June 5, 2012

Mahiya's mood has been changing quite a bit these days. She has cycles of good and bad days. Any change in one of numerous medicine she take make her crabby. She is super sensitive to these changes, with every change in her meds high tone kicks in. She will have higher tone for even a week. High tone make it very difficult to give her any therapy. She will be pushing her head back as hard as she can that it could stop blood flow in my arms when I am holding her head. High tone kicks in her arms as soon as I touch her arms to do some therapy.

PT at the U of M is going well. She will be upset but still the PT keeps doing and after sometimes she gives up and starts cooperating. She is doing stander these days. It is not easy, she has to be rocked in the stander all for just 10 minutes of standing. 10 minutes in the stander is the best we could get out of her. Hope she improves.

We are looking to switch in-home OT to Gillette OT, because she does not do well at home. Now a days I feel like she is getting stranger anxiety. It is a good thing but still not good for therapy. As soon as she hears a new voice in the house she gets upset. She knows if someone else is holding her other than me, my husband or her nurse.

Mahiya's sleeping pattern is very disturbed. In the day-time she refuses to take a nap even if she is super tired. She will be cranky but will not go to sleep and by the end of the day she will be so exhaust.

This week is Mithilya last week of Kindergarten and it is going to get hectic for me starting next week.

Thanks everyone for your continued support...

Six Months update

Can't believe six months has passed since Mahiya's sudden cardiac arrest. Six months have gone with little improvement. I am not sure if the improvements are good enough for six months or if it is too little. When she woke up after her arrest she was not able to move her body at all. Zero neck control and her eyes would stare away to the ceiling. Now she has some neck control. Follows toys sometimes. Tries to bat at toys, but her arms still won't come to the front, it is usually to her sides. And when she initiates a movement her upper body stiffens out. She has been pushing her head back so hard these days, she was doing that 3 months before but after her baclofen it got better. But recently it is getting worse.

For some reason she does not like to sit leaning against her back, but she like sitting leaning forward. The school has loaned us different therapy chairs but she does not sit on any of those chairs. She spends half an hours a day sitting in a adult rocker leaning backward. She is prop sitting with boppy pillow arround her tummy for 10 mins . Sometimes she can prop sits without the pillow. She was getting in-home PT therapy once a week and she absolutely hated it. She will be crying through out the session and we decided we need to make some changes. So we cancelled in-home therapy and started center based therapy at the UOM. Hope she does better with the change. I am learning lot of new stuff from the new therapist S. I feel like a stupid not to know all the great information for 6 months that S gives me. But I am alteast glad to have made the change.

Last week when giving Mahiya her bath I took her clonidine (for BP) patch off. And I forgot to put a new one on. Her sleep cyclewas totally messed up, but she was very alert. She did not want to sleep she just wanted me to keep her busy. She would be happy with just 3 hours of sleep in the night. And won't even take a nap. Her blood pressure was also fine. But her tone increased significantly and she was arching her back so much. But I could not understand why. Then after 3 days I realized that the patch was not on. But with her BP being in a good range I thought it would be good to keep it off. Then the next day her BP was high and I got scared and put the patch on. It has made her too drowsy. She has been sleepy the whole day. Now I feel like it will be a very good idea to wean her clonidine slowly because it makes her drowsy. I feel like it will help her alertness as well.

After passing swallow study, she is taking some formula orally. She takes 40 to 50 mls in a day. She is still getting most of her feed through J-tube. Hopefully some day we could go with just G-tube for her feeds and meds.

Passed Swallow study!!

Mahiya had a swallow study done yesterday. And she passed it. hurrah!!

I drove Mahiya to the hospital for the test and thought I will try putting her in her stroller. She usually gets mad in the stroller, but yesterday she was happy in the stroller and had a good sitting posture as well. We went to the Radiation center and were waiting for the test. A nice lady from child family came and explained about the test and said she has to sit in a tumble form chair (it is just a chair with good support) during the test. I thought, oh no.... she is gonna get mad and will not cooperate for the test. But she proved me wrong. She was very calm and happy in the chair. She was given a thin barium liqiud in a syringe. It was apple flavoured. To start with, she was very slow to swallow as she has not had anything orally for almost 6 months but once she got a hang of it she did good. We could see the liquid go down as she swallowed in the continuous X-ray video. She did a good job, did not aspirate. Then she was given a semi-solid barium paste. She was confused first when she had it and then she swallowed it. So the speech therapist has given a OK to oral feed her. We are excited but still are worried abut her throwing up and not getting her medicines.

Yesterday I was holding her and was eating a sandwich and she smelled it and she wanted to have it. She was moving her arms attempting to get it and had her mouth wide open...... ha....ha....but she cannot have it....not yet . Then I tried to give her a bottle and she was biting the nipples but did not attempt to suck on it. But she drinks out of a syringe. It is going to be a very slow process...... we will take it slow.... as long as there is some progress.....

Thanks everyone for your continued support!

swallow evaluation

Mahiya when she woke up after her code, was evaluated by speech therapist (ST). At that time she had her jaws tight and did not open much, the ST called it tonic bite, and said this is due to her severe brain damage (which I hated to hear). Then the ST put her finger to see if she had gag reflex. NO.... then an ice chip in her mouth to see if she would swallow. She did swallow but ST said she is very slow to swallow waits until her mouth is full. So we were given a big NO to oral feed. After couple of days I saw her mouth was very dry,so I started giving her water in a syringe and she loved it. Couple of weeks later ST came again and said the same thing again...........we were very upset first and then we understood that aspirating could cause even more set back. So we have been tube feeding (J tube) Mahiya since her code.

After coming home, we have been giving her little taste of food that she loved before her code it seemed like she was liking it. Last week we had another evaluation and the ST gave her half a baby spoon of apple sauce. She opened her mouth and bit on the spoon and won't let go. Again the ST started "she still has the tonic bite". NOT AGAIN!!!. But she let go in few seconds and swallowed the apple sauce. ST looked inside her mouth to make sure there was no residue. There was  no residue and Mahiya took another spoon. Drank water out of a cup. ST was happy with her and said that she does not feel like she is aspirating but still she wanted a swallow study to be done before we feed her orally. Swallow study is done under continuous X-ray. She will be given some liquid to drink mixed with barium (tasteless). This barium can be watched under X-ray to see if she is swallowing without aspirating. But ST is OK with giving her little pureed food until the swallow study. Liquids have higher risk of aspiration.

Mahiya has not had much solids even before her code, so she it is new to her. Hope she starts eating solids and likes it.

Whew...

Mahiya does not like to be laid on the floor. She wants to be held all the time. She was like that even before she coded. She would be crying the whole time during her each diaper change. The scary part is if she gets really upset she will hold her breath and her heart rate drops. Usually her breath holding spell lasts for few seconds and she will start breathing normally. Blowing on her face helps, but after transplant I am scared of doing that, as I don't want to give her my germs. This afternoon, she was in my lap and then I had to go send my older one off to school, so I asked the nurse to watch her and put her down. She immediately got very upset and started crying. She held her breath, I immediately picked her up and was trying to calm her. But she would not calm, she held her breath for so long that I started freaking out. She could not let go. Her face started turning little blue, I thought she was going to pass out. I got so scared that I could not think, then suddenly blowing came to mind. I did not care about germs and blew on her face twice and she immediately stopped crying and took a deep breath. Then she just had her eyes wide open and staring at me without any expression on her face, as if she was in a shock. I got very worried. I wanted to make sure that she was OK. I grabbed my phone and played one of her favorite video and she started smiling.... whew what a relief.....

What matters most?

We had an neurologist appointment. We were really scared before the appt, as we did not want to hear anything negative about Mahiya's neurological state. I don't know if we are cheating ourselves or just don't want to face the truth. But the most important thing is we don't want anything or anyone to do even a slightest damage to our hope. Hope is all left in our life and it is our strength. The appointment went well. The Dr. J was a very nice person. He is one of the best in our area. He spoke for 30 mins and it was very pleasant. He neither gave us false hope or ruin our hopes. He exactly understood how devastated we are and the pain we are in. One of his point made a great impact on me.

He said "I have treated so many neurologically affected kids, kids who will never walk or talk to kids who top their class, but they all are happy. They don't care about their disability. They are happy the way they are. It is like happiness is in a separate gene. Keep them happy. Just enjoy the time with them. It does not mean that you have to forget about their disability, but you have to learn to lower your expectation for your kid. Keeping them HAPPY matters the most."

It was such a great statement. We don't know our destination, but will keep moving with hope and try our best to keep her happy.

When we bring up any issues with Mahiya to the doctors, they say "she is very complex it is not easy to tell anything or pinpoint a reason". We don't know what to do sometimes.Wish there was a specialist who is an expert in all fields. Mahiya's progress has been really slow. Patience is a virtue. Hope our patience will be rewarded.

First month at home - Post cardiac arrest

Each day is getting harder these days. I can't stop thinking of all the worst things that happened. I try not to think and concentrate on Mahiya's needs, but it is very hard. I wish we had not put Mahiya through the transplant OR I wish the ICU team had trusted my instincts the day before the code OR I wish she had responded to the CPR sooner........ Wish I could go back in time and set the clock back. I know crying my heart out is not going to help but still, I am just a human being. I just want her back the way she was. Her therapist keep saying celebrate every little improvement that she makes. I am trying to focus on little progress she does and tuck all the bad experience in the back of my head. I came across a inspiring phrase that says,

"HOPE"

 Its just one little word, but sometimes it means everything

I felt like that this phrase was just the right one in our situation. We will hang on to HOPE.

Now updates on Mahiya...... It has been almost a month at our home and Mahiya is irritable most of the time when she is awake and sleeps a lot in the day time. It could be because she is on high dosage of prednisolone(steroids). Keeps me up all night.  She is not making much eye contact these days. she will look at me for a second and turn away. She responds to sound and music but cannot located the source. She get PT once a week. They have set a goal of sitting up in 6 months from now and are working towards it. There are days when she gets really upset from the beginning of the session and does not get enough therapy. She has a very tiny window of time when she will cooperate, which will not coincide with the PT session. So they try to show me how to work her out and I try to do it when she is more up and alert. She gets OT once a week. They are working on feeding, reaching, grasping and tracking. In addition she get the OT from school as well. they also work on fine motor skills and the school loan us lots of therapy stuffs to try with her. She is holding her head better these days.

She gets lab done twice a week. We get in-home nursing for port access and blood draw. We see the doctor once a week. I wish we don't have to go back to the same hospital again, but we have no choice, we have to go there for our transplant follow up clinical visits. Even though we are very disappointed with the ICU team who did her post transplant care, we like our surgeon Dr.C and he is very involved in Mahiya's care. We see Dr.C every other week.


They are slowly weaning her steroids, hope she gets less irritable soon. We don't her out much except for clinic visits. And it has started to snow here. We feel like taking her out will help with stimulating her senses, but very worried about germs. Mahiya gets 8 hours of home nursing from 10 AM to 6 PM. But still I can't let them do all her cares. Our surgeon wanted us to try home nursing. We actually did not want to have one but we respect him and so we gave it a try. I do most of her care and give her meds. The nurse is more like for emergency. Mahiya  is on continuous feed through J-tube. (She got a GJ placed as she was throwing up often and the doctors were worried that she won't be getting her meds). She gets some time off her feeds for therapy. Because of the fear of aspiration she is not getting anything orally. We might get a swallow study done on her soon. After that she can feed orally.

Thanks everyone for your continued support!

Transplant - Long story short

On Sep 9, Friday I got a call around 9.30AM, it was from Dr. E. My heart started racing. She said we have a perfect liver for Mahiya. She asked how do you feel? I could not speak for few second. It was very emotional moment I felt really sad for the family who just lost their little one and very stressed thinking about Mahiya having to go through the major surgery. My legs started trembling. Dr. E said come to the hospital before noon. I called Raj at work and told him. He rushed home. We packed our bags and started at 11.15 AM leaving behind my older daughter in tears. Mahiya was admitted and was started on IV fluids and numerous blood work was done. It was around 11.00'PM when they took her to OR. All the way from the room to OR she was smiling at her dad and patting him on his face, as if telling us that I am happy like this don't put me through this surgery. We did not understand it until later. Surgery started at 11.30 PM. The surgeon was out at 4.30 AM and said he is pleased with the surgery and said he may have to go back in to make sure everything looks good in 3 days, but it also depends on how she is doing. By the time they came to get us it was 6.30 AM. She was in the ICU with breathing tube and lots of IV line and meds running.

The next day they wanted to extubate her. They tried pressure support trails and she started to breath very shallow and her breathing rate was 120. Then they stopped and decided to do it the next day. On sunday her ammonia was 74, I started worrying, the surgeon said "I know it is hard, but you have to take ammonia off of your mind now." But I could not rest, the next day I asked them to check her ammonia and it was 45, it was a great relief. They could not extubate her the next day as well. Now everyone started turning their attention towards her lungs. Her lung volume is small.....Has she had any breathing issues before?.... And it scared us.  Then couple of my friends told that their kids also had the breathing tube in for a while. That helped a little. Mahiya was retaining a lot of fluids everyday as it is the stress response of the body to such a major surgery. She was started on diuretics to help her remove fluid from her body. Finally got extubated on the 7 th days (Friday) after surgery. She sounded like a cat that day. Poor thing. The rest of the day she was alert and was watching videos and needed me by her side. Her liver enzymes were getting very close to normal and they were planning to move us to regular floor. We felt like back to square one, but a better one with all her new less restricted diet and new meds schedule.

 

The next day (Saturday) she was looking very tired and her eyes were sinking. I keep insisting that she looks very dry. They said that they want her to be dry. If she get more fluid up then she will have trouble breathing and has to intubated again. Her port needed to be reaccessed Saturday night. She was tired but awake at that time. When the port was reaccessed she did not show even the slightest resistance or cry. It is not her at all and I felt there is something terribly wrong. She needs 3 persons to hold her down to access her port. I asked to see the attending doctor. He came and examined her and said she is dry, but she is still OK, and we are going to go up on her fluids by 10mls an hour and see. And also he ordered for a blood work at 10.00 PM. Her hemoglobin was 8.6 and her potassium was low. So she was giving potassium. He asked for another lab at 5.00 AM the next morning. At around 2.00 AM her breathing rate started to go up she was breathing in upper 50's. I asked the nurse to keep a close watch on it. early morning her breathing rate was in 70's and her blood pressure was dropping. But the nurse said that it was OK. Her blood pressure is dropping because she is dry and her breathing rate is fine as her SPO2 was 100%. In the round we told the doctor about our concerns and he said she is dry and we will give her a bolus of liquid. For some reason 5.00 AM lab was not done. Lab was done at 9.00 AM hemoglobin was not done ????? Her pressure kept going down and her feet was getting very cold. But the nurse was not concerned at all. He said he is going to give the fluid bolus soon. At 10.00 AM her breathing rate was falling rapidly and her heart rate started dropping as well. we were standing right next to her and were begging the nurse to do something or call the doctors.  They were very cool and were dialing the doctors. we got really frustrated and asked them to press the code button. Finally when the code was called she had stopped breathing and her heart rate was falling. The ICU team rushed in and we were taken out of the room. They started CPR immediately. We were in a shock and tears and did not know what to expect. We thought we were going to lose her. Our metabolic doctor stayed with us all the time was giving us hope and updates every 5 mins. In 10 mins she went to check and said her heart is pumping now and she was intubated, it was such a relief, which words can't explain. Then they said her hemoglobin was 3.6 at the time of the code and her potassium was too high 12. The reason for the hemoglobin being this low could be caused by bleeding internally. An echo was done, ultrasound was done. They saw lot of fluid in her abdomen. She got lot of blood products. Then the doctors who attended her code came and talked with us, they felt like it was very quick so any damage that was caused should be reversible. And her brain should be fine. That evening the surgeon examined her abdomen and she open her eyes and looked at him, which he said it is a very good sign that her brain is OK. Her liver enzymes that evening was in 4000's. A CT scan was done at 9.00 PM to conform the fluid in her abdomen was blood and it was blood. At that time her liver enzymes were in 7000's. The surgeon said it is not good for her new liver so he has to go back in immediately and wash out the blood. She was taken to the OR immediately. The surgeon could not find the source of bleeding but he said it was from all the surrounding tissues and so after wash out , a glue was applied to stop bleeding and a drain was put in. For couple of days she was bleeding 30 ml an hour from the drain it was very scary. She was transfused everyday. And the day after the code her BP was out of control. That is when they doubted brain damage and the neurologist came to see her. But she was sedated heavily. They put in EEG leads to monitor her brain activity. We heard everything that we did not want to hear. The neurologist said that she just has one fifth of activity, it could be sedation, but still he thought there was severe damage due to lack of oxygen to her brain at the time of the code.

 

We are so heart broken that we hate life. But our older daughter Mithilya needs us, she is the only one who is holding us. Mahiya believed in us and trusted us, but we feel like we betrayed her. We can never forgive our self for putting her through this. Everyone saying God is watching. Yes he is watching but not helping. why?????????? Doesn't she deserve the littlest mercy????? What we were fearing will happen without the transplant happened just after the transplant. Our fight for her brain has not stopped after the transplant. Few weeks ago she was so full of life smiling, walking , talking .............. and now her whole future is filled with uncertainty????? Pre-transplant she cannot even lay down for few minutes for a diaper change and now she is laying in her bed all day.

 

They insisted getting a MRI to see the level of damage, but we denied it as there is no change in management or treatment after MRI. It would just be an emotional stress for us. Our surgeon agreed with us and he keeps saying "I don't believe in prognosticating with an MRI. Kids are resilient. Give her a chance. Only thing we could do is wait and let her tell us what she can do".

 

She opened her eyes briefly for the first time, 1 week after the code. It was exciting. And she moved her hands towards her head when the EEG leads were taken off. She was extubated 2 weeks after the code. She did not open her eyes much for the next 24 hours and I asked the attending doctor about it. She said "probably she won't mom, because of the hit her brain has taken". It was like a stab on my face. I just stood there. How could she say that right to my face, in such a hurting way?  She had opened her eyes even before extubation and this is what I hear from the doctor. The doctor could be right, but she has no proof. We did not do the MRI and how could the doctor just say that she has suffered a major damage. I stopped asking any question to her from that day. Everyday in the rounds the attending doctor would relate any issues that she has to neuro, like high BP, fever, bradyc. And would say not much we can do. She would say we have to get her off of all IV meds and make it oral and transfer her to the regular floor. Everyday her BP meds were titrated to control her high BP. Her liver function started improving. It came down to 300's and started going back up. She had her first liver biopsy on OCT 7. Biopsy is a procedure in which a needle is inserted into the abdomen and a small piece of liver is taken out. It is done under sedation. From the extracted piece the pathologist can see if it is rejection or other issues with the liver. It was done by Dr. H. He is very confident man. He did the biopsy bedside in the ICU under sedation. He made it sound so simple. Pathologist had different view with her biopsy. One thought it was rejection and the other thought it was poor blood flow. Ultrasound was done and the blood flow looked fine. She was treated with THYMOGLOBULIN, an anti-rejection meds. It is administered once daily , it runs for 6 hours through IV. It is a very portent drug. It could cause fever, chills, trachycardia, high BP...... so premeds were given 30 mins before stating thymo (Tylenol, benadryl and steroids). She was treated for 3 days. This time she did not have any adverse reaction to this drug. The liver enzymes were improving but not as much as they would like to see. So they stopped treating and ordered for another biopsy to make sure it is rejection for sure. They switched all her IV BP meds to oral and transferred her to regular floor on Oct 10.

 

She had her second biopsy on Oct 12, it was in the OR this time as Dr. H was not available. This time everyone agreed that it was rejection. THYMOGLOBULIN was started again for 5 days but this time first 2 days went OK but she started developing a fever from the third day. They would stop thymo for sometime and start it back again when her fever comes down. Part of the reason for fever could be she was not getting steroids as premeds as they were worried about over suppressing her immune system. Again the enzymes were coming down slowly from 1000, after the fifth day of treatment the enzymes were in 400's. The surgeon thought that she has a very strong immune system, that is why it is not coming down as fast as expected. She was given steroids every other day after the thymo and the enzymes are coming down.

 

Oct 27, the enzymes were in 200's.  On oct 30 enzymes are in 100's. If it keeps trending down and they will plan on getting us home on Nov 2.

 

Neurological Status: Couple of days after extubation, Mahiya was awake more. But we could not say if she recognized us. It was so painful for us. She would just stare at the ceiling or to her sides and won't even blink for a long time. If we get very close to her or talk to her or touch her, she will start crying. She wanted to left alone all the time. Her muscles were getting stiffer everyday. PT and OT were working with her even before extubation, to stretch her legs and hands. Her left leg and hands were more stiffer than the right side. Her foot was always pointed down. So they made a soft boot for her to break the tone in her legs and keep her foot at right angle to the leg. She had to keep it on for 4 hours and off for four hours through out the day. But even with that she was getting stiffer. PT and OT show me how I can work her out and I was trying my best. She slowly started developing a trust on me and her dad and won't cry when we stand by her. She briefly started making eye contact with her dad. She won't respond to my voice, but responds to her dad's voice. I won't take it in a normal day, but now I LOVE IT, any improvement is great!!!! even if she just wants to be a daddy's girl!!!!! On Oct 14 she smiled for the first time after her code at a toy. She was started on Valium, which is a muscle relaxer medicine to help with her stiffness. In couple of days we saw good improvement in her tone. She was not very stiff and was easy to work with her legs and hands. On Oct 22 dad got a smile, I got a smile the next day. She sure is a daddy's girl!! She is tracking toys with her eyes. She does not have much neck control. She does not turn her neck much. We try to sit her up and she is slowly getting better. She holds her neck for few seconds and sometimes turns it to track a toy. She has a very small window when she does lot of stuff and other time she is not interested. She likes her wagon ride in the hallway. She calms down to music. She loves to watch video pre-transplant, but now she is not. It is probably too much stimulation for her at this point. We will keep working with her. She will be getting in-home PT and OT. In addition to that the early intervention team from the school will be working with her. We want to be positive and try to bring her back. We think when we get home, being around her sister will help her as well.

 

Restless long weekend.....

Aug 31: Mahiya was yawning a lot, took her to standing lab. (Hate going to the ER as they want to weigh her and get vitals before accessing her port and she freaks out as soon as we enter the ER. She will be screaming the whole time.) Standing lab is better as it is the same nurse all the time and she knows Mahiya very well. She will have everything ready before we enter the room, she will access her and draw blood in couple of minutes. Ammonia was 163. Did not expect that!!. I asked the doctor to transfer us directly to the floor bypassing ER and she did. Mahiya was started on D10 and lipids. Usually our team starts lipid above 150. Evening 120. Next morning 63. Evening 48. Friday morning when the lab person came to draw blood it was not drawing. Again her tissues got swollen. Had to take the needle out. Hot pack helped. Accessed again in couple of hours to draw blood for ammonia and was locked. Ammonia 47. In the evening again 47, they let us home. ....... got home for the long weekend!!! for how long ????

We have totally lost confidence in us catching Mahiya's ammonia early. Every time we go for a clinical visit thinking she is fine, but her ammonia would be above 100. So these days we rush to ER even if she gets a little fuzzy.

Sep 4, 2011: Mahiya was kind of little dull in the afternoon. Then had a couple of hours of good nap. When she woke up at 9.30 PM, she was very cranky. We did not waste any time and rushed to ER. At midnight ammonia 73. But her white Blood count was elevated 23 (normal below 17) So the doctor wanted to culture her urine to see if she had any bladder infection. I was so scared of catheter and asked for bag. But the doctor said bag is no good, it could give false positive and there will be a lot of process after that. So they insisted catheter. We said OK half hearted. But it went very quick. They did an ultrasound to see if her bladder had some urine and then did the catheter, it took less than a minute. A 45 minutes test said her urine was clean. And a culture has been started on the urine as well. It will take 2 days . Mahiya was admitted not because of her ammonia but her WBC. Chances are there that any infection that she is fighting could rise her ammonia. D10 was started. Ammonia in the morning was  68 and WBC 16. So fluid was stopped. Ammonia at 2.00 PM was 42. Got home at 4.00 PM.

4 days at home, is it too much already....??

Aug 24, 2011 : Mahiya was irritable in the morning. So we thought we need to check her ammonia. Went to the ER. A nurse in the ER tried to access her port and pushed the needle into her tissues outside the port and it was not drawing. She tried to flush it and it flushed fine. I felt that the needle was higher than its usual spot, I kept telling her, but she wont listen. Mahiya's tissues got puffy with the flushed saline. Then the nurse pulled the needle out. The fluid started coming out of her skin. It freaked us out. Then came another nurse, she accessed it and drew blood. Ammonia was 66 hurray...... They sent us home but we left the port accessed as we had a follow up with metabolic doctor the next day.

Aug 25, 2011: Next day ammonia 89. The doctor wont let us go, she was admitted. I was not expecting the hospitalization, because the doctors have told anything above 100 is when they admit. But our doctor said looks like she is trending up, so we need to start fluids.Was started on fluids right away and ammonia at 8 PM was 54. At 1 AM 52. Morning went back up to 80. ........ huh ....
2.00 PM - Ammonia 28. When drawing blood, the lab tech said it is kind of hard to flush, but after laying her down it flushed fine. Then Mahiya got very cranky. She was trying to pull on the port needle. She always does that. So I was trying to divert her, but she was getting restless. I was confused why is she acting like this when her ammonia is 28. That is when I doubted that something is bothering her???? probably her port and I pulled her shirt to look at the port sight and it was so swollen, I got really scared and immediately stopped the pump. I though the pump would beep if it is very hard to infuse. The nurse tried to flush and it was not flushing at all and the needle was only half way in so all the fluid was going to the tissues not the blood vessel. So the nurse had to pull it out. I felt good as it was taken out, as they were going to stop her fluids anyway. In few seconds, I realized the port was not locked with heparin. (heparin is a solution that is flushed into the port before de-accessing. It prevent any blood clots in the port when it is not used). The nurse said she has to talk to the surgery team before re-accessing her port, because it is so swollen. She called the team, they gave a OK. but the nurse could not feel the port, her tissue was very hard with all the liquid. So they gave me ice packs to put on her skin. Mahiya was screaming the entire time. I was so worried about the port clogging. Then they said using hot pack is better and it actually helped. A chest X-ray was done to located the port and the port was in place. Hot pack for 30 mins helped with the swelling. They they tried to access her with a longer needle and it worked. Blood was drawing fine. As they used a longer needle, it was sticking out, but it was OK, it was a great relief to us, but it was very hard on Mahiya. The whole process of re-accessing the port took 2 hours. Ammonia next morning was 46. They let us go. She is very sore from all the port issue and she needs a break ........ ....please lord keep her stable...

As we wait......

We pray for Mahiya's stability until a liver becomes available. It is killing us to pray for a faster availability as it has to be a deceased donor. We just pray for Mahiya's stability.

Mithilya is starting her Kindergarten this fall Sep 7,2011. She is very excited. We are excited too, want her to get out and have a normal life, but still we are scared of all the germs that she is going to bring home.

When Mahiya undergoes transplant, we will pull Mithilya off her school and home school her for sometime. The tutor has accepted to teach Mithilya at our home after transplant.

Mahiya is walking holding on to furniture. Mahiya loves her sister. She says few words and ""akka" is the predominant one. "akka" means older sister in our language - Tamil. Whenever she gets excited she says "akka". She loves watching her sister and her hair. She loves pulling her hair. Mithilya loves Mahiya too, but sometimes she won't share toys with her.

Mahiya can do some things when asked for like, she claps her hands, she will bounce up and down in sitting position, she will turn towards her sister when asked for. She loves to climb steps. She will climb all the way up. She loves her bath time, there is not a single time she has cried.

My in-laws are leaving to India by the end of Aug. My parents are coming here in the first week of September.

Start of the crazy time...

On June 15, 2011: we went for a regular metabolic blood draw and her ammonia was 180. We could not believe it, She was looking just fine, we had no clue. We thought that it was a false number and asked for a redraw.  The redraw number was 112. Unfortunately at that time, all out metabolic team was out of town for a conference in DC. Our nurse practitioner spoke with the doctor and said we need to admit her. But I was not convinced at all. Then the doctor called me and said I cannot let you go home. Let us be conservative and start her on fluid. Then we admitted her. She was started on fluids. In couple of hours her ammonia was drawn, it was 198. Our team start ammonul above 200. So they transferred us to the ICU and drew one at midnight, before starting ammonul. They said everything depends on this blood result. If it is higher than 200 we have to put in a central line to start her treatment. It scared us to death. It was 1.00 AM and I was desperate to talk to our doctor, I told my nurse. Our doctor immediately called me and explained me the plan. I asked why not use the same line on her hand for ammonul and arginine. She said it is too strong that it could burn her veins. We were sitting in the ICU scared and her ammonia came back 140. It was such a relief. By morning it was 77. They transferred us back to regular ward. But every time the lab draw was too exhausting to Mahiya and she would be screaming. That is when we decided to put in a port. In a week she had her port and her ammonia went up to 90 the day her port was placed. We were in the hospital for another day......

July 5, 2011:  Mahiya was little off from her normal. She was very quite and dull. But still we didn't think of high ammonia. When I put her to bed that night, she fell asleep right away it was so unusual. She usually takes at least 10 mins to sleep. It got me worried and I told my husband, we have to go to the ER. We got ready and put Mahiya in the car seat, it woke her up and she looked fine, she was alert and smiling. For a minute we thought she is fine. But still we did not feel comfortable and headed to the ER. Her ammonia was 170. Another 5 days in hospital......

On July 23, 2011: again something was not right. Trip to ER. Ammonia 100. Spent 4 days. July 27 was her birthday. We were in the hospital on her first birthday. The doctors wanted us to get home on her birthday and we got home by 5.00 PM. When we were getting ready to go home Mahiya was playing in her crib and she was pulling up to stand for the first time. We were so happy. We went to the temple that evening.

Aug 10, 2011: regular metabolic visit. Ammonia 188. Redraw before admission 112. Hospitalized for 7 days.

Aug 18, 2011: metabolic follow up. Ammonia 112. Redraw before admission 105. 2 days in hospital.

Doctors have no answer for her so frequent episodes. They said her honeymoon period is over. She has been lucky so far. And this is her disorder, it is very severe.

With every episode we were slowly deviating towards listing her for the Liver transplant. As she does not show obvious symptoms even at 150 scares us. NO MORE DAMAGE......

While we were in the hospital we meet the transplant team and the surgeon. We are glad that UOM has the metabolic and transplant team. Both team are working together on Mahiya. The surgeon is a Indian, to be more specific south India - Andra, we are from the neighbouring state - Tamilnadu. He went to medical school in Tamilnadu. He was very friendly and explained us everything that we needed to know. He explained the risks involved as well. He also said "If she was my child I would have done the transplant even earlier. It is a very dangerous disorder and her brain is very precious." It was stuck in our mind.

One other thing that was up in our mind was our metabolic doctor's words. The same doctor who was not very happy with her development at 3 months said, "She looks developmentally normal, like any other baby and I want her to stay that way and that is the reason I want you to list her for the transplant. I have seen so many UCD kids with disabilities and that hurts me" We love our doctor and she has gone beyond her duties to care for Mahiya. She cares for her as she was her own child. We consider our doctor as a GOD....

Aug 11, 2011: Listed for Liver transplant.

Mahiya - 7 to 10 months

Our bi-weekly appointment became monthly. Blood is drawn at our every visit to get a panel of things tested. Few of many things are ammonia, prealbumin, Glutamine...etc..But Mahiya was a very hard poke and every time the Lab people will struggle to find a vein. Sometimes she needed an ultra sound to put in a line to draw blood off of it. Our doctor was asking us to consider to put in a port-a-cath. Which is a small medical appliance that is installed beneath the skin. A catheter connects the port to a vein. Under the skin, the port has a septum through which drugs can be injected and blood samples can be drawn many times, usually with less discomfort than a more typical "needle stick". But we did not want to put her through another surgery. We were holding back.

Mahiya was sitting unsupported for few mins at 6 and a half to 7 months. There was not much improvement and I asked the early intervention team to evaluate her. They were actually happy with her sitting. And they said she is not very far from sitting longer without support.

Then soon she started rolling from one end of the room to the other end. At about 7 and a half months she was sitting very well. Then she was trying to get on her fours and rock, I was expecting her to crawl but did not happen soon....... I was really getting worried. It was at that time she learned to sit from laying down position. She was practicing that for couple of weeks. She will go from sitting to on her fours and then come back to sitting. When she was very close to 10 months, she started crawling. We were so excited. She would crawl from the living room to kitchen and explore the dish washer!!

At our may visit Mahiya was 10 Kgs and the doctor said she is a a good size now to think about liver transplant. It blew us away. She has been so stable this far and why would they ask us to list her. The doctor said that she has a very severe mutation, because there is a total stop in her urea cycle, she might get unstable at any time. We came home confused. But my husband Raj was open for talks with the transplant team. But I was very hesitant. He said I don't want anymore damage to her brain, if this is what needs to be done to protect her precious brain, we have to go ahead. I could not make up mind at all......... a month went by .......

Another hard blow!!

At our February appointment our metabolic doctor heard a murmur in her heart and she ordered for an Echo the week after. We were for some reason very confident that she is totally fine. But the shock was waiting for us. On Feb 16, she had an Echo and was diagnosed with Hyper tropic Cardio Myopathy. It is a abnormal thickening of heart muscles. Because of that her heart has to work harder to pump blood. We were so depressed and helpless. Everything was blurry and we were just lost. They wanted Mahiya to be admitted for couple of days to get a baseline.We did not know what to do with our older daughter. We had no family here. So we let her in our friend place. It was the first time she was away from me and it was so hard on her. But I am very grateful to the friend who took care of Mithilya for 4 days.

It was the time when we were kind of getting used to live with UCD, this shook us again. We had to build up our confidence again, which was not easy. In the hospital, Mahiya was started on meds to reduce her blood pressure. For now they are just monitoring her. There is not much answers like what caused it , when did it start, is it from birth..... ??????

When in hospital, she was having running nose and cough and her ammonia went up to 90, but she was started on D 10  immediately and ammonia was in control in 24 hours. Then we got home after 4 days. We realized that it is not possible to live here (US) without family support and my in-laws accepted to come from India and help us out.

Life goes on......

Mahiya till 6 months

After about 2 weeks of hospitalization after birth we got home. Mahiya was refluxing very bad at that time. The first week at home was very hard, we could not lay her down at all, she would throw up immediately. So me and my husband took turns holding her in our arms through the night for a week and slowly her reflux improved with prevacid. But not a minute went without worrying about her ammonia. I decide to quit my job. For the first 4 months we had weekly appointment with our metabolic team. Mahiya was basically a sleepy baby and so every time we see the doctor, she would think that she is hyperammonimic and test for ammonia and most of the time it would be less than 9. We had too many questions about citrullinemia but we also did not want to hear bad things and loose sleep over it. We were so worried about the extent of brain damaged caused by her initial ammonia at birth, but the doctors could not tell us anything, we asked about MRI scan, but they said she is too young for that and it is not going to show much. The thought of even a common cold or fever causing her hyperammonimia was very depressing.

As she had bad reflex we did not give her much floor time. At an appointment at about 3 months our doctor said Mahiya does not hold her head up as much as I like her to, so I think she has suffered some brain damage. I did not expect that and I could not take it. When we got home we could not bear it we were all in tears. Then I told myself, it could be because she was not given any floor time. From that day I tried to give her as much floor time as I could and she was holding her head up better. At about 4 months Mahiya's oral feeding slowed down and in a week she totally stopped feeding orally. All her feeding was done through G-tube.

The early intervention team from our School started their visit at about 4 months. At their first visit they said she is within normal range of development. It felt good.

Our appointment after four months was every other week. Mahiya rolled over at 5 months. She was sitting for few minutes unsupported at about 6 and a half months.

We stopped our older daughter from school for the fear of infection. Our early intervention team helped us find a tutor for my older daughter, Mithilya. We are very thankful to them. Mithilya loves her teacher and learning a lot from her.

Oh God...... why???

We are Hindus. According to the Vedas, if one sows goodness, one will reap goodness; if one sows evil, one will reap evil. Karma refers to the totality of our actions and their concomitant reactions in this and previous lives, all of which determines our future. We don't know what sin we did in our previous birth to have been cursed like this in this birth. It is killing us every day.
In this birth we have been good people, at least not bad. Please help us lord!!