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.

HIV/AIDS & Cancer Palliative Care

by Bamuturaki Musinguzi, Kampala, Uganda (June, 2006)

Nurse Jerith, a CPCC Nurse, shows a patient and family how to measure out liquid morphine.

For a price of two or three loafs of bread cancer and HIV/Aids patients in Africa can now afford cheap pain killers for a week after the continent obtained affordable oral morphine in 1990, which can be used in a home and controlled by the sick.

This also followed the international community declaring “to be free of pain” a human right. This

brought up a challenge for medical and health workers to manage and control pain and give palliative care (a holistic approach looking at the spiritual, physical, social, cultural and economic) of HIV/Aids and cancer patients. It costs Hospice Uganda Ushs. 20,350 ($11) per week to care for a patient. Hospice asks the patients for a contribution of Ushs. 5,000 ($2.7) per week towards the cost of medicines, and less than 1/3 of its patients can afford this. It assists 60 per cent of those who cannot afford.

“It was a big break through because it’s now cheaper to bring in the morphine powder and make it here in a pharmacy,” said Dr. Anne Merriman, director of policy and international programmes, Hospice Africa Uganda.

“ If to be free of pain,’ is a human right then people should ask their MPs to have this service in their constituencies,” Dr. Merriman suggested.

“Its difficult to abuse oral morphine, there is no addiction or diversion compared to the tablets,” Dr. Merriman said.

“When taken by month it dose not give ‘a high’ if you have pain.” According to Dr. Merriman the pain of cancer never goes until one dies:

“We can control the pain of cancer of 98 per cent of the patients we treat and have the pain controlled. For HIV/Aids patients they usually get severe pain from infections such as Kaposi’s sarcoma (commonly called Kisipi in Uganda). If they have access to the antibiotics/fungi then as the infection is cured then we can withdraw the pain killers.” Dr. Merriman, who was the first director of the Nairobi Hospice in 1990 and later left to start a model Hospice in Uganda in 1993 where other countries would learn by taking care of the African cultural and economic aspects, believes making palliative care affordable has been Hospice Africa’s greatest objective on the continent.

Hospice Uganda is the model Hospice for Hospice Africa. Hospice Africa was founded in 1993, to promote the initiation of Hospice in those countries in Africa who have not yet got assistance of palliative medicine.

Uganda was the third country to commence palliative care in sub-Saharan Africa (excluding South Africa). Zimbabwe was the first (1977) and Nairobi Hospice the second (1990). The objectives of Hospice Africa are to provide and promote a palliative care service to patients and families, within a 20 kilometer radius of Hospice. To carry out education programmes in palliative medicine, to health professionals at undergraduate and postgraduate levels throughout Uganda so that this form of care can be available to all in need. It also encourages the initiation or consolidation of palliative care in other African countries, by providing a facility at hospice Uganda fro training, and experience of palliative care working in the African context.

Each year Hospice Africa Uganda has a Palliative Care Week to promote palliative care and to let people know that they have a human right to be free of pain before they die. This years week from May 8 -13th, 2006 under the theme, “We want to see that this Medical Specialty Come to Your Home,” climaxed with a hospice sponsored charity walk on May 13th from the Constitution Square in the capital city and finishing at the hospice headquarters in Makindye a suburb of Kampala to raise money for the terminally ill patients in Uganda. Hospice Uganda is entirely dependent on the goodwill of donors. In UK support comes from two charity shops in Liverpool and Ainsdale, run by volunteers. Donations come from other organizations and individuals all over the world on an ad hoc basis. WHO in 1996 recommended that nurses would be allowed to prescribe morphine in countries where there are insufficient doctors.

Uganda is the only country in the world where nurses and clinical officers have undergone training at Hospice Africa Uganda in palliative care. They can prescribe morphine without a doctor after the Ugandan government amended the law. Hospice Africa Uganda has trained over 2,730 health and non-health professionals in 21 districts of the country of which 325 are community volunteers. Hospice Africa Uganda that started with three staff in 1993 has now grown to 107 workers in three Hospice centers in Kampala, Hoima and Mbarara districts. In the last 13 years it has looked after 9,000 patients 6,000 of whom have been on oral morphine. It’s estimated that up to 60 per cent of its cancer patients also have Aids. With the conquering of infections diseases by improved sanitation and the recent arrival of Aids with its associated cancers, the causes of death are now changing. In those countries without disasters of war or famine, cancer is the first or second cause of death. Unfortunately less than 10 per cent of resources committed to cancer control are available to patients in the developing world where the biggest increase in cancer is taking place.

Hospice Africa says: Patients seek medical care with already advanced cancers and with severe consequences of pain, symptoms and gross disfigurement. Most are sent home from conventional medical establishments and clinics with few simple analgesics at eh most, as there is nothing left to be done fro them. “This leads to untold suffering for the patients and family.”

Currently in Uganda 1.5 per cent of its total population develops cancer each year. The raise from 1 per cent is due to Aids associated cancers in Uganda. Hospice Uganda looks after cancer and or HIV/Aids patients by bringing the modern methods of pain and symptom control.

“Aids has brought an epidemic of death and increased the urgency fro palliative care services not only in hospitals but in the community and reaching to village level where up to 57 per cent of the population may never see a health professional,” Hospice Africa says. “Palliative care must reach these people through training of health and non health professionals who live in the villages. 20-50 per cent of patients with HIV/Aids have severe pain. Aids has brought a great increase in cancers and Kaposi’s sarcoma is now the highest occurring cancer in Uganda.”

There is much attention given to the procurement of ARVs for Africa at present, Hospice Africa observes. “However this will not mean there is no need for palliative care for all, even those who are rich or powerful enough to access them will need palliative care when their time comes.”

Conference in Uganda defines importance of Oral Health

by Pius Sawa Murefu, Kampala, Uganda (6 june 2006)

"Oral health in the developing world is at its worst", says Dr. Shewine Shinne, president of Smile Foundation in the US, who was speaking at a health conference in Uganda recently. She also pointed out that there is now a need for dentists in these areas to be equipped with more knowledge and skills.

The conference underlined the relationship between general health and oral hygiene. A team of students and a professor from the University of British Columbia presented their research concluding that poor oral health can increase the risk of heart disease, diabetes and premature labor.

It was also noted that oral health in relation to HIV/AIDS has rarely been mentioned. Dr. Mulubya Gordwin at Uganda's Mulago hospital, says the oral cavity is the first site in which symptoms of HIV/AIDS become present. He explained that oral manifestations of HIV infection are a fundamental component of disease progression and occur in approximately 30-80 percent of the affected patient population. Symptoms such as Candiasis, leukemia, HIV gingivitis, periodontitis and ulcers are common to those who are HIV positive.

The conference, entitled, "Epidemiology, Impact Challenges and Management of Oral Health in Developing World" was held in Kampala between 5th and 6th June.

Reproductive Health Care: A Basic Human Right

by Rafiqul Islam Azad, Bangladesh "Madam we have come again today. For, my wife has conceived again—a fourth time. We have already two children and we are not in a position to have the third. Last time, her M.R. was done in July last under your care but after that her menstruation became irregular. And we failed to notice it. As a result, we became careless in using contraceptive.” This is what a Senior Assistant Secretary of the government was telling to Hasina Muqtadir, a Senior Counsellor working at the Reproductive Health Services Trainings & Education Programme (RH STEP) located at the 2nd floor of Dhaka Medical College Hospital (DMCH) a couple of weeks ago. “Ok, I am taking care of her case,” the Counsellor assured the man, a resident of Mugdapara area in the city. Being assured by the Counsellor of taking proper care of his wife, the man left for his office leaving the wife at her care saying, “I am coming back at noon.” This was the conversation between the two that was heard by this Correspondent at about 9:45am on a Sunday last month while waiting for talks with the said Counsellor on the activities of the RH STEP. After few minutes, another woman came to the Counsellor from Arihajar upazila of Narayanganj district for her follow-up treatment. The woman, a mother of four daughters and one son, had earlier done her Pep’s Smear test in the center on July 12 last year and she received treatment as she was suffering from severe inflammation found in the test. “Though I feel comparatively better than previous time but I have still some complications,” the woman, aged about 40, told the Counsellor, who advised her to continue the treatment. It may be noted that the government began a special project namely Menstruation Regulation Training and Service Programme (MRTSP) in 1989 to promote the reproductive health. Later on, the project turned into an NGO called as RH STEP in October 1983. The RH STEP now is playing a complementary role to support and supplement in consonance with the GoB’s national health programme targets in reducing maternal mortality rate and morbidity and other reproductive health hazards in Bangladesh. Not only government officials or housewives, Hasina Muqtadir said, women and adult girls from all walks of life come to the RH STEP everyday with their various problems relating to the reproductive health and for treatment and counselling. “The clients are given counseling first. If the counseling is of not considered sufficient to heal their complains, then other actions are also taken as deemed fit for,” she said. Sometimes, the Counsellor said, “We have to do M.R for street girls and unexpected pregnancies of adolescent girls who are victimised socially considering the human ground.” “Everyday 10 to 15 patients are being attended by us,” she said adding that for a single case of M.R an amount of Tk 120 to Tk 130 is charged for. However, if the case is complicated one, the amount may rise to Tk 500. “Most of the women generally come to the RH STEP for M.R. Besides, Pep’s Smear, pregnancy test and such tests that are necessary are done here. When the women leave the center, they are provided with health education and contraceptive pills,” said the Counsellor adding that treatment for uterus infection, which is found common among the mothers or women of child bearing ages are also given to them. A total of 18 RH STEP centers, mostly located at the government hospitals attached with medical colleges and major district hospitals, have been working from 1983 to facilitate the reproductive health care in the country. The RH STEP, funded by the Swedish International Development Agency (SIDA), is engaged in an unabated fight to reduce the maternal morbidity and mortality rate due to induced and septic abortion by providing M.R training to doctors, FWVs, paramedics, nurses etc. It has been providing M.R and contraceptive services besides counselling to M.R clients for follow-up visits, the Executive Director, Quazi Suraiya Sultana said. According to its annual report, the RH STEP has performed over 58 thousands M.R cases during the last fiscal year when about 63 thousands pregnant women were counseled during the period. Of them, maximum clients (28%) are of 25 to 29 age group who were provided the M.R services while only 5.20% clients of 40 and above age group were in the minimum level. The percentage of M.R recipients of 20 or below age group were 5.44. Of the M.R recipients, highest number of 17,160 women with two children were provided the service. At the same time, contraceptive M.R training was imparted to 782 persons including, government and private doctors, FWVs, paramedics, nurses and medical assistants. The report shows that contraceptive services were extended to 68 thousands women and girls when 8,926 patients were given treatment after pep’s smear test during the period. Besides, the RH STEP also provided antenatal care, post-natal care, family planning and other services to more than 20 thousands clients. In addition to the initiative, all the government medical college hospitals, districts hospitals, specialised hospitals, Upazila and Union Parishad level health complexes and thousands of health workers across the country have been providing the reproductive health services. Health experts say there are excellent GO-NGO infrastructure across the country in providing the reproductive health but many of them alleged that most of those remained unutilized due to inadequate supply of medicines by the government and also non-availability of funds. Sources said significant awareness has already been created among the people due to the GO and NGO effort but there are still 30 to 40 per cent women and adult girls in the country who remain unaware of the reproductive health issues. Sources said maternal mortality rate has significantly been reduced to 3 per cent per thousand from the 6 per cent of the seventies. The institutional delivery is also increasing day by day, sources said, it has now stood at 11 per cent with one per cent enhancement than that of last year to reduce child and maternal mortality rate. According to sources in the Family Planning Directorate, about 23 thousand family welfare assistants and 15 thousand health assistants are working at the grass root level for providing basic health and family planning service delivery. About 30,000 “satellite clinics” are also organised at ward and community levels every month all over the country aiming to bring the service facilities at the door step of the people. In that package we deliver antenatal care, family planning, health education and EPI services, said a highly placed official of the directorate. MA Akmall Hossain Azad, Director General of Family Planning Directorate, said that under the directorate reproductive health service and education are being given from its 80 maternal and child welfare centers across the country. On average some 5,000 safe deliveries are being conducted from the centers in each month, he said. Besides, about 3,500 Family Welfare Centers are also proving reproductive health services including free distribution of contraceptives, the DG said. Of the specialised government hospitals, Azimpur Maternity Hospital provides all sorts of maternal services including safe delivery and counselling to the mothers and adolescents. Dr Md Ziaul Karim, Superintendent of the hospital said around 500 expecting mothers come to the hospital for taking health services. He added that over 500 delivery cases are conducted at the hospital in each month. Of them, about two-third are being conducted by Scissorian section operation as most of the complicated cases are referred to this hospital for its reputation. The Urban Primary Health Care (UPHC) Project, assisted by the Dhaka City Corporation, also provides reproductive and general health services to urban people through its 38 centres in the city. There are also many NGOs, which have been working in the field of reproductive health care along with the government initiative. Of them about 15 leading NGOs are specially dealing with the reproductive health. Different GOs and NGOs like the RH STEP are implementing “Behaviour Change Community (BCC) activity to promote the awareness, particularly among the adolescents about the reproductive health so that they could protect themselves from deadly diseases like HIV/AIDS and STDs. Under the Reproductive Health Initiative for Youth in Asia (RHIYA) project funded by EU and UNFPA, five leading NGOs—Concerned Women for Family Development (CWFD), Family Planning Associations of Bangladesh (FPAB), Marie Stops Clinics Society, Bangladesh Red-Crescent Society and Save the Children UK— are also working with the adolescents. Some NGOs, notably BRAC, has a programme on sex education. Many NGOs have already developed culturally appropriate IEC materials regarding the issues for their campaign.