Monday, May 31, 2010

what are the complications of HSP??

Henoch-Schonlein purpura, also known as anaphylactoid purpura, is a disease characterized by an inflammation of the body's smallest blood vessels throughout the body. While the cause is unknown, it seems to be triggered by a recent respiratory infection, either viral or streptococcal. Children ages two to ten (particularly boys) are most commonly affected, although the condition can develop in adults as well. The older the child (or adult), the more likely the disease is to be serious.
The disease may begin suddenly, with rash, abdominal pain, and other signs, or it may develop more slowly over a period of weeks. Henoch-Schonlein purpura is characterized by:
·A rash, which is always present, begins with small hives, or red patches, which appear anywhere on the body, but especially on the legs and buttocks. This rash represents swollen blood vessels, and the spots blanch with pressure, since pressure moves the blood along the vessel. The allergic nature of the rash often makes it itch. As time passes, blood leaks from the swollen vessels, the rash changes from red to a bruised, purple color (hence the name purpura), and the rash no longer blanches when pressed. As the tiny bruises heal they turn to a rust color, and then fade. Each spot lasts for about five days. Often the rash comes in several crops, and a single child may have a rash of a variety of different colors. The skin rash is the most obvious, and most common, finding in Henoch-Schonlein purpura - but by no means the most serious. Blood vessels in other parts of the body can also be involved --most frequently in the joints, the intestines, and the kidneys.
 ·Joint symptoms (arthritis) occur in two thirds of the children. The knees and ankles, particularly, often become swollen, tender, and painful with movement. This arthritis can be quite debilitating, but usually resolves in just a few days. No permanent deformity results, even with the most severe arthritis.

key points:
  • Henoch-Schonlein purpura is a short term inflammation of certain blood vessels thought to be triggered by a malfunction of the immune system.
  • Symptoms include a purple spotted skin rash, abdominal pain swollen painful joints, and gastrointestinal upsets such as diarrhea.
  • Children (particularly boys) are most commonly affected, although the condition can develop in adults too.
  • In most cases, the symptoms disappear by themselves within one to four weeks.

  • ·Gastrointestinal symptoms occur because of inflammation of the blood vessels of the GI tract. Most of these children experience abdominal pain, often quite severe. They will often vomit -- sometimes vomiting blood. More than half of the children with Henoch-Scholein  will have bloody stools. Serious short-term complications most often come from the GI involvement. of pain and gastrointestinal bleeding, with possible complications of bowel rupture or intussusception
  • ·Kidney disease occurs in up to half of affected children, usually manifest by blood and protein in the urine. While more severe kidney involvement can occur and may even become more or less permanent, most children with kidney involvement recover fully. A very few do not clear up fully and may go on later to develop more serious chronic kidney disease.
·Angioedema - massive transient swelling of the scalp, eyelids, lips, ears, backs of the hands and feet, back, scrotum, and perineum is common and may be striking, especially in young childrenMost children recover fully. The disease may be quite mild and last only 2 or 3 days. For those with moderate to severe symptoms, though, the disease lasts an average of 4 to 6 weeks, with relapses occurring up to a year later (particularly after the child gets another cold or is re-exposed to the offending agent).
Rarely, children die from complications experienced near the time of diagnosis (bowel perforation, hemorrhage, seizure, stroke, etc.). Having made it through the acute crisis, the long-term outcome depends on the extent of kidney involvement. With no kidney involvement, full recovery is the rule. One-fourth of the children with kidney problems will still have detectable problems years later. 
There is no specific treatment for HSP. If the initiating trigger is identified, everything possible should be done to remove it. If the cause is thought to be a bacterial infection, such as Strep throat, prophylactic antibiotics are often given once the infection is eliminated, to prevent recurrence. Anti-inflammatory drugs, and sometimes immunosuppresive drugs, are used to provide much-needed symptomatic relief. Steroids may cause dramatic reversal of GI or brain involvement, but have not been shown to be very effective for the kidneys.

HSP is a very serious disease. Thankfully, it is also very uncommon.

Saturday, May 29, 2010

Our HSP Story (part two)

Over the next couple of days Nathaniel's rash continued to spread over his feet, ankles, legs, bottom and arms. He ran a fever, complained of constant pain and could not mange to bear any weight on his legs.  He was taking antibiotics for the Strep throat and Tylenol for the pain.

By Wednesday March 30th (just over one week after he first started feeling poorly) he became very nauseous and started vomiting.  I like to believe I am from the school of parenting that is Non-Panicking but when it became more blood than vomit I called the doctor.

When the Dr. office called us back - it was not reassuring.  'Take him to Dorenbecher - to the emergency room.  We are calling ahead so they know he is coming.'  Oh dear!  So we loaded up the boy, his Lambie and his 'bowl'  and headed into Portland.  Here is what he looked like on arrival:

They started an IV and pumped him up with anti nauseates to stop the vomiting.  The ER doctor decided to call in a consult from the Paediatric ward, who suggested that he must have had a stomach bleed (due to the purpura in his stomach)  and should be kept for observation.  So Thaniel was admitted and moved to the childrens ward.

Due to the bleed in his stomach he was not allowed any food or drink and they started him on 40mg of Prednisone to stop the bleeding.  After a battery of blood work and testing we settled in for the night.  By morning Nathaniel's body was covered in bruises and his joints had swollen enormously.  Here are some pics of the swelling:

 Yep - thats MY thumb and Nathaniel's hand!!  And here is what his face looked like - just from sleeping on that side of his body!!  Poor baby,

Friday, May 28, 2010

HSP overview:

What is Henoch-Schönlein purpura (HSP)?
Henoch-Schönlein purpura (say: hen-awk shern-line purr-purr-ah) causes blood vessels to get inflamed (irritated and swollen). This inflammation is called vasculitis. It usually affects the small blood vessels in the skin (capillaries). It can also affect blood vessels in the bowel and the kidneys. When the blood vessels get inflamed, they can bleed into the skin, causing a rash that is called purpura. The intestines and the kidneys may also bleed.
What causes HSP?
HSP is caused when a person's immune system doesn't fight an infection like it's supposed to. It occurs most often in the spring, usually after an upper respiratory infection, like a cold. HSP occurs most often in children from two to 11 years of age, but it can occur in anyone. Its exact cause is unknown. It might be triggered by bacterial or viral infections, medicines, insect bites, vaccinations or exposure to chemicals or cold weather. You may catch an infection that caused someone's immune system to respond with HSP, but HSP itself isn't contagious. Doctors don't know how to prevent HSP yet.
What are the symptoms of HSP?
HSP causes a skin rash, pain in the joints (such as the knees and ankles) and stomach pain. The rash looks like small bruises or small reddish-purple spots. It's usually on the buttocks, around the elbows and on the legs. HSP can also cause fever, nausea, vomiting and diarrhea. The symptoms usually last for about four to six weeks. If the blood vessels in the bowel and the kidneys get inflamed, HSP can make you bleed when you have a bowel movement or when you urinate. Serious kidney problems don't happen very often, but they can occur. In rare cases, an abnormal folding of the bowel called intussusception (say: in-tuh-suh-sep-shun) can occur. This makes a blockage in your intestines that may need surgery.
How is HSP treated?
There is no specific treatment for HSP. Medicines can help you feel better and treat an infection that may have triggered HSP. Fortunately, HSP usually gets better without any treatment. Anti-inflammatory medicines, such as ibuprofen (brand name: Advil) or naproxen (brand name: Alleve), can help the pain in your joints. Sometimes medicines like prednisone can help people with severe stomach pain.
What happens to people with HSP?
Most people do fine. Usually, HSP gets better on its own and doesn't cause lasting problems. About half of people who had HSP once will get it again. A few people will have kidney damage because of HSP. Your doctor may want to check urine samples several times after your HSP goes away to check for kidney problems. Be sure to see your doctor as many times as he or she tells you to.

Our HSP Story (part one)

Monday March 22nd - Nathaniel woke up with a fever and sore throat.  I let him stay home from school for three days.  By Thursday he was feeling better but that night Eric noticed what he thought were bug bites around Nathaniel's feet and ankles.

We were headed to the beach for a couple days and began to notice that what we thought were bug bites - were actually spreading up Nathaniel's leg and growing into dark purplish red splotches.  We sent an email to our doctor because I was worried that it may be somekind of secondary infection from the bug bites.

By Saturday morning (the 27th) Nathaniel was having a very hard time walking and was complaining of pain in his feet, ankles knees and hips.  By the end of the day he was crawling across the floor instead of walking.  We decided to cut our trip short and get him back home and straight to the doctors office.

The Pediatrician on staff ran a rapid strep test which can back positive and she recognised the rash right away.  She explained that it was not bug bites at all but HSP.   She sent us home and told us to keep a close watch on him over the next week or so.  Here is what some of his rash looked like:

Wednesday, May 26, 2010

Weird but yummy!

Easy Stir Fry Peppers

2-3 cups sliced (any combination) green, red, yellow or orange sweet peppers
1 teas olive oil
1 teas minced garlic
2-3 teas (or to taste) Worcestershire sauce
2 tbs Ketchup (yep!)

Heat over med-high until peppers are tender crisp (about 5mins) I love this as a side dish.