Living with aplastic anaemia, rare blood condition
Sandra Wekesa @wekesa_sandra
In 2011, as a 16-year-old, Aminah Paul was always vibrant in school and happened to be the best in athletics and music.
He was basically doing all the normal activities of a teenager. However, every time he participated in such vigorous activities, he would experience sharp pains in his chest, which led to shortness of breath.
After a year, he started to become extremely fatigued, and in no time, he had to dismiss any activities in which he had to exert himself just to see if the exhaustion would go away.
But after months of laying low, the symptoms intensified and others appeared.
One day, he became dizzy and fainted, he was rushed to a local German hospital in Mathare and after several blood tests, results showed a low blood count and ruled out possibility of sickle cell anaemia.
They needed to transfuse Paul, but in the process of looking for blood, he had to be put on oxygen.
Tesrs and biopsies
“When I got to Kenyatta National Hospital (KNH), they opened my file, but later terminated it because they didn’t have enough blood supply in the bank. I was later put on blood supplements.
After two days I travelled to Kisumu, but on my way, I became so weak that I had to be taken to Marie Stopes Kisumu, where I was admitted for two months.
The condition started affecting my sight and I was referred back to KNH for a detailed examination,” he explains.
All along he was filled with many questions and was completely clueless on how to address the situation and how to feel about this new life of walking in and out of hospitals.
With his eyesight already affected, he hoped the blurry images were part of a dream he could soon wake up from once he got to KNH.
“They told me to book for a bone marrow aspirate tests, which turned out negative so the doctors ruled out aplastic anaemia and concluded that I had anaemia.
I then continued with the iron supplements and a good diet to boost my blood,” he recalls.
Bone marrow aspirate is a procedure that involves taking samples of the liquid part of the soft tissue inside the bone to determine whether a patient has any condition including anaemia, other blood cell diseases, bone marrow diseases and other conditions.
For the next five years, Paul’s health improved until August 2018 when the same symptoms recurred.
He underwent blood work at Mama Lucy Hospital and was put back on iron supplements.
“In 2019, I got ill again and went for a blood work at Mercy Sisters in Makadara and the result showed I had a haemoglobin concentration (hb) of 1.6 and sickle cell negative, this was threatening so I had to get an urgent blood transfusion.
I was rushed to Kijabe Mission Hospital for a blood transfusion and a bone marrow aspirate; unfortunate they never had a bone marrow specialist.
I received only a unit of blood and was released in the morning with a hb of four ,” he explains.
A week later, he was back at KNH for readmission. His symptoms had flared up again.
He spent several hours unattended, and was forced to move to Jaama Hospital where he got a unit of blood as an outpatient. He was admitted the following day and stayed for three weeks.
His health began to deteriorate and that is when he went to MP Shah Hospital for a bone marrow aspirate.
The report didn’t give a clear conclusion, so he was advised to undergo a trephine or bone marrow biopsy, a process where cells are checked for diseases.
He was diagnosed with aplastic anaemia.
According to Dr Sarah Bosire, a doctor at Kenyatta National Hospital, Department of Oncology, aplastic anaemia is a condition that occurs when your body stops producing enough new blood cells, as a result of damaged bone marrow.
“With this condition, the stem cells are damaged and as a result, the bone marrow is either empty (aplastic) or contains few blood cells (hypoplastic),” she explains.
According to studies, aplastic anaemia is a rare life-threatening disease. The incidence and median age at diagnosis vary according to geography and ranges from 1.5 to about seven cases per million from age 25-60 years respectively.
But since the introduction of immunosuppressive therapy and allergenic stem transplantation, which Paul is set to undergo, the outcome has improved considerably and the five-year survival is reported to be 70-80 per cent in selected patients.
“Aplastic anaemia is not as rare as it has always appeared. In many cases, there is a misdiagnosis of the condition, which then makes many people suffer in silence.
The belief that the condition is a result of witchcraft causes patients to resort to treatments when it’s too late,” Dr Bosire says.
Although it is often likened to sickle cell anaemia and leukemia because of recurrent transfusions, patients of aplastic anaemia have lower counts of the three blood cells; red, white, and platelets.
This means that the bone marrow does not produce enough blood cells to replenish dying ones.
“In most cases, the patient has immature blood cells, which make them prone to infections, spontaneous bleeds, and low haemoglobin count,” adds Dr Bosire.
While anyone can get aplastic anaemia, it is more likely to happen to teens and the elderly.
Acquired aplastic anaemia is the most common, and accounts for 50 per cent of cases with unknown causes.
However, researchers believe it can be triggered in the immune system.
Such triggers include; viruses such as HIV, certain medications, toxic chemicals like insecticides, organic solvents, paint removers, and herbicides. Also, radiotherapy or chemotherapy treatments play a major role.
Symptoms depend on the blood cell count, which in some patients could be of all the three blood components.
“Patients could get shortness of breathe, dizziness, pale skin, headaches, infections, high fever, easy bruising and bleeding, bleeding of gums, chest pains, and irregular heartbeat,” Dr Bosire says.
She adds that diagnosis is the most important part of knowing whether you have the condition as symptoms may not always present themselves. Usually, the process involves a blood test and bone marrow biopsy.
In the blood test, a sample of blood is drawn out to check on cell count, and whether the red and white blood cells are in a normal range.
If they are seen to be low, then the bone marrow biopsy will be conducted.
“In the bone marrow biopsy, a sample of the marrow will be removed and examined under the microscope to rule out other blood-related diseases.
In case tests reveal few blood cells then they confirm diagnosis of aplastic anemia,” says Dr Bosire.
The treatment will be done according to the severity of the condition. One of these is blood transfusion, which will be able to provide some blood cells the bone marrow isn’t able to produce.
“With aplastic anaemia, you will find a patient walking with very low hb, as low as two yet a normal person should have 14.
In this case, they will require a blood transfusion regularly to substitute the low blood count,” says Dr Bosire.
While there is no limit to the number of transfusions one can get, there can be a downside of multiple transfusions, which is accumulative iron in the body that has to be treated to avoid further complications.
The body can also develop antibodies to transfused blood cells, making them less effective at relieving symptoms.
The use of immunosuppressant medication makes this complication less likely.
Another treatment option is stem cell transplant. This is usually successful as it rebuilds the damaged one, hence stimulating a normal supply of blood cells. However, it is only possible if there is a donor.
After the procedure, a patient will be put under observation and on drugs to prevent rejection by the bone marrow.
If a bone marrow transplant can’t be done due to the extreme damage, patient can get on immunosuppressive therapy (IST) with anti-thymocyte globulin (ATG), a drug that helps improve blood cells count.
Unfortunately, not so many people can afford the treatment, as most processes have to be done abroad.
“Oncological issues faced in Kenya such as lack of advanced techniques for testing and surgical problems, especially when it comes to complex issues such as bone marrow transplant, makes it impossible for people to be treated locally.
That not so many families are capable of taking their kin to another country for treatments makes it hard for the disease to be treated,” Dr Bosire explains.
Another challenge patients face is psychological torture of having to think about their life expectancy and the continuous questions from people who don’t understand their need for regular blood transfusion.
They also have had to grapple with demystifying the condition’s link to witchcraft as some people believe.
For Paul, the only solution for medical condition is a bone marrow transplant. Unfortunately, it is expensive for him, just like the alternative-medication that costs Sh18,000.
The 28-year-old is optimistic that he will be able to get Sh4.5 million to undergo surgery and restore his lifestyle.
He’s turning to wellwishers, who can donate through Paybill 891300, account AMINAHPAUL.