ILM ~ James "J.W." Mount

About me:

Our son James William (J.W.) Mount was born April 23, 2004 he weighed 7lb and was 20in. long. When he was born we had no idea that anything was really wrong with our son, the only problem we knew he had was a slow heart beat and the doctor told us he always would even as he grew older. We noticed J.W. had a sucking problem, he wouldn't eat a 3oz. bottle at all. They put him into the NICU for a week until they could get him to drink a 3oz bottle within a half hour. After we brought him home he started losing weight. The doctor (Dr. Devanesan) didn't think we was feeding him right because I told him that it took J.W. an hour to eat a ounce so he admitted him into the hospital to monitor his feeding's. He seen we was feeding him right and that it did take a while to feed him anything, J.W. put on 2oz. so the doctor let him go home. Three weeks later we took J.W. back to see Dr. D. and again he said J.W. lost more weight, he sent us to the hospital for blood work and then told us to come back to his office, after we got back to the office he again wanted to put him back in the hospital to monitor his feedings, I got really upset and told him he wasn't going to put my son back in that hospital for anything, I fed my son. We decided to grab J.W. and leave, as we got out to the parking lot the doctor ran out and said he found the problem, J.W.'s liver enzymes were triple the amount they should have been. Dr. D. told us that we couldn't leave the doctor's with him having liver problems. He admitted us into the hospital and then transferred us to a bigger hospital the next day. After getting transferred to Palms West Hospital we found out that J.W. had several problems that hadn't been discovered at birth. They did a liver biopsy and the doctor said that J.W.'s liver did not look good at all. They sent the biopsy to Miami and they said he didn't have Allagile's Syndrome (which they thought). The doctors ran a number of test on J.W., checked everything from head to toe "Thank God". They did a reflux test and said he had reflux severe so they wanted to do a flundoplication (stomach wrap). After that test they did a swallowing test and found out that J.W.'s upper palate was as long as his esophagus and the whole time he could have aspirated while eating, that he couldn't hardly swallow do to it. They got him right into surgery for a feeding tube and the fludo. to be done. After the surgeon opened him up he said that J.W.'s liver was so enlarged that he had to cut a big piece off from it and move it out of his way to perform his procedure, he also said it was like brownish-red in color and looked horrible. The next thing they found out was that J.W. had the PDA of the heart that never closed, he was also having low heart rates when he slept, going apnea. They planned on doing the catherization to his heart when he was 6 months old and/or bigger, but it never got done because he never really got bigger. They sent the second biopsy to Miami as well, this time they said he had Allagile's and that only 30% of his liver bile ducts worked. The GI doctor (Dr. Hernandez) looked at the biopsy when it came back to Palms West, he said I do agree that only 30% of the bile ducts work but I don't agree that he has Allagile's, so we were racking our brains hoping he didn't have it but still having our droughts (now we wish that he would have had that and not what he had). Dr. Hernandez told us that he wanted to send his biopsy's, blood, and photo's of all our faces to CHOP so Nancy Spinner could do research to see if it was or wasn't Allagile's. He still wasn't gaining weight so every one was puzzled on what we could do to help him without knowing what he had for sure. Three months later, late September, Nancy Spinner called me personally before speaking to the doctor's and said J.W. didn't have Allagile's that he did not even meet criteria for the Syndrome. We said then what does he have, it killed us when she said she didn't know. Here we were still hopeless on helping our son, all we knew was that J.W. wasn't gaining 1oz. and when he did he would lose 2oz. every time. We took J.W. to get the result's of his 24hr. EEG and the doctor couldn't read the results but yet told us that J.W. was fine. I went to see Dr. D. and told him that I wanted a second opinion on J.W.'s brain. He sent us to Miami Children Hospital with the MRI films, the doctor took one look at the films and said go see the Genetic doctor (Dr. Jayakar) now, we went straight to her office, we talked over our situation with her, she said ok let's get blood and urine and see what we can come up. In the mean time J.W.'s eyes started jumping, they called it "dancing eyes." We seen two different eye doctor's and they both said he had red spot's all over in his retina, that he would be almost if not completely blind by the time he was about a year old. Here we was with our son having a bad liver, PDA and and another hole of the heart, going apnea, almost blind, fatigue, and he couldn't hold his head up hardly, he didn't have any muscle tone, and his spleen was also enlarging. Dr. Jayakar got her result's back and called Dr. D. and told him that she thought that J.W. had a Peroxosmal Disorder. Dr. D. called me and told me what she said and I didn't know what kind of disorder it was because I never heard of it. I turned around and called him right back and asked him "how do you spell the disorder?" he told me but asked me not to look it up because they wasn't 100% sure and didn't know which one he would have if any. Like any parent I didn't listen to him, I jumped right online and checked it out. I always wondered where his space between his eyes came from (that was one thing I said right after I held him at his birth because it was a little strange). As soon as I read up on Zellweger's and the other disorder's I told his Mami (grandma) I was scared because J.W. had a lot of the effects of the worst one "Zellweger's" and that the life time wasn't very good. In Feb. 2005 I went to change J.W.'s diaper and when I opened the diaper it was filled with blood. I called Dr. D. and he said bring him now and he met us at the hospital By the time they got us to the back (in a matter of 2 minutes) J.W. already filled another diaper with blood. They did blood work and said he needed a blood transfusion, so they started giving him blood, the doctor came in and told us that we needed to be admitted and that Palms West had no beds, I told him I didn't care what he had to do but we was not staying in that hospital (it is only a hospital for mainly the elderly, they do not deliver babies or anything) D. D. went back out the room and came back in and said that we could go to Miami but we would have to wait until the next day because we had no way to transport him that late, I told him I didn't care how we got there but we was going if we had to take him ourselves, he walked back out and right back in and said he was having J.W. airlifted out to Miami Children Hospital to the PICU until we found out what was causing the bleeding. After getting to Miami J.W. had to have a platelet transfusion. His bleeding stopped so they moved us to ground floor, the GI doctor from M.C.H. said that he would have to go down J.W.'S throat with a scope to see where the bleeding came from. They had to give him platelets and plasma transfusions before they could perform the procedure, next his blood sugar was going wacky so we had to postpone his test. When they finally got to go down with the scope the Dr. said that J.W. had varicose veins (exploding) due from his liver and his spleen being bad. We talked about putting a shunt in to bypass the spleen. After that it was like everyone was giving up on him, I got upset and walked down to Dr. Jayahar's office and sat there until she could talk to me. Dr. Jayakar took me to a room and asked me what she could do for me? I told her how I felt and asked her if she knew why everyone was acting like they were not interested in helping us anymore (the GI doc. already signed off from him), she told me it was because there was nothing that could be done for him and I lost it. I had to go upstairs and tell my husband that there was nothing to be done for our son, that was so hard! Dr. Jayakar came to our room and told us that we were going to have a meeting with all of the brain doc. at MCH and her that Friday, she also said they wanted to do another MRI of the brain to see if any changes were made since the first MRI, we told her ok because we still were not being told what J.W. had but deep down I knew what it was. The Hematologist came in and told us that he thought that J.W. had a severe bleeding disorder and that he wanted to send some blood to Wisconsin, we let him send the blood out, and he told us that if they put J.W. asleep to do the MRI then J.W. may never come out of his sleep so we told them they couldn't do it. At the meeting they told us our our biggest fear's, they talked about the way he looked, his eye's, chin, ears, forehead, the whole nine yards. Then they told us that his life time wasn't long, I asked them how long was their time limit, they told us to wait outside while the team of doctor's talked. Us getting nerved up, the doc. finally walked out and said J.W. has Zellweger's and only had a very little life time left, she said the life time is from 6 months old to 1 year old. I was in shock and nothing seemed to be registering, I looked at her and said so are you telling us our son is dieing and she said yes. J.W. was already 9 months old at that time. They got J.W. a 24hr. feeding pump and sent us home within 2 day's. After we got home Hope Hospice stepped in and Dr. D. continued seeing J.W., after I fought him not to give up on us. J.W. made it up to 11lbs. 3oz. then went back to 10lbs. 9oz. before he passed away. The last 2 months of his little life was really tough. One day we heard he was having heart failure the next day we was told he wasn't. We noticed his body changing and his eye's looking worse. The day he passed away Michael had a Dr. appointment with Dr. D., we left J.W. with his Aunt and when we got home J.W. was screaming and we couldn't calm him down. I called the nurse (Monica) to come out, she called the Hospice Dr. (Dr. Longmore) and told him about J.W., while she was on the phone with him I noticed that J.W.'s spleen was bulging out of his side and we never seen that before. Dr. Longmore told us to take J.W. to Dr. D's office to let him take a look at him, after we got there Dr. D. could see that J.W. was in a lot of pain. He sent us to have x-rays done and then told us to come back to his office, when we got back to his office he said that the results were good, he said he thought of another x-ray that needed to be done and he thought it was a good idea to check his blood, so we went back to the hospital and we drawled blood first because his blood sucker "Angela" was already there waiting on us, J.W.'s veins were so bad and his arms were to small for her to even touch, she called Dr. D. and told him she refused to draw his blood, he told her to do a heal stick. While she was doing that J.W. passed out, I figured he was just tired from all the screaming because he never cried hardly at all. When we took him to get the other x-ray J.W. just rolled out of my arms and didn't move, I knew something wasn't right at all, I picked him up after the test and handed him to his Mami and said somethings not right at all. While she was walking out of the hospital J.W. started shaking really bad and his eyes were wide open, we never seen anything like it so we ran to the doctor's office. The doctors ran, grabbed J.W. and started oxygen on him, and ran all kinds of test because Dr. D. was at the hospital looking at the x-rays. They called him and he ran over and told us that he believed that J.W. had a seizure and said "Jaimie he's dying and we can't help him anymore this is it". He called the hospital and they said his blood sugar was only 13 so he said not even to feed him anymore because his liver and spleen were shutting down soon. We took him to the hospital because he wouldn't let us bring him home like we had planned for him to be when the time came. After we got to the hospital he started to have more and more seizures, they gave him Morphine and said if you feel he needs more let us know, the nurse never made it out the door he started to seize again...I told her to come back here and give him more Morphine now...I couldn't set there and hold my son in my arms and let him continue to do what he was doing and being in the pain he was in. The nurse gave him more Morphine and he rested comfortably until he took his finale breath's and God took his little Angel home to the heaven's above. J.W. passed away 3 days before his first birthday with Liver Failure, Heart Failure, Hypoglycemia, and Zellweger's Syndrome.