Update 11 (8/30/98)

Julia's Journey: Update #11 8/30/98 day T+10

Good news: Yesterday Julia's white blood cell count went into the measurable range at .1, and remained there today. She has a very definite rash, looking like a thousand little red dots on the soles of her feet, palms of her hands, neck and trunk. And, for

the third day in a row she started a fever at around 5 in the morning, up to 39 C or so, disappearing around 11AM. All of these are hopeful signs that engraftment is starting to happen!

Through all of this Julia is becoming a very happy kid. Those of you who saw her back in June and July know that this is a major departure from what had become the norm. Now, when she's not febrile, she is looking around, maintaining eye contact, moving her arms, and doing lots of "snake tongue", her communication for 'happy'. She loves her physical therapy, and yesterday was actually holding her thumb in her mouth (with mom's help) for a brief time! Her hands are much more relaxed than the former tight fist. Yesterday she did not cry at all, and has been sleeping through the night, or at least from about midnight until 7 or 8 AM. She is clearing the extra saliva caused by the mucositis effectively by making a very loud bear growl sound: music to my ears!

Now for the amazing part: she hasn't had ANY sedative for more than a day! Her last dose of Ativan was at 8 AM on Saturday. We are also removing the Fentanyl (narcotic painkiller) pump because she hasn't used it in four days. I know that the doctors say that we should not be seeing any direct improvement for many weeks or months, but anyone who listened to Julia cry for those weeks on end could not help but make the comparison between then and now. We know that when engraftment is in full swing she could again become very irritable, so the absence of sedative may be temporary, but it's still amazing that it's happening at all.

For those of you out there for whom eating is something of an issue, and you know who you are, {no names mentioned here ;-} let's review some of the basic facts. You procure food, you put it in your mouth, you eat it. Done. Not so for a kid with a gastric tube. Julia began her feeds again two days ago, which is great. Since she still receives only breast milk the hope is that maybe the immunities will give her a little extra boost. So, we put the milk in the pump. The pump pumps it into her tube, and her tube... STOPS! Traffic jam somewhere inside. Pretty soon the milk is backing up, going nowhere. What to do?

Pull out a plunger! Yup, push that plunger into the syringe at the end of the pipeline attached to the tube, and plunge. In and out, gently at first, until, POP! Sediment dislodged, traffic jam solved, feeding progresses.

Gee whiz, how I long for simpler days!

When I think of all the procedures I have had to learn in the last month I just know I should put in for my honorary nursing certificate. Everything from suppositories and temperatures taken with mercury thermometers to priming pumps and cleaning g-tubes are now part of the daily regime. And this from a person who used to be squeamish just thinking about newborn navels! {You must be laughing hysterically, Judy =} I can now even calculate centigrade to Fahrenheit, milliliters to ounces, and kilograms to pounds in my head.

I must admit that I would gladly trade all of my current new knowledge for my old ignorant bliss. I will forever more be grateful when someone in my house spills their milk to know that first they can drink milk and second that they can spill it. Julia can do neither now. But, someday...

Thanks to all of you who keep those prayers and well wishes coming. A special request: A little girl on our floor, Kelli, passed away this morning. Kelli's parents very generously gave Julia one of Kelli's special dolls, which we will always treasure. Please add Kelli and her family to your prayers. Every child is so precious.

~Julia and her mom

Update 12 (9/01/98)

 Julia's Journey: Update #12 9/1/98 day T+12

Great news: two days ago Julia's White Blood Cell counts reached the measurable range at .1. Yesterday she was at .3. And today she is at .6! What a hard working girl! And what a hard working rest of us.

Yesterday was a bit of a trial. Julia needed hemoglobin and platelets, which is not too unusual for transplant patients. Prior to getting these infusions it is standard procedure to give benadryl and tylenol, which Julia received around 8:00 AM. Julia is so sensitive to anything sedating. It appears as though something in one of these medications put Julia to sleep for hours.

At first a snoozing baby, especially a Krabbe baby, sounds like a mother's dream. But Julia's deep sleep prevented her from doing bear growls to clear her throat of the thick mucous produced during the mucositis stage. And, various of the medications are also making her retain water. The result was that her oxygen saturation began to fall well below the near 100% where it should be. First we gave her some blow-by oxygen, then a nasal cannula, and finally an oxygen mask, which she is still wearing to keep her sats up to where they should be.

Julia still was not awake through the evening hours. It was getting more important to see some motion because the afternoon's X-ray showed a little of her excess fluid was beginning to leak into her lungs. I tried hugging, holding, dancing, pinching, poking, and prodding, but Julia still wanted to sleep. I even tried singing, which normally would wake Rip Van Winkle.

Finally by midnight I was dripping some ice water on her toes. She opened her eyes very wide, and looked straight at me in utter disbelief as if to say, "Mom, you're supposed to be on MY side!" She's been a little whiny ever since, but continues working hard at moving the mucous out of her passageways, with lots of grunts and groans. We just had another X-ray taken to see if the fluid situation is improving. It is fascinating to me to watch the endless array of gadgetry in this hospital. When you need an X-ray there is no need to get out the map and go to radiology. They just wheel the X-ray machine into your room. And, the oxygen pump is hooked up to a humidifier that blows bubbles through water. The result is that when you walk in here it sounds like a babbling brook. I couldn't resist the temptation to put on one of Julia's relaxation CD's, "Mountain Stream", to enhance the effect created by the humidifier, and give the whole room that woodsy-by-the-stream feel. One of the nurses said she felt like she should be swatting for mosquitoes! :-)

Allie, the Krabbe baby who was transplanted 12 days before Julia, gave us some much needed inspiration yesterday. Her mom had her in the hall WITHOUT her pole of pumps and IV's! They will probably get to leave the hospital by the week's end. Allie went through all of this a couple of weeks ago, and she's now so much better that she's on her way home. Way to go, Allie! We're looking forward to joining you in the outside world soon.

The many well wishes and prayers are working, thank you all.

~Julia and her mom

PS: If you've tried to call and couldn't get through, try again! Your luck will be better this time. ;-}

Update 13 (9/03/98)

Julia's Journey: Update #13 9/3/98 day T+14

Julia must like bad weather. Between Hurricane Bonnie and Tropical Storm Earl her counts have really gone up. Yesterday her white blood cells count was .9, and today it was at 1.6! According to the sheet that we are given each day quoting all of these statistics, white blood cells are considered normal at between 3.8 and 14.0. We are well on our way!

Today another count became very meaningful, Absolute Neutrophil Count, or ANC for short. For those of you who are interested, this number is calculated as follows:

total WBC X % (segs + bands) = ANC

WBC = white blood cells. There are two kinds of white blood cells: --lymphocytes, which fight mostly viruses --granulocytes, which fight mostly bacteria and fungus Segs and bands are two types of granulocytes. Consequently, of your total white blood cell count you want a large percentage of them to be granulocytes, or bacteria fighters, which are also known as neutrophils. If you don't have very many of them you are known as neutropenic. Being neutropenic means you are immune deficient, and very likely to catch infections because your body has nothing to fight the germs.

So, what's the practical application of this? When your ANC rises above 500 for three days in a row you get to leave your room without a mask! You are no longer considered neutropenic, and that is a joyous occasion. Yesterday Julia's ANC was 288. Today her ANC was 624! In just two more days, if all goes. Well, we will get to introduce Julia to everyone else on the floor who also can leave their rooms. This will definitely be a good time to put on her best outfit and get the camera ready.

Besides growing cells Julia's biggest challenge lately continues to be eliminating fluids. The second X-ray we had taken a couple of days ago showed her lungs to be clear, but the butterball babe still weighs about 20% more than she did when she arrived here.

Hence, I once again find that the way I define a good time these days is radically different from anything in my past. For example, I LOVE, ADORE, COVET, and DREAM of big juicy wet diapers. Yes, it's true. It is really necessary for Julia's output of urine to exceed her input of fluids, so I find myself celebrating when we have a urinary event, and being ecstatic when the diaper is totally saturated. I know that some of you think we get bored in here, but I get to peek in Julia's diapers at very regular intervals just to check on how we're doing. It's definitely better than TV!

It is now time for me to do exactly that: confirm that we are now positive output, or more coming out than going in. I'll find out soon, but you will all have to wait two whole days to know the answer. ;-}Meanwhile, please keep those well wishes and prayers coming. They are working!

~Julia and her mom

NEWSFLASH!!

Great news here: Julia's white blood cell count this morning measured 1.8, up from 1.6 yesterday. This afternoon it jumped to 2.6! Remember, 3.8 is considered the beginning of normal range. And, she has started to show a small amount of GVH (Graft Versus Host) rash, which is a hopeful indication that the new cells she is growing are donor cells, and not her own. A little GVHD is a good sign, just not very much. We just wanted to make your day a little nicer!

~Julia and her mom

Julia's Journey: Update #14 9/6/98 day T+17

Two days have passed and Julia remains at the same weight, same intake, same output. So, diapers remain a high priority. If Julia should happen to start urinating on a large scale then diaper rash becomes a real possibility, something she has never experienced in a major way.

I assumed that of course there would be a eminently scientific solution for the problem of diaper rash. There is. It's a unique blend of Peru Balsam-Zinc Oxide Compound Ointment, that goes by the highly technical name of 'Dr. Harris' Buttock Paste'. Now, that's some terminology we all can understand! Even the directions are in layman's terms. All it says on the label is, "Caution: DO NOT TAKE INTERNALLY". I have come to really appreciate simplicity.

Another ingenious invention that has become my favorite of all the interesting gadgets and gizmos here is some nifty elastic material by the name of "Co-flex". Co-flex looks like a cross between duct tape and an ace bandage. But rather than having a sticky surface on just one side, this "cohesive flexible bandage" is a sort of rubberized stretch material that can be used repeatedly, on just about anything.

The best part is that it comes in a wide variety of fashion colors! We have used Co-flex to hold Julia's g-tube in place, to separate the spaghetti coming from the pumps to her central lines, and to keep her thumb splints in place, always coordinating with the outfit she is wearing on that particular day. No sticky residue, as you would have from tape! Some of the teenagers here decorate their entire rooms with the stuff. Now, that's a thought...

This is the fourth day that Julia's ANC has been well above 500, so we are eligible to leave the room and go into the hall without a mask. The only hurdle to overcome before we do this is to finish weaning Julia off the oxygen she is still getting through her nasal cannula. Her high fluid retention contributes somewhat to making it a little harder for her to breath, as it would in anyone carrying a lot of extra water.

But, the bigger problem is the mucous that is in the back of her throat, too far to be suctioned. This is similar to the feeling that adults get when they have a cold and the phlegm settles just out of reach, making it hard to either swallow or expel. Julia's tries to move this mucous with huge bear-like growls and grunts, but is only effective at it when she is awake. She received an Albuterol breathing treatment yesterday, which seemed to help somewhat. But still, every time she goes to sleep for any length of time the mucous settles, and starts to occlude her airways.

Her pulse oximeter sounds an alarm when her oxygen saturation goes below 87%, and we rush to wake her quickly so she can move some more air. This is usually no problem, because she's not very happy at being awakened in such a rude fashion! I call these events "sats-attacks". They have caused no permanent damage to Julia, but I get a couple of more worry wrinkles every time we have one.

Dr. Kurtzberg says that the mucous buildup may continue in this fashion for another couple of weeks, but that Julia's overall condition right now is good. Julia continues to do things that she hasn't done in a long time, like look around at her balloons, purse her lips while holding her mouth closed for long periods of time, and relax her thumbs. She has not cried for more than 15 minutes in a day for a couple of weeks. She still hasn't had any sedatives or narcotics since August 29th. :-)

Some of the other Krabbe's babies are reacting similarly, exhibiting some of the behaviors they had earlier lost, although no one here has performed any new milestones that they hadn't already achieved before the onset of Krabbe symptoms. One theory is that the chemo drugs may eliminate some of the possible inflammation in the brain that may be caused by toxic buildup resulting from the missing enzyme. It's hard to say why the symptoms are less pronounced, but we are grateful for every day that Julia feels better. She's building cells rapidly. Today her white blood cell count is 3.4.

So, please keep your prayers and well wishes coming. We very much appreciate everyone's good thoughts.

~Julia and her mom

Julia's Journey Update #15 9/8/98 T+19

 Yesterday Julia's white blood cell counts reached the low end of the normal range at 3.9! Dr. Kurtzberg gave us the good news that we are now at the stage of reducing Julia's medications and working to get her ready over the next couple of weeks for life outside of the hospital. Of course, all of this is predicated on her continued improvement. We still are weaning her off oxygen, which continues to dip slightly every time we remove the cannula completely. But, we have now gone 24 hours without the sat-attack alarm going off, even during sleep!

We are still waiting on more wet diapers, although I had a 4:00 AM thrill when I rolled over on to a big wet spot. Yippee! That was a 330 ml diaper, absolutely huge. The more the better! I was happy about it even at 4:00 AM. Julia's weight increased yesterday again, but the X-ray showed that her lungs remain clear. Fortunately, this wee-pee problem has remained manageable, although annoying.

Dr. Kurtzberg agrees that Julia is looking better now than when she first came in, for whatever reasons. Last night we were able to leave our room and wander the floor in our stroller, dragging our 100 pounds or so of IV's and pumps with us. But, out of the room at last, for the first time in 27 days! Julia was bright-eyed and happy to see a new set of walls, and to meet some of mom's new friends, the parents of other kids also here for transplants.

Today Julia practiced holding her head up with the physical therapist's help. Julia really worked hard at this, as though she understands what a much broader world she will again be able to view when she can move her head of her own accord. She was very good at this when she was first born. Her physical therapist was amazed to see her progress in regaining this skill, along with the amount of motion she was making voluntarily with her arms. Later, when she was sitting in her red TV chair she dove forward while our nurse was watching. We were both astonished! I'm so glad I'm not the only person to see these Julia-stunts.

Julia also had the chance to get her 15 minutes of fame when Time Magazine sent some photographers and reporters to our floor. They are documenting some what happens at Duke University Medical Center for an upcoming October special issue on health care. The photographers took lots of pictures of her and many of the other patients, but we will need to wait and see if any of them make it into the issue. She acted pretty bored by the whole event and never really opened her eyes, and so may have missed her first opportunity for fame. No matter. This transplant will give her a chance to live a long life and have plenty of other occasions to broadcast her cuteness!

Today three of our floor friends left to begin their outpatient stay. It was so wonderful to see them ready to go, but, I must admit, I feel a little void when walking past their empty rooms. Allie, one of the other Krabbe's babies, was one of the kids to leave, along with Sarah, our immediate neighbor, and Andy three doors down. We all live so close together on this floor that it begins to feel a little like extended family. But, new kids will move into those rooms almost immediately, so we'll have the chance to get to know some new families. And we very definitely look forward to joining them in the outpatient clinic waiting room.

That's all for now. I'm off to take Julia on her second stroll, and see who new has moved in. Please keep those prayers and well wishes coming. We're doing well today, but we still have a way to go.

~Julia and her mom

Update 16 (9/10/98)

Julia's Journey #16 9/10/98 day T+21

Today is our one month anniversary of our arrival in the hospital, August 10th. Julia continues to move right along. Yesterday her white blood cell count was 4.4, and today it was 6.6! I have noticed a correlation between when Julia's counts are going up very rapidly and how much she sleeps. Generally speaking, the faster her counts are going up, the more shut-eye she gets. Yesterday and today she slept a great deal.

Sleep is a definite five letter word around here. It is always a topic of conversation: who slept when and how much, and why did they either sleep or wake up? At one of our parent support groups this topic took a new twist. Instead of discussing the kids' sleep we had a good talk about the parents' sleep, particularly how we sleep around total strangers.

This support group meeting was entitled "Thematic Imagery". We all sat in a darkened room, with soft music, and our social worker's crooning voice reciting sweet images of far away places. We were supposed to transport ourselves to these wondrous lands and relax our tension away.

Well, I knew within the first 20 seconds or so that this would be an hour of trying desperately to stay awake. Pinch, pinch, pinch. Sixty minutes later we were all supposed to rouse ourselves gently from la-la land and drop anchor gently back on the pediatric transplant floor. Then came true confessions! No one likes to sleep in front of perfect strangers, something we do all night long (if we sleep) as the nurses come in and out of our rooms. One mom was afraid she slept with her mouth open, and another wondered if the nurses talked about her snoring. I thought that it doesn't matter much in my case: I just never sleep!

But, as I said, Julia has been sleeping fairly well lately. So well, in fact, that today we noticed a bed sore on her back about the size of a half-dollar. It is located in a place where we almost never would see it, because she lays on her back most of the time due to the g-tube. When I hold her, bathe her, walk her in the stroller, and play with her, it is hidden from my direct line of sight. And, we may not have found it today, except... WHOOPS! I pulled out the g-tube once again yesterday. I had to post a "BEWARE: G-tube menace-mom in this room" sign on the door. Fortunately we have been trying to enlarge Julia's feeding capacity to prepare her for life outside the hospital. Dr. Martin kindly took mercy on this remorseful mom and called my faux pas an opportunity to have a larger gauge tube installed.

The flexibility of this larger tube made me less hesitant than usual to lay Julia on her stomach, so we decided to try it today during physical therapy. And, voila! We noticed that Julia has a great big bedsore about two inches above her tail bone. The good news is that she now seems to really enjoy laying on her stomach. Her sats remained very high as she lay on her stomach this afternoon, and she even occasionally made rudimentary efforts at raising her head. Julia and I had worked very hard at what we called "tummy-time" back in April and May, and never had any success. She screamed and became rigid back then. She accepted it very well today, and even eventually fell asleep.

We are still working on a couple of challenges before we can become outpatients. We are trying tonight to wean from the nasal cannula to blow-by oxygen. Julia must be completely off oxygen before we can leave. The blow-by set-up is giving me heightened incentive to get Julia well: it makes the very loud continuous noise of hissing gas. I much prefer the babbling brook sound of the last few days! Also Julia's water retention is too high. She needs to loose close to a kilogram of fluid. And last, Julia regularly gets an early AM fever, sometimes as high as 102 F or 103 F. These three challenges could be met in a matter of days, or a couple of weeks. It is very difficult to predict a time frame with transplant kids.

All of the staff and families on this unit work very hard to be upbeat and cheery, because it helps our children get better faster. We all know the seriousness of the situation, and the possible outcomes, both good and bad. Yesterday, another little boy on our floor, Colt, passed away. He was due to leave with Allie, Sarah and Andy two days ago, but took an unexpected turn for the worst. Colt was a very courageous young boy, and his parents are so loving and kind. We now must mourn his passing but still maintain a light-hearted mood for our own children, who are fighting the same odds.

As always, I thank you for your continued well wishes and prayers. It means a great deal to us to know that all of you are out there cheering us on to the big goal, life for these precious kids. Thanks.

~Julia and her mom

Update 17 (9/13/98)

Julia's Journey #17 9/13/98 T+24

Julia's white cell counts are soaring: 9.3 on Friday, 14.4 on Saturday, and 18.8 today! If her counts continue to rise so rapidly she will soon be able to stop receiving Filgrastim (G-CSF), the drug used to stimulate the body's production of blood cells. Then Julia's counts will likely cut in half, which is normal range. This is progress!

We still have the same three big challenges to overcome before we can progress to outpatient status. Julia continues to run a fever most mornings. Today it went up to around 101 F for a few hours. It seems to be due to cell growth, at which her body is definitely working hard. Nonetheless, we need to take cultures on a regular basis because it's hard to tell positively that the temps result from cells coming in and not from infection. Once the fevers disappear for a few days in a row, and all the cultures are negative, then we can discontinue her antibiotics. That would be one step closer to discharge!

The second challenge remains eliminating the sats-attacks. A couple of days ago Julia's right upper lung lobe showed some additional fluid. This condition is called "collapse", which sounds really awful, but only means that the fluid does not allow the lung to expand to the full space of the chest cavity. In a different setting it might be considered extremely serious, especially if the entire lung collapsed, but on this floor it is within the range of "to be expected". {Quite a range, that ;-} A subsequent X-ray showed the situation to be improving, but Julia still needs oxygen, due in part to the lung, and also to the continual mucous secretions in the back of her throat. The nasal cannula we were using caused a 2" long, but very thin blister on her cheek, so we had to go back to blow-by oxygen.

Blow-by oxygen could also be referred to as a "jet engine" if only the sound were taken into account. Our nice pleasant atmosphere now is reminiscent of a pneumatics factory. Julia is on a very low rate of oxygen, 28%. Room air is usually around 21%, so the machine has to suck in a lot of room air to mix with the pure oxygen to give Julia just the right amount. That sucking sound would make Ross Perot proud. It is LOUD. What did you say? I said

L O U D.

Even the chronic pump beeping is diminished to a distant tweet in relation to this sound. I am heartily inspired to do everything possible to encourage the mucous to go away, so we can resume our nice quiet abode in our little room. I am not complaining, mind you , about anything that helps our little girl survive. I'm only going on record as being wistful about the babbling brook sound of a week ago.

The third challenge remains fluid intake versus output. Two days ago Julia took in about 500 ml more than she put out, a huge amount on an already bloated baby. Yesterday she topped 19 pounds, the record so far. She only weighed about 15 pounds when she got here. We have tried a number of diuretics and other tricks, with still a few more to go. The cell walls that contain fluid become "leaky" after a time of continued onslaught from all the medications, so the fluid goes from the vessels where it normally is contained into the surrounding tissues. This is called "third spacing", and makes Julia appear very large. Even her hands and feet become hard with the major amount of fluid contained in them. Her tummy reminds me of a baby Buddha.

So, diaper-watch continues. I have taken to playing a new game with the nurses. I change her diaper and we guess how much fluid is contained within. Then it's weighed. The closest person gets a sticker. Lately the average weight has been about 40 to 60 ml. You'll hear me celebrating from wherever you are when we break her all time record of 330 ml!

And so, life goes on at DUMC in Durham. Thanks once again to everyone who sends their well wishes and prayers, they are so much appreciated.

~Julia and her mom

Update 18 (9/15/98)

Julia's Journey #18 9/15/98 T+26

This is definitely a place to put a mother's intuition aside. For almost nine years I have been honing my child-comforting skills, and I must have gotten pretty good at it, because now Julia is TOO comfortable. We play a wide variety of music all day to relax and grow neurons; we massage, we sing, we hug. The result: no crying for several weeks now. The problem: Julia is still not moving the mucous. Good lusty cries will help. The solution: Make her cry. Easier said than done! Julia's own sweet temperament is working against her in this situation.

Jenny, the wonderful physical therapist who does the inpatient therapy here at DUMC, showed me how to do respiratory therapy today. Julia's most recent X-ray showed that her upper right lung love has collapsed again. So we started on that side, cupping our hands and pounding rhythmically with a clapping or popping sound over that area of her chest for two minutes. Then her left lung. Then her right side and her left side, right back, left back, each with some shakes in between. The objective is to get the mucous and fluid moving.

When I first saw Jenny doing this I thought Julia would hate it. Not so! She loved it. She was even doing snake tongue (Julia's happy signal) to get Jenny to do more. It was difficult at first for me to imitate Jenny's motions, because it's counter-intuitive to pound on your baby. But, it became easier when I was how much Julia liked it.

When Julia sat up she was really wide awake, and gave Jenny a very long stare, sans nystagmus, with a hilarious expression that said, "WHAT were you doing to me?!" Julia held her head up on her own for many seconds, and purposefully turned her neck and eyes to look at me when I spoke. What a reward for a long hard day.

Even so, the mucous and sat-attacks, overabundant fluid, and morning fevers remain as much of a challenge today as they have been over the last couple of weeks. I missed the "dog days of summer" in Michigan. Instead, these are proving to be the "dog days of Durham" at Duke. It is difficult to retain cheery optimism with so little positive movement recently on these three issues. The doctors remind me, however, that Julia's counts are good (18.5 this morning), she is tolerating her feeds beautifully (completely off TPN!), and she seems quite happy and comfortable. I see significant improvement over her status from when she arrived, in both alertness and ability. Really, all we need to make my day are big fat diapers to clear the excess fluid!

As always, thank you to everyone who sends their well wishes and prayers. Maybe if we all dream of big fat diapers something will happen. Yellow has most certainly become my favorite color. As I said above, none of this is intuitive. First I want my daughter to cry, and then I want her to wet. So be it! For a long life...

~Julia and her mom

Update 19 (9/17/98)

Julia's Journey #19 9/17/98 T+28

 Yesterday was a great day. All that diaper dreaming must have paid off. Julia's output was slightly more than her input! She was awake and very alert most of the day, even uttering one syllable sounds, and definitely interacting with various people entering our room by using eyes, tongue, and hand movements. Dr. Martin even quietly mumbled something under his breath about "turning the corner" and the possibility of talking next week about a date in the future for discharge. Yes, a glimmer of light at the end of a very long tunnel!  

Today Julia's white blood cell count is 25.7, up from 20.9 yesterday. And, as always, when Julia's white count skyrockets she is sleepy. It has been hard to get her to keep her eyes open all day long, which also means that she is not clearing her throat. So, her sats are a little lower again. She also has a mysterious lump under her left arm that comes and goes, possibly related to big white counts and her lymphatic system. The lump seems to come at night after Dr. Martin goes home, and leave when he sets foot in the building. I am always very careful to have the nurses check the lump when I feel it, just to get a third party opinion.  

Today we get to increase Julia's feeds to 45 ml every 3 hours, and remove even more meds: completely off Heparin, decreased antibiotics and growth factor. I estimate that the IV pole now weighs about 80 pounds, instead of 100, and is getting easier to push on our nightly walks up and down the hallway. I, Med-Mom, am working hard to learn the nuances of how to understand the gurgles one hears in a stethoscope, so I'll know what I'm listening to when we are outpatients. The respiratory PT I need to give Julia every two hours or so throughout the day not only shakes that mucous loose, it's getting my arm in shape too. The other area I keep massaging is her cheeks, ever upward, waiting for that next smile.  

Julia finally got to play on the big ball today, the one that Alison and Cameron so admired when our Michigan physical therapist first showed it to us in July. Julia worked very hard to move her head ever so slightly, and was able to do it when positioned on her ball! Once again this little trooper astonished us. She is holding her head up more and more frequently, with big bright blue eyes open, eyebrows knitted together, and an expression that says, "This is SO hard, but I'm going to do it!" I managed to get a couple of pictures of this today, and will net-post them as soon as they are developed. Her trunk control is still negligible, but we are learning exercises to turn those little soft spots around her neck and spine back into muscles. Lately Julia's physical therapy sessions have been the highlight of our day. She does something new and different each time we work with her, giving us reason to be optimistic about the future. 

Julia will have the amount of protein in her spinal fluid measured next week, probably on Tuesday. The lack of galactocerebrosidase (the missing enzyme in Krabbe's disease) causes this protein count to be abnormally high. If the cells that Julia is growing so rapidly are the donor's cells, then hopefully Julia's protein count will be lower than it was when she began the transplant. This would indicate that some new galactocerebrosidase is entering her brain and breaking down some of the byproducts of myelin production. We would be able to take this as the first scientific proof, other than our subjective observations of her increased activities, that she is truly getting better.  

But for now, we are still on diaper-duty and sats-watch. Yes, we still need to hope for big diapers. The rest seems to be coming along very nicely, as of right now. Thank you all as always for the many prayers and well wishes. We very much appreciate your good thoughts. It's working!  

~Julia and her mom

Julia has been having more fun than she has in a long time. She is moving a lot more, making more vocalizations, and keeping her eyes open more frequently. Yesterday her counts were 28.8, and today they are 29.6! We were prepared for a big drop due to cutting the growth factor two days ago from 10 to 5, but haven't seen it yet. So now we're going down to 2.5, on the way to zero. Julia's hemoglobin count has been holding its own in the 10 to 11 range for a few days (10.5 to 13.5 is normal). This means that she doesn't have to have as many transfusions, hence less fluid intake, making it easier to balance intake and output. All good news!

Yesterday Julia had me engaged in a game. She would hold her arm up, bent at the elbow, and wait for me to kiss it. I would kiss it, and she would put it down to her side.

Then, she would put it back up, wait for me to kiss it, and put it back down. If I hesitated she would say "ugh" in a pretty ornery fashion until I followed her directions. At first I thought it might be coincidence, or some new kind of reflex, but she did it 34 times in a row! Towards the end she had to struggle, and I could tell she was getting very tired. Finally, after the 34th time she fell fast asleep. I feel that she is trying to duplicate a game we used to play when she was about three months old, only at that time we did it with both hands, and lots of smiles. We're getting there.

Julia had so much fun on the physical therapist's ball that we bought a similar one at a local toy shop. Now we can practice as long as she can stay awake, pushing up, building back muscles. She can hold her head up for a minute or two at a time with this new strength, when her trunk is supported in a sitting position. This is such an improvement. It allows us to walk around with Julia in her carrier, giving me free hands to hold cords and push poles, without needing to worry so much about a floppy head.

If you download the attached picture you will see Duke Blue Devil Julia sitting on mom's lap in the family room at the new computer. She is holding her pink jacks, which she has in her hands almost all the time. They are light so that she can easily lift her hands, but they still keep her thumbs in the right position. She almost never puts her thumbs tightly to her palms anymore, which is a vast change from before the transplant.

You will also see that Julia's eyes are closed, and she looks like she's sleeping. In fact she is wide awake, but still won't open her eyes in bright lights, or when there is a lot of commotion. She keeps them open about 2 hours a day in total, but only with indirect lighting. I couldn't take the picture with dim light, so we have bright light and closed eyes. Although her mouth is open in this picture, she now keeps it closed most of the time, again in large contrast to a few weeks ago. At that time chapped lips were almost always a problem, but no longer. And note the hair doo, about 15% of its former volume, but Clairol would be proud of the color.

Julia's sats and fluids are slowly improving. She is still on blow-by oxygen, and her sats still drop when it is removed, although not to quite the same extent that they did in the past. Her I's and O's (Intake and Output) are closer to balancing so that we have been able to increase her feeds again. She is no longer on continuous feeds during the day.

All of this is in the right direction to leaving the hospital, hopefully in a week or two!

Please keep your prayers and well wishes coming. As we become ever more hopeful about gaining outpatient status it is important to remember what good fortune we have. Another special little boy on our floor, Brandon, passed away last night. Always we are reminded that we mere mortals are not omniscient, and need a little divine help to do the extraordinary.

~Julia and her mom

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All images and text copyright 2000 Wendy Beatty. All rights reserved.