Leah Rose's Mom ~Princess in Training Blog

*** September 19, 2003 ***


While I was in recovery, the pediatrician arrived to inform me that Leah Rose was born with a cleft palette in the soft palette of her mouth and the nurses were having difficulty feeding her.  Leah was brought to me and I held her for about 15-20 minutes, but I was shaking too badly to be able to enjoy it.  Plus she was screaming at the top of her lungs.  I tried nursing her, but she was too upset.  I was able to pump, and she took a small amount from the bottle and the nurse felt encouraged.


The pediatrician also informed me that Leah's left fingers were curled in and drifted sideways and one foot was turned in.  She might also have to have surgery in about a year to repair the cleft palette.


*** September 20, 2003 ***


The pediatrician arrived to inform me that the nurses had been in contact with her throughout the night and that feeding had not been successful.  The food was coming back up Leah's nose.  She also advised me that part of Leah's ear cartilage was folded over, and that her toes and fingers were slanted.  The decision had been made to transport her to the intensive care nursery (ICN) at a children's hospital in a nearby town that was more equipped to handle her problems.


I was able to spend about 30 minutes holding her before the staff arrived from ICN to transport her.  My mother and cousin were in town and they followed the ambulance to the hospital so they could be with her to talk to her and hold her hand.


At this point, I'm still handling things calmly until later that evening when it finally hit home that I wasn't holding her in my arms.


At the ICN, a heart murmur was detected and they also felt a distended belly.  She was put on IV fluids and a feeding tube.  According to the nurse, Leah took a while to learn the rules.  "You don't take out anything you didn't put in."   Leah learned the trick of pulling out the feeding tube very quickly.  She will be seeing a kidney specialist, gastroenterologist, a heart specialist, and an orthopedic specialist.


*** September 22, 2003 ***


The nurses have been looking in their medical books and have found a syndrome called Cerebro-Oculo-Facio-Skeletal (COFS).  The characteristics are small jaw, small recessed eyes, overlapping fingers, and rocker bottom feet.  Leah displays all of these.  It has been determined that her bladder is thicker than normal, her kidney is tilted, but functions well.  The heart has a small opening that opens and closes intermittently causing the heart murmur (or PDA).  The DNA blood work has been sent to the genetics specialist at the University of Florida in Gainesville.


I saw Leah for the first time in two days.  She looks so tiny laying in that little bassinet all wrapped up.  I was allowed to feed her, but I wasn't very successful.  The trick was to squeeze the bottle at the appropriate time and to help hold her bottom jaw closed for better suction.


*** September 23, 2003 ***


Leah passed her hearing test, and a follow-up is recommended in six months.  An eye exam has been requested.  No anomalies were found in the CT scan of the head.  An MRI scan is recommended because of one little thickening area on right side.  Renal exam was okay except for right kidney tilted.  Recommends follow-up at regular intervals.  She may be prone to kidney or bladder infections and needs to be monitored closely.  All internal bony structures are normal.  Small jaw and other facial features may change as she grows and reaches more normal proportions.  If it continues to cause problems with eating, reconstructive surgery can be done.  As for the cleft palate, surgery would not be done before six months in the hope that the opening will close by itself or become smaller so that less reconstructive work would be necessary.  Feeding tube was reinserted.  At time goes by, it may be necessary to surgically implant one in the tummy.  Learning to work with getting her to nurse is the major concern at this time and will be the critical factor in determining when she can go home.  The heart rate has not dropped today and hopefully the opening will close on its own.  Echo cardiogram was normal.  The opening is from the aorta to the lungs and normally closes when the baby's cord is clamped.  It seems to be closing on its own at the present time.


I am visiting Leah each day, but it's a hard trip for me.  I am recuperating well from the c-section, but I tire easily.  It's about a 45 minute drive to the hospital from where I live.  I can only visit with Leah about four or fives hours before I give out.  I wish more than anything that I could stay and hold her all day and feed her and love on her, but it's just not possible.


*** September 24, 2003 ***


The eye doctor examined and dilated her eyes.  Everything looks fine and recommended a follow-up visit in three months.  Her eyes are very small and she keeps them closed tightly.  The doctor had difficulty prying her eyes open.  She can open her left eye at times, but appears to have difficulty opening her right eye.


Feeding is sporadic.  Sometimes she will eat all of it, and sometimes she won't.


She was examined by the plastic surgeon today for the cleft palate.  The midline cleft is in the soft palate and the uvula is also split.  The repair is recommended between 9 months and one year of age.  This time frame provides the best results for speech and ear development.  The growth process of the mouth also helps so that less work has to be done surgically.  She may need to have tubes put in ears at the same time.  The surgical procedure is done in three layers.  There should only be one small scar in the midline area when healing is complete.  Surgery would last 2-3 hours, with a 3-4 day hospital stay.  She will have to learn how to breathe through her nose after the surgery.


The orthopedic surgeon will be called in to examine her hands and fingers.  May put a splint on to help the fingers grow more normally.  Surgery may be necessary for the right thumb.


IV to be removed tomorrow.


*** September 25, 2003 ***


Continues to lose weight.  Down to 7 lbs.  May add supplement to the milk to keep 2400 calories.


The orthopedic surgeon examined her fingers and thumbs.  Will probably splint her thumbs, but not for a couple of months.  Hips and knees seem to be okay.  Right foot and toes may need splinting or casting or surgery possibly at eight months of age.  Ordered x-rays of the spine.  An MRI of the head has also been ordered.


*** September 26, 2003 ***


Physical Therapist examined her hands and fingers.  She recommended massaging her hands, fingers and thumbs along with moving her arms and shoulders in the normal range of motion.  Leah appears tight in the shoulders.  Her legs and feet should be massaged and moved also.  A physical therapy schedule may be started while she is in the hospital.


The MRI of the head is scheduled for this afternoon.


I tried breast feeding her for the first time today.  It wasn't successful.  The shape of her face and mouth prevents her from latching on.  The important thing right now is getting her to eat and gain weight.  Not too much talking and playing because her endurance is very limited.  Her weight was steady - hasn't gained or lost.


*** September 27, 2003 ***


No anomalies were noted in the MRI of the head.  Some prominent space in craniofacial features.  Weight is 7 lbs. 5.6 oz.  Physical therapy has been ordered, but won't start for another two days.


I bathed and dressed her for the first time today.  She didn't like it too well.  Her big brother, Ted, from Georgia came to visit her today.  He was very comfortable holding her.  See picture here.


*** September 28, 2003 ***


A doctor from the local Naval base was in attendance today.  He does not feel that the diagnosis is COFS syndrome.  So far, she doesn't fit the picture of anything that he has found.  He took pictures with my permission to share with his naval colleagues to see if they could help.  He could not say what her life expectancy would be.  Life expectancy for babies with COFS syndrome is less than five years.


I tried to feed her again today, but I wasn't successful.  However, the nurse was able to get her to take the rest of the bottle.  She lost a couple of ounces.  Weight:  7 lbs. 2 oz.


Spinal X-rays are normal.  There had been some concern regarding the cervical area, but it was okay.


*** September 29, 2003 ***


Leah was released from the hospital today.  She is feeding well and the tube has been removed.  Her weight is 7 lbs. 4.8 oz.  I was in a panic because the successful feeding had not been due to my efforts.  I still hadn't been able to feed her a complete bottle yet.  We met with a genetics counselor today to review the family history.  She will provide the information we gave her to the genetics specialist in Gainesville.