4/17/11 9:30 am
I have decided to start each update as a new post since we will be staying in the hospital for a while. When we first came, we never imagined that we would be here for two weeks with no discharge in sight so it made sense to just continue to update the same post. That no longer makes sense.
We did not move floors as we thought in the last post; Nadia actually took a turn for the worse. James and I got a call at 3:00 am on Saturday morning in our sleep room telling us that Nadia was having severe trouble breathing. They needed to intubate. We immediately rushed from our room and had to wait outside the ward until they finished the procedure. Dr. Holmes came out and explained that as her stomach kept increasing in size, it was becoming more and more difficult for Nadia to breathe. A blood gas showed that she needed immediate assistance. The additional oxygen flowing into her nostrils had been enough to help until this point, but was no longer cutting it. They had to put a breathing tube down her throat and into her lungs. This took two attempts and she fought until they gave her a drug to temporarily paralyze her. She was put on fibrinogen to keep her sedated so she doesn’t fight the tube. The breathing tube has a built in suction device that our nurses manually do once an hour. At first, the gunk they would pull from her lungs was bloody or tinged with blood (from the entry of the tube) but now it is mostly clear. Because her mouth is taped open, the nurses do mouth care once an hour to keep her tongue and mouth lubricated and moist. They needed more access to Nadia’s system so they put in two new IVs while they were dealing with the middle of the night emergency: one in her head and one in her foot.
The doctors said that her breathing trouble is because her tummy has gotten so big, it was pushing on her lungs and not allowing them to fill or clear completely. Again, they compared it to being pregnant (how toward the end of a pregnancy a woman cannot catch her breath because the baby is pushing so high in the abdomen). Obviously, Nadia’s issue is more severe than the pregnancy example. Her tummy girth has continued to increase but thanks to the breathing tube she is stable. Her tummy girth was 40 centimeters when we went to bed on Friday night.
Dr. Holmes was the first to mention DIC as a possibility as to why Nadia’s blood is not clotting. Disseminated intravascular coagulation (DIC) is a complex systemic thrombohemorrhagic disorder involving the generation of intravascular fibrin and the consumption of procoagulants and platelets. The resultant clinical condition is characterized by intravascular coagulation and hemorrhage (emedicine.medscape.com). They FFP (fresh frozen platelets) that they had been giving Nadia were not getting the results they desired in improving Nadia’s INR (International normalized ratio, a laboratory test measure of blood coagulation). INR should be below 1.0, Nadia’s started at close to 3.0. Each FFP lowered it some but she only got down to 2.6. They added some vitamin K through her IV to help lower her INR. As of last night, she had an INR of 2.3, today it was at 2.1, the immediate goal is to get under 2.0. (Later in the day yesterday, Dr. Allison Peroskie (hemeotology) asked that a blood test be done immediately after some FFP and it showed that her INR did drop significantly with the fresh plasma,but that the number increased as time went on. She doesn’t maintain the INR as time goes on…mystery. Dr. Peroskie does not feel that DIC is reallyour problem but can’t yet explain what is).
Nadia was uncovered for about 2 hours while they worked on her and put in the breathing tube. Her temp dropped significantly. When we were allowed in the room at 4:00, her temp was only 93 degrees. They put another tube down her nostril that is able to monitor her temperature. Nadia’s blood pressure has had moments of being high but always stabilizes; she has never been put on any meds for blood pressure (much to the surprise of her entire team as they would expect it with this much going on). This is encouraging that she has a strong heart. Nadia has continued to have trouble regulating her temp. She has warming lights about 2 feet above her bed that can be turned on as needed to raise her temp. The room temp is 80 degrees to help keep her comfortable.
The surgical team came by very early Saturday to consult. The PCCC (pediatric critical care) doctors convinced them to wait for a CT scan. They also ordered an ultrasound of both belly an head (to check brain response), a GI consult, and another x-ray. The CT scan showed that her liver was slightly large, but not nearly as large as expected. It also showed no blockage or abnormalities in her bowel which held off surgery and yet another strike against Hirschsprungs Disease. The CT scan did show that she is completely full of fluid. Completely full of fluid,no air present at all in her abdomen. Her lungs had fluid in them, especially her left one. This was overflow fluid from her abdomen. The fluid was solid all the way down, well past her bladder and kidneys (which looked ok). Her liver was surrounded by a ring of the mystery fluid. I say mystery fluid because the doctors do not know what it is or what is causing it. The medical term for this is ascites. Defenition according to medicinenet.com: Ascites is the accumulation of fluid (usually serous fluid which is a pale yellow and clear fluid) in the abdominal (peritoneal) cavity. The abdominal cavity is located below the chest cavity, separated from it by the diaphragm. Ascitic fluid can have many sources such as liver disease, cancers, congestive heart failure, or kidney failure. It is a mystery because her liver is not functioning as well as it should but is not failing, her heart is good and not the problem, and her kidneys are ok and not the problem. So…the textbook things that cause an ascites have all been ruled out as not being the cause in Nadia’s. Nadia’s liver: slightly enlarged, synthetic functions not functioning properly (the protein producers). They did a doppler ultrasound of her liver to make sure that the flow was moving the right direction and it was.
It was then decided that Nadia would have a catheter inserted into her belly to drain the fluid. Since she is high-risk due to her clotting issues, the surgical team was called in to insert the catheter. Before this procedure, the PCCC doctor wanted more access to Nadia in case of emergency. He asked James and I to leave the room and took two hours to put an Art Line into a major artery in her groin. This line is different from the PICC Line as it goes into bigger vessel and can handle larger doses. He inserted something into her toe to monitor blood pressure and also ran a catheter into her bladder to pull and collect urine output. Measuring all output is now more important than ever as we don’t want to add even more fluids. It becomes a tight rope as so many of the things needed to keep her healthy have significant volume. The surgery team came back and put the tube into her abdomen. They pierced her belly with a long needle, threaded a line through the needle, withdrew the needle, and then threaded the tube over the line. The tube was sewn into place on her side and left to drain. They pulled off 60 ml of clear, yellow fluid during the procedure. She started draining really well. They warned that the tube should be clamped off for outputs more than 30 ml per hour to prevent more problems. Her internal organs are floating around in this fluid and need to settle back into the correct positions slowly. They sent some of the fluid off to the lab, which will take 72 hours for the cultures to grow to look for bacteria, parasites, etc. We do know that there were no Leukemia cells in this fluid which is great! We also know that the fluid was clear and not cloudy or mucky which would be obvious infection. The Oncology Pathologist that was able to see no Leukemia cells in the sample only noted white and red blood cells.
Blood products were ordered: more FFP, cryoprecipitate, red cells to replace what keeps being taken for tests. A lot of volume but needed to keep her going.
Nadia is on three different antibiotics: Ampicillin, Gentamiacin, and Fentinol (attacks fungal infections). The Gent levels are checked often as this antibiotic can hurt the kidneys if abused.
Nadia’s belly catheter stopped draining last night at midnight. No one really knows why. It could be plugged, it could have cleared all the fluid in the immediate area, maybe some of her organs have shifted and blocked the tube. They don’t know. What we know is that her belly girth has continued to climb as has her weight (not good weight, weight from all the excess fluid). Her girth last night (Saturday) at 6:00 was 42.5 centimeters, which has continued to climb to 45 centimeters by 2:30 this afternoon. Nadia’s weight went from barely 5 pounds two weeks ago to 7 ¾ pounds yesterday morning to 9 ½ pounds this morning. Nadia’s breathing is good. Even though her belly girth is increasing, her respiratory compliance is good (they even adjusted her levels down today). They are watching and making sure she breathes 28 times per minute. They started her on Lasix, a med that pulls fluid from the limbs (which she responded wonderfully to) and Albumin, which pulls fluid from the blood vessels. These meds make her pee which at this time is her only output (she is getting out 20-25 ml per kilogram per hour, a healthy infant would only pee out 0.5-1 ml per kilogram per hour). She needed some potassium and some protein as well.
She is very swollen and puffy. Everyone here keeps telling us today to wait and watch for the next step. Surgery does not want to adjust/remove the belly tube as they don’t feel it is worth the risk since she is breathing so well. They will wait for her INR levels to level out so she can clot. They feel like it is a victory that she is maintaining her success following such a rapid decline the night before last. They did take her off the sedation to make sure she would respond on her own, she did wake up a bit and even opened her eyes. She is exhausted from her ordeal and from the Chemo but is still feisty enough to wiggle around when she’s being messed with which is exactly what we all wanted to see. They believe that her body may just absorb the excess fluid on its own.