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Hamman-Rich Syndrome: Facts, Symptoms, and Treatment

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Acute Interstitial Pneumonia (Hamman-Rich Syndrome): Facts, Symptoms, and Treatm Olesya Shelomova/fotolia

What is Hamman-Rich Syndrome

Hamman-Rich Syndrome is also known as acute interstitial pneumonia. It is an uncommon type of pneumonia for which researchers have not yet found a cause (idiopathic). It affects otherwise healthy individuals. The underlying condition may worsen without symptoms appearing for a long period of time, but when the symptoms do manifest they come on very suddenly and "acutely".

The condition received its name from the doctors who discovered the disease in 1939, Drs. Hamman and Rich.

Acute interstitial pneumonia has similar symptoms to acute respiratory distress syndrome, and may be misdiagnosed as such.

Pneumonia results when tissues of the lungs become inflamed in reaction to infection. Interstitial pneumonia, by comparison, is a long-term condition that affects the connective tissue of the lungs. The inflammation is caused by the build up of white blood cells and plasma in the alveoli (the tiny sac that facilitate the carbon dioxide/oxygen exchange in the bloodstream). With interstitial pneumonia, the inflammation also extends into the bronchioles - small airways that branch off into the lungs.

If the inflammation lasts long enough, the fluid hardens into scar tissue (fibrosis). If there is enough scar tissue, over time alveoli will be destroyed and the space filled with cysts. Over time, the bronchi and the walls of the bronchi widen, or are destroyed resulting in the lungs shrinking.

Sixty percent of those with acute interstitial pneumonia die within six months of the appearance of symptoms.

Symptoms of Hamman-Rich Syndrome

As stated earlier, symptoms of Hamman-Rich Syndrome or acute interstitial pneumonia are similar to those observed in acute respiratory distress syndrome, which are:

- shortness of breath
- rapid, shallow breathing
- crackling or wheezing sounds in the lungs
- cyanosis (blue tinge) to the skin
- heart and brain function issues (rapid heart rate, difficulty concentrating, forgetfulness) because of long-term reduced oxygen supply

Diagnosis and Treatment of Acute Interstitial Pneumonia

Diagnosis of acute interstitial pneumonia is usually confirmed through CT scans, lung biopsy, and tests that measure pulmonary function.

The goal of treatment is to prevent complete respiratory failure and keep the patient alive and comfortable until the condition resolves. Treatment often involves use of a ventilator in the event of respiratory failure, and administration of corticosteroids (although it is not known how effective these medications are).

For those who survive, lung function will improve over time, but the condition may return.

Sources: www.uptodate.com; www.wrongdiagnosis.com; www.medfriendly.com; www.merck.com

Add a Comment72 Comments

HERWriter (reply to Anonymous)

Thank you for sharing your story. So sorry for your loss. That's the hard part about this disease; it's often too late when they do figure out what's actually wrong. Hopefully, as research continues into this disease, it will be easier for doctors to distinguish between Hamman-Rich and pneumonia so appropriate treatment can be started earlier.

November 29, 2011 - 1:56pm
HERWriter

I hope so, too, Joshua. So sorry for your loss. My heart breaks for both you and your children. Obviously, this is a tough time of year for you.

August 19, 2011 - 8:45am
EmpowHER Guest
Anonymous

My wife passed from this disease last year Oct 2010, at the age of 36 years old leaving behind a 17yr son, 16 old daughter, 2 (8yr olds son/daughter) and a 4 yr old daughter. It started out from a small rash that developed into a Christmas tree pattern on her back, which bought on shortness of breath an caused her fingers to become ruff and hard to grab a hold of things. After going from hospital to hospital before actually finding out what she really had, we were getting several responses that she had psoriasis which it was not. Finally after getting upset with the responses that we were given, we finally decided to go back to the first hospital an was not going to leave without a more clear explanation of what was going on with her. After several test and being in the hospital for 2 months, my wife passed in the initial hospital that informed us that it was a skin condition. I truly hope that the hospitals and doctors find a better way to treat an cure this disease that attacks healthy individuals. I love an miss my wife (Simone Croxton) your husband Joshua L. Dixon.

August 19, 2011 - 7:41am
EmpowHER Guest
Anonymous

Darlene. Thank you for the information you've shared as well as those who have dealt with this type of pneumonia. I do wish there were more research on it. I lost my mother to Hamman Rich Syndrome and I'm devastated. What we thought was just a bout of bronchitis coming on ( mom was susceptible to this as well as colds), was much worse than we could have imagined.

We begged mom to see her doctor, being mom, she said, I'm fine! Well, finally on 09.10.10, she went and the doctor would not let her leave the office after checking my mother out. Mom went to hospital via ambulance. We waited for 2 hours in the emergency room with her, the doctor explained her xrays showed she had double pneumonia, mom was admitted and we were led to believe she would be home by late the following week. After 3 days the doctors wanted mom moved to icu. We were told it was to keep a better eye on mom since the treatment wasn't going as planned. A pulmonary specialist ordered a culture that was sent to Mayo Clinic. From there, the daily xrays showed no improvement. The test results came back and it was the Specialist had suspected, Hamman Rich Syndrome. Though tough to fight, we had hope.

The lung doctor wanted to place mom on a ventilator to give her more oxygen and bring up her levels. After a day, the doctor decided mom should be placed in an induced coma. After two days of this, her white blood cell count skyrocketed, she had a fever off and on during this time, Mom also had a mild heart attack later in the week. All during this time they did take good care of mom. After that, the second week was hope, prayer and taking every bit of advice we could get. On 09.24.10, my family and I were told that since they had taken mom off the drug that induced her coma 4 days before, she wasn't likely to wake up. Also, her kidney's were failing. I refused to simply let her go. I wasn't going to even consider it until the pulmonary specialist came back (she had been gone for a week). This doctor called every morning and explained how mom was doing. She did the very best she could for my mom. When she did call on 09.27.10, she explained that after seeing mom's xray's with a fresh eye, the xray's were a thousand times worse. She also explained that mom wasn't going to wake up and when she was taken off the ventilator, she would pass away.

I asked the Specialist if there's any information on how my mother could have contracted this. She told me there wasn't much information available. She's right, I went all over the web looking for something, anything. There's not much out there.

This is the absolute worst thing I've ever had to do. My family and I were with mom when we let her go. Every day I ask, why didn't she listen? Why didn't she go to see her doctor sooner? I know that second guessing is natural. But I miss her so very much and only wish that there was something available that could have saved her life. If you know anyone who's ill and has those symptoms that seem mild but they're not quite sure, don't wait. See a doctor!

Thanks for taking the time to read my information.

December 6, 2010 - 8:28pm
EmpowHER Guest
Anonymous (reply to Anonymous)

I just lost my mom to acute interstitial pneumonitis. She went to the hospital with what she thought was bronchitis and was first told it was double pneumonia. Within a week she was in the ICU and then had a lung biopsy. They put her on a ventilator and tried steroids but they didn't work for her. Within 18 day of her first going to the hospital, we were told there was no hope and that we needed to make a decision about taking her off the ventilator. My mom was a beautiful, active 76 year old and that makes it so hard to believe. It is a devastating loss. I had never even heard of AIP and it took my mom so fast with no warning. There needs to be more research for a cure.

January 2, 2012 - 7:55pm
HERWriter (reply to Anonymous)

So sorry for your loss and you're right more research needs to be done.

Even finding information to include in the article was challenging. I had to really dig for it. There really isn't a whole lot out there because I think it catches doctors by surprise.

It should be a standard test now for otherwise healthy people who come in with pneumonia- or bronchitis-like symptoms.

Research does continue. Unfortunately, these things often take time.

January 4, 2012 - 7:30pm
HERWriter (reply to Anonymous)

Thank you for sharing your story. So sorry that you've lost your mother. It's a natural part of the grieving process to second guess, but you did everything humanly possible. There isn't a clear definition of how Hamman Rich develops compared to pneumonia or any similar lung condition and because the symptoms present like bronchitis or pneumonia it is hard for doctors to narrow down until the "traditional" medications don't work.

I'm glad I posted this article, too. It seems this information was needed and I'm glad it has been able to help and provide families with these details.

Darlene

December 7, 2010 - 11:32am
EmpowHER Guest
Anonymous

Six weeks ago I became ill while in my hotel room. The next day I was so sick I called 911. I am currently still in the hospital. I was in a drug induced coma for more than 3 weeks. They tell me that it is a miracle that I am still here. If anyone here is suffering from this condition or has a loved one that has it I would love to talk to you. I can't find a lot of info on this and I would like to hear other peoples stories. Its so crazy to me that something like this could happen. I am only 29 years old. My e-mail is [email protected]

November 4, 2010 - 7:18pm

An update on my mom, the dr's have her on prednisone and she has been responding to treatment. She is still on oxygen 24 hours a day. The dr says she is still not out of the woods, but they have been real pleased with her progress. She was in the hospital for over a month and in ICU for a week, has been home almost two weeks. She doesn't know how serious this is, she doesn't want to know. She is living her life the best that she can and we are hopeful about the future. At her last dr apppointment they said she still had the germ, but her lungs were sounding much better. She has pulmonary fibrosis and we are unsure of how much damage this pneumonia has done to her lungs. The dr's had done a lung biopsy on her, sent it to the Mayo clinic and it came back as Hamman Rich and the dr's immediately changed up all her medications as from what I understand antibiotic's won't touch this disease. The lung biopsy was hard on her physically but in the long run we believe that it saved her life. What is scary to me about this disease is the fact that there is so little known about it, I have talked to several dr's and nurse's who have never even heard of it. I am sending prayers for all those who have been affected by this disease.

October 12, 2010 - 7:33am
HERWriter (reply to Poppie)

Thanks for the update, Poppie.

I was astonished at how little information I found to write the article.

Darlene

October 12, 2010 - 12:20pm
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We value and respect our HERWriters' experiences, but everyone is different. Many of our writers are speaking from personal experience, and what's worked for them may not work for you. Their articles are not a substitute for medical advice, although we hope you can gain knowledge from their insight.

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