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Let’s see, 2 divided by 3 equals?

Clark’s doctor made a funny comment the other day.  When we were talking about the bone marrow transplant process, she mentioned that one of us would need to join Clark in isolation when he is getting his bone marrow transplant. So I asked how parents putting a child through a BMT typically cope with 2 children.  She then said that siblings would not be allowed in isolation (due to the risk of viruses and bacteria), and that most parents simply divide the responsibilities and each take a child.

This prompted me to ask a simple but important question: who in the family goes to work in this scenario?

She looked stunned for a moment, and then recovered and asked if we had a family member that could watch our older son during the day.  I replied that we have no family living in the state.  She then asked if we had a nanny or daycare.  No to both; Beth is a stay at home mom, which is how we survive on one income.

This apparently was too much for her.  She said that many families go through this, and they somehow “manage”, and that we would be fine.

I assume by “manage”, she means “go bankrupt”, as medical expenses are the single leading cause of bankruptcy filings in the US.  While I would gladly take the role of watching my 3 year old son full time, someone has to go to work and keep the medical insurance current.  The costs with medical insurance are surprisingly high – I can’t imagine going through this without insurance.

But what was really surprising, is the disconnect between those providing medical insurance, and the individuals receiving this insurance.  Even car insurance has become smart enough to realize that when your current car is out of service, you may need to be covered for a loaner car – and hence they provide a mechanism to pay for this.

When it comes to medical insurance, if the parent responsible for watching your children isn’t available – you are on your own.  I almost wonder if the people who come up with this coverage all have nannies and full time daycare, and never think that there would be a scenario where a parent might stay home and watch multiple children.  It’s clear the medical industry doesn’t have a clue about this.  Even our “assigned” medical insurance social worker was at a loss.

In the end we have been pointed to a website that allows us to hire a caretaker for our oldest boy.  Daycare isn’t an option, as we can’t have Connor exposed to sickness, unless we want to keep the boys separated for 8-12 months.  Costs for a nanny would be in the neighborhood of $3200 a month.  Can you imagine paying this out of the current budget you have?  We can’t.

It is clear the system is very broken.  I’m sure every parent who goes through this type of situation probably thinks the same.  But until you do go through this, there is no way to conceive of what a real medical emergency looks like.

And I suspect this is why the system remains broken.

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What is the procedure to recieve a bone marrow transplant?

Here’s what it will be like for Clark to get his bone marrow transplant.

  1. Over the next two months, UCSF and bethematch.org are working together to identify the best possible bone marrow donor they can find for Clark.  The ideal match will need to be young, of similar genetic background (Irish and German primarily), not have any life threatening diseases, and has never been exposed to the CMV virus.
  2. After identifying a match, they then begin the process of double checking to see if the person is still willing to donate, going through a ton of paperwork and more detailed tests, and if this individual still doesn’t bolt by this stage, the donor will finally be ready to set a date to extract.
  3. Clark will need to be brought into the hospital ahead of the extraction date, and started a conditioning treatment (Chemotherapy).  This treatment will effectively kill all the white blood cells as well as the bone marrow in his body.  It will return Clark to a state of zero immunity, similar to how he was in the uterus 4 months before he was born.
  4. He will then be given the bone marrow cells in the form of a drip. This part of the procedure is very quick (and very cool).  The cells just need to be dropped into the blood stream.  They know where to go and what to do.
  5. Once given the drip, Clark will begin to re-grow his bone marrow.  But during this time, he will be susceptible to bacteria, virus and fungus.  So Clark and Beth will need to be kept in a special isolated room for the first 6 weeks of treatment.  This room will have special ventilation that will be purifying all the air coming into the room. Even with filtered ventilation, bacteria found naturally on and in the human body will also be a risk for Clark.  Antibiotics, anti-fungal medicine, as well as antihistamines will be given regularly during this time.
  6. Connor will not be allowed to visit at all due to the risk of virus and bacteria.  I will be allowed to visit, assuming I don’t have a hint of cold or sickness, and I will have to be scrubbed down before entering.
  7. After the 6 weeks in intensive care, we will hopefully see white blood cells rising.  If this is the case, we are on a track to success (but far from cured).  We will then be sent home to continue recovery.
  8. Clark will need to continue to be kept in isolation after returning home.  If we want Clark and Connor to spend time together, then Connor will need to be isolated from other children as well.  No pre-school or playing at the park with other children.
  9. Hopefully between 4-9 months after being sent home, all 3 of Clark’s blood cell counts will rise, and he will effectively be cured.  If so, he will have brand new bone marrow and will not need to take any ongoing drugs.

This is the plan, as long as there aren’t complications… Complications on this path are far and wide.

  1. Finding a good match is the first major hurdle. Doctors have assured us that this should be fairly successful, as the vast majority of the donor pool is of European decent.
  2. We have to be worried about his body rejecting the cells that are donated to him.  This rejection can happen in one of two ways:
    A) His body could attack the cells as they are coming in, never letting them root.
    B) Or worse, something called Graft-versus-host disease (GvHD).  This is where the new donated bone marrow takes root, and then starts to attack the recipient’s host cells.  This can take up to 2 years to show itself!
  3. Through the whole procedure, we have to be constantly vigilant to ensure Clark doesn’t get a whole host of problems while his immune system is compromised.  Pulmonary infection is the leading cause of mortality with children receiving bone marrow transplants.

We are cautiously optimistic about this procedure and wary of its risks, but we are very glad there is at least hope in curing Clark.

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What is the procedure to donate bone marrow?

When it became clear to us that Clark’s brother might have to donate bone marrow to keep Clark alive, we were very curious as to what the procedure was like, and how painful it was.  So here is the low-down:

When you donate bone marrow, liquid is essentially drawn from your hip bone with a needle. This liquid contains cells that will be put intravenously into the recipient.  Depending on the amount that is needed (babies need a very small amount, adults need more), and how “robust” your bone marrow is (generally based on how old you are), this may be a couple to a few pokes into your hip to draw liquid.  The hip bone is the primary source for extraction, as it is the largest and easiest bone to draw from.

The entire procedure is done while under general anesthesiology, so you will be fast asleep when it happens.  When you wake up, you will have an achy hip for a day or two.  Somewhat like if it was bruised from a “hip check” in soccer or hockey.

And that’s it, you are a hero, and you’ve saved someones life.  No long term side effects.  Well, not physical ones…

Now is surgery emotionally traumatic?  I would say yes.  I’ve had my bicep sewn to a bolt that was drilled into my forearm.  I was very nervous going into that procedure, as there was a chance I would never use my dominant hand again.  And when I woke up, it was a year long recovery.  Compared to that? Well, let’s say bone marrow donation is a walk in the park.

That said, surgery is surgery, and it can be emotionally traumatic no matter if it is your molars or a hip replacement. So you do have to come to terms with that. That said, for all the different acts of heroism in the world, this is definitely one of the easiest by far.

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What does it take to be a bone marrow match?

I get a lot of great friends and family asking if they can be the ones to donate bone marrow to Clark for his transplant. It’s a great question, and we really appreciate the offers, but unfortunately most will not be a match.

About 70% of patients who need a transplant do not have a suitable donor in their family. Half of Clark’s HLA genetic markers are inherited from myself (his mother) and from Patrick (his father). Each sibling has only a 25% chance of matching. We already know that Connor is not a match (unfortunately). Likewise, it is highly unlikely that other family members will match Clark. Under very rare circumstances, family members other than siblings may be tested.  Case in point might be where 2 sisters married 2 brothers.  Cousins from the other couple might be a great match.  No such luck in our case.

Human leukocyte antigen (HLA) typing is used to match Clark with a donor for bone marrow. This is not the same as ABO blood typing. HLA is a protein – or marker – found on most cells in your body. Your immune system uses HLA markers to know which cells belong in your body and which do not.  It is “acceptance” that is being sought after here.  If the body rejects the transplant, it can be a catastrophe.

There are 12-13 genetic markers that are tested to define a bone marrow “match”. The odds that two random individuals are HLA matched exceeds one in 20,000.  This is why it is so important for eligible donors to register.

The factors for Clark are largely Irish and German (with some Welch, Norwegian and other European Countries). So if you are full Irish, or half Japanese, then you most definitely won’t match. But if your grandfather was Irish, and your grandmother was German, there might be a match there. We are basically looking for people that might have been from the same “village” (back in the days those existed).

However, even if you are of a different nationality than Clark, please consider donating “in kind” for Clark. Donating actually isn’t a huge procedure, and you very easily could save someones life.

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Revelations on being a parent

I was suddenly struck with a revelation when thinking about Clark’s condition the other day.

When my wife and I decided to become parents, we thought we knew what we were signing up for. We take on the responsibility of raising the children, and if we do a good job, they move out and become responsible adults. 18-25 years, and we would be free to resume our intimacy and privacy.

In Clark’s case, it suddenly struck me that – beyond our best efforts – he might be unable to be an independent adult. That my wife and I may have unwittingly signed up for a life long commitment.

Which made me wonder…

In this day of knowledge, medicine, and (somewhat) economic stability – have we lost touch with the real responsibility of choosing to become parents??

Did parents of decades ago choose to have 5+ children, knowing that some of them may need the help of their siblings? Would couples in today’s age have only 1-2 children, if the odds of a life long debilitating disease was a “high” likelihood?

Would you think twice about having children faced with a possibility of something like this?

I look into Clark’s eyes, and I know the answer in my case. He gives it to me in one very large, and very loving, smile. I’ve chosen my path, and will remain steady through good and bad. Having seen the love in Clark, I know I would have regretted any other path that would have avoided such loveliness in my life.

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Is a helmet for a mild hemophiliac worth it?

We debated for more than 6 months before realizing a helmet would be necessary for Clark. At first, we did not really believe that Clark needed a helmet, since he only had mild hemophilia.  But after 2 back-to-back visits to the ER for mild head bumps, we realized any prevention is much better than any trip to the hospital.

So off to buy a helmet.  The only problem was finding a helmet that provided enough protection, and yet didn’t look too much like a medical device.

After looking at some terrible helmets, we came across the “No Shock”.  It’s a soft, adjustable helmet that looks more like an equestrian hat. It was one of the cutest helmets we could find that actually looked like a childs hat. It also has open spots on top to make it more breathable. A breathable helmet is more comfortable on a hot day in California.

We ended up buying the “No Shock” on Amazon.com.  It has turned out to be a fantastic helmet, and we’re looking at getting a second.  Easy to wear and size, and very versatile.  In this case, sometimes less is more, and the lack of cartoon characters makes it seem like we are simply overprotective parents, rather than carting around a child with a debilitating illness.

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Hemophilia vs. Aplastic Anemia

Clark was born with mild Hemophilia.  And then, at his 1 year birthday, was also diagnosed with Aplastic Anemia. Both blood disorders affect clotting and bruising, and both could be fatal.  But how do they differ and how do they relate?  What does it mean to have both?

Hemophilia is genetic

Hemophilia was passed down to Clark genetically, and it is related to a missing “factor” called Factor VIII (pronounced “Factor 8”) or Factor IX (pronounced “Factor 9”).  When missing, the blood in a person isn’t able to congeal, or scab.  In severe Hemophiliacs, a person could get a small cut, or bump into a chair, and literally bleed to death.  In Clark’s case, he is mild.  Which means unless he gets into a car accident, or is having surgery, he really doesn’t have to worry too much.

Mild hemophilia, on its own, isn’t a life or death concern.  You can plan around it, and generally have a very full (albeit cautious) life with this condition.

Aplastic Anemia is deadly

Individuals can get Aplastic Anemia through several means. There are dangerous chemicals that can cause it (Benzene) and there are genetic conditions that can cause it (DKC), or it can happen without any clear cause (idiopathic).

Aplastic Anemia is where the body stops producing new blood cells.   It is characterized by 3 blood cell counts being low:

  • platelets
  • white blood cells
  • red blood cells

As these blood counts drop, an individual is prone to spontaneous bleeding (low platelets), getting a bacterial infection and not recovering (low white blood cells), and to becoming anemic (low red blood cells).

Aplastic Anemia will kill you.  It does it from the inside out, killing your body’s ability to fight back or repair itself.  It is a very dangerous and deadly disease, and can only be treated by a couple methods.  Both of those methods have to do with repairing the bone marrow in the body.

Where they intersect

So in the case where a person with hemophilia has Aplastic Anemia, you have an individual which has both low Factor VIII and low platelets.  These two items work together to create a scab.  Platelets form a “framework” and Factor VIII creates “fibers” that bind the framework.  Not having one or the other can be a big issue, not having either can be a HUGE issue.  Where Clark is only a mild hemophiliac today, we have to treat him as though he was a severe hemophiliac when he has low platelets.

Transfusions

In both cases, there are transfusions available to shore up numbers of either Factor VIII or Platelets.  Factor VIII only lasts a couple hours, so it is only given when an injury has occurred, or a surgery will occur.  Platelets last a little longer, often a couple days to a week depending on their age and quality.  These are often given once a week depending on when a patient’s platelet numbers have declined.

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Nosebleeds: How something so simple, is now… complicated

When Clark’s older brother got a nosebleed, we quickly calmed him down, pinched his nose and tilted his head forward.  The bleeding usually stopped within a minute and we would then dust him off, and send him out into harms way again.

With our second child, Clark, it is amazing how different this simple injury has changed for us.

Clark woke up the other day with bloody sheets and a nose slowly oozing red. There are lots of reasons he might have a nosebleed:

  • banging his head accidentally when he was sleeping
  • itching his nose
  • dry summer air
  • etc…

As well, of course, spontaneous nose and/ or gum bleeds from his Aplastic Anemia.  Low platelets is one example of something that might cause this.

Nosebleeds can also be difficult for someone with hemophilia since it is difficult to stop the bleeding.  Couple this with low platelets, and we figured we were just seeing a little more active bleed than normal.

Clark was scheduled to get his platelets that day, so at first we weren’t concerned at all.  Our first thought was: get him his regular platelet transfusion, and it should help stop the bleed.  Clark received the platelets, and all was looking well. He had a little more color in his cheeks, and the bleeding has stopped.

Until the drive home. :(

When I arrived home and took Clark out of his carseat, I noticed that his nose had started bleeding again and blood was all over his face. I immediately gave him a dose of Amicar knowing that this drug helps to prevent saliva/ mucous from breaking down a clot. Unfortunately, an hour later and his nose was still bleeding. A quick call to the hematologist has us trying to squeeze Clark’s nose shut for 15 minutes. (This, let me tell you, Clark did not like!  I think he may never let us touch his nose again.)

Squeezing didn’t help either.  So I ended up driving back to the clinic for Clark to get an infusion of Factor VIII, hoping this might be the issue.  (Factor VIII gives him the clotting factor that hemophiliacs are missing.)  While at the clinic getting the factor treatment, we did another CBC.

This is where things got interesting.

It turns out Clark’s hemoglobin (red blood cells) were low.  He was at 6.7 and the doctors like to transfuse at 7 or less (normal healthy people are closer to 10).

At the time, my first thought was what would this have to do with it?  Red blood cells aren’t used for clotting.  When you are low on red blood cells, you are getting less oxygen (carried by red blood cells) to your organs, which can manifest itself in a child being lethargic.   No signs of that with Clark at this point.

But what we didn’t think about, was the fact that while Clark’s energy appeared normal, he was maintaining this energy by having his heart work twice as hard to get oxygen around his body.  Which means his blood pressure was also elevated.  As such, his nose wasn’t able to heal due to the pressure of his blood circulating around his body.  Think of it this way, if you had a bloody nose, and then started to sprint as hard as you could – would your nose heal?  No, it would probably start to gush.  This was effectively what was happening to him.

Sure enough, once he got his blood transfusion, his nose stopped bleeding altogether, and he (and I) could finally calm down and relax.  Once again proving, that with a Hemophiliac / Aplastic Anemia child, even the simplest of issues can cause huge problems.

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What is Aplastic Anemia?

When we first learned that Clark could have Aplastic anemia, we were completely naive on the disease and its effects.  Here’s a simplified version of what we’ve learned so far.

What is it?

Aplastic anemia (a-PLAS-tik uh-NEE-me-uh) is a blood disorder in which the body’s bone marrow doesn’t make enough  new blood cells.  There are 3 types of new blood cells affected, red, white, and platelets.

Red blood cells carry oxygen to all parts of your body as well as carbon dioxide back to your lungs to be exhaled. White blood cells help your body fight infections. Platelets are blood cell fragments that stick together to seal small cuts or breaks on blood vessel walls and stop bleeding.

As your body stops producing these blood cells, there are many health problems that can occur.  Heart failure, infections and spontaneous bleeding are some of the top issues.  Gone untreated, severe Aplastic anemia will most certainly lead to an early death.

Aplastic anemia and Hemophilia are completely separate diseases, and have no commonality or causality. Clark has both diseases, unfortunately.  Read this post to understand how the two relate, as well as differ.
How do people get the disease?

People of all ages can develop Aplastic anemia. However, it’s most common in adolescents, young adults, and the elderly. Men and women are equally likely to have Aplastic anemia.

The disorder is two to three times more common in Asian countries.

Your risk of getting Aplastic anemia is higher if you:

  • Have been exposed to toxins (such as Benzene)
  • Have taken certain medicines or had radiation or chemotherapy
  • Have certain infectious diseases, autoimmune disorders, or inherited conditions (such as Dyskeratosis congenita, DKC)
Is it curable?

Short answer is: yes, but the success rate is less than 100%.

How is it cured?

Treatments for Aplastic anemia include blood transfusions, blood and marrow stem cell transplants, and medicines.  Blood transfusions help only temporarily.  There are 3 ways to cure Aplastic anemia, in order of success rate:

  1. Bone Marrow Transfusion from a sibling (80% chance of success)
  2. Immunosuppressive Therapy (70% chance of success, if you don’t have DKC otherwise much less)
  3. Bone Marrow Transfusion from a stranger (varies, depending on the quality of the match)

When it comes to Clark, #1 will not work for us, as Connor is not a match.  #2 will not work as it is suspected Clark has DKC.  So we are left with only #3, and are in process of finding a match.

What is the likelihood of finding a bone marrow match from a stranger?

To make a bone marrow transplant successful, it requires that there is a genetic “match” between the donor and the recipient.  Read more about the requirements for a bone marrow match here.

What is involved in a bone marrow transfusion procedure?

In short, it is a 1 year treatment, in which the recipients body is taken to a state of zero immunity for a period of 2 to 4 months, while the transplanted bone marrow starts to grow and “take root”.  It is a complicated procedure with many risks.  Read more about the procedure for getting a BMT.

Where can I learn more?

The National Heart, Lung and Blood institute is the best reference I’ve seen to date on Aplastic anemia.  If you prefer to read offline, use this link to print all the topics at once.

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6 days in pediatric care

On April 22, our doctors came to us more bad news (a recurring theme in this whole fiasco).

If the doctors aren’t sure that you have Aplastic Anemia, but are pretty sure the CBC blood counts you have are from this disease, they might continue to test your bone marrow until the disease “declares itself”.  Bone marrow declares itself when the cellularity of the marrow is less than 10%.

From the continuing declining numbers in Clark’s blood counts, we would need to do another bone marrow biopsy, as well as platelet and blood transfusions to ensure Clark’s survival. They were still certain that Leukemia had been ruled out, but wanted to check to see if Aplastic Anemia was going to declare itself.

Up to this point, even an attempt to pull blood had been extremely difficult.  Our last time we tried to put an IV in Clark, we had a horrible 20 hour stay in ER and still didn’t get it in.

So we had to make a decision.  The stress of attempting to find veins in Clark was physically and psychologically damaging.  And if he needed an IV for a bone marrow biopsy, plus transfusions twice a week, plus pulling blood samples twice a week, plus (possibly) chemo therapy… We needed a better solution.

So we asked our doctors to implant a Broviac.  You can read more about the device here. By placing this “central line” inside Clark, doctors could easily give or take liquids, without the use of needles.

So in 1 days notice, we packed everyone up, and went for an extended stay in Kaiser’s Oakland pediatric facility. We arrived late in the evening to “check-in” and give us “in patient” status. This allowed us to start Clark’s surgery 1st thing in the morning.

Clarkie’s room had a crib, fold out couch, a private bathroom and a ton of medical equipment. This was a very cozy (as in, tiny) room for 4 of us to spend the week. We were able to ask for a hospital bed instead of a crib. This allowed Patrick and Clark to shared the bed, while Connor and I slept on the couch.

The morning of the 25th, Clark went in for surgery. Factor VIII was given before and after the surgery. They performed the marrow biopsy, and then the broviac insertion, followed by platelet transfusion and more factor. The surgery went well with no bleeding issues and the broviac was able to be used immediately.

The hospital stay lasted 6 days (4 people, small room). Clark received factor treatment 9 more times during the visit. Not because of any bleeding, but evenly spread out as a precautionary treatment. During our stay, we received training on how to care for his broviac and to wait for his test results to come back.

Sure enough, this time Clark was diagnosed with Severe Aplastic Anemia. He has a 5% cell growth. 10% is considered Aplastic Anemia. Clark is now being tested for multiple genetic links to Aplastic Amemia (approximately a 10% chance), as that may change the course of treatment. Patrick, Connor and myself were tested to see if we are a bone marrow match. The results will be available in a couple weeks.  Connor stands the best chance of being a possible donor.