Patients often have skin-related questions and wonder what types of things will influence their PC symptoms and what types will not. Please always feel free to connect with us and get the answers you need! We are here to help you gain the understanding and knowledge you need.
Alphabetical list of question topics:
Question from K6a parent: “I had a consult with a doctor today and my son definitely needs spacers and braces. I hadn’t really thought about how it would affect him. Have you or any of your boys had braces? I am a little concerned about the friction it might cause in his mouth. I wasn’t sure if he’d blister.”
Answer from K6a patient: “I had braces and so does my oldest child. He’s had them for almost 2 years now. He also wore spacers. He did not have any PC-related problems at all. For my son, sometimes the insides of his checks get irritated by the braces, but I’m not sure how much that is from PC or just because those braces rub. He loves the wax the orthodontist provides. And I figure, because they provide the wax, my son is not the only one who is bothered by braces rubbing against the inner sides of the mouth. No blistering, though — nothing. I didn’t have any problems either.”
K6a patient 1: “I was unsuccessful at breastfeeding either of my children. It was excruciatingly painful and continued to be for the duration of our experience. I worked with a lactation consultant who assured me that our latch was correct, but the pain never subsided. I had persistent cracking of the nipples…almost blistering and a couple of bouts of mastitis. As you know, life with a newborn is quite chaotic, so I honestly can’t recollect if this thought was on my mind at the time. In retrospect, I’ve often wondered if I was unable to breastfeed because of my PC — if the friction of the nursing was just too much for someone with PC. I, of course, was a bit devastated at the time, because I (and perhaps many others) desire to breastfeed and somehow equate that with being a ‘good mother.’ It was difficult for me to ‘give up’ on it, and I remember mourning it a bit…”
K6a patient 2: “I, too, struggled with breastfeeding. I think I had all of the things you mentioned and I also wondered if it had to do with my PC. In addition, the first two of my three boys have PC, and so, when the first had a hard time sucking, I knew (from my mom telling me about my own time trying to suck as a baby) that he might have trouble. I ended up using bottles that were so squishy and with big openings so that I could squeeze the milk or formula into both of my sons’ mouths because it was painful for them to suck. But still, because I was trying to be a ‘good’ mom, and since they couldn’t suck well, I pumped my breast milk for months for my first baby. My breasts were a mess with blistering, and sometimes they would bleed so much that I just threw out the milk. With my second baby, I pumped for a much shorter time. My third baby didn’t have PC and was an awesome ‘sucker,’ but even in the hospital my breasts started to blister, and I said ‘forget it, I’ve got three little boys to care for’ and went straight to the formula bottle. I still felt the guilt somewhat because I know about all the benefits of breastfeeding, and I believe them. However, all three of my boys are in the gifted programs at school (OK, yes I’m bragging) so I think they survived just fine with good old formula! Whether or not it was related to PC, I don’t know, but one other thing I figured is if feeding my babies was a dreaded thing because of the awful pain and terrible toll on my skin, and not a bonding, loving thing (which it was with the bottle), then I figured the agony of breastfeeding defeated one of the best benefits of feeding my babies.”
K6a patient 1: “I finally came to the same realization that we were losing out on the part we both needed/wanted the most — the calm, relaxing, joy of bonding during feeding. The tears and frustration — for both of us — were just not worth the potential added nutrition. Our stories are so amazingly similar…I pumped for weeks as well, just praying that both my children got the magical ‘mommy elixir’ that is so important at the beginning of life. We did the SAME thing with my son’s nipples…found the ‘squishiest’ we could find, and enlarged the openings with large needles. What a support we could’ve been to one another during those times! I pray that if a new PC mom or mom to a PCer finds these messages, they can reach out to one of us in a time of need!”
K6a patient 2: “Yes, I sure wish we would have known each other back then. I bet my own mom would have liked the support too as she was trying to feed me! Still, it’s nice to know I wasn’t the only one. Somehow, hearing your story makes me not feel so odd. For the record, to this day, I don’t enjoy ice cream cones because it irritates my tongue to lick. I don’t enjoy suckers either. Unfortunately for my waistline, I find eating ice cream wonderfully satisfying with a spoon!”
K17 patient: “I have also had a terrible time with breastfeeding! My first son with PC never understood how to suck from the breast. After five weeks of terrible pain, fear of being a bad mother, bleeding nipples, and an infected breast (which I had to go to a hospital for and to get the puss out in a surgery), I gave up!!! I didn’t get any help before it was too late. My second child without PC had no problem with sucking — he ate as much as possible. He could eat constantly, but my breasts didn’t like it. I got blisters and cracks from the start. They were bleeding and extremely painful! I tried everything but nothing helped. I got help from the hospital, but even if I told them about PC, it wasn’t helpful. After about five weeks, I gave up and started to pump my breasts, but it hurt too so I couldn’t continue doing it. After about two months, I heard about a moistening creme made especially for your nipples, and when I used it, the cracks and sores became better! I also gave him bottle to drink from and only breastfed him 2 times a day (afternoons and nights). This summer, I stopped breastfeeding him in the day. I have tried to give up the feeding, but he has this problem where he wants to drink something else at night, and he wakes up several times, but I’m too lazy to get up so I give him the breast instead. But I’ve had two mastitis lately, and this morning I woke up with a blister on the nipple! So I have to stop soon…”
K6a patient: “I have breastfed all of my children. In all three cases, I have developed what looks like could be a callous on one of my nipples. I didn’t know if it was related to the issues I was having with breastfeeding (in all 3 children, I’ve had really sore nipples which even cracked and bled) or if it was from PC. When I spoke with lactation consultants, they didn’t really say whether the nipple looked normal or not, but when it comes to sore nipples, I think they come in all different shapes and forms.”
Although there are people with PC who have excessive ear wax, we have learned two things over these past 12 years: (a) The incidence of excessive ear wax is much higher in the general population than it seems to be with those with PC and (b) the ear wax is not the source of the short ear pain experienced by some PC-K6a patients.
“First Bite Syndrome” is the only term we have found that comes close to describing the short ear pain for some PC-K6a children. We believe the pain is related to salivary glands, but have been unable to sustain an in-depth study, partly because there are so few patients, and partly because the condition usually diminishes greatly after about age 16. We often don’t know of the problem from patients, as parents take the children to an ENT or other ear specialist assuming it is ear pain and not identifying it with PC.
Hyperbaric Oxygen Therapy (HBOT) is the medical use of oxygen at a level higher than atmospheric pressure.
K6a patient asked: “I’ve been battling with this painful sore on my right foot for over two weeks now, and I was wondering if you would ask the physicians whether they think Hyperbaric Oxygen Therapy would help heal PC sores/blisters like these.”
PC physicians’ comments:
1. There is no data on HBO therapy for PC.
2. The basis of the HBO therapy is to force oxygen to sores that will not heal over periods of time due to a lack of oxygen to the sore (lack of circulation, etc.).
3. There is no rationale for HBO therapy being effective for PC and none of the physicians felt this would be effective or an option they would recommend.
K16 Patient question: “Would laser hair treatment have an effect on my PC symptoms?”
Physician response: Dr. Hansen — a dermatologist who consults for the IPCRR members — indicated that we have never seen any connection or problem from PC with laser hair removal. Of course, every individual may react differently, but there should be no concerns specifically because of the keratin mutation you carry which causes PC.
K6a patient question: “Will getting a tattoo create any PC complications?”
Physician response, Sancy A. Leachman, MD, PhD Medical Advisor PC Project: “I understand that you have PC, are interested in getting a tattoo, and want to know if there is any contraindication to the tattoo given your condition. This is actually a complicated issue as there have been no reports in the literature from which we can draw, and none of the patients we have enrolled in our registry have reported getting a tattoo (much less the outcome). However, based on our current understanding of the cause of PC, you should probably use care. We know that biopsies performed on PC patients heal fine — maybe slightly slower, but usually without any worse scarring. This suggests that the tattooing process is unlikely to be much different on your skin long-term than on non-PC patients. On the other hand, you should use care in selecting the site of the tattoo, because there are certain places — like the foot, hands, and possibly buttocks/knees — that may not heal as well, and you may be left with a bit of thickening in the area of the tattoo that persists for quite a while, maybe indefinitely. Finally, I do not want to fail to mention that it is very important that, if you choose to get a tattoo, you select a reputable business, because many of these establishments are not hygienic and many innocent people have been infected with untreatable viruses due to exposure during the tattooing process.”