Julia's Journey


Julia’s Journey #75 2/5/00 T+536

Greetings to all on the occasion of a prominent anniversary for Julia. It has been one year since Julia’s med flight from North Carolina to Michigan. We have come so far! Good fortune plus diligent hand washing seem to have paid bountiful dividends. Julia’s last hospital stay was in June, almost eight months ago.

Thanksgiving and Christmas flew by. Julia really enjoyed the sights and sounds of the holidays. We decorated our tree in red and white lights, since red is the color she is most likely to see. Alison and Cameron were concerned that Santa wouldn’t leave any presents this year because Julia resides in the living room, and has been known to stay awake at night. No problem though, Julia must have tripped Santa on his way up the chimney, because he certainly dropped a lot of presents by the fireplace for her and her siblings. One of the most useful is Julia’s mini recliner, which gives her a new and very practical place to sit and survey household affairs. Attached you will see a picture of Julia in her Christmas finery.

We rejoiced to have Julia at home on December 27th to celebrate her 2nd birthday. What a difference a year makes! On her first birthday we were in room #5212 on the Duke transplant floor, wondering if she would survive the night, one of her worst. This year she sat on my lap at the dining room table, while her brother and sister helped open her presents. Thankfully, the coming of the year 2000 was as uneventful here as it was for the world at large, merely the changing of the calendar. After the excitement we endured in 1998 and 1999, it was a pleasant change of pace.

Julia continues to progress in different areas at varying rates. At her neurology appointment in January, Dr. Leber congratulated us on having reached one of the goals we had stated during the previous visit. Last August Julia was on the ventilator 24 hours per day. She is now OFF the vent twelve hours per day, as long as she is not sick. She still takes a tiny amount of oxygen, 1/16th to 1/32nd of a liter, a few extra molecules per minute. Her oxygen saturations are in the high 90’s with the O2, and in the low 90’s without it. Although this is a very small amount for a more normal person, it seems to make a big difference to Julia.

Julia keeps her eyes open more frequently than in the past, not all day, but virtually always when she is upright. Although she continues to prefer low light levels she opens her eyes in bright light if there is an attractive sound. She likes to watch her Baby Mozart tape in a darkened room, and works hard to orient her head toward the TV. Many of our nurses have commented on how she follows me with her eyes. It takes 7 or 8 seconds on average for her to move her eyes in a full sweep from right to left or back, with the movement being very jerky. She fixates with a steady gaze more often. I constantly try to ascertain how much she can perceive. Up until recently she would not react when her eyes were wide open if I put my hand right in front of her to occlude her view. Now she reacts with nystagmus, not the desired response, but still a response.

Julia’s hearing remains acute. She especially enjoys one new sound, her own voice! Because of the trach bypassing her vocal cords she is vocally silent when she is off the vent. When she is on the vent she can make sounds upon inspiration. She sounds just like a lamb: baaaaa... baaaaa... baaaaa. We can tell that she very much enjoys this game. She also lets us know immediately if she is unhappy, with a quite normal sounding multi syllable cry. She has a sweet little voice! We are exploring the possibility of a special valve that she could wear during the day that would allow her to hear her voice more often.

Julia is able to sort noises that merit attention from noises to be ignored, much better than she could earlier. In the past she startled with every closing clipboard, every spoon on a plate, every pound of a foot on the floor. She still startles, but much less readily. For several months it was very difficult for her to fall asleep, because she constantly startled awake to the slightest internal or external signal. For the last month or so she has settled quite well into a normal two year old schedule.

The most exciting progress has been with Julia’s head control. For so long lifting her was like lifting a newborn. We had to hold her head and carefully be sure that it didn’t flop back or to the side. Now, she holds it all by herself so well that we don’t worry about it! When she is doing tummy exercises on the therapy ball she can hold her head up for about a minute in a position similar to a crawling toddler. She moves her head from side to side quite readily. She still prefers the right side, a source of concern because she has developed a 40 degree scoliosis. We are seeing more initiation of left sided movement, especially when stimulated. Our next big challenge is for Julia to make the connection that she can reach and grab. We have seen a lot of new leg motion, but not nearly as much arm motion. One of our therapists theorizes that the weight bearing Julia does in her stander is stimulating neural pathways in her legs, so we are working on developing weight bearing arm exercises, not so easy with the trach! Our efforts may be paying off because just this past week we have been seeing more bent arms and arm rotations.

Julia continues to hone her eating skills with various frozen juices in nipples, and now with yogurt. Her swallow is delayed 7 to 10 seconds, so she does better with foods of higher consistency that give her tongue more feedback. We have noticed a pattern in the length of delay for responses. It takes 7 to 10 seconds to swallow, move her eyes across a field of vision, initiate a rolling response, react to warm water poured over her, and many other mild stimuli. In contrast, she reacts almost immediately to unpleasant stimuli, such as a painful poke, loud clap, or something very cold. Thankfully, she once again exhibited all reflexes at her recent neurology visit, as well as a few extra ones on the side opposite from the stimulus. :-) She habituates to stimuli rapidly. We are likely to get a response to most things, but once she has reacted she is ready to go back to rest.

We have been fortunate to have had no major medical challenges over the holidays. In December Julia caught a cold, and survived it with a few albuterol treatments, some extra vent time, and no hospital. In January her port clogged, a g a i n. (Oh, this story is so old!) Now we can not draw blood at home, and monthly labs have to be done by finger or heel pricks. We hope to resolve this issue soon. The only other problem has been a Clostridium difficile infection, essentially an overgrowth of bad bacteria in the gut due to too many antibiotics. Julia has been on Bactrim since a year ago, first for pneumonia prevention, then for urinary tract infection prophylaxis. Now she is on Vancomycin, another very powerful antibiotic, to fight the clostridium, in addition to the Bactrim. I am hopeful that she can avoid another Clostridium difficile infection until the Bactrim can be removed permanently. Julia continues growing rapidly both in height and weight, soon to catch up with her age peers. One area she lags behind in is head circumference, which has not grown any larger in the last year, but hopefully soon this will begin to catch up too. We know that the neural pathways are growing because she does so much more than she did a year ago, but she still needs a very substantial increase in white matter to avoid permanent microcephaly.

As always we are grateful for all of your well wishes and prayers. Julia has come so far, and although the road ahead is very long, we know that she will exceed everyone’s expectations. We are pleased to hear that a number of other Krabbe children have been transplanted recently at Duke, and wish them great success.

~Julia, her mom, and her whole family

Julia’s Journeys are archived at