The Details of Judah's Condition and Care

Since Charity Air Ambulance posted Judah's story, we've gotten a lot of messages, especially from other parents of Potter's Sequence children, asking how Judah is being cared for. I thought I would post something then that explains it in detail so we can hopefully answer all the questions.

Firstly, Judah's condition is called Potter's Sequence, previously called Potter's Syndrome, with the specific type being bilateral renal agenesis (BRA), or the absence of kidneys. The building blocks for kidneys are developed in the first 28 days of gestation and begin working during the 14th week and take over the production of amniotic fluid from the placenta. This is where Potter's turns deadly. Without the amniotic fluid, the babies suffer deformities and their lungs fail to develop, making it impossible for them properly breathe and oxygenate their bodies once the umbilical cord is cut. They die of respiratory failure before they die of kidney failure. Potter's Sequence can come in various different forms, including having kidneys but the kidneys aren't functional for one reason or another. Potter's occurs in 1 in 4000 births and is usually 100% lethal. Up until 3 years ago, there were no survivors. Then one set of courageous parents decided to try and do something about it and set in motion the treatment that led to our Judah surviving so far.

The first step in Judah's care was the placement of an amnioport in my abdomen to deliver normal saline into the amniotic sac three times a week to help him grow and develop his lungs. We had to travel to Cincinnati for this because, to my knowledge, only three hospitals in the U.S. will do amnioinfusions willingly without being pushed. Because they puncture the sac, I leaked fluid throughout my entire pregnancy following the surgery, had contractions if I got out of bed for more than using the bathroom or going to appointments, and went into premature labor twice. His prenatal care, in relation to his postnatal care, was relatively simple. He was born at 33 weeks and 3 days gestation after my water broke and we could no longer do infusions or stop labor.

Judah being intubated right after birth

When Judah was born he didn't cry but he did try to breathe. They intubated him when he was five minutes old and managed to get him stable enough to take him from the operating room (he was a C-section) to the NICU. Following his birth he was given a chest tube for a pneumothorax and then transferred to Cincinnati Children's Hospital (he was born at a different hospital since CCH currently doesn't have a labor and delivery floor). His blood pressure was extremely low when he was born and the doctors said they "threw everything and the kitchen sink through his UVC line" to get his blood pressure up. Once they considered him a pulmonary survivor (at three days old), they did surgery at his bedside (because he was too unstable to move to the OR) to place a hemodialysis (HD) catheter in his neck and a peritoneal dialysis (PD) catheter in his abdomen. They tried a gentler form of hemodialysis called aquapheresis first but his blood pressure immediately crashed and they had to take him off of it. They waited another day or so and then started him on PD. This gave him enough time to come down off his blood pressure medications (he had been maxed out) enough that when his PD catheter ultimately became ineffective because of leaking, they were able to do aquapheresis again and give him good enough nutrition to heal his PD surgery site.

Judah's problem list at birth included bilateral renal agenesis, small lungs, low blood pressure, pulmonary hypertension (high blood pressure only in the lungs), two hernias (common in preemies), and a pneumothorax. He was on dialysis, nitric oxide therapy (pulmonary hypertension), flolan (pulmonary hypertension), epinephrine and vasopressin (low blood pressure), a ventilator, a chest tube, and TPN (total parenteral nutrition - basically IV nutrition) and lipids for his food. He was extubated at 2 1/2 months and off all the IV meds except the nutrition over that period of time as well.

Judah on his due date

He remained on PD for around 3 1/2 months until his PD catheter became clogged and they had to do surgery to fix it and then surgery again 2 days later when it stopped working again and they had to replace it with a bigger one. Following those two surgeries they put him on hemodialysis for 3 days (surgery recommended 2 weeks) and he didn't tolerate it well so we had to go back to PD. Just before Christmas he was transferred to Texas Children's Hospital which was back home for us. Following his transfer home, he developed a hernia at one of the surgery sites and began leaking PD fluid. He was then put back on hemodialysis for 2 weeks until he developed peritonitis (assumed - we never found anything on the cultures) at which point his blood pressure got so low that he had to be put back on medication for it - this time dopamine and epinephrine. He also had to be re-intubated because he was breathing too fast and hard and they were worried he would wear himself out. They put him on another form of dialysis called continual renal replacement therapy (CRRT) which is a gentler form of hemodialysis that runs 24/7. He is staying on that currently until they can get him back off the blood pressure medications and get him more stable. The issue with CRRT is it can be very dangerous to start them on the machine (example - Judah coded by dropping his heart rate the 3rd time they tried to start him on it) but once they are on it is very safe and gentle.

Judah's first Iron Bowl (We shout War Eagle)

Judah's current problem list is bilateral renal agenesis, small lungs, low and high blood pressure, peritonitis, respiratory failure, and resistance to sedation. He is on CRRT, dopamine and hydrocortisol (low blood pressure), fentanyl and versed (sedation and pain management), antibiotics, antivirals, and antifungals (covering their infection bases), TPN and lipids, a ventilator, saline mixed with heparin to keep one of his PICC lines open, and a warmer (CRRT drops the body temperature). Before getting sick, he was only on TPN, lipids, fortified breast milk (for more calories), hemodialysis, and 7L high flow nasal cannula.

His ultimate goal is to get him onto a dialysis cycler that he can go home on. He will then have to be pretty isolated (renal babies are extremely susceptible to infection) until he reaches 20lbs (around 2 years of age - it's hard for renal babies to gain weight because of fluid restriction) at which point he can receive a kidney transplant. He will then be on immunosuppressants for the rest of his life to help keep him from rejecting the kidney and will need another transplant in 15-20 years under current technology.

How It Works: Peritoneal Dialysis

There has also been some confusion as to how it is exactly that peritoneal dialysis works. Without getting too technical it's basically the following:
A catheter is surgically inserted into the peritoneal cavity, a spot between your abdominal wall and your organs that contains many, many blood vessels. Once the site has been allowed to heal (which isn't usually the case with babies) for 1-2 weeks per the surgeon's recommendation, it can be used for dialysis. With this type of dialysis no blood has to leave the body so there is less risk for infection (of the blood at least) and it is less draining and stressful to the body. PD runs in cycles - fill, dwell, and drain. I am not aware how long it takes to dwell in adults, but with Judah it would be 30-45 minutes to dwell with 10 minutes to drain and typically 2-5 minutes to fill. The fluid used for PD is a special type of sugar and salt water specially prescribed by the nephrologists based on the electrolyte and fluid balance needs of the patient. The peritoneal cavity is then filled with dialysate using an amount prescribed. Judah started at 20mL because of how tiny he was and we had worked our way up to 50mL before his most recent leaking. The goal for him is 150mL before he can go on a cycler, move from the NICU to the renal floor and from there work on going home. Once the cavity is filled, it dwells for 30-45 minutes, depending on how many cycles you want to get in per day. Since the fluid put in has no toxins in it and the blood does, the toxins and extra electrolytes and fluid are pulled into the peritoneal cavity through diffusion (toxins) and ultrafiltration (water). When the cycle is up, the stopcock on the tubing is turned to drain and the fluid drains out over 10 minutes. You want to get out the same amount or more than you put in and output is carefully measured. The cycle is then repeated. This is done manually by a nurse 24/7 until a high enough volume is reached for the cycler to work at which point dialysis can be done over 12 hours and the patient can live a relatively normal life during the other 12 hours.

Hopefully this helps explain things for anyone who is curious and helps anyone who may be looking for help for their recent diagnosis of Potter's in their baby. This post will become updated with his treatment as time goes on.


Judah passed away at 11:04am on February 25, 2017 after taking a sudden turn for the worst. We're still unsure what caused him to get so sick so quickly but we know he is healed and happy in Jesus' arms and we are looking forward to the day that we see him again.

Holiday Round-up

Happy New Year y'all! How was your Christmas? Did you make any resolutions?

This holiday season was especially busy for me and Brendan. We had originally planned, before we got married, to spend our first Christmas alone, with just the two of us. Those plans changed when Brendan got a new job (and, subsequently, a few more vacation days) and my family decided that I really needed to come home (I did too-I was so homesick).

Because what else do you do while you're waiting for
the show to start?

The week before Christmas one of my best friends from nursing school, Bethany, flew all the way to Houston just to see me. And we got into some shenanigans! The first night she was here we stayed up till probably one in the morning watching Psych and funny youtube videos. We had tickets the following day for Houston's First Baptist's Celebration, a Christmas show. I was really impressed by the quality of the show (and I rarely am-comes from being in theater since I was a child). They really went all out and held nothing back. I need to get in on this show next time they do it!

The following day Brendan had to work and we started doing damage to our bank accounts. We went shoe shopping, clothes shopping (multiple times that week), got manicures, went out to lunch, drove around Houston, recorded a song using nothing but garage band on my iphone, went out to see Christmas lights, went to Christmas parties and anything else we could find to do. I felt like I was back home and back in my first year of college. What we did that week were all of our destressers (is that a word?) from those first two semesters of trying to get through our pre-clinical classes.

We also baked (what's Christmas without Christmas cookies?) and made a gingerbread house. The cookies we made are called Italian Knot Cookies and I encourage to try the recipe (found here) because they are so so so good! Probably not good for your New Year's resolutions, but we won't talk about that. Our gingerbread house was a kit we got from Walmart on a shopping run later that week. I've never made a gingerbread house from scratch but I have a dream to someday do it! And if I get really good at it, my mom has an old Christmas cookie book that has the recipe and pattern to make a Notre Dame cathedral gingerbread house (would it still be considered a house at that point?). I've been wanting to make that thing since I learned to bake as a teenager. So one of these days, possibly in the far, far future, I will make that gingerbread house/cathedral and then probably refuse to let anyone eat it till it goes bad or falls apart. I mean, you wouldn't eat artwork, right?

At the end of the week, both of us had a flight back to Baltimore. Our original problem was that we were flying out of two different airports. Bethany's was north of the city and ours was south of the city. Thankfully, Bethany has an aunt who lives in Houston and she spent the afternoon with her and therefore had a ride to the airport.

One of my terribly cute traveling buddies

Our flight was scheduled to leave at 8:30pm. It was our first time flying with our dog so we decided to leave an hour earlier just to make sure we wouldn't have any problems with checking him in and getting him through security. We arrived only to find that our flight had been delayed to 9:20, giving us about three hours to make it through security and to our gate. There was a storm literally cutting off the west side of the country from the east side of the country from Canada to the Gulf. So we picked up some dinner from a wonderfully overpriced airport Wendy's and then settled in to our gate to wait armed with iphone games and a magazine with health studies in it for me and a novel for Brendan. And so we waited. And waited. And waited.

We assumed that we would begin boarding around 9pm. That time came and a plane, full of passengers, came into the gate and started off loading. And so we waited some more. Boarding began around 9:30 and, being in the last section to board, we probably got on the plane around 10. Once we had finally found a seat (I'm not sure how much I like Southwest's open seating policy), I took a nap and Brendan (probably) kept reading his book.

We landed around 1:30am and made it back to my parent's house around 4 in the morning. And boy were we exhausted. We settled in for about 6 hours before we woke up for church. I was excited to go back to the church I had been attending since infancy and I thoroughly enjoyed the worship and sermon, that, strangely enough, didn't feel so much like home any more.

On Monday, my older sister Sarai, Brendan and I took my two littlest sisters, Eliana (age 5) and Sofia (age 3) out to Chick-fil-A for lunch and then to the mall for last minute Christmas shopping and a movie so mom could play Santa and wrap up their gifts. We all met up at the Chick-fil-A were I got to see some of my old coworkers and Eliana and Sofia got their much loved nuggets and fries. After lunch we went across the street to the mall where we sent Brendan off to do his own shopping so I could get him his Christmas present, a new wedding band. He lost his a couple months ago and I couldn't get him a new one in Houston because he checks our bank account almost daily and I didn't want to tip him off. But at my parents' house, where his use of the internet is more limited, I was able to pull it off.

After we finished our shopping, we took the little girls to see Frozen. I highly recommend this movie. It's very well done, great for smaller children and bigger children alike, and the songs are terribly catchy. It was my little sisters' first time to the movies so they were a little scared when the lights went out and Eliana was too light to keep her chair seat from popping up, but all in all I think they enjoyed themselves.

On Christmas Eve my family drove up to Connecticut to spend the night at my aunt and uncle's house. Brendan and I went and had breakfast at IHOP with Jaimie and Bethany before spending the day relaxing and watching Christmas movies. That night we attended Sarai's church for the Christmas Eve service before heading back to my parents' house to finish present wrapping and put them all under the tree. Our family is continuing to grow-my brother is on his second child and I got married this past summer-so there were quite a few presents under the tree. It took us a good few minutes to bring them all upstairs from the basement and get them all in a spot where they wouldn't be tripped over or fall and break.

Christmas morning didn't have much to remark. Brendan and I slept in for the first time since our flight and Y'shua, Katie, Dixie, and Sarai all came over. We opened a few presents from each other before they headed off to Katie's mom's house for dinner. We started working on Christmas dinner and watched a DVD Brendan had gotten me for Christmas. My family finally made it back around 4pm and while dinner was being finished, the mania of passing out presents and opening them began. It's quite an event at our house and takes two or three trash bags to keep the mess contained. There were quite a few squeals of delight from the babies as they opened their gifts and got things they really wanted. My favorite gift was one from Brendan-a Canon Rebel T3i that I had been wanting for a really long time.

Dinner was when things got really interesting. Between in-laws and "adopted" family we had about twenty or so people and not enough seats to put them in. So we just got really creative. Refer to the following pictures.


This is the normal dining table (most of it)



The foremost end of the table

This is one of the kitchen counters.
Tony built it to be like a bar but it only has
three chairs. Everyone else was standing

Sarai and Keturah grabbed the chairs from the office
and used the counter to hold their plates and drinks.



Houston Zoo Lights

We spent the rest of the week doing various different things. We went ice skating with Bethany, had doggy play dates with Henry (Sarai's dog), ran errands, went to see The Desolation of Smaug, I got my hair done by the best hair stylist ever (Jen who works at La Bella Vita) and played with the little ones. All the while we anxiously waited for Katie to go into labor. We would still be waiting when we left that weekend. Saturday came and it was time for us to go back home to Houston. We packed up most of our things and I ran over to Jaimie's house to drop off a Christmas card and present. We sat and talked about various things for a while before I had to head back to finish packing before it was time to leave. We made it back, safe and sound, on another delayed flight around midnight and that night I thankfully collapsed in my own bed.

Delaware has been my home since I was born. I traveled but always came back. When I moved to Houston almost 7 months ago, I was terribly homesick and had a large amount of culture shock. All I wanted to do was go back to Delaware. But this trip made me realize something-my roots may be in Delaware but, and I never thought I would say this, Texas is my home. And there truly is no place like home.

That awkward week that separates Christmas from New Years was uneventful for us. Brendan went back to work, we tried to catch up on errands, and I did what seemed to be fifty loads of laundry. But New Years Eve we all got what we had been waiting for. Katie had her baby!

William Levi was born at 11:23am at a whopping 8lb, 7oz and 22 inches long. He's the first boy in my immediate family to be born in 25 years! Unfortunately he had swallowed something called meconium before he was born and it got in his lungs. He has spent his whole life in the NICU thus far but God has answered so many, many prayers that were raised up for this little boy and his is now off of his oxygen, IV and draining tube and just remaining there for a few more days to make sure he's all right. If everything turns out fine, he gets to go home tomorrow! He's a little fighter and thank God for answered prayers.

So this is how my family rang in the New Year-worried, anxious and praying for this little boy's life to be spared. Nobody wanted to lose this precious little one. But I know, just as God has worked a miracle and saved little Will, so will He work so many more miracles in this coming year both for you and for me. I've already gotten my first one-I got into Microbiology after fighting with the school to let me in the class for several months! So as we go into 2014 all I can say is praise God for all His marvelous works!