Lukiah L. Nakabembe Mulumba, RN, BSN, Chair, Uganda-American Sickle Cell Rescue Fund, USA
Dear Colin (listserv moderator),
I would like to Thank you and the Commonwealth Health and Media Partnership, for connecting me to the right people. My efforts to assist those suffering the affects of Sickle Cell Disease all started with our initial contact via your website almost three years ago, now.
I was able to visit Uganda earlier on this year, I met with Pius, the journalist you connected me with. I also met with Ruth, Uganda's Sickle Cell Association's Chairperson. It was a positive week here in the fight against sickle cell Anaemia in Uganda. Please fell free to read the articles below and see some of the activities that happened.
Peace, Lukiah.
http://www.connectuganda.com/content/view/237/204/ http://allafrica.com/stories/200801210747.html
http://www.monitor.co.ug/artman/publish/coffee-break/Daring_to_dream_different.shtml http://allafrica.com/stories/200801210747.html http://www.monitor.co.ug/artman/publish/health-and-living/Sickle_cell_patients_still_in_need_of_help.shtml http://www.zibb.com/article/2533608/The+Trauma+of+a+Sickle+Cell+Baby+Pushed+Her+to+Help+Others http://www.newvision.co.ug/D/8/19/570294
Showing posts with label Sickle Cell. Show all posts
Showing posts with label Sickle Cell. Show all posts
February 13, 2008
October 05, 2006
Sickle Cell Disease Association 34th Convention
by Lukiah and Abudallah Mulumba, founders, uganda-american sickle cell rescue fund, USA
The 34th Annual Convention of sickle cell organized by the Sickle Cell Disease Association of America ended on Saturday September, 31st 2006 on a promising note. The convention which started on the 27th of September drew thousands of member organizations from across the United States, leading researchers in the sickle cell disease and physicians plus professors from across the world. Many stake holders from all over the world such as India, Nigeria, Zimbabwe, and Mali also attended. The convention was held at Hyatt Regency Dallas Reunion, Texas.
Iron overload
Some of the most promising presentation was presented by Dr. Whitley on iron overload in sickle patients. One of the most underlying complications of the disease is the development of stroke due to vasoconstriction of critical blood vessels that supply the brain with oxygen and nutrients. Studies done concluded that monthly transfusion of these patients indefinitely will reduce these strokes. On the other hand, however, there is evidence that patients who are transfused frequently do end up with iron overload since the amount of iron taken in is excreted by the body in the same amount. This iron overload causes other major complications including congestive heart failure, liver problems, and kidney problems.
Treatment of iron overload
Until recently the only available medications to help in the excretion of this excess iron otherwise known as cheleters was a cumbersome effort which could only be delivered subcutaneously through a pump because the half life of the medicine was only 20 minutes and so there was a need for continuous delivery of the medication. On a good note, oral cheletors by the names of exjade and deferoxamine have been developed. Such new techniques of treating sickle cell complications are very important to be used in developing countries with higher incidence of sickle cell disease such as Uganda. For example, using a Transducer Cranial Doppler (TCD) in many sickle cell children enables physicians to identify patients who are at risk of developing strokes in time for interventions. Jell is applied around the child’s facial area above the eye brows and the screen would be taking pictures and numbers of which the neurologist physician bases the finding.
Another important aspect of this convention was the call by many presenters that it is time to change the management of sickle cell disease by looking at the transition of sickle cell disease children to adulthood. Better management techniques have greatly reduced the level of mortality in children with sickle cell disease. Yet no efforts have been made to direct a smooth transition to adulthood and yet sickle cell disease management in adulthood continue to be neglected. There was therefore a call to come out with strategies of managing the disease in adults. In case of the United States, many adult patients find themselves unable to secure medical insurance since they lose most of the available free medical care offered by most of the states by the age of 21 years. Frequent crises also means frequent visits to hospitals with hostile care providers who look at the patients as drug seekers!! Management in childhood is always a teamwork comprised of hematologists, registered nurses, nurse practitioners, social workers, psychologists, and lab technicians. This team gets used in giving primary care to the patient until adulthood (teen age, which is 18 years in the United States). Unfortunately there is no such approach in sickle cell disease management once patients reach their adulthood. Patients find themselves being seen by multiple physicians some of whom know little or nothing on sickle cell disease. The bond and trust that had been built (for 18 years) during the early years between the therapy team and the patient is therefore broken during adulthood. Calls for smooth transition are just timely.
We do know that this kind of problem does exist in Uganda today and that there exist minimal number of hematologists specifically trained to deal with sickle cell disease adult patients. I hope that Uganda do learn from the new experience of treatment strategies for adult sickle cell patients.
Genetics
During the conference there was significant contributions made by doctor and Professor Betty S. Pace of the Department of Molecular and Cell Biology and Director of sickle cell disease research center at the University of Texas in Dallas. Professor Pace shed some light at how far researchers for sickle cell disease are in finding a cure basing on the information available from the Genome Project. The Gnome Project was completed in 2003 and it is basically a blue print of human body having been able to identify all the necessary genetic information regarding humans. Researchers in sickle cell disease just like in other incurable diseases continue to have high hopes in manipulating DNA of patients to cure diseases. It was quite refreshing to note that the National Institute of Health (NIH), which is the leading research umbrella in the health sector in the United States, is collaborating with other countries including developing countries in the search for cures of different diseases including sickle cell disease. Uganda can explore such opportunities that exist with sickle cell disease. We mentioned to Doctor Pace about our plans of starting such educational sickle cell disease conventions in Uganda, she immediately volunteered sponsor her trip (ticket, lodging etc) with others to join us in Uganda.
DNA
Another current on going research was presented by Doctor Mathew Porteus, PhD. His lab is currently trying to fix the mutation that causes sickle cell disease. The field of gene therapy developed as a way to treat genetic diseases, such as sickle cell disease, by changing the DNA of the cell for therapeutic benefit. Doctor Porteus said that, there are a number of different approaches to gene therapy, but his lab has focused on trying to “fix’ the mutation that causes sickle cell disease. His goal is to remove the blood stem cells that contain the sickle cell mutation in both copies of the b-globin gene from a patient, fix one of both of the genes so that they no longer have the sickle mutation, and then return those cells back to the same patient. He hopes that by “repairing” the sickle cell mutation in enough of the patient’s blood stream cells that he and his team can cure the disease. He was very impressed so far and discussed his progress towards this long-term goal. It may take 5 to 10 years. We are keeping our fingers crossed!!!!!!!
Nigerian experience
We were so thrilled about the support from our sister countries Nigeria and Ghana in relation to the struggle of sickle cell disease in Africa. Doctor Ramesh C. Pandey of Xechem, Inc, New Brunswick, New Jersey, presented about the new anti-sickling natural herbal drug Nicosan / Hemoxintm in Nigeria. Various basic and clinical studies of Nicosan formerly known as Niprisan have been performed over the past 10 years in Nigeria at the National Institute for Pharmaceutical Research and Development (NIPRD) and for over five years at several laboratories in the United States. Dr. Pandey was hired directly from the United States by the Nigerian government to take part in this research. He has been involved in research of this drug for a period of over five years. Nicosan is an extract of four plants which has been used by traditional health providers in Nigeria. Phase I/II clinical trials showed a decrease in the frequency of painful episodes and an increase in school attendance of people with sickle cell disease. Laboratory work carried out at the NIH NHLBI Sickle Cell Disease Reference Laboratory at The children’s Hospital of Philadelphia in United States (SCDRL-CHOP) showed that NICOSAN has strong anti-sickling effect. Their studies in vivo using transgenic sickle mice that express human sickle cell hemoglobin showed that NICOSAN not only decreased the formation of sickled cells in blood, but also prolonged the survival period of mice that had been exposed to severe hypoxia (5% oxygen / 95% nitrogen). Other studies performed at Xechem Laboratories in New Brunswick, New Jersey in United States and Xechem Nigeria in conjunction with SCDRL-CHOP have shown that NICOSAN contains various aromatic aldehydes that combine with sickle hemoglobin and inhibit sickling erythrocytes at very low concentrations. Further studies showed that NICOSAN contains chemicals that inhibit cell sickling by hydrating sickle erythrocytes. According to Doctor Pandey, NICOSAN is a new effective anti-sickling natural herbal drug available in Nigeria. The studies conducted in Nigeria, will be repeated in the United States so that it can be available over the counter by patients of sickle cell disease. We are very happy for Nigerians. Some patients/parents etc have been contacting loved ones in Nigeria to send them NICOSAN to United States.
Hydroxyurea
Currently hydroxyurea continues to have more success in treating painful events. Clinical trials conducted in 1995 showed a 44% decrease in medical contact for treatment of painful crisis. Whereas, hydroxyurea was thought to work better due to toxicity concerns, the HUG_KIDS trial demonstrated that children from five years and older could also be given the same dose basing on body weight and experience the same benefits. During the convention proceeding, there were testimonies from patients using hydroxyurea on its efficacy. While speaking about sickle cell disease in Uganda, we were told that hydroxyurea is not available at the Ugandan market and that they only read about it despite the fact that it has been on the market in the United States for over ten years now!
Bone marrow
Bone marrow transplant is still the only documented cure for sickle cell disease. Its limited use can be explained from the many problems associated with it such as finding a matching donor, surviving chemotherapy, and finding enough stems cells for therapy. The later is made easier for patients who are lucky to find a matching donor from cord blood stem cells.
The 34th Annual Convention of sickle cell organized by the Sickle Cell Disease Association of America ended on Saturday September, 31st 2006 on a promising note. The convention which started on the 27th of September drew thousands of member organizations from across the United States, leading researchers in the sickle cell disease and physicians plus professors from across the world. Many stake holders from all over the world such as India, Nigeria, Zimbabwe, and Mali also attended. The convention was held at Hyatt Regency Dallas Reunion, Texas.
Iron overload
Some of the most promising presentation was presented by Dr. Whitley on iron overload in sickle patients. One of the most underlying complications of the disease is the development of stroke due to vasoconstriction of critical blood vessels that supply the brain with oxygen and nutrients. Studies done concluded that monthly transfusion of these patients indefinitely will reduce these strokes. On the other hand, however, there is evidence that patients who are transfused frequently do end up with iron overload since the amount of iron taken in is excreted by the body in the same amount. This iron overload causes other major complications including congestive heart failure, liver problems, and kidney problems.
Treatment of iron overload
Until recently the only available medications to help in the excretion of this excess iron otherwise known as cheleters was a cumbersome effort which could only be delivered subcutaneously through a pump because the half life of the medicine was only 20 minutes and so there was a need for continuous delivery of the medication. On a good note, oral cheletors by the names of exjade and deferoxamine have been developed. Such new techniques of treating sickle cell complications are very important to be used in developing countries with higher incidence of sickle cell disease such as Uganda. For example, using a Transducer Cranial Doppler (TCD) in many sickle cell children enables physicians to identify patients who are at risk of developing strokes in time for interventions. Jell is applied around the child’s facial area above the eye brows and the screen would be taking pictures and numbers of which the neurologist physician bases the finding.
Another important aspect of this convention was the call by many presenters that it is time to change the management of sickle cell disease by looking at the transition of sickle cell disease children to adulthood. Better management techniques have greatly reduced the level of mortality in children with sickle cell disease. Yet no efforts have been made to direct a smooth transition to adulthood and yet sickle cell disease management in adulthood continue to be neglected. There was therefore a call to come out with strategies of managing the disease in adults. In case of the United States, many adult patients find themselves unable to secure medical insurance since they lose most of the available free medical care offered by most of the states by the age of 21 years. Frequent crises also means frequent visits to hospitals with hostile care providers who look at the patients as drug seekers!! Management in childhood is always a teamwork comprised of hematologists, registered nurses, nurse practitioners, social workers, psychologists, and lab technicians. This team gets used in giving primary care to the patient until adulthood (teen age, which is 18 years in the United States). Unfortunately there is no such approach in sickle cell disease management once patients reach their adulthood. Patients find themselves being seen by multiple physicians some of whom know little or nothing on sickle cell disease. The bond and trust that had been built (for 18 years) during the early years between the therapy team and the patient is therefore broken during adulthood. Calls for smooth transition are just timely.
We do know that this kind of problem does exist in Uganda today and that there exist minimal number of hematologists specifically trained to deal with sickle cell disease adult patients. I hope that Uganda do learn from the new experience of treatment strategies for adult sickle cell patients.
Genetics
During the conference there was significant contributions made by doctor and Professor Betty S. Pace of the Department of Molecular and Cell Biology and Director of sickle cell disease research center at the University of Texas in Dallas. Professor Pace shed some light at how far researchers for sickle cell disease are in finding a cure basing on the information available from the Genome Project. The Gnome Project was completed in 2003 and it is basically a blue print of human body having been able to identify all the necessary genetic information regarding humans. Researchers in sickle cell disease just like in other incurable diseases continue to have high hopes in manipulating DNA of patients to cure diseases. It was quite refreshing to note that the National Institute of Health (NIH), which is the leading research umbrella in the health sector in the United States, is collaborating with other countries including developing countries in the search for cures of different diseases including sickle cell disease. Uganda can explore such opportunities that exist with sickle cell disease. We mentioned to Doctor Pace about our plans of starting such educational sickle cell disease conventions in Uganda, she immediately volunteered sponsor her trip (ticket, lodging etc) with others to join us in Uganda.
DNA
Another current on going research was presented by Doctor Mathew Porteus, PhD. His lab is currently trying to fix the mutation that causes sickle cell disease. The field of gene therapy developed as a way to treat genetic diseases, such as sickle cell disease, by changing the DNA of the cell for therapeutic benefit. Doctor Porteus said that, there are a number of different approaches to gene therapy, but his lab has focused on trying to “fix’ the mutation that causes sickle cell disease. His goal is to remove the blood stem cells that contain the sickle cell mutation in both copies of the b-globin gene from a patient, fix one of both of the genes so that they no longer have the sickle mutation, and then return those cells back to the same patient. He hopes that by “repairing” the sickle cell mutation in enough of the patient’s blood stream cells that he and his team can cure the disease. He was very impressed so far and discussed his progress towards this long-term goal. It may take 5 to 10 years. We are keeping our fingers crossed!!!!!!!
Nigerian experience
We were so thrilled about the support from our sister countries Nigeria and Ghana in relation to the struggle of sickle cell disease in Africa. Doctor Ramesh C. Pandey of Xechem, Inc, New Brunswick, New Jersey, presented about the new anti-sickling natural herbal drug Nicosan / Hemoxintm in Nigeria. Various basic and clinical studies of Nicosan formerly known as Niprisan have been performed over the past 10 years in Nigeria at the National Institute for Pharmaceutical Research and Development (NIPRD) and for over five years at several laboratories in the United States. Dr. Pandey was hired directly from the United States by the Nigerian government to take part in this research. He has been involved in research of this drug for a period of over five years. Nicosan is an extract of four plants which has been used by traditional health providers in Nigeria. Phase I/II clinical trials showed a decrease in the frequency of painful episodes and an increase in school attendance of people with sickle cell disease. Laboratory work carried out at the NIH NHLBI Sickle Cell Disease Reference Laboratory at The children’s Hospital of Philadelphia in United States (SCDRL-CHOP) showed that NICOSAN has strong anti-sickling effect. Their studies in vivo using transgenic sickle mice that express human sickle cell hemoglobin showed that NICOSAN not only decreased the formation of sickled cells in blood, but also prolonged the survival period of mice that had been exposed to severe hypoxia (5% oxygen / 95% nitrogen). Other studies performed at Xechem Laboratories in New Brunswick, New Jersey in United States and Xechem Nigeria in conjunction with SCDRL-CHOP have shown that NICOSAN contains various aromatic aldehydes that combine with sickle hemoglobin and inhibit sickling erythrocytes at very low concentrations. Further studies showed that NICOSAN contains chemicals that inhibit cell sickling by hydrating sickle erythrocytes. According to Doctor Pandey, NICOSAN is a new effective anti-sickling natural herbal drug available in Nigeria. The studies conducted in Nigeria, will be repeated in the United States so that it can be available over the counter by patients of sickle cell disease. We are very happy for Nigerians. Some patients/parents etc have been contacting loved ones in Nigeria to send them NICOSAN to United States.
Hydroxyurea
Currently hydroxyurea continues to have more success in treating painful events. Clinical trials conducted in 1995 showed a 44% decrease in medical contact for treatment of painful crisis. Whereas, hydroxyurea was thought to work better due to toxicity concerns, the HUG_KIDS trial demonstrated that children from five years and older could also be given the same dose basing on body weight and experience the same benefits. During the convention proceeding, there were testimonies from patients using hydroxyurea on its efficacy. While speaking about sickle cell disease in Uganda, we were told that hydroxyurea is not available at the Ugandan market and that they only read about it despite the fact that it has been on the market in the United States for over ten years now!
Bone marrow
Bone marrow transplant is still the only documented cure for sickle cell disease. Its limited use can be explained from the many problems associated with it such as finding a matching donor, surviving chemotherapy, and finding enough stems cells for therapy. The later is made easier for patients who are lucky to find a matching donor from cord blood stem cells.
November 11, 2005
Uganda's Forgotten Disease
by Pius Sawa Murefu, Kampala, UgandaSickle cell disease is a serious public health problem in Uganda and completely ignored by the government here.
Twenty percent of Uganda’s population has sickle cell traits and 25,000 children are born each year with sickle cell.
There are approximately 2000 charities working on HIV/AIDS in Uganda. Not a single one on sickle cell disease. The country has only one daycare sickle cell clinic: nothing but a small room made of plywood - no windows or facilities. There are 5000 registered patients at the clinic with only one dedicated doctor; and, he has to work from morning to evening, treating 200 patients a week. Many Ugandan doctors work at HIV/AIDS clinics because of higher pay.
The problem gets even worse in the rural areas. People are dying due to lack of treatment. They cannot afford to travel to the clinic in Kampala.
The Sickle Cell Association, run by two female “sicklers”, has been visiting districts offering counseling services. The turnout is always overwhelming.
Ruth Mukiibi, the chairperson of the association, says people in severe pain: children with swollen spleen, for example, and an anemic requiring a chronic transfusion, but they can’t help them. One parent says that he knows nothing about sickle cell apart from dying children. Others think it is a form of witchcraft.
Ruth says, “It took us two years to see the Ministry of Health about this problem. The Health Minister says was that there are more serious issues such as TB, Malaria and AIDS to tackle”.
Professor Graham Sergeant, from Sickle Cell Trust, in Jamaica, has been funding the Sickle Cell Association for the last five years. He wonders when the government will recognize that people are dying of the disease every day.
He says government needs to be aware of the frequency of this disease, and get involved in public education, and funding should be provided for research and disease management.
The Sickle Cell Association is appealing to the government to incorporate sickle cell awareness into the school curriculum. In addition, the Association has been soliciting for $130 000, to set up the first phase of a sickle cell center. The Association has also threatened to organize a demonstration at the parliamentary buildings if government continues to ignore the disease. Patients are ready to travel and join them.
What is Sickle Cell disease? Sickle cell disease describes a group of inherited blood disorders characterized by chronic anaemia, painful events, and various complications due to associated tissue and organ damage.
Because sickle cell diseases are characterized by the rapid loss of red blood cells as they enter the circulation, they are classified as haemolytic disorders, "haemolytic" referring to the destruction of the cell membrane of red blood cells resulting in the release of haemoglobin. [Source: Health A-Z website]
Some history: Sickle cell disease has been known to the peoples of Africa for hundreds of years. In West Africa various ethnic groups gave the condition different names:
Ga tribe: chwechweechwe
Faute tribe: nwiiwii
Ewe tribe: nuidudui
Twi tribe: ahotutuo
In the western literature, the first description of sickle cell disease was by a Chicago physician, James B. Herrick, who noted in 1910 that a patient of his from the West Indies had an anemia characterized by unusual red cells that were "sickle shaped.".
Some good news: Many children with sickle cell disease could be cured with stem cell transplants, according to new medical research from France. Doctors say they that 85% of children given the treatment are alive and disease-free after three years. [Source: BBC news]
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