Category: Clark’s Progress

In preparation for Clark’s BMT this Monday, we needed to have his single line broviac removed and replaced with a double line version.  The double lumen broviac is ideal for placing medicine (or in this case chemo) at the same time as pulling blood samples.  Single line would require switching lines and flushing between pushing and pulling liquids.

As with every surgery Clark has had (this was his 4th so far), we are always nervous about putting him under the knife.  Luckily all went well, and he is recovering nicely. Clark didn’t appear to be in any pain, until lunch came.  He must have been starving as he completely devoured all of his food, and then ate some of Beth’s.

We are scheduled to remain at Kaiser hospital until Sunday, and then transfer directly to UCSF for the start of the BMT.

 

After pulling an all-nighter at ER the night before, Patrick and I hit the sack early to get some well deserved sleep.

But it wasn’t meant to be.

At 3am in the morning, we were jarred awake by the sound of the phone ringing.  When I answered the phone, there was a very urgent tone coming from the person talking.  It was the lab technician who was analyzing Clark’s blood culture.  He said Clark’s blood culture had grown and was showing signs of a category of bacterial infection called “gram negative rods”.  He said we needed to go to Oakland ER immediately for antibiotics, as this strain of bacteria can be very dangerous if not treated properly.  And just to understand how dangerous he was talking about, E-coli is one of the bacteria found in this category.

We quickly packed our overnight bags and headed to the Oakland ER. The trip turned out to be a very long one.  Over the next 8 days, we “lived” in the hospital while Clark was given antibiotics and a series of blood culture tests.

By the 4th day, the doctors were finally able to identify the strain of bacteria. Unfortunately, the antibiotics Clark had been on up until then were the wrong ones!  Clark had a rare bacteria that the current antibiotics were not effective at killing. The bacteria, Pseudomonas, can be normally found in dirt, plant spores or just blowing around. Most people are not affected by it, but as it so happens, this bacteria loves broviac’s, and people with compromised immune systems.

Since Clark wasn’t showing many signs of an infection (no fever and he was happy and energetic), the doctors assumed the bacteria was harboring inside of Clark’s broviac. So they decided to have his central line removed. This on its own is not a big deal since we were planning on switching it out for a double line this week anyways. The problem was that we would not be able to put the double line Broviac in until the bacteria is completely out of Clark’s system. And without some type of central line, giving Clark infusions or medicine would be difficult (or near impossible).

So, during the surgery to remove Clark’s original broviac, Clark had a PICC line inserted in his arm. This is basically a mini broviac, or catheter that was inserted in his arm to receive his medicine, or blood products if needed.

Late on the 8th night (Monday August 4th) we were finally sent home with oral antibiotics and instructions on how to care for Clark’s PICC line.  We were very glad to be heading home.  It gave us 2 days to plan for Clark’s upcoming BMT, and more importantly, some much needed TLC with Connor.

With less than 2 weeks to be admitted for his BMT at UCSF, our only goal is to keep Clark free of colds and sickness, as these are the only things that can derail the upcoming procedures.  But even with our absolute best efforts, we couldn’t seem to catch a break for our young boy.

After a great Sunday afternoon celebrating Connor’s 4th birthday, Clark woke up at 10:30pm crying and vomiting, with a fever slightly over 102 degrees. This was a panic moment for us.  Fevers this high generally mean there is an infection. Since Clark has a low white blood count (WBC), he cannot fight off bacteria infections on his own. We called the hematologist, and (as expected) they told us to go to ER in Oakland immediately.

We really don’t like going to ER.  It could be deadly for Clark to wait in the general waiting room filled with other sick patients and furniture covered in germs. Whenever we go into ER with the kids (aka the little germ magnets), we try to avoid touching anything and keep the sanitizer close at hand.  Lucky for us and him, since Clark has a compromised immune system we are put on the fast track after arriving in ER (where others may wait for hours).   With Clark being immunocompromised, we are given a private room (with a door) usually within 10-15 minutes.

As soon as we entered our room in ER, Clark had a blood culture taken. The blood culture determines if he has a bacterial infection, and if so, what type of bacteria infection he has. This usually takes 24 hours to get a negative or positive result plus another 24-48 hours to find out what strain of bacteria it is.  As well, he also received a blood draw for his complete blood count (CBC).  The CBC is to check his WBC count. If his numbers are high enough, he can fight off most infections on his own.  The CBC test generally comes back in only an hour.

After taking these, they immediately started Clark on an antibiotic delivered intravenously through his broviac. They do not mess around with immunocompromised children.  By giving this treatment immediately, his body can start fighting off possible bacteria before even getting the culture test results. As well, if the bacteria is in his broviac line, by putting the antibiotics through the line they can also make it is also clean from bacteria.

Within an hour, Clark’s CBC came back, and his WBC was higher than normal. This was great news ! It meant his body was attacking the bacteria. It meant we could go home after the 1st round of antibiotics, and come back in 24 hours for 1 more dose.

However, as always, there was a catch.  The CBC also showed that his hemoglobin (hgb) was down to 6.4.  The doctors generally recommend we do a blood transfusion when Clark is under 7 g/dL. The low hgb is a complication due to his Aplastic Anemia. While this result had nothing to do with his fever of bacterial infection, it did mean we needed to stay in the hospital “a little longer” to receive the transfusion.  We knew there was nothing little about the stay we were having.

The reality is that it can take some time to get a blood transfusion. There is time needed for the logistics of acquiring the blood for the transfusion, as well as the time needed to do the procedure itself.  First the doctors need another blood draw for confirmation of blood type. Then they need to order the blood from the blood bank, which usually takes a couple hours.  Finally, when they hook Clark up  the transfusion, the process of transfusing usually takes 3-4 hours.

We immediately knew we were not leaving the hospital that night.

Thankfully we have been through this before, so we knew it would be beneficial to request to be admitted into a room on the pediatric floor (rather than staying in the less comfortable ER). And as we expected, by the time the transfusion was complete it was 10am on Monday morning. Patrick and I (and Clark) were all exhausted from the long night.

And then we got more good news… The doctors decided it would be best to keep Clark “just a little longer” so they could administer his 2nd dose of antibiotics.

By the time they administered the 2nd dose, we were finally sent home.  It was 6pm Monday evening.  We had been in the hospital for almost 20 hours!!  At least we were able to come home early enough to give us a chance to play with Connor, have some dinner, and sleep in our own bed.

That is, until the phone rang at 3am with the blood culture result (to be continued…)

We are very close to the start of the bone marrow transplant (BMT). Officially, the timer will start when UCSF calls us and says they are  “activating” a donor. When we get this call, we will be in a 3 week countdown for the transplant.

We had a tour of the UCSF BMT facilities the other day. It is very interesting in how they have it set up. The room Clark will be treated in is behind another room they call the “anti” room. The “anti” room is a “prep room” where Beth and I will store and eat our food, as well as scrub up and prepare to visit with Clark. Clark will not be allowed out of his treatment room for the entire 6 weeks he is being treated at UCSF.  Beth and I will be allowed out, but we will need to sanitize up to our elbows before entering Clark’s room.

As well we learned that Beth will not be allowed to shower in Clark’s room, but she will be able to use the toilet.  If Clark was potty trained, then this wouldn’t even be allowed. There is concern about spreading outside bacteria by steam (shower) or having him touching the toilet (if he was potty trained).  Beth will have access to a shared shower room that is on the floor.

The most dangerous area in Clark’s treatment room is the floor.  Clark will not be allowed to touch the floor without being on a plastic mat or wearing some type of protective foot gear.  If he drops a toy from his crib, it will need to be completely sanitized before being handed back to him. As you might suspect, plastic toys that can be easily doused in disinfectant are preferred to stuffed animals (which have to be washed). Clark loves fire trucks, and action figures that ride in fire trucks, so we think this is a small blessing.

Clark will not be able to eat any home cooked food.  It will either need to be made by the hospital, or in a sealed package (Larbar is a good example).  After opening a food item, it will have to be eaten or thrown away within 2 hours.  Bethany can bring her meals into the anti room, but not into Clark’s room.  This includes drinking water as well.  There can be no risk of Clark reaching for someone else’s food, and being exposed to foreign bacteria.

The plan of action, looks to be that Beth and I will switch off staying with Clark. Beth will stay Sunday to Friday, and I will cover Friday to Sunday.  That way both Connor and Clark can see both of us over time.

The facilities overall are very nice.  They have many areas designed to allow people of all ages to decompress and de-stress.

It has taken a couple trips to the emergency room to have us re-orientate our thinking around what preventative measures are best for Clark.  One example of this was when we realized having him wear a helmet was better than having to take him into the hospital for random head bumps.  In our latest visit to ER, we’ve also realized that drawing blood or giving medicine with needles or an IV to Clark, also required a new approach as well.

After Clark’s first bone marrow biopsy, we were instructed that if he got a fever above 101.5 after the surgery, that we should immediately call the doctors office and let them know. A fever could mean there is an infection, and since Clark has a low white blood count, he would be unable to fight off bacterial infections without strong antibiotics pumped directly into his blood stream.

Exactly 1 week after the biopsy, Clark had a temperature of 102. So, while we were not surprised when the on-call hematologist sent us to the ER, we were still very nervous about getting blood drawn or an IV.  Patrick and I have learned from previous blood draws, that Clark was not an easy boy to stick with a needle. He has cute chubby arms with tiny hidden veins.

We arrived around 7pm, and of course the first item on the agenda – a blood draw. They wanted to check his CBC (complete blood count) and to check for any obvious bacterial infections.  This initial blood draw took 3 hours, 4 pediatric nurses, 1 anesthesiologist and finally a lab technician to get the minimal amount of blood needed for a small vial.  Through this first adventure, they had blown 4-6 veins in the largest and easiest spots to access.

Knowing more was coming, this was a disheartening start to our visit.

Then came another challenge.  While in ER, the nurses wanted to put in an IV line.  Why, we asked?  Because it “might” be needed later in the visit.  At first I refused, explaining how hard it was to get to Clark’s veins.  As well, I knew that:

1. they most likely would not get an IV in, and would ruin more veins in the process
2. or, the IV wouldn’t stay in a 1 yr old who is thrashing around

But fighting with ER nurses takes wisdom and practice, and on this visit, I was still learning both.  So sure enough, they placed the IV while Clark screamed and fought.

The ER doctor also wanted to give Clark a catheter for a sample of his urine (to check for a bladder infection). This I definitely had to question.  If the staff can barely get a needle in my 1 year old’s arm, I’m definitely not letting them jam a catheter into his junk.  Luckily this was one small skirmish we were able to win.  As it turns out there is a cute little bag that they can use to collect his urine sample. No needles, just a harmless sticker. I guess my persistence paid off. The sample was ready a bottle of milk and an hour later.

By the time 11pm rolled around, Clark’s blood tests came back.  He had low WBC, but with high Neutrophils (900) which is the part of the white blood cell that attacks bacteria.  So some good news, we did not end up needing the antibiotics…

BUT….

Unfortunately the tests did show that Clark’s platelets were dangerously low at 14,000 (normal is 150,000- 450,000).

Because of this, we were admitted at midnight to the pediatric ward for a platelet transfusion.  Most transfusions are done when the platelets drop below 10,000 but extra caution is needed because of Clark’s hemophilia. Platelets and Factor VIII both affect how well his blood will clot. At this point Patrick and Connor arrived at the hospital for support and for more information. Thank goodness, I couldn’t have gone through this without Patrick’s help.

As we had predicted, the IV put in from the ER nurse didn’t stay in.  Another vein ruined, and one fewer spot to place another IV.

Nurses have a funny way of slighting each other.  In this case, our pediatric nurse said that the ER nurse had wrapped Clark’s IV using the same practices as an adult, and not the appropriate method for a child.  My first thought was, why not go down and tell the ER nurse this info – maybe someone else would be saved the suffering of their child being repeatedly being poked with needles?

As this “knowledgeable” pediatric staff started to place a new IV into Clark, we learned the hard way that the best medical care Clark can possibly receive, starts with determination and persistence from his own parents.

Over the next 10 hours, multiple attempts were made to put an IV into Clark.  Each time escalating to a new set of “experts” on placing IV lines.  Pediatric nurses, to ER nurses, to pediatric doctors, to anesthesiologists. By the time the anesthesiologists (who we do consider experts) arrived, they were left with the smallest and remotest of veins.  Again, no luck.  Clark had been in the ER for 15 hours at this point, being woken up every 2 hours for doctors to attempt poking him again.  And each time, there would be 4 of us holding him down while he looked directly at us, pleading for the torture to stop.

And so at this point it stopped.

After the 4th attempt at the new IV, we said stop.  That was enough.  We questioned how badly he needed platelets, and the answer came back that we probably didn’t need the transfusion.  Apparently it wasn’t worth escalating the procedure of placing an IV into his bone.  The next step would have been to push a needle directly into Clark’s thigh bone, and feed directly into his marrow.  They felt that the platelet count was high enough not to warrant this emergency tactic.

When we finally left the hospital the afternoon of the 2nd day, it was with a tired little boy full of holes and low on platelets. His little pokes turned into bruises at every needle site. It took 2 full days of recovery before Clark is turning into his regular, happy, energetic self.

Here’s the benefit of what we learned from this visit:

1. Pediatric anesthesiologists are our best bet for placing an IV in Clark, although we’ve had success with regular anesthesiologists in the case a pediatric version isn’t available.  Everyone else is simply not allowed to try.
2. When needing to pull blood, ask for someone from the lab to come up to do it.  They are experts with pulling blood, as it is all they do all day, every day.  We’ve had 100% success with these folks doing the work.
3. NO TRIAL RUNS!  Don’t waste time with nurses or doctors (ER, pediatric, etc…) attempting to place an IV or pull blood.  SAY NO WHEN THEY SAY: “I’ll just take a look”.  This is code for “I’m better than the last nurse…”, but believe us, it isn’t true.  The truth is that each attempt just ruins the veins that are needed by the anesthesiologists or lab techs.
4. Arms are the best bet for placing a line.  Forget the ankles.  We have had 0% success with placing lines in the legs.  As well, attempt to place a line in this area is extremely painful for Clark, as well as awkward to attempt to place.
5. There is a ultrasound machine used in surgery to help Pediatric Anesthesiologists place lines in small children.  Suggest this if the risk of placing a line is high.

April 8th, 2014 was when the real adventure began for our medical journey with Clark.  While we had several key medical issues leading up to this date (Clark’s mild hemophilia, problems with his left eye, head injuries,etc…), it was on this date that we received confirmation that the smaller issues we were seeing might be tied to a bigger problem.

Two days earlier, Clark had a “comprehensive exam” at the Hematology clinic. A comprehensive exam is a way for us and Clark to meet all our doctors, nurses, physical therapists and social workers in the hematology clinic.  As well, it gives us a chance to address any questions we may have about Clark’s hemophilia as well as his physical and mental development.

In conjunction with attending the comprehensive, it is routine for the patients to have a blood draw and a CBC (complete blood count) done. This is to check factor VIII levels, platelet count, WBC (white blood count), RBC (red blood count) and liver functions.

On April 10th, after getting the results of the blood draw, alarms went off.  Our doctors noticed that Clark had low platelets, low WBC and low RBC. We were told to go in immediately for another round of labs to see if there were any mistakes. 3 separate blood tests all confirmed that his numbers were low, and decreasing.

2 conclusions were immediately drawn.

• Something was VERY wrong
• It might be either Leukemia or Aplastic Anemia.

We were immediately scheduled for a bone marrow biopsy to check for both of these diseases.  At the age of 1, Clarkie was to go into his first surgery.