• drowsiness,
• nausea,
• vomiting
• diarrhea,
• constipation,
• mucositis,
• anorexia,
• stomatitis,
• bone marrow toxicity (which may take 7–21 days to recover after the drug has been discontinued),
• alopecia (hair loss),
• skin changes,
• abnormal liver enzymes,
• creatinine and blood urea nitrogen

Due to its effect on the bone marrow, regular monitoring of the full blood count is vital, as well as early response to possible infections. In addition, renal function, uric acid and electrolytes, as well as liver enzymes, are commonly checked.

On a positive note a 2005 study with sickle cell patients indicated that long-term hydroxyurea treatment can improve height, weight, and spleen function, and reduce episodes of acute chest syndrome. Patients in the study started the treatment as babies, and most patients took the drug for at least 4 years.

Hydroxycarbamide has been used primarily for the treatment of myeloproliferative diseases, which has an inherent risk of transforming to acute myeloid leukemia. There has been a longstanding concern that hydroxycarbamide itself carries a leukemia risk, but large studies have shown that the risk is either absent or very small. Nevertheless, it has been a barrier for its wider use in patients with sickle-cell disease.

One of the main reasons that Hydroxyurea has been recommended for sickle cell is that there is evidence to show that it increases the production of fetal haemoblobin (HbF) which appears to be able to carry more oxygen. The mechanisms are not fully understood but would appear to revolve around breaking down sickle cells and increasing the production of Hb.

I’d always prefer to look for non pharmaceutical lifestyle changes first and that has taken me in the direction of the following.

Cytidine Analogues. Cytidine analogues increase HbF production by affecting the genes that regulate it. Decitabine is one such drug that was developed to treat leukemia and other blood malignancies. Early studies are suggesting that it significantly increases HbF production, even in patients who have failed hydroxyurea. Only minor toxic side effects have been reported to date. So my question is where could the body get naturally occuring cytidine precursors. The answer is from eating meat from organs such as liver, kidney, heart etc. These are rich in Dietary sources of cytidine and include foods with high RNA (ribonucleic acid) content, such as organ meats, Brewer’s yeast, as well as pyrimide-rich foods such as beer. During digestion, RNA-rich foods are broken-down into ribosyl pyrimidines (cytidine and uridine), which are absorbed intact. In humans, dietary cytidine is converted into uridine, which is probably the compound behind cytidine’s metabolic effects.

Butyrates. Butyrates are natural fatty acids, which are the end-products of fermented carbohydrates in the intestinal tract and they are also metabolized from fiber. One derivative, arginine butyrate, has been under investigation for some time in sickle cell for its role in stimulating production of HbF. Intermittent therapy using intravenous administration has achieved increased levels. In a promising 2002 study administering arginine butyrate improved ulcer healing by ten-fold. Because its actions are different from hydroxyurea, experts hope the two drugs may eventually be used in combination. However, arginine butyrate is difficult to administer, and experts are looking for different forms that might make it simpler to use.

So my advice is to eat food before resorting to drugs. Eating a diet with plenty of liver and kidney could be beneficial. Try for yourself and let us know how you get on.

Now I applaud the lateral thinking of those who came up with the research but my inclination is always to ’swim against the current’ – I started by looking at our own experience with sickle cell and huntingtons and the results we’ve seen (not scientific studies but consistent results based upon experience). A sugar called Trehalose has interested us for a number of years and if you do a little research about its activity with regard to inflammation (just google trehalose inflammation and see what comes up). Trehalose interrupts the cytokine process very early and has a very potent anti-inflammatory effect.

So my little mind is wondering why medical research is focussing on the worshipped statins – (they even want to give them to children now) and not on Trehalose – it could be the positive effects of Trehalose match or exceed the benefits of statins for these children with sickle cell. The huge advantage with trehalose is that there are no side effects – only benefits. Trehalose doesn’t make money for the drug companies – increased uses for stains would make huge profits for these predatory giants.

I would suggest that before trying Statins you try something that is natural and safe and possibly just as effective – maybe more effective.

So thank you for this research but did we really need to spend this money to prove what we already knew.

For years, clinicians have routinely advised patients with sickle cell disease to dress warmly and avoid extremes of temperature, especially cold weather, based on laboratory evidence that more red blood cells undergo sickling with temperature changes.

According to lead author Wally R. Smith, M.D., professor of medicine and chair of the Division of Quality Health Care, the team has found for the first time that pain in adults with sickle cell disease is affected by climate and geographic location.

Wrap Up Warm

“This research confirms pain is worse in fall and winter, better in spring and summer, and pain is worse in colder climates. This has public health and policy implications since advice to patients to dress warmly and avoid extremes of cold can now be stronger,” said Smith, who is scientific director of the Center on Health Disparities at VCU.

“Similarly, the fact that cold weather does affect these patients’ health should be disseminated to employees, employers and schools, and could potentially be used in disability determinations for these patients,” he added.

Sickle cell disease is a genetic blood disorder that affects more than 70,000 Americans, and is characterized by pain. Severe pain related to sickle cell disease is known as a crisis period and typically is treated with fluid, rest and opioid analgesics.

The study included 21 clinical sites in eastern North America and a total of 229 participants who recorded their daily pain intensity – measured on a scale from 1 to 9 – and pain frequency in a diary for a period of four years.

In the study, researchers analyzed the data from these diaries, as well as monthly climatologic data, including temperature and barometric pressure to examine the relationship between climate conditions, geographic location and monthly intensity and frequency of sickle cell pain.

They observed a cyclic pattern of pain intensity and frequency with peaks in late fall and early winter and a trough in the spring. Smith said that higher monthly temperatures were significantly associated with both lower pain intensity and pain frequency, but higher monthly barometric pressures were significantly associated with greater pain intensity and frequency.

Sickle cell disease affects the body’s red blood cells. Normal red blood cells carrying hemoglobin are smooth, flexible and donut-shaped and can travel easily throughout blood vessels. However, sickle cell patients have stiff, crescent-shaped red blood cells that do not flow freely through blood vessels. Pain results when the irregular-shaped cells clot together, causing blockages that may lead to potentially dangerous complications that can compromise a patient’s organs.

The majority of patients are African-American, but the disease can affect people of Spanish, Portuguese, Italian and Greek decent. About 2.5 million Americans have sickle cell trait, which is the gene that causes the disease.

The findings were reported in the November 2009 issue of the journal Pain, the official publication of the International Association for the Study of Pain. The patient sample was taken from a large clinical trial known as the Multicenter Study of Hydroxyurea. Hydroxyurea is the only Food and Drug Administration-approved drug to prevent painful attacks of sickle cell disease and works by preventing cells from sickling.

Editors note – just because something is approved by the FDA doesn’t mean it is right or the best – just approved – there are many things you can do without drugs.

Payout for Marston Green man denied jab

Jan 6 2010

A MARSTON Green man who was left blind and disabled after blundering doctors failed to give him a ‘simple injection’ received a substantial payout last week.

Raymond Stanley, 42, who was born with Sickle Cell Disorder and thus susceptible to pneumococcal infections, was not given the recommended jab to prevent the deadly disease.

Over eight years, five different GPs failed to provide the former postman with the vital vaccine, which would have prevented him contracting the infection and suffering a heart attack.

“My life has been turned upside down due to the infection I contracted and the health problems it caused,” said Raymond, who is still very ill at the moment.

“I can no longer work, and my wife, Kim, has also had to give up her job in order to care for me full-time at home.

“Hopefully the settlement we’ve received will ensure I get the best possible health care for my disabilities and health problems in future.

“I would strongly urge anyone who has Sickle Cell Disorder or has had their spleen removed, to take it upon themselves to insist on a pneumococcal vaccine from the doctor – I’ve learnt the hard way what the consequences can be if you don’t.”

The Department of Heath recommends that patients with Raymond’s condition be inoculated to prevent pneumococcal infections, but between 1996 and 2004, no injection was given.

Raymond contracted a severe pneumococcal infection in 2004, which led to a heart attack and septicaemia, leaving him blind and with kidney damage. Also, during his seven-month hospital treatment he developed heel ulcers which became infected with MRSA and have still not healed to this day.

“This case constitutes a gross failure in patient care from a number of doctors who did not provide the necessary treatment for Mr Stanley’s condition,” said solicitor James Bell of Russell Jones and Walker, who represented Raymond.

“It’s frightening to think how many other people may not receive the simple vaccine which could ultimately save their lives.”

Fifteen athletes, most of them college football players, have died in the past decade from a condition that is benign in most circumstances. They were carrying the sickle cell trait.

Kourtni Livingston, a 14-year-old girl who played basketball for DeSoto High School near Dallas, died running laps outdoors in August 2002. Then University of Missouri linebacker Aaron O’Neal died during a voluntary workout in 2005. Devaughn Darling died shortly after complaining of dizziness and chest pains during off-season conditioning exercises in February 2001 at Florida State University. Experts have found that under conditions such as extreme exertion, high altitudes and hot climate, sickle cell trait is associated with a potentially deadly blood disorder called “sickling.”

In January, the National Collegiate Athletic Association is expected to decide whether it will be mandatory for colleges and universities to test athletes for sickle cell trait. But some experts are concerned that this could lead to athletes with the trait being singled out and excluded from sports.

Millions of people in this country have inherited the sickle cell trait, an estimated 1 in 12 African-Americans, but also Hispanics, Mediterranean people, others from parts of the world where malaria is common, and about 1 in 2,000 to 10,000 Caucasians, according to the U.S. Centers for Disease Control and Prevention. Having the sickle cell trait is known to provide some protection against the malaria parasites. This is not the same as having sickle cell anemia, a painful and debilitating disease that affects about 70,000 people in the United States.

In 1998, the federal government began requiring that all infants be screened at birth for the sickle cell trait. Since test results conducted in-hospital may take weeks, that information is seldom passed along to the parents or the child.

Checking for sickle cell trait is not something that ever crossed the mind of Bridgette Lloyd. Her 19-year-old son, Dale Lloyd II, had been an athlete all his life, excelling in football and baseball. He was offered an athletic scholarship to Rice University, the big Division I school in Houston.

“Dale was a gift from God, truly. And I would always instil in him, ‘Son, you are truly blessed not only athletically, but academically,’ and he would say, ‘Mom. You know, you’re right.’ ”

Before he headed off to Rice in June 2006 to prepare for the upcoming fall season, his parents said they had him checked out from top to bottom. More than anything, they wanted to know his heart could withstand the pressure of strenuous workouts.

“We would always look forward to make sure he had a good heart,” Lloyd says, “because they always say young people getting out there and working out, sometimes their heart gives out.”

In late September, the football team had just returned to campus after a 55-7 bruising. The 190-pound freshman defensive back headed out for an evening with friends. The next day, team members told her, Dale was back on the field for a light workout that included 16 100-yard sprints.

“The light workout turned into a bit more strenuous workout,” she says. “They did weightlifting and a lot of running. During the running portion he collapsed.”

When she and her husband, Dale Lloyd Jr., arrived at the hospital that afternoon, they were met by doctors who were baffled by his condition.

Bridgette Lloyd says, “They had no idea what they were looking for and what was going on with him. They were trying to get all kinds of information from us. Late into the night someone asked … if he had the trait.”

She means the sickle cell trait. The Lloyds had no idea. It had never occurred to them to have him tested, and no one had suggested that they should. It turned out that Dale Jr. carried the gene and had passed it to his son.

“He passed away the following day,” Bridgette Lloyd says.

The cause of death was rhabdomyolysis, a condition associated with sickle cell trait and brought on by extreme exertion, high altitudes and hot climate. The flat red blood cells sickle, stiffen, blocking microvessels that carry the blood, starving tissue of oxygen.

Taking Precautions

Many coaches and trainers are still ignorant of the problem, says Scott Anderson, director of athletic training at the University of Oklahoma. He became aware of it about 14 years ago when one of his players collapsed. “The physicians at the hospital recognized that he had sickle cell trait, but they said that’s not what his problem is,” says Anderson.

That athlete survived his collapse, but Anderson wasn’t entirely convinced of the doctors’ explanation. He undertook an investigation and found that in fact, the athlete’s collapse was similar to other cases that had not ended so well.

“Deaths were occurring,” Anderson says, and even as athletes were dying those cases were not communicated to the profession as a whole. They were singular cases in isolation and oftentimes individuals would think that it was a unique occurrence.”

So Anderson convened a task force of the National Association of Athletic Trainers to study the problem of sickling in young athletes. In 2007, the group released a consensus statement calling for screening of all athletes for sickle cell trait.

“It doesn’t matter whether they are playing football or another sport, male athlete or female athlete,” Anderson explains. “It doesn’t matter whether they are African-American, Caucasian-American, Native American or whatever.”

The point is not to exclude anyone from sports, Anderson says, but to make sure that all precautions are taken, with a particular emphasis being placed on educating athletes about their condition. Most of the incidents occur during conditioning training, when these highly competitive athletes are pushing themselves or being pushed to the breaking point. Anderson sets limits.

“We make them aware of signs and symptoms of exertional sickling. If they are experiencing undue fatigue, shortness of breath, a sensation at the lower extremity, a lower back cramping spasm,” Anderson says, “we want them to report those symptoms immediately.”

At the University of Oklahoma, Anderson says, any athlete would be withdrawn from training at this point to be given time to recover. Rest periods between activities are longer for athletes with the trait than for other players. They may have fewer sprints, for example.

At the same time, trainers make sure that the athlete’s capacity to be competitive is not compromised. In fact, in surveys done by the National Football League, it’s estimated that 7 percent of the players have the sickle cell trait. Anderson proudly notes that that has included some of Oklahoma’s best.

“Curtis Lofton is one. He was an All-American linebacker for us at the University of Oklahoma. He currently plays for the Atlanta Falcons. He’s a defensive standout for them.”

Unfortunately, Bridgette Lloyd did not know any of this when she sent her son off to Rice University, but she says the NCAA knew about the deaths of young athletes associated with having the sickle cell trait, and so her family sued.

“I found out when Dale passed away, that you will find names and names and names of kids,” Lloyd says. “Young people who should have had young and productive lives that were cut short because the NCAA didn’t inform them that this situation could affect their health.”

As part of a settlement the NCAA agreed to recommend that all universities and colleges offer athletes screening for the sickle cell trait. And Rice University will propose a bylaw at the association’s annual meeting to make that mandatory. Currently, 64 percent of colleges screen for the sickle cell trait.

But some African-Americans and sickle cell experts, such as Dr. Elliot Vichinsky, a hematologist at the Children’s Hospital Oakland, worry that the mandatory testing policy hasn’t been given enough thought.

In the past, African-Americans were blocked from military service and certain kinds of employment if they had the sickle cell trait.

“Given that history,” Vichinsky says, “if you just have a willy-nilly ‘go get tested and then go see somebody for counseling’ [policy], there is a tremendous medical, legal pressure on them to do this. There is inherently a medical-legal risk.” He argues that coaches will say to themselves, “Why would I put a trait person out to play on Sunday when it’s really hot? I may be liable.”

Instead of screening, Vichinsky recommends further study of a problem that he says has not been appropriately investigated. In the meantime, he says, colleges and universities should be required to use preventive regimens of rest and rehydration for all athletes during training, noting that just 5 percent of the more than 136 sudden deaths in athletes during training have been attributed to the sickle cell trait.

Former Idera Pharma CEO at Helm of AesRx, New Startup With Sickle Cell Drug

Ryan McBride 1/5/10

Stephen Seiler has been hunting for cash to advance an experimental therapy for sickle cell disease into initial human studies. And the veteran biotech executive, the former CEO of Cambridge, MA-based Idera Pharmaceuticals (NASDAQ:IDRA), has founded a startup in Newton, MA called AesRx to carry out his goal.

Seiler said he has provided an undisclosed amount of money from his own pocket to support the young startup, which was formed in November 2008. Now he’s looking to raise about $10 million in a Series A round of venture capital to advance the startup’s lead drug, called Aes-103, into a Phase I clinical trial next year for sickle cell disease. He’s making the case that the current treatments for sickle cell disease (sometimes called sickle cell anemia), which affects about 75,000 Americans, have left patients with far too few options to treat the genetic, lifelong illness that causes pain, infections, organ damage, and can often be deadly.

The firm’s drug is a small molecule called 5-hydroxymethyl-2-furfural, or 5HMF, which is derived from sugar and can be found in everything from caramel and cookies to prune juice. Based on preclinical studies, according to Seiler, there is evidence that the drug could be less toxic than previous treatments for sickle cell disease. For example, the standard treatment for the disease, called hyroxyurea, is typically prescribed only when patients are experiencing pain and other complications of sickle cell because it can cause side effects such as vomiting and lowered red blood cell production. And while bone marrow transplants have been able to cure the illness, there are challenges to making this therapy available to most patients because a matching donor is needed and it’s too expensive for them.

“Although it’s still pre-clinical, we know a lot about this drug,” Seiler said. “Therefore we believe our odds of success are better than a random pre-clinical compound.”

For now, the company is supporting some of its claims about the potential utility of its treatment with outside, peer-reviewed studies that validate the importance of the target that AesRx’s treatment binds with, as well as the drug’s intended mechanism of action. The compound was also studied extensively by researchers at Virginia Commonwealth University who first discovered its potential to treat sickle cell disease. (Seiler purchased the experimental drug after its former owner, New Jersey-based Xechem International, filed for Chapter 11 bankruptcy protection in fall 2008, he said.)

by Paul Louis, staff writer

(Natural News) An epidemiological study in Israel that included 84,832 babies born at Soroka Medical Center, in Beer-Sheva concluded that medications taken during the first trimester that block folic acid more than double the risk of congenital malformations.

The study team involved Epidemiologists, Pediatricians, Clinical Pharmacologists, Obstetricians and Gynecologists who examined birth and abortion data collected in Israel between 1998 and 2007.

The medications that act as folic acid inhibitors are the antibiotics trimethoprim, sulfasalazine for treating ulcerative colitis, and the chemotherapy drug methotrexate. This group of drugs prevents folic acid from being converted to its active metabolites.

Anti-epileptic drugs and cholesterol lowering drugs are among the group of medications that lower serum and tissue concentrations of folic acid.

All about folic acid

Folic acid (B9) is also known as folate or folacin. It is essential for building new cells, and everyone needs it. But it is especially crucial for a woman’s physiological fetal function during pregnancy. Abundant folic acid during early pregnancy is important for preventing neurological and spinal birth defects.

Doctors are now recommending extra folic acid intake for women during the first trimester of pregnancy. Folic acid is abundant in leafy green vegetables, grains, dried beans, peas, nuts, and fruit. Adding daily supplements of folic acid is usually recommended.

The most common major birth defect from folic acid deficiency is spina bifida, or open spine. It is the result of the fetal spinal cord not closing completely during the first month of pregnancy. Nerve damage can result in the child’s paralysis of the legs, fluid in the brain, learning difficulties, and urinary or bowel problems. There is no cure for this birth defect.

Sources for this article include:

http://www.eurekalert.org/pub_relea…

http://www.nlm.nih.gov/medlineplus/…

http://www.nlm.nih.gov/medlineplus/…

http://www.upi.com/Health_News/2009…

http://visitbulgaria.info/11732-fol…

Wrap Up Warm

For those of us in the Northern Hemisphere winter will be rolling in soon and we need to get ourselves ready so that it does not catch us unaware. There are a lot of things you can do to keep yourself warm and healthy, which is the most important thing during winter. First of all I found out that if the weather is cold take warm drinks which helps to warm the body up and reserve heat in the body. Also eat regularly, instead of eating three full meals in the day try splitting it into six. You will find that, you have more energy and would not get cold easily.

Wrapping up warmly in winter, especially when going out, is very important, because those with sickle cell lose heat very quickly, it is advisable to wear a lot of warm clothing. The easiest places to loss heat is from the head, neck, hands and feet. That is why it is very important to keep them wrapped up, as for the head make sure you have a woollen hat or a cap that is warm and would cover the ears. Get a scarf to wrap round your neck, this would prevent heat loss from the neck. I found out that knitted gloves are not that warm, you can either get very warm gloves from shops that sell clothings for those that go hiking or out door sports. Or you can get hand warmers with the woollen gloves, these can be put in the pocket and they heat up to warm your hands. As for your feet make sure you get a good pair of boots, that you can wear socks in.

However do not forget your supplements, these would do you a world of good keeping up your health. Make sure you keep up your drinking of water, but making sure it is lukewarm not cold. Cold water or anything cold is a shock to the system, and you do not want to do this because it may set off a painful crisis. If you want to drink any thing hot you could go for green tea, with trehalose which is a healthy sugar, or any other fruit tea, but nothing with caffeine in it. Also remember your omega 3 it would help a lot with the joints.

Exercise is another good way of keeping yourself healthy and warm. Exercise little and often-  it helps to prevent muscle wastage, and keeps the body healthy and prevents you ageing prematurely. You do not need to join the gym, but simple exercises in doors would keep you in good health.

Ruth Akiboye

Paul Barton

I have long been a campaigner for help for people with sickle cell and have recently teamed up with Paul Barton. I know what it’s like and have suffered for years. In the last few years I’ve taken a different and much more proactive stance and as a result I’ve seen some remarkable improvements:

• haemoglobin levels increased by close to 10%
• virtually eliminated crises
• reduced sickling pain to very low levels and go long term without any pain

This e mail is to invite you to join in our ongoing exploration of what we as sickle cell sufferers is possible, without resorting to pharmaceutical intervention. If you don’t want to join in this discussion at the moment please click here and you can unsubscribe and won’t receive any e mails from here on. You can always come back at a later stage or you can watch the growing discussion on-line at our website Hope For A Healthier Life

If you’ve not listened to the earlier recordings about sickle cell with Dr Emil Mondoa MD then you can do so here.

The diet should include:

• Lean meat, skinned fish and chicken
• Eggs
• Skimmed milk
• Low fat cheese, yogurts and spreads
• Soups
• Salads
• Grilled fish and chicken
• Legumes

Foods that need to be avoided:

• Fried foods
• Spicy food
• Whole grain breads
• Cereals, seeds and nuts
• Baked beans
• Broccoli
• Cabbage
• Brussels sprouts
• Cauliflower
• Peanuts
• Pastries

After the removal of the gall bladder, it is very essential to maintain a low carbohydrate and high protein diet. Cooking meat and fish at low temperatures and draining off the released fats helps a lot. It is also advisable to adopt six smaller meals rather than three large ones. Without the gall bladder, digestion does become difficult and hence this shift in the diet plan is required. A liquid diet for some time is ideal. However, if it is not completely possible, then you could consider semi liquid meal components that are non-fatty and low-carb, essentially.