Home of the Four Trans

Justin is really small. I mean reeeaaaal small for his age. He’s eighteen months, and I feel like he’s been a baby forever. And, no, I’m not just saying that because I see him everyday. Loi and I measure him (length and weight wise) often.

He hasn’t grown spectacularly well. His weight fluctuates up and down. He is now at a constant 18 pounds. As for his height? He’s about 29 inches. He’s a full head shorter than other kids his age. He is not on the charts for weight or height, as in he’s below the 5th percentile.

I wouldn’t worry so much about this if he were following his “own” curve, growing at his own pace. But, for a couple of times, he fell off his own curve, and this got me worrying.

Through the medical and personal accounts that I’ve read, CHD patients are expected to be smaller for their age. Through my experience, now I see why. CHD patients don’t normally eat that well because they often tire before eating enough. They’re gastro-intestinal system is not as efficient in absorbing food and distributing the nutrients and calories because they’re heart isn’t beating at its optimal. The calories that do get absorbed are mostly allocated to the functioning of the heart (which works harder than a normal heart), or the brain. So, with the little calories that they do consume, the majority goes to their heart or brain, leaving little else for the development of other parts of they’re body.

So, Justin’s small-ness can be attributed to nutrition, or the lack of.

What makes me wonder if it’s just Justin’s diet that is causing his inability to thrive is this: when I read about other CHD children, or ask their parents about their development, I find that although the children are small, they’re still within the normal curve. Justin is waaay below it.

Let me also point out that Loi and I are not particularly tall people. I’m at a borderline 5′0, and Loi is 5′7. With this observation, doctors are also quick to point out that Justin’s size may have to do with his parents’ petiteness.

Ok, fine. But, he’s NOT really growing.

This leaves me to wonder, with the inside of his body as messed up as it is (stomach on the wrong side, malrotated intestines, no spleen, hanging pancreas, large liver), not to mention his heart defects, I wonder what other systems in his body are affected, too. Most particularly, his endocrine system. As complex as the human body is, where every system works in accordance with the others, I wonder, how his heterotaxy affects his endocrine system.

I can’t find information anywhere about this. I’ve asked this question on the online support group board. These children vary in sizes and complexity, so it’s hard to compare.

My next resort, after speaking to his cardiologist and pediatrician, of course, would have to be testing. As in, poking and blood drawing. This is another can of worms I must think before opening.

Justin is happy. Should I just leave well enough alone?

Justin and I met with ID to discuss his aplenia and a fever plan.

Meeting with the ID doc helped clarify my main concern of what to do when Justin develops a fever. We discussed how to get through the ER without having to wait if Justin develops a fever (by announcing that he has NO SPLEEN), what to expect at the ER (blood must be drawn for a blood culture and an antibiotic injection must be given to protect him over the next 17 hours as the culture grows to show if he has an infection). After giving him the antibiotic injection, we would be sent home to wait for the results. If there is an infection, he would need to be admitted to the hospital for treatment.

Of course, the ER is secondary to the doctor’s office. If we’re able to get to the doctor’s office, we should do so first. The same procedure would be done at the doctor’s office.

He urged us not to treat Justin with Tylenol or Motrin to reduce his fevers. Justin’s ability to produce a fever is like a smoke alarm telling us there’s something going on in his body. He does not want us to turn this off with medicine. It is crucial that he is seen right away when he does develop a fever so that the doctor can take the necessary precautions. It is also important to watch how his fevers develop. The fever should always improve, and not spike upward (let’s say from 101-104) in the course of 4 days. If it does, we need to report it to either him or his pediatrician.

I asked if the fever itself could harm Justin. He answered no. There are some children who develop febrile seizures with high fevers, but that does not necessarily damage a child and his brain.

Because Justin is up-to-date with his immunizations (minus the Hib booster and MMR), and is on a prophylactic amoxicillin, he is protected from the main forms of encapsulated bacteria, mainly, pneumococcal, meningcoccal, and influenza. However, not all. Prevnar (vaccine for pneumococcal) protects him from only 5 different strains.

The ID doc made a point to say that 99% of the time asplenic children develop fevers, it will not be a result of an infection. But, for that 1%, we must be careful all of the time. He stresses that once the child has an infection, and we do not take the necessary measures to care for him, he can go downhill very quickly.

This doctor is much more aggressive in his treating method than the other (not ID) doctors that I’ve encountered. As I listened to him, I understood why. During our meeting, he explained that he has been in the business for 17 years, and he’s encountered 2 asplenic children who have contracted an infection…one didn’t make it through. His perspective is that unless a doctor’s experienced how it’s like to treat a child, and watch a child with no spleen fight a bacterial infection…and see one not make it…they will not understand why it is important to be aggressive from the get-go with treating these children.

With that said, he also acknowledged how “intelligent” our pediatrician is, and will understand if he has his own method of treatment. Although the ID doc’s method is “extreme,” according to him, he’d rather be safe than sorry. But, each doctor will have his own opinion.

We also discussed Justin’s immunization schedule which he will dictate and send a copy to our pediatrician. He explained that Justin will be on amoxicillin until he’s 5 years old. Between the ages of 5-17, his immune system will still be weaker than normal, but strong enough to fight off the bacteria, so, he does not recommend the amoxicillin during those years. If he does develop a fever, then we are to take him to the doctors or hospital right away. The course of treatment at that time will depend on how he is from a clinical standpoint, and we’ll discuss further as we approach that point. By around 17-20 years old, we can worry less, though the risks are still there. His body, by that time will be a little stronger (not taking into account his heart condition).

He gave me a copy of his “bible” that he refers to on treating asplenic children (photocopied pages from his reference book) that he told me to give to every single one of Justin’s doctors, and all those who are treating him. He asked me to bring it to the hospital as well.

I am grateful for having met this doctor. He had great compassion and imparted me with more knowledge than I had before meeting him. I hope by sharing it with all of you, you’ll gain a better understanding, and encourage you to ask more questions.

From the beginning, doctor after doctor have shied away from having discussions with us about Justin’s asplenia. I would ask, and they would tell me that it’s not a big deal, essentially. According to them, the immunizations and amoxicillin are enough to protect him.

Perhaps it is part of my nature to always want to know more so that I can prepare. I was on the brink of annoyance with the brush off, but really pushed the issue and was referred over to infectious diseases by Justin’s cardiologist.

Will all this information and fever plan completely shield my child from harm’s way? No. Does it allow me a better understanding of how to care for and protect my child in the best of my ability? Yes.

I’m a mom, and I’m just trying to do my job.

We ended the meeting with the ID doctor telling me this: “The best way to protect your child is to have a good mom. And, you’re a good mom, a terrific mom. Keep him up to date with the immunizations and keep up with the amoxicillin. He’ll be fine.”

I don’t like being called “super mom” or being told how great of a mom I am (because I’m not. Let’s face it, we all have our moments). But, from him, I’ll take that remark as a compliment because it is coming from a medical professional who acknowledges the role of a parent in her child’s care, even if she’s not a doctor. Thanks, Doc.