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Julia's Journey #51 12/5/98 T+107
We received great news on Thursday: Julia's MRI results showed improvement over the previous MRI! The attached picture of Dr. Kurtzberg and Julia was taken shortly after Dr. Kurtzberg gave us the good news. We had expected Tuesday's MRI to be worse, because Julia demonstrates a fair amount of disfunction, especially in the areas controlled by the brain stem (lack of swallowing, high and low heart rates, and consistent apnea). The theory says that we would not likely see improvement in the MRI until 6 to 12 months post-transplant. But, the T+103 day MRI showed improvement in many areas, including the brain stem! Everyone, including Dr. Kurtzberg, was surprised. There is no sign of remyelination yet, but the inflammation is reduced and the ventricles are smaller. The only negative is that there are some additional hematomas near the shunt site, possibly from when it was last put in. Now we must wait for these to break down and dissolve on their own, or we end up back in the OR if the shunt gets clogged again.
A different difficulty has reemerged, however, such that Julia is back in the hospital again for observation. She started having trouble getting her oxygen saturation into the high 90's. Sats are always an interesting statistic with Julia. She has two "normal" types of desaturations. When she tries to cry and holds her breath her sats will drop from the high 90's to very low depths (teens or twenties) until she remembers to exhale and breathe again. These events require pushing her jaw up and down or bagging her to remind her what to do next. The other type of apnea is the cyclical up/down/up repetition of forgetting to breathe when transitioning from sleep to wake or vice versa because her brain is slow to react to lack of oxygen. Both are caused by Krabbe's, and will improve as the Krabbe's decreases.
Today Julia was trying to breathe, but couldn't draw in enough air. She always sounds congested because she can't currently remember how to swallow, so she "gargles" her saliva, and frequently has to be suctioned. Today, despite repeatedly suctioning and repositioning her, I was unable to get her to breathe deeply enough to get her sats into the nineties at their high point. For about five minutes she kept gasping for breath, and started turning blue, with sats in the forties. When I finally was able to get her sats up, they would only go into the 80's without oxygen. I called and went to the hospital. Interestingly, Julia's apnea disappeared while she was laboring to breathe.
The x-ray technicians put Julia into a piece of equipment that I have never seen before, called a "Pigg", or bicycler. This is a clear tube that fits around her while she is in a sitting position with her arms extended over her head. She was wearing a Santa cap and snowman slippers, and that was all you could see sticking out of the tube. Our old buddy-nurse, Tracy, was with us, and we both agreed that Julia looked so funny with only her Christmas extremities and very dark knit eyebrows at either end of the tube that we were not sure whether we should laugh or cry. I didn't have my camera on hand, or I could have finally paid Julia back for the famous Time picture.
Ultimately the chest x-ray showed a small spot that might be fluid overloaded in one of Julia's lungs. So, we are now camped out in room #5206, five doors down from our old hangout, waiting for a better x-ray tomorrow. Many viruses are going around, and the big fear is always that these transplant kids will catch one before their immune systems are able to handle them. We've had Julia's lines and secretions cultured again, and are hoping that all these test come back negative. One of the aides punned that with the large numbers of cultures Julia has had in recent days she should soon be speaking 3 or 4 languages and singing opera.
So, I am writing this on a quick trip back to the hotel while Julia rests in a new craft-matic in the hospital, which we've explained does not belong to her. I've promised to ask Make-a-Wish for a craft-matic if she promises to get us out of the hospital for good. Her sats are in the high nineties on a quarter liter of oxygen, but drop to the low eighties without it. We're directing our thoughts to nice clear lungs. Your well wishes and prayers are appreciated as always.
~Julia and her mom
Julia's Journey #52 12/7/98 T+109
Julia really likes that Duke North address. For two days now we have worked on weaning her off the oxygen. One-half liter, one-quarter, one-eighth, now back up to one-quarter liter. Julia's sats drop to the mid eighties within a couple of minutes every time we take the oxygen cannula away from Julia's face. There is no question that she needs this little oxygen boost, but why is a mystery. She has had a x-ray every day for the last three days, each one showing us no large scale areas of lung distress. Julia is rousable, but definitely not active. She does not want to play snake tongue or kissing hands, and remains basically limp throughout physical therapy. She's definitely not up to her peak performance, although a little better than she was on Saturday when we first came in. I very much want to return to the hotel, but we will need to stay put until we know Julia is able to be off the oxygen for substantial periods of time.
We are on a big search to find the cause of the desats. Julia's x-ray today appeared basically clear, so another nasal battery of cultures was done. It takes a few days for viruses to grow in these cultures, so we won't have any answers from that test for a while. In the meantime Julia continues her antibiotics. If the nasal cultures remain negative but the symptoms don't abate then we will need to do a bronchioscopy. Julia would be intubated with a probe that would go into her lungs to take a small sample of tissue which would then be tested for a variety of problems. Unfortunately, Julia would need to be sedated for the procedure, something to be avoided with a Krabbe child, especially one who is already not breathing well. Julia's better option is to breathe long and deep on her own and avoid this test! :-)
There are currently three Krabbe's families on the floor, Julia, Mikey, and Joseph. Joseph is Matthew's little brother. Matthew was the second Krabbe's patient transplanted at Duke. He is now three, and speaks several words, smiles, and eats on his own. An inspiration! Two more Krabbe's families toured the unit today, here for a consultation, one from Alabama, and the other from the state of Washington. Each of these families found out about the possibility of transplant via the internet. It is good to see that this option is being discovered hopefully in enough time to help kids recover from this disease.
Meanwhile, my goal is to get Julia out of the hospital to make room for other kids to have a chance at overcoming their illnesses. So, help her out by sending her all the deep breath good thoughts that you can muster. She needs more and more well wishes and prayers. It might sometimes seem that we can let up, but I have come to realize that Julia demands full attention. When can we rest? When Julia herself tells us, in English, a year or two from now!
~Julia and her mom
PS: Note to newcomers to these Julia's Journeys: Archived updates are located at http://www.krabbes.com/juliasjourney/
Julia's Journey #53 12/11/98 T+113
Julia was released from her second hospital stay last night. Her sats were finally rising to the high 90's in between apnic sessions, which is where we were before she came down with whatever was bothering her last weekend when we went in to the hospital. We are now on oxygen, just a small amount, all the time, however, to keep those sats up where they need to be. It is becoming a real project to walk across the street with baby, pulse oximeter, and oxygen tank all in tow.
Julia had a four channel pneumogram test on Wednesday to determine the cause of her apnea. This test correlates heart rate, respiration,oxygen saturation and CO2 and prints out lots of statistics on how often the patient doesn't breathe. Julia was hooked up to these machines from 9:45 AM until about 7:00 PM, and was supposed to "sleep" and not play. Finally! A task to which Julia could attend with flying colors! I was proud of her. She slept most of the time and had a great day. The test showed Julia to have 142 desaturation events in the nine hour period, the longest of which was 110 seconds. Try to hold your breath for 110 seconds, and you will quickly notice that if Julia does this very often she has a rather serious problem.
The diagnostic service that graded the test determined that Julia's apnea is obstructive in nature, not generated in her central nervous system (brain). We think that she may not be able to control the muscles to her tongue that keep the tongue forward while she attempts to take a breath. In this sense, the intial problem is CNS, but becomes an obstructive situation. The answer may be to mechanically open the airway. We tried that today in clinic, by inserting a "trumpet", which is a tube that extends out her nose down her throat to make a clear passage for breath. The first four hours of observation went well, and we returned to the hotel tonight just after dark.
When I took Julia out of her stroller and removed her coat she desated into the low 70's for about 10 minutes, arriving at a rather unbecoming shade of dusky blue. She was unable to draw breaths through the extra secretions caused by the procedure. I called the doctor on duty, removed the trumpet, and trotted right back over to the hospital before we even had the chance to settle down for dinner. Julia received some deep suctioning, and I had another conversation about what to do next. Julia has been breathing much easier without the trumpet, which probably was not inserted in the right position, so I am hopeful that we will last the night here in the hotel. Tomorrow we reevaluate the merits of the trumpet, and discuss the likelihood of a tracheostomy.
Our several day stay in the hospital made me realize how weary it is possible to become after several months of making hourly decisions about the survival of one's child. A couple of other outpatients have had to return to the floor along with Julia, although the reasons for their temporary change of residence varied from ours. We all agreed that the second tour is hard on us mentally, even though we were already seasoned veterans at maintaining good mental attitudes because it is necessary for the well being of our children. In my case, I refused to bring all the creature comforts back to the hospital from the hotel, Julia's toys or mine. It was really obvious to me how much I relied upon these diversions to keep my mind occupied and happy for the last few months. During the last several day visit I had all day and night to ponder unanswerable questions like "When will we go home to Michigan?" "When we go home how long will it be before life is normal?" "Will I ever be able to have another job (much less career), or will I always need to take care of Julia?" "What month or year will be the first time I get to sleep just once through the night?" and of course the most insidious of all, "Why me?"
But, now we are out, and trying very hard to stay that way. It is a pleasure to catch up on emails and cards, in the midst of keeping on top of Julia's therapies and meds. {Please be patient if you haven't heard from me in a few days!} Julia is responding very well by moving her ankles and legs without stimulation, and making a wide variety of facial expressions I haven't seen for months. No, she doesn't swallow or breathe regularly, but her progress in other areas makes me believe she will. I really want to keep her out of the tracheostomy operating room in anticipation of her regaining basic functions, but am glad to know that the option exists if necessary. Please keep those clear air well wishes and prayers coming for this little girl who is remembering how to do what we take for granted.
~Julia and her mom
Julia's Journey #54 12/14/98 T+116
We nearly lost Julia today, code blue in clinic, 911, mouth-to-mouth resuscitation, ambulance, emergency room, PICU... Guess she wants her tracheostomy.
We had a great day yesterday. Julia's number of movements and range of motion has been increasing day by day. When I pour water on her legs during bath she now draws them up to her stomach, bent at the knee. She keeps her eyes towards wherever she hears my voice. Unfortunately, one area that seems slow to improve is swallowing. Yesterday she sounded congested, but actually effectively coughed up mucous mid day, leaving Julia mucous free for several hours. I had considered taking her over to the hospital for deep suctioning prior to that, but yesterday ended up being our first truly hospital-free day since July, not even any blood draws.
Around 2:00 AM this morning Julia began sounding congested again. By the time we went to clinic she needed the respiratory therapists to do deep suctioning. After that, and a detailed conversation with Dr. Martin about tracheostomies, we came back to the hotel. Julia's sats began dipping into the 70's, but not rising beyond the 80's, despite repositioning, suctioning, chest PT, and anything else I could think of. Her secretions were so dry that they were like glue. I couldn't move them. I called clinic; over we trotted.
We decided to try some saline water to loosen the mucous. Our nurse practitioner spritzed just a bit in her nasal passageways and I suctioned. Ann left the treatment room to go get more supplies, and I kept suctioning. Julia made a low growl, and turned dusky. I opened the door to our room but didn't see anyone, so I upped her oxygen tank to the highest setting and packed her up to take her to the room where oxygen is available in higher concentration. She turned full blue and froze in one position. I began running to get to the room a quickly as possible and telling everyone I saw in the hall to get some help. I wheeled her into the Rainbow Day Hospital and kicked a table out of the way to get Julia and her stroller (to which she was attached to her pulse oximeter) in a position to reach the oxygen. She emitted a primal scream and pushed much of the mucous out of her mouth and nose, but could not draw a breath. She was deep, deep blue. Her saturation read 1, and her heart rate had dropped from the 180's to the low 50's.
By this time the other patients in Rainbow Day were rushing out and people who could help were rushing in, doctors and nurses followed by emergency workers and ambulance personnel. I stood back and watched, turning down an offer to leave the room. I was frightened, but felt that if she had to go through this the least I could do is be there if there was any way to help. It was not evident for some moments in time if she would survive. My thoughts were rapid and adrenaline driven.
Julia received mouth-to-mouth and was resuscitated. We were able to transport her to the emergency room in the ambulance, with a kind clergy person following with our stroller and oxygen tank. She opened her eyes several times located me, and even gave me a kissing hand while we were in the ER. Julia became stable and is now in PICU, resting peacefully. It appears that our God-given fortuitousness in being in the right place at the right time to have this happen has not only saved her life, but hopefully prevented brain damage. We would not have been so lucky if we had still been in the hotel or in transit to the hospital.
So now Julia has been on the 5200 floor, 5300, 5100, PICU, clinic, an ambulance, several operating rooms, and the ER. We know most of the hospital personnel. In fact, it is nearly impossible to go anywhere without meeting someone we know, and there are only 27,000 people who work here. We met the otolaryngeal doctors tonight, as they inserted a scope to view the Julia's respiratory tract. If only she would find the tour to Michigan as interesting as the tour of Duke Medical Center!
Julia will likely receive her tracheostomy on Thursday, after a couple of days of stabilization and observation. The central line removal and lumbar puncture that we were due to receive tomorrow is now on hold. Our journey continues to lengthen, and although I am weary, I am very grateful to have the opportunity to continue. I am thankful that this happened now, and not on Saturday, when we had been scheduled as of a couple of weeks ago to be en route to Michigan. Julia seems to have a way of telling us what is best.
Please keep those prayers and well wishes headed our way. Julia must remyelinate the area of her brain that tells her tongue to swallow. This is a race for the enzyme to quickly cross the blood-brain barrier and act to grow myelin and repair already damaged areas, before the genetically preprogrammed disease progresses too far. These setbacks seem momentous, but there is evidence in Julia's actions that she can win the battle.
~Julia and her mom
Julia's Journey #55 12/19/98 T+121
Initially Julia's planned central line removal and port placement on Tuesday were cancelled, due to the respiratory arrest on Monday. But, luckily we were able to assemble all of the necessary surgeons on Tuesday along with Julia's favorite anesthesiologist, Dr. Alison Ross, and have not only the line changes but also the tracheostomy on the same day. It's important with Krabbe's kids to keep the number of times and amount of anesthesia to a minimum, and doing both procedures on the same day with Dr. Ross accomplished this.
When Julia first came out of surgery her eyes were disconjugate, and the right pupil was far larger than the left. Julia had had to be reclined with her head lower than her stomach during part of the surgery, which didn't allow the shunt to drain, probably causing some temporary high intracranial pressure. We left the operating room to go to the CT scanner after surgery. Fortunately this seemed to be a transitory phenomenon, and the rest of our post-op went well. We were soon in PICU for recovery. On Wednesday Julia began playing a few games and generally seemed much more comfortable breathing than she had before the surgery. Julia had improved so much that we were able to go back to the transplant unit, room #12, Wednesday evening. This puts us on a different wall and gives us a different view than we had in rooms #1 and #6. I need to impress on Julia that nothing rivals the view of the lake from the our home in Michigan!
Julia has certainly had some invasive procedures done, the G-tube, ventriculostomies, shunts, but none requiring the intense perpetual care that the tracheostomy does. My training to be the caregiver began on Thursday. Julia still generates mucous, which must be suctioned through the tracheostomy tube. This is a sterile procedure for the time being, although down the road it will eventually become "clean", meaning that we will not need to have all equipment sterile for each suctioning. Currently we are suctioning about every three hours, but hopefully this will become less frequent as time goes on. She will also need to have the trach tube changed every couple of days, although the first one needs to remain in for a week so that the tract can form, similar to a pierced ear. The first trach change will be performed by the surgical team. I will do all of the others after that.
Trachs are very high maintenance items. It is my great hope that Julia will remyelinate the part of her brain that controls her swallowing so that eventually she will outgrow the need for the trach. As it stands now, I must suction her (a sterile procedure), change the trach tube, keep her away from any sort of particulate that normally would be filtered through her nasal cavity since the direct line to her lungs does not provide any filtration, and keep her oxygen source moisturized so that she doesn't dry out. When we travel it will be necessary to take the oxygen and moisturizing source with us, along with suction catheters, ambu bag, extra trach tubes, and so on. Everything is considerably more complicated than it was before, and it already was quite an ordeal. You can get an idea of what caring for a trach is like and learn some interesting trach details, such as how a trach might have saved George Washington's life, by viewing the tracheostomy website at http://www.cntn.net/cbissell/trach.htm . An especially interesting page on this site that applies to us all is the one on infection control. http://user.aol.com/trachtube/infect.htm It is imperative that everyone around Julia avoid catching a cold. If Julia comes down with a cold the trach care and secretion management will be that much more difficult.
The good news is that Julia seems to be adjusting relatively well to the new apparatus, and is resuming her old games. Yesterday she wouldn't let me stop playing kissing hands, for about three hours! She would raise her reflex hand, "Kiss me!" Then again, again, again... I would hold her hand. Up comes the other (non-reflexive) hand! Again, again, again... I would hold both hands and she would either hold her breath or move her legs! There is for me no greater pleasure than seeing these little advances. Each one is better than any Christmas present. Two nights ago Julia contorted her little face into a true baby crying expression for the first time in many weeks. Of course she can't make any noise because of the trach, but her emotions are very evident. She also is starting to raise the corners of her lips more and more, working those muscles until one of these days we will see a real smile. :-))
We will need to stay in the hospital through at least two trach changes, so it looks like we will probably be inpatients through Christmas and perhaps Julia's first birthday on December 27th. No matter. I will be happy to celebrate with lots of hugs and kissing hands no matter where we are. Another patient on our floor, Cheli, lost her long and hard fought battle last night. We are ever mindful of how lucky we are to have made it to this point, and grateful for all of your prayers and well wishes for an uncomplicated recovery from this surgery. Please continue to keep Julia in your thoughts, and perhaps add an extra prayer for all the other children and families going through transplant as well.
~Julia and her mom
Julia's Journey #56 12/24/98 T+126
Someone up above must have heard our wish to return to a colder climate for the holidays, and arranged the next best thing. We awoke today to trees drooping with ice and a Durham wondering how to handle the precipitation. Julia's motto: If you can't go to Michigan, have Michigan come to you!
Julia's tracheostomy is healing well according to the surgeons who did her first trach change yesterday. Until Julia learns to cough the mucous she has up and out we must use a small catheter to go down her tracheostomy tube and remove the secretions before they obstruct her airway. I am working hard to learn all of the cues of when she needs trach care, which, like many things with Julia, can be somewhat mysterious. The suctioning, bagging, and dropping saline water down her tube is becoming more routine, although it's a little bit like diving for oysters in the dark. Did I go too deep and biopsy her lung? Did I overinflate with the bag? Or, did I not do anything far enough, and miss the mucous that will cause a problem? The respiratory therapists that do this task here in the hospital have several years of schooling and plenty of experience. I've got another week or so to become proficient.
Julia had almost no apnea for several days after the surgery, except one night when she was over oxygenated by too much bagging. She also began playing quite a few games, including one three hour session of kissing hands on Sunday. When I would hold the kissing hand, she would raise the other hand! We were beginning to think that we really had finally found a solution to the breathing problem. Then, at about 3 AM two nights ago she began having severe apnea every five or ten minutes. The next day she was not interested in playing, or even in opening her eyes. Everyone became quite concerned that there was a new problem, but finding the cause has been difficult.
Julia was once again cultured for just about everything. All the cultures have been negative so far, except for some bacteria growth in the airway secretions. Julia is back on three of the most powerful antibiotics to help prevent sepsis, but it is unlikely that the mucous bacteria is causing her apnea. There was a thought that maybe her behavior is some type of seizure. Julia underwent an EEG (ElectroEncephaloGram) yesterday, which ruled out that possibility. This is very good news, because many anticonvulsive medications have a sedative effect, which is one thing Julia doesn't need. We also did another CT scan, just to be sure that her intracanial pressure wasn't high again. This morning Dr. George gave us great news: the CT scan was the best he has seen of hers. Her ventricles continue to reduce in size and there are no new areas of concern that can be seen on the scan.
This still leaves us with the mystery of why Julia appeared to be backtracking the last couple of days. Dr. Kurtzberg mentioned the possibility that the Krabbe's is still getting worse in isolated areas of Julia's brain, despite what appeared to be overall progress on the last MRI. This would certainly be a serious problem, because many kids with early onset and rapidly progressing Krabbe's like Julia has do not live much past their first birthdays. As we've said before, the race is on to get that enzyme everywhere it needs to go, and quickly. The transplant is the only fix for this problem, so now we wait and see what happens.
In the meantime, we are enjoying the holiday spirit and the wonderful winter weather. Many groups have been through the floor wishing good cheer and handing out goodies, including Miss North Carolina, who gave each of the kids a teddy bear. Julia doesn't get to meet everyone in person to cut down on possible germ contamination, but they all get to see her in her Juli-elf outfit through the windows to our room. Please enjoy the attached Merry Christmas photo taken of Julia when we were in the family lounge. Note her smile-grimace, as she works to get her mouth into a smile that she can't quite make... yet.
We certainly never expected to still be in the hospital at Christmas, but I am very happy to hug and hold Julia wherever we are for her first Christmas and birthday. There will be a Christmas celebration on the floor today. Of the sixteen rooms four of them now have Krabbe's children receiving transplants, and I have heard of at least two other families in contact with Duke to see if their children are eligible for this chance to live. Julia, Mikey, Joseph, and now Victoria all have a chance that a very short time ago would not have existed. That is certainly something to celebrate this holiday season.
Thank you to each of you who continues to send so many wonderful prayers and well wishes our way. I feel those prayers at work every day. The gift given to the world on the first Christmas came in the form of child, and the meaning of that gift has never been so clear to me as it is this year. It is our wish that all of you who have worked so hard on our behalf for so many months receive whatever gift you wish for during this holiday season.
Merry Christmas!!!
~Julia and the whole Beatty family, Rob, Wendy, Alison and Cameron
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