Thursday, June 20, 2013
Ezra is two months old today! My, oh my, what a blur the last two months have been...both the longest and shortest two months of our lives! Love this picture that Josh captured on his phone this past Tuesday while we were up at Doernbecher's...what's funny is this was right after he puked ALL over me (I had a change of clothes for him, but not for me)! He was ALL smiles after totally sliming mama :) We're starting to notice a pattern: long car rides to Portland + Doernbecher's = MASSIVE spit-ups/pukes. They happen nearly every time we go up to Doernbecher's. At least now we've recognized a pattern, we'll be more prepared with back up clothing for ourselves!
I just read Judah's two month blog post...oh how times have changed! I wrote about dealing with a nasty diaper rash infection and being scared for his first round of shots. Those things seem so insignificant in comparison to what Ezra has already been through in his first two months of life. Oh, to only be concerned about a diaper rash infection and vaccinations!
I've said it before, but Ezra is such a good, sweet baby despite all he's been through. Such a precious little guy. Since we've been at the doctors on a regular basis, we've been keeping track of his growth based on his weight. He seems to be gaining really well but I think he is still on the short side, though...so much opposite from his big brother who has always been at the top of the charts for height and bottom of the charts for weight. Oh no, we're comparing our two boys already, not sure they're going to like that!
Wednesday, June 19, 2013
|Josh and his mini me :)|
Now, yesterday's appointment. Since most of Ezra's test results have been normal and all the experts seem to be scratching their heads about our little guy, our next specialist visit was to a geneticist (we are up to four specialists now). After a bunch of family medical history questions, reviewing Ezra's medical history, and another examination, we had yet another specialist scratching his head. Ezra just doesn't look like anything is wrong with him. However, "after some digging," the doctor came up with one possible diagnosis...probably the scariest one yet. The disease is called Wiskott-Aldrich syndrome (WAS) which is an immunodeficiency disease that occurs almost exclusively in males. "It affects the function of the white blood cells, making these patients susceptible to serious infections. There is also a significant reduction in the size and number of platelets...Affected patients show a wide variation in the severity of the disease. Severely affected patients typically have excessive bleeding, are at risk of serious infections, have eczema, and are at a high risk of developing autoimmune disorders and certain types of cancer." Yikes. You'll have to click the link to read more about it.
Although we have NO idea what is wrong with Ezra, we are NOT jumping to the conclusion that WAS is what Ezra has. The hematologist has never noted that the size of Ezra's platelets are small, Ezra seems to be producing a normal number of platelets (according to his bone marrow biopsy), he does not have eczema, he has not had excessive bleeding...and there are other characteristics of the disease that just don't seem to apply to Ezra. Does he maybe have a more mild form of the disease? Possibly. But like I said, we are not going to jump to this conclusion. And even if we wanted to jump to this conclusion (why would we!?), we can't. It will take up to three weeks to receive authorization from our insurance to get the test ordered and once the test is sent out, it will take another 4-6 weeks for results. And as luck would have it, Josh's insurance is changing the beginning of next month, which means that we probably won't be able to get the ball rolling on this until July 1st. So, we're talking about 2+ months of waiting to see whether or not this diagnosis is a possibility. I'd say Josh and I are pretty patient people, especially considering everything we've been through so far... but it's safe to say this is certainly going to test our patience in a big way.
Since starting this post, we've gotten a call from the hematologist about Ezra's CBC yesterday. Unfortunately the huge increase of platelets last week (it jumped up to 120/93) seems to have been a fluke. Ezra's count is back down to 49. His white blood cell count is still super high. And now his hemoglobin level is quite low (for the past few weeks Ezra's number has been around 13, but it is now down to 9.9 - if it goes under 6, he will need a transfusion). Sigh.
As hard as it is to remain positive through all of this, like I mentioned on Facebook last night, we are counting our blessings that Ezra is as healthy as he is. We have seen so many children up at Doernbecher's in much worse shape. We continue to remain hopeful that one of these days, Ezra will be as healthy on the inside as he looks on the outside, and we can put all of this behind us! In the meantime we are praying fervently for an extra measure of patience, peace, strength, courage, and so many things that we need to get us all through this incredibly trying time! Lots of love to all of you for continuing to pray for Ezra and for us...you are a blessing.
|Big brother Judah has been such a trooper coping with all of Daddy & Mama's time away with baby brother.|
Wednesday, June 12, 2013
|Trying to wake up from sedation. Such a trooper through all of this!|
That evening after two attempts to get an IV in (this is always such a traumatic part of any of Ezra's procedures, unfortunately he inherited my tiny and very uncooperative veins), they took a blood sample for a CBC so we could know how much platelets to order. Remember, Ezra's platelets are usually 25-35 but they've been as high as 50 (but never, ever higher). Well, after that CBC, we found out Ezra's platelet count was up to 120. WHAT!!?? Everyone was in shock. For Ezra's platelets to have gone up at all would have been amazing, but that much? Wow. Josh and I were very reluctant to get too excited, we've been falsely optimistic before. But, after it took FOUR attempts to draw blood the next morning to do a recheck (because Ezra was too hard to get blood out of with just a heel stick) we were pretty confident that Ezra's count was still up...and it was!! 93 this time. Of course we would have liked if it had gone up even further and not dropped some, but we've been doing this long enough now we're just happy when it's over 35.
So what does a platelet count of 93 mean? Well, it means that Ezra didn't end up needing a transfusion before his procedure, hooray! Transfusions can be really scary so it was awesome that we had just one less thing to worry about. The sedation for the procedure went well (anesthesia is always scary too) and the doctor was able to get some good pictures and biopsies during the scope. She found some small ulcer-like spots and inflammation in stomach that are indicative of milk protein sensitivity, but no vascular abnormalities that would explain platelet destruction. We should know more in 7-10 days when we get the biopsy results.
While we are rejoicing with Ezra's unexplained platelet increased, we are doing so very cautiously. We don't want to get our hopes up just yet. Next week we will have our weekly blood draw and we should know some more about what his platelets are doing then. And, unfortunately, with every good piece of news another challenge seems to follow. Along with an increase of platelets, it has been brought to our attention that now Ezra's white blood cell count is extremely high. So what does that mean!? Ezra threw up (not just spit up) while we at the hospital, but other than that and the tiny ulcers/inflammation in his gut, he has no fever and isn't showing any other signs of infection.
Sigh. Glory be to God for so many things that have been going well and for favorable test results, but we still have so many unanswered questions about Ezra's platelets and now a new challenge. The doctors are so perplexed by all of this and the path forward seems so unclear. What we do know, though, is that Ezra's counts need to be monitored very closely so we will continue with our weekly blood draws. We will also be seeing a geneticist in the near future to see if they can shed some light on any of this and to see if something has been overlooked.
I know many of you have asked how you can help and it's hard to say what you can do besides keep praying! But, if you are wanting to help in a more tangible way, meals or restaurant gift cards would be very much appreciated. We seem to be averaging about two days at Doernbecher's a week, and by the time we get back from those trips we are completely exhausted and making a meal when we get home seems like a pretty big task. If you know Josh and I, you know we don't like asking for help, so we are very humbled to do so...and I know some of you are tired of our "we don't need anything" response :) So, yea, my diet is very restricted these days. Gift cards to places with large vegan offerings like Laughing Planet, Cafe Yumm, Nearly Normals, Baja Fresh, Spice & Ice, the co-op or Market of Choice would be super helpful, especially since we don't always know what days we will be in Portland or what nights we will be getting home late. But, if you're really wanting to drop off a meal, it would probably be easiest to make something non-vegan for just Josh and Judah (their favorites are comfort foods and not a lot of veggies...go figure!).
Again, glory be to God for continuing to watch over our sweet boy and for helping us all get through this. And many thanks to all of you following along, for continuing to pray and for offering your support and well wishes.
|Ezra getting a ride in his cage (aka, hospital bed crib) back to our room after the procedure.|
Sunday, June 9, 2013
In this post I'll try to sum up Ezra's medical history over the course of his first seven weeks of life, that way from this point forward we can try to post what is currently going on with our sweet boy for all of you who are following along.
04/25/13 - Appointment with our pediatrician
05/21/13 - CBC at lab
04/25/13 - Appointment with our pediatrician
- Ezra looks great. There are no real physical signs that anything is wrong with him.
- We need to go to the lab to have a complete blood count (CBC) to check Ezra's platelet count and to see if his red blood cell count has continued to go back towards a normal level.
- Our pediatrician has been consulting with a hematologist at Doernbecher's children's hosptial. Ezra needs to have head and abdomen ultrasounds done to see if there is an abnormal concentration of platelets somewhere.
- Red blood cell count is headed in the right direction
- Platelet count is 51. Not good. We need to go up to Doernbecher's to meet with the hematologist
- Ultrasounds are clear! Josh and I are elated. We assume that nothing serious is going on with our sweet boy, he must just have some treatable type of blood disorder. (We were falsely optimistic)
- Platelets are down to 45
- This appointment lasts nearly 2 hours. Ezra is examined. Hematologist wants to find a diagnosis for Ezra that encompasses all his tiny little issues into one package.
- Based on combination of complications after birth (low blood sugar, high red blood cell count) and possibly enlarged soft spot and possible separation in abdominal wall/slightly herniated umbilical among other things, he orders test for Beckwith-Wiedemann Syndrome (blood sample will be sent to St. Louis, results take a month)
- While we are there, other blood work is done including CBC
- Platelet count is down to 36
- (A month later we find out Beckwith-Wiedemann Syndrome test was negative, he does NOT have the syndrome)
- We find out that Ezra's abdomen ultrasounds may indicate some bladder reflux. We will need to see a urologist at Doernbecher's for follow-up.
- We will now have weekly CBC's done to monitor Ezra's platelet count
- Karyotype test is also ordered (to rule out Downs, etc. A month later we find out everything looks NORMAL)
- Platelet count is up to 50, we are falsely optimistic things are improving
- That morning we discover blood in Ezra's stool
- In addition, Ezra's belly button is bleeding, pediatrician cauterizes it
- After consulting with a GI specialist at Doernbecher's, the suggestion is that I go off dairy and soy for 2-3 weeks to see if Ezra has a milk protein allergy (after 2.5 weeks, there is no improvement, pediatrician and hematologist do not think it's allergy-related and okay going back on dairy)
05/21/13 - CBC at lab
- Platelets have dropped to 20
- Admitted to hospital for transfusion at 6PM. It takes SIX attempts to get IV placed. Platelets finally arrive and are started around 3AM. We are discharged around 1030AM the next day.
- Platelets go up to 48
- Platelets go down to 34
- Platelets go down to 26
- IV goes in on FIRST attempt, Ezra does great with sedation and procedure
- Platelets are 34, preliminary results of bone marrow biopsy are NORMAL
- Ultrasounds of bladder and kidneys look NORMAL, no bladder reflux repair surgery needed
- Blood in stool continues, doctor wants me to go off dairy for 2 months to rule out milk protein allergy
- We also need to schedule a GI scope (endoscopy and flexible sigmoidoscopy). This procedure will require sedation and another transfusion because of the risk of bleeding (due to Ezra's low platelet count)
As many of you know, our sweet little boy, Ezra, has had a rough first few weeks in this world. As of now, the root of his troubles are a mystery. We are so looking forward to the day when we can get some answers and some treatment. To chronicle this journey, I thought I would start at the very beginning. I'm going to try to fast forward through most of the details of the birth story so we can get to the part where our beautiful little boy was born...and then when things started to get more complicated.
A little history. When I was pregnant with Judah, I tested positive for Group B Strep (GBS+). One in four to five healthy pregnant women carry the GBS bacteria. Mothers who test positive are treated with antibiotics via IV to reduce the risk of passing the GBS bacteria to the baby. Antibiotics need to be started as soon as the mother's water breaks, or, if the water hasn't broken, at least 4 hours prior to delivery. It's possible to test positive for GBS during one pregnancy and not during another, but as luck would have it, I tested positive again. With Judah, it was easy to know when I needed to go to the hospital to get started on antibiotics because my water broke. I didn't have a choice, I had to go right then. This time around my water didn't break. My contractions REALLY started around 1030PM on April 19th (just as Josh was getting home from a lacrosse game - I was already in bed and I was so exhausted, I didn't even have the energy to tell him I was having a contraction). I continued to have contractions every 15-30 minutes until about 1230AM on April 20th. At that point I woke up Josh to tell him what was going on. We finished packing our hospital bags and I told Josh to go back to sleep. I tried sleeping. No use. I went downstairs to have a snack, watched a little TV, and by 3AM I was timing my contractions about 8-10 minutes apart. I thought for sure it was time to go to the hospital (being GBS+), but after calling the midwife, she told me to wait until my contractions were 5 minutes apart (even though I stressed I was GBS+). By 4AM, my contractions were 5 minutes apart. We were to the hospital by 445AM. While we were parking I had a contraction and by the time we walked to the check-in desk I was having another. Things were really progressing. When I got up to the delivery room, the nurse checked my cervix, I was only at 3cm (but still having intense contractions every 4-5 minutes). They said they wanted to wait an hour to admit me to make sure I was really in labor...WHAT!!?? FINALLY after walking the halls for about an hour and dealing with seriously intense contractions that were getting closer and closer together they rechecked me. I was 5cm. They decided to admit me and got my IV in and ready for the antibiotics, it was about 6AM by this point. Finally at 7AM, they started the antibiotics (why were things moving so slowly!? Remember, I was supposed to be on antibiotics for being GBS+ for at least 4 hours before delivery). For the next hour my contractions were insanely intense and coming faster and faster together. It's hard to even put into words how much pain I was in from pretty much the time I got to the hospital to this time...don't get me wrong, I experienced an extreme amount of pain with Judah, but this was so much more intense, and so much faster. Finally by about 830, my pain was so intense I could hardly breathe and I could not keep myself from pushing. In the back of my mind, I knew I needed the green light from the midwife before I started pushing, but I couldn't not push. We had to run out of the room to get someone! About three big pushes later, Ezra Joseph Parker was born at 837AM!
Okay, this has already been too long, so let's switch to bullets to recap and tell the rest:
- 3AM Saturday - called midwife, said I was GBS+, said my contractions were 8-10 minutes apart
- Midwife said to wait until contractions were 5 minutes apart to come to hospital
- 445AM - got to hospital, contractions were 4-5 minutes apart, was checked at 3cm, was told they were going to wait an hour before admitting me
- 6AM - was 5cm, got admitted, got IV started
- 7AM - finally got antibiotics started
- Around 830AM we ran to get someone, I NEEDED to push
- 837AM - Ezra was born
- I had only received ONE dose of antibiotics (instead of the 4 hours of four doses I should have had before delivery)
- 842AM - midwife decided to clamp umbilical cord FIVE minutes later (we were COMPLETELY unaware of the delay until after the fact. Ezra was PURPLE, I kept asking, "Is he okay!?" Midwife answered, "Oh yes, he's okay." I kept trying to pull him up closer to my face so I could see him, and the midwife said, "Be careful, he's still attached!" What?? It never even occurred to us that she had decided to delay cord-clamping and cutting. That was NOT apart of our birth plan! (This part is important later)
- A couple hours later we were moved to another room.
- A doctor or nurse informs us that Ezra has low blood sugar, rapid breathing, and a high red blood cell count. They're going to have to give him some formula to try to bring his blood sugar up. I am distraught, Ezra has already been nursing, VERY well, I don't understand why they need to give him formula. I don't want to do anything to interfere with breastfeeding, but they tell me they have to give him formula, and I want to do what's best for Ezra. It's the tiniest amount of formula, but they say it will be enough to get his blood sugar up.
- Ezra's blood sugar doesn't go up after the formula. Because of his symptoms, they must get him on an IV with glucose, and also want to start antibiotics since I had only gotten one dose of antibiotics for being GBS+ when I should have had four doses. Again, I am distraught. I'm unsure of the impact 48 hours of antibiotics at birth will have on Ezra's immune system for the long run. I'm SO upset that I was told not to come to the hospital when I called at 3AM. If I had come to the hospital at 3AM, I would at least have been able to get three doses of antibiotics, maybe even all four. Even if I had been admitted at 5AM when I got to the hospital (instead of them waiting until 6AM to admit me, and not starting my antibiotics until 7AM), I could have gotten 2-3 doses in!!
|In the nursery|
- Ezra has to stay in the nursery overnight. Again, I am distraught. When we had Judah, he never left our sight.
- Ezra is still being monitored by machine, but he gets to come back to our room late the next morning (Sunday)...hooray!
- At some point, his IV starts bleeding (it is still in place for his antibiotics). After SIX attempts to get a new IV placed, they give up. That means he will have to get six ADDITIONAL pokes because now the remaining antibiotics will have to be administered by injection. At this point we have lost track of how many times Ezra has been poked or had his heels stuck.
|Failed IV attempt in Ezra's head :(|
- By Monday we are totally stir-crazy, we just want to leave the hospital, bring our sweet boy home, and be back with Judah. But the doctors need to keep watching Ezra a while longer.
- Early evening, the pediatrician on shift gives us an overview of what is going on with Ezra. He's never had a fever, there is really no evidence to support that he ever had any type of infection (so the 48 hours of antibiotics were probably totally unnecessary). We still don't know why he had low blood sugar, though, or why his red blood cell count was so high. The doctor says that sometimes the red blood cell count can go up when the baby receives too much blood from the placenta, it's essentially like a massive blood transfusion. LIGHT BULB! The midwife didn't clamp the cord for FIVE whole minutes, plus Ezra was PURPLE. Surely this is what happened, right? We look up "delayed cord clamping" and find that while this is widely popular these days, we read that complications can include polycythemia (high red blood cell count, just like the doctor said). We go on to read that polycythemia in newborns can cause the following: deep reddish-purple coloring, lethargy, rapid breathing or respiratory distress, jaundice, and low blood sugar. LIGHT BULB! Ezra had ALL of those things! Surely this is the explanation for everything going on with Ezra.
- Then we get another curve ball. It has not been brought to our attention until this moment when the doctor tells us that Ezra also has a low platelet count. What!? Why are we just now hearing about this?? Anyway, we are still discharged late Monday night with instructions to see our pediatrician in a day or two. In the meantime, we need to watch Ezra for any abnormal bleeding. The platelet issue is now our main reason for concern.
|Ezra's purple feet|
It's hard to conclude all of this except to say, this story is just the beginning of an already long journey. In the next post I'll try to sum up Ezra's medical history over the course of his first seven weeks of life, that way from this point forward we can try to post what is currently going on with our sweet boy for all of you who are following along. Words cannot express how thankful we are for your continued love, support, and prayers...please keep it coming!