What Is Childhood Henoch Schonlein Purpura?

Henoch Schonlein Purpura, called HSP in short, is childhood vasculitis. It can occur at any age and peaks around 4-6 years old. It is a non-thrombocytopenic, small-vessel vasculitis that typically presents acutely. Ninety percent of cases occur under the age of 10 and are often seen in boys.

It is very little understood about the exact pathophysiology of this condition. It is a condition that involves the swelling of small blood vessels. The swollen blood vessels leak into the skin, joints, intestines, and kidneys. It is regarded as an autoimmune disorder. 

This is where the body’s immune system attacks the body’s own cells and organs. This immune response may be caused by an upper respiratory tract infection, and other immune triggers may include an allergic reaction, medicine, injury, or being out in cold weather.

Symptoms

Each child’s symptoms can vary, but common symptoms are the following:

Severe abdominal pain

Joint pain and swelling

Rashes caused by blood leaking into the skin

Swollen lower extremities

Renal involvement, including blood in urine

Severe nausea and vomiting

Bleeding in the digestive tract, which includes the mouth, oesophagus, stomach, and intestine

It can be further diagnosed by biopsy, blood and urine tests, and ultrasound.

Treatment and Management

To date, no form of therapy has been found to shorten the duration of this condition to any significant degree. Therefore, treatment remains primarily supportive in most cases. This is consistent with the understanding that it is a self-limited disease. The majority of patients recover within several weeks, whereas some patients may take several months to recover.

There is only supportive treatment for this disease, which includes the use of non-steroidal anti-inflammatory drugs for joint pain and fever. But these drugs should be avoided in the presence of gastro-intestinal or renal manifestations, as they have been shown to aggravate these symptoms.

Complication 

Around 37 per cent of the cases show renal involvement, and among them, 1 per cent result in end-stage renal disease. 66 per cent of children may experience gastro-intestinal symptoms such as abdominal pain, intestinal bleeding, and intussusception.

HSP might recur in up to 33 per cent of affected children. Recurrences appear to be more common in children 8 years of age and older at onset and in children with renal involvement.

A personal case

My younger daughter was diagnosed with HSP on February 25, 2022. My family had just arrived in the United States in November 2022. As a registered nurse in the US, I was there for six months. My daughter had never been admitted to a hospital in Nepal. She was a healthy kid.

In the initial stage, my daughter showed signs of knee pain and joint pain. We thought she might have gotten hurt in school while playing. My husband took her to urgent care for X-Ray. No abnormalities were found. After a few days, she started vomiting. We thought it might be due to food poisoning, which could get better after a few days. But it recurred with severe abdominal pain. We suspected appendicitis and took her to Cooks Children's Hospital.

At that time, I noticed that my daughter had a few dots on her legs, which were not that noticeable. I showed the dots to the doctor, and he said that it might be HSP, though they were not sure yet. After a blood test, urine test, and ultrasound, they were sure it was HSP. They admitted her. They gave her an NS (normal saline) drip for hydration and a mild analgesic. She was kept for 5 days for observation.

The doctors didn’t have a definitive answer for its treatment, and the prognosis of the disease was unknown. As parents, we were worried as the kid was diagnosed with some kind of auto-immune disease without definitive treatment. 

It might take several weeks, months, or even years to heal, and there is also a chance of recurrence. It might also affect her kidneys. We were terrified by the situation. She had flare-ups every 4 to 5 days. She used to have severe abdominal pain and vomiting, and her rashes started to be prominent, with big ones occurring especially in her lower extremities and her buttocks. She used to cry the whole night in pain. We gave her Tylenol for her pain four times a day according to the doctor's prescription, but it didn’t help her.

Her affected areas were swollen. We had to take her to the hospital and admit her every couple of days. We were tired and exhausted. Doctors told them that it was a severe condition of HSP, so they started with Steroids (prednisone) for several weeks. Still, she had the flare-ups, and then they started IVIG (Intravenous immunoglobulin) drips. There was still no improvement.

We had to take her back and forth to the Hospital and home. Doctors then gave her the immunosuppressant drug Cellcept (Mycophenolate) for a couple of weeks, and they told us that it was a rare case they had seen because usually most kids get healed after steroids. But in our daughter’s case, it didn’t work. They also made us aware that if Cellcept didn’t work, they were left with the last option, which would be chemotherapy. 

We felt shattered at that moment. We didn’t know what to do. I had lost hope, but when I saw my daughter’s face fighting this disease in the hope of getting cured, it gave me hope and courage.

By God's grace, my daughter slowly started to recover. Cellcept's medicine started to work. Thank God she didn’t have to go through chemo sessions. What a sigh of relief! Slowly, her symptoms subsided.

The main objective of this article is to create awareness among people. Not many people know about HSP because it is quite rare. I hope my personal experience will be useful for all. I am thankful to the doctors and nurses and the team of Cooks Children's Hospital, as well as my family and friends, who helped me, and my family overcome this difficult situation.

(The author holds a Bachelor of Science in Nursing and Masters in Chid Development and Gender Socialisation.)