Why being told “ your son is dying, give up” turned out to be the best words possible

Being told “your son is going to die, make the most of what time you have left” is every parent’s worst nightmare but for me, this was a reality.

My little Benjamin was just two years old when doctors diagnosed him with Hurler Syndrome (MPS 1), a progressive genetic disorder that affects just 1 in every 100,000 births.

For those of you who aren’t familiar with the disorder, it’s a progressive illness which creates a build-up of sugar in the body, slowly attacking the major organs.

The memory of that moment I was told about his illness stays with me as if it was yesterday.

It felt like my heart had stopped beating, everything was going in slow motion and I remember saying ‘this must be a misunderstanding. There were so many questions running through my mind - he has what? How did it happen?what should we do? How would he die?

I was told the illness would claim his life in 10 years if we were lucky, or five if we weren’t, which meant I would lose my little boy before he even became a teenager.

That beautiful day on September 29th in 1996 would forever haunt me.

On that day, the words which hurt me the most were being told to “make the most of what is now, then move on afterwards. Quit your job to stay home with him.” I used to hear them whispering to me at night when I tried to sleep. I was so angry - who were the doctors to tell us what to do? 

It was as if everyone had given up hope on my son. Well, not me. These words would turn out to be the key to Benjamin’s life and is one of the reasons why 25 years today, he is still with us and is possibly one of the happiest 25-year-olds I know.

Never let other people’s limiting beliefs become your beliefs

We must all take responsibility for our own actions or lack of action. Sure, we should expect the experts to tell us what to do, but in my experience we know that is not always the case. I wasn’t going to rest at the news of my son’s impending death. I had a choice to either let it paralyze me or use it as a strength to make a difference in this world.

I had done this once before, when we were told Benjamin would die at six months from the bacteria Clostridium. I introduced the solution of using feces in a saltwater mix, to cure him, a treatment later used around the world. 

In January 1997, I contacted Dr Emil Kakkis at UCLA after I came across an article written by him. They were close to getting started on an enzyme replacement therapy on humans, which was music to our ears - somebody was actually working on a treatment for my little boy.

Because the pediatricians at the hospital noticed we took an active role in Benjamin’s health, and contributed constructively, they listened to us. I was eager to talk to them about this therapy treatment but I’m not sure if they believed it was possible. They just repeated the message “there is nothing to do, move on”.

I still find it shocking how professionals preach their limiting beliefs: What could possibly be more important to us than this? How could they know that we should give up? There were no medical professionals that could stop me believing that I could make a difference. Under no circumstances would I allow their limiting beliefs become my own beliefs. 

In 1999 Dr Kakkis and his group were looking for children to participate in a study. It was time to move the trial from mice to humans. Sadly, even though Benjamin was doing very well for a five-year-old he had the most severe form of MPS 1 so we were told he would not receive treatment. It was hard news to receive.

However, this was a step in the right direction for us and for other children. I spoke about this life-changing treatment to everyone I believed could make a difference.

Looking back, I realise that none of the doctors I spoke to believed in the treatment, yet alone mention that this study that was going on to other families and patients. Perhaps fear held them back, fear of raising families and patients expectations for a treatment that might arrive too late for them. I believe they should have told parents about the study being conducted. It would have given a glimmer of hope to stricken families who were being told there was nothing more to do. It would give them a feeling that there are people that really cares, and want to help.

Death and Life

As time went by, Benjamin’s health was deteriorating. In 2001, when he was six-and-a-half, he was hospitalised due to respiratory problems. He was given oxygen through a mask to help his oxygen levels but, because he couldn’t exhale, it caused a build-up of CO2 and he slipped into a coma.

It was terrible to watch. As each anaesthetist tried and failed to get a tube down his throat, I remembered admitting defeat and saying: “You had a good life Benjamin, and I am glad you could go to sleep now. We have been so blessed to have you with us.” Then I heard the words, “There is nothing more we can do”.

The door suddenly flew wide open, another anaesthetist,who was about to visit Benjamin heard something was wrong. He threw himself around and started to work the tube again, pushing it down, maneuvering it through the tight trachea. It was all in slow motion, I can’t recall hearing any sound, like a silent movie. I was exhausted and in shock. Then finally I heard the words “Rita, he has a chance.”

A second chance!

It made me realise that all the work I did to make sure they knew about enzyme replacement therapy might not benefit Benjamin, but it could still help other children and this gave me more strength to carry on. I knew I was given an opportunity to make a difference to others, and I chose to carry on.

The hope we had and the knowledge that somebody was working on a drug for Benjamin and others with MPS 1 really was of great help in the years from when he was diagnosed up until now. I realised that Benjamin might not live to see the day of Aldurazyme, the name given to the drug, but the hope made me feel alive. I understood that life was to be loved and lived each day to the fullest.

In 2002, I remember attending our first MPS get-together in Norway and I was so optimistic as I knew it was just a matter of time before the drug would be released. How disappointed I was when I understood that hardly any of the MPS families knew what was about to happen.

Why did they not know? Some had seen the specialists I talked to about the treatment many times. One mother was furious as to why she had never been told. She did not believe there would be anything to do for her child but just knowing that something was being done would have helped her emotionally. Parents going through such turmoil shouldn’t be expected to do their own research, why can’t doctors talk to them about possible future treatments?

Hope finally arrives, or does it?

In the spring of 2003 we finally got the word that Aldurazyme had been approved by the Food and Drug Administration and was to be released on the European market shortly. This was exactly what we had hoped for. 

Benjamin, then nine, had to start treatment before he was 10 so we sent our application immediately, convinced we would be rewarded for the hard work informing the right people about this treatment.

Sadly, our application was turned down six months later - because it was too expensive and nobody could afford to pay for it. So we include prisoners in the national budget and are willing to spend even more money on each of them to keep them safely behind bars, but treating a child with a medication that will extend his or her life doesn’t count?

Opportunity comes knocking

In 1996, we were told Benjamin would only live a few more years. In 2003, he was way past that prediction, he was living on borrowed time.

When you know your purpose, fear of failure and taking risk disappears.

I was on a mission. I had been given an opportunity to make a difference and I would not let anyone stop me.

We didn’t have time to send letters and become entangled in bureaucracy, but a story on one of Norway’s largest TV channels in March 2004 forced the Ministry of Health to review the case, which stunningly took only two hours before they instructed the local hospital to treat Benjamin.

One month later, he got his first injection and the physical improvements were overwhelming. Every week for five years he got the enzyme replacement therapy at the hospital, now he receives it at home.

His weekly presence at the hospital also changed many lives of the people working there by giving them a feeling of making a difference. This is important for Benjamin, and for us, as it promotes being positive and deep listening. 

We experienced that as individuals we can have an enormous impact and influence. We saw doctors increasingly listen on a different level, have meaningful and respectful conversations with their colleagues, the nurses, the parents and patients and it has changed us as parents. 

We truly understand the value of being part of a team, we all need each other. We have also learned the value of good communication and trust. By exercising those two skills there is no room for misunderstandings as everybody involved has one interest, and that is to make Benjamin’s life better. As a result of this other families and patience get to experience the same. 

The reality

Has it since been a walk in the park? Not at all. We have since been faced with making the decision to end Benjamin’s life after three months in coma, just to experience once again his life being rescued in the 12th hour through the connection I had built up around the world, this time the rescue came from Royal Manchester Childrens Hospital. 

We spent five years fighting the government’s decision to send Benjamin’s friend and assistant  Maria back to her home country, and finally won the case after much time in court. 

He stopped speaking when he was nine-and-a-half years old, so it is like having a toddler in the house for 23 years. He needs a wheelchair and can be awake for 48 consecutive hours. What I feel deeply, that I know to be true, is that he is possibly the happiest 25-year-old there is, because his happiness has been the highest priority. He also brings joy to everyone in his life.

Now what? 

As parents we have decided it is not about life expectancy but quality of life. This is a decision we often bring up when Benjamin is acutely sick and is suffering. He must live his life for himself, not for us. 

I look back on that day in 1996 and I am grateful for the words “there is nothing to do but to make the most of what is now. And then move on afterwards” because they made me realise that we had to fight for hope, take personal responsibility, be leaders and make decisions regardless of how hard it would be to be judged for doing all the opposites of what the ”experts” would advise. The words were brutal, but they massively triggered my drive to prove them wrong.

Changing the perception from “why is this happening to us” to “why is this happening for us” made the big difference in the fight and kept Benjamin alive long enough to see the day that we saw hope when he started his treatment.

What have I learned?

Benjamin is the oldest known living person with Hurler Syndrome, today he turns 25. Each day is a gift. He has taught me the most important lessons in life, one of them to listen to my gut, another to always challenge the status quo and, importantly, never second guess my own impact to influence outcome and find solutions, and last but not least the importance of asking and receiving help.

I know from experience that taking risks, speaking up and sharing ideas, regardless of how crazy they sound, save lives and is crucial in order to create sustainable solutions. 

Benjamin and his diagnosis is the reason why I help women to create more influence, confidently communicate their ideas wherever needed, regardless how crazy they might sound.

In each person I meet, I see an untapped reserve that sits with a solution we need access to.

We all have it, men and women ( parents or not) that drive, that fire within us, that when fueled with passion, can create impact and transformation, beyond our wildest dreams. 

We are all leaders in our own lives, and it begs the question; What are you waiting for to happen to light your fire and create extraordinary results? Every day challenge is an opportunity to grow.

Today my son Benjamin is 25 years old, his life is an extraordinary result from turning the impossible dream into reality, from changing the perception “why is this happening to us” to “why is this happening for us” and make a difference.

Rita Hausken is an international speaker, leadership strategist and coach for women in male-dominated industries. You can learn more about her work at her website, www.ritahausken.com.