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 ♥
Wednesday, January 30, 2013
Sunday, January 27, 2013
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.
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.
Friday, January 25, 2013
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 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 ♥
Wednesday, January 23, 2013
New day, new plan
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.
Monday, January 21, 2013
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
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
Saturday, January 12, 2013
Save the Date!
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 :)
Wednesday, January 9, 2013
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 :)
Monday, January 7, 2013
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 :)
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 :)
Saturday, January 5, 2013
102, 81, and 21
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!
Friday, January 4, 2013
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 ♥
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 ♥
Tuesday, January 1, 2013
2013 & Facebook
HAPPY NEW YEAR!
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