Clark’s white blood count (WBC) continues to approach zero, as expected. The drop is a result of the conditioning (chemo) from Day -10 to 0. Once it bottoms out, and the new marrow starts to engraft, we will slowly see his counts rise again.
With his low, or absent WBC, Clark is prone to illness. This includes viral, bacterial and fungal infections. Right now, Clark is in the “danger zone”. His body is unable to fight off a mild cold, let alone anything more serious.
Clark has received an allogenic transplant. Meaning he is using marrow from someone other than himself. Patients undergoing allogeneic transplantation are at a greater risk of infection because of a longer time to achieve engraftment (prolonged neutropenia) and the added risk of GvHD.
Engraftment is the process in which the transplanted stem cells find their way to the bone marrow spaces in the centre of the large bones of the body. Only then can the transplanted stem cells begin to produce new blood cells. Engraftment happens in stages:
The first sign of engraftment is the gradual rise of Neutrophil (WBC). This can occur as early as 10 days after transplant but is more common around 14-20 days.
Shortly after Clark’s WBC starts to show signs of engraftment, his platelet counts will start to rise. A platelet count of 20,000 to 50,000 is a sign of platelet engraftment.
Lastly, we have red blood cells (RBC). The engraftment of RBC’s Usually occurs a couple of weeks after everything else. There is no “exact” time frame for engraftment to occur. Many things play a roll in how Clark’s body reacts, such as his specific disease, his age, and the quality of his old blood cells.
While there are many risks involved in Clark having a bone marrow transplant, the hope is the benefits outweigh the risks. In Clark’s case, without a BMT, he would most definitely die at a very young age. But thankfully, with a successful transplant, Clark can at least live a life without the need of regular blood transfusions.
After Clark ate a full breakfast, we had a “surprise” visitor… Let’s call him “The Book Fairy”. The book fairy came and read stories to Clark! Only moments after story time was over, the “Music Man” arrived. Clark was serenaded, and given musical instruments of his own to play along. And that’s not the end of it…
Next was Physical Therapy. This may sound like work, but for a child who is not in physical pain, this is actually closer to “playtime”. They walked around the room, climbed on my bed, and basically just played around! All the excitement of the day made for an easy transition to nap time. This was probably the most “full” day Clark has had since he came into the hospital.
About 2 weeks before Clark came to UCSF, he had a mandatory dental exam. The purpose, was to identify potential sources of oral infection that can lead to bacteremia (bacteria in the blood). It is important to prevent or eliminate dental infection before chemotherapy or other medications that lower the ability of the body to fight infections. Severe gum disease, tooth decay, tooth abscesses and poor oral hygiene can lead to pain and/or bacteremia before, during and after chemotherapy. Thankfully Clark had a clean (dental) bill of health.
It is extremely important to keep the mouth clean and healthy during the bone marrow transplant procedure to help reduce the risk of infection and bleeding. Research has shown that mouth sores are less severe in people with excellent oral hygiene. Some of the preventative medications Clark is on are usually very effective in lowering the risk of viral and fungal (thrush) infections in the mouth as well as in other areas of the body. Fewer infections in the mouth results in less pain and better nutrition.
Clark being silly while Mom brushes his teeth.
With that in mind, one of Clark’s daily rituals, is brushing his teeth! Unlike big brother, Clark actually loves brushing his teeth. This is wonderful, since we have to brush them 4 times a day! He uses a new disposable sponge brush each time he brushes, and instead of toothpaste, Clark uses Sodium Bicarbonate (basically a liquid baking soda)….which, oddly, he likes the taste. Flossing and the use of a normal toothbrush are not recommended when the platelet count is lower than 50,000. To date, Clark has had no signs of mouth sores!
A vital part of Clark’s care revolves around his central line, aka double lumen broviac. This line is key to Clark receiving his medicine and having blood drawn. Without this, Clark would be covered in holes and bruises from ongoing attempts to put IV lines in his arms, legs, or anywhere else they might have luck getting a vein. The double lumen broviac has 2 lines, one red and one purple.
A couple of days ago, Clark’s red line (the one they typically use to draw blood) started to feel sluggish. Instead of having an easy time drawing blood, there was resistance. And over the days it only became more resilient.
To prevent clotting in the line, Clark has a “maintenance” drip of saline constantly going into both line when there are no medicines being administered. The resistance in his line is most likely from blood clotting in his line, or from a medicine that leaves residue inside the lining of his tube.
Instead of waiting until nothing would draw at all, it is best to do something to clean it out ASAP. At home, when there are no maintenance IV lines pumping liquid through, we do a Heparin lock (aka “Heplock”). Heparin fills the line and helps prevent clots from forming. We would do a Heplock every 24 hours. In this case, we already had a clot or build-up, so we needed something stronger to clear it up. So last night we put tissue plasminogen activator (TPA) in his line for two hours. TPA is a protein involved in the breakdown of blood clots. This is only used in the line, and does not enter his body. Once the TPA has been in the line for approximately 2 hours, it is drawn back out with a syringe. So today, after using the TPA, his line is clean as a whistle!
Today was another great day! The nurses and doctors believe Clark is having very mild side effects of the chemo. We’ll take that… and we are grateful for it.
Nachoooooo Libre
So many of you have been asking about the plane that scared Clark. Well, here it is. It definitely isn’t the friendliest looking thing, that is for sure. We think it’s the engine sounds frightens him. No worries, say the word little man, and I’ll get Daddy to take this to a quiet farm up in Northern California, where it will live out the rest of its days playing in the fields and sunshine.
Day +6 started REALLY early for us. At 1:30am, Clark started whining, sat up, and started emeses (plural form of emesis). Thankfully this was short lived, and he fell back to sleep once we changed his sheets.
Clark loved the car-go-round
Clark had an exciting morning while playing with the Child Life Specialist. They played with puzzles and a toy car slide while sitting on a large mat on the floor. There was also a cool airplane that made flying sounds. For some reason the airplane freaked Clark out! Once he started using the “all done” sign, I decided it was best to hide the airplane in the closet. :)
Throughout the morning, I have noticed a small rash on Clark’s body that comes and goes. At first it was on his inner thighs, then his back, followed by his cheeks. One minute they will be there, the next they are gone. A rash can be a sign of GvHD, so I told the doctor once he came in the room to do his rounds.
But not this time… I was told the GvHD doesn’t usually show up until the engraftment stage (still about 2 weeks out). So the doctor isn’t worried about this rash being GvHD. Instead, there is a chance this could be something called Peri Engraftment Syndrome (ES). It was described to me as a slight reaction to the new bone marrow. This can cause an unknown fever, rash, and occasionally inflammation of the lungs. This is usually treated with corticosteroids. Once treated, it usually lasts 1-5 days. We are not at the point of starting corticosteroids yet, because we are not sure if ES is the culprit. More watching, more waiting…
While Clark is in the hospital building up his immunity, he is on a long list of IV fluids and medicines. One of his many transitions in preparation for going home, is to switch his IV medication to oral meds (can you imagine us at home with an IV pole? No way!) Today was Clark’s first switch! He started his Cyclosporin (Neoral) orally at 7am, and will be given this every 8 hours for the next few months.
Clark’s Neoral levels will be closely monitored until the doctors feel he is getting the proper dose before going home. Cyclosporin helps prevent GvHD, but there is definitely too much of a good thing when it comes to this medicine. Side effects from too much Neoral include damage to the liver and kidneys. It can also cause elevated blood pressure, shakiness and rare cases, seizures.
We also meet with the nutritionist weekly. She makes sure Clark is eating enough foods and gives advice on how to increase his calorie intake. If Clark stops eating, he will be put on an IV nutritional supplement. Loss of appetite could be due to mouth sores called mucositis, or from nausea. Both are common short term side effects caused by the damage done by the chemo.
We would prefer to avoid IV nutrition, or at least prolong his eating as much as possible. We can help prolong his intake by “giving in”, and feeding him cookies and ice cream for dinner! The nutritionist has some better/healthier ideas, such as adding sour cream to his soup or drinking pediasure as a supplement. I am not a huge fan of high fat or high sugar diets, but I would prefer this (for the short term) to using IV supplements since they can have a host of side effects including GI issues, liver damage and more.
Overall, Clark’s day was quiet without any (obvious) nausea. Here’s a interesting word of the day for you… “Emesis” is the medical term for vomit. Doesn’t that sound nice? Clark was emesis free today. Clark’s nemesis is emesis. Ha, ha…
Clark had a long nap this afternoon and finished out his day with a fun visit with daddy.
This is the dreaded Day +4. This marks the starting of Clark’s lowest counts. This is the beginning of when we expect to see his negative reactions to the chemotherapy, as well as when he is the most susceptible to infection. It is also the ideal opportunity for the new bone marrow cells to start growing.
Patrick and I had the opportunity to go outside and grab some lunch this afternoon. Clark has just fallen asleep, and we had an hour and a half until he needed his next set of oral meds. We made sure the crib railings were secure. The nurses turned on the camera and sound for the room, and we were on our way!
It was such a beautiful day outside, with the sun shining and a cool breeze blowing. The neighborhood around UCSF is very San Francisco-esk with the tall 3 story homes and the steep hills. We made it to a nearby taqueria and had just enough time to order our burritos when my phone rang.
It was the hospital… Apparently (in the 15 min we had been gone) the nurse had to turn Clark over because he was squishing an IV line (this happens often). Clark started coughing, which turned into full blown vomiting! It sounded like the nurses were having a hard time calming him down, and needed us to come back ASAP!
Luckily, the nurses managed to calm Clark down before we returned. We were very relieved to see this, since Patrick and I were a 15 minute walk away when we received the call! Unfortunately Clark’s nap ended up being only 15 minutes long due to the unkind wake up, and he wasn’t able to fall back to sleep. The night ended with no additional signs of upset stomach, but he was a little grumpy from being sleepy. (Heinrich?)
It’s difficult to tell the reason why Clark started to vomit. It could have been a cough that triggered his rather sensitive gag reflex, followed by the fact that neither of his parents were there to calm him. Or this could have been nausea and vomiting due to his chemo. Clark has a daily drip of anti-nausea medicine. This is needed to keep his stomach calm. But it is very possible that the medicine is not strong enough some days.
So, hopefully an isolated incident, and not the “wrath of Day +4”. We spent the end of the day playing by the window and enjoying cookies. If Clark is going to start rejecting food (due to chemo), Patrick and I decided we should start including foods he never says “no” to. That way we can see if the problem is the type of food, or his stomach. Cookies and ice cream accompany every dinner because of this. Plus… YOLO, hombres!
One of the things we ask the doctors most often is… how do we know if Clark is doing well? This isn’t like a cut, where you can see it scabbing and know it is healing. Nor like a muscle, when you can measure its range of motion and strength. So, what do we have to look at to measure progress in this phase?
As we talked about earlier, when destroying his existing bone marrow, we were most interested in Lymphocytes. And sure enough, these were “crushed” to an immeasurable amount just as we entered transplant. But what are we watching for now?
It turns out there are both positive and negative indicators that we will be focused on over the next 2-4 weeks. The positive ones relate to growth of the new bone marrow, and the negative ones relate to attacks on his body externally and internally.
The positive measures will take some time. This will come in the form of ANC, neutrophils and white blood counts rising. We don’t expect to see any change in these numbers until about day +14. This is about how long it takes for the bone marrow cells to take root and start producing.
Until then, we are really just on the lookout for any negative reactions. There are several measures that we are watching diligently.
One is fevers. During the time Clark is Aplastic, he is really running without any immune system. So basically he is an open smorgasbord waiting to be devoured by viruses, bacteria, and fungus. While we have several prophylactic drugs running through his body to prevent anything from attacking him, these are general drugs, as we cannot give him a drug to fight everything. So we watch for fevers, and if he gets one, then we have to take a blood culture immediately and make sure it is a bacteria that we are addressing through the existing medication. If not, we have to start giving additional medications.
The other more concerning negative indicator is (of all things) a rash. A simple rash that grows worse over a couple days will be the number one thing we have to watch for. Why? This symptom is a strong indicator of graft-vs-host disease (GvHD). If this occurs, we will have limited options available to us. Most likely we will immediately start immunosuppressive therapy, to try and suppress the immune system and help the bone marrow “reset”. This would be days of additional drug therapies, and it would be very stressful.
So, in a nutshell, we are trying to keep Clark healthy until the new bone marrow takes root, and at the same time hoping the new bone marrow doesn’t reject its host. This two week phase is the toughest part of the treatment process, as it is simply a waiting game – void of many indications of success, but riddled with possible failures.
Over the last week, Clark has had some mysterious night fevers. During the day Clark is happy, and his energy level is high. Nothing out of the ordinary; however, starting around midnight, Clark has a fever for a few hours!
This is puzzling the doctors. Clark continues to have blood drawn to check for bacterial and viral infections. Today he also had an ultrasound for his spleen, kidneys, and liver to find out if there is an abscess on any of his organs causing infection. The most likely cause of this fever is a reaction to one of his medicines. The precautionary tests are purely to rule out a more serious cause.
Since Clark is now technically 2 people, I decided that Clark’s new alter-ego needs a name. When Clark is acting like himself, he will be know as Clark – obviously. But when Clark starts acting unusual, or just not like himself, or simply grumpy… He will be known as “Heinrich”.
It took some time deciding on a name for this alter-ego, but since the donor is European, we thought it only appropriate to give it a strong European name. In the case of a bone marrow donor, race is a big factor that makes the HLA markers match. Our doctors noticed mostly Irish and German markers in our DNA. So I decided to go with a classic German name to give homage to our German DNA. Heinrich was the obvious answer. I don’t know why, but I love this name. Especially when spoken with a strong German accent!
Clark’s bone marrow is now (almost) empty, and we are awaiting the new bone marrow cells to take root. This is the start of the “Aplastic” phase. It will take approximately 2 weeks from today for the marrow to start to show solid signs of growth. In this phase, Clark is at his highest risk of catching a virus, bacteria, or fungus. We have to be extra careful during this time.
If all goes well, our Aplastic phase will be pretty boring. To give you a taste, here’s what Clark and I go through on a daily basis. This is an example of what each day over the next 4 weeks should Iook like:
The start of our morning is generally at shift change for the nurses. This occurs at 7am. After meeting our new nurse for the day, Clark receives his 1st set of his eye drops for his glaucoma in his left eye. We repeat these drops 4 times over the course of the day.
Next, Clark receives his oral meds. One to protect his liver, an antiviral and anti-nausea med, and vitamin D3. Occasionally he will also have an antibiotic or blood pressure medicine when needed. Then we brush his teeth with a special “sponge” toothbrush to help reduce the acidity in his mouth and protect his gums and cheeks from bleeding. This is also done 4x throughout the day.
I’ll then pick up the phone and order breakfast. They deliver food on a “room service” model. We can order up to 5 times a day for Clark. They have a large and diverse menu, and we can order Clark whatever we think he will eat. They then deliver the food within an hour. Normally he eats a pretty diverse breakfast of oatmeal, eggs, bacon, vanilla yogurt and milk. We try and pick several different foods, knowing that his appetite is dwindling and that we might get lucky and have him take a few bites of several different foods before he tires of eating and goes back to playing.
In between breakfast and lunch, Clark has playtime in his crib, or by the window. Around this time, we are usually visited by the attending doctor and the resident doctor for their “rounds”. This is when we get a chance to ask the doctor any questions we might have. The doctor uses this time to see how Clark is doing while informing us of anything new we will be doing that day. For example: today we were told we will be getting a platelet transfusion.
Lunch varies, and I just try to keep things interesting with Clark’s food order, only so that he doesn’t associate one particular food with his hospital stay. For lunch, we’ll typically get something like soup, chicken quesadilla, banana, yogurt, and a milk.
After lunch, Clark usually takes a nap. After his nap, I will give him his daily “bath”. This is actually a chemical bath with chloro prep wipes, since we can’t actually put his broviac bandage in water. We then use this opportunity to change the linens on Clark’s crib. My bed also needs new linens daily, so I’ll usually do them at this time as well. I don’t mind getting fresh linens every day, since thankfully I don’t have to do the laundry!
I try and get Clark dinner somewhere between 5-6pm. This meal often includes mac & cheese, grilled cheese sandwich, soup, cottage cheese, whole milk and brown rice. If Clark has been really good, we sometimes add in a chocolate chip cookie.
After dinner, the day winds down at about 7pm with another nurse change. At about 8pm we do a whole host of oral meds and other IV medicines. I will generally try and get Clark to go to sleep at about 8:30pm. This varies greatly depending on how much he slept during the day (less sleep = later bedtime, surprisingly).
As well, throughout the day, there are also several diaper changes, IV fluids changed, and Clark’s vitals are frequently checked. The routine can become a little boring over time, but every time a nurse pops in, it is a chance to chat and do something that takes my mind off of things!
Boring is good at this point in Clark’s healing. Surprises tend to be bad. So as “boring” as it is, I kind of hope it continues.
This marks Clark’s official second birthday. Coincidentally, it is also Patrick’s birthday! Happy Birthday Patrick!
Clark celebrating his second birthday after the transplant.
The actual transplant was pretty straightforward. They gave him some pre-meds (Benadryl, cortisone and Tylenol) to help manage any allergic reactions, and then intravenously pushed in the donated bone marrow over a 2 hour period. Clark did amazingly well. The Benadryl made him drowsy, so he was asleep the entire time. The really good news was that he didn’t get a fever or show any signs of an allergic reaction.
Clark sleeping through the transplant process. If you look closely, you can see the red line containing marrow.
I won’t say the process was stress free, as we were told that the bone marrow donor was a different blood type (ABO) and a different RH (-/+). These two together represented a small (but higher) risk of allergic reaction. So the nurses and doctors were in the room the entire 2 hours, taking temperature, heart rate, and blood pressure every 5 minutes. Yes, every 5 minutes.
During the 2 hours of the transplant, we had time to talk to the doctor about what the process to extract and deliver the bone marrow is like. It is a pretty amazing story.
There are several steps leading up to the donation of bone marrow where they check, and then double check they have a good candidate. But we were really interested in hearing what happens from the extraction to when it arrived in the syringe for implant. Especially since we were aware this donation came all the way from Europe!
Each pull gets 10ml of liquid. For Clark, 500ml was required.
The extraction process starts 14 hours before Clark’s transplant, where the patient is admitted to the hospital and prepped for surgery. After prep, they are then taken into surgery and put under general anesthesia. This is because the process of extraction is somewhat intrusive. A needle is pushed into the lower back (if you have back dimples, this is exactly where they enter) and about 10ml of liquid is extracted. Then the needle is pulled out, and re-inserted into the same place, but at a different angle. They repeat this until they get enough liquid. The amount depends on the size of the recipient, but in Clark’s case, they extracted about 500ml. this means the donor had approximately 50 insertions. Most likely 25 on each side of the pelvic bone.
After the liquid is pulled, some quick tests are done to ensure they have pulled enough stem cells, and then all of the extracted liquid is thrown into a cooler. There are cases where the liquid has to travel longer, in which case a preservative is added and the liquid is frozen. In our case, because Europe is only 10 hours away, no freezing is required. They just need to keep it at room temperature the entire way.
The cooler is then handed to a volunteer delivery person, who immediately jumps in a cab and goes to the airport. The “courier” will have 2 tickets for flights to the destination city via 2 different airlines. This is done just in case a flight is delayed or cancelled. The bone marrow is carried by the courier the entire way, and they are not allowed to store it in checked baggage nor do they allow it to go through scanners. This can be very stressful for the person charged with carrying the marrow. The upside is that the volunteer gets a free flight to the destination city, and usually books some extra days of vacation before they return.
When they arrive in San Francisco, they immediately jump in a cab and drive to UCSF. The UCSF lab receives the marrow and starts to process it further. In our case, the bone marrow donor had a different blood type than Clark, and the lab needed to remove red blood cells before transplanting. Due to time constraints, they can only do one pass of this process, which did leave a small risk of allergic reaction because of the residual blood cells.
Finally, the remaining liquid is loaded into an oversized syringe, and brought up to Clark’s room for transplant. It is then placed into a drip line on his broviac, and administered.
A wild ride, all timed out and executed perfectly.
Another interesting fact we learned today, is that Clark will actually have 2 blood types. More likely than not, the donor marrow will only constitute 80-90% of Clark’s bone marrow. The other 10-20% will be his original marrow. Both marrows will continue to produce their respective blood types. This is actually considered a good thing, and will greatly help reduce the chance of Graft-vs-Host-Disease. If the bone marrow were completely empty, and then the donor marrow took root (and was 100% of Clark’s bone marrow), this would almost guarantee GvHD. Having a tiny bit of the original marrow acts as a cushion, dramatically reducing the chance of GvHD. Now, why not aim for 50-50% of old and new marrow? Because the body will most likely just reject the new marrow. It is a balancing game that is almost all science, but also a little art.
We are both blown away by the process, and in awe of the donor who went through this with us. Thank you again to our generous and kind donor who gave their marrow for Clark. We are very, very grateful for your donation. Thank you!!
