Ep 488 – Spherocytosis: “I Have a Client Who . . .” Pathology Conversations with Ruth Werner

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0:01:05.4 Ruth Werner: Hi and welcome to "I Have a Client Who... " Pathology Conversations with Ruth Werner, the podcast where I will discuss your real-life stories about clients with conditions that are perplexing or confusing. I'm Ruth Werner, author of A Massage Therapist's Guide to Pathology, and I have spent decades studying, writing about, and teaching about where massage therapy intersects with diseases and conditions that might limit our clients' health. We almost always have something good to offer, even with our most challenged clients, but we need to figure out a way to do that safely, effectively, and within our scope of practice. And sometimes, as we have all learned, that is harder than it looks.

0:01:52.6 RW: Dear listeners, I promise I thought this month's episode was going to be short and simple. There's my first mistake. I ended up having such an interesting time with this topic, and I hope you do too. Here is the story from our contributor. "Ruth, I was wondering if you have any information on this. I have never heard of a blood disorder called hereditary spherocytosis. Are there any problems with getting a massage?" I asked the client to ask their doctor. I read about that the blood cells are abnormal and can cause spleen problems. The client is coming to see me this week. I've been reading about this but I can't find anything on massage. I asked a doctor friend, not the client's doctor, and they said this was sort of similar to sickle cell disease. The client is 40 and wants a massage for his birthday. But when he filled out his medical form, I was like, yikes. He had brain tumor surgery three years ago. The brain tumor was not cancer, thank goodness. But this blood disorder is making me really cautious. Do you have any thoughts about what I could do? 

0:02:58.3 RW: I've been researching a more gentle Swedish massage and no deep tissue is what I'm looking at. And here's how I replied, "I think it has to depend on his general resilience. Can he exercise, play sports? What happens if he gets a bruise? What meds is he using and what are their side effects? And mostly of course, what does he want from his massage. So if he wants a relaxing treat you're probably on safe ground once you gather this core information but if he wants anything more specific like deep work on his legs or something you might have to be more cautious." These by the way listener are the words of someone who doesn't know very much yet about the condition that we're about to explore. Our contributor responded, he takes no medication for this. He told me he's had jaundice before. He said, "I don't take medicine or have any problems with physical activity. I did get a note from my doctor today." This is Ruth again. You all know what I think about doctors' notes.

0:03:57.9 RW: However, I want to add that this interaction was done through MyChart and this kind of portal can be extremely helpful to massage therapists and their clients. The contributor said, I asked what's your goal for massage relaxation or pain relief and the clients reply was, "Both. I work in a factory, I lift a lot at work, my back hurts after a while, gets better over the weekend and then starts all over again on Monday." He said he doesn't bruise easily. So, my listeners, to review, here's a 40-year-old man. He has a physically demanding job. He wants massage for his birthday. Don't we all? And also for both pain relief and relaxation. Not that those are two different things. But he had brain surgery for a non cancerous tumor three years ago and he has a condition I have never heard of, spherocytosis.

0:04:54.1 RW: The brain surgery doesn't appear to be relevant anymore, hooray, but the other thing, spherocytosis. Take that word apart and what do you get? A sphere, a cell, a condition. Condition of spherical cells, specifically red blood cells. Okay, before we take this any further, let's do a quick review of red blood cells and how they interact with the spleen and the liver. You will recall, I'm sure, that red blood cells are manufactured in the bone marrow. They are our most numerous cells. We have 20 to 30 trillion of them and they account for about 70% of all of our cells. Red blood cells carry oxygen to our other cells. They live for about 120 days and when they can no longer fulfill their function, the spleen filters them out of the bloodstream and recycles several of their components. One byproduct of red blood cell breakdown is bilirubin. This travels from the spleen to the liver via the hepatic portal system, and the liver uses bilirubin to make bile.

0:06:05.2 RW: As it happens, bilirubin, which is golden brown, is also the thing that colors our poop. And that little factoid can take us down all kinds of detours that I am just not going to follow right now. So our red blood cells merrily do their thing. They make the complete circulatory circuit about once every minute. They come whooshing out of the heart and aorta and into the smaller arteries and arterioles. And then they line up one by one to squish through those tiny capillaries and they drop off their payload of oxygen and then they go back and do it again. After about four months they begin to degenerate and on their next trip through the spleen they are pulled out of circulation and recycled. In the meantime, our bone marrow continues to produce red blood cells at the same rate that we lose them and everything balances out and that system works great. However, sometimes things go very wrong. In one condition that you have probably heard of, hemoglobin molecules inside the red blood cells are abnormal and the shape of that cell, which should be a biconcave disc, which is to say a disc that's a little thicker around the edges, the shape of that cell collapses and it becomes a sharp-edged curve.

0:07:25.2 RW: And what I've just described is sickle cell disease. This is the result of an inherited genetic anomaly and it affects mostly African Americans and people of Mediterranean descent. Sickle cell disease can cause all kinds of damage especially when the sickled cells accumulate in small arterioles and capillaries and these are painful sickle cell crises. These cells have a much shortened lifespan, and because this is a genetic disorder, treatment options are very, very limited. However, we have some data that massage has been found to be really helpful for pain and anxiety and depression with people who have sickle cell disease especially for children. Hereditary spherocytosis has a lot in common with sickle cell disease. It is also related to an inherited genetic anomaly. In fact, five specific genetic changes have been identified, although it can also be the result of a spontaneous mutation.

0:08:26.3 RW: Like with sickle cell, these anomalies change the shape of the affected red blood cells and shorten their lifespan. But these are two quite different disorders. Where sickle cell disease affects the hemoglobin molecules inside red blood cells, hereditary spherocytosis affects proteins in the red blood cell cell membranes. The cell membranes adopt a spherical appearance. Think of having millions of tiny ball bearings in your bloodstream. But that's not all. The spherical red blood cells turn out to have less surface area than normal ones and they are rigid rather than elastic and squishy. They rupture rather than squeeze and rebound. Hereditary spherocytosis affects a different portion of the population too. It is found most often in people of northern European descent. In fact, according to at least one source, hereditary spherocytosis is the most common kind of anemia among this ethnic group occurring in one out of every 2,000 live births.

0:09:34.0 RW: So here's the situation, where some percentage of red blood cells, and I was unable to find out what that percentage might actually look like, but some percentage of red blood cells are not normal. And instead of being part of our oxygen delivery system for about four months, these cells last for about 10 to 30 days. Let's track that through and see where it leads us. Firstly, because I know, or at least I hope I know, that this is how massage therapists' brains work, let's talk about blood clots. Blood clots are a big issue in sickle cell disease with those sharp, pointy, distorted cells. But this turns out not to be the case in spherocytosis. So as a number one caution, we can take issues around blood clotting and embolism and thrombosis off our list of concerns. Phew! When these non-functioning red blood cells report to the spleen, they are removed from circulation and destroyed. This is called hemolysis, destruction of blood. But when this happens on a large scale, some bad things can develop, starting with hemolytic anemia. That is, lack of red blood cells because they are being destroyed faster than they are replaced.

0:10:53.7 RW: Now think about that poor spleen working in hyperdrive every minute, all the time, to keep bad red blood cells out of our circulation. The spleen is extracting a boatload of bilirubin, which then travels to the liver to be made into bile. More bilirubin than we need. Lots more. And then more on top of that, and eventually all that bilirubin can do a couple of things. The first one is that it can back up into the bloodstream and eventually be deposited into the skin and the mucous membranes and the sclera of the eyes. Do you remember what color bilirubin is? Golden brown. When we have way too much, we turn yellow. And this is jaundice. That excess bilirubin can also accumulate in the gallbladder, where it forms a specific kind of gallstone. About half of all people with moderate to severe hereditary cirrhositosis end up having a gallstone at some point. Jaundice is usually a sign that the liver's having a problem and things are getting backed up and we see it with hepatitis and cirrhosis and some other liver problems. But in this situation, it's just a reflection of having loads and loads and loads of bilirubin.

0:12:09.4 RW: In the meantime, there's the spleen sucking up more bad red blood cells and more and more and spleens get mad when things like this happen and they get big. This is called splenomegaly. And the big problem with splenomegaly is it makes the spleen more vulnerable to injury and it's located just under the left side of the rib cage. With the right kind of trauma, an enlarged spleen can easily rupture, and that means life-threatening internal bleeding. So what I've just described are what might be called the clinical sequelae of hereditary spherocytosis, hemolytic anemia, jaundice, and splenomegaly. And this is, of course, where the symptoms develop. The main signs of hemolytic anemia are pallor or grayish skin, shortness of breath, low blood pressure because of low blood volume, and tachycardia as the heart tries hard to push oxygen-poor blood to all of our suffocating cells. Jaundice, we know, yellowing of the skin and mucus membranes in sclerae, plus of course that risk of gallstones.

0:13:17.5 RW: And spinal megaly may not have big symptoms. Hereditary spherocytosis can occur in a range of severity from barely noticeable to extremely severe. When it is severe in young children, it can result in delayed development and short stature. It can cause complications in pregnancy when that anemia can get very severe. And there's a phenomenon called a hemolytic crisis. This happens when some stressor or aggressive infection can trigger a sudden onset of excess spleen activity with anemia and jaundice. So how is this treated? Well, just like with sickle cell disease, this is a genetic disease. It can only be treated symptomatically. For people with really severe hereditary spherocytosis, they may need a splenectomy. The spleen is removed and then the liver takes up the tasks of homolysis and bilirubin recycling. I looked for any information on whether this is just too much to ask of the liver. And the consensus seems to be that now the liver can keep up just fine.

0:14:25.8 RW: However, remember that the spleen, in addition to being our blood filter, is also our largest and busiest lymph node. And someone who doesn't have a spleen is now considered to be immune compromised and vulnerable to infections for the rest of their life. People with hereditary spherocytosis may be counseled to supplement folate, that's a key nutrient for the production of red blood cells, and if things get very, very severe, they may need a blood transfusion. Happily, those super severe cases of hereditary spherocytosis are pretty rare, and most people with this disorder have a completely normal lifespan. So let's return to our client. Remember he's 40. He has a physically demanding job. He wants relaxation and pain relief from his very first massage. He takes no medication and while he reports having had jaundice, there's no indication that he's at risk for spleen problems. However, that should be a follow-up question from his massage therapists.

0:15:27.5 RW: Enlarged spleens are a caution for massage, not because I think we will rupture them, but because splenomegaly is a sign that something hard is going on with blood in circulation, and let's not challenge a system that is already really challenged. So, my advice to our contributor in this situation is to proceed with joy. Maybe lay back on pressure until we know more about how the client takes it in, but given his overall picture of health and resilience, he seems a great candidate for the kind of massage session that he hopes to get. This might not be true for every person who has hereditary spherocytosis. So as always, make sure to get a thorough health history. And in this case, with special attention to spleen function, immune system health, and anemia. Spherocytosis, who knew? 

0:16:27.6 RW: Hi friends, Ruth Werner here to talk about my current absolute favorite online self-paced continuing education class and bonus. It is NCB TMB approved for ethics credit too. It's called A Doctor's Note is Not Good Enough... And what is better. And in this fun for all we go through why we think doctors notes matter. Spoiler alert they don't do what you think they might do. And we talk about how to set up lines of communication that will get you what you really need. Information to help you make good clinical decisions. Please join me for A Doctor's Note is Not Good Enough... And what is better. You can do it anytime that works for you and anywhere you have Wi-Fi. Visit me at ruthwerner.com to register today.