Brenna aka "The Plum"
Hello, my name is Robyn Johnston. My daughter, Brenna, was born May 14, 1996, with Crouzon Syndrome. She is our first baby. My husband, Erin and I call her the "Plum", short for "Sugar Plum" because she is such a sweet little girl. She has recently been categorized as having a rare form of Crouzons which affects the FGFR3 gene, (most Crouzons are FGFR2). We will probably have her genetically tested at a later date to confirm. She has most of the typical Crouzon symptoms but a moderately restricted nasal cavity.
A brief view of our history. I had a very healthy and easy pregnancy except Brenna was breach and I lost too much amniotic fluid at the end. I had to have a C-section to deliver her. When Brenna was born, the doctors weren't too sure what she had. They knew she had craniosynostosis and checked for brain damage. Luckily her brain was normal. The rest of her body was unaffected. She had severe restrictions of her nasal cavities, and had to stay at the NICU for 4 days. We were released with a monitor after 4 days. She was a very noisy breather, but it was music to my ears to know she was breathing. At 3 weeks of age, genetic specialists form Portland diagnosed her with Crouzon Syndrome. We were referred to a neurosurgeon in Portland, OR. for future surgical intervention.
SURGICAL AND MEDICAL HISTORY
At 4 months of age, her weight dropped to the bottom of the weight chart, so her pediatrician recommended we see a otolaryngologist. As she was growing, her restricted nasal passages became less efficient. Her breathing became more labored and it was hard for her to suck and breath at the same time. Her ENT suggested she needed immediate surgery to put Stents (tubes) in her nose. The other option was a tracheotomy. We chose the stents as I was scared to death of a tracheotomy at the time. Looking back now, the trachea might have been better over the long haul because we still continue to battle her breathing issues. The stents were successful, but the cleaning was a chore. We also switched to a bottle designed for babies with cleft & palate (Mead Johnson) which helped reduce the bottle pressure while sucking. We also increased the calories of her formula.
Her weight came back up on the charts and her cranial/forehead advancement was set for 6 months of age. The stents came out at the same time as they are short term. She had some complications during surgery, her skull bones were underdeveloped, so the doctors did not use the technique they had planned. They had to piece her skull together with many plates and screws. They are now discussing redoing the surgery. One side of her forehead above her eye is recessed. They want to bring it more forward to create more of an orbit for her eyes before her mid face advancement. We have taken Brenna to another craniofacial team in Seattle for a second opinion. My husband and I have chosen to use them for any remaining major Craniofacial surgeries. They have suggested waiting to redo her surgery between 3 and 4 years of age when she has an adequate bone supply to use.
Brenna has glasses that we have recently gotten her to wear at 19 months of age. Her eyes tend to wander outward when she tries to focus (strabismus), and she has moderate astigmatism. The glasses seem to help both. It was hard to find someone in our town who knew how to fit a baby with a craniofacial difference. Her nose is very small so the glasses constantly slip down. We got her to wear them by bribing her with toddler computer games! Hey - whatever works!
She had tubes put in her ears at one year of age because she had constant fluid in her ears which caused significant hearing loss and repeated infections. Draining the fluid improved her hearing loss dramatically. We have had no problems with her ears since she has had tubes.
She also had stents put back in her nose at the same time, because she has moderate sleep apnea and her oxygen levels got low at night. (confirmed with a sleep study). Unfortunately we had them removed 2 months later because they did not help and there were so hard to clean and maintain on a toddler. The ENT also removed all excess soft tissue in the area. Immediately after surgery, her breathing improved for a couple of months. Now, as she is growing she is having trouble with apnea again. We believe that her tonsils and adenoids are beginning to develop obstruct her airway, (Her ENT's warned us this may happen this year). They will most likely be removed sometime in the near future.
Brenna is beginning to talk but we are teaching her sign language as extra language. She is very chattey and vocal, but may have some trouble with speech as her mouth is small and her tongue gets in the way of word formation. She has also had a hard time learning to eat solid foods as her mouth and airway are so small. She still prefers to eat small pieces of food and softer textures. At first, she choked a lot and refused any food. We had to introduce solid foods very slowly and start out with very soft "melt" in your "mouth" textures.
She is currently being followed by an early intervention team regarding eating, speech and motor development. They have been very helpful. Her motor development was slow at first, but she is now catching up by leaps and bounds.
Overall, Brenna has been extremely happy, good natured baby for all she has been through. My husband and I feel very fortunate to have Brenna in our lives. She has become strong willed and independent as well as very loving. She is very full of life, and loves to read and play and is very observant of her surroundings. These are traits which we will continue to promote as they can only help her in life.
Erin, Brenna and I have had very full and stressful 2 years. I think we are finally adjusting. Brenna is now seeing a wonderful new team of doctors from Seattle who specialize in children with similar syndromes. It has been a blessing to find them. Brenna's medical concerns seem to be slowing down finally! Her next planned craniofacial surgery is not expected until she is 3 or 4 yrs old. After 4 past surgeries in a year, we hopefully have a much needed break for a while.
I am currently taking a leave of absence from my job to make sure she continues to get the adequate medical attention she needs. It has been rewarding for the whole family, for me to be able to stay home with her. Brenna has thrived, and is back on tract developmentally since I have been able to stay home with her. It has really made a difference. We are very fortunate to be able to do this.
Brenna is going to make a wonderful big sister to her new baby brother
who is expected to enter the world at the end of March '98. I can already
tell he going to be the wild one of the family. He doesn't slow down in
mommy's womb from dawn until dusk. We will have our hands full then.
MY BIGGEST ADVISE FOR NEW PARENTS IS...
Be thankful and appreciative for what we have on a daily basis. Never forget that. Give your little angels a big hug and kiss every night. They can never be spoiled with too much love.
Keep open communication with your partner - even if you do not have the same opinions. It is important to communicate. You are each others support.
As hard as it is, make time for you and your partner to spend time alone - no matter what it takes.
If you and your partner are having trouble, see a marriage counselor BEFORE it is too late. Having a medically fragile child is VERY STRESSFUL. It is ok to seek help.
Do not be afraid to discuss topics with 'others'...You will be surprised how many 'others' have "been there" also.
Ask your childs doctors all the questions that you can possibly imagine. They are working for you and you need to feel absolutely confident in what they are doing.
Find out how often the doctor does surgery on children who have similar needs to your own. Get references!
Do not be satisfied until you receive all of the answers that you came for.
Do not be afraid to get second opinions. Everyone is entitled.
Do not be afraid to ask why - or challenge the system, if you do not understand or agree with the way something is being done.
We are our childrens best advocates. We need to put our own personal issues aside and do what is best needed for them.
Education is the key to harmony. Do not be afraid to educate anyone
who is willing to listen. Learn as much as you can yourself.
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Created: Sat., March 14, 1998
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