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Kim's Story: Elena had an uncomplicated delivery at 40 weeks. Her birth weight was 7 pounds even. After the customary parental inspections she was moved to the well baby nursery.
Within 6 hours of birth Elena’s nurse discovered her to have a low body temperature, the nurse tried to correct the low temperature. Within another hour she was even colder, floppy and changing colors rapidly. The doctor ordered a blood glucose check and found that her blood sugars were low for her age. They placed her on light therapy and started an IV feed of dextrose. After five days of sugar water it was determined safe to send her home. During those days in the hospital her feeding was below normal levels, but it was thought that this is due to her receiving an IV. Elena was discharged after 8 days in the nursery. Elena was readmitted after 24 hours at home with seizures and low blood glucose readings. After being transferred to a level 4 Neonatal Intensive Care Unit [NICU], the course of care was for the low sugars. No tests or studies were done in the next 6 weeks except for blood. When she was discharged again her weight was now 4 pounds, she was jaundice and could only eat 1-1/2oz an hour. She was labeled Failure to Thrive [FTT].
Our first stop was the Gastrointestinal Doctor at UCLA. UCLA did a battery of tests related to feeding issues. She also had 2 liver biopsies. All the results showed were giant cell hepatitis, and an absence of the gall bladder. After 3 weeks she was discharged home again on a complicated feeding schedule with many additives. She still wasn’t eating and she was starting to have episodes of blacking out and lethargy.
We were making many trips to the ER with an unconscious child and no answers to any of her episodes. UCLA kept checking systems and found that her kidneys were not growing and had problems. We went through the summer and at a follow-up visit at UCLA with the GI team they told us she is falling off of the growth charts and maybe we would like a consultation with the endocrinologist when they were done with her. She was readmitted at that visit for Ph studies and consideration of changing from a nasal gastric tube to a g-tube. The next morning an endocrine fellow visited us and said he would like to do some fasting studies before GI does anything else. That evening Elena went all night without food, about 5am, the nurse noticed her getting lethargic and changing colors. They immediately checked her blood glucose and determined it was 9. They fed her and send her down for a MRI; within the hour we knew she was panhypopituity. The following afternoon we had a supply of Growth Hormone from Genetech and started it that night. Within the week, her color improved her eating increased and her whole attitude was brand new. Prior to admission she was doing 2 month old activities. Within a month of discharge her weight was improving. At one year she weighted 12 pounds and at two years she was 25 pounds.
We jump ahead to pre-school and we had a very interesting time. We were living in a mountain community, small school district no doctors and a six bed hospital for sports injuries. The school worked with us, they provided an aide on the bus for Elena and set limits for her attendance according to her blood sugar readings.
When Elena entered kindergarten, her first teacher refused to have her in the class if she had to check her blood sugars with needles. We ended up moving Elena to a school in the middle of town. When she was in pre-school the district was happy with a morning reading of 70 or greater, the new school district wanted 50 or better for her to attend. Since the only opening for kindergarten was in the AM. She missed several sessions. In first grade she moved back to her home school and things have improved.
In the summer of 2002, Elena was attending the annual MAGIC convention and started having problems with headaches and vomiting. We administered her emergency solu-cortef [see instructions from NIH]. This continued through July and August. When Elena had her annual eye check, the pediatric ophthalmologist observed pressure in her eyes, he sent us for an MRI and then a cross town drive through Los Angeles on a Friday evening to Children’s Hospital Los Angeles [CHLA]. They were able to determine she had increased Inter Cranial Pressure. Elena was taken off of the GH, she had many visits with the eye doctors and endocrinologist. At her 6 month check it was finally observed that her GH had leveled off and she could start it up again. During the time she was off of GH, we noticed a drop in her attention spans, her muscle tone and general health seemed to decline. Our next dilemma is dealing with her weight gain.
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