Day 167

Josh informed me that my posts are way too technical and hard to understand, so I'm going to try keep the post simple this time.

Jacob had another echocardiogram today which showed that the fluid around his heart looks about the same. I was hoping it would start looking better now that he's been off a couple of his more dangerous medications for a week, but I guess the good news is that it hasn't gotten worse. The doctors are thinking that it is probably a viral infection at this point. We go back for another echocardiogram on October 1st.

Jacob's blood work showed some improvement, so it looks like for now we will not have to go back to Seattle any time soon. The doctor decreased the dose size of a couple of medications to see how he does, so we will hopefully be able to come off of all immunosuppression in about a week and a half. Also, Seattle received the slides from Jacob's gut biopsies and confirmed what Kaiser had already told us about Jacob's gut not having any gvhd.

Jacob did well with his electrolytes on 5 nights of iv hydration this week, so we are decreasing him to 3 nights a week and will hopefully be done with that entirely in a couple of weeks. I'm hoping after that we can get his central line removed. That hardware coming out of his chest has been a part of him for 18 months. It's going to be strange and wonderful to not worry about caring for it any more. I'm sure it will take some getting used to for Jacob, too. He's had it for as long as he can remember.

So all in all, I think Jacob is moving in the right direction. It's just going to take him a long, long time to clear the virus that he is fighting.

Day 161

After rechecking his heart on Thursday, Jacob was discharged from the hospital. The fluid has remained about the same. The cardiologist said based on the size and location of the fluid build up, the risk of piercing his heart to try to drain it would be too great. So for now they are monitoring him closely to make sure it doesn't get worse. They have no way of knowing what caused the pericardial effusion without actually testing some of the fluid. It is most likely a virus based on the positive rhino nasal culture he had this week and the low grade fever he's had for the past couple of weeks, but other causes could be medication or gvhd. He also came back positive on a nasal culture for mrsa, something he most likely picked up from the clinic or hospital. It's not causing any problems right now. It's a type of bacteria that he would not be able to fight with CGD, but they think his new cells can keep it in check. We just have to monitor him closely for signs of infection, like always.

While we were in the hospital, Jacob stopped his tacrolimus and switched over to sirolimus for his immunosuppressant. For 2 days in a row his lhd improved, which seemed to indicate that the tacrolimus was the offender causing his red blood cell destruction. However, on the third day, his lhd jumped right back to where it was. Jacob is now switching off sirolimus to cellcept to see if we have any better luck. If our Kaiser team is unable to see improvement in Jacob within the next couple of weeks, then Jacob and I are heading back to Seattle. Kaiser has already started the authorization process.

Jacob was able to stay off tpn and even came off feeds Wednesday as he began to eat more. His favorite food in the hospital was chicken, but now that we're back home, he's all about the chips and salsa again. He still gets iv night hydration to get his electrolytes and to fill in any gaps in his fluid intake, and I can supplement his diet with some feeds if he is not eating enough.

Naturally with everything that has been happening to Jacob, I've been struggling with exhaustion and discouragement. It's difficult to hold on to hope when things continually go wrong for him. I still second guess myself if we made the right decision pursuing bone marrow transplantation. Some lucky CGD patients actually live a long and relatively healthy life. We seemed to have made matters worse for him.

Ethan's 7th birthday is right around the corner. When we first returned home from Seattle, I thought we'd be in a good spot with Jacob that I could throw a party for him. Now I'll be lucky if I'm even in town. It's hard making future plans and moving on with life when things change so quickly from one day to the next. I've learned I have to always have the laundry done just in case we have to whisk Jacob back to the hospital for a week or more at a time. I thought being home would be easier, but to be honest, there is just more to try to keep up on in addition to Jacob's weighty health issues.

Day 158

We are back in the hospital. I brought him in on Monday because his temperature started getting a little higher and his heart still seemed to be working really hard even though he got blood on Friday. The doctor also wanted to watch his electrolytes as he stopped his tpn and started on feeds. The transition off of tpn has gone smoothly so far. He tolerated feeds for 15 hours yesterday and he actually ate some dinner. This morning he requested milk and water and had several bites of a peanut butter and jelly sandwich. I asked them to hold the feed to give him a chance to eat more, and they said that was fine. Now onto the bad news. Monday night he had a chest X-ray to check for pneumonia. Thankfully his lungs looked fine, but his heart appeared enlarged compared to past X-rays. So yesterday he had an echocardiogram to investigate further and it turns out he has fluid built up around his heart which is making it have to pump harder. The inflammation doesn't appear to be blood pressure or virus related. He has a repeat echo today to see if it is worsening. We are hoping and praying that it will clear up on its own. Also, his blood cells continue to self destruct. As he comes off tacro this week, we hope that issue will resolve.

Day 153

Jacob continues to struggle with various problems. Earlier this week we noticed him getting tremors and getting dizzy and discovered that he was hypoglycemic. Further investigation revealed that his cortisol level was zero, which indicated that his adrenal glands were not working from long term use of steroids. He started on an oral hydrocortisone immediately and seems to be doing better in that aspect. Another problem he's been having is a low grade fever for the past week. All cultures have come back negative, so we are going to stop the neupogen injections to see if he was having a reaction to that. The final problem is that his hemoglobin continues to drop. He is getting a blood transfusion today, so that will hopefully perk him up. He is going to taper off his other immunosuppressant, tacrolimus, because his doctor has ruled out everything else and he believes the problem is medication related. Luckily that is an option because he does not have any evidence of gvhd. Jacob is eating a little, but he is still on 16 hour a day tpn and is now also tolerating some slow feeds, so we are hoping to slowly transition him off of the tpn. It's hard having him hooked up to pumps for the majority of the day. We have to follow him around the house with his iv pole any time he wants to move. It's rough, but better than having him stuck in a bed in the hospital. I'm glad we are able to care for him at home.

Here are some pictures from today's visit. Our appointment has lasted 8 1/2 hours and we still have to stop by the pharmacy. Plus now we are going to hit LA commute traffic... But like I said, at least we are not in the hospital and we can sleep in our own beds.