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Tuesday, April 9, 2013

a long-winded update.

About 6 weeks old, before he was diagnosed with a heart defect. Photo taken by his Auntie Amie.

It occurred to me that maybe it’s a good time to write an update on Elliott’s health. I haven’t shared too much in detail here about every little thing to note in Elliott’s health but it’s been about 4 months since he was discharged from the children’s hospital after his heart surgery. Developmentally he’s been astounding me on a daily basis but that hasn’t meant he hasn’t progressed in other areas too! We’ve seen a lot of specialists in those four months, a lot. If I had to go back and recant them all I am not sure I could do it without looking at a list.

Elliott’s need for a variety of specialists comes from him being born with DiGeorge syndrome, also known as Velo-Facial-Cardio syndrome but most accurately described as 22q deletion. You can read a full scientific account of DiGeorge syndrome, especially since some of my usage of certain scientific terms may not be accurate. The fact is that Elliott’s Digeorge is what has caused his heart defect and that there is a chance that Taylor or I is a carrier of this deletion. It is also widely possible a mutation in the gene happened randomly during conception. I have been told by my genetic specialist (that is monitoring my most current pregnancy; we did not do genetic testing with Elliott) that Taylor and I could opt into genetic testing for the two of us to see if one of us is the carrier, but there would be a small chance that such a test may not be 100% accurate. It may have been something we looked into later on, prior to us planning the next pregnancy, but, as you see, we got quite a surprise with little one #2. In any case, if one of us is truly carrying the deletion, the chances of us having a baby with this deletion is 50%. If one of us is not a carrier, the chances that our second baby is born with the syndrome is drastically reduced.  

We’ve been told by several doctors and specialists that Elliott does not have a “severe” case of the syndrome, and so far many of the more common complications or “symptoms” of DiGeorge haven’t shown up in Elliott (with the exception of the cardiovascular problems). This is very fortunate, many kids with DiGeorge are born with other big issues like immune system problems or cleft palates. In spite of knowing that his case is “less severe,” it still requires that we are proactive about Elliott’s health and visiting all of these specialists with some regularity, to monitor possible issues as Elliott grows.

Basic updates: 

Has gained on average a little over 1 pound a month since being released from the hospital. Elliott’s release weight was a little over 8 pounds and now he over 13! He’s also grown a few inches. He was clearly still in newborn-sized clothing when he was released and now has nearly outgrown 0-3 month and 3 month sizes. 3-6 month sizes are starting to fit well! (Although, sizing is so different between brands…. A few things still fit him in a newborn size, and some 6 month items fit too!) Elliott has been getting a once-a-week visit from the home health care nurse to check his weight and monitor his health, which has been a mind-freeing thing for my husband and I, to know we can count on someone if we have a concern before we make a trip to the pediatrician or urgent care. 

Elliott was released with 5 prescriptions (6 if you count an as-needed prescription to Acetaminophen), but is now down to 2 (a blood pressure medication and an anti-seizure medication) and has no need for his pain medication since about 4 weeks post-hospital. We are hoping to get him off everything by is 10thmonth birthday!

The before he was discharged after his heart surgery repair.
Heart:

Obviously, Elliott’s heart was the big hitter. If you’re a newer reader, here’s a quick recap: Elliott was diagnosed with Truncus Ateriosis type 1 (his own variant on type 1) in October when he was a teeny tiny eight weeks old. Essentially Elliott was born with a single arterial vessel instead of the pulmonary artery and the aortic arch, and had a hole between his right and left ventricles. Elliott's repair included patching that hole and creating a conduit to act as the pulmonary artery. Elliott's recent visit to the cardiologist was positive, but they had a bit of a rough read on his echo-cardiogram. They thought there was a possible tiny jet of blood from his left ventricle to his right where the hole was patched, but due to Elliott being a wiggly infant they also thought the read could have been bad. They'll be doing another echo cardiogram in a few months to make sure.

The good news is Elliott was taken off one of his heart medications called Lasix and is doing well. They are keeping him on his blood pressure medicine until they know for certain there isn't an issue with the patch.


Immune system:
Immune system disorders are high on the list of issues kids with DiGeorge face. We recently visited the Immunologist and she mentioned they had tested Elliott's T-cells during both stays in the hospital and determined he was within the normal range. Being that he has not exhibited many other symptoms of fungal or bacterial infections, has only one cold given to him by his mama that knocked him on his butt (pre-heart surgery), has braved two other colds post surgery just fine without a problem, and exhibited signs of an active immune system after getting immunizations (a fever that lasted less than 24 hours) the immunologist currently feels he does not have an immune deficiency. We were given a broad list of things to look out for in the interim, but we won't be visiting that clinic for another 6 months.


Speech/swallowing

Readers who have been around a while might remember Elliott's feeding tube. In the hospital a speech therapist came to visit while Elliott bottle fed and had some concerns about his swallowing. After doing an x-ray test in radiology they found he was silently aspirating, i.e. allowing some of his feeds to go into his airway without noticing/coughing it back up. It may have been because he was on medication that can impair his movement or cognition, I felt. For this reason Elliott was on a strict tube-fed only diet until we got another visit to radiology well after he had healed from surgery. The test indicated that he was still silently aspirating on water-thin liquids, but he was handling a slightly thickened formula with a little rice cereal! So we are still on that diet until we get our regular speech therapy visits in place. The delay is one part exhaustion/an overwhelming schedule/laziness on our end, one part difficulty getting the speech therapy appointment set up on the specialists end. There is a lot of appointments to handle and the thought of weekly appointments in speech therapy on top of our weekly visits from Elliott's teacher (more on that below) and the home health care nurse and whatever other specialist appointment we have that week is enough to make my head spin.

It is an important thing that we really need to make a priority soon. Kids with DiGeorge can have palatal problems and speech problems, and the same muscles that cause issues with swallowing can cause issues with speech. Elliott doesn't have a cleft palate, obviously, but there is a small chance he could have a less noticeable palate problem. We've been told by a few doctors and specialists that he likely doesn't, but at some point we'll be visiting with a plastic surgeon just to make sure.


About 5 months old, 4 weeks after hospital discharge.
Learning/development:

Kids diagnosed with DiGeorge have a 90% chance of having some learning disability, so my husband and I have already taken the steps to make sure he is on the right track to develop the way he should. I have touched on his development here in his monthly updates, and he is a tad behind in a few areas, but he is never not progressing in an area. The State of California has sponsored Elliott in a developmental program where a teacher visits our home once a week to work on a variety of things, fine and gross muscle development, social development, and cognition. This week she was extremely pleased with the progress he's made in his muscle movement since last week. He is heart baby who was sedated in a hospital for a length of time so it is only natural for him to be a bit behind. Cognitively Elliott is doing very well and doesn't seem to have many problems, but we prefer to be proactive to make sure he is reaching his full potential!

Physical growth:

DiGeorge kids are smaller in stature as a rule of the syndrome. Elliott has smaller parents (I am 5' 3'', Taylor is 5' 6") and heart babies tend to be smaller too. It's no wonder Elliott is in the 4th percentile when compared to other babies in the rest of the country! He's maintained his spot in the 4th, however, and hasn't waivered below it by constantly gaining weight and growing, so I am pretty pleased by this. And has it happens, I've looked up his spot on a growth chart for DiGeorge kids, and he is in the 25th! Not too bad, I think. There is nothing wrong with being little and cute.

Neurology:

The first night Elliott was in the hospital, they called to tell me they thought Elliott was seizuring. He was doing some rhythmic tongue thrusting and it was concerning to them, so they put him on anti-seizure medication and performed an EKG. They did not spot any seizures, but they did see some abnormal spikes so they still felt he was susceptible to them. The EKG after his heart surgery found that he had had 4 mini-seizures the day of surgery, which is not abnormal for a person who had been on the heart-lung machine for bypass surgery. The following days showed more of the abnormal spikes but no seizures. Currently Taylor and I are on a mission to have another EKG and get him off his anti-seizure meds, which we feel is unnecessary. I am pretty convinced that the "tongue thrusting" they saw was a combination of a hungry baby on heavy sedatives with a ventilator tube shoved down his throat. I have seen the same thing happen on occasion when he falls asleep with a bottle in his mouth and I take the nipple out. His sucking reflex kicks in for just a second or two.

Whew! Is that everything? I am not sure my tired brain can wrap that all up neatly in a bow, and let's face it, we can't wrap our life into a neat little bow either. Things are challenging, but Elliott is turning into the sweetest, happest nearly-8-months-old little boy I have ever met. Kid, you are worth all of this craziness and hoopla. You are worth every minute.



About 6 months here?






 

3 comments:

  1. Oh this little boy breaks my heart/makes my day every time you post. He's such a brave little thing, I hope he keeps right on getting better and better xxx

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    1. Thank you Charlotte! It's nice to know we have support across the pond! Hope you are doing well! :D

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  2. His progress is just astounding! That is a crap-ton of medical nonsense to have to go through when you're so tiny, but that little boy has come through it all with a smile on his face. I'm so proud of him!

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