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.

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.