What is 4p- and What is Dialysis

Wolf-Hirschhorn Syndrome (WHS)

Deletions of the short arm of a B group (4-5) chromosome was initially thought to be associated with the cri du chat (cat-cry) syndrome (also known as 5p-). However, patients were noted with many of the characteristics of the syndrome but without the catlike cry. In 1965 Wolf et al. and Hirschhorn et al. published reports showing that there was a syndrome with a deletion on the short arm of the fourth chromosome.

The amount of material deleted may range from about 50% of the short arm to a small break that can't be detected by normal chromosome analysis. In 10-20% of the cases the deletion can occur as a result of a translocated chromosome in the parent.

Because of this wide range of deleted material, the effect on our children varies widely. Some can walk, talk and assist in their daily care while others are not verbal and require constant care. Some are near normal height and weight while others at ages 20-30 weight only 35-50 lbs and are only 45-55 inches tall. The medical involvement also varies from near normal to severe heart and other problems. However, a common trait seems to be that our children are (for the most part) happy, loving children.

Major symptoms may include severe growth and mental retardation, microcephaly, "Greek Helmet" faces, and closure defects (cleft lip or palate, coloboma of the eye, and cardiac septal defects) and other physical disabilities .

The life expectancy for WHS children is unknown. The oldest member of our group was born in 1949, and several are in their twenties and thirties.

Mark's case being a little different, he doesn't have cardiac issue, but does suffer from kidney issues and seizures.  No two kids are alike!

What is dialysis?

Dialysis is a procedure that is performed routinely on persons who suffer from acute or chronic renal failure. The process involves removing waste substances and fluid from the blood that are normally eliminated by the kidneys. Dialysis may also be used for individuals who have been exposed to or ingested toxic substances to prevent renal failure from occurring. There are two types of dialysis that may be performed on your child: peritoneal or hemodialysis.

Peritoneal dialysis - Peritoneal dialysis is performed by surgically placing a special, soft, hollow tube into your child's lower abdomen near the navel. After the tube is placed, a special solution called dialysate is instilled into the peritoneal cavity. The peritoneal cavity is the space in the abdomen that houses the organs and is lined by two special membrane layers called the peritoneum. The dialysate is left in the abdomen for a designated period of time which will be determined by your child's physician. The dialysate fluid absorbs the waste products and toxins through the peritoneum. The fluid is then drained from the abdomen, measured, and discarded. There are two different types of peritoneal dialysis: continuous ambulatory peritoneal dialysis (CAPD) and continuous cyclic peritoneal dialysis (CCPD). CAPD does not require a machine. Exchanges, often referred to as "passes," can be done three to five times a day, during waking hours. CCPD requires the use of a special dialysis machine that can be used in the home. This type of dialysis is done automatically, even while your child is asleep.

Hemodialysis - Hemodialysis is performed in a dialysis center or hospital by trained healthcare professionals. A special type of access, called an arteriovenous (AV) fistula, is placed surgically, usually, in your child's arm. This involves joining an artery and a vein together. An external, central, intravenous (IV) catheter may also be inserted, but is less common for long term dialysis. After access has been established, your child will be connected to a large hemodialysis machine which drains your child's blood, bathes it in a special dialysate solution which removes waste substances and fluid, then returns it to your child's bloodstream. Hemodialysis is usually performed several times a week and lasts for four to five hours. Because of the length of time hemodialysis takes, it may be helpful to bring games or reading materials for your child, to occupy him/her during this procedure.

Mark is currently on Hemodialysis.

Information found on the 4p- and Childrens websites!