Need Support and/or Information on Arhinia AKA Arrhinia??

Looking for support and information on dealing with arhinia AKA arrhinia or related conditions....  I formed a group on Facebook called Arrhinia and other Rare Conditions involving the Nose at https://www.facebook.com/groups/536799623068278/  You must be a member of Facebook to join the group.  It is a closed group.

You can also send me a friend request at Facebook to see what it is like to be an adult with arhinia AKA arrhinia....  I live a pretty ordinary life. :)  Here's a link to my Facebook profile at https://www.facebook.com/kristilbranstetter

You can also follow me on Twitter....  Just look for Kristi Branstetter there!

Discovered Photos of me in a Medical Journal Article and Book without my Consent

Watch out if you have a rare condition like me!

I had found an abstract of a journal article about my condition, arhinia, in October 2010.  I noticed that my surgeon from France was one of the authors.  I emailed one of the authors to see if my case was part of the cases.  The reply that it was.  I finally got around to purchasing the article on January 1, 2012.  Not only was my case part of the article but there were photos of me.  I never consented to anything appearing in any journal article.  The authors stated that written permission had been given and it was not.  I was both shocked and horrified! 

The author told me that he was publishing a book about my former French plastic surgeon and that I should read it.  I finally got around to purchasing it this month.  Had to spend over $90 to buy both volumes!  I discovered my photos in the book!  Not only that my case is to be part of a museum in France.  WTF!

I was recently asked to submit my story and photos for my employer.  (Yeah I have been working since August 2011!)  I have to sign permission slips for my photo to appear on the website.  It is too bad that the medical profession is above that!

My mission is to stop the practice of sharing medical information among medical professionals whether are retired or not!  What has happened to me is NOT right especially in this day and age of HIPPA!

A Poem

The following poem was sent to me via email in the summer of 1998.

If the world was blind,
maybe then we'd see
the true side of beauty,
deep within you and me.

No one could judge us
by our outer looks,
like the fancy covers
on expensive books.

We'd see with our hearts,
and not just our eyes,
and what we discover
might be a surprise.

There would be no need
to try to impress,
with the way we look,
or the way that we dress.

The only thing that would matter
is the way we would feel,
not phony or cheap,
but truthful and real.

(anonymous)

Reconstructive Surgery

Disclaimer

You MUST always discuss the risks of any reconstructive surgery with your surgeon doing the reconstructive surgery on you and/or your family member.

I have been thru many reconstructive surgeries over the years. I don't like to call myself an expert because I am not. But I've seen and experienced many things that many others have not.

Parents

I have met many parents of children with craniofacial anomalies thru the years. I am amazed at how much or how little they know about reconstructive surgeries. I think that it is great that there is so much information out there for parents. There was next to nothing when I was born.

When it comes to risks, I believe that parents know very little or information that is incorrect. Parents don't ask their healthcare providers enough questions about risks. I know the risks when it comes to having surgery. I know someone who has gotten the wrong blood during surgery and nearly died from it. I know three people who have died from complications from surgery. I take a risk every time I have surgery just like anyone else.

I think that parents are not fully prepared when it comes to seeing a child after surgery. But then again, what really prepares anyone for the sight of child whose features have changed so radically? My dad has told me how he nearly passed out after seeing me in ICU after my October 1978 surgery. My mom told me that she didn't recognize anything but my fingernails and eyes after that surgery.

I believe that some parents do not have realistic expectations of their children's surgical treatment. Some are very dramatic about their children going thru surgery. My parents were very realistic about their expectations. And I have never seen them get upset outwardly. I know they were nervous though like me.

I bet not many parents have seen the anguish of another's parent's face when something has gone wrong. Until she passed away, my mom can still recall the look on the stepdad of a child whose surgery had gone terribly wrong. That happened in October 1981.

Children, Teenagers, and Adults

I think that children whose parents cope better with the child's craniofacial anomaly and the subsequent reconstructive surgeries do better with the surgeries.

All children are nervous about having surgery. I get rather tired of hearing or reading about adults who share how horrible their experiences were. For many of those adults who go thru such horrible experiences, many opt to go thru more surgery. For one thing, things are so much different today then they were thirty-five years ago. Nowadays, hospitals do a lot to prepare children for surgeries, such as offering pre-op parties and tours of the hospitals. Before the 1970s, hospitals did not do that.

I think many teens and adults go into surgery with the idea that they are going to come out looking so much different. I think that many of them think that they are going to look like Miss America of Mr. America afterwards. Well it is not going to happen. Then they are disappointed afterwards because they had too high expectations. The more surgery one has on a particular area, such as the upper lip, the more scar tissue there will be.

I have not been too disappointed over surgery results. The only times I have been upset are when complications happened, such as when my columella pulled away twice in the 1980s. It was more frustrating than anything.

I notice that many adults want surgery as they get into their twenties, thirties, and later. For me, I am just ending my surgery road and have NO desire to have any more surgery. The only way, I would have anymore would be if I had a problem with a wire trying to work its way out.

Procedures

Not all procedures work all the time.

Cleft Palate Repair

In cleft palate repair surgery, there is a risk of a fistula forming in the palate after surgery. I think that it is a greater risk than everyone thinks. It is a known fact that some children will develop a fistula after surgery, even with all the care that is taken.
When bone grafting is done to the avoelar ridge (gumline), the graft does not always take. It might not take because cadaver bone is used (has a high risk of rejection by body), the age of the person, disobeying postop orders, or reabsorption of the bone by the body for some unknown reason.

Skull Reconstruction Surgery

In skull reconstructive surgery, there are many risks. There is the risk of brain damage (small but possible risk), infection (true with any surgery), and man-made bone or cadaver bone not taking (rejecting). There are two procedures widely used: traditional skull surgery (making an incision across the top of the head from ear to ear and peeling the skin back) and the endoscopic surgery (the use of endoscopy). Traditional is more widely used. Endoscopoc is really limited due to age of child (must be six months or younger).

Midface/Jaw Advancement Surgery

I think that going thru midface/jaw advancement is a once-in-a-lifetime experience. I had mine done when I was fourteen years old. And at age twenty, I was told that I needed done again. Both my parents and I agreed that I was not going thru it again. It had been too hard on all of us the first time. I knew someone who went thru it two times. The second time, her jaws were wired much longer than the first time. To this day, my bite is off one-half inch. It was an elective surgery the second time around for me and I chose not to do it again.

Distraction is used to bring the midface and jaw out without having to use bone grafts. During the procedure, the bones near the ear are broken. Then pins are inserted. For several weeks after the surgery, the pins are turned each day until the desired gap in the bones is reached. Eventually, new bone will grow. After the pins are turned, the device is left in place for several more weeks.

I think distraction used in craniofacial reconstruction is not a good idea. For one thing, the device that people who have it done must wear. Some devices look like a halo. I would not be a good candidate because I like to sleep on my side. Plus, great care must be done everyday to clean the pins that are attached to the device thru the skin. Distraction is not for everyone.

Some craniofacial surgeons still do the traditional jaw advancement, which involves bone grafting. Some surgeons do not wire the jaw shut even though they use bone grafting.

There are pros and cons to all three procedures, just like any surgery.

Anesthesia

I think that very few people think about the risks of going under general anesthesia even in the twenty-first century. I know that I will come out alive but I never know what is going to happen from the time I am put out until I wake up after surgery. Anything can happen.

Pictures

I think it is great for people to share before and after surgery pictures. That's why I have my pictures on the web.

Today's parents have advantages that my parents never had: The ability to see before and after pictures.

However, I don't agree with sharing pictures of children in the first day or two days recovering from surgery. I would not feel comfortable showing a child of mine pictures of a child in ICU with tubes. Besides, how many young children are allowed into ICU? You will never see any pictures of me within the first few days after surgery. You will only read my words about the experiences.

I am not against reconstructive surgery at all. I just think people need to look at each procedure very carefully. I just believe that people should not take any reconstructive surgery lightly.

Reconstructive Surgery for People with Down Symdrome

Please note: The opinions expressed here are solely mine. I would be more than happy to debate the issue in email. But I will not remove this page from my website.

Craniofacial reconstructive surgery is usually done to improve function, such as feeding, breathing, and seeing. Craniofacial reconstructive surgery is usually not done for cosmetic reasons alone. There are a handful of craniofacial surgeons doing reconstructive surgery on children with Down Syndrome. The reconstructive surgery for people with Down Syndrome is a controversial subject. I oppose reconstructive surgery for people with Down Syndrome because I find it totally UNNECESSARY.

"Skin Deep"

On Sunday, February 21, 1999, I watched a program called "Skin Deep" on the Discovery Channel. The program was about the parents deciding on reconstructive surgery for their son who has Down Syndrome.

A Brief Synopsis of the Program

The show began with the parents preparing for their son's surgery at their home in Paola, KS. During the show, the film crew interviewed a group of people with Down Syndrome and their parents about having reconstructive surgery. None of the people they spoke with would have the surgery. Then the parents and little boy drove down to Dallas TX for the surgery. Dr. Kenneth Salyer and his craniofacial team at Medical City did the surgery. (By the way, the surgery is not covered by insurance and costs approximately $17,000.) The parents and the child met with Dr. Salyer and his team the day before the surgery. The parents viewed computer images of their son before and after. The father really took in the imagery. The next morning the little boy had the surgery. They showed clips of Dr. Salyer and the team doing the surgery. The blow came when the parents went to recovery and saw their son for the first time after the surgery. The child had bandages covering most of his face and had blood on some of the dressings. The father was in shock. Then they showed the parents with the little boy in a hospital room later in the day. Dr. Salyer came to visit the parents and the little boy and spoke about the pain as if it were minimal. A couple of days after surgery, the father commented on how he was focusing on the computer images. About three months after the surgery, the parents and the little boy went back to Dallas for a followup appointment. There had been some scarring of the boy's eyelids so Dr. Salyer and his team did a quick procedure to reduce the scarring. The program ended with the family picnicking with family members and friends.

My Opinions of the Show

I found the program to be disturbing for several reasons:

1. The Parents

It was quite obvious that the parents were thinking of no one but themselves. It was quite clear that they did not accept their child's disability. The father kept wanting the child's face to look normal. They would go to any lengths. I like what what one of my friends said, "I feel bad for this sweet little boy having to grow up with them as his parents." Someone else said, "self-centered yahoos." Another friend suggested abuse. Why would parents want to put their child through something that is so unnecessary?? My answer: They were only thinking of themselves!! Very, very sad :-( The bottom line was they did not want to have a child who looked different. I wonder how they would cope with a child who had a craniofacial condition, such as arhinia??? I thank God for my parents every day of my life.

2. The Plastic Surgeon

I was utterly disgusted with the way Dr. Salyer described people with Down Syndrome. Remember this doctor is one of the first doctors in the United States to ever perform craniofacial reconstructive surgery. The plastic surgeon downplayed the whole surgery. I must say the best quote has been: "Does anyone know the difference between God and a plastic surgeon? God doesn't think He's a plastic surgeon." (The quote came off one of the listservs for individuals and families dealing with Down Syndrome.) I just don't get into the God complex from any doctor. Even my own plastic surgeon knows when enough is enough!! Thank God my plastic surgeon doesn't have the God complex. If he did, I would have been gone a long, long time ago.

Judging by the parents reaction in the recovery room leads me to believe that they were not fully prepared for how their son would look after surgery. I really feel that very few doctors prepare anyone for after surgery. In 1978, when I had my first Tessier surgery, my parents walked into ICU and nearly passed out after seeing me for the first time. They had no idea what I would look like after that surgery. Mom has always told me the only features she could recognize were my eyes and fingernails.

3. The Child

I did not like how the parents or Dr. Salyer did not direct anything towards the child. The child may have been three years old but must have had some degree of understanding. I have a friend who has a son who is two years old. Even tho her son is young, she still explains everything to him. I never heard the parents of the little boy talk about the surgery with him and what would happen to him. They were spending way too much time concentrating on how different he would look after the surgery. The parents should have waited until the child was older to include him in the decision-making process. I didn't care for the way Dr. Salyer told the parents how "We can do touchup surgery." Don't children have a say in their lives?? I wasn't too much older than the little boy when I was asking everyone questions about the hospital.

The child was beautiful before the surgery. Personally, I didn't see too much of a major change in his appearance after the surgery. No amount of surgery is going to take away the characteristics of Down Syndrome. Just like no amount of surgery will ever make me Miss America on the outside. But you know what?? I CONSIDER MYSELF TO BE A BEAUTIFUL PERSON INSIDE AND OUT!!!!!!!!!!!!!!!!!!!

4. Facial Anomalies

As someone with a facial anomaly, I personally took offense to how Dr. Salyer described children with Down Syndrome. To describe a child's facial characteristics as a "Mask of Deformity" is totally UNPROFESSIONAL. He referred to the face of a child with Down Syndrome as deformed. He is WRONG. The faces of children and adults with Down Syndrome are characteristic of the syndrome. There are many beautiful people with Down Syndrome. I hope that I never hear the phrase of "Mask of Deformity" again from the mouth of any plastic surgeon. It sounds like the plastic surgeon is doing a favor to anyone with a facial anomaly by reconstructing our faces. Does the surgeon think that he can magically make facial anomalies go away with his scalpel??? Someone said that she wishes she could be in the same room with Dr. Salyer and I for two minutes. If it would happen, this is all I would say: "It is what is on the inside that counts.. spoken from the mouth of my own plastic surgeon!!!!" Then I would turn and walk away.

My Ponderings

1. Doing Surgery on a Child with Mental Retardation

Most craniofacial surgeons don't do surgery on children with profound mental retardation since they would not benefit from the surgery. In fact, Dr. Tessier would not operate on a child with mental retardation unless he or she was high functioning, meaning that he or she could learn basic life skills. I have a friend with mental retardation who went thru some craniofacial reconstructive surgeries at the same time as I did. She is high functioning. She is able to live on her own with limited supervision. Her mom is a retired nurse who only put her thru the surgeries to save her sight, which she ended up losing after her near-death experience. When my friend nearly died after her surgery in the early 1980s. her mom said no more reconstructive surgery. My parents and I met several other children and adults with mental retardation who went thru craniofacial reconstructive surgeries. They were high functioning. Sometimes craniofacial reonstructive surgery is necessary in certain cases of craniosynostosis. I met a little girl who was born with Pfeiffer Syndrome (cloverleaf skull). She was blind and deaf and had a tracheostomy. I think that she had some degree of mental retardation. Dr. Tessier had to rebuild her skull.

2. Putting a Child through Unnecessary Reconstructive Surgery

Craniofacial reconstructive surgery is hellish for functional purposes. But putting a child thru it for the sole purpose of cosmetic is unthinkable. There is nothing wrong with the appearance of child or adult with Down Syndrome!! Parents don't want their children teased. But, the fact is ALL children get teased whether they have distinct facial features or not. If the child wants the surgery, the parents should wait until the child is old enough to be involved in the decision-making process.

My Views

You may or may not choose to agree with my views. That's fine. Please do NOT email me with your opinions. Please do NOT email me to remove the opinions expressed within this blog. Everyone has a right to express his or her opinions. This is where I express mine.

Waiting for Surgery

Please note: Any comments made here about my former PS are not a slam.. He is a great man. I would just get frustrated over waiting for surgery, especially when it was expected. I wouldn't have changed PS' for anything. I liked my PS a whole lot because he does a lot of great things for people. It's just feelings I dealt with at that time in my life. BTW, my PS really did understand me.

This was written when I was waiting for my surgery in January 1999 to be scheduled.

It seemed like every time I turned around that I was playing the waiting game. It can be very hard emotionally. If you have never played it, you will not understand it at all. But I will try to explain it as best I can.

Between March 1997 and January 1999, I played the waiting game with my life. I sat thru six months of waiting to have my surgery in September 1997. Then there was a 13-day delay. (I think the surgery date for that ended up being changed four times. LOL) I thought that I would be home free without any surgeries for awhile. Then in November 1997, PS tells me that he is considering opening up my nostrils more. (I had someone tell me that I shouldn't bother since the surgery would be only for looks, blah, blah. Geez that surgery made a difference in my speech. I am glad I went for it!!) When I went back to see PS in early February 1998, he schedules me for surgery in April 1998. (Actually I chose the date. LOL)

I had known about the January 1999 surgery since the night of my surgery in April 1998 because my mom told me that PS couldn't do everything he planned. I accepted that. I would rather my PS not do too much than have something fail because he did too much. At first, I was first told that I was going to have the surgery three months later. Then I went in for a follow-up appt. in May 1998. At that time, PS decided that he wanted to wait six months before he did anymore surgery. OK I dealt with that. I spent the summer doing online stuff and bowling. I went back on Sept. 1, 1998. At that appt., he told me that he wanted to wait and see how I do with beginning of the weaning from the nasal tubes. Well I've been there done that deal and I can tell you what will happen. All the surgery to open up my nostrils will be done for nothing. Everything will start closing up. I looked at him and said that I knew what was going to happen. I KNOW MY BODY. (And yes, I did say that to him.) Then he gave me some line about my cleft being worse on the right side. Hello was I not born without a nose??? (I really need to remind him of that. ) I am sitting there thinking: It's because of the way the flap for the columella was tunneled under my right nostril. Gee he was there. Now I wish I would have said something. :: SIGH :: I know more about my condition than any doctor does. Good thing I don't accept everything any doctor tells me because I would be up a creek without a paddle. No doctor knows everything.

I had a burning question to ask my PS when I saw him in October 1998. My question to him: Why was he so hesitant about doing my columella reconstruction? Was he afraid that I might be pissed if it didn't work?? I love the saying, "You won't know until you try." Been there done that at least two times in my life.

I am the one who made the choice to finish my reconstruction and I knew that I have to live with my decision. I certainly didn't like the waiting game. I accepted it even tho it was very frustrating and hard at times. I got over one hump and then another one would come along. I don't regret going thru the last three surgeries. They definitely made a difference. I just felt like the most critical part of my reconstruction at that point was eluding me!!! It was like.. I had waited forever for this surgery. I never dreamed that that part of it would be dragged out over this many years. (I had been waiting for nearly seven years to have the next stage of my columella reconstruction.) My patience was running thin.

I had used the stents since May 20, 1996. I played by the rules. I had the tubes out of my nose for a total of 61 days between May 20, 1996 and October 30, 1998. I thought about having the tubes bronzed.

I really liked my former PS. I tried to be patient but it was sooo hard sometimes. I completely understood that the surgery had a risk. I knew that there was a ten-percent chance of it not working because my PS told me. Back in November 1997, my PS told me that he would not do it for another year. (You should have seen the look on my face back then. ) I guess I was just wanting to get the surgery done and over with.

I was hoping to get a possible surgery date in October 1998. But I did not.

In October 1998, I finally found the root to my problem. I was feeling resentment and anger because of being given timelines all the time. It was very hard for me to deal with timelines for surgeries. I am going to talk to PS about this.

1998

Sun., Oct. 25, 1998

It's been almost two weeks since my last visit with my PS. I have to wait a little bit longer. He wants to see me in early Dec. I actually go back on Dec. 8th. I was disappointed at first. But now I am OK with waiting. This is something that cannot be rushed into. PS and I talked about the wait. He wants NO pressure on the columella after surgery. The slightest pressure could sever the blood supply to my columella. Now that would be hard to deal with because that would mean starting at square one again.

Thurs., Nov. 5, 1998

I am dealing with the waiting just fine. I understand the reasons for the wait. It is simple.. My PS does not want to rush into it. I think that my PS is wise for waiting. I do hope that he can give me an idea of when he wants to do the surgery when I go back to see him in Dec. I wish more people had the kind of PS I do.

Sun., Nov. 29, 1998

I am down to about nine more days of waiting to see when PS might do next round of surgery. I've been stent-free for just about a month now. The nostrils haven't closed up too much. I think that I breathe out of my nose now than I have in a long time.

Wed., Dec. 30, 1998

The wait is nearly over. I finally got my surgery date when I went and saw my PS on Dec. 8th. The surgery is scheduled for Tues., Jan. 12th. I've been OK with the wait. My greatest worry is getting the approval from my HMO to use St. Luke's. I hope to hear something soon.

1999

Thurs., Feb. 11, 1999

The wait was well worth it!!!!! I ended up having a successful reconstructive surgery on Tues., Jan. 12, 1999. I may have been mad at my PS back in Sept. and Oct. but now I am not. I am very happy with the results of the surgery. Now I just wait for the final round and I am no hurry to do it since it will be minor in comparison to what I've been thru in the last three years and the preceeding years.

2000

Sun., April 16, 2000

There will not be a final round of surgery. I've decided that enough is enough and that I am happy with things the way things are. Why mess with perfection?? NO MORE WAITING FOR SURGERIES!! YAY!!!