*****I started on this post in March 2012, in fact, the title was, "March, already". Well, here we are in July 2012 and the post has never been finished and lots has happened including a trip to Italy and back. I will try to make all of this make sense.***** Me again...it is now February 2013 and I am determined to finish this post today. I am so sorry for an entire year without a post but our home is full of activity and the last thing on my mind is sitting at the computer for any huge amount of time. It is also sometimes an emotional process letting our lives pour out onto the computer screen and I admit to avoiding that. I tried to sprinkle photos throughout the post from our year...some taken with a "real" camera and some cell phone shots.
I really can't believe it is May (haha) already. The hours turn to days, turn to weeks, so fast. I don't know if this is a blessing or a curse. Time seems so much more important to me now than before and the passing days seem like they inevitably bring us closer to the day when Liviana will no longer be with us. Despite that looming thought in the back of my head I try to live each day one at a time and cherish every smile, glance, giggle and snuggle from sweet Liviana. I have been reminded all too well, with the passing of some of her leukodystrophy pals lately, that the days are entirely too short for the love we have to give to these fighting, loving, living children of ours.
Since my last post Giovanni returned from Italy in February (2012) with A+ reports. Overall, all of his tests, including the MRI, revealed no change since his June testing. This is AMAZING news. We of course didn't have MRI results for Liviana before we knew about MLD but we could assume she would have shown signs of demyelination during the same time period. Brad discussed with the doctors, at length, his night waking and what seemed to be intense pain in his hands and feet. His nerve conduction test did not reveal peripheral neuropathy and was unchanged from his last test in June. This would imply that the pain was not related to neuropathy. The conclusion was that the pain was related to growing pains. Now, Giovanni is a very tall boy for his age and he did indeed grow A LOT since June of last year but I still find myself unsure that he could have such severe pain in both his feet and hands (which is not a typical location for growing pains). One of his annual tests is a bone scan that reveals bone age based off of the size and other equations of the hand. When he had just turned 1, his scan revealed the hand of a 2 year old and likewise, his scan this past January, right after turning 2 revealed the hand of a 3 year old. In addition, his body has had to catch up from a short period of growth arrest with his chemotherapy. When he had an x-ray in October 2011 after jumping off the couch his pediatrician said his x-ray showed obvious growth arrest and continued growth lines because of his treatment. It is
possible that the pain is intensified because of his body playing catch up and an already aggressive growth patter given his size. I am happy to say that the night waking, pain and screaming has improved. He pretty much sleeps through the night now but does wake a few times a week in the morning with what seems like pain but he gets over it quickly. In February he started sleeping with his shoes on. I normally would not be too keen on this idea but he seemed to get comfort from the shoes and sure enough, he started sleeping better. I did not know if the pressure feeling from the shoes helped his feet or if this is all the crazy observations of an obsessive mother :). He eventually stopped wearing the shoes after a few weeks and had nights on and off with pain but overall things are MUCH improved. I like to think it is because his treatment has kicked MLD butt! ***
Update from early 2012...G was awake from 12:30am to 4:00am crying and screaming in pain. When I would ask him what hurts he would point to his feet and cry. He was also shoving his hands in any tight spot he could find because they hurt too. Last night he slept fine. It ripped my heart out holding him all night trying to help him sleep through the pain. IT was so much like many nights with Liviana. I hope he never has a night like that again*** ****since this update Giovanni has been sleeping fine at night and waking up fine as well. Overall, night waking issues seem gone. He was attacked by what seem to be mutant mosquitoes while in Italy and has been waking frequently from that but I was glad to have the explanation this time.****
2013 Update: G is waking at night and having a hard time getting to sleep. He moves and twitches his feet and hands around. He doesn't cry but he is definitely uncomfortable. He is a big boy. He can wear Mile's socks (who is 6) and has grown just since November. I suspect this night time discomfort to be just part of growing for him but I am sure we can rule out disease processes on our visit next week.
Giovanni is by all accounts a normal 30 month old (this was typed last year obviously). He runs, climbs, plays..does everything he would be expected to do. By this point with Liviana there were several things showing up that were concerning. It was almost exactly 2 years ago (In May) that Brad and I had our first conversation about Liviana. I remember the day so clearly like it was yesterday. We had just gotten home from the Farmer's Market sans children and Liviana was standing by the closet in Aria's room whimpering and seeming to be in pain. I picked her up and held her and was unsure what was causing the tears. I sat her down on the couch and looked at her legs. Her right leg was looking very different from her left. It was beginning to turn inward in a knock kneed and hyperextended position. This happened SO fast. I have a photo of her from the last week of April standing with perfectly "normal" looking legs....2 weeks later there are noticeable changes. We stood there that day discussing how we both had noticed she seemed to fall down more and have balance issues. Those two things combined with a host of little things....her shaking and unable to balance if she was put on the counter or other surface with her legs hanging down to sit, shivering uncontrollably after nap and especially during bath times, overheating easily...were all screaming, "there is something wrong" in my head. G doesn't have any of these issues. I have "tested" him, often afraid of what I will find but overjoyed when all seems normal. He has buttoned his shirt (something Livi's shakey fine motor skills would not have made possible), climbs up and down on tall surfaces, sits on the counter when I let him :), stands on his tippy toes for long periods of time to reach things...he really leaves me in awe every single day. To only be the second child in the world to have undergone this type of treatment for MLD...I am really speechless and full of so much hope all the time.
***2013 Update on G*** One of G's favorite things to do is jump. When he is excited he jumps, when he tells you something he jumps, when Daddy gets home he jumps. It is so awesome to me every single time his feet leave the ground. He goes up the stairs one step at a time holding 2 fire trucks and little people, he jumps on the bed, he rides bikes outside, he pulls the wagon around...he is an amazing, normal, 3 year old little boy. So amazing!
Giovanni and I will go back to Italy in July 2012. This will be a VERY telling visit. By that time in Livi's life she had already had an appointment with her geneticist to figure out what was going on with her. I remember her standing in the room in her cute denim dress in the middle of several people. She was so cute and entertaining but too quick of a turn and she would lose her balance. It is hard for me to imagine Giovanni having a dramatic change downward in just 2 months but then again...MLD has done that to us already with Liviana. All the good thoughts and prayers for his continued well being are welcomed.
He is still not talking as much as they would expect at 27 months. He communicates easily, just not with a ton of words. I doubt this is MLD related since both Aria and Miles had expressive language delays. He has the same pattern they do, accept he is ahead of where they were at the same age. His testing reveals at age level or advanced...we just need to get him saying more. He has about 15-20 words and repeats things randomly on some days. He says, "Daddy" all the time. I feel anxious about him losing is speech to MLD before I ever get to hear him call me Mama. I am going to start him on speech in this summer and hope to see some improvement.
*****Inserting July update on Giovanni's visit to Italy*****
Giovanni, Adelina, Tarah and I headed off to Italy on July 2nd. I had such mixed emotions going into this trip. I hate flying. It is an irrational fear that developed after having kids. I think the only time I wasn't stressed is when we were all together because if something happened I wouldn't be leaving any children behind. I also was very afraid of leaving Liviana and fear that she would get sick while I was gone. It can happen in an instant. One minute she is smiling and calm and the next her breathing is labored and she needs oxygen support. I had trust in Brad though to take awesome care of her and knew they would have fun while I was gone since it would be easier for him to get out with just the 3 of them. They went bowling, to the Children's Museum, out to lunch, the Farmer's Market and played outside in the pool. I was so happy she was able to get out and enjoy the week, even if it did get horribly hot.
I was also dreading going back to Italy. Despite the image people have in their head, it is not all glorious. When Brad was there in February, his friend Jason said he didn't even believe they were in "Italy" until a couple of days into the trip when they went to a neighboring town with friends. The idea of being back at the same hotel, in the same hospital rooms we visited, with that same smell in the hallways felt overwhelming at times. There isn't anything inherently wrong with the place, it is just the memory of that time last year going through it all with G and the rest of the family that I try to avoid. At the same time, I was so excited to show off our G-man. He is so stinking cute and he is, by all accounts, still a normal 2 year old.
In a summary, all of his tests came back normal and unchanged. His nerve conduction tests, MRI, motor skills evaluations, psychological evaluations...all normal. His hemoglobin which was always consistently around 9 is now comfortably at 13.6 so no more concerns over iron supplementation. I attribute that in part to his love of Odwalla Green Machine :). I feel like I just smiled at him all the time when we were at the hospital. I am so proud of him...so proud of all he has been through and how happy, well adjusted and social he is, proud of his accomplishments, proud he is so strong, proud that his test results show hope for other children in the future. I feel like I (we) have so much to learn from him, as we do Liviana. He and Adelina were both so awesome on this trip. Our longest flight was 10.5 hours and we had people coming up to us comment on how well behaved they were. Their smiling faces made that unusually long flight home more bearable for many of the passengers. I love seeing our friends in Milan...making these new memories there is important to me so I can slowly remove the negative feelings from his days in the transplant ward and the difficult days in Italy. It also helped that we were there in July so the sun was shining...something we didn't often see during our first few months there.
Onto sweet, beautimus Liviana. She keeps us on our toes...she likes it that way. A few days after Brad and G got back from Italy, on February 14, 2012, we took Liviana to the ER. G was sick first, right after returning home and it passed onto Liviana and Adelina quickly. When Livi gets sick it comes on quickly and gets scary fast. She starts seizing and pretty much becomes unresponsive. She doesn't make eye contact, she doesn't react to our voices, smile...nothing. Her breathing was rapid and I had been unable to get her fever to come down, which causes more seizing. We were lucky to have our sitter in town so Brad and I took Liviana to the ER that night. Her fever upon arrival was 105.2! I was only getting a 103 reading at home (I have since bought a new thermometer I trust more. X-ray revealed a little bit of "something" in her right lung...she was quickly admitted. She ended up testing positive for RSV. After being in the hospital for a day and a half she was put on oxygen, as she was no longer able to maintain her saturations. I was devastated when I got the text while I was on my way into the hospital. I know oxygen needs are common with RSV but her situation is not normal, her ability to fight this and cough is not normal and it seemed like such a back slide. It was, for me, the scariest time I had with her to that point since I gave birth to her and wondered if she would survive her CDH. I laid with her in the hospital bed, still unresponsive, on oxygen, and ran my hands through her hair, like I always do when I hold her. It hit me like a ton of bricks...when we lose her, I will never be able to touch her hair again. I could not stop the tears this time, and I am usually pretty good at that. I felt desperate and helpless. I felt insane for mentally refusing to lose her EVER. She ended up in the hospital for a week. Not only is the hospital challenging for us with 4 other children but the whole time we just want Livi home with us. It does not feel right to not be all together. I spent the week running back and forth from home to hospital and in the middle of it all took Adelina to the ER too because the RSV in her, being so young, was scaring me too. Our amazing babysitter, Annie, happened to be off work that week which was a an absolute God Send. She was able to say with the kids so I could go up for a few hours and come back because of a nursling at home. Brad stayed at the hospital most nights but I ended up staying twice and kept Adelina in the room with us at the hospital.
The entire week was a absolute blur.
After discussions with our doctors at Children's Hospital it was decided that Liviana being on Hospice care at home would be best for her. This allows her to stay home more when she gets sick, allows doctor visits to the home as well as nurses that check in on her once a week. It doesn't have the same connotation as "Hospice" for adults...it just allows us to have an appropriate team in place and nursing care for when that time comes since things can be more unpredictable with children. It also doesn't include a nurse being in our home taking care of her all the time. We are Livi's parents and caregivers. The nurses are here for about 30 minutes once a week to check on her, take vitals and see if we need anything. It works out perfectly. We met with the Hospice team the day after she came home. I was an absolute ball of anxiety and nerves leading into that appointment. I knew there could be challenging discussions and it is all so new and unknown to us. I think we keep a pretty tight circle around Liviana and letting in people we don't know is hard for me. They were all wonderful and good at putting my mind at ease. We were faced with needed to discuss signing a Do Not Resuscitate form for Liviana. Not something any parent should have to face signing for their child. I held onto the paper for many weeks...not ready to sign it. I know we can change our mind at any moment but it is the whole concept of having to sign something like that for my children...knowing that that one, short document will come into play one day.
Deliveries of medical equipment started...suction machines. oxygen machines, oxygen tanks for travel and power outages, IV pole. Our living room was transformed overnight but I had peace in it all knowing that we could take care of Liviana at home the next time she was very sick. It is the only place I feel all is well...when we are all together as a family, sleeping under the same roof.
A new type of anxiety comes with having a child with a terminal illness. Things you never imagined could make you anxious do...simple things like being at the grocery store too long or pretty much any important date on a calendar. You learn to really live one day at a time since a day, an hour or a minute your whole live could change.
I really have to work hard to not get lost in sadness. It isn't a pervasive feeling, just fleeting moments when some memory catches me, or I think of Liviana running up and greeting Miles as he comes home and them running off outside to play. We have 5 friends who have little girls who turn 4 years old, just like Livi, within a month and a half of her birthday. I can picture them all giggling in a corner or playing together. I imagine her still always wanting to wear dresses like her big sister and probably annoying Aria by always wanting to take her stuff and play in her room :). I am sad that Aria will miss out on that traditional sister relationship with Liviana. That was one of the first things that made me cry myself to sleep after she was diagnosed. I was so sad for Aria's loss. I can see that in Aria too. She lays with Livi and talks to her. She talks about, "If Livi was sharing a room with me". I can't tell you how much I would LOVE to hear the girl squabbles. The, "That is MY shirt", "Did you take my pink jacket". It would be music to my ears. The biannual clothing rotation for seasons is always difficult. I have one tub that says, "2T" with a big heart drawn next to it. I'm not sure how easily I will be able to let Adelina wear those clothes. I can see Livi still walking in them...they are the last clothes she walked in and then the clothes she stopped walking in. They are the clothes that she wore as she sat in her favorite spot on the couch in Milan, the swimsuit she wore on the beach in Sardegna and the tutus and sweaters that so many came to recognize as her. The awareness that these "things" are all we will ever have of her one day changes the way we look at them. I find myself putting anything that reminds me of Livi, that was her favorite things, the book she read most, her photos...in one tub that is tucked into the back of my closet. I will keep these items and cherish them for the rest of my life. When she is no longer with us they will warmly remind us of her. I got this uncomfortable feeling one day as I added something to the Livi tub...what will happen when I am gone? What happens to Livi's things? Will she still be remembered and cherished since she herself won't be here to keep that legacy going? I don't ever want her forgotten...that smile, the way her eyes twinkle with her happiness, her long lashes, her long, curly hair, her fighting spirit, her humor, her love of pretty dresses and Calliou. I want her to live forever and since I can't stop time I want her legacy to live forever. This all makes me feel quite irrational at times...desperate and oddly clausterphobic...like I can't "fix" things and make the outcomes perfect. So those pretty dresses and favorite books mean so much more than anyone could ever know.
I have this odd sense some days that I don't even recognize her. I don't mean in the basic sense of recognizing someone but rather, my memories of her walking and talking are of a 2.5 year old...even after her 3rd birthday she still was talking. Now, before me, as I dress her and talk to her and hold her in my lap I see a very tall, 4 year old girl with hair half way down her back that curls up tight after a bath. I see my 4 year old girl and I would do absolutely anything to know what Liviana would have to say to me as a 4 year old girl...Absolutely.Anything. It is a sad feeling. I am sad for her, as much as selfishly sad for myself. I have told myself that I am not missing those moments because they were never meant to be part of Liviana's life but are rather are just part of my imagination. When I look at old photos of her, as I so often get lost in, It is as if I am looking at photos of someone else...it is an odd experience. As a mother though I can't help but look at Miles and Aria and wonder about the wonder in Livi's face as she learns something new, or makes a new friend, scores her first goal or talks about her first day of school, which would have been this fall 2012. She would have been playing on Mile's soccer team this year with him...how awesome would that be to watch? Miles was acutely aware of that one day as we headed off to his soccer game. He told me, "Livi would have been playing with me, right mom". I said, "Yes sweetie...she would have loved that". He gave her a kiss as he ran off to the field and told her he would win the game for her. Love that kid!
I can't focus on that stuff though...it is a quick way to a downhill spiral. I focus on who she is instead. I wake up seeing her smiling face looking right at me. She gives me the sweetest sounds and when she looks right at me it melts my heart. She can still answer affirmatively with an, "Uh Huh" if you ask her a question. I LOVE that. She falls asleep almost instantly when we hold her and she loves to laugh at her brothers and sisters, especially Giovanni. He is her little entertainment.
When she was an infant she would reach up and hold my hair in her hand when she nursed. When she stopped nursing she would still hold my hair to get herself to sleep. Those nights of her feet hurting and difficulty sleeping it was my hair that would eventually help her fall asleep. She was slowly losing the ability to actively hold my hair on her own during our time in Italy. When I would visit from my time in the hospital and get her down for nap I realized she could no longer hold my hair, as her motor skills had declined and her fists were held tightly clenched. That was heartbreaking. For every night that I laid there with a to-do list in my head, wanting to get up while she played with my hair...I would take them all back in a second. When she is having a hard time settling down I have started putting my hair in her hand, which is usually tightly held in a fist. She makes the happiest, sweetest sound of contentment. I wish she always had that contentment.
I have been obsessed with finding all of our old videos and photos of Liviana. I was going through photos and videos from Brad's old phone the other night and found a video he took of Livi with his US phone while we were in Italy that I had never seen before. It was so weird and awesome to see her reaching her hand up to scratch her head and reach for the phone. We take for granted our kids being able to move their arms on their own. I would run a million miles to just feel her hug me again. A HUG! That is why I always say, "Hug your babies". You have no idea how amazing a hug from them really is.
***May 2012 posting to present***
I knew when Miles got sick last week (May) that it was probably going to eventually make its way to Liviana. He sits with her all of the time and plays with her hair and she always gets big kisses at bed time from everyone. Miles rarely gets sick and if he does it is pretty short lived. This seemed to be a nasty virus though and he ended up home for 2 days with a fever, eventually going back to school on Wednesday. Liviana started her fever on Friday. We acted quickly since fevers increase her seizure activity. She started having respiratory difficulty and we had to dust off her oxygen machine after not having to use it since late February. With the illness that meant we had to make sleeping arrangement changes. She needs to stay downstairs with oxygen so she slept on an air mattress and Brad slept next to her on several rolled up comforters on the floor. I stayed upstairs with the nighttime nurser. We had several nursing visits and the Hospice doctor visited on Tuesday. He said if she still had a fever the next day they could consider starting her on an antibiotic since it likely indicated the virus had caused a bacterial pneumonia.
On Wednesday morning she woke up doing great. I got some smiles and she looked directly at me (a big deal since she is pretty unresponsive when she is sick). She was fever free and I was able to take her off oxygen. I was ecstatic. I talked to my mom and told her the good news, sent a text to Brad...it looked like she was getting over the virus...yay! I gave her her first albuterol breathing treatment in the morning as I was getting the kids ready for school. This was a burn the breakfast, running between Livi and finding socks and shoes type of morning. She was doing well and by the time the kids left for school she was ready for a nap and slept peacefully. I sent a text to Brad saying that she was sitting in her seat (she breathes better laying down when sick), sleeping, fever free, off oxygen and saturations were great. I noticed she seemed to be getting more uncomfortable starting around 10am. I switched her positions, gave her a little ativan and tried to figure out what was wrong. I took Adelina up for nap at 10:30 and when I came down Livi's face was BRIGHT red. I was startled and obviously wondered if it was reaction to albuterol. I called her nurse and she thought the 3 hours between it showing up and the treatment was unlikely. She seemed distressed and uncomfortable so I held her and tried to help her feel better. By this time Miles was coming home from school and things really just went haywire. Her extremities were ice cold. She was moaning and looking at me with wild, scared eyes. The oximeter was not giving me good readings. I normally will look at her rather than the machine but she was in such distress that the all over the board readings scared me to death. I called Brad, called her nurse and asked for someone to come over right away. I was terrified! She was in my arms screaming and her heart rate was reading over 200 and her saturations were sometimes in the 80's but sometimes in the 70's and then the machine would go all over the place. Brad showed up, the nurse showed up and she confirmed her HR was in the 190's. We gave her some more ativan and it didn't do much to help her. Our nurse looked at us both and said she was worried that Livi would not be able to maintain her HR that fast for long. I had the WORST feeling in my stomach as I held her and just realized I could be suddenly and unexpectedly losing her right there. Brad ran over to the school to get Aria out of school so she could be home with Liviana. The look on her face broke my heart as she walked in the door and came over by me. My head was spinning. I was NOT ready for this at all. I knew she still had more life to live. Our other nurse showed up and our doctor (one of the Hospitalist from Children's who has seen Livi a few times in the hospital). On the phone our doctor had said to give her another dose of Ativan and by the time she got there Livi had finally calmed down and was breathing more normally (on 3 liters of oxygen), but had started running a fever again. The doctor said that while an elderly adult could not sustain HR above 200 that long that a child could do it for days. I do not fault our nurse for alerting us to the seriousness of the issue though. It was a frightening situation. The doctor believes that she recovered from the virus but the bacterial infection kicked in and that is what caused the sudden onset of issues and distress that morning.
We sat in the Living room, while Aria and Miles drew Get Well card for Liviana at the dining room table, discussing what our options were for Liviana. The 300lb elephant in the room was of course that we could lose her and that it is usually a pneumonia or other serious illness that takes our MLD kiddos from us. We discussed said elephant and what our options were. We could go to the hospital, get a chest x-ray and have her taken care of there. That would of course separate our family and put Livi in a less than comfortable situation away from home. We could start her on an antibiotic at home and hope it showed improvement for her in 12 hours or we could do nothing and free he from fighting this monumental fight. We chose to start her on an antibiotic. This was the first experience with the house full of doctors and nurses and machines humming. I know Aria and Miles were worried and I hate that their lives have to hold such fears at such young ages. We tried to explain to them that she was very sick and we hoped the new medicine would help her. It was just two day of the Cipro and Livi woke completely improved. Hallelujah she had kicked this. So humbled by this little girls strength.
Up until this illness Livi was still getting 3 "meals" a day that I made her through her g-tube. She got 3-210 ml meals and then two more 210ml rounds of water in between meals. About a week after she got over the illness she suddenly started vomiting during her feeds. I started lowering her volume slowly each meal...trying to get to a point she could tolerate. This is typical with MLD disease progression. The body just stops working effectively and can't tolerate the same volume of food. Just when I thought she was at a safe point for her meal volume she lost an entire feed all at once. Bless her sweet heart. It is so scary when she throws up because she immediately starts aspirating since she can't swallow. We try to rush to sit her up and get her suctioned. Then I feel so bad that she is covered in all of her food. The worst was when I had just wheeled her outside to get some sunshine and fresh air. I went back in then to get Adelina and she had thrown up and was covered in her entire feed...which contained sardines. I also put Turmeric in her food so the yellow staining never came out of her favorite pants. Such a silly thing that makes me sad for her. She doesn't react much, doesn't cry, doesn't seem to get upset but I know it upsets her anyway...she takes in life just like any child her age would...she just does it silently. Eventually, I had to change Livi to continuous feeds to make her get enough volume throughout a day. She has now settled on 85ml's an hour that runs all day, splitting between her food and water. So far she has held steady at that 85mls even though her big illness this past December. Once we went to continuous feeds it became harder to keep the weight on her. She overall has lost about 1.5lbs which seems like a lot since she has gained so much length and is already so tiny. I know she is healthy though because her hair and nails grow like crazy but I still keep trying to beef her up without overloading her too much in each meal hour.
Spring and summer is so busy. We wrapped up the spring and the school year with soccer games. I am pretty adamant that either we all go to games together or I or Brad will stay home with Liviana. It just doesn't feel right to me to leave her behind. The only reasons we have had to make the decision for one of us to stay home is if the weather is too cold or too hot or too much travel is involved. Aria's final soccer game rolled around though...right after Livi's big May illness and Brad and I both really wanted to be there. Livi was still recovering and on antibiotics and even though the game was up the street at the school, we thought it would be too much to bring her out when she wasn't a 100%. Our wonderful hospice nurse, who is like a member of our family, offered to come by off duty and sit with Livi so I could go up to the game. I was so happy to be able to see Aria's last game (It always seems that she scores her goals at the games I miss) but felt awful going with Livi at home. I agreed to have Carolyn stay with Livi and I loaded Adelina up in the sling to make the walk up to the soccer field. I felt sick to my stomach that entire walk. I can't even describe the feeling of leaving her at home. It didn't feel right but neither did missing Aria's last game. I know it doesn't seem like a big deal to other people but this was the first time ever we left her home without one of us...even if it was just Adelina...she was without her family. I had tears welling up in my eyes when I got to the game but didn't want people to think I was a freak so I pretended all was well, all along feeling like only part of me was there. When we got home she was in good hands...snuggled in Carolyn's arms sleeping. That made me feel so much better knowing she was getting loved and snuggled while we were away.
There are strange dynamics that take over when you have a child in a health situation like Liviana. We seem to sometimes have multiple families in one. Sometimes I feel like Brad, Aria and Miles are one unit. They get to go more places together and live more of a life outside our home. It isn't easy to get Liviana loaded up and isn't always comfortable for her and although we had put in for her stroller/wheelchair request in late April, it took a couple of months into summer to finally hear something and get it for her. At one point I had gone 2 months without leaving the house. Brad did grocery shopping, running the kids places, friends picked up the kids. I stayed home with Adelina, Livi and G. The heat of summer is hard on Liviana and I could not wait for cooler temps since we had her new chair, to be able to take her places. Once the new school year rolled around we had a whole new round of activities that included soccer 5 times a week. And guess what...I can only think of two games across both Aria and Miles that we didn't all go to. It was actually really, freaking awesome...loading up Miles and Aria and G, Livi and Adelina and heading out 5 times a week for soccer. I loved it! It was controlled chaos and more than once, as I navigated the 5 of them by myself when Brad couldn't make it, through the soccer field...holding Adelina, pushing Livi's wheelchair and monitoring the other three as we entered the parking lot, had people either look at me like I was nuts or want to high-5 me. I am mama hear me roar....and all together is the ONLY way I feel complete. I can't wait for soccer season to start again. I look forward to getting Liviana out and seeing G and Miles play during Aria's games, Adelina learning to kick the ball and chatting up the other parents. It all brings me so much happiness.
As we got closer into fall I knew I wanted to go down to Wichita to do a couple of photo sessions for friends. I wasn't looking forward to going without the whole family though so Brad and I spent a lot of time discussing if it would be possible for us all to go. Aria many times throughout summer and into the beginning of school, as kids would talk about their vacations would say, "Why can't we go anywhere". I always felt so bad. I don't want them to resent Liviana (although the love her to death and would never intentionally resent her) for us not being able to easily do things but is certainly is not easy for us to travel with her. Because I make her food it would entail a lot of extra travel gear, blenders, oxygen tanks, feed pumps, supplies for a crisis, food already made, cooler, etc. We just flat decided we would make it work in October though and we would all go down to Wichita. We really were like the traveling Griswolds. I don't think you could have squeezed anymore into that van. I brought some premade food for Livi but otherwise brought the blender and it the grocery store to make her meals while we were there. The trip really went well. We stayed with my mom and my brother, his wife and their daughter were there also. We went to the zoo and the kids played outside. Adelina and Jensen played together and Livi just took it all in. Like I mentioned before, Livi is silent but she takes it all in and loves all of the activity. She will laugh at the most surprising times and smile and interact in her own way. She seemed really comfortable the entire trip until our ride home. I realized when we got home that she had a pressure sore on her spine from the combination of her stroller and carseat. I felt awful and now we know to give her extra padding on the back of her seats. Even our trick-or-treating outing on the bumpy sidewalks was uncomfortable for her.
Sometime in September I was contacted my Alessia in Rome. She is with the Fondazione Telethon and they fund the clinical trial that G went through in Milan. They did a day of filming while we were in Milan in July but really wanted to get the whole family so they planned to send a producer and crew from Italy to do a short film to be used for the December Telethon to raise money for rare disease research in Italy. I really can't say enough about all they are doing for rare disease research. Maybe they just keep it all a secret here in the US but it really seems to go leaps and bounds above what we are doing here. I am proud to know them and let our family do all we can to help them raise more money. We were very lucky to have Max Croci, who did our filming in Milan join us here in Omaha at the beginning of November. They hired a local film crew from here in Omaha and they were great to work with also. It means so much to me to have this footage of our kids and Liviana especially. I will cherish it forever. Thank you Max and Telethon!
Thanksgiving was wonderful and low key. We had made plans that my brother, his wife and their daughter would come for Christmas so just my mom visited for Thanksgiving. I appreciated the low key holiday. Aria's birthday always falls over Thanksgiving break so she had friends over for our first ever house-full-of-kids birthday party. It was great!
At the beginning of December we headed out to Santa's Woods for our Christmas tradition of picking out our Christmas tree. I really don't love this time of year. It just brings me back to when Livi was diagnosed. I wish I could escape the season but the kids look forward to it so that brings joy to us too. The Christmas tree was positioned this year right so Liviana could see it from her bed. She beamed when she first saw it put up and the lights turned on.
Everything was going well leading into Christmas break when our little, already fragile world came crashing down again. Adelina, who was now 13 months old, had been increasingly fussy for a couple of weeks. Wanting to nurse all of the time, whining, horrible sleeping. I noticed she had 4 molars coming in all at once so that seemed to explain the agitation. On Sunday, December 16th she seemed fine in the morning and upon me getting home from the store she ate almost an entire container of blueberries (her favorite). She then had two pouches of banana puree. A few minutes later she she threw up. At first I thought, "Oh wow, she ate too much" but then I got worried that she had gotten sick from our friend who mentioned not feeling well after being with her a couple days earlier. I messaged her but she didn't report any vomiting issues with being sick. I kept an eye on Adelina and she just sat on the couch like a lump. She ended up throwing up again all over Aria who was so sweet and didn't even freak out but kept holding her, telling her it was alright, until I could get to her. It really bothered me that she seemed sick but wasn't running a fever. As the day went on her breathing seemed to get heavier but she didn't have any respiratory congestion. I had a nagging feeling something else was wrong but researching didn't turn anything up...friends said that the fever would probably show up soon. That night I laid awake all night with her laying on my chest, breathing heavy. She seemed so out of it. I know now, and have read other parents telling their story say the same, that she was dying. That morning I told Brad first thing that we needed to get her into the doctor. Of course we thought worst case scenario was a horrible virus we would treat and move on from. When the doctors office was full I said I we just needed to take her to the ER. She was hardly responsive at this point, breathing heavily, not opening her eyes much. Walking into the ER at Children's they immediately said, "2-2.5 hour wait". They heard her breathing though and sent a nurse out to listen to her. When she heard that her chest was clear they went ahead and brought her back right away. They weighed her and did the usual assessments and asked the usual questions we were finishing up and as an afterthought I mentioned that for the last two weeks I noticed she had been peeing more. It coincided with when she started drinking out of a sippy with water so while I wrote it off, it also bugged me. I sat in the room holding my little baby with a halting, heavy breathing and after initial blood test they came in to tell me she was in a severe diabetic state and that she had Type 1 Diabetes. She was in a diabetic coma, Diabetic Ketoacidosis and her blood PH was 6.8 upon admission. Anything below 6.8 is considered incompatible with life. We were immediately admitted to the ICU where she stayed for 2 days and another 3 on a regular floor, learning all we could absorb about our new life with Diabetes. I sat in the ICU room holding her, still out of it, still being slowly nursed back from near death with IV's and wondered What The H*#@? My, still breastfed, healthy eating, no family history, miracle surprise, MLD free, little darling now had to live her life with this autoimmune disease (yes, Type 1 is an autoimmune disease and has nothing to do with diet and exercise) that will REQUIRE her to have daily insulin injections to keep her alive. WTH? It felt overwhelming at the hospital learning about carb counting and insulin formulas but once we were home it seemed easier than it seemed while in the hospital. So, so many people stepped up to help during that week. Ww had not done our Christmas shopping yet, mainly because it is hard to get a sitter with Livi's medical needs and the other kids. We had a long standing "date" to Christmas shop that week because a friend had offered to come over with her kids and do crafts and have fun while we went out. Obviously that wouldn't happen. So here we were one week before Christmas with no shopping done, a week stay in the hospital, more time off work for Brad holding down the fort at home and a bunch of friends and angels from our church jumped in and did Christmas shopping, made meals, shoveled our driveway, helped at home and made sure we were set and didn't have to worry about anything. I cannot begin to thank everyone enough. As if I didn't dislike this time of year enough, we now had this horrible dx and hospitalization right at holiday time. Thank you, thank you, thank you to EVERYONE who helped make it a great Christmas for the kids on such short notice and planning. We love you all and are blessed to have you in our circle of friends. Julie...you are a rockstar :).
So, we have a new lifestyle at home. Sleepless nights as I lay awake staring at the clock waiting to test her blood glucose again. We have had one major hypoglycemic episode that caused seizures in Adelina very suddenly. It just.isn't.right and yet she handles it with grace and awesomeness, often saying, "Thank you" after I give her insulin injections. My mind is filled with keeping two of my kids alive in my home each day.
Less than a week after Adelina got home, a couple of days after Christmas, Liviana started breathing heavy suddenly. I checked her oxygen saturations and she was 88-89 so I dusted off her oxygen machine that sat unused for months and got her on oxygen. My biggest fear had happened. Aria had been sick with a nasty respiratory virus the week that Adelina was in the hospital and Liviana had gotten it, along with Adelina (which made her blood sugars a nightmare). This then took us on a journey that at first just seemed like an illness we had to get her over, like before. Her oxygen needs would increase, her fevers would come and go and she became less responsive to us. Things changed one day and suddenly, about a week later and her Heart Rate was dropping way down into the 30's and 40's and it was taking rousing to get her to take a breath again. One afternoon I could not get her saturations up with the machine we had, which only went to 5 liters. Her HR was dropping way low and she was having multiple bradycardia episodes. I was home by myself, literally keeping Adelina and Liviana alive, while keeping G happy and made a couple of calls to get a nursing visit to check on her. They knew the situation was serious when I called them because I never ask for help. About 30 minutes later a nurse and our Hospice caseworker showed up. They thought Livi sounded awful but didn't have the "end of life" signs they often look for. At the same time, Brad and I were told to be prepared and asked if we had let her know it was ok to let go. We had not...I wasn't ready for that...it didn't seem like time...I NEED Livi here with us. The Home Health team had to bring out another oxygen machine going to 10 liters, as her needs were going above the 5 liters we could give her. Brad was on nightime duty with Liviana downstairs while I was on nightime duty with Adelina's diabetes upstairs. One night, at 2am, we passed in the kitchen as I dosed insulin and got something for Livi. We were both bleary eyed each morning and scared.to.death. We were experiencing this all differently as well. He felt like he saw Livi's fight dwindling and I felt like this wasn't her time. Her respirations were low and her exhalation was longer than inhalation which I clearly remembered as a sign of end of life. This startled me and I texted Brad and he was equally concerned. We were able to get her on Cipro again, like we did when she was sick in May. I prayed that she would start to get well after a couple of days like last time. I didn't sleep each night I went upstairs for fear I would not see her again. I wanted nothing more than to just sleep in her bed, beside her and hold her all night. It is one of my greatest stresses...that I can't give her 100% of myself, as her mom all the time. After a few days on Cipro Livi did start to improve. Her Bradycardia episodes continued until she was off oxygen completely and then they stopped too. It blows me away to see her off oxygen, smiling and doing so well after so much fear that we would lose her. So many people from all over the world sent cards and letters to her. I would sit on her bed each day and show them to her and read them and then we taped many of them up on the wall next to her to remind her how much she is loved. I have no doubt that all of the prayers and cards and letters make a difference to her. She knows how loved she is and knows she has much more life to give and lessons to teach. I'm not sure I could have ever expected her life to help so many people. The messages we get from people talking about how much she has taught them means so much to me. She has taught me to live differently, love differently, laugh louder, smile more, don't sweat the small stuff, hug more, snuggle longer, work less, play more and live life with what is best for others in mind. I can't imagine life without her by my side.
The rest of January calmed down after Liviana was well. On January 16th, Giovanni turned 3 years old and on January 18th, Liviana turned 5....FIVE. I can't believe it. We did things pretty low key without a house full of people this year....too risky with illness season (maybe we should start celebrating their birthdays in July). We had a dear friend, who is a firefighter, come by with his crew on their birthday weekend to surprise Giovanni. We wanted G to see a firetruck and firefighters (he is obsessed with fire trucks) but didn't want to leave Livi at home for the festivities. Thank you so much Drew for coming by with your truck! G was very serious the whole time...staring, quiet, pensive. Firetrucks are serious business for a 3 year old boy :). I feel like sometimes G gets lost in the middle of it all. Miles and Aria are older and get to do more, Adelina is the baby and Liviana is always the center of attention and G is off to the side playing with his firetrucks, wearing his tools and sitting in his "office". Don't get me wrong, he gets lots of snuggles and play. He is my little buddy during the day. He brings me so much joy. His, "Thank you mama" and "Ok mama" is the cutest thing every single time I hear it. He is so cute and funny and sweet. He cares about Adelina and Liviana so much. I think we worry so much about Aria and Miles and how they will handle things when Livi is gone (we had a big talk with them this last illness round) but I worry quite a bit about G because he won't understand why she is gone...where she is being take and why she isn't here anymore. That breaks my heart and I hope she is here for many, many more years so he can learn to love her even more than he does today.
I was thinking the other day that one day that G will probably ask me about Liviana and what she has and I will have to explain Metachromatic Leukodystrophy to him and tell him that he has it too.....Wow. I can't imagine that conversation and the look on his face and how that will change him forever. When I look at G I see amazing things in his future. I get this feeling that he will be something amazing when he grows up and what he has been through and all that he can teach others is a foreshadowing of his future.
We are less than a week from our trip back to Milan for Giovanni's 2 year follow up. We will be gone February 16th to March 1st. This is the entire battery of tests again that he had at baseline. The MRI, EKG, EEG, bone marrow aspirate, csf draw, x-rays, ultrasounds, heart echo...the whole shebang. This is the big appointment....the one that tells them more than any other how this gene therapy has worked. I am excited and anxious all at once. I am optimistic that we will have amazing news and can take yet another deep breath until the next visit. I am probably most anxious about traveling with Adelina and all of her specialized medical needs with diabetes. We will be traveling with a bunch of new paraphenalia and pray that she has no major hypo or hyper episodes while we are away.
It has been a difficult 2013 already for MLD and Krabbe families. I think we all lost a little piece of our hearts when Gummy (Montgomery) Maughn passed away on February 3rd. His mom is very dear to me. She is the MLD mom who immediately reaches out to and "meets" all the new MLD families. She has surprised us with a new blender for making Livi's feeds and a beautiful fruit bouquet last January when Brad was away in Italy with G and I was a pacing basketcase the whole time. She is a mom who has been there 3 times over with MLD. She has lost two babies to this horrible disease and is nurse-mom to beautiful Tabitha who still fights and conquers MLD each day. We love you Cindy and your family is in our prayers. Livi has a new, very special angel looking over her along with Matthew, Rebecca, Josi and too many others who have flown high already this year.
Every night before I go to bed I give Livi a big kiss. Even if she is sleeping, I always tell her to have sweet dreams. When she is awake I talk to her about what dreams she will have that night...what we will be doing...where she will go. She always gives the biggest smile when I ask her about going to the beach. Hot air balloons, climbing mountains, going to an ice cream shop can't compare to a beach and sand and water to her. I cherish the moments we had in Sardegna and thank Erica from the bottom of our hearts for inviting us along on that trip during her conference. The gift of that time to Liviana is simply priceless. Toes in the sand, carefree, joyful. Those are the moments that carry us through the rough times around here. We have photos of the smiling faces all around us. Although I see a very different Liviana it is clear that the same spirit is still alive and well behind those amazing, glorious eyes. Her sweet face and toes in Sardegna.
I am beginning the ritual of packing, preparing and organizing for our trip these next two days. Aria started coughing yesterday and I want to zip her in a bubble to keep my two mini-travelers from getting sick and keep Liviana well while I am gone. My biggest fear is getting a call or message that Livi has gotten sick and isn't doing well while we are gone. Please send all the prayers and healthy vibes her way that she is able to stay healthy and happy during our two weeks away. Send well wishes that my mini-travelers stay well too. Illness is havoc for Adelina's diabetes and can get scary fast. I will swear away anyone who is coughing or hacking near her during out trip :).
I know the year was much more filled than I could get into this blog post. Those on my FB page are better able to keep up with our busy little family and share in our daily joys, love and fears. I love you all and love the little family we have built around us.
I will be posting updates of Giovanni's testing and our trip, as I can on the
Liviana and Giovanni's Journey Page on Facebook. I am very happy to have my friend Kelly going with me to help with Adelina while I am in testing with Giovanni. I hope we can see some sights so she doesn't think Italy isn't all like the area around the hospital.
Thank you for the continued prayers and love for our kiddos.
Hug Your Babies!
Amy
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