Our 2016 Send a Kid to Camp Campaign is Live!

As many of you know, the NFED kids have a great time at Kays’ Kids Camp during conference! They create meaningful friendships, play,  have fun, and get to enjoy moments with others without having to feel different from their peers.

IMG_4884As many of you also know, it may be a difficult time financially to send your children to camp because of the $125 fee.  It may even be a deciding factor if you will be able to attend Family Conference at all. That is where our motto, “Supporting You. Supporting Each Other.” comes into play. We are so blessed to have many contributors who have given to this campaign in the past, and we would love to have support from you and others by making a contribution AND spreading the word! We are all here for each other.

This year during Ectodermal Dysplasias Awareness Month, we want to try to reach out to people in our NFED circle and beyond who may know someone affected by ectodermal dysplasias or who just genuinely care about children having friendships and moments that build self-esteem and embrace their uniqueness!

Click on this link https://www.crowdrise.com/sendakidtocampfund and make a donation. It all adds up. No gift is too small! We need as many people as possible to share this link with friends and family, especially during Awareness Month. What better way to show what we do then by sharing our video and photos of kids having a wonderful time during Kays’ Kids Camp.

When we reach our goal of $10,000, one extremely generous contributor will match our goal 100% with a $10,000 gift. The possibilities are so exciting right now! Join us!

Teen Group at FC CO

Thank you!

DONATE NOW

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BE HEARD. INSPIRE OTHERS. BE YOURSELF.

Speak Up for Ectodermal Dysplasias

Speak Up for Ectodermal Dysplasias

Wow! Can you believe it is February already?  We all know what happens in February – Valentine’s Day, Ground Hog Day, the Super Bowl.

But more importantly, it is Ectodermal Dysplasias Awareness Month, #EDAM2016.  This month, we are striving to raise the positive awareness of this condition which affects an estimated 2 in 10,000 births.

The 2016 theme is “Be Heard. Inspire Others. Be Yourself.”  We encourage you to always NFED-Profile-FebBE YOURSELF and do not let ectodermal dysplasias define you.  We have a memorable month planned but we still need one key ingredient: YOU!

We need you to Speak Up for Ectodermal Dysplasias! We have the tools and resources to make your participation easy and free.

Please check out the Ectodermal Dysplasias Awareness Month Page on the NFED website for details.  Here are some ways you can BE HEARD while you INSPIRE OTHERS:

  • Watch and share our brand new video!
  • Share your story. Tell your story to anyone who will listen.
  • Like, share, retweet, and pin our posts.
  • Download our Social Media Guide for sample posts you can do all month.
  • Write blog posts about the ectodermal dysplasias.
  • Hang fliers around your neighborhood.
  • Talk to dentists around your area.
  • Learn and share the facts of ectodermal dysplasias.
  • Hold fundraisers or an awareness both.
  • Wear your NFED gear.
  • Attend or support events.
  • Create your own video and post online.
  • Follow the DIY Calendar and for a different idea for every day.
  • Send press releases to your local media.
  • Read more on our Awareness Month page.

Most importantly, always smile, show the joy in your heart, and BE YOURSELF! Let’s speak up and tell the world about ectodermal dysplasias – together.

Be sure and post your activities on our Facebook page or email us.

If you have any questions, email or call me at 618-566-6875.

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Treating Common Nasal Problems in Ectodermal Dyspalsias

The following is an excerpt from the NFED’s “A Guide to the Eyes, Ears, Nose and Throat in the Ectodermal Dysplasias.”

The common nasal problems of ectodermal dysplasias revolve around the management of nasal dryness and are usually managed without surgery. Surgery may be may be of some benefit if there is an associated internal deformity such as a deviated nasal septum or external nasal deformity resulting in a cosmetic change in the appearance of the outer nose.

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A saddle nose deformity is often seen in  ectodermal dysplasias.

 

A saddle-nose deformity (or “ski-slope” deformity of the outer nose) is often seen in ectodermal dysplasias. Its correction probably will not improve breathing. If done for cosmetic reasons, however, it should be done by an experienced surgeon. Cosmetic and functional concerns (breathing freely) of the nose are managed together when both exist.

What Can Be Done About Crusty Nasal Secretions?

Nasal crusting is caused by abnormal mucous production or excessive accumulation. The mucus is thick and does not drain normally. Large crusts may form that can interfere with breathing. Some form of treatment to improve the flow of mucous and provide improved humidification to the inside of the nose can be very helpful.

Preventing Nasal Crusting

Humidification is critical to prevent and treat nasal crusting. Humidification can be provided by commercial saline (salt water) drops or a spray such as Alkalot, Ayr, NaSal, or Ocean. A simple preparation may be made daily by mixing one-half teaspoon salt in one pint of water; one eyedropper full of such a solution can be placed into the nose to soften the crust and repeated as often as necessary.

Humidification in the bedroom while sleeping is also helpful. In addition to humidification, nasal irrigation with salt water under pressure using a squeeze bottle or bulb syringe several times a day is helpful in clearing crusts and thick mucus from the nose.

Although both humidification (misting) and irrigation (forceful washing) can involve the use of salt water, the irrigation offers the advantage of a forceful stream, which is more effective at washing sticky, thick secretions from the nasal cavities.

Decongestant sprays and drops such as Afrin, Dristan, and Neosynephrine may be helpful but should not be used for more than three days in a row and only with a physician’s supervision and approval. Routine antibiotic treatment does not prevent crusting but may be necessary with an acute upper respiratory illness.

The nasal crusting may be accompanied by a foul odor. Again, humidification, gentle nasal suction, or nose blowing may be helpful. If the quality of the mucus changes, consultation with a physician is appropriate.

Are Nosebleeds A Problem In Ectodermal Dysplasias?

Nosebleeds may be an occasional or a chronic problem for people affected by ectodermal dysplasias. The bleeding is usually the result of chronic dryness.

Several measures can be taken to stop the bleeding.

  1. The person should be placed in a sitting position and asked to tuck their chin to their chest so the blood doesn’t run down the back of their throat and cause nausea.
  2. Then, the fleshy part of the front of the nostrils should be tightly pinched shut between the thumb and first finger for ten minutes by the clock.
  3. If bleeding continues, consultation with a physician is urgently necessary.

Preventing Nosebleeds

Because the nosebleeds may be recurrent, preventive measures are appropriate.

  1. Avoid picking one’s nose or placing instruments (Q-tips) inside the nose is key.
  2. Avoid forceful noseblowing.
  3. Humidify the air, moistening the nose with one of the previously mentioned saline nose drops, and application of a sterile lubricating ointment such as DuraTears, Lacrilube, or Vaseline may be helpful.
  4. Apply the ointment liberally just inside the nostril with a finger tip several times daily.

Crusting often causes nosebleeds and prevention is just as important as treatment. A physician should evaluate recurring bleeding.

Information on treatment is suggested in this blog as a guide to what might be expected or recommended by physicians who are consulted by our families. It is important for you, the reader, to understand that the statements are generalizations about the many ectodermal dysplasias  and no absolute recommendations are made or implied. These recommendations therefore should not take the place of the advice of a qualified medical professional. Trust the physicians you consult to do the right things, but also be an advocate for yourself or your child. Your doctor may appreciate a copy of this booklet to increase his or her awareness of the  syndromes. The mention of any “brand name” drug is presented as an example only and must not be interpreted as endorsement of any specific product or brand or company by the National Foundation for Ectodermal Dysplasias (NFED) or its Scientific Advisory Council.

 

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Smiling About Snot and Spit

Breast Implants

Smiling About Snot and Spit

How Little Liam is Doing 11 Months After Receiving EDI200 in Clinical Trial

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Liam was the 9th baby to participate in the Newborn XLHED Clinical Trial.

Liam is a happy little guy whose smile lights up the room – and your heart. Like most 11-month-olds, he drools constantly and often has a runny nose. Unlike most kiddos his age, that saliva and mucous is a welcome surprise for his parents.

Liam is affected by x-linked hypohidrotic ectodermal dysplasia (XLHED) which can affect, among other things, the nasal glands, causing nasal concretions, and the salivary glands, causing dry mouth and swallowing difficulty. When he was just days old, Liam participated in the Newborn XLHED Clinical Trial and received five doses of EDI200, a lab created protein that is missing in people affected by his condition.

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Parents Beth and Steve are glad they had the opportunity to participate in the Clinical Trial and help advance XLHED research.

 

It is too soon to know if the EDI200 did indeed have a positive impact for Liam. But his parents, Steve  and Beth, are grateful that Liam’s glands seem to be working correctly. Who knew we could be so excited about snot and spit?!

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A dental assistant looks in Liam’s mouth to see his dental ridge while Steve watches. They were collecting data for the Extension Study.

The family was at St. Louis Children’s Hospital this week for Liam’s one-year checkup as a part of the Extension Study for the Clinical Trial. We, the NFED, announced earlier this month that the Clinical Trial didn’t achieve its expected outcomes, and therefore was not continuing at this time. While it is certainly disappointing, we are grateful that Edimer will collect data for 10 years on the 10 babies  through this Extension Study.

Liam’s dad, Steve, shares our hope for what this data could tell us.

“It’s going to be four to five years before we will know about the number of teeth these babies will have,” Steve said. “We might yet learn that EDI200 did impact their dentition. We must stay the course. We are not giving up, for Liam’s sake and for his kids and the next generation.

“It’s what hasn’t happened in the Clinical Trial that is just important as what has. Like in the animal studies, there has not been one negative side effect for the babies who received EDI200. That’s important and advanced research,” Steve said.

Beth said, “I admit that I was disappointed when I first learned the news about the Clinical Trial. I was thinking, ‘Why are we stopping now? What does this mean for Liam and his future?’ But it doesn’t change how we will raise Liam.

“In our case, we are better off having participated since Liam does get runny noses and has saliva. His skin has been normal. He has not had any respiratory issues that are common in XLHED, and his nails are good. He’s tall. He’s underweight for his age but we know that’s typical of the condition. He has not had any teeth erupt yet but the dentist thinks he will have six baby teeth. Given what I lived through with XLHED, my greatest hope is that Liam will develop teeth. These are all things which have us hopeful for the future,” Beth said.

Both parents say their participation was always about the bigger picture: a research breakthrough for XLHED.

“We had the opportunity to help advance research in a health way. We embraced that opportunity,” Steve said.

Beth agreed, “We will continue to advocate and support efforts from families, the NFED and Edimer to keep this research at the forefront.

Steve said, “We love that Liam is a part of what ultimately will be a solution. It’s pretty cool that one day, he could say to his kids, ‘I did that for you.’”

LiamThumbsUp

Liam gives one thumb up!

 

 

Other Articles Related to the Clinical Trial:

Critical Update: Newborn XLHED Clinical Trial

XHLED Family in EU Needed for “Trial to Cure” Project

 

Breast Implants

Editor’s note:

Lack of breast development is common in certain syndromes of ectodermal dysplasia. We wanted to share a few stories of affected women who experienced lack of breast growth either in one or both breasts. Two of the women interviewed chose to get breast implants, while the other did not. Names and identifying details have been omitted to protect privacy.

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Story 1

I had not been aware that a lack of breast development was a possible symptom of ectodermal dysplasia. When I was about 13, I noticed that my right breast was not developing. For many months, I chalked it up to them just not developing at the same rate. After a while, it couldn’t be denied that the right breast was simply not coming.

If I recall correctly, when it was discussed with my pediatrician, he suggested that it may or may not be part of my syndrome (I have HED). At the time, I believed it was just my luck to have another rare deformity. My everyday life went on as it always had, but, I was very upset about it. I didn’t let on too much to the emotional distress it caused.

Eventually, when I could no longer hide it with big shirts, my mother started sewing pads into the right side of my bras. It got to the point where I usually slept in them. It was strange; for most of my adolescence, I felt like I had to put myself together every day. Get up, put in my dentures, put on the “prosthetic” bra. I would even dream about it. To this day, I will still dream about leaving the house without dentures or all my hair has fallen out. The silver lining was that I had a doctor’s note that excused me from swimming for both 9th and 10th grade!

When I was about 16 years old, (I’m 42 now) I went to see a plastic surgeon. He had me put my hands together and he felt my sides. He said I was done growing and he could do surgery. We went with silicone, cause, what did I know. My parents’ insurance covered 10%. I believe this was due to the fact that it was a congenital defect. They paid out of pocket the rest. At the time, I think it was $3,800.

Surgery was a success. I was thrilled. For the first time, I could purchase bras that weren’t white or nude. They could have lace. I was pretty psyched. About 2-3 years later, I started having a lot of pain. My breasts were also changing shape. This is also at the height of the Dow Corning lawsuit. Everyone was talking about ruptures and women were suffering from all kinds of ailments due to leaking silicone implants.

They thought I was suffering from a rupture. So, I had exploratory surgery. Turns out, it was capsular contracture. The surgeon cleaned the scar tissue and placed them in the same spot. Insurance did cover that procedure. It wasn’t even a year later that I was in pain again. My surgeon, who was the best in my city, tried to tell me it was my menses.

That certainly didn’t explain why I was deformed again. I got another surgeon. He decided that we should take them out, replace them with saline, and place them under the muscle, which would limit the likelihood for the scar capsule to grow again. I believe my insurance covered the removal, but not new implants. He was a newer surgeon, so his fee was $2,000. This surgery was a success. I was about 22 or 23 at the time. For the next few years, things went along well until at age 26, I found a small lump that turned out to be a pocket of blood and tissue. Not sure if that was from all the surgery or not. But, I am told at my mammograms that they don’t see that every day. Story of my life…

The initial surgery was painful to be sure. It probably took a good two weeks to not feel so sore. And wow, nothing prepares you for what it looks like when you take the bandages off. The bruising, swelling… you feel like it will never be right. But, after each implant surgery, with about equal time for recuperation, I was pretty good. I was told by the doctor that it takes about a year to fully heal.

Then, I had kids…my first at 31, second at 33. My breasts got jacked up again. I went back to the surgeon who had done the last procedure. I was completely deformed again. My husband’s insurance paid for certain things regarding removal, but, of course, there was some loophole to get out of paying. It was over $7,000 out of pocket and a 3-4 hour procedure to fix me.

This time, I had them replaced, and evened out. I got a little bigger (those guys always want to make you bigger), but, I guess he was right. It suits me. This was the worst recoup of all my surgeries. I came home with drain tubes and was in a lot of pain. I was sore for weeks and weeks. But, like the other times, they healed and I’m pretty happy with the results.

I had to have follow ups for a year and then I “graduated.” Knock on wood, I won’t have to do it again. Can’t say there is anything I would do differently. I mean, what happened to me is simply luck of the draw. When I had to have this last surgery, I did seek out the opinion of a different surgeon, simply because I had never really done that before. I was in desperation mode the last two times. The first surgeon had a stellar reputation, but I just lucked out with the doc who did the initial replacement. So, I tried to do my due diligence and take my time before this last one. But, when all was said and done, I did go with him anyway. And he was amazing.

Having my implants does a great deal for my confidence. There was no way I wasn’t going to do it. There was also no way I wasn’t going to rectify the capsular contracture either. And I’ll tell ya, you get used to seeing your breasts the way they are. After surgery, each time I was fixed, I’d go back to the surgeon and he’d show me the before pics. They would upset and astound me to see how deformed they were. I have never regretted any of my operations.

Story 2

I had deep suspicions that my lack of breast development was related to ectodermal dysplasia, but really I just thought, because I’m smaller it was more fitting for my body shape to be smaller (though that didn’t explain the nonexistence!) Also, I did a lot of gymnastics growing up. Gymnasts are usually small, right?

I didn’t really get a confirmation of my repressed suspicions until I was 25 years old and was connected to the Women with ED Facebook page. Apparently my mom knew all along though. To be honest, I had never looked that much into ectodermal dysplasia until I turned 25. I was mixed with anger (I think just over having to deal with ectodermal dysplasia in general, even if it is more mild) and a feeling of relief from knowing why.

My lack of development didn’t affect me growing up. I think ignorance is bliss. And I wasn’t very sexually mature either while I was in high school. However, it’s increasingly affected my self-confidence with dating. As far as I know, I was not teased for being flat-chested while I was in school.

I’ve worn a kid’s size bra since college, probably the smallest remotely correctly fitting bra I’ve found. I guess the fact girls are getting more developed earlier has worked in my favor. I’ve tried the padded ones on but I just feel fake and not confident with them even in the dressing room so I’ve never brought one. I mostly did sports bras in high school. Now I find I’m going braless more often. It’s so much more comfortable. But I don’t feel my age physically.

I have considered getting implants. I still do. I haven’t yet for several reasons. First is cost. And I’m upset health insurance won’t cover even part of them. They do for breast cancer patients. Not saying they shouldn’t for us, but it’s not like we choose to have ectodermal dysplasia, or that it doesn’t affect us mentally either. Secondly, having had surgeries and working in the medical field, I know it will hurt. And I know things can go wrong. I’m in a constant debate for the future.

I wonder if getting implants would make me more confident in the dating scene. I’m sure it would help. I feel like I’m stuck in a child’s body. I’ve rarely gotten romantically close with men because of it. Though I have an internal battle (same feeling went for getting my teeth redone) if people don’t like me for me, $#%@ them! So I think that and the fear of the pain stops me. And the money. I could travel to so many cool places with that money!

My advice to other women with ectodermal dysplasia, is that implants are a very real possibility. Think about it. Listen to yourself. Do what you think is best for you and not for society’s idea of a perfect body.

I’m crying writing this. Maybe I should get implants.

Story 3

I am now 58 years old. My HED is mosaic and affects one side of my body so one breast developed, the other didn’t. We had no idea about ectodermal dysplasia at the time.

I would say it had a major impact on my teenage years. I felt like a freak. I only ever told one friend at school. Changing for sports in a communal area was my worst fear. No one ever said anything, so I doubt they noticed.

My first implant was at 17. I am in Australia and my health fund paid for it no problem.

Because there was absolutely no development, it was initially agony. I had a cage over the bed to prevent even a sheet touching me. Any movement sent me through the roof. That didn’t take too long to settle.

When I got home, I was so excited and wanted to show everyone but obviously couldn’t. It was the best feeling to be “normal.”

I had them changed two more times when they went “wrong,” but still have the last one from 1985.

I had three children. They tried to get me to feed on that side to no avail because there really wasn’t a nipple to attach to well. I had no trouble breast feeding on the other side alone though, I guess I looked very lopsided at the time!

My eldest daughter went through the same thing. I thought because I had been there, it would be helpful. But, she was badly affected emotionally, shied away from people and dressed in baggy clothes. She had an implant at 17, too. If it was possible, I would have done it much earlier and changed a few times to help get through the horrible teen years.

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