Max and I left for CHOP at 5:30 am and got there a little bit before 7:30 am. We beat everyone to the oncology floor but Max found a book and played with the Thomas the Train set that they have setup. We met with the nurses in the oncology unit and talked briefly about what would be happening. There was some confusion as to how Max would get today’s chemo treatment but we got setup so that they drew a little blood from his middle finger with just a prick test and then we headed down to the Pediatric Outpatient facility to have his port installed. Max got his does of Versed at about 9:20 am which made him very thirsty but he was able to focus on Dora and Blue’s Clues until about 9:35 am when they started the IV in his hand. He whimpered a little bit but he really didn’t think it was too big of a deal.
The surgeon came in and explained how the port would work. His explanation was very helpful. He explained that they would make two incisions, one on his chest below the collar bone and another by the jugular vein in his neck. The run the line from the lower incision up to his neck and then back down to a vein by his heart. The port will be on the right side of his chest (of course, I had him practice with a dressing of gauze and first aid tape on the left side!). The bump is very small. A woman whose daughter is sharing the recovery room with Max has breast cancer and she was willing to show us her scar and the bump. The scar is actually more noticeable. The doctor explained that the port is NOT permanent but could typically be left in place for a year or longer. They would not leave it in forever because of the risk of infection but if he needs it for an extended period they may have to pull the existing port and put a new one in later. Time will tell.
The risks involved in today’s port placement are infection, damage to a vein, an air embolism. The doctor explained the steps that they took to minimize these risks by using a sterile techniques, an ultrasound and fluoroscopy to guide them inside Max’ chest, and extremely small needles.
Max was taken down the hall just before 10:00 am is expected back in about an hour. They believe it will take about an hour for him to wake up and then we will head up to oncology for his first chemo treatments. So far he has been a real trooper. He actually thought getting his finger pricked to draw blood earlier was a little ticklish.
Max didn’t come back until 11:40 am. They said everything went fine. He was still asleep but stirred when they took some of the tape off of him and hooked him up to the monitoring equipment in recovery. He went right back to sleep though. The dressing by his neck is fairly small. The nurse said that he has some liquid skin and some butterfly bandages there. He’ll need to keep the dressing on for 2 days and leave the butterflies on until they fall off. She said it was okay to leave the dressing on longer if he is likely to pull the band-aids off. The dressing where the port was placed is somewhat larger and will need to remain in place for 7-10 days. That has internal stitches which will self dissolve. The nurse said he is pretty thin so there will be somewhat of a bump in his skin. She also said that the numbing cream will make it less painful for the needle stick but that he’ll still feel it go in. Right now he is sleeping and they expect him to wake in about an hour when we’ll take him back to oncology to start his treatments. They said that we can use a wheelchair for that trip because he’ll still be groggy.
Max finally woke up enough to drink a juice box and eat some cheerios at 1:30 pm. It took a while to get him unhooked from the IV and get him dressed. He wanted to make sure he was going to see the metal ball display in the lobby and play with toys but he is essentially completely drunk. We got him a wheelchair to come back to oncology and met with Dr. Belasco to discuss some things before treatment. She said that any hair loss would be temporary. She also corrected a misunderstanding about deep tendon reflexes – those problems will reverse themselves once treatment stops. We’re supposed to call them with any issues at all so that they can keep Max free from any issues related to his treatment so that he does not start to see it as a problem. This week he has been asleep but in the future he should be able to play with things here.
Dr. Belasco said she had consulted with a neuro-psychologist and it was her opinion that he should have a full neuro-psych profile because he may not have Aspergers but something related to the NF-1 which is very commonly associated with learning disabilities, social problems, etc. Much like Aspergers. She said to try first with the school but that CHOP could do it if need be. It needs to be done by someone familiar with all of the diagnoses and would different than the normal school test which is geared towards IQ and attention problems.
Dr. Belasco also suggested contacting the Commission for the Blind which is a lot like Early Intervention but geared towards addressing Max’ visual problems. They would be able to make suggestions that would improve Max’ learning at school This could be as simple as where the teacher should stand in relation to Max to getting him any mechanical aids that he needs. She thought that they might suggest that he learn braile as a second language – if only to help him educationally since he already has exposure to Spanish. That was just a thought that she had, she said that they would be the best source of information and is yet another resource that we can try to tap early for Max before it becomes an issue. Her greatest concern educationally was that he continue with special instruction for Aspergers but finds out later that something else is the cause. She said it is not urgent but the neuro-psych evaluation should be done.
They gave me a sample of the cream that will be used to numb his port and told me that it could be put on in the parking garage. It only needs 15-20 minutes to be effective. There could be an issue with skin irritation if it is left on for longer than 90 minutes. If we forget, there is a spray that can be used right before hand that works immediately.
Today has gone pretty well so far. We had an early appointment so we tried to leave by 6:30 am but didn’t really leave until just before 7:00 am. We hit some pretty major traffic just North of Philly but still got here at 9:30 am so it wasn’t too bad. We were seen pretty quickly and we appear to on track for getting lunch at the Franklin Institute.
I talked to Dr. Belasco about the newest findings in Max’ MRI. We looked at several views of the brain and I think I wrote down the right numbers so that I can share the same images with anyone that wants to see. Anyway, it turns out that the optic gliomas pretty much run into the hypothalamus and pons (part of the brain stem). In the MRI it almost looks like one big glioma. The optic gliomas only affect vision. The other gliomas are more of a concern because they are in the part of his brain that controls many different body functions. The hypothalamus could affect the thyroid, growth, metabolism, emotions, learning, memory, hunger and other ‘regulators’ that would control obsessive compulsive behaviors, temper, etc. The pons tumors are in the part of the brain stem that controls all upper body movement (eyes, lips, mouth, face, etc.). There is also a large occurrence of spongiform in the thalamus. According to Dr. Belasco, it is not possible to tell the effect of spongiform (she explained spongiform as similar to the spots on your skin – there but often not an issue) so it will be monitored but it will be more important to watch Max for changes In essence, many of the Asperger symptoms that Max has could have been caused by the tumors – there is no way to know how long they have been there. So from early on when he did not make appropriate eye contact, took longer than normal to ‘learn’ social skills, etc. could all be related to NF-1. For now, there is no change in his treatment as what he doing should be effective for all of the tumors. The major change will be in monitoring his condition which will become a life long effort because the tumors in the hypothalamus and pons are not self-regulating with age. The good news is that there are a number of cases of NF-1 patients having their tumors disappear between MRI’s.
They had run some endocrine blood tests after his last MRI and Dr. Belasco said those results were all normal. His blood counts are also well into the normal range although they are declining slightly due to his treatments. Max did not lose any weight since last week – that is first time since he started treatment so that is good news. To be clear though, Max had only lost about 2 pounds which they said was not a cause for concern and was very normal for his course of treatment.
For the next two weeks, Max does not have to take the carboplatin so his treatments will be an hour shorter. That is somewhat of a bonus because he’ll only be hooked up to the IV for 30 minutes or so while they draw blood, run some tests and give him 15 minutes worth of medicine.
Today has gone pretty well. We found out last week that Max has lost his reflexes in his legs and has a slight droopiness to his eyes. These are both side effects of the Vincristine drug that he is receiving. They fully expect that his reflexes will come back when he done with all of his treatments - Nov 2005. They may come back during his 3 week down times but that isn't as common. Anyway, there is nothing that gets done now to try and get his reflexes back but we are to watch Max more closely for trouble with his feet and legs. His strength in his legs is still fine so the folks at CHOP are not concerned. We're to watch for changes in the way that he walks or problems with stairs. Should those problems arise, they'll alter his levels of Vincristine. The fact that Max no longer has jaw pain when he eats is a good sign that might be related. His blood counts have all been doing pretty well. Certainly there have been significant drops because of the chemo, but nothing to be concerned about yet. Actually, we've been told that there is still a lot of cushion before we get to levels that we would be a concern. Max has lost about 1 pound overall and has maintained a steady 22.3 kg for the past 3 weeks.
Max still has signs of thrush even though he's been taking an oral medication for almost 2 weeks now. The nurse-practitioner has prescribed something stronger instead too make sure it goes away.
Today the trick of the whole process was explained to Bob by Cindy, Max's nurse today. Basically, after we see the Dr/Nurse-Practitioner, we should go have lunch because it takes a while to make the drugs. In fact, they take their time making the drugs because they assume we are going to lunch. The nurse assumed that we knew this. Everyone else assumed, but I end up feeling like the ass - hardly seems fair - but at least now I know. Max and I got lunch and his meds were ready when we returned. He is napping now and we'll probably sleep through the entire treatment. He is to get his flu shot today so that will probably wake him up because they can't give that to him through his port - it is a 'real' shot. Can't imagine that will be fun, but maybe that will take his mind off the tape coming off - he hates that more than you could probably imagine.
Two more weeks to go before Max gets his first 3 week break. We have appointments 10/19 at 10:30 am and 10/26 at 9:30 am. Max will have off from then until 11/9 when he and Bob will go back to CHOP for a hearing test (just to be safe - no signs that anything is wrong) and a follow up MRI. We'll be taking a family vacation from 11/13 until 11/20 and then Max will start his first round of maintenance 11/23. That will go for 4 weeks. He'll get about 7 or 8 follow ups which are 4 week sessions. There is a 3 week break between each. That would seem to put us into November 2005.
Today is Max's last day of the induction phase. We got up at 5:30 am and left at 6:20 am. Traffic wasn't a problem until we got to Philly but we were here at 8:30 so we made great time. Max started his meds at about 11:30 so we should be out of here by 1:00 pm. Max has been asleep since shortly after getting hooked up but he was up early so I let him rest. Besides, it is embarassing to have him yell "What is your problem, I was sleeping" and "Sorry doesn't cut it mister" with 15 other parents/patients sitting in close proximity.
Max still has a bit of Thrush so he has a new, stronger medicine to take when we get home. They think this will knock it out for a while.
They gave me a number to call in Orlando for the clinic there and they also gave me forms for his records. We have canceled the visit for Max's MRI reading (his MRI is 11/9) and we'll go over it on 11/23 when Max starts his first round of maintenance. Barbara Ann, the nurse, told me that I could call here on 11/11 and she'll fax me the radiology report for 11/9 so that we'll have it in Orlando should they need anything. Max shouldn't have to go the clinic in Orlando, but they have asked to know when patients are in the area so that they are better prepared in the event that Max runs a fever and needs to be seen.
On Tuesday, November 23, Bob, Tracy, Cindy and Max met with Max’s doctors to go over what to do next for Max. First, it is important to stress that while Max’s situation did get a little worse he is not in any graver danger than he was over the summer. Dr. Belasco stressed that it is important to hope (and it is reasonable to believe) that the current tumor started in the optic chiasm because in kids with NF-1 it is very common for the body to regain control of the growth of this type of tumor around the time that puberty starts.
In going over his most recent MRI we confirmed that the tumor is inoperable. We also learned that radiation would be a last resort because in addition to destroying the tumor, functional brain cells would also be destroyed and there is no telling exactly the function of those cells but they are in the part of the brain that involves the optic chiasm where vision is transmitted from the eyes to the part that interprets what is being seen. There is also tumor throughout the hypothalamus which is where a lot of processing of information is done, autonomic processing is done, and short term memory is handled along with several other functions. We learned the tumor continued to grow during Max’s most recent chemo treatments and that means that the treatment he was receiving was ineffective for Max. On the bright side, the growth was relatively small.
One option would be to discontinue Max’s treatments entirely. While his tumor is slow growing it is in parts of the brain where even minor changes could pose very large problems for Max. A large concern is Max’s vision. Without further treatment the Dr. felt that Max’s sight would continue to deteriorate and he would likely eventually become blind. In addition, further growth in the hypothalamus would likely start to affect his ability to learn and to process what is going on around him. This could lead to loss of control of his emotions (anger being a big issue since Max is already quick to anger), appetite control problems that could lead to severe obesity, and dementia. The tumor also surrounds major arteries and any interruption of blood flow would result in a stroke. There is no telling if the stroke would cause minimal, profound, or life ending damage. Finally, the tumor is also around the ventricles (‘drains’ for brain fluid) and if they are affected it could lead to a build up of fluid in the brain which would cause further problems for Max. These are all felt to be somewhat likely if treatment were to be discontinued. However, even with treatment, these are all possible.
Another option is to change Max’s chemo to a drug called Temodar. It is believed that Temodar is 65% effective (the previous chemo is known to be 70% effective with no life threatening side effects). The most concerning side effect of Temodar is leukemia. There are currently no statistics available for the risk of developing leukemia as Temodar has previously been used to treat terminal adults who did not live long enough to know the long term side effects. The Dr. told us that the risk is less than 10% but is thought to be 1-3 %. If leukemia occurs, it is not likely to do so for 2-3 years after starting treatment. It could happen anytime after that but we were told that after 8-10 years the chances were far smaller. The problem is that if Max does develop leukemia it would be fatal unless he able to get a successful bone marrow transplant. We didn’t go into the details of that but it sounds like those stats are not in his favor.
The nice thing about Temodar is that it is an oral medication so Max can take it at home. Treatments would be on a 4 week cycle. Days 1-5 Max would take Zofran (to handle nausea) at least an hour after dinner. 30 minutes later he would drink 3-4 ounces of apple juice mixed with the Temodar. At the end of the third week, Max would go for a blood test somewhere local. At the end of the fourth week Max would return to CHOP for another blood test and a new set of meds for days 1-5 of the next cycle. Treatment would last for 1 year. If Max’s red blood cell count or platelet counts drop too low, his dosage might be reduced and he might also need a transfusion. The transfusion would have to come from non-family members as there is a chance that Max could develop antigens to the blood donor which would then rule that person out as a possible bone marrow donor if that becomes necessary later. Transfusions could all be done through Max’s port though they would probably be done at CHOP and take many hours to complete though they are seen as somewhat routine. I’ve put a complete list of side effects in the Medicines section.
In order to participate in a current study of Temodar’s effectiveness on low grade gliomas, Max will need to have an MRI, EKG, Chest X-Ray and blood work prior to starting treatments. The study is being sponsored by Duke University. Participating in the study only offers the ability for others to gain a statistical insight into the effectiveness of this treatment, there is no reduction in costs. We will need to talk to the pharmacist at CHOP to determine how to get Oxford to cover the expense of the drug (about $2,000 a month).
Dr. Belasco was able to tell us that there are currently about 10 children that are part of the Temodar study at CHOP and some have been in long enough to be off the drug for a while and they have not seen any of the severe side effects and they have had success in stopping the growth of the tumors.
The second option available to Max is another chemo called PCTV. It is an acronym for 4 different drugs, some oral some intravenous. The risk of developing leukemia from this treatment is higher and effectiveness is believed to be about the same so we didn’t discuss this for very long. One thing that Cindy had learned from her own research was that Max wouldn’t be able to eat milk, bananas, cheese, yogurt and some other foods for 2 days each month. This would significantly impact his diet on those two days.
We have a follow up with Max’s neuro-ophthalmologist on Thursday, December 3. We’ll also be seeking a second opinion from another doctor. Right now we are likely to proceed with the Temodar but we wanted to get at least one more opinion before starting his treatment.
Note: After we met with CHOP, Max had a follow up eye exam where his vision was tested and appeared to have improved to 20/100 and 20/60 (used to be 20/250 and 20/100). We then made a trip to see the doctors at Robert Wood Johnson for a second opinion on the next course for Max. After that and another talk with his Dr. at CHOP, we decided to keep Max on his original protocol of Carboplatin and Vincristine. He started his first of 8 maintenance rounds on December 14 and will finish January 4. He has an MRI on Jan 11 and an eye exam Jan 13. We're hoping that things will remain stable and maybe even improve. If that is the case, Max will start another 4 week cycle on Jan 25 followed by another MRI and eye exam. If things don't go as planned, Max will probably start Temodar around Jan 25.
Today we met with Dr. Belasco and she brought in a neurologist, Dr. Sullivan, to look at Max. He concurred with her opinion that Max is not autistic. He and Dr. Belasco found a problem with Max seeing peripherally with his left eye (his bad eye). Dr. Belasco reviewed Max's Nov MRI with me because she has spoken with Dr. Pan from Robert Wood Johnson and wanted to explain where they are of different opinions. The review did not change her opinion that Max is ok continuing his original protocol of vincristine and carboplatin but she is stressing that it is important to have an MRI and follow up with the eye doctor during Max's 2 week break. Max is going to get chemo 12/21, 12/28 and 1/4 and has an eye doctor visit 1/13. I still have to schedule his MRI but that will likely be either 1/11 or 1/18. If Max vision is found to be deteriorating we'll need to look at switching to Temodar in an attempt to preserve his vision. For now, we'll just continue praying that things don't get worse and hope that they get better.
We were at CHOP for his third maintenance chemo. His blood work still looks good. Max hasn't been sleeping well at night and this could be a side effect of the Vincristine that he is taking. We're going to wait and see if it gets better over the next week - maybe it is just the excitement of Christmas and all the new toys. If not, we'll consider giving him something to get him to sleep through the night.
Max still has thrush so he is going to keep taking the medicine for that for one more week. He is also still not sleeping at night ; when I got him up at 5:30 he was already wide awake and had setup his blankets and pillows on the couch downstairs and returned to his room. Tracy gave him breakfast and found that he had also been into the cookie/fruit candy jar this morning. We're a little concerned about him sneaking down to eat before the MRI next week so they've suggested using Benadryl at night to see if that will help him sleep through. In case it doesn't work and he gets hyperactive or it works too well and he can't stay awake, we'll wait to try the first dose until Friday evening. Max has been very receptive to the sedation drugs so far so it will probably work ok for him. They've also suggested using it the morning of his MRI next Tuesday to keep him 'out of it' until after his MRI so that maybe he won't complain too much about not being able to eat. His last solid food has to be consumed by 4:45 am but he can have clear liquids until 10:45 am. Max's blood counts were better than last week (which were also good) so still no worries there.
The plan for next week is to get the MRI on Tuesday. Then call CHOP on Wednesday just to remind them that I'll be down Thursday so they can make sure they review his MRI Thursday morning. Thursday morning Max meets with his eye Dr. at 10:00 and then will stop by the clinic to see if anyone is available to quickly go over the MRI. If not, they'll call me in the car because I have to be back to work at 3:00 pm for a meeting.
Max had an MRI Tuesday and we were back today for an eye exam. Today his vision tested at 20/400 and 20/70. The left eye is significantly worse than other testing by the same doctor but matches what Dr. Chen found over the summer of 2004. In talking with the doctor after the tests it did not seem like Max was cooperating as well as he did during his last test. I told him that I had not noticed any significant change when Max reads the temperatures off the weather map in the mornings other than he reads double digit numbers correctly now (forty-four instead of four and four). As we were leaving he tried to test Max's peripheral vision in his left eye and Max did terribly mistaking 5 fingers for 1 even though he had moved his eye to stare directly at the Dr.'s hand. While it was disheartening, it really is where we thought Max was over the summer. Max seems to be doing quite well with the vision that he has so with the MRI findings, we'll be starting Max's chemo again Tuesday, January 25.
Today Max started his second round of maintenance and it will last 4 weeks. That'll be followed by an MRI and follow up eye exam. His dose of Vincristine has been cut in half for this round because he has had some pain in his left knee. He's going to start taking a prescription antihistamine at night to try and help his insomnia.
Max had a problem with limping at school yesterday and complained about neck pain at the same time. Today, his limp was pretty noticeable and he had a hard time jumping on just his right foot and could not jump on his left at all. They believe that this is related to the Vincristine so they didn't give him that drug today. He just started his Carboplatin so we should be able to leave by around 2:30 pm today.
They didn't see anything wrong with his neck and think it could be just dry skin or possibly the clothes he was wearing yesterday. We also talked about getting some of his meds at a facility closer to home. We could do this in New Brunswick at Robert Wood Johnson but another option that they mentioned is Lehigh Valley Hospital Muhlenberg at 2545 Shoemersville Rd in Bethlehem PA. They mentioned Lehigh Valley because it is a satellite and it would be easier for them to share their findings. I believe that they are the same distance from home but I have to confirm that still. Because of his problem with his legs though they want to keep him here for at least the next two weeks.
One thing that they mentioned that might be a problem later on is that he might stop tolerating the Carboplatin. If that does happen, he would have to get the treatments here because it would change from taking 1 hour to taking 8 or more hours. Ugh. Hopefully that won't happen.
Oh, they said it was okay for him to take over the counter medicine for gas and that possibly we could cut his dose of the laxative he has been taking. It will be nice to put a stop to some of the gas.
Max has had a cold this week so he had to wear a face mask today. That didn't go over so well because he believes that he can't smell with it on (he initially thought he couldn't breathe but he got past that). His blood work was good so they believe that whatever he has is viral and not bacterial. They tell me here that viral is good because bacterial could mean that there are much larger issues than a cough. Nice to get some good news I guess.
They also feel that Max's neuropathy (side effect of Vincristine causing his walk to be awkward and some tightness in his legs) is improving. They aren't going to give him Vincristine today or next week and try to let him get a lot better before they start it up again. A lot of kids don't make it this far before the dose has to be reduced and/or stopped so this isn't uncommon. Cindy was able to show us some stretching exercises that might help and also suggested an exercise trampoline to improve his leg strength and stamina.
It looks like we'll be getting out of here around 3:00 pm or so today.
Things today have gone quite well. There was very little traffic both on the road and here at the hospital. It isn't even noon and Max has already started his last med so we should be out of here by 1:30. That hasn't happened in quite a while.
Max's neuropathy is still getting better but they are going to wait until his next maintenance round begins to decide if he'll start Vincristine. The hope is that he will recover enough strength and flexibility to be able to better tolerate the Vincristine then. Max has an eye exam next Monday afternoon. He and I will stay in Philly that night because he as a 9 am MRI the next day. Max then has off until March 8 when he starts round 3 of maintenance. The first visit will be here in Philly that day but we expect to be going to Lehigh Valley (much closer with less traffic) starting March 15. His Dr. here will still be in charge and will continue to see his eye Dr. here and get the MRIs here but 3 weeks out of every 6 will (hopefully) be much shorter and easier.
Finally, for sleeping at night, they want us to give Max melatonin at night. It is an herbal sleep aid that they are okay with using but don't have much experience in dosing. We're going to start giving him an adult dose before bed (he was on half that) to see if he can sleep through the night. This morning, Max was already downstairs searching the toy room, in the dark, for a 1.5 inch copy of the Scooby Doo Mystery machine.
We had about 6 inches of wet heavy snow over night but the drive down here was one of the fastest yet. Probably a combination of the holiday and the weather keeping people off the roads. We didn't have to be down here until 3:30 pm so there was plenty of time to shovel out.
Max's eye exam today went really well. Dr. Liu feels that there is definite improvement in his right eye. Max's left eye remains at 20/400 but with his right eye he has improved to 20/60 and he got 7 out of 8 color plates. They dilated his eyes to get a better look inside and they found a type of freckle in the right eye. It won't affect his vision but it is a common trait of people with NF1.
The plan had been to go to McDonald's in the hospital for dinner and then to the hotel but Max was so vocal about not being able to see (the eye drops) that we couldn't stay in the McDonalds. Instead, Max watched a big exhibit with bouncing balls in the atrium while Bob looked up directions to the hotel (the directions I had were still at home on my Palm pilot). Max had some French fries but when I offered him a chicken McNugget he screamed that he told me only to get french fries and a toy. I stopped offering nuggets since it didn't seem worth it.
We drove over to the hotel which really could've been simpler had it been daylight. I'm now old enough to have trouble reading signs at night even with my glasses so the first wrong turn that we made was the wrong way into the Four Seasons Hotel entrance. No one was happy about that and then Max added his two cents when the cabbies started honking their horns. Max felt that I probably needed a map. On our third trip past the Four Seasons, Max again said that I should've brought a map and pointed to a WAWA store that he felt might be able to help me out. By that time I had figured out the one way streets and we got into the parking garage for our Wyndham Hotel. This is much nicer than the Sheraton that we stayed at last summer but this city strikes me as seedy. It is kind of like Worcester but at least back then I knew the city, I was only 18-21, and I didn't have anyone with me that couldn't fend for themselves. Also, any worries were probably tempered with a modicum of alcohol.
Anyway, the hotel really is pretty nice. We've already called home and said our good nights so we're pretty much set for the night. Hopefully Max will eat something but I think I'll be lucky to get a bagel into him. Tomorrow's MRI is either 9:00 or 9:30, can't remember which, but we'll be there plenty early since Max will be up at 6:00 or so. No breakfast tomorrow but we should be done by 10:30 or 11:00 and he can eat and drink on the way home. He won't be able to go to school but I'm hoping to get into work for half a day.
tIt turned out that 6:00 am or so was wishful thinking. Max first woke me up at 2:47 am. I know this because his first words were, "Look Dad, it is two seventy-four." I don't think he ever went back to sleep. I would make him get back into bed and I would wake up 20-30 minutes later with Max looking out the window (we were on the 20th floor so he had a good view) or dancing in front of the full length mirror on the bathroom door. Needless to say, we made it to CHOP with time to spare. Good thing because one of the MRI machines broke down. We suffered only a slight delay so Max was home by 1:00 and Bob got to finish the day at work. I heard back from Dr. Belasco that the MRI was stable so we'll be heading back to CHOP on March 8 to start round 3. Assuming we can get insurance worked out we'll be at Lehigh Valley from March 15 - 29.
Today we went over a lot with Dr. Belasco. Max's MRI appears to be stable and might be showing signs of some limited shrinkage (could only get a 'might'). I think they were hoping to see something more definite. They want to play things cautiously so we sort of met half way and agreed that Max will see his eye Dr. in 5 weeks but will NOT get an MRI. At that time he also have another audiogram to make sure there is no hearing loss (none is suspected but again they are erring on the side of caution). We agreed not to the MRI for three reasons: 1) There hasn't been any noticeable change since January and really even November, 2) There is always a risk to Max with sedation even though it is becoming somewhat routine (never thought I'd think that of an MRI), and 3) the cost is just phenomenal.
Max will get an MRI and a follow up eye exam the week of May 17. We'll likely repeat these every 12 weeks (3 months) until something more definite shows up.
Dr. Belasco was happy with Max continued improvement for his neuropathy (sensation problems mostly in his left leg but affecting him everywhere). The work with the exercise trampoline seems to be doing something more than just making him laugh so that is good. They are going to keep his vincristine (responsible for the neuropathy) at 1/2 dose for this round because they are worried it would affect him right away and they'd have to stop all together. I did learn that vincristine is used in almost all occurrences of brain tumors - not just NF-1 or tumors like Max has. It is unclear if the role it plays is as an additive (both fighting the tumor at some level) to the Carboplatin that he gets or as an enabler simply making the Carboplatin more effective. There is no way to test for Vincristine levels in the blood so they don't know if he has a big problem with it or if instead he just metabolizes it differently than other patients and it stays in his body longer. It is clear that it leaves the body via the stool so the constipation that he suffered from earlier certainly made any problems worse. Thankfully Max is now Mr. Regular so that worry is somewhat abated.
Next week we will start the first of 3 weeks of chemo at Lehigh Valley Hospital with Dr. Monteleone. Tracy is working on insurance coverage for the new location now but everything looks to be good to go. That means that next week Max can start the day at school and that we'll only have an hour drive instead of 2.5+.
On the way down here today it was raining but it was snowing by the time we came upstairs at 9:30 am. It is currently (2:10 pm) snowing harder but not sticking to the roads. It looks like we'll get out of here around 3:00 so hopefully the roads will stay clear. I bought a new street mapping program that came with a GPS last night and it was pretty cool on the way down. I knew the way so I was really just looking to see how easy it was to follow the spoken directions from the program. Seemed pretty good so on the way home I'm going to let it decide how to go. Max thought it was very cool to be able to say "Computer, WHERE AM I?" and have the computer tell him "You are currently in Philadelphia in Philadelphia County Pennsylvania. You are currently traveling Southbound on US Route 95 at 75 miles per hour." Being a total geek I probably found it cooler than he did.
Above is what I wrote before we left. We did end up leaving the parking garage by 3:10 so that was pretty good. But it really went down hill from there. It took just over 3 hours to get home with the majority of the problems in Philly and again on Route 206 (the home stretch).
Two things I forgot to mention were that Max is now up to a strapping 60 pounds and the Dr. said to just stop the medicine for thrush since it no longer seems to be responding. She felt that if it was truly thrush, Max would've complained about a change in the taste and/or texture of foods but neither has happened. We'll see if taking him off the medicine makes any noticeable difference.
Today we started at Lehigh Valley and it is certainly different than Philly. For starters, Max was able to go to school until 11:00 am. We put his numbing cream on at school and then made the 1 hour and 5 minute drive to the hospital. There was no traffic at all and we don't really expect any on the way home either. I did have the new GPS/mapping software setup because I hadn't been here before but I did know that there were only 6 turns involved. We'll I guess I got what I paid for because several time on Route 78 the GPS lost track of us. I had to shut it off when we got near to the hospital because it kept saying it was rerouting us. Thankfully, I could see the hospital from the highway and there really is a big blue H on the front of the building that we had to walk through. Max thought it was funny. It is at least 30 feet tall but could well be more than 50 I suppose.
Everyone here has been fabulous. To be fair, Morristown, CHOP and Robert Wood Johnson were all very friendly as well. Here, it was a little over the top. Everyone was expecting Max and he showed everyone the whole in his teeth (he lost them 5 months ago, at least). After filling out the paperwork Max and I got to play the video game Combat. It was a little sad because they have a lot of very nice stuff here that all has a dedication to some child that has past. The video game is a relatively new game from an arcade, not a playstation type game. There is also a foosball table and a big salt water aquarium with most (maybe even all) of the fish from Finding Nemo. That is the pre-waiting room. In the day hospital, where Max gets his meds, there are 19" TVs build into the ceiling and each has its own Sony Playstation and DVD player.
Max is up to 61 pounds now. His other vitals are good. I believe he is 125 cm (4' 1.2") tall as well. We got a tour from his nurse, Karen, and then she set Max up in a room with A Shark's Tale playing on the DVD player. He was also allowed to play with a bunch of toys and books while she asked some questions. Max said that the needle didn't even hurt - probably because the numbing cream was on for so much longer than normal. Oh, on the tour, Max go to see the slushie machine that the have here (same as you see in DQ). The kitchen also offered milk and animal crackers so he took those and headed for the Thomas the Train set. He played for a little bit and then we met Dr. Phil (Phillip Monteleone). He is very nice and actually did some work at CHOP previously. He took a history, did a quick check of Max and then had us go back to play while the drugs were made. Max was watching Finding Nemo when everything started but he's fallen asleep. We should be out of here by 4:00. I still have to make the appointment for next week but they've told me that Tuesdays are typically slow here so we should be okay for the next two weeks.
Friday night was the start of it all. Max and Gwendolyn were watching Space Jam together in the kitchen. Gwendolyn came running to Tracy and me at about 8:30 saying that Max was throwing up and couldn’t stop. We’ll, he certainly was throwing up. Since I can’t smell it was my job to get Max cleaned up. The whole time I was wiping down his hands and face, he was continuing to watch Space Jam. He is nothing if not focused. He didn’t believe he was going to be sick again so we got him in the shower, cleaned up and into bed. Around 3:30 am he woke Tracy because he was again throwing up and this time there was some diarrhea to go with it. I called CHOP to see if we should do anything and the fellow on call said that if Max was done, it was okay to just give him some liquids.
Everything seemed fine until Saturday afternoon when Max was a little flushed and had a fever of 100.1. I called CHOP and they said to check him once an hour for two more hours and if it stayed up or went to 101.4 or higher that I had to take Max to the ER. Sure enough, his temp stayed up so we headed down to Philly at about 3:15 pm. The drive down had a surprising amount of traffic between Bridgewater and Flemington and then again around Philly. Max watched a movie and had his giant yellow pail with his name on it (from Emma’s birthday party quite a few years ago).
We got here around 5:00 and after figuring out where to park – which would’ve been impossible had we not been coming here for past 7 months – we were met by the woman at the Welcome Desk with a friendly, “You must be Max.” I have to admit that the thought of coming to the ER in Philadelphia on a Saturday night had me thinking of all kinds of nightmarish problems. Service was so fast that the triage nurse didn’t have time to look him over before the room was ready.
We’ve been here about an hour now giving histories and discussing what to do. Max still hasn’t had his port accessed but that will happen shortly. They don’t expect to keep him but believe he’ll be going home with some meds to take to knock out the fever and keep it away. His neutrophils have stayed up so they don’t believe he’s in any danger but they’re going to check his blood counts just in case.
… the rest was added after the fact because I needed some time to think about what had happened …
The first nurse had trouble accessing Max’s port so another attempt was made by his other nurse. This went better but Max shed a lot tears; I think it was more from fear because of the new surroundings – I hope so anyway. His blood counts were fine but it turns out that I had missed a very important piece of information. Apparently because of Max’s central line there is a very grave concern about infection and he is treated for EVERY fever of 100 or higher that lasts 3 hours or as soon as a fever reaches 101.3. I had thought we’d be going home once his blood work was confirmed ok but instead I was presented with the option of keeping Max at CHOP until Monday morning or having him take part in a study to evaluate home treatment. This didn’t happen until 9:00 pm. Needless to say, we chose to participate in the study because Max had already had a very very bad day. Unfortunately we didn’t get out of there until 11:30 and didn’t get home until close to 2:00 am. Oh, and then to top it all of Bob ended up with what Max had for Sunday. Ugh.
Today's visit was mostly uneventful. One nice thing was that a boy named Jeff being treated at Lehigh Valley had raised a lot of money for the hospital and some of that money had gone to a String of Strength – a bead necklace that the kids at Lehigh Valley make. He gets beads for the milestones of his treatment and each visit to Lehigh Valley we’ll add his newest beads.
Today went very well. Max enjoyed having Gwendolyn and Amy along for the day. Gwendolyn was a little bit shy but otherwise okay. She thought the Slushie machine was cool but didn’t like the taste. Amy helped Gwendolyn with a bunch of different crafts – stencils, paint, glitter, etc. while Max played with Play-Do or watched TV. Max never took a nap today and surprisingly not all that cranky. We’re done for two weeks until round 4 starts April 19 back at CHOP.
Today started very early - 5 AM but really 4 AM because we just changed the clocks. Ugh. We didn't leave the house until 6:00 am. We lucked out in that we were going the 'good' way to Philly because the traffic on I-95 crossing the Delaware River was backed up at least 10 miles going to NJ. We got there at 8:30 for our 9:00 am audiogram and we got in to see the audiologist a little before 9:00 am. Max liked the Dr. and was very cooperative. Everything was normal and we'll probably go back again in 6 months to make sure the chemo isn't affecting his hearing.
We went and got a late breakfast / early lunch in the cafeteria and then Max watched the giant 'mouse trap' in the hospital lobby. I was on my laptop checking on work but Max sat perfectly still for more than 10 minutes just watching the balls move through all the different parts of the machine. When I was done I walked over to him but he got up as I got near. He didn't see me behind him and I thought he was going to panic that I wasn't there but when he didn't see me at my seat he just wandered off to another toy. When I asked him if he was worried he asked me what I was talking about. Huh.
We went and hung out in Dr. Liu's waiting room for the next two hours because our appointment wasn't until 1:00 pm (and he doesn't get there until then). We watched some TV and talked and Max was pretty good about it. We got in to see Dr. Liu and everything is pretty much the same as last time. His left eye could be 20/300 (improved from 20/400) but Dr. Liu was not confident in the test but didn't want to give Max drops to improve the test since there wasn't a lot to be gained. Max's right eye stayed at 20/60 so that is still good news. Dr. Liu asked that we Max's local ophthalmologist for the next 6 week follow up so that she can update his eye glass prescription if need be.
Well Tracy and Bob got up at 5:00 am to get ready so that we could be at CHOP with Max by 8:45am. Bob found Max downstairs in the kitchen with a mouthful of Easter bunny marshmallows. There is just no telling when he got up but he was wide awake. We were able to get everyone showered and out by 5:48. Tracy slept much of the way down but Max watched movies and looked at the scenery - often remarking that the sun was not yet awake. We got here early enough to eat breakfast but we ended up waiting 45 minutes because there was some confusion as to where we were supposed to wait. Dr. Armstrong was very nice and Max spent most of the day with Jennifer who works for Dr. Armstrong. He didn't finish until a little after 4:00 pm but Jennifer said that he had done a great job. We'll be hearing from them in a about 2 weeks to schedule another meeting to go over their findings.
Max is up to 63 pounds and has gotten a lot (maybe most) of his reflexes back so he's back on a full dose of vincristine for the first 3 weeks of this round. Max did a great job today but was napping when he finished so taking the tape and tube off made him extra grouchy but it had been a long day so we didn't make too big a deal out of it. In talking to Dr. Belasco today, Max will be done with this chemo at the end of round 8 (October 25, 2005) at the latest. He may have to stop early if he develops an allergy to any of the meds. For the first year he'll get MRIs every 3 months. After that it will be at least every 6 months until he's 10 but more frequently if needed.
The next 3 weeks will be at Lehigh Valley then we go back to CHOP May 17 for an MRI and Morristown for an eye exam and updated prescription for his glasses.
Today's appointment at Lehigh Valley wasn't until 2:00 pm so Max got to go to school for half a day and Bob got to go to work. Max was asleep when I picked him up but Miss Alicia, his aid, said that Max had eaten a lot today including 7 tater tots because he thought they were french fries. Not the healthiest food in the world but certainly it increases the variety a little bit.
Max has some slight weakness in his left foot and is more tired than normal but otherwise seems to be fairing pretty well. He only has one more week with the vincristine so hopefully he can get that next week and then recover while he's off that drug for almost a month. Today was the first time he's ever been uncooperative with the Dr. Dr. Leslie was trying to get him to walk in a straight line, heel touching the other toe, but we wouldn't even try. Instead he went back into the exam room, sat in a chair and started eating his snack of milk and animal crackers. Not bad for the first resistance in 8 months.
Max slept through most of the carboplatin and somehow didn't wake up extremely grumpy. We left at about 5:15 pm and got home a little after 6:15 pm. The great thing about Lehigh Valley is that we will always manage to get home in about an hour because traffic is so light.
Today's appointment went pretty well. Max brought his collection of View Master 'discs' to show the nurses because the room where they access his port has a new Incredibles View Master viewer that he thought was great last week. Max has about 20 'discs' and the nurses sat through them all. Max was a very good narrator and kept the flow going by simply ignoring all questions from the nurses. He really was entertaining. Max was able to get the high score on the video game in the lobby but we didn't get to put his name in because it was time to see the doctor and he wasn't 'dead' yet. He was actually pretty good natured about just walking away once the doctor was able to get his attention away from the game. We met Lori, the nurse practitioner, and she saw the same problems with his walk and strength on the left side that the doctor had seen last week. He got his full dose of vincristine and he is not scheduled to start that again until May 31. Hopefully we can get his stamina up so that he can tolerate the full course again next round.
Today went pretty well but Max didn't sleep so there was no time to write. He napped on the way there and on the way home but we were either playing video games or watching Sesame Street at the hospital. Max still has a little bit of trouble with his left leg while walking but it is nothing compared to a few months ago so hopefully he'll be able to get all of his meds next time around. Max has an MRI next Tuesday at 7:45 am in Philly. Then he is off until May 31! I'll be meeting with the doctor that did his neuro/psych evaluation on Thursday of next week and then I join Max for some time off.
Max's MRI was essentially uneventful. It was a long day for both of us but he did just fine. He is more worried about getting eye drops tomorrow than he was about having his port accessed today. We talked about it last night at the hotel and I said that maybe a really good toy or movie would make the eye drops not so bad. He didn't realize that I was being a little sarcastic and it was cute to see him seriously considering what prize would make the drops hurt the least.
Anyway, the eye doctor is tomorrow afternoon. He'll probably come away from that with a new perscription for his glasses as his vision is testing better than it did before. I'll be taking a CD with his MRI images from today but I don't know that she'll be able to shed any light on them. I'm still only able to pick out the trouble spots and can't really tell how they've changed. We should have the reading back Thursday. Also on Thursday, I'll be down in Philadelphia to meet with his neuro-psychologist and discuss his evaluation from a few weeks ago.
Hope everyone had a good Memorial Day Weekend. Our was mostly quiet but that worked out well for us. Gwendolyn had her Brownie parade and then she, Max and Tracy went to play mini golf with the Brownies. Apparently Max was only interested for a little while and then started throwing rocks into the water traps and putting extra rocks in his pockets for later. I stayed home with Olivia and we worked on our napping skills :).
Traffic on the highway was nothing compared to the traffic at the hospital. Because of the holiday, it is very very busy here today. We did get into to see the doctor pretty early and we were able to go over Max's most recent MRI. Things look to be stable and it could be argued that they are improving. I'm supposed to get a copy of the reading today so I should be able to update Max's website later this week.
The doctor did confirm that Max has started puberty and it is far too early for that. This is likely related to the tumor in his hypothalamus but she wants Max to start seeing an endocrinologist here to better track what is going on with Max's hormones. Unfortunately she said there is a 3-4 month delay in getting to see them. So, Max will be starting Lupron this afternoon as the doctor believes it needs to be started right away. The company that manufactures Lupron has a website for kids with precocious puberty called www.toosoon.com. It is pretty informative. Max will start the injections today (shot in the thigh/butt) and get a shot every 28 days until the endocrinologist tells us to stop - from what I gather around 12 years old (around 2012). Max and I talked about the shot at lunch and while he is apprehensive (his first words were "No way, no how" - not sure where that came from as I don't recall saying it in the past 10 years or so) he seems on board with the fact that it will hurt but it means an extra prize when he gets home. I'll update his website later with info on how the shot goes. (The shot actually went just fine. He shed tears for a second or two but then got laughing and was okay. Said his butt still hurt when we got home.)
Tomorrow we'll be doing Max's IEP at school. Hopefully everything for that will go quickly and Max will again get everything that he needs. I'm hoping that he is able to continue learning Braille over the summer so that he doesn't lose what he has already learned.
Today has been essentially uneventful. Max got a toy Stitch (Disney character for those that haven't seen the movie) that oozes green goo from his eyes, nose, and mouth when you squeeze his head. It was a big hit with everyone here but is much harder to squeeze than it seemed. Hopefully Max will be able to get some reaction out of it with lots of practice but for today he's relied on handing it to me for the 'real' demonstration of how cool it is. His Dr. happened to have a similar Sponge Bob toy that just sort of bulges when you squeeze him. Most people just make the eyes bigger but Max quickly determined that it could be made to look like he just had an accident in his pants if you squeezed his head just right. Ah, my boy.
Looks like we'll be out of here before 4:00 pm so this is a good week.
Today went pretty well. I had to sign a release for Max so that they could take pictures of him for the Lehigh Valley Website. Max hammed up the test shots quite nicely. When the photographer found out that Max wouldn't start his chemo for an hour or so, she decided it would be good to get shots of him getting accessed. Accessing went better than normal because Max kept looking for the camera so that he could smile.
Max did react a little to the carboplatin again. He didn't wheeze or cough but did develop a little rash. They gave a small dose of benadryl and said they would do that to start next time to see if they can't take care of it before it starts. Oddly, Max didn't sleep on the way home and seems ready to go outside. Outside is only somewhat hotter than inside as the A/C stopped working last night. This does not appear to have been a Max related incident but I did check to make sure he hadn't filled the compressor full of stones ;)
Max got Benadryl to start today and slept for the entire treatment. He had no reaction at all to the carboplatin this week.
The Lupron shot went okay. It appears to be pretty painful (the Doctor didn't even stay in the room) but Max did very very well. Just a few tears and then some laughter. I'm realizing now that I'm woefully far behind in updating this site but I'll try to get to that over the holiday weekend.
Today Max and I traveled to Philadelphia to meet with his newest doctor, Dr. Sogol Mostoufi-Moab and her attending physician Dr. Thomas Moshang, Jr (search his name on the Internet and you'll find tons). I didn't think it was possible but the Endocrinology department seems even busier than Oncology. Our choices for appointments 4 weeks ago were today or the end of August. Last week we got a letter in the mail that said how important it was that we make our scheduled time and that 30 minutes had been included just to complete paperwork. Our appointment was for 1:00 pm and we got there at 12:40. The man ahead of us in line had apparently missed his appointment and was rescheduling for the end of September (the earliest available appointment - we will not be late for these appointments!)
In spite of the near horrific number of people that were in the waiting room, we were called by the nurse within minutes of signing in. Before she could introduce herself, Max looked her square in the eye and announced, "I have brown hair all over my peeper". His tone was exactly as it had been when he announced the loss of his teeth so many months ago but the content was enough to leave me able to say only, "Hello, I'm Bob". Even the nurse paused and then told us that her name was Rita. Max got his height (4' 2"), weight (63 lbs) and blood pressure (106 / ??) and we were shown to a room which the doctor entered only a minute or too later. Dr. Mostoufi-Moab was very nice and asked several questions of Max and I before starting to examine Max. Max had been prepped that the examine would include parts that only doctors, nurses and his parents were allowed to examine and the Dr. started with a similar explanation to reassure Max. He waited for her to stop talking and then asked, "Are you going to pull out the hair around my peeper?"
Everything so far is pretty much as expected. Max does have precocious puberty. Even though he turns 6 tomorrow, his bone age is that of an 8 year old and his genitals put him at about 12 years old. They took some more blood today to test his hormone and growth factor levels. The results won't be back for about 2 weeks. They should see a decrease since the blood test that was done the day he got his first shot on May 31. If not, they will adjust the dose that he gets. They did tell me that any registered nurse can give him the shots so we don't have to go to the hospital every month until he is 12. When it works out with his other visits to the hospital, we can do it then but otherwise we'll probably try to work something out closer to home.
The only other concern is that his Growth Factor was at 700 but for a 16 year old male at the height of puberty it should be a max of 600. This typically is NOT a side effect of precocious puberty but they want to wait for the blood work to come back before discussing it further.
Next Tuesday we are back in Philly to start the next round of chemo.
Today went okay but we learned a little more about Max's recent reaction to the Carboplatin. The feeling is that we are close to the end so they want to try and get him to not react to the Carboplatin. In addition to the Benadryl (25 mg) that he got last time at Lehigh Valley, they're giving him Tylenol (4 children's chewable) and we are supposed to give that same to him tonight. This is head off any potential fever because if he does run a fever we'll have to go back to the hospital. In addition, they are going to slow down the rate that he gets the drug from 1 hour to 2 hours so our trip will be longer but their experience is that this makes a reaction less likely. In the event that he ends up with a significant reaction at some point (hives, fever, and/or breathing trouble) they will slow him down to 8 hours and give him the medicine in the ICU - just in case.
Max is now up to 4'5" (I must have miss-heard last week's 4'2") and 64 lbs. I was able to see his blood test results from last week and all but the Growth Factor numbers are at or close to normal. The Growth Factor has dropped from 700 to 450 but that is still very high. I have to wait for the Endocrinologist to call to find out more about what that means and how (if?) it gets treated. For the next two weeks we'll be going to Lehigh Valley as long as the Carboplatin goes ok.
For the first week of August we'll be here all day on Monday August 1 and Tuesday August 2. Max will get chemo Monday morning and see the eye doctor in the afternoon. Tuesday he has an MRI at 11:30. After that, God willing, Max won't see a doctor or hospital again until August 23 when he starts round 7 and gets his 4th Lupron shot.
Today we tried starting at 11:30 am at Lehigh Valley but construction on Route 78 made us about 30 minutes late. They were ready for us anyway and got his blood work going right away. His treatment was preceeded with children's Tylenol and Benadryl. Max slept for the entire treatment and didn't show any reaction.
Today we got a letter in the mail from the Endocrinologist. It is about 3 pages but the summary is that Max has precocious puberty which they believe is caused by the optic and hypothalmic tumors. It is a little hard to follow the letter because it was composed the day after our visit but includes an addendum that was added after the lab results were back.
Anyway, Max had 2 out of 7 test items related to puberty return to the normal level. There are 2 other test items that are noted to be elevated but improved. The final 3 items are just marked elevated. While they had talked about the possibility of additional testing/treatments for Max's elevated growth factor, they feel the lab results are encouraging and will wait to do further testing during our next visit in December. If the levels do not continue to show the decreasing trend, then they will administer an oral glucose surpression test to further assess him.
There is an informative website (http://www.chclibrary.org/micromed/00049860.html) that talks about the test and also briefly describes acromegaly. Acromegaly is mentioned in the letter but only in the discussion of the glucose suppression test, not Max's diagnosis.
Max got his chemo and Lupron shot at Lehigh Valley. Everything went just fine. He got the shot in his chair in the day hospital, instead of moving to a room. He still cried a little bit because it hurt but Lauren, a young girl who is also at Lehigh Valley with NF and is on her 14th Lupron shot, brought him some tissues and that made him feel a little bit better. The Benadryl had him asleep a few minutes later.
While Max was sleeping, I got several calls from work that resulted in quite a few stares. It wasn't that I was too loud, it was because I changed my ringtone for work calls to the theme from Halloween. It seems that no one there expected that from me as I'm normally just the quite guy working on his computer.
Today went very well for Max. We left the house at 5:45 am, made a pit stop in Pennington for BBQ potato chips and Swedish Fish (for later of course) and to put on Max's numbing cream and then ended up making it to Philly by 7:30 am - one of our best times yet. We somehow made it through the construction before the traffic really started. Max got to pick the story of the day in the playroom and was rewarded with a toy carrier pigeon. Things went pretty quickly and Max was soon asleep from the Benadryl. He slept right up until 2:00 pm when they finished his treatment. We went and got lunch. Max had HoneyNut Cheerios because they didn't have regular Cheerios. There was a concern about him having a nut allergy similar to Gwendolyn's but the allergist had said the blood work showed no problems at all. I figured, what better place to put it to the test than in the middle of the hospital (he had just had Benadryl and I had Gwendolyn's Epi-Pen with me so I was hedging a lot anyway).
After lunch we went to see Dr. Liu to have Max's eyes checked. As always it was phenomenally crowded and it took a while to even get a seat. Once we did sit down, Max complained that he need the entire couch so that he could lay down. When I informed him that I would be glad to have either his head or his feet across my lap but that I would not be vacating the couch, he had a minor tantrum where he told me that I was to go home and Mommy could come get him when he was done. It is these precious moments in the midst of a few dozen people that I don't know that I treasure the most ;) I guess the silver lining there is that I don't get nearly as brilliantly red as I did a year ago; maybe with some more Max therapy I'll be able to hold a killer hand in poker and not have my cheeks let the room in on my secret. Max was little difficult for the first Dr. to check his eyes but when Dr. Liu came in for the follow up Max was fine and they have decided that, again, his vision is essentially unchanged.
Tomorrow Max has his MRI at 11:30 am. We're staying at the Wyndham in Philly which should work out pretty well as it is just a few minutes drive in the morning to the hospital. With a little luck we should be home tomorrow before traffic starts to get heavy.
Vacation is over and today it was back to Philadelphia for a review of Max's MRI, the start of round 7 and another Lupron shot. While there was very minimal growth in the size of the overall tumors in the chiasm/hypothalamus, his Dr. is calling it stable and said that no change in protocol or timing of future MRIs is needed since he is already set to scans every 3 months. One change that they'll watch is that there is a cystic mass on the back right side of his hypothalamus that is different than before. It appears to have a fair amount of fluid that is not clear like the ventricular fluid. She said this is because there is protein in the fluid that the tumor (outer wall of the cyst) is feeding off of. The location makes it important to watch Max for changes in appetite, behavior, and mood. While he ate voraciously at times while on Block Island, the Dr. and I agreed that it was likely due to his increased daily activity. We'll have to watch him though.
Max did great for the neuro exam and was pretty brave for the Lupron shot. The shot was a little weird because he broke down and cried a few minutes after the shot. His nurse had him laughing pretty quickly and all was ok. He is now sound asleep and we'll be here until about 4:00 pm. We're going to be back here in 3 weeks so that they can do some additional blood work to see how the Lupron shot is affecting his hormone/growth levels.
While eating lunch earlier, Max had asked for one of my Cheddar/Ranch potato chips. He seemed to like it. Then he asked me for the ingredients for the potato chips, the honey nut cheerios and the fruit loops. When I later offered him another chip, he told me no. When I said, "But it looked like you enjoyed the first one, what's wrong?" He replied, "Blue Cheese .... I don't like Blue Cheese" That had been listed as the next to last ingredient. I then offered him a chip that I assured him had no Blue Cheese. He looked at it and pointed out a dark brown spot that he said was probably Blue Cheese. I bit that piece off and offered him the chip again. He looked at me, smiled his toothless grin, and said "I don't eat chips that other people have already eaten .... that's gross"
Today was at Lehigh Valley and so far ... so good. Max got soaked when the Sponge Bob squeeze toy that Dr. Phil showed him broke during a 'demonstration' by Dr. Phil. After shouting that his clothes were RUINED Max calmed down a little bit. Dr. Phil felt pretty bad but it was really not his fault. Max felt better when the nurses told him that they would get him a squirt gun (syringe) to use on Dr. Phil when his meds are done but Max has been asleep since shortly after getting the Benadryl and I suspect that he'll have forgotten. The nurses might remind him though as they seemed to like the idea of Max squirting Dr. Phil.
Today has gone pretty well. Max watched a Mickey Mouse Christmas movie on the way here today (Tracy has been telling him it was 'lost' so that she wouldn't have to listen to Christmas movies in the summer but somehow it 'showed up' today - shocking) The movie had him in good spirits when we got here. We were surprised to find that we were still essentially alone since yesterday was a holiday but it was a nice surprise. Lunch also went exceptionally well as Max ate all but 1 yogurt that Tracy had sent along and he did it in about 20 minutes. Normally lunch is an hour and there is constant bartering about what will actually get eaten. Max was watching Toy Story but the Benadryl knocked him out so I got to turn that off. He fell asleep before the Dr. could see him so they are waiting until the end to finish their evaluation but his blood work was fine and his breathing and heart rate were also good. It looks like we could be done here before 4:00.
Max starts school next Monday and then on Tuesday we travel back to Philadelphia so that they can run some more endocrinology tests before he is due for his next Lupron shot the following week.
Today has gone well so far. We left the house a little bit late (6:15 instead of 6:00 - ugh) but we still got here before our 9:00 am slot. Max was unable to find the book of the week (Clifford's Halloween) so Kim, who is in charge of the treasure chest as well as many other things, told Max that he could pick a new story and come up with a new question. We read the Lion King and I thought it would be easiest to ask "What is Hakunah Matatah?" Since I thought that Max would certainly know that - he's seen the movie at least twice (he says knowing that the count is closer to 200). We'll we had to practice quite a lot with "What is the question? - What is Hakunah Matatah?" and "What is the answer? - Have no worry." Max kept saying something about fear. Anyway, Max 'won' 3 hotwheels cars which had him very excited.
Today, for the first time since at least last October, we actually got to see Dr. Belasco at 10:00 am. This worked out really well because there were no tests to review and Max has been doing well so we talked about what would happen at the end of his next round as he is very near the end of his chemo. I knew about MRI's and eye exams every 3 months for a year but there was more that I had not considered. Max will have an MRI on Tuesday, Nov 1 and already has an eye exam scheduled for Nov 3 at 10. We're going to make his Thursday really busy by doing an audiogram at 8:00 and seeing Dr. Belasco at 12:30. The audiogram is just precautionary as hearing loss is one of the side effects of his chemo; there are no signs of any trouble. The meeting with Dr. Belasco will be to review the MRI of 2 days earlier and to talk about the eye exam's findings. The following week, Max will need to have his port removed. I think that is mostly good news but it means that he'll need to get IV's for the rest of his MRIs. While I'm not really looking forward to that, Max has been surprising me more and more with what he is able to tolerate. Even his Lupron shot goes pretty well now and I can see that shot is painful.
By the time we were wrapping up with Dr. Belasco, we only had time to make some appointments before Max's meds were ready. He is already asleep from the Benadryl and he should be starting his 2 hour carboplatin before 12:30. With a little luck, we'll be done by 3:00. We didn't eat yet so I expect we'll need to do that before we leave but we should be able to get ahead of the worst of the traffic. Happy Happy Joy Joy.
It is now the beginning of the end, 3 more weeks of chemo to go and we're done. We didn't get out of the house and on the road until close to 7:00 but we managed to get here at just a shade past 9:00. Much of the construction on 95 just North of Philly is now complete so traffic is not nearly as bad as in the past. Max is somewhere between 68 and 70 pounds now - can't remember if it was 30.3 or 31.3 Kg. He is still 4'5". Everything else was pretty normal.
We did go over his endocrinology labs from a few weeks ago and while most of his hormones are back to where they should be his growth levels have gone up in spite of being on the Lupron. I've written to the endocrinologist to get some idea of what it all means but from talking to Dr. Belasco, this is in all likely hood being caused by the tumor around his hypothalamus. Apparently it is not uncommon for seemingly stable hypothalamic tumors to cause the elevated growth levels. More questions - I'll let you know when I have any kind of answer.
After that we shot downstairs for a quick lunch at McDonald's - the cafeteria is apparently closed at 11:20 in preparation for lunch. I should mention that for the period of time that the cafeteria is closed - it looks like a very very fun place to work. The music was blasting and everyone looked to be enjoying themselves (we could see them through the gate). While I wouldn't say that there are normally only dour faces and bad attitudes in there normally, I certainly don't recall thinking of it as festive. At McDonald's Max told me that the Happy Meals toy "sucks" and he didn't want it but it is cheaper to buy the nuggets, milk and fries as the meal. When he pulled the toy out the box - he must have briefly forgotten is earlier statement - he told me, "See, I told you this was stupid. Why didn't you listen." He did this in a nice way - not the normal snotty tone I get when he is tired or mad.
Max again fell asleep within minutes of getting his Benadryl. I was tired so instead of hooking up my laptop and drying my eyes further, I read a little bit. That was the plan any way. Instead I fell asleep. I knew it was coming and tried to fight it because I'm really terrified of snoring in here but I lost the battle and awoke with my book in my lap and my head leaning forward - thank god. I don't think I can snore that way and no one has given me funny looks so I think I'm okay. When I slept on the plane with my head tilted back, even if Shawn hadn't been laughing, I could've told from the looks of my fellow passengers that I had not slept quietly.
While Max was sleeping, a guy named Woody Wolfe dropped by with a what looked like a lot of over night equipment. It turned out to be a guitar and a bunch of other equipment and he was here to sing songs for the kids. It was really nice. I tried to wake Max up because he knew some of the songs but it just wasn't meant to be. Woody did leave a CD for Max. The website on the jacket is http://heartohandministries.com.
It looks like we will be done around 2:30 or sooner so all in all, a pretty good day.
Today was a pretty good day. Everything has gone fine but we'll be ending with Max's flu shot. Can't imagine it could be any worse than last year when he jumped off the table with the needle sort of in his arm but if it is I'll write about that tonight from home. Max got to watch Jay Jay (a toy airplane show on PBS) which made him very happy because we don't get it at home. After that he fell asleep and just woke up when they put numbing cream on his arm for the flu shot to come. We should be out of here a little after 4:00 pm. There is really not much else to report.
This is the last week of Vincristine for Max. That is the drug that was causing problems with his reflexes. Next week will be the last round of chemo for Max! Today was a series of minor problems that started when I picked Max up from school and learned that I had purchased the wrong type of bandage for his numbing cream. I was supposed to get just a clear plastic dressing but what I got was more like a band aid with an absorbent strip in the middle that I was sure would just suck up the cream. So I loaded him up with far too much cream and hoped for the best.
When we got to the hospital, I opened my backpack to include his blanket and boppy and dumped cheerios all over the inside of the bag and the back seat of my car. It had seemed like such a small bag but Cheerios produce an disproportionate amount of dust so it looked like an awful lot. Oh well, I thought, the birds can have a little picnic and I'll just have to remember to vacuum before the mice figure out what I've done.
The extra cream mostly worked out but he felt the needle going in a little and moved so that the first try missed and just made a little hole and produced a little bit of blood. The second time worked okay though and Max didn't have any tears because of it (that really is the goal and the important part). Max wanted to play with toys before he ate lunch but lunch always takes to long with him that playing first isn't really an option. We haggled for a little bit but when I saw I wasn't going to win I told him that he wouldn't get the prize for the day unless we ate lunch first. This was effective but left him angry with me so he tried to beat me to the elevator so that he could leave without me. Thanks to my exceptionally long stride, Max was unsuccessful but I took the time that we had in the elevator together to remind him that we had, just last night at Tiger Cubs (pre Cub Scouts), discussed the dangers of getting lost. Now that I'm writing this I'm realizing that last night was also lost on me because I didn't go the extra step and setup a meeting place for the hospital in case we ever do get separated. I have to pay more attention at those meetings but I could only think about whether there were enough chocolate chip cookies for the adults as well as the kids ;)
We got through lunch with Max eating one and half chicken fingers - he doesn't like them at all. I only got the extra half into him because he wanted hints for his prize. He was able to guess that he was getting a Scooby Doo toy but knew that he had to wait until we got back to the day hospital to see it. He was very excited to find that Scooby was wearing a pirate costume for Halloween and that he played the Scooby Doo theme song while dancing. Using the theme song as a reward, we were able to read 3 books that had been sent as part of his homework package. Max was able to fly through them and we were about to start other work when Lori, the nurse practioner we saw today, came over to talk to us and Anne Marie brought over Max's Tylenol. Max smelled the Tylenol and said it smelled like cheerios. He got the two nurses to also smell then started laughing when they asked if I was going to smell. He told them that I can't smell because I smashed my head into a wall and broke my nose.
While I corrected the story Max got his Benadryl and then we headed back to an examine room so that Max could get further checked out. Just walking back there you could see the effect that the Benadryl was having - next time the Benadryl will come after the exam. Max made it back to his chair but was already falling asleep. He made it through 3 pages of the story he had picked before I realized he was gone. I took out my laptop and got ready to get connected, do some work, and compose this letter only to find that my cellular card wouldn't turn on. I took it out and reseated it but still couldn't get it to turn on. I turned off the computer, waited a little bit, still no joy. Then I noticed there was little bit of dust on my leg where the card goes into the laptop. Huh. I took the card out and found more dust on the card. Looking into the slot I found the remains of a stray Cheerio. Ugh. I got a straw and sliced it up and got a lot of Cheerio bits out but there are still some that are very stuck among the pins and my card won't turn on. Thankfully I got the Complete Care package for the laptop and I've double checked with Dell that they will replace the motherboard if I'm unsuccessful with a can of compressed air later tonight. Ugh.
Max still has his Lupron shot to go but even that is getting easier for him to tolerate. Last night, even though I reminded him that it was coming today, he did not tell me that was a little worried. He just said, "In the tush?" "Yeah bud, the one in the tush." "Goodnight." ... It is amazing what has become routine for him.
You won't get this until we're home, but since I couldn't work I was able to spend more time just writing
The shot went ok but Max did try to talk his way out of it. We're going to try some numbing cream for next time to see if that makes it any easier.
Today didn’t go nearly as well as last week, but it has been mostly inconvenience. The trouble started on Route 78 just past exit 16. We stopped dead and sat for almost 90 minutes. Because it was raining pretty hard when we left home, I decided that we did not need the whole case of movies. Ah well, it is good that we brought a Peter Pan movie because even though it is short he doesn’t mind watching it again and again. Max ate his lunch and made the best of things. He saved 4 of his candy corns to give to the staff because “sharing is nice”.
This week is sort of Halloween week at the Hospital for the kids, the staff has Halloween colors and stuff but they don’t wear costumes until next week. This year my initial idea for a costume was a little too scary to wear here so I decided to pull out my long blond wig (well, I asked Tracy to do that) and Tracy purchased a beautiful brown dress with blue and green flowers. It is lovely. I had a set of fake boobs that I had worn years ago but they came with very pointy nipples. Instead of butchering the old boobs, as pointy nipples seemed inappropriate for the pediatric ward, I went to Wal-Mart this morning and grabbed the largest bra I could find. It is about 10 inches too small but has stretched quite nicely. It is filled with socks so my chest is quite warm but the rest of me was pretty damn cold when I was outside as it is blowing 30-40 mph, raining very hard and only about 50 degrees (pointy nipples might not have seemed so out of place). Sitting in traffic on 78 with all of the trucks looking down into the car made me feel embarrassed but I only got a few weird looks.
Walking into the hospital was a lot different. I didn’t have the wig on in the car but I put it on outside so I wouldn’t have to muss too much inside. All I got outside and in the lobby were weird looks so I just kept my conversation with Max going and tried to ignore everyone. Finally, as we got to the elevator, 2 women and a man burst out laughing. I had been a little worried that no one would get it or think it was just weird or twisted but the laughter made me feel a lot better. The receptionist was little skeptical but thought it was funny. She told us the most everyone had gone to lunch but not to worry being late was not a big deal.
The staff had a blast. Several outsiders have been called into to gawk including Dr. Phil’s wife because everyone decided they now knew what Phil would be dressed as but his wife said it was okay he didn’t have to wear a dress on Monday. Max’s costume has also been a hit because it is of such high quality. He is, of course, Peter Pan but several people have made the mistake of calling him Robin Hood (and I, Maid Marian) but Max always corrects them. He is curious to know how they all know it is Max in the costume. I’m not sure if he really thinks he looks like Peter, just looks very different, or what but it is cute to see him struggle with it.
We talked to Dr. Phil a little longer than normal because Max was a somewhat different boy yesterday. He was a little confused and disconnected. Today he seems back to his old self but he had us a little bit worried. Anyway, things seem to be fine and we’re into the last few hours of his last chemo treatment. Max might have reacted to the Zofran – he sneezed and had a coughing fit right after he got it – but they think he is going to be fine. His breathing never changed and he didn’t get any hives. He is now sleeping soundly.
I have found, much to my chagrin, that the Cheerios have done some lasting damage to something. I’ll need to troubleshoot further at home but something isn’t working right so this message will get sent from home.
Max finished his Chemo close to 5:00 and then they had a little celebration for him since it was his last chemo. He got a new bead that has a Hot Air Balloon on that symbolizes his soaring away – although he’ll be back once a month for a while for the Lupron shots. They also gave him an Olympic type medal that says “You Did It” and a cupcake. Max was very cute about everything. With a mouthful of cupcake he looked up and said to Ann Marie, Karen, and Nancy, “Do you know why I gave you the Candy Corn?” One of them said, “No, why did you give it to us?” and he replied “Because I love all of you.” It was great to be there for that.
We ended up getting home around 6:45 pm so there was rush to eat and get to bed. Max and I talked a little on the ride home and he does seem thankful that it is over. He knows there is more to do in the next two weeks and that he still needs to get the Lupron. He did think Lupron shots were done but I told him that we’d be going back once a month to get the shot and see everyone at Lehigh Valley. He didn’t really say anything more to that.
We'll be in Philly next Tuesday, Thursday and the following Tuesday but then we are done until December. I think.
Today was pretty uneventful. Our appointment wasn't until 9:30 am but we left at 6:00 am because we weren't sure how the SEPTA strike would affect Philly traffic. I don't know if it is just too long in between trips but the traffic down there sucks. It took us just over an hour to get within 7 miles of our destination but then it took 90 more minutes to go those 7 miles. No accidents, just the traffic down there. It made me wonder why I had made this Thursday's first appointment for 8:00 am. No changing it now but leaving at 5:00 am feels like it will be a challenge. Today was made a little worse by the fact that Max has been getting at 3 am or so for snacks and stuff. Having bags and buckets of candy lying around made me extremely worried that he would venture out to eat and wreck any chance of getting the MRI done. I couldn't sleep because of the worry so I was up at 2:30 am standing guard in the living room. Max, of course, slept soundly until 5:40 am when we roused him for a shower.
Max's nurse today was Lilly and she was very friendly. I had planned on going in with Max just to see what was what but realized I was just too tired so I asked Lilly when kids started doing the MRI without sedation. She was extremely friendly and informative. The gist of what she told us is that Max can always start without sedation since he will need an IV for the contrast dye anyway. Then, if he can't do the whole thing without, they can put him to sleep. The big challenge for Max will be the scan of his orbits because his eyes really need to remain still. While he is forever surprising me with his tolerance for what he has been put through, I don't know that asking him to look straight ahead, or even just close his eyes without 'looking' around, is going to work out. On the bright side, if they can get him through the brain scan with his head still and only need to sedate him for 20 minutes for his eyes, they can use different, shorter acting meds. After we talked, Lilly suggested showing him the room as the first step toward getting him ready. That went okay until Max went in and was talking to the tech and somehow got his thumb pinched in the sliding bed. He was really crying and seemed to have suffered quite a bit of pain. Unfortunately, I had my cell phone, car keys, wallet with credit cards, and several security cards with me so I didn't run in to help. I'd been told to stand at the door unless I wanted to replace all of the credit cards and I've read about how powerful the magnets are and had visions of getting sucked across the room and making things worse. Anyway, the tech took Max's hand and was rubbing it but he just kept crying and saying how badly it hurt. After a minute without any pause in the crying the tech asked him where, exactly, it hurt and he held up the thumb of his other hand. That made for a bunch of laughter. He was okay by the time we got back to the sedation room and there was no perceivable damage to either hand.
Max did very well with the sedation and started his scans right at 10:30 (our appointment time). They had given him as much of his meds at the start as they could so that he would be better for the drive home. This seemed to work out pretty well because about 45 minutes after he came out he seemed to be stirring a little bit. I turned the TV as I've learned that it usually better than my soothing voice but he didn't seem interested. I tried moving him a little bit but got nothing. I asked if he wanted to go home but I only got an eyebrow twitch. Then I whispered, as quietly as possible, the words "Candy Corn" in his ear and his eyes popped open. Thankfully he was still a little confused by the medicine and didn't remember what I had said, only that he was hungry (I didn't have an candy corn). He ate a small bag of chips and took a sip of apple juice and then we were on our way home. The ride home and the rest of the day have been mostly uneventful.
We head back to Philly Thursday for an ear test at 8:00 am, an eye exam at 10:00 am and a follow up with Dr. Belasco at 2:30 to go over the MRI and talk a little more about the future.
I was right about the challenge of leaving by 5:00 am. We got up at 4:15 am but didn't manage to get out of the house until 5:15 am. Thankfully traffic was thinner at the hour so we made it there by 7:30 and Max had his ears tested right on time. Max's hearing is normal but there is an OAE test that checks the cochlea in the middle ear that showed Max had no response in his right ear. This doesn't seem to be impacting him but they want to see him again in 3 months just to check. That fits into the schedule that we did this time so it shouldn't be a big deal. Dr. Belasco didn't believe that this was really an issue.
We went to Dr. Liu's office next and somehow we got in and out prior to 10 AM. It was a major miracle that he readily acknowledged. He told us not to expect that in the future but today was one of those rare occurrences where things were running well. Max was first seen by Dr. Osborne - this is the same Dr. that Max the very first time we came to Dr. Liu's office last year. Max was antsy and wanted to see a Teletubbie doll but he managed to sit through a lot of bright lights in his eyes. The long and the short of it is that Max's vision is basically unchanged. I did learn that Max does have peripheral vision in his left eye but his central vision is almost non-existent. This time they put his vision at 20/300 and 20/60. After that we went upstairs to see Dr. Belasco. As always, it was very busy up there but we ran into friends that we hadn't seen in a long time. When we did get to go over his MRI, things appear to be stable and there is actually a decrease in the amount of tumor that is enhancing which means that is no longer drawing blood. Max still has no reflexes but those are expected to come back in time. Everything is ready for Max's port to come out next Tuesday.
After that, we'll be following up around Feb 2 with an MRI, ear and eye exam. In December we meet with the endocrinologist. He also will continue the Lupron shots ever 4 weeks.
We have to be back in Philly Tuesday, November 8 at 8:00 am for his port removal.
Oh - on our way out today we ran into Dr. Phil, from Lehigh Valley, in the parking garage. It was nice to see an unexpected and familiar face.
Today, as with last week, we left at 5 am. Unlike last week there was almost no traffic so we got here by 7 - a full 75 minutes early. Max has expressed some concerns about today but has told me that he will try to be brave. He is most concerned about wearing a bandage for many (never say 'a couple' when you estimate around Max as it is ALWAYS 2) days.
Max says . "I miss you all very very much and I love you." Right now he is watching PBS while we wait for his procedure to start.
He has been worried about this for a while but the doctor had him laughing right before he went in. I could hear him asking for blankets to get comfortable and also trying to start a round of 'the movie game' - he always makes me smile.
He went in at 9:20 and came out at 10:20. They expect him to sleep at least an hour. Once he gets some food and liquids down we'll be able to head home.
Max's chest is healing pretty nicely since having his port removed last week. He started showering again Monday morning and that seems to be going pretty well so far.
Today we went to Lehigh Valley for a Lupron shot (for early puberty). For the first time we tried using the numbing cream on his butt beforehand. Max complained quite a lot initially but was okay for the ride there. We didn't have to arrive until 3:15 so Max got to spend most of the day in school. Unfortunately when I got to school Max was asleep in the nurse's office. He's been getting up very early lately so it wasn't all that shocking. We'd been talking about not getting up early so while Max got to see his prize for the day he won't get it until he can stay in his room until 7:00 for 3 consecutive school days. He's got a chance at Friday but I wouldn't bet too heavily on it.
At the hospital someone had left the balls for the foosball table in/on the table, which is the first time we've seen that. Max wanted to play very badly so after we said hello to everyone I proceeded to completely take him to school. He thought it was pretty funny and was more interested in figuring out how the ball got into the pocket on the side than in winning. Luckily he never got hurt banging his head on the sliding rods.
He tried to delay his shot as long as possible but finally lay down on the table 'in the position'. While the cream worked and Max did not seem to even notice the needle going in - he definitely felt the serum going in. The tears were there but were pretty minimal. After that he showed the nurses some pictures from Halloween and we said our good bye's.
Today Max weighed in at 74 pounds and stands 4' 5" tall. We chatted with the doctor a little bit because our pediatrician had raised a few questions about Max’s early puberty. The doctor feels that Max early puberty was caught soon enough so that the overall impact to his ‘natural’ height will be minimal. There is a concern about his growth factor levels because they had risen again while his hormone levels had stayed down at the pre-puberty levels where they should be.
They drew blood and are retesting his hormone and growth factor levels; we should have results around Christmas. They also order another bone age x-ray. Max’s ‘growth velocity’ is accelerated so they expect that his growth factor levels will be elevated. If they are elevated, Max will need to undergo a glucose load test sometime in January. The glucose test could be oral and might be able to be done at Lehigh Valley but we’ll find out more when the blood work results come back.
Right now they believe that Max is suffering from acromegaly which is also known as gigantism. Max’s doctor was only able to find research on 5 or fewer (“can be counted on one hand”) cases that are similar to Max where the child had NF-1 with precocious puberty and elevated growth factors. That doctor’s attending had only treated 3 children during his entire career that have symptoms similar to Max but all of 3 of them had pituitary tumors which Max does not have. The problems that this cause are that both tests and treatments are geared towards adults. The big concern with acromegaly is that Max’s organs, especially his heart, will continue to grow. This would ultimately lead to cardiac problems. The treatment that we talked about would be a shot similar to the Lupron shot that Max is currently receiving. I did ask if he could get the medicines in the same needle and she answered that she had never been asked but would certainly find out for me. There is still some time before we get the results back so for now we’re just going to plan and look forward to the holidays.
Max tried again to delay the shot for as long as he could but he still ended up getting the shot. Same story as last time, the needle going in was okay because of the numbing cream but the serum really hurt. The nurses got him thinking about other things pretty quickly and they showed him the new beads for his necklace that he gets for the Lupron shots. That got him to stop crying. He made the rounds giving everyone Christmas hugs and then we headed home.
I forgot to write this up originally but the day was pretty good. We learned that Lehigh Valley will be able to do the glucose load test for Max before our next Lupon shot in February. That's good news both for distance and because Max likes going to Lehigh Valley.
Tracy actually had to wake Max up at 4:20 for his shower. It was refreshing to know that he could sleep that late. He was kind of funny because he claimed to not know where he was. He was refreshingly nice this morning. Even when Tracy told him that she couldn't find his 'prize' for today and that he would have to wait until he got home, he maintained his good attitude. Of course at that hour there was absolutely no traffic and we only hit rain as we pulled into town so we were here at 6:20 am.
We met one of the most pleasant receptionists we have ever run into at CHOP (not to say they aren't all nice - she was REALLY REALLY nice). Sheila said that Max reminded her of her own son and told us a story about a chemistry set that his Dad gave him many years ago (I suspect he is around my age, not Max's). Everything was okay with the set and nothing exploded but it was nice to have someone to talk to that early. Max had gone around the corner and was laughing at something but he was all alone without a TV so we didn't know what he was doing.
We got to get setup for the MRI early and Max started his sedation at 8:00 am. Not sure what order they use for the drugs, but the first one is certainly psychedelic. He was sort of waving his finger with the pulse/oxygen monitor (glows red like ET) around in front of his eyes but was unable to focus. The second drug seemed to knock him out but while they were giving him the third and final drug he opened his eyes and stared at the ceiling (very small pupils at that point). He heard me ask if he was okay but took many seconds to answer that he was fine. Right before he lost consciousness he looked at the nurse and said, "this is how I wink" and he winked at her with only his left eye. That is something he is unable to accomplish sober - then it is more of a two-eyed blink. Not sure what to make of that but it was amusing.
The MRI went pretty well but Max started to wake up near the end and required a second dose of meds. That kept him asleep for more than 90 minutes after it was over and he was pretty grumpy. He slept for most of the ride home but was still pretty disoriented when we got home so Bob had to pick Gwendolyn up from school (Tracy can't carry our 80+ boy anywhere anymore).
Max's vision and tumors are stable which is good news. I had written for about 90 minutes when my computer crapped out and I don't have it in me to try again now, sorry. It was a very long day because we got stuck in traffic, missed the audiology appointment, ran long with our vision check and oncology had an emergency in the morning that set everything back so that we didn't get in to see Dr. Belasco until close to 2:00 pm.
The highlights were that Max was a really great kid today and very pleasant to be with. Tracy and I were both concerned about the results but his vision is mildly better and the tumors, size wise anyway, are unchanged. The MRI shows a slightly different blood flow through the tumors but they don't feel that is anything to worry about now. We go back in 3 months to repeat everything and we'll make up the missed audiology appointment sometime before that locally.
This morning started early, again, because Max decided to wake me at 4:45 am to find out what time we were getting up today. I must have neglected to tell him that we could sleep until 5:30 am. Our trip out to Lehigh Valley Cedar Crest was pretty uneventful. Route 78 going the other way looked horrific but our way was mostly clear sailing except for the truck that had driven through the yellow warning barrels and onto the Jersey Barrier. They already had two big construction trucks there with arrows on the back diverting traffic so it must have been there for a little while but the driver was still sitting in the cab of his truck chatting with the state trooper down on the ground. Can't imagine he thought that was a good start to his day either. Guess it really is all relative.
We got here about 20 minutes early so Max got to play with his new Leap Frog hand held game. For 8:00 am in the morning, this place is pretty busy but also pretty organized. They give everyone pagers, just like many of the restaurant chains around here, so they don't have to announce names. We met Bert (Roberta) and she took us back to Max's room for the morning. He was pretty worried about things today but the numbing cream on his arm worked pretty well so there were no tears this morning! The hard part, as we sort of suspected, was getting Max to drink the glucose in orange soda concoction. Max shook with the first several sips and it looked like it would be trouble but Bert started counting to see how long he could sip for (and Max negotiated for two prizes since the drink was "really awful"). The whole thing took less than 5 minutes I think. We started the 2 hour timer and Max has been watching Nick, Jr. ever since.
We finished at about 10:30 and went to the cafeteria for a bite to eat since Max had been fasting since last night. They were in between breakfast and lunch but Max was able to find a banana, a piece of white bread, a bowl of cheerios, a container of milk and a couple packets of ketchup. While we were eating, Nancy from the 'normal' Lehigh Valley called to see what they were supposed to be doing for Max today. They knew about his Lupron but couldn't remember what they had told him they would do for the movie. Since we had forgotten to call them yesterday I offered that we would pick up the popcorn for the movies and be there at 1:00 pm. On the way over to the other hospital we stopped at a Giant to get popcorn and Target to get Max's prizes for the day. We made it with a little time to spare. Nancy came out to see what movie Max had picked but said she needed 5 minutes to tie up some work. Karen & Anne Marie took the opportunity to rush Max into a procedure room for his shot so that all he had to do was watch the movie. For whatever reason, Max wanted to lie across my legs so I sat on the stretcher in the room and he did lay across my lap - it looked ridiculous since I don't exactly fit on the stretcher but Karen stuck him and got the serum in before he really had time to cry. Still looked like it hurt but he didn't cry. First time for that and it was over in just a few seconds. What a change - he was really looking forward to the movie.
The movie was great and Max really enjoyed himself. He picked a Scooby Doo video that he has and everyone joined him in chairs and bean bag chairs. It was pretty comical to see. Even Dr. Phil sat for a few minutes and had a bag of Smart Food. They couldn't stay for the whole movie but Max kept shouting to everyone with updates so he was happy. He'd been looking forward to this for the whole month and it really made his day.
Bob talked with Dr. Moustofi today. The results appear to be what was expected and Max is likely to start Sandostatin treatments soon. Dr. Moustofi and Dr. Moshang want to meet with Tracy and Bob at CHOP on March 29 to go over the results and treatment options. Max doesn't need to attend so it shouldn't be too much of an impact. Dr. Moustofi assured me that there is nothing they aren't telling me, they just want to go over everything in person before treatments start.
Bob met with Doctors Mostofi and Moshang at CHOP to talk about starting Max on Sandostatin. In talking he learned that Max has pituitary gigantism and this will be reclassified to acromegaly (also known as gigantism) when he gets older. They were able to find 5 other children that had NF-1 and were being treated with Sandostatin. Because the numbers are so limited there just isn't a lot of guidance for what to expect for Max since most recipients of Sandostatin are adults. The good news is that the side effects of Sandostatin aren't as bad as the other medicines he's already taken.
There is a 20% chance that Max will have trouble with his Gal Bladder. It is unlikely that he'd get gal stones. Instead it would be kind of a sludge that would cause abdominal pain and could lead to problems with digestion and moving his bowels normally. If this does become a problem there is medicine that can help. Unfortunately because he'll be taking Sandostatin for the rest of his life, if he does have problems with his gal bladder there is a further likely hood that he'd need to have it removed. Not horrific but certainly something he could do without.
The consensus is that Max's elevated growth levels are a result of the tumor around the hypothalmus and pituitary. His tumors are inoperable and because he is young and has NF-1 it is unlikely that he'll receive radiation - the other 'common' way to try and stop elevated growth levels from pituitary tumors. Unless his treatment for NF-1 changes or his tumors change significantly they believe Max will need to take Sandostatin for the rest of his life.
Prior to starting Sandostatin they want to take one more set of blood tests to be clear on his hormone levels. Also, they want an ultra sound of his gal bladder so that they have a baseline in case he does start to have trouble with his gal bladder. Max is set to see the doctors again near the end of June. They'd like him to start in May so that he has two doses by the next time they see him. Max will be able to get the Sandostatin at the same time as the Lupron so it shouldn't be too much of a change for him. Hopefully he'll make the majority stats this time around and NOT have any reaction.
Max got his Lupron shot and additional blood work to check his hormone levels today. Sometime in the next week or two he had an ultrasound of his abdomin to get a baseline of his gal bladder and other organs in the event he suffers from the Sandostatin. Everything went well and Max is going to start Standostatin, get an MRI, eye exam and follow up with his oncologist the first week of May.
Being somewhat tired from TR & Kathy's wedding this past weekend, Max and I headed down to Philly last night to stay at the Wyndham. I had recently gotten a new laptop (mine suffered some trauma when it hit the floor in Florida last month) and I did not realize that my new laptop had no built in microphone. That made using the mapping software a little bit harder but it seemed to be enough to get us where we wanted ... until we got to Philly where I needed it to find the hotel. This is the same hotel that Max and I tried to find at night a while ago and ended up driving around the Four Seasons. I set everything up before hand on the computer and things seemed pretty good. We got off at different exit than before but it put us closer to the hotel so that seemed like a positive step. Then I realized that we'd started at 15th, were crossing 12th and wanted to go to 17th but the computer had been quiet for a while. Turns out it was too busy figuring out a new route to say anything. I ended up having to turn it off because it kept telling me to turn left when I knew I wanted to go right ... stupid technology. Max and I ended up going in two full circles before we saw the tiny Wyndham sign at eye level. Everything else said Sheraton or was really high up. I'm still trying to figure out what went wrong but I'll be using the software again this afternoon to get us up to Lehigh Valley since we haven't traveled that route before. Hopefully it will do better where the roads aren't so close and stacked two high like here in the city.
Max had slept in the car for most of the trip so he was a little awake when we got to the hotel. He made light banter with the ladies at the front desk and had I taken him to the bar I'm sure he could've made a few more dollars (he made $2 at TR's wedding doing bar tricks). Max was asleep again around 10:30 and then we were both up at 6:30 this morning. Things went well this morning and we got the CHOP almost an hour ahead of time. That has worked out well as our start time was 9:30, it is now 9:33 and Max is already 50 minutes into his scan. I talked with the nurses about trying to give him enough up front to keep him down for the entire scan because last time he needed more right at the end and that ended up making him angry Max for a good two days.
When he gets back to this room our plan is to get him dressed and wheel him down to the cafeteria so that we can get some food for the road before we head to Lehigh Valley for his Lupron and Sandostatin shots. If we finish as early as I think we will I'll have to call them to see if they can take him earlier since our appointment there isn't until 2.
Since I got burned once before by typing a really really really long email and then losing it, I'm going to update you in waves today.
Just a quick update ... the nurse just came in to tell me Max woke up and had to be re sedated. Should prove to be a nice long day.
It took about an hour for Max to wake up and the trip to Lehigh Valley was pretty uneventful. Max slept and the computer tried to take me there in a round about way so I shut it down ... again. We ate lunch in the hospital cafeteria and ran into the nurses while we were there. Max was a little grumpy and very un-balanced so we took our time and I tried not to do anything to start him on a tirade. I let him get french fries and potato chips for lunch. I'm sure the nurses thought that was a pretty good lunch for a growing boy.
Max's sandostatin drug is a little different than the lupron shot. The sandostatin must be prepared and injected in a very short period of time so we got to meet the pharmacist today. His nurse thought the needle was way to big so we went with a smaller one. Unfortunately the whole process of getting Max on the table for his shot took to long and the drug wouldn't go through the needle. He didn't cry for the first poke but he also didn't get any medicine. After some work on the sandostatin, Max got his second poke this time with medicine. This is much thicker than the lupron and, in fact, the residue on his butt afterward turned into a crusty paste pretty much right away. Needless to say he cried but not very hard and not very long. He was very brave but also thought two was enough. It took some convincing to get him to take the lupron. He was already tired so there were a few more tears but again nothing too bad. I was amazed that he didn't even say anything for the first poke so I was very proud of him.
It was at this point that angry Max came out and started to lecture me about many things so we headed home. Thankfully he fell asleep. He's on the couch now but I suspect, as does Tracy, that he'll be home tomorrow because he just can't go to school that angry. On Thursday he and I head back to CHOP for his eyes and to talk to his oncologist about the MRI and how things are going. Max will need blood work in two weeks to see how the Sandostatin is doing and then he'll be on monthly injections of both drugs for a while.
Today we had to be in Philly at 10:30 so we left at 7:30 to be safe. I had forgotten how bad traffic on 206 can be so we went the scenic route through Califon and High Bridge. Much nicer but a little bit longer. We arrived early for our appointment and Max got to play with Ben and his younger brother (two new friends). He was really great about sharing his L-Max (hand held electronic game) and his new Chitty Chitty Bang Bang car. I was pleasantly surprised. We learned from Dr. Liu that Max's MRI on Tuesday showed a stable tumor and his vision is also stable. He actually tested a little bit better with his left eye, 250/20 up from 300/20 last time, but they felt it was probably behavior related and he probably was always at 250/20. He is calling it stable and would like us to come back in 6 months but we have to check with his oncologist this afternoon to see if we're making it 3 months.
Max is now at 74 pounds and stands 4'7". I don't think that is changed from my last report but I can't find that right now.
It is now 1:10 pm and we've eaten lunch. We're going to go and do some homework while we wait to see his oncologist.
Max would like to say, "I hope that everyone that is sick gets better. And I miss all of you."
We got to see Dr. Belasco after doing some homework and she said that his MRI was stable. She agreed with Dr. Liu that eye exams could be done every six months from now on but he'll have another MRI in 3 months. We talked a little bit about the problems Max has had with sedation and we'll be switching to a modified General Anesthesia for his next scan.
We had a 2:00 pm appointment so I picked Max up from school at 12:35 and we went home to put some numbing cream on his butt. We got to Lehigh Valley by 2:00 pm and we played with Play-Doh while we waited for his injections to be prepared. When we got his pants set so that he could get his injection, the nurse said, "Aren't we doing two shots today?" Of course, I had only applied numbing cream to one cheek so I had to answer, "Yes, but I'm an idiot." because I'd forgotten to do both cheeks. We decided to put the Sandostatin in the numbed cheek and that actually went well. Max tensed up but watched his movie, Monsters, Inc., through getting stuck and receiving the medicine. The same for the Lupron shot in the other cheek that hadn't been numbed. I'm grateful that he is so brave for the shots but also a little saddened that he's had the opportunity to get so used to everything.
Today Max had his MRI scheduled for 9:30 am and we were to be here at 8:30. We left home at 5:35 am (after some debate about going down the night before but finally deciding it wasn't worth it) and we got here at 7:20 am. Unpredictable Philly traffic can be so much worse. It worked out though because Bob got to talk to everyone about the problems we had last time with Max taking two days to metabolize the drugs (and missing extra school) and also about the confusion with switching to General Anesthesia from his past sedation. There is more involved in GA that we didn't know about including a pre-visit down here to discuss things and meet with the Doc that would be with him during the scan. After our talk, they decided this morning to try a slightly different sedation method so he was given liquid versed to start. This is the same as his very first two MRI's. That got him groggy and silly (if you have heard Max tell jokes recently, I can assure you that it can, and does, get worse with meds). They are also planning on giving him additional meds with about 30 minutes left in the scan rather than a larger does at the very end. Finally, they're going to run a full liter of fluid through him during the scan to see if they can't jump start the metabolizing of the meds so he isn't mean for so long. Of course, I stopped bringing a complete change of clothes this time. Here at CHOP he gets special pajamas for the scan but keeps his own underwear. Hopefully Max won't have to go Commando for the rest of the day because I'm certain that everyone in the elevator and at Lehigh Valley would be told of this exciting new fashion.
Max did manage to make it through the scan dry. He slept off some of the drugs for about an hour and then we ran to the bathroom. We were able to make our appointment at Lehigh Valley where Max talked through his shots with only a brief pause. He's still pretty unsteady and very chatty but otherwise seems ok. We'll see how tomorrow goes.
Today we had a 9:00 am appointment to review Max's MRI with the oncologist as well as have him checked out. Not having been to the oncology clinic for a while the first thing that we noticed that was different was a maintenance woman who wiped down everything (chairs, doorknobs, blood pressure cuffs, etc.) after it was used. I had noticed that the woman who checked us in used a hand sanitizer after doing the paperwork but I didn't think too much of that. After Max got weighed, measured and had his blood drawn, we went to play area and noticed that there were far fewer toys. Max headed right for Kim, who runs the Child Life Clinic, to see what the story of the week was. Kim, among her other duties, dispenses prizes to kids who get the question about the Book of the Week correct. We read a book about a rain forest and Max got the question right but this time the prize was stickers. Max was clearly disappointed but actually handled it pretty gracefully. It seemed odd because Kim knew Max and knew that he always picks a car out of the toy bin (which is mostly McDonald's, Burger King, etc. Happy Meal Toys and pencils) but that wasn't offered.
I thought that maybe August was "Extra Extra Over the Top Germ Free Month" and that Scrooge had taken over the toys but I soon found out what was going on. When we saw Judy, the nurse practitioner that we first met at Lehigh Valley, she asked if we had gotten "the letter." At Lehigh Valley that always meant some kind of cool activity (talent show, meet Santa, meet the Eagles players) but at CHOP it means something is wrong. Anyway, the letter stated that there had been an outbreak of VRE which is some sort of nasty that is not normally harmful but can be a big problem for compromised immune systems. It is easily transmitted by contact but can live off of a person (on a toy perhaps). As a result, there are hand sanitizers all over and they removed the majority of the toys. One other thing that was different, as a result, was that Max was given a coloring book, crayons, and a goody bag that had some additional prizes but was 'sealed' with a sticker. The intent of the bag, I believe but could be mistaken, was to have the kids take home a couple of toys since there wasn't much to play with. As Max figured out, and I'm sure others did as well, the 'seal' was easily broken and the toys were quite playable. I was able to get Max to change to coloring a picture for Mommy pretty easily and we colored while we waited.
The wait really wasn't too long but we spent some of it at the Art table with a young boy who seemed to be there with his grandparents. I'm not sure what was wrong but his temper was very short and he said some pretty mean things to his adult companions. After the first such comment Max looked at me and said, "Wow, that's rude." loud enough for all to hear. Horrified, I leaned over to Max and whispered (praying that he would not repeat this) "Please don't say anything more about him until later, something is wrong, ok?" He astounded me by gently pulling away from me and giving me an exaggerated wink. I was so grateful but also curious as to where this boy was when we marched in two parades over the summer and he could not stop yelling about how this marching was ridiculous and he was calling the police because I was being a bad parent. Max is quite like a Forest's box of chocolates.
Finally, the good news! Max's scans were stable and that, as you've already learned, is good news. Of course we went over where everything was and how it is pretty incredible that his tumors are so close to such important parts of the brain (then again it is difficult to find a part of the brain that is expendable I suppose). The only sort of bad news is that his reflexes are still mostly gone. That is the result of one of the chemo drugs (vincristine) but she said in time his reflexes really should come back.
The plan now is to go back in November and have another MRI, an eye exam and a follow up oncology visit. If all continues to be stable then we will move to a schedule of repeating the visits every 6 months. That'll be a welcome change. He'll continue his monthly hormone shots and Endocrinology is likely to want to see him at different times but those visits aren't nearly as long lasting
Today Max has his MRI at CHOP in Philly. He is scheduled for a 10:00 am scan with a 9:00 am arrival so we tried to leave by 6:00 am in case we hit traffic. We didn't make it out until 6:15am so Bob didn't get coffee but we made it deep into Philly before we hit traffic. We got here at 8:30 and we were checked in by 8:40 am. Max is now 96 pounds and 4' 8" tall. Although we missed the traffic, for the most part, on the highway, there is some traffic here and it looks like we'll be delayed at least 15 minutes. Not a big deal but we'll see how close that estimate is when we get there. For now, Max has decided that he is tired and is taking a nap (several people have expressed concern that he's already sedated because he's sleeping so soundly).
Max got sedated and went into the scanning room at about 10:30. They seem to think it will take an hour but experience tells me 90 minutes. Anyway, I ran downstairs and grabbed something to eat quickly and now I'm in the MRI waiting room sort of hanging out until Max is done.
It was actually a little more than 90 minutes. Max was snoring so they had to intubate him via his nose to make sure he was getting enough air and to stop the snoring because it was making the images blurry. That must not have been comfortable because it was sort of long but thankfully at dinner tonight he had no memory of it at all. He also had to get an extra dose of meds right at the end so he was a little grumpy. The grumpy turned to mean when we had to wait 30 minutes for a wheel chair and he started saying, pretty loudly, "This is ridiculous and it is ruining my plan. It is not what I expected."
In event, we got home around 5:00 and Max slept the entire trip. He can't go to school tomorrow because he won't have metabolized the medicine until around noon tomorrow but otherwise he seems to be fine. We go back Thursday for an eye exam and to find out how the scan went.
Wow - it has been too long since I've update this site. Sorry but things have been busy and Max is doing well. His November scan was stable and his eye exam was fine!
Max had shots and a blood draw at Lehigh Valley today. The blood draw was so that his growth factors could be checked to see if the Sandostatin is still effective enough. Max, the nurses, and the social worker there sang "Part of Your World" from the Little Mermaid while he got his shots. There were some stumbles on the exact lyrics but all in all, it was NOT melodic. It took Max's mind off the shots and that's what counts. He chatted with the nurses for about 30 minutes after the shots about things like Peter Pan movies. Oh, and he looked at pictures of a cat in a toilet bowl - not sure what that was about but he certainly thought it was funny.
Max has been doing very well with the shots which are essentially his only treatment now. He is getting new glasses soon but that is just to update his frames and lens' prescription. We follow up with an MRI in May. If he's stable I think we stay on 6 month follow ups. He also has an appointment with Endocrinology in June when we'll find out if his Sandostatin and Lupron injections are still effective.