Baby Boston's Marathon

Family Pictures

2010-02-03T18:17:00.001-08:00

Happy Birthday!

2010-02-03T04:30:00.000-08:00

Our baby boy is 1 year old today! I am filled with joy that our path has led to this wonderful day. The most fitting words I can say are the lyrics from Matt Redman's "Let My Words Be Few":

You are God in heaven
And here am I on earth,
So I'll let my words be few-
Jesus I am so in love with You.

And I'll stand in awe of You,
Yes I'll stand in awe of You,
And I'll let my words be few-
Jesus I am so in love with You.

The simplest of all love songs
I want to bring to You,
So I'll let my words be few-
Jesus I am so in love with You.

We have some wonderful pictures of Boston's year that will be posted soon!

Update

2009-06-30T14:48:00.000-07:00

Boston is now almost five months old. His latest lab results have confirmed that he is completely healed. He is a perfect little baby...so easy, happy, and lovable. He always has a ready smile and is developing beautifully. He loves to look at faces and watch others interact. He will coo and sing along with us. He is grasping and throwing his toys, rolling over, sitting up and starting to stand. He and his brother share a mutual affection, so much so that Clark first said "Love you, Bahs-sah" before he told anyone else that he loved them. Boston has been released from Children's Mercy care; all of his future testing, which will just consist of a yearly urine protein test, will take place at his regular checkups. We are truly blessed.

Boston in the tub.

Playing in the exersaucer. He can throw his ball two feet!

Father's Day!

My (hot) boys.

Standing up. That boy has strong legs!

Milestones and Dedication - You're Invited!

2009-05-04T07:29:00.000-07:00

We've hit a big one! Boston is now three months old. This is especially significant to us because the technical definition of congenital nephrosis states that it will develop by three months. We are so blessed that Boston has made it to his three month birthday with no symptoms and has in fact been completely healed.

It seems fitting, therefore, that we are able to celebrate his birth and dedication so near to this milestone. Boston will be dedicated next Sunday as part of our morning church service. We will be having an open house from 2-5 in the afternoon and invite everyone to stop by to see Boston and share in our happiness!

The details are:

Dedication
11:00 am
Heartland Community Church - South Campus
15100 W. 127th St.
Olathe, KS 66062

Open House
2:00 - 5:00 p.m.
14630 W. 85th Terrace
Lenexa, KS 66125

We hope you can come!

Our Miracle

2009-04-03T22:05:00.000-07:00

Boston has been completely healed! The lab results today indicated that the level of protein in his urine is normal. When he was born, his protein to creatinine ratio was 7 to 1; today it was 0.38 to 1. When he was born, the actual protein level in his urine was 350; today it was 2. When he was born, the protein level in his blood was 3.1 (normal is above 3.59); today it was 4.3. The doctors told us that he is completely normal and that there is nothing to indicate that he will have any issues as he grows up.

Additionally, the genetic tests came back and were exactly the same as before he was born, which is additional reassurance that he only has one broken gene, not two, and therefore will only be a carrier of Pierson Syndrome rather than have it himself.

We will follow up with the docs in 2 months, and then again in 6 months, and then every year after that to make sure that nothing changes, but the docs are happy to see us as little as possible.

We understand that we are living a miracle; that God has blessed us beyond our wildest imaginings; that our son could have died and instead has been healed...and we feel grateful and blessed. We are thankful for your prayers and for God's endless grace and mercy. Amazing grace. Our son has been healed.

In addition to this wonderful news, we also celebrate that Boston is 2 months old now. We not only got to hear the great news about his labs, we also got to visit his run-of-the-mill pediatrician for his 2 month check-up. He is 14 pounds! He is in the 97th percentile, but still on the chart. He has gained the maximum amount of weight on the "normal" growth chart. It is such an answer to prayer because before he was born we were not sure if he would grow at all.

Our family of four is loving life and our time together. I did not anticipate how hectic daily life would be and how little time I would have to spend on my own pursuits, but I love my boys and would not trade time with them for anything. Hopefully, I will have more time to blog soon :)

Here is Boston on his 2 month birthday!

Clark and I at the Wichita zoo looking at the cows.

Brian came home one day to find both boys asleep on my lap. We were watching Jeopardy!

Boston asleep in the sink (Brian thought this would be a funny picture).

Clark on the first night he slept in his big boy bed.

Clark getting his hair cut at Shear Madness.

The women of Anthony, KS who prayed for Boston's healing (Brian's mom Diana is holding Clark).

This is how Clark looks when you ask him to say "Cheese!" for the camera.

Boston smiling in his sleep.

Good News at the Doctor's

2009-03-03T09:41:00.000-08:00

We were back at Children's Mercy today for another visit with Dr. Blowey. Boston looks great and is growing well. He is at the top of the growth charts: 94% in height at 22 inches; 98% in weight at 11 pounds, 7 ounces and 97% in head circumference at 40 cm.

Boston's labs looked great. His protein level in his blood was stable again at 3.7, which is normal. All of his blood work was normal. We are waiting for the actual ratio of protein to creatinine in his urine, but the dipstick was negative for protein.

With all of this good news, Dr. Blowey said we can wait another month before having labs drawn again, and if those are normal, we will not have to go back to Children's Mercy until June.

We should get the results of the repeat genetic test back from Athena Diagnostics in a couple of weeks.

The Power of Prayer

2009-03-02T19:12:00.000-08:00

Since we found out about Boston’s condition we have been earnestly praying and we wanted to give an update about how our prayers have been answered. Most of our prayers focused on Boston's gestation and birth and have been answered. It was amazing to see how God answered our prayers. What was even more amazing was to see him answer prayers we did not dare to pray and shower us with blessings for needs that we did not know how to articulate. We did not expect that the labor would be so short and easy; that Sarah could avoid a c-section; that Sarah would recover so quickly from the labor and delivery; that Boston would be so calm and easygoing; that Boston would be able to go through 5 days of photo therapy without issue; that Boston would take to breastfeeding and bottle feeding so easily; that Clark would adapt so quickly and so well to being a big brother; and that we would be so supported and uplifted by friends, family, and strangers alike.

Our prayers that were listed on the right hand side of the page have been answered in the following ways:

Prayer: That Sarah will be strengthened with all of God's glorious power so she will have all of the endurance and patience she needs to finish the pregnancy with a cheerful spirit. Answered: Within a day after posting this request, Sarah felt uplifted and strengthened by God’s power. She was able to faithfully wait out the rest of the pregnancy. Sarah had an easy labor and delivery and recovered quickly.

Prayer: That Boston would be completely healed and that he will be born healthy with no complications; That Boston be healed completely; That he has a mild form of disease that does not affect his functionality.

Answered: In God’s wisdom, Boston was born with a body that compensates for an extra loss of protein in such a way that he has no other complications. Because of this, he is currently not classified as having any form of nephrotic syndrome. This is an impossible miracle that we could not have understood nor prayed for, but we are exceptionally grateful for God’s understand of the complex workings of our bodies.

Prayer: That Boston’s kidneys will continue to improve and show no sign of “texture” or “brightness” on the ultrasound.

Answered: Boston’s kidneys improved and the ultrasound after Boston was born showed no abnormalities: the kidneys were formed and function correctly, although they do not filter as expected.

Prayer: That he will grow quickly and gain weight well in order to reach transplant size.

Answered: This prayer has been answered in abundance. Boston made it to almost 39 weeks and weighed in at 8 pounds, 3 ounces. At one month he weighs 11 pounds, 7 ounces. The normal weight gain range for a breastfed baby is 5 to 8 ounces per week, so he had a target weight gain of 20 to 32 ounces. He gained 52 ounces! This weight gain is his protection against any future problems and brings him closer to a transplant weight, should he ever need one.

Prayer: That Sarah and Brian will be able to prepare for the worst without losing perspective on the big picture.

Answered: We found this to be much harder than we anticipated, especially in the first few days of Boston’s life when we were told that he was losing protein and to expect him to get sick. We know, however, that this preparation would have been impossible without the knowledge of God and our relationship with Jesus.

Prayer: That the doctors will find a cause for his ridiculously high AFP and be able to treat it accordingly.

Answered: The cause of Boston’s high AFP was the protein that he is passing into his urine. Miraculously, however, it requires no treatment at this time.

Prayer: That the level of his amniotic fluid will stay normal in order to allow his lungs to develop and to reassure us that he does not have complications.

Answered: this was answered in abundance: Boston’s amniotic fluid stayed normal until the final weeks of his gestation, allowing his lungs to fully develop.

Prayer: That he will have use of his eyes and not be blind.

Answered: Boston’s eyes are normal at this time and he is meeting all of the development markers for focus and tracking.

Prayer: That his muscles will be strong and his brain alert.

Answered: Boston has a strong vice grip in his hands. His head and neck strength are increasing every day and he is ahead of the curve on being able to support his head on his own. He is also advancing in his head and shoulder strength during his tummy time.

Prayer: That Sarah will be able to learn and understand about the disease and treatments.

Answered: Through the support and encouragement of the medical team, Sarah was able to gain all the information necessary to converse with the doctors in a knowledgeable manner about Boston’s condition. It was a compliment to her to be regarded as an “expert” on Boston’s condition.

More Pictures

2009-02-22T20:30:00.001-08:00

This pic showcases Boston's chin that looks like Brian's.

Eyes open.

On our last day at Children's Mercy in his going home outfit.

Asleep at home.

Update to Volunteering/How Can You Help?

2009-02-17T14:33:00.000-08:00

We have been abundantly blessed by everyone's outpouring of gifts and wanted to keep everyone updated on what would be most appreciated as our needs change.

Now that we are home we have really appreciated the meals and gift cards to restaurants that have been given. It is such a blessing to not have to go to the store and prepare meals.

Play dates for Clark are also something we are looking to set up. The best times are 9-11 and 3-5. If you would be willing to have Clark over, please contact us with available days and times.

Gift cards to Babies R' Us or Wal-Mart -- for diapers, burp rags, pacifiers, breast milk storage bags, and other baby essentials that always seem to be in high demand.

Prayer -- we can never have enough. And why stop now when our prayers have been answered so abundantly?

Contact us at ihearttheprestons@gmail.com if you are willing to help. Donations and gifts can be mailed to us or dropped off at 14630 W. 85th Terrace, Lenexa, KS 66215. We can't tell you how much we appreciate all of your prayer and support.

Changed Diagnosis

2009-02-17T14:23:00.000-08:00

Boston and I went to Children's Mercy yesterday to have his labs drawn and then were back again today to talk over the results with Dr. Blowey. His lab work remains stable. His protein to creatinine ratio in his urine was 5.81 to 1. Anything over 0.5 is abnormal, so 5.81 is definitely an indicator that he is dumping protein in his urine. The expectation has been from the beginning that as he continues to dump protein in his urine that his protein level in his blood would drop too, and thus cause all of the complications, but we have not seen this happen. His protein in his blood (albumin) was 3.3; he has ranged from 3.1 to 3.8 with 3.5 being the bottom end of the normal range.

Because of Boston's miraculous stability and the fact that he has protein in his urine, but none of the other signs of congenital nephrosis, Dr. Blowey changed his diagnosis from congenital nephrosis to proteinuria (a diagnosis that means he has protein in his urine). Dr. Blowey says he looks great and we have done all that we can, so now we just watch him and keep our fingers crossed. Our next follow up appointment is in two weeks.

This is such a cause for praise in our household. I have been challenged to be completely and utterly joyful and giving of praise to God for what he is doing in Boston's life. My tendency was to be "cautiously hopeful" and to not fully embrace Boston's health as a miracle out of fear that he could take a turn for the worse. Over the past couple of days I have come to realize that God is not one who makes us "knock on wood" that he won't take our blessings away -- He doesn't punish us for being too happy with his gifts. And so we embrace Boston's health for what it is--an absolute miracle and answer to prayer. And we praise God for his goodness and mercy and for our beautiful boy!

On our way to Michelin Baby #2

2009-02-17T14:12:00.000-08:00

Clark was famously chubby during his first six months of life. He gained weight extremely quickly, outgrowing his clothes at record speed. He was only in 0-3 month clothes for a little over a month and by his two month birthday had moved to 6-9 month clothes. By 3 months he weighed over 20 pounds, which is traditionally seen as more of a one-year-old weight.

Miraculously, Boston seems on track to match or surpass his big brother in weight gain. Boston had a bit of a leg up at birth, regained his birth weight a bit quicker, and is currently packing on the pounds. At one week, Clark weighed 7 pounds, 10 oz; Boston weighed 8 pounds, 4 oz.

Today at Boston's two week check-up we found out he weighs 9 pounds, 6 oz, a weight gain of about 18 ounces in a week! (The average baby gains 5-8 ounces per week).

This is such a blessing because often the biggest hurdle in the treatment of conditions like Boston's is getting the baby to grow. Boston's healthy appetite and his steady growth are the best indicators for his long term survival.

Clark and Boston: Brotherly Love

2009-02-17T14:07:00.000-08:00

Our two boys are getting along beautifully. Clark is very inclusive of Boston. Clark doesn't want to go anywhere without Boston and doesn't want Boston to go anywhere without him. He will try and help carry the car seat with Boston in it, and when we return home will refuse to do anything until Boston is out of the car seat. He won't put his hat on unless Boston has a hat on too. He won't eat dinner unless Boston is sitting in his bouncy seat next to the table. And if we are going upstairs or downstairs, we all have to go.

Clark is also very protective of Boston. At church on Sunday he actually pushed a friend of ours' hand away when she tried to touch Boston. (She was able to hold Boston later when Clark wasn't around).

It is wonderful to see how quickly Clark has adjusted to having Boston in our lives and how loving he is toward him. We are so blessed!

Home!

2009-02-11T19:31:00.000-08:00

We are home! We were discharged from the hospital yesterday at 2:30 and have spent a whole day at home adjusting to our lives as a family of four. My mom has been visiting to help us with the transition and she has been a big help.

It is wonderful to be home!

Blessed Be the Name of the Lord

2009-02-09T13:43:00.001-08:00

After rough news to start and a few uncertain days, we have reached a point of cautious optimism and wholehearted praise to God as Boston remains stable. Today his levels were improved to such a point that we have been told we may be able to go home tomorrow!

He is still showing no sign of swelling. His bilirubin was low enough that all phototherapy could be stopped. He is back in his clothes and out of the warmer and in a regular crib. He is still a champion eater and sleeper.

It is still possible that Boston's condition could worsen and so the doctors are developing our follow-up plan that will involve weekly visits to the docs and labwork. We are focusing on the fact that today he is doing great -- and not worrying about what tomorrow holds. Our doctors can't explain our little hero's miraculous recovery -- we give all the credit to God.

During our week in the hospital we have had lots of ups and downs -- we have been in the land of the plentiful and we have walked through the desert place, sometimes in the space of a few hours. Through it all we say "Lord, blessed be Your name."

2009-02-09T11:33:00.000-08:00

He must be laughing at the item of turning into the Michelin baby.

This is how babies get a tan.

Stable on Day 5

2009-02-08T14:13:00.000-08:00

Boston's labs came back stable this morning. He is still not swelling at all (yay!) and is eating great, which are two signs that he is holding his own. He still has protein in his urine, but for now it is not causing any serious side effects.

His bilirubin was down today enough that we could take him off the spotlight and just leave him on the paddle. It goes inside his blanket next to his back and we can hold him while he is on it.

He is gaining weight and is at 7 pounds 10.5 ounces, up from a low of 7 pounds 8.7 ounces a couple of days ago. The norm is for babies to regain their birth weight within two weeks and he seems on track to do that.

Boston has had the same day and night nurses over the weekend, which has been really nice to have that continuity. His day nurse, Stacy, is awesome, and we have been swapping baby stories. She has an eight month old daughter. The normal ratio of babies to nurses is 2 babies to each nurse, but there are two babies on our pod that are critical enough to need their own nurse. A new baby was admitted today after being born at only 25 weeks and was assigned to Stacy. She said she could take her because I pretty much take care of Boston by myself. It is nice that he is so easy to take care of. I just do the normal things, like feeding and changing him, but I also know how to fix his wires and alarms in case anything goes off, and I can navigate through all of the machinery to get him in and out of his bed without any help. I can't wait until it is just him without any wires or paddles or masks or foot wraps.

2009-02-08T12:15:00.000-08:00

Boston on 2-7

A Few of My Favorite Things...

2009-02-08T07:30:00.001-08:00

1) When Boston wakes up and stretches his arms over his head like Superman.

2) My orange bracelet that lets me go in and out of the hospital without going through security.

3) When Boston opens his eyes and looks around with his mouth in a little "o."

4) The free meal vouchers to the cafeteria - yum, chicken fingers.

5) The little half-smiles Boston makes right after he eats and has fallen back asleep.

6) The Ronald McDonald room where I get to sleep in between night feedings -- did I mention it has a sleep number bed?

7) The fact that Boston is able to sleep on his bed under the bililight without fussing.

8) Getting to go home at night for a few hours to put Clark to bed.

9) Lab updates in the morning when there is good news.

10) The support of my friends and family :)

A Good Day

2009-02-07T11:08:00.000-08:00

Boston is having a good day -- his labs came back with good levels. His protein to creatinine ratio in his urine is 3.69 to 1. Congenital nephrosis is considered to be anything above 3 to 1. Yesterday it was 7 to 1 and the day before it was 5 to 1. The doctors told us to not get too excited because the ratio can fluctuate from day to day and we are looking for trends, not snapshots. But for today, his ratio is good. Let's pray that tomorrow it will be good as well.

He had lots of good wet diapers during the night and so we no longer need to supplement him with a bottle after he is done eating.

He gained weight since yesterday -- just about 40 grams, but that is moving in the right direction. He had been down about 8% from his birth weight.

His protein in his blood is still normal. It is 3.5, which is the bottom of normal. It was 3.4 then 3.8 then 3.6 and now 3.5 -- still in the good range. If his blood protein gets too low then he will start to swell which will cause problems. Even if he has protein in his urine, if he still has a normal level of protein in his blood then he will not have the complicated side effects.

His immune system levels were good -- normal white blood cell count, etc.

The only number we are still watching is his bilirubin level. This is the number that tells us if he is still jaundiced. His number went up from yesterday and so he will be on the blanket with the light for at least another day, maybe two. His number is not awful, just high, and so they don't have to take any more drastic measures than that. It is a blessing that he is sleeping lots and doesn't mind being on the bed. We are so lucky that he is a sweet, content baby. He sleeps for three hours, eats and smiles at me, and then goes back to sleep to repeat the cycle.

It's been quiet today because I've asked people not to visit. Boston is doing great and I wouldn't mind visitors, but the Intensive Care Nursery is not a very visitor friendly place. This morning there were two emergencies with two other babies near Boston and it was like watching drama tv -- when it was all over the other members of the staff were telling baby's nurse how she had saved his life. She was visibly shaken by the ordeal. It was amazing to see how wonderful the staff is and how they are able to handle everything in a crisis situation.

Day 3 - Friday

2009-02-07T07:31:00.000-08:00

Boston spent the day under lights getting rid of his "tan." His jaundice hadn't improved and so we added a lamp in addition to his blanket to help speed things along. He looked really funny in with his blindfold on.

We were able to get all of the ultrasounds and the eye exam today -- even though we had thought we would have to wait until Tuesday. No cyst in front of the tailbone; head ventricle still on the high side, but not concerning; and the kidneys are formed correctly, although slightly bright. Dr. Van de Voorde was not concerned with the ultrasound as it did not affect our plan of care.

Our best news of the day came with the eye exam -- Boston's eyes are normal. If he ends up being confirmed for Pierson's we will have to continue to watch them, because they could all of a sudden get worse - but it is great news that his doesn't have any blindness, retinal detachment or pinpoint pupils right now.

I am continuing to stay at the hospital so I can feed Boston every three hours. At night I have been able to find a bed in the Ronald McDonald rooms, so I can sleep for an hour and a half in between night feedings.

Boston will probably be under the lamps for another day or so depending on what his levels are on Saturday. We will continue to watch him closely over the weekend, as this time is when the other effects of losing protein, such as swelling and lack of appetite start to occur. Whether he gets worse over the next few days will tell us a lot about the plan of care we can expect.

2009-02-06T10:49:00.000-08:00

Taken on 2-6
Taken on 2-5

More Pictures

2009-02-06T10:38:00.000-08:00

Taken on 2-4

The testing begins...

2009-02-06T03:49:00.001-08:00

Good morning! Sarah here. Boston says "hi" as he sleeps contentedly (his only 4 jobs being: eating, sleeping, pooping, and wait for it...awesomeness). We have had a magical first few days with him. It is hard to believe that he was born only 54 hours ago.

We were transferred to Children's Mercy yesterday afternoon around 3. Thanks to everyone who called or stopped by while we were at St. Luke's. It was a whirlwind of activity as we crammed as much visitor time as we could into his first day and a half in the world. Last night I got to sleep about 5 hours, but before that I had slept maybe four hours since Monday night.

Medical news: Boston's initial urine test at St. Luke's showed that he had large amounts of protein in his urine, which was sufficient for the kidney doctors at Mercy to say "Okay, yes, there is something wrong with the way his kidneys work." Having large amounts of protein in your urine is the hallmark sign of congenital nephrosis. Our plan for testing and care has not changed -- we will still need at least a week here at Children's Mercy to get a more accurate picture of what is going on with his body -- the doctor's look at trends, not snapshots, so while we will try to be as forthcoming with the updates as possible, be prepared for us to not know anything certain for a while.

More medical news: while Boston does have a large amount of protein in his urine, and thus likely has congenital nephrosis (which would mean a kidney transplant), the other signs of "Pierson Syndrome" (which can be just congenital nephrosis or congenital nephrosis and other things) are not presenting initially. His big brown eyes do not have the "pinpoint pupils" but we are going to have a full opthamalogic exam next Tuesday. He has good muscles and reflexes. He is eating really well. All of these things could change, as this syndrome can be progressive (things not being there at first and then showing up later).

Support: we would appreciate it if people would not ask us for medical updates or about what is going to happen with Boston's condition. As far as we are concerned, he is just a normal baby. We get comments from the doctors and staff that he is a normal baby; that he "looks great" and is doing all of the normal things that normal babies do, except he has a large amount of protein in his urine. It is too early for us to speculate about what his tests could mean; it will just take time to know anything. The earlier posts in the blog have more information about congenital nephrosis and the tests that are going to be done this week. Also, while this news is not "optimal," it is what we were planning for and we are proceeding with the plan that we had set up before he was born. We are in good hands with the doctors here. We don't want people feeling sorry for us or treating us or Boston any differently.

Fun news: Boston really is awesome. He is beautiful and smells good and loves to cuddle. He knows his momma and his dadda and while he spends most of his time sleeping, he also has alert times when he just stares up at us. Yesterday he was awake and kept trying to grab Clark's shirt with his hand, but Clark was not interested. I don't think Clark has figured out that Boston belongs to us yet. I am sure that day will come. :)

Communication reminder: Children's Mercy is a blackout zone for cellphones, so if you get sent to voicemail, you can bet that we're here.

2009-02-04T00:14:00.000-08:00

Baby Boston Ethan Preston was born at 11:15 pm on February 3, 2009!!!
He weighed 8 lbs. 3 oz.
He is 20.25 inches.
Mommy is doing well.

No Baby Today

2009-01-29T13:11:00.000-08:00

This morning Boston had flipped and was head down! Yay! So no baby today, but we are scheduled to go in for an induction on Monday night, meaning that he will likely have a birthday of Tuesday, February 3, 2009!

More Than A Feeling...

2009-01-28T10:21:00.000-08:00

The new song on the playlist is Boston's "More than a feeling." I decided to not put it on the playlist until we were close to delivery...and it looks like we are. This song is on my "Greatest Hits" list (although Boston's name is unrelated to my interest in the band). As Boston has gotten bigger and bigger and his movements stronger and stronger, I have come to realize that soon he will be "more than a feeling." We are about to meet our little man - and I am so excited.

Tomorrow morning we need prayers -- I have my regular NST at 8:30 and then at 9:00 we are going to be facing some decisions. On Monday during my office visit it was discovered that Boston had flipped from his previous head down position to a head up/face up breech position. He has so much fluid to move around in that it allows him to turn when most babies would not be able to. At 9:00 tomorrow I have what Lynn called a "position check." If he is still in breech, then Lynn said that we will "talk." This "talk" will focus on the decision of whether to try and turn him manually into a head-down position and then most likely induce right then. I'm not sure what my options will be if he is head down or if he refuses to turn. I know that if I go into labor while he is breech then I will almost definitely have to have a c-section. The risks of cord prolapse are even higher when the baby is in breech and most doctors just don't try vaginal breech delivery any more -- the risks are just to high.

We will try to update as fast as we can tomorrow -- but be expecting some news :)

Caden's Cubs for Clark

2009-01-26T14:57:00.001-08:00

We were blessed by the Carlson family with the opportunity to take Clark to create a special Build-a-Bear in anticipation of Boston’s birth. The Carlson’s set up this project called “Caden’s Cubs” to honor their son Caden who was stillborn last summer. To read more about the project, check out the January 5, 2009 entry on their blog, http://thecarlsoncrew.blogspot.com/2009/01/special-announcement.html

Clark, Brian and I had a great time on Saturday building his bear. Clark and I had previously done a “scouting mission” just the two of us earlier in the week when it was less busy in order to make sure Clark had the necessary time to pick out what he wanted. I took each bear, dog, cat, turtle and other animal off the shelf and handed it to him. He shook his head almost every time, rejecting them one by one. I knew we had found his bear when he reached out excitedly and said “Heart, Heart.” He had chosen “Champ,” a champion fur kids. It was fitting, because part of the purchase of Champ goes towards the Bear Hugs Foundation which makes grants to causes that support children’s health.

Clark next picked out a sound for Champ. He decided on the “Roaaar.”

Clark’s favorite part was watching Champ get stuffed and getting to put the heart inside. He had hearts in both fists and wanted Mom and Dad to kiss them before they went inside.

Dad helped pick out some cool clothes and sneakers.

Because the store was so crowded (it was Saturday afternoon), we took Clark to get a pretzel and dress Champ where it was quieter.

Since we have brought Champ home, Clark has gone to Champ’s house several times to get him out, give him a kiss, and then put him back. It was great to have this special time to spend with Clark, in what will probably be our last family outing before Boston’s arrival. Our family is about to be changed forever and it was wonderful to have this memory to share. Thanks to the Carlson family for allowing us to honor Caden and Boston in this special way.

Waiting PATIENTLY

2009-01-23T14:43:00.000-08:00

I have been waiting, but not patiently, for Boston to be born. In Latin, the root word of patience "pati" means “to suffer.” The Greek word “hupomone,” which is often translated as “patience” in the New Testament, means “cheerful or hopeful endurance.” That is my aim for the rest of this pregnancy. As I wrote in the November 13 entry, Dr. Blowey wants Boston to stay in utero as long as possible because my kidneys keep him healthy and allow him to grow without putting any strain on his little body. If there is not a significant medical reason for him to come, then just we should wait on him. I didn’t realize at the time how tempting an elective induction would be. With his size and all of his extra fluid, I am SO uncomfortable. Noticeably uncomfortable. Uncomfortable to the point of pain and tears.

I read in Ecclesiastes 7:8 today that “Finishing is better than starting; patience is better than pride.” It made me realize that as much as I am eager to start Boston’s life, it is better to finish this pregnancy with a strong and valiant spirit, with “cheerful and hopeful endurance.” Many verses in the Old Testament talk about waiting patiently. Psalm 40:1 says “I waited patiently for the Lord to help me and he turned to me and heard my cry.” Psalm 27:14 says “Wait patiently for the Lord. Be brave and courageous. Yes, wait patiently for the Lord.”

This is definitely an area where prayer would be appreciated. I want to be filled with the calm and peaceful assurance that God has determined all the days of Boston’s life, including the day he is to born. In the first chapter of Colossians, Paul is outlining all the prayers that he has for the church in Colossae. He closes that section of the chapter with this message: “We also pray that you will be strengthened with all his glorious power so you will have all the endurance and patience you need. May you be filled with joy, always thanking the Father.” That is my prayer: that I will be strengthened with all of God’s glorious power so I will have all the endurance and patience I need.

Volunteering/How Can You Help?

2009-01-21T09:12:00.000-08:00

We have received several inquiries and offers of help and because we want and appreciate the offers and will need your help, I wanted to provide more information about what we need and when.

This whole pregnancy has been filled with unexpected events and as much as we have tried to plan ahead of time, there are still many things that are uncertain. The traditional, typical needs of a family with a new baby will not be our needs; we will not be the family at home with a healthy baby, although we hope that someday we can get to that point. I will provide as much information as we have now, with the understanding that we will update the info as it comes available and as our needs change.

What we know we can use:
· Gas cards – we will have to make trips back and forth to St. Luke’s and Children’s Mercy several times a day and the gas will add up.

· Non-perishable food and snacks - for at least the first week we will be in the NICU, so easily portable food that we can take with us will be appreciated. Things like Progresso Soup, dried fruit and nuts, packets of instant oatmeal, etc. would be appreciated. Also, apparently Children’s Mercy has one of the best cafeterias around, so money for a hot meal there would also be appreciated.

· Frozen casseroles/dinners – we are not sure when we will be at home, but are planning on spending one meal a day (probably dinner) at home as a family. Food that can go from freezer to oven would be appreciated. We even bought a full-size freezer to put in our garage for this very purpose.

· Clark care/play dates - Brian’s parents are going to try and come up for at least part of that first week to stay with Clark, but if we have to be in the hospital for longer than a week then we could use some help in watching Clark so Brian can be at the hospital too. Also, even if we get to come home, it is likely that Boston will be more susceptible to infection and thus not able to have a lot of visitors or leave the house. I am sure Clark will not like being cooped up and so play dates would be appreciated.

· Rides to the hospital – this is a “we hope not” request, but if Sarah has to have a c-section then she will not be able to drive for a couple of weeks. Because she wants to spend a lot of time with Boston, we would appreciate having some people to call on for rides.

· Gifts – because we don’t know when we will get to use any clothes or diapers, we are asking people to hold off on these gifts for now. I looked online for ideas and several websites suggested: books to read to baby, books and music for Mom and Dad to get through long bedside vigils, toys for the older siblings, photo frames to put by baby’s warmer in the NICU, movie tickets (for a break for Mom and Dad), gift cards to Wal-Mart and Target for necessities, etc.

· Congratulations – we plan on celebrating Boston’s birth with all of our hearts and invite you to join us. He is our little boy and we are going to shower him with love and affection from the moment he is born. Cards, emails, balloons, flowers, etc., will all be appreciated as you join us in celebrating Boston’s arrival.

· Prayer - once Boston arrives I am anticipating having an abundance of prayer request and praise reports to pass along. We will keep the blog updated with the specific prayer needs that we have and would love for you to target your prayers in kind.

A few more things:

Updates – we know that you will want to know how Boston is doing and how we are dealing with things, but we do not anticipate being able to make, take, or return many phone calls, especially since cell phones are not allowed in the NICU. We will try to update the blog regularly and will rely on you to check it if you want information. Please understand that we appreciate and acknowledge your interest, but have been told we will not likely have the time to respond personally to every call or email.

Visitors – we rely on you for support and comfort. We have been told that parents are divided on the issue of visitors in the NICU. We know the NICU has strict rules on who can visit and when and that some parents are overwhelmed by visitors. When we have a better idea of what is going on we will let you know. Also, we know that anyone who visits needs to be very healthy, so keep that in mind.

Homecoming – we are hoping that our homecoming will be as soon as possible. We were told that coming home with a new baby is cause for celebration and a time for renewed interest from others, a time for gifts and dinner, visits and help.

Paranoia – we were also advised to let people know that we may be overprotective of Boston if he has to spend much time in the hospital. In case we are cautious of other people holding him, don’t get out much, or decline to attend crowded events, please understand that we are doing so with Boston’s health in mind and hopefully it will pass as he grows in strength and health.

If you are willing to help with Clark care, rides, or meals once we get home, please email us at ihearttheprestons@gmail.com and we will contact you when we know more.

Donations and gifts can be mailed to us or dropped off at 14630 W. 85th Terrace, Lenexa, KS 66215. Please specify what you would like it to be used for, unless it is food, in which case we can figure it out on our own :).

We can't tell you how much we appreciate all of your prayer and support. It is what gets us through.

Baby Alcatraz Part 2: St. Luke's is in lockdown

2009-01-20T08:27:00.001-08:00

Ashley Bates, the NICU coordinator at St. Luke's (who I love), called this morning to let me know that the NICU at St. Luke's is also in lock down to everyone except parents (which is stricter than Mercy). I told her that she did not have a very fun job today calling all these moms, but that I was okay because we expected that Boston would room in with me at St. Luke's and that we would be transferred to Mercy pretty quickly anyway. However, I wanted to give everyone a heads up in case Boston has to be in the NICU and not with me in my room.

The waiting is the hardest part…

2009-01-19T15:10:00.000-08:00

Monday morning I had the whole enchilada of testing – NST, Ultrasound, and Office Visit. The NST was uneventful: I had virtually no contractions while I was hooked up, Boston slept through the whole thing, and my blood pressure was normal. All good things. Boston would get zapped with the stimulator, do what he needed to do, and then promptly fall back asleep. I told Julie, the RN, that he was taking after his dad, who is nearly impossible to rouse when he is sleeping.

I am a big fan of Tom Petty and the Heartbreakers. I bought their greatest hits when I was high school and spent many a day singing along to their lyrics. Often those songs will come to mind, as one did this morning. It’s the chorus to “The Waiting” by Tom Petty (sing it with me):

The waiting is the hardest part
Every day you see one more card
You take it on faith, you take it to the heart
The waiting is the hardest part

That pretty much sums up how I feel right now. Boston could come at any time within the next three weeks. We don't know when, and there is no way to know. I have trouble making plans beyond today because I don't know if I will be able to keep them. We just have to wait -- take it on faith and take it to the heart.

The ultrasound revealed five noteworthy things:

1) Our prayers for Boston's growth have been answered in abundance!!! Yay God!!! If he does have any sort of congenital nephrosis, his size will give him a great head start on the growth that is needed for him to reach transplant size. Right now on the ultrasound he is measuring 8 pounds 5 ounces, give or take a pound. The fact that he is somewhere between 7 and 9 pounds makes me soooo happy! Trust me, I can feel how big he is; he is definitely taking up a lot of room in there.

Because of his size, the docs think he could come any time. He is head down and in position. They have scheduled another growth scan for three weeks, if I am still pregnant then. If during that scan he measures, say, 10, 10 and a half pounds, they are going to punch his ticket and induce. This gave me at least some measure of comfort to know that there is an end date in sight, even if it is still three weeks away.

2) Boston's amniotic fluid level is high. We have been praying "that the level of his amniotic fluid will stay normal in order to allow his lungs to develop and to reassure us that he does not have complications." That prayer was answered as his level stayed normal/high normal throughout the pregnancy; however, he has moved out of the "high normal" range into the "high" range. Normal levels are between 5 and 25; Boston's is 34.11. The sonographer called it his "swimming pool." High levels of amniotic fluid occur in about 1% of pregnancies and while it is not harmful to either Boston or I, it could indicate an underlying problem, such as a congenital defect or block in his gastrointestinal tract. High levels of fluid happen because he is producing a lot of fluid, or not swallowing enough fluid, or a combination of both. The complications I have experienced so far include discomfort for me, because he and his fluid are taking up so much space and squishing everything, and uterine contractions (ouch!). High levels of fluid also indicate that labor could happen soon -- I am basically a water balloon that could pop any time. The doc told me to expect him any time and to keep extra towels in my office and car. High levels of fluid also increase my risk for an emergency c-section, if the placenta ruptures or the cord enters the birth canal before Boston's head does.

3) The doc spotted a fluid-filled cyst in front of his tailbone. Boston has gotten so big now that it is hard to see anything clearly on the ultrasound, but the doc thought she saw something in front of his tailbone. She did a thorough check to make sure that his back and spine were closed, and she was reassured that they were, but still saw the cyst. This is not something they have seen before. We discussed the possibilities of what it could be: nothing, part of his intestine that has dropped down, or a block in his rectum. This finding doesn't change our plan for labor and delivery, but we added a pelvic sonogram to the list of tests that Boston will have once he is born. He also will not be able to eat until they can rule out a block in his rectum.

4) The ventricle in his brain was still enlarged: anything over 1 cm is large and his was 1.2. Because it has been consistently just a bit over 1 cm throughout the pregnancy, we were already planning on doing an ultrasound of his head.

5) Otherwise, Boston looked great. We got to see his chubby cheeks and all the fat he has put on, his big head of hair and his hands and feet. His kidneys looked normal, but to remind everyone, that doesn't mean he doesn't have kidney problems. Boston's AFP level was 65 times too high, and 98% of babies with an AFP over 10 are born with abnormalities. That percentage increases as the level increases. From a medical article: "the difficulty in confirming congenital nephrosis before the baby is born is the lack of ultrasound findings. Although bright and slightly enlarged kidneys have been described, in most the kidneys appear normal, the amount of amniotic fluid is normal, and a large placenta, which may occur, does not appear until late in the pregnancy." Basically, what the article says is that if you have an extremely high AFP and everything else looks fine, you can't rule out congenital nephrosis until the baby is born.

These statistics encourage me to put my faith in God. They tell me that medically, logically, it is likely that Boston will not be born okay. Because the odds are against him, and his chances by the world's standards are not good, it makes me realize how important God's intervention is. We cannot do this without Him. This is too big for us; we cannot do it on our own. God will have to heal Boston.

That's the nice thing about waiting -- it gives you more time to pray :)

Tour de NICU

2009-01-16T12:50:00.000-08:00

This week I met with the neonatologists from St. Luke’s and Children’s Mercy, on Tuesday and Wednesday, respectively, . I also got to tour the Neonatal Intensive Care Units (NICUs) and talk with the coordinators about the NICU experience, visitation, etc. It was very informative and not as altogether unpleasant as I had been expecting.

St. Luke’s: I met with John Callenbach from the neonatology department and he outlined Boston’s initial plan of care. Because we do not anticipate any problems with delivery, he said I should be able to deliver in a normal room, as opposed to an operating room where there would need to be ventilators, more staff, etc. Once Boston is born, he will be immediately admitted to the NICU, although this is more of a paperwork thing than a location thing. Provided there are no imminent threats to his health, he should be able to “room in,” i.e., stay with me, any time he is not having tests done.

Pierson Syndrome is virtually impossible to diagnosis in the first couple of days because Boston’s kidneys won’t have kicked in yet and a lot of his numbers will reflect my physiology and not his. It also means that if he does have the syndrome, we are hoping to get to spend a couple of days with him before he gets seriously sick.

In the first couple of days, Dr. Callenbach anticipates monitoring his blood and urine for protein, doing an eye exam and an ultrasound of his head and watching his growth, eating patterns, and for any sign of swelling.

We do not plan on being at St. Luke’s for more than a couple of days. I hope that Boston can stay there until I am discharged from the hospital, which will be after 48 hours, or 96 hours if I have to have a C-section.

The visitation policy at St. Luke’s is extremely lenient on the mother-baby side and provided everything is going okay, the first couple of days will also be prime visitor time for those of you who want to meet Boston because once he is at Children’s Mercy, it is pretty much Baby Alcatraz and only two people can be by Boston’s side at a time and that includes parents, so if Brian and I are both there, no one else can be (more on Baby Alcatraz below).

Children’s Mercy: After our first couple of days at St. Luke’s, Boston will be transferred to Children’s Mercy for at least a week. We were hoping that if everything was going well that we would be able to go straight home from St. Luke’s with follow up at Mercy, but during my Wednesday meeting with Dr. Kilbride (neonatologist a.k.a. baby doctor) and Dr. Van de Voorde (nephrologist, a.k.a. kidney doctor), they told me that they wanted Boston to come to Mercy A.S.A.P. They said that it wasn’t safe for him to go home because even if he looked like he was absolutely perfect, he could deteriorate quickly, so it was necessary to keep him under close supervision for at least a week. They seemed kind of on-board with waiting until I got discharged from St. Luke’s, but they didn’t want a lot of testing done until he was transferred to Mercy. If he gets sent to Mercy right away, then I will have to get a day pass to go visit him there, not something I want to be doing so soon after he is born.

Once he is at Children’s Mercy, they will continue to do the same blood and urine testing and monitoring. They will also do renal function tests and likely an MRI and a test to look for other eye defects. If necessary, his treatments could include protein infusions, nutritional supplements, a drug to reduce any swelling, and antibiotics.

Our best case scenario, if he is doing absolutely perfect, is to get to go home from the hospital after a week or so. It is not uncommon for babies with mild nephrosis to stay for weeks and the more serious babies stay for months before going home, and some never go home at all.

So we are preparing for Children’s Mercy to be our “home away from home” for a while. I called it “Baby Alcatraz” because of their strict, albeit necessary, visitation policy. Only 2 visitors are allowed at the bedside, including parents, which means if I am there (and I plan to be there a lot), only one other person can be there with me. I can’t have both my parents there at the same time, and if Brian is there with me, then no one else can be there. However, I am sure I will welcome the company and be more than excited to share Boston with the world; I just want to warn everyone that this will be on a one-at-a-time basis.

The thing that upsets me the most about the visitation policy is that right now the NICU is on “Sibling Lockdown” due to flu season, which means Clark can’t visit at all. I am working through the sorrow of knowing that once Boston is admitted to Children’s Mercy and until the ban is lifted or he can go home, we will not be able to be together as a family. We will try and cram as much “family time” into the first couple of days at St. Luke’s as possible. I know that this will not be very long (if we get out in a week) but the ban may not lift until March, and if we are there long-term, then it will be very strange to not have Brian, Clark, Boston and I all together.

The NICU at Mercy is very open – the babies line the walls rather than being in their own rooms. So every few feet there is a baby with a couple of chairs in front of it. I plan on camping out in one of those chairs and becoming very well known to the staff. I can have water while I sit next to Boston, but no food. Breastfeeding moms get four meal vouchers a day to use at the cafeteria, which is directly below the NICU. They also provide “nap rooms” and showers to use during the day. Even though we are only going to be (for sure) there for about a week, I am reading about parents and the NICU, how much time to spend there, how to schedule the day, etc. I am planning on being home in the evenings with Clark from at least 5-8 pm, so we can maintain our usual schedule, and I am planning on sleeping at home, and the rest of it we will just figure out. There are no cell phones allowed in the NICU, so I will have to take regular breaks to get messages and send updates. There is a computer for parents’ use in the NICU, so I will be able to update the blog from the hospital (yay!)

I can’t wait to meet this little guy! Even though the beginning of his life will be unconventional, I feel better having a plan and knowing what to expect in the event of the unexpected. Let me know if you have questions; there is probably something I left out.

Update on NSTs

2009-01-12T07:15:00.000-08:00

My NST this morning was not as complication-free as my previous ones have been. I was having contractions throughout the testing, although some of them were mini-contractions that Julie just called "irritability."

Boston wasn't reacting the way he should -- although he was moving around a lot, we weren't seeing the same good accelerations in his heart rate that we had seen in the past.

After 45 minutes on the monitor (as opposed to the usual 20), Julie took my results to be analyzed by Dr. Lu. This was a first, as they are usually clear cut enough for Julie to release me on her own. Dr. Lu gave me permission to leave, but asked about whether I was feeling the contractions and to be extra-vigilant about baby-movements.

The plan right now is for me to come back in for regularly scheduled testing on Thursday.

The Importance of Being Earnest

2009-01-12T07:07:00.000-08:00

I am a go-getter, an information seeker. I like to know things for myself firsthand. As a result, I have done a lot of my own research on Boston's condition. Some may call this overkill, but for me, I feel it is necessary to make sure that I am doing everything I can to be a good mom for Boston.

I received a benefit from my overzealous pursuit of information last week when I found out that Boston's kidneys had been "bright" again on his November ultrasound, a fact that the doctor's failed to tell us. I requested copies of my ultrasound reports to send to the doctor's at Children's Mercy and as I was reviewing them, I saw that on his November ultrasound, the doctor had reported that his kidneys were large and "mildly echogeneic." Echogeneic means that there are more sound waves (echos) in that area, resulting in the area looking bright. I was shocked when I read this, because I always ask lots of questions during our ultrasounds and the impression that I got from the doctor was that everything was fine during our November ultrasound.

We have had another sonogram since then, and the ultrasound report from that sonogram reported that Boston's kidneys looked normal.

The two things I took away from this experience were:

1) We have thought that Boston's kidneys looked normal since his September sonogram; now we know that is not true: they were bright in November.

2) I need to make sure that the doctors are giving me all of the information, even if that means reviewing my chart after every visit.

Blog Updates

2009-01-07T15:46:00.001-08:00

I have been researching ways to best notify people on blog updates, and have decided to do two things:

First, there is now a link on the right hand side called "Subscribe to Baby Boston's Marathon." This will allow you to add Baby Boston's Marathon to your homepage and see in an instant if there have been updates. I use this method to stay up-to-date on the blogs that I follow and can check all ten of them at once.

Second, for those who are interested, I am willing to send out an email notifying you that I have updated the blog. Send me an email at ihearttheprestons@gmail.com if you would like to be added to the email notification list.

If anyone has other ideas on how to notify readers about blog updates, I would love to hear them.

Thanks!

NSTs -- Non-stop testing

2009-01-05T11:26:00.000-08:00

I went in this morning for my fifth non-stress test (NSTs). For four out of five of the tests I have been with Nurse Julie -- who is great. She asks me questions about Clark and my family and we pass the 20 minutes in relative uneventfulness. Boston has a tendency to fall asleep during these tests -- probably because I am reclining and giving him plenty of room to relax. I don't blame him -- if the tests were longer I would probably fall asleep too -- after all, it is 7:30 in the morning. For every NST except the first one we have had to use the fetal movement stimulator, which is a handheld buzzer that Julie presses against my abdomen. It is basically a baby alarm clock and gets Boston to jump and hopefully wake up and move around so we can record the necessary heart accelerations. We have not had any problems with the testing -- his heart responds effectively every time he moves; it's just the getting him to move that is the problem. Because of this, we have a tendency to go beyond the minimum 20 minutes into the 30 minute range.

This morning I also had an office visit with Lynne, the nurse practitioner. I was happy to report that I was experiencing some relief from such bad heartburn and that I had no issues to report other than the run-of-the mill pregnancy woes. She measured my abdomen and said I was right on track. She also used a portable ultrasound machine to check out Boston's position and fluid level. That was really cool. The picture on the machine was grainy, but she was able to find out essential information in just a couple of minutes, like the fact that he had plenty of fluid surrounding him and that he was almost head down, but not quite. If you painted a clock on me, with 12 at my head, he would be between 7 and 8.

I have another NST on Thursday, and two more next week, and then a growth scan on the 19th. Those are my favorite, because it is basically a 45 minute show all about Boston. I will keep everyone updated on the testing -- and hopefully there won't be too much more before Boston arrives!

He's Always Been Faithful

2008-12-24T07:49:00.000-08:00

Yesterday we had another amazing sonogram! Boston had a growth spurt in the last month that exceeded both mine and the doctor's expectations. He is now an estimated 6 pounds 5 ounces. He is measuring like a baby who is full term -- 37 weeks! That is almost a full month ahead of my due date. The doctor commented on his super-long limbs (just like his daddy), his gigantic melon (the biggest measurement was his head -- means he must be smart) and my favorite comment from her was -- "Oh my goodness, look at all that hair!" I am picturing lots of dark hair like his daddy (even though I know Brian was blond when he was born). It was a great time to spend with my baby boy and to see how he has grown. The fluid level is still good and his kidneys did not look enlarged or bright.

What this ultrasound means for his diagnosis: nothing. Unfortunately, regardless of how magical and special each positive sonogram is and how much we treasure getting to see our sweet little boy, nothing done prior to his birth can erase the doctor's early findings of a high AFP and the genetic testing. A baby can look perfectly fine on the sonogram (like Boston) and still be born with Pierson Syndrome, so we will still have to wait for him to be born to know for sure.

I also had my first non-stress test (NST) yesterday. It is a very simple procedure where they hook up monitors to my belly and listen to Boston's heart. They are watching for movement and heart rate accelerations that accompany the movement. Each NST is 20 minutes long and they are looking for at least 2 accelerations in the 20 minutes. Boston had his first two within the first minute or so, but we still had to wait the full 20. I will be getting NSTs every 3-4 days until he is born. Luckily, I snapped up all of the 7:30 am appointments so I won't have to miss any work.

Yesterday I also met with Elaine Swinehart, the Maternity Care Coordinator, who helped me fill out all of my admission paperwork and consent forms. She read through a copy of our birth plan and said it was very well done. She took me on a quick tour; I will have a more extensive one when I go back to meet with the neonatologist. I was not expecting how the visit to the NICU would affect me.

I walked into the NICU and all of a sudden it struck me that this was not a place I wanted to be. Although they try to make it as friendly as possible, you don't have to be there for more than a few seconds to know that the babies here are sick. On the ceiling, part of which you can see in the picture, is the phrase "Wish I May, Wish I Might, Grant the Wish I Wish Tonight." It hit me that the wish these parents wanted granted was for their babies not to die, and that was overwhelming -- a parent's plaintive plea for their child to survive. All of the doors are glass for security reasons and so you are surrounded by room after room, bassinet after bassinet, of babies who are gravely ill. I teared up as I realized that Boston could be coming here and I had such a surge of protective mother instinct -- thinking "I don't want my baby to be here -- I don't want him to be sick. I don't want to have to wish that he would live." I am pretty sure that this aversion to the NICU will pass; after all, I want Boston to be where he needs to be in order to get better. But I was surprised how much it affected me.

The title of this blog is from a Sara Groves song. As we celebrate our positive sonogram and wait for Boston to be born, I recognize God's faithfulness. We will not be tempted to fall into sorrow or despair, or to let our hope wane. As Paul says in 1 Corinthians 10:13 No temptation has seized you except what is common to man. And God is faithful; he will not let you be tempted beyond what you can bear. But when you are tempted, he will also provide a way out so that you can stand up under it.

Here are the lyrics to Sara Groves' song "He's Always Been Faithful"

Morning by morning I wake up to find
the power and comfort of God's hand in mine.
Season by season I watch him amazed,
in awe of the mystery of his perfect ways

CHORUS: All I have need of his hand will provide.
He's always been faithful to me

I can't remember a trial or a pain
he did not recycle to bring me gain.
I can't remember one single regret
in serving God only and trusting his hand.

CHORUS

This is my anthem, this is my song,
the theme of the stories I've heard for so long.
God has been faithful, he will be again.
His loving compassion, it knows no end.

CHORUS

Getting ready...

2008-12-22T08:36:00.000-08:00

This weekend we made some exciting progress towards Boston's birth.

On Saturday, Brian washed Boston's clothes while I set up the nursery. For right now, we have put all of Boston's stuff in the guest room because we don't know when (or if) we will come home from the hospital or what kind of set-up we will need when we get there. There may be some major furniture rearranging depending on what we need, for a dialysis machine, feeding apparatus, etc. But for now, I blissfully arranged his Winnie-the-Pooh baskets on his shelves, filling them with onesies and sleep and plays and baby washcloths and booties and other assorted baby-morabilia. I separated out the smallest items in case he is less than 8 pounds and we need some preemie or newborn clothes. They are the tiniest of outfits and could fit on his teddy bear! Clark helped arrange things. He was highly disappointed that he could no longer fit into his swing. He looked adorable as he peeked over the bassinet to see what was inside. For now, it only holds a green teddy bear and a blanket, but hopefully he will soon get to peer at his baby brother! The new paint in the guest room looks great with all of Winnie-the-Pooh decor.

Our second baby-related event happened overnight on Saturday. I tossed and turned all night and had major lower back pain. I couldn't figure out what was going on until Sunday morning when I got out of my bed and realized that Boston had majorly changed positions. I will find out at the sonogram tomorrow whether or not Boston has officially "dropped" but I can tell you that it sure feels like it. I used to have a shelf that I could rest a drink on, but now he has moved down much lower. Although I can breathe easier, it has made walking much more difficult. For first time moms, the baby can drop weeks ahead of time. Since this is my second pregnancy, I was not expecting Boston to move down quite so early, but hopefully I still have a few weeks left before I deliver. The doctors have agreed that it is medically impossible for my due date to be Feb. 12, so I have a due date "range" of Feb. 1 to the 12, making my due date 6 to 7 1/2 weeks away. I want to get everything done at home and at work so I don't have procrastination stress.

My sonogram is tomorrow at 4 and I have my pre-admission appointment at the hospital at 6. I am excited to see how Boston is growing and to get a tour of the maternity ward at St. Luke's.

My two boys

2008-12-10T12:36:00.000-08:00

We had 3d pics taken of Clark as well, and here are a couple of side by side comparisons. We will see how they play out when Boston is born.

More than halfway there!

2008-12-10T11:57:00.000-08:00

We found out about Boston's likely diagnosis 10 weeks ago today. His due date is 9 weeks from tomorrow. It is hard to believe, but we are more than halfway through this first period of prayer, the waiting for his due date. The hardest thing about the four month period between the time we found out about his possible diagnosis and our due date is the uncertainty -- knowing that all we can do is wait and pray and trust in God. The most fun thing about this time is that right now Boston is doing great! He is living his little life to the fullest, kicking and growing and moving around. I can only wonder about what his little brain is thinking -- most likely something along the lines of "my Mom is awesome; she takes such good care of me...keeping me fed and warm and letting me listen to good music. My dad is awesome too...I like it when he talks to me and tells me how I am going to look just like him. I like Clark too, but I wish he wouldn't always sit on my head."

The comfort of knowing that he is okay right now is also part of the uncertainty -- not knowing if right now is the only time he will be "healthy" and okay. So while I am anxious to meet him, I also want him to stay right where he is for as long as possible, because I know he is safe and taken care of. This is the part where God comes in...where he tells me that "My grace is sufficient for you, for my power is made perfect in weakness." (2 Cor. 12:9). God will perfect his power not only in Boston's weaknesses, but in my own. And so I trust in that. And as Paul put it so perfectly: May the God of hope grant you all joy and peace as you trust in Him, so that you may overflow with hope by the power of the Holy Spirit. Romans 15:13 Can you picture that? Someone overflowing with hope? Gushing with hope? Spraying hope like a firehose? Sweating hope? Walking around with an aura of hope? Beams of hope shooting from their eyes? It's a visual picture. And not just hope, but ALL joy and peace as well. I can't imagine a more perfect place to be then filled with all joy and peace and overflowing with hope. Call it that "pregnancy glow."

2008-12-10T08:04:00.001-08:00

A view of Boston's right side: his head, shoulder and right arm and a bit of his chest.

Boston's face and a bit of his foot.

A shot of his lower face and chest.

Boston's face.

Boston rubbing his left eye with his left hand; right hand tucked under chin.

2008-12-10T08:03:00.001-08:00

Boston grabbing his foot with his hand.Rubbing his right wrist against his mouth -- all tucked up.

A shot of his five fingers :)

Curled up -- arms crisscrossed across his face, belly in the middle and crossed legs.

One beautiful foot

2008-12-10T08:02:00.000-08:00

A shot from the back -- Boston's legs crossed at the ankles and the right side of his bottom.Boston's right leg.

Profile pic -- left arm by left eye

A closer view of Boston

2008-12-10T07:57:00.000-08:00

We had our 3-D sonogram of Boston last Wednesday. My mom and dad came with Brian and Clark. We brought the portable DVD player so Clark spent more time watching "The Wiggles" then he did watching his baby brother. We were grateful that he was so entertained by the show that he didn't try to pull me off the sonogram table or turn off the big screen tvs. It was wonderful to see Boston close up. He looks so cute! The biggest difference that we saw between him and Clark were their feet. Boston only has five toes, as opposed to Clark's six, and his feet look so narrow compared to Clark's. Enjoy the pics!

Happy Thanksgiving

2008-11-27T06:49:00.000-08:00

The news yesterday was all GOOD! Our beautiful baby boy is now 4 pounds, 1 oz, has fat on his face and is growing hair. He looked great all over. He had normal amounts of fluid, normal looking kidneys, and FIVE fingers and toes on his hands and feet. He is still measuring big with an estimated due date of January 23. We took a good look at his heart and it is beautiful. Patti Lewis from Alexandra's House came with Brian and I while my mom stayed at home with Clark. We are so blessed.

Christmas is coming!

2008-11-25T07:51:00.000-08:00

Sometimes I can't believe that I will still be pregnant at Christmas! Clark was born on December 12, and so we got to have him with us at Christmas. This year I will be about six weeks from delivery at Christmas, give or take. I could play Santa Claus with my belly full of jelly. Ho ho ho!

There is another effect of Christmas coming and not having Boston with us and that is the thought of the unknown. Thoughts like...what if Boston never gets to celebrate Christmas? It is not a new thought. I had the same thoughts on my birthday...that this could be the only time I get to celebrate with Boston. Because of that, we are trying to incorporate him as much as possible into the holidays.

The other effect of Christmas coming is the comparison of our baby to baby Jesus. There is a song on the album City on a Hill: It's Christmas Time and when I listened to it yesterday I broke down in tears. I was listening to the cd in the car and not paying much attention till this song came on. The song is "Child of Love" by Sara Groves. Here are the lyrics:

Child of wonder, close Your eyes
Rest here in my arms tonight
Someday You will save the world
But tonight I'll hold You, right here in my arms

Chorus:
Precious miracle of life, child of love
Gift of hope, the gift of light
From the Father above
And You were made for all mankind
But You will always be mine, child of love

Gabriel's promise has come true
God has blessed this world with You
And as I humbly hold You now
In my heart I know I'm holding heaven's child

You can listen to the song here: http://www.amazon.com/gp/product/B00138DJPQ/ref=dm_mu_dp_trk6

It is such a powerful song of Mary's love for her baby Jesus and the bond she felt for him, the need to protect him and comfort him, even while knowing that he was destined for so many great and terrible things. It reminds me of the wonderful joy that is coming when I get to hold Boston in my arms and he is real, tangible, and mine. Knowing that heaven has destined for us to have some time together is such a blessing, whether it be weeks, months, years, or a lifetime.

Sonogram tomorrow!

2008-11-25T07:34:00.000-08:00

We have so much to be thankful for this year: for the beautiful gift of Baby Boston, for family and friends lifting us up and surrounding us with prayer, for all of the ways God has come near to us, comforted us, and encouraged us, for a wonderful year spent growing closer to each other in our marriage and as a family, and for Clark, who gets more and more amazing every day.

Tomorrow we hope to have another blessing to be thankful for...we have another sonogram at St. Luke's. We are praying for Boston's growth, first and foremost. We are praying that his kidneys look good. We are praying for healthy fluid levels. While these things don't tell us whether or not he has Pierson Syndrome, they allow him to stay in utero longer and continue to grow, which will give him a better chance of survival once he is born. Normal fluid levels tell us that his kidneys are still functioning, meaning that he may not need to be put on dialysis right away after he is born. Normal looking kidneys mean there are no tumors or cysts. And growth means just that...he is growing. At our last visit the doctors told us that one of the primary things they look for when there is a high AFP is a baby who does not grow.

Another thing we are thankful for is that we are going to be able to receive a 3D/4D sonogram and see Baby Boston up close. A week from tomorrow, on December 3rd, we are going to be able to receive this wonderful gift from Jeanette Burlbaw at Prenatal Imaging. We were connected to Jeannette through Alexandra's House. Jeanette offers her services for free to families in situations such as ours. She has scheduled us at the end of the day so we can have plenty of time to see Boston. When I was talking with Jeanette, she was astounded to hear that Boston is sonographically normal. So many babies who face terminal diagnoses also have physical deformities that appear on the sonograms. While Boston's outward physical perfection is a gift from God, it is also something that I struggle with, knowing that my baby who appears perfect could have a life-threatening disease. I want to scream "look at him, there is nothing wrong with him, he is perfect!" But we are so blessed and excited to get to see his perfection up close. We are going to be looking for six fingers and six toes :) and hoping that he looks like Brian. I tell Brian all the time that Boston is going to be a mini-version of him...dark curly hair, lean features, and long legs and arms. I don't know if he believes me. As cute as a mini-Brian would be, who could not want the alternative? Another mini-version of me, who looks just like his big brother :) We will post pictures of Baby Boston next week after the 3D/4D sonogram. To see samples of Jeanette's work, visit her site at: http://www.prenatalimaging.com/

Alexandra's House

2008-11-25T07:21:00.001-08:00

Last Thursday, November 20th, I met with Patti Lewis from Alexandra's House. Alexandra's House is a charitable perinatal-infant hospice house and refuge for abandoned or neglected babies. They provide spiritual, grief and practical support to families pregnant with babies who have a terminal diagnosis. They attend medical visits, develop compassionate and comprehensive birth plans, go to labor and delivery, maintain vigils through the babies' death, bathe and dress the babies, and participate in funerals and in long-term bereavement care, following up with families for three years after the babies' death. They are the "high touch" complement to "high tech" obstetrical care. Patti Lewis founded the house following her experience with her niece Alexandra's diagnosis of a fatal genetic disorder. She lives in an actual "house" near Westport, making it an ideal proximity to both Children's Mercy and KU Med.

I emailed Patti after we got Boston's diagnosis and explained our situation, outlining that Boston's diagnosis was not definitive and would not become any more definitive before he was born. I explained that because of this uncertainty, I wasn't sure how to prepare. I told her we still have hope that Boston could be born normal or with a mild form of Pierson Syndrome that may allow him to survive, which forces us to prepare both for his life and his death at the same time. She wrote me back and said she thought we would be a great match for our services. In situations like this, they help parents prepare a plan for the worst then put that on the shelf, so to speak, and enjoy the pregnancy.

So I met with Patti last week and she was wonderful! I was there for over two hours, but the time flew by. I took Brian and Clark back on Sunday for an open house and they got to meet Patti and look around the house. There are two apartments upstairs for people to stay in. If necessary, we would be able to come to the house for respite, naps, showers, etc., if Boston is in the hospital for an extended period of time. I am so grateful that such a giving place exists to meet our needs. The website for Alexandra's House is http://www.alexandrashouse.com Please check out the site and pray for the volunteers. The personal stories from the parents about their children are powerful and moving; I haven't ever been able to read one without crying.

Meeting with Children's Mercy Doctors

2008-11-13T08:32:00.001-08:00

Last Friday, November 7th, I had the opportunity to meet with Dr. Blowey from Children's Mercy.

The link to his page at Children's Mercy is here: http://www.childrensmercy.org/findadoctor/view.aspx?id=3241

From the moment I first sat down with him I knew that God had His hand on Boston and on the doctors that will be involved in Boston's care. The miracle of all miracles is that one of Dr. Blowey's colleagues in the Nephrology Department, Dr. René G. VanDeVoorde, has personally worked with a child with Pierson Syndrome!!!! Not only that, but he co-wrote one of the leading articles on Pierson Syndrome. This article was the first to examine a case of Pierson Syndrome in the United States. It was written in 2006 when Dr. VanDeVoorde was in Cincinnati. It is so awesome that one of the few people in the entire WORLD who has worked with Pierson Syndrome will be able to work with us. Pierson Syndrome is so rare; there are very few cases and even fewer doctors. We truly feel blessed and it is beyond mere coincidence that Dr. VanDeVoorde is available to work with us.

The link to Dr. VanDeVoorde's page at Children's Mercy is here: http://www.childrensmercy.org/findadoctor/view.aspx?id=9179

The link to Dr. VanDeVoorde's article on Pierson Syndrome is here: http://pediatrics.aappublications.org/cgi/reprint/118/2/e501

If you type "Pierson Syndrome" into Google, Dr. VanDeVoorde's article is the first entry.

From my meeting with Dr. Blowey, I got the following impressions:

1) They (the team at Children's Mercy) totally know what they are doing. They have more experience with congenital nephrosis than I expected and are on the cutting-edge of treatments and procedures. Dr. Blowey has personally seen about 5 patients with congenital nephrosis. Although it doesn't seem like a lot, given how rare the disease is, it was more than I was expecting.

2) They have a great respect for life and are very aggressive in their treatments and procedures. This is in contrast to other stories I have heard about mom's being told not to pursue treatment. It is also significant because the biggest factor in survival is the aggression and dedication of the parents and medical team, basically if they are "sold out" to the survival of the child.

3) They love what they do. Dr. Blowey referred to the patients as "kiddos," which I loved. It was such a personal reference and really brought home that he cared about the kiddos that he sees and works with.

4) They don't know what to expect. Dr. Blowey said that kids with the Finnish type of congenital nephrosis always look and act the same way so the doctors can tell you what is going to happen. Pierson Syndrome is different; there are mild forms, forms with kidney problems but not eye problems and some forms that don't present right away. His plan is to know all of the different possibilities and be prepared to act on what happens.

On the practical side, I got answers to all of my questions and have a good idea of the game plan once Boston is born. I also got a little bit of insight into how life workswith a baby who has congenital nephrosis.

Here's my best explanation of what I learned:

Prenatally
Although there are no more tests that can be run, Dr. Blowey wants the perinatal docs to look for a few things on the ultrasounds: bright kidneys, fluid level, and placenta size.

Dr. Blowey wants Boston to stay in utero as long as possible. If there is not a significant medical reason for him to come, then just wait on him. All this means is that I shouldn't have an elective induction or a planned C-Section -- if it is hard for me, too bad, 'cause as long as he's doing okay, he's staying in there. As a side note, Kansas just received a grade of "D" for pre-term labor. Apparently, 1 in 8 pregnant moms deliver early. This is due to an increase in elective inductions and scheduled C-sections that are done for the mom's convenience. In general, this practice is discouraged as it is not optimal for the baby.

Initial testing
Once Boston is born, we will likely do the following tests:

Physical exam (standard for all babies): we will be looking particularly at his eyes to see pinpoint pupils, as well as any other physical abnormalities that didn't show up on the ultrasounds. This will be done at birth.
Blood tests: checking the blood for creatinine, protein, and fat levels. This will be done daily. The levels at birth are not always accurate, so it could take up to a week of daily tests to make sure that what we are reading is accurate.
Urine output: is he peeing enough? The answer is probably yes. Most babies with CNS (congenital nephrosis) have functioning kidneys, meaning that the kidneys are filtering fluid; it is just that in the normal amount of urine they are dumping huge amounts of protein. Urine output can also take a couple of days; some babies don't pee on the first day.
Urine tests: check the urine for protein. This will be the most important. If he is dumping massive amounts of protein, then that would be what gets him bumped immediately from St. Luke's to Children's Mercy. The doctors will know that they can't mess around and have to start some aggressive interventions immediately. With all of the prenatal testing, we have a heads-up on what we are dealing with and don't have to mess around with a bunch of treatments that aren't going to work. That is what happened in Dr. VanDeVoorde's case in Cincinnati -- they didn't know what it was, so they spent a week or two trying things that didn't work.
Ultrasound: to get a better look at his kidneys up close.
Genetics: send his blood back to Athena and have them redo the genetic test.
Kidney biopsy: we aren't for sure on this test because it is painful and dangerous, so we are going to explore the benefits/risks before deciding to do it.

With all of these tests, we are planning on being in the hospital at least for the good part of a week, although Dr. Blowey advised that we could be there for months. I kind of skidded past the "months" issue, but I am sure we will discuss it more in the future. We have specified in our birth plan that we would like to have as much testing done at St. Luke's until I am released. Barring any complications with me, I don't expect to stay more than 48 hours before I am discharged. Once I am discharged, I don't have a preference where the tests are done. Dr. Blowey said that unless there are high levels of protein in his urine, we should be able to stay at St. Luke's for a couple of days. He said even if the team at St. Luke's gives Boston the all clear, he wants to take a look at him before we go home.

Even if things go great at birth, because CNS can be progressive our care will still be heightened for a while. We will have weekly visits for a month or two, then bi-weekly visits for a while, then monthly visits. That is if everything goes well. If not, we will be back to visiting the doc more often. Thank goodness the IRS counts mileage as a medical expense!

Feeding
I was happy to hear that breastfeeding is preferred. Most of the time supplements will also be necessary. Dr. Blowey said that all the kiddos he has seen that have congenital nephrosis have also needed a feeding tube, either one through their nose or one that goes directly into their stomach. He said a lot of kiddos don't eat very well -- they can't/don't nurse. But he supports and encourages breastfeeding, and since I have "super milk," I was glad to hear it.

Treatment
New information for me was that sometimes in treating kiddos with CNS, they preemptively remove both kidneys and start them on dialysis before the kidneys have actually failed. This stops the kidneys from dumping huge amounts of proteins and thus relieves the secondary conditions like swelling, high cholesterol, high blood pressure and risk of infection. Of course, the younger the kid is when you do this, the more risky it is. A baby on dialysis has increased risk of death, infection and is more likely to be restricted in its growth. This looks like a really tricky balancing act to decide when and if to preemptively remove the kidneys. Once the kidneys shut down, you don't need to remove them.

Dialysis
If necessary, we will do dialysis at home at night with support from a home health nurse. The type of dialysis that the use is through the tummy and it is automatic, which means I don't have to get up 3 or 4 times during the night to do the exchange. There is an alarm on the machine that will let me know if something is wrong.

Kidney Transplants
Most kidney transplants are done around 2 years of age. Dr. Blowey said it was not just the weight of the kiddo, but their general size. Their body cavity has to be large enough to fit the kidney.

Donors
The most important factor is blood type and then the general health of the donor. It is not whether or not the donated kidney will screw up the kid, but whether the donation of the kidney will leave the donor too bad off. He thought Brian might get knocked out because of his myotonia.

Follow-up
Although Dr. Blowey only meets with prenatal moms one day a month, he told me that he recognizes that this is a special case and is treating it as such. He told me he was going to talk to Dr. VanDeVoorde this week and that we would talk again this Friday, November 14.

If you read this and think of other questions, please email me or comment. I am sure there are other things I haven't thought of.

Boston's latest ultrasound looks good!

2008-11-03T07:20:00.000-08:00

We had our ultrasound on Friday. I was tempted to paint a big orange pumpkin on my belly, but I thought it might get a little messy with the sonogram goo. My mom went with me to the appointment and we were delighted to see that Boston is GROWING!!! One of the risks with a high AFP is "growth restriction" where the baby isn't growing like he should be. Boston is a champ and is nearly hitting the 90th percentile for his gestational age. He is already 2 pounds 2 oz. We got a detailed look at all of the parts of his little body. Checking in from last month, the look of his kidneys continues to improve. This time, there was "no discernable brightness." Yippee! We prayed "that Boston’s kidneys will continue to improve and show no sign of “texture” or “brightness” on the ultrasound." That prayer has unequivocally been answered, at least for this month. The doctors do not know what this means overall for his health because Pierson Syndrome has not been studied in utero, so we don't know if Boston could have "normal" looking kidneys and then still have the disease. They also don't know the significance of the "bright" kidneys earlier in the pregnancy. My doctor reiterated the seriousness of my AFP levels and said that to call them "high" would be the understatement of the century. The doctors seem genuinely amazed that Boston is still alive. We are genuinely amazed at God's grace and power.

In other great news, Boston's lungs have developed to the point where the amniotic fluid level is no longer necessary for their development. We have prayed "that the level of his amniotic fluid will stay normal in order to allow his lungs to develop." That prayer has been answered.

We will meet with Dr. Blowey this Friday to talk about what to expect when Boston is born and what kind of testing will need to be done. That appointment will wrap up our "information-gathering phase" and allow us to prepare our expectations for the birth.

We won't go back to the hospital for another month -- our appointment is the day before Thanksgiving. We have so much to be thankful for already -- and God continues to abundantly provide.

Laminin and the Cross

2008-10-28T11:21:00.000-07:00

Louis Giglio, while touring with Chris Tomlin on the “How Great Is Our God” tour, gave a speech about laminin. Laminin is the protein gene where doctors have found at least one of Boston’s mutations. We are waiting to find out how damaged Boston’s laminin gene is and the effect it will have on his functionality. Giglio’s goal on the tour was to give the hearers of his message “the confidence that [God] is able to hold onto us and hold us together no matter what circumstances come our way in this lifetime.” Giglio’s point about laminin was its shape – the cross. Giglio suggested that when we peek into the micro-biology and building blocks of life we find that we are held together by a cross shape glycoprotein matrix. The rebar of the human body is in the shape of a cross. He jumps to Colossians 1:15-20 where Paul in discussing the supremacy of Christ says, “He is before all things, and in him all things hold together… making peace through his blood, shed on the cross.”

How great is our God that in this time of waiting for Boston to be born He draws our attention to the laminin gene – a gene in the shape of a cross – to remind us that Jesus’ sacrificial love on the cross is what truly holds us together, even when the building blocks of life break down.

For a link to Louis Giglio's sermon on youtube, click here: http://www.youtube.com/watch?v=_e4zgJXPpI4&feature=related

High-Risk Pregnancies

2008-10-24T07:23:00.000-07:00

I wanted to clear up any confusion about Boston's diagnosis and what it means for me and the pregnancy. Physically, I am absolutely fine. Pregnancies can be classified as "high-risk" for two reasons: either (1) there are issues with the mother and/or (2) there are issues with the baby. We are at St. Luke's because of the second reason: there are anticipated issues with Boston. There are, however, no issues with me. My blood pressure is good; my kidneys work great; there are no problems with my blood or urine. I have even kept my weight gain down to only a few pounds (which for me is an accomplishment).

Additionally, there are not any anticipated problems with the progression of the pregnancy. There are no more tests that have to be done to put Boston at risk, no in utero kidney biopsies or surgeries would be helpful. I shouldn't have to be on bed-rest or anything like that, and as of right now, we are not anticipating that Boston will need to be born early. Personally, I don't think there is any way that he will make it to his actual due date of February 12, because every sonogram that we have had shows him about 2 weeks ahead on his growth. So I think he will be born closer to Feb. 1, which is what I thought my due date was to begin with.

Also, Boston's kidneys shouldn't cause him any problems in utero. Right now, my kidneys do all of the heavy lifting for him, filtering all of the blood and fluid that goes to him. And my kidneys work great, so that means his kidneys get to take a break. It is like being on free mommy-provided dialysis.

The only thing that we are watching for that could cause Boston to come early is low levels of fluid around him. A lack of fluid means his kidneys have shut down completely. The fluid is necessary for his lung development. So far the fluid level has been great - just the perfect amount. Each week that passes with Boston still inside is another week for him to grow and develop and become a strong, healthy baby. Grow, Boston, grow!

Upcoming Appointments

2008-10-24T07:07:00.000-07:00

Although there are no other tests that can be done to make Boston's diagnosis any more or less conclusive, there are a couple of upcoming appointments that we are preparing for.

October 31 - Sonogram and doctor's appt: one week from today I have my "new patient" appointment with St. Luke's Perinatal Center. Until now, I had been seen by Dr. Magee and Crystal Murphy at Women's Care Group at Shawnee Mission Medical Center and had been going to St. Luke's for specialist consultations. With the likely diagnosis of Pierson Syndrome, it was recommended that I transfer all of my care to the Perinatal Center to work with Dr. Elizabeth Wickstrom, who is a perinatal specialist, meaning that she works exclusively with high-risk pregnancies. It will be nice to have all of my care located in one place because it means fewer doctors visits and more centralized care. All of my visits will be downtown, so I won't have to miss much work to go. Even though I have been there before, this appointment is classified as a "new patient" appointment. I will spend about an hour getting a detailed level 2 sonogram done, which is what I am most looking forward to. The sonograms really let me "spend time" with Boston, getting to see him as a real person and watching him move around. It is so neat to see what is causing all of the bumps and movement in my belly. I am also to eager to see how his kidneys are doing. How they look will give us a better idea of what to expect. If they look "bright", then that will give us a better idea to expect issues at birth. If they look normal, then we will have reassurance for another month that his kidneys look normal. It doesn't guarantee that he will have no problems when he is born, but it could tell us that at that moment they look fine.

I will also have a "nurses appointment" and a "doctors appointment." Since I haven't been a regular patient there, I don't know what these will consist of or why they are different. I will be glad to have my mom there for support through all of the appointments.

November 7 - Meeting with Dr. Blowey: Dr. Blowey is a baby kidney doctor at Children's Mercy Hospital. On the first Friday of every month he meets with pregnant mommies who have kidney diagnoses for their unborn babies. I will be interested to find out from him the ins and outs of baby kidney disease and what will happen when Boston is born. I am going to ask him what kind of tests they will do and when, what will be the circumstances where he has to stay in the hospital, how long will it take to find out if he has this disease, if he is born "normal," how long will we know that he really is "fine," and anything else that I can think of. I have been writing down questions as I think of them, but I welcome suggestions of things to ask. If you have had any questions about Boston's condition, please send them to me so I can make sure to ask Dr. Blowey about them. I am looking forward to talking with Dr. Blowey because he is the one that will be able to answer all of the questions about what will happen after Boston is born.

Updates to the blog!

2008-10-13T10:30:00.000-07:00

I have put up a new picture of Boston, a list of prayers and accompanying bible verses, and (my favorite feature) a playlist at the bottom that plays the music we are listening to for comfort and support. Enjoy!

Prayer

2008-10-07T15:16:00.000-07:00

We want to keep track of who is praying for Boston so we can update you directly when we list new prayer requests and so we can delight in the power of prayer and strength in numbers. We think it will be a wonderful testament for Boston to see how many people were praying for him before he was born. Please email us at ihearttheprestons@gmail.com or leave a message in the comments.

Also, we are asking that we be added to any prayer chains or prayer ministries you have at your place of worship. If you are willing to involve Boston in corporate prayer, please let us know that as well and include the name of your organization.

If we didn't mention this before, please feel free to share this information with anyone that you know. We are not shy about making Boston's condition known and are hungry to connect with people who can offer support, encouragement, information, or experiences through like circumstances. Pass along our contact info and the link to the blog.

Thanks!

The results are in!!!

2008-10-05T13:00:00.000-07:00

The results are in and the marathon continues! We were waiting in anticipation for the results from Athena Diagnostics. In addition to testing for the two main causes of congenital nephrosis, mutations of the NPHS1 and NPHS2 gene, the lab also tested two of the rarer causes of baby kidney disease, mutations of the WT1 and LAMB2 genes. Unfortunately, the lab was not able to provide any definitive answers. We know for sure that Boston does not have congenital nephrosis of the Finnish type, because there were no mutations of the NPHS1 or NPHS2 genes. However, there were issues with the other two genes that make the doctors think it is likely that Boston will be born with a fatal syndrome that can also cause congenital nephrosis. We will not know for sure if he has these syndromes until he is born, but there are markers we will be watching for along the way that will tell us if it is more/less likely. We view this as an opportunity for increased prayer and faith in God’s unending grace and mercy, not to mention his unlimited power to heal both body and soul.

Our doctors received the lab results on September 30, 2008, and spent 24 hours researching and making phone calls to make sure that they had all the available information they could get. We are grateful for their diligence and hard work and are confident in both their knowledge and dedication to finding the correct diagnosis for Boston. Only two labs in the world test the LAMB2 gene, our lab, Athena Diagnostics, and a lab in Germany. The WT1 and LAMB2 genes were so recently linked with congenital nephrosis that Athena Diagnostics just started testing for them in July 2008. Boston is the first prenatal screen of the LAMB2 gene that has been performed; all other screens for the gene were done after the baby was born and the external indicators pointed in the direction of the disease and the genetic screen was done to confirm the syndrome. In our case, we are trying to diagnose the disease based on the gene alone, and will not have the external confirmations until Boston is born.

Our doctors were frustrated by the non-decisive results, but did their best to explain the outcomes and options. There are no additional tests that can be performed to give us more definitive results. Our answers will come after Boston is born. Until then, we wait, prepare, and pray.

We will do our best to try and explain the test results and what the doctors communicated to us. As we have stated previously, the first red flag was the incredibly high AFP result that came with the amniocentesis and the “bright” kidneys on the sonogram. All of the typical diagnoses for a high AFP were eliminated. Two explanations remained: testing Boston’s genes for some known causes of CNS and testing and testing Sarah for some sort of cancer/disease. Sarah has been all but ruled out as a cause for the high AFP.

We were expecting the results to focus around the NPHS1 and NPHS2 genes, because these are the most common. Instead, our results concerned the WT1 gene and the LAMB2 gene, which are the rare, newly discovered genes. The results from the tests of these two genes is where the uncertainty lies.

For each of Boston’s genes, he has two strands, one from Sarah and one from Brian. Understanding these tests requires knowledge of genetics from the freshman year of biology. Each trait, whether it is brown hair, blue eyes, or Pierson Syndrome, is either dominant or recessive. With a dominant trait, you only need the gene from one parent in order to have that trait. With a recessive trait, you would need a gene from both parents in order to have that trait. WT1 is a dominant trait, meaning that only one of the genes that Boston received would have to be mutated. LAMB2 is a recessive trait, meaning that both of the genes Boston received would have to be mutated. In looking at the genes, the lab technicians lay the two strands, one from Sarah and one from Brian, on top of each other and look for changes (variants) or mistakes (mutations).

The WT1 gene came back with what the doctors’ describe as a “variant.” This means that there is a change in the way one of Boston’s WT1 gene reads, but the doctors do not think it is enough to change the way the body performs. The example that our genetic counselor used was to look at the genes like a recipe. If the recipe calls for “sugar,” a mutation would change the word “sugar” to “salt.” This would mess up the whole batch of cookies. A variant is changing the word “sugar” to “suggar.” An experienced cook would look at the word “suggar” and know to add “sugar.” An inexperience cook might not know what “suggar” means and they may leave it out or just quit cooking. What we don’t know is what type of cook Boston is and whether his body will know what to do with the variant. Because mutations of the WT1 gene are dominant, it would only take one mutation for Boston to have this disease; however, so far the change has been classified as a “variant” and not a “mutation.” Athena Diagnostics’ response is that the variant is of “unknown significance.” Our educated guess is that Boston’s body will handle the variant just fine. There are some markers for a mutation of the WT1 gene that we can watch for on the sonogram and so far none of those are present. WT1 gene was so named after “Wilms Tumor,” which is a large tumor that grows on the kidneys when this mutation exists. So far, the sonogram shows that Boston’s kidneys are tumor-free. Another feature would be pseudohermaphroditic genetalia, meaning that we wouldn’t really be able to tell if Boston was a boy or a girl by looking at him from the outside. He is developing into a normal-looking boy, which is another reason we don’t think that the variant of the WT1 gene will cause any syndrome or disease.

Our prayers are going to be focused on the results of the LAMB2 gene. Mutations of the LAMB2 gene are known to cause a disease called Pierson Syndrome. Pierson Syndrome is a recessive disease, so both the gene from Sarah and the gene from Brian would have to be damaged in order for Boston to have Pierson Syndrome. The results from Athena Diagnostics told us that one of the genes is damaged. It was more than just a variant, it was a definite change in the gene and was classified as a mutation. If we knew definitely that only one of the genes was damaged, we would rest a little bit easier, because that would mean that Boston would be a carrier for Pierson Syndrome, but he would not have it. The doctors have told us that they cannot guarantee that the other gene is fine; all they can say is that they only found one mutation. It is possible that the other gene is mutated but they didn’t find it. Our prayers are focused squarely on this hope: that Boston has one broken gene and one normal gene and that he will be born without Pierson Syndrome.

Athena Diagnostics’ conclusions about this were as follows: Boston is at least a carrier for diseases related to mutations of the LAMB2 gene. The fact that one mutation was found “increases the likelihood that [Boston] may be affected with [baby kidney disease], even though the second mutation was not identified. However, the result is not definitive for the diagnosis of [baby kidney disease] since known or predicted disease-associated mutations in both alleles were not detected.”

Because of Boston’s high AFP, his “bright” kidneys, and the fact that he has one broken gene, we are preparing for the likelihood that Boston will be diagnosed with Pierson Syndrome after he is born. However, while we are preparing for that possibility, our prayers and hopes will be focused on Boston being born healthy and without complications.

If Boston does have two broken genes, it is likely that he will have Pierson Syndrome. Pierson Syndrome is extremely rare and new on the scene. The first diagnosed case in the US was in 2006. Only 30 cases have been identified in the literature so far worldwide. As we mentioned, Pierson Syndrome is caused by a mutation of the LAMB2 gene. The LAMB2 gene is so named because it produces the B2 chain of the laminin protein. There are twelve genes that help make the laminin protein: LAMA1-LAMA5: LAMB1-LAMB4; and LAMC1-LAMC3. Laminin is critical in forming the structural scaffolding of all of our organs, known as “basement membranes.” A mutation of the LAMB2 gene causes defects in the “glomeular basement membrane” of the kidney. Malfunctions of the GBM can lead to congenital nephrosis, which is discussed in detail below. A mutation of the LAMB2 gene also causes defects in the basement membrane of the eye. Babies born with Pierson Syndrome often have “pinpoint pupils,” called microcoria, due to a weakening of the muscles that dilate the pupils. Some kids are blind because of the changes in the eyes. The mutation of the LAMB2 can also cause a weakening in overall muscle strength (hypotonia) and a slowness of the brain’s reactions (psychomotor retardation).

As is evident from the above description, Pierson Syndrome is very, very bad. Of the 30 cases reported so far, none of the children have survived beyond age 5, usually due to complications with the nephrosis. This was devastating news to us when we heard it. We have taken time to adjust to the idea that we could lose Boston at a very young age.

However, we are encouraged by the fact that so few cases of Pierson Syndrome have been reported. We have recently been provided with studies that outline mutations of the LAMB2 gene that do not cause full-blown Pierson’s. These children, while they still have congenital nephrosis and some ocular abnormalities, generally have milder cases and therefore a more positive outlook. We are looking for information about Pierson Syndrome and are reading everything that is available about the disease. The “newness” of this mutation discovery puts us at an information disadvantage, but it also gives us hope that Boston could have a chance at survival if he does have the disease.

Nephrotic syndrome can be the side effect of some disease or can be the primary effect of certain syndromes. On its own, nephrosis is just a description of symptoms: lots of protein in the urine (proteinuria or albuminuria), swelling of the body (edema) that can cause high blood pressure (hypertension) and high levels of fat in the blood (hyperlipidemia). The flushing out of the proteins also means that a lot of antibodies are flushed out too, so there is also a higher susceptibility to infection. Nephrotic syndrome that occurs in older children and adults can be treated with steroids and so the prognosis (predicted outcome) is positive.

Congenital nephrosis is a different story. It is extremely rare and the prognosis is poor because it is resistant to steroids and other medications traditionally used to treat nephrosis. Congenital nephrosis will usually progress to end-stage renal disease within childhood (with Pierson Syndrome it is usually in the first year). When end-stage renal disease occurs, the kidneys shut down completely and the child can only survive through the use of dialysis. However, dialysis is not a long term solution and the child must receive a kidney transplant. The main complication is that babies/children have to reach at least 20 pounds before they can undergo a kidney transplant. Once end-stage renal failure occurs, it is very difficult to gain weight. The reason no child has survived beyond age 5 with Pierson Syndrome is because most the babies reach end-stage renal disease in the first few weeks or months of life, before they are 20 pounds. Then they can’t gain any weight and so the chance for a transplant is gone. Children with other forms of congenital nephrosis may not reach end-stage renal disease until they are older and so the option of a transplant is more available.

Bonus news about growth: as many of you know, Clark was breastfeed exclusively when he was a baby. Although he was only 7 pounds 10 ounces when he was born, he reached an unbelievable 20 pounds in a mere 3 months! Most babies do not reach 20 pounds until they are at least a year old. We would be blessed and thrilled if Boston followed in his older brother’s growth-steps and could put on weight so quickly. We can’t really count on it because the side effects of congenital nephrosis include a lack of appetite and problems with malnutrition. Most babies have to be on a battery of supplements and formulas, sometimes even intravenously, but we would love to have the chance to try and breastfeed Boston and see if he will super-grow.
A lot of times the best match for a kidney transplant will be one of the parents. One of the sites we have been visiting is www.cota.org, Children’s Organ Transplant Association. Most of the kids under the “kidney” link have received transplants from either their mom or dad. One of our prayers includes Brian or Sarah being a viable match for a transplant. Although a baby-sized kidney would be better than an adult-sized ones, the availability of a parent match is usually seized upon. Brian and Sarah have the benefit of both having the blood type of A negative, which is dominant, meaning all of our kids will hopefully have an A negative blood type as well. This should give us a great leg up for the transplant possibilities. For those who are wondering, Clark is out of the running to be a transplant candidate because (1) we wouldn’t put him through that and (2) even if we would consent to the testing, we couldn’t. Because the donation of a kidney puts you in a weakened state kidney-wise, he has to be of a certain age to consent to such a major surgery and we cannot consent on his behalf.

Whew! As you can tell, this is quite a bit for us to process. What we know right now is that Boston is doing great. Our ultrasound on October 1, 2008, told us that he is doing awesome. He is growing like a champ. He is topping the 90th percentile in size for his age. This ultrasound showed us that his kidneys didn’t look “bright,” but the doctor thought they looked “textured.” We will go back in four weeks for another sonogram. Most kids who have congenital nephrosis will have a couple of signs on the sonogram: (1) bright kidneys and (2) low levels of amniotic fluid. Both of those looked fine this time, and we will continue to watch for these signs.

Here are our prayers:

Our ULTIMATE prayers:

That Boston will only have one broken gene, not two, and that he be born normal and healthy with the correct production of laminin.
That we will be able to teach Boston how much God loves him and raise him to know Jesus as his Lord and Savior.
That this experience will strengthen our relationship with God and be an opportunity for us to share our faith with others.

Prayers for the pregnancy:

That Boston’s kidneys will continue to improve and show no sign of “texture” or “brightness” on the ultrasound.
That the level of his amniotic fluid will stay normal in order to allow his lungs to develop and to reassure us that he does not have complications.
That we will focus on the joys of each day and be reassured that “Today Boston is GREAT” and not borrow the unknown sorrows of the future.
That every day we will “Live, Love, Forgive, and Never Give Up.”

Prayers if Boston is born with a disease:

That he be healed completely.
That he has a mild form of disease that does not affect his functionality.
That he will have use of his eyes and not be blind.
That his muscles will be strong and his brain alert.

Prayers if Boston needs a kidney transplant:

That he will grow quickly and gain weight well in order to reach transplant size.
That Brian or Sarah would be a match for the transplant.

We will be updating this site with new information about Boston and information about the possible disease. We will also be including songs and bible verses that have been impacting us, and prayers that we are lifting up.

Bible verse: Psalm 37:23b (New Living Translation) “The Lord delights in every detail of our lives.” This short verse reassures us that as God was makes Boston, he delights in every detail, including the writing of his genes.

Full Chromosome Analysis Is Negative (Which is GOOD)

2008-09-12T17:42:00.001-07:00

Susan, our genetic counselor, called today with the results of Boston's full chromosome analysis, which analyzed the genetic structure of all of Boston's chromosomes. No chromosomal abnormalities were detected. These were the results we were expecting, as our concerns have been focused on Boston's kidneys, not his chromosomes. The amniocentesis looks at two things: the AFP level and the full chromosome analysis. It is always great to hear good news, and the lack of abnormalities in his chromosomes rules out things like Down Syndrome and other chromosome disorders.

Baby Boston's Marathon

2008-09-08T14:13:00.000-07:00

Sarah and Brian are thrilled to be expecting another son to be born in February 2009. His name is Boston Preston, and he is a confirmed boy with XY chromosomes. He is growing and developing well; however, there may be something wrong with his kidneys. The thorough and dedicated doctors (especially Susan, our genetic counselor) at St. Luke's hospital have been wonderful. Boston, like all other babies, produces hormones and proteins that we adults do not make. One of these proteins, the alpha-fetoprotein (AFP) is MUCH higher than normal. The reading in Sarah's blood is 20 times higher than normal. After performing amniocentesis, the doctors discovered that the AFP reading in Boston's amniotic fluid was 65 times higher than normal.

We have already been blessed, because 90% of the babies with an AFP reading as high as Boston's are either already dead or dying. Our baby boy, however, looks great on the ultrasound, with the exception of a couple of "bright" kidneys. He is growing great and all of his developmental markers are being met. The doctors have ruled out most of the causes of such a high AFP, including spina bifida or other open neural tube disorders, any sort of bleed or rupture around the baby, and Sarah having cancer or liver disease.

The remaining item on the doctors' "what could be causing this" list is a rare (as in 1 in a million) and life-threatening genetic disease called "Finnish Congenital Nephrosis." It is so rare there is not even an entry for it on Wikipedia. Roughly translated, the name means "kidney disease you have from birth." The "Finnish" part indicates the fact that while the majority of the people in the world (including us) have a 1 in a million chance of having congenital nephrosis, but people in Finland (for whatever reason) have a 1 in 8000 chance of having congenital nephrosis.

Lucky for us, there is a genetic test to diagnose this disease and it has a 95% accuracy rate. Because the disease is so rare, there is only one lab in the country that does the test. Coincidentally, it is also the same lab that did the genetic testing for Brian's myotonia. Athena Labs, we thank you. The lab will actually take a grown culture of Boston's DNA and read it forward and backward, looking for the genetic marker. Because of the involved nature of this test, it will take 2-3 weeks from the time the lab receives the sample before it can get us the results. The lab should get the sample sometime within the week, so we should have the results within a month or so, around the first of October.

Although the disease is life-threatening, there is a definite plan of action that is taken for babies with this disease. Provided that the baby makes it to birth, the baby is put on dialysis until he is big enough (20 pounds) for a kidney transplant. If the kidney transplant is successful, the baby will take anti-rejection drugs for the rest of its life, but there are no other anticipated complications.

Regardless of whether the test for "Finnish Congenital Nephrosis" comes back positive or not, Boston still has a ridiculously high AFP. As a frame of reference, babies with spina bifida have an AFP that is 3 or 4 times too high; babies with congenital nephrosis usually have an AFP that is maybe 8 or 9 times too high. Boston's is 65 times too high. Like all Prestons, he seems to be an over-achiever :) Provided this AFP stays high, which without God's miraculous intervention it is likely to do, Sarah will have increased monitoring to track Boston's growth and confirm his survival. Every time it is confirmed that his heart is still beating, it will be a miracle.

As we wait a month or so for the results of the test, we have some specific prayer requests. We are not timid in asking God for what we want, and ask that you do the same:

That Boston would be completely healed and that he will be born healthy with no complications.
That the doctors will find a cause for his ridiculously high AFP and be able to treat it accordingly.
That Sarah and Brian will have God's peace that passes all understanding as they love and care for Clark and Boston.

Please pray for us and for our smallest son. We will keep you updated.