- Blood Draw
- Nurse Visit
- PA/Doc Visit (We met with Natalie Riotto, PA-C & Dr Ann Woolfrey)
During the first day of LTFU, we go over everything that has transpired in the last 12 months. *whew* Then we go over his list of appointments for the week and decide if anything needs to be removed or added. It was decided that his scheduled Pulmonary Function Tests needed to be cancelled. He is just too small to do these tests and get accurate results. Instead, we added a chest x-ray and a high resolution CT with sedation.
We left clinic, did some REAL grocery shopping and returned to RonDon. That night there was a family meal. These meals are amazing. Volunteers come in, from various organizations, and prepare meals for the families staying at RonDon. This is also the first time Gregory has been able to participate in these meals. He was tickled. We had meals every night. The second night I asked Gregory if he wanted to go down and see what was for dinner… he said: “We are having ANOTHER FEAST! Eeeeeeeeee!” We also had entertainment Monday and Tuesday night. Monday night’s entertainment was hilarious. For everyone. Devin Reynolds was his name. Seriously. If you are in the Seattle area and need comedic entertainment, this guy is fantastic. Once again, for all ages. Just enough of that adult innuendo that is above kids’ heads to keep adults giggling in their seats. Gregory got to help with one of his tricks and wouldn’t you know it, Devin made a rabbit appear. Wait for it. Here is comes. As soon as Devin lifted to cover off the pan containing the rabbit, in a raised voice I tell my child (and the rest of the room) “Gregory! Don’t touch the rabbit!” Ugh! Yes, he is still immune compromised and I am anal retentive. Hey, it’s boded well for us, so far. Keep reading, you’ll understand soon. Gregory was, once again, filled with glee to to do this. Can you tell why I heart the RonDon so much. It is such a bright spot for us families.
Tuesday we had:
- Physical & Occupational Therapy Exam
- Dental Exam
- Eye Exam
The PT/OT provider was AWESOMESAUCE! We were once again back to SCCA. Little did I know that they had recently brought in a PT/OT person just for peds. She was wonderful with Gregory. He had a blast while she evaluated him. Over all Gregory is doing great. He still has some tightness in his ankles, refuses to hop on one foot and insists on going down stairs one at a time. Meaning, down one step with both feet. Not one foot per step. Make sense? These are minuscule things that we are now working on and Gregory is fantastic at knowing he need to do these things and beaming with pride when he practices……”See Mommy!”
Dental was not a surprise. I must admit, brushing his teeth is really low on my priority list. Yet, it’s been 2.5 years since he has had a dental cleaning. He cannot have one until he is free of immune suppressing medications. He has eight crowns and four caps on his teeth and they help to create gingivitis. Which Gregory has a
healthy dose significant amount of.
We had to travel to Swedish for his eye exam. They were not able to get us in with Seattle Children’s ophthalmology, which is where he is usually seen. This turned out to be a great thing. Gregory saw Dr Richard Bensinger. He was able to answer my question about cataracts, gave me some great information and is now in our back pocket should Gregory ever develop eye GVH. My question about cataracts was…. WHY do steroids cause cataracts? What is the physiological answer? He quite simply answered with: We don’t know. Which is an A-O-K answer. So… we do not know why steroids cause cataracts, they just do.
Wednesday we reported to Seattle Children’s for a DEXA scan and bone age x-ray. The DEXA scan looks at his bone density. With Gregory’s prolonged usage of steroids, we monitor the density of his bones. Steroids also cause us to lose it. Gregory does have Osteopenia, but it has not progressed. *whew*
Early Thursday we reported, again, to Seattle Children’s for his chest x-ray and High Resolution CT with sedation. On Wednesday night Gregory started with a wet cough. It was not productive, he did not have nasal secretions, he did have sneezing. It was too late to call Seattle Children’s to talk about his sedation the next day, so we just proceeded as planned. He had his chest x-ray, then we went over to Nuclear Medicine. We had a chat with the anesthesiologist and it was decided that the risk of sedating him with any kind of respiratory symptoms was not worth proceeding with the CT. Gregory has had High Res CT’s every six months for the last 18 months. Each one has yielded no results. Clear CT’s every time. So we headed back to RonDon and hunkered down. Gregory was starting to feel run down and now that he had respiratory symptoms, he was on house arrest. Confined to our room. In order to protect the other families, if you have symptoms you are sequestered to your room. Which was OK. We both needed the rest.
When we were at Children’s on Wednesday, we went to the Hem/Onc unit to visit and catch another of my favorite docs. Dr Lauri Burroughs was attending on several occasions during transplant stay. It was another opportunity for one of Gregory’s docs to see him post transplant and doing well. We did get The Look from several of our previous nurses, yet this time I knew it was coming and quickly assured them that we were there just to visit during our LTFU. While watching the relief flood their faces. It was soul filling to see Lauri. For both of us. Not only are these doctors managing patient care, but they all have areas of research. They leave clinical practice and work in the lab, every month. Yes, I am in love with our treatment center. Fiercely.
Friday we had our conference to discuss the results of the weeks worth of tests. There was an additional lab draw added and we had to have that done first thing in the morning. When we returned in the afternoon to meet with Dr Woolfrey, we were pleasantly surprised to find that Dr Burroughs was covering the out-patient clinic. (Nothing against Dr Woolfrey, I just really, really like Dr B!)
- Gregory remains 100% donor and no evidence of disease.
- His cataracts remain. Plan: continue to monitor every six months.
- His Osteopenia remains, no progression.
- Chest X-Ray: clear
- Bone Age ~ a little over 5 years.
- Despite what I feel to be low counts, they are considered normal. Even though they are not in the ‘normal’ range. (Hematocrit, Platelets, White Count & ANC)
- He has Iron deficiency. Which is weird. Transplant patients receive so many red cell transfusions that more often than not, they end up with iron overload. Gregory is now on a twice daily iron supplement.
- Gregory has: Methemoglobinemia. Methemoglobin is an oxidized form of hemoglobin that has an increased affinity for oxygen, resulting in a reduced ability to release oxygen to tissues. Which is most likely why he has decreased oxygen saturation. This can be caused by Dapsone. We have discontinued his Dapsone and replaced it with Bactrim. SCCA recommended that we have a pulmonology consult to rule out any other causes for his decreased oxygen saturation, including a home Pulse Ox to spot monitor him and a sleep study. The reason Gregory is on Dapsone to begin with is that Bactrim suppressed his counts, immediately post transplant. The theory is that since he is so far out from transplant, this should not happen again. We will see at his next clinic visit on the 28th. Dapsone/Bactrim are antibiotics that we use as a prophylaxis to prevent Pneumocystis Pheumonia.
- Gregory’s immune system is not successfully reconstituting. From what I gather… Gregory is producing white cells. The cells that fight infections. What he is not making are antibodies. The cells that recognize what needs to be fought, then signal the white cells to fight. He has bee receiving IVIgG, since about three months post transplant, every time his level falls below 400. Which has equated to every 4-8 weeks. IgG are donor antibodies. It is separated from whole blood donations and comprised of antibodies from 1,000 different donors. We now have a plan in place for Gregory to receive IVIgG every four weeks. Keeping his level above 600. This also means that Gregory cannot be re-immunized. Not until he is making his own antibodies. We will continue this therapy until Gregory is able to sustain/increase his IgG level. Once that happens, we will discontinue IVIgG, wait three months, then re-check his immune status. *sigh*
- We have been given the green light to discontinue his Sirolimus. Provided he does not have any GVH flares. Gregory will have his labs checked at his July 28 appointment and then we can simply STOP the sirolimus.
I’m sure I’ve left out several details. This is what I have from memory. Once I receive the LTFU packet of results from SCCA, it will refresh my memory and I can fill in the blanks. I do not know what the last three items mean in terms of being able to recover. The data review conferences happen so fast that I often can’t formulate good questions until after I’ve walked out the door. I do have a list of questions for Dr J when we see her later this month. Gregory see his endocrinologist on July 14. It does sound like Growth Hormones may be off the table. *squee* Hopeful, but not holding on to that. We did learn that one of Gregory’s chemos leaves him with a 30% chance of thyroid problems. The risk does not decrease as he ages. At any time it could crop up.