Iron Deficiency Anaemia and PKU patients
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Iron Deficiency Anaemia and PKU patients

Iron Deficiency Anaemia and PKU patients

Iron Deficiency Anaemia can be a common problem in PKU patients who either do not take their PKU formula or struggle to take their supplements consistently. Iron is used to produce red blood cells, which help store and carry oxygen in the blood. If you have fewer red blood cells than is normal, your organs and tissues won’t get as much oxygen as they usually would. Iron is available in two different forms: heme and nonheme. Heme iron comes from animal-based foods, including dairy products, meat, poultry and fish. nonheme is the only type of iron you’ll get from plant-based foods.
Iron deficiency anaemia is the most common type of anaemia, others can be caused by a lack of vitamin B12 or folate in the body (previously discussed).

Many people with iron deficiency anaemia only have a few symptoms. The severity of the symptoms largely depends on how quickly anaemia develops. You may notice symptoms immediately, or they may develop gradually if your anaemia is caused by a long-term problem, such as a stomach ulcer.

The most common symptoms include:
– tiredness and lack of energy (lethargy)
– shortness of breath
– noticeable heartbeats (heart palpitations)
– a pale complexion

Less common symptoms include:
– headache
– hearing sounds that come from inside the body, rather than from an outside source (tinnitus)
– an altered sense of taste
– feeling itchy
– a sore or abnormally smooth tongue
– hair loss
– a desire to eat non-food items, such as ice or paper (pica)
– difficulty swallowing (dysphagia)
– painful open sores (ulcers) on the corners of your mouth
– spoon-shaped nails

Many dietary sources of Iron are not allowed or restricted in the PKU diet, so taking your formula as prescribed is essential Good dietary sources of iron include:
– Meat, especially liver
– beans
– nuts
– dried fruit – such as dried apricots
– wholegrains – such as brown rice
– fortified breakfast cereals
– most dark-green leafy vegetables – such as watercress and curly kale

The amount of iron you need is:
8.7mg a day for men (19-64 years)
14.8mg a day for women (19-50 years)
8.7mg a day for women (50-64 years)
Women who lose a lot of blood during their monthly period are at higher risk of iron deficiency anaemia and may need to take iron supplements.

Vitamin C, also called ascorbic acid, strongly enhances the absorption of nonheme iron, but it must be consumed at the same time as the nonheme iron. When they’re digested together, vitamin C combines with nonheme iron to form a compound that’s more easily absorbed. If the iron in your meal comes from plant-based foods, you should include at least 25 milligrams of vitamin C in the same meal. You’ll get at least that much vitamin C from one serving of cauliflower, cabbage, broccoli, sweet peppers, tomatoes and Brussels sprouts, and double that amount from citrus fruits and juices.

Your dietitian can assess your iron intake and a blood test will check your levels. If levels are low, this can be treated with iron tablets prescribed by a GP and by eating iron-rich foods. All protein substitutes contain iron and vitamin C.

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PKU and Vitamin D Deficiency

PKU and Vitamin D Deficiency

For the next topic in our series about why you should always take your metabolic formula if you’re restricting your diet, we’re going to discuss Vitamin D. In PKU deficiency is common, and following a restricted diet without taking your PKU supplement increases your chances of developing this deficiency.

Vitamin D regulates calcium absorption and homeostasis. That means it’s super important for bone health! It promotes calcium absorption from the gut, enables mineralisation of newly formed osteoid tissue in bone and plays an important role in muscle function (NOS 2013). Over the last decade, evidence has suggested that low serum concentrations of 25-hydroxyvitamin D, 25(OH)D, are associated with a number of non-skeletal disorders including cancer, heart disease, high blood pressure, diabetes, age-related cognitive decline, Parkinson’s disease, multiple sclerosis and arthritis. However, whether low 25(OH)D is the cause or result of ill health is not yet clear (Autier et al. 2014).

UVB sunlight exposure is the main source of Vitamin D, however, synthesis of vitamin D varies during the year with peak levels recorded following the summer months. In the U.K., sunlight is not strong enough to trigger synthesis of vitamin D in the skin from October to March. Therefore, we rely on dietary vitamin D and body stores to maintain a healthy vitamin D status, particularly during the winter.

The UK National Diet and Nutrition Survey 2008–2012 reported approximately 40% of males and females age 11-64 years have Vitamin D levels below the recommended level between January & March and 25% year round. Vitamin D status in post-menopausal women is of interest because of the association of low vitamin D status with increased risk of osteoporosis and fracture.

Treatment is either with capsules (adults) or liquid for children, in severe cases an intramuscular injection may be needed.

Vitamin D deficiency can be prevented. Do you know your Vitamin D status? Ask your GP or metabolic clinic for a blood test to check your levels, and remember to be reviewed regularly at a metabolic clinic even if you’re not on diet.

References:
Spiro, A, and J L Buttriss. “Vitamin D: An Overview of Vitamin D Status and Intake in Europe.” Nutrition Bulletin / Bnf 39.4 (2014): 322–350
NOS (National Osteoporosis Society) 2013. Vitamin D and bone health: a practical clinical guideline for patient management.
Autier P, Boniol M, Pizot C, et al. Vitamin D status and ill health: a systematic review. The Lancet. Diabetes & Endocrinology. 2014;2:76–89

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PKU and B12 Deficiency

PKU and B12 Deficiency

Did you know that following your PKU diet without taking your supplements is dangerous and can put your health at serious risk? We get it – taking your supplements can be hard, but it’s important you find one that works for you and that you can tolerate. Restricting protein while not taking your PKU formula can lead to a range of debilitating deficiencies, some of which can cause irreversible damage. Many teens and adults who struggle with formula compliance find Glytactin RTD and BetterMilk a more palatable and even enjoyable option that they are able to take consistently without experiencing a range of GI issues (stomach aches, nausea, bloating etc) commonly reported as a side effect of traditional amino acid supplements. If you’re struggling with taking your supplements then please get in touch with us for a sample of our products – we’d love to help you be the healthiest you that you can be! Simply email sarah@cambrooke.com to request samples.

B12 deficiency is common in teens and adults with PKU that restrict protein but don’t take supplements. Untreated B12 deficiency or folate deficiency anaemia may cause serious neurological disease that, if left unaddressed can become irreversible. The symptoms usually develop gradually and worsen over time if untreated.
Anaemia is where you have fewer red blood cells than normal or you have an abnormally low amount of a substance called haemoglobin in each red blood cell. General symptoms of anaemia may include:

• extreme tiredness (fatigue)
• lack of energy (lethargy)
• breathlessness
• feeling faint
• headaches
• noticeable heartbeats (palpitations)
• loss of appetite and weight loss

Symptoms of vitamin B12 deficiency
If you have anaemia caused by a vitamin B12 deficiency, you may have other symptoms in addition to those listed above, such as:
• a pale yellow tinge to your skin
• a sore and red tongue
• mouth ulcers
• pins and needles
• changes in the way that you walk and move around
• disturbed vision
• irritability
• depression
• changes in the way you think, feel and behave
• a decline in your mental abilities, such as memory, understanding and judgement.

How Your Metabolic Clinic Can Help – On or Off Diet!
Even if you’re off diet it’s super important that you attend a metabolic clinic every year for monitoring. At these visits you should have your vitamin and mineral levels checked through a simple blood test that will highlight any deficiencies that need addressing. If any deficiencies are found, your metabolic consultant and/or GP will be able to determine the best course of action to address it.

References: J Walter / Mol Genet Metab. 2011;104 Suppl:S52-4
Dr John Walter is a very experienced paediatric metabolic consultant at The Willink Unit, Manchester Children’s Hospital

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International PKU Day is almost here!

Every year on the 28th of June, the PKU community joins together to celebrate International PKU Day. It’s one of the most talked about days in the PKU community calendar, and a time where families, patients, charities and companies join together to raise awareness of Phenylketonuria.

Raising Awareness

A brief chat with different people in the PKU community will show you that International PKU Day means different things to many of us. Personally, I feel International PKU Day serves its biggest purpose to PKU families by acting as an annual encouragement to raise awareness of Phenylketonuria. Many people within the community take part in fund raising activities, share their very own personal PKU stories (all packed with emotion) and broadcast the existence of PKU to the world. This year, following on from the formation of an All Party Parliamentary Group for PKU, the NSPKU has brought several British MPs on board to do the diet for one day on 28 June. As a community we need to shout, and we need to shout loud. Our international day is an opportunity to do exactly that!

PKU is such a rare disorder, so its huge to make ourselves known to the rest of the world, not only to help raise funds for research, but also to help in a political struggle for PKU treatments to become available in the UK (which are already available elsewhere in Europe).

Remembrance to Robert Guthrie and Horst Bickel

International PKU Day also serves as a remembrance to two men who were born on 28th June. Robert Guthrie, born 28th June 1916, developed the first newborn screening test, and Horst Bickel, Born 28th June 1918, was the first to develop a low protein diet for PKU infants in 1951.

Without these amazing findings from Robert Guthrie and Horst Bickel, those with PKU would almost certainly by subject to mental retardation, with untreated newborn babies being dangerously affected by protein in foods and milk.

Anybody born with PKU genuinely owes their quality of life to Guthrie and Bickel, and their mutual birthday is certainly a fitting occasion to celebrate PKU as a successful, life changing discovery which the whole community is indescribably thankful for.

A Reason to Celebrate

With the raising awareness and remembrance of the two men who discovered a successful low protein diet for PKU, we really feel that International PKU Day gives us something to celebrate.

Some members of our tight-knit community might be celebrating by running a half marathon to raise money for PKU, others might bungee jump off a bridge or do a skydive, but getting together to enjoy the company of other people who also share the same day to day challenges is golden to those who live the PKU lifestyle.

There has never been a better time to get involved in raising awareness of PKU. Start a fundraising campaign in your community, talk to your local press, share your PKU story with the NSPKU or ask your friends, family and local MP to join the NSPKU’s Diet for a Day Challenge.

What does International PKU Day mean to you, and how are you going to get involved? Let us know in the comments!

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A PKU Return (and the benefits!): Paul McKellar

It’s too easy to be drawn in to simply describing my experiences of returning to PKU after 17 years of PKU neglect, but I’d like to do a little more than that here. I’d like to add an extra dimension to my experiences by offering further insights into the mistakes I’ve made and revealing how PKU adherence opens unlimited opportunities.

Following a childhood of being brilliantly organised and cared for by my parents, I went completely off the PKU radar by the age of 11. I hadn’t felt the impact of high phenylalanine levels until I was regularly visiting the shops, either alone, or with friends, and by this age I had been doing so for several years and rejecting any knowledge of negative impact of the high protein intake. It’s not that there wasn’t any negative impact, it’s more that I was so young I simply didn’t know what I was looking out for – and didn’t want to notice any unpleasant symptoms!

I should have been on 5 exchanges in my teenage years, but (despite the dissatisfaction of my parents) the doctors at my local hospital had relieved me of my PKU duties and insisted “there is no point in continuing with treatment if he is continuing to cheat the way he does”, and I was officially off the diet from the approximate age of 12 without returning until I was 27 years of age.

Albeit, the first few years of non-adherence was being unofficially off treatment, I spent approximately 17 years eating all the wrong things without realising the effects these foods were having on the way I was feeling, or how these foods were restricting my potential. I was eating chocolate as I made my way to school, drinking milk (on my milk round) before I even set off and had a lunch of either pizza, burger, sausage, kebab – but whatever I decided on was always served with a full portion of chips and I wasn’t taking any supplement at all.

I never realised how may eating habits affected my social habits at school, and this still has an impact on my life, not because I’m still affected in the same ways, but because people remember me from my school days. Eating a high protein diet, and going against the way my body is built, was affecting my concentration levels (and I’m sure many of you reading this will relate). So, I wasn’t responding to conversations as sharply as I could do if I was on treatment. Despite having plenty of friends, a jovial personality and a silly sense of humour, I was repeatedly walking away from conversations either not responding much at all, or not being very convincing in my response, because protein is toxic to my brain and my brain was too lazy to come up with a quick answer.

Being so young, and repeatedly finding myself in these situations, saw my confidence in social situations decline. I relied on a sense of humour but you can be viewed as only a clown if you constantly go about your ways in an overly silly manner, so I was forced to take the back seat to the sharper, more confident guys in the crowd. Now, I did have some good times back then and I had some great friends too – that’s not the issue. The issue is that I could have been one of those sharper, more confident guys. I didn’t need to seem so timid and vulnerable when I was being confronted or challenged with witty banter during my school days, but this is what a lazy, phenylalanine rich mind did – and, as for my GSCE grades, I passed one (A-C), which was English literature.

We’re not born to live in limited ways. If we were, then we’d have been born with PKU in the 1800’s (long before it’s discovery), but we’re here now. We have a choice!

This lifestyle/mindset continued until I returned to the diet at 27. I worked in some under inspiring workplaces (which needed little to no qualifications) and I had very little ambition, but this all changed when I returned to PKU. I’d been hooked on jokes for several years, and after only a few months of being back on treatment I was invited to perform stand-up comedy at a charity fundraising event. I accepted the invitation, came 3rd out of 7 acts, and went on to do a handful of comedy attempts at other local charity events and comedy clubs (something there is absolutely no chance I could ever have managed off the diet).

I would never have even considered getting on stage if I was feeling the same as I always had been, but the improvement in my thought processes didn’t only improve my performance at work at that time, it also inspired me to get on stage, which, in turn, had me realise my passion for writing. This realisation is now seeing me study an English literature degree and I’ve recently began talking at PKU events to help inspire other (struggling) PKUers and PKU families.

I’ve certainly made mistakes, and there’s no doubt I’ll make more mistakes in the future, but I’ve learned that the PKU diet allows me to express myself confidently. It allows me to understand what I want. It allows me to visualise how I can achieve, and it allows me to help other people.

We’re not born to live in limited ways. If we were, then we’d have been born with PKU in the 1800’s (long before it’s discovery), but we’re here now. We have a choice!

I’ve found my return to the PKU diet to be almost an awakening of my natural, organic alertness and confidence which was lying dormant for so many years while I was denying the reality of who I was – yet knowing the condition I was born with.

We don’t need to be restricted. We all have a life purpose!

Paul McKellar is a PKU adult and comedian. He takes Cambrooke’s Glytactin RTD and Glytactin BetterMilk formulas, and says that returning to the PKU was an awakening for his alertness and confidence.