Interview: Babies Die of Sickle Cell Yearly.

- Dr. Denis Nsame, Dir. L’be Reg. Hospital

Cameroon on 19 June 2018 commemorated world sickle cell day under the theme "sickle cell and blood donation, a gift for life.” Sickle cell is a chronic inherited disease affecting mostly Africans. Reason why the government of Cameroon is taking all necessary measures to step up its fight and prevention. In the following interview The Median’s Ticha Melanis sought to know from the Director of the Limbe Regional Hospital, Dr. Denis Nsame, the cause, manifestation, incidence and management of the disease in Cameroon.

Dr. Denis Nsame

*What is sickle cell anaemia and how is it contracted?

      Sickle cell is anaemia is a chronic disease and hereditary disease. Hereditary meaning you cannot have it if not from birth. Sickle cell is the malfunction of the hemoglobin (red blood cells). This is because the red blood cells assume a crescent shape instead of the normal disc-shaped. When this happens, the red cells cannot carry oxygen adequately to nearby tissues. This is because with its abnormal crescent shape the diseased red blood cells cannot flow through smaller blood vessels.

*How does one know they have sickle cell anaemia?

      You cannot read on the face that someone has sickle cell. It is a genetic disease transmitted from parents to their children. To be sickle cell patient, it means you have the gen of SS which indicate the parents were carriers either AS and AS or AS and SS. Those suffering from the disease suffer a lot of pains and are generally anaemic because their red blood cell are always destroyed and they are prone to infections since their blood stagnates and allows for any germs to grow causing headache, passage of blood in urine, shortness of breath amongst others.

*How is sickle cell diagnosed?

     Diagnosis is through a blood test. It is done through electrophoresis of the hemoglobin which will indicate if you are AA, AS or SS. If you are AA it means you are normal, AS indicates sickle cell trait while SS indicates you have sickle cell anemia. The result of the test usually takes up to 14 days to come out.

*How can sickle cell be prevented?

        It is advisable for people wishing to get married to carry out a blood test so as to know their status. If a couple is AS/AS there is a 25% chance of them having a sickle cell child. But it is up to them to decide. Doctors are just there to advice and inform them on the risks involved. It is advisable for an AS person to partner with someone who is AA.

*Can the disease be cured?

There is no cure per say but it can be managed. There is bone marrow transplant for children with severe case of the disease but in Cameroon, it is much more refined medically and there is still a lot of research going on. The bone marrow transplant alleviates your condition and reduces the risk of the patient having a crisis. But it won't change your genetic status. If sickle cell patients don't have treatment they may die before the age of 5 because of the complications that come with the disease. If they have good medical follow up, they can bypass this age and live for more than 20 years with less crisis. The crises are more in the younger age.

*what is the incidence of sickle cell in the world?

Globally, 1000 babies die of sickle cell disease each year in Africa and Asia. And most of them die before their 5th year. One out of every 30 new born has sickle cell in Central Africa. At the Limbe Regional Hospital between 20-50 cases of sickle cell patients are presently being followed up.

*Why the choice of this year's theme for the Sickle Cell Day?

       It was chosen because the most frequent problem of sickle cell patients is reccurrent anaemic and pains due to infections. So these patients need blood to be donated to them when in crisis. And when you donate your blood you save a life. Without blood, we cannot live because blood carries oxygen to the organs. We recently lost a girl of 18 years from sickle cell complications. So I beg on the public to come and donate blood. Those eligible to donate are people from 18years above and who don't have allergies.

*Apart from anaemia what are some of the complications that come with sickle cell?

You can have liver and kidney problems because of your kidney will not be filtering blood very well but filtering bad blood into your system. The patients might have frequent infections because their blood stagnant. And when blood stagnant in the bone, the develop osteomalasia that is having pus inside the bone which will need surgery. Some patients may develop a stroke or paralysis. Since these patients are generally anaemic, if a disease just as malaria sets in, their situation becomes worse.  

*How can a patient with sickle cell keep fit?

     By being clean that is proper hygiene on yourself and environment to avoid getting infections, do regularly checkups in case of any slight problem, take medical advice and vaccination especially vaccines like meningococcal to prevent them from frequent infections.

*So what are some of the things RHL do to fight against sickle cell?

   We work with the department of social affairs & NGOs to support these families. We also have a social service in the hospital that registers these patients to make sure they follow up their condition. We equally exonerate patients from many medical bills, encourage them to join social groups to share experiences on how to cope with the pains, educate parents on the fact that the disease is inherited and not caused by witchcraft. We also have blood donors in the communities that we call at midnight in case of an emergency.

*what are some of the challenges RHL face in the fight against sickle cell?

There is the problem of managing the disease in some patients, availability of blood especially rare groups like 0-. Some people who refuse to open up about their status for fear of stigmatization and more.

*so what will RHL do to step up the fight against sickle cell?

     We will continue to inform the public of the importance of blood donation and the necessity to know their status. I will take this opportunity to thank the students of the faculty of health sciences in UB for their blood donation on world blood donor day and to our community donors. These people I must say know the importance of blood donation.

Pic

Dr. Denis Nsame