Perspective

I posted a status to B's Facebook page yesterday and received an overwhelming response. Since it seemed to make people stop, think and gain a little perspective -- as I had hoped it would -- I thought I would share it here...

My name is Brady. In my short seven months I have spent more than 150 days in the hospital. I cannot breathe without tubes in my nose giving me oxygen. I don't get to nurse from mommy, drink a bottle or eat food. My food goes through a tube into my tummy. It has to go really slowly so I'm attached to a pump all the time. I take medicine six times a day to keep me healthy enough to be home. I can't go many places because it isn't safe for me to be around germs. I don't get to take real baths because I've had an IV in my arm for most of my life and for the foreseeable future it will stay there. It makes it hard for me to use that arm the way I would like. I'm not very strong because I am so sick. I cannot roll over or sit up or easily reach for my toys to play. I get very tired and need to sleep a lot. I spend as many as 4 or 5 days a week going from appointment to appointment where doctors and nurses and therapists poke, prod, and examine every inch of me. My blood cells are "broken", my liver is sick, and my lungs don't work right. I don't know when or if I will get better. I don't know if I'll ever be able to run and play with my sisters and brother. I don't know if I'll ever get to play little league or ride a bike or go swimming or go to an amusement park. I don't know how many tomorrow's I will get. But, I LOVE my todays. I smile, A LOT. I'm a happy little guy no matter what I go through. Maybe next time you're about to post a status telling the world how terrible YOUR day has been, how hard YOUR life is, maybe take a second and think of me. Think of all the other kids just like me.
Now, is your life really that bad???

Team Brady is growing!

I spent the morning welcoming the newest additions to TEAM BRADY. It was an incredible honor to meet the students of Mrs. O'Connell's Eight grade class from St. Bernadette's School and personally welcome them to the TEAM BRADY. These incredible young women & men have been offering up their prayers in support of B and fund-raised to purchase their own bracelets. Our family is truly touched by their kind-hearted gesture.




Now, for an update...

Brady went for his six month check up with Dr. Miller today. She was very encouraged by the progress he has made the last few weeks. He tipped the scales at 8 pounds and 10 ounces. That means he has gained more than a pound over the last two weeks -- way to go Bug!! We discussed my concern that Brady has been having frequent episodes where he gags and chokes on his own secretions. The episodes can last as long as ten minutes and often result in significant desats and sometimes cause blue spells. It's something I've brought up during hospital stays in the past but haven't had any luck getting a response to. I think maybe these episodes sound like no big deal until you witness one. Well, B was ever so cooperative and happened to give a live demonstration during our visit. So, Dr. Miller called B's pulmo and they discussed what's been happening. They both feel Brady needs deep suction at home and have already ordered it, to be delivered today! They also decided that he needs to have a swallow study done - we tried once before but Brady was admitted that day for jaundice - and that he needs an ENT eval. Those appointments are being coordinated by the peds office and we should get that done soon. Suction will be delivered tonight and VNA will be here tomorrow to show us how to use it. I feel so much better knowing we will have a safe way to clear his airway when he has trouble! Otherwise, since coming home he has done amazingly well. All his labs have been stable and March 31st will mark two whole months with NO TRANSFUSIONS!! I honestly can't believe it! Dr Higman likes to remind us that right now, he is riding the Rituxan wave so to speak and it's very likely he'll relapse at some point but for now we're just enjoying a little less time at the hospital.

Next week we finally have our intake meeting with the Medicaid RN to continue the process of getting Bug a homecare nurse. We're really hoping it won't be too much longer. Nights have been the hardest part for Tim and I. Between Brady waking frequently, having gagging episodes, desats, IV meds, etc. we don't get much sleep -- we're both exhausted! In the meantime, we've been referred for a few hours a day with a home health aide. Hopefully, that will make it a little easier to manage all that needs to be done during the day. Right now B gets meds six times a day, he is on a twenty-four/seven feeding schedule, he gets IV medication 12 hours a day, we need to perform GJ tube care every eight hours, he needs his PICC lines flushed every twelve hours, we need to vent his G tube for half an hour every three hours, he gets his temperature, respirations, and heartrate checked twice a day, and we need to weigh him daily. Now we'll be adding frequent suctioning to the list. Admittedly, Tim and I have gotten pretty good. We can handle a lot. But even if we had no other responsibilities that is a lot to manage. Factor in three other kids, the house, laundry, cooking, his work schedule... and forget it! We're barely treading water. It's overwhelming! We're just trying to hang on while hoping that help is on the way soon. So keep your fingers crossed and say a prayer that we get approved for the help we need!

As always, thank you for your continued support. Without all of the help and prayers and well wishes we would be truly lost!

With Love, The Getty's

Lessons from Brady

Someone asked me an interesting question today. They asked if having a child with complex needs has changed me and how. It has absolutely changed me.

Lessons from Brady

He has taught me that I am strong; that I can do so much more than I ever thought I could.

He has taught me how to endure. That the bad does eventually give way to moments of goodness, even if they're fleeting. Keep going because those moments are priceless. Those moments are worth the struggle.

He has taught me to love with my whole heart.

He has taught me that laughter heals. Even when things are bad it's okay to laugh. Actually, it's important. When everything seems to go wrong and the day seems endless and the exhaustion is all consuming a good laugh really does make me feel better.

He has taught me to forgive. A person is flawed but people are good. Everyone makes mistakes. They say the wrong thing. Do the wrong thing. But most often, they make up for it ten fold if you give them the chance. And even if they don't, it takes so much more energy to begrudge them than to just let it go.

Because of him I've learned to stop and take things in more. I notice the sweet smell of his skin. How soft his cheeks are. How infectious Ethan's full belly laugh is. How sweet Miranda's dimples are. How Kristyna smiles with her whole face.

I've learned what matters. Time matters. Now, instead of brushing the kids off I try to stop what I am doing and focus on them. We play more. We laugh more. We snuggle more.

I've learned that love and support comes from the most unexpected places. In a world with so many problems there is still so much goodness. We live in a wonderful community and the kindness of strangers is incredible. I've learned that it's okay to accept help when it's offered. It's okay to reach out and ask for help.

I've learned to find joy in the small victories. The daily accomplishments. The quiet moments. There is something so incredibly amazing about watching a child learn something new, discover how things work. It's a gift to watch them blossom into themselves.

I have learned to trust Tim. He might take a little more time than I do but he always gets there. He can do everything for Brady that I can do. So much of caring for Brady is way beyond his comfort zone but he has learned to do it any way and you know what? He has learned to do it well. I've realized that for so long I have underestimated him. Given the chance, he really has become a better parent. I think I was holding him back in a way but Brady has given us the opportunity to grow as a couple. We still struggle sometimes but I know I can trust him when it really matters. When push comes to shove, we are in it together.

I have learned to let go. Anyone who knows me knows I struggle here. I tend to want to do everything myself because I need to know its done right. But I can't do everything. I cannot be in two places at once. I cannot take care of everything all the time. I have to let others step in and trust that it will be okay. It might not be the way it would have been if I were doing it but just because it is different doesn't mean it is wrong.

I have learned to have faith. It's still an ongoing challenge but I am learning to trust God. There have been so many times since Brady was born that I have felt so lost. So desperate. When I was sure we could not get through it. Somehow we have gotten through all of it. There have been a few times when we have been down to our last dollar and then money comes in from somewhere or someone unexpected and we squeeze through. When I feel like I'm starting to break something simple renews my strength. A card from someone we have never met. A comforting word from someone unexpected. Something simple to smile about. And what I've realized is that those perfectly timed little blessings? That is God at work. He is trying to show me that He is with me, with Brady, with our family. We are not alone in this fight and I need to learn to trust Him to lead us through this. I now understand what people mean when they say "Give it to God". My faith still falters but I am finding it more and more.

Because of Brady I have learned how to really live and I am a better person because he is in my life.

Blogger Fail

I know, I know. Worst blogger ever. I'm sorry :(

Just a reminder: if you're not seeing blog posts you can always check out Brady's facebook page -- Sweet Brady -- for up-to-date info.

Now, a long overdue update...
B is back in the hospital. Thursday 2/23 he started running low grade fevers off and on. He would feel slightly warm, I'd check his temp and it would be hovering around 100 and then when I'd recheck an hour later he would be fine so we decided to just keep an eye on it. Then Saturday night I noticed he felt pretty warm. I unwrapped him and changed him into cooler jammies and his temp normalized. But by Sunday morning we knew he wasn't feeling well. His fever was ranging between 100.0 and 101.5 and he was starting to show dips in sats, hanging out around 92%. He was also having more frequent and borderline diarrhea poops (TMI, sorry!). We called the pediatrician and they said for us to take him to the pediatric urgent care in Williamsville. We got there around 11:30am and knew right away things were not good. When we arrived there his temp was 103 and his sats were worsening. They drew labs and said he was dehydrated and his liver numbers were elevated. They felt he was too unstable for us to take him to the ER and called the WCHOB Stat Team to transport him by ambulance. They kind of down played things and were really calm about the whole thing so to be honest we didn't really think things were too bad...until we got into the ambulance and they said we were going "hot", meaning with lights and sirens! Um, okay. Never a good sign. When we arrived in the ER they explained that Brady's sodium was very elevated and that that put him at high risk of having a seizure. He was admitted to the PICU :(

He spent eleven days in the ICU and was transferred to the 4th floor yesterday evening. Turns out he caught rotavirus and because he has no immune system he could not fight it off. They ended up putting IGG (antibodies) into his gut through his GT. The IGG fought off the infection that his body couldn't and he stabilized pretty quickly at that point.

Between the crazy leaking from the tube and the rotavirus he's lost a lot of weight again. He dropped down to just 7 pounds. He has started gaining a little again and is up to 8lb 1oz. They've switched his G-tube to a GJ and he is currently getting all his food through the J port, which feeds directly into his jejunum...



The hope is that this will give his stoma (hole in his tummy where the tube goes in) a rest and allow it to heal so that it won't leak any more. They've also dialed down the caloric concentration of the formula (which may have contributed to his diarrhea) and changed it to Pregestimil which is specially formulated with a different type of fat, called MCT oil, to be gentler on his liver. We're starting out slowly. The rate is being increased by just a few ml's per day. The hope is that by using the J he will be able to eventually tolerate a higher volume and get the calories he needs without having to concentrate his formula so much. Fingers crossed!!

For anyone asking about Team Brady bracelets, we apologize but they are currently sold out -- wow! More are on the way. Watch for facebook updates for when the new order arrives. Chinese auction baskets are being collected and I believe they're hoping to have the majority of them collected by March 31st so if you have a donation call any of the committee members to arrange a pick up time. Tickets are selling fast too so please don't forget to get yours! We have been completely overwhelmed by the incredible response to this benefit. We are truly blessed with an amazing community, family, and group of friends. Thank you times a million!

Love & Hugs,
Shari

Team Brady

My apologies for not updating sooner -- things get busy around here as I'm sure you can imagine!

Bug is doing pretty well. He went to WCHOB on Tuesday for his 4th rituximab treatment and, as always, handled it like the tough guy he is! His labs looked stable still and his hemoglobin was 9.4 -- woot woot!! If all goes well and stays stable he won't have to go back until March 12th for his monthly IVIG.

Wednesday he had his Early Intervention evaluation. He qualified for both PT and OT services and will also get help with feeding. He is currently at the developmental level of a two month old but is showing some scattered 3 month old skills. Hopefully he won't get any further behind now that he'll be getting therapies weekly.

Next week he has appointments with his surgeon, his GI, and his nutritionist. He's continuing to lose weight despite being on a crazy high calorie formula blend and his G tube is still leaking. Hoping they can put their heads together and come up with a solution to get some weight on my tiny little guy!

TEAM BRADY bracelets have arrived -- If you're interested in purchasing one you can make a $3 donation to Brady's paypal account (TeamBradyGetty@gmail.com) and then send an email (same email address) with your name, email address you used to make the donation, & address. Your bracelets will be shipped to you ASAP! If you live in the Buffalo NY area then message Sweet Brady on facebook to arrange a local purchase :)

Break up and make up

Well, we're being discharged Wednesday and BCH is breaking up with us, so to speak. Lol

Since we broke up with WCHOB before transferring and now we'll be getting Bug's care there again I guess it's time for us to make up. But it's hard. Our trust was definitely broken and it will be a long road to rebuild it. They say time heals all wounds and I hope that will prove true but for now we are definitely feeling anxious about placing our Sweet Brady back into their care. We've loved the whole team centered approach they've taken here at BCH and that team spirit was something that was sorely lacking back at home. We're hoping things will be different now. We hope we've made it clear what our issues were with the care Brady received and that our concerns will be addressed. Only time will tell I guess.

Apparently this blog has been read by B's Buffalo care team and they weren't super thrilled with what they've read. I'm not happy that anyone may have been upset or hurt by what was posted here, that was not the intent, but I will not apologize for speaking my mind either. Nothing was said here that wasn't or wouldn't be repeated directly to any of his doctors. We made it clear, or so we thought, that we were not happy with the course of treatment. We asked for a transfer. We made it known we wanted a second opinion and why. To the opposite end, I've also shared here when I felt like someone did something positive. I've raved here, and to anyone who will listen basically, about the stellar level of nursing care we received every step of the way. But that doesn't change that mistakes were made. Things were missed. As a result our peanut suffered. The past cannot be erased. But we will move forward making every effort to work cooperatively with his doctors back home in Buffalo. All that matters to us is that Brady gets the care he needs!

Down the rabbit hole

It seems like I've been struggling these last months to catch up to all that is happening in my life. I get to where I think I'm within reach of getting a handle on things and then something else jerks it away and I'm struggling again. The other day they were playing Alice in Wonderland at the hospital and all I could think is that I fell down the rabbit hole. This new world is the same as my old world and yet it's so different. Nothing is as it appears. Most days my baby is yellow. Feedings involve tubes & pumps not bottles. Beeping is so common I don't even notice. Burping my baby involves a syringe not a cloth. Asking "Did you flush?" has absolutely nothing to do with a toilet. There is a constant loud hum in the background. This world is filled with poles, and pumps, and tubes, and cords, and syringes, and medicines, and tanks. My cell phone recognizes more medical jargon than a second year med student and I spout it out like a second language. This is my world. This is Tim's world. This is Kristyna, and Miranda, and Ethan's world.

And this is Brady's world. My beautiful beautiful boy. Who looks absolutely perfect. Who is so very sick. His body is betraying him. It is slowly destroying itself. And it is slowly breaking my heart. But I have to be strong. Strong like my boy. He fights so hard so I will fight with him. He will never ever carry this burden alone. Mommy will be by his side always. We've gone down the rabbit hole together and no matter how crazy things get down here in Wonderland I will hold tight to my precious Brady. I will never give up hope that one day we will find our way and come through the looking glass and the world will be the way it should be once again. I just have to hold on...

Biopsy Results

Hello. This post is brought to you by Liane (Shari's sister/Brady's aunt).

After Brady’s liver biopsy on Friday, February 1st, he was diagnosed with Giant Cell Hepatitis (GCH). When you combine GCH with Brady’s AIHA, the prognoses is poor. There will be a family meeting on Friday with Brady’s medical team and social worker, where Shari and Tim will learn more about this rare disorder and what the future holds. However, after doing some research of our own, the outcomes do not seem favorable. Here is a link to a case study that will explain this rare disorder better than I can:

Giant Cell Hepatitis with Autoimmune Hemolytic Anemia

It is important to remember that every case is different and there is no way of knowing how Brady’s body will respond to treatment. At this point Brady’s liver is stable, but things could change rapidly. You may not see a blog update for a while, as this is a very emotional time for Shari and Tim. They just need some time with their family to process this unsettling news. Please keep Brady in your prayers and never lose hope that our little fighter will get through this!

The wait is on

Bug's biopsy went really well yesterday. They used moderate sedation so that he wouldn't need to be intubated. He was in the OR about two hours and after a quick half hour in recovery we were back in his room. His pain didn't seem too bad. He had two doses of morphine and thanks to the anesthetic in his system he spent most of the day napping. He did awesome overnight too and I was able to get the most sleep I've gotten since we arrived in Boston! This morning he was pretty fussy and couldn't get comfortable so I asked about possible pain and they gave him another dose of morphine. He's resting comfortably again.

I'm so relieved the biopsy is behind him. Now its waiting time. They're hoping to have some preliminary results back on Monday but the full pathology work up will take 5-7 days. So much is riding on those results that I swear I'm going crazy waiting :(

B's hemoglobin is stable today at 7.8 so that's good. That means he didn't have any bleeding from the biopsy. Since he was transfused Thursday that means today is day 2 with no transfusion. Hoping he starts to stretch longer in between at least. I really don't want to have to put him through another major surgery but if he doesn't stabilize then the splenectomy is really the best option. He'll get his second dose of Rituxan Monday so that will hopefully help!

B's benefit really seems to be coming together. I am absolutely humbled by the overwhelming response so far. I think it's safe to say that Bug's event has gone mini viral with over 5,000 invites going out in less than a day! Amazing. A huge thank you to all of you who have generously offered to make donations, seek donations, volunteered your time, are preparing baskets, or just spreading the word. It all means so very much to our family. Truly. TEAM BRADY committee -- you're doing such an awesome job and we're so grateful you're working so hard for Brady! A special thank you to ComDoc & Travis Weiss for doing all the tickets, flyers, stickers, etc absolutely free of charge. Everything looks great!!

Guess that's it for now. I'll update again as soon as we have some results from the biopsy.

Shari ♥

Four Days

Brady hasn't had a transfusion in four days. If he isn't transfused today then this will mark the longest he has EVER gone!! His hemoglobin is still not at a normal level but it has been relatively stable. Today it is 7.4, yesterday it was 7.6, and Monday it was 7.3 in the morning and 7.5 in the evening. That means it has even risen slightly on its own a few times!

He's been working with the feeding team here and making slow progress. They feel he should adjust to oral feedings without any major problems. The only concern is how much volume he will be able to tolerate so a GI specialist will be consulting on how best to transition him to larger volume feeds rather than continuous feeds.

Yesterday he had a follow up xray. He will have a follow up hearing screen today. On Friday he will have a liver biopsy and a complete cardiac echo. Then next week, if he is still stable, he will be scheduled for an MRI.

He is trending in a positive direction overall but there are definite concerns still and always the possibility of a setback. Splenectomy is not off the table but if things continue this way that may be able to be avoided.

Things have been a bit of a struggle as far as staying here with B. Hospital policy is to only allow one parent to remain overnight which leaves the other of us with no place to stay. We paid for a hotel room one night, spent one night in an Parent Sleep Space for ICU patients, and spent one night at an off site Ronald McDonald type house (Yawkey House) which offers a reduced rate. They do have a RMcD house here but it is reserved exclusively for cancer patients & families. Yawkey House & another like it, Autumn house, do not have any more availability for us until mid february. With the cost of a hotel room near the hospital averaging $200+ for one night that just isn't an option at this point. So the social worker on the unit spoke with the powers that be and Tim was allowed to stay in the units treatment room last night. So long as it isn't being used then he can spend the next few nights sleeping in there. Its pretty uncomfortable but at least we know where we're both laying our heads!!

Things aren't anywhere near normal and we miss the other kids like crazy but we're pushing through it. Both Tim & I will do anything it takes to continue to get the care Brady needs to get well. We thank you for all of your continued love, prayers, and support during the most difficult thing our family has ever endured.

All our love, The Getty's ♥

Hello Boston

This blog update is coming to you from the Medical Intensive Care Unit (MICU) at Boston Children's Hospital in Boston, MA. That's right, at just 4 months old our little Bug went on his first plane ride yesterday. He was airlifted here when doctor's at WCHOB said he was in liver failure and they no longer felt they could adequately care for him.

But, today is a new day. A fresh start. Brady has a brand new team of doctors with new ideas and perspective. Here at Boston Children's (BC for short!) they take a very team centered approach, prioritizing his diagnosis and then assigning teams accordingly. In B's case his most critical issue is hematologic so the hematology team is his primary team. Secondary to them is the hepatology (liver) team, and then pulmonology since his lung disorder is stable.

So far we have been blown away by BC. They've been right on top of things and making decisions from minute one...and...WE HAVE A DIAGNOSIS!!!!! Brady has Evans Syndrome.

Information about Evans Syndrome can be found here.

The hematology team is starting a medication called Rituximab this afternoon. This is a B cell suppressor that will hopefully knock down his immune response and slow his hemolysis. The issue is that he has been being transfused for so long that his transfusions are devastating his system with a toxic amount of iron. Another week of daily or almost daily transfusions would likely kill him. They need him to respond extremely quickly to this medication. If not, he will need to have a splenectomy as early as Friday or Monday.

Tim & I are feeling extremely angry about the care he's received the last few months. How could his WCHOB doctors not know that he would not survive continuing transfusions several times a week and not going forward with treatment. Instead of helping him they were essentially poisoning him. His body is so overloaded with iron that he could have permanent damage. Continued iron exposure in those doses would overwhelm his system to the point of multiple organ failure. One by one his liver, kidneys, lungs, and heart would fail. They almost killed our baby! We're relieved to have transferred and wish now that we had stuck to our instincts and done so back in December. We mistakenly believed what we were being told. We trusted that his doctors knew what they were doing. We were very very wrong.

NOT pneumonia

Final conclusion from Lung Center: NOT pneumonia. They say it is either a mucus plug or a small area of atelectasis, both common issues with interstitial lung disease. So, they're now trying once again to explain to the anesthesiology team that he does not have pneumonia and is not any more of a risk to go under than he ever is. Haven't heard a response but keeping my fingers crossed that they finally relent!

New problem though. A few hours ago Bug's nurse Kristen and I noticed that his belly was starting to look distended. Now, in the last hour & a half its so distended that his belly button is popping outward and its pushing his gtube out. The docs on the floor just ordered a bunch of blood work and he'll probably get an abdominal ultrasound to try and get a handle on what's going on. I swear, it never ends!!!

Its been so bad lately here. Yesterday I talked to another children's hospital in Boston and they're opinion is that B belongs in intensive care. They wanted us to initiate a transfer and have B airlifted to their ICU immediately. I had Dr. Higman paged and told her we were seriously ready to request a transfer. Surprisingly, she said she had already been considering it herself. She asked that, so long as B does not worsen before then, that we give her one more week to try this new blood theory/treatment plan. If by the end of next week he is not doing SIGNIFICANTLY better then she will give her blessing for a transfer, help us choose which hospital would be best suited for his needs, and coordinate the whole process with us. So we agreed to give it a shot. ONE WEEK. If Bug seems to be sliding downhill before then though, all bets are off. It will be very difficult to separate our family and be out of state with Brady but priority number one is making sure he is getting the best care possible so we will do whatever we need to. Pray pray pray it doesn't come to that though!!!

I'll update again later if I can. If I don't have time to get here I almost always take a minute to make important update to his Facebook page so go "like" there for more up-to-date information.

Lots of love, Shari ♥

New day, new plan

This is going to be a long and somewhat technical post, please bear with me. I'll do my best to explain things, it'll just take some extra time...


Yesterday was an absolutely nutty day. We brought B into the hospital Monday night for his Tuesday morning liver biopsy, as planned. However, nothing else went as planned!

When we got here I couldn't switch B over to the hospital's oxygen because there was no flow meter hooked up. The nurse paged respiratory. They came and put in the flow meter, hooked up sterile water to humidify it, and switched B over for me. All set right? Um, NO. About ten minutes after the RT left the room B started gasping and his pulse ox started alarming. His sats were down around 87. I scooped him up and tried to help him clear his throat since I figured he just had some excess secretions. At that point the shift had changed and the night nurse, Colleen, came in to check on him. I asked her to flip on the light. By now B was getting a grayish pallor and was blue around the mouth. His sats were down around 83/84%. Colleen reached to check the oxygen and we quickly realized something was wrong there. No air seemed to be coming through the flow meter and the water wasn't hooked up properly. Before we could react B got a nose full of fluid and began to choke again! The tubing had filled with water and shot into his nose. I pulled the cannula down out of his nose while water continued to drain from it. Then I grabbed our portable O2 and hooked him up to that while Colleen paged RT. They came and switched the flow meter and attached the water correctly. The whole episode lasted maybe fifteen minutes and then Bug settled down and went back to sleep. I think Colleen and I were both shaken up but everything seemed to be okay at that point.

A few hours later as I was getting Bug changed and ready for bed I noticed his breathing was more labored and he was having moderate retractions (What are retractions? Click here.) I called Colleen to look and she called in the resident to make her aware. As the night progressed things worsened. B's respirations were often into the 100's and his retractions worsened. By early morning the resident called the attending and a portable chest xray was ordered. The docs rounded about 8am and it was during rounds the preliminary results of his xray came back...aspiration pneumonia. Aspiration pneumonia is an inflammation of the lungs caused when a foreign material, like food or formula, is breathed into the lungs. It can be very serious for anyone but for someone with lung disease, like B, its especially dangerous. They immediately started him on antibiotics. At this point his blood work had also come back showing his hemoglobin was very low and his bilirubin was very high. They ordered a blood transfusion and paged GI as well as anesthesia. It was decided then that B was too unstable to safely be put under anesthesia and the biopsy was cancelled. With his bilirubin above 11 we were of course very worried and wanted to know what they planned to do to treat it. We were told they weren't going to treat it, We couldn't believe it!! Why wouldn't they help him? We also asked if he wasn't stable and his sats were poor, was it safe for him to remain on the heme-onc unit or should he be transferred to the PICU? They didn't really address our concern, just said he would be staying on the floor. Um, okay. Also during rounds they had discussed that his labs showed his platelet count was rapidly dropping. This hasn't been an issue for B, only RBC's, so we asked about this too. They said they didn't know why and left it at that. By that point, Tim & I were a wreck. Clearly, our baby is very sick and they weren't really doing anything to help him.

At this point we needed an ally and decided to call Buggie's pediatrician. The nurse at the office told me she was off until Thursday but a while back Dr Miller had given me her personal number and told us to please call her if we were ever concerned. After a lot of back and forth we decided it was important enough to go ahead and call her at home. I explained to her what was going on and that we were concerned there didn't seem to be any kind of plan of treatment and that none of the various departments were really communicating with one another. She promised to log onto the hospitals network, review his labs & chart, contact the docs here, then get back to us. From there, we could see the domino effect of her phone calls.

Dr Alkhouri from GI called to talk with me. She explained that she had just had a meeting with the entire GI attending staff regarding Brady. They came to the agreement that more than likely, his liver issues are secondary to his hemolysis. If he stops hemolyzing then his liver function will return to normal. If he continues to hemolyze he could do permanent damage to his liver so stopping it is top priority.

Dr Bye from lung center also stopped in. He said he wasn't entirely convinced B actually does have a pneumonia since babies with SP-C deficiency can often show areas of infiltrates and haziness but he had no problem with them continuing the antibiotics any way.

The day resident from Heme-Onc as well as the covering attending came in to talk to us again and explain better this time. The only concern with the platelet count would be in relation to surgery since that would put him at risk for bleeding. Otherwise, they were not dangerously low and often platelets can drop in response to infection.

As for the hemolysis being more out of control (5 transfusions in 4 days), Dr Higman (B's primary hematologist) came by this morning to discuss it and she feels she may have somewhat of an answer...

Here, I'll need to give a little blood cell lesson. First lets define a few things.

• B Cell - A type of white blood cell. Its main job is to fight infection.
• T Cell - A type of white blood cell. Signal B cells to respond.
• K Cells - Recognize any antigen a fetus is exposed to during gestation as autonimous, or belonging to the fetus. Stops an immune response to these antigens.
• Antigen - A substance foreign to the body that causes an immune response.
• Antibody - Identifies and neutralizes foreign objects. Produced by B cells.
• Self-Antigen - Antigens inherent to a person which the body recognizes as not foreign but belonging to itself thereby not stimulating an immune response.

As you probably know, everyone has a blood type. That blood type is determined by two main factors: One is the presence of (self) antigens A, B, or both. If neither antigen is present that person has type O blood. If both antigens are present that person has type AB blood. If only A is present, A blood. Only B, B blood. Simple enough, right? Then there is an antigen called Rh. If Rh is present on a persons RBC's they are said to be Rh+. If not, they are Rh-. So, let's use B as an example. His RBC's have A antigens and Rh antigens so he is blood type A+. That means, in theory, B can safely receive blood with no antigens (type O-), blood with only A antigens (A-), blood with only Rh antigens (O+), or with both A and Rh antigens (A+). But, in some rare cases, its not quite that simple. There are over 600 other subtypes of antigens, or minor antigens. Brady seems to have antibodies against quite a few minor antigens. Recently, they've found that he is making antibodies against a particular antigen which they're referring to as Big E. When he receives a transfusion of blood containing Big E antigens his body recognizes them as foreign and attacks those cells (hemolysis). In order to better control Bug's hemolysis they are now filtering his donor blood to exclude any minor antigens to which he has antibodies. This should allow those donor cells to survive longer and space out his transfusions more. Her treatment plan is to give Brady a strong immunosuppressant medication. This would put his B cells out of commission. They would no longer recognize and attack all the little antigens in his blood. Since his bone marrow is now working as expected and kicking out his own baby red blood cells this would hopefully give him time to make more and more of his own blood without it being destroyed and eliminate any donor blood from his system. Then, once his body contains only his blood they could stop the suppression and the hope would be that his B cells will have reset, recognize his blood as his own, and will stop causing hemolysis. However, there is potential that the problem is actually with his T cells. Since his T cells direct his B cells, if they are not functioning properly they may be telling his B cells to attack. If that's the case, as soon as we stop suppressing his immune response the B cells would still be being incorrectly directed to destroy his RBC's and the hemolysis would kick right back in. So, she called for a consult from Immunology to help determine if both T & B cells are involved. If both are in fact causing the hemolysis then we would need to suppress both. This would require stronger medications and leave him even more vulnerable to opportunistic infection so its important to make sure its absolutely necessary. Also, before being able to safely proceed with that course of treatment she needs to make sure we're absolutely certain there is no infectious process going on in B's liver so the biopsy would be essential. The problem is getting anesthesiology to agree to the biopsy now rather than making him wait six to eight weeks and delaying treatment that much longer.

So today has so far consisted of the great pneumonia debate. Is it or isn't it? Is he stable enough for the biopsy? If they hold off on the biopsy will he be any more stable in eight weeks or could things potentially be much worse?

If I'm brutally honest with myself, I'm not sure he can take another eight weeks at the rate he is hemolyzing. It's destroying his body. Seems to me the risk of a few days on a ventilator is worth it if it means being able to start the treatment that could save his life. Actually, its really very simple. He needs this treatment. He needs the biopsy in order to get the treatment therefor he MUST have the biopsy. It truly could be the difference between life and death for him. It's up to anesthesia now. I pray they make the right decision.

Another hurdle to jump

Apologies for not updating sooner but it's been a totally crazy week!

We took B in for a transfusion on Sunday 1/13. Business as usual, in and out in about 5 hours. Monday morning he had his surgical follow-up. Dr Bass said she would like to start him on a new medication to help with his continual gagging & retching since she feels it is due to B having very delayed motility. We were told that surgery would contact our pedi and we would be given a script. So far, no script! Grrr. Tuesday we followed up with the pediatrician. Nothing new there, just a weight check and a quick exam. Wednesday things started to head down hill. For some reason, VNA screwed things up and took B off their schedule. This meant that by Wednesday he hadn't seen a nurse since Sunday. So I called and was told they couldn't get anyone out to see him until the next day, Thursday. Then I called and left a message with Lynn, the Heme NP, to see about Brady's weekly IVIG. Never heard back. By late into the night Wednesday I was getting pretty concerned about his color, heart rate, and respirations. I called Tim at work around 4am and asked him to leave early so we could take him in ASAP. I then paged the on-call hematologist to give them a heads up that we would be on our way. Tim took him in and they got there just before 6am. They tried to draw blood and realized that his PICC line wasn't working. From that point nothing went right. It took the docs all day to decide what they wanted to do about it. Tim & I switched off at the hospital around 4:30pm. Finally, around 5pm they brought in a NICU nurse to place a temporary IV line and then at like 9pm started a blood transfusion. After waiting nearly 14 hours his hemoglobin had tanked and was down to 4.1! Since then we've been struggling to get his hemoglobin stable again. After that initial transfusion he only came up to 5.5 so they transfused again and discharged him late Friday night. By Saturday evening he was really struggling to breathe and just did not look good so we rushed him back in and he was transfused yet again. This time Mommy through a fit and demanded they give him a larger volume of blood. His hemoglobin went from 5.3 to 8.8 after the larger transfusion and we were discharged again last night.

Today, he will be readmitted! Throughout all this they've been tracking his liver function also. His enzymes are remaining elevated and his bilirubin is steadily on the rise. They don't yet know why but its clear B's liver isn't functioning properly. So tomorrow he will have a liver biopsy. In most cases, its a simple outpatient procedure but of course nothing is simple with B so he needs to be admitted and observed closely. The biopsy will be done in the OR under general anesthesia so he will need to be intubated. In the past, he has struggled with getting extubated and needed some ventilator support but we're really hoping that won't be the case this time. If all goes well and he breathes on his own post-op then he will stay the night on the heme-onc floor and be discharged the next day. Otherwise, we're looking at yet another PICU stay :(

So we are once again asking you for prayers. His procedure is scheduled for 11:30am so if everyone could just say a quick little prayer for him at that time we would be extremely grateful.

Love & thanks as always <3

Save the Date!

Friends and family are planning a benefit for B on Saturday April 20th -- more details to come!!

B has been doing well since we got home. He doesn't sleep great and is pretty demanding of our attention so I haven't had much time to update, my apologies. He has appointments with the surgeon and the pediatrician this week so I'll have more of an update later this week :)

Grow baby grow!

Brady weighed in at 8 pounds 5 ounces today -- woot woot!!

And...

We get to go home tonight!! Brady's liver scan looked great, his bilirubin is back to normal and his liver enzymes are almost there. They'll monitor him closely for a few weeks but there's no reason he needs to be here :)

Overwhelmed

Just asked one of the residents to come in and give me a run down of where we're at with B...and wow, a lot to process.

So...one of the tests they did, Anti-HBs, did come back positive. This means that B has antibodies to Hepatitis B. More than likely, Brady received the antibodies (which anyone who has been vaccinated against or has had Hep B in the past would have) during an IVIG transfusion and does not have Heb B himself, but, there is a small chance (1 in 200,000 to be exact) that he could have contracted Hep B through one of his blood transfusions. The test that is coming up positive is the first one that would show up positive during a specific window of time after contracting the virus when others, Anti-HBc & HBsAg, would still be negative. They are sending out another test tomorrow morning that will provide the definitive answer. Hepatitis B is obviously THE worst case scenario but, it's also the least likely, so I'm trying not to panic unnecessarily. If the test comes back negative for Hep B then that leaves two other likely causes. He could have what they call a "Heme stone" in his liver, which is caused by iron build up from transfusions and/or hemolysis, and that is blocking one of his ducts. Or, he has hepatotoxicity from the chelation medication his takes. They've temporarily stopped that medication. If its determined that is what caused this then he would not be able to continue that and would need to try other chelation meds. The problem is that those medications are less effective and still have the possibility of being hepatotoxic, meaning toxic to the liver.

B's hemoglobin is 7.8 so its holding out nicely. His bilirubin is down to 1.7 and his liver enzymes are around 500 -- way down from over 2000 at admission -- but still a long way from normal, which would be below 35. The docs did say that they're low enough that if he wasn't scheduled for a hidascan and IVIG wednesday then he would be being discharged so that's awesome. At this point, they're fairly confident he can be discharged wednesday night.

I'm not sure if I mentioned that they changed his formula again, actually back the the Enfacare that he originally transitioned to when he ran out of my milk. The Nutramigen was running right through him causing diarhea and a horrible diaper rash. Also, the Enfacare has extra nutrients that boost babies who are preemie or "failure to thrive". They also upped the calories per ounce from 24 to 27. To put it in perspective, normal formula or breastmilk contains about 18 calories per ounce. Brady burns so many calories just to fight the anemia and lung disease that he needs to take in roughly 50% more calories than a healthy baby would.

Well, think that's it for now. Thank you for all of the prayers and support through this. Things were pretty scary for a day or two but B is tough little guy and things are looking up :)

102, 81, and 21

Brady is 102 days old today! He has spent 81 of those days in the hospital and 21 precious days at home.

The doctors told us today that B will be here until at least Thursday. He needs to be monitored while he is on phenobarb, his liver function needs to be rechecked at the end of the course, and because he is still so small he needs to gain 20 grams per day minimum. For a teeny tiny like Brady that's a lofty goal!

Things are looking up though. His bilirubin was down to 4.1 as of 4pm today and his liver enzymes are slowly coming down too. They drew even more labs for even more tests this morning but they say we won't have anything back for at least 48 hours.

His hemoglobin is still pretty good, at 8.8 this morning. Dr Higman sent out a specialized test for something called a warm IGA autoantibody. One of the hematologists they reached out to in Texas said he knew of ONE CASE where there was a 3 year old boy with unexplained hemolytic anemia and it turned out it was due to this autoantibody. ONE CASE. So, its unlikely but worth looking into. I'm not sure how long this test will take to come back but I'm hoping it isn't long!! Before this liver episode they were about ready to start B on the immunosuppressive therapy I wrote about before and now this has set that plan back. So frustrating. The sooner we start trying alternative therapies the sooner we reach potential for a remission!

Back in the hospital

Wednesday we brought B in for his regular IVIG therapy and also a blood transfusion since his hemoglobin was at 6.7. We spent the day here, he got his infusion and blood and after about 12 hours or so we went home as usual. Then yesterday morning we got B up for an appointment with the feeding clinic and were immediately concerned about his color. He was looking jaundice and still a bit pale.

We brought him in to his appointment but while we were waiting I called the nurses on the hematology floor and explained to them my concerns. They grabbed the resident on the floor who told us to take B up to the hematology NP, Lynn, after our appointment. We went up to see Lynn around 11:30. She drew some labs, told us to grab some lunch, and she'd meet us in the waiting room in about an hour with the results...

The results were not good. Brady's hemoglobin was only 7.6 (less than 12 hours after a blood transfusion), his liver enzymes were very elevated, and his total bilirubin had gone from 2.1 to 6.9 in less than 24 hours. (A normal bilirubin level would be >1.) Lynn told us they were going to be admitting him and we needed to head down to the ER so they could do an abdominal workup. The concern was that he may have some kind of blockage in his liver.

We got down to the ER around 4 and were told they didn't have a bed for us. The charge nurse there sent us up to the heme-onc floor. When we got to 4 they told us they were completely full and B was supposed to be in the ER. We explained that they had sent us up, some calls were made, and we were shuffled back down to the ER. Since B is immune compromised we were held in a triage room until there was a bed available for him.

Once back in a room they drew TONS of labs and sent B for an abdominal ultrasound. The ultrasound looked clean but his labs were getting even worse. We we're told we were being admitted under the GI service but they weren't yet sure if he needed to go to the PICU or could go to a regular bed. Finally, about 8pm the decision was made to send him to the floor. Since the hemot floor is full we were put on Variety-8, Brady's old stomping grounds!

And here we are! The nurses put us in a family care suite (much bigger than a regular room and with a bed for mommy) and got us comfortable. Overnight they held his IV med and gave him another blood transfusion. When I got him up for a diaper change around 3am I thought his color was starting to look better and this morning the GI fellow said that his labs have improved slightly. His hemoglobin is up to 9.9 so at least that's good for now. He'll go for a more thorough ultrasound sometime today and they're running blood tests for metabolic disorders, hepatitis, epstein-barr, and also to check to see if he's producing any antibodies against the liver.

We've been told not to expect to be able to go home today. For now, that's all we know. If something significant comes up I'll be sure to update. Until then, please continue to pray for Brady!

With Love, Shari ♥

2013 & Facebook

HAPPY NEW YEAR!

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