Geographic Tongue, Aquagenic Skin Wrinkling, and Other Oddities in CF
- CFAA
- Oct 9
- 3 min read
Updated: Nov 9
There are lots of little weird things in cystic fibrosis that you eventually stumble upon, here are some BUT please leave comments if you have noticed or experienced other unmentioned oddities with Cystic Fibrosis.
Salivary Gland Obstruction in the Young CF Child
One of the possible effects of cystic fibrosis is salivary gland obstruction. We noticed that our first CF infant would not eat dry solid food. He would put it in his mouth, to be sure, but then it would come out. And it came out about as dry as it went in! This was very troubling to us, as it meant that he was not getting enough food to sustain his caloric needs. We discovered that he was suffering from obstruction of his salivary glands. From then on, when we gave him solid food, we made sure that it was moistened. He became a great fan of all types of gravies and sauces. With sufficient moisture content in the food, he was able to eat solid food and gain weight. If your very young CF child has a problem eating solid food, try moistening it. This is something you may not hear from your CF doctors, because they do not consider it a major symptom of CF--but it becomes a major symptom when it prevents the child from enjoying food, which is a barrier to weight gain.
Pathophysiology of cystic fibrosis with emphasis on salivary gland involvement: https://pubmed.ncbi.nlm.nih.gov/2442229/
Here's three more to add to your collection:
1) Geographic Tongue. Some CF persons develop strange raised circles on their tongue, almost looking like crop circles. No, the aliens haven't invaded your tongue; this is called "geographic tongue," and can be a marker of a nutrient deficiency or of excess inflammation, both of which CF persons may have. In our family's experience, our first child with CF developed geographic tongue as a baby. Though we have no idea if this can be generalized, when we added to his regimen a health food drink designed to provide mineral micro-nutrients, the geographic tongue disappeared and has not recurred. Levels of zinc, magnesium, and other minerals may be depressed in CF persons, and developing a slight mineral deficiency is common.
2) Aquagenic Skin Wrinkling: If you have kids with CF, you know that if you put them in the bath or the pool, they come out with intense skin wrinkling on the palms and inner fingers (and soles of the feet). The wrinkling may be so intense that a layer of skin may in fact slough off as a result. Scientists have now determined that this is in fact due to CFTR mutation, though they still do not completely understand the mechanisms at work. While we can just laugh at the wrinkling, the skin sloughing is another matter. You may wish to moisturize the palms and soles if your child will be soaking in water for a long time, or you may wish to limit the time they do soak.
3) Finger Clubbing: Over time, the fingertips of CF persons become more bulbous. Interestingly, after a lung transplant, this finger clubbing goes away. Scientists still do not understand the exact mechanism of finger clubbing, but some have speculated that in severe lung disease, the body releases a circulating vasodilator of unknown identity that is causing the clubbing.
4) Salty Skin: One of the "oldest" known oddities of CF. Recognized as far back as 15th Century. People with CF excrete abnormally high levels of salt in their sweat, making their skin taste salty.
5) Nasal Polyps & Chronic Sinusitis: These are growths inside the nasal passages and sinuses that are common in people with CF, likely due to chronic congestion and infection.
6) Male Infertility: Many men with CF have congenital bilateral absence of the vas deferens (CBAVD), meaning they are born without the tubes that carry sperm, leading to infertility.
7) Pancreatitis: Pancreatic duct blockages can lead to pancreatitis, an inflammatory process that can cause severe abdominal pain, vomiting, and diarrhea.
8) Cystic Fibrosis-related Diabetes (CFRD): Damage to the pancreas can lead to a form of diabetes known as cystic fibrosis-related diabetes mellitus (CFRD).
9) Malnutrition: Thick mucus in the digestive tract and the lack of pancreatic enzymes to help with digestion can put people with CF at risk for malnutrition.
10) Osteopenia and Osteoporosis: The inability to absorb nutrients in the digestive tract can lead to conditions that make bones too thin.
11) Pregnancy Complications: CF can affect the digestive tract and cause poor nutrition, which can increase the risk of pregnancy complications, including preterm birth.
12) Meconium ileus (MI) : in infants and distal intestinal obstruction syndrome (DIOS) in older individuals are serious intestinal obstructions associated with cystic fibrosis (CF). These obstructions result from the abnormal function of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) protein.
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