Select by Update

41 | 42 | 43 | 44 | 45 | 46 | 47 | 48 | 49 | 50 

Updates 1-10    Updates 11-20    Updates 21-30    Updates 31-40    Updates 51-60    Updates 61-70    Updates 71-80    

 

Update 41

Julia's Journey #41 11/2/98 T+74

Julia had a great weekend. She has made great progress on crying, and hasn't needed any help breathing through a cry for a couple of days. The caffeine experiment has required some adjustment. She had exhibited the effects of both too much and too little: heightened sensitivity to environment a little while after a dose (Whew, am I speaking euphemistically. Julia will startle if she hears you breath.), heightened irritability (withdrawal headache?) prior to the next dose. We've been working at getting the dose optimized. The great news is that the caffeine seems to be keeping the apnea well under control.

Julia has been moving much more. Today in physical therapy she held her own head up while on the ball! She used to knit her little copper tone eyebrows when we first tried this in September. She looked positively fierce today knitting those Juli-anderthalian big black brows. She really seemed to enjoy the session after she figured out that this wasn't specially formulated as a torture device. She loved it when we bounced her on the ball, but did not enjoy rocking at all.

Julia was allowed to leave on a pass last Friday, to go to the hotel to get her Halloween costume prior to the floor's party. (Please check out our little clown on the attached picture!) She was able to meet all the other kids whose counts allowed them into the family room for this event, including Mikey for the first time. On Saturday Julia went on a four hour pass to show Aunt Lynn the Duke chapel and gardens. Julia's opinion of the outside is mixed. Riding in the stroller is okay, so long as we don't hit any bumps. The low hat hood to keep the sun out of her eyes is not her favorite. And, she absolutely hates the particulate mask she is required to wear when around other people. The pediatric masks are still big on her. We have to cut and shape them so that they don't cover her eyes. It's never a good fit. The result is that the hat is low, the mask is high, and there's about a quarter inch of face visible. She loves to chew on the masks, which makes them slimy inside. Maybe the real goal here is to exercise Julia's jaws in preparation for the day when she can eat by mouth again!

All of this is in preparation for the unmentionable "D" word again. We've now been at this point a couple of times, so no one even brings it up. Rumor has it that something could happen later this week, but we're not telling Julia. I'm not taking down any decorations, moving any stuff to the hotel, or doing anything special, just in case she notices, and thinks we mean PICU. No way! We're just going to surprise her when the big day comes. I think our 84 days here are starting to make me superstitious.

Something else that our long stay has done is to turn me into a hand washing aficionado. The other day a hospital employee was entering the airlock at the same time I was. She didn't wash her hands. When I reminded her she became very defensive with the belief that she wasn't required to do this, despite the numerous signs indicating that there are NO exceptions to this rule. We eventually worked everything out, but it made me curious about the rationale for the rule.

I found some extremely interesting research on this subject. It turns out that the #1 way to prevent infection is hand washing. Studies have been done on this subject in hospital isolation units. In one group everyone entering the unit was required to suit up in sterile hospital "bunny suits", caps, masks, and shoe covers, versus the other unit where only hand washing was required. The infection rate was the same. This is why we are required to wash our hands here, but not suit up. The vast majority of germs are carried on our warm and moist hands. Bacteria can live there indefinitely, transmitted from one person to another. Germs die rapidly on cold dry surfaces or in air, which is why relatively few infections are spread or caught by breathing. Most nosocomial (hospital spread) infections come from lack of clean hands.

Much of the research indicates that few people take this seriously, however, or somehow think that their germs aren't germy enough to make the hand washing rule apply to them. One study about an ICU unit in a famous hospital we've all heard of (NOT in North Carolina or Michigan) found that health care workers washed their hands before only 37% of the patient contacts made during the study period. The amount of time spent washing was inversely proportional to the rank of the person making contact in some research results. The study did not tell how many patients in that unit came down with infections.

Every time I wash my hands before touching Julia I now think of the following statistics:

---The minimum amount of time spent washing must be greater that 15 seconds (less than 9 is virtually like not washing at all);

---The water must be greater than 104 F but less than 114 F (promotes chapping if too hot);

---The soap should contain an antibacterial agent of between 2% and 4%;

---All areas up to 4" about the wrist should be covered with the soap, with special emphasis on rings and fingernails;

---Drying is as important as washing: paper towels remove 42% of remaining bacteria, clean and not previously used cotton towels remove 10%, and hot air dryers may add up to 500% of the bacteria you started with, because 97% of the time their nozzles have been contaminated. If you must use a hot air dryer NEVER stop until your hands are completely dry. The drying action itself kills many of the germs.

So, how have we all survived this long if we haven't always washed our hands with clinical perfection? That is the wonder of the healthy immune system. A working body fights off the onslaught of diseases every day. Immune compromised adults and kids like Julia must adhere to these rules with great rigor, as well as anyone caring for them, along with all healthcare, food service, and child care professionals. And, it won't hurt the rest of us either.

Now you see it -- here's what happens to someone's mind when they've been here this long. I not only looked all this stuff up, I actually enjoyed reading it! EEEEEEkkk... It's time for us to move on to a new residence with a little broader range of activities, hopefully soon. As always, your well wishes and prayers are welcome and needed: clear and draining fluids for Julia, and a little respite for her mom.

~Julia and her mom

PS: Tomorrow I will be on the "Al Kresta Live" radio talk show from 12:45 PM to 1:00 PM, talking about Julia's experiences. This show is broadcast from WDEO 1290 AM in Ann Arbor, MI, and simulcast on WCAR 1090 AM in Detroit. I will talk to Al from here in our hospital room. Wish me luck! Remember, our quiet little Julia has been learning how to cry, and is not afraid to practice... :-)

Return to Index

 

 Update 42

Julia's Journey #42 11/5/98 T+77

Rumor has it that tomorrow is "D" day. No one is mentioning it to Julia, but plenty of hints are being dropped. For example, she no longer has any medications by IV, and mom can now draw blood and change dressings with relative ease. The d-i-s-c-h-a-r-g-e (we don't think Julia knows how to spell yet) nurse has been hanging around with stories of life in clinic, and old friends are stopping in to say "see you around". So far tonight, so good. So, I spent the evening removing many of our three months' worth of accumulated goodies from our room and taking them to the hotel. All that remains here besides Julia and me are her toys, and mine (the computer, of course).

Today Julia had a follow up CT scan, which showed that her ventricles have not changed size from the time of the last shunt revision. This means that the intracranial pressure likely has remained stable in the normal range, and that the shunt is working. I have learned that shunts are frequently very high maintenance devices. We will be on ICP watch for a very long time.

It could be many weeks before Julia's ventricles start to shrink visibly on the scans. Because she can not tell us in obvious ways when something is bothering her, we will need to constantly observe her very subtle signals to see if anything is different. Signs of problems in the past included higher blood pressure, lower heart rate, hyper tonic limbs (rigid legs in her case), hypo tonic limbs (limp arms in her case), disconjugate gaze, increased nystagmus, clonus, grunting as if making bowel movements, and so on. The difficulty is that all of these high ICP signs are also symptoms of her disease. I could get very anxiety stricken about being out there in the big wide world (across the street, that is) on my own having to interpret whether or not we've got a situation we need to act on. But, instead I'll put my 'Physician's Assistant' sweatshirt on, and take comfort in the idea that everyone here thinks that mom's intuition is able to do the trick. And, we do get to visit clinic nearly every day when we first leave, so that we don't have too large of a withdrawal from our dependence on the hospital staff.

So, keep those well wishes and prayers headed to Durham, a block from the hospital. As I look around our now almost vacant room, I am very grateful for how far we have come. I also know that this is no time to become complacent. Julia must maintain a working shunt, keep growing cells that will get more and more enzyme into her brain, remyelinate and regain lost functions (like eating and smiling), and go forward to learn and grow like other kids do. The next step is the little shower of confetti the nurses toss over the PBMTU graduates as the go into the airlock on their way out of here. It is hard to put into words how fondly we all anticipate that moment.

~Julia and her mom

 Return to Index

Update 43

Julia's Journey #43 11/8/98 T+80

Someone must have told Julia that we were thinking of changing our residence, because she decided Friday morning to drop her sats enough times that we had to stay here for a couple of more days until we could be sure that the medications, especially the caffeine, were optimally effective. Grandpa and Aunt Cheryl arrived from New York late Friday night, expecting to see us in the hotel room, but finding us still in the hospital.

Saturday was a pretty good day, though, and we were allowed to go out on a 4 hour "therapeutic" pass with our new pulse oximeter. We were strolling around campus showing our relatives the Duke gardens and chapel, when we rolled over a metal grate. OH NO! Metal on metal sounds still make Julia very unhappy. She started to cry, and then... held her breath... until she turned blue. Fortunately she started breathing again with a little help from me pumping her chin, but the trip ended up being not very "therapeutic" for me, or some of the bystanders for that matter. But, at least we were out in the beautiful fall air for a little while. The trees are in peak color down here right now.

We weren't able to go out on pass today, however. Early this morning Julia's blood pressure dropped very low. One of the nurses admitted to saying the "D" word outside our room in the hallway, since we had decided on Friday that Monday would be the big day. Well, no need to move the computer to the hotel tonight. We're blaming that nurse for jumping the gun and tipping Julia off about the impending move.

Low blood pressure is something we haven't seen in Julia before. Extremely low blood pressure can be indicative of sepsis, or blood infection, so Julia has had to go back on pumps again for IV infusions of different antibiotics. She also has had a lot of extra fluid pumped into her today to raise her blood pressure into a more normal range. She's now off feeds for a while until we have a better indication of what's going on. Blood infections are not that uncommon in transplant kids because bacteria can enter their blood stream in areas where the mucositis has opened the walls of the digestive track. It usually takes a while to get rid of these infections because of the kids' weakened immune system. Julia has the additional burden of needing to avoid an infection in her shunt, which connects to her peritoneal cavity. That is definitely a complication we don't want to have.

Julia's sats have been cycling in the 70's for the last couple of hours, and she is not as responsive as she was yesterday. Her arms are quite limp, but she has rigidity and clonus in her legs. Her disconjugate gaze and nystagmus are a little worse. All of these symptoms sound a bit like what has happened prior to other episodes of high ICP, but she still had a nice long game of kissing hands this afternoon, and her blood pressure has normalized slightly on the high side. It is very hard to know what is causing these symptoms, so the doctors have ordered a CT scan to take a look inside and be sure that the ventricles aren't getting larger again.

In the meantime we sit and wait, look and watch. Right now Grandpa and Aunt Cheryl are out getting a bite to eat while Julia sleeps here in her 'craftmatic' bed, listening to her Baby Mozart CD. Dr. Howrey gave us the fantastic news yesterday that all of the new cells that Julia is growing are donor cells. She is now growing enough of her own platelets that she is well into the normal range, on her own. We had to give her a little bit of growth factor today to spike her white blood cells, just in case she is starting an infection somewhere, even though this count is currently in the normal range at 7.3, without having had any growth factor for many days. It will be possible to begin seeing true cure and remyelination taking place in the near future. Everything is in place; we now just need to get past all of these complications!

Well wishes and prayers are continually appreciated. We have to get our strong willed little Juli-anderthal to understand that the hotel is even MORE fun than the hospital, and that she will really enjoy outpatient status. We need normal right now: normal blood pressure, normal ICP, normal breathing patterns, normal heart rates, and normal temperatures. Nothing too high or too low, but right in the middle.

~Julia and her mom

 Return to Index

 

Update 44

Julia's Journey #44 11/10/98 T+82

Julia's blood pressure continues to go up and down sporadically, 80 over 19 one minute, 116 over 71 a little while later, 92 over 41 a few minutes after that. Now we're limp on the left and rigid on the right. A little while ago we were limp all over. This morning we were rigid in all extremities. Her heart rate can vary 30 or 40 beats per minute in a half an hour's time, all while she's sleeping. Geesh! Trying to determine which symptoms are important and which to ignore is definitely a full time job. There is only one certainty: Julia is always full of surprises!

The reigning theory at the moment about these vast fluctuations is that they probably indicate that the Krabbe's is affecting the parts of her brain that regulate basic functions. As with most things here, this bit of news can be considered both good and bad, if it is in fact what is really happening. The bad part is that we definitely do not want any more parts of Julia's brain affected by Krabbe's than already have been. The good part is that if it is Krabbe's then we are already doing the most we can to fix it. If the problems are being caused by Krabbe's then they are not symptoms of a more emergent problem, like high ICP, that would require an immediate fix. Julia's new cells are cranking out the formerly missing enzyme, and day by day we come closer to having more enzyme reach its destination. Enough enzyme will correct the problem. We just have to pray that a large enough quantity gets where it needs to go in enough time, before the disease progresses too far.

Since everyone seems currently to believe that the interesting fluctuations in all the vitals are due to Krabbe's, which is not fixable by sitting in the hospital, we are back on target for the unmentionable departure, possibly tomorrow. Once again, I haven't raced to get the computer over to the hotel room. So far all of the cultures have not grown, but Julia is on antibiotics just in case she is still septic. The most recent CT scan shows no change, making high ICP less likely, unless it's intermittent. So, we're assuming the unusual stats are either Krabbe's, or...

Temperament. Yes, it's a possibility! Julia can't talk yet, after all, but she has had ample opportunity to figure out how to hold her breath and get a lot of attention. Biofeedback used to be pretty popular. Hmmm... can one control her own blood pressure just by thinking about it? Julia's favorite doctor, Dr. Sherry Bayliff, came to visit tonight again. Julia always becomes very unhappy when Sherry leaves. The last time Sherry visited Julia had her first big "blue" breath holding session, three weeks ago, not more than a few seconds after Sherry left the room. Tonight everything was fine as Julia and Sherry talked and played, until Sherry left. Julia's blood pressure plummeted into the teens almost immediately. Granted, Julia doesn't have a lot of ways to express her heart's desire, but this method of communication is a little extreme!

So, let's hope that Julia's downs and ups can be attributed to temperament. Maybe she's getting it all out of her system before she's a teenager. I certainly hope so, and I also hope that we can have a permanent change of residence to the hotel soon. No high ICP, no low blood pressure, high sats, normal heart rate, active and alert baby: our goals for the next few days.

~Julia and her mom

Return to Index

 

Update 45

Julia's Journey #45 11/13/98 T+85

Julia has had two good days and nights, now, with nothing happening that was very unusual or too out of the ordinary by her standards. So, today when Dr. Szabolics was in our room doing rounds he casually mentioned the "D" word and Monday in the same sentence. I caught my breath and quickly looked at Julia. She appeared to be soundly asleep. I sighed and allowed myself a flicker of hope that maybe she didn't hear him and it might really happen on Monday. Dr. Szabolics went on to say that Julia's engraftment looks as good as it can: her platelets are high into the normal range (180's consistently!) and she's definitely making everything on her own; they are all donor cells; GVHD is not in appearance anywhere at this point so we can lower the steroids even more; we have reason to believe that the shunt is working; etc. All of this is fantastic news. :-))

Dr. Szabolics left the room and went down the hall to round on another patient. I sat down to have lunch and contemplate a possible change of surroundings. The alarm went off for vent tachy, fast heart rate, and Julia began breathing very rapidly. We are now waiting for the respiratory people to culture her newly copious nasal secretions to see if she has caught the virus that a couple of other kids on the floor have. Sigh. Ninety-five days spent in the hospital may have served to turn me into a superstitious person, or Julia really is a super-savant and can fully understand English at 10 months of age, but there seems to be a definite correlation between vocalizing that "D" word and postponing our departure! Let's hope that the nasal secretions are a fleeting phenomenon and that Julia proves my superstitious worries to be unfounded.

A quick word on hand washing and colds: One of Julia's favorite nurse practitioners here told me that transplant families benefit from their knowledge of the importance of effective hand washing. The other members of the family of a transplantee have significantly fewer colds during the year following transplant! Judging by some of the emails I have received in response to the hand washings update, clean hands are probably one of the most underutilized disease preventatives available to modern science. One mom told me about her daughter's preschool class where all the children rinse their hands in the SAME basin of warm soapy water prior to snack. Another mom decried the lack of any type of soap in the restroom of a local restaurant's kids' play place, and management's lack of concern. The employees who cook and serve the food have to use the same restroom, so, how do they get their hands clean? The amazing part about the many stories relayed to me is that most of us are as healthy as we are. A working immune system is an amazing bit of physiology.

Many thanks to everyone who sends well wishes and prayers. We are grateful for your continued efforts, and we feel the effects of those efforts on a daily basis. We will soon be at the 100 day post transplant assessment, and Dr. Szabolics is confident that from a transplant standpoint we will be in very good shape. Of course, we still have the complications to attend to... shunt functions, Krabbe's progression, relearning how to breathe and cry at the same time, swallowing without aspirating, avoiding viruses and infections of all types. Nonetheless, I'm hoping to be living in a latitude more conducive to snow by Christmas Eve. Michigan would be nice!

~Julia and her mom

Return to Index

 

 Update 46

Julia's Journey #46 11/15/98 T+87

Here we are at another eve of a proposed exit day from the hospital to the hotel. Hmmm, let's see if we can accurately assess the chances that we really make the move or not. Today Julia avoided any major crises. We left the hospital on pass and went for a walk in the warm fall day, and spent some time in the hotel getting Julia accustomed to the scenery. Her caffeine level must be effective (80mg 4x/day, about the same as 5 cups of coffee) because we had virtually no apnea spells, a record in the last few weeks. Julia also did not turn blue while trying to cry

even once, for the first time in several days. The labs values as drawn at 4 AM were all within normal range, platelets at 160, white blood count 10, red blood cells 11. Yeah! And, fortunately, so far she has not developed any more signs of the flu-like virus that a couple of other kids on the floor have.

But, of course, there are the puzzling new symptoms, a daily event. Julia was VERY tired today, although able to be roused. Every time I woke her, she glared at me with knit brows similar to those on the attached picture, as if to say, "Go away! I need to get my beauty rest so I can grow some more of these eyebrows!" Then, she would go limp and go back to sleep. No kissing hands today -- too tired. Also, we spent a lot of time the past couple of weeks trying to keep her heart rate above the 90's. Over night it crept up to the 150's, and has stayed there all day, a significant departure from the recent baseline. All the other vital signs remained at their former rates, including blood pressure. Hmmm. What's up?

The current theory about the heart rate is that since Julia has finally reached the "therapeutic" blood level of caffeine the higher pulse may be her new resting heart rate. Six cups of coffee a day would probably make my heart beat at about the same rate, so this theory sounds reasonable to me. We figured out that a probable reason why her blood pressure had been dropping every morning around 6 AM was because she would slide down in her craft-matic until her head was at the same height as her stomach. When this happened the gravity operated shunt couldn't drain. Since we learned to prop her higher the low pressure hasn't come back. The only remaining puzzle with out a probable cause is why she is so tired. Just the fluke of the day, or something to be concerned about? It certainly does not appear to be indicative of a problem serious enough to prevent us from leaving, but the door at the front of the hospital opens two ways. We definitely do not want to revolve out, and revolve right back in after a couple of days.

So, we will wait and see. The decorations and computer are still in our room, but everything else is gone. We'll get the word from the doctors in the morning, and can be done moving after a couple of quick trips to the hotel. What do you think? Will we stay or will we go? Please keep those well wishes and prayers coming, and stay tuned for the next update to find out if Julia decides to change her address or not.

~Julia and her mom

PS: The attached picture was taken on September 17th, the day before Julia first exhibited symptoms of high intracranial pressure. She is on the therapy ball working with Jenny, her physical therapist. It is a favorite of mine because Julia was just starting at that point to exhibit enough disposition to make her feelings known, and she's really glaring at Jenny for making her work so hard. You can clearly see how much hair Julia had left after the chemo. Several days after this it was shaved off for the first ventriculostomy. The fantastic news is that Julia is now back to doing the same tricks on the ball, and glaring at Jenny just as she was in this picture, taken two months ago. And, her hair is growing back!

Return to Index

Update 47

Julia's Journey #47 11/19/98 T+91

WE'RE OUT!!! Yes, it's true. We were able to leave the hospital and become outpatients on Tuesday, exactly 99 days after we entered. Tuesday was an exciting day, packed with last minute things to do, and not a small amount of trepidation. After all, we have been within hours of leaving several times in the past only to have our plans altered at the last moment. We had spent the afternoon on Monday dipping Julia's feet in ink and stamping cards, rather a fun time all around. Monday evening after considerable thought I decided that I really felt like this time we were finally leaving. Julia and I had a little "talk" in which I told her that I've left it up to her in the past, but this time I was taking control. I went around the room removing all of her toys and decorations and took them to the hotel. The only personal things remaining were the signs on the door and the computer. {I was confident, but not foolhardy! Remember, we had had egress nixed on the scheduled day more than once before.}

The next morning I woke up and quickly put one eye on the monitors to see if all the stats were within healthy range. Check. Everything looked normal. Then I put the other eye on Julia, who opened her eyes and gave me a snake tongue. Check. Julia looked willing to leave. The doctors arrived not long after, and check, we all agreed that today was the day! From that point came an avalanche of activity: instructions on how and when to draw blood, times and amounts of medications, symbols and signs to watch for, names and numbers to call, CPR refresher course, sad and happy good byes. I finally got to eat breakfast by 3:00 PM. Thanks to Mikey's and Brooke's moms' help, it only took three trips with the wagon to the hotel to get us moved. One trip was for Julia's oxygen tanks, hospital supplies and personal effects, another for the computer, and the third for the desk and chair we had purchased in August so that we could have a full size computer in the room. We looked a little strange wheeling that across the street on a Little Tykes wagon. We were finally able to leave after waiting for the pharmacy to concoct a month's supply of Julia's caffeine. The nurses tossed tissue paper confetti as we exited the unit into the big wide world.

The real activity began when we got to the hotel. The home health care provider arrived to teach me all I need to know about Julia's new equipment. I learned how to hook up and adjust the oxygen supply, and change the tanks. It's very important to pay attention to the little details, like remembering not to light any candles around the oxygen, or WHOOSH! The resultant flames would probably dwarf our current set of problems. The visiting respiratory therapist also gave me a lesson on the apnea monitor. After extensively tutoring me on the intricacies of where to connect Julia to the machine and how it works, he told me that the machine was set go off if it didn't detect a breath for more than 10 seconds, or if her heart rate was above 130 beats per minute. The alarm sounds like the fire alarms most of us have in our homes. I laughed out loud. Julia doesn't breath for at least 10 seconds just about every minute! And, the doctors had just spent several days convincing me that I shouldn't fret unless her heart rate was above 180. Maybe it was a typo, 180 rather than 130?

But, no, those were the stats. He could not change those settings unless Julia's doctors ordered them changed. Julia's doctor was probably home and preparing for the next day by this time. So, just like a normal kid, when we hooked Julia up while the therapist was here Julia breathed regularly and kept her heart rate at 125 or below. Then the therapist left. The door closed and two minutes later it began.

BBBEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEPPP!

BBBEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEPPP!

BBBEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEEPPP!

The memory on the machine was 99% full within the first two hours. I pulled the plug by 10:30, wondering how safe it would be to try to catch a few winks, since the pulse oximeter alarm is not loud enough to wake me. I decided to suspend my anxiety and try to act normally. I set Julia and her machinery up close to the shower so that I could hop in and hear her when she decided to cry. She rocked her head back and stopped, turning blue, holding her breath. I picked her up to stimulate her, began pumping her chin, pumped harder, the pulse ox dropped to the 20's. I laid her down and hooked up the oxygen and put the cannula on her face, still stimulating her and pumping her chin. I looked at the machine which now said "3" in the oxygen saturation column. Julia was turning a deep shade of periwinkle, but not giving an inch. I grabbed the phone to dial 911, when finally she broke and emitted a cry. Sigh. If all nights were going to be like this I was going to move back to the hospital. I'm pretty calm through most situations, but I must admit that my adrenaline was pumping pretty high this time. I moved Julia right into the bathroom with me while I took a short shower. I was not comfortable sleeping without the loud apnea alarm, so I dozed sitting up next to Julia with my head a couple of feet from the pulse oximeter. The rest of the night went smoothly.

I gave Julia her magnesium and feeds at midnight, caffeine and feeds at 3:00 AM, and rose at 6:30 to draw blood and administer the first morning's set of medications. I was confident that I could figure out the doses and volumes of the seven different meds that I was to give Julia that morning. Drawing all the meds that went into the 10cc syringes was easy. What I didn't anticipate was the difficulty I would have in drawing meds into needle syringes. I tried and tried. And, tried and tried. Urgency was added to the situation because Julia had spiked a temp to 39.6 C. After fifteen minutes of trying to get .28cc of Cyclosporine into the needle syringe I decided I had better ask the nurses in clinic what the secret to using the needle syringes is. Simple they told me: Remove the needle!

From what I hear, I had a pretty standard first night out of the hospital from the standpoint of anxiety provocation, although the specifics vary case by case. I was happy to get to clinic Wednesday morning, where we determined that after a dose of antibiotics we would watch Julia from the hotel rather than readmitting her to the hospital for observation because of the fever. Fortunately her fever disappeared quickly, and we went on to have a couple of very good days yesterday and today. Julia seems to be settling in to life here at the hotel. Her balloons are floating around, and all of her toys are within an arm's reach. Our good music is here, and the weather outside has been conducive to nice walks home from clinic. We are adjusting quite nicely. Julia's grandmother will be arriving tomorrow to allow me to sleep more than a couple of hours at a stretch. She will stay up all night while I sleep so that we can be sure Julia doesn't pull a deep sat "blue", with me too tired to wake to the alarm.

Julia is doing very well from a transplant standpoint. We now must wait for the missing enzyme to reach its destination and hope for rapid remyelination of the parts of Julia's brain that have been compromised due to her disease. We will be able to return to Michigan when Julia has reached a point of stability in all of her basic life functions. In the meantime we will enjoy our new surroundings. I appreciate all of your well wishes and prayers more than ever. We have achieved one huge milestone, and look forward to working equally hard on achieving many more.

~Julia and her mom

Return to Index

Update 48

Julia's Journey #48 11/24/98 T+96

We've been out for a whole week! What great news. Julia has given us a few scares, but we've managed to avoid being readmitted. Besides the usual sats problems Julia has gained about a pound and a half of fluids since becoming an outpatient. She had to have Lasix as an outpatient twice so far. Lasix is rapid acting diuretic, which she had all of the time during the massive fluid imbalances she experienced during the early days of the transplant and after surgeries. By Friday of last week she had very swollen feet and hands, so she received a quick dose of Lasix in the clinic. We had to stay in clinic for about 20 minutes after the dose ended so we could make sure she would urinate, usually a flood after Lasix. I removed Julia's outfit to avoid having it get all wet and waited for what I hoped would be a deluge.

That's when things got exciting, only this time Julia wasn't the cause. Julia had just had a sinus sample taken to check for viruses, along with some deep suctioning. I started to smell something. I am getting used to this feeling of experiencing symptoms of whatever Julia is feeling, such as headaches during times of high ICP or stomach aches during intestinal upsets. Sinus samples and deep suctioning are not fun for the kids, so I wasn't surprised to feel some sympathy symptoms. I investigated further and thought, "Gee, this smells really awful!" Pretty soon I thought I saw smoke, so I opened the door to the room and, yes, I saw a LOT of smoke. And, a lot of people leaving! The nurse said that there was a small fire, so we all left (without taking time to dress the wee one, who post-Lasix could have put the fire out by herself) and went outside to watch the fire trucks and police cars line up. They quickly doused the small fire that had broken out in the dirty laundry section behind the check-in counter at the clinic. A wicked thought went through my mind. Some poor patient must have waited just a couple of minutes too long in line for his paperwork.............. ;-}

Yes, lengthy periods of waiting comprise the major story of clinic. We are required to do our blood draws early in the morning and take them to clinic by 8:30 AM. The subsequent wait for paperwork, exams, lab results, medications and so on can be QUITE extensive, sometimes several hours. But, it is broken up by seeing old friends from the floor, doctors, nurses, other parents, and kids all doing well. Julia heard her favorite doc talking outside the room, and demonstrated her affection for Dr. Sherry by starting her kissing hand and snake tongue before the doctor entered our room. Julia also has a new game of holding hands. When you try to let go she grasps your finger more tightly, making it obvious that she doesn't want to stop playing. An interesting side note is that just as Julia lost most of her hair and is now growing a new doo, she is also getting a new set of fingernails which appear to be growing underneath the old pre-transplant set.

We have been required to go to clinic everyday for the first week, but today we have a scheduled day off. This will be the first day since August 9th that we haven't been in the hospital, and only about the 10th since mid-June that we haven't been in either a hospital or clinic. Let's hope it stays that way! Julia has a 103 F temperature right now. She has run a temperature spike every morning since we became outpatient. She was receiving antibiotics, but since all of the cultures keep coming back negative, we now think that the fevers are the result of the Krabbe's erratic temperature regulation. If the fever doesn't break within a couple of hours we will have to wait until another day to have our first full day out of the hospital.

So, please continue to send those well wishes and prayers our way. We have our 100 day tests next Tuesday. We will probably get a projected date to return to Michigan after we receive the test results. Christmas is getting closer and closer... :-)

~Julia and her mom

PS My computer has been having some problems connecting to the net and sending/receiving email, which seem to be repaired now. If you didn't receive update #47 or sent me something within the last week to which I didn't respond, please let me know. Also, please note that Julia's website has a new address: http://www.krabbes.com/juliasjourney/

 Return to Index

Update 49 

Julia's Journey #49 11/27/98 T+99

Yes, that's right, 99 days out from transplant, and ten days out of the hospital! Next week we will be doing our 'hundred-day' tests, which will tell us how well Julia's transplant is progressing, along with a prediction for when we will be able to return to Michigan. Her transplant statistics currently look very good. She is producing lots of cells on her own, platelets (clotting cells), red blood cells (oxygen carriers), and white blood cells (infection fighters). Her shunt seems to be working, so long as I am always very careful to keep her in an upright position. Although she's a little fluid heavy, it is nowhere as bad as it has been at times in the past. The doctors are starting to say the coveted words, "successful transplant", when discussing Julia's condition.

All of this good news made for a very nice Thanksgiving Day. We received turkey dinner with all the trimmings from David's (the Krabbe kid in town) parents' church. We definitely have a great deal for which

to be thankful. Julia's cell counts, shunt function and balanced metabolism were all hard fought for, and we know how lucky we are to be where we are today. We also know that this is just the start of a long road ahead. Julia's disease has progressed since August when she had the transplant, and it will be hard work to keep her going through the next several months before enough Galactocerebrosidase, the enzyme she is missing, is carried to the parts of her brain where it is needed to ensure that she will keep living.

Julia's last MRI showed significant brain stem Krabbe's involvement, and it is very evident on a day to day basis that the primal functions controlled by this part of the brain are affected. Her heart rate fluctuates dramatically (90's to 160's while sleeping), her temperature ranges from 33.5 (92) to 39 (103) within hours, she only swallows her saliva a couple of times each day, and she forgets to breathe on a regular basis. These symptoms are indicative of the later stages of Krabbe's. We are in a big race to see if enough enzyme gets where it needs to go before the genetically predetermined Krabbe's time bomb takes too great a toll.

Once the enzyme finds its mark and the disease progression is stopped, then Julia will need to undo the damage that has been done. We work daily on helping her remember to swallow. I position her like a Buddha, insert a variety of occupational therapy devices into her mouth to motivate her to perform a normally reflexive swallow, then massage her trachea until I hear a "gulp". Sometimes I only hear one or two gulps per day. Yet, it is important to keep trying because the less she forgets, the easier it will be for her to learn more abilities, like eating food rather than only swallowing.

Breathing is much the same. It is very common for Julia to neglect to breathe for 20 or 30 seconds. In most of us the lack of oxygen and build up of waste gas in our brains would excite us to gasp for air after not taking a breath for that long. But the lack of myelin in Julia's brain makes the BREATHE NOW message take a very long time to get to her lungs. Julia also seems to have returned to a no-cry state, so I am hopeful that she didn't forget how again.

The reason why these neural messages travel so slowly in Julia is because the missing enzyme prevents the myelin sheaths that surround her nerves from forming correctly. This is very similar to trying to send a telephone message if many of the wires were missing the rubber insulation that surrounds the copper. The messages would short circuit and become jumbled, taking a long time to reach their destination. Julia must remyelinate if she is to have any hope of normal function. By normal function I am referring to breathing regularly, swallowing, and even blinking. Advanced functions such as talking, walking, hugging, and smiling are a LONG way off, and in no way a foregone conclusion. The good news is that there has been proven remyelination after transplant with this disease. Due to the rapid progression of her disease, Julia will have to remyelinate substantially just to achieve the basics. If you are unclear about what I mean by this concept, please rent a copy of the movie "Lorenzo's Oil", with Susan Sarandon and Nick Nolte, and you will soon understand what will happen without remyelination.

Yet, we are hopeful. Julia is young, still at an age when kids myelinate rapidly. And, we stimulate her day and night, every moment she's not sleeping, through all of her senses, to keep those pathways open. It is clear that she responds to this stimulation, and that she very well understands love. She can't smile as we can, yet, but when she's really happy she works her facial muscles into what might look like a grimace to those who don't know her. But we know what she means!

On this Thanksgiving weekend I am very very thankful to have her with us this far, to get those grimace-smiles, and to have the chance to complete the rest of the journey. Please keep sending your well wishes and prayers. Our little grimacer is smiling inside in response!

~Julia and her mom

PS: Sorry for the wrong web address in the last update. Julia's site has changed to

http://www.Krabbes.com/juliasjourney/

Return to Index

Update 50



Julia's Journey #50 12/2/98 T+104

We are more busy than ever. Yesterday Julia went for many of her 100 day tests, to determine when she will be eligible to go home to Michigan. At 9:00 AM she had her ECG, 10:00 AM vitals and statistics (she grew an inch and weighs about 4 pounds more than she did in July), 11:00 AM blood draws (30 cc or so to many different labs), 12:00 PM pulmonary function test. Time for lunch. Up to the pharmacy, over to the business office, and back to clinic to schedule the rest of the week and discuss Julia's progress with Dr. Kurtzberg, who gave us the fantastic news that Julia's missing enzyme is at 100% normal levels in her blood! Finally, back in the hotel by 7:30 PM, in time to prepare for an early bedtime so we could arrive on time for our 7:30 AM MRI this morning.

Julia's test results indicate that everything is in order to have her central lines removed and a port installed so we can still draw blood easily while cutting the risk of infection. Central lines are the small white hoses that have extruded from Julia's chest since August. They connect to the blood vessels close to her heart, allowing rapid access so that it isn't necessary to poke Julia with a needle every time she needs new medication or to have blood drawn. These central lines prevented Julia from receiving thousands of pricks over the last four months. The lines have caps on the end that seal them from the germs circulating in the outside world. But, despite the most conscientious of efforts, central lines still sometimes get pulled out or infected. NO THANKS! The alternative is to have a port put in, which is a reservoir of blood under the skin that makes blood draws easier than searching for a vein each time. Because it is covered by a layer of kin it is much less likely to become infected then central lines are, and even a G-tube menace like me would have a hard time pulling it out. The port installation operation will take place in about two weeks.

We will need to wait about a week after the port is installed to be sure there are no side effects or infections from the operation, and then we will be free to go home, hopefully just in time for Christmas. The doctors here have started to transfer information to the doctors at home. I have started calculating the easiest way to transport Julia and all of her new equipment back Michigan. When we arrived in July I had one baby, one stroller, and one overnight bag. I now have one room nearly full of all sorts of stuff that definitely will not fit into my suitcase. Suction machines, apnea monitors, pulse oximeters, toys, clothes, syringes, tubes, medications... not even a Houdini could get this mass quantity on a plane under one ticket.

It will be great to be home, though I am fond of North Carolina. Two days ago I had shorts on, when I saw several Christmas trees on top of cars in the parking lot next to the hotel. A nurse standing in the hospital lobby was wearing snowman earrings. I've even seen some houses decorated with Christmas lights. HOW can these North Carolinians think about Christmas when the weather is appropriate for Labor Day weekend?! I have a hard time playing Christmas CD's when it's in the mid seventies outside. I've explained to Julia that even bona fide Southerners like to be where it snows for the holidays, just in case she starts thinking that the hotel is really home. Besides, Alison and Cameron are really worried that Santa Claus might not know where Julia is if she's not home with them for her first Christmas. They definitely do not want any presents being delivered to the wrong address! ;-}

It is so nice to be considering all of the details of the trip home. I am very thankful that we have made it this far. Another little Krabbe kid, Joseph, just began chemo here two days ago. He is only a month old, and without symptoms. His older brother, Matthew, was one of the first two children with infantile onset to be transplanted. Matthew is an inspiration to the rest of us. He had fairly advanced symptoms when she received his transplant two years ago, but now interacts with those around him and gave me a big smile a week or so ago. I have also heard that there are two other Krabbe's kids who will be transplanted soon, getting their second chance to live. The word is out that Krabbe's may not always be fatal!

Julia has a wonderful group of friends back home who are helping to spread that word. They are putting together numerous activities to raise awareness and funds, such as planning a candlelight vigil in Michigan in conjunction with Hunter's Hope on Valentine's Day, which will be Hunter's second birthday. You can read more about this on Hunter's website, http://members.aol.com/hunterhope. Julia's Friends are looking for good but easy recipes to go into a cookbook they are compiling. They have also spent a lot of time hand tying ribbons the same color blue as Julia's eyes, with white rosebuds in the center symbolizing Julia's pure will to live. They are asking a $1 donation for 2 ribbons, funds to go to Julia's Friends. Please email Julia's Friends at norahjoyce@hotmail.com for more information on ribbons, events, how you can help, or just to say "hi" to Julia's dear friend, Mary.

And, be sure to take a moment to view the attached photo of Julia decked out in her favorite hat, jacket, flip-lens sunglasses, and all-important mask. Yes, that's Julia's really in there, ever grateful for all of your prayers and well wishes! Please keep them coming. We're closer to home, but still have a way to go.

~Julia and her mom

PS: A special HAPPY #1 BIRTHDAY to David, Julia's Krabbe friend, celebrating his birthday today. Lots of love to you, David!

Return to Index

Next Page 

 

Back to Krabbe's Kids

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