When the body makes too many red blood cells

Kelly* sat back in her chair holding her little electric fan in frustration. The January heat was killing her. It had been an extremely hot start of the year but for Kelly, she felt like the sun had been sent down to specifically torture her.

Kelly had just recently started seeing me. Despite Kenya being home, she had hardly lived here for the past 20 years. Her work assignments took her all over the globe, but she was now craving some sort of roots. She wanted to sit on her front porch and work from her laptop, not packing a bag every few years and starting all over again.

Kelly had done amazing work for her organisation for the last 30 years while single-handedly raising her children. She lost her husband to a grisly road accident when she was barely 25, with her twin babies losing their dad when they were barely out of diapers.

The grief was overwhelming. She had never imagined raising her babies without her childhood sweetheart Sam*. The two had dated since high school, hence it was no surprise that after college, they got married right away, with their babies coming a year after.

Moving away to Rwanda was what Kelly needed at the time. It was a fresh start for her and her babies, to heal and recollect herself. Her company was just setting up a new office and she jumped at it. She was not too far from home and her mother was happy to come with her and babysit. This marked the beginning of her globe-trotting. Her babies spoke six languages, courtesy of them being global citizens.

Kelly was now heading to 50. She had had enough. It was time to come home. Her babies were now young adults who did not need her. They were charting their own path in different parts of the globe and she wanted to reconnect with the part of her she had been away from for too long. Her mum, her partner in crime in all these travels, had also been laid to rest.

When Kelly first came to see me, she had undergone a hysterectomy five years prior. She had suffered fibroids that caused her to bleed too much. She opted to remove the offending uterus altogether since she had no plans of having any more babies.

With the bleeding caused by fibroids, Kelly had lived right on the edge with constant anaemia from the excessive monthly blood loss. Post-surgery, with the bleeding having stopped, her haemoglobin levels had risen to a normal of 12g/dl to 13g/dl. She had never felt better. She was able to focus on her fitness and even started hiking.

She had enjoyed a blissful three years before she started wondering whether menopause was coming early. What bothered her the most was the heat. Kelly has always enjoyed her hot showers. The heat helped her unwind her sore muscles. It was her therapy. Now she could not tolerate her hot showers anymore. She would get horrible itching afterwards, turning all red and hot.

She had also developed intolerance to her coffee and wine. She has always thrived on coffee, it was her ‘pick-me-up’ morning drink. Now all she got after her cup was extreme discomfort in her tummy, worsened by wine and acidic fruit. She had to give up the chilli as it would cause her stomach to burn. She knew her ulcers were acting up.

Going through the rest of her reports, her hormonal checks were fine, menopause wasn’t what was causing her to feel so uncomfortable. The thyroid gland was working just fine and her blood sugar levels and cholesterols were all good. The only noteworthy report was her complete blood panel.

The haemoglobin level was certainly not normal. It was at 17.8g/dl! Sitting there holding her little electric fan, looking excessively flushed, I had a light-bulb moment. Kelly was suffering from polycythemia vera! It explained why her liver felt slightly enlarged during her physical exam a month ago, as did her spleen. It even explained her recent increase in blood pressure.

I explained my suspicions to Kelly and this not being my area of specialisation, I sent her off to the hematologist. Kelly underwent a series of tests, including biopsy of the bone marrow, and her diagnosis was confirmed.

Polycythemia vera is a condition that causes excessive production of red blood cells by the bone marrow. The red blood cell production can also occur at other sites, not usually expected to make the red cells in adults, such as the liver and spleen, explaining why they get enlarged.

While having a high haemoglobin level is a good thing, improving oxygen delivery to your body and keeping the bodily functions running at optimum level, there is such a thing as too much of it. The red cells become too many, hampering blood flow around the body. The slowed movement increases the risk of forming blood clots in the blood vessels, a life-threatening condition.

The condition also leads to an overproduction of histamine, a chemical compound released by the body, causing the itching that was bothering Kelly and also causing development or worsening of stomach ulcers. It is more common in men than women, especially in persons over the age of 50. Five per cent transform into leukaemia.

Kelly had to contend with weekly removal of some of her blood to keep down the number of red blood cells and dilute the blood by giving her intravenous fluids. This improved blood circulation around the body, reducing her risk of clots that could potentially cause her complications such as stroke, heart attacks and embolism in the lungs. Kelly has to take a daily dose of hydroxyurea to suppress the excessive production of the red cells. She also takes daily aspirin to protect her from blood clots. She will remain on constant long-term follow-up to look out for any possibility of leukaemia. She feels much better, has resumed her physical activity and is now able to enjoy the hot showers. I haven’t even seen the little electric fan anymore!

Dr Bosire is an obstetrician/ gynaecologist