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I was born in Sep 20th 2007. It is the golden-pig year in Chinese, meaning a child born in that year would be healthy and happy. Mom and Dad planned well for me to be born before November because Nov is the end of school year enrollment. Mom and Dad love classic name, they decide to call me Emily Victoria Chao. They wished me to be a real lady, elegant and beautiful. They also have a Chinese name for me. It is "雅馨"(YaXin), meaning elegant and fragrant. 
Mom and Dad were very surprised at my size when I was born , only 5 lb 9 oz in weight and 18.5" in height. Mom is 5'6" and Dad is 5"10'. They wondered how come I was such a small baby. The OB Dr. said the cord was thin and placenta was small, that might be the cause. Other than that, I looked pretty healthy, 10 toes, 10 fingers, and loud crying . Mom said she can absolutely differentiate my voice from other baby because my crying sounds sweeter. :)  
 I am living with Mom, Dad and grand parents happily in California, US. I grew very fast in first three months, from 25% in weight chart to 100%. Mom and dad was glad. They always made silly sound around me and try to make me laugh. I looked like normal baby except one thing, I am a bit floppy. Since I was their first kid, they lack enough knowledge and experience to tell if I am abnormal. They thought it is because I was born small,  it would take longer time for me to reach the milestones. About 4 months, I rolled from belly to back completely, which gave my parent a lot encouragement. Mom taped the moment and they were so glad for me. I can hold my head up to 90 degree on elbow for a few minutes when they put me on my belly. What they didn't know is that it was the one and only one time I rolled over completely by myself in my whole life. 
At 5 months old (Feb 2008) my parent mentioned the floppiness concern to my dear pediatrician. He said I should be fine, every baby has its own pace, but just in case, he order the 1st round test for the normal condition that caused floppiness. He also recommended a few specialists. My Dad believed I was a late boomer, but Mom felt something not right. She made appointment immediately. My Mom said it is so hard to make an appointment. it would take 3 or 4 months to make an appointment with the Dr. in CHOC. The app was scheduled on May. Since she was worried, Mom made another app with a private specialist. It was in March. The private neurologist checked me over and told my parent that I was just a bit overweight, nothing wrong. We were sent to home happily. I started eating first stage baby food and cereal, I like sweet potato very much. I also have the polyvisal everyday which was recommended by the pediatrician.  
(My half year birthday!)  At 7 and half months old (May, 2008), I still can not roll and sit by myself. I can sit with support though. Dad took me to see the CHOC neurologist and the second round test started, that include blood work for metabolic, muscle dystrophy, and SMA (Spinal Muscular Destrophy). When Mom heard the SMA, she was so scared. She googled internet a lot and didn't believe I was SMA. She was relived after the result come back normal. 
At 8 months old (June 2008), Mom contacted Regional Center for early intervention. They provide physical therapy for developmental delayed kids. After the evaluation, I was reported to have gross motor skill as 3 month-old baby. Then I was scheduled the physical therapy program. Mom took me to Foothill ranch for the PT two times per week. I actually loved the private PT, the therapist put MOM and me onto an huge trampoline, Mom hold me tightly and jump together. I was excited, giggled and laughed a lot when we jump. This was my favorite game. Mom said she would buy one trampoline for me since I loved it so much. It is my best time in my life.
 
As time went by, Mom took me to see different specialist. Pediatric, Genetics, and Neurologist. Some of them believed I was CP (cerebral Palsy), one of them believe I was muscle dystrophy, and some of them had no idea of what was going on with me. I have brain and full spine MRI, EEG, EMG , and lots blood works done. None of them showed abnormal. The EMG showed possible myotonic dystrophy, but it was controversial as another neurologist pointed out later.  (EEG test looks scary, but it is the most comfort test I has gone through. The nurse just glued a few electrode onto my head and that is.)
I didn't feel lots pain or uncomfort since I was sedated, but Mom was very sad when she watching me went through these tests. The MRI showed delayed mylination which conformed with my development. Dad and Mom kept searching for professional and very experience specialist because they wanted to find out the real cause. When we went out, people always said I looked sleepy or tired. Mom made appointment with specialists in UCLA and UCSD. Though Mom was so worried and anxious, the appointments need 3-4 months.  Mom joined hypotonia/cp/myopathy discussion group and learn a lot from these friends. She was told that the CP baby can make huge progress via PT and OT before age 2 because the brain is still forming. Mom worked very hard to do the PT every day for me. She wished me could correct the brain by these therapies. She has learned some English/Chinese kids songs and Lyrics because I love musics. I will stop cry and start to smile if she sing my favorite songs. 
Here came my 1st birthday, September again! Wow, what an exciting event. Mom bought shining Tierra, beautiful dress, and a huge birthday cake for me. It is mango mouse cake which is my favorite. She also took me and Dad to JCPenny for portrait. She want me to be the most happy and beautiful girl in the world. Since my head is large, I didn't like the Tierra much. I made faces to Mom when she put it on my head, Mom LOL. It was so funny. 
After my birthday, I started another round Doctor visit. Both neurologist in San Diego and UCLA said I was Moderate Cerebral Palsy, I probably would not 'recover' but should be making progress. I felt Mom and Dad loved me more after that. I don't understand these terms. I can only tell how much Mom and Dad love me. They love me so much that I know no matter what I would be, they would take very good care of me and accompany me, fulfill my life with happiness and wonderful memories. 
On Monday, Oct 13th 2008, I was almost 13 months, Mom and Dad took me to see Dr. Marylyn Jones in San Diego. She is a nice, very knowledge and experienced genetics, she has helped a lots kids to find the real cause of their problem. After she checked me out, she didn't say anything conclusive. She recommended us to see a Ophthalmology specialist and checked out the fundus. I believe she had something in her mind but she was not sure so she didn't tell my parent.  Mom and Dad just thought my vision might not be good, but it is not big deal, we have lasik (We find out later this Ophthalmology visit is such a big deal that can turn our world up side down). Beside that, she ordered three tests, micro array which is requested by Mom, lysosomal storage enzyme panel, and CDG. Dad believed I was CP, and he searched hard for CP treatment, include herb and acupuncture. Mom still had question.  She said she would agree I was CP if anyone can explain why I could had very good tummy time when I was 4 months old but could not do it any more. While Mom and Dad are trying hard to find out the rootcasue and treatment, Grand parents are taking very good care of me. Grandpa put me into stroller and go to park twice a day. The PT said I have a good team, Mom is my commander who is in charge of the doctor visits and physical therapy; Grandma is my nanny who is responsibel for the feeding and sleeping; Dad is the entertainer who play music for me; Grandpa is my grandma's assisstant to take care other misc things. I am so happy living with them. Early November, Mom took me to see the Ophthalmology Doctor. On Nov 14th, she got the note from him: "bilateral 'cherry-red spot' involving entire macular areas. Impression: possible glycogen-storage disease ? Tay-Sachs disease or other metabolic condition". It is first time Mom heard the word. She googled Tay-sachs and scared to death. But she still had hope since Tay-sachs is prevalence in Jewish people. We are Chinese, so maybe it is not Tay-sachs. Dad found a disease called Sandhoff which also caused cherry-read spot. The disease is more rare, progress faster, and is found in all populations. The founding sinks our heart. 
Mom called Dr. Marilyn Jones immediately and asked if Tay-Sachs was included in the tests, the answer was yes. Both Tay-sachs and Sandhoff were included in the lysosomal enzyme panel test. Next a few days, Mom googled sandhoff presentation and found I am fit in. I have most of them, easily startled, not much eye traction, floppiness, and regression. I can see her red eye although she didn't cry in front of me. On Nov 18th, Dr. Jones called, after she said she was Dr. Jones, Mom knew the worst fear was come true. The world crushed. Dr. Jones said I was Sandhoff and she was very sorry. She asked if we could go to her office so that she can talk with us face to face on this rare matter. On Nov 20th, I was 14 months old, Mom and Dad took me to San Diego to talk with Dr. Jones. After we arrived the hospital, I vomited hard. All food I ate that morning went out, the clothes are all wet.  I thought it might be because of long distance driving, or I was not feel well that day. But Mom was very sad, she couldn't hold her tears. Dr. Jones explained the disease and prognosis which is not much different from what Mom and Dad knew. The most concern Mom had was if I would suffer. Dr. Jones said no, I would not suffer. She said she was very sorry but there was nothing she could do. She suggested hospice service. It is a srvice for the patients who will not look for the treatment any more, the purpose is to make patient comfortable in home, such as oxygen supplier, monitors and etc. Mom rejected the proposal immediately, she was not ready to accept the crucial fact. She can not believe that I will die before age 3 while I am only 14 months old. She still has hope for the possible treatment. She thought I was stable and healthy at that moment. 
While Mom and Dad worked very hard to find a treatment for me, they decided to take me to explore the world as much as possible. On Dec 7th, we went to disneyland. I think I like it, but it is very noisy because I am easily startled by sound. The christmas parade is great. I like the snowman, they were just as cute as me. 
I was tired after the parade, Mom took me to Minnie for a photo. Minnie likes me a lot, and tried to figure out why I was so sleepy. She made a lots poses and expressions to make me laugh, I really like her. I know I am floppy, you can see how hard it is for Mom to hold me. There are few experimental treatment for the disease although officially there is no cure no treatment. Bone marrow transplant, cord blood stem cell, and Zavesca medication. The Doctor in Duke University who is in charge of bone marrow and cord blood transplant said it would not benefit me since I still can not sit, meaning my brain is already damaged much by the GM2. The transplant is very risky with high dose chemotherapy and a chance of death of 20%. The only substrate therapy medication is Zavesca which is FDA approved drug for Gauche disease, another lysosomal storage disease. Dr. Tifft, Division Chief of Genetics & Metabolism Center for Neuroscience and Behavioral Medicine had a clinical trial for using Zavesca to treat Sandhoff kids. She said "I only have one Sandhoff patient whose family felt that it stabilized the child's disease, although I have not seen that patient back in many months". Mom and Dad decide to give it a try. on Dec 10th, 2008, I started taking Zavesca. The Doctors warned my parents that the drug is very bitter, it would be very difficulty to give it to me, Grandpa tasted it first and said wow it is very very bitter. Mom carefully put the white crystal in the middle of two layer of fruit puree with honey, make it like a tiny sandwich, and gave it to me. Although it is very very bitter, I swallow it down. Mom is very glad. I took Zavesca on Dec 10th and Dec 11th without problem. In the afternoon of Dec 11th, I had diarrhea which is expected side effect of Zavesca. At night, Mom feed me 2ml of Imodium which is used to control the diarrhea.  On Dec 12th early morning, I stayed with grandma. She noticed my lips was blue, I was unconsciousness, drooling and pale. The heart rate was 180-200. Parents and grandparents were all scared. Mom hold me tightly and didn't know what was going on. I kept drooling, half-unconsciousness, and fast heart rate for one hour. Mom called the nurse in CHOC, and asked if we should go to ER.   After the nurse checked with the on-call doctor, she said yes. At that moment, Mom noticed that my lips started twitching. It is my first seizure. Although they know seizure was expected as the disease progress, they didn't expected I would had one at this early age. Mom, Dad and Grandpa immediately rushed to the car and sent me to CHOC ER. At ER, they did a regular blood work and CT scan, they found I had no virus, no fever, and no bleeding in the brain. The CT scan showed my brain stem was atrophy. The seizure start from left lips, then left arm, then left leg. My head was jerking too.  At 10:00AM, I was injected with Phenobarbital. After a few minutes, the seizure stopped. I felt asleep, very tired and quite. I was transfered to CHOC and stay there overnight. Mom and Dad stayed there and accompany me. The hospital doctor consulted with the metabolic specialist and the neurologist. Both of them said the seizure was due to the progression of the disease, not the side effect of Zavesca. Mom and Dad decide to dis-continue the Zavesca temporarily to confirm it is not the drug. After I was discharged, I was very weak. It is difficult for me to suck the bottle and eat from a spoon, which made Mom very sad. Mom called hospice to send the oxygen supplier and suction machine just in case. I got better after one day. 
I can drink formula from bottle and eat my favorite yobaby yogurt. But in deep, something have been changed. I can feel I am not as alert as before, I am wondering in my world more, giggling less, and much inactive. My whole body is floppier, I can not raise my arm to bat my most favorite toys. I can recognize Mom and Dad's voice and hugs. It is difficulty for me to control the head. As usual, I love to be cuddled, and I am always be cuddled. On Dec 18th afternoon, I got my 2nd seizure. It is 5:35PM. After three times lip twitches, Mom gave me DisStat. It is very helpful, immediately, I am released and fall a sleep. That night, Mom consult with neurologist, and decided to put me on seizure control medication witch is Keppra. I started to taking Keppra 0.5cc twice a day. Mom and Dad was hoping I can be comfortable and seizure free with this drug. But unfortunately, it is not a magic bullet, I started another cluster seizure on Dec 22nd 8:20AM. My lips are twitching, and my hands are shaking. After consulted with nurologist's assistant, Mom gave me another Diastat. The oxygen supplier and suction machine are helpful when I was short of breath and have difficulty to swallow the saliva. 12/30/2008: The seizure seems very hard to control, It came back again. On Dec 24th, 27th, and 28th, I had these cluster seizure every 8-10 minutes, my lips were twitching hard, and my hands were shaking hard.  Occasionally, my head were jerking too, up and down, or left and right. I was half-conscious when I was having seizure, but conscious between them. I can smile to Mom and drink formula between the seizure. On Dec 27th, after I had more than half day fight, Mom decide give me Diastat on 4:07PM. But this time, the medication only last 4 hours. At 8:00PM, the seizure came back. Since we were run out Diastat and the pharmacy was closed, Mom immediately called Hospice and asked for the drug. Fortunately, hospice informed us that CVS has the DiaStat, so Dad went to CVS and picked it up. Now, we have emergency drug, and I was under control. Mom discussed with the neurologist, and decided to increase the Keppra dosage to 2cc twice a day. Beside the seizure, I am all right. Dad take me to sun bath everyday. I like it very much and feel very comfortable. Mom said I smelled like a sun. 1/11/2009: I am deteriorating rapidly, and I had a very bad New Year holiday. Starting from the Dec 31 2008, I had consistent seizure. I twitched my lips every a few minutes, it is almost non-stopping. I couldn't drink formula because of the seizure. Occasionally, I got one or two hours rest without seizure and I fell deep sleep. I was with oxygen tube 24 hours a day. Around 6:40PM on Jan 1st, Mom noticed my lips turned blue, Immediately I was given a DiaStat at 6:48PM. My heart rate was above 160 and my respiration rate was above 60 consistently. Both my arm were extremely stiff and locked. I was unconsciousness and can not swallow my saliva. Mom called Vitas Hospice and got instruction to give me another Diastat. The nurse came in around 8:00pm. She said my lung had water. She used the suction machine to clean my upper respiration system a bit, which made me felt better. She said that such high heart rate and respiration rate was the beginning of downhill. Usually, the patient with this condition would not last 72 hours. I couldn't believe what I heard. I didn't agree with her, neither did Mom. After more than 3 hours with high heart and respiration rate, I relaxed and fell asleep. Thanks double DiaStat, I had almost no seizure that night. Mom had to keep sucking out my saliva to prevent me from choking because I can not swallow. On Jan 2nd, my nurse, Virginia, came in the early morning. She is a very nice lady. She stayed with us almost whole day. Although Virginia said I should not feel hungry or thirsty, Mom wanted to get nutrition and hydration for me ASAP since I hadn't eat for almost three days.  They discussed the feeding tube. It is either a Nasal Feeding tube or a G-Tube via a surgery. NG tube sounds disgusting and G-tube sound painful. I like neither. The neurologist said that the earliest time for me to get a G-tube done was Jan 5th because it was a long holiday. Furthermore, because of the water in my lung, I might not be able to get a G-tube surgery before the infection is gone. Mom decided to get a NG tube for me temporarily and then discuss what to do next. She called CHOC but Choc said they were over swamped. The nurse suggested to go to CHOC in early morning next day since the night was the busiest time. I didn't want a feeding tube, so I tried very hard to drink a few ounces juice and pedialite in the night.  Mom had to use the suction machine to suck out the fluid that went to my air way every a few swallows. Mom and Dad felt a bit relif and not that extremely desperate after I drink. On Jan 3rd, I had blue and purple bruises around both of the eyes, my face was swollen. Dad said I looked like been punched hard. I had very weak suction and can almost swallow nothing. It is very tricky to get formula into my mouth. It took me 2 hours to drink 4 ounces milk. After one week intensive care, I was stabilized a bit. I can swallow better and better, and finally can drink 6 ounces formula thicken with cereal without coughing. Unfortunately, neither I nor the drug can control the seizure. It comes back every a few minutes consistently for a few hours, then give me a break for a few hours. 
1/25/2009: My lip is twitching hard all the time because of the seizure. Meanwhile, I got diarrhea. Mom suspected it is because of Phenobarbital. After discussed with Doctor, she changed my medication back to Klonopin on Jan 17. I am doing pretty good with this drug for three days and another four days after the dosage increased to 1.5mg. The seizure is better controlled so I can drink well. I am going down hill rapidly after first seizure on Dec 12th, 2008; At this stage I almost lost my head control. It is very difficulty to hold me, or change my position. Occasionally I can flap my left hand, cooing, and grin; most of time, I am very quite and wondering in my little world. I have very cute little hands and feet. My hands are samll becase I dont' use them lots, my baby feet are small because they do not bear weight. I have very fine skin, so I get lots kisses everyday. My major food is formula, pedialite, first stage baby food and pediasure. I can not eat from spoon, so Grandpa blends everything into a bottle. All food are thicken with cereal to decrease the chance of coughing. My head is growing larger, the nosebrige is getting thicker, and my face is a bit bulky. I think it is because of the GM2 deposit. But even with these changes, I am still beautiful. I don't think I have good vision, but my hearing is very good. I can hear Mom's whisper and music clearly, and am easily startled by sound. Most important, I can feel more love from Mom, Dad, and grandparents. We got a pulse-oximeter from hospice which is very nice. It alarms adults that my oxygen saturation is low, or heart rate is high. In last a few days, they have to wake up several times during the night. My heart rates went up around 170 consistantly. Parents and grandparents are taking shift to take care me. Today is Chinese New Years' Eve, I am stable. Happy 牛(Niu, ox) Year! 1/29/2009: In the morning of Jan 27, I had consistent seizure again after I finished 8oz food. The heart rate is 160-180, 170 at most of time. The doctor instructed us to increase the Klonopin to 2mg per day. But it didn't help. After 24 hours, I still had non-stopping seizure and high heart rate, and can not sleep well. Mom called Hospice several time and try to get a solution. In the afternoon of Jan28, the hospice Dr. came for a home visit, and decide to increase the dosage to 2.5mg. The reason they were very hesitated to increase the dosage is that I am a baby. Too much sedation would be very dangerous. I am very quite all the time, hardly move or make sound. But at 4pm, I shouted loudly. Mom noticed my heart rate was 206 and immediately gave me a DiaStat. I was calmed down after that but heart rate still around 170 for a few hours. This morning, it finally droped to 120. Today is a nice day. Mr. Chris, the in-home PT, came in and helped me stretch a bit. I feel comfortable and fell deep sleep. Mr. Chris said I have a ET light on my toe. 
2/1/2009: At 4:30AM on Jan 30, my pulse went up to 200 again. It was between 190-203 for almost one hour. Even DiaStat can not bring it down. Hospice sent an on-call nurse who didn't know I am a baby. I had low fever, the temporature was 101F degree. My little body was fighting hard. Parents didn't know how they could help but pray. Mom put cold towel onto my head. Finally, the pulse went down to normal at 7:00AM. Since most patients of Hospice are elder people, Mom is asked if she is my daughter almost everytime she called. The 2.5mg Klonopin works good. I am comfortable except some mild seizures. I can even grin, that make Mom and Dad heart melt. Thanks Jo LABEDZKI who is from New Zealand, she posted my website to CaringBrige (www.caringbridge.org/visit/brookeforever), so I have a lots friends now. I am very glad to hear the hellos from all over the world. Thank you for your caring and prayers! I only hope that more and more people are awaring of this dreadful disease, so that less and less family will be affected. (Beautiful Sundy afternoon, swing with Mom in backyard.) 2/8/2009: I am kind of stable in this week except some mild seizures, Mom can sleep better too. I have weaker suction, so I can not seal the nipple completely and there always are some noise when I drink. It takes me longer time to finish a bottle. On Friday, I had consistent seizure, Mom gave me DiaStat, it really helped. I was happy and feel comfortable during that night. I grinned a few times. I even giggled. Mom is not sure if I am giggling or having laugh seizure. This condition lasted over night. I still can grin on Saturday morning. I guess that the DiaStat somewhat suppress the disease, so temporarily I am myself again. Here is copy from http://sandhoffdisease.webs.com, it describe what exactly the laughing seizure is: "During a laughing seizure you will notice the child all of the sudden giggling or laughing out loud, this is one of most parents favorite kind. That may sound crazy but for children that have stopped making noise and lost the ability to smile or laugh, to see if just for a minute the child giggle, is just somehow peaceful". Talking about laughing seizure, I may have it when I was very young. I remembered that I laughed loudly for a few minutes when I was 7 months old. The giggle came from no where and sometimes I had frightened look. At that time, parents didn't know it was seizure, they just thought I was happy and active at that particular moment. Even Ped said it's a good sign since I didn't make much noise. Online friends told Mom that Tompmax can be used to control seizure, so Mom is checking with doctors and see if it is safe to have that drug. 
2/16/2009: I am about same this week, sleep a lots. It is might because of the anti-seizure drug, or I just want to sleep. The Klonopin dosage is increased to 3mg. Everyday, there are some mild seizures. When I have no noticeable seizure, everyone has a good day. When I have consistent seizure, everyone is plagued. Yesterday and last night, I had more seizure than usual, but I went through it. I can drink food pretty good when I has no seizure, I coughs but we can handle it. Sometimes, I have very shallow and labored breathes. Today, I am good and grinned once. Though it is raining, the grin brought sunshine into whole house. (my grin.) I was pretty serious on every things when I was 'young', eating, playing even poo-pooing. Previously, when I was poo-pooing, my face turned very red and I was using whole body strength. If I felt somebody watching me, I relax and the face color back to normal. Then I looked at you with an innocent and curious expression "what is happening?" :) 2/20/2009: I still sleep lots and almost seizure free, Mom feed me well.  When I am sleeping, the pulse is between 90 to 110, when I am awake, it is below 130, when I am drinking, it is up to 150. Grandparents help me stretching my arm and legs everyday. Thanks God, life is kind of peaceful now. 2/22/2009: I cough more and more when I drink food, sometimes the food come out my nose with breathes. Because of my weak cough, Mom need to use suction machine to clean my airway a few times every feed. Other than that, I am pretty comfortable. My heart rate is slower than before, sometimes it drops below 80 when I am in deep sleep. 3/4/2009: I am alright these days, sleep alots and almost no seizure. When I am sleep, I looks like an angel, very peaceful and comfy. I eat all kinds of food: meats, fish, shrimp, veggies, and fruits. Everything is pureed well. We get support from friends, which is what we need most. Although I can not express myself and move my little body, parents love and care me much. They can understand my feeling. To prevent chocking, I always lay on my side. Parents and Grandpartens help me stretching my body everyday for the tightness and lockup. Sometimes, I can sleep even when they stretch me. I know I would have a condensed life, but the love and caring I would get in my life is not less than what other people get. 3/10/2009:  I got fever two days ago, the temperature went up and down. The highest number measured is 103.6F. I have no appetite and am lethargic. When body heat goes up, the pulse goes up too (160-180). That caused the pulse-oxi-meter alarming consistently. Luckily, the alarm is trigger by fever not seizure. Yesterday, I had 10oz food for breakfast and lunch, I didn't eat well at night. At 6:30PM, when Mom tried to clean my air way,  the suction machine triggered a gag, so I vomit lunch out. Mom felt guilty for my vomiting. But actually I felt better after that. The food is a burden for me since it can not be digested well. Anyway, the temperature went down this morning after 2.5ml Motrin, and I am much better now. I had some formula, pedilite and sweet potato for breakfast and then go back to sleep. 3/12/2009: The fever is kind of stubbon, and the temperature goes high and low. It is low during morning, and high after noon. Last night, it went up and kept high until 6:00 AM this morning. Hopefully, it can stay low in the rest of day. I havn't eat today since I am very tired and in deep sleep. Everytime before the temperature goes high, I am shivering and feel extremly cold. My hands and feet are very cold, and my lips are bit purple. I am wrapped with lots blankets to keep me warm. After the temprature goes high, my face is very red, and whole body is hot. Then I am only covered with a thin blanket to keep me cool. 3/17/2009 On March 14th, the fever was gone. We were kind of released. In the daytime of March 15th, I was good too, no fever and high heart rate. Unfortunately, I started coughing in that night and the fever came back. I coughed very hard, I felt I almost coughed my lung out. It is very difficulty to give me food or medicine. I am floppier and weaker than a new born baby. Mom tried hard to feed me, but I just lost appetite and strength. It would take more than one hour to finish 2 or 3 ounces food. Beside, Mom need using suction machine frequently. Sometime she felt that the food I took in is less than the food she sucked out. She also used syringe to inject food into my mouth slowly, and hoped I can get more nutrition. I was with 24-hour oxygen for almost two days, it bring the saturation to 95-98, but hardly reach 100. Without it, my oxygen saturation drops to 85. Most of time, the heart rate is above 160. Mom called Hospice, the nurse said she would come in shortly. I guess I have pneumonia again. 3/23/2009 I am declining and not doing good. Last a few days, my temperature was up/down/up/down. It came back to normal yesterday. Hopefully, it will not go up again. Mom used antibiotics to help me fight the pneumonia. The lung sounds clearer now, but I still cough. I don't have much strength so my little body fight very hard when I cough. Suction machine is used extensively to clean the mucus in the mouth and nose. Beside, I don't has desire to eat. I drink 1-4 ounces formula per feeding. Today, it take me more than one hour to have one ounce formula. Mom kept putting bottle into my mouth, I just do not swallow. Parents reduced anti-seizure drug dosage a bit and hoped I can eat more because of less sedation. But the seizure came back, we have to restore the dosage. Occasionally, I stop breath for a few second. When I stop breath, the oxygen saturation dropped to 60. 3/27/2009:  With antibiotic and extensive care, I am almost recovered from Pneumonia. I am a true baby fighter, and I win the battle again. The fever is gone. I cough less and less. I still need 24 hour oxygen, without it, my saturation drops to mid 80. Occasionally, I cough very hard for a few minutes for no reason.  We are trying to figure out why I cough so badly and haven't got any answer yet. we also understand that the stop breathing is one type of seizure. It disappears if the correct medicine is used. My appetite is getting better and better, today, I had 7 ounces food for lunch. Though I still need suction machine to help me clean the air way but I CAN swallow. That really make parents reliefed. A few days ago, I just didn't have desire to swallow anything. " I know us grieving parents often use the roller-coaster ride analogy to describe the extreme emotions of having a terminally ill child, but there’s an element of excitement or enjoyment in that description that doesn’t fit. I think it’s more accurately a ride to hell and back over and over again. And then after so many trips, you’re finally able to claw your way out of the deepest, darkest pit of your life and stay on solid ground. Once I was out of that pit and able to feel the sun on my skin again, day after day, I realized that I’d climbed my way into a different world. Nothing would ever be the same again. My heart had been changed, and that changed everything.
... I remember shortly after Amelia was diagnosed, one of Maddie’s preschool teachers said to me “I don’t understand how God could let this happen.” That was the first time the question occurred to me – did Amelia’s disease have anything to do with God? At the time I didn’t know. I had a hard time believing that it did. A year later, there’s only one explanation that makes sense to me – this has everything to do with God. From a non-spiritual perspective, my child getting Tay-Sachs disease and dying is inconceivable. There is nothing okay about a baby losing all of her physical and mental abilities to the point where she can’t even swallow. There’s nothing fair about it. There’s nothing right about it. Children are not supposed to die. There’s no way to make sense of it. And I’m one of the most logical people that I know. I got my bachelors degree in math – everything is supposed to add up somehow. I like to put hundreds of numbers in tiny boxes on a spreadsheet and make them all reconcile. Just like for the kindergarteners, explaining this by genetics doesn’t do it for me. This whole situation just doesn’t add up, unless I look at it from a spiritual point of view. ... " 4/3/2009: I am kind of stable. Average feeding time is 90 minutes. Mom bought premie nipple for me. It is thinner and softer than the one I used. I like it very much. Mom made the nipple hole bigger, so I can get the food out the bottle easier. But still, I am easily get chocked when I drink. Suction machine is used frequently during the feeding. Another good thing is that I am off oxygen supply two days ago. My santuration is around 94. The noise from the oxygen generator is pretty loud, so my home is quite now. 4/12/2009: I vomited a few times during last week. Sometimes it happened after a feeding, sometime it happened after a bad cough. We are suspecting that my stomach could not hold much food. The good news is that without oxygen, my saturation can reach 98. 4/15/2009: I was kind of rough last night. For no reason, I got fever again. Around 11:45PM, I was shivering, and my hand and feet were cold. The temperature was 102 degree, pulse was above 160, and the oxygen saturation was low. Mom fed me Tylenol, and gave me oxygen. Gradually, the temperature dropped to 98 degree after 1 hour. Today, after 1:00pm, the temperature went up again. Virginia, the nurse, came in around 6:00pm, she said there is some sound in my lung, meaning the fever is caused by the aspiration. Mom chatted with her and found out the existence of Tylenol suppository. That is very helpful. When I am in bad situation (fever or seizure), It is very risky to put anything into my mouth. It might cause aspiration since I can not swallow anything, even Tylenol drops. Mom requested the G-tube placement last week. The PCP was out of town, so we are waiting for his coming back. Previously, Mom didn't want the feeding tube on me. First, she didn't want me to suffer the surgery. Second, she didn't want me to lose the ability of tastes. She always hoped that I can live without feeding tube. But now, feeding become more difficulty and risky than ever. Sometimes, it is unpleasant and unsafe. If I had G-tube, I would have less chance of aspiration. 4/18/2009: Mom discussed the G-tube placement with PCP and requested to have it done without GI doctor visit. The surgery was scheduled in mid or late next week. I didn't have fever for two days, but today afternoon, it came back again. We dont' know the reson since the lung sounds clearer. ---------------------------------- I think the thing that is so hard about Tay-Sachs disease is that it is progressive. You don’t get a diagnosis, go through the grieving process, get to a place where you can cope and then get on with life. Because it always changes (for the worse.) And each new disease progression has to be grieved. It wasn’t that hard to accept Amelia being “special,” because she’s still such a beautiful child – a blessing and a joy just like any other child. And I feel like I’ve done that. I’m not saying that I like it or that it has gotten any less painful, but I feel like I’m at peace with who Amelia is and the life she’s been given. What I have not come to terms with is the death sentence she’s been given. Most of the time I get to put Amelia’s inevitable fate out of my mind. That is until she gets sick. ... Then I got equipment and medications that greatly improved Amelia’s respiratory function. So it’s only when she’s sick that her breathing becomes labored. But because of her disease progression, her breathing is much more labored, her oxygen drops and her heart-rate skyrockets. So when she’d get sick, my fear had a new friend – Panic! I remember the first time Amelia’s oxygen monitor said 85. I thought it must be a mistake. I was trembling with fear, my heart was pounding, I could barely breath. I didn’t know what to do. I couldn’t think straight. ... After Amelia’s RSV/pneumonia and stay in the hospital in February, I realized two things. One, when she actually did come close to death, I wasn’t terrified like I had been in the past. And two, Amelia gets better care at home than she did in the hospital. I have all of the same equipment and medications at home, and she gets one-on-one loving care at home. ... The doctors were looking to me to assess Amelia, since they don’t see many patients like her. The day that I thought she turned a corner, I told them I was basing this on her hiccupping, having seizures again, and arching her back that day. She hadn’t done any of those things in a week. How on earth could anyone else know that except for me? So even though she was at the hospital, I was still calling the shots, doing the care, and assessing her. Which led me to the next stage of this process. Now I know that no doctor, nurse, respiratory therapist, or hospital has anything more to offer Amelia than I do here at home. Which means there’s nothing more to do for her. Which means that when Amelia’s oxygen is 85 and her heart-rate is 195 and her lips are blue, I can do all of her respiratory care to try and help her breath better, put her on oxygen, start her on antibiotics or an oral steroid, and give her medicine to make her more comfortable. After that, all I can do is hold her. There is absolutely nothing peaceful about that feeling of being stuck, being helpless - paralyzed. ... All I can do is love her and hope she gets better. Now that I’ve seen first-hand and know for sure that I’m giving Amelia the best care possible, the fear and the panic have subsided. I still feel helpless, but not the crawling out of my skin, claustrophobic kind of helpless that I used to feel. I’ve found over and over again since Amelia’s diagnosis that once I work through the fear, anger, anxiety, and all of the funky unhealthy emotions, what’s always left is deep sadness. Anytime that Amelia gets sick and has a hard time breathing, the possibility that this will be the sickness she doesn’t survive makes me so sad. It’s a sadness that at times literally knocks the breath out me and drops me to my knees. My heart is too heavy to get back up, so I lie on the ground and weep. ----------------------------------  4/22/2009: The G-tube placement is scheduled at tomorrow, April 23rd. We are requested to go to hospital in early morning at 7:00AM, then have the surgery around 2:00PM. We should be discharged in Friday if everything goes fine. At this moment, my PCP is communicate with CHOC to get the hospital admission. 4/26/2009: We were admitted into CHOC at 9:00AM on Thursday, Aprial 23rd. At 2:30PM the G-tube was placed. It went smoothly without complication. Parents were not allowed to enter the operation room. The whole procedure took about half an hour. One doctor performed anaesthesia, one doctor put the light tube into my stomach, then the GI doctor perform the cut, and connect the stomach to the outside tube. It was not that scary as we thought. I was on IV on Thursday and Friday. In Thursday night, I had fever due to possible infection. Nurse gave me tylenal, and the fever was gone in early morning. At 4:00AM in Friday morning, I was fed at the first time by G-tube. I was given 90cc pedialite in one hour. The fluid was pumped into G-tube via a pump slowly. Then at 7:00AM and 11:00AM, I was given 240cc pediasure in one hour. I tolerate the feedings good. Mom afraid that I was in pain although I wasn't, she requested tylenol plus codeine to make me comfortable. The GI doctor came to checked me out in Friday afternoon, she said I was ok to go home. At 2:30PM, we were discharged. It was sad to have to go through this, but we feel we made a right decision to have the tube placed. We are in home for 2 days now. There is no more worry about the choking, cough, and aspiration during the feeding. I just lay on the bed and I am full! Dad said I seems gain weight already.  5/5/2009: Hospice delivered the enteral pump and Pedisure to us. Before the delivery, Mom use the big sringe to put the food into the G-tube. It is a little difficulty to control the feeding speed. I vomited once becasue of fast feeding. With pump, feeding is much easier. The Pedisure is poured into the bag, then the pump slowly pumps the food into G-tube. the speed is 240 ml/hour. Mom use pacifier to satisfy my sense of fullness. Furthermore, she dip the pacifier with honey or milk to give me sweet taste. I like it very much. 5/10/2009: The wound in my stomach is almost healed. The yellowish secretion is less and less. Mom keep changing the sponge pad with polysporin powder. It protects the wound, and helps healing it. I had a few very brief seizure in last 36 hours. When I had seizure, I stretched my arm and leg straight and outward, and my neck backward. My oxygen santuration dropped to 60 suddenly and back to normal within one or two minutes. Mom guess that we might need to increase the anti-seizure drug dosage since I grow bigger. 5/17/2009: I had fever this morning. During the breakfast time, My face is not rosy and fresh, it is a bit dark. Mom checked me out, and found my temperature was 100.4 and pulse was 150, my hand and feet are very cold. Immediately, Mom stopped the feeding and gave me Tylenol via G-tube. A few minutes later, I vomit all feeding out. The temperature and pulse keep going higher until 103 degree and 204/minute. Mom hold my little soft hand and wish my pulse can go back to normal. It stayed aroud 200 for almost an hour, then went down slowly, 190, 180, 170... After 3 hours, both the temperature and pulse go back to normal. Mom guess that I might caught cold after the bath last night. We don't understand why a common cold can cause such fast heart beat. I have no appetite for rich food, so Mom gave me some diluted apple juice for lunch, and four ounces Pedicure for dinner.  It seems I can easily catch cold after bath although Mom keep me warm all the time. She even heat the air before she put me into my infant bath tub. Mom and Dad need to cooperate very well to give me a bath. One need to hold my heavy head steady, while the other make me clean. I can feel their love in every minute I with them, every cuddle, every touch, and every wisper. I am always their precious and beautiful little baby girl. I have weaker cough than before. It is difficulty for me to cough out the mucus. Suction machine is used to help me clean the airway. I lay on my sides almost all time to prevent the chocking from my saliva. 5/26/2009: I am about same last week, sleep alot peaceful. 5/27/2009: At 4:00AM this morning, I have labored breath and my oxygen santuration drops to 80 for no obvious reason. After a few minutes, there is no sign I can recover myself, oxygen was provided. My heart rate went up 160 without fever after breakfast. No one knows what is going on, Grandpa noticed my G-tube is full of food, so he dained some food out of stomach. It came out good. My pulse back to mormal slowly after that. My head swell larger compare to a few monthes ago, the circumference is off chart with an unbelievable number. My cutist expression is yawn and sneeze, that is the best movement and noise I can make. Occasionly, I can recognize Mom and Dad's voice, and respond them with a very slight lip twitch or very weak coocoo. Such moment can make us very happy. 6/14/2009: Nothing has been changed significant, but I am getting floppier everyday with tiny things: Lower fever in short period, brief seizure, labered breath, and weak cough. Mom said I am a sweet and happy angel. I come to earth for a short visit, then go back to heaven when time comes. My life is short, but it full filled with care and happiness. More important, it makes people appreciate life and health more. 6/23/2009: 21 months old Sleeping Beauty... 6/30/2009: Emily is very sick today. Around 6:00AM, her breath is short and shallow. Her oxygen saturation dropped to 65 even with oxygen supply, and gradually come back to 90 after half an hour. After breakfast, she vomit 20-30cc fluid out. Then she had 2nd attempt to vomit around 10:30am with obvious upper body movements. Her hands are very cold and around 12:00, she had fever. Her temperature is 101 and heart rate, 155-165. Grandpa gave her 1.6 ml Tylenol at 12:30.
we are doing our best to make Emily comfortable, and hope she can survive as before. It makes us very sad knowing the possibility that this might be the sickness she couldn't survive. We take best care of her and pray that she can stay with us as long as possible. Emily is such a beautiful and happy baby but she will never get better. She is losing her swallow ability slowly, so if she is awake, the suction machine is used extnesively to get her saliva out of the air way.  7/5/2009:
Emily is stabilized. But she relies on oxygen more than before.. 7/10/2009: Emily's respiration system seems getting worse. For last a few days, we need put her on oxygen almost all the time. The oximeter alarms the saturation level every a few hours. with oxygen supply, the level drops to mid 70s and stay at that range more than half an hour. When it happens, Emily has very labored and fast breath. You can tell she is breathing hard to get more air. I don't know what I can do, but hold her soft hand and try to calm her down. 7/13/2009: Emily is off oxygen supply yesterday which is good. She is calm and sleep peacefully. I can tell she still know things. If you talk to her, her face would be relaxed. She bite her teeth tightly sometimes and don't want the suction machine get into her mouth. I guess she just does not like it. 8/6/2009: Because Emily can not cope with her regular saliva, we use suction machine 24 hours to get her saliva out of her airway.  Even with suction, her oxygen level goes down often. I have heard from other parent to use medicine to control the saliva secretion. I didn't know if the drug make any difference, but we have to give it a try since the situation is getting worse. After consulted with Jo and doctors, we decide to put Emily on Robinul to control her saliva a few days ago. It comes out pretty good. After we used the drug, we still use suction machine but less frequent. Emily looks better too. She hasn't use the oxygen supply for 3 days and the oxygen level is almost 100. Emily is a happy baby, it is her nature. If she knows you are talking to her, she will respond with a Emily style smile: right side lip goes up a bit. it is not a real smile, but it is still heart melting. 9/15/2009: Emily's birthday is coming. She is almost two years old. We are glad she is still here with us. There were very hard times in past 10 months, but we all make it through. Emily is a true fighter with our extensive love and care. I am very sad thinking of her birthday. Emily is complete silent for more than half year. She can not enjoy the birthday party like other kids, she can not eat her birthday cake, she can not enjoy her pretty dress, she can not go to theme parks, she can not jump around and ask for gifts. But I know she still know things, in Emily style. So we are going to have a Emily style birthday party. We will celebrate. We will celebrate her accompany in last two years, the happiness and wonderful memory she brought to us, her sweet and touching smile, her vivid and innocent expression when she laugh, giggle, crying, and cooing. We will celebrate. 9-20-2009, Emily, Happy Birthday!  
 10/26/2009: Yesterday, I cuddled Emily in backyard, for sunshine and gentle swing. I can tell she still love them. Her face is relaxed and tried to cooing. Before doing that, we used suction machine to get her saliva out completely. I don't dare to hold her upward for long time, so we went inside after about 15 minutes. Holding Emily is like holding a noodle, she doesn't has any strength at all. But last night she looked a bit alert than usual. I guess the natural breeze waked up some cells in her body. Emily is very pretty, she is growing big (32 lbs) and tall, but she still has baby size foot and hands. We avoid all chance that could make her saliva get into her lunge. We even avoid the bath. Sponge bathes are given but not full bath. We just want her to stay with us as long as possible. Seeing her beautiful face makes us happy everyday. It seems she tolerate PediaSure worse. Every time after the feeding, her heart rate goes up to 140-150. We decrease the amount per feeding, that makes her feel better. Mean while, I use syringe to inject the mixed baby food (meat, veggies, and cereal) via G-tube. She tolerate the baby food good. Maybe it is easy to digest. 11/04/2009: Sweet Amelia has a new update, I wish she can get stable soon. Here is a copy of her jounal (http://www.caringbridge.org/visit/ameliaalford/journal): "I was just thinking about a description of special parents called “The Strength of an Egg.” (I’ve decided to call myself a special parent since I have a special child.) The author compares us to an egg, seemingly strong as a rock on the outside, but under the right amount of pressure, easily shattered. After two years, I don’t know why it is such a surprise to me each time I’m reminded of how fragile I am. I can carry on for days, sometimes weeks, feeling strong and full of hope and faith. But it just takes one little thing to completely crush me. And each time it happens, I feel just as heartbroken and hopeless as the last time. And each time I’m taken back by it. I don’t why. It seems like I’d be used to it by now. Today it was standing at the pharmacy counter saying “I’m picking up my daughter’s Phenobarbital and Morphine.” Just something about saying those words out loud made me fall apart. Amelia’s hospice wants her to have an end-of-life kit at home (morphine, phenobarbital, and ativan), even though she’s not actively dying. The Strength of an Egg
by J. Freitag Parents of children with a terminal illness are often referred to or viewed as having strength “like a rock.” Albeit flattering, it isn’t quite true. It is more like the strength of an egg. An egg, you ask? Yes! If you’ll think about it, you’ll see my point. An egg has a polished, smooth outer appearance, with no cracks or weak spots visible. It seems almost inconceivable that the inside might not be so smooth or solid. Most children, at some point are shown the famous egg trick. An egg set at just the right angle can withstand enormous amounts of pressure and cannot be cracked or broken. Yet the same egg, tapped gently at an ever slightly different angle, will break. The contents, once so neatly concealed, will come spilling out. The no longer perfect shell will be crushed. It looks so fragile that it seems inconceivable that it ever held any strength. A rock, on the other hand, is solid all the way through. To break it is almost impossible. If you succeed, you will find that there is nothing inside but more rock. It takes a lot more than pure hardness to hold the hand of hope. Parents of [medically fragile] children are not solid all the way through. We hurt, we fear, we cry, we hope. It takes a very careful balancing act to keep the shell from being shattered. “Balancing an egg” while running a household, going for doctors’ visits and hospital stays, keeping the family together, and holding on to the constantly unraveling ties of your sanity can be very tricky indeed! Occasionally, the angle will be off and the shell will break, shattering hope and all the neatly secured appearances of a truly fragile existence. Unlike Humpty Dumpty, though, parents of medically fragile kids will pick themselves up and put themselves back together again." 11/15/09:  The Hospice nurse came in today for regular check. She visits Emily every two weeks for general chechup, medication refill, medical supplies, and monitor her condition. Today, she listened Emily's lung, and said it sound very clear. Thanks suction machine, Robinul and g-tube. Without them, Emily couldn't be with us for such long time.
12/11/09: Last Dec 12th, Emily had the first seizure and was sent to ER. After that, there was pneumonia and more than one month non-stopping seizure. I was extremely desperate watching her went thought these. The memory is so vivid that I can feel my heart is just as painful as one year ago. Since then, she went downhill rapidly and lost her ability one by one. In last a few months, Emily's seizure is controlled well under the Klonopin; But recently, we have seeing more obvious seizures than before. Since Emily has no strength, you will not notice it if you don't watch closely. Her eye blinks and her lips twitches. If you hold her hand, you can feel her hand shakes slightly too. We have no choice but increase the dosage of seizure control medication. We also increased the dosage of the soliva secretation drug. They work better but makes Emily sleep more. When Emily is sleeping, you can not tell she is different from others kids, she is just beautiful, peaceful, and adorable. We don't know how long Emily can be with us, we don't know what would happen tomorrow. We have learned living by days. Today, Emily is still with us, no obvious seizure; Today, all of us still talking, walking, working, watching, eating and laughing. I will say it is a great day. 1/4/10: Little peasant lady. :-) Emily is under extensive care for a few monthes. We flip her over every 3 hours (from left to right, or from right to left), feed her every 4 hours, give her medication every 8 or 12 hours, and get her saliva out frequently. While I am not in home, Grandpa and Grandma are taking care of Emily as full time 'nurses'. With their tremendous help, Emily's lungs stay clear. Her oxygen saturation has been almost 100 since we put her on the saliva secretion control drug. Even with these cares, she still get some bad days: fever, seizure, and vomiting. I know in the last stage of some kids' life, their lung collapse or they can not digest anything. All the feeding get into the stomach will come out of the mouth eventually. It needs extreme strength to imagine the situation and speak it out. I am still lacking of it. I am fooling myself that as long as Emily is doing OK today, I don't need to think about what will happen tomorrow. Grandpa made a peasant hat for Emily. She is so cute with it. 2/12/2010: Emily is doing good recently. Her lung is clear, and oxygen level is almost 100. she hasn't vomit for a while, and seems tolerate food ok. She seems kind of stable at this stage. We still seeing seizures everyday, but we don't know what else we can do except increase the drug dosage. She sleep a lot silently. Even Emily is growing bigger and taller, she is extremely fragile and need lots attention. I thinks the 'stable' is formed by growing and deteriorating. Growing make her stronger while deteriorating make her weaker. 3/26/2010: It’s been a while since my last update. I think I should let everyone know that Emily is doing good. She hasn’t changed much on her condition, but growing heavier, longer and prettier. Her head is even bigger than before. One of my friend said she looks like Hello Kitty, I agree no more. She is just like a little kitten, quiet and peaceful. When the weather is good, we put her on her mattress in backyard for sun bath for an hour. I think she likes it.
Yesterday, I read Miss Amelia’ latest update (http://www.caringbridge.org/visit/ameliaalford/journal) and found out she got a hospital bed. Immediate, I think it is a great thing to have. Since we need to bent to flip her over, feed her, use suction machine and change diaper, we feel a lots pressure on back and neck. And with her disease progress, her head and chest need to be on a higher position to prevent the food come out. A hospital bed can definitely help us on this. I called hospice today and ask them if they could provide it, they said yes and they were going to order one for Emily. It will be delivered next Monday. 4/28/2010: Emily is about same. She got her hospital bed, which is very nice. We can raise her upper body a bit when we feed her. She vomited a few times, and had low fever for a few days. We are still seeing seizures, but we don’t know what else we can do since the sedation drug is at its maximum dosage. Sometime she twitched her lips very hard with her eyes widely opened, sometimes she just roll her eyeballs left and right silently. Most of time, she is quite and peacefully.
Emily is ‘eating’ good. We feed her table food everyday for nutrition balanced meals. Furthermore, Grandpa feed her black fungus jelly to prevent the GM2 formation, grandma does Qigong on her to push out the ‘bad brain cell’. She weights 38 lb and is 39 inches in length. Her weight is above 95% on the chart, but I believe her head is about 1/3 of her total body weight. 5/25/2010: Emily is not doing good since last Friday night.
She vomited twice that day. Because of things that got into her lungs, she started pneumonia, low fever (101F) and very labored breath. Her lungs sound pretty noisy. She is breathing hard using her mouth, her little belly is breathing hard too. This is first time we see her breathing by mouth. She relies on the oxygen concentrator consistantly to get 98% saturation. Without oxygen supply, her saturation dropped to below 90. We gave her Tylenol and antibiotic, and finally the fever is gone today.
She had high heart rate for three and half days already, it is between 150-160. Nothing can bring it down, even after the fever is gone. The Hospice Doctor said it might caused by fever, aspiration infection, nerve disorder, on something else.
Emily tolerate food badly. If we pump the liquid at 100cc/hour, she throw it out immediately. We had to provide the liquid at 20cc/hour to keep her hydrated.
Emily hasn’t open her beautiful eyes since then. We are hoping she can recover from the pneumonia soon so that we can see them again. In general, she is better than yesterday. The fever is gone, and her breath is not as labored as yesterday. The Hospitc Doctor and nurse said she looks comfortable. Jun 3, 2010: Emily got her angel’s wings peacefully in the morning on Friday, May 28th. She is free now, no more seizure, no more pneumonia, no more suction machine, and no more sedation. In the first time, she can do things that she was not able to do, sit, crawl, walk, jump, talk, and fly with her tiny white wings. She gets back all lost abilities and can laugh, giggle, and see the world again.  Emily had with us on earth for 2 years 8 months and 8 days. She was such a lovely, beautiful and sweet little baby girl, it was extreme sad to watch her went away. We had enjoyed every moment when she with us, and appreciated every tiny thing that seemed so easy and natural to other kids but very difficult to her, like finger move, swallow, grin, and breath. Emily was strong and a great fighter. On Thursday, we thought she was getting better and would recover because her heart rate dropped to 120 from 150, her aspiration rate dropped to 30 from 50, and her oxygen saturation was better with less oxygen supply. But after midnight, she turned bad suddenly. At 2:00AM, the oximeter alarmed because it lost her heart beat. Her heart beat was very weak, and her breath was shallow with max oxygen concentrator output. Although I was afraid that she might not able to survive this time, at that moment, I knew she wouldn’t. Her heart or/and lung were failing. I held Emily in my arm and rock her gently. She was comfortable and the saturation went back to 92. At 6:30AM I put her down and tried to get her mucus out. I didn’t get much out, but her saturation dropped to 70 and her hands and feet were very cold. We rubbed her hands and feet to keep her warm. Her oxygen saturation kept dropping like freefall within next one and half hour. At 8:05AM, the saturation dropped below 10 and she passed away. She looked like in deep sleep as before, comfortable and peacefully, the only difference was her face was a bit pale. Emily, our precious daughter, we love you, we love you forever. 
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