Sarcoidosis

Sarcoidosis? What the hell is that? This is what my husband lives with every day. I realized I have never blogged about it and yet it's something that lingers in our home every day, some days more than others. Here's a medical entry regarding the disease followed by our personal story. Sarcoidosis http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001140/ Last reviewed: June 2, 2011. Sarcoidosis is a disease in which inflammation occurs in the lymph nodes, lungs, liver, eyes, skin, or other tissues. Causes, incidence, and risk factors The cause of the disease is unknown. In sarcoidosis, tiny clumps of abnormal tissue (granulomas) form in certain organs of the body. Granulomas are clusters of immune cells. The disease can affect almost any organ of the body, but it most commonly affects the lungs. Possible causes of sarcoidosis include: • Extreme immune response to infection • High sensitivity to environmental factors • Genetic factors The condition is more common in African Americans than Caucasians, especially in Caucasians of Scandinavian heritage. Females are usually affected more often than males. The disease typically begins between ages 20 and 40. Sarcoidosis is very rare in young children. A person with a close blood relative who has sarcoidosis is nearly five times as likely to develop the condition. Symptoms There may be no symptoms. When symptoms occur, they can involve almost any body part or organ system in your body. Almost all patients have lung or chest symptoms: • Chest pain (most often behind your breast bone) • Dry cough • Shortness of breath Symptoms of general discomfort or uneasiness often occur: • Fatigue (one of the most common symptoms in children) • Fever • Joint achiness or pain (arthralgia) • Overall feeling of discomfort, illness, or lack of well-being • Weight loss (one of the most common symptoms in children) Skin symptoms: • Hair loss • Raised, red, firm skin sores (erythema nodosum), almost always on the front part of the lower legs • Rash • Scars that become raised or inflamed Nervous system symptoms may include: • Headache • Seizures • Weakness on one side of the face Eye symptoms include: • Burning • Discharge from the eye • Dry eyes • Itching • Pain • Vision loss Other symptoms of this disease: • Dry mouth • Fainting spells if the heart is involved • Nosebleed • Swelling in the upper part of the abdomen Signs and tests A physical exam may show the following: • Abnormal breath sounds (such as rales) • Enlarged liver • Enlarged lymph glands • Enlarged spleen • Rash Often the disease is found in patients with visible physical signs who have an abnormal chest x-ray. Different imaging tests may help diagnose sarcoidosis: • Chest x-ray to see if the lungs are involved or lymph nodes are enlarged • CT scan of the chest • Lung gallium scan To diagnose this condition, a biopsy is needed. Biopsy of the lung using bronchoscopy is usually done. Biopsies of other body tissues may also be done. This disease may affect the results of the following lab tests: • Calcium levels (urine, ionized, serum) • CBC • Immunoelectrophoresis - serum • Liver function tests • Quantitative immunoglobulins (nephelometry) • Serum phosphorus Treatment Sarcoidosis symptoms will often get better on their own slowly without treatment. Patients whose eyes, heart, nervous system, or lungs are involved may need to be treated with corticosteroids (prednisone or methylprednisolone). Therapy may continue for 1 or 2 years. The most severely affected patients may need lifelong therapy. Drugs that suppress the immune system (immunosuppressive medicines) are sometimes also needed: • The drug used most often is methotrexate, but azathioprine and cyclophosphamide are also sometimes recommended. • Hydroxychloroquine is useful for skin sarcoidosis. Rarely, some people with end-stage heart or lung damage may need an organ transplant. Expectations (prognosis) Many people with sarcoidosis are not seriously ill, and the disease may get better without treatment. About 30 - 50% of cases get better without treatment in 3 years. About 20% of people whose lungs are involved will develop lung damage. The overall death rate from sarcoidosis is less than 5%. Causes of death include: • Bleeding from the lung tissue • Heart damage, leading to heart failure and abnormal heart rhythms • Lung scarring (pulmonary fibrosis) Complications • Fungal lung infections (aspergilloma) • Glaucoma and blindness from uveitis (rare) • Kidney stones from high calcium levels in blood or urine • Osteoporosis and other complications of taking corticosteroids for long periods of time. • Pulmonary hypertension In the spring and summer of 2008, we actively pursued having a third child. I took chlomid and we hoped it would happen. After three years of infertility with Gabriel, we were unsure if it would happen without assistance. In the early fall, Andrew developed an ache in his lower back and I urged him to go to the doctor. The doctor had an xray done and they found a large mass in his lung. He was sent to a pulminologist where we were told that it could be a number of things - a fungal infection, cancer, and more. At that point, I quit taking the chlmoid and decided to have my tubes tied in January or convince Andrew to have a vasectomy. I didn't want to risk a rough pregnancy and deal with any health issues with my husband on top of it. The thought of lung cancer shattered my world but little did I know how bad things would get for us. My parents drove from Wisconsin to help us when Andrew had a bronchoscopy. I was able to go with him and I have to tell you that the numbing procedure prior to the bronchoscopy is horrible. It needs to be tweaked. The test was inconclusive, leading Andrew to more tests and both of us to more questions. Nothing was ruled out. When the pulmonologist gave us the news, he wanted to admit Andrew to the hospital immediately in order to have an open lung biopsy performed. We were able to schedule the surgery after the weekend so Andrew could figure out everything at work first. Andrew had an open lung biopsy performed. If I thought the numbing procedure was horrible, this was disgusting. He had a tube coming out of his side that was full of blood and he spent three days in the hospital recovering. The good news was that the surgeon got good tissue samples and didn't think it was cancer. He didn't think it was a fungal infection either. So what the hell was it? This seemed like GREAT news! I was relieved. Time passed and the sample was sent to Mayo to be diagnosed. I found out I was pregnant with #3! The end of November, I surprised Andrew with the news. (I don't know which of us was more surprised)

We later learned that Andrew had sarcoidosis. We were happy that Andrew didn't have cancer and couldn't understand why the pulmonolgist still seemed bleak and serious when he delivered the news. Anything is better than the "c" word. Andrew started chemotherapy and prednisone in January. We were a mess. I was very ill from my pregnancy and Andrew felt like crap. The medications took a toll on him but somehow, with the help of our Sioux City friends, we made it through this difficult time. The boys were 2 and 4 years old and VERY active. Andrew still went to work despite the side effects of the drugs.

Andrew was sent to Omaha, one of three places that performed a specific cardiac MRI. The results from the MRI were not good. Andrew was told that he had sarcoid in his heart. This was devastating news - he was given 2-5 years before he would have a massive heart attack. If he survived, he would have a defib put in and then wait on the heart transplant list. I couldn't believe this was our future. I lived in constant fear. If he took too long getting home from work, I would get a sick feeling in the pit of my stomach. If he didn't rouse from sleep fast enough, I would panic. How would I raise three young children by myself? We had to get our financial situation in order and make a big decision about our third baby. Should we give another couple the greatest gift we knew - the gift of becoming parents? It was a heart wrenching and private decision process for us. In the meantime, I went in for my 24 week appointment. The baby didn't cooperate at the 20 week ultrasound so we were given another quick peak. A girl! A girl?? We are girl makers??! As my pregnancy progressed, I was miserable. I was on bed rest and had a Zofran pump that meant shoving a needle into my leg several times a day just so I could function through the day and avoid hospitalization. I did not want to go through another round of mag in the hospital - that was pure hell. It was then that I knew I couldn't hand my baby to another person. We would find a way to make this work. Fast forward through the NICU ... autism... more tests and procedures for our family... to a visit in November 2010 to Mayo clinic in Rochester, MN. Mayo is another place that performs the cardiac MRI. We all traveled to Rochester and spent a few days there for Gabriel's 6th birthday.

You know those two years I spent panicking about my husband's heart? It wasn't necessary. The orignial MRI was read incorrectly and Andrew does NOT have sarcoid in his heart. Not now.. not yet... HOORAY! Fantastic news! We know that it could still go to his heart one day but it's not there now. I might have punched the radiologist who misread his first MRI if that person was in front of me. I've come to hate medical mistakes with a passion. Sarcoid today means no medication. It means less panic for me. Andrew has days when his joints hurt. He can't walk up stairs on those days. His knees give out. He has days where his lung hurts so bad he can't move and wonders if he should go to the hospital. He gets weird rashes that won't leave his body. It's a huge hurdle filled with daily challenges but we are grateful that we don't have to worry about cardiac sarcoidosis at this time. Thank you to those of you you continue to pray for Andrew. For those of you who ask about his disease and for your continued support, we are grateful.