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Life is complicated when you don’t have the tools you need

RAQUEL

Spain

Raquel has a vibrant personality. It is hard not to smile and be positive around her. Despite having to navigate the often difficult Spanish medical environment as a rare disease patient, she is thankful for the care she has received from her primary physician, and the relationship that they have built. She remains, however, adamant that more tools are needed to support adults with PKU.

Like most PKU patients, Raquel was seen by a paediatrician until her late twenties.

“In Galicia, we are lucky because we have a great team,” said Raquel. “But when I made the transition from the paediatric unit to the adult one, I was 27, because they don’t have enough patients to justify the existence of an adult unit. It’s not easy living with a rare metabolic disorder like PKU because it means that doctors and HCPs don’t have any experience or information with treating the disease. My doctor used to call me ‘the rare’ because his only experience was learning about it during medical school. If this is rare for a doctor, how can a person who never studied medicine really understand it?”

Now 32, Raquel does not let her condition stop her from living her life to the fullest. Her parents, her husband and her friends are her support system. Alongside her hobbies, they act as ‘alerts’, as she calls them, for when her blood phe levels are higher than usual and the symptoms begin manifesting.

“I roller-skate to check my levels. I know there’s a problem when I spend too much time learning something new or trying a technique that I already know. My mum acts as an alert to my PKU for me, too. When she sees me in a bad mood, she always asks about my Phe levels, and so does my husband. He always pays attention to how much protein I have, what I eat when we go out, and how I feel”.

When it comes to PKU symptoms, Raquel feels that the struggle to concentrate has had the biggest impact on her life. “It takes away everything. You are not the same. Your anger levels also change, even your smell changes. I used to skate in national and international competitions and you need to concentrate to coordinate with your team. Because of this you have to be alert every day. You need to know yourself, know when you are losing control and need to control your levels”.

Photo of Raquel, arms folded against a white wall.

Having learned the intricacies of PKU and the dangers that consuming too much protein can have at a young age, Raquel has been managing her condition her entire life.

This is why she thinks it is extremely important for every patient to be independent and really know themselves. Equally, she wants to see more support from the healthcare system and in particular, better communication between healthcare professionals.

“In some places like Madrid, they have two reference clinics and most patients have their primary doctor in the same place as their specialist one, they are partners, so they talk. In my case, we have access to a team and a unit who manages our diet and our blood results to make sure everything is okay. But my primary doctor is not in Santiago. If something happens, they don’t have a protocol. They have the information they just don’t have the communication”.

Looking at the future, Raquel feels uncertain, but she wants younger patients to learn about her experience.

“I think one of the things most PKU patients my age are concerned about is what will happen in the future. In my case, I am the first generation detected at birth so there isn’t any information available about a person aged 50 in this moment. It does make me feel scared, I don’t know what to expect. PKU is a serious condition and if you don’t treat it from the beginning, it can have consequences. You need to follow a serious treatment. My wish is that I will be a reference for younger generations with PKU and they will learn from my experience.”