The road from diagnosis to support

High blood pressure in the lungs is the simple explanation for a complex disease — November is Pulmonary Hypertension Awareness Month

In just a few months, Louise Taylor went from living an active life filled with good health to one that turned her into a fatigued, house-bound patient.

“Suddenly I was very short of breath.  Next came five months of tests: heart, lungs, sleep apnea and many blood tests,” she said.

In October 2014, Taylor was diagnosed with scleroderma-induced Pulmonary Arterial Hypertension (PAH), which  meant her heart could no longer pump blood and oxygen to her lungs with ease.

“Just breathing was a task, and in a matter of weeks I was sent to Vancouver General Hospital (VGH) for another heart test that confirmed this rare disease was due to scleroderma,” she said.

Scleroderma is an autoimmune disease, whose characteristic feature is the presentation of hard, thickening of the skin, and the lesions that occur in small blood vessels.

For Taylor, her diagnosis of PAH has involved a variety of treatments and a number of trips back and forth to VGH.

“I was put on Opsumit (a drug that works to open narrow blood vessels between the heart and lungs) while we awaited the home visit from a nurse.”

The nurse came to Taylor’s house twice daily for two hours at a time for a week, teaching her and her husband how to build cassettes of Caripul, a medication for the long-term intravenous treatment of primary pulmonary hypertension (PH) and secondary PH due to scleroderma.

“Next came a trip back to VGH for 10 days to have the Hickman, or intravenous line, inserted into the chest,” she said. “While there we were taught how to clean and care for the site, flush the lines as well as how to change the bandages. Every possible emergency situation was reviewed.”

The Caripul is pumped into Taylor’s heart through an IV line attached to a hip pump that is worn 24 hours a day, seven days a week. Five boxes of supplies are ordered and delivered to the house every four weeks.

“Mixing the saline vials with the powdered Caripul vials via syringes and then injecting that solution into the cassettes can take up to an hour each,” said Taylor. “The cassettes can be built up to eight days ahead, kept refrigerated and are changed daily.

“Every time I leave the house, an extra cassette and pump must accompany me.  An emergency kit with all possible supplies is kept in the truck in case something is needed.”

Taylor said the equipment she wears has made for interesting conversations with security staff at the airport.

“Going through the airport is interesting as you have to get staff to understand you can’t take the pack off, it really is surgically attached to me and the medical supplies accompanying me are a necessity,” she said.

Taylor’s life has changed radically since her diagnosis of PAH, but she’s happy that this past summer she returned to her work as a forestry recreation site host, where with a helper she was able to complete the daily maintenance and camper tasks.

Thanks to the wonderful care of nurse practitioner Lisa Lee, cardiologist Dr. Nathan Brunner, the Caripul pump and the support group, I have now returned to an almost normal lifestyle,” she said. “Everyday life now requires up to 12 hours of sleep nightly, and over-taxing the system can take up to three days to recover,” she said. “I must return to VGH at least every four months for ongoing tests and care.”

When Karen Bouchard was diagnosed with PH in 2007 she had already spent months undergoing medical tests followed by strong treatment and its myriad side effects.

“I had been diagnosed with pulmonary fibrosis, which required daily oral doses of chemotherapy,” said Bouchard. “I became nauseated, lost my hair and had my white and red blood cells almost totally decimated.

“I had to go off the chemo drug until my immune system recovered a bit, then back on it again through the winter.”

While going through all of this, Bouchard received a Saturday morning call from her doctor, advising her to travel to VGH to meet with a PH specialist.

“I must have asked what the possible treatment would be and he said they would probably put me on Viagra. It’s a good thing I was sitting on the floor, because I fell over and laughed myself silly. I wasn’t sure that I had heard correctly, but the doctor confirmed that at least my hearing was still working.”

She met with the specialist in April 2007, who ordered a series of blood tests — 14 test tubes’ worth. Bouchard ended up with a diagnosis of PH and a prescription for Viagra.

“You have no idea how funny it is to take such a thing to the pharmacy. The male pharmacists are all trying to avoid looking you in the eye, the female pharmacy assistants are laughing their heads off. It’s horrendously expensive and the doctor had to get special authority from Pharmacare, and I had to arrange for Blue Cross to pick up the tab. The first two to three weeks cost us about $600.”

The active ingredient in Viagra — sildenafil — works by opening the blood vessels in the lungs to allow blood to flow through more easily.

Bouchard said she has what is considered a fairly mild form of PH at this point, but it’s one more diagnosis after two decades of health challenges.

“First, I received a diagnosis of rheumatoid arthritis, which two years later was reassessed to be polymyositis (inflammation of many muscles).  Either the illness or the medications — or neither— may have been responsible for my development of pulmonary fibrosis, and that may have led to the PH.

“The pharmacy visits have improved — it reminds me a bit of the theme song to the TV series Cheers as I go where ‘everybody knows my name.’ I’ve learned a whole lot of medical vocabulary, learned to take someone with me to doctors’ appointments and met some wonderful people in our local support group as well as the online support groups.

“I’m glad that my condition is under control with medication, and that I have a group of friends who have experiences that I can learn from if and when the disease worsens. And I am still able to work, enjoy life with my family, and laugh.”

For Joan Nemeth, Doreen Millar and Marilyn Lane, Pulmonary Hypertension Awareness Month is just that: a chance to share awareness, both with the general public and with the medical community. It’s a task made more difficult due to the rarity of PH.

“Many doctors don’t know what it is, and it’s hard to diagnose,” said Nemeth, a non-smoker who had enjoyed good health until she was diagnosed with PH six years ago. “I knew there was something wrong but I kept pushing to get a diagnosis.

“When I was diagnosed, I was given three months to live; I’m now in my sixth year.”

Millar said the support group gives anyone living with PH a chance to share with others who understand.

“The important thing for all of us is the understanding, the caring, the sharing and the laughter,” said Millar.  “It’s so fun to meet with these gals and they know exactly what you’re talking about.”

Nemeth said when the group gets together, members share tricks that they’ve learned, to make living with PH a little easier.

“Tricks such as having a bed that is much higher so I don’t have to bend too far to get into it, or having two laundry hampers, one on top of the other, so it’s not too far to reach. We get out of breath easily, so anything we can do to make life easier, is great.

“And if you see a handicapped sticker, but we don’t look sick, don’t judge. It’s there for a reason.”

Lane is a retired nurse who was diagnosed with PH in 2008 and said she was lucky as her doctor picked up on her symptoms immediately and referred her for tests.

“People often don’t understand because you look well,” she said. “The support group is great — we share solutions and how we manage things, and it’s wonderful not to feel alone.”

The group is supported by retired nurse Betty Ross, a board member with the B.C. Pulmonary Hypertension Society. For more information, contact Ross at 250-861-1961or email Br.20@shaw.ca

 

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