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!!