Dim The Lights, The Results Are In

If you know my kids, you know they have relatively weak immune systems (It is my little superheroes' kryptonite)

   

 

Ryder has been seeing an allergist and immunologist at Children’s since January 2011.  We first took him because he was ALWAYS sick, specifically with ear infections and chest congestion. After blood work, we discovered (I think to both my surprise and to my doctor’s surprise) that he had a Compliment Protein Deficiency.  What is that you ask? Well, here is a brief and broad description:

The complement system is part of the innate immune system. The complement system plays an important part in defense against pyogenic organisms. It promotes the inflammatory response, eliminates pathogens, and enhances the immune response. Deficiencies in the complement cascade can lead to infection. In addition to playing an important role in host defense against infection, the complement system is a mediator in both the pathogenesis and prevention of immune complex diseases, such as systemic lupus erythematosus (SLE). These findings underscore the duality of the complement system. It has a protective effect when functioning in moderation against pathogens; at the same time, the inflammation promoted by complement activation can result in cellular damage when not kept in check. The complement cascade consists of 3 separate pathways that converge in a final common pathway. The pathways include the classical pathway (C1qrs, C2, C4), the alternative pathway (C3, factor B, properdin), and the lectin pathway (mannan-binding lectin [MBL]). The classical pathway is triggered by interaction of the Fc portion of an antibody (immunoglobulin [Ig] M, IgG1, IgG2, IgG3) or C-reactive protein with C1q. (Chaganti)

Ryder showed a deficiency in the Classical Pathways of his Complement Proteins.  I honestly am not sure how deficient; I do remember Dr. Hains saying he had less than half of what was normal.  Basically, a deficiency in this area can lead to multiple infections that are recurrent, and can even include the development of Lupus and high susceptibility to meningitis depending on what areas are deficient.  When Ryder was retested to see where he was specifically deficient (there are apparently 9 divisions of the Classical Pathways), we thankfully had good news.  His test came back with much higher numbers, which put him in the very low range of what could be considered normal concerning his Complement Protein. 

How did this change? I have no clue (prayer maybe?), especially since I understood it to be something his body was simply missing, but whatever it was, I was glad it was a much better outcome than we originally thought.  He was placed on a year and a half regimen of a prophylactic antibiotic, just to help him fight the infections his immune system was not strong enough to fight by itself.  A year and a half later, I am thrilled to report we have just recently (March) come off of that antibiotic and are hoping he does well enough to stay off (there is a chance he may have to start them again in the fall if he starts getting sick again, since that is the time in the year when sickness increases).  

If this wasn’t fun enough, we added Sawyer in the mix.  (This actually brings me to the relevancy of why I posted this today.) We decided to have his immune system tested as well since he has had so many ear infections and sinus infections this past year, not to mention Ryder’s low immune system. Yesterday (May 22, 2012), we went for the lab results. He had overall a healthy report (like Ryder) but (also like Ryder) had some low or slightly abnormal results (again, nothing too serious).  He also tested in the low average range for his complement proteins.  Apparently this was no huge concern, but was very similar to Ryder’s results the second time he was tested.  It does mean he is more likely to develop ear infections (um, no kidding) and other upper respiratory infections than most kids (the average child in daycare, according to Dr. Hains, makes about 7 trips to the doctor due to basic illnesses in a year.  Kids with Ryder and Sawyer’s immune deficiencies make typically around 15 trips—Great, roll of eyes!)  Somehow, his immune system is just weaker than most.

The other area where Sawyer’s numbers were not normal concerned his pneumococcal titers. (Again, I am no doctor; this is my layman’s terms for all of this. Brad and Allison may laugh at my awkward description of all of this.) Apparently, a titer tells how much immunity someone has built up after getting a vaccine.  Sawyer had no protection against pneumococcal bacteraemia (presence of bacteria in the blood) despite having had his required Prevnar13 vaccines (to date).  Now, this could be because he hasn’t finished all of his vaccines, but Dr. Hains thought it was concerning since he has had at least two vaccines and still has no antibodies. This bacteria, she explained, can be a major cause of ear infections and sinus infections (well that explains a lot!) and Sawyer has no protection against fighting off this when he comes in contact with it. He isn’t making any antibodies against it. When he gets older (over 2) he can have the Pneumovax 23 vaccine (I think that is right).  This will offer him a different type of protection (I honestly don’t know what is different; I just know what I was told). I don’t think it is given to most kids, but since he isn’t responding to the other vaccine, he will need it. I found this when researching this:

“Evaluation of their [their meaning subjects studied concerning the pneumococcal vaccine]response to pneumococcal vaccine can be used as a marker to determine their ability to make antibody specific responses to multiple infectious agents. The failure to make a specific antibody response may be one factor in the susceptibility of these patients to recurrent infections.” (pub.med.gov)

SO, Sawyer, like Ryder will also have to take a year dose of prophylactic antibiotics to help his low immune system (what are the odds?) beginning in August provided he can remain ear infection free until then (currently Sawyer is without an infection and hasn’t had one in about 3 ½ weeks, which is the longest stint we have gone sans ear infection since October.)  

ON TOP OF ALL OF THIS, there was major concern over his weight, which is high 18- low 19 lbs and hasn’t really changed since February.  (The big concern here is that he hasn’t gained, not just that he is simply 19 lbs.) This I have been a bit worried over, especially when I discovered his weight was in the 4^th percentile at his 1 year checkup. I was immediately sent to see a nutritionist; she placed him on two cups of Carnation Instant Breakfast a day, as well as his regular meals and suggested he stop nursing (um, no.)  I honestly think she suggested this because she couldn’t understand why I would still get up to nurse him at night and thought he wanted it because he was hungry. (Just a note, it was the PA and nutritionist, not his main doctor who suggested he stop nursing. His doctor said obviously human milk is made for human babies over cow's milk, but did state that after a year the benefit isn't anything more than simply being a comfort and a healthy drink, not magic potion. And since we don't live in a famine, stopping at 1 year is reasonable.) Sawyer doesn’t really nurse much in the daytime because I am at work, etc.  Therefore, our nursing happens mostly in the evening or at night.  It is tiring at times but I wasn’t complaining. Half the reason he wants it is comfort, and I am okay with that-he is still a baby. And just for good measure, I DO NOT starve him so that he will nurse.  I offer him whole milk all day, he just barely sips it. He has never wanted to drink a bottle, despite what was in it—breast milk or formula or cow milk (so good luck with the Carnation Instant Breakfast)—and despite who gave it to him—daycare workers, me, my mom, other family. That has driven me to keep nursing him, especially since he doesn’t take in the recommended 24 ounces of milk a day. However, I don’t see how taking away extra calories will help. He just isn’t a vigorous eater.  Anyway, to help his appetite, (he does eat okay, he just doesn’t always seem interested) the doctor put him on an appetite stimulant to try and encourage him to eat more.  (Honestly, that surprised me a bit.) But maybe this will help him eat more and get his weight up some. 

SO, there you go, a 1200 word account of my craziness that is my life as a mom.  I guess I will just expect that my next baby will be the same way. Maybe I should start saving for her (okay, or his) doctor’s visits now!

Sources Referenced:

Chaganti, Krisha, MD. "Complement Deficiencies." Medscape Reference. WebMD LLC. July 9 2009. Web. 23 May 2012. 

Zora, JA, Silk, HJ and Tinkleman, DG. Annals of Allergy. pub.med.gov Apr. 70.4 (1993). 283-88.
          Web.23 May 2012.