Hi! The Beatty clan is finally back on line, and anxious to let everyone know how Julia is doing at Duke. We have set up a mini-office here in the hospital room and will send out an update on Julia's progress with each milestone. We would love to hear from you, so please note the new e-mail address, and let us know how your summer is going!

 We arrived in Durham two weeks ago, Wednesday, July 30th, and began some intensive testing on Thursday and Friday. On the weekend we participated in a Krabbe's Kid conference at our local hotel, where three Krabbe's babies, Judson {TX}, David {NC} and Julia {MI} met while their parents asked each other at least 100 questions about life in other Krabbe households. Julia had her MRI on Monday, August 3rd, the test that we determined ahead of time would indicate whether or not the bone marrow transplant would really be a possibility.

 Julia moves very little, does not follow with her eyes, can not coordinate swallowing with eating, and generally appears to have a rather advanced case of Krabbe's. We were prepared for the worst. Julia's MRI showed a fair amount of damage in her cerebellum, the area of the brain that has a lot of control over motion, as well as certain other functions. But, there was NO damage in her brain stem where many primal functions are controlled, or cerebrum, where most cognitive thought occurs. A MIRACLE!!! The news was the best possible for us, and a certain indication to go ahead. We know that even with this very good MRI result there are no guarantees about the result, but are committed to doing the best we can to have a good outcome.

Alison and Cameron's HLA typing showed them not to match Julia for a bone marrow transplant, but a cord blood match was found. We had Julia's central line surgery on Tuesday, 8/4, but needed to wait until the following Monday, 8/10, for a bed to open up in the Bone Marrow Transplant floor for chemotherapy to begin. The transplant happens 10 days after chemotherapy begins, so Tuesday, 8/11 became day #-10 when we began busulfan treatments at 4:00 AM.

Julia has tolerated the first two days of chemo fairly well, sometimes happy, and sometimes pretty irritable. :-) She gained about a pound with the extra IV fluid, but other than that is doing quite well. She's sleeping soundly beside me right now, and ready for another day tomorrow on the way to wellness.

Rob, Alison and Cam are enjoying their visit with us, swimming in the hotel pool (the air conditioning was out for two days at the hotel), decorating Julia's room, and playing lots of video games. The will leave for home in a few days, and won't likely be back down. Julia and mom are firmly entrenched for the duration: a couple of months in the hospital and a couple of months as outpatients. We're looking forward to being home by Thanksgiving!

As I said earlier, I will send updates with each milestone met, and I hope also to hear from you. So please note the new address and send us your hello's when you get the chance. Let's keep in touch since technology makes it so easy!

~ Julia & Wendy

Update 2 (8/14/98)

Julia update #2 8/14/98

We are at day #-7 in our cord blood transplant, and thus far Julia is tolerating the chemotherapy rather well. She remains rather sleepy due to the medications she's taking, and sometimes irritable, but also has some good alert time. She LOVES riding what Cameron calls the "craft-matic" bed, with Cam at the controls: ^UP^ |DOWN| >FORWARD> <BACK< over and over again! We're hoping he doesn't burn out the controls, but Julia's physical therapist says it's good for her to get her blood circulating. This is one for the video library!

As well as Julia is doing in general with the chemo, it is still possible to see the progression of the Krabbe's, probably somewhat exacerbated by the chemo drugs. For example, yesterday one of her pupils became much more dilated than the other, but we believe this and other symptoms such as tongue fluttering will diminish after the treatment. Always there is a reminder of the gravity of the situation, despite the gravity defying acrobatics she and her brother are performing on the "craft-matic". Alison made a sign for her door that reads "Wanted (by as soon as possible) A cure for Julia!"

The hospital works hard to accommodate the parents of transplant patients. They bring breakfast, lunch and dinner on trays to the room, as well as providing meal tickets to the cafeteria, so we are well fed. They have available in the family area a kitchen stove, washer/dryer, and shower in the family area, along with many videos. They involve us quite heavily in our children's daily care, and keep us well informed on their progress. They really seem to go out of their way to help make a stressful situation as good as possible.

Alison and Cameron are really enjoying their 'vacation', as they call it. Cameron has nearly overworked the Sega Genesis machine in Julia's room. We don't have video games at home, because as Cameron knows our TV is too "old", at least as far as video games are concerned. ;-) So, Cam thinks that North Carolina is the vacation hot spot of the world. They have video games here as well as 'craft-matic' controls: What more could a six-year-old boy want?!?! Alison is spending as much time as she can in the whirlpool and pool at the hotel. What a novelty, swimming without the turtles nibbling at her toes.

Thanks again for all the well wishes!

~Julia and her family

Update 3 (8/16/98)

Julia-News Update #3 8/16/98 day -4

Well, below is the update that I was going to send yesterday, but then, just after Julia and I finished looking out the window I accidentally pulled out her G-tube (feeding tube that was inserted through the wall of her stomach at UM on 7/17). Whoops! The good news is that although mom feels pretty sheepish, taking the G-tube out doesn't hurt, but is somewhat like removing a pierced earring on a bigger scale. Julia didn't seem to notice a thing, but we did have to call in the doctors to reinstall a different tube.

Today Rob and the kids left to return to Michigan (11 hour drive), to go back to work, and let Julia recover without sibling germs. This is important during the worst of the immune deficiency created by the chemo, which allows her body to accept the new stem cells she will receive during her transplant.

Tonight Julia had a wonderful period of following voices, albeit slowly, with eyes and head, and moving her arms. This is not evidence of the beginning of a cure; that's a long way off, but rather a brief moment when the neither the chemo or Krabbe's were enough to prevent her from doing a little bit of what babies are meant to do. Alleluia for these precious moments! 8/15/98 Julia has started losing her hair, beginning with the eyelashes. This sounds sad, and it is a bit to see those really cute spun bronze Gerber Baby locks falling out, but we're viewing it as part of the road to wellness. I took a lock of Julia's hair before we began, so we'll always have that part of her, but we much prefer the new doo if it's a symptom of progress. Actually, the other kids want to stick around until it all falls out so that they can "soft" her cute little bald head!

She has finished with her first chemo drug, Busulfan, and is moving on to the second, Cytoxan. It is amazing to see how many different medications, procedures, processes and so on accompany each stage. For this stage an EKG must be done each day to monitor heart function, a catheter installed to prevent irritating buildup in the bladder, CV monitor during infusion... each for a good purpose. The phrase "WIRED" takes on a whole new meaning!

Another new implement that Julia just received is a set of thumb splints. One of the earliest symptoms of Krabbe's is clenched thumbs. Julia's occupational therapist custom fit her with a set of splints to keep her thumbs nice and relaxed. This will allow the muscles to develop properly to more easily enable grasping down the road. She really seems to like them, or at least not mind them.

We seem to have figured out the appropriate sedative dose level for the moment and she is awake, alert, and only a little irritable. She is enjoying sitting on my lap, looking out the window, munching her mint-flavored sucker-sponge, and watching mom try to type with one hand. Life is good!

Much thanks for those prayers! They ARE working!

Love Julia and her mom

Update 4 (8/18/98)

Julia-News Update #4 8/18/98 day -2

 Julia is nearing the end of the chemotherapy, meaning that most of her bone marrow has been removed in preparation for new marrow that will allow her to produce the missing enzyme, GALC. Yesterday she began a different drug called ATG. This drug is made from horse serum, and destroys the last part of the immune system that would otherwise reject the new stem cells she will receive in her transplant. For the next several weeks the talk of the day will be "counts": The chemo has driven her white blood cell count down 1.5. It should be at 0 by transplant day on Thursday. This means that she will have no remaining white blood cells, which is one of the main defenses our bodies have against disease. In this case they must be removed so that the new cells Julia will receive on Thursday will be accepted. The side effect of this is that she is now very open to infections of all kinds, so much of the daily routine is spent preventing her from coming into contact with germs. The fight against germs takes on a whole new dimension here! To enter this unit you must first press a button to open a large door and enter an anteroom, where you must wash your hands thoroughly for 15 seconds. One of the patients made a sign that read, "IF YOU HAVE HANDS YOU MUST WASH THEM!" Then you press another button and a door on the opposite side of the room opens and you enter the Pediatric Bone Marrow Transplant Unit, or PBMTU for short.

All of this sound perfectly normal until you're in a hurry. The anteroom creates something of an airlock, so one door can't be open until the other door closes. And, these automatic doors are very s-s-l-l-o-o-w-w. Some humorous situations have been witnessed by this mom as people in the know have seen other people coming from one direction, when they want to go in the other direction, and have had to race to hit the button before the other party gets to the other door. Whew! And, these are grown-ups, too! ;-)

Julia has weathered all of this quite well so far. The ATG is frequently the worst of the pre transplant regimen, but so far her temperature has remained low, and there has been relatively little rash. Her cute little hair doo is taking on a new thinner appearance, but hair grows back with a growing child. I'm not washing it these next couple of days because it falls out so quickly when I do. She no longer smells like a baby girl, but rather like a new chemical soup! Her doctor says she's right on track, and we're all very happy. She's resting next to me right now, looking at her big toucan balloon, listening to a little Bach, getting better and better!

Thank you all once again for all of your well wishes,

~Julia and her family

Update 5 (8/19/98)

Julia-News Update #5 8/19/98 day -1 

Leopard Baby is here! The ATG (immune system suppressing drug that Julia began on Monday) frequently causes a spotty hive-like rash, which Julia has. She's receiving numerous medications that reduce the symptoms, so she's rather sleepy and not much bothered. We feel that Julia has simply learned that the world is a jungle, and has modified her appearance accordingly so that she fits in. ggggrrrrrooooowwwllllll !!!

Leopard Baby Julia had a bit of fun tonight. I put a tiny taste of frosting off of my birthday cake on her tongue. WOW, what a sensation! Remember, she's never eaten any food, and has only ever tasted breast milk and medicine before she got her G-tube. Her eyes were wide open and her little tongue was nearly flying in and out of her mouth with this taste treat! If I keep this up I could have a lot of coaxing to do someday when I start her on peas and carrots. 

We have been lucky with the ATG in that we did not get the high fevers frequently accompanying this drug. Even the leopard spots are rapidly fading. Julia is receiving her last dose of ATG now, in preparation for the big event tomorrow:

 TRANSPLANT DAY!!!

Julia-News Update #6 8/20/98 Day 0 - Transplant Day!!!

We did it! Julia received her new cord blood stem cells today at 10:15AM. All went well. As a matter of fact, she had a fantastic day all around! She was awake and alert throughout the entire process, which is somewhat anticlimactic in relation to all of the chemo.

A bone marrow transplant is not an operation like surgery. It is more a case of many hanging drip bags, all performing different functions to prepare the body to accept new cells when they are dripped in. The actual event consists of hanging one more drip bag on the pole, and letting it drip by gravity into one of the central lines over the course of 20 or 30 minutes. The hard part is all of the pre-transplant tests, chemo, ATG, and post-transplant balancing act to fight infection and avoid GVHD (Graft Versus Host Disease).

The transplant itself may not be dramatic, but it is very significant. It has only been a few short years that doctors have known that umbilical cord blood actually contains more of the all important stem cells than bone marrow does. Once the stem cells enter the body, provided they aren't rejected, they will multiply and grow, eventually allowing Julia's body to produce the enzyme that her errant DNA decreed she would be missing. Before today there was no chance that Julia would survive. NOW THERE IS A VERY SIGNIFICANT CHANCE THAT SHE WILL LIVE!

As I said, that little drip bag may not be dramatic, but it's a definite cause for celebration. We took lots of pictures, hung up a very nice certificate, and are saving the bag in which the blood arrived. Julia must know what a happy day this is, because she was in a super mood all day long. She was alert, following my voice and moving her arms until evening when she settled down for a little nap.

An interesting side effect of the transplant will please anyone who likes creamed corn, which is one of Julia's dad's favorite vegetables. The preservative that the cord blood comes in makes the kids give off a VERY powerful creamed corn odor. It's so powerful that you can smell it outside of the room in the hall. In fact, one of the nurses on the unit calls transplants "corn-bakes" because the aroma is so obvious! Julia smells like about 20 cans of creamed corn cooking at once. Ummm Ummm Good!

Today was a great day. Dr. Driscoll has warned us that there will still be some hard days ahead of us: avoiding infections, fighting the side effects of chemotherapy, dodging GVHD. We know all of that, but we also know that as of today the tools that Julia's body needs to survive are in place. So we are even more thankful for today's nice moments.

Keep those prayers and happy thoughts flowing!

~Julia and her family

PS: Take a moment to check out our beginning web sites, attached to Haley's and Joseph's sites. Look up the following pages then take a peek at some of the world's cutest kids: Julia, Judson and Allie, along with several others! www.Krabbes.com/haleys_hope/ >>>go to the bottom of the page for our kids' links home1.gte.net/robmil/bb.htm >>>for stories home1.gte.net/robmil/children_iii.htm >>>for pictures BIG thanks to Haley's and Joseph's parents for adding the Duke group to their sites!

Update 7 (8/22/98) 

 Julia's Journey: Update #7 8/22/98 day +2

 Julia has had several good days in a row. Yesterday she still smelled a little like creamed corn, but more like a "corn kernel" as one friend put it, rather than a whole pot of it. Today that odor is gone, but the beginning stages of mucositis have set in. Mucositis is the inflammation of most of the mucous linings of the body with red sores, a side effect of chemo and all the different drugs. The kids who can talk report it as very painful. We are required to do mouth care four times each day to help lessen the effects of mucositis.

Mouth care consists of swabbing the inside of Julia's mouth first with sodium bicarbonate (yuck), then with Periogard mouthwash (double yuck). The funny part is that Julia loves it! She could be the poster-child for mouth care here, so we spend an hour or more each day swishing the sponge this-a-way, that-a-way, massaging her gums and staving off mucositis. I really wonder what this kid will think when she starts eating real food!

Many of us assumed before we arrived that we would be very bored once we got here. In fact, we are kept quite busy performing myriad tasks in the care of our children. Bored I'm not! We sure keep busy. This is something like being in a Bone Marrow Boot Camp, or BMBC for short. You are pretty well consumed by it. When you walk outside you immediately say to yourself, "Oh! There IS life off the unit! Wow!"

There are really three different categories of information you need to learn about when you enter into a program like this:

1. Your responsibilities and all the rules you need to follow. The parents are given a fair amount of responsibility for caring for their children, which we feel is a wonderful advantage to the child. The parents continue to do the day to day tasks of amusing, diapering, hugging, bathing, loving the child. This gives the child a sense of continuity. But along with it come various rules and protocols, such as using rubber gloves to change diapers during chemo, mouth care on a schedule, picking the child up without damaging all the tubes and connections, incessant precautions to maintain sterility, changing feed tubes or dressings or whatever, etc. This takes up a big portion of the day, and night too.

2. The medical terminology and information pertaining to your child's illness, and treatments. This is especially imposing for people who don't have medical backgrounds (I do not) but who want to understand what is happening and why. Merely pronouncing some of the pharmaceuticals is daunting! But, as time goes on we are all beginning to feel like we have taken on a new career and can recite stats with the best of 'em.

3. Hospital life itself: routines, hierarchies, who to talk to and where to go to take care of necessities. Where do you get stamps? Is there a sign up sheet for the washer and dryer? What's the difference between a P. A. and N. P.? When do you beep the nurse, and when do you push code blue? When do the doctors usually do their rounds? What can you put in your room (NO live plants or rubber balloons)? It takes a while to get the routine fully down pat.

And, after all, this is just a parent's perspective. Julia can't talk, but I am certain it's a lot more intense for her and for all the kids here than it is for us. We grown-ups get our hour or so a day to reacquaint ourselves with mother earth; they never leave the floor. So, with that in mind I'll sign off and begin another round of mouth care. Keep those thoughts and prayers coming, for increased counts and cell growth, and decreased mucositis.

~Julia and her mom

Update 8 (8/24/98) 

Julia's Journey: Update #8 8/24/98 Day+4

We've reached the rocky road part of this journey. Yesterday Julia began the morning with a small hoarse cry. An hour later she had spiked a fever of 102.2 F, which is extremely high for a girl whose average temperature is around 95 F. The mucositis has set in. Chemotherapy works by destroying cells in the body that regenerate quickly. That's why it is effective against cancer, which is defined as cells growing out of control, and bone marrow cells, which are normally some of the fastest growing cells in our bodies. Another group of cells that regenerate quickly are those that generate mucous, lining the digestive tract from the mouth to the rectum. One major side effect of chemo is that these relatively fast growing cells are damaged too, a condition known as mucositis.

There was evidence of the onset of mucositis late on day +2 in Julia's diapers, and it's now clear that she has a pretty advanced case. So far we can see no signs in her mouth, happily, due to all that mouth care. But, the site of her g-tube is quite inflamed indicating that the mucositis probably has hit the lining of her stomach and caused an infection.

So now Julia is an example of better living through chemistry! We've had to completely stop feeding her through her g-tube, because the lactose in the milk causes the mucositis sores to be very painful. Instead we have an amazing array of machines hooked up to do everything for her. There are six large pumps and three drip bags on the pole beside her, and no more room. If she needs another machine we'll need to start pole II.

You can get a feeling for what it takes to get these kids through this stage by looking at Julia's diet of medications and drips for today:

Dripping into her using gravity drip bags:

Julia is receiving Fentanyl in her PCA pump. One of the side effects of Fentanyl is listed as "confusion". Do you think the confusion is on the part of the patient or the parent?!

My objective here was to provide everyone with an idea of the complexity and balancing act of this operation. As I look it over myself I am even more amazed when I see it on paper, and I am sure that there are probably a few items I've missed. According to our doctor, the "bad" days are usually #7 through #11. We're only on two antibiotics now; it is usual to have five or more!

Our white blood cell count is still <.1, and it may take as little as a few days up to a few weeks to start having a count. Pray for white blood cells to get here quickly, before the pharmacy runs dry!

Love to you all, and thanks for your prayers and well wishes.

~Julia and her mom

PS: UPDATE! In just the few minutes it has taken me to write this we've added three more drugs to the mix: Lasix, Benedryl and Tylenol. And, the doctors haven't even been to our room yet this morning on rounds. I think I'll go have breakfast.

WHOA! Update again! New arrival of PRBC's, Packed Red Blood Cells, to go under the category of gravity drip bag mentioned above. Julia's hemoglobin count was low. I need to type and run soon before something else shows up, or I may miss lunch too, much less breakfast. :-)

 Update 9 (8/26/98)

Good morning from sunny Durham, North Carolina! Yes, the sun is actually casting a shadow outside right now, despite hurricane Bonnie just off shore, only a couple of hundred miles from here at this very moment. I can see some high bands of clouds out my window, but no raindrops within viewing distance. Without becoming too philosophic, it seems that there are parallels that can be drawn between today's weather and daily life. Imminent danger just off shore, but no more than a hint that something might be up: an apt description of Julia's symptoms prior to diagnosis. I don't want to be paranoid, but I probably will overreact to every health related symptom my kids have until they're at college and I can't see what they're up to anymore!

Julia's mucositis seems to have abated, and she has been a very happy girl yesterday and today. She has been doing some incredible things too: following me with her eyes and some slight neck motions, reduced nystagmus (back and forth twitching eye movements), lifting her arms, one syllable verbalizations, and general alert contentedness! This is really amazing for a girl who virtually could not move when she got here. Yesterday she only cried for about fifteen minutes all day! I would think I was imagining things, but even her Physical Therapist was amazed at Julia's range of motion. Jenny was able to get Julia's hand to her mouth, whereupon Julia really went at licking her fingers. In the past she became too rigid to bend her elbow that far. Julia's newborn fencing reflex was somewhat reduced (this is good), and she was making rudimentary efforts to hold her head up when we did our sitting exercises Whoopie! I was telling all of this to Dr. Driscoll this morning, who was acting just a little dubious, to say the least, but humoring me in a good natured way. Then when he spoke and Julia looked right at him with big blue ALERT eyes I think even he started to become a believer! :-)

Of course, all we have proved is that Julia is feeling better, not that a cure has taken place. We have reduced her Ativan (sedative) to an even smaller amount (.05 mg 3x/day) than it as been, and she has had no Fentanyl (narcotic painkiller) for more than a day. Hopefully we are over the hump on the mucositis, although Dr. Driscoll, ever the bearer of good tidings, continues to warn me that days T+7 through T+11 are usually the worst. Our white blood cell count is still at zero, and there are no overt signs of engraftment yet. She is still fighting the infection at her g-tube site, which remains red, but not pussy. I learned the hard way here that puss is composed of white blood cells, which Julia doesn't have right now, so infections look significantly more benign than one would expect, but definitely a cause for concern.

Still, it's hard to be anything but optimistic. Dr. Wenger, the guy who does all the testing for this disease, told me yesterday that galacocerebrosidase, the enzyme Julia is missing, becomes measurable only a couple of weeks after transplant. It's easy to extrapolate that maybe some enzyme might already be where it needs to be. Yes, let's be cautiously optimistic, and wait for those white blood cells to increase.

Meanwhile, let's just continue to enjoy the day. Eternal thanks to all of you who are sending best wishes and praying for cells.

~Julia and her mom

Update 10 (8/28/98)

Julia's Journey: Update #10 8/28/98 day +8

We continue to have some great days while waiting for our counts to come in. Yesterday, again, Julia astounded and amazed both mom and her physical therapist by showing even more flexibility and alertness than in the previous couple of days.

She was bending her arm at the elbow just a bit, and easily allowing us to help her get her fingers to her mouth without becoming rigid. Her eyes are remaining fully open for long periods of the day, and she was moving her lips in a "smacking" motion without the sound. It is so wonderful to see her doing these motions!

We are working hard on her ability to track objects. Yes, she's definitely back to watching the Weather Channel, wide eyed and full of fascination. If she could talk, she would happily tell you that Hurricane Bonnie was a bad one, but that it missed Durham by just a few miles. For two days we watched the clouds swirl on TV and overhead, but received only a few raindrops and a little breeze. The TV doesn't move like the clouds do though, so it doesn't help her to learn tracking. For that we have balloons.

She first received a big red toucan with brightly colored feathers a week or two ago from the child life specialist here. I tied it to the table at the foot of her bed. The air filtration system drafts here make Toucan fly back and forth and bob up and down. At first I thought I was seeing things, but it's true! Julia really does track Toucan! Her eyes still flutter back and forth, but it is easy to see that she really makes the effort to follow where Toucan is going.

So, yesterday I added Minnie Mouse to Toucan. Minnie's balloon is mostly blue and a different shape. Julia definitely seemed to notice the variety. We'll do whatever it takes to get a few additional neurons firing!

We are still less than .1 white blood cells, but may be experiencing our first indication of something in the works. Julia woke up this morning with a fever of 40.4 C (104.8 F). There is no discernible infection, and she's already on several different antibiotics (standard around here), so there's hope that she's beginning to engraft. If she gets one new cell for every hair that falls out then we should be out of here in no time. Every surface she lays on takes on the appearance of an angora rabbit: very very furry. I won't let anybody touch the top of her head, nor will I wash it, so she still has her little cardinal's peak doo right in the front. Fortunately she is just as cute as a baldy as she is with hair, so no matter.

~Julia and her mom

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