Don’t you love those acronyms? I have come to really appreciate the time they save, the space they save, and that I can safely dodge oodles of mispronunciation. For example, until 2012 those letter sequences meant nothing to me. If someone would have told me years ago how much I would come to know about their meaning I would have laughed. I would have laughed a lot, and then I would have freaked. out. As I write this, it occurs to me that it might look as though I ripped my information from a medical journal. Rest assured, this all came spilling out of my brain and heart. If there was a site or a journal to credit I would. Perhaps some of you parents can relate. If you have a child with acronyms in their medical file – you tend to know stuff. You tend to become an expert about those acronyms, about what they stand for, what procedures are required to treat and diagnose and repair. I can hardly remember what I walk into Target for on any given day, but if someone asks me what TOF means I will promptly spew forth so much information in a matter of seconds that I hardly recognize myself.
Moms of kids with acronyms unite!
We brought home our daughter Grace, from China in May of 2013. You can read all about our miracle girl at our blog. We started our journey to Grace in January of 2012, and it has been quite the adventure. I’m forever in awe of how God knit us together as a family. We went from a family of four with zero “special needs” to a family of 5 with a bunch. There were scary times, scary terms, scary travel with a cyanotic baby, and scary surgery (all kinds of scary) – but for all of the scary we have survived together it’s in the top 5 all time favorite seasons of our life as a family – for sure. I have learned a lot as a mom of a child with multiple congenital defects and I am passionate about sharing what I’ve learned because sometimes acronyms can scare you right out of someone amazing and I cannot imagine my life without our daughter, acronyms and all.
TEF: Tracheo-esophageal Fistula: A congenital (born with) defect where the trachea (breathing tube) and esophagus (food to stomach tube) are connected. A baby born with this defect will get very sick fast since half of what they drink goes to their stomach and the other half the aspirate into their lungs through the trachea.
Grace was born with this, I’m sure it is why she was extremely sick when she was found at approximately one week old. She was immediately hospitalized and had the TEF repair surgery at three weeks old. She also had a raging case of pneumonia among other things. When this necessary and time sensitive repair is done (removing the connection between the trachea and esophagus that are meant to be close but separate) there is inevitable damage to both the trachea and the esophagus. The trachea, which should grow and become more rigid with age is left with a bit of scar tissue at the repair site. This almost always leaves the trachea “floppy” which makes for several challenges and concerns: it’s noisy, its a trap for bacteria and things that shouldn’t be in the trachea, it can easily become irritated, it can (in severe cases) fold in on itself cutting off air supply. Next door, the esophagus is also left with some scar tissue, which can and often does cause stenosis or narrowing of the esophagus. When this happens and you’re on a liquid diet it’s hardly, if ever, noticeable. When you begin to introduce solid food, it’s inevitable that unless you are very carefully taking and thoroughly chewing small bites of food, and following it up with a drink of something, what you swallow will get stuck. It’s not choking (choking happens in the trachea) it’s that feeling that there’s too much in there and it will either have to make it’s way down – or come back up.
The second result of the esophagus repair is how it affects the chain of nerves that make the esophagus squeeze food to the stomach in a matter of seconds (peristalsis). When that series of nerves is interrupted – the esophagus cannot function the way it should. It might have some function – but truly gravity is doing most of the work to bring the food into the stomach. We knew Grace had the repair. We did not know until after we were home all that the repair had created. It is very manageable in her case. We closely monitor what she eats and as she’s growing she is learning to drink between swallows and chew well. There is a ton of progress in her eating and swallowing since we brought her home.
TOF: Tetralogy of Fallot: This is one of the most common congenital heart defects (CHD). There are four defects that are accounted for in TOF, but two are necessary to be repaired and the others you live with. One of the two that must be repaired is a Ventricle Septal Defect (VSD) which is an opening between the ventricles. This allows oxygenated and deoxygenated blood to mix before entering the body, resulting in insufficient oxygen saturation throughout the body. The other repaired defect is a poorly or non functioning pulmonary valve.
When we met Grace in China her oxygen saturation on a good day was in the 70’s. When she was upset, terrified, or crying they would drop into the 50’s. At 19 months old she was 16 pounds and had blueish gray finger tips and toes and sometimes lips, she was cyanotic. Her overall color had a grayness to it.
Her body had adapted to her condition to survive in this way but the older you get and the bigger you get, the more oxygen you need, the harder your heart works. Her heart was enlarged and when she laid down there was a noticeable difference in her rib cage from where her heart was larger than it should be. The doctors in China were ready and waiting for months to operate but she was chronically sick with pneumonia. She had a chance once and spent over a week in the hospital preparing for surgery but the noise in her trachea was concerning and they refused to operate. I’m so glad they did. We traveled to and from China with a portable oxygen concentrator (POC) that helped some when she was especially cyanotic.
We returned home from China on May 19, 2013 and on June 21, 2013 we got to be her family when she went from blue to pink and her oxygen saturation read 99%.
It was a glorious glorious day and we praise God for how He brought about healing in her tiny body at just the right time. Today I’m happy to say that in the almost 10 months since we have had Grace as our own, she has only had three colds (after 19 months of chronic pneumonia) and her cardiac function is great. It’s not even a part of our daily life any longer, other than her “battle scar” reminding us that she is a walking miracle.
V: Vertebral anomaly (congenital scoliosis for example)
A: Anal (anal atresia or some other type of malformation in that area)
C: Cardiac (congenital heart defect)
T: Trachea (tracheoesophageal fistula)
E: Esophagus (esophageal atresia)
R: Renal (kidney abnormalities)
L: Limb (defects in legs, arms, hands, feet)
When you receive medical information with a referral for an adoption, most agencies if not all agencies suggest (and some require) that you submit that information to “International Adoption Clinic” where doctors are familiar with looking through referrals carefully and giving you a clearer picture of what needs are diagnosed. When we received our response from the international adoption clinic in Minneapolis we were very well educated in what to expect from the cardiac defect, and the TEF repair was explained. What I didn’t know anything about was the suggestion of “VACTERL association”. Even in all of my researching of medical conditions while we were paper-pregnant I had never come across that term.
VACTERL is a collection of congenital defects that often are associated. For example, after returning home we met with a genetic counselor who explained that while there’s nothing really to be done for VACTERL association – it’s a map and fits a fairly common pattern for other potential anomalies that may not have presented in a child yet. If a child presents with two or more congenital defects it is often recommended that the other systems within the VACTERL association be examined as well. One
doesn’t have to have every letter accounted for to be considered someone with VACTERL association but more than 2 suggests a possibility.
At the time of referral, I didn’t think much of it. I researched it, I was prepared to have our docs look into it upon returning home and I brought it up to every specialist we have seen. All agree she fits the pattern – especially when she was diagnosed with congenital scoliosis due to a hemi-vertebrae (a uniquely formed vertebrae which sets everything above and below it a bit curvy. She doesn’t have anal atresia, no obvious limb defects, but she was born with hydronephrosis (one kidney larger than the other) which has since resolved. She may have a tethered spinal cord but shows no symptoms currently (which is a post for another day… perhaps April).
Sometimes it’s comforting, in an odd, way to have a pattern that makes some sense, you know? It has been noted, and docs nod and say – “yes she fits, so we will look into other things to make sure there’s nothing we are missing”.
…Good heavens… What else could be missing?? Sometimes, I have to look back and remind myself she was created, I believe, this way on purpose.
“For you created my inmost being; you knit me together in my mother’s womb. I praise you because I am fearfully and wonderfully made; your works are wonderful. I know that full well. My frame was not hidden from you when I was made in the secret place; when I was woven together in the depths of the earth your eyes saw my unformed body. All the days ordained for me were written in your book before one of them came to be. How precious to me are your thoughts, God! How vast is the sum of them! Were I to count them they would outnumber the grains of the sand. When I awake I am still with you.” — Psalm 139:13-18
What do you tell a child born with multiple defects why they were created “like that.” The same thing you tell a child who was created with one – or none. You tell them Psalm 139:13-18. You tell them they were fearfully and wonderfully knit together by a God who has ordained each one of their days, and that nothing is hidden from God even though it’s sometimes hidden from us, and that nothing surprises God, even though it might surprise us. We brought a child home with known congenital defects – and we brought that same child home with even more unknown congenital defects. We were surprised by some, but God wasn’t surprised by a single one. We either believe that or we don’t, and I believe it as much as I will believe that the sun will rise tomorrow. I don’t know why a good God creates some people with no “defects” and others with several, but I believe He is good. I have seen how the hard things in Grace’s life have not just been redeemed but have been used for good. I have seen time and time again the miracles that occur in people with congenital defects, the healing that doesn’t make sense to the experts, the children who out live the odds, and that is precious and rare to behold. The reality is, we live in a world where bad things happen that we can’t explain, can’t prevent, and through no fault of our own – exist. It’s what we do in those moments, and how we live with those things. That is what defines us – not the “defect”.
The “defect” never defines us. The “defects” never define the child. The acronyms, the medicines, the scars, the prognosis – must not define our children and it must not define our life.
What if we would have known it all ahead of time? If you read our story to Grace you’ll know it was far too late to say no. By God’s grace, and through no effort of ours, we were matched with the one child we already so loved. She could have had two heads and we would have brought her home. What if before that we would have said “no…defects are too scary…acronyms are too hard…she will need surgery(s)? I can’t watch a child suffer like that…” What if we had said no to it all at the very beginning. I shudder to think of all we would have missed out on. I spend my days with the most beautifully, spunky, energetic, loving, hilariously sassy, independent, brilliant, adorable Chinese girl with more “defects” than I can count on one hand.
I wouldn’t change one thing.