A chronicle of our trials and triumphs as we battle Hyper-IgE syndrome (Job's disease) with our baby boy Sam.
"Immune dysregulation" is the latest term to describe Sam's condition. If it's unclear, you're not alone. The positive news is that Sam's mytogen responsiveness (ability of cells to fight off infection) was markedly higher than it would be if he had a SCID. So, we're out of the woods on a "bubble-boy syndrome". Which leaves us...still guessing. Immunology told us today that we may never have a name for his affliction. Not exactly what a parent wants to hear. It's frustrating to hear conflicting messages from our doctors (even if they only differ slightly). We are well past them needing to sugar coat a diagnosis to keep us happy. We've been up and down enough already. For now, we know his immune system is performing only moderately well with a steady steroid dose to fend off the eosiniphils. He needs to wrap up a couple more days of IV Bactrim, then we can continue the bulk of his treatment at home. We're preparing for some significant life changes to accommodate at least the next few months until we can see the progress of his weight gain, steroid treatment and milk allergy. We've prepped the kids for the change; we're getting ready to keep him mostly isolated without him feeling isolated. In the meantime, we are trying to focus on the fact that he's happy, he's here, and we're a family.
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We received some disappointing news today. Up until this morning we held hope that Sam was immune compromised only due to his milk allergy/malnutrition + steroids. We found out today from some additional bloodwork ordered yesterday that he is immune compromised despite the other factors. We are back to waiting for the Mayo results, which may or may not be definitive. We need to find out what kind of immune deficiency he has and how bad it is. We are quite devastated. We are getting really tired of the roller coaster of good news/bad news. It is truly exhausting to have such emotional highs and lows every day. It's so hard as a parent to not hope for the best, but the associated reality slap that comes when your hopes are dashed is emotionally draining. Your continued prayers and support are well-appreciated and needed. We thank you for all the support you give us.
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In contrast to last Wednesday when he was admitted, Sam is the picture of health. He is happy and smiley all the time, he's eating well, and he has some color back. We often find ourselves asking why he's still in the hospital. They are slow to release him for three key reasons: 1. He needs to have had 5 days of IV antibiotics for the pneumocystis due to the superior absorption of the IV version of Bactrim. 2. He has not yet gained weight. He's eating enough to maintain his cuurent weight, but not enough to gain. Since he's still significantly below the threshold for a baby his age, they want to wait. Malnourishment or undernourishment are key causes of some of the symptopms he's been having. 3. Even if he does go home, he needs the same strict protective isolation here as he's in at the hospital. Until all the SCID tests come back as definitively negative, we have to be very careful. So, he's not coming home today, but there's a chance he'll be discharged tomorrow. Right now we're just working on coordinating his follow-up visits and making sure HealthPartners will cover the Amino-acid based formula he needs to drink now.
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Sammy did well today. The test result for both CMV & HIV have come back negative; we aren't surprised by that. The feeding tube was turned off today and Sam has been taking bottles well. The decision to take the tube out completely may happen as soon as tomorrow. He is doing well on his new hypo-allergenic formula. He seems to be gaining more strength and trying new things. Another bacteria was discovered from his bronchoscopy today, mycoplasma. As a result, he started on another antibiotic. The good news is that his current IV has been in place for over 24 hours without incident. The longer this one stays in place, the less likely it is we have to do a central catheter.
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We have 3 departments concsulting with each other on Sam's case: Pulmonary, Infectious Disease, and Immunology. We feel like we have a great team of doctors and that they are finally figuring things out. The latest: -The infectious disease doctor strongly believes that his current immune-compromised condition was caused by his treatment from his last visit to the hospital; by being malnourished and getting a strong steroid treatment. -The preliminary test for cytomegalovirus has come back negative, but the final test is still pending. -Sam's x-ray of this morning showed improved function of the lungs. -He has been able to take several bottles today along with his feeding tube. They even turned it off for 3 hours today and let him bottle feed. He did well, so they will most likely keep moving in that direction tomorrow. -He lost his 2nd to last IV this morning. He has been poked so much that he has little pricks all over his hands, arms and feet. (along with the bruising that comes with each failed attempt.) If he looses this one, they will either leave it out or put in a central line, depending on what treatments he still needs. Over all he is looking good. He smiles and even tries to laugh. He is capturing the nurses hearts with his bright eyes and flirty smile.
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Well, Esther stayed the night at home last night, and I camped at the hospital with Sammy. He did very well, and he's starting to get some appetite back.
By far the worst part of the day was at about 7:30 am the morning shift nurse came on to check vitals and noticed Sam's IV was occluded and had dumped a couple hours' worth of solution and antibiotic into Sam's arm, not his vein. His whole arm was swollen around his tape, and was very tight. It looked excruciating, but he did not complain. I was irritated that the night nurse either did not notice it, or decided not to do anything about it at the end of her shift. I'd like to assume the former.
The problem, though, is the fact that every (and I mean every) useful vein has been tapped and has subsequently occluded and/or collapsed. We're being told he needs to be on IV antibiotics for 10 more days, so there's a possibility of having to install a PICC line or some other semi-permanent peripheral line. Yuck. This poor kid has been stuck so much lately. Last night on the third IV attempt he didn't even flinch. I will take that as an answered prayer.
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The implications of the pneumocystis diagnosis have been weighing on us today, with all the worst-case scenarios playing out in our heads.
In 99% of the pneumocystis cases there is an underlying severe combined immune deficiency (SCID) that requires treatment. That could range from HIV (which has been ruled out) to hyper eosiniphilia or cytomegalovirus.
In 1% of cases though, the best-case scenario is that an aggressive steroid treatment combined with a sever milk protein allergy and malnutrition creates a temporary weakness of the immune system and causes the T-cells to stop functioning properly. Fortunately, this matches Sam's history and may be the only root cause of the pneumocystis. Meaning, he is very allergic to milk protein, and needs a hypoallergenic diet until he grows up a bit at least.
There is still a chance that this is not the cause and he is severely immunocompromised. For now, they are treating this as a SCID and he is in strict protective isolation to protect him from any other illnesses that could set us back again. The test results to rule out the other SCIDs are in the works now at Mayo, and will take at least until next Friday, possibly Monday. So, we are here for at least another week.
We're trying to focus our positive thoughts and prayers on that best-case scenario, though. His treatment is going well; he's still being tube-fed and is regularly taking a couple ounces of hypo-allergenic formula to top off.
Good night, and thank you for your support and prayers!
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One of the rounding clinicians came in last night and informed us that his lung tissue sampled positive for Pneumocystis. http://kidshealth.org/parent/infections/lung/pneumocystis.htmlPneumocystis is obviously not the concerning part of the diagnosis, what's of concern is the underlying lack of immune system defense that allows a common fungus to act the way it does in Sam's body. He started on another PCP-specific antibiotic and an aggressive steroid treatment. We are anxiously awaiting yet more test results to help us understand the underlying immunodeficiency and he we can treat it. None of the options sound very good to us. We appreciate the prayers and support from all of you. More later...
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The bronchoscopy and Upper GI scope both went relatively well. They decided to add a feeding tube to the equation as his failure to thrive recently is just as concerning as his pulmonary issues. Both surgeons took sample tissue from their scopes for additional testing. The GI surgeon said that the passageway from Sam's stomach to upper intestine was smaller than he expected, which may be contributing to his diminished appetite and failure to grow. We should be getting some definitive results from the tests on Friday. They did not have to put him in the ICU, rather he is in the Intermediate Care Unit (IMC) with a dedicated nurse for the time being.
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Sam was panting tonight, apparently really laboring to breathe. It was 3AM and he was not sleeping, just working on inflating and deflating those little lungs. We called our pediatrician's emergency nurse line. When she heard him breathing she told me to "hang up the phone and call 911". Even as we got him ready to rush off to the hospital in the middle of the night and gave him a blessing he was smiling and cooing at us. The bronchoscopy is scheduled for 4:30PM Thursday.
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During the past two weeks we've noticed occasional wheezing and some labored breathing. Esther has said Sam looks a little gray, though I haven't noticed it. She took him in for his 4-week follow up with Dr. Wheeler the pulmonologist on Wednesday and due to the symptoms, he ordered a CT ( Computed Tomography) scan. The results were not good. We find ourselves with still diminished lung capacity and still significant growth of infiltrates in the lungs. They are still talking about reflux or aspiration of food, but as Dr. Wheeler has said, "Nothing is ever easy with Sam." He is ordering a Bronchoscopy, Upper GI scope and lung biopsy for later this week.
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 I can't believe we are all back at home! The 4 doctors consulting on his case all agreed that he was looking well, and since he had been off oxygen for 24hrs was okay to come home. The pulmonologist said he didn't want Sam catching anything else in the hospital, so after stocking up on nebulizers, steroids and antibiotics, he was finally released! The nursing team of Children's Hospital's 7th floor is the best we've ever seen. They were courteous, caring, attentive and inciteful. They offered sound advice and frank feedback, even when it was hard to hear. We know that several of them were heaven-sent and arrived just when we needed them. They were indeed angels unaware. We are so happy to be ending this hospital blog. We can't do so without expressing our humble gratitude to all of you. To the house cleaners, the kid watchers, the caring phone callers, the soup/casserole/carrot cake makers, the care basket deliverers and the grocery shoppers. To Esther's family (who all about hopped on a plane Thrusday when they heard of the bad turn Sam had taken) and to the sister who did end up coming. To Steve's family who dropped their lives to be there for anything we needed. To all who sacrificed time with their own families to make sure we were comfortable. To those who took on shuttling and feeding and playing with our kids. To the shoulders we cried on, and those who cried with us, as well as those who listened and empathized. Especially for the prayers and positive thoughts sent our way. We were overwhelmed with phone calls and door bells and emails and foodstuffs (mostly General Mills!). We are more than humbled by your sacrifice. We feel so very blessed and loved. For the burdens you truly lifted we are indebted to you. You have done more than take a bad experience and make it better, but you have taught our children what a great blessing family and friends can be, and that charity is the pure love of Christ. -----------------------------------------------------------
These pictures were all taken Saturday when Sammy was feeling better...
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The swallow study will now be done in clinic sometime next week. The pulmonologist really wants Sam out of the hospital before he catches something else. Arrangements are being made for them to come home tonight! Sent via BlackBerry by AT&T
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Sam had a great night off of oxygen. He looks great again today.
All the doctors have agreed that he should be able to return home today.
Steps are being taken in that direction as well! Yeah!!!
His swallow study is still pending, but if that comes back negative then we should be able to leave.
He will have several follow up visits in the next few weeks and will need to continue a nebulizer for the rest of virus season...
Thanks again for all your help, thoughts and prayers. We couldn't have made it through this without you all.
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Sam is still doing well off of oxygen. He has had 3 doctors consulting about him this morning and I'm waiting to hear from pulmonary.
His swallow study is rescheduled for tomorrow morning, and I'm waiting to hear when we will go down for his chest x-ray today.
His coloring looks so good and he continues to be a very happy baby!
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Sam is still doing well off of oxygen. He has had 3 doctors consulting about him this morning and I'm waiting to hear from pulmonary.
His swallow study is reschedueled for tomorrow morning, and I'm waiting to hear when we will go down for his chest x-ray today.
His coloring looks so good and he continues to be a very happy baby!
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Sam is looking even better this morning - the oxygen was disconnected this morning, and last I heard, he has been breathing air on his own for over an hour, maintaining safe blood-oxygen saturation levels. One cord down, two to go. Rumor has it we'll be able to get rid of the IV later today as well. One of the doctors came in this morning and said he'd need to stay a few more days to finish the antibiotics (I'm sure HealthPartners would love that!), and Esther insisted we could finish that up at home. It's looking REALLY good for a Tuesday discharge. On the docket for today: xray at 1:00, hoping for some signs of improvement there. Swallow study thereafter to look for any ingestive abnormalities. More on test results later...
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Dr. Wheeler (pulmonary specialist) came in today and cautioned us about our surging optimism. He made a good point: the patient presents well and obviously feels better, but the xray and test results still indicate there is a problem. The xray he said is way outside the range of "normal" baby pneumonia. Since the test results are only ruling things out, we have no clear answer on what is going on. The bronchoscopy is still very much on the table, if not while still hospitalized, maybe in the following weeks in the clinic if a cause is still not clear. He indicated they'll run at least 10 tests from the bronch samples. Also a possibility is a lung biopsy. Long story short, something more complicated and less obvious is still going on. They plan to do a swallow study before we're discharged to see if the food goes down the right path, and if there's any reflux. We are not talking about discharge yet, and since he's still on oxygen (70%), we're still at least a couple of days away...
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Well, according to this morning's xray there was little (if any) improvement since Thursday. But, as we've heard, the chart is less important than the patient. Sam the xray film is very sick, but Sam the baby is having a very good day. Spirits are very high as he has really bounced back from a bad morning. He has been alert when he's awake, coughing less, needing less oxygen, and sleeping soundly. In fact, for the first time in over a week, Esther left the hospital for a couple of hours, and he took a bottle from dad! It's been a long time since he's done that. We are cautiously optimistic at this point that he really is making strides in fighting this off. Whether it's clinically attributable to the Azithromycin or the steroid, I know that the prayers have lightened our load.
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Dr. Wheeler said the xray from this morning shows a very slight improvement, and feels more comfortable with continuing the steroid treatment than proceeding with a traumatic bronchoscopy. So, for now we are just holding tight, looking for signs if improvement. If he's better by monday, there's a good chance he'll go home early next week. Sent via BlackBerry by AT&T
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He slept fairly well, and we got a good xray. Waiting on radiologist interpretation as well as consult from the pulmonologist as to whether to proceed with bronchoscopy. Our layman's interpretation of the film is no change. Third round of azithromycin and second round of steroid given. Still waiting on some test results. No food since 4am, so he is grumpy.
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The outpouring of help, food and general support from our family and friends has been staggering. We can't keep up with the phone calls offering support. The fridge is full, the house is clean, the kids are happy, and we are humbled. Thank you. I truly can't imagine how we would have been able to make it through without you.
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Sam started out well, then struggled a bit in the late morning. The clinic doc came in today while he was down a bit, and our request for a pulmonologist was entertained. We spent a good portion of the day making calls to get advice and request consults. Both Jim Sidman and Bruce Ferrara from Children's Surgery International (CSI) helped the cause move forward. Dr. Wheeler (pulmonologist) consulted on the case today, and we really made some good strides from his visit. He prescribed a dose of steroids to try and get the inflammation and irritation down. Sam was SO alert and fun tonight - for almost two hours he was smiling; almost laughing, and very aware and lucid. His IV was not flushing tonight, it was bent inside him, and he got his 3rd IV tonight, this time in his right foot. Tomorrow will be a decisive day. Dr. Wheeler has provisionally scheduled a bronchoscopy for 8AM, and will proceed if the morning's xray does not reveal an improvement.
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We spent a lot of time today working on getting the right people involved in Sam's care plan, calling in favors from our friends at CSI and leveraging some of the best nurses on the face of the earth. It was, again, a day of high highs and low lows. Our clinic doc always comes in the morning when he's doing well; then when he struggles later in the day it's just Esther and the nurses pushing him through. Today Sam and his mom hosted multiple new doctors and Sam's blood was distributed to labs for testing, trying to determine the cause of the pneumonia and why it was not getting better. Today we installed IV number two, this one in his left foot.
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Wednesday was when things really took an unexpected turn for the worse. After some concern over his labored breathing pattern and severe coughing bouts, another xray revealed that the pneumonia had actually spread to both lungs since beginning treatment. As the antibiotics were not improving the situation, the pneumonia was either viral or a more severe bacterial infection. Until now, we'd really felt that any day now we'd be heading home, well on our way to recovery at home. The events of today really dealt a severe emotional blow to both of us. Esther had not left the hospital since arriving Saturday and was really looking forward to some time with the kids and a good night's sleep. I arrived at the hospital after the kids were in bed and we had a good cry together. Dr. Amanda came in and we had a lengthy conversation about next steps and how we ensure that progress is made and Sam gets appropriate attention. It was time to step it up. We gave Sam another blessing tonight, and we had a great experience together.
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