Julia's Journey
#31 10/9/98 T+50
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Update 31
Julia's Journey
#31 10/9/98 T+50
Julia's surgery went well. Her neurosurgeon said that we need to hope for "no fevers, no bad stuff, no complications" for the next many weeks. Every date we pass without these things will bring us closer to a worry free day. It will be several weeks until we can be fairly sure that Julia's clots have not dissolved into bits of debris that could clog her shunt, but that instead they have enzymatically dissolved, been absorbed into the C S F, and processed by her body.
Yes, I do love the neurosurgery team, and especially their calm and relaxed demeanor. I asked how long it will be until we can feel fairly confident that there will not be a clog. They said, "Oh, we'll get a CT scan in a month or two." Meanwhile we just watch to see if we see any signs of infection or rising fluid intracranial pressures. The shunt will stay with Julia permanently, draining extra liquid from her brain into the peritoneal lining in her abdomen when there is an excess. It is similar to a telephone cord and will grow as she grows unless she gets taller than 6 feet 2 inches. What a wonderful thought! :-)
The aftermath of this surgery thus far has been much better than the other surgeries. Julia has gotten to know the whole surgical team so well that they had Time opened to her picture and waiting for her arrival. They even play her favorite music for her in the operating room now. She is particularly fond of her anesthesiologist, Dr. Allison Ross, and always gives her lots of snake tongue just before going to sleep. Dr. Ross has really perfected the art of almost no anesthesia, but just enough, so that Julia can wake up afterward. Julia was awake in the recovery room this time, and has maintained an almost normal sleep/wake routine ever since. She was in some pain the day of the surgery, which we were able to treat with massage, music, hugs, and Tylenol. All of this paid off with no tremendous fluid retention or collapsed lungs due in large part to an alert and moving baby. What a relief!
Julia's big quest at present is to have a bowel movement. Yup, somehow it always seems to come back to diapers. She has been groaning about this for several days now, so we have had to give her a laxative. Nothing yet.... Although this sounds rather humorous, her constant bearing down causes extra intracranial pressure, which causes her to eject more fluid into her peritoneum, which puts more pressure on her bowels. All of this will get better with one big exodus, after which we will dance in the halls singing, "I feel good, do do do do do do do. So good, so good..."
Rob and the kids arrived last night from Michigan. What a sight for sore eyes! Yesterday was exactly three months from the day we received the official notification that Julia has Krabbes. What a long road since that day. It was two days shy of two months ago when we began chemotherapy, and also two months since we had seen the other members of our family. The kids have grown, but they're still the same kids, and Julia recognized their voices. Cam already has permanent thumb imprints on the video game controls. Alison needs to wear a mask due to a small allergic sniffle, so she knows a little bit about what it's like to be a kid on this floor for a long time.
Three transplant kids have died within the last day, two on our floor, and one who had to move to PICU some time ago. Each battle was extremely long and hard fought. Two of the children were infants, and I did not know their parents very well. I knew the parents of the older boy well, and am awed by their strength and courage. Time Magazine called the regimen these kids go through "brutal". They were understating the reality of it. But yet, yesterday a child left here to become an outpatient. He will live, whereas before he had no chance. The cost is high, the price is worth it.
As always, thank you for the prayers and well wishes. To quote Dr. George, our neurosurgeon, once again, "No fevers, no bad stuff, no complications." Dream of distilled water-clear cerebrospinal fluid bathing Julia's brain. And a little boost on the bottom end for big diapers, both kinds. :-)
~Julia and her mom
PS: Another picture! This is of Julia playing kissing hands, back when she still had the entriculostomy. You will see a small cord leading from her head up the bed with a tag. The tag reads, "cerebrospinal fluid". Although Julia looks covered with tubes in this photo, she actually was in a great mood, quite active, and very playful.
Julia's Journey #32 10/13/98 T+54
Julia, the little trooper with the big will, is doing well today. She has been on full feeds of 75 ml every three hours, complete with regular bowel movements. Our world revolves around diapers, and they are a gratifying sight. She is reactive and playful, although still a little waterlogged, so she's not opening her eyes very far due to puffiness. She remains in a state of acute startle-ability: the sound of the door closing, something hitting the garbage can, the blood pressure machine going off, etc. causes her to startle and to emit a small scream. But, really, this is a good sign. She's alert and interacting with her surroundings. Rob, Alison and Cameron returned to Michigan yesterday, and Julia now seems a little lost without the other kids' voices in the room. We both miss having our family here. It was a great visit, but video game Toy Story level 5 remains uncracked. Everyone will just have to come back soon.
The samples all went bad that were sent to New York three weeks ago to ascertain the percentage of donor vs. host cells, for Julia and several other kids. We took more samples and sent them yesterday, but now have to wait another three weeks for proof positive that all these cells are donor cells. The doctors have assured me many times, however, that there is ample evidence that Julia is producing donor cells. The aspirate was very cell rich, and with many of the "funky" cells present in cord blood, as opposed to the more mature cells that she would produce. Her last C S F sample showed a lower protein count (about 110) than she had when she arrived here (154), hopefully meaning that the missing enzyme has arrived and has started to work.
Julia was able to begin her physical therapy again today. The three week vacation she had to take from her exercises are very evident in the regression of her muscle tone, especially in her back and arms. I am amazed at how quickly the muscles deteriorate. We could all learn a lesson from this, particularly anyone prone to couch-potatodom, or hospital incarceration. Julia made her displeasure at being forced to move quite evident.
Julia gets to start trying some taste treats, like apple juice on her mouth care sponge. We've been giving her more and more medications through her GA-tube as opposed to intravenously. So, we decided to take the medications that the older kids report as tasty, or at least not horrible, and let her try them orally. The Tylenol was a hit, but the potassium bolus was only so-so. My objective is to keep those taste buds awake and functioning. We will need to do a swallowing study before she gets more than a drop of anything, but by that time she should be so happy to taste real food that I have a hunch she'll start eating easily. In the meantime, she is starting calorie enhancer added to her milk through her GA-tube so that she can keep on growing, since she can't handle any more fluids than she is currently getting, but needs more nutrition.
We are so grateful to be back in our room, and hopefully on the mend. Dr. Howrey even uttered the big "D" word, for sometime next week. (Shhhh... don't say it out loud, or even think it too strenuously! :-)
The last several weeks have been very frightening and worrisome. We are not completely in the clear yet, because we are still waiting for the ventricular clot to dissolve without clogging the shunt and starting the high intracranial pressure problems all over again. Every day we go without a complication the greater the chance we have of surviving. We know how lucky we are to be in this position, and believe that your thoughts and prayers have helped immensely.
One of the other Krabbes babies here, Judson Peacock, needs a boost. His transplant was the week before Julia's. He has a very severe case of GVHD, graft versus host disease. The next day is critical for him. And, another Krabbes baby, Gabi, who received her transplant at Columbia in June, is working to recover from GVHD and a blood infection. Please give all of these kids your well wishes and prayers. Transplant kids live on the edge for a long time. The disease they suffer from is vicious, and in many ways the treatment is equally so. Let's continue to hope and pray for the best possible outcome for everyone.
~Julia and her mom
Julia's Journey #33 10/17/98 T+57
Julia has either resumed her ability to squeal or she's practicing her cat yowls for Halloween. She has become quite able to voice her opinions in the last several days, frequently emitting high pitched squeals of great length and many decibels. This happens upon startling, gas bubbles, vitals being taken, the door opening or closing, lights being turned on or off. Basically, pretty frequently. She still does not cry, but the squeals definitely fill that void. ;-) I see them as a positive sign of increased alertness, because she definitely seems to alertly be on the lookout for new stimuli that merit squeals. I can hear her from the nurses' station, way down the hall, "Moooooooooooooooooom, I'm caaaaaaaaaaaaaalling you!"
Julia seems to be in a stable phase. We continue with our situations du jour. Yesterday she ran a fever that peeked at 38.2 C (100.7 F). Dr. George had said, "No fevers...", so we immediately put her back on Vancomycin and Ceftaz, the antibiotics we had earlier discontinued. Today she showed some blood in her stool again. I've told Julia repeatedly that I like to be bored, and don't need any more excitement. So, we'll see if she pays any heed to my admonitions, and hopefully not have any additional situations pop up, once we get these resolved.
Julia has started to grow some new hair. We worked so hard to save her copper tone topnotch, not washing the top front of her head for a month, not combing it, not touching it for pets or kisses. And, we were pretty successful, until the ventriculostomies required having it all shaved off. Finally it has started coming in again. At first I thought she had bruised eyebrows because she looked black and blue in that area. On closer inspection I could see that what she really has is a five o'clock shadow. Her eyebrows are returning full force, but the hair is black. The heavy steroids cause very thick hair growth, which frequently is in a different color. That medication also causes very chubby chipmunk cheeks, giving Julia an appearance something like this:
..
(( -- ))
These effects eventually go away. We are just overjoyed to have had her make it to this point. The doctors keep talking about the big "D" word, for next week. We had a visit this morning from Carmen, the "D" nurse, who sets you up with home health care, makes sure you have learned how to draw blood, schedules your tour in clinic, teaches you what you can and can't do on the outside. Julia is now off of all of her pumps about 22 hours of the day, and will actually have weekend passes tomorrow. She will get to go outside to feel some fresh air, for the first time in 67 days! Still we must watch for signs of high intracranial pressure, but since high ICP is first noted by observing her behavior cues we can do it as outpatients. We also need to have a couple of other issues resolved before we exit, such as doing away once and for all with the oxygen blow-by and no fevers for several days. But, I am happy to report that Julia's fluid balance finally appears in line. She is back down to 8.4 kilos, from a high of about 9.5 kilos. This is good!
Another little Krabbes kid, Mikey from Idaho, just finished having his G-tube installed, and is scheduled to begin his chemo on Monday night. We wish him and his family good fortune. This is a very long road. Our friend Judson, the Krabbes kid from Texas who had his transplant six days before Julia, has been in critical condition for several days due to severe complications from GVHD. His parents continue to do everything they can to make him comfortable. They have asked everyone to continue to hope and pray for a miracle. All of the families here develop a bond born of working relentlessly for the same goal, doing anything it takes to have our children survive their terminal illnesses. I feel especially close to Judson's family, since our kids have the same disease. Judson has been through a tremendous amount, and is a valiant fighter, as are his parents. Please add them to your prayers.
And, once again, thank you so much for all of your good thoughts and well wishes. We have come a long way, but still have a very long way to go. First we must make it to outpatient status, then home hopefully by the end of November, then rebuilding the many abilities lost since symptom onset. One step at a time.
~Julia and her mom
Julia's Journey #34 10/17/98 T+58
Tonight, Julia's journey included losing a friend who fought the same battle. Judson Peacock, the same age as Julia, died just before midnight. His was a very long and hard fought battle, lost to graft versus host disease. His parents, Tami and James came to our door just before they left the hospital, to say good bye. We had the most brief of smiles at the two phrases that drive Krabbes parents crazy: "You're so strong", and "God wouldn't give you more than you can handle".
I could write volumes about the unfairness and injustice of the suffering of this innocent child, an overwhelming reality. But I choose now to focus on the inner reserves that somehow his parents seemed to find. It is inspiring to know that all of us, ordinary people, somehow manage to access the strength necessary to do what we have to do for our kids. I saw it in a profound way tonight.
We will miss Judson, who has begun a new and less painful journey. And we will miss our daily visits with Tami and James, who return to Texas to raise their other son, Jase, as the extraordinary parents they are.
~Julia and her mom
Julia's Journey #35 10/19/98 T+60
The last couple of days have been very busy, as we prepare for "D" day, currently scheduled for Wednesday. I have learned how to draw blood and prepare it for labs, do fecal occult tests, and administer medications through the syringe pumps. I took the exit tour of the clinic in which we are taught where and when to show up on a daily basis with our samples. I went over a ream of paperwork for the home health care company that will visit our hotel room to ensure that we're doing everything correctly, and talked with the insurance company to make sure we're following all the rules and guidelines. And, I actually started moving some of our four month supply of STUFF to the hotel. This is a very pleasant kind of busy.
Julia has been able to join in the fun. On Saturday she had her first pass! She was allowed to leave the hospital and go outside for the first time in 69 days. We walked over to Duke Gardens and strolled around with Kelly and her son, David, the Krabbe family that lives in town. Julia had to wear her mask for this adventure. The mask catches most particulates circulating in the air, and hopefully most of the germs as well. It's going to take some getting used to, especially because the smallest mask available would probably fit a child who's about 4 or 5 years older quite well. It covers Julia's eyes as it is. We have to fold and tape it to allow her to see, which makes it a little more difficult for her to breath. I feel the need to check her frequently for pinkness. But, it's worth it. She seemed to enjoy the walk, but emitted little squeaks every time we went over a sidewalk bump.
Julia has been given the okay to start taste testing. We have a little thimble-like rubber device with bumps that fits on a finger. I dip this in various juices and stick it in her mouth. She tries to suck it, and makes great faces. She especially enjoys lemon juice. :-o We will be doing a swallowing study this week, and hopefully actually begin taste testing solids soon after. Julia received the biggest size g-tube today. The next step is a button, which will finally allow us to not have any tubes dangling. What a pleasure!
Julia's was born with O- blood. Her blood type is now O+, that of her donor. This is great news, and proof "positive" that the multitude of cells she is producing are donor cells! We sent a sample today to the lab in Philadelphia that tests the amount of the formerly missing enzyme circulating in her blood. I'm willing to bet it's quite a bit. She has started voicing her opinions on many things, with a cry that sounds more and more like a normal baby. It's music to my ears. All of this good news doesn't mean that we no longer have our situation du jour, however. Just as I finished bathing Julia tonight we got some labs back quoting a very high potassium level, meriting an on-the-spot
EKG to check for heart problems. Fortunately the test results were fine, but still the question remains about why her potassium level could shoot so high so rapidly. It will be a long time until we are no longer confronted with a wide and unpredictable variety of questions like this , always with the big question looming, "Is this significant?" As usual, we must wait and see.
We know, though how lucky we are to be in this position. Each time I opened my door this weekend I saw Judson's empty room, always keenly aware of the loss of our friend. Judson's parents have set up a fund called "Judson's Helping Hands", which will be used to help defray the massive expenses incurred by Krabbes families going through transplants. Similar funds exist for other diseases, but since we are pioneers, none has existed before now for the disease our kids have. The address is as follows:
Judson's Helping Hand
Nation's Bank
2615 Calder Ave.
Beaumont, Texas 77702
Please keep Judson and his family in your prayers. Mikey, the little boy from Idaho, has arrived on our floor and will begin chemo at 4:00 AM. One more child will now have a chance. Thank you once again for the many prayers and well wishes you have sent our way. These kids fight so hard to hold on to life.
~Julia and her mom
PS: Please note that when we leave the hospital our address and phone number will change as follows:
Julia Beatty
University Inn
502 Elf Street
Durham, NC 27705
919 286-4421
Update 36
Julia's Journey #36 10/21/98 T+62
Today is Julia's scheduled day for going "home" to the hotel. She had a fantastic day yesterday, sitting in her TV chair, turning her head from side to side as she followed me around the room while I packed and tidied. We packed just about everything, and carried most of it to its destination. The University Inn nicely cleaned and detailed the room we will call home for the next several weeks. We finished signing papers, scheduling appointments, meeting the home health care personnel. We even had our swallow study in preparation for trying real food with the outpatient occupational therapist.
It was interesting to watch in real time what happens when Julia swallows. She was fed some cherry flavored barium, which was X-ray filmed while it went down. The good news is that she still swallows and gags, even though she hasn't eaten by mouth since the beginning of July. {As an aside, I will forever be grateful to our University of Michigan neurologist, Dr. Leber, for finding Julia's gag reflex after her g-tube surgery. We would not have been able to have the transplant if Julia couldn't gag. She was still a little groggy from the surgery when he checked her, and he had to work hard to find her gag reflex. He did finally find it. That gag reflex enabled Julia to eventually remove all the mucous generated by the mucositis, without getting severe pneumonia.} The bad news is that her swallow is delayed such that some of the liquid dribbles down her windpipe before she swallows it into her esophagus, so she aspirates some. Hence, we can still give her tastes only of clear liquids on the end of our finger squeegie, but it will probably take many months of practice until she has enough control to risk more substantial foods.
We had a number of people stop by our hospital room in the last couple of days to see us off, including our friends on the cleaning crew, nurses' aides, nurses on their days off, and a few of the doctors we've met along the way. Last night Dr. Sherry Bayliff, Julia's all time favorite who had recently been rotated off our floor, dropped in for a good session of kissing-hands and snake tongue with Julia. Sherry stood up, went out the door, and I laid Julia down to change her diapers. Julia emitted a screech, and then stopped breathing. Her oxygen sats hit a low of 26 (out of 100), and she was quite blue. It took her primary nurse, Kate, and I a minute or so to get her out of this stage and back into the pink range. The oxygen was hooked back up, and we all wondered how this could happen on the night before we were slated to move to the hotel.
The sats attacks continued on and off through the night, never as low, but still into the lower 80's and upper 70's on a periodic basis. At 8:00 this morning we agreed to postpone becoming outpatients for a day or so to evaluate. An hour later when Julia became limp and unresponsive it became clear that the "D" word would go on hold for some time. Downstairs we went, STAT, for another CT scan. We found out that Julia's ventricles were enlarged again, an indicator of increased intracranial pressure. I put Julia's favorite CD on, Bach's Brandenburg Concertos, for the neurosurgeon who came to our room to tap the shunt reservoir. (He was a music major as an undergrad, and studied at Interlochen in Michigan.) He found a slightly elevated intracranial pressure, although nothing like the extremely high pressures of the past. All ports were cultured, labs drawn, tests run; multiple discussions as to cause and course of action continue.
It is now 11:00 PM, and we are still watching Julia's sats, heart rate, and awareness level rise and fall similarly to other times when her intracranial pressure was high. Things go down, we call the neurosurgeons, they get here, things go up. Intermittent, just like the car business. I offered to trot down to the operating room and handle a couple of surgeries for them (I am becoming a regular down there after all), if they would stay up in Julia's room, since she looks great whenever they're around. No takers yet, but I'll keep working on them.
So right now it's up to Julia. We'll keep a close watch on her tonight, and send her for another CT scan tomorrow to see if we can determine any difference in the ventricles, indicating a possible cause for the strange behavior. At this moment she's playing her favorite games, albeit a little sluggishly, even though an hour or two ago she was limp and non-responsive to all but painful stimuli. I prefer the games.
Please keep the prayers and well wishes coming.
~Julia and her mom
Julia's Journey #37 10/22/98 T+63
Julia woke up
today in a much better way than yesterday. We had a follow up CT scan this
morning, and then a fun afternoon of going to the gym to get fitted for a
"Panda Seating System Stroller" that will enable Julia to finally
straighten her back. She had been completely weaned of IV drugs during the day
in preparation for outpatient status, but has had to go back on several due
since her low sats episodes began. We were able to cap her off (remove her from
all IV's) for the CT scan and jaunt to the gym. It is always fun to be without
wires and constraints, although it is currently still necessary for Julia to
have a pulse oximeter so we can tell before she's blue if her sats are going
down.
The physical therapy gym is located on the floor above us. It was very interesting to see the wide variety of equipment available to help kids of all ages relearn the skills we take for granted. Julia will need to learn nearly all basic skills, from reaching and grabbing to crawling and walking. Just a few short months ago she was nearly indistinguishable from any other baby her age. The tone in most of the muscles in her body has atrophied substantially since then. Her back and shoulder muscles are particularly at risk, because her stomach muscles are stronger, so she is almost always "hunched" over. We had to fit and measure her for three pieces of equipment:
1. car seat
2. bath chair
3. seating system stroller
When we sat Julia into the sample stroller and adjusted it to allow her back to straighten out she became so obviously more comfortable and happy that we called back down to our floor to have Carl, the director of the Child Life program, come up and take a picture. Be sure to download the picture to see Julia in a state of ease. (You can also see a couple of the suture spots on her head from the ventriculostomies.) This seating system is something that Julia will probably spend a lot of time in, because it straightens her spine so that it is much easier for her to breathe, reach, and avoid clonus. Amazing!
It was nice to be allowed to have that respite of fun time, which we had scheduled back several days ago. Julia was definitely in an improved state today from yesterday, although still not quite 100%. She wanted to play her games and turn her head to watch what was going on right up until naptime. We haven't had any more extremely low sats attacks, but have regularly dipped into the low 80's. A couple of times today Julia has become limp and somewhat nonreactive, indicating that although improved, the underlying cause is not completely gone.
Julia's CT scans showed that the blood clot from a month ago has dissolved, and that there are no new bleeds, FANTASTIC news. Julia's ventricles still have not reduced in size from yesterday, however, indicating a possibility of shunt malfunction, maybe due to a clog. This may be intermittent, sometimes clogged, sometimes not. Her low heartbeat, high blood pressure, and unusual patterns of apnea and non-responsive behavior fit that paradigm, and might explain why the tapped intracranial pressure was nominal. Hence, it is necessary to look at the shunt itself and see if we can solve the mystery, since it doesn't seem to be going away by itself and Sherlock Holmes is currently unavailable for comment. We can't be released to roam around town unless we can be sure that Julia's hydrocephaly is safely under control.
So, tomorrow at 7:30 AM Julia is slated for another surgery with our favorite neurosurgeons. Dr. George will examine the shunt in pieces to see if all parts are working, and fix or replace as needed. Once again, well wishes and prayers are appreciated. Julia needs to have a permanently operating shunt, and her cerebrospinal fluid needs to turn crystal clear, and easily drainable. Her post operative period is always a trial, when she needs to become awake and alert, and breathe on her own, or we end up back in PICU. PICU is a great place for those who need it, but we prefer our own room sweet room if we must be in the hospital. I am very glad that if a complication had to arise, it did so on the night before we were to leave, rather than the night after. I would also be very happy to have no more complications after this one, but a straight shot to the pleasant life of a PBMTU outpatient and her mom, gleefully enduring the boredom of hours at clinic rather than days and weeks on the fifth floor of the hospital.
~Julia and her mom
PS: The new Krabbe's family on our floor, Mikey and his parents Tina and Mike, began chemotherapy on Tuesday morning at 4:00 AM. You may contact them at TMTtorres1@aol.com if you would like to send them your well wishes.
Julia's Journey #38 10/23/98 T+64
Julia's AM surgery went well, according to Dr. George. He did a "shunt revision", taking out the first shunt and replacing it with a shunt that drains to a slightly different part of her stomach. He was pleased as he gave me the post-op debriefing, with the usual caveats: "No fevers, no bad stuff, no complications."
As one friend put it, today Julia turned the situation du jour into the situation de l'heure. Her post-op recovery looked good, and we were able to come back to our room relatively quickly. Julia played snake tongue in the recovery room, and greeted our friend Vickie, who just arrived today from Michigan for a visit, with a bit of kissing-hand. Soon after Julia's heartbeat started to drop, first to the 80's, then 70's. She began a new routine of normalcy, then staring, then apnea, then sats, then even lower heart rates, then normalcy. This caused quite a stir among the doctors. Up came the neurosurgeons; down we went to the CT scanner and then to radiology for a shunt work-up. A friend here asked us as we were being wheeled off the floor just exactly how many cats are we planning to scan. I guess we're aiming for an entire pride, but I'll have to get that answer from Julia. The good news is that there have not appeared to be any more bleeds, but the ventricles are still large.
The cycles kept getting closer together throughout the afternoon, with lower dips, heart rate now in the 60's. One of the surgeons tapped the reservoir, which only helped a little. We needed to go back to the operating room. Dr. George "vacuumed" Julia out, draining over 60cc of cerebrospinal fluid, and used a scope to determine that in fact this shunt is really working. The problem is that the distal part of the shunt, the end in Julia's peritoneal (stomach) lining was malfunctioning due to having a higher pressure than the end in the ventricles in her brain. She seems unable to absorb the amount of liquid that is draining from the shunt into her body, and has a backlog.
Julia also recovered well from this surgery, but was this time put in PICU so that she could be very closely monitored for de-sats, and bradycardia (low heart rate). She was left on the respirator for a while because she had a couple of periods of apnea in the operating room. We were able to remove the tube a couple of hours ago, meaning that she will hopefully stay in PICU only until tomorrow, if she can maintain her oxygen saturation levels.
Julia MUST find a way to absorb and dispose of this fluid, or we will soon be back in the operating room for another external shunt. Dr. George felt that this could go on anywhere from a short period of time, to several weeks. Amazingly, her function level when she is not having apnic spells is quite high. When I was first allowed to enter PICU and she heard my voice she began a wonder series of snake-tongues and kissing-hands. This, from a girl who had just gone through two brain surgeries!!!
So, again, thank you for sending all of your prayers and well-wishes our way. Julia is working very hard to overcome this tricky problem, and your good thoughts on her behalf are much appreciated.
~Julia and her mom
Update 39
Julia's Journey #39 10/26/98 T+67
Our little Julia is full of surprises. She must have thought we said "PICU" last week when we said "HOTEL". Our visiting friend from home, Vickie, had to emphasize to Julia repeatedly that it wasn't necessary to provide such a tour of the hospital on Vickie's behalf, she would have been just as happy staying in room #5201. Hopefully we've redirected Julia's thought patterns on the subject: Let's save the touring for Duke Gardens.
We were able to leave PICU Saturday morning and return to our room, but not before we had the opportunity to learn plenty of new facts. For example, I did not know that when people are on respirators a tube goes in between their vocal chords and they can not make any sounds. It was fascinating to see the respirator machine at work, and to learn the meaning of the wide variety of statistics it generates. But, from now on, I really prefer to learn these things at times other than when my daughter is using them! Fortunately we were able to remove the respirator a few hours after surgery. Julia's apnea continued, probably as part of her disease, but not seriously enough that a respirator was necessary.
We discovered something interesting about temperature regulation while in PICU. Julia's average temperature is around 33 C, equivalent to about 92 F. This measurement is probably so low because the Krabbe's has done some damage to the temperature regulation area of her brain. In PICU they didn't realize that this extraordinarily low temperature was her baseline, so they heated her up with the "vacuum cleaner / inner tube" heating device detailed in a previous update. Her heart rate went from the low 60's to the 100's-120's. We were able to move the warm air hyperthermia system into our room, which has helped us keep her heart rate a little closer to where it should be.
Julia began to regain her nice pink color on Saturday, and returned promptly to playing her games. She seems to be having an easier time controlling her body fluids than she has had after other surgeries. She is actually down to 8.1kilograms, from an all time water logged high of around 9.5 kilograms a few weeks ago. The big issue now is having her drain her excess cerebrospinal fluid, and being able to absorb it from her peritoneal lining into the rest of her body. It may take some time for her body to completely repair all of the damage that is done during chemo and transplant, so that it can easily absorb and dispose of the fluid. Right now we are watching her day and night, searching for any signs of high intracranial pressure, which would indicate that she's still not absorbing the fluid in large enough quantities. If her pressure goes high again she will very likely have her shunt externalized. Dr. George will find a place somewhere in the current shunt and run a drain to the outside. There is a very significant risk of infection by doing this, so we really want Julia to figure out how to absorb those liquids on her own. This is the current most important quest.
Julia's skin is changing colors due to the steroids and other drugs. She is a little dark and light patchwork quilt in some areas. Anything sticky, like band aids, lead connectors, and dressings always remove the top layers of skin when removed, unless removed with sticky tape remover. When Julia had her unscheduled surgery on Friday night, the surgeons needed to get her prior dressings off quickly. The result is that these areas are now quite raw. The good news is that not all layers of skin came off, as in the past. This, coupled with her lower fluid retention, is good evidence that Julia is making progress in her recovery from the transplant.
Even better is how Julia looks. Her body is returning to her former shape, instead of being so bloated. She looked a little green the other day, but now is quite pink. When nurses who haven't seen her in a few days walk into our room they are always happily impressed to see how much better she looks. She is beginning to get bristles of new hair on her head where what little was there was shaved off for one or the other of her five brain surgeries. This should end up being quite a hair style. Her eyebrows though...... eeek! Big, black, furry, wow. Soon they'll be one, right across her face. And, she's growing a bumper crop of hair on her forehead, chin, and upper lip. A new name comes to mind: "Juli-anderthal". That's just fine with me, so long as we continue on the getting better path!
Our Juli-anderthal has also become quite practiced at letting out primeval screeches. She has started doing this to indicate she wants to be held. It is very nice to have her so aware of her surroundings that she can tell us "HOLD ME NOW". She can pick up the cues by the sound of voices, and will try to turn her head in that direction. If she doesn't get any attention, she lets us know. Her cries are sounding more normal as well, but are still just a couple of syllables and of short duration.
Please be sure to keep your prayers and well wishes coming for Julia to continue on her upward trend. She has worked incredibly hard to come so far. The transplant itself is working. The big challenge now is that she must be able, somehow, to absorb the extra cerebrospinal fluid, the sooner the better. Tomorrow she goes for yet another CT scan to see if her ventricles that were enlarged by the excess pressure have started to shrink. We need low pressures, and shrinking ventricles. Julia is doing the rest.
~Julia and her mom
Julia's Journey #40 10/29/98 T+70
Today is our
80th day in the hospital. It brings to mind the book, "Around the
World..." Certainly every day here with Julia has been one kind or another
adventure. Sats-attacks are nothing new, but the low numbers we've reached in
the last couple of days are. Two days ago Julia hit 34%, 26% and 16%, in
progressively deeper shades of blue. The doctors and I were very concerned, and
spent considerable time trying to determine cause and course of action. Julia's
CT scan showed the same or slightly smaller ventricles, so this wasn't the
problem. The EEG did not indicate any seizure activity. We could see that the
deep drops most often occurred just after Julia started to cry (YES! I said
cry!), when she would take a deep breath and then hold it for a minute or more.
We watched this phenomenon time and again, and have finally concluded that Julia is simply relearning how to cry. She hasn't cried in full gusty tones for about two months. It seems that now, as she is relearning this skill, she gets stuck at the intake. She inhales all the air and then freezes, mind and body, until something finally reminds her to exhale and breath again. We have been able to help her do this by grabbing her chin and forcing her out of her frozen position by closing her mouth. The oxygen is always right next to her, a dose of which helps make those first few breaths more powerful. A couple of times we have had to "bag" her, which means to use the blue bladder-type device with a mask. When the bladder is squeezed it forces oxygen into her lungs, and breaks the her position lock. We will be seeing a speech pathologist this afternoon in the hopes of discovering more tricks to help her learn that while crying is good, breathing is better. ;-)
Right now Julia is on a 100% watch. She really seems to be enjoying her new ability to cry, which makes sense if you understand that she has been unable to express how miserable she has felt for the last two months. Now she has the means to communicate her displeasure, and she is making use of it. There must be someone watching her at all times, though, because you never know what might set her off, and if she's going to lock and desat so that she needs help breathing again. She starts to cry every time she's moved, when the blood pressure cuff expands, when her temperature is taken, when she burps, when the phone rings, when the "golf-ball dropping" noise from the pressure stabilization unit occurs, and occasionally for no determinable reason. Each time she struggles to get past the intake. She seems to be making progress, however. Thus far today the longest she has help her breath is 55 seconds, desaturating only to 42%. Learning is taking place, but she still can't be left alone for even a minute, in case she needs help.
Julia's other breathing problem, not to be confused with the one above, is sleep apnea. This is a common Krabbe's symptom. Her breathing pattern has always been irregular, especially when she's in the transition period between waking and sleeping, or vice versa. Her breaths per minute frequently show as zero on the monitor during this time, and she desats to the low 80's on a cyclical basis for a half hour or so every she takes a nap or wakes up. But, there is a remedy for this: caffeine! I had to laugh when the doctors told me today that Julia could use a cup of coffee. In fact, I had only semi-jokingly suggested this to Dr. Martin last month when we had exhausted virtually all other diuretics. But, Dr. Howery was serious about giving it a try this afternoon, so Julia just had her first dose down her G-tube, 80mg, about the same amount you would get in a good-morning mug from a drip machine. This is a "loading dose" for her. The half life in infants is 100 hours, whereas it's only about 6 hours in adults. I had to ask about this because Julia will only get her caffeine once each day. I know that I've never had a cup of coffee keep me going for the whole day, but apparently it's different for babies. It seems to be working. She has drifted off to sleep for her afternoon nap, and only desatted once, to 87%. That's a huge improvement.
The other issue we are working out is temperature regulation vs. heart rate. Julia seems to drop her heart rate about 10 beats per minute with each degree centigrade she looses. So, the trick is to keep her at the right temperature, 37 C, without heating her up or cooling her off too much. Easier said than done! We have the warming inner-tube/vacuum, but there are only three settings, 90 F, 100 F, 110 F. Last night I carefully dressed her and arranged sheets, blankets, and inner-tube to hopefully keep her at 37 C. Then I fell asleep, a rare occurrence that necessarily happens every so often. Over the course of a couple of hours her temperature rose to 38.2 C, just above 100 F. So, we had to take cultures on everything and give her a quick dose of antibiotics, just in case it was a real fever not caused by the warmer. This is just one more small example of the balancing act it takes to keep these kids going, every day!
The very best news is that Julia's fluid seem to be in alignment right now. We initially thought that the low sats were an early indicator of increasing intracranial pressure, but now believe that they are separate Phenom ena. Today was the first day in many weeks that Julia's weight was below 8 kilograms, right where it should be. She is getting rid of excess fluids on her own, and hopefully absorbing from her peritoneal cavity the extra cerebrospinal fluid. Today is a good day. Julia sat in her swing and seemed to really enjoy it. She is following me around the room by moving her head and eyes. She just started squeezing her eyes tightly shut as she wakes up, as if to get them moving again. And, we are all getting lots of snake tongue and kissing hands. Yes, it's a great day! Let's pray for many more like this one. I even received notice that I FINALLY was excused from jury duty, which I had been scheduled to serve next week.
Attached is a picture of Julia hugging her terry cloth teddy and wearing her new booties, given to her by Michele and Peter, parents of Joseph, and authors of his web site. Michele and Peter are visiting the Krabbe's kids in town this week. Those of us here now owe them a tremendous thanks for putting the information that led us to Duke on Joseph's page. And, as always, thanks to everyone out there who has sent prayers and well wishes our way. I am amazed when I contemplate the numerous people who are contributing in so many ways to help Julia and the other kids here with their chance to survive.
~Julia and her mom
PS: Please note that Mikey's parents' email address is TMTorres1@aol.com
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