Note: Portions of this section reprinted with permission from Iron Disorders Institute
Iron stored in tissues and organs is toxic and opens one up to infection, cirrhosis, liver failure, heart failure, arthritis, diabetes and a host of cancers. Early diagnosis and removal through phlebotomies is crucial to positive outcomes.
Iron is removed therapeutically from the body in two ways, by blood donation (phlebotomy) or chelation therapy (pharmacological removal). The method of first choice is phlebotomy. as it has the least side effects. Chelation therapy is recommended if the patient can’t tolerate phlebotomies, but can have significant side effects.
A phlebotomy is blood removal; when used as a treatment, it is called therapeutic phlebotomy (TP). The procedure is identical to blood donation, except that TP requires a doctor’s prescription.
Physicians prescribe therapeutic phlebotomies for patients who have too much iron stored in their bodies and whose hemoglobin levels are sufficient to tolerate blood removal.
Each blood donation, or phlebotomy treatment, removes about 500cc of blood and reduces the amount of iron in the body 250 milligrams.
Phlebotomy Phases of Therapy: induction, transition, and maintenance
Induction is when therapy is initiated. Transition is the phase in which iron reduction is taking place to return iron levels to a normal healthy range. Once iron levels reach satisfactory and healthy levels, a patient enters the maintenance phase.
Key to successful iron reduction without generating symptoms or accelerating disease depends on several important factors. The single most important factor is how the doctor writes the order for phlebotomy. Besides this critical first step other important factors are patient compliance with scheduled phlebotomies, diet and behavior modifications and continued education.
With few exceptions Iron a pre-treatment hemoglobin of 12.5g/dL. is recommended. Exceptions will include some females or patients with liver or blood diseases. A fairly standard schedule of phlebotomies during induction is 500 cc’s per week. higher iron levels can call for twice weekly draws.
During this phase iron levels may be erratic or fall predictably. The frequency of phlebotomies may be reduced. Generally when a patient’s serum ferritin is less than 500ng/mL, the frequency of phlebotomy can be slowed to once a month. Each person is different and responds to treatment in a unique way. Much depends on age, the extent of iron saturation, serum ferritin levels, hemoglobin response, one’s physical condition including symptoms, and the speed with which an individual unloads iron. Also, compliance with therapy schedules and care with diet will impact this phase tremendously.
The goal of the transition period is the safely usher the patient from therapeutic phase into the maintenance phase without bottoming out, thus causing anemia and/or iron avidity. During this period, if iron levels are allowed to fall steadily, over time and not too rapidly, the body can reach healthy iron levels and possibly without competition from the genes that are programmed to load iron.
Maintenance: Maintenance patients are those who have reached healthy iron ranges and who can remain within those healthy ranges by donating blood periodically.
A healthy range for ferritin is 25-150ng/mL.
The patient may donate blood routinely as defined by attending physician for optimum quality of health or may have periodic therapeutic phlebotomy by doctor’s order.
As of May 2015 the FDA lifted all variances, waivers and restrictions on the donation of hh blood.
Blood Journal notes that the use of hemochromatosis blood could augment the US national blood supply. The results of a study at the National Institutes of Health demonstrated that recruitment of healthy hemochromatosis donors augments the intramural blood supply significantly. Patients with iron overload are allowed to donate blood more frequently than other volunteer donors. A healthy hemochromatosis patient will donate up to 60 pints of blood in the first year of treatment and, on average, 8-12 pints per year thereafter
In Canada, blood from hemochromatosis patients has been used for transfusion since 1991. In Wales and Sweden, hemochromatosis blood used for transfusion impacted blood shortages.
For years pint after pint, week after week has gone into biohazard bags, classified as hazard waste increasing disposal costs which are passed on to the public.
To recap; HH blood can be used in transfusions. As of May 215 all procedural restrictions were lifted, HH donors are highly motivated “super donors”. Use would reduce blood shortages and eliminate disposal costs.
The crucial next step is getting blood banks, hospitals and the Red Cross to acknowledge and educate staff to new standards of donation acceptance, and actively recruit and advertise for HH donations.
If you are interested in donating your blood, rather than seeing it thrown out, contact your blood bank, hospital or Red Cross directly and demand they accept it for transfusion/educate them and direct them to ironitout.org for information and to answer further questions.
“For years now, we’ve been using blood from the phlebotomies of hemochromatosis patients for transfusions. The patients are pleased to help others in this manner, and the practice has helped greatly in a time of blood shortage.”
-Vincent J. Felitti, MD Southern California Kaiser Permanente San Diego, California
Frequency of donation or therapeutic phlebotomy will depend upon patient’s Personal Health Profile as observed by patient and attending physician: age, weight, response to treatment, symptoms, rate of iron unloading and general physical condition. At anytime during treatment you experience symptoms of heart irregularities or severe abdominal pain or symptoms of anemia, alert your physician immediately.
Symptoms of anemia can often be mistaken by a patient as symptoms of iron overload. Symptoms of anemia or iron overload can include: fatigue, heart arrhythmia, headache, sensitivity to cold, shortness of breath, dizziness and restless legs syndrome. Lab tests are needed to distinguish between the two.
If excess iron has had enough time to damage critical organs, one may never restore these damaged organs to full function. Medications may be required to address these symptoms or disease that has developed. This is why early diagnosis, monitoring of iron loading and treatment are crucial.
During the maintenance phase, and so long as a patient remains symptom-free, the doctor will re-test iron levels about every 3 months. The initial three-month exam upon reaching healthy iron levels will provide a baseline or the first set of numbers after a series of phlebotomies whereby a person’s pattern of un-loading can be established. These numbers are very important to the management of iron balance.
The amount of time required to reach the maintenance phase of treatment will vary. Thereafter, how often that person must have a phlebotomy to keep iron levels in a normal range will depend on compliance. Many hemochromatosis patients abandon therapy once they achieve normal iron levels.
This sets them up for irreversible organ damage and the need for repeat series of therapeutic phlebotomies. For the hemochromatosis patient maintaining iron balance is a life-long effort—timely blood donation is their “drug”.
Serum Ferritin and Phlebotomy Frequency:
In most cases the serum ferritin will drop by about 30ng/mL with each full unit of blood removed. This helps the physician to form an estimate of when the serum ferritin is will be below 1,000ng/mL.
For patients whose initial serum ferritin (SF) is greater than 1,000 ng/mL, phlebotomies can be as frequent as twice a week. The SF should be evaluated every 4-6 weeks until it is lowered below 750 ng/mL. SF above 750 but below 1,000 is still a very high and may require continued weekly phlebotomies. However, issues such as inflammation and alcohol consumption should be taken into consideration when maintaining weekly or adjusting the frequency of blood removal. A patient may need to continue on aggressive removal (at least once a week) if iron levels are dropping at a fairly steady pace. When serum ferritin falls dramatically, highly suspect are inflammation or other factors that influence serum ferritin levels.
Ferritin drops by about 30-50ng/mL with each full unit of blood removed. Ferritin levels can be distorted. Individuals who have liver damage such as cirrhosis will unload iron faster than those without liver damage. Other factors that may skew ferritin include presence of inflammation or infection and alcohol or nicotine consumption (gums, patches, chews). Checking ferritin periodically can protect against undue iron deficiency anemia caused by overbleeding.
Patients undergoing de-ironing are at risk for overbleeding that can take place in the latter part of de-ironing. Bleeding a patient until the hemoglobin is below normal and the patient has mild iron deficiency anemia is no longer the best practice. There is no known benefit to the forced-sustained anemia, a practice that was established nearly three decades ago. Iron Disorders Institute Advisory Board recommends against phlebotomy (with few exceptions) for patients whose hemoglobin is lower than 12.5g/dL.
Prior to treatment, a patient will have a serum ferritin greater than 200ng/mL (females) or 300ng/mL (males) with an accompanying transferrin-iron saturation percentage greater than 45%.
When ferritin is above 1000ng/mL phlebotomy treatments will be aggressive usually as frequent as twice weekly while tolerable and until ferritin drops below 1000ng/mL.
Once serum ferritin is below 500ng/mL, the frequency of treatment may slow down from twice weekly to once a week or even to every other week depending upon the patient’s condition, behavior (eating habits) and ability to unload iron.
During de-ironing: blood contains water and other nutrients. Adequate fluid intake guards against dehydration. Patients are encouraged to take a once-a-day multi-vitamin without iron to support the loss of nutrients. Look for vitamins with minerals (except iron) and B complex.
Otherwise, serum ferritin can be checked periodically such as every 3-6 months to determine the patient’s unloading pattern.
Most blood donation centers allow one donation every eight weeks. If you are a candidate for maintenance, then a periodic blood donation will suffice. If you are found to need treatment, needing more than one extraction in eight weeks, the attending physician will provide you with the necessary order for additional phlebotomies. Your gastroenterologist or hematologist may refer you back to your family physician for the maintenance phase of your therapy. Afterwards, you may resume a normal, happy healthy life with only a small adjustment to your schedule: a life-saving, blood donation every 8 to 10 weeks.
My Iron Levels
Body iron levels are determined by measuring serum ferritin, fasting serum iron and total iron binding capacity (TIBC). A patient’s transferrin saturation percentage (TS%), an important measure of the body’s capability to bind, transport and maintain safe levels of iron. TS% is calculated by dividing serum iron by TIBC multiplied by 100%. TS% is normally about 35-40%.
Serum ferritin is a measure of stored or contained iron. SF varies by age and gender. INSERT Ferritin chart
With the exception of infants and newborns, iron overload is suspect when both serum ferritin and TS% are elevated. There are other conditions of high body iron where only the serum ferritin is elevated. These individuals can benefit from periodic blood donation as elevated serum ferritin is a possible early warning that disease is forming.
Hemoglobin indicates the amount of functional iron, that is, the iron that is carrying oxygen to tissues so that organs can function.
What is the role of hemoglobin in phlebotomy?
Hemoglobin value will determine if a person can tolerate phlebotomy to reduce iron levels. If the hemoglobin value is too low, but iron overload is present, iron chelation therapy is used to reduce excessively high iron levels.
For most hemochromatosis patients the hemoglobin levels will rebound and remain in a normal healthy range following phlebotomies or blood donation. Patients with significant iron overload can tolerate frequent treatments because their hemoglobin will rebound very quickly. As the body manufactures new blood to replace that removed, the new liquid acts like a sponge; it draws the iron out of the body tissue (stored in ferritin) to synthesize hemoglobin in the new blood. Once iron becomes part of hemoglobin it can be safely removed with the next treatment.
Hemoglobin levels prior to blood donation should be 12.5g/dL with few exceptions.
Why do I need a phlebotomy?
Phlebotomy reduces excessive and life-threatening levels of body iron. Some patients diagnosed with hemochromatosis have already built up 5 or 10 grams (or more) of excess iron in their bodies. These patients will require aggressive treatment and sometimes will be phlebotomized (or give blood) as often as once or twice a week. For individuals whose iron levels are only modestly elevated, blood can be simply donated every eight weeks without a prescription.
If serum ferritin is less than 1,000ng/mL at the time of diagnosis, the chances of liver cancer is less than 1%. But if diagnosis occurs after serum ferritin has risen above the 1,000 mark, the risk of liver cancer jumps 20-200 fold depending on the individual.
Pretreatment Lab work:
Before the phlebotomy may be done, hemoglobin or hematocrit must be checked. Usually centers have labs on site where the results are forwarded to the attending nurse or phlebotomist. With few exceptions, if the hemoglobin is too low (below 12.5g/dL) the phlebotomy should be postponed to avoid the risk of over-bleeding and unnecessary iron deficiency. Otherwise a person can become iron avid and set themselves up for increased symptoms and struggles to achieve iron balance.
Where are phlebotomy treatments performed?
Phlebotomies might be performed at a blood donation center, as an outpatient in a hospital or even in a doctor’s office. Your doctor will probably advise places that provide the treatment. Consider convenience of location, cost to do the phlebotomy, and how responsive the center is to your situation.
The Therapeutic Phlebotomy (TP) Procedure Described: The process varies slightly depending on the treatment site; following is a general step by step of what takes place.
After the preliminary tests for hemoglobin and hematocrit are finished, a phlebotomist prepares you for the procedure. The phlebotomist can sometimes be a nurse or doctor, but regardless, this individual is trained to perform needle sticks.
If your hemoglobin-hematocrit are sufficient and in accordance with the doctor’s order, you will be prepared for blood removal. Usually you will stretch out on a comfortable recliner chair. The attendant takes your blood pressure, temperature and heart rate (pulse). These numbers will be recorded on your medical chart for future reference.
An elastic band is tied around the upper part of the arm. This helps the vein to stand up. You may have to squeeze a soft rubber ball or make a fist several times to help the vein remain accessible. The nurse then swabs an iodine-based antiseptic on the vein and all around the area near the vein. This is to disinfect the area where the needle is to be inserted and to make certain no bacteria gets into your system during treatment.
A special needle is then inserted into the vein. You might feel a little pinch, but it lasts only a second. A piece of tape is placed over the needle to keep it stable; you just sit back and relax. The needle used is large enough for blood to flow quickly without clotting. Some patients have a fear of needles or vein access difficulties. There are ways to address these issues but they are not routine and require assistance from the healthcare provider and in some cases involve a surgeon. See Phlebotomy Problems and Issues
While the blood flows, some like to bring a headset with earphones or a good book to read during treatment. While relaxing, the blood flows from the needle, into a tube, and then into the blood bag. The blood bag sits onto a scale that measures the weight of the blood. When 500cc (about one-half pint) of blood is removed the donation or phlebotomy is complete. Some blood centers can offer Double Red Cell Apheresis (DRCA) treatment to hemochromatosis patients. DRCA removes more red blood cells but the amount is less than double.
About 250 mgs of iron are removed with each unit of blood removed. The procedure will take as few as ten minutes or as many as thirty; again, it depends on how hydrated you are, your vein access and the thickness of your blood.
After the Treatment:
After the phlebotomy, the nurse will remove the needle from your arm. You may need to keep the area bandaged or you may need to apply mild pressure if bleeding continues. You should rest for about 20 minutes following therapy. This is a precaution to insure you do not get weak or dizzy. You may be given a snack while you are resting and it is suggested you eat something after your therapy.
Your blood will be discarded unless you educate the treatment center , blood bank or Red Cross branch of the new standards and demand they begin using it for transfusion.
To prepare yourself, be sure that you are getting adequate fluids. A daily multi-vitamin without iron is recommended because with treatment iron is removed but so are other nutrients. Be careful about water consumption. Gulping great amounts of water prior to therapy is not wise; you may actually cause yourself to become water intoxicated, a serious condition that can result in death. Hydrate yourself slowly over days; do not wait until the last moment.
Eat more fiber, refrain from slow-cooking in an iron skillet, and limit Vitamin C to 200 milligrams per dose. Fiber impedes iron absorption while vitamin C enhances iron absorption. Drinking tea with meals is helpful as the tannin in tea also impedes iron absorption. Iron absorption is not known to be affected by caffeine. Nicotine will increase iron levels; nicotine gums and tobacco products should be avoided. Smoking should be discontinued; tobacco is rich in iron and inhalation of this smoke directly or second-hand is harmful to your lungs. Hydration can be accomplished by increasing consumption of fresh fruits and vegetables. There is no need for concern about the vitamin C or A content of fresh fruits or vegetables, although the less sugary choices are best. Fresh, whole fruits and vegetables provide antioxidant protection against harmful iron triggered free radical activity. It is this action that is believed to do the most damage to vital organs.
Regular, intense exercise or taking aspirin daily will cause some blood loss and thus iron loss. However, you should consult your doctor before incorporating any of these practices into your daily routine. Intense exercise (or marching) can result in a condition called “March Hemoglobinuria”, which causes the premature breakdown and destruction of red blood cells that results in anemia. Excessive aerobic exercise can create an environment for increased oxidative stress (free radical activity). Low impact exercise such as walking appears to offer the best benefit with the fewest injuries or side effects. Try to walk 10,000 steps a day if you can. Pedometers can record the number of steps that you take. You might be surprised to learn that most Americans take an average of only 2,500 steps a day!
WARNING: Aspirin can be dangerous for youths with fever and it can interact with some drugs. Your pharmacist may be able to provide you with drug interaction advice; if not, contact your doctor.
Problems and Issues:
Fear of needles is a powerful deterrent to iron reduction. It is a common complaint expressed by men and women alike. There are not too many alternatives for such a patient, but there are some steps that can help. Taking a light sedative (prescribed by the doctor) can relax a person sufficiently to insert the needle. Having a support person present to distract a nervous patient can be helpful. Listening to music through a headset can also be distracting. Double Red Cell Apheresis (DRCA) is a good choice for many of these patients. One stick allows nearly double the iron extraction saving the person from the anguish or anxiety of twice a week punctures.
Numbing agents such as Emla Cream can help in some cases. These creams temporarily numb the skin allowing the needle to be inserted. Check with your doctor about a prescription but first see if the blood center has any objections to the use of such a product.
Vein access is another issue. When patients require multiple and numerous blood extractions sometimes a chest port is an option to consider. Otherwise, veins in the legs can be used to remove blood.
Since scar tissue can build up significantly over time, a person whose arms are scarred might consider wearing a medical ID bracelet. This can distinguish them from individuals who may be chronic drug abusers.