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.