update...

Today was a very long and stressful one, but as of a bit ago when we left the hospital, Jacob was still off the ventilator (and has been since 9 this morning). He spent most the day very upset and unsettled but he has worked hard to breathe and hopefully we're going to make it over the hump. He is one tough kid! Please continue to pray that his airway swelling decreases and that, in the meantime, he has the strength and stamina he needs to breathe through a smaller and swollen airway. 

jacob

I wanted to write to ask you all to pray for Jacob tomorrow. Tomorrow morning they're going to try to extubate him - ie, take out the ventilator tube - AGAIN. They attempted on Friday and Sunday with no success. As I said in a previous post, the problem seems to be upper airway swelling (from the vent tube being in there for so long AND the trauma of having to reintubate him after the failed extubations) and NOT due to respiratory issues. He desperately needs to get off the ventilator, so we're praying that the different steroids they're giving this time around do the trick in keeping the inflammation at bay so he has a chance with CPAP. 

As for other updates, the kidney doctor said he was "very guardedly optimistic" that the steroids could indeed be having an effect on Jacob's kidneys. He said it's way too soon to say anything definitive, but we continue to pray that they are helping heal his kidneys and thus his problem isn't genetic but curable! We also found out yesterday he has a hernia, which I guess can be common for premie boys. That poor kid will have to undergo yet another surgery at some point before he's discharged, though it's hardly urgent at this point. It seems almost certain now that he will be the toughest kid on the playground.

As for Sawyer, he continues to do well, though today he has some yellow/green stuff coming out his nose so we're praying it doesn't become a more serious infection.

We will keep you posted. More photos soon! Thank you for praying!

new family photo

6 weeks old!

The boys are 6 weeks old today! The last couple days have been eventful ones. We'll start with Sawyer, who, within the last 24 hours has had some great progress: first bath last night (and now the fuzzy hair looks extra blonde!), he's again off the nasal canula and is doing great breathing on his own, AND he got promoted out of his isolette and into an open air little crib! This means he can regulate his own body temperature and is thus another step closer to going home. Yah!

Jacob has had a fairly good week. Overall he seems to be feeling well and has great times of alertness. (Unfortunately none of these moments get captured well by the camera since he's usually in his isolette.) He had a few protein plasma doses over a week ago and since then the protein levels in his blood have remained stable, so again we're grateful for his body's response in managing his protein leaking. The protein in his urine has gone down some and his swelling is almost gone. His UTI never progressed to something terrible (thankfully!) and so today he was able to start the steroids as a step to treating his kidneys. We are praying this works, as it means his kidney issues are most likely curable. He will be on steroids for 2 weeks and we're praying especially that he'd be protected against the potential side effects - ulceric stomach and suppressed immune system. He'll be on Pepcid and an antibiotic preventatively because of the side effects. We should know within the 2 weeks if this is working and thus if he has Minimal Change Disease. He also had some genetic testing done, so if he doesn't have MCD, we should know the results of his genetic testing in 2-3 weeks. 

He is still on the ventilator to help him breathe, but the doctors have been talking for weeks about trialing him off it. Well, yesterday they did it with no success. It was a traumatic hour for him (and me!) as his vent tube was taken out, CPAP put on, a miserable Jacob wheezing and struggling to breathe through a tiny airway, and then a couple attempts to put the vent tube back in. It turns out that his lungs are most likely strong and capable to be off the vent, but he has a lot of swelling in his vocal cords and throat from having that tube in there for almost the entire time he's been alive! The solution for the swelling is steroids - so the idea is that the ones he's just recently been put on for his kidneys should help with that. Consequently, tomorrow they'll take him off the ventilator again ... hopefully with some success, in which case he'll be on CPAP (the snorkel gear). We're really hoping and praying this is successful! We can't WAIT to have him off the ventilator at long last - and to be able to hear him make noise again!

I think that's all the news for now. We're especially excited to share the photo with the boys together. It's the first time they've been together since they left the womb!

the boys together again

update

We were very gung ho to start the steroids today as a possible diagnostic and treatment for MCD. He was all set to begin and then one of his urine tests came back with bacteria suspicious of a Urinary Tract Infection. Since his situation is more precarious regarding infections, the doctors are taking no chances and have put him on antibiotics just in case. Apparently steroids  compromise the immune system, so, as a result of the possible UTI, he has to wait a week to start the steroid treatment. This is a bit disappointing because we were so eager to start and thus get closer to an answer and possibly even a cure. So we won't know for a bit but will be sure to let you know when we do! Jacob's protein level in his blood has remained good, as has his urine output. We are so grateful for his progress.

Something else we're grateful for - weight gain! Sawyer has officially graduated to the "5 pound" club, having weighed in at 5 lb, 1 ounce tonight. Woo hoo!

more visitors!

sweet jacob

sawyer, eyes open

sleeping Sawyer w/ Debbie & Judy

p.s.

THANK YOU to the SFC folks for the adorable baby gifts and their incredibly generous contribution toward a larger item or two (I'm thinking stroller or car seats!). I thoroughly loved my mini-shower! We are continually overwhelmed by your continued love, thoughtfulness, and generosity! 

THANK YOU!

Update

Regarding Jacob's neuphrotic syndrome and the results we were expecting to get today...

Unfortunately, we didn't find out today exactly what the cause of Jacob's protein leakage is, but we did get some answers. (They couldn't tell from the biopsy if things were such due to Jacob's prematurity or due to something else.) They have concluded that there are 3 possibilities to explain his issues (3 instead of about 15). The first two are genetic defects and the 3rd is Minimal Change Disease (which is extremely rare in premies - ie, there are a handful of cases worldwide). If it is Minimal Change Disease, due to his young age/premature birth, the doctors believe that the normal course of treatment, corticosteroids, could/would actually cure him of his protein leakage issues. Cure! So they are putting him on these steroids, along with a couple other medications to treat the possible risks/side effects, for 2 weeks. If, by the end of that time period, things haven't changed then they will rule out MCD. If what Jacob has is indeed MCD, we should see improvement within the 2 weeks and they will continue the steroids for a number of subsequent weeks. In the meantime, Brad and I will have some blood work done for genetic testing to determine or eliminate the two genetic possibilities.  So obviously we are fervently praying that Jacob has MCD and that, consequently, he would be cured of his kidney issues. The doctors plan to start him on the steroids soon.

The doctor said there is nothing now that indicates or leads him to speculate which of the three it is. He did say, however, that he is pleased with Jacob's response thus far to the plasma protein and diuretics and that that gives him a good prognosis for the future, whichever of the three paths it may be.  

We feel relieved to have some more answers and continue to wait expectantly for more answers. We continue to ask God to miraculously intervene and provide a prognosis that includes complete healing. Until then, we will keep you posted... THANK YOU for praying!

In other news, Sawyer is off the nasal cannula and spent the day breathing 100% on his own. He has been doing fabulously thus far! In addition, he is just starting to breast feed. We are extremely proud!

Also, we have had a visit from two dear friends who worked at the high school in San Diego with us come visit for the past couple days. We have LOVED seeing Debbie and Judy and introducing them to the boys. They have been an enormous help with meals, errands, and overall moral support! Thanks for making the long journey from CA to visit us and big HUGS to all our fellow Eagles in San Diego! We miss you!

S&J's first visitors!

Uncle Jonathan & G-ma Ramey visited back in January

G-ma Nancy holding hands w/Jacob

Arja & Nancy holding Sawyer

more photos!

jacob's scar #2

sawyer, bright eyed & bushy tailed

a happy jacob

sawyer sleeping

just a short report

Jacob continues to have albumin (protein) doses and that combined with his diuretic medication have really helped reduce his puffiness and helped him lose weight. The protein level in his blood has increased a bit and the protein in his urine has gone down quite a bit. He has also started to look around more (thanks to being able to open his eyes finally!), smile, and overall seem to be back more to his old self. We are so pleased to see some progress at long last! Thank you for your continued prayers for this guy (and us!) as we wait to hear the results of the biopsy.

Sawyer is doing great! He continues to gain weight and will be approaching 5 lbs before we know it! He has been doing great with his breathing (needing almost no oxygen), so soon they'll try removing the low-flow nasal canula and see how he does breathing 100% on his own!

That's all for now...

s&j photos

sawyer & brad's ring

jacob smiling

sawyer KCare w/brad

jacob & dad

Update

Today was another full one! We met with the kidney specialist again and learned more regarding Jacob's condition. Like we mentioned before, he has a neuphrotic (kidney) syndrome, which means his kidneys are unable to retain sufficient amounts of protein. It is either congential (genetic) or acquired (non-genetic - in his case perhaps from medication). Regardless of the type, it is serious in that it affects his overall nutrition (which affects growth), causes higher cholesterol, and causes higher risk to infections and blood clots, to name just some. (I'm not going to attempt to explain why this protein leakage results in these risks, though if you're really interested you could check out wikipedia.) So the biopsy results will tell us if it's genetic or acquired, as well as, if genetic, if it's two of the possible 10 types. In addition, in three weeks we'll learn the extent of damage of his syndrome, regardless of the type. While we learned all that is potentially involved in either scenario, at this point there's not reason to go into it until we know more. All I can say is that the genetic types aren't really treatable but are able to be managed with different levels of medical intervention. The acquired ones are often treatable through medication, protein supplementation, and overall self-repair. We were told we'd find out the results late Tuesday afternoon at the earliest. So until then, we're praying that it is acquired and that God would miraculously heal the tough little body of Jacob's that He so wonderfully created. 

Last night Jacob had his first dose of protein (albumin, for you medical junkies) in the form of blood plasma via IV, along with a diahretic medication to help flush out fluids. That helped his urine output increase dramatically (yeah!) and helped him not gain weight last night and thus is less swollen. In addition, his first blood test showed increased protein, which is good, AND his urine has showed decreased protein from the last day or two. (Simply put, in his case increase in protein in urine = bad; increase in protein in blood = good.) We are so grateful and relieved that he has made great strides already! They'll monitor that level to determine when he'll need another dose of protein. His recovery has been good; he's back to full feeds of breast milk and he's back to his pre-surgery ventilator settings. Again, we are very relieved and proud of his recovery! 

So thank you in advance for joining us in prayer and praise for Jacob's health and recovery. We are praying that his kidney issues are not genetic and that God would miraculously intervene to heal him. We'd also ask you to pray for discernment for the medical team as they determine the juggling act between restricting his fluids until his fluid retainment problem resolves, yet getting enough calories for him nutritionally (since breast milk is obviously a fluid) and overall fluids for his hydration. This is unusual so there are no textbook formulas. 

The words in Isaiah 41:10 kept coming to my mind yesterday - to not fear because God is with us and will strengthen and help us. We are thankful for God's presence, strength, and peace; we need it!

Thank you so much for your prayers and support! We'll update you on Tuesday or whenever we learn the results.

P.S. So as not to neglect the "older" brother, Sawyer is doing great! His feeds just increased and he continues to do really well and grow. (Oh, and he's earned quite a reputation as a super pooper in the ICN... you can use your imagination on that one!) He is now 4 lb, 10 oz! We are so thankful for his health and great progress!

update

Jacob ended up having the surgery today to remove some of his kidney tissue for a biopsy - and he just got back. We are grateful that it went well and he is now recovering. He's now got a scar on his lower back right to complement the one on his upper left.  We'll know more regarding the results of the biopsy in the next couple of days - hopefully by Monday. Thank you for your prayers! We are so relieved that he is back and it all went well!

Baby Jacob

We just had a text msg from Brad and Jacob was taken to surgery for the kidney biopsy.  please pray for his safety and wisdom for the doctors.  Thank you!

 

Grandma Sharyn

J update

Friends,

Yesterday we got very devastating news back from Jacob's kidney consultation. In short, he has an unusual congenital kidney syndrome that is very serious. He will have surgery probably today or, if not, tomorrow to get a biopsy of his kidney tissue to determine if the syndrome is the result of a genetic defect or of some potent medication he took to try to close his duct (which didn't work and thus he had the heart surgery). Obviously we're hoping it's the latter, as anything genetic isn't really treatable and would potentially affect Sawyer and future kids as well. But either way, the best case scenario is a very long, slow, and difficult road. We feel weary, scared, and disappointed as we anticipate another procedure as well as the outcome. We covet your prayers, especially for Jacob's safety and healing.

I will have more info once we meet with the kidney specialist again today and also when we know the results of the biopsy. For future updates, instead of sending out emails, we now have a blog that you can check or subscribe to: www.regiertwins.blogspot.com

Thanks to Meredith for setting it up and maintaining it.

In Him,

Brad and Arja

32 weeks

Hello everyone,

Today the boys are at 32 weeks! It's crazy to think that if they had been born today, they'd still be TWO MONTHS EARLY! Anyway... just wanted to fill you in. Since Jacob's update is longer, I'll start with Sawyer...

Sawyer is doing "terrific" as everyone in the ICN keeps telling me. He's on a low flow nasal canula (1/4 liter of pressure) and room air for oxygen. This means that he really needs almost no support breathing; just a tiny bit of oxygen when his belly is full after eating. He weighs 4 lbs, 7.5 oz. That's right, folks, almost 4 1/2 lbs. Wow! He's eating well, growing ... nothing too new with him, as he continues to be the MVP of premies. 

Jacob continues to be doing just okay. We've learned a little more about what seems to be the issue: that his kidneys are spilling a lot of protein. Instead of utilizing the protein, he's peeing it out. This has caused the fluid retention and consequent puffiness. The good news is that his urine output has increased and is now good AND that he has finally lost weight (ie, fluids) the last couple of days. The bad news is that he still has a lot of protein in his urine and the doctors are completely puzzled. They said they don't typically see it in a baby this late after birth. His kidneys are clearly functioning, but they can't figure out the protein leakage.They originally thought it was linked to the staff infection and/or nutritional deficiencies, but he's now done with the antibiotics and the staff infection seems to be gone, AND he's had good feeds for a bit now. So those don't seem to be why. We are waiting on a consultation with a renal specialist and hope to get some answers there. It's interesting because in looking through his chart, I realized that he hasn't always done this. Interestingly, it started at the time they discovered the staff infection and started him on some potent antibiotics. It's obvious his kidneys were taxed. He's still needing the ventilator to breathe and, because he's on such low settings, it seems to be linked to him just not feeling good. I think he's close to getting off but not sure he'll be able to make the leap until the swelling is down and he's back to feeling like himself. They've restricted his fluids so his breast milk feeds haven't gone up, which has raised concerns that he's getting enough food to grow. So I'd continue to ask for prayer for this little guy - that God would give wisdom to the doctors and that ultimately God would touch and heal his kidneys & his body would be able to utilize the protein - and then he can move forward- get off the ventilator, increase feeds, etc.  Otherwise, he eats well, poops and pees well, his lungs look really good in the xrays, and his heart is great - so everything else seems to be normal. They guess that his real weight (minus the fluid puffiness) is 3 lbs, 12 oz. 

As their parents, we know we have much to be thankful for, and also obviously have some big things to be concerned about. One of the Bible verses that has meant a lot to me since I found out I was pregnant with the boys is Psalm 33:20-22 which talks about WAITING IN HOPE for the Lord. And that is what we are continuing to do: wait in hope.

Thank you so much for praying!

Love,

Brad and Arja

p.s. The photo is of Sawyer with Brad's mom, who just came to visit and was an ENORMOUS help! Thank you, Sharyn!

G-ma Regier w/Sawyer

Sawyer in clothes!