Julia's Journey #21 9/20/98 T+31

Julia has been having some great days and doing some fun things, as you have been reading in my last few updates. Unfortunately, she had a setback this morning. I received many, many phone calls today regarding Julia's status, and I thank you all for your concern. The following chronicles the events of the day and should answer some of your questions. For those of you who prefer the bottom line first, the answer is that while Julia does not appear to be in any immediate danger, her situation is considerably more complicated than it appeared a day ago, when she seemed well on the road to mending.

Julia woke me with a sat-attack at about 6:00 AM, which is nothing unusual. But I noticed that every few minutes her legs would twitch slightly, and unlike clonus, neither the position in which I put her nor massage and pressure would stop the twitching. Yet, the twitches were small and subtle, and several minutes apart. By 8:00 AM they were somewhat more pronounced and closer together. The nurse and I watched her intensely for a few minutes. When I tried to rouse her from her night's rest she remained nearly completely unresponsive. This was a definite indication of something unusual. We called the doctors in to examine her.

Julia was not exhibiting any obvious outward signals of distress, but several neurological signposts such as pupil dilation were diminished. After discussing their findings the doctors decided that we should do a CT scan to see what was happening on the inside. So, we were wheeled down to the CT scanner, as it turns out, by someone from our home town in Michigan.

The CT scan showed that Julia has a large accumulation of extra fluid on her brain. The doctors were able to determine that this was very recent, because there is no evidence of damage in her retinas, which would occur after as little as a day or two of extra pressure. The cause of Julia's excess is unknown, but they do not believe it has anything to do with the extra body fluids about which I have so often written. In any case, her body fluid balance has been close to normal for the last couple of days. The diffusion of fluid on the CT scan appears somewhat similar to that of a child with meningitis, which is an inflammation of the lining of the brain that stops the clearing of unneeded fluid, although Julia does not exhibit other symptoms generally associated with that disease.

By this time we began seeing other external symptoms, such as bent knee rigidity, which she has never exhibited. The neurosurgeon examined her, and discussed doing an internal shunt to drain intracranial fluids to her stomach. This would have been a permanent solution to an unknown problem because the shunt would likely remain in place for the rest of her life. Why do brain surgery twice, once to put in, and once to take out, if you can avoid it? Ultimately it was decided that Julia would have a lumbar puncture (spinal tap) to drain and culture the fluid. This would remove some of the pressure, give us a sample to send to the lab, and buy us some time to look for possible causes.

Julia had the lumbar puncture this afternoon on the bed next to me while I began typing this. {I needed to stay out of the way, but didn't want to leave.} The doctor doing the tap told me that although they would check the protein in this fluid it is likely to be higher than normal, so I should not get my hopes up for a great result. Julia will probably end up with several spinal taps to drain fluid between now and the shunt. Normally this is a painful procedure. Julia did not receive any anesthesia before the puncture, and she did not react what so ever during the whole ordeal.

The test results are back, and Julia shows no sign of infection. Her protein count was 263, as opposed to 154 when we arrived for the transplant, not a good result, although possibly due to thicker viscosity of the fluid. The pressure was very high, in the 40's, where the mid teens is normal. The technical term for this condition is 'communicating hydrocephalus'. There is no evidence of any infection such as would be present with meningitis.

The decreased pressure as a result of draining the fluid will hopefully allow Julia's brain to resume the "normal" status of the past few days, as opposed to her current non-reactive state. When she becomes more aware she should be quite upset and very much in pain. So, today the prayers are for crying and irritability and wakefulness. A tearful, crying, awake Julia would make me very happy right now!

~Julia and her mom

Update 22 (9/22/98)

Julia's Journey #22 9/22/98 T+33

A small sigh of relief: Although we have not yet found the cause of Julia's excess cranial fluid, we are able to address the symptoms. As a matter of fact, we are again addressing the symptoms at this very moment, 3:03 AM, when Julia is receiving another lumbar puncture right next to me, again as I type.

Yesterday after the first puncture Julia began reacting to her surroundings at about midnight, when I received a most welcome "I love you Mom" in the form of a tiny snake-tongue motion from my wee one. The next morning she was very groggy in appearance when her neurosurgeon appeared in our room around 8:00 AM, but gave definite signs of awareness. We took our first excursion off the floor to go get a CT scan sometime after that.

It is worth a minute to describe how one "leaves the floor" these days with a child in Julia's condition. It's a little more complicated than merely getting out of the airlock. First we unplug the pole, putting the pumps on batteries. Then we pick the child up (without pulling out the G-tube ;-). Then mom sits on the wheel chair with babe in arms, while the oxygen is hooked up and protective air filter mask is put on. All the pole cords, G-tube, and oxygen paraphernalia are aligned. The attendant then pushes pole and mom/babe combo in wheelchair out the airlock, while mom carefully holds mask, tubes and baby in place. This takes about 30 minutes to accomplish, the same amount of time it takes to get a whole class of elementary kids ready to play outside in the snow. Think of us the next time you just walk out a door to go somewhere! When we got to the hallway by the patient elevators, Julia opened her eyes widely to look around. What a sight for my sore eyes. She got to watch us ask the other patients to get off the elevator, because Julia still needs to avoid close confinement with people who may harbor infectious germs.

Finally we arrive at... whoops!... the X-Ray machine. The scheduling was a little mixed up, but since we were here we took the chest X-Ray we needed but normally did back in our room. Then back to the room to wait for the CT machine to become available. Then, pack 'er up and off again. Then back to the room. Then waiting, then pack 'er up one more time for the MRI.

A wonderful side benefit of the MRI was that Julia had to be disconnected from her pole. MRI, or Magnetic Resonance Imaging, entails being placed inside a machine with very strong magnetic fields. It is imperative that there be no iron-based metals anywhere in the MRI room, or they and anything attached will go flying, sort of like the junk yard car-wreck magnets you see on kid's construction videos. (I'll lend you ours if you haven't enjoyed this viewing pleasure. I recommend 'Heavy Construction Equipment', available at your local video store.) So, we had to leave the pole behind, clamping off the G-tube and central lines. WHOOPIE!!! I have not picked Julia up and flung her over my shoulder sans dangling accouterments in a long, long time! We were twirling in the hallway, making our neighbors envy our freedom.

As the day wore on Julia became much more interactive with her surroundings. By mid afternoon she was at about the same level she was two or three weeks ago. After the MRI she wanted to play hand-kisses, like she did a couple of days ago, with wide blue eyes and lots of snake-tongue. But, her legs were beginning to stiffen and her eyes were becoming more disconjugate (not functioning together). By 2:00 AM her blood pressure was rising and her heartbeat was becoming somewhat erratic, signs of increased pressure due to too much spinal fluid. The stage was set for the 3:00 AM lumbar puncture mentioned above.

zzz (couple of hours of sleep)

Time has passed, and it's now afternoon. We just got back from our bone marrow aspiration, from which we will know whose cells Julia is growing so rapidly. Pray for the donor's!!! Unfortunately, the test results won't be back for about three weeks. As usual, I scrubbed up to watch the proceedings. Dr. Martin told me to tell all my friends that I stayed for the whole event and didn't become squeamish. These guys are going to give me my honorary medical degree yet. Personally, though, I think that medical school would be easier and definitely more lucrative.

So, the way we stand this afternoon after talking with the neurosurgeon, team of four neurologists, and the usual transplant doctors is that we will hold the same course for now. The risk of infection from inserting either the permanent or a temporary shunt in Julia's current condition is too high (somewhere between 10% and 30%) unless we know for sure what is actually causing the problem. We will continue to receive lumbar punctures to relieve the pressure on a periodic basis. I am carefully watching all the behavioral signs, and of course the usual vital statistics are being scrutinized to determine when another puncture is necessary. It is a great challenge for me to know what nuances of behavior to report. Julia doesn't move or react too much these days. Everything she does is pretty subtle.

While Julia's condition is quite serious, it is not critical. Whatever is preventing her CSF (Cerebral Spinal Fluid) from draining is not known. That means that it could disappear as rapidly as it happened. There is no obvious blockage, according to the MRI, which is good. Julia's body fluid balance continues to be good, and her mucous seems to be decreasing. While the CSF situation is a major annoyance, it is manageable via the punctures to drain the excess.

Whew! The thought crosses my mind that perhaps a nice mountainside villa or seaside resort would be good for Julia's CSF balance. It would go a long way in balancing the anxiety level in my CSF! But, then again, when I think about all of the rigmarole just to transport Julia a couple of floors down to get her scans I think I'll hold off right now on requesting a two week vacation pass for Julia and me from Dr. Martin. Maybe next week.

Thank you as always for your continued well wishes and prayers. We feel the effects of your good thoughts every day, and it is very important to our continued success. We've gotten through intake vs. output -- diapers, and mucous removal -- bear growls. We still need some good alert irritability, on Julia's part that is, not mine.

~Julia and her mom

Julia's Journey #23 9/24/98 T+35

Julia is pretty sleepy this afternoon, and has good reason to be. It has been a busy couple of days. A few hours after the last lumbar puncture as detailed in the previous Julia's Journey it was necessary for her to have another one. Julia's cerebrospinal fluid (CSF) pressure jumped higher and more quickly with each puncture. I have learned that we all actually produce CSF all the time, and fairly rapidly. Julia is draining some of her excess on her own, but not all of it. We don't know whether the drainage system is stopped up, or if she is producing an overabundance. But, she again started becoming symptomatic of high pressure very rapidly after this last puncture, with stiff jerky legs, rising blood pressure, lowered heart rate, and decreased responsivity. At 4:00 AM two nights ago she had what appeared to be a seizure sort of event, where her whole body became rigid and she had a fixed disconjugate stare.

The next morning it was decided to proceed with the ventriculostomy, the surgical procedure in which a shunt is placed in the ventricles of the brain to drain excess fluid. The surgery transport assistants showed up this time to take Julia to surgery not with a wheelchair, or even a stretcher. We just packed up the whole bed and moved it to surgery! Once again, when we got to the big windows next to the patient elevators with the great view of Duke chapel, Julia opened her eyes bright and wide, despite the fact that her whole face was covered by the oxygen mask. She LOVES the outdoors, and it was so nice to see her taking in the scenery.

The surgeons reported that everything went well. I wasn't allowed to stay for the whole procedure this time, but was able to be with Julia until the very minor amount of anesthesia caused her to sleep. Two hours later she was back in our room, the best possible outcome, as opposed to PICU (Pediatric Intensive Care Unit) where she would have been if she was unable to come off of the respirator or if anything else went wrong. Our little abode had looked so empty without our wee one and her big bed in it. Fortunately the worst side effect of the whole ordeal appeared to be the removal of her skin when one of the leads was pulled off. Ouch!

The doctors had decided that it is currently worth the increased risk of infection associated with a temporary shunt that drains to the outside, instead of a completely closed permanent shunt, which she may still end up with at some point. This way we can measure the pressure and amount of cerebrospinal fluid, and collect samples to be sent off to lab, hopefully enabling us to find the cause of this problem. CSF should be absolutely clear. Julia's is currently yellow and slightly cloudy, indicating that possibly her problem is being caused by a small hemorrhage somewhere in her brain. The small amount of red blood cells in the fluid reported by the lab results add credence to this theory.

Once again, I am awed by the ingenuity of some of the equipment used to help Julia get well. The CSF collection device is basically a plastic bag with a ruler on the side into which the fluid flows as the pressure builds in Julia's brain. The trick is to keep the bag at the same height as her ear. If it's too high then all the fluid flows back into her from the bag. If it's too low then all the fluid flows from her into the device. No different than your average garden hose! It was already tricky to hug and hold Julia with the central lines and G-tube. Now it's really becoming intimidating. I already have an established history of unauthorized G-tube extraction. The ventriculostomy tube extends into the depths of her brain, so it's really important to avoid unsanctioned removal.

Julia's heart rhythm printout was showing an extra blip after the surgery, so the cardiac folks were sent up to run an EKG. {I must take an aside here to say that the acronym 'EKG' is really a left curve. Any self-respecting linguist should be very upset about this: Electro-Cardio-Gram should not be abbreviated e-K-g, but e-C-g. Sometimes I feel like there's a plot afoot to prevent us lay people from ever learning the vernacular.} When the EKG folks were done they handed me the strip. I asked them who I should give it to, and they said, "Aren't you the nurse?" Geesh! Now I know I've made the grade in my quest for a Med-Mom degree. I felt like I always feel when I'm in a crystal department and another customer asks me if I can help them.

Fortunately the heart blip turned out to be inconsequential. Julia's heartbeat normalized, her blood pressure stabilized, and the deep suctioning that the anesthesiologists did during the surgery removed enough mucous to keep Julia's sats in the high 90's. I decided to take a breather and headed for the hotel and a lobster-back shower. I walked back into the hospital room an hour later and greeted our family helper, Beth, who babysat while I was away. Julia greeted me upon hearing my voice by opening her eyes, raising her kissing-hand, and doing snake tongue I-love-you's. I adore that kid! She may be small, but she has a huge personality.

This morning Julia's vital signs are stable, although not necessarily the same as yours or mine. Krabbe's disease affects the part of the brain that regulates temperature. Julia has been measuring between about 90 F and 92 F, and really needs some help to keep warm. So, I am going to brave the wall of equipment between me and her and figure out a way to transfer some of my body heat to her little self, without pulling out any tubes or messing up the measurements. Hugs are great therapy for the thermometer and the soul.

Please keep the prayers and well wishes coming. We need lower cerebrospinal fluids, lower body fluids, less mucous. Lower, lower, less... until normal. :-)) Thank you.

~Julia and her mom

Update 24 (9/26/98)

Julia's Journey #24 9/26/98 T+37

I must begin this journal with a small matter of personal pride. For some unknown reason the spell check on this program consistently changed the initials C S F, as in Cerebro Spinal Fluid to CSF in the last journal. So, if you see the acronym CSF, please know that my intention was C S F. {I really have to question, WHAT does the spell checker think a 'CSF' is, anyway? Chronically Fatigued Speller? The possibilities are endless....) Perhaps I finally have the answer about why an electrocardiogram is sometimes referred to as an EKG instead of ECG.

Yesterday morning a member of the neurosurgery team was in our room to draw off some of the C S F from the intracranial pressure monitoring device when she noticed that one of the valves was leaky. This is a rather scary situation because this should be a totally closed system. Otherwise the possibility is much greater for infection, a very serious potentiality given the direct access to Julia's brain.

I fretted about this all morning, with my only piece of mind being the selfish thought that I knew that this time I did not cause the problem, unlike prior G-tube extractions of a far less serious nature. When Julia was napping I decided that it was time to break this rumination cycle, and that I needed a break. I left the room to go to run an errand. When I returned 45 minutes later I noticed that there was blood in Julia's ventriculostomy bag. Some break! I set into worrying overtime while waiting for the neurosurgeon to finish his other surgery and come look at this.

What a guy! When he arrived after my afternoon of total fretting he took one look and said, "Don't worry! This is almost nothing. If it were really bleeding you would know it." My relief was palpable. He thought that perhaps the cells at the surgery entry site could have dislodged during rolling Julia or some other similar circumstance. He set my mind at ease... somewhat. Telling a pediatric bone marrow transplant unit mom not to worry is like telling the sun not to rise.

I decided that it was time to go for a walk again. I had a different kind of surprise waiting for me when I returned, much more pleasant this time. Haley's parents, Greg and Erika, had made the trip up from South Carolina and were here to visit! We went to dinner and talked into the night. Julia was a happy kiddo, and very appreciative of having more people ready and willing to play kissing-hands with her.

This morning the C S F flow rate seems to be diminishing, and it is now transparent yellow instead of opaque pink. Hopefully we are resolving that crucial situation of yesterday and moving on to less serious considerations. The new worry point du jour arrived at about 5 AM, when Julia woke me with an elephantine squeal. I jumped up from a rare sound sleep, tripped on the blankets (as usual) and raced to her side to see what was wrong. She had her tongue sticking out and was rolled up in a tight fetal position, purple with exertion. I called the nurse.

It turned out that it was "just" blood pressure. It had gotten a little high (120's/80's), so Julia had received some Nifedapine sublingually to keep the numbers in line. And, she HATES it! This little kid would stop at nothing to get rid of that taste, hence the loud noises and contortions. Yuck, yuck, yuck! I laughed out loud. We have been through so much that I'm starting to mistake normal reactions for abnormal ones.

It must have done her a lot of good to have such a normal reaction, though, because she has had a great day since then. She had bright eyes again on the way to the CT scanner, and since back has done nothing but request more kissing-hand play. Dr. Martin said he had never seen her so alert. He also told me that although we do not yet have the official results of the bone marrow aspiration back from the lab (another couple of weeks), hers was one of the richest cell aspirations he had ever seen and that there is very high likelihood that these are donor cells. Julia is also completely off growth factor, and still has a white blood cell counts above normal, which is great news. She finished the day yesterday with about 200 ml more out than in, and showed no lung collapse on her most recent X-ray. She is back on regular feeds, 60 ml every three hours.

Truly one never knows what the day will bring around here. Seven days ago we were thinking about when we could leave. Then we had the most intensive week of worry I have ever experienced, topped off by a little brain surgery. I wonder how long it will be before I can go to bed at night without the latent fear of what will happen when I wake up. Today though, the indications are positive. All we still need to leave the hospital is to have adequate C S F reabsorption so as to get rid of the external shunt, make sure our blood pressure is under control, not have any sats-attacks for several days, and loose about another liter of fluid. Yup, I probably will have enough time to finish this baby blanket I have been learning how to crochet before we become outpatients, lest I worry about keeping Julia warm. ;-}

Please keep your prayers and well wishes coming. They are keenly felt and appreciated, particularly during the moments of highest concern.

~Julia and her mom

Julia's Journey #25 9/28/98 T+39

We had a great weekend. We received visits from both Haley's and Dalton's families, staging a mini Krabbe's convention. Julia demonstrated her willingness to play kissing-hands with Haley's parents, Greg and Erika, over and over again, and missed their company after they left. Dalton's parents, Tommy and Dianna, brought both Dalton and his twin sister Dakota to Durham for a day long visit.

Dalton was the first early infantile onset Krabbe's patient transplanted at Duke, and he is doing very well. He was transplanted at two months of age, before he had many symptoms. He will be two years old next week, and is doing everything a normal two year old does, including talk and pull his twin sister's hair, except walk. He is very close to that, though, standing and even taking steps, although he still needs help balancing. Seeing Dalton is inspiring, although we know that Julia has many more challenges ahead of her than Dalton had, because she was very symptomatic at the time of transplant.

The results of the MRI taken last week show that Julia's Krabbe's has progressed significantly since the one taken in early August, to be expected. This is really a simple race against time: new cells producing GALC and getting it where it needs to go in the brain before the genetically predetermined Krabbe's time bomb takes over.

The good news is that it appears as though Julia's cell production also appears to be on the fast track. She is beginning to grow her own red blood cells and platelets, which at not quite forty days is very early after a transplant. Dr. Martin still feels that there is good reason to believe that these are mostly donor cells, although we must wait until the results of the bone marrow aspiration are returned to us in a couple of weeks. If these are the donor's cells, then it's all the sooner that Julia will be making galactocerebrosidase and ridding herself of the Krabbe's.

Julia's overall demeanor is returning to what it was prior to the overload of C S F. The current theory about what happened to cause the original problem is that her platelets dropped too low, causing a small bleed in her brain. Platelets are the cells in the blood that clot, preventing bleeding. Once there is blood in the C S F the drainage system can become clogged, because it is set up to clear C S F cells, not blood cells. Hence, the backlog and the pressure buildup.

We continue to have a small streak of blood in her shunt line each day, usually around mid afternoon, but this appears to be only of minor concern, and may still be left over from the placement of the shunt. The neurosurgeons want to challenge Julia to absorb more, which can't currently happen, because every time the pressure rises naturally, as when changing positions, the excess is discarded through the intracranial pressure monitor. If we keep doing it this way we will never know if she needs a permanent shunt, or if the former problem is gone and she can handle her own fluid. So, we have to hook the collection device up to a transducer that will measure the pressure without removing the fluid.

The bad part about this is that we can not use the transducer here in Julia's room. The doctors are working hard to come up with some sort of arrangement where she can be cared for by nurses who understand transplant patients, along with nurses who are well trained in C S F transducer readings. Wow, what a mixed bag of emotions! As tired as I am of the view out this window, having Julia go to another room for as many as several days is not a thrilling thought. Our music, our visual stimulation, and our routine are here. Home Sweet Home aside, so are the hepafilters and more highly tuned infection control for the kids who are without white cells. (Fortunately Julia's counts remain in the high teens.) Julia's fluid level is down and her oxygen saturation remains steady through most of the day. She is down to only one pump for part of the day unless she needs to receive blood. As Doctor Martin says, if it weren't for the shunt we'd probably be outpatients by now. But, let's face it...it IS for the shunt, and here we sit.

So, we will probably move to a different room tomorrow for a few days. We'll see how Julia does processing all of her own fluid, so we can determine if it is safe to have no shunt, or if we need a permanent shunt. Either way, we should be back in this room after several days, and then out of the hospital completely some time after that. Please keep those well wishes and prayers coming.

~Julia and her mom

Julia's Journey #26 9/30/98 T+40

Julia's intake and output is finally really on track. She has moved up to 75 ml of milk every three hours, and is having some great diapers. HURRAY! The sats-attacks are minimal, and we should be able to completely do away with the blow-by oxygen soon. Last night she had a wide-eyed great session of all of her favorite games: kissing hands, snoodles, thumbs-up, and snake tongue, among others. This morning I even held her for a moment after her daily weigh-in, although this extra motion may have been partly responsible for the red streak in her shunt tube.

We go through so much every day that the thought occurred to me that there are actually people who wake up in the morning who don't fear what critical crisis du jour their child will face today. (Geez, a few months ago I was one of them!) Since everyone we see or talk to on a daily basis has one critically ill kid in their family, and we have, after all, been here a long time, it is easy to forget that not everyone in the world has at least one really sick child. That all out gut wrenching fear of 'what next?' is par for the course. Stop someone in the hall and ask, "So how's the little guy doing today?", and you could hear "Oh, his bilirubin is at 26 and he looks like a pumpkin." Or, "Her fever was at 104.5 for twelve hours, but it's starting to break." Or, today, in Julia's case, "Well, there's another streak of blood in her shunt tubing, but her neurosurgeon says it's not a huge concern."

It really seems strange to me these days that parents get to discuss grades on tests or athletic scores, instead of showing their child's gastroscope pictures (the inside of the stomach or bowel, as photographed by tiny cameras), pouring over minute nuances of CT scans and MRI's, and rattling off hemoglobin counts and plasma serums as if we invented the measurements. This is really a world apart from the world "out there" that we can see from our windows.

My hat goes off to the doctors and nurses who can stay here all day and then make the break for home and still act like normal. Of course, it's not their child.

The doctors have decided to remove Julia's temporary shunt today, because after a week the risk of infection increases too dramatically. We will rely on other observations to tell us if her brain pressure is increasing too much, and then do lumbar punctures. If the situation still does not resolve itself we will have a permanent shunt installed. So, we must hope and pray for Julia to be able to absorb all of her cerebrospinal fluid. These shunts and lumbar punctures aren't fun! I much prefer physical therapy, which we've had to stop for a while, and games.

We are at day 40 post transplant. We have been in North Carolina exactly two months, and have another 60 days to go until we are at 100 days post transplant, when transplant patients are usually allowed to go home. We have made it to the midway point, and now have cells growing with the needed enzyme. This is a big reason for optimism despite the frequent complications. I just keep reminding myself that complications are to be expected, and that you need to fight hard for the things that are really worthwhile. Thank you as always for your continued well wishes and prayers. They are very much appreciated, as well as news about your day. It's fun for us to hear about and look forward to the daily trails of a normal routine.

~Julia and her mom

PS: Note the attached picture of Julia snoozing with her snuggle toys.

Julia's Journey #27 10/2/98 T+43

Shortly after I sent Julia's last update the streak of blood in her shunt line became full red, and the issue of whether or not to remove and keep out or replace the shunt became a more poignant. Down to the first floor for a stat CT scan. This time we didn't wait for the transport team to arrive; the neurosurgeon, nurse and I rolled Julia's bed down ourselves.

The CT scan showed that as the ventricle where Julia's shunt had been placed reduced in size, the blunt end came in contact with a vein that then began to bleed. In fact, this was not a big cause for concern. OHMYGOSH, do those neurosurgeons ever have nerves of steel. By this point I was beyond anxiety stricken, but they assured me that they had seen this before with a good result.) So, she was able to have the temporary shunt out, and no new shunt put in.

This leaves us now in a position of closely monitoring her behavior and vital signs to see if more lumbar punctures are necessary. Monitoring Julia's behavior is my specialty, since what little behavior we have is rather subtle, and not always easy to interpret. For example, I wanted her to remain awake all day today to see what her behavior would be. After a few hours of keeping her stimulated she just couldn't go on without a nap. She rolled her eyes backwards, stretched her arms into a rigid position, and seemed to fall asleep.

That was different, nothing I had ever seen before. So, is it significant? Do I report it? Does it mean anything? This is not your average 8 month old. Mom's intuition doesn't go very far on a kid going through all of this. That was 2 1/2 hours ago, and we still don't know if it was important.

But, Dr. Martin assures me that having the shunt removed is a positive stride forward, and that we are one step closer to outpatient status. It certainly is better from an apparel standpoint. Julia hasn't been able to have a shirt on in a week because every article of clothing we have here needs to be stretched over her head, an impossibility with a transducer attached to a shunt. It is still possible that the bleeds from today will gum up the C S F collection points in Julia's brain, and that more punctures will be necessary, but Dr. Martin and all of our neurosurgeons felt good about how well Julia had been absorbing the fluid on her own. So, pray, pray, pray for happy cerebrospinal fluid absorption. Then we're out, and we can work on remyelination, the next big task on our to-do list.

~~~ the next day...

Some good news: Julia's most recent C S F sample showed reduced protein, down to 130 from 150 when we first arrived at Duke! While not totally conclusive, this is directionally correct. The lack of the enzyme makes the protein in the fluid high, so a reduction is hopeful evidence that the enzyme is in place.

~~~ and today...

Julia became less and less reactive as yesterday progressed. A couple of times she had mini events of staring accompanied by low sats that may possible have been seizures. A little after 3:00 AM the sats alarm woke me, and we had a difficult time rousing Julia back to a pink status. A little while and a lot of oxygen later we took her for a CT scan, which showed little change from the scan prior to that. The ventricles may be slightly larger, and the blood clot from the last shunt is about the same size.

Julia just finished having an electroencephalogram, here in our room, to look for possible seizure activity. If she is having seizures then medication can be prescribed. Now she is undergoing another lumbar puncture to ascertain if the C S F is rising. The neurosurgeon team has already been here this morning. A permanent shunt is looking more and more like a real possibility. This procedure would be somewhat more difficult in Julia than in someone else because the surgeons would need to circumvent their normal route from the brain to the stomach due to Julia's central lines.

Once again, we are grateful for your prayers and well wishes. We anticipated the possibility that Julia would need at least a couple of lumbar punctures after the external shunt was removed. We need to have the clot in her brain dissolve, and the cerebrospinal fluid absorbed at a normal rate.

~Julia and her mom

PS-- Please be sure to check out the attached picture of Julia enjoying some of mom's birthday cake. This is the only "food" Julia has ever tasted. As soon as this C S F situation is resolved I will be trying to convince her that carrots and peas are just as tasty as vanilla frosting. :-)

Return to Index

Julia had her second ventriculostomy this afternoon, after having a great deal of C S F buildup in the prior 36 hours. Her neurosurgeon reported that the procedure went well, and when I saw Julia in the recovery room a little while ago she recognized me and did a lot of snake tongue as if she wanted to tell me all about the whole experience. I really love that kid! What an incredible inner spirit. I very much look forward to having her more interactive than she has been the last day and a half, now that her intracranial pressure will be again at the right amount.

We will not be able to stay in our now comfortable room this time, however. We are being sent to a step down intensive care unit so that the cranial pressure monitor can be continuously watched. We were lucky enough to avoid moving last time, but this time the pressure will be monitored on the transducer from the beginning, and we will be where there are many nurses trained to read this particular machinery.

The neurosurgeon was able to see through a minuscule sized camera the blood clots in Julia's brain. The breakdown of this extra blood in the ventricles of her brain may be what is clogging her absorption of cerebrospinal fluid. The clots will continue to break down and disperse over time. The ventriculostomy will help Julia drain fluid until she is able to drain it all on her own. Let's pray for this to happen as soon as possible, so that we can hopefully avoid having the permanent shunt installed to take care of what is probably a temporary problem. Julia's counts and fluids have balanced enough that were it not for the overabundant C S F we would now be outpatients.

Please keep those well wishes and prayers coming, overtime! Julia needs to absorb C S F from her brain, and the blood clots need to disperse. Those two events will make for a very happy day. :-)

~Julia and her mom

Julia's Journey #28 10/5/98 T+46

Our stay in the step down unit uncovered two pleasant facts. First, the water in the shower gets much hotter than what it does in this unit. Second, the lack of a hepafilter system means that you can smell the microwave popcorn really well, a particular favorite of mine.

We are no longer on that unit, however. We have had to move to PICU, the Pediatric Intensive Care Unit, due to some external bleeding that Julia had from her ventriculostomy site. This bleeding continued on and off yesterday until the surgeons did some extra stitching and Julia received some extra coagulants. The really good news is that the reason why the bleeding appeared so heavy is that it's from the scalp and not internal. This ventriculostomy seems to be clearing the extra CSF quite well, and the CT scan we had last night showed that the internal clots are the same ones that were there a few days ago.

Julia herself is back to playing her favorite games, snake tongue and kissing hands. She is trying hard to adjust to PICU, whose beeper/noise overload has kicked her startle reflex into high gear. We are allowed to play our music, and it helps. Julia also has a really nifty warming device that looks like an inner tube with holes hooked up to a vacuum cleaner. It blows warm air under her sheet to keep her warm. The bleeding has abated and she is in stable condition. I am not allowed to sleep or eat in the PICU, both of which I have to do sometimes. When I try to leave Julia gets agitated upon hearing the tone of my voice saying "I'll be back soon." We hope to return to our PBMTU room sweet room very soon where I can be with her all day and night.

Your continued prayers and well wishes are very welcome and necessary. Julia needs to dissolve the blood clots in her brain, absorb her cerebrospinal fluid on her own, and not bleed anymore. It helps me to think of that inner GALC glow that Julia is getting, GALC being the enzyme that was missing before the transplant, but that is now growing and will allow her to live. Thanks to all of you for sending all of your good thoughts our way, they really help when we really need them.

~Julia and her mom

Update 29 (10/06/98)

Julia's Journey #29 10/6/98 T+47

Julia is definitely far more active and quite cheerful, although I don't know how long she can hold out against sleep. She hasn't really slept since 9:00 AM Sunday morning, mostly due to stimuli overload in the PICU. Hardly a moment goes by without that famous metal on metal sound that seems to put Krabbe's kids on edge. A clip board snaps, a door closes, a buzzer buzzes, a beeper beeps. They all keep her going. I hope that at some point she can block it out enough to sleep just a bit. She, like all of us, needs some rest to help her body get well.

Thankfully her condition continues to improve. We have had no more bloody bandages, and her ICP (IntraCranial Pressure) remains at an acceptable level due to the ventriculostomy. She does continue to drain C S F, however, meaning that she is not absorbing enough of it on her own to keep the fluid pressures safely low without an artificial vent. It will take some time for the clots in her brain that are clogging the C S F absorption mechanism to dissolve on their own, but we must remove the ventriculostomy within the next few days because the risk of infection becomes too great to keep it in any longer. It's looking more and more like a permanent shunt, tricky in Julia's case, because it will need to be inserted down her back because of the central lines already in place in her front. So, we'll keep hoping for crystal clear cerebrospinal fluid draining rapidly.

When Time came to our room during Dr. Kurtzburg's rounds they shot dozens of pictures from many angles. You get a good idea of the size of our room and the number of pumps to which Julia has been attached from the photo they decided to print. It is fun for me to look at pictures of Julia back when she had hair. The 15% that was left after chemo has been shaved off for the recent ventriculostomies. Please ignore the part of the picture with me in it. But if you do look at it, remember that I do smile once a year or so, in case you wondered. ;-}} (Julia, you owe me for this!!!) I assume that given all the hundreds of pictures Time shot on this floor they must have been looking for THE one where mom shows the stress of the moment.

As always, please keep the prayers and well wishes coming. Julia is doing her part. We'll keep calling on all the powers that be to help her along.

~Julia and her mom

Update 30 (10/07/98)

Julia's Journey #30 10/7/98 T+48

Julia was able to move out of PICU (Pediatric Intensive Care Unit) today, and back to our room sweet room on the PBMTU (Pediatric Bone Marrow Transplant Unit). This has been great for both of us. There are many hours of the day if your child is in PICU when you can not be with her. You must leave when the doctors round, from 6:00 AM until 9:00 AM, and 6:00 PM until 9:00 PM. You must leave to eat and sleep. And, you must leave when certain procedures are done with any of the kids in the unit. Now we are snug in our room where I can catch every nuance of her personality.

One remnant of the PICU stay is that Julia remains hypersensitive to the metal noises, an old Krabbe trait that she seemed to regain in PICU where there is an ongoing cacaphony of hard metal day and night. Every time Julia startles she ejects cerebrospinal fluid into her ventriculostomy, so she has cleared quite a bit in the last couple of days. Nonetheless it is still necessary for us to proceed with the permanent shunt, which will be put in tomorrow.

So, we spent the day today preparing for that surgery: CT scans, bleeding tests, urinalysis, coagulant draws, and so on. This will be Julia's fifth surgical procedure, so we pretty well have the routine down. The only difference today is that now Julia is famous due to the Time Magazine article, and well recognized it seems wherever we go. As we rounded the bend with her bed on the first floor into radiology on the way to our CT scan I heard a couple of patients in the waiting room say, "Look! Isn't that the baby in the Time picture?" Next they'll be asking her for her autograph! It was a special pleasure for me to receive a phone call tonight from a dear old friend from high school with whom I have not spoken for several years. She got home from work, sat down to relax, and started leafing through her subscription copy of Time. SURPRISE!

Although I personally am not so fond of the photo, I am glad that the word "Krabbe's" received a bit more exposure. Hopefully as the news of this disease spreads it will become easier for more pediatricians to suspect it and diagnose it rapidly, before children become too symptomatic to allow transplant. I met a new child and his parents who arrived at Duke today to be accessed for transplant viability. They discovered the cord blood option over the internet, as did the rest of us. In the old days it would have probably taken a few years for this information to become mainstream, and certainly unlikely that many Krabbe's families would be in contact. There are only about 40 of us diagnosed in the country each year. The internet certainly can be misleading, but used with circumspection it can be a lifesaver. Please add Mikey to your prayers.

Julia must be enjoying all of her publicity. She had a fantastic day, full of kissing-hands and snake tongue, and even some leg movements. It really looked like she wanted to add kissing-feet to her repertoire of games, with a little more practice. She seems to be normalizing her sleep/wake schedule after her PICU experience. Her eyes were bright and wide most of today, and she was doing very well holding her gaze. Days like this are truly joyful. The surgery tomorrow morning is fairly short, but Julia usually takes a couple of days post-anesthesia before she opens her eyes and becomes alert. She tends to gain fluid and have oxygen saturation problems during that suppressed state, not a lot of fun.

As always, your prayers and well-wishes are definitely necessary and very appreciated. We want Julia to have an easy surgery, quick recovery, and above all, NO MORE complications. We still have not received the proof positive test results from the bone marrow aspiration that all those new cells she is growing are donor cells, but the doctors here are optimistic. I personally am too, based on her ability to do some things that she was unable to do just before the transplant. If the surgery and recovery go well, and if the complications are behind us, then we can work on remyelinating and learning how to be a kid. That's a lot to pray for, but just look at her now famous picture. She's worth it!

~Julia and her mom