Pulmonary Veno Occlusive Disease

We wanted to take a moment to give you the update on Cynthia! They are getting ready to go up this week for the proceedure and where they are very excited they are also very nervouse and worried about the expense. She will have to stay up north for around 6 months so they are having to pay for a place there as well as maintain their Lake Worth Home not to mention the expense going back and forth for her family. If you would like to contribute to their fund please contact us at nhnproject@gmail.com or rolohlakeworth@gmail.com for the information. Please keep Cynthia and her whole family in your prayers over the next 6 months and together we will make a difference in this very tough journey! As I stopped by to visit a neighbor who had called me yesterday it was quite a shock and a surprise. Cynthia Mehl answered the door of her very charming home while the trucks outside were swapping out her Oxygen Tanks.

What I expected was to be greeted by someone who would be frail and weak but was surprised to be greeted by a very joyful, upbeat loving individual who immediately invited me into her home. As I walked in the first thing you can't miss is the shear beauty and charm of her home...and then the 5 or 6, 3 feet in diameter, 3 to 4 feet tall canisters of Oxygen. Lined up and ready to be used.

See Cynthia was diagnosed with Terminal Lung Cancer in 2003 and survived. Then was diagnosed with Pulmonary Hypertension in 2004 and kept on going. Then in 2009 she was diagnosed with Pulmonary Veno Occlusive Disease (see below for details or click this article to read more about PVOD.)

Cynthia looks just like you and me and had all the energy and joy of a normal person except for the fact that she has to be on Oxygen 24/7, What will help her? A Double Lung Transplant at Duke Medical Center.

Why do you ask that I want to write about this? One she is my neighbor and now a dear friend, two we in ROLOH as in many of our neighborhoods care about each other and third my own father passed away from Lung Cancer. Cynthia has to go through a very serious procedure, Double Lung Transplant....Recovery will be 4-6 Months if not longer depending on complications...

What is needed to help Cynthia? Prayers, good wishes and happy thoughts and knowing that people care about her and this rare condition. Where Cynthia and her husband Gordon made wise decisions with their health insurance that still does not cover the cost of traveling back and forth, hotel stays, gas cost and many other ancillary expenses that arise in a situation like this. Not to mention they still need to maintain there very lovely home in order for Cynthia to come back and enjoy for many years to come.

Cynthia and Gordon have set up an account at Wells Fargo and donations can be made direct to Cynthia's Wells Fargo Account If you would like to make a donation please email nhnproject@gmail.com and I will forward you the flyer to complete and drop off at a Wells Fargo...She should be heading to Duke Medical in September and then she will have a long road ahead before her from that point. Below is more information on PVOD and remember in Giving in tough times even the smallest of donations are a blessing and lets not discount the power of Prayer!

Sincerely,
Robert Waples

Pulmonary veno-occlusive disease is an extremely rare form of high blood pressure in the lung area.

See also: Primary pulmonary hypertension

Causes:
In most cases, the cause of pulmonary veno-occlusive disease is unknown. The condition may be related to a viral infection. It may occur as a complication of certain diseases such as lupus, or as a complication of leukemia, lymphoma, chemotherapy, or bone marrow transplantation.

The disorder is most common among children and young adults. As the disease gets worse, it causes narrowed pulmonary veins, pulmonary artery hypertension, and congestion and swelling of the lungs.

Symptoms:
•Shortness of breath
•Fatigue on exertion
•Fainting
•Coughing up blood
•Difficulty breathing while lying flat

Exams and Tests:
The doctor will perform a physical exam.

The exam may reveal:
•High blood pressure in the veins of the lungs
•Increased pressure in the neck veins
•Fingernail clubbing
•Bluish coloration of the skin due to lack of oxygen (cyanosis)
•Swelling in the legs

Your doctor may hear abnormal heart sounds when listening to the chest and lungs with a stethoscope.

The following tests may be done:
•Arterial blood gases
•Chest x-ray
•Chest CT
•Cardiac catheterization
•Pulmonary angiogram
•Echocardiogram
•Lung biopsy

Treatment:
There is currently no known effective medical treatment. However, the following medications may be helpful for some patients:
•Vasodilator drugs (drugs that widen the blood vessels)
•Drugs that control immune system's response (such as azathioprine or steroids)

A lung transplant may be needed.

Outlook (Prognosis)
The outcome is often very poor in infants with a survival rate of just a few weeks. Survival may be months to a few years in adults.

Possible Complications
•Progressive difficulty breathing
•Pulmonary hypertension
•Right sided heart failure (cor pulmonale)
•Coughing up blood

When to Contact a Medical Professional
Call your health care provider if you have symptoms of this disorder.

Alternative Names
Pulmonary vaso-occlusive disease

References
Barst RJ. Pulmonary hypertension. In Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 67.

Channick RN, Rubin LJ. Pulmonary hypertension. In: Mason RJ, Broaddus CV, Martin TR, et al. Murray & Nadel's Textbook of Respiratory Medicine. 5th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 52.

Update Date: 6/2/2010

Updated by: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.