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Julia's Journey #61 1/17/99 T+150
We are keeping busy here in Durham! Julia has received her Panda seating system stroller, which allows her to be positioned in the very best way to optimize breathing and bone formation. After several months of inability to move Julia's back is becoming quite rounded (also partly due to steroids), and her weight bearing bones and muscles show some signs of atrophy. When she sits in her new stroller, which she now does several hours per day, she breathes more regularly and her back is in the correct straight position. When we stroll around the hospital to and from appointments she opens her eyes and looks around the inside of her canopy, which protects her not only from raindrops but also from wandering germs.
Julia's apnea continues to worsen at night, however. She simply forgets to breathe when she sleeps. Last night she filled 41% of the apnea monitor's memory in one 8 hour period. Julia takes a deep breath, then several smaller breaths, then none for 30 to 45 seconds. She starts to turn dusky blue, her sats drop to the 30's or so, BEEEP BEEEP BEEEP, and then she takes another big deep breath. So far, she has always managed to remember to take that next breath. This chain of events happens dozens of times a night, interspersed with frequent suctioning. Last night I suctioned Julia 13 times between 1 AM and 9 AM. She sleeps through the desaturations, apnea monitor, and suctioning. Needless to say, I don't, and I finally have a whole bunch of gray hairs to prove it! :-/
On the up side, Julia does remember to breath when there is a lot going on during the day. Yesterday we visited another transplant patient's room. Collin is three years old and his brother is one. Julia's eyes lit up when she heard the other kids' voices! Usually we must avoid other children, except transplant kids in the same circumstances, because of germ potential. Julia loved the stimulation and kept opening her eyes to see what was going on. I can't wait to get her home around her brother and sister, who will surely bring out the best in her.
Speaking of going home, we will have been in Durham six months to the day on January 30th. The good news is... if all goes according to plan... we just might be able to go home on the 30th or 31st! I am so excited that it's hard to keep a lid on the rumor! The lucky folks in Michigan are having the best winter ever, lots of snow and ice, my favorite kind of weather. I dream of cross country skiing and ice skating across the lake. Yet, here we sit, in shirt sleeve weather! I realize that when I get home my duties with Julia will not allow me to do much playing outside, but I really look forward to looking out the window at the winter wonderland. Rob and the kids have mobilized the cleaning efforts into high gear in anticipation of our arrival in a couple of weeks-- washing the draperies, cleaning the carpet, boxing the unused toys to make room for all of our new paraphernalia. It will be a grand homecoming. We all understand that it will be a very big adjustment for everyone. The kids will not be able to bring any friends over for a very long time, and they will have to wash their hands much more frequently than any kid their age would ever want to do. Everyone will have to learn to sleep with all the new sounds that will resonate throughout the house all night long: apnea monitor beeps, and suction machine rumbling.
Nonetheless, I truly look forward to leaving our hotel room, and Durham, although I have seen very little of this city beyond the hospital. But, taking a breathing Julia home is a wonderful gift, even if the breathing is a 'little' irregular. At home we will meet new challenges and learn a new way of life. We appreciate your prayers and well wishes as much as ever. Julia's remyelination must be rapid if she is to gain much function. She currently is mostly limp and non-responsive much of the time, except for the occasional open eyes and snake tongue "I love you". We see some improvement in her ability to eject some of the secretions from her lungs, but she needs to swallow on her own if we are ever to be rid of the trach and g-tube. She currently swallows a couple of times per week at best. She will need to regain a lot of basic function beyond what she currently has to be able to lead a rich and full life. Please continue to send your prayers and well wishes that she is able to remyelinate quickly.
Sadly, I must report that another Krabbe's transplant child has passed away. Brenden lived not far from us in Michigan, but had his transplant at Johns Hopkins in Baltimore. Please keep him and his family in your prayers.
~Julia and her mom
Newcomers to Julia's Journey may read old updates on Julia's website:
Julia's Journey #62 1/24/99 T+157
After another week of trying to keep up with Julia's antics, we are on track to go home a week from today. Julia had been doing some new tricks a week or two ago: raising both arms and legs at the same time as she coughed secretions, squinting her eyes, pursing her lips. She always would do what we call "hand huggies", holding your finger tightly and refusing to let go. Her eyes were opening more and more every day, sometimes for an hour or two at a time. She would slowly follow my voice around the room. She particularly seemed to enjoy the sounds of animals that would come on TV, and always opened her eyes in that direction. Her greatest joy was rolling around in her new stroller, when she would open her eyes and look all around.
All of these abilities seemed to slow down, and then stop, while the apnea increased to greater and greater levels. By Wednesday we were virtually filling the apnea monitor memory in a few hours at night. We discussed every possibility with the doctors, and could come up with no firm conclusions. The big fear is always that the disease overtakes the cure, despite the increases in abilities we have been seeing. We can't predict where in Julia's brain the enzyme will work first, or how rapidly. By Thursday Julia appeared completely limp and non-reactive to any stimuli. It was getting more and more difficult to keep a positive attitude about the situation.
Julia has frequently spiked high temperatures, always in the morning, over the last few months. Exactly why this happens, most often at the same time of the day, has always been something of a mystery. When it happened on Thursday morning I didn't perceive it as unusual. But, when it hadn't come back to normal by noon I decided we should have it checked at clinic. By the time we waited a few hours in the treatment room the fever had gone back to normal (around 93 F for Julia), but she remained non-responsive. The same thing happened on Friday. Cultures were taken of Julia's port, and another of Julia's all time favorite doctors, Dr. Shannon, decided to take a urine culture via catheter, since all of the blood cultures had always come back negative.
The urine came out very cloudy, and the sample showed a substantial amount of bacteria. This is one case were an infection indicates relief! Finally, we found something we could treat. Julia was given an immediate dose of IV antibiotics. Literally, as the dose was finishing, the nurse and I watched Julia open her eyes for the first time in two days, and grasp my hand! There is still a huge question about what really occurred, because the antibiotic couldn't possible have cleared her urinary tract in a half hour, yet we saw something happen. If she were septic (infection in the blood) the reaction could have been immediate, but still all the cultures of her blood remain negative.
She has continued to get the antibiotic for 3 days in a row. Julia is regaining some of the personality we had been starting to see emerge, although she is still has a distance to go before she reaches her high point of a couple of weeks ago. Last night Joseph's mom, Michele (author of Joseph's page at http://home1.gte.net/robmil/index.htm) visited with Julia, who is making Michele wonder what all the recent fuss has been about! On Wednesday I had suctioned Julia 67 times. Today she has only needed suctioning a mere 16 times! The apnea monitor only advanced a scant 7% overnight! It looks like this infection made Julia really feel miserable, and she is very happy to be feeling better.
I am very happy to report that she seems to be doing better, and we will go forward with our plans to return home next Sunday. This is a very major undertaking! We have a six month accumulation of equipment, toys, clothes, and STUFF here in our very cramped hotel room. I will be packing it all and sending it north this week. It took some time to convince one of the airlines that they really did want to fly us home, complete with oxygen, pulse oximeter, ambu bag and other emergency supplies, suction machine, and Panda stroller. The home health care companies will need to meet us at the airport on both ends of the trip.
We are very anxious to get home and have our whole family together again. It will be a big adjustment for Julia, who always indicates her displeasure at missing the friends she's made at Duke. After all, she has spent half of her life here! It will also be a big adjustment for the folks at home, who will be getting to know a very different Julia than the one who left, not to mention a mom who now has gray hairs! Nonetheless, we are up to the challenge. Saturday will mark the six month anniversary of the day we arrived in Durham, and six months is long enough.
So, please keep us in your thoughts and continue to send your well wishes and prayers that we have a non-eventful journey home, where Julia's journey can continue into new and uncharted territory.
~Julia and her mom
Julia's Journey #63 1/27/99 T+160
Aaaargh! Julia's dad says that we should start calling Julia "Dixie" since she seems to like it here in the south so much. She's back in the hospital again.
By the end of the weekend it looked like once we fought off the urinary tract infection that Julia's apnea was getting much better, her alert status was significantly improving, and the copious secretions were considerably reduced. We were right on all counts, but didn't anticipate the next situation. Although none of Julia's respiratory cultures have ever turned positive, she obviously had some sort of small cold. All of us have experienced the frequent runny nasal secretions that you get at the beginning of a cold, which then eventually become thick and dry toward the end of the cold. The same thing happens to Julia, only when her secretions thick and dry they occlude her airway if we can't get out the mucous plugs. Imagine trying to blow your nose through a coffee stirrer straw, with no other way to get rid of the mucous, and you will see how hard it can be to keep Julia's air passage clear. The suction catheters are about the same diameter as the stirrer. It sounds funny or gross, depending on one's age, but the consequences for Julia can be deadly if we aren't successful.
On Monday morning I heard Julia trying to pull a breath, and when I looked over she was purple with wide open eyes, fists clenched, arms and legs in the air. The pulse ox went off but I didn't have time to watch it and see where her saturations were going. I tried to suction, but the mucous was so thick and sticky that I couldn't draw it through the catheter. I had to do a quick emergency trach change, which pulled the plug out and allowed Julia to keep breathing. We both made a huge sigh of relief, got dressed, and trotted over to clinic.
Julia receives moisture through a humidification system that pushes vapor through a hose to a collar that she wears around her neck. All day on Monday we had a little cloud of vapor saturating Julia's trach to hopefully moisturize that mucous and make it flow. It seemed to be working in that Julia's sats remained high all day and evening. At about midnight she started making a little clicking noise with each breath, just as I was getting her ready for bed. A few minutes later another plug occluded the trach just as one had done earlier that day. This time we went over to the transplant floor to get admitted and see what we could do to prevent this from happening again.
Julia began receiving breathing treatments to loosen the gook (a highly technical phrase I picked up from some of the therapists ;-) yesterday, but still required another trach change last night. The ambu bagging that she received reset her central nervous system oxygen/CO2 sensor, causing her to "forget" to breathe for a few hours, because she thought she had enough oxygen on board. The good news is that today she is breathing on her own, with minimal apnea, and her secretions are a little more loose, although she is requiring oxygen to maintain saturations in the 90's.
We have had to revise our plans to return home this weekend. I had planned to pack and ship everything this week, but since I've spent most of my time in clinic and the hospital it has been almost impossible to accomplish this task. I was extraordinarily grateful to receive a call from Lawrence Transportation Systems, an employee owned moving company centered in North Carolina, South Carolina, and Virginia, who in a wonderful act of kindness offered to transport our belongings home for free. The generosity of these good people is beyond commendable.
Initially we had planned to drive home, as a way to transport both ourselves and all of our six months of accumulation. Dr. Kurtzberg was a strong advocate of cutting Julia's transport time down to the minimum, so we had made arrangements to fly on a commercial jet, pulse ox, extra trachs, ambu bag, suction machine, and oxygen in tow. Now we now may need to change those plans to a med flight, since emergency trach changes on commercial airliners probably would cause a rather strong reaction from the other passengers. There is a significant concern that the low humidity en route would make those dry and sticky secretions even worse. The question is: When will we get to go? Only Julia knows for sure, and, as usual, she's not talking. We are all hoping for next week, giving us time to settle in before the Julia's Friends & Hunter's Hope Vigil that our many, many good friends are holding to fight this disease on February 14th. Please look at the invitation below for more details.
We feel the power of your thoughts, prayers, and well wishes every day. Julia needs to breathe, swallow, and cough. Julia's will to live is very, very strong, and especially evident during crisis moments such as emergency trach changes. Somehow she tells us to look her way when it's critical that we must, and somehow I know that she will learn to breathe, swallow, and cough on her own to survive.
~Julia and her mom
Julia's Friends invites you to join us during our ~~Candlelight Vigil for Julia Beatty~~
on Sunday, February 14, 1999 4-5 p.m. Spiritus Sanctus Academy 2600 Via Sacra, Ann Arbor (at Domino's Farms)
Donations of $5 for a powder-scented candle will go to Hunter's Hope for research. Candles will be available for purchase starting at 3:45 p.m.
This Vigil is being held in conjunction with Hunter's Hope to honor all the victims of Krabbe's Disease -- a vicious, genetic disorder which destroys an infant's nervous system and usually leads to death before the age of 2. Currently, the only treatment that will allow these kids life is bone marrow or umbilical cord blood transplant.
Please join us to celebrate Julia's life, to pray for her healing, and to raise funds for research so that future children and families will no longer suffer from this and other demyelinating diseases.
If you cannot attend but have questions, or would like to purchase a candle or make a donation, please contact JuliasFriends@hotmail.com
Julia's Journey #64 2/2/99 T+166
A little more than six months ago I arrived in Durham with a baby, a stroller, and an overnight bag. Tomorrow I will leave with a bigger baby, a much bigger stroller, and a suitcase filled with medical supplies. I am ecstatic!
Julia got out of the hospital again last Friday. Her secretions had finally loosened to a somewhat manageable stage, only plugging to the point of causing a big adrenaline rush three times in the first 24 hours we were outpatients. Fortunately none of those plugs required emergency trach changes or massive ambu bagging. Throughout the weekend the secretions have become thinner and more watery, which allows me to suction them without undue fear of occlusion. Unfortunately, however, Julia's apnea has again reached a severe state. For the last couple of nights she repeatedly desated into the teens and twenties on 5 minute cycles, causing great concern in this very sleep deprived mom.
The good news is that Julia seems to be remembering to swallow! She began to swallow when she was coming out of low sats on Saturday. She ended up swallowing several dozen times that day, repeating the feat on Sunday and today. She also spent each of these days with long periods of open eyes, following her favorite voices around the room. Her new game is to raise both hands at the same time with a big frown after coughing. We keep telling her to "Breathe, swallow, cough. Breathe, swallow, cough." Maybe she's listening!
Well, the time has come to unplug the computer. Our room is packed and ready to be moved home to Michigan, and the computer is, as usual, the last thing to go. This is the mark of us being serious about leaving! Julia's Journey will continue in Michigan, updating you whenever Julia does something new and interesting. Please note that our email address will change to email@example.com as of the date we arrive at home, but please do not send any email to that address until you receive our next update. We are happy to be on our way, although not without a little trepidation at saying goodbye to many good friends, and leaving behind the security of having the incredible medical team that has worked so hard at saving Julia's life right across the street. Please keep your prayers and well wishes coming for Julia to "breathe, swallow and cough" happily all the way home.
~Julia and her mom
Julia's Journey #65 2/18/99 T+182
Hello from Ann Arbor! Yes, we really did arrive, via Medflight, two weeks ago. The flight itself went well, and we were met at the entrance to the University of Michigan hospital by Julia's Friends carrying a "Welcome Home" sign on a big batch of balloons. We were so happy to get here that I was tempted to kiss the ground.
Julia settled in almost immediately and began showing all the new doctors and nurses what she could do: a new urinary tract infection and multiple desats in the twenties and thirties. It wasn't too long before we were trying out some new flavors of antibiotics (the current variety gives her a very distinctive aroma) and discussing any number of ways to convince her to breathe.
Our new pulmonologist, Dr. Brown, brought up an interesting point. Julia forgets to breathe on a very regular basis especially while asleep. Every time she had been forgetting for much more than 40 seconds I had been caressing her cheeks or moving her around to stimulate her, essentially waking her up. Sometimes I would give her a whiff or two from the ambu bag as a reminder. Eventually she would always remember to start breathing, but I was becoming exhausted with reminding her that "breathing is fun-damental" twenty four hours per day. Dr. Brown queried that if I was so tired keeping Julia awake all the time as I reminded her to breathe, how tired was she from never being allowed to sleep? What would happen if she were well rested?
Apnea patients are sometimes put on a form of a ventilator called a "bi-pap" which sends a whiff of air down their windpipe at regular intervals (less than 40 seconds apart ;-) that reminds them to breathe, essentially what I had been doing manually or with the ambu bag. We decided to give it a try with Julia.
The first night was a success! She only desated half a dozen times, and then only into the 80's. Hurray! We tried it again the second night, and then the third. Unfortunately, it seemed as though she was acclimating herself to the machine, and finding a way to become more apnic as time went on. The desats were becoming more frequent and lower with each night.
The good news was that during the day Julia appeared more alert, eyes open more frequently, and arms raising happily to play kissing hands. It seemed as though the experiment was a mixed success. A little sleep was going a long way. What could we do better?
We decided to try a different type of machine that would still aerate Julia, but based on volume of air rather than pressure. This is the approach we are currently using, and so far it seems to be working quite well as we continue to tweak all the settings a little up on one dial and down on the next. I receive my formal training tomorrow.
The great news is that Julia seems to love being able to sleep while her sats remain in the high 90's. She is becoming more reactive than I have seen her in months. She has taken a strong liking to her new physical therapist, and is doing well on holding her head up by herself for several seconds at a time. She is back to playing kissing hands for hours, and now swings her arm up and then out, sometimes above her shoulder. She is making new facial expressions and really likes the orange and pineapple flavors the occupational therapist has let her sample. She continues to swallow, although not as much as she was before being ventilated. Her favorite time to swallow then was just before breathing during a low sat. Now she isn't having low sats, so the swallowing is more random.
Julia is more reactive to her environment than she has been for a long time. She regularly cries whenever she hears the door close, unless I'm hugging her. And, now she cries audibly! Two days ago she figured out how to force the air from around her trach up past her vocal chords, and now does this frequently, making a little cry sound. Under normal circumstances I would be unhappy to hear my child cry. In this case I'm ecstatic! My next goal is to teach her to do it during the daytime. She prefers to do all of these tricks from about 10 PM until 2 AM. We'll have to work on that. She also is opening her eyes more frequently, but she doesn't like bright lights, which may explain why she is more active at night.
In two days it will be Julia's six month anniversary of her transplant. The theory behind the transplant leads us to believe that the formerly missing enzyme should be hitting its mark somewhere between six and twelve months post transplant. We are buying Julia time by letting the vent breathe for her when she forgets. Although Julia has definitely been doing more the last few days, it is hard to say whether this new activity level is from remyelination or a reaction to having more oxygen in her brain. I prefer to be an optimist and think that it's a little of both. Please keep your prayers and well wishes coming that the pinhead full of enzyme circulating in her blood finds its home closer to the beginning of the six months post-transplant than to the twelve month anniversary. Julia's updates will continue more frequently now that I have a computer here in our new hospital room. (Thank you Heather!) We are very grateful to all of you for keeping us in your thoughts during the last two weeks while we were unable to communicate via email, and especially to all of our good friends at Duke who, I am told, have been going through withdrawal at not having any Juli-antics to tend to up on the floor or in clinic. Please make note of the new email address, firstname.lastname@example.org , and look forward to the next update which will include a report of the Valentine's Day Julia's Friends' Vigil and Julia's recent television debut.
~Julia and her mom
Julia's Journey #66 3/4/99 T+196
Today marks one month since our Medflight to Michigan. What an adventure to be back in our home state! It's amazing to me how much has changed here in the six months we were gone. On my first driving expedition I went through our local downtown, and in the dark of night nearly ran over all the new curbs that had been placed in such a way that they now stick out into the street. No more dodging the cars who are making left turns in downtown Dexter! There are new grocery stores in places where for as long as anyone can remember there have only been fields. The old restaurant that was across the street from the pharmacy for the last 20 years has been turned into a health food store. All the kids in Alison's class are taller with bigger front teeth. Cameron looks like a different kid now that he has lost his front teeth, which were firmly in place when Julia and I left last summer.
We are still in the University of Michigan hospital, however, having resigned ourselves to a much longer stay than originally anticipated. Julia currently has two small issues that require hospital attention. The port that was installed under her skin to facilitate blood draws and IV infusions has become clogged, and must be replaced surgically. Also, Julia has had some stool blood ever since having an upper GI about two weeks ago to ascertain stomach wrap function. Both of these issues will hopefully be resolved shortly, leaving us with one big problem. There are no home health care nurses available to care for Julia during the hours when I am asleep. Right now there is a nursing shortage in this area, and despite have run ads, there are no nursing agencies with enough nurses to give us enough coverage to allow us to go home. What frustrating circumstance.
None of this matters much in light of the big picture, however. Julia is doing very well. The theory behind the transplant says that remyelination should hopefully start to become obvious between six and twelve months post transplant. February 20th, our six month anniversary of transplant, came, and went, and ...
Within the last couple of weeks Julia has become more alert and active than she has been at any time since before we went to Durham last July. Every day we see improvements in the variety and magnitude of Julia's responses to stimuli and amount of independent motions. Whereas we formerly had the medical situation de l'heure, now we have the du jour developmental step forward. All of these small strides are very obvious when comparing the notes that the physical, occupational, and speech therapists made when we first arrived here to now. Here are some of the accomplishments Julia has demonstrated today:
Head control: We now have a new game called "sit-ups". While Julia lays down (30 degree incline) I grasp her hands and pull her arms up slowly. I can now raise her slowly to a sitting position, while she holds her head upright! She can maintain neck control for up to several minutes, even while I rock her slightly from side to side, still holding only her hands.
Facial expressions: Julia has been making a cry face off and on for a couple of months, but she has now become very proficient at it. She can even vocalize her cry through the trach, and does frequently. It is fun to watch her making more frequent smile-grimaces, yawns, frowns, and knit eyebrows. We haven't seen a big smile yet, but we exercise the muscles needed for that action by massaging the sides of Julia's mouth one at a time, which makes her mouth twitch similar to Samantha on the old TV show, "Bewitched".
Tongue motions: Julia has been trying tastes of orange, cherry, pineapple, strawberry. Today she had apple juice. When Dale, her speech therapist, put the juice on Julia's tongue she slowly licked her lips four or five times and then swallowed twice. Julia sticks her tongue out when coughing, rolls her tongue, and smacks her lips loudly enough to startle herself several times per day. We have been practicing with a pacifier recently, to get Julia accustomed to the feel of having her tongue in a normal position. Last night Julia "sucked" on her pacifier for about a half hour, pushing it out with her tongue and pulling it in with her bottom lip, without letting it fall out. She sometimes stops making a cry face when the pacifier is used to massage the inside of her mouth.
Hands and arms: One side benefit of massaging Julia's cheeks is that she almost always raises her kissing hands to this stimulus. In fact, Julia will raise her hands to almost anything these days! She waves her arms out to the sides, then bends them at the elbows, raises them high, and lets them drop, sometimes in tandem, sometimes one at a time. She flexes her fingers back and forth at the second knuckle. The most exciting motion she has made is to extend her thumb and forefinger like a gun, the exact opposite of the Krabbe's fist.
Legs and feet: Julia rarely moved her legs and feet in the past. Now she continues to bend her knees and draw her legs almost to her tummy when she first feels the water being poured over her in bath. Her feet exhibit much more independent motion in reaction to stimuli, although they still hyperextend and she still has frequent toe clonus (rapid back and forth movements due to lack of control).
Eyes: Ahhhh! Those beautiful blue eyes! We now get to see those cerulean orbs more often than we have for months. Today Julia had her eyes open for a total of three or four hours. She can now keep them open when looking straight ahead for several seconds, although they still eventually wander to the upper right or left. Her nystagmus is slower than before, and sometimes she will actually fix on a friendly face or colorful object such as her Rusty raccoon. She has demonstrated to some of our doctors and therapists how she slowly follows my voice around the room. She even gradually jerks her head from one side to the other to be able to follow my voice.
WOW! It's exciting to watch this progress. Today, Julia had her pacifier in her mouth, arms extended with a rattle in each hand, her eyes wide open, and her newly grown spiky hairdo. I looked at her and startled, "She looks just like a normal baby!" What a treat for me to see her this way after all this time, even for a fleeting moment. We still have a long way to go, but it's great inspiration to be able to perceive some advancement. It is also great to be so close to home that we were able to attend the candlelight vigil on February 14th held by Julia's Friends in conjunction with Hunter's Hope. I was overwhelmed by the support of so many friends and acquaintances who gave their time to arrange and attend this event. WDIV TV in Detroit came to our hospital room the day before, putting together a three minute segment on Julia, Krabbe's, and cord blood transplants for their evening news. We also had a radio interview on WDEO in Ann Arbor and its sister station in Detroit, and several newspaper articles in the Ann Arbor News, Credo, The Dexter Leader, and the Chelsea Standard. Julia is doing her part to publicize the need for research on this and similar diseases and the possibility of remyelination.
Hundreds of people have worked very hard to bring Julia to where she is today. One of the nurses here and I were trying to figure out exactly how many doctors and nurses, not to mention support people, Julia and I have met since our first appointment last May, as I struggle to keep all the names and faces straight at this hospital. (I keep mistaking people here for people at Duke. Whoops, that's not Dr. So-and-so or Nurse Such-and-such! Wrong hospital!) I remain very grateful to everyone who has helped us in so many ways. Julia is now starting to show us with all of her new abilities the cumulative effect of each person's hard work, prayers and well wishes.
~Julia and her mom
Please note that our new email address is email@example.com. We love to hear from you!
Also note that archived Julia's Journeys may be viewed at http://www.krabbes.com/juliasjourney/
Julia's Journey #67 3/27/99 T+219
It's hard to believe that March is almost over. Yes, we are still in the hospital, but there is light visible at the end of the tunnel. Our discharge date has been set, for next Wednesday! Julia is surprising us with new abilities on a daily basis.
Julia's neck strength has increased so that she now lifts her head forward up off the bed, as though she is trying to sit up. She can hold this position for many seconds, the only problem being that her many chins eventually occlude her trach. So long as one is careful to pick Julia up off the bed slowly, giving her plenty of warning about what is to come, it is no longer necessary to support her head as she is lifted. The best part is that when Julia is sitting on my lap she can hold her head up without help for a half hour or more! She will look around the room, moving her head back and forth with a slow jerky motion. This makes it difficult for her to keep her artificial nose in place, because as she turns her head the nose rotates to ninety degrees, a position through which it's difficult to breathe. This type of breathing problem is far better than some we've had in the past.
We've been having a lot of fun with taste treats, too. Julia's reigning favorite is apricot nectar. We put a little on a Q-tip and dab it on her tongue. Clamp. Her jaws munch and munch, she licks her chops, and swallow swallow. Eyes open, her head turns toward the source and her mouth opens for more. Her little feet bend up and down at the ankles, and her kissing hand goes way up in the air. MORE, please! Our speech therapist asked our permission to video Julia enjoying her lunch this way, definitely one for the record books.
Julia is more alert and awake most of the day than she has ever been at any time since before transplant. Interestingly, she is no longer on any caffeine or its relative theophylline. This was discontinued a couple of weeks ago when her liver function blood tests showed her AST, which had always been in the 20's, to be very high, in the 900's. Julia was full of surprises that week. Besides the high liver functions, her heart rate was in the 200's, temperature in the normal human range (which for Julia indicates a fever), she was having higher blood pressure, 8 to 12 stools per day, and not doing much in the way of fun and games.
What was going on? The attending physician for infectious diseases appeared in our room and announced that he was the Bug Detective and was on line to track down whatever bugs were in there doing this to Julia. Best of all, he has a full arsenal of weapons to beat back those enemy bugs and put them in their place. He declared open war on the little monsters, and I just knew that if anyone could find and annihilate them he could. If I had had a doctor like this when I was a kid I would have LOVED going to the doctor's office. It is nice to see medicine becoming so patient friendly.
All of this happened just after the port clotted, and just before the scheduled surgery to replace it. Since the port was no longer functional, Julia had to undergo a separate poke for each blood draw, and have an IV to do medication infusions. We had to pull the old port out in one trip to the operating room, and wait for Julia to get better after several days of antibiotics before we could introduce the second port, so as to avoid additional risk of infection. She did incredibly well with each surgery, spontaneously breathing while still in recovery, her best post-ops ever. We now have Streptokinase, a drug that will break up line clots, right at our bedside, in the hope of avoiding this problem again.
Julia is becoming very reactive to her environment. She will roll her head and upper trunk from one side to the other to follow whatever action is in the room. She startles very easily to any noise that sounds like a fork hitting a plate, although this reaction is significantly less than it was a few weeks ago. Back then we went through a three day period when Julia startled roughly every 10 seconds, every moment she was awake, hour after hour. The EEG determined that she wasn't having seizures, but didn't tell us exactly what she was having. One interesting theory came from sister Alison, who hypothesized that Julia had a bad case of hiccups. Fortunately we are now usually able to ascertain the source of most startles, which can be reduced in frequency by having the music we play at the right loudness to block out as many of the noises that she doesn't like as possible.
Julia is back to watching TV for an hour or two a day, although the weather channel is not available on the cable system in this hospital. We will be watching the Michigan State - Duke basketball venture. As University of Michigan alumni, our sentiments will necessarily fall with Duke, since no self-respecting Wolverine could support the small school to the northwest of Ann Arbor.
It is most fun to pick Julia up, hug, hold and caress her, especially now that she has even more hair EVERYwhere than she has ever had. Not only is she still taking Cyclosporine, hair growth drug extraordinaire, but now she is also on Minoxidil, to help control her blood pressure. This little Juli-bear's favorite way to be calmed when crying is to have the hair on her forehead stroked opposite the way it grows. We've had to cover Julia's ears from jokes about braiding her hair, on her arms, that is. Big sister Alison has developed a new exercise formulated to eliminate stress through laughter, inspired by the thought that Julia's copious hair growth is part of her healing process. Alison spikes her hair into an electric shock sort of format, and holds up a sign saying "HAIR THERAPY" whenever a nurse enters the room. The gales of laughter that follow are definitely therapeutic. The hospital's art therapist was especially impressed.
This is an exciting time. Every day we make an advancement forward. Today Julia's great grandmother visited her, and it was wonderful to watch as they talked and held hands. Julia has a most endearing new ability. She does real kisses when you put your cheek on her lips. She Pull her bottom lip in and makes a "smack" sound. Great grandma melted when Julia showed off her new talent. This was especially effective yesterday when we met the home care nurses who will care for Julia while I sleep. Smack, smack. Who could resist those big blue eyes, long eyelashes, furry spike doo, and little loving kisses? They were hooked!
And so, we're looking forward to Wednesday-'n-OUT. The home nursing agency was able to fill most of the month of April with nurses willing to care for our Pubbles. This time I'm the one turning blue in our family by holding my breath, hoping that Julia stays well enough to actually get discharged, so that we can finally go HOME. Wednesday will be the 8th month anniversary of our first full day in North Carolina. Our journey has been very long, but I am ever conscious of how lucky we are to make it. Our next door neighbor on the transplant unit at Duke, Sara, passed away after a relapse of her disease post transplant. Sara was a beautiful girl. Her parents were very helpful to me when I was a newcomer to Duke. Sara's bright smile is very missed.
Sadly, also, Julia's good friend David, whose parents, Kelly and Zig, were so helpful to us during our stay in Durham, died a week ago. His very loving parents were at his funeral while we were here at our discharge planning meeting. David was loved and cherished during his short life. The way that his Krabbe's progressed meant that he did not have the second chance for life that Julia has been given, and his parents weren't allowed the pleasure of watching him gain function post transplant as we are now. Your well wishes and prayers on Julia's behalf have helped us considerably and are greatly appreciated. Please also add a prayer for David and Sara and their families, who miss their children tremendously.
~Julia and her mom
Julia's Journey #68 4/8/99 T+231
We had the very best April Fool's Day ever. It was Julia's first day AT HOME!!! That's right, at home, in our house, in her crib, not in a hospital or hotel room, for the first time since last July. Julia looked around her new abode in open-eyed wonder and amazement. We all did! Home looks a little different now, with distinct similarities to a hospital room, but definitely much more pleasing.
Julia kept us guessing whether or not we would really get discharged, an old game of hers, right up to the last moment. She was supposed to get a dose of IVIG the day before, but there was a snafu in the orders. We noticed when we awoke on Wednesday, the big day itself, that the IVIG hadn't actually been given. Since this is an IV infusion drug it must be given while still in the hospital, unless we involve yet another set of home health care professionals, equipment, and lessons to administer the dose at home. Since we already have a large crew of home care nurses, home care equipment providers, and mounds of necessary supplies, it seemed more efficient to take the time to give the med while in the hospital. Our task force of parents, relatives, friends, nurses, and home health care providers were all ready for the big exit at high noon, when we made the decision to wait for the IVIG to be given. After all, what's another hour or two in the hospital after 8 months?
Julia, of course, had other plans. A patient receiving IVIG must have their vital statistics monitored closely during administration to be sure that she is tolerating the dose well. Julia has had IVIG many times in the past with negligible side effects. This time, though, her blood pressure started to rise, and rise, and rise. First she received Monoxidil. Then a shot of Hyperstat. Then some more Hyperstat. We all sweated it out waiting until it appeared safe to leave. Finally at 9PM we were given the okay by the doctors to head home. The only person disappointed was Cameron, who had worn his thumbprints into the Nintendo player just the way he likes them.
The medical equipment provider helped us set Julia up in the car for her first ride home. Traveling with a baby on a vent, with a battery, and a pulse-ox, not forgetting the suction machine, can be a real experience the first time you try it. Our caravan took off for home and the several hour old pizza dinner waiting for us. A half mile down the road I pulled over suddenly, throwing the several cars of helpers who were following us into a panic. Was there an emergent situation? No. I realized that in the half hour it had taken to get Julia into the car, none of us had bothered to put her seatbelt on her!
Once we made it to our door the transition went rather smoothly. Our living room is set up with Julia's crib, vent/humidifier/pulse-ox/suction equipment, shelves of supplies, and plenty of paperwork. An hour or two later the set-up crew left and we were officially home. What a wonderful feeling to have our whole family under the same roof again after so long!
We began adjusting to our new life, figuring out what was normal, what worked, what was best placed where. When I discovered that I had accidentally unplugged our freezer chest full of food, which was completely lost, I knew I had rediscovered normal. Nope, normal isn't always fun, but situations like the disposal of a few hundred pounds of rotting comestibles (and associated guilt at having caused the problem are a relief from several months of wondering if your child will survive.
Julia did well the first day, and the second. The other kids were on Easter vacation, and anticipation was in the air. Since we had missed spending Christmas together, as well as all of our birthdays, we had a large pile of unopened presents waiting for a celebration. Easter Sunday arrived, and the kids were up very early, calling the day "East-mas" and "Chris-ter". The Easter Bunny hid the baskets deep within the presents. Grandma and Aunt Lynn arrived. Alison and Cameron had a great day, and Julia had one of her best days ever.
On Easter Sunday, Julia sat on my lap, supporting her head BY HERSELF, for almost 45 minutes as the other kids opened their presents. Her eyes were open much of the day. She turned to look at the action, wherever it was, especially following the kids' voices. If I placed her in her crib with her body oriented to the left, and the action were to her right, she would tense, lift her head off the wedge, and orient herself to the right. While this is a long way from rolling over, it is a major step in the right direction. Best of all, Julia did very well sprinting off her vent. She even tried some pear mush from a jar on a cotton tipped applicator for dinner, and licked her lips.
On Monday Julia seemed tired after all the excitement. I was unable to take her off the vent without desaturations. By late afternoon I had her on a half liter of oxygen. Her blood pressure had been on the high end over the weekend, and that had normalized somewhat, but Julia just wasn't acting as she had been the prior few days. Even though she appeared better by the next day, her blood pressure was again on the rise. When we received her lab results Tuesday night we learned that her sodium, potassium, and chloride were dangerously low, and her Cyclosporine was dangerously high. We drove back to the hospital, and were admitted first to the Emergency Room, and then placed in PICU.
Hence, this update is being sent to you from the Pediatric Intensive Care Unit at the University of Michigan Medical Center. Julia obviously had not finished touring the hospital as of our departure last Wednesday. Julia's chemistries were very askew, calling into question her kidneys' ability to function. Further testing showed very low thyroid. Julia's blood pressure has been reaching some record highs, 170/117 on Wednesday morning, 149/105 earlier today. This trip to the hospital we were introduced to two more medical disciplines in addition to the multitude were already knew: nephrology and endocrinology. That's enough!
Fortunately, Julia's ECG today showed that so far there has not been any danger to her heart from these tremendously high pressures, and she is responding to the Isradipine being used to treat it. Her most recent labs are much closer to normal than they have been, and Julia herself seems fairly comfortable. Although she is primarily sleeping, she is able to be roused, especially when being poked for blood draws. (Yup, that darned new port will flush but will not draw.) Julia has been moving her legs today more than she has since before the transplant. She is reacting to massage by wiggling her toes purposefully, bending and flexing her ankles, bending her knees, pulling her legs up near her tummy, and moving faster than I have seen since early last year! The nurses can't believe that two months ago she "rarely move(d) her legs" as it was phrased on our discharge summary from Duke. Hooray for Julia!
Our great hope is to get out of the hospital soon. I am unable to stay with Julia 24 hours/day while she is in PICU, and I know that she was happier at home. As always, your good thoughts, well wishes, and prayers are very much appreciated. Julia is making great gains in her abilities, but we are once again back to an old issue. Diapers! Fluid balances, ins and outs, electrolytes, and so on. We were home for six days. We can be patient, and know that we will have the chance to improve that record soon.
~Julia and her mom
Julia's Journey #69 4/21/99 T+244
Julia maintained her short PICU stay record, for the fourth time, when we were released from PICU on Saturday, April 10th, after 4 1/2 days. Her condition had become stable, her chemistries normalized, and her immune system determined by the doctors to be strong enough that she could go to the Stable Vent Room. This is a four-patient room set up for vent-dependent kids where there is a nurse 24 hours per day. In the past Julia had to be in a private room due to immuno-suppression issues. We were surprised that she was ready to be in the same room with other kids. This is progress! Julia had more stimulation in the SVR than she has had probably since she was in PICU at Duke, which, until their new children's hospital is completed next year, is still a large room with quite a few patients. (The PICU at UM has rooms that are enclosed.) When Julia was in Duke PICU she went into hyper-startle-drive every time she heard a clipboard or notebook making that most hated noise when open and shut. She became excited by many of the sounds in the Stable Vent Room too. We do not know what Julia can see with her big blue eyes, other than the difference between light and dark, but she definitely can hear well, ~startle~ ~startle~.
We stayed in the Stable Vent Room for six days. We were in the far corner of the room, and really enjoyed getting to know the other families. A couple of the other kids were in the same situation we were last month, waiting for available home nursing care so that they could go home. A couple of kids had conditions that science hasn't yet been able to find a name for, perhaps leukodystrophies, but a variation where the specific missing enzyme or other factor hasn't yet been identified. There is little that is lucky about having Krabbe's, but it is worth remembering that the missing enzyme for this leukodystrophy is
one that has been identified within the last several years. I have met a number of people who told me of loosing their children within the last 2 or 3 decades to unknown neurological disorders. If we had not been able to positively know which disease Julia had, then we could not have sought the transplant treatment, because not all neurological disorders would benefit from transplant.
Last Wednesday Julia was acting a lot more like her Easter Sunday self:
lots of kisses, open eyes, large motor motions. Her sodium, chloride, potassium, and Cyclosporine had normalized. We prepared for discharge on Thursday. But, ho ho! Julia has NEVER exited the hospital on the presupposed schedule. On Thursday she was quiet and didn't want to open her eyes. Although her blood pressure was under control, her heart rate was moderately high, in the 150's. A number of times in the past this has indicated an impending problem. When her attending Bone Marrow Transplant doctor, Dr. Hutchinson, came to see her she promptly desatted to the seventies, despite being on a quarter liter of oxygen. So, in true Julia fashion, we remained in the hospital for another day.
Friday rolled around and even though Julia still wasn't a ball of fire we decided we would go home on oxygen and watch her closely from there. We finally arrived home at 9PM, keeping another record intact, that of never getting home from the hospital before dark. Fortunately we had a nurse scheduled for that night, and the transition went smoothly. We are once again adjusting to being at home. I still invariably dial "9" to access an outside line every time I pick up the phone to call someone. And, I must say that I really miss the nifty coffee machine on the floor of 6 Mott, where you can program whether you want fresh brewed coffee, hot chocolate, plain water for tea, and even whether or not you want 100%, 50%, or 0% caffeine. Whoopie! We long term hospital med-moms really enjoy the small things in life.
Yesterday was our first clinic day since we have been back to Michigan. Quite an experience! Here are Julia's basic necessities for transport to clinic.
4 lbs enteral feeding pump (which Julia has been using again since her diarrhea problems in March)
4 lbs suction machine and go bag
8 lbs diaper bag/notebook/spare food
6 lbs pulse oximeter
20 lbs "panda" seat
20 lbs stroller base
8 lbs oxygen tank
10 lbs vent and battery trays
35 lbs ventilator and attachments
24 lbs ventilator battery
22 lbs one very cute baby = 161 lbs to carry to and from the car every time we go anywhere, all while ambu-bagging Julia every time she forgets to breathe.
After a couple of weeks of carting all of this around, I will look like the one who's on steroids, not Julia! I am, needless to say, on a huge mission to wean her from all of this equipment. Our many hours sprinting off the vent on Easter Sunday make me very hopeful that this could happen one of these days very soon, at least when Julia is awake. It is always one step back after two steps forward as Julia recovers, though. We haven't been able to take her off the vent much at all since Easter. Alison calls Julia "Beeping Beauty", since it is a common sound to hear "beep beep beep" throughout the house whenever Julia decides to fall asleep.
We know that we're making progress on some fronts, even if she's not improving equally in all areas. Julia's extremities are moving more, stronger, and faster. Super-Baby now regularly lifts her head forward from where ever she's sitting, and turns from side to side. This makes trach changes much more tricky. She also has another new look. She's lost her eyebrows and eyelashes for the fourth time. Every couple of months her skin grows very thick and then flakes off taking her eyebrows with it, similar to cradle cap in newborn infants. Then the brows and lashes grow back, thick and luxurious, only to fall out again. It is possible that the thickened skin growth is due to the steroids Julia has been receiving to suppress her immune system so that it doesn't reject the transplant. The good news here is that she had her last dose of Prednisone yesterday, since there is no evidence of GVHD. Now we will watch for the chipmunk cheeks and other signs of steroid use to reduce.
Our great hope now is to stay out of the hospital long enough to find out what routine is! Our friends have helped in many ways, especially by providing home cooked meals while we were in the hospital and now that we're out. Julia still is at her most alert during those night owl hospital hours, which will take some retraining. A year ago I was reading books on "The Self-Comforting Baby". Now I'm trying to get her to stay awake through the day. Today, during physical therapy, Julia worked to extend her back muscles, needed for sitting, muscles she hasn't used for a long time. We see something new every day from this very special little girl, with the help of many friends and professionals. Please keep your good thoughts and well wishes coming. We are making progress!
~Julia and her mom
Julia's Journey #70 5/12/99 T+265
We're breaking records! We've now been out of the hospital for a full three and a half weeks! Our previous record was 2 1/2 weeks, the first time we were released from Duke. Julia currently has a cold, with a lot of secretions and a slightly elevated temperature (she's hanging out around 96 degrees, warm for her). But she has some advantages this time that have allowed us to stay out of the hospital that weren't present before: she now cries and coughs, which help move fluids out of her lungs; she is able to take an extra 240 ml of water per day which keep her secretions loose (she was too edemic before); she is now off Prednisone and Cyclosporine, the two immuno suppressing drugs that allowed her body to accept the transplant, so she can develop her own immunity to the cold. This cold has been tenacious. In fact, Julia's primary care nurse caught it, and has been unable to work for the last three days! Julia has been able to fight off the worst of the cold with the help of the antibiotic Cefzil, and despite the usual discomfort that happens to anyone with a cold, and we're still at home.
Our family has adjusted to having nursing care in our home. When we first came home after being gone for more than eight months it was a little disconcerting having people we had just met spending so much time with us. Julia resides in our living room, central to our house, and the nurses usually arrive at about the same time Rob brings the other kids home from school. It was somewhat awkward at first, feeling that as you sit down to dinner there is someone you don't know a few feet away listening as you discuss the events of the day. But we have gotten to know everyone, and their presence is now part of our routine. Most often the nurses arrive at 7 PM, and stay until 7 AM. We have an extensive schedule of care items that must be done with Julia, and it is a great aid to have the nurses here to help. Otherwise this little cute girl would easily take up 23 of my 24 hours every day! Here is an idea of how our day goes:
Every 3 hours: vitals taken (temp, blood pressure); diapers changed; suction/percussion; repositioning; hand splints put on or removed; lip and skin care; pulse ox probe change; bolus 30 ml water; enteral supply and volume check; chart and record events
Every 12 hours: <9 AM and 9 PM> medications drawn and given (this takes about 45 minutes); catheters changed; trach and g-tube care
Every 24 hours: <9 AM> replace artificial nose; lab draws or port flush; home therapies (physical, occupational, speech -- up to an hour each, depending on Julia's tolerance)
Every 24 hours: <9 PM> fill humidifier with water; bathing, clothing change, weight taken; suction machine cleaned; trach collar changed; enteral bag changed
Once a day: Our chore for the week
Monday-- trach change
Tuesday-- sterilize syringes, wash trach ties
Wednesday-- medication check and reorder
Thursday-- clean, organize, inventory supplies and reorder
Friday-- change vent tubing and intake filters; check and recharge vent batteries
Saturday-- monthly chore (varies by week)
Sunday-- review go bag/accessories/batteries so ready to travel emergently; very thorough hair wash and nail clipping
I haven't added up the total number of minutes each of these task takes. I haven't had time! :-) But, I can tell you that most of my day is taken up with Julia's care and thus I am very grateful for the nursing help. Realistically, if Julia were a normal active 16 month old kid her care would still be very time consuming. The major difference is that in her current state Julia requires nearly as much care when she's sleeping as she does when she's awake. Suctioning alone is a round-the-clock venture. The nursing care allows me to say a quick "Hi" to my husband and our two other kids, and a chance for me to sleep at night. Anyone contemplating a transplant for their Krabbe's kid should be aware that just because the transplant is over doesn't mean that the care reduces any time soon. When Julia has a cold or other issue the amount of care she requires grows even more intense. On top of all of this I still need to clean and get the laundry done, and I still like to spend some time just having fun hugging and loving Julia!
So, what is Julia doing to deserve all of this attention? She continues to move more rapidly and frequently. Her legs respond virtually always to touch by bicycling, usually three or four times in a row. She is starting to move her wrists from palms up to down and vice versa action, and do ankle twirls. These motions are very slow and deliberate. I no longer need to worry about her ability to hold her head up when I lift her. Yesterday when Julia was sitting in her bouncy chair she held my gaze at mid line. Very slowly I moved to the right. She was able to follow me all the way to the side with her eyes, and then back to the left. I can not tell if she was looking at my shadow (difference between light and dark), or responding to the sound of my breathing, or just truly SEEING, but her actions were full of purposeful intent. And, the snake tongue was going full speed ahead! Julia's hearing remains very acute.
I will be leaving Julia at home with dad and our nurses on Friday as I go to the Hunter's Hope symposium in New York for just that day. It is amazing to me that this symposium is about 10 miles from where I grew up! I will be squeezing into one day both the lectures at the symposium and a quick lunch with family and friends from my home town, many of whom I have not seen since before Julia was sick. Although I wish I could make my visit longer, I am happy that Julia's improving health is giving me the chance to participate at all.
Julia is doing well from a transplant perspective, and is making slow but steady progress regaining function as she begins remyelinating. Your well wishes and prayers remain as important as always. Julia is on the vent most of the day and still also requires a little oxygen as she completes this cold. Her number one priority is to sprint more and vent less, which considerably broadens what we can do to stimulate her mind. Carting the 161 pounds of vent and equipment around certainly restricts what we can do to introduce Julia to the pleasures of the world around her. Just moving her over to the TV to watch the Weather Channel requires a nearly Herculean effort. Julia swallows better, and enjoys tastes of food much more without the vent. The vent serves its purpose in bridging the gap between Julia's remembering to breath and how much breathing she needs to maintain her oxygen, but now we need to move on. So, that is our goal: more breathing and less vent. Breathing is fun-damental!
~Julia and her mom
PS: Please note that our address has changed! I am finally up and running on the home computer, using the following NEW address. Please change your address books to firstname.lastname@example.org to contact me. I love hearing from you. Sending emails to this address will ensure that I get your message. Sorry for the inconvenience!
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