Lung transplant only cure for pulmonary hypertension
When Wendy King started experiencing shortness of breath in the fall of 2010 she thought she had developed asthma. Never did she imagine that less than a year later she would be given a diagnosis of pulmonary hypertension (PH).
© Photo courtesy Coadi Byrne Photography
Wendy King of Grand Falls-Windsor has been battling Pulmonary Hypertension since 2010 with the support of husband Stephen and son Kenneth.
Fast forward five years and she finds herself preparing for assessment for a double lung transplant.
King, 39, is married and has a seven-year-old son. Her life in Grand Falls-Windsor was fairly typical when her symptoms began.
“I never would have thought it would be this rare incurable disease,” she said recently when asked about her PH journey thus far. “They tell you what to expect as the disease progresses but you don’t realize how sick you actually are. You still want to go, go, go in your mind, but your body won’t let you.”
A teacher at Woodland Primary and then Millcrest Elementary up until December 2014, King had energy to spare. When she wasn’t teaching she and husband Stephen went to the gym, enjoyed cycling and she was always up for extra activities. Her son Kenneth is involved in a variety of sports and King would most often be there with him, involved in parents’ groups or cheering from the sidelines. And you could usually hear her before you saw her. Now going up the seven steps to the bedrooms of their home is a challenge.
After going up those steps to read Kenneth a story before bed, King said she has to catch her breath before coming down.
“It hurts,” she explained. “You can’t even laugh without running out of breath and seeing stars.”
What is PH?
PH is a disease characterized by an increased blood pressure in the pulmonary arteries (the arteries of the lungs) with values above the normal baseline. The pulmonary arteries become narrowed and can be scarred to the point of being closed. The heart is also affected as the right ventricle (the lower chamber on the right side) has to work harder to pump blood through the lungs. PH patients suffer from shortness of breath, fatigue, dizziness and fainting, which tend to get worse with continued activity.
As King has learned, pretty much any task leaves her breathless. Getting dressed to go to medical appointments is exhausting as are the everyday chores of preparing meals and doing housework and laundry.
Working out and cycling are things of the past. It’s cruel irony that she was in the best shape she’d be in in years when the shortness of breath set in.
“All those things that define you as a person slowly change because you can’t do those things any more,” King said. “They are robbed from you. That’s the hard part.”
She added if it wasn’t for the support of family and friends she’d never be able to get through it. “The support we have received has been overwhelming and has made the journey that much easier. Knowing you have the support of your community, friends and family means a lot.”
If you’ve never heard of PH before now it’s not surprising. According to the Pulmonary Hypertension Association of Canada (PHAC) it’s more common than previously believed, but it’s not clear exactly how many Canadians might suffer from the disease. There are several reasons for this – the incidence of PH hasn’t been specifically studied in Canada; there are many kinds of PH; many patients with early or mild PH may not have symptoms and, as a result, haven’t yet been diagnosed.
In King’s case, while it took less than a year for the PH diagnosis to be made, it was only after a year or so of testing that it was determined she has idiopathic PH which means there is no known cause. This applies to a small number of PH patients and is more common to females than males. And as Wendy points out, it makes her PH more difficult to treat.
King’s condition was managed without medication until February 2014. At that time her right ventricle pressure (RVP) was high enough for doctors to decide it was time. She said the average RVP is 15. When she started medical treatment hers was 75. Today it’s 102. Her current drug, the third she’s been on, is administered by continuous infusion through a Hickman line in her chest.
As King pointed out, the drug is supposed to lower her RVP, but isn’t working. At best it is maintaining it.
King is at the end of the road for trying new drugs. And even if there was another one to try, medications don’t usually cure PH, according to the PHAC website. Transplant of the lungs or both lungs and heart is often a last treatment option in certain patients with PH. This is where King is now. She said they’ve known this was coming, just not this quickly.
In St. John’s last month for a week of appointments and tests, including ones specific to a six-centimetre aneurysm that has developed in her inferior vena cava (one of the large veins that carry deoxygenated blood from the body into the right atrium of the heart), King was hoping to get some answers and narrow down the details regarding a double lung transplant.
On Feb. 1, the day after she returned home, she found out she’s to be in Toronto on March 29 to begin assessment for a double lung transplant.
There is much to be done between now and then and like many people King said she didn’t know anything about the involved process that has to be worked through prior to leaving for Toronto. She now has a social worker in Toronto attached to her case and that person has been “a great source” of information. So far King has booked the flight for both her and Stephen (who is her primary support person) and has a hotel room booked for the week of assessment. If she qualifies for a transplant they will be in Toronto until the organs become available. And no one knows how long the wait will be. They will have to rent an apartment near Toronto General Hospital and there will be groceries to buy. There could be some medical equipment needed for before and after the transplant that may not be covered by MCP or private insurance. On top of all this, arrangements must be made for care of Kenneth and their two dogs.
It's no exaggeration that everyone connected to the King family wants to do something for them. At this point fundraising is at the top of the list and no moss has grown under the feet of the 10 family members and friends who met Feb. 5 to get things rolling.
Stephen’s father, Carson King, is chairing the steering committee. Following the meeting he said there was no shortage of ideas. A Facebook group (Wendy’s PH Journey: Join the PHight) has been established to provide not only information on Wendy’s ongoing journey and events planned by the committee, but also a venue for messages of support for Wendy, Stephen and Kenneth.
A GoFundMe account is in the works. Collection jars are being placed in a number of businesses. A special purple T-shirt (purple being the colour for PH) is available for sale. An account will be opened at CIBC and donations may be made at any branch of that bank.
King said the committee was formed to have a co-ordinated fundraising effort, having everyone on the same page but not at the same time when it comes to direct fundraising such as ticket sales. He added he knows there are many who want to help and has encouraged committee members to go to those people when help is needed.
The first major event is well in hand. A dinner and dance is being planned for Sat., March 5 at the Royal Canadian Legion Branch 12 in Grand Falls-Windsor. The meal will be prepared by chef Allan Milley. Dinner music will be provided by well-known local musician Michael Snelgrove and there are three bands booked for the dance, all donating their time and talent. There will be smaller fundraisers held during the night. Details are being finalized.
Tickets are available by contacting Carson King at 290-4889 or committee member Jeff Power at 290-4969.
King isn’t involved directly in fund-raising. Her job is to take care of herself and stay as strong as possible prior to going to Toronto.
“Doctor’s orders,” she explained.
That being said, she does have input. When she learned of the March 5 event one of her comments was “I really would like to tie in organ donation as well. It’s so important. Organ donation can do so much for someone else.” To that end, information about organ donation will be available at the event.
There are two websites that provide facts and answers to frequently asked questions: www.servicenl.gov.nl.ca (search organ and tissue donation) and www.easternhealth.ca (organ donation is listed under Services Quick Links). Individuals can indicate their intention of being an organ donor on their drivers licence application form. A small red heart will appear on the lower right hand corner of the licence. For those who don’t drive or want to indicate their intention before their licence must be renewed there is an organ donor card available for download on the Eastern Health website. Even if both these things are done, the Eastern Health website urges everyone to share their wishes with their family.