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Nutrition for Rare Diseases

A Case Study Involving Stevens-Johnson Syndrome / Toxic Epidermal Necrolysis.

Around 7000 people in New Zealand have rare disorders, of which 50% are children. In Australia,this figure is estimated to around 1.2 million with a third being children.

Integrative nutrition therapy can potentially make a big difference in both the management of the particular disease symptoms and give sufferers better quality of life.

What Is A Rare Disease?

A rare disease is one that affects only a small amount of people in a population, according to Ministry of Health (NZ) website.

Excerpts from MoH (NZ) website says the following: There are many different kinds of rare disorder, and combined together they are relatively common. They are often genetic – they run in the family and people have them from conception. Many of these disorders have no effective treatment but health professionals need to know where to find up-to-date information and ways in which the people with these conditions can be supported.”

Actually, I am not sure about this statement. The last two clients I had had no family history and were very likely related to triggers (medicinal drug reactions) that overwhelmed their physiological system.

Why This Blog? Raising Awareness

A client recently came to me with symptoms of IBS and reproductive issues. That client had been diagnosed of Stevens-Johnson-Syndrome 20 years ago.

Stevens-Johnson-Syndrome (SJS) is a disease triggered by drugs mostly and at times by infection. The incidence of SJS 3-5 persons per million.

Management of Rare Diseases

At the acute stage, the person’s outer skin (epidermis) detaches from the body. SJS affects less than 10% of the skin. When it is more severe, it is classified as Toxic Epidermal Necrolysis. The person also develops visual ocular issues (they cannot see properly due to eye going dim or the eyelids sticking to each other. They also develop light sensitivity).

Since a majority of the research literature for this condition is based on acute management, only focuses on the skin and eye issues, it seems to be that most treatments are only focused on these two issues.

Chronic manifestations of these diseases are usually ongoing and develop in stages between flare ups and remissions.

Other Symptoms of SJS /TENS

An extensive literature review was undertaken by Saeed J et al (2016) regarding multi-organ impacts of SJS/TENS. Only 138 of the 4944 articles discussed chronic sequel of SJS/TEN (3%).

Apart from skin and eye issues, the review found six other organs that were affected long term by SJS/TENS.

Respiratory – bronchial tubes, lungs
Gastro-intestinal system – ulceration of the lining of the bowel, tightening of the oesophagus, necrosis of the mucosal layer, diarrhoea or constipation and abdominal pain.
Oral – dry mouth, decreased saliva production, leading to dental issues
Ear, Nose, Throat – ulcers and formation of fibrous bonds
Gynaecology / Urinary System – 9% of the patients had indicated their soft privates parts fusing and closing up. Frequent Candida infection and UTIs were also noted.
Kidney: difficulty to urinate. Chronic renal changes showed development of adhesions, thickened filtering areas (Bowman’s Capsule).

Gastro-intestinal involvement with SJS/TENS was also discussed by JHA et al (2019). They stated that “Common GI symptoms were diarrhea, intestinal bleeding, and severe appetite loss.”

The Need For Nutritional Support Including Supplementation

Raj E et al (2019) case study highlighted the need for adequate protein energy and micro-nutrient supplementation in acute phase as this phase is very catabolic (uses a lot of nutrients and body breaks down). E Raj emphasized this same concept to me through this email. Individualized nutrition intervention is crucial and necessary.

Another multi-centre study showed that 14% of the suffers will need long term and on going support.

In case of this particular patient, when I reviewed all the symptoms, every organ system had been affected. The patient had also developed intolerance to Sulphur and dairy, histamine, dairy and many other food types. The client’s hormonal systems also were playing up. There was also a side reaction of auto-immune disorder. For this patient, there was acute manifestation of problems and then over the years, continual flare ups and recessions with increasing multi-organ symptoms.

So Where Was the Holistic and Integrative Component?

Not mentioned at all until I met the patient. It seemed that every other specialist involved treated only a particular symptom as the patient when to them for that symptom.

So issues around the privates were tested for STI which came back negative, respiratory issues were diagnosed as “Fibroid of the Lungs” but not linked to SJS/TEN chronic symptoms, on-going pain as fibromyalgia.

This is one case where my years of working in various areas of medical field become useful.

So apart from providing guidance on all the food intolerances and allergies, I tested the patient’s hormones and provided interventions for these. I also advised on nutrition for every other organ system. Definitely extra nutritional supplementation was needed. We are slowly making progress to hopefully refreshing the client’s body system.

Advocating For Integrative Nutrition Therapy For Rare Diseases

In all my articles, I have advocated for a comprehensive management plan for medical conditions with Nutrition as being an integral part of the treatment. I do believe your body is a finely tuned so that anything out of alignment will affect other parts of the body. In the case of most diseases including rare ones, we need to see all the systems connected to each other like a spider web and then unwind one part gently to connect to other part.

Testing can help in complex conditions — read more about our fructose breath test kit.

For individuals with digestive complications caused by rare diseases, understanding the types of laxatives available in New Zealand can be crucial for symptom management.

References

Graves et al. Nutrition in Toxic Epidermal Necrolysis: A multi centre review. Nutr Clin Pract (2016 (31)6: 836 -840

Jha AK, Suchismita A, Jha RK, Raj VK. Spectrum of gastrointestinal involvement in Stevens – Johnson syndrome. World J Gastrointest Endosc 2019 February 16; 11(2):115-123

Raj E et al: Nutritional Management In Steven Johnson’s Syndrome. IP Journal of Nutrition, Metabolism and Health Science, January – December , 2019;2(1):20-22

Saeed H et al. Complications of Stevens-Johnson Syndrome beyond the eye and skin. Burns (2016):42:20-27