Friday, March 14, 2008
On Tuesday I took Chet in for counts. I could tell he was looking a bit low, and wanted to make sure it was safe for him to try to earn his swimming merit badges that night. His platelets were at 5,000, and his other lines were down as well. The arrangements were made to take him to get the platelets, and since we thought that was all we would be doing we just got in the car and left thinking we would be home in a few hours. From now on, we are going to have an overnight bag ready to go at a moment's notice.
He was given his platelets, and we were on our way home surprisingly early. He was released at 11:30 pm. We were halfway home when he began to tell me his stomach was hurting, and it was getting worse. Thombrosis (clotting in the stomach and brain) is a problem for PNH patients, so I immediately called the doctor on call at St. Jude who told me to get him to an ER to see a pediatrician. I called his local doctor to see which ER I should go to since we were about the same distance from the one we came from and the one closest to our house. He sent me back to the city, and we got back about 12:30am. My cell phone battery had died, so I couldn't call ahead to the ER to tell them what was happening and that we were on the way back. (The next purchase I need to make is a car-charger)
The rest of that night was spent in the ER. Chet had had a pancreatic attack, which was causing the severe pain. He had bright red blood in his urine (which is another problem from the PNH... it means he was experiencing an increase in the breakdown of his red cells. There is a medical term for it I cannot think of right now.) He was given morphine, and he was finally able to sleep, and hooked back up to an IV. I have no complaints about the doctors and nurses there, and this isn't meant as a complaint, but since this is such a rare disease we spent quite a bit of time that night sharing what we did know about this disease with the doctors there.
At 6 am he was admitted to the hospital and put in a room. Jack had already gone home to make sure the kids were ready for school, and Chet and I went to sleep for a couple of hours. By the time we woke up he was no longer in pain, and the blood in his urine was dark brown instead of bright red. The doctos at St, Jude asked the doctors there to have an ultrasound done to check for clotting, and that showed everything was normal. (I told him that the good news is that he isn't pregnant.... he giggled at that.) By the end of the day he was eating again and his urine was clear. We thought we were on a fast track home. Then the doctors said they wanted to see what his counts were in the morning to make sure his liver and pancreas were completely normal.
So the next morning the counts were taken and his platelets were falling extremely rapidly. His other bloodlines were falling too. His red cells and white cells were very low, and the doctor said if he didn't know he had PNH he would think he had developed severe aplastic anemia again. He called the doctors down at St. Jude again to see if he should go ahead and give him more platelets, or possibly whole blood, and his doctors decided they wanted him down here for evaluation. (His doctors and nurses were rather fascinated by him there... they don't treat very many patients with such rare diseases, and he looks so perfectly healthy as well as being such a calm patient that I think he really won some of them over.)
An ambulance was ordered, and within an hour we were on the way. I tried to follow the ambulance, but once we hit the freeways I never saw them again. We had made arrangements to meet Jack in Conway, and he brought Beth and some changes of clothing to me there. Again we got on the freeway, and finished heading into Memphis, and again I never saw the ambulance again. I got pulled over just outside of WestMemphis, but when I let him know why I was driving so fast the officer let me go. He even told me that he had seen the ambulance and I wasn't too far behind.
After arriving, I found Chet in the treatment room, and his doctor came and did an evaluation. Then they taught me how to flush his IV line. I have never wanted to be a medical practitioner of any sort, but I have been receiving a lot of new medical knowledge this last month. After another set of counts showed he wasn't in any immediate trouble, we were sent to the family hotel to sleep. Oh heaven...real beds.
Today we went in for the first set of testing, and his counts are still falling. His platelets fell another 3,000 in just the few hours between being discharged last night and the early am appt today. I don't know yet what the PNH test level results were.
Chet has had some terrible IV experiences the last couple of weeks, so a ChildLife specialist came in and talked to him about it for a while. She brought an IV doll and went over the entire procedure with him, explaining every step. Then she taught him how to try to relax and be calm through the process, and gave him a "coping bag" for his next IV stick. It is full of stress balls, a 20 Questions game, and a pinwheel to focus breathing with. I think she did a great job, and he seems to feel better about the idea of having to have IVs. Beth took the little IV class with him. I have some pictures I will upload later when I can get them loaded.
We are going to be here until at least Tuesday. There are several more tests being planned, and the results of those will determine what happens next.There has been talk a couple of times about the possibility of seeing if it is time to have the marrow transplant. Chet feels fine, though, and looks good, so that makes all of this lot easier to handle. Jack and the kids wanted to come down last night for the weekend, but there have been a lot of thunderstorms with tornado warnings there and here. If we are still here next weekend they will try to come down, but we are hoping to be home by then.
Tomorrow is a free day as far as testing goes, but I think we are going to stick close by. He is still getting fatigued extremely quickly and his immune system is down, so there is no reason to take any risks that can cause him more problems. My phone should be working tomorrow since we won't be in the actual hospital building, so feel free to call if you need to. I will post when I hear more.
Thank you to everyone who comes together to help out in so many ways when these crisis times come. We are so grateful for all of you.
Tuesday, March 4, 2008
Unfortunately, his medical issues have caused him to exceed the allowed amount of abscences. I have to go tomorrow to take care of more paperwork as far as that is concerned, and see what can be done to try to keep him on track as much as possible in school. He is so happy about going back to school. Let's hope he stays on a healthier track and doesn't have to miss anymore for the rest of this semester.
I think I have the major points covered in updating this blog. Now I should be able to just come and post updates as things happen. We are going back in for new counts next Wednesday, and will be watching this week for anymore unwelcome symptoms. Chet wants to be able to go earn his swimming badge in Boy Scouts next week, so hopefully this upward trend continues to let him do that. One of the badges requires swimming 300 meters, and he is worried he will get too tired before he can complete it. So if you are praying for him this week, will you please add in a little request for an extra boost of stamina and endurance to let him complete this task? I know it would mean an awful lot to him.
The worst part of the morning was Chet getting his IV. His internal port was removed when he was four, and he doesn't remember the big pokes, and for the first time through any of this he really cried. He had an allergic reaction to the tape, and he is so sensitive to pain that he never got used to the IV being there. For him, the best part of the day was getting it taken out. Along with the blood tests, he was admitted to the surgical outpatient to have a bone marrow aspiration and bone biopsy. I was able to stay with him until the anaesthetic put him to sleep. The very first thing he said when he woke up was, "Can you take this IV out now?"
Because a bone marrow aspiration makes your hip sore, he rode in a wheelchair out of the hospital. Target was throwing a big Valentine's Party for all the kids and Chet wanted to go see what they were doing, so we stopped there on the way across the parking lot back to the hotel. He stayed in the chair for the most part, but when Beth asked him to take a picture with her he stood up, put on the silly red hat, and smiled for the camera. I thought that was really sweet of him. We didn't stay very long because the pain killers were wearing off and he wanted to be back in the room.
The doctors called us and asked us to come back to the clinic that afternoon. The preliminary results were back, and they found that Chet had developed PNH. He is no longer considered an aplastic anemia patient, and unfortunately the PNH is even rarer and more difficult to cure than the aplastic anemia. Worse, it has more life-threatening manifestations (blood-clots), and because it is so rare there isn't a lot of research on this disease. The biggest paper ever written on it is only 6 pages long. The worst thing about diagnosing a new disease is that the doctors have to tell you every worst possible scenario. I was given a lot of frightening information that afternoon, but the thing the doctors emphasized over and over is that although there are some scary aspects of this disease, Chet's case will be handled according to how it develops in him. At this point 20 percent of his cells are affected by the PNH, and although he has begun to develop jaundice which is typical in PNH patients, it doesn't show up physically in his skintone or eyes yet. On Friday, we went back and they confirmed that he doesn't have any cancers right now. At this point they plan on having bone marrow aspirations at least every six months, check-ups in the clinic at least every three months, monthly counts here locally, and ultra-observance to watch for blood in his urine, headaches or stomachaches that can indicate blood-clotting, and increased bleeding and petichaei.
We didn't have any appointments on the 14th while we waited for the rest of the results, so we went to Graceland. (Chet and Matt are both huge Elvis fans.) Graceland gives free admission to St. Jude patients and their families, and they are only ten miles from the hospital, so it seemed like an ideal way to spend the day. Chet, Beth and I had a lot of fun there, and it was a nice way to take our minds off the stress of the trip to Memphis. Chet wants to go back again sometime, and he wants to go see Sun Studio where Elvis recorded his first albums. I am so glad there are interesting things we can go do during the stays down there. It makes it easier to deal with the medical issues.
Here are some photos from Graceland.
The next morning we met Chet's doctors, and they did their initial examination and reviewed his case history with us. After counts, we found out that his platelets had dropped to 22,000, which was the lowest they had been in over 8 years. When the doctor told us that, our hearts just stopped a bit. His immune system was really low, so the doctor told us to keep him home from school until his next appointment to give it a chance to recover. Then they told us that they wanted to bring him back the following week to have a bone marrow biopsy (which we expected) but they also wanted to run some tests to see if he had developed another disease. They told us that they wanted to check for leukemia, MSD, and Fanconi Anemia. We knew it probably wasn't the Fanconi since he had previously been tested for that, and we knew there was a chance that aplastic patients can develop MSD, and we knew there was a chance for leukemia, but it is still a terrifying feeling when doctors run off a list of tests for diseases that they want to exclude.
Here are some pictures from that first trip.
Chet and the St. Jude statue. I hate that he has to start a new treatment regime, but I am so grateful, beyond words, that he can go to St. Jude for treatment. They are every wonderful thing you have ever heard about them.
Here he is playing a puzzle game at the Cracker Barrel. We stopped there to eat on the way to enjoy some time together and to let him play with the checkers they have there. You can tell by the look on his face that he wasn't feeling great, but that didn't stop him from trying to beat that game over and over!
He and I decided to play with taking some pictures together in the hotel room. Chet loves the hotel rooms. He loves sleeping in the beds, being able to play in the arcade they have for the kids, and he adores having an unlimited supply of Mountain Dew.
I took a shot of the Welcome to Tennessee sign. It hangs right over the bridge that crosses the Mississippi River, and he really enjoyed seeing the river itself. The next time we go down he wants to go ride the steamboat on the Mississippi.
Chet was diagnosed with severe aplastic anemia at 2.5 years old. It was devastating, but as time went on we learned to deal with things as we needed to. We knew the limits and guidelines - not enough platelets? no rough housing and avoid bruising - not enough red cells? boost the iron intake, eat extra veggies, and rest - not enough white cells? avoid crowds, wash your hands even more often than normal, take airborne. We got used to living with aplastic anemia, and although he wasn't really cured (his platelet counts never did return to normal) we found that we could all find a way to live with this disease until he was either cured or had a remission.
In June of last year, we took him back in for yearly counts, and realized that he had lost over half of the platelets he had maintained for several years. The doctors here began to watch his counts more regularly, and within a few months it became apparent that his counts were rising and falling in an irregular pattern. He still hadn't become so low that he needed transfusions, but it was obvious that something serious was happening again. In December he was referred to St. Jude Children's Research Hospital. When we got the phone call that all of the arrangements had finally been made and his appointment was set, the reality really began to sink in deeply. Once again our little guy was really sick, but we had no idea what was ahead.