Adjustments

Our family is slowly adjusting to what our new "normal" looks like. I am feeling less exhausted each day. I am so glad our other three kiddos are not preschoolers anymore. At 12, 9, and 7, they are really quite helpful. They can make oatmeal, heat up cans of soup, and even make pancakes and waffles! And with some encouragement, they can even do quite a few chores inside and outside the house.

The two boys are still struggling with bronchitis. It just keeps on hanging on...since the end of July. Nathan just started on antibiotics a couple days ago and Micah just finished a week's worth. He's been feverish most of the day today and just moping around feeling lowsy. I'll probably take him back to the doctor tomorrow. Poor buddy! Please pray that Aliyah doesn't get this nasty respiratory illness that even I haven't gotten rid of 100%.

This summer, the boys have memorized large amounts of Scripture and done an inductive study of the book of II Timothy in preparation for the Bible Bee. The local contests will be held this Saturday all over the nation. They have worked so hard for this opportunity to compete; we pray that they are well enough to participate. We are so proud of them for putting so much effort into hiding God's Word in their hearts.

The learning curve for Aliyah's IV antibiotics administration was steep especially with a sleep-deprived brain. It is becoming easier now. As of today, I don't have the 16-step list in front of my nose that walks me through the whole sterile process. In brief, each time I give her IV meds, which is 5 times during a 24-hour period, I have to push 4 things through her PICC line in a very specific order... 5mL saline, antibiotic using an IV pump, 5mL saline, Heparin. One of the antibiotics takes 45 minutes on the pump and the other takes about 30 minutes. I start the first one at 6:30am and the last one of the day is done about midnight. The amount of medical supplies needed for just one week of this is astonishing. The bottom crisper in our fridge is filled, not with apples, but bags full of prefilled syringes of two different kinds of antibiotics and Heparin. Aliyah is still needing meds for pain management. Her knee is still warm and she still won't extend it voluntarily. I think this is to be expected. We're just doing gentle range-of-motion exercises right now. Her reflux has been horrible! She is swallowing frequently at night in effort to keep everything down. Another odd thing is that she is urinating excessively with no change in her diet. I called her doctor today and he said to monitor her for signs of dehydration. We have a followup appointment on Monday with both orthopedics and Infectious Disease. Also, the drain hole in her knee which was dry and scabbed over is now moist and oozing a little, so we're keeping our eye on that.

Home

I'm so glad to be home! Aliyah looked visibly more relaxed from the moment we put her in her carseat at the parking garage. We arrived home at 6pm and not 10 minutes later, a home health nurse showed up at our house to train me on how to administer the antibiotics with an IV pump. She didn't finish with all the paperwork, documentation, and training until 9:15pm. I just started Aliyah's first antibiotic on my own and should be finished completely with this set of meds by 12:45pm/1am. I'll have 5 1/2 beautiful hours of sleep until I need to wake up and prep for the next set of meds. I'll tell you more about that whole process later. Emails are preferable to phone calls at this point. I really need some uninterrupted sleep. :-)

PICC line success!!!

There were 2 failed attempts on her left arm and success on the right. They took an x-ray to verify that it was in the right place. It was 4cm too far in, so they just removed the dressing (while I held her in my arms) and pulled the line out a little bit. It has cm markings printed on it, so it was pretty straight forward. Apparently, the line was in the lower part of her right atrium instead of at the top near the superior vena cava. And yes, I had to look that one up. I'm getting an anatomy lesson over here. We got the next x-ray back, and it has been confirmed that the line is in the right place now. We're heading back to our room soon. Thank you for praying!

Things I'm thankful for...

1. A research hospital only an hour away from home
2. Doctors who listen carefully to Mom and Dad's concerns
3. The Ronald McDonald House room where I could get a quick snack, deodorant, toothbrush, toothpaste, shampoo, and coffee
4. Clean clothes
5. Prayers and encouragement from friends and family
6. Quiet nurses at night
7. Visits from friends and family
8. Kind and attentive doctors and nurses
9. Doctors who are humble and teachable enough to think outside the box
10. Kind and helpful "neighbors" in the next room over and their sweet little boy who brings a smile to Aliyah's face

Update

Aliyah's new IV had to be removed mid-morning due to leakage. The doctors don't want to torture her anymore, so she will receive antibiotics through her g-tube until her PICC line is inserted tomorrow morning. She has to be without food after midnight, so they will drip pedialyte through her g-tube rather than IV fluids. The anesthesiologist will have to start an IV in the morning to sedate her; I sure hope they put her under a little bit with gas before doing it. I will request that, of course. The antibiotics are already causing frequent and very loose stools, so I'm going to start her on probiotics as soon as I can to try to ward off these side effects that can occur from being on a lot of antibiotics. She'll also need barrier cream for the duration of the antibiotics to prevent them from burning her little hiney. I've been able to get Aliyah up and in her wheelchair for feeding and short walks around the hallways! The change of scenery has been nice for both of us. We're pretty much back on her regular feeding schedule now. Her gag reflex does seem to be overactive though, so she's thrown up a bit and has been choking more frequently. Her pain level seems a little bit worse today, probably due to more movement of her leg. Overall, I would say that things are going pretty well. We both had a fairly good sleep last night.

Plan B

We cannot be discharged until Aliyah has the PICC line inserted. Plan B is to have her go under general anesthesia at 8am on Monday morning. With her body completely relaxed, they are hoping the procedure will be successful. The same nurse will be attempting it using the ultrasound equipment for guidance, just as before. We will be able to go home Monday afternoon if all goes well. A home health nurse will meet me at home and teach me how to give Aliyah the IV antibiotic infusions. I expected today to be uneventful, but it started out with a bang. I woke up to Aliyah fussing and a nurse telling me that she had to remove Aliyah's IV because it was leaking. Ugh! Remember that Aliyah is a "hard stick". The first IV nurse tried 3 times and failed. Aliyah giggled the first time, looked worried the second time, and cried terribly the third time. A more experienced IV nurse was sent in who also tried 3 times unsuccessfully. Then, the Pediatric ICU cardiac team came in with ultrasound equipment for assistance and got it in the first time. That was seven pokes and digs, people! My poor girl was so upset. It's so hard to watch her go through that.

PICC line

Unfortunately, the PICC line insertion procedure was unsuccessful. They allowed me to be in the room the whole time! I had to wear a blue surgery hat over my hair and a face mask since it had to be a sterile environment. They even recruited me to hold her arm down under the sterile cloth for an hour. I am going to be so sore tomorrow. I had to switch hands in the middle of it because my right arm was starting to shake with fatigue. I think I told you that Aliyah typically has myoclonic spasms only as she is drifting off to sleep. Once she reaches a certain depth of sleep, they completely subside. Well, the sedation that they gave her for the procedure put her in that "drifting off to sleep" stage. When they first put the medication through the IV, she started making this choking, loud snoring sound. At the same time, her upper body started violently spasming. It was very disturbing to see her like that! I asked the doctor how she knew those weren't seizures. She said that she didn't. Not comforting! She was going to give Aliyah some seizure medication, just in case, but the spasms settled down to their normal rhythmic pace. Even with me firmly holding her arm down, it looked as though her arm had hiccups. After 3 sticks and digging around for an hour, they decided to stop and go with Plan B. It's too bad...if they had gotten the PICC line in today, then they would have been able to let us go home tomorrow afternoon.

The Plan

Aliyah started IV antibiotics last evening. They have her on two different ones to cover all possible strains of infection. Even though the doctors highly suspect Kingella Kingae, they want to play it safe since the bacteria cultures haven't had time to grow out yet. That can take anywhere from 1 to 7 days. Once they have the problem bacteria identified, they can tailor the antibiotics. In the meantime, she is on Clindamycin 3 times a day which works well against staph and strep. The second antibiotic, Ceftriaxone, given twice-a-day, works well against Kingella Kingae. Aliyah will be receiving antibiotics for at least 4 weeks, longer if there is bone infection as well. She will get IV antibiotics for at least the first two weeks, probably all four. In preparation for that, she will have a PICC line inserted around 2pm today. This is a long catheter that will be threaded through a large vein in her arm, up through her shoulder, and into her upper chest near the heart with the guidance of ultrasound. I have been assured that this is a low risk procedure. Aliyah will be moderately sedated for this one hour procedure that should take place in the treatment room right across the hall. All things considered, Aliyah had a good night last night. Her pain was well managed by the IV Tylenol. They are going to stop the IV Tylenol and go to regular Tylenol and ibuprofin given through her g-tube as needed. She'll also have oxycodone as a backup. The drain line in her knee was removed this morning. It had partially slipped out of place, but there was only a small amount of fluid being discharged anyway. One less tube! Yeah! Now it looks like Aliyah will be discharged on Monday at the latest. MAYBE even tomorrow! She has certainly perked up today...lots of smiles and some giggles, too.

Diagnosis

We had our consult with the Infectious Disease team. I would say that they are 95% sure of the diagnosis -- Septic Arthritis caused by a bacteria called Kingella Kingae. Kingella is a normal bacteria that is found in the throats of preschool-aged children. If something disrupts the mucosal lining of the throat, like an upper respiratory infection,the Kingella can cross the epithelium by using the RTX toxin to break through the tissue. Then it enters the bloodstream which allows it to reach deeper tissues like bones and joints where it seeds itself and multiplies. It most often affects the hip or knee joint which receive a large blood supply during these years of fast growth. Kingella is slow-growing festering kind of bacteria, unlike the more familiar strep and staph bacteria which cause fast and furious infection. The 35,000 white blood cell count also supports this diagnosis. The head of the Infectious Disease team said that he saw what appeared to be a small amount of bone irritation on the x-rays that were taken. Only a bacterial infection, not a viral one could cause this. To know for sure whether there is a bone infection, we must wait for one of two things: the bone biopsy to come back and/or a followup x-ray in 2 weeks and another in 4 weeks. If there is a bone infection, the x-ray will show that as time passes. As the bone heals, it would show bony changes on an x-ray. We found out a couple of interesting things. Cerebral Palsy has nothing to do with this infection. It was just a random, rare, and unfortunate occurrence. In fact, her doctor, the senior orthopedic specialist at Duke, has never treated another individual with CP who had this infection. Aliyah will not be more susceptible to this type of infection in the future, and there should be no negative long-term effects since we caught it so early. We are so thankful that it is an infection and not an autoimmune disorder; we can get this cleared up and leave this experience behind us. We know it has an end.

Post-op Surgery Consult

The orthopedic doctor made an incision on one side of Aliyah's knee. Another small incision was made on the other side of her knee to allow for drainage of the 9 to 10 liters of antibiotic saline that would be flushed through there. He said there was a lot of pussy fluid that had to be washed out. He was quite confident that there was infection in the fluid because it was so cloudy. He didn't see any evidence of bone infection, but he went ahead and took a biopsy of the femur just to be sure. He sewed up the main incision and placed a drainage tube in the small incision. The tube is about a foot-and-a-half long with a suction bulb connected to it. It will be left in place for a few days to continue to pull fluid off the joint. Her leg is currently wrapped up in a soft bandage with access to the tube for periodic drainage. Once the drainage has stopped for 12 to 24 hours, her orthopedic doctor will remove the tube. I've been told that removal of the tube is painless. It just feels like a noodle being pulled out of your leg. Nice, huh?

Smile

Aliyah just gave us one of her sweet smiles. She doesn't open her eyes more than a squint to peek at us. She's so out-of-it. Oh! She's asleep again! She'll be given Tylenol through her IV for the next 24 hours to help control the pain.

Fever

She has a fever of 101.8. We're supposed to keep it below 100.4 due to her susceptibility to seizures. We just gave her Tylenol and are waiting to hear what the plan is for intravenous antibiotics. The Infectious Disease team will consult with us about their recommendations later today.

Recovery Room

It took 3 hours to get Aliyah's pain under control. Poor baby! We are now back up in her regular room. She's dealing with nausea/vomiting probably from the anesthesia. She has been given medication to alleviate that, as well. She is now sleeping comfortably. Please pray that the myoclonic spasms (involuntary body spasms that occur rhythmically) subside. The spasms increase her pain greatly because they repeatedly jolt her swollen knee.

Surgery

Aliyah just went back into surgery. I had the opportunity to speak with her orthopedic surgeon for a few minutes. Apparently, Aliyah's case is continuing to stump the doctors. An accurate diagnosis is evading even the senior orthopedic specialist at this time. We know that Aliyah has excess fluid and inflammation in her knee joint. We also know that she has a very elevated level of white blood cells in the fluid that was aspirated yesterday. We do not know at this time whether her joint contains infected fluid or not. A staph infection would cause a higher white blood cell count. It's possible that a different bacteria strain is causing a lower level infection. The doctor will take a sample of the synovial membrane tissue for testing, as well. It could show whether an autoimmune disorder is going on. Regardless, her doctor felt it was prudent to flush the joint rather than risk having infection eat away at the cartilage in her knee which would cause permanent knee damage.

The Treatment Plan

The MRI showed inflammation of the knee joint and an abnormal collection of fluid within it. It also showed that the pocket of fluid just above the knee was connected to the knee joint. The good news is that it didn't look like the infection had gotten into the bone at all. It looked like it just involves the synovial fluid. The testing of the synovial fluid that was aspirated from her knee showed an elevated white blood cell count. Normal synovial fluid has less than 500 white blood cells per ml. A septic joint has 50,000 to 100,000 WBC. Aliyah's has 35,000. The senior orthopedic specialist, which we found out happens to be her regular orthopedic doctor, has decided that since the inflammation has been going on for nearly two weeks now, that it would be best to treat it as a septic joint. At 7:30am tomorrow morning (Thursday), Aliyah will go into surgery. It will involve making an incision along the side of her knee and washing it out with 10 liters of antibiotic saline. Ten liters! Isn't that wild? This should clear out all of the infected fluid. They will also look carefully at the joint, bone, and surrounding tissues for signs of deterioration/dead tissue. They'll clean out anything that they see at that time. The procedure is called Arthrotomy Irrigation and Debridement. If all goes well and looks good, they will be able to stitch up her knee and get her started on antibiotics. Thanks for keeping Aliyah, our family, and her doctors in your prayers!

MRI

Aliyah got an MRI at noon today. Because of her young age, it required sedation. When she arrived back in the recovery room, the orthopedic team descended on the anesthesiologist requesting to do an aspiration of her knee joint. They had seen some of the MRI films as they were being taken and saw fluid inside the knee joint. They wanted to get a sample of it for testing and to start a culture growing. It takes 24 to 48 hours for any bacteria to start growing. It is important to know which bacteria we're dealing with so the most effective antibiotics can be used. Anyway, they re-sedated her with a pain-killing drug this time and pulled about 6ml of cloudy yellow fluid from the joint space under her kneecap with a 16 gauge needle. I was told it looked like cloudy mountain dew. I'm so glad she was sedated for it! They almost did it when she was awake last night. Now we're waiting for the orthopedic doctors to do an initial test on the fluid, evaluate the MRI films, and determine the plan.

Knee Problem

Twelve days ago, on Friday, I noticed that Aliyah was sitting oddly in her wheelchair. Her right leg, which is always extended out, was flexed (bent) at a 90 degree angle. Upon further observation, I noticed some swelling and warmth just above her right knee. She would not voluntarily extend the leg, nor would she push hard into a surface that touched the bottom of her foot (a typical response for her). She also showed some signs of stress when I was pressing on her knee. The inflammation seemed a bit worse the following morning (Saturday), so I took her to the Duke ER. They took x-rays and bloodwork. Both looked fine. We were discharged at 11pm with instructions to followup with her regular orthepedic doctor in 7 days.

A day-and-a-half later (Monday), I called her orthopedic doctor and demanded that she be seen "today". The swelling and warmth was spreading down her knee. Her orthopedic doctor expressed appropriate concern. He sent her for an ultrasound which showed no difference between her left and right knee. He said that ultrasounds are very limited in their diagnostic ability for musculoskeletal issues, and also that x-rays wouldn't be conclusive about a fracture because her bones consist mainly of cartilage at this age. He thought that she likely had a fractured patella (kneecap) or a partially torn patellar tendon. The best way to diagnose these things is through MRI imaging. He said that regardless of the diagnosis, he would treat it the same...wrapping her leg in a soft cast for two weeks to give it support and keep everything on alignment during the healing process. He would remove the cast in two weeks and the knee should be healed up at that point. He said to use ibuprofen to help with pain management. The support that the cast offered seemed to make Aliyah more comfortable. I gave her ibuprofen that Monday, Tuesday, and then just Wednesday morning. She didn't need it again until Sunday afternoon when she started showing definite signs of pain and had a fever of 101.3. With renewed concern, I called her orthopedic doctor on Monday to fill him in. He did not feel that the fever/pain was associated with her knee, but was more likely the beginning of a viral infection. (Four of us in the house have been sick with bronchitis over the last three weeks.) He said to take her to her regular pediatrician if her fever went above 101.5 and have her checked out for a virus. I went ahead and took her in that same afternoon; her lungs sounded clear...everything seemed fine as far as illness was concerned.

Yesterday, Tuesday morning, Aliyah woke up at 6:30 crying hysterically with pain. When I picked her up out of her crib, she screamed worse...until her face was purplish and she was blue around the lips. She looked at me with wide terror-filled eyes. I gave her Tylenol, was finally able to resettle her back in bed, but she awoke in agonizing pain again two hours later. Feeling SO strongly that her knee was involved with this pain and fever, and with the encouragement of Aliyah's nurse aide, I removed the soft cast. Poor baby! Her knee was much more swollen than the week prior when it was wrapped and it was super-hot to the touch. She wailed at the slightest touch or movement to the area. After administering ibuprofen, at the urging of the on-call orthopedic doctor at Duke, we headed to the ER. This time at the ER, there were two inflammatory markers from her bloodwork that were abnormally high, the x-rays showed knee inflammation, and the ultrasound showed a fluid pocket above the knee and on the outside of the knee joint. The radiologist himself came in to see the ultrasound done in "real time". It was unclear whether the fluid was actually within the joint itself. The radiologist was going to consult with his senior advisor and research out what he may be looking at in her knee. It was decided that she needed to get an MRI done to get an accurate detailed picture of her knee for a proper diagnosis. We were admitted into the hospital last night around 10pm.