- Doctors list measures to alleviate condition
BY KATE HALIM
When Chidinma Ubani was born 25 years ago, her parents were overjoyed. They welcomed her arrival like a princess. She was the centre of their world and the rainbow that coloured their lives. But as she grew older, she became a sickly child.
Her mother frequented the hospital because of her and her father’s purse was being drained of money. Her hands and feet swelled whenever she suffered from this ailment that was alien to her parents then. They first attributed it to spiritual attack until their doctor told them to conduct a genotype test for her.
The result of the test was heartbreaking. The apple of their eyes has an SS genotype! She is stuck with sickle cell disorder for life.
Chidinma’s mother couldn’t hold back the tears and her father questioned God for giving them such a difficult child. Since then, this once happy family has been threatened by their daughter’s condition.
Over the years, the poor girl herself has been a regular visitor at hospitals. She goes for check-up every month. For Chidinma, life has been filled with pains, but she is determined to live it to the full.
“I have been in and out of hospitals because of sickle cell crises. I have felt unimaginable pains. I don’t do things like others. I have felt like dying sometimes, but when I look at how supportive my mum has been, I decide to live life to the full,” she said. For Shirley Hills, her life of over 30 years has been filled with pains and drugs. She is still recovering from crises that glued her to the hospital bed for over 12 days. Shirley revealed that the pains of having her son was nothing compared to what she felt for days.
Said Shirley: “I know what I go through during crisis. I lost my sister to the disorder and my family hasn’t remained the same after that. I am determined to do everything within my power to live and eat right, so that I can minimize the number of times I suffer from crisis. I no longer drink cold water. I take warm water only.”
Sickle Cell Disease at a glance
Sickle Cell Disease (SCD) is a major genetic blood disorder that affects the haemoglobin within the red blood cells. The recurrent pain and complications caused by the disease can interfere with many aspects of the patient’s life, including education, employment and psychosocial development. The family is not spared the ordeal.
As blood circulates to all parts the body, “sickling” can occur anywhere resulting in a range of complications. Symptoms usually start after the age of four to six months and can include pain. Because of the sickle cell shaped blood cells, they sometimes get stuck in the small veins and prevent normal blood flow. These blockages cause pain in the arms, legs, back and stomach. Sometimes this pain is quite severe.
Sickle cell disorder can also cause swelling of the hands and feet, with stiff painful joints and extreme tiredness. Episodes of severe pain are known as “crises”.
Sickle pain can happen at home and at school – in fact anywhere – so it is important for families and sufferers, as well as care givers and teachers, to know the best ways of coping.
Crises and sickle cell disease patients
According to Dr. Gabriel Omonaiye, people suffering from SCD are mildly affected and largely free from pain, while others have frequent and severe pains. He notes that the pains they suffer during crises differ from one individual to the other. Omonaiye reveales that there are different kinds of crises which include Vaso-occulusive, Aplastic crises, sequestration crises.
He said: “Vaso-occulusive crises presents as painful episodes of ischaemic necrosis (tissue death from lack of blood supply) commonly involving bones, liver, brain, penis and spleen. The lungs can be involved too and this is a potentially dangerous crisis known as acute lung syndrome due to pulmonary (lung) inflammation that impedes lung blood flow. A viscous cycle of sickling and occlusion hinders lung function, worsening pulmonary and systemic hypoxia.”
Dr. Alex Nwadibia reveals that crises triggers infections, thirst and dehydration caused by not drinking enough, even if thirst is not felt, over-exertion, over-excitement, cold weather and cold drinks and swimming have all been reported to cause pain.
“Bangs, bumps, bruises and strains have also trigger crises. Stress triggers pain in adults, but does not seem to do so in children. Children and families can often tell when a severe sickle pain is coming on by thirst, or the eyes turning yellow (jaundice), or by the sufferer being more irritable or tired than usual. These features may not be present, but one or more may predict crisis.”
Dr. Omonaiye adds that most commonly, children and adolescents report sickle pain in hands, arms, legs and lower back, with pain in the joints being less usual. Headaches and pains in the chest and abdomen aches characterize crises in sickle cell patients.
He said that: “Boys may get priapism, which is a painful, stiff penis, often noticed as pain in the groin. They may be too embarrassed to mention this, but it is important to get medical attention, because severe sickling in the penis can lead to impotence.”
Treatment and possible cure
According to Dr, Gabriel Omonaiye, bone marrow transplant, also known as stem cell transplant, offers the only potential cure for sickle cell anaemia disorder. The drawback, however, is the exorbitant cost. As a result, treatment for sickle cell anaemia is usually aimed at avoiding crises, relieving symptoms and preventing complications. Treatments might include medications to reduce pain and prevent complications, and blood transfusions, as well as a bone marrow transplant.
Omonaiye says: “Bone marrow transplant is usually reserved for people younger than age 16, because the risks increase for people older than 16. Finding a donor is difficult, and the procedure has serious risks associated with it, including death.”
Living with Sickle Cell Disease
Shirley Hills who is nursing a nine- month baby says living with Sickle Cell Disorder is so painful and expensive. She spends N15, 000 for medical reviews every month. Her weight is checked as well as her red and white blood cells count. “The financial implication of taking care of my health as an SCD person is weighing me down. I used to buy Hydroxyurea, one of the drugs I use to manage my condition for N6, 500, but now, it’s N11, 000. It’s a pack of 100 tablets that will last for a month, because I take it three times a day.”
Treatment and management of SCD
Dr. Nwadibia says people with the disorder especially need folic acid to prevent them becoming run down. They frequently have a mild jaundice (yellowing in the whites of the eyes). This is not a cause for concern unless it becomes noticeably worse.
Dr. Omonaiye says that the most important components of treatment are early intervention with analgesics, antibiotics, rest, good nutrition, folic acid supplementation and high fluid intake.
“At times, invasive procedures such as blood transfusions and surgery may be needed. Psychological support is an important tool in caring for them as they have a lot of emotional issues to contend with and questions that plague their minds,” he says.
Medications used to treat sickle cell anaemia include Antibiotics such as penicillin. Antibiotics prevent infections, such as pneumonia, which can be life-threatening to someone with sickle cell anaemia.
Pain relieving medications also come in handy in treating patients with SCD. When taken daily, hydroxyurea reduces the frequency of painful crises and might reduce the need for blood transfusions and hospitalizations.
Hydroxyurea seems to work by stimulating production of fetal hemoglobin — a type of hemoglobin found in newborns that helps prevent the formation of sickle cells.
Hydroxyurea increases your risk of infections, and there is some concern that long-term use of this drug might cause problems later in life for people who take it for many years. There is a limit to how much pain relievers a SCD patient should take, because their hearts pump blood faster than any other genotype.
Government’s intervention needed
According to Dr. Omonaiye, government can help SCD patients by subsidizing or bearing the costs of laboratory investigations, drugs and other medical consumables used in treating them as some of these are costly and are beyond the reach of many of the sufferers living both in the urban and rural areas. The need for government support is further underlined by the fact that the sickness is a chronic one.