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Nager Syndrome (4 posts)

  • Started 10 years ago by zachsmom
  • Latest reply from zachsmom
  1. zachsmom

    zachsmom
    Member

    Hi my name is Janice and Zachary is my Blessing, he was born February 1998 @ Medical City. Zachary met Dr. Genecov when he was about 15 minutes old.  Zachary has Nager syndrome. We are blessed having been born into the craniofacial family @ICI., Dr. Genecov is an amazing Person and doctor who has an enormous heart. Plus he has the most wonderful staff to walk you down the path life has set you on.They Are Family who supports us with their Beautiful Hearts and Knowledge.I thought this information about Nager Syndrome might help someone.

    Have a Beautiful Life!

    Nager syndrome (or acrofacial dysostosis) is an extremely rare congenital defect that has only had around 90 documented cases to date (02/06/07). Only 25 or so of these people survived. Nager Syndrome displays several or all of the following characteristics: underdevelopment of the cheek and jaw area, down-sloping of the opening of the eyes, lack or absence of the lower eyelashes, kidney and/or stomach reflux, hammer toes, shortened soft palate, petite, lack of development of the internal and external ear, possible cleft palate, underdevelopment or absence of the thumb, hearing loss (see hearing loss with craniofacial syndromes) and shortened forearms, as well as poor movement in the elbow. It is rare for sufferers to develop vertrebral anomalies and sufferers do not usually have problems with their reproductive organs. Usually sufferers have "normal" levels of intellect and lead productive lives. Inheritance pattern said to be autosomal but there are arguments as to whether it is autosomal dominant or autosomal recessive. Most cases tend to be sporadic. It is unknown whether someone with Nager syndrome can have surviving children. Nager syndrome is also linked to five other similar syndromes: Miller Syndrome, Treacher-Collins, Pierre-Robin, Genee-Wiedemann, and Franceschetti-Zwahlen-Klein.

    External links

    Posted 10 years ago
  2. cranium

    cranium
    Key Master

    WOW!!!  Amazing post.  Thank you so much.  We've registered a lot of users, but only a few have posted so far.  You not only embody the spirit of this site, you are full of incredible information too... that I know is going to be helpful to many.  Incredible.  

    Posted 10 years ago
  3. zachsmom

    zachsmom
    Member

    My how the time does fly it has been 4 years since we have done a cranio distraction surgery and i am sooo grateful for the supporting team at ICI. It's been so nice and easy not having to go through the stresses that come with cranio surgery but it is time to move again and do what must be done. GOD's plans are perfect and HE Blessed us that scary day Zachary was born 15 years ago when HE gave us David Genecov. He is still an Awesome doctor after all these year, a Doctor/Person whom i have such a great respect and heart for, the cranio team he has built are our family and i can't ever truely express in words what a treasured impact they have made in our life. They have seen me at my worst and best and still hold my hand and heart, with such caring hearts of their own, through the hardest of times. I Love this cranio team they help me to face and do the things i don't want to do cause i'm tired and it hurts so much to do what must be done for my baby (he is the air that i breathe).  We have surgery on monday 9/23/13 and our lives will become a lil twisted for a time but it's not my first parade and GOD equipped me for this along time ago.  I just wanted to share with parents facing Mandibular (Lower Jaw) Distraction information on useful supplies and things that may come in handy and help with the healthy healing for your child. Have your primary care doctors prescribe the following things for you, most insurances will cover the supplies  and what is not covered is usually affordable.

    Be sure you drop off and pick up the medicine prescription as soon as you get them so that you have them when you get home.

    Liquid Nutrition (cranio team will advise about this and usually prescribe) 

    Toothettes (preferably the spongy kind) for cleaning lips and tongue

    Qtips for cleaning and applying ointment if needed

    Sterile Saline for rinsing and cleaning the distractor sites or inside mouth using 10cc syringe to flush area.

    10cc syringes pointed tip for flushing distractor sites(this helps to keep the the device from getting wet too much) also handy for feeding and flushing the inside of the mouth out so that it stays clean from most bacteria caused by liquid or soft foods.

    This is all i can remember so far but will continue to update as we begin the Life of Distraction again (LOL accidential play on words) and work through each new day before us. Hope this information will be useful

    Posted 6 years ago
  4. zachsmom

    zachsmom
    Member

    Try to get your supplies ordered about two weeks before surgery because it could take one week to 10 days for insurace approval and a day or two for delivery. most insurances will not make medical supply deliveries while you (child) are admitted to the hospital so early ordering is best so you have what you need once you get home from the hospital and aren't struggling without them when you need life to be as simple as possible.

    Posted 6 years ago

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