pulmonary capillary hemangiomatosis :: Article CreatorHow One Woman Went From A Double Lung Transplant To A Gold Medal
Tennis is everything for Genie Kilpatrick.
Ever since her mom signed her up for classes when she was young, "tennis has been my life," she said. "It saved my life in so many ways."
"I was a chubby little kid and needed some sort of a -- some way to exercise," Kilpatrick said.
Tennis was also her window into social life and belonging in high school.
She even went on to get a full-ride college scholarship for her talents. After graduating, she became a tennis coach for a living and met her wife, Sheri Norris, through the sport.
Genie Kilpatrick recovering in the hospital with wife, Sheri Norris, by her side.
Sheri Norris
But in 2015, at age 48, she started experiencing shortness of breath and it turned her world upside down.
At first, doctors had trouble diagnosing Kilpatrick, but the root of her illness was finally discovered: pulmonary capillary hemangiomatosis.
PCH is a disease where the vessels in both lungs grow abnormally and make it difficult to breathe. The estimated survival without treatment is three years, according to a study published by Radio Graphics in 2007.
The only cure is a double lung transplant.
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As her lungs failed her, she was "not able to walk from one room to the other end without my machine," she said, "dragging the 21-liter oxygen tank with me."
"It was tough because I didn't think I'd ever have a racket in my hand [again]," she added. "I'd sit at the window and just watch people jog by and run by: 'If only I could just move again.'"
Her return to tennis began when she was put on the transplant waiting list.
Five months later she got the call that there was a donor and that she'd have a shot at a transplant.
Genie Kilpatrick recovers in the UT Southwestern Medical Center after her double lung transplant in 2016.
Sheri Norris
"It's an exciting call," she said. "But you know that some somebody somewhere ... Has died and it's tough because someone in some family somewhere is dealing with loss. And we're dealing with a second chance of life."
She went to the University of Texas Southwestern Medical Center in Dallas, where she underwent the delicate, challenging and, ultimately, successful surgery. Along the way, she never took her eye off the prize.
Genie Kilpatrick wanted to start practicing tennis as soon as she could, so she had her racket in the hospital after surgery.
Sheri Norris
Her wife made big tennis posters for her to hang up on the walls, and for two months she says she was "in that hospital room and holding that racket in my hand. I was bumping the ball up and down the hall when I went to walk, said, 'I'm not going without my racket!'"
Genie Kilpatrick wanted to start practicing tennis as soon as she could, so she had her racket in the hospital after surgery.
Sheri Norris
Right before her surgery, she had learned about the World Transplant Games and made it a goal to compete.
(MORE: This woman donated her kidney to a co-worker and now they hope to send a message)
According to Kilpatrick, the majority of participants in the World Transplant Games have undergone transplants of organs such as the kidney, liver or pancreas. Lung transplants are less common, with University of Michigan Medicine reporting about 2,000 Americans receive a new lung compared to the 18,000 kidney transplants that take place each year.
And Kilpatrick was a rarity with her double lung transplant.
Genie Kilpatrick poses with her gold metal at the 2019 World Transplant Games.
Sheri Norris
Regardless, she gave it her all and came home with gold during the August 2019 games.
"Just to take home the gold and represent your country was pretty incredible," she said. "I don't know what it feels like to be a kidney or liver [recipient] or anything else, I just feel like I'm the same as everybody else because I had a lung transplant."
She now spreads awareness for how organ donation changes lives, even going to the tennis tournament where she once coached to set up informational booths about becoming an organ donor.
"It's all about giving back and spreading the word. I'm happy to do anything that I can to get the word out," Kilpatrick said. "If one more life can be saved, it's going to be all worth it."
She's so grateful for everything she has, and wants others to have that same chance at pursing their dreams.
"I was so lucky to receive an organ and have a second chance," she said. "I just want everybody that is stricken with a disease to be able to be in the same boat."
RHC Still Recommended Over Echocardiography For Detecting COPD-PH
In a single-center, retrospective analysis, echocardiography demonstrated low sensitivity but high specificity for diagnosing pulmonary hypertension (PH) in advanced chronic obstructive pulmonary disease (COPD).
Echocardiography has high specificity in the diagnosis of pulmonary hypertension (PH) in patients with advanced chronic obstructive pulmonary disease (COPD); however, it has low specificity for detecting PH in these patients, according to a recent study published in The American Journal of the Medical Sciences.
Detecting PH in patients with COPD is critical. Patients with COPD combined with PH carry increased risks for hospitalizations, mortality, and other exacerbations, and their PH status can influence candidacy for lung transplantation.
Holding Lungsimage credit: Katerina - stock.Adobe.Com
Right heart catheterization (RHC) is the typical approach for diagnosing PH, but the use of echocardiography could provide lots of benefits to patients. Comparatively, echocardiography is less expensive and invasive, and performs well in screening a patient's pulmonary arterial pressure (PAP); however, the efficacy of this approach in identifying PH in patients with COPD is not sufficiently studied. Furthermore, at present RHC is relied upon for multiple transplant criteria including the management of transplant listing, identification and treatment of left-sided heart disease, as well as decision-making for single or double lung transplants. To address this gap, researchers conducted a study to evaluate the usefulness of echocardiography as a less-invasive diagnostic tool for PH in patients with COPD.
Patients with COPD being assessed for lung transplantation who also underwent RHC for PH diagnosis were evaluated between January 2011 and May 2019. Echocardiograms were performed at a median of 3 days from the time of RHC, with the majority occurring beforehand (76%). Individuals were classified as having low, intermediate, or high probability of PH according to their tricuspid regurgitation velocity (TVR) and the presence of echocardiographic evidence of PH. Echocardiographic criteria for PH included at least 2 of the following: detection of a right/left ventricle basal diameter ratio above 1, interventricular septum flattening, right ventricle outflow doppler acceleration time under 105 m/s, mid-systolic notching, or early diastolic pulmonary regurgitation velocity above 2.2 m/s-1.
A total of 111 patients with severe COPD (defined according to the 6th World Symposium of Pulmonary Hypertension) who received RHC were evaluated. Of these 111, 82 patients were identified with COPD-PH (74%). Overall, patients had a mean PAP (mPAP) of 30.3 mm Hg, systolic PAP (sPAP) of 45.8 mm Hg, diastolic PAP (dPAP) of 21.8 mm Hg, pulmonary vascular resistance (PVR) of 4.0 mm Hg, diastolic pulmonary gradient (DPG) of 8.5 mm Hg, right arterial pressure (RAP) of 9.7 mm Hg, and pulmonary capillary wedge pressure (PCWP) of 13.5 mm Hg. Those with COPD-PH were noted to have significantly higher PAP levels compared to those without.
According to the echocardiographic criteria, 20 patients (18%) had a high likelihood for PH, 20 (18%) had intermediate, and 71 (64%) had low. In these cases, the sensitivity, specificity, positive predictive, and negative predictive values for echocardiography diagnosing COPD-PH were 43%, 83%, 88%, and 34%, respectively. Additionally, for the diagnosis of COPD–severe PH, these values were 67%, 75%, 50%, and 86%.
In light of their findings, the authors note that the echocardiography's high specificity and positive predictive values demonstrate its value in the diagnosis of COPD-PH; however, it exhibited limited sensitivity for ruling out COPD-PH and its efficacy was impacted by the presence of significant hyperinflation or sever emphysema in these patients. The authors conclude by mentioning their results were not consistent with recommendations for identifying COPD-PH with noninvasive methods for advanced COPD; therefore, pretransplant RHC should still be considered.
Reference
Younis M, Al-Antary N, Dalbah R, et al. Echocardiography and pulmonary hypertension in patients with chronic obstructive pulmonary disease undergoing lung transplantation evaluation. Am J Med Sci. Published online November 13, 2023. Doi:10.1016/j.Amjms.2023.11.006
Pulmonary Vascular Disease
Pulmonary vascular disease is the medical term for disease affecting the blood vessels leading to or from the lungs. Most forms of pulmonary vascular disease cause shortness of breath.
The definition of pulmonary vascular disease is simple: any condition that affects the blood vessels along the route between the heart and lungs.
Blood travels from the heart, to the lungs, and back to the heart. This process continually refills the blood with oxygen, and lets carbon dioxide be exhaled. Here's how the process works:
Oxygen-poor blood returns from the body's tissues through the veins back to the right side of the heart. The right heart pumps oxygen-poor blood through the pulmonary arteries into the lungs. This blood becomes filled with oxygen. The oxygen-rich blood returns from the lungs back to the left side of the heart. The left heart pumps the oxygen-rich blood into the body through the aorta and many other arteries. Any part of the heart-lung blood circuit can become damaged or blocked, leading to pulmonary vascular disease.
The causes of pulmonary vascular disease vary according to which of the lungs' blood vessels are affected. Pulmonary vascular disease is divided into several categories:
Pulmonary Arterial Hypertension: Increased blood pressure in the pulmonary arteries (carrying blood away from the heart to the lungs). Pulmonary arterial hypertension can be caused by lung disease, autoimmune disease, or heart failure. When there is no apparent cause, it's called idiopathic pulmonary arterial hypertension.
Pulmonary Venous Hypertension: Increased blood pressure in the pulmonary veins (carrying blood away from the lungs, to the heart). Pulmonary venous hypertension is most often caused by congestive heart failure. A damaged mitral valve in the heart (mitral stenosis or mitral regurgitation) may contribute to pulmonary venous hypertension.
Pulmonary Embolism: A blood clot breaks off from a deep vein (usually in the leg), travels into the right heart, and is pumped into the lungs. Rarely, the embolism can be a large bubble of air, or ball of fat, rather than a blood clot.
Chronic Thromboembolic Disease: In rare cases, a blood clot to the lungs (pulmonary embolism) is never reabsorbed by the body. Instead, a reaction occurs in which multiple small blood vessels in the lungs also develop blood clots. The process occurs slowly, and gradually affects a large part of the pulmonary arterial system.
The symptoms of pulmonary vascular disease vary according to several factors:
The suddenness of the process affecting the pulmonary blood vessels Which pulmonary blood vessels are affected (where the pulmonary vascular disease is) How much of the pulmonary vascular system is affected For example, a sudden, large pulmonary embolism blocking a large pulmonary artery can cause severe shortness of breath and chest pain. But a very small pulmonary embolism (blocking only a small blood vessel) may cause no noticeable symptoms.
Although symptoms of pulmonary vascular disease can vary widely, each of the causes of pulmonary vascular disease has a set of usual symptoms:
Pulmonary arterial hypertension: This most often causes slowly progressive shortness of breath. As the condition worsens, chest pain or fainting (syncope) with exertion can occur.
Pulmonary embolism: A blood clot to the lungs typically occurs suddenly. Shortness of breath, chest pain (often worse with deep breaths), and a rapid heart rate are common symptoms. Pulmonary embolism symptoms range from barely noticeable to severe, based on the size of the blood clot(s).
Pulmonary venous hypertension: This form of pulmonary vascular disease also causes shortness of breath, due to the congestive heart failure that's usually present. Shortness of breath may be worse while lying flat, when blood pressure is uncontrolled, or when extra fluid is present (edema).
Based on a person's symptoms, signs, and history, a doctor may begin to suspect the presence of pulmonary vascular disease. The diagnosis of pulmonary vascular disease is usually made using one or more of the following tests:
Computed tomography (CT scan): A CT scanner takes multiple X-rays, and a computer constructs detailed images of the lungs and chest. CT scanning can usually detect a pulmonary embolism in a pulmonary artery. CT scans can also uncover problems affecting the lungs themselves.
Ventilation/perfusion scan (V/Q scan): This nuclear medicine test takes images of how well the lungs fill with air. Those images are compared to pictures of how well blood flows through the pulmonary blood vessels. Unmatched areas may suggest a pulmonary embolism (blood clot) is present.
Echocardiography (echocardiogram): An ultrasound video of the beating heart. Congestive heart failure, heart valve disease, and other conditions contributing to pulmonary vascular disease can be discovered with an echocardiogram.
Right heart catheterization: A pressure sensor is inserted through a needle into a vein in the neck or groin. A doctor advances the sensor through the veins, into the right heart, then into the pulmonary artery. Right heart catheterization is the best test to diagnose pulmonary arterial hypertension.
Chest X-ray film: A simple chest X-ray can't diagnose pulmonary vascular disease. However, it may identify contributing lung disease, or show enlarged pulmonary arteries that suggest pulmonary arterial hypertension.
Pulmonary angiography (angiogram): Contrast dye is injected into the blood, and X-ray images of the chest show detailed images of the pulmonary arterial system. Angiography is very good at diagnosing pulmonary embolism but is rarely performed anymore because CT scans are easier, less invasive, and have lower risk.
There are many different treatments for pulmonary vascular disease. Pulmonary vascular disease is treated according to its cause.
Pulmonary embolism: Blood clots to the lungs are treated with blood thinners (anticoagulation). Treatments include the medicines are betrixaban (BEVYXXA), enoxaparin (Lovenox), heparin, and warfarin (Coumadin).
Chronic thromboembolic disease: Serious cases of thromboembolic disease may be treated with surgery to clear out the pulmonary arteries (thromboendarterectomy). Blood thinners are also used. Riociguat (Adempas) is a drug approved for use after surgery or in those who can't have surgery, to improve the ability to exercise.
Pulmonary arterial hypertension: Several medicines can lower blood pressure in the pulmonary arteries:
These drugs have been best shown to improve idiopathic pulmonary arterial hypertension.
Pulmonary venous hypertension: Because this form of pulmonary vascular disease is usually caused by congestive heart failure, these treatments for heart failure are usually appropriate:
If pulmonary vascular disease is brought on by another condition, treating that condition might improve the pulmonary vascular disease:
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