Sammy

Apologies for the delay in posting - it's been a whirlwind for the last two days trying to get everything ready for Sam. He is now comfortably resting in his own crib. Even though it seems we're at the beginning of this medical maze rather than at the end, it's great to be home together again.

I'll continue to update the blog as we attempt to learn more of Sam's condition and how it will impact him and us going forward.

Once again, the silver lining in this ordeal has been our friends and family who have sacrificed much to help us get through. We are humbled by your love and compassion.

Well, it's been a good couple of days. Sam is taking well to the new antibiotic, and is not fighting the oral prednisone (steroid) too much. He actually had a solid stool today (which doesn't sound like a big deal) but after 6 weeks of diarrhea, that's a big deal. Plus, his blood work from today is the best he's had in 4 days.

We are fairly certain we'll be discharged tomorrow, and we are doing all we can to have his room ready. Without a real diagnosis, we are being very cautious with his home treatment. Here is what we know:

1. This is more than just a milk allergy. He has some mild to moderate immune deficiency.
2. His eosiniphil count is very jumpy. When those get too high, his immune system is compromised. The steroids help keep the eosiniphil count down.
3. His neutrophil count is very jumpy. You need neutrophils to fight infections when they first start.
4. He still has a very dangerous form of pneumonia that requires 3 weeks of treatment.

After that, there's not a lot we do know. We are going to do our best to protect him from school germs and other general nasties until we have more answers. His only outings for the next 6-8 weeks will be to the immunology clinic. Stating that is just as much for mom and dad as it is for the reader. It's been difficult coming to grips with the long-term nature of his illness. We are getting better, and we'll take the victories, no matter how small.

Our hope and prayer is that in due time we will have a diagnosis, or at least a long-term treatment plan and prognosis. This little man has big plans, and we need to keep him healthy!

The lab is checking Sam's counts daily now, watching for reactions to the various medications. The doctors think he is having an adverse reaction to the Pneumocystis antibiotic Bactrim. They've moved him to an oral antibiotic now instead (Mepron). I guess they're hoping that will stabilize his white blood count and eosiniphils. The steroid prednisone is now being administered intravenously instead of orally.

Our discharge date was pushed back again (not that we ever really had one expressed, only implied). It looks like Monday or Tuesday now, assuming we can get Sam to react well and take both the antibiotic and the steroid.

Not surprisingly, our doctors do not all agree on everything. We can understand and even appreciate that, but we get a different picture painted for us every time the door opens.

"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.

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.

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.

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.

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.

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.html

Pneumocystis 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...

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.

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.

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.