Hey girls, Angie asked me to post a little something about TEF which is the medical condition our newest little Sapphire is dealing with. I asked Josh to explain it all to me and I will do my best to interpret it as best I can. I am also going to post an explanation from a medical journal website that you all are welcome to read through, but it can be a little confusing so I'll try to explain it a bit first. TEF or tracheoesophageal fistula is a birth defect that develops very early on in pregnancy. It is where the trachea (windpipe) and the esophagus (the tube that food goes down into our stomachs) do not develop and separate properly. A connection fuses somewhere between the trachea and esophagus. Consequently, when the baby tries to eat, the food spills from the esophagus into the trachea and then, sometimes, into the lungs, causing aspiration. It will make the baby choke and struggle to breath. It is a pretty serious condition because the baby cannot feed until it is resolved. This is why Katie would have had surgery so quickly yesterday. During surgery, the doctors go in and tie off the fistula (connecting tube) between the trachea and esophagus. Incidentally, the TEF is likely why Liz went into preterm labor. The baby is not able to swallow the amniotic fluid adequately during pregnancy and there develops an excess of fluid in the uterus, causing preterm labor. There are actually several different types of TEF and sometimes it is necessary for the doctors to reattach pieces of the esophagus that have separated from each other. I have posted a diagram below of the different types of TEF. The picture above with the baby is the most common (87% of cases) and is most likely what Katie has. But I don't know for sure. The first tube in the pics is the trachea that goes into the lungs and the second tube is the esophagus. You can see how it connects to the trachea in some pictures and how it is gapped and would need to be reconnected to itself in several pictures.
The good news about TEF is that it can usually be surgically resolved, which it sounds like is the case with Katie. The danger after surgery is acid reflux problems that can cause scarring and ulcers in the little baby. Also, feeding can continue to be difficult because of the challenge of contracting esophageal tissues needed for swallowing after surgery. In some, not all,(approximately 50%) of cases of TEF... the condition can be linked to other birth defects or other physical conditions, usually cardiac disorders. Sometimes it can be linked to birth defects involving vertebra, anus, renal anomalies, or limb anomalies (radial dysplasia). Some of these would have been obvious immediately and can be ruled out already. I'm sure the doctors are looking for any of these possible links in Katie and we just need to be praying that she is perfectly healthy other than the TEF. We also need to be praying for quick recovery for her and that she does not struggle with acid reflux problems or swallowing problems. I hope this info. helps a little. I have posted an article below that explains the condition more medically if you are interested. I'm sure it will fill in any gaps I have missed as well. Love you girls!
WHAT IS TEF?
At the beginning of a pregnancy, the trachea ("windpipe") and the esophagus ("foodpipe") are one single tube, called the primitive foregut*. Between the 23 rd and the 28th day of pregnancy, a time when many women are not even sure they are pregnant, this single tube divides into two tubes – the trachea, which takes the air you breathe to the lungs, and the esophagus, which takes food, water, and saliva to the stomach. If something should ever go wrong with this division, as it does in approximately one out of every 3,500 – 4,000 births, the baby is born with a medical condition known as esophageal atresia and/or tracheoesophageal fistula. The anomalies are thought to arise from 1) a defective division of the primitive foregut into the trachea and esophagus, 2) defective growth of the entodermal* cells leading to atresia, or 3) incomplete fusion of the lateral side walls of the foregut during separation of the trachea from the foregut causing a tracheoesophageal fistula.
Atresia means an absence or closure of an orifice or tubular organ. In this case, the esophagus has a gap between its upper and lower segments, so that nothing can pass through, not even saliva. The fistula is a connection between the esophagus and trachea, which, depending on its position, either lets food or acidic stomach juices into the lungs. When a fetus has esophageal atresia, the inability to swallow and eliminate amniotic fluid may result in polyhydramnios.* This is the first sign of possible atresia at some level in the upper gastrointestinal tract and may lead to premature birth.
Many babies born with this defect have other medical problems – all associated with organs that were being formed during the same period at the beginning of the pregnancy. At least 25% of these babies are premature, and 25% have additional critical problems such as congential heart disease, Down's Syndrome*, hydronephrosis*, duodenal atresia*, and tracheomalacia*. Ten percent of the babies have an imperforate anus*, and one-half have vertebral or skeletal anomalies. Researchers found that it was not uncommon to have vertebral (V), anal (A), tracheo-esophageal (TE), and/or radial limb or renal (kidney) (R) anomalies, which was abbreviated as VATER syndrome (or VATER association) by Quan and Smith in 1973. This syndrome is also currently being termed as VACTERL, the C referring to cardiovascular problems, and the L to limb defects, because of the high percentage of children with VATER syndrome born with these latter anomalies.
How is this anomaly discovered?
In type A atresia, both esophageal segments are blind pouches, and neither connected to the trachea. It maybe termed as pure esophageal atresia and may be considered either short or long gap, depending on the space between the segments. An infant born with pure esophageal atresia may appear normal at birth, but as they swallow, secretions fill the esophageal pouch, causing an overflow into the oropharynx*, and the infant will drool excessively. When the infant is fed, vomiting and respiratory distress follow (aspiration). Continual suctioning temporarily relieves these symptoms. Both the excessive secretions and drooling in a newborn strongly suggest esophageal atresia.
In type E (or H-type tracheoesophageal fistula without atresia) the fistula may occur anywhere between the level of the cricoid cartilage* and the mid-esophagus. However, the fistula is usually higher in the trachea than in the esophagus. A fistula such as this may be as small as a pinpoint. Symptoms that may signal this H-type fistula are repeated episodes of lung inflammation (pneumonitis), infection of the lungs or respiratory system (pulmonary infection), and swollen abdomen (abdominal distention). When a child with this disorder drinks, he coughs, chokes, and becomes cyanotic (bluish discoloration of the skin and mucous membranes). The reasons for this are that excessive mucous builds up in the esophagus, producing abdominal distention. This type of tracheoesophageal fistula may be overlooked, since such a child may appear normal at birth. It is possible for diagnosis to be delayed as long as a year.
In types B and D, the upper portion of the esophagus opens into the trachea, a life-threatening condition since an infant with this anomaly can aspirate saliva or food into the trachea. Both type B (proximal fistula and type D (fistula to both segments) cause immediate inhalation of saliva into the trachea airway and bacterial pneumonitis*.
The most common tracheoesophageal fistula is Type C, which includes esophageal atresia. The upper section of the esophagus ends in a blind pouch, and the lower section ascends from the stomach and connects with the trachea by a short fistulous tract. A newborn with tracheoesophageal fistula with esophageal atresia appears to swallow normally, but soon after swallowing coughs, struggles, becomes cyanotic (skin turns blue in color), and stops breathing since he is inhaling aspirating) fluid returning from the blind pouch of the esophagus through his nose and mouth. In this infant stomach distention may cause respiratory distress. Also air and gastric contents (bile and gastric acid secretions) may reflux through the fistula into the trachea, resulting in chemical pneumonitis*.
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