Day 28

Noah's fevers have stopped and he is looking very well. He certainly looks like he shouldn't be here anymore. However, his electrolytes are still an issue, and we continue to make small changes each day in the hopes that soon he will be stable enough to finish off his treatment at home. He is getting quite bored here and wants to be entertained all the time, so we are getting to the playroom and spending lots of time wandering the halls. All good signs that hopefully home is not too far away! We need to get out of here before Noah catches something new! Thanks for all the continued prayers and for the encouraging comments. We appreciate it so much!!

2 Steps Forward, 1 Step Back...

Noah has been spiking fevers again the past couple of days. So far his latest blood cultures are negative, so we are not sure what is going on. It is suspected that these fever spikes may be coming from his bone infections. If that is the case, then we just need to carry on with what we are doing. He has now completed 2 weeks of Ampho and antibiotics, and he has 4 more weeks to go. We are still unsure as to whether we will be able to get Noah stable enough to finish out the treatment at home, although his electrolytes are looking better the past couple of days. Noah's chest is much better and he is off the oxygen during the day now. We are still doing hypertonic saline masks 3 times a day which really helps loosen up the mucous in his lungs.

We are so tired of hospital life and feeling pretty frustrated these days. Thankfully Kailyn & Joshua were able to go to Mom & Dad's for the long weekend. Brad is at the hospital tonight, so I am able to sleep in my own bed tonight. How wonderful it is to be at home, even if it is only for a few hours. When I drove up to our house today, I saw the snowmen that the kids built this past week when we had our first good snowfall. It brought tears to my eyes...a reminder of all the simple things I am missing out on at home.

Day 20

Not much to update these days. Noah is doing well and we are just waiting it out here. His bloodwork was sent yesterday to confirm the latest diagnosis. The Ampho continues to deplete Noah's potassium levels and so he continues to need daily potassium infusions. The latest we have heard is that Noah will need a total of 6-8 weeks of Ampho. We are hoping that if we can ever get him stable on the Ampho, that we could finish some of it as an outpatient, however we don't know how long that may take. So for now we just continue to put in our time here and try to be patient.

More Rough News

Noah is doing much better today. Still no more fevers, his bronchiolitis is improving and today he was actually sitting up and playing in his crib. His platelets are beginning to come up on their own. His hemoglobin is still low, but no longer dropping. We are having a hard time keeping enough potassium in his body, as the Ampho B has been depleting his body of potassium, so he continues to get potassium boluses. Still no plan on how long we need to keep him on the Ampho, and so we continue to take it one day at a time.

However, we recieved some difficult news yesterday from the Metabolics doctor. They have diagnosed Noah with a metabolic disease, based on a metabolic workup that was done. The final bloodtest to confirm the diagnoses will take a couple of weeks, and so we won't know for sure for a while, but the doctors are quite certain from the high level of MPS found in Noah's urine that he has Sanfilippo Syndrome (MPS Type IIID). We are only just learning what this means, but basically Noah's body is missing an essential enzyme that breaks down a complex body sugar called heparan sulfate. The sugar slowly builds up in the cells over time, causing neurological regression and death usually in the teen years. It is a degenerative brain disease with no cure. So even if Noah is able to fight through all the health problems his chromosome deletion has caused and even if through hard work he learns to walk, talk and eat, there is this disease lurking in the background waiting to take it all away. This is so discouraging and just so very sad. I'm pretty overwhelmed by it all right now and it is so hard to try to look at the short term and not think too far ahead. Sometimes I think it's better not to know. And so I must ask "why God?". Does Noah not have enough to deal with? Does he really need a double whammy? It seems that we just manage to make it through a crisis, when another one is right in front of us. I know I will never understand these things and the future looks even more frightening right now.

No Fever!

We've made it 24 hours now with no fever spikes, and Noah's latest blood cultures are still negative! All good news! However, Noah seems to have picked up something new here. Lots of coughing and wheezing today, and needing ventolin masks every couple of hours. The chest xray from today didn't show any new change from his last one, so likely a virus. Between that and the hours on the Ampho (which today gave him a crazy rash), he doesn't get much happy time. He did make it to music time on the ward though, which is always a highlight for him!

The bone scan results are back, and it looks like osteomyelitis again. The infection has seeded in 2 places: one of Noah's lower ribs, and his T10 vertebrae. This doesn't really change to much as far as treatment, except perhaps how long ID decides we need to treat for. Noah's platelets have draopped again today and he's been having nosebleeds. His hemoglobin is also back on its way down. But for now, we are still holding off on more transfusions. Hopefully his body with begin to rebuild once this Ampho really starts taking effect. His kidneys are looking ok, and we are doing what we can do with extra fluids and sodium to protect them. ID is still quite concerned with the combination of drugs he is on, but they are undecided about how many more days of Ampho B we will do.

We've also come up with a new plan regarding Noah's line care. He now has his own protocol written up in his chart. No more alcohol swabs, but rather chlorhexidine on his line. An each time his line is accessed at all, the nurses have to glove, gown and mask, and use sterile technique. It may seem a bit extreme and it makes a whole lot more work, but we are hoping to prevent any new hospital bugs from making their home in Noah's line.

Hospital Update

Things have been up and down here with not alot of change. Noah is still spiking fevers, although today he only had one spike, so that's an improvement. He is in a very precarious situation right now. Having yeast growing in his bloodstream is very dangerous, and very hard to clear. But it was almost inevitable though, after all the antibiotics Noah had been on. We have 2 services with completely differing opinions right now. ID (Infectious Diseases) wants Noah's line out. And our surgeon refuses to pull the line. She is not convinced that pulling the line out would help, since we already have an infected clot and possibly a bone infection (still waiting on the final bone scan results). And right now, because of the remaining clots, Noah has only 1 site left to put a line back in. So it is critical that we try and save this line. There is no right answer here, and either way, we may lose this battle. And so it really is a waiting game at this point to see what Noah's body will do and we continue to reassess the line situation daily.

Noah started the Ampho B (antifungal) early Saturday morning. It's nasty stuff that takes 5-6 hours once a day to be infused. It makes Noah itchy, gives him a low temp and basically just makes him miserable. So far his kidneys are handling it ok and they are being watched closely. His platelets and hemoglobin have been dropping and he's been requiring more oxygen, so yesterday evening he recieved a blood transfusion. His hemoglobin is looking better today, however his platelets continue to drop, so he may need another one in the next day or two.

Thank you for all your thoughts and prayers. Please continue to pray for wisdom to make the best decisions for Noah.

Yeast

Things just aren't improving here. The line tip from the old line that was sent for culture came back growing Staph & Yeast. And now Noah's latest peripheral and new line blood cultures are also growing yeast. The lab is still trying to identify what type of yeast. This is very dangerous for Noah and yeast is difficult to treat. He continues to spike fevers as all these antibiotics aren't touching the yeast. So our brand new line is in jeopardy. ID wanted to pull the line tonight, however, our surgeon is out of town until the morning, so we decided to wait until she is back tomorrow before making any decisions. He had another bone scan done today to see if the yeast has seeded anywhere else. No results on that yet.

Noah will be started on Amphotericin (an antifungal medication), however the drug is very very harsh and can have bad side effects, including toxicity to the kidneys. He is at even greater risk as he still needs the antibiotics (Vanco & Gent) to cover the Strep bugs. These alone are hard on the kidneys. However, we have no other options at this point.

More Fevers

Noah had a pretty good day yesterday, but today has been spiking fevers once again, with his brand new line! His last cultures showed 2 different Strep bugs growing. Today we drew new cultures, so once again we wait for results. Until we can figure out what is going on, ID has added a third antibiotic (Vanc, Gent & Piperacillin). He is still needing the extra oxygen and is getting chest physio 3 times a day. His blood pressure has stabalized and platelets are coming back up to normal. The ECHO that was done in PICU showed another new clot in his right atrium.

Hoping the fevers are just viral. When his fever is down, he is actually in pretty good spirits despite it all! Thanks for all your prayers, visits and food!!

On The Road To Recovery

Noah is doing much better today. Surgery went well on Saturday and he now has a new line tunnelled into his left external jugular. He began to improve dramatically after his "sick" line was removed. We are still waiting the final culture results, but it looks like he is growing a Strep type bug this time. Yesterday he was going to be moved out of PICU, but he began to have some respiratory difficulties in the afternoon. A chest x-ray showed a small atelectasis (lung collapse) in his right lower lobe and his lungs were "wet" from all the fluid boluses he recieved during his rescuscitation. After some Lasix to get rid of the extra fluid, today he looks better. His respiratory rate is still high and he is still needed extra oxygen, but he was able to be moved out of PICU today and back on to the ward. He really gave me a scare this time and I am so thankful to see him looking improved. Praying that things continue to go in the right direction here. Thankfully Brad is back from Florida tomorrow!!

PICU

Hi. Just a quick update. Noah was admitted Thursday morning with line sepsis. He started going into respiratory failure on the ward yesterday morning. The rapid response team was called and he was taken to PICU. We thought we'd have to intubate, but with lots of rescuing with fluids and O2 he has somewhat settled, although is still spiking fevers on antibiotics, so he is going to the OR this afternoon for a line change. Hoping to see improvements after that.

Starting November in Hospital

This is Brad posting from FL. Nichole had to take Noah into the hospital yesterday due to difficulty breathing and fevers. He was also having difficulty maintaining a steady blood pressure. With fluid treatments, they have him stabilized and await the results from blood cultures to know if it is a bacterial infection or a virus.

Please pray that this will be a short stay for Noah and Nichole.