Living with Pulmonary Hypertension: A Family’s Struggle to Deal with the Fatal Disease

Living with pulmonary hypertension - A family’s struggle to deal with the fatal disease

12.01.2015

Source: Strathroy Age Dispatch

1297778486666_ORIGINAL.jpgOn Monday, Nov. 16, Everleigh Pierce turned five years old.

Two days later, her school, Caradoc Public School in Mt. Brydges, had planned a special event to celebrate her birthday.

The school was decorated and the hallways looked like a scene taken from a Western University’s homecoming day, as all children in the school were wearing purple shirts in honour of their classmate.

“Is this all for me, mommy?” Everleigh asked her mom, Kerry, with a huge smile on her face, as they both entered the school.

“Is this really all for me?” she asked again in disbelief. “Wow! This is the best day ever,” she added.

For Kerry and Craig Pierce, like any parents, seeing their daughter turn five was a special day; but for the Pierce family, who lives in West Elgin, Ont., this milestone was also extremely important because, unfortunately, they don’t know if they will be able to see Everleigh turn six.

Three years ago, Everleigh was diagnosed with a rare disease called pulmonary hypertension (PH). There are approximately 5,000 known cases of people living with the disease in all Canada, but the Pulmonary Hypertension Association of Canada (PHAC) estimates that number is probably twice as big, but half of the people have yet to be properly diagnosed.

Scientifically speaking, PH is high blood pressure inside the lungs’ vessels, but its effects on people’s health are much more serious, said Dr. Sanjay Mehta, chair of the PHAC. The disease causes the vessels inside the lungs to get narrowed, impeding the normal flow of blood from the heart through the lungs, and forcing the heart to work harder to push the blood through the body and weakening it overtime, Dr. Mehta, who is also the director of the Southwestern Ontario PH Clinic, explained.

In layman’s terms, living with PH is like trying to run a marathon… with a bag over your head. The amount of air entering the lungs is the same you would get by inhaling through a straw. The air is so scarce, that common day activities like walking or climbing two flights of stairs become exponentially harder.

And as of today, there’s no cure for the disease – the life expectancy of someone diagnosed with PH who receives proper treatment is about seven years.

Two years ago, doctors gave Everleigh three to five years.

*****

1297778490671_ORIGINAL.jpgThe day Everleigh was diagnosed with pulmonary hypertension (PH) remains in Kerry's head like a blur, like a really bad nightmare in which it’s hard to remember exactly what was going on or to make sense of it.

It all happened during a snowy day in February of 2012, but the agony had been going on for much longer.

“I knew something was wrong with Everleigh when she was eight-weeks old,” Kerry remembered.

As she had done with her two older children, Kerry had enrolled Everleigh to a newborn introduction-to-water swimming class. The day of the first class, when the session was over, Kerry noticed Everleigh had turned “quite blue” in comparison to the other babies, she said.

Thus, she decided to take Everleigh to the hospital, in what would become the first of dozens of trips to medical centres where she would receive the same answer for almost two years.

“‘This is very normal. There’s nothing to worry about,’ they would tell me,” Kerry said.

But with time, despite family doctors, dietitians, and pediatricians telling her otherwise, Kerry’s suspicions that something serious was going on with her daughter only increased.

"There were just a ton of tiny little things about her that were so different," she said.

Kerry recalls that Everleigh would always seem tired and sad; that she would take four-hour naps every day; that you could barely hear her cry; that she never wanted to eat; that even when she wasn’t doing anything, Kerry could see and feel Everleigh’s heart going really fast; and that after reaching some milestones like crawling or walking, she would simply stop doing them.

And yet, doctors didn’t see anything abnormal. The problem with PH is that its symptoms are so general – shortness of breath, tiredness, and when patients are very sick they get lightheaded and pass out – that unless doctors are looking for the disease specifically, it’s very difficult for them to see it.

"I started to take my mother-in-law with me to the doctors appointments because I started to think that maybe I was crazy... or that I wanted my daughter to be sick," she said. “I was very frustrated because I knew something was wrong, and nobody would listen. I took Everleigh so many times to the doctor’s office that they were sick of me there.”

But that day in February, something was different.

Kerry noticed Everleigh’s hands and feet were turning really purple and, despite the bad weather, she decided for the first time to take Everleigh to London.

“I don't know to this day what told me that day to take her to the Children's Hospital because I had never taken her there before, and it was a very snowy day,” Kerry said. “But if I hadn’t, Everleigh probably would have died that day.”

Once there, Kerry received a then-familiar response, and Everleigh was added to the non-emergency list.

But seven hours in, Everleigh visibly worse, Kerry approached another nurse and asked her to check Everleigh again, she said. At that point, Everleigh’s oxygen levels were very low, and she was rushed in for care and some tests.

What came next, Kerry has some trouble remembering. The only thing that is still vivid is the pain she felt when she saw a number of doctors walking towards her.

"I just knew it was bad, before they said anything, I knew it was bad," she said.

The hospital called a cardiologist who gave the first PH diagnosis.

But it took almost two weeks before Everleigh was stable enough to move her to the Hospital for Sick Children in Toronto, where she spent another month, and the PH diagnosis was confirmed.

*****

Everleigh’s care is very complex. Everywhere she goes, she must carry a small backpack with her medicine that is administered to her through an IV. A nurse is with Everleigh at all times – at school taking care of her, at home when her parents are at work. The nurse assists the five-year-old in carrying an oxygen tank, which is needed 24 hours a day, seven days a week. In addition, Everleigh has to take oral medication around 12 times a day. Against her wishes, her physical activity is very limited – a fall or a hit in her chest could be fatal. A simple cold or a mild fever could keep Everleigh in bed for weeks.

But the most complex part of her treatment is that Everleigh has reached a point where her medicine dose is as high as it can be before it stops working, making a heart and lungs transplant the next step in her treatment. But moving ahead with the procedure is very tricky; basically, Everleigh needs to be sick enough to need the transplant, but healthy enough to survive it. Among other things, this would allow her body to grow enough to be able to receive bigger organs – Everleigh is very small, and her body could only receive organs from other kids at this point, making finding a donor that much harder. Still, getting a transplant is not a cure, but a way to extend Everleigh's life. In average, lungs transplants keep a person alive for six years. It could be many more years or it could only be a few weeks; the longer Everleigh can live with her own organs, the longer her life expectancy becomes.

*****

“Every single time I go for an appointment, and I know it’s silly and sounds almost juvenile, but every time when we get in the car I pray, and a little piece of me thinks that we're going to get there and they're going to tell me they were wrong. Every time, and I'm three years in, and I still do it... and that never happens; but that is how it feels. And the part that is the most unfair is that (Everleigh) doesn't know. She thinks she's going to grow up and have babies and get married, and do all of those things that we know she won’t. And that's the part that makes me sad, because when you have children you have this dream for them, and nobody expects to be told that your child is terminally ill... and to go home and grieve her until that they comes... because that's what happens. When an accident happens and you lose a child it's terrible and it's traumatic and it's awful, but I feel like we live it everyday. I live every single day grieving her, and she is here... does that make sense? It's a terrible feeling... and she is such a joy to be around. Being home with her is easy; the hard part is going to work everyday because her medicine is expensive, and not spending this time with her, and she’s having to spend her time with nurses, and her memories are being with nurses... life it's not fair; nobody said that it was, and not because she's my daughter, but she's just such a special little girl; she's so smart and her love for creatures and animals; she's everything that is good in the world. It's getting a crappy deal. I'm not sad for me as her mom. I'm said for her as her mom. I can take the pain; I just don't want her to. I just wish there was something I could to take it away from her, but I can't... and to have to do things to her that hurt her and make her cry... but we have no choice but to poke her with needles and change her IV bag every other day... the whole thing is unfair. It feels like we are in autopilot most of the time...” Kerry said.

*****

1297778497196_ORIGINAL.jpgIf having to face PH didn’t bring enough hurdles, the Pierce family, like thousands of patients in Canada who live with rare diseases, also has to live with the fear of losing the assistance from the government to purchase the medicine that is keeping Everleigh alive, said Dr. Mehta.

Right now, there are a total of nine medications approved by Health Canada for the treatment of PH, which have proven to be safe and effective at increasing the life expectancy of patients.

The issue, however, is the cost of the drugs.

The cheapest medication, which isn't enough to treat the disease by itself, goes for $1,000 a month, with the pills prescribed more commonly sitting at $4,000 a month. Once the severity of the disease increases, the next step is to prescribe liquid medication that is infused in the patient's veins or under the skin and which costs around $10,000 a month.

And while studies have proven the effectiveness of these medications, it is the Canadian Agency for Drugs and Technologies in Health, CADTH, which decides which medications should be funded by the government.

“CADTH recently said that government should only fund the cheapest drug because it is the cheapest and it seems to work okay for three months, but you can't look at a study for three months and say that's ok for a disease that's progressive and it's fatal. How does that make any sense?... So Ontario is looking at CADTH, and we are very nervous they are going to restrict funding for these drugs.” Dr. Mehta said. “"Most families, including my family, cannot afford the medications for PH. It is not reasonable to expect people to pay for them.”

Dr. Mehta said Canada is the only country member of the G20 without a unified policy to treat people living with rare diseases.

“Since these are rare diseases, (governments) can kind of ignore these patients because it doesn’t lead to a lot of outcry,” he said.

But for him, the only real solution is people taking action. That’s why PHAC is constantly conducting awareness campaigns and looking the support from citizens.

“People need to understand that governments respond not to doctors or physicians, but to people and communities. Contact you MPP and tell them that something is wrong if we are not paying for these drugs for these patients,” he said.

“All we are saying is, gives us the flexibility to choose the right drug for the right patient,” he added.

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