difficulties with oral feeding, both Dr. Penalver and her dietitian (Tran) mentioned the possibility of getting a G tube placed instead of her NG tube. A G tube requires a small surgery to place a tube from the outside of Shirley's abdomen directly into her stomach. It has a small button that can be opened for feedings and closed when not in use.

Initially, this would seem like a step backward, but it really isn't. There are several issues that could be resolved by switching the NG tube out for a G tube. First, the NG tube holds Shirley's esophageal sphincter open, which causes reflux and throat irritation; both make it more difficult and uncomfortable for her to feed orally. Second, the NG tube also causes Shirley to have a heightened gag sensitivity, since the tube is hovering at the back of her throat; this also makes oral feeding more difficult. Third, the adhesive used to keep the tube in place on Shirley's face really irritates her skin. It is an obvious discomfort for her, and the older she gets, the better she is at ripping it off and pulling her tube out. For the past several weeks we have considered ourselves lucky if we could keep a tube in for more than 3 days. Although we don't like the idea of Shirley going through another operation (albeit a very minor one), the NG tube has been such a headache for all three of us that the idea of a G tube is beginning to appear like a breath of fresh air. Also, selfishly, I would like to see Shirley's beautiful face without that pesky yellow tube getting in the way!

We have not made a definitive decision to move forward with the G tube yet. We're going to see if her oral feeding improves over the next couple weeks and then make a decision.

Meds and Labs

The other positive thing that came from her cardiology appointment was that she was taken off digoxin. She is now only taking three medications multiple times a day. Digoxin was never meant to be a longterm treatment for Shirley's heart failure and because it is by far the most dangerous drug with the least benefit, we were all itching to be done with it. If you don't remember digoxin is the drug synthesized from foxglove. Shirley also had her blood drawn last week and this revealed even more positive news. If you recall a post a while back about BNP you will be glad to hear that Shirley's BNP levels are at 63. Thats down from about 3500 at its peak in the hospital. It is still high, relative to the average child, but its not that bad for a child with heart failure! It also confirms that she really didn't need to be on digoxin anymore and that her current meds are doing the job just fine.

Shirley is still the happy, chubby, sweet baby she's always been. See pictures below:

-Brianna

Breakthrough

Best Yet

Shirley is breastfeeding like a champ as I type this! She has been at it for a solid 20 minutes and doesn't show signs of wanting to stop! Brianna has been working so hard with Shirley ever since she has had the opportunity to attempt nursing around 3 weeks old. It has not been easy. There have been a lot of tears and mixed emotions surrounding nursing over the last two months but right now it's euphoria. For a mom who wants to breastfeed, not being able to do so is extremely challenging. There have been times that Brianna wanted to throw in the towel. Failed attempt after failed attempt took a toll on her but she never stops trying and it paid off big tonight! Hopefully this is just the first of many nursing sessions for this little girl and her mom.

Other Shirley News

She is a whopping 9lbs, 8oz as of her last appointment! That is a full two pounds more than her birth weight! When we first got home from the hospital Shirley was just about 8lbs and during the first few weeks we could literally feel her gaining weight everyday! The doctors want her to pack on as many pounds as possible. In the event that something unforeseen happens, they want her to be stout and able to handle whatever intervention she may need. Each Dr. appointment goes better than the last it seems. Each cardiologist raves about how great she looks in light of her heart failure. There was also some concern about her hormones being out of whack but the endocrinologist ran some tests and said that she has no concerns for Shirley and doesn't expect we will need to visit her again! 

Shirley was enrolled in "early intervention" which is a program for children that helps with developmental delays. Because Shirley spent so much time on her back the first few weeks of life she still has rather poor head control and will behave much like a rag doll if we don't support her. She is also going to get some help with her oral skills due to the fact breast feeding has been such a difficult task for her. It's unlikely that Shirley will have any pressing developmental delays and even if she didn't go to her little appointments she would probably be just fine. Cognitively, Shirley seems to be doing very well. Her drugs can make her drowsy at times but when she is awake she is alert and very interactive! 

New Work

We have been very busy over the last few weeks and its been hard to do much writing. I started a new job a few weeks back! No more UPS!!! No more brown! No more packages! UPS was a great job for what it was and I'm very thankful to have had the opportunity to work there. UPS bought us our house, put food on the table, and paid our medical bills. I didn't love my job but that didn't matter. Our needs were met and sometimes thats all you need to get out of work. 

During the majority of Brianna's pregnancy I was interviewing with different local law enforcement agencies. The week after Shirley was diagnosed I had an interview with the City of Federal Way. The interview went well and a few weeks later they called me in for a background interview. A couple weeks after that I was sitting with the Chief of Police and some of the command staff. My first interview was Oct 10th and I had an offer of employment on Nov 29th. I related the complication of Shirley's condition to them very early on and they were more than willing to work around Shirley's birth and hospital stay. Four and a half months after being offered a position I started as a recruit police officer. There were a few times I thought that I was making a mistake and being irresponsible by leaving my job at UPS during such a critical time in our lives but I'm very glad I did. It wasn't ideal quitting while Shirley was in the hospital recovering form open heart surgery but sometimes you just have to take risks. The Federal Way Police Department has been unbelievably supportive of our needs over the past few months and I couldn't be more excited to start a new career with such a great agency. 

Brianna has been a trooper taking care of Shirley on her own over the last few weeks. It certainly hasn't been easy for her but She really is doing a remarkable job! 

Not sure how many of you saw this but Jimmy Kimmel recently had a son the was born with CHD his announcement on the show was tear jerking. 

Thats all for now I think! Enjoy this little video of Shirley being adorable!

-Joseph

Poets and Puppets

Mystery

Time and time again since Shirley's birth I have marveled at the immense amount of joy I get from being a father. Maybe someone can explain it to me better but I've been thrown for a loop thinking about what love is and how it comes about being manifested in our lives. I love Shirley; but why do I love Shirley? It certainly isn't as simple as the fact that she is my blood daughter. When I look at her I have a sense of joy but it is so hard to understand from where that joy spawns. Looking at her may trigger something in my brain which produces a response that correlates with joy and feelings of love but why it does so is a much harder thing to understand. Obviously, I have a knowledge that Shirley is my daughter and that might shed some light on why she evokes such a strong response but even that doesn't seem to be enough. There must still be something that transcends simple knowledge, the senses, and chemical reactions. Something truly metaphysical, producing in me the the foundation that links these things together and enables me to having feelings of love for her. The same can be said for my wife. Beyond simple physical attraction there is something much deeper that evokes a sense of love for her. There is something immaterial that I can't explain that must be at work. To chalk it up to instinct seems like a bit of a cop out. That it might be evolutionarily favorable to have feelings of love towards my family is equally weak in my book and if it were the case it's certainly not the least bit romantic. I would like to hear a lecture on how the idea of romance evolved. Instinct is just another way to say, "we really don't know why such and such a thing happens." That was a long rant that I'm not quite sure has a place but it is something I have been pondering over the last few weeks.

Poets

I don't think I'm that much different than the average guy and I would be willing to bet that many men have fancied themselves poets in the face of love. For us that have no real knowledge of how to craft a poem, nor the vocabulary to express our feelings, we are left to daydream and let our imaginations be our pens. It is the worst kind of poetry, though, because it is so hard to replicate what has been imagined and materialize it for the object(s) and inspiration of that affection. I have written multiple letters to my wife most of which were penned before we wed. Shirley also has a little notebook, with her name on it, that I have written in for her to read when she is able. Also, most of which I wrote before she made her entrance. There are so many times, though, that my mind has been overrun by the most poetic and romantic little daydreams. Things that will never make it to paper. I am not even that romantic. I'm a lazy romantic at best. Ask Brianna how romantic I am. She probably won't have much to say... Most of my romance is wasted passing time behind the windshield of UPS truck. Brianna is always asking me to write her letters like I used to. She wants more of the romance from our pre-marriage days. I bet this is common of many relationships. The romance is still there though and often times the romance was inspired by time apart. Brianna and I might as well be attached at the hip these days and the time apart is hardly time that I have for picking up a pen. I wake up every day next to the object of my affection. She is not far off, she is not out of reach. The depth contained in the the phrase, "good morning, I love you," is very great and not understood well enough. Nor is a something seemingly as simple as kiss on the nose before getting out of bed to start the day. I don't really like hearing, "tell me you love me." Don't get on my case too much for this, but I think the best reply is, "you know that I love you." I generally disagree with the idea the the word love is misused. Often times people point to the fact that Greek had four unique words for love to show that we have no idea what the word love means anymore. I was never a fan of that either. Context has always been more than enough for me. When I say, "I love food" it should be obvious that it is nothing like when I say, "I love my wife." All that to reenforce "good morning, I love you," as something much more than superficial. Though it is a small thing, it lacks depth only if you choose not to look at it closely enough. Like a cell under a microscope made up of many organelles, made up of many proteins, made up of many atoms, made up of sub atomic particles, and who knows what else. If I could only get my wife to look at it that way... I don't think she would ever ask me to tell her I love her again. She may, every now and then, think about it that way and hopefully she will smile a bit to herself with the knowledge that I do love her and the same goes for Shirley. I'm also certain that she will ask me to tell her again and I'll be able to smirk and tell her "I love you. I do. I do. I do."

Puppets

There is also this sense of being a puppet on strings, a marionette, dancing about at the hands of my master(s). In my case, Shirley and Brianna. It is probably true of many love relationships that there are certainly times you find yourself acting in a way that does not resemble your normal self. It's not a facade, not the real you; it's an inspired you, that needs some tickle of romance before, like a peacock, you will show your feathers. Isn't it kind of funny that in the world of birds the males are the pretty ones. Shirley and Brianna both have, at times, seemed more like puppeteers in a non-manipulative way (if there is such a thing). Nevertheless, its a wonderful feeling being at the whims of love. Romance, I think. is the real mystery that I have been trying to plumb and I don't expect that I will ever know its depth.

Wow

It has been a long time! It took me days to write the last few paragraphs in between feeds, drugs, dishes, diapers, cooking, cleaning, shopping, appointments, and the bits of sleep we get. We are still alive, albeit sometimes it doesn't feel much like life. If I'm not getting enough sleep I can be a monster and in the last few days the long nights caught up with me. I am a pretty easy going guy and I often have a good deal of patience and do not get frustrated easily. Over the last few days though I have found myself apologizing to Brianna many times over. I'm probably more like a child that is in desperate need of a nap. Brianna has had bountiful grace for me, though, and it is a good insight into myself that I often times forget about. I have been thinking about how to take those moments captive, when I know I'm tired, and instead respond in a way appropriate for the situation, refraining from making a difficult time more difficult. I hope that what I wrote earlier made some sense or that it resonates with some of you.

-Joseph

Good News

Routine

We have been home for a few days now. Shirley is one time consuming child! With her current feeding and med schedule it’s more or less impossible to string together periods of sleep longer than 2.5 hours. Stumbling through the house at 3am to prep milk and draw meds is almost comical. I’m very serious about her medications and somewhere deep in my subconscious I have an irrational voice that tells me I am the only one that can do it correctly. When 3am comes around though I’m probably no better than mole that has wandered to the surface (click here for a visual). Once I’ve made it to the kitchen I go up to the fridge and look carefully at her schedule posted there. I wait as long as possible to turn on the lights but I don’t know why! I have yet to draw her medications in the dark and I could probably save some time by flipping the switch earlier but instead I wait until I’m about to draw her med and then turn the lights on to ensure I’m dosing the right one. Now that I have completely destroyed my night vision I go back to her crib and search about for the NG tube. It really would make for a funny video. It’s a ridiculous process that must be undertaken to really understand why it’s funny! If you imagine myself as the aforementioned mole it really is the best way to read this first paragraph. We love this time at home though, when we go to bed and look back on our day we just smile to ourselves and think, “what an interesting, crazy, life we have.” Brianna and I started to talk about having kids probably around week two of our dating relationship and this is certainly not what we had in mind. Life rarely goes as we expect though and the ability to adapt to the reality of things is very important. Not putting too much stock into your expectations of the future is something I have learned the hard way a few times but I have never been disappointed by the outcome. We love our life and we love caring for Shirley, no matter how time consuming it may be. I hope you are not reading negativity into the phrase “time consuming.” I can’t see anything wrong with the fact that our time might be consumed with our daughter.

Adventures

Going anywhere is an adventure. We have ventured out a few time over the last week and each time has presented us with challenges and obstacles. Two of Shirley’s medications must be kept refrigerated. That, along with the breast milk for feeds requires carrying around a small cooler everywhere we go. Our diaper bag has all the normal stuff parents might pack for the day and topped off with syringes, large and small, a stethoscope and other random medical supplies required to adequately care for Shirley. We tried to go to worship service on Sunday and we made it there but after a big spit up in the car, changing clothes and diaper, feeding, and giving meds we spent a total of ten minutes in the actual service. It gave me a whole new understanding for why some people with small children might just stay home some weekends. Nevertheless we were glad we went. Our church community has been so supportive throughout this process and many people were itching to meet Shirley. Unfortunately, I have no idea what the sermon was about but we certainly heard the Good News.

We have gone out a few different places and each time has offered us a new scenario to learn from. The other most notable trip was to Seattle on Sunday afternoon. Many, many people have offered us televisions and dvd players to replace ours. Friends, family, and family friends alike have offered to replace the stolen goods. One gentleman even offered us his entire entertainment center complete with TV, Apple TV, Blu-ray player, sound bar and cabinet! We don’t have room for anything like that so we respectively declined not wanting to cherry pick the man’s goods. It was quite the offer though and we were very humbled. Instead though we drove to Seattle to meet with a wonderful couple (the Kirby's) that contacted Q13 after seeing our story on the news. They were wanting to just drive to the hospital and drop off a brand new television they had acquired but instead I convinced them to at least get coffee with us after we were discharged. So on Sunday we packed Shirley up and headed to Green Lake and had coffee with the Kirby’s. We had a wonderful time meeting these complete strangers and talking for a couple hours before loading up the car with a new TV. We didn’t really have any way to decide who to accept replacement goods from, but after the Kirby’s contacted us it was a no-brainer for me. Shirley’s life has put us into contact with so many people that we wouldn’t have met outside of her being with us. I love meeting new people and to have complete strangers be compelled to reach out in generosity towards us was something I didn’t want to pass up. 

Medical

Shirley had her first follow up appointment with the HFT at Seattle Children’s today. It went surprisingly well! Her echo revealed that her heart function had increased slightly from the previous echo. The doctors were pleasantly surprised and said that they don’t think that she needs to be monitored as closely as they originally thought. So now, instead of going to Seattle for appointments with the HFT we can simply drive down the road to a SCH satellite cardiology clinic in Tacoma! Shirley is also gaining weight consistently and we can see the pudge being added to her legs and cheeks. It’s very cute.

We may never be back to life as normal but we are finding a new normal and that is ok by us.

Thanks to everyone that has helped with meals since we’ve been home! It made adjusting to our new routine a little easier without trying to fit meal prep in between everything else.

-Joseph

Homebodies

First Night

Shirley had a great night at home. It was a busy night for us but Shirley slept the majority of the time. Her med schedule is pretty intense! There are 7 different med times between the hours of 3am and 10pm. We have been giving meds for about a week now but yesterday was the first day we had to prep her meds. Being that it was our first time dosing her drugs, it took a while but I think it will get easier as the days go on. On top of her med schedule she has a pretty strict feeding schedule. Shirley still doesn’t feed very well at the breast but she is working on it. Including breastfeeding there are 4 ways we feed Shirley. I’ll try to get a picture of her gravity feed and pump system. Every three hours we pick a method, sometimes two methods, and make sure she gets her nutrients. Hopefully Shirley will begin to put on some serious weight and take the majority of her feeds by mouth. Once that happens the NG tube will come out and feeding will feel much more natural. It’s kind of strange but we have to haul around all of these interesting things if we want to go somewhere. I have a feeling we are going to be homebodies for the next few months. Who wants to set up an IV stand, hang a bag, load the milk, prime the pump, survive the 30min run time all for a 12oz cup of coffee? Not me! I think it will probably be a circus trying to get from point A to point B but who doesn’t like a circus??? Always something interesting going on and you may even laugh. No doubt we will be the spectacle of many a people watcher.

Sleeping in your own bed, assuming you like your bed, for the first time in 5 weeks is an eye closing experience! Even though we didn’t sleep a ton last night, the sleep we did get was fantastic! I can tend to be a picky positional sleeper, rolling from side to side, flopping back to front, but last night it didn’t matter! When my head hit the pillow, no matter how contorted my body was, I was out cold. I was a little scared when I woke up thinking about how poorly I had actually been sleeping over the past month or so! I think it also had to do with simply being in the comfort of our own home again. The other thing that worried me when I woke up was what the heck I was going to eat! I haven’t prepared my own meal in the last five weeks, breakfast lunch, or diner. After writing that I ALMOST (keyword) feel a hint of shame. The food options at the hospital were phenomenal. The fear didn’t last however, I made a few simple breakfast sandwiches comprised of a piece of Dave’s Killer Bread (DKB), avocado mayo, spicy kimchi, and an over easy egg. I’m not sure how that combination of ingredients came into play but I’m glad to say that it works well.

Waking up this morning was many years of dreaming come true. Brianna got Shirley out of her bedside crib and had her all snuggled up and for the first time we had a morning in bed as a family. Having little Shirley close at hand and being able to doze off then wake up and smile at my wife and child was a fantastic feeling. Marriage and now being a parent have certainly yielded my happiest days, as I often thought they would. I look forward to more of the same.

BTW Shirley’s instagram is up and running! There will be a separate link under the news tab that will display photos from instagram. So if a few days pass and you need a Shirley fix, there may be little piece of info attached to a cute photo of her there. You can follow her @shirleysgrandadventure 

-Joseph

Home Sweet Home

Home

We are finally home! Our wonderful family and friends cleaned our house from top to bottom. There will be a lot of adjusting but it will be good. I could go on and on but I think that keeping it short is best.

We are thankful to have Shirley home. Thank you all for your continued prayers and support. This is only the beginning of Shirley's Grand Adventure! 

Tonight we will sleep in our own bed with Shirley under the same roof. 

-Joseph

Meds and More

Managed

Shirley started a new medication to help manage her heart failure long term. Digoxin has been working well so far and she will continue to receive it for now but she was given carvedilol for long term use. You can read up on it if you want but the hope here is that with years of use it will help her heart function return to normal by a process call "reverse cardiac remodeling." I have no idea how this actually works but there are multiple studies that show heart function returning as a result. We really like the primary heart failure doc we have had so far and she is very hopeful in regard to Shirley's condition. The first time we met her she talked so much about nutrition and breastfeeding that I thought she was a nutritionist. It wasn't until halfway thorough our conversation that I started to question how this nutritionist knew so much about meds and heart failure. 

Close

We are almost out of here! As of today are expected discharge date is 3/29! That is this Wednesday! Brianna and I are almost all trained up on Shirley's daily routine! She will be going home on lots of meds that we will have to administer multiple times a day and with a NG tube. We have learn how to place the NG tube also. It's really not a fun process getting a tube from a child's nose to the their stomach but it can be done with tears and crying from both parties. Shirley isn't taking her feeds entirely by breast or bottle as we hoped she would but we will continue to work on that at home and she will be off the NG tube soon enough. We sat through infant and child CPR today and it seems very ironic sitting there learning chest compressions while thinking about sternal precautions for your child that just had OHS! I laughed to myself a few times. We are very excited to bring little Shirley home.

Robbery Update

There was some initial communication with TPD in the days following the break in but it never amounted to anything. I have been leaving messages every now and then and I tried one more time today. I was able to talk with someone and they put me in contact with a detective and someone from forensics! Unfortunately, I don't think they will find much evidence in our home now that it's been nearly a month and a lot of stuff has been picked up and cleaned since then. Who knows? 

-Joseph

Outside

Today wasn't very eventful in regard to Shirley's medical condition but it was a great day. We now have a room all to ourselves (neighbors went home) and Shirley's bed gets natural light for the first time. Out of the blue the nurse asked us if we wanted to take Shirley on a walk! Shirley got to take a stroll around the hospital, made a Starbucks run (there are three in SCH), and went outside into the fresh air for the first time! 

This is the kind of update that I'm thinking will be better for Instagram. 

The natural light really brings out Shirley's highlighted hair as well as her blue eyes. Not sure if either of those traits will last but they sure do make her look cute right now.

-Joseph

Practice Makes Perfect

Feeding

Shirley has been getting consistently better at eating by mouth. Her feeds are so condensed now that what she used to take in over the course of three hours she now receives in 30 minutes. The OT/PT team has been working hard on her oral skills and three times a day Shirley eats by bottle, if she is up for it. The last three days have been hit and miss as far as her actually getting a sizable portion by mouth but today we saw some real progress. She drank 25ml from her first bottle this morning and 35ml from her bottle this afternoon. The OT that swung by this afternoon was shocked and was probably more excited than we were. It was difficult to get her to take 10-12ml by bottle the last few days and even those feeds were celebrated. If Shirley can keep it up she won't need to go home with an NG tube placed. We are confident that she can do it.

Next Steps

Thankfully there hasn't been much to report concerning Shirley's condition in the last few days and we hope that is a continuing trend. Shirley is still monitored very closely and tomorrow is lab day... not her best days in the past. Shirley is very hard to get blood from for whatever reason so let's hope tomorrow goes smoothly. Labs will tell us a lot about how she is responding to her medication and if it is going to be something viable for us to continue at home. The doctors expect that it will be. She will be starting another heart failure drug in the next few weeks that will also be a longterm addition. Our days here are coming to a close and you can almost feel it. The D word was used this morning (discharge), but with no definitive date attached. There is a lot to be done before we can walk out the doors with Shirley. Brianna and I will both have to complete infant CPR classes and Shirley will have to pass the "carseat challenge." The test involves securing Shirley in her car seat while in the hospital room hooked up to all the monitors. Shirley has to prove that she will be able to handle the trip home. Two hours seems like an awful long time to have her in there but I think it will go fine. She sleeps so much that I can't see her being awake for the majority of it anyway. Hopefully I don't have to eat my words.

Insta

Yep, I'm so hopelessly obsessed with taking pictures of Shirley that I've decided to take it to the next level: Instagram. That is only a part of the reason. Although I plan on keeping this site and blog updated, I may start to spread them out over a few days or weeks. A lot of people follow Shirley's progress and Instagram may be a better way to do short updates. Shirley will be in and out of the hospital and cardiology clinics her entire life and we hope that documenting it will provide hope to other families that find themselves in similar circumstances. We have been greatly encouraged by others and we can only hope to do the same. 

-Joseph

One Month

Fast Month

Today marks a month with our little Shirley. It was a quick month, literally and figuratively. February 23rd to March 23rd is exactly 4 weeks. I know there are some people that calculate months in terms of weeks but I can't really make any sense of that. Children born in February on a non-leap year have a short first month (4wks) but after that it's simply back to however many days make up a calendar month. I know there are people out there that say pregnancy is ten months but just because it's 40wks doesn't make it ten months... The same logic would yield a 13 month calendar year... Seems more than a little fishy to me. Sorry for all this nonsense. It really has seemed to have gone by quickly for us. Hard to believe that we checked in 4wks ago with a cone-headed little Shirley. It has been such an amazing month! So many blessings, so much love. We have many people to thank.

Easy Days

I hope every day from here on out is as uneventful and enjoyable as the last few have been. We feel an awful lot like average parents. We didn't get much sleep the last couple of nights due to the simple fact that little Shirley needed a little attention. We aren't the "cry it out" type so we just took her cries as wonderful excuses to get up and soothe her back to sleep. She is a very cuddly little baby and we are very cuddly parents. It works out well! Now that we are in the step down unit there is much less supervision by nurses and the docs only come in periodically to look at Shirley. Brianna and I pretty much get to hold Shirley all day long, give her bottle feeds, change her diapers, and dote.

Pastime

I thought reading was going to be the pastime most associated with our stay here but it hasn't. This blog has certainly taken up much of my time and I'm very thankful for it. Also, Brianna and I go on three dates a day: breakfast, lunch, and dinner. We are spending so much time together and we love it. Before Shirley was born we pretty much did everything together besides go to our separate places of work. For the last 4 weeks we haven't spent more than two or three hours apart and the vast majority of days we don't spend more than 5 minutes apart in a 24 hour period. Brianna and I truly are the best of friends and we rarely look for opportunities to "get some space." Lots of time is spent talking with Shirley, taking every opportunity to bond with this sweet girl. I must admit though that we have binged on rerun episodes of "Forensic Files" late at night while Brianna pumps. In the last few days I have been glued to the senate confirmation hearings for Judge Gorsuch! It obviously hasn't been all roses and butterflies though. There have been some very difficult days but they have been sandwiched between the best of days as well. We often reflect about being on cloud 9 while walking the hallways and hours later be in the "depths of despair"  contemplating the unknown. As cliche as it may be, I wouldn't trade these days for anything.

Perspective

The perspective shift, for myself, has been very noticeable after Shirley's birth. I have always loved children and my mom may know best how much I have coveted the idea of family, marriage, and having children of my own. I used to love babysitting for friends from my previous church community and feel very fortunate to have had the trust of those wonderful couples! So many hours with those cute little babies just made me want my very own. It seems like a long time coming though, considering that in my early teens I often thought and hoped I would be a father by 20. I may have been nearly 10 years late according to my own schedule but it was worth the wait. I also may have scared more than one woman away because they thought I was too serious about getting married, Brianna included. I also used to have thoughts about what my children would be like and who they would become. When Brianna was 20wks pregnant, we found out about Shirley's condition as well as the possibility of her being born with a genetic disorder called, among a host of other names, 22Q. Shirley has been tested a couple of times for 22Q and each has been negative for the syndrome. Once during her pregnancy via Brianna's blood and the other after Shirley was born. The prenatal test is about 80% accurate and we thought about the results often. We welcomed the idea of Shirley with a genetic disorder but we were uncertain about how it would affect her life and, if I'm honest, how it would affect mine. The night Shirley was born though put all of that to rest and it wasn't because of some genetic screening result. It was very simply put to rest by Shirley herself and the love of a parent that, hitherto, I had never fully understood. While sitting in a recliner with Shirley in my arms I began to cry thinking about her and saying to myself, "I don't care what defects or disorders you may have, I just love you." Her second screening came back negative a few days later and there was no sigh of relief, just us saying, "ok, thanks" to the doctor reporting it. Shirley's full screen is still not complete and there are other possibilities as far as her genetics are concerned but they don't worry us. As we walk the halls of this hospital there are children with every kind of condition imaginable. Now, more than ever, I don't see them defined by their genetics or whatever it may be, I simply see beautiful children and their proud parents. I look forward to what else being a parent may change in me.

Last Thing

A wonderful woman contacted me about a week or so ago and said she had heard about Shirley through the American Heart Association (AHA) where she works. Her name is Kami Sutton and she too has CHD and has had upwards of 20 procedures here at SCH. Because of her fundraising efforts the CICU is named in her honor. We had actually heard about her after Shirley's surgery when our nurse was talking about caring for the woman whom the unit was named for. Shirley and Kami shared the same nurse nearly 14 years apart! To have Kami contact us a few days later was very special to us. Kami sent Shirley a few crocheted items made by volunteers of the AHA here in the Puget Sound and also a wonderfully crafted card with a hand written note. We continue to be in awe of the support we have received and the people who have encouraged us out of the kindness of their hearts. The card made our day, thanks Kami!

-Joseph