Annie Whitaker - phaware® interview 394

Nov 29, 2022

PHA Australia CEO, Annie Whitaker, gives a raw account of her connection to pulmonary Hypertension as carer to her son Tim, who lost the PH War in 2006. Annie discusses how one promise to her son has helped her work through grief.

Good day from Australia. My name is Annie Whitaker, Annie Boxsell Whitaker on Facebook. I've been in the land of pulmonary hypertension for 23 years online and personally. So much has happened. Where do I start? Maybe where I am now and then I'll go to the start of a disease I thought was harmless and was just hypertension. However, it took the life of our son, Tim.

Now I am CEO of Pulmonary Hypertension Association Australia. Also, a founder, a carer, and a bereaved parent. I’m honored to have been awarded the Periwinkle Pioneer [award] in Dallas in 2016, as well as this year right down to the final four of Australian of the Year, which is a big deal down under. I don't do this work for these accolades, however, they do keep you going and give you a boost.

For the start of my journey in this space, I'll be using PH to cover all aspects of this disease, as back in 1999, that there were no other classifications. You just had pulmonary hypertension. You didn't have CTEPH or anything like that.

Tim, our son, was very intelligent. He moved into his own apartment. At 19 years young, Tim developed a blood clot in his right arm that grew to nearly 25 centimeters. Then it started to break off into his lungs, which is now called CTEPH. He was not living at home and wanted space. In saying that, I noticed a change in his color when I saw him, a general slowing down of pace. When he was walking, he was a bit short of breath. He asked for my antacid, as he had chest pain. He was an adult, albeit a very young adult, totally responsible for himself. When I brought all these issues up, it was because of lifting furniture. He said he'd pulled muscles wrong. From a young, independent adult that does not like being told to do when he left home, we wanted him to go to hospital at a very least and have an ultrasound on his swollen arm. The parent eventually won, as Timothy knew, he was getting worse.

There were many pulmonary embolisms in his outer arteries. We had to go do five doctors to get to this point. You know your child or anyone you love deeply, their color, the way their eyes are, the way they walk, do they suffer from pain. You just know by looking at them, you can tell probably better than they can, especially as a young adult. Tim said 19-year-olds don't get blood clots, right? We were actually battling to get him to a doctor before we started the battle to find a doctor.

Tim actually relented and referred himself to a professor in Sydney, New South Wales. She said to get to her now. It was a three-and-a-half-hour drive on a good day, six, if the highway was blocked. She herself, this professor, saw his GP and got a referral to herself. We got to her in October. She said, “If he keeps going like this, he won't open a Christmas present.”

She put him on Viagra. The cost was a horrendous. It was over $300 a day, Just because my husband is a physician, (he works for the health department), so the money was not up there with what she was getting. My husband didn't get paid per patient or anything. He just got a wage.

The headaches nearly killed Tim. He really struggled with the headaches. It got to a point where she said, “He's going to die within a couple of years if we don't do something else. Have you got more money? If you can give me $600,000 a year, I might be able to keep him alive for transplant.” Well, no, we didn't have $600,000 a year.

We bought medications from America at our cost to keep him alive because time is hope. The more time you have with pulmonary hypertension, the more hope you have because you think something's going to come in. Surely there's going to be access to medication. Surely somebody's going to do something. So we found a surgeon that worked under Jameson in San Diego who did the PTEs back then (Pulmonary Thromboendarterectomy). Now they're called PEAs. I wish they wouldn't stop changing all the terminology. It makes it confusing for people going back, looking at stories such as this. We went to several surgeons, and they said, "No, it's too risky. He only had a 40% chance or 30% chance of coming through the surgery."

He as an adult, took on this surgeon and they did the PTE. It was a very long day. I think he was in surgery for 14-hours or something like that. I just sat there and stared at a spot on the wall for that amount of time. I was too scared to even go to the toilet in case I missed something. I'll never forget that day as long as I live. He made it through. However, when the surgeon finally came out, he said we could not get it all. It kept breaking off, so all his capillaries and arteries and things were not cleared. He said, “That is going to calcify and build up again, so we'll see you on transplant.” So that was the end of that.

Then he started on oxygen, and we bought an oxygen concentrator for home. We had cylinders to travel with. We got to that point and then we were still paying a fortune for all these medications. Our specialist was writing letters to pharmaceutical companies to try and get some medications on compassionate grounds, as Tim would do trials. He was on everything. There was nothing in the world he wasn't trialed on. If you go on a trial, you are able to stay on the medication if the trial's successful at no cost of your own. So he went on any and every trial he could. The doctors were really keen. There were only six people [in Australia] that had that PTE surgery. He was the sixth. They were really keen to get him on as many trials as they could, because he was living, and the other people had died.

It's 2002 by then, so this is three years after his diagnosis when they said he wouldn't know, but a Christmas present. We just kept fighting and couldn't believe what we were in and couldn't understand it. My husband being a physician, had heard of pulmonary hypertension, but again, this word “aware.” He wasn't aware of it. He didn't know anything about it. So he was researching and looking up studies and things that the average normal Joe couldn't actually get access to.

We just kept continuing to buy medications or get onto trials where he had a chance of surviving. In the meantime, there were some horrific moments and scary moments, and I won't go into all of those. Anyone that's a carer would know those… chest pain, arm pain, “is this it,” falling over, passing out.

He also worked full time. His pressures were about 130. He was an IT manager. So a lot of doctors we went to before accused him of being lazy wanting him to get out and walk. “You sit on your backside all day and you’re just unfit”. We had to sort of jump those hurdles a lot with a lot of people, including people that were in cardiac rehab and things like that. Staff members and so forth didn't understand the importance of this disease and how it was going to affect his life.

He did get married to his girlfriend. He did buy a house. We did everything we could to fit normal life in, because we knew it was going to run out. His wedding was in 2002. He could just do a little slow dance and that was about it. He was on oxygen. He died in 2006. We did pretty well to keep him alive for seven years after we were told he only had three months.

The thing to do is you really have to advocate for yourself and not look at all medical practitioners as them knowing everything. His biggest thing to keep himself alive was to get to this PH Center and this PH specialist, not just a cardiologist or a respiratory physician or a GP, his general practitioner. He had to get to the top. My biggest goal in PHA Australia is to fast-track people to the right people and get them under somebody's umbrella that actually knows what pulmonary hypertension is.

Tim died and we had what I call the death honeymoon. After that, we sort of went along on a bit of an adventure and moved houses. My husband, Richard changed jobs. My youngest son, who was a radiographer at the time, changed jobs. He's a dentist now because he couldn't stand x-raying lungs. So our whole world was tipped upside down completely. But I wasn't depressed, and I never cried at all. When Tim and I were talking about him dying and planning his funeral and all that sort of stuff, I never shed a tear. It was my business hat that was on.  You know they're watching themselves die, and that's really hard to deal with. He was very brave and very strong, and if he could be that and it was him dying, then I had to step up and my husband had to step up and his brother had to step up. We were a very tight little unit.

In the end, his death was very different to most deaths for PH, because at 4:00 in the morning we all woke up. We were in an apartment in Sydney, which also cost a fortune like 300 or $400 a night. We all woke up at 4:00 and were sitting out in the lounge room and I said, "I'm going to text Tim. Something's going on here." I texted him in the hospital and he said, "Can you come in and watch the sunrise with me, because today's the day I'm going to die." I said, "Well, you don't know what day you are going to die." He said, "Yes, I do because you've run out of money. You've lost everything. We haven't got anything left and I'm going to withdraw my treatment at 7:00 in the morning. I know how many nanograms per kilogram I weigh that I'm getting. I envisage I will die between 10:00 AM and 10:30."

So we went in and we watched the sunrise. I never cried. I can cry now about it. He died at 10:22 after he spoke to all of us individually about what we meant to him and his life. That was the bravest thing I've ever seen anyone do. Like press the button. Pull the lines out. He was actually had sepsis in his Hickman's. They'd taken him off the transplant list. We were on and off the transplant list for on note about a year. They weren't keen to transplant PHers. They were more keen to transplant CFers Also, he needed a heart lung transplant by then. They decided that it's better to give two people a chance than one. So the decision was made that he was not to be transplanted and he would die over the next few weeks. He decided to just cut the pain short and get it over and done with. That's what he did.

I will never be the person I was before we went through this journey of PH - let alone losing the war. We called it the PH War, because we were always fighting with somebody about something to get something to try something, to do something to fundraise, to get more help to pay mortgages and bills and things like that. Afterwards, when he did die, I had my business hat still on. We had a funeral to do. We had 800 people at the funeral. We had just so much to organize and work out. Plus, he now had a wife, which we had to deal with. She called the shots. When your children are adults and they have a partner, it becomes more complicated.

The death honeymoon I call it now. I was probably on it for about five years. My husband was more realistic being a physician. My youngest son and now only son really went down pretty bad with depression. We had all these aftereffects. It’s like a tsunami came and hit us and then you've got to deal with the outcome of this tsunami of death. I actually went back to university to learn IT and freshen up on my grammar and math and everything to help run PHA Australia. So I was living the dream. I was going back to university with 17-year-olds. I was sort of on this permanent holiday for a while until things started to hit us that he wasn't on a holiday. He wasn't overseas. He was not coming back.

Then it hit me at about the five or six-year mark that this is really happened. It's not some fantasy show I've watched on TV. I haven't seen Tim for a long time now. Now it's too long. When it gets to longer and longer and longer, I'm finding it's getting harder and harder and harder because you just want one hour with them or something to make sure they're okay and you can't get it. You could make excuses for not seeing them for a few years. He could be a boarding school or anything. You make up things all the time subconsciously, Actually, you don't know you're doing it, but you are because you have to save yourself from having a huge collapse and going over everything over and over and over in your head and going mad.

Our brains have the ability to change the way we think about this very close death. Grandparents dying is different because you expect it. Even your parents dying, even if they're younger, you sort of expect it that you'll see them out. When it's your child, it doesn't compute like your brain just closes shop on that and lets you recover. It just depends on the age. The relationship you had with that child and how long the battle was that you fought to keep them alive.

We migrated north. I continued with my education and when that finished, I went thump. I didn't have anything to get out of bed for anymore. I hit a huge depression. The only thing I could do was make sure that nobody, no other family went through what we did. Tim made me promise the day he died, "Mum, keep going,” because we had a Yahoo page. “Keep going.” It was before Facebook. “Don't give up. Don't let anyone else go through this. Mom promise me." I've kept my promise. I found about a hundred other people in the end. I found a static page from another mother, bereaved mother that Actelion at the time had put up for advertising in memory of Sarah. Sarah died 48 hours after childbirth and was diagnosed with PH on autopsy.

So people say, "Oh, well you fat, you need to lose weight. I was tired when I was pregnant. I was tired when I had little kids. You need to get out and exercise more. You're not doing enough.” There's every excuse in the book. So rather than ruling PH, I would rather see it ruled out as a priority rather than wait two and a half years or whatever before someone goes, "No, this isn't just laziness, this person's really sick." It's a simple ultrasound to get an echocardiogram, right? So let's rule PH out. If someone's really flailing and struggling like super tired or getting niggly chest pains, or maybe they have one limb that swells up, which would indicate they had had a clot… let's rule it out rather than wait two and a half years for it to be ruled in.

By the time that comes around, it's a much harder fight to stay alive. Then they've got to work out your medicine combination, as well. That again is individual. Some things work for some people, some things don't. You're a walking test tube basically once you are diagnosed. It's a bit of this, a bit of that. We'll try this, we'll try that. You just hope that what they've done is going to at least give you some length of time on this earth like the pumps have now and things like that that weren't around when Tim was diagnosed. They weren't here, they were there. I think they were in Europe, but we didn't have them.

It's just a matter of fast tracking people. Also, Australians have this affinity to be loyal to their local doctor, right? Being a sparse country with the size of America, with the population a bit over Los Angeles. We are scattered everywhere. For me now to go to a PH Center, it's 3000 kilometers. If you're on oxygen, you have to have an altitude test and get signed off by the airline company. It's a big deal to fly. So some people take three or four days to drive to their specialist. I encourage people to do that. I know it's hard and I know it's expensive and I know it's just an absolute pain, but you've got to try and keep someone alive, so you do what you have to do to do that.

In closing, the thing that got Tim in the end were two main things. Fluid and sepsis from his line. When you get sepsis, you don't have to have a red spot on the outside of the arm. I notice on a lot of posts everyone says, "Do you think I'm infected?" But you can be infected from the inside out and not have a red spot. The other thing is fluid.

People should ask their doctors how much fluid they're allowed a day. It's easy in Australia because we are imperial. So a 1000 mil, which is a liter, and most people start on two liters a day fluid and might go down to one and a half liters. One-liter weighs one kilo, right? So you go and buy very good scales down to the 100 grams. I don't know how many ounces that is, but people can convert that. What he put in and what he ate and drank during the day and what he put out in a bottle was all measured and he should have been that exact weight.

Now, people think if they're eating, they're not having fluid. What happens if you put an apple through a juicer? You get fluid. You know what happens if you have a casserole or a bowl of soup? It's fluid. Fluid does not mean water. It means anything you put in your body that's wet. Tim was cheating because his body was telling him he was dehydrated and he had a great big tummy on him. He had to have tummy taps. In one tummy tap, they took 13 kilos. That was 13 liters off his stomach.

He'd been cheating, because the doctor told me that in some of the patient's room they have to take their handles off the taps. Patients get so desperate and dry, their bodies believe they're dehydrated when they're not. It's just gone into all the wrong places. He was cheating by drinking in the shower. So we'd do this measurement thing and he'd be two kilos heavier the next day and you can't put on two kilos of fat overnight, especially when you're on a low fat diet. Like you just can't. He was that desperate to drink.

The doctor even told us that people drink the flower water out when they're given flowers. They drink the water in the bottom of the vase. It's a desperate thing. There's an aboriginal trick in Australia when they're traveling across the bush. They put a pebble in their mouth and it makes saliva. I don't know if you've got Tic Tacs over there or up there. We used to pop one of those in his mouth to get the saliva going so his mouth at least was wet. If we did give him fluid in the form of food, I froze it like frozen grapes, frozen bananas.

Fluid overload will kill you. He went into renal failure that was part of his demise. He did help himself die in the end anyway by cheating on the fluid. But it's a question that you need to ask. Am I on fluid restrictions? How much can I have a day? Get your scales and measure your input and measure your output. Get on those scales. They should be the same as they were yesterday, if you're doing the right thing.

My name's Annie Whitaker. If you want to find me on Facebook on Annie Boxsell Whitaker. Thank you for listening by the way, because it's a hard story to tell. I'm aware that my son Tim was rare.

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