The Start of Peritoneal Dialysis

On February 17th, we got the word that Dialysis had to start.  That was hard to hear, we thought he was doing well enough to hold out longer, the longer we wait the better he will tolerate it.  The other Nephrologist on call said we would possibly go home before we needed to start dialysis.   Even though it was hard to hear, I felt peace about it, one step closer to going home with our little man, I remembered thinking.  Ezekiel has been such a brave little fighter his whole life so far and I know he will get through this too.  

However his electrolytes were slowly becoming unbalanced and it needed to be started.  Working kidney’s keep all your electrolytes in balance and since he only has 10% function on the right, it is hard for him to do so on his own. Our Nephrologist also said it is recommended to start dialysis on patients when one only has 10-15% function left. So we are so thankful that we were able to prolong dialysis long enough to let everything heal up good from his last surgery.  

What is peritoneal dialysis and how does it work?  Here is a good website if you want to read more about what it is all about.  But basically we infuse dialysis solution (water with salt and other additives)  into his peritoneal cavity (lining of abdomen) and let it dwell for a designated time and then we drain it out.  During the dwelling time of the solution is when dialysis is occurring, waste products and excess fluid pass from the blood through the peritoneal membrane into the dialysis solution.  The peritoneal membrane acts as the filtering device, similar to what the kidneys should do.    

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Our Nephrologist had him start dialysis with low volumes (50 ml each cycle) and will then work his way up to the desired amount for the home dialysis machine, which is 100 ml.  Each day they evaluate how Ezekiel tolerated the dialysis and each day we were able to increase his volumes.  It took him 5 days to work up to the 100 ml goal of dialysis solution in his peritoneum.  While we were working up to his goal, the nurses had to perform dialysis manually.   Now that he is up to the goal volumes they put him on the home dialysis cycler, all we have to do is learn how to program it and hook it up to his PD catheter.  It does everything for us.

Ezekiel has currently been on the home dialysis cycler for about 6 days and doing great.  He is even gaining weight, which was a struggle for awhile.  Infants and children grow a lot slower and have trouble gaining weight we were told, and could take up to 2 years or longer to get to the desired weight for a kidney transplant (which is 20 pounds).   We finally figured out what will help him gain weight, and we are sticking to it as of now.   Currently he is receiving my breastmilk with a special formula designed for kidney patients to increase the caloric intake as well as a liquid protein supplement each feeding.  We give him bottles during the day and then at night he is on a continuous feeding through his g-tube while dialysis is occurring.  We are doing this because he currently does not have enough energy and strength to take the amount of calories he needs every 3 hours.  

Our next step is to learn how to use the dialysis cycler at home. We had our first training session on Sunday, and then again today (Tuesday) and will have one more tomorrow morning.  After setting up the machine a couple of times we both feel very comfortable doing it.  It is a very simple process we just have to make sure when we connect the fluid bags and when we connect his catheter we are as clean as possible.  Any contaminations during the process could lead to peritonitis, an infection in the peritoneum (the lining of abdomen), a very serious infection.  During the process we have to close all doors/windows, cover all vents, wear masks and wash/sanitize our hands many times during setup and especially when we connect the machine to his PD catheter.

Our goal for home is this FRIDAY!!!!  As long as Ezekiel corporates, which we aren’t telling him so hopefully he does.  Here in the NICU it is common when we tell babies they get to go home on a certain date it doesn’t happen.  So don’t tell him :)  We all want him home.  Every day Addilyn asks “Does baby Ezekiel get to come home today or after bedtime?”  I can’t wait for the day I get to say YES he’s coming home!!!!

David in training

Katie in training

The dialysis machine

Ezekiel's Lungs and Kidneys

During my pregnancy, the lungs and kidneys were his main concerns, this blog will explain those in more detail - see my last post to read about my pregnancy.  Somewhere between my first initial sonogram at 8 weeks and the 20 week sonogram is when the LUTO (lower urinary tract obstruction) occurred. Which means that amniotic fluid levels were low, and pulmonary hypoplasia was happening (small underdeveloped lungs).  With that, the kidneys were also being damaged at this time due to the pressure build up in the bladder and ureters.

The Lungs
To develop, the lungs need amniotic fluid. At 24 weeks I had another sonogram and this is where things turned around and the Urachus popped open and allowed the urine to escape into the amniotic space.  Amniotic fluid levels were normal and his lungs could have a chance to grow and develop.  Since working in the NICU, I had the chance to ask around and do some research to see if there are any cases that this has happened to. Unfortunately, there was no research out there about having to low of fluid at the beginning and then normal fluid levels.

I remember all the doctors saying, if there are no lungs then we can’t work on the kidneys.  So the plan was to deliver Ezekiel on February 15th at 39 weeks via c-section and for me to receive 2 doses of Betamethasone prior to delivery.  Betamethasone is a steroid that reduces the risk of lung complications in preterm infants, or in my case, the unknown of lung development.  However, on February 1st at 37 weeks pregnant I had a sonogram.  We knew my fluid levels were on the lower side of normal, but we didn’t know if it was due to normal pregnancy or due to lack of kidney function.  So the sonogram revealed that fluid levels were too low to stay pregnant and was whisked up to labor and delivery to prepare for the c-section.  I asked what about the steroids and they said they didn’t have time. 

Ezekiel was then born 3 hours later at 12:17pm and he came out crying!  All the doctors have been very pleased with his lungs.  We had everyone at the delivery as well: 2 Neonatologists, 3 Neonatal Nurse Practitioners, a Respiratory Therapist, and the Transport RN.  (It helps to know people!).  At birth Ezekiel only needed a little support and was placed on Nasal IMV.  Over the next couple of days he was weaned off all oxygen support and was on Room Air. Even after his surgeries he was able to come off oxygen support quickly.  Very proud of our boy!

He did have a little set back about a week ago with oxygen and needed to be placed on Nasal CPAP.  We think we just changed too much all at once for the little guy.  At that time we were increasing on the amount of food he was bottle feeding quickly, was on room air, and doing mini cycles of dialysis to keep the PD catheter clear of blood.

Currently Ezekiel is doing much better.  The doctors put him on a slower feeding schedule to ease up the volume to full feeds, and holding his oxygen steady on a High flow Nasal Cannula at 2 Liters in 21%. Here in the NICU we can increase the amount of oxygen very slowly and goal is always 21%, which is room air.  The plan is for him to get to the full volume of feeds then take him off the nasal cannula.  One thing at a time is the key with this little guy.

Praise God for working lungs! Because the lungs are good we are able to focus on the kidneys...these are a whole different story.


The Kidneys

Right after birth we were talking to the Urologist and Nephrologist every day.   The second day of life they preformed the Mag 3 Lasix test to see how much kidney function he actually had.  The results came back and they were not good.  His left kidney had no function and the right only had about 10%.  The Nephrologist says that it is recommended to start dialysis when there is 10-15% function left.  So the next day, when he was only 3 days old the urologist placed bilateral ureterostomies so there was some place for his urine to drain and kidneys and ureters to decompress.  Our hope was then he would produce a lot of urine to help stabilize his electrolytes.   

During the bilateral ureterostomy surgery they learned that his kidney’s were half of the size they should be and we definitely would be looking at dialysis and kidney transplant in the future.  Our hope was to then get the PD catheter in place and be able to hold off on dialysis for at least 2 weeks so everything could heal.  Over that following weekend the doctors watched his electrolytes very closely and we prayed for pee.  If his urine output stayed up then we would have a great chance of holding dialysis off.  

On Monday, at 6 days old, he had his second major surgery.  That was full of waiting.  Surgery APN called early in the morning saying his surgery could be anytime from 10:30am to 5pm that night.  He ended up going into surgery that day at 2pm.  I was able to snuggle him all morning and was able to talk to the surgeon and all the other doctors.  He ended up having a Peritoneal Dialysis catheter placed, abdominal hernia repair, bilateral orchioplexy, and a G-tube.  Our goal was to fix everything so that things had a time to heal properly before we start dialysis.  

Everyday they check Ezekiel’s electrolytes to make sure they are not becoming elevated.  Some of the main labs they look at are sodium, potassium, BUN, and creatinine.  Our goal is to hold off dialysis as long as possible and if his labs stay stable and his urine output stays strong then we will be able to wait for everything to heal.

That is the story of his lungs and kidneys. We thank God for working lungs and continue to pray for kidney function as we begin Peritoneal Dialysis. I will share more about Peritoneal Dialysis in my next post. In the mean time, be sure to find me on Facebook for updates and pictures.

Thanks everyone!

Beginning the Journey of Ezekiel Joseph

So now that all of you are up to date on our happenings here in the NICU.  We would like to share a little of our roller coaster of emotional pregnancy journey.  When I found out we were pregnant, I told our girls (Addilyn is 4 and Eleanor is 2) that they were going to have a baby brother or sister coming.  They were so excited, especially Addilyn, who automatically said she wants a baby brother not another baby sister.  We told her that we don’t get to choose, God chooses for us, but she could pray to God to have a baby brother, and she did.

God gave us a baby boy!  However, it took us awhile to find that out... and this is where our roller coaster begins.  David and I went in for our 20 week anatomy sonogram.  They started the sonogram and I automatically knew something was wrong.  The only thing we saw on the screen was a big fluid filled cyst like structure, we could not see the baby’s arms or legs, just a head and the cyst.  The doctor was called into the sonogram room and told us that they didn’t know what that was and referred us to the Maternal Fetal Specialist. They drew some blood for different tests and still did not know if our baby was a boy or girl.  

Notice his little head; the black circle is his bladder

The next day we got an appointment and we were so nervous.  We were then given the diagnosis of a Lower Urinary Tract Obstruction (LUTO), which means his bladder has no way to empty, so no fluid would be able to get to his lungs thus lungs would not develop.  The big cyst like structure that we saw was his bladder filled with urine.  With LUTO, the kidneys become severally damaged as well.  LUTO is a rare birth defect that occurs in 1 in 5,000 to 7,000 births, most commonly in males.  Other diagnosis we heard that are a result of LUTO were pulmonary ,.   and prune belly syndrome.  Our doctor gave us some options to do but none gave us much hope and the end result would be the same.  We were looking at comfort care, end of life care, when he was born and would meet with everyone at our next appointment to come up with a plan for his passing.  We were going to loose our baby.  It was just impossible to think of.  To carry him to full term and then loose him was so devastating, and we still were not able to see the gender.  The lab results finally came in a couple days later and we found out we were having a BABY BOY!!!!

Our next appointment with the Specialist was not for another month.  We lived, I should say just survived a day at a time, knowing we were going to loose our baby boy.  I was then 24 weeks pregnant and we went in for another sonogram.  The sonogram looked totally different, there was fluid around him.  Not knowing what could happen was hard.  They did say that the bladder could rupture from all the pressure of urine inside and we automatically thought it was that.  However the doctor came in and said things have changed a complete 180.  From all the pressure in his bladder, the urachus popped open and allowed the urine to come out freely into the amniotic space.  He now had fluid to breath, his lungs had a chance.  The doctor said we were no longer going to talk to palliative care, and he was very optimistic about Ezekiel's chance to live. From them on throughout the pregnancy we had sonograms every 2-3 weeks to check fluid levels and anatomy. The doctor said there is a change that the urachus -that popped open- could close back up and we would be back to square one. 

After weeks of sonograms and various meetings with different doctors, Ezekiel Joseph made his entrance into this world on Wednesday, February 1, 2017 at 37 weeks.  I had a 37 week sonogram to check fluid levels and the doctor came in and said today is the day to have a baby, my fluid level was too low.  It scared us so much.  Our planned c-section was not scheduled for another 2 weeks.  February 15th was supposed to be his birthday, we had plans, plans to receive steroid injections prior to delivery to help his lungs.  But Ezekiel had other plans.  He came out crying and only needed a little oxygen support.  We both immediately burst into tears.  It was so good to hear him cry.  Praise the Lord!

Welcome to our Journey

Hello friends and family!  Welcome to our blog, God Will Strengthen.  This title was a no brainer because Ezekiel is a miracle and we know God is with us.  Ezekiel means God strengthens.  We had this name in mind before we were even pregnant with Ezekiel.  

As of today, our sweet little miracle is 12 days old.  I (Katie) am sitting here watching him sleep so peacefully as I write to all of you, his prayer warriors.  We cannot thank you all enough for the prayers, love and support you all have shown our family.  We could not have made it this far without all of you and the faith of God.  We would like to bring you all along on this journey of Ezekiel Joseph.  Not sure how consistent we will be about writing but we will do our best.  

Before we share how our journey began we would like to update you all on how Ezekiel is doing.  Ezekiel was born on Wednesday, February 1, 2017 and then was whisked away to the Neonatal Intensive Care Unit.  The Friday after he was born he had to undergo surgery to have bilateral ureterostomies placed, which allow him to have a place where urine would escape.  Then on Monday he had another major surgery to place his Peritoneal Dialysis catheter, a G-Tube, along with fixing his abdominal wall hernia and bilateral orchioplexy.  After that surgery the plan was to let him rest for a couple of weeks to allow everything to heal.  However, the PD catheter started to leak that night and blood got in. So the next day we had to start doing mini cycles of dialysis to flush out the blood in the catheter so it would not clot off.  If it did clot off,  he would have to get a new one.  So we have been doing mini cycles of dialysis for the next 3 days.  Ezekiel had some ups and downs but was able to come off all his oxygen completely for a few days.  

The night of 9th, we got 2 dreaded phone calls.  Its never good when they call you at night.  Ezekiel was working harder to breath and was just very irritable and not making his oxygen saturation requirements.  So they put him back on a nasal cannula.   The nurse called everyone in to look him over.  They got x-rays, drew blood cultures, labs and they sent off fluid from the PD catheter.   That morning when we arrived, they were doing an abdominal sonogram to make sure nothing was no leaking from his surgery, and placing him on a Nasal CPAP machine. He needed a little extra help breathing.  After they put him on CPAP he was a lot more relaxed and slept the rest of the morning.  David and I hung around till the afternoon and Ezekiel was feeling much better so we both got some good snuggle time in with him. 

Thanks for reading our first post.  Next we will update you on how our journey began.