Treatment is a 2 part process:

First, the goal behind any treatment is to stop or slow the production of the amyloid. If the production is not stopped, whichever body system, soft tissue or organ(s) are being effected by amyloid deposits will continue to deteriorate. This is why it is important to get a conclusive and accurate EVALUATION/diagnosis and treatment plan as soon as possible.

Second, the affected system, tissue or organ(s) must be dealt with. The goal is to restore as much function as possible. This is typically done with medication, diet, exercise and in some cases an organ transplant or surgery. It is the diseased or damaged organ(s) that put the patient at the most risk.

Financial Aid for Amyloidosis Treatment

If you’re having trouble paying for treatment, the following strategies may help.

• Enroll in a clinical trial - Drug makers or the National Cancer Institute often provide free drugs to participants of clinical trials. Check http://www.cancer.gov/ or http://www.clinicaltrials.gov/ or contact patient advocate groups or your doctor for information.
• Contact the Patient Advocate Foundation - The, foundation, at http://www.patientadvocate.org/ helps insured patients get coverage for cancer drugs. The. Group’s new assistance program, http://www.copays.org/ , offers up to $2,.500 to help meet co-payments, premiums. and out-of-pocket expenses.
• Find out if you are eligible for Medicare - Cancer patients disabled by their disease may quantify.  A lottery program offers help paying for some oral medications. Go to http://www.medicare.gov/ and click on Medicare Replacement Drug Demonstration.
• Contact the manufacturer of your cancer medication - Many offer free medicine to the poor or uninsured. For a list of companies with assistance .programs, go to http://www.phrma.org/ or http://www.helpingpatients.org/.  http://www.themedicineprogram.com/ helps patients find out if they qualify for various programs; $5 fee per medication.
• Tell your' doctor - Oncologists can steer patients to available assistance programs and clinical trials.  They may be wiling to contact drug companies on a patient's behalf.
• Go to your hospital's or cancer center's patient-services department - Most have someone on staff to help patients work with insurance companies to get coverage. They frequently know of other financial-assistance programs.
• Contact a patient advocacy group - Many know of financial assistance programs for patients.

 

7. What does Amyloidosis with heart involvement mean?

It means the patient has "restrictive cardiomyopathy" which is rare in the United States and most other industrial nations. In this disease, the walls of the ventricles stiffen and lose their flexibility due to infiltration by abnormal tissue such as amyloid deposits. As a result, the heart cannot fill adequately with blood and eventually loses its ability to pump properly.

In the United States, restrictive cardiomyopathy is most commonly related to the following: amyloidosis, in which abnormal protein fibers (amyloid) accumulate in the heart's muscle; sarcoidosis, an inflammatory disease that causes the formation of small lumps in organs; and hemochromatosis, an iron overload of the body, usually due to a genetic disease.

Typical signs of the condition include symptoms of congestive heart failure: weakness, fatigue, and breathlessness. Swelling of the legs, caused by fluid retention, occurs in a significant number of patients. Other symptoms include nausea, bloating, and poor appetite, probably because of the retention of fluid around the liver, stomach, and intestines.

NT-proBNP Blood Test for AL Amyloidosis Heart Involvement

4/28/2003

In a Clinical Investigation Report published by the American Heart Assoc., researchers at the University Hospital "IRCCS Policlinico San Matteo"–University of Pavia, Italy and the Department of Experimental and Clinical Biomedical Sciences, University of Insubria, Varese, Italy, have concluded NT-proBNP appeared to be the most sensitive determinant in cardiac AL amyloidosis. It adds prognostic information for newly diagnosed patients and can be useful in designing therapeutic strategies and monitoring response. NT-proBNP is a sensitive marker of heart toxicity caused by amyloidogenic light chains.

For more information link to the American Heart Assoc:

 

Generally, Amyloidosis is a very uncommon condition. And not all forms of amyloidosis are hereditary.Typically, families know when they have this because of similar symptoms and causes of death among blood relatives usually showing up in their mid-forties.  However, new variants are still being discovered.  So family history is a key indicator.  If you know the country of origin of your family member who has/had amyloidosis, especially if they are Swedish, Japanese, Portuguese or Italian, there are research centers in those countries.

There are 2 tests for the hereditary version; DNA testing & blood tests.  The DNA test would indicate the potential to develop the disease (i.e. carrying the gene).  The blood test would look for the abnormal protein, indicating the disease is active.  So, prior to the disease onset, a DNA test would show if you are at risk. After onset, both the DNA & blood tests are used to confirm and properly "type" the specific variation of familial amyloidosis.

As to whether you should get tested, this is a very personal decision.  If you suspect amyloidosis due to your family history, then you should start by getting your relative’s medical records.  Then your local doctor, consulting with an amyloidosis reference lab, can conduct the necessary blood & DNA tests on blood relatives to determine if you have the amyloid protein variant.  "Transthyretin", is the most common type of hereditary amyloid and is produced in the liver. Treatment would potentially include a liver transplant.

Reference labs in N. America are at Boston Univ., Memorial Sloan-Kettering in New York City, the Mayo Clinic in Rochester Minnesota and Indiana University School of Medicine Amyloid Research Group Department of Pathology and Laboratory Medicine.  Outside the US, labs are at the Royal Free Hospital near London, University di Pavia Hospital in Italy, and Groningen University Hospital in the Netherlands have established amyloidosis practices.  Contact details are on this web site under MEDICAL HELP. 

Keep in mind, Amyloidosis is sufficiently uncommon that primary care physicians do not recognize when or how they must test for this disease.  Seek the most experienced doctor you can.  Privacy is a major issue and for those on private medical insurance, never let your insurance lapse after discovering you are at risk for this disease.

People who are considering donating their body to science generally are advised to contact their nearest university medical school, to make transport of the body easiest.  Many universities bear all the costs associated with body donation, as long as the body is nearby. Other institutions might require family members to arrange for transport.  In most cases, the institution promises to use the body for teaching purposes or medical research. But that can include a variety of uses; so potential donors should ask for a detailed explanation of what types of research might be performed.  Although the institution typically decides how and where a body is used, sometimes potential donors and family members can specify the ways they don’t want the body used or direct the donation to a specific researcher.

Many cadavers donated to universities are used for medical students, who dissect every inch of the body.  Some questions potential donors should ask are:

• What steps are taken to preserve the dignity of the deceased, such as wrapping the body and keeping the head covered?  Many schools have their own rituals that students use to show respect, including memorial services and "blessing" ceremonies.
• What happens to a body if a university doesn’t need it, and whether auditing systems are in place to track how the body is used?
• Does the university itself distribute the bodies to other researchers and institutions or do they employ a private body broker, which is less accountable to a donor’s family?
• What happens to a body when the research is complete?  Many medical schools cremate the bodies. Some hold memorial services, and family members may be allowed to attend. A few universities promise to return remains to family members.

People who work in “willed-body programs” say the most important thing is for potential donors or family members to make all of their concerns known.  For example, in the case of amyloidosis patients, to will their body provides the medical student with first hand knowledge of the disease.  Hopefully, experiencing amyloidosis now will aid the student in diagnosing and treating a patient in the future when that student when practicing medicine.  Also, perhaps it will attract another “mind” to do research in human amyloidosis that we so desperately need.

For more information contact Anatomical Gift Association Of Illinois. 2240 West Fillmore Street,Chicago,Illinois 60612 [312-733-5283] [1-800-734-5283 ] http://www.anatomical-gift.org/