Altitude Sickness

When you go to the ocean the altitude level is equal to zero (0). When you travel up a mountain the altitude level increases and the air pressure decreases. At places like the Grand Canyon the altitude goes up to over 6,000 feet elevation, and in the Rocky Mountains of Colorado it can peak to over 14,000 feet elevation. As the elevation increases, the altitude is higher and the air pressure is lower. The lower the air pressure, the lower the number of oxygen molecules available to breathe. This is also known as thin air.

For people with Sickle Cell Disease, less oxygen means SICKNESS.

My altitude sickness symptoms begin around 3,000 feet. It starts with a headache, feeling tired, and dizziness. If I don’t get out of the mountains, then all of these symptoms get worse. At 4,000 – 5,000 feet, I begin to get confused and agitated. I have shortness of breath, my spleen, hands and feet begin to swell, and my head aches with no relief. At 6,000 feet, I descend into needing hospitalization. I throw-up, more headaches, dizziness, lips and palms turn white, and my body slowly collapses into a full Sickle Cell crisis.

How do I know so much? I found out about altitude sickness by accident. After taking a few vacations to the Grand Canyon and Utah I experienced altitude sickness first hand, the hard way. I travelled to these high places, got sick, and learned my lesson. I discovered that I can not go higher than 4,400 feet elevation EVER (without an oxygen tank). In winter, because oxygen is thinner by nature, I can not go above 3,000 feet elevation.

People with Sickle Cell disease have physical limitations, as well as geographic limitations. I know that I can’t visit the Swiss Alps, unless I have an oxygen tank strapped to my back and a mask flowing cool, clean oxygen into my lungs. That's ok with me. I am informed about my limitations and I can accept them. Information is power.

Now take a minute to inhale, now exhale. Enjoy each and every breath you have because it's very precious.

Sickle Cell In India – Part 3

Some people have asked why I posted two previous blogs from a health-care worker in India?

Well, in my opinion, Sickle Cell Disease and Thalassaemia are global problems that are growing. Ignorance and avoidance have limited the recognition that these disorders have a true impact on society. With global immigration increasing, we have to look at the global impact these diseases have on children, healthcare, healthcare cost, and education worldwide.



Currently, there are American voices crying out for people suffering with these diseases, and they are getting things done in America. Thank God. There are no global voices, however, telling the worldwide story of pain, suffering, and health-care limitations related to these two diseases.

As Sickle Cell Disease and Thalassaemia are silently transferred from one generation to another, all voices are important. Hari Prabhakar, from the Sickle Cell Disease Center at Gudalur Adivasi Hospital in India offers his voice, and I gratefully listen.

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Working at the Sickle Cell Center at Gudalur Adivasi Hospital over the past 5 years has taught us a wide variety of things from both a patient and provider perspective.

Existing literature and health authorities continually stress the importance of cultural sensitivity and community consensus as a means of developing an effective healthcare management system. We have certainly found this to be true, but still face challenges in the implementation of effective healthcare programs and must overcome obstacles such as:

Providing awareness of the disease in the Adivasi population - This is extremely difficult when dealing with a population with a strong and widespread belief system. We use members from the Adivasi community as key health educators (in the villages) to help in gaining acceptance and support for the program. However, a lot more needs to be done regarding genetic counseling managed internally and that does not offend Adivasi group’s beliefs.

Confidentiality of test results - Given that awareness of the disease and its causes is still unfamiliar, there exists a fair amount of shame when an individual is labeled with the disease. It is interpreted that a curse is upon them. Cards are given out to document test results of individuals and are coded to be interpreted only by healthcare providers and patients. Explaining blood test results is also the responsibility of an Adivasi health worker, who we feel can best explain the disease to the patient and instruct them on the appropriate course of action.

Getting medications - Medications like Hydroxyurea (a rather expensive cancer drug used for the treatment of SCD) is hard to get in developed countries where there are areas of geographical isolation. Transportation is too difficult or the patients are migrant workers with little time to take care of themselves. We have tried to stock supplies of critical medications like Hydroxyurea in area Centers throughout the Gudalur valley, where patients can also get their blood tests taken. And, a Sickle Cell Disability Fund is in place to help cover patient transportation costs and make up for lost time in an already unsteady employment situation.

Funding is always a major problem - With the prospects of offering “free” treatment to Adivasi patients and inpatient treatment for complications, we pursue public-private partnerships, private donations/grants, and cross-subsidize from non-Adivasi patients to ensure equity and access in services. Community-based insurance for SCD is also a possibility in order to ensure long-term sustainability and empower patients to become an active part of the financing process.

Researching and developing cheaper more sustainable medicines - In a resource-strained environment where other basic public health needs are minimal, it raises to question the use of Hydroxyurea, the expensive tertiary-care cancer drug. As a result, we have team working to develop a plant-based anti-sickling agent that may be cheaper than Hydroxyrea and more sustainable to produce. In Nigeria, for example, the plant-based drug, Ciklavit, has been shown to have positive results, and we hope that have something similar which could be used locally and internationally.

So, what can we learn for use in the United States?

Regardless of whether we are dealing with a rural village or sprawling metropolis, developing skills of health workers to serve as liaisons to the community is important in gaining support and acceptance. A health service with little confidence from the public will ultimately fail.

There certainly needs to be a push for greater training in SCD among health workers, many of whom could be chosen from the local community to conduct education programs, promote counseling, etc… Complete coverage of the SCD population in the US should be done in tandem with local communities.

Most importantly, however, listening to patients and determining their needs, capabilities, and limitation will play a crucial role in expanding the role of sickle cell programs in the US into a wide-ranging program rather than top-down vertical program.
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( HARI……. Thanks for your contributions to this blog and your suggestions.)

Resources in India

Sickle Cell Sabbath

There is a lot of money to be made by non-profit organizations that support specific “diseases.” Patients, with those diseases, may or may not benefit from those organizations unless they seek them out. One thing that every patient with Sickle Cell disease can benefit from is the donation of blood.

I recently read an article about a young girl with Sickle Cell disease who has received over 400 blood transfusions in her short lifetime. She is now working with community churches to encourage donations as part of “Sickle Cell Sabbath.” This effort was directed to community churches because they are a symbol of goodwill and a pillar God’s love. Together with a local blood center, this young girl has begun a quest to ask churches for donations of blood, rather than money. A donation that we all know will be put to good use.

We can all learn a lesson from this young girl, who has moved past her personal struggle to enhance the lives of others. We can find creative ways to help people and meet their needs, as we care for our own. We can give money, if possible, and we can give love, always. We can also encourage the world to give blood and make a donation to someone’s life. xoxoxox

http://www.redcross.org/

http://www.kentucky.com/139/story/712761.html

Sickle Cell In India – Part 2

This post is part 2 of a contribution from Hari Prabhakar, from the Sickle Cell Disease Center at Gudalur Adivasi Hospital in India.

The recent 2007 World Health Organization (WHO) and Thalassemia International Federation (TiF) report (see link below) speaks on the need for comprehensive sickle cell centers in developing countries to manage the burden of SCD, and also notes that in the USA, existing centers only cater to 18,000 of the 80,000 affected. In India, there are around 5 million carriers and up to 20 million affected, and most of those affected, primarily Adivasis, live in areas with little access to basic health facilities.

In the south Indian state of Tamil Nadu, in the Gudalur Valley of the Nilgiris District, there is a tribal (Adivasi) population of over 25,000 who have lived in the area for thousands of years. Until 1913, there was a high prevalence of malaria, which could explain why the sickle gene and sickle cell disease has persisted for so long. Within the community, historically mysterious deaths and suffering were regarded as “Doshams” or curses. There was almost no awareness or concept of genetic diseases.

In the past, hydration therapy and vitamin supplementation remained the primary means of dealing with patients at Gudalur Adivasi Hospital. The hospital has doctors, nurses, and health workers, a large percentage of whom are Adivasis trained from the community. Unfortunately, we didn’t have a good idea of the prevalence of the disease in the area.

After reviewing some of the existing literature, doing some internal studies, securing startup funds, and talking to some hematologists, we launched the Sickle Cell Disease Center at the Gudalur Adivasi (Tribal) Hospital. The platform of our Center, now 5 years in existence, has been to emphasize screening, treatment, education, and research, and to serve as a national model for SCD nationwide. Realizing the cost of management of the disease would be out of reach for the population, we decided to offer all services free.

The first step in ensuring the effectiveness of the program was to bring awareness of sickle cell disease to the local population. Thanks to the hospital’s longstanding presence and community outreach, we have been able to deploy the Adivasi health workers to educate, while also providing comprehensive services including Hydroxyurea, penicillin, and pneumococcal vaccinations. Unfortunately, the pneumococcal vaccine for children under 5 is not yet available, but thanks to public health professionals throughout the world, it will be available in India for our patients soon.

Every month, there are screening camps in the villages to identify patients and carriers, as well as presentations to villages on SCD, led by our health workers. Those identified as patients are brought to the main Center in Gudalur and given appropriate treatment according to their needs.

In the summer months, the monsoon season hits the area and make transportation a tremendous issue. The terrain and lack of roads in the area make it difficult for those who need to come to the main Center for picking up their medications and getting monthly tests. We have stocked the appropriate medications in 8 area Centers of the Gudalur area to enhance patient accessibility

As of now, we have screened around 6500 and have 185 patients, many of them who are non-Adivasi but come from a community called the Chetty’s who also have a high prevalence of the disease. There are still thousands more in the villages who have not been screened and are undetected, and we hope to reach them in the upcoming months and years. Genetic counseling is certainly an important part of the program, and we are working on deploying a Center and field-based program that is culturally sensitive and appropriate.

(see report from World Health Organization on Management of Haemoglobin Disorders)
http://www.who.int/genomics/publications/en/

Good luck Hari with your efforts in India….there IS hope.
THE WORLD IS LOOKING….NOW.

Resources in India

Young People “Don’t Give Up”

I’ve heard too many stories about young people (ages 13-30) with Sickle Cell Disease who just give up on life. They believe they've gotten a bad deal from God, that life is unfair, and it's not worth living if it includes suffering.

In result, these young people drink alcohol, smoke cigarettes (and whatever else); and push their bodies beyond its limits. Trying to keep up with their friends, these young people end up in the hospital every time.

I know it’s hard to take care of yourself when you’re suffering and sad. I know that life seems unfair when you’re the only one with Sickle Cell Disease that you know. I know you want to live, like everyone else, and I also know how it feels to be 20 years old (with life ahead of you), and Sickle Cell dims your hopes for a future.

I have to tell you young people “Don’t give up!”

There is hope past the pain. There is life beyond blood transfusions. You’ve gotta just live. Live well and take care of yourself. I promise you there are happy days ahead….live to see them. There is wellness ahead….do your part to have it. There is a future for you....to do all the things you want (or can) do. Live to see it.

I tell you this (young people), from one SC sufferer to another, cry if you want to cry…then wipe your tears. Get up, dust yourself off and get on with your life. Go ahead and dream big dreams, and then do whatever you can to make those dreams come true. God will do the rest.

When you get weak and you fall, get up, dust yourself off and try again, and again, and again, and again, and again. Then, just smile, because you're not going to be defeated by anything…. not even Sickle Cell Disease.