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Idiopathic Pulmonary Hemosiderosis
Tuesday, 19 October 2004
Monday, 29 November 2004

What

Idiopathic pulmonary hemosiderosis (IPH) is a rare pediatric disease characterized by repeated episodes of bleeding into the lungs. The many episodes of bleeding can make your child anemic. The body is able to remove most of the blood from the lungs, but a large amount of iron is often left behind. Over time, this iron, called hemosiderin, can cause permanent damage to the lungs.

Who

Idiopathic pulmonary hemosiderosis usually affects children ages one to seven, but can occur from birth up to early adulthood. The disease is very rare and happens in about one in a million children. Rarely there have been reports of IPH occurring in families, but this has not been confirmed. In children under ten years of age, boys and girls are equally affected. For children older than age ten, IPH is twice as common in boys than girls. In Japan, it seems that girls are affected two times as often as boys.

Signs and Symptoms

The majority of the signs and symptoms occur after chronic blood loss or blood in the lungs. The blood in the lungs acts like an irritant and causes children to have cough, difficulty breathing, wheezing, or turn blue from not getting enough oxygen. This makes many people think the child may have pneumonia. The children will either cough up the blood in their lungs or they will swallow it and eventually throw it up or pass it in their stools. When the lung bleeding starts, children will often get a fever and a fast heartbeat. The amount of bleeding into the lungs is different for every child. As time goes on, the repeated blood loss causes an anemia, and the child will start to look pale. They may also get tired more easily and be short of breath. The iron left over in the lungs is not available for the body to make more red blood cells, so the anemia lasts for a longer period of time. Some children with IPH will not grow as well as other children.

Possible Causes

Unfortunately, the cause of IPH is still unknown. In general, bleeding in the lungs could be caused by infections, antibodies to lung tissue, milk allergies or heart disease. In children with idiopathic pulmonary hemosiderosis, however, none of these factors are present. Some researchers speculate that certain environmental toxins or exposures, like molds, could cause IPH. This was found in one published study, but others have criticized the design of this study. There is currently no consensus about whether molds are responsible.

Diagnosis

First, all other causes of bleeding into the lung must be excluded. Blood tests may show an anemia with low iron levels. In IPH, all antibody tests will be negative. A chest x-ray during an episode will show patchy areas of involvement in both lungs that usually improve completely in two weeks. A CT scan of the chest is another option to get a better idea if the lung damage is due to bleeding or infection. In a technetium scan, red blood cells from the child can be marked and then checked to see if they end up in the lungs, demonstrating bleeding into the pulmonary spaces. Flexible bronchoscopy is a way to collect fluid from the lungs. After a child has been given medicine to help them sleep, a flexible tube with a camera will be inserted down the windpipe and into the lungs to collect fluid. The fluid is then checked for bacteria, viruses and fungi that could be causing an infection. In IPH, the fluid will show cells that are full of hemosiderin, but no other signs of infection. In some cases, it may be necessary to biopsy a tiny part of the lung for examination under the microscope to help make the diagnosis.

Treatment

A pediatric pulmonologist should be involved in your child’s care. Therapies are directed towards managing the consequences of the bleeding in the lungs. Blood transfusions may be necessary if there is a large amount of bleeding. The blood in the lungs can make breathing more difficult and your child may need oxygen. If it becomes too difficult for a child to breath on their own with oxygen, a breathing tube can be inserted that will help them breathe until their lungs have had a chance to recover and get stronger. In some children, steroids are useful in controlling the immediate bleeding phase, although this is not true for every child. If your child does not respond to steroids then other medications that suppress the immune system can be used. Any time a child is on these other medications, their immune system will not work as well as it should and they are at a higher risk of not being able to fight off viruses, fungi or bacterial infections. If a child is on steroids for a long time, their growth and blood pressure may become affected.

Prognosis

Idiopathic pulmonary hemosiderosis is a rare disease, so it is difficult to predict how well each child will do. Children who do not have a large amount of pulmonary bleeding can do well, while others with large amounts of bleeding may only live for a few years. In a small study of 30 children with IPH, it was shown that girls do a little better than boys and that the older a child is at age at onset of disease, the better they usually do. Even if there is a severe onset of disease, this does not mean that your child cannot do well long term with medication and supportive care.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Unfortunately at the present moment, there are almost no websites or support groups with information on idiopathic pulmonary hemosiderosis.

http://emedicine.com/ped/topic970.htm
A more compreensive synopsis of idiopathic pulmonary hemosiderosis along with a medication summary.

Google Search for Idiopathic Pulmonary Hemosiderosis

References and Sources

www.emedicine.com/ped/topic970.htm Update: Pulmonary Hemorrhage/Hemosiderosis Amoung Infants-Cleveland, Ohio 1993-1996. MMWR. 2000; 49:180-4. Behrman, R.E., R.M. Kliegman and H.B. Jenson. Pulmonary Hemosiderosis (Pulmonary Hemorrhage). Nelson Textbook of Pediatrics. 2000, p 1296.